WorldWideScience

Sample records for tissue sarcoma patients

  1. Quality of life and patients' expectations in soft tissue sarcoma.

    Science.gov (United States)

    Jones, Robin L; Cesne, Axel Le

    2018-05-01

    Assessment of health-related quality of life (HRQoL) is essential for holistic care. Greater efforts are required to incorporate HRQoL measures into clinical trials and daily practice. Considerable HRQoL data are available for localized soft tissue sarcomas (STS), particularly in the orthopedic setting. In future, HRQoL is expected to become increasingly important in the evaluation of palliative therapy in advanced STS. A patient-centric approach is advocated for STS management. Greater awareness of STS by nonspecialist clinicians, and timely referral to specialized sarcoma reference centers, is crucial for patient welfare. The patient is central to shared decision-making during consultations and during case review in tumor boards. The management approach to STS should be collaborative, involving a multidisciplinary team, multiple centers and patient advocacy groups.

  2. Clinical practice guideline: 2006 update of recommendations for the radiotherapeutic management of patients with soft tissue sarcoma (sarcoma of the extremity, uterine sarcoma and retroperitoneal sarcoma)

    International Nuclear Information System (INIS)

    Le Pechoux, C.; Pautier, P.; Le Cesne, A.; Delannes, M.; Bui, B.N.; Blay, J.Y.; Bonichon, F.; Bonvalot, S.; Morice, P.; Chevalier-Place, A.; Taieb, S.; Coindre, J.M.; Ray-Coquard, I.; Stoeckle, E.

    2006-01-01

    Context. - The National French Federation of Comprehensive Cancer Centres (FNCLCC) initiated the update of clinical practice guideline for the management of patients with soft tissue sarcoma in collaboration with the French Sarcoma Group (GSF-GETO), specialists from French public universities, general hospitals and private clinics and with the French National Cancer Institute. This work is based on the methodology developed in the 'Standards, Options and Recommendations' (SOR) project. Objectives - To update SOR guidelines for the management of patients with oft tissue sarcoma previously validated in 1995. Methods. -The methodology is based on a literature review and critical appraisal by a multidisciplinary group of experts who define the CPGs according to the definitions of the Standards, Options and Recommendations project. Once the guidelines have been developed, they are reviewed by independent reviewers. Results. - This article presents the updated recommendations for radiotherapeutic management. The main recommendations are: 1) irradiation before or after surgical treatment is the standard for soft tissue sarcoma of the extremity and uterine sarcoma; 2) no systematic irradiation should be done in case of retroperitoneal sarcoma. (author)

  3. Soft Tissue Sarcoma

    Science.gov (United States)

    ... muscles, tendons, fat, and blood vessels. Soft tissue sarcoma is a cancer of these soft tissues. There ... have certain genetic diseases. Doctors diagnose soft tissue sarcomas with a biopsy. Treatments include surgery to remove ...

  4. Collecting and Storing Tissue, Blood, and Bone Marrow Samples From Patients With Rhabdomyosarcoma or Other Soft Tissue Sarcoma

    Science.gov (United States)

    2017-12-11

    Adult Rhabdomyosarcoma; Childhood Desmoplastic Small Round Cell Tumor; Chordoma; Desmoid Tumor; Metastatic Childhood Soft Tissue Sarcoma; Nonmetastatic Childhood Soft Tissue Sarcoma; Previously Treated Childhood Rhabdomyosarcoma; Previously Untreated Childhood Rhabdomyosarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Childhood Rhabdomyosarcoma; Recurrent Childhood Soft Tissue Sarcoma; Stage I Adult Soft Tissue Sarcoma; Stage II Adult Soft Tissue Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma

  5. Anthracycline-induced cardiotoxicity in patients with paediatric bone sarcoma and soft tissue sarcoma.

    Science.gov (United States)

    Bini, Ilaria; Asaftei, Sebastian D; Riggi, Chiara; Tirtei, Elisa; Manicone, Rosaria; Biasin, Eleonora; Basso, Maria Eleonora; Agnoletti, Gabriella; Fagioli, Franca

    2017-11-01

    Anthracycline cardiotoxicity is an important side-effect in long-term childhood cancer survivors. We evaluated the incidence of and factors associated with anthracycline cardiotoxicity in a population of patients diagnosed with bone or soft tissue sarcoma. Materials and methods We retrospectively enrolled patients diagnosed with bone or soft tissue sarcoma, from 1995 to 2011, treated with anthracycline chemotherapy at our Centre and with a follow-up echocardiography carried out ⩾3 years from cardiotoxic therapy completion. Cardiac toxicity was graded using Common Terminology Criteria for Adverse Events version 4.0. A total of 82 patients were eligible. The median age at treatment was 11.9 years (1.44-18). We evaluated the median cumulative anthracycline dose, age at treatment, sex, thoracic radiotherapy, hematopoietic stem cell transplantation, and high-dose cyclophosphamide treatment as possible risk factors for cardiotoxicity. The median cumulative anthracycline dose was 390.75 mg/m2 (80-580). Of the 82 patients, 12 (14.6%) developed cardiotoxicity with grade ⩾2 ejection fraction decline: four patients were asymptomatic and did not receive any treatment; six patients were treated with pharmacological heart failure therapy; one patient with severe cardiomyopathy underwent heart transplantation and did not need any further treatment; and one patient died while waiting for heart transplantation. The median time at cardiac toxicity, from the end of anthracycline frontline chemotherapy, was 4.2 years (0.05-9.6). Cumulative anthracycline dose ⩾300 mg/m2 (p 0.04) was the only risk factor for cardiotoxicity on statistical analyses. In our population, the cumulative incidence of cardiotoxicity is comparable to rates in the literature. This underlines the need for primary prevention and lifelong cardiac toxicity surveillance programmes in long-term childhood cancer survivors.

  6. Primary soft tissue Ewing's sarcoma of the maxillary sinus in elderly patients: presentation, management and prognosis.

    Science.gov (United States)

    Dutta, M; Ghatak, S; Biswas, G; Sen, A

    2014-06-01

    Nonosseous or soft tissue Ewing's sarcoma is a rare form of Ewing's sarcoma/primitive neuroectodermal tumour that seldom affects the head and neck region. Involvement of the nose and paranasal sinuses is extremely uncommon, with only eight of such patients being reported to date, mostly affecting adolescents and young adults. To our knowledge, this study is the first comprehensive report of primary soft tissue Ewing's sarcoma involving the paranasal sinuses in an elderly patient who successfully completed treatment. We herein discuss the pathogenesis, management and factors affecting the prognosis of this rare group of tumours involving the nose and paranasal sinuses, in relation to the available literature.

  7. Adipose tissue and muscle attenuation as novel biomarkers predicting mortality in patients with extremity sarcomas

    International Nuclear Information System (INIS)

    Veld, Joyce; Vossen, Josephina A.; Torriani, Martin; Bredella, Miriam A.; De Amorim Bernstein, Karen; Halpern, Elkan F.

    2016-01-01

    To assess CT-attenuation of abdominal adipose tissue and psoas muscle as predictors of mortality in patients with sarcomas of the extremities. Our study was IRB approved and HIPAA compliant. The study group comprised 135 patients with history of extremity sarcoma (mean age: 53 ± 17 years) who underwent whole body PET/CT. Abdominal subcutaneous adipose tissue (SAT), visceral adipose tissue (VAT), and psoas muscle attenuation (HU) was assessed on non-contrast, attenuation-correction CT. Clinical information including survival, tumour stage, sarcoma type, therapy and pre-existing comorbidities were recorded. Cox proportional hazard models were used to determine longitudinal associations between adipose tissue and muscle attenuation and mortality. There were 47 deaths over a mean follow-up period of 20 ± 17 months. Higher SAT and lower psoas attenuation were associated with increased mortality (p = 0.03 and p = 0.005, respectively), which remained significant after adjustment for age, BMI, sex, tumor stage, therapy, and comorbidities (p = 0.002 and p = 0.02, respectively). VAT attenuation was not associated with mortality. Attenuation of SAT and psoas muscle, assessed on non-contrast CT, are predictors of mortality in patients with extremity sarcomas, independent of other established prognostic factors, suggesting that adipose tissue and muscle attenuation could serve as novel biomarkers for mortality in patients with sarcomas. (orig.)

  8. Adipose tissue and muscle attenuation as novel biomarkers predicting mortality in patients with extremity sarcomas

    Energy Technology Data Exchange (ETDEWEB)

    Veld, Joyce; Vossen, Josephina A.; Torriani, Martin; Bredella, Miriam A. [Massachusetts General Hospital and Harvard Medical School, Division of Musculoskeletal Imaging and Intervention, Department of Radiology, Boston, MA (United States); De Amorim Bernstein, Karen [Massachusetts General Hospital and Harvard Medical School, Department of Radiation Oncology, Francis H Burr Proton Therapy Center, Boston, MA (United States); Halpern, Elkan F. [Massachusetts General Hospital and Harvard Medical School, Institute of Technology Assessment, Boston, MA (United States)

    2016-12-15

    To assess CT-attenuation of abdominal adipose tissue and psoas muscle as predictors of mortality in patients with sarcomas of the extremities. Our study was IRB approved and HIPAA compliant. The study group comprised 135 patients with history of extremity sarcoma (mean age: 53 ± 17 years) who underwent whole body PET/CT. Abdominal subcutaneous adipose tissue (SAT), visceral adipose tissue (VAT), and psoas muscle attenuation (HU) was assessed on non-contrast, attenuation-correction CT. Clinical information including survival, tumour stage, sarcoma type, therapy and pre-existing comorbidities were recorded. Cox proportional hazard models were used to determine longitudinal associations between adipose tissue and muscle attenuation and mortality. There were 47 deaths over a mean follow-up period of 20 ± 17 months. Higher SAT and lower psoas attenuation were associated with increased mortality (p = 0.03 and p = 0.005, respectively), which remained significant after adjustment for age, BMI, sex, tumor stage, therapy, and comorbidities (p = 0.002 and p = 0.02, respectively). VAT attenuation was not associated with mortality. Attenuation of SAT and psoas muscle, assessed on non-contrast CT, are predictors of mortality in patients with extremity sarcomas, independent of other established prognostic factors, suggesting that adipose tissue and muscle attenuation could serve as novel biomarkers for mortality in patients with sarcomas. (orig.)

  9. Exatecan in pretreated adult patients with advanced soft tissue sarcoma: results of a phase II--study of the EORTC Soft Tissue and Bone Sarcoma Group

    DEFF Research Database (Denmark)

    Reichardt, P; Nielsen, Ole Steen; Bauer, S

    2007-01-01

    No standard treatment is established for patients with advanced soft tissue sarcoma after previous chemotherapy with anthracyclines and ifosfamide, given either in combination or sequentially. Exatecan (DX-8951f) is a totally synthetic analogue of the topoisomerase I-inhibitor camptothecin, which...... was synthesised to impart increased aqueous solubility, greater tumour efficacy, and less toxicity than camptothecin itself, topotecan or irinotecan. Since some activity against soft tissue sarcomas, especially leiomyosarcomas, has been reported for topoisomerase I-inhibitors, a study with a new and more potent...... agent seemed justified. We report on a prospective multicentre phase II study of Exatecan in adult soft tissue sarcomas failing 1 or 2 lines of chemotherapy in advanced phase, performed within the STBSG of EORTC. Thirty-nine patients (16 leiomyosarcomas and 23 other histologies) were included in two...

  10. The modified Glasgow prognostic score in patients undergoing surgery for bone and soft tissue sarcoma.

    Science.gov (United States)

    Morhij, Rossel; Mahendra, Ashish; Jane, Mike; McMillan, Donald C

    2017-05-01

    The prognostic significance of markers of the systemic inflammatory response in patients with soft tissue and bone sarcomas remains unclear. Therefore, the present study aimed to compare the prognostic value of markers of the systemic inflammatory response in patients undergoing surgery for primary soft tissue and bone sarcoma. Patients who underwent resection of primary soft tissue/bone sarcoma between 2008 and 2012 and had pre-operative measurements of the systemic inflammatory response [C-reactive protein, albumin, white cell, neutrophil, lymphocyte and platelet counts, and the combination of C-reactive protein and albumin (mGPS)] were included in the study (n = 111). The majority of the patients were ≤50 years old (84%), were female (63%), had soft tissue sarcoma (62%), and had tumours >10 cm (52%), mostly of high grade (85%). The median follow-up of survivors was 50 months (range 34-78); 24 (21%) developed local recurrence, 35 (31%) developed distant metastases and 30 (30%) died of their cancer. On univariate analysis, tumour size (P sarcoma. Copyright © 2017 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.

  11. CMG2 Expression Is an Independent Prognostic Factor for Soft Tissue Sarcoma Patients.

    Science.gov (United States)

    Greither, Thomas; Wedler, Alice; Rot, Swetlana; Keßler, Jacqueline; Kehlen, Astrid; Holzhausen, Hans-Jürgen; Bache, Matthias; Würl, Peter; Taubert, Helge; Kappler, Matthias

    2017-12-07

    The capillary morphogenesis gene 2 (CMG2), also known as the anthrax toxin receptor 2 (ANTXR2), is a transmembrane protein putatively involved in extracellular matrix (ECM) adhesion and tissue remodeling. CMG2 promotes endothelial cell proliferation and exhibits angiogenic properties. Its downregulation is associated with a worsened survival of breast carcinoma patients. Aim of this study was to analyze the CMG2 mRNA and protein expression in soft tissue sarcoma and their association with patient outcome. CMG2 mRNA was measured in 121 tumor samples of soft tissue sarcoma patients using quantitative real-time PCR. CMG2 protein was evaluated in 52 tumor samples by ELISA. CMG2 mRNA was significantly correlated with the corresponding CMG2 protein expression (r s = 0.31; p = 0.027). CMG2 mRNA expression was associated with the mRNA expressions of several ECM and tissue remodeling enzymes, among them CD26 and components of the uPA system. Low CMG2 mRNA expression was correlated with a worsened patients' disease-specific survival in Kaplan-Meier analyses (mean patient survival was 25 vs. 96 months; p = 0.013), especially in high-stage tumors. A decreased CMG2 expression is a negative prognostic factor for soft tissue sarcoma patients. CMG2 may be an interesting candidate gene for the further exploration of soft tissue sarcoma genesis and progression.

  12. CMG2 Expression Is an Independent Prognostic Factor for Soft Tissue Sarcoma Patients

    Directory of Open Access Journals (Sweden)

    Thomas Greither

    2017-12-01

    Full Text Available The capillary morphogenesis gene 2 (CMG2, also known as the anthrax toxin receptor 2 (ANTXR2, is a transmembrane protein putatively involved in extracellular matrix (ECM adhesion and tissue remodeling. CMG2 promotes endothelial cell proliferation and exhibits angiogenic properties. Its downregulation is associated with a worsened survival of breast carcinoma patients. Aim of this study was to analyze the CMG2 mRNA and protein expression in soft tissue sarcoma and their association with patient outcome. CMG2 mRNA was measured in 121 tumor samples of soft tissue sarcoma patients using quantitative real-time PCR. CMG2 protein was evaluated in 52 tumor samples by ELISA. CMG2 mRNA was significantly correlated with the corresponding CMG2 protein expression (rs = 0.31; p = 0.027. CMG2 mRNA expression was associated with the mRNA expressions of several ECM and tissue remodeling enzymes, among them CD26 and components of the uPA system. Low CMG2 mRNA expression was correlated with a worsened patients’ disease-specific survival in Kaplan-Meier analyses (mean patient survival was 25 vs. 96 months; p = 0.013, especially in high-stage tumors. A decreased CMG2 expression is a negative prognostic factor for soft tissue sarcoma patients. CMG2 may be an interesting candidate gene for the further exploration of soft tissue sarcoma genesis and progression.

  13. Clinical management of soft tissue sarcomas

    International Nuclear Information System (INIS)

    Pinedo, H.M.; Verweij, J.

    1986-01-01

    This book is concerned with the clinical management of soft tissue sarcomas. Topics covered include: Radiotherapy; Pathology of soft tissue sarcomas; Surgical treatment of soft tissue sarcomas; and Chemotherapy in advanced soft tissue sarcomas

  14. PET/MRI for Preoperative Planning in Patients with Soft Tissue Sarcoma

    DEFF Research Database (Denmark)

    Loft Jakobsen, Annika; Jensen, Karl Erik; L�fgren, Johan

    2013-01-01

    Clinical positron emission tomography (PET)/magnetic resonance imaging (MRI) acquisition protocols may improve the evaluation of soft tissue sarcomas (STS) prior to surgical planning. We examined two patients with lower extremity STS using a Siemens Biograph mMR PET/MRI scanner and the glucose...

  15. Ether à go-go potassium channel expression in soft tissue sarcoma patients

    Directory of Open Access Journals (Sweden)

    Stühmer Walter

    2006-10-01

    Full Text Available Abstract Background The expression of the human Eag1 potassium channel (Kv10.1 is normally restricted to the adult brain, but it has been detected in both tumour cell lines and primary tumours. Our purpose was to determine the frequency of expression of Eag1 in soft tissue sarcoma and its potential clinical implications. Results We used specific monoclonal antibodies to determine the expression levels of Eag1 in soft tissue sarcomas from 210 patients by immunohistochemistry. Eag1 was expressed in 71% of all tumours, with frequencies ranging from 56% (liposarcoma to 82% (rhabdomyosarcoma. We detected differences in expression levels depending on the histological type, but no association was seen between expression of this protein and sex, age, grade or tumour size. Four cell lines derived from relevant sarcoma histological types (fibrosarcoma and rhabdomyosarcoma were tested for Eag1 expression by real-time RT-PCR. We found all four lines to be positive for Eag1. In these cell lines, blockage of Eag1 by RNA interference led to a decrease in proliferation. Conclusion Eag1 is aberrantly expressed in over 70% sarcomas. In sarcoma cell lines, inhibition of Eag1 expression and/or function leads to reduced proliferation. The high frequency of expression of Eag1 in primary tumours and the restriction of normal expression of the channel to the brain, suggests the application of this protein for diagnostic or therapeutic purposes.

  16. Postradiation osteogenic sarcoma of bone and soft tissues. A clinicopathologic study of 66 patients

    International Nuclear Information System (INIS)

    Huvos, A.G.; Woodard, H.Q.; Cahan, W.G.; Higinbotham, N.L.; Stewart, F.W.; Butler, A.; Bretsky, S.S.

    1985-01-01

    Sixty-six patients with well-documented osteogenic sarcomas arising in bones and soft tissues after exposure to x-rays, which represent approximately 5.5 percent of all osteogenic sarcomas registered since 1921 at this institution, were studied. These secondary sarcomas occurred in equal proportion in both sexes, with the sixth decade of life being the most common age. In 42 patients, the bone had been normal at the time of irradiation, whereas in 24, the radiation was directed against an osseous tumor or tumor-like lesion. The median latent period was 10.5 years in both groups, ranging from 3.5 to 33 years. The radiation varied from diagnostic quality to 1 MeV x-rays. The dose was variable, but none was less than 2000 rads. Postradiation osteogenic sarcomas most commonly arose in the bones of the pelvic and shoulder regions. Histologically, the sarcomas were mostly of the fibrous type (46%) and radiographically showed a destructive bone lesion with or without signs of radiation osteitis. The cumulative disease-free survival rate at 5 years was 17%, with a median survival estimate of 1 year

  17. Management and prognosis of patients with high-grade soft tissue sarcomas

    International Nuclear Information System (INIS)

    Alvegaard, T.A.

    1989-01-01

    Doxorubicin is one of the most active single agents in metastatic soft tissue sarcoma. This was the rationale for the evaluation og doxorubicin in an adjuvant situation for patients with high-grade soft tissue sarcoma in a randomized, Scandinavian, multicenter joint care program, which was carried out from January 1981 to February 1986, and included 240 patients. These patients comprised the base series for the investigations of the importance of the surgical margin and radiotherapy for local tumor control; the accuracy of malignancy grading and tumor typing; prognostic factors, including DNA content; and epidemiologic risk factors. The results showed that the use of doxorubicin as adjuvant chemotherapy did not have clinical benefit in patients with high-grade soft tissue sarcoma. The overall local tumor control was high (94 %) for radically operated on extremity-located tumors due to strict classification of surgical margins. The only risk factor for local recurrence was marginal surgery without radiotherapy with a four times higher risk than after compartmental or wide surgery. Twelve percent of the operations reported as radical were classified as marginal, demonstrating the importance of reevaluation of surgical margins, and in these patients the local recurrence rate was 37 %. The 5-year metastasis-free survival for the whole patients series was 55 %, with the extremes 79 % for the patients with no or only one risk factor and 0 % for those with four or five risk factors. The five prognostic factors found could be used for selection of high-risk patients for adjuvant chemotherapy in the future. The epidemiologic study gave limited support for an association between occupational phenoxy-acid exposure and soft tissue sarcoma development. (98 refs.)

  18. Functional and psychosocial effects of multimodality limb-sparing therapy in patients with soft tissue sarcomas

    International Nuclear Information System (INIS)

    Chang, A.E.; Steinberg, S.M.; Culnane, M.

    1989-01-01

    We have documented functional and psychosocial changes in patients with extremity soft tissue sarcomas who have undergone multimodality limb-sparing treatments. In 88 patients, parameters related to economic status, sexual activity, pain, limb function, and global quality of life (QOL) were recorded prior to surgery and every 6 months postoperatively. Changes from the preoperative assessment for every parameter were analyzed in each patient. Six months after surgery, there was a decrease in employment status, sexual activity, and in limb function in a significant number of patients. At 12 months, these decreases were still evident. Despite these changes, global QOL measured by a standardized test showed at least some improvement in a significant proportion of patients at 12 months. These findings highlight the difficulty in defining QOL. It could not be ascertained if radiation therapy and/or chemotherapy were causative factors in specific changes because of the small numbers of patients in each subgroup. However, among 60 patients with high-grade sarcomas, significant wound problems developed in 10 of 33 who received postoperative radiation therapy in combination with adjuvant doxorubicin and cyclophosphamide chemotherapy compared with one of 27 patients who received adjuvant chemotherapy alone (P = .016). Also, among high-grade sarcoma patients with 12-month follow-up, six of 19 patients who received radiation therapy and chemotherapy developed joint contractures compared with zero of 15 patients who received chemotherapy alone (P less than .04). The combination of postoperative radiation therapy and chemotherapy appeared to be associated with significantly more tissue-related injury in patients with high-grade sarcomas compared with chemotherapy alone

  19. Functional and psychosocial effects of multimodality limb-sparing therapy in patients with soft tissue sarcomas

    Energy Technology Data Exchange (ETDEWEB)

    Chang, A.E.; Steinberg, S.M.; Culnane, M.; Lampert, M.H.; Reggia, A.J.; Simpson, C.G.; Hicks, J.E.; White, D.E.; Yang, J.J.; Glatstein, E. (National Cancer Institute, Bethesda, MD (USA))

    1989-09-01

    We have documented functional and psychosocial changes in patients with extremity soft tissue sarcomas who have undergone multimodality limb-sparing treatments. In 88 patients, parameters related to economic status, sexual activity, pain, limb function, and global quality of life (QOL) were recorded prior to surgery and every 6 months postoperatively. Changes from the preoperative assessment for every parameter were analyzed in each patient. Six months after surgery, there was a decrease in employment status, sexual activity, and in limb function in a significant number of patients. At 12 months, these decreases were still evident. Despite these changes, global QOL measured by a standardized test showed at least some improvement in a significant proportion of patients at 12 months. These findings highlight the difficulty in defining QOL. It could not be ascertained if radiation therapy and/or chemotherapy were causative factors in specific changes because of the small numbers of patients in each subgroup. However, among 60 patients with high-grade sarcomas, significant wound problems developed in 10 of 33 who received postoperative radiation therapy in combination with adjuvant doxorubicin and cyclophosphamide chemotherapy compared with one of 27 patients who received adjuvant chemotherapy alone (P = .016). Also, among high-grade sarcoma patients with 12-month follow-up, six of 19 patients who received radiation therapy and chemotherapy developed joint contractures compared with zero of 15 patients who received chemotherapy alone (P less than .04). The combination of postoperative radiation therapy and chemotherapy appeared to be associated with significantly more tissue-related injury in patients with high-grade sarcomas compared with chemotherapy alone.

  20. Role of radiation therapy in management of patients with sarcoma of soft tissue

    International Nuclear Information System (INIS)

    Spiro, Ira J.

    1996-01-01

    Soft tissue sarcomas (STS) are relatively rare malignant neoplasms arising from the mesenchymal connective tissues. There are some 5600 newly diagnosed patients with STS per year. These tumors occur at all anatomic sites within the body and are of many histologic subtypes. Etiologic factors, including occupational risks, the role of environmental carcinogens, radiation and genetic diseases in the development of these tumors will be mode. The molecular biology of soft tissue sarcomas including the role of several oncogenes and suppressor genes (e.g. Rb, p53, MDM2) will be reviewed. Cytogenetic alternations with an emphasis on molecular diagnostic techniques will be reviewed. The natural history of these tumors will be described with reference to local invasion and spread to regional and distal sites. The evaluation of the patients suspected of having a sarcoma of soft tissue will then be considered including the relative roles of various imaging modalities. The timing and type of biopsy (including FNA, core needle biopsy, incisional biopsy or excisional biopsy) for tumors at various sites and sizes will be addressed. Assessment of histopathologic subtype of the tumor by standard H and E stains, immunohistochemistry, electron microscopy and cytogenetic studies will then be discussed. The principal role for radiation in the management of patients with sarcoma of soft tissue is in combination with surgery. This may be: 1) pre-operative and or post-operative use of external beam photons, electrons, and protons, and 2) intra-operative use of electron beam techniques, or 3) post-operative brachytherapy. Results of these various treatment options with respect to local control, disease-free survival and overall survival will be considered for each of the various techniques with respect to size, grade, histologic type, surgical margin status, anatomic site, primary vs. recurrent disease. Similarly, the factors associated with delay in wound healing are to be considered and

  1. Role of radiation therapy in management of patients with sarcoma of soft tissue

    International Nuclear Information System (INIS)

    Spiro, Ira J.; Suit, Herman D.

    1997-01-01

    Soft tissue sarcomas (STS) are relatively rare malignant neoplasms arising from the mesenchymal connective tissues. There are some 5600 newly diagnosed patients with STS per year. These tumors occur at all anatomic sites within the body and are of many histologic subtypes. Etiologic factors, including occupational risks, the role of environmental carcinogens, radiation and genetic diseases in the development of these tumors will be made. The molecular biology of soft tissue sarcomas including the role of several oncogenes and suppressor genes (e.g. Rb, p53, MDM2) will be reviewed. Cytogenetic alternations with an emphasis on molecular diagnostic techniques will be reviewed. The natural history of these tumors will be described with reference to local invasion and spread to regional and distal sites. The evaluation of the patients suspected of having a sarcoma of soft tissue will then be considered including the relative roles of various imaging modalities. The timing and type of biopsy (including FNA, core needle biopsy, incisional biopsy or excisional biopsy) for tumors at various sites and sizes will be addressed. Assessment of histopathologic subtype of the tumor by standard H and E stains, immunohistochemistry, electron microscopy and cytogenetic studies will then be discussed. The principal role for radiation in the management of patients with sarcoma of soft tissue is in combination with surgery. This may be: 1) pre-operative and or post-operative use of external beam photons, electrons, and protons, and 2) intra-operative use of electron beam techniques, or 3) post-operative brachytherapy. Results of these various treatment options with respect to local control, disease-free survival and overall survival will be considered for each of the various techniques with respect to size, grade, histologic type, surgical margin status, anatomic site, primary vs. recurrent disease. Similarly, the factors associated with delay in wound healing are to be considered and

  2. Postirradiation soft tissue sarcoma occurring in breast cancer patients: report of seven cases and results of combination chemotherapy

    International Nuclear Information System (INIS)

    Kuten, A.; Sapir, D.; Cohen, Y.; Haim, N.; Borovik, R.; Robinson, E.

    1985-01-01

    Seven cases of soft tissue sarcoma developing after primary or postoperative radiotherapy for breast carcinoma are reported. The sarcomas occurred within the irradiated volume, after a latent period of 4-26 years. These cases conform well to established criteria for the diagnosis of radiation-induced sarcoma. Chemotherapy, consisting of the four-drug combination CYVADIC (cyclophosphamide, vincristine, adriamycin, DTIC) was employed in six of the seven patients. Only two of them achieved partial remission, lasting only 2 and 3 months, respectively. The effectiveness of adriamycin-containing chemotherapy regimens in soft tissue sarcomas as well as the remote hazard of radiation-related sarcoma in primary or postoperative breast irradiation are discussed

  3. Eribulin in the management of inoperable soft-tissue sarcoma: patient selection and survival

    Directory of Open Access Journals (Sweden)

    Thomas C

    2016-09-01

    Full Text Available Colin Thomas, Sujana Movva Department of Hematology/Oncology, Fox Chase Cancer Center, Philadelphia, PA, USA Abstract: Patients diagnosed with metastatic soft-tissue sarcoma (STS have a poor prognosis. Additionally, after failure of first-line therapy, there are relatively few treatment options from which to choose. The novel tubulin-binding drug, eribulin, with a unique mechanism of action from taxanes or vinca alkaloids, has shown clinical activity in several different types of cancers. Eribulin has been approved by the US Food and Drug Administration (FDA for patients with metastatic breast cancer previously treated with an anthracycline or a taxane and has recently been FDA approved for patients with unresectable or metastatic liposarcoma who have failed a previous anthracycline regimen. Here, we review current standard treatments of STS, a background of eribulin, the studies that have propelled eribulin to FDA approval for liposarcoma, and future directions of the drug. The benefits of eribulin in STS are discussed in detail, especially with regard to the recent pivotal Phase III study comparing eribulin to dacarbazine for leiomyosarcoma and adipocytic sarcoma. Keywords: eribulin, tubulin-binding drugs, soft-tissue sarcoma

  4. Soft Tissue Sarcomas In Children And Adolescents

    International Nuclear Information System (INIS)

    Bajciova, V.

    2008-01-01

    Soft tissue sarcomas are rare tumors, they may occur at any age. It is heterogenous group of different histology types, different biology and different clinical behavior. Different treatment strategy is used for children and adults. Adolescents with sarcomas could be managed by both pediatric and medical oncologists. Decision regarding location of treatment should be based on the best patient interest. (author)

  5. Ovarian tissue cryopreserved for fertility preservation from patients with Ewing or other sarcomas appear to have no tumour cell contamination

    DEFF Research Database (Denmark)

    Greve, Tine; Wielenga, Vera Timmermans; Grauslund, Morten

    2013-01-01

    The chemotherapy required to treat patients with sarcoma may as a side-effect induce infertility in girls and young women. If these patients have ovarian cortical tissue cryopreserved prior to chemotherapy, they may, if necessary, have the tissue transplanted and restore their fertility. The aim...

  6. Role of radiation therapy in management of patients with sarcoma of soft tissue

    International Nuclear Information System (INIS)

    Suit, Herman D.; Spiro, Ira J.

    1995-01-01

    Soft tissue sarcomas (STS) are malignant neoplasms arising from the mesenchymal connective and supporting tissues. These tumors occur at all anatomic sites within the body and are of many histologic subtypes. There are some 5600 newly diagnosed patients with STS per year. Epidemiological and etiologic factors, including the role of environmental carcinogens and radiation in the development of these tumors will be made. The role of several oncogenes and suppressor genes (e.g. Rb, p53, MDM2) and cytogenetic alterations will be reviewed. Consideration of the epidemiology and role of environmental carcinogens and radiation in the development of these tumors will be made. The natural history of these tumors will be described with reference to local invasion and spread to regional and distal sites. The evaluation of the patients suspected of having a sarcoma of soft tissue will then be considered including the relative roles of CT, MRI, PET, and US. The place of core needle biopsy, incisional biopsy or excisional biopsy for tumors at various sites and sizes will be addressed. The histopathologic subtype assessment of the tumor by standard H and E stains, immunohistochemistry, electron microscopy and cytogenetic studies will then be discussed. The principal role for radiation in the management of patients with sarcoma of soft tissue is in combination with surgery. This may be: 1) pre-operative and or post-operative use of external beam photons, electrons, and protons, and 2) intra-operative use of brachytherapy or intra-operative election beam techniques. Results of treatment with respect to local control, disease-free survival and overall survival will be considered for each of the various techniques with respect to size, grade, histologic type, surgical margin status, anatomic site, primary vs. recurrent disease. Similarly, the factors associated with delay in wound healing are to be considered and strategies to reduce wound morbidity. Functional outcome after limb

  7. Imaging of soft tissue sarcomas

    International Nuclear Information System (INIS)

    Vanel, D.; Le Treut, A.

    1988-01-01

    Modern imaging of soft tissue sarcomas now includes ultrasounds, CT and MRI. These new techniques allow a better evaluation of initial local extension, of the response to treatment and are able to detect local recurrences early [fr

  8. Impact of geriatric factors on surgical and prognostic outcomes in elderly patients with soft-tissue sarcoma.

    Science.gov (United States)

    Tsuda, Yusuke; Ogura, Koichi; Kobayashi, Eisuke; Hiruma, Toru; Iwata, Shintaro; Asano, Naofumi; Kawai, Akira; Chuman, Hirokazu; Ishii, Takeshi; Morioka, Hideo; Kobayashi, Hiroshi; Kawano, Hirotaka

    2017-05-01

    Patients aged ≥65 years requiring surgery for soft-tissue sarcoma are a concern in an aging society. We aimed to reveal the association of clinical/geriatric factors with survival period or postoperative events in such patients who underwent surgery. We enrolled patients aged ≥65 years who underwent surgery for localized soft-tissue sarcoma at five institutions. We retrospectively collected clinical/geriatric factors and laboratory data, and analyzed their association with outcomes using univariate and multivariate analyses. Among the 202 patients included, mean age at presentation was 73 years. Surgical margin was R0 in 139 patients (69%). The Eastern Cooperative Oncology Group performance status was ≥2 in 15 (7%). Thirty patients (15%) showed thinness (body mass index sarcoma-specific survival (hazard ratio for R1 vs. R0, 3.17; P = 0.001) and event-free survival (hazard ratio for R1 vs. R0, 2.56; P sarcoma-specific survival (hazard ratio for ≥2 vs. 0 or 1, 2.15; P = 0.038), and higher sensitivity-modified Glasgow prognostic score was significantly associated with poor event-free survival (hazard ratio for ≥1 vs. 0, 1.74; P = 0.046). Severe thinness (body mass index sarcoma patients. © The Author 2017. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

  9. Radiosensitivity of soft tissue sarcomas

    International Nuclear Information System (INIS)

    Hirano, Toru; Iwasaki, Katsuro; Suzuki, Ryohei; Monzen, Yoshio; Hombo, Zenichiro

    1989-01-01

    The correlation between the effectiveness of radiation therapy and the histology of soft tissue sarcomas was investigated. Of 31 cases with a soft tissue sarcoma of an extremity treated by conservative surgery and postoperative radiation of 3,000-6,000 cGy, local recurrence occurred in 12; 5 out of 7 synovial sarcomas, 4 of 9 MFH, one of 8 liposarcomas, none of 4 rhabdomyosarcomas and 2 of 3 others. As for the histological subtyping, the 31 soft tissue sarcomas were divided into spindle cell, pleomorphic cell, myxoid and round cell type, and recurrence rates were 75%, 33.3%, 16.7% and 0%, respectively. From the remarkable difference in recurrent rate, it was suggested that round cell and myxoid type of soft tissue sarcomas showed a high radiosensitivity compared to the spindle cell type with low sensitivity. Clarifying the degree of radiosensitivity is helpful in deciding on the management of limb salvage in soft tissue sarcomas of an extremity. (author)

  10. Expression of human Piwi-like genes is associated with prognosis for soft tissue sarcoma patients

    International Nuclear Information System (INIS)

    Greither, Thomas; Taubert, Helge; Koser, Franziska; Kappler, Matthias; Bache, Matthias; Lautenschläger, Christine; Göbel, Steffen; Holzhausen, Hans-Jürgen; Wach, Sven; Würl, Peter

    2012-01-01

    Argonaute genes are essential for RNA interference, stem cell maintenance and differentiation. The Piwi-like genes, a subclass of the Argonaute genes, are expressed mainly in the germline. These genes may be re-expressed in tumors, and expression of the Piwi-like genes is associated with prognosis in several types of tumors. We measured the expression of Piwi-like mRNAs (Piwi-like 2–4) in 125 soft tissue sarcoma (STS) samples by qPCRs. Statistical tests were applied to study the correlation of expression levels with tumor-specific survival for STS patients. In multivariate Cox’s regression analyses, we showed that low Piwi-like 2 and Piwi-like 4 mRNA expression were significantly associated with a worse prognosis (RR = 1.87; p = 0.032 and RR = 1.82; p = 0.039). Low expression of both genes was associated with a 2.58-fold increased risk of tumor-related death (p = 0.01). Piwi-like 4 and combined Piwi-like 2 and 4 mRNA levels correlated significantly with prognosis (RR = 3.53; p = 0.002 and RR = 5.23; p = 0.004) only for female but not for male patients. However, combined low Piwi-like 2 and 3 transcript levels were associated with worse survival (RR = 5.90; p = 0.02) for male patients. In this study, we identified a significant association between the expression of Piwi-like 2 and 4 mRNAs and the tumor-specific survival of soft tissue sarcoma patients. Furthermore, a connection between sex and the impact of Piwi-like mRNA expressions on STS patients’ prognosis was shown for the first time

  11. A Longitudinal Study of Functional Outcomes in Patients with Limb Salvage Surgery for Soft Tissue Sarcoma

    Directory of Open Access Journals (Sweden)

    Eunsun Oh

    2018-01-01

    Full Text Available Background. Many studies have reported on the surgical outcomes of soft tissue sarcoma. However, there was no longitudinal cohort study. Because time is the most valuable factor for functional recovery, adjusting time value was the key for finding the causal relationship between other risk factors and postoperative function. Therefore, existing cross-sectional studies can neither fully explain the causal relationship between the risk factors and the functional score nor predict functional recovery. The aim of this study was to determine important predictive factors that affect postoperative functional outcomes and longitudinal changes in functional outcomes in patients who had undergone limb-sparing surgery (LSS for soft tissue sarcoma (STS. Methods. Between January 2008 and December 2014, we retrospectively enrolled 150 patients who had undergone LSS for STS and had been assessed for postoperative functional outcomes with questionnaires. To evaluate functional outcomes, we used the Musculoskeletal Tumor Society (MSTS score and Toronto Extremity Salvage Score (TESS. Multivariate generalized estimating equation (GEE analysis was used to identify the predictive factors, including size, stage, and anatomic location of tumor, bone resection, flap reconstruction, age, and time after surgery. Each continuous variable such as age and time after surgery was explored for statistically significant cutoff points using the Wilcoxon rank sum test. Results. Functional scores significantly improved until the second year after surgery and plateaued for the rest of the 5-year period. Age p<0.0001, bone resection p=0.0004, and time after surgery p<0.0001 were identified as significant predictive factors. The functional score was significantly higher in patients younger than 47 years old. Conclusions. Functional outcomes can improve until the second year after surgery. Patients who were older than 47 and underwent bone resection may have poor final functional

  12. Post-radiation soft tissue sarcoma

    International Nuclear Information System (INIS)

    Tomita, Yasuhiko; Kuratsu, Shigeyuki; Myoui, Akira; Ohsawa, Masahiko; Aozasa, Katsuyuki; Uchida, Atsumasa; Ono, Keiro

    1993-01-01

    Seven patients received radiation for malignancies, and two received for benign tumors. The latency period from radiation to symptom ranged from two years to 36 years (mean 17.2 years). Post-radiation soft tissue sarcomas (PRS) comprised six cases of malignant fibrous histiocytomas, two leiomyosarcomas, and one angiosarcoma. The five-year survival of PRS was 16.7% showing a worse prognosis than spontaneously occurring soft tissue sarcomas. Seven PRS occurred superficially, and two were deeply located. Four cases occurring in the superficial tissues had histories of radiation-induced dermatitis. The radiation-induced dermatitis was suggested to be a risk factor for development of PRS. (author)

  13. [Update on soft tissue sarcomas].

    Science.gov (United States)

    Bui, Binh Nguyen; Tabrizi, Reza; Dagada, Corinne; Trufflandier, Nathalie; St ckle, Eberhard; Coindre, Jean-Michel

    2002-01-01

    Important refinements have taken place in the diagnosis of soft tissue sarcoma with extensive use of immuno-histochemistry. New entities have been described, while malignant histiocytofibroma, the most diagnosed sarcoma type during the last two decades, has been dismembered. As for prognosis, the new UICC classification is effectively more discriminating in the definition of prognostic groups; but the usefullness of new biological or genetic markers remains to be assessed. Several breakthrough have taken place in the last years in the treatment of soft tissue sarcoma. Isolated limb perfusion with TNF, hyperthermia and melphalan have proven its efficacy, and is now an alternative to preoperative chemotherapy and/or radiotherapy for limb sparing treatment of the primary tumor site or to amputation. For systemic treatments, novel cytostatic drugs have been shown to be active in sarcomas, including ecteinascidine (ET743) and Glivec (STI571). This last drug has been shown to be remarkably active in c-kit+ stromal sarcoma of the gastro-intestinal tract. It can hopefully regarded as an example for targeted therapies, which may come with a better understanding of the molecular mechanisms triggered by the fundamental, specific genetic alterations shown in sarcoma.

  14. Soft tissue sarcoma - diagnosis and treatment

    International Nuclear Information System (INIS)

    Ruka, W.; Rutkowski, P.; Krzakowski, M.

    2009-01-01

    Significant progress in the treatment of soft tissue sarcoma (STS), both primary tumor and local recurrences/metastatic disease, has been achieved in recent years. Surgery is essential modality, but the use of combined treatment (standard combination of surgery with adjuvant radiotherapy, chemotherapy in selected cases and perioperative rehabilitation) in highly-experienced centers increased possibility of cure and limitations of extent of local surgery. Current combined therapy together with the use of reconstructive methods allows for limb-sparing surgery in majority of soft tissue sarcoma patients (amputation in 10% of cases as compared to approximately 50% in the 1960 - 70s). The slow, but constant, increase of rate of soft tissue sarcoma patients with long-term survival has been observed. Contemporary 5-year overall survival rate in patients with extremity soft tissue sarcomas is 55 -78%. In case of diagnosis of metastatic disease the prognosis is still poor (survival of approximately 1 year). Good results of local therapy may be expected only after planned (e.g., after preoperative biopsy - tru - cut or incisional) radical surgical excision of primary tumor with pathologically negative margins (R0 resection). Following appropriate diagnostic check-up, adjuvant radiotherapy is necessary in the majority of patients treated with radical surgery need, as well as long-term rehabilitation and follow-up examinations in treating center are needed for at least 5 years. The progress is due to the introduction of targeted therapy acting on molecular or genetic cellular disturbances detected during studies on etiopathogenetic mechanisms of sarcoma subtypes. In view of rarity of sarcomas and necessity of multidisciplinary therapy, the crucial issue is that management of these tumors should be hold in experienced oncological sarcoma centers. (authors)

  15. Evaluation of Quality of Life at Progression in Patients with Soft Tissue Sarcoma

    Directory of Open Access Journals (Sweden)

    Stacie Hudgens

    2017-01-01

    Full Text Available Introduction. Soft Tissue Sarcoma (STS is a rare malignancy of mesodermal tissue, with international incidence estimates between 1.8 and 5 per 100,000 per year. Understanding quality of life (QoL and the detrimental impact of disease progression is critical for long-term care and survival. Objectives. The primary objective was to explore the relationship between disease progression and health-related quality of life (HRQoL using data from Eisai’s study (E7389-G000-309. Methods. This was a 1 : 1 randomized, open-label, multicenter, Phase 3 study comparing the efficacy and safety of eribulin versus dacarbazine in patients with advanced STS. The QoL analysis was conducted for the baseline and progression populations using the European Organization for Research and Treatment of Cancer 30-item core QoL questionnaire (EORTC QLQ-C30. Results. There were no statistical differences between the two treatment arms at baseline for any domain (p>0.05; n=452. Of the 399 patients who experienced disease progression (unadjusted and adjusting for histology, dacarbazine patients had significantly lower Global Health Status, Physical Functioning scores, and significantly worse Nausea and Vomiting, Insomnia, and Appetite Loss (p<0.05. Conclusions. These results indicate differences in HRQoL overall and at progression between dacarbazine and eribulin patients, with increases in symptom severity observed among dacarbazine patients.

  16. Olaratumab for advanced soft tissue sarcoma.

    Science.gov (United States)

    Tobias, Alexander; O'brien, Michael P; Agulnik, Mark

    2017-07-01

    Olaratumab is a humanized IgG1 monoclonal antibody that blocks the platelet-derived growth factor receptor alpha (PDGFRα). Its antagonistic behavior inhibits the receptor's tyrosine kinase activity, thereby, turning off the downstream signaling cascades responsible for soft tissue sarcoma tumorigenesis. In October 2016, olaratumab received Food and Drug Administration (FDA) approval for its use in combination with doxorubicin for treatment of advanced soft tissue sarcoma. Areas covered: This drug profile takes a comprehensive look at the clinical studies leading to FDA approval of olaratumab as well as its safety and efficacy as a front-line treatment option for sarcoma patients. The literature search was primarily conducted using PubMed. Expert commentary: The combination of olaratumab plus doxorubicin has provided a new front-line therapeutic option for soft tissue sarcoma patients. An open-label phase Ib and randomized phase II trial in patients with advanced soft tissue sarcoma demonstrated that the addition of olaratumab to doxorubicin prolonged progression-free survival by 2.5 months and overall survival by 11.8 months when compared to doxorubicin alone. Of importance, this clinically meaningful increase in overall survival did not come at the expense of a significantly greater number of toxicities. A phase III confirmatory trial (ClinicalTrials.gov Identifier NCT02451943) will be completed in 2020.

  17. Soft tissue sarcoma of the extremity.

    LENUS (Irish Health Repository)

    Cooper, T M

    2012-02-03

    A retrospective review of 33 cases of soft tissue sarcoma of the extremity presenting over a 10 year period was undertaken. The history, patterns of referral, diagnostic investigations, procedures undertaken and outcomes were studied. We found there was a frequent delay in diagnosis and sometimes misinterpretation of biopsy specimens. Patients were seen by a variety of specialists from disciplines such as general surgery, plastic surgery, orthopaedic surgery and rheumatology. Considerable progress has been made in the treatment of soft tissue sarcomas, often allowing local control of the tumour without amputation. We believe there should be early referral of patients having these tumours to a centre where a combined multidisciplinary approach can be undertaken.

  18. Cyclophosphamide versus ifosfamide for paediatric and young adult bone and soft tissue sarcoma patients

    NARCIS (Netherlands)

    Mulder, Renée L.; Paulides, Marios; Langer, Thorsten; Kremer, Leontien C. M.; van Dalen, Elvira C.

    2010-01-01

    BACKGROUND: Alkylating agents, such as cyclophosphamide and ifosfamide, play a major role in the improved survival of children and young adults with bone and soft tissue sarcoma. However, there is still controversy as to their comparative anti-tumour efficacy and possible adverse effects.

  19. Cyclophosphamide versus ifosfamide for paediatric and young adult bone and soft tissue sarcoma patients

    NARCIS (Netherlands)

    Mulder, Renée L.; Paulides, Marios; Langer, Thorsten; Kremer, Leontien C. M.; van Dalen, Elvira C.

    2012-01-01

    Background Alkylating agents, such as cyclophosphamide and ifosfamide, play a major role in the improved survival of children and young adults with bone and soft tissue sarcoma. However, there is still controversy as to their comparative anti-tumour efficacy and possible adverse effects. This is an

  20. Cyclophosphamide versus ifosfamide for paediatric and young adult bone and soft tissue sarcoma patients

    NARCIS (Netherlands)

    Mulder, Renée L.; Paulides, Marios; Langer, Thorsten; Kremer, Leontien C. M.; van Dalen, Elvira C.

    2015-01-01

    Alkylating agents, such as cyclophosphamide and ifosfamide, play a major role in the improved survival of children and young adults with bone and soft tissue sarcoma. However, there is still controversy as to their comparative anti-tumour efficacy and possible adverse effects. This is the second

  1. The Scandinavian Sarcoma Group Central Register: 6,000 patients after 25 years of monitoring of referral and treatment of extremity and trunk wall soft-tissue sarcoma.

    Science.gov (United States)

    Trovik, Clement; Bauer, Henrik C F; Styring, Emelie; Sundby Hall, Kirsten; Vult Von Steyern, Fredrik; Eriksson, Sigvard; Johansson, Ingela; Sampo, Mika; Laitinen, Minna; Kalén, Anders; Jónsson, Halldór; Jebsen, Nina; Eriksson, Mikael; Tukiainen, Erkki; Wall, Najme; Zaikova, Olga; Sigurðsson, Helgi; Lehtinen, Tuula; Bjerkehagen, Bodil; Skorpil, Mikael; Egil Eide, Geir; Johansson, Elisabeth; Alvegard, Thor A

    2017-06-01

    Purpose - We wanted to examine the potential of the Scandinavian Sarcoma Group (SSG) Central Register, and evaluate referral and treatment practice for soft-tissue sarcomas in the extremities and trunk wall (STS) in the Nordic countries. Background - Based on incidence rates from the literature, 8,150 (7,000-9,300) cases of STS of the extremity and trunk wall should have been diagnosed in Norway, Finland, Iceland, and Sweden from 1987 through 2011. The SSG Register has 6,027 cases registered from this period, with 5,837 having complete registration of key variables. 10 centers have been reporting to the Register. The 5 centers that consistently report treat approximately 90% of the cases in their respective regions. The remaining centers have reported all the patients who were treated during certain time periods, but not for the entire 25-year period. Results - 59% of patients were referred to a sarcoma center untouched, i.e. before any attempt at open biopsy. There was an improvement from 52% during the first 5 years to 70% during the last 5 years. 50% had wide or better margins at surgery. Wide margins are now achieved less often than 20 years ago, in parallel with an increase in the use of radiotherapy. For the centers that consistently report, 97% of surviving patients are followed for more than 4 years. Metastasis-free survival (MFS) increased from 67% to 73% during the 25-year period. Interpretation - The Register is considered to be representative of extremity and trunk wall sarcoma disease in the population of Scandinavia, treated at the reporting centers. There were no clinically significant differences in treatment results at these centers.

  2. Exposure-survival analyses of pazopanib in renal cell carcinoma and soft tissue sarcoma patients: opportunities for dose optimization.

    Science.gov (United States)

    Verheijen, R B; Swart, L E; Beijnen, J H; Schellens, J H M; Huitema, A D R; Steeghs, N

    2017-12-01

    Pazopanib is an angiogenesis inhibitor approved for the treatment of renal cell carcinoma and soft tissue sarcoma. Post hoc analysis of a clinical trial demonstrated a relationship between pazopanib trough concentrations (C min ) and treatment efficacy. The aim of this study was to explore the pharmacokinetics and exposure-survival relationships of pazopanib in a real-world patient cohort. Renal cell cancer and soft tissue sarcoma patients who had at least one pazopanib plasma concentration available were included. Using calculated C min values and a threshold of > 20 mg/L, univariate and multivariate exposure-survival analyses were performed. Sixty-one patients were included, of which 16.4% were underexposed (mean C min   20 mg/L was related to longer progression free survival in renal cell cancer patients (34.1 vs. 12.5 weeks, n = 35, p = 0.027) and the overall population (25.0 vs. 8.8 weeks, n = 61, p = 0.012), but not in the sarcoma subgroup (18.7 vs. 8.8 weeks, n = 26, p = 0.142). In multivariate analysis C min  > 20 mg/L was associated with hazard ratios of 0.25 (p = 0.021) in renal cancer, 0.12 (p = 0.011) in sarcoma and 0.38 (p = 0.017) in a pooled analysis. This study confirms that pazopanib C min  > 20 mg/L relates to better progression free survival in renal cancer and points towards a similar trend in sarcoma patients. C min monitoring of pazopanib can help identify patients with low C min for whom individualized treatment at a higher dose may be appropriate.

  3. Soft tissue sarcoma - Compliance with guidelines

    NARCIS (Netherlands)

    Nijhuis, PHA; Schaapveld, M; Otter, R; Hoekstra, HJ

    2001-01-01

    BACKGROUND. Because soft tissue sarcomas (STS) are rare, guidelines for the diagnosis and treatment of patients with STS were developed. Because the diagnostic management is essential for definitive treatment, adherence to these guidelines is important. METHODS. Primary STS registered by the

  4. The tolerance of skin grafts to postoperative radiation therapy in patients with soft-tissue sarcoma

    International Nuclear Information System (INIS)

    Lawrence, W.T.; Zabell, A.; McDonald, H.D.

    1986-01-01

    During the last ten years at the National Cancer Institute, 11 patients have received 12 courses of postoperative adjuvant radiation therapy to skin grafts used for wound closure after the resection of soft-tissue sarcomas. The intervals between grafting and the initiation of radiation ranged between 3 and 20 weeks, and 4 patients received chemotherapy at the same time as their radiation. Ten of the 12 irradiated grafts remained intact after the completion of therapy. One graft had several small persistently ulcerated areas that required no further surgical treatment, and one graft required a musculocutaneous flap for reconstruction of a persistent large ulcer. Acute radiation effects on the grafted skin sometimes developed at slightly lower doses than usually seen with normal skin, but these acute effects necessitated a break in therapy on only five occasions. Concurrent chemotherapy and a relatively short interval between grafting and the initiation of radiation seemed to contribute to more severe radiation reactions. This experience indicates that postoperative adjuvant radiation therapy can be delivered to skin grafted areas without undue fear of complications, especially if the graft is allowed to heal adequately prior to initiating therapy and if chemotherapy is not given in conjunction with radiation

  5. Extremity Soft Tissue Sarcoma: A Review of 19 Cases. | Eyesan ...

    African Journals Online (AJOL)

    Background: Although soft tissue sarcoma is a rare tumour, it accounts for a significant proportion of malignancies seen in many orthopaedic practices. The objectives of this study are to evaluate the pattern of presentation of extremity soft tissue sarcoma and the treatment outcome in our patients. Method: This is a 3 year ...

  6. Tumor-targeting Salmonella typhimurium A1-R is a highly effective general therapeutic for undifferentiated soft tissue sarcoma patient-derived orthotopic xenograft nude-mouse models.

    Science.gov (United States)

    Igarashi, Kentaro; Kawaguchi, Kei; Kiyuna, Tasuku; Miyake, Kentaro; Miyake, Masuyo; Singh, Arun S; Eckardt, Mark A; Nelson, Scott D; Russell, Tara A; Dry, Sarah M; Li, Yunfeng; Yamamoto, Norio; Hayashi, Katsuhiro; Kimura, Hiroaki; Miwa, Shinji; Tsuchiya, Hiroyuki; Singh, Shree Ram; Eilber, Fritz C; Hoffman, Robert M

    2018-03-18

    Undifferentiated soft tissue sarcoma (USTS) is a recalcitrant and heterogeneous subgroup of soft tissue sarcoma with high risk of metastasis and recurrence. Due to heterogeneity of USTS, there is no reliably effective first-line therapy. We have generated tumor-targeting Salmonella typhimurium A1-R (S. typhimurium A1-R), which previously showed strong efficacy on single patient-derived orthotopic xenograft (PDOX) models of Ewing's sarcoma and follicular dendritic cell sarcoma. In the present study, tumor resected from 4 patients with a biopsy-proven USTS (2 undifferentiated pleomorphic sarcoma [UPS], 1 undifferentiated sarcoma not otherwise specified [NOS] and 1 undifferentiated spindle cell sarcoma [USS]) were grown orthotopically in the biceps femoris muscle of mice to establish PDOX models. One USS model and one UPS model were doxorubicin (DOX) resistant. One UPS and the NOS model were partially sensitive to DOX. DOX is first-line therapy for these diseases. S. typhimurium A1-R arrested tumor growth all 4 models. In addition to arresting tumor growth in each case, S. typhimurium A1-R was significantly more efficacious than DOX in each case, thereby surpassing first-line therapy. These results suggest that S. typhimurium A1-R can be a general therapeutic for USTS and possibly sarcoma in general. Published by Elsevier Inc.

  7. The Diagnostic and Prognostic Value of Hematological and Chemical Abnormalities in Soft Tissue Sarcoma: A Comparative Study in Patients with Benign and Malignant Soft Tissue Tumors.

    Science.gov (United States)

    Ariizumi, Takashi; Kawashima, Hiroyuki; Ogose, Akira; Sasaki, Taro; Hotta, Tetsuo; Hatano, Hiroshi; Morita, Tetsuro; Endo, Naoto

    2018-01-01

    The value of routine blood tests in malignant soft tissue tumors remains uncertain. To determine if these tests can be used for screening, the routine pretreatment blood test findings were retrospectively investigated in 359 patients with benign and malignant soft tissue tumors. Additionally, the prognostic potential of pretreatment blood abnormalities was evaluated in patients with soft tissue sarcomas. We compared clinical factors and blood tests findings between patients with benign and malignant soft tissue tumors using univariate and multivariate analysis. Subsequently, patients with malignant tumors were divided into two groups based on blood test reference values, and the prognostic significance of each parameter was evaluated. In the univariate analysis, age, tumor size, and tumor depth were significant clinical diagnostic factors. Significant increases in the granulocyte count, C-reactive protein (CRP) level, erythrocyte sedimentation rate (ESR), and γ-glutamyl transpeptidase (γ-GTP) levels were found in patients with malignant soft tissue tumors. Multiple logistic regression showed that tumor size and ESR were independent factors that predicted malignant soft tissue tumors. The Kaplan-Meier survival analysis revealed that granulocyte counts, γ-GTP levels, and CRP levels correlated significantly with overall survival. Thus, pretreatment routine blood tests are useful diagnostic and prognostic markers for diagnosing soft tissue sarcoma. © 2018 by the Association of Clinical Scientists, Inc.

  8. Follow-up of recurrences of limb soft tissue sarcomas in patients with localized disease: performance of ultrasound

    International Nuclear Information System (INIS)

    Tagliafico, Alberto; Truini, Mauro; Spina, Bruno; Cambiaso, Paolo; Zaottini, Federico; Bignotti, Bianca; Derchi, Lorenzo E.; Martinoli, Carlo; Calabrese, Massimo

    2015-01-01

    To evaluate diagnostic performance of ultrasound in the detection of local recurrences in patients with localized soft tissue sarcomas of the limb. An analysis of patients treated for soft tissue sarcomas between 2005 and April 2014 was performed. Sixty-eight patients (men/women, 36:32; age range, 18-84 years) were evaluated. Sensitivity, specificity with 95 % confidence intervals (CIs), positive predictive value (PPV), pre-test probability (the prevalence), negative predictive value (NPV), likelihood ratio for positive results (LH+), accuracy and post-test probability (post-P) of ultrasound were reported on a per patient basis using surgical findings and clinical follow-up as reference standard. Effects of independent variables (US equipment, age and sex, body mass index, radiologist) were considered. Comparison with MR was also performed. The overall sensitivity and specificity were 0.88 (0.60-0.94) and 0.94 (0.86-0.98). PPV, pre-test probability, NPV, LH+, accuracy and post-P: 0.83/0.25/0.96/14.9/0.92/0.83. There were two false negative cases both graded as G3 and deeply located and three false positive US cases. Diagnostic accuracy was not dependent by US machine (p = 0.08), age and sex (p = 0.16), body mass index (p = 0.07) and radiologists (p = 0.07). Diagnostic accuracy of ultrasound was relatively high. Negative US results excluded the presence of a local recurrence with acceptable accuracy. (orig.)

  9. Quality of Life and Utility in Patients with Metastatic Soft Tissue and Bone Sarcoma: The Sarcoma Treatment and Burden of Illness in North America and Europe (SABINE Study

    Directory of Open Access Journals (Sweden)

    Peter Reichardt

    2012-01-01

    Full Text Available The aim of the study was to assess health-related quality of life (HRQoL among metastatic soft tissue (mSTS or bone sarcoma (mBS patients who had attained a favourable response to chemotherapy. We employed the EORTC QLQ-C30, the 3-item Cancer-Related Symptoms Questionnaire, and the EQ-5D instrument. HRQoL was evaluated overall and by health state in 120 mSTS/mBS patients enrolled in the SABINE study across nine countries in Europe and North America. Utility was estimated from responses to the EQ-5D instrument using UK population-based weights. The mean EQ-5D utility score was 0.69 for the pooled patient sample with little variation across health states. However, patients with progressive disease reported a clinically significant lower utility (0.56. Among disease symptoms, pain and respiratory symptoms are common. This study showed that mSTS/mBS is associated with reduced HRQoL and utility among patients with metastatic disease.

  10. Drugs Approved for Soft Tissue Sarcoma

    Science.gov (United States)

    This page lists cancer drugs approved by the Food and Drug Administration (FDA) for soft tissue sarcoma. The list includes generic names and brand names. The drug names link to NCI's Cancer Drug Information summaries.

  11. Intracellular vorinostat accumulation and its relationship to histone deacetylase activity in soft tissue sarcoma patients.

    Science.gov (United States)

    Burhenne, Jürgen; Liu, Lu; Heilig, Christoph E; Meid, Andreas D; Leisen, Margarete; Schmitt, Thomas; Kasper, Bernd; Haefeli, Walter E; Mikus, Gerd; Egerer, Gerlinde

    2017-08-01

    In the regulation of chromatin-structure and histone function, histone deacetylases (HDACs) are key enzymes and thus modulators of epigenetic regulation and gene expression. Accesses of the HDAC inhibitor vorinostat to intracellular compartments are essential to exert epigenetic effects. In ten sarcoma patients receiving oral Zolinza (400 mg qd) vorinostat concentrations in plasma and peripheral blood mononuclear cells (PBMCs) were quantified using validated LC/MS/MS assays to determine intracellular and extracellular pharmacokinetic data. Cellular HDAC activity was evaluated using a fluorogenic assay. Concentration-response relationships were established between intracellular and extracellular vorinostat concentrations and HDAC inhibition in PBMCs. Pharmacokinetics of vorinostat and its two main inactive metabolites were determined over 8 h in plasma and PBMCs. Steady state AUCs (±SD) and T 1/2 (±SD) were calculated to 4.61 ± 0.87 h µM and 1.73 ± 0.69 h (plasma) and 15.2 ± 9.03 h µM and 5.30 ± 4.27 h (PBMCs). Intracellular accumulation of vorinostat was determined together with prolonged vorinostat elimination in PBMCs. Cellular HDAC inhibition increased parallel with vorinostat concentrations in plasma and PBMCs. For effective inhibition of cellular HDACs (IC 50 ) vorinostat concentrations of 0.05 µM in plasma and 0.17 µM in PBMCs were necessary. HDAC inhibition closely followed intracellular vorinostat concentrations and was short-lasting, which may contribute to the limited efficacy seen with vorinostat in solid tumors so far.

  12. Costs Associated with Intravenous Cancer Therapy Administration in Patients with Metastatic Soft Tissue Sarcoma in a US Population

    Directory of Open Access Journals (Sweden)

    Mei Sheng Duh

    2013-01-01

    Full Text Available Background. The most common chemotherapies in metastatic soft tissue sarcoma (mSTS require intravenous (IV administration. This often requires patients to make multiple outpatient visits per chemotherapy cycle, possibly impeding patients’ daily activities and increasing caregiver burden and medical costs. This study investigated costs associated with IV cancer therapy administration in mSTS from the payer perspective of the health care system. Patients and Methods. From the Experian Healthcare database, 1,228 mSTS patients were selected. Data were analyzed on outpatient visits during 2005–2012 involving IV cancer therapy administration. Costs were estimated on a per patient per visit (PPPV and per patient per month (PPPM basis. Results. The mean (median cost of IV therapy was $2,427 ($1,532 PPPV and $5,468 ($4,310 PPPM, of which approximately 60% was IV drug costs. IV administration costs averaged $399 PPPV and $900 PPPM, representing 16.5% of total visit costs. Anthracycline and alkylating-agents-based therapies had the highest PPPV and PPPM IV administration costs, respectively (mean $479 and $1,336, resp.. Patients with managed care insurance had the highest IV administration costs (mean $504 PPPV; $1,120 PPPM. Conclusions. IV administration costs constitute a considerable proportion of the total costs of receiving an IV cancer therapy to treat mSTS.

  13. Local control and survival in patients with soft tissue sarcomas treated with limb sparing surgery in combination with interstitial brachytherapy and external radiation

    DEFF Research Database (Denmark)

    Muhic, A.; Hovgaard, D.; Mork, Petersen M.

    2008-01-01

    %. Nineteen (49%) patients suffered from some degree of decreased force or function of the affected extremity, 16 (41%) suffered from oedema, 12 (31%) had persistent pain, 8 (21%) suffered from wound complications, and in 4 (10%) of these patients plastic surgery were required. CONCLUSION: Limb sparing...... surgery, combined with PDR BRT and EBRT can result in good local control in patients with soft tissue sarcomas. BRT is an effective modality with good cosmetic results and acceptable toxicity Udgivelsesdato: 2008/9...

  14. A Device and Procedure for Immobilization of Patients Receiving Limb-Preserving Radiotherapy for Soft Tissue Sarcoma

    International Nuclear Information System (INIS)

    Dickie, Colleen I.; Parent, Amy; Griffin, Anthony; Craig, Tim; Catton, Charles; Chung, Peter; Panzarella, Tony; O'Sullivan, Brian; Sharpe, Michael

    2009-01-01

    The purpose of this study was to determine the accuracy and efficiency of a custom-designed immobilization device for patients with extremity soft-tissue sarcoma. The custom device consisted of a thermoplastic shell, vacuum pillow, and adaptable baseplate. The study included patients treated from January 2005 to March 2007, with 92 patients immobilized with the custom device and 98 with an established standard. Setup times for these cohorts were analyzed retrospectively for conformal and intensity modulated radiotherapy techniques (IMRT). Thigh tumor setup times were analyzed independently. A subset of patients treated with IMRT was analyzed for setup error using the radiographically verified isocenter position measured daily with electronic portal imaging and cone-beam computed tomography. Mean setup time was reduced by 2.2 minutes when using the custom device for conformal treatment (p = 0.03) and by 5.8 min for IMRT of thigh tumors (p = 0.009). All other setup time comparisons were not significant. A significant systematic error reduction was seen in all directions using the custom device. Random error standard deviations favored the custom device. The custom device offers immobilization advantages. Patient setup time was reduced for conformal techniques and IMRT of thigh tumors. Positioning uncertainty was improved, permitting a reduction of the planning target volume margin by 2 to 4 mm.

  15. Histology and imaging of soft tissue sarcomas.

    Science.gov (United States)

    Kind, Michèle; Stock, Nathalie; Coindre, Jean Michel

    2009-10-01

    Imaging and histology are two complementary morphological techniques which play a fundamental role in the diagnosis and management of soft tissue sarcomas. Imaging allows to identify some pseudosarcomatous benign lesions such as myositis ossificans, intramuscular hemangioma, angiomyolipoma, intramuscular lipoma, giant cell tumour of tendon sheath, desmoid tumour and elastofibroma. There is no formal criterion for diagnosing a sarcoma on magnetic resonance imaging (MRI) but malignancy is strongly suspected with the presence of necrosis and vascular, bone or joint invasion. Imaging may also suggest some histological types of sarcoma such as well-differentiated liposarcoma, dedifferentiated liposarcoma, synovial sarcoma or extraskeletal osteosarcoma. Imaging is also extremely helpful in determining the appropriate kind of sampling to carry out and in guiding the performance of a microbiopsy. The appearance observed on imaging should always be taken into consideration for the interpretation of the microbiopsy by the pathologist.

  16. Histology and imaging of soft tissue sarcomas

    International Nuclear Information System (INIS)

    Kind, Michele; Stock, Nathalie; Coindre, Jean Michel

    2009-01-01

    Imaging and histology are two complementary morphological techniques which play a fundamental role in the diagnosis and management of soft tissue sarcomas. Imaging allows to identify some pseudosarcomatous benign lesions such as myositis ossificans, intramuscular hemangioma, angiomyolipoma, intramuscular lipoma, giant cell tumour of tendon sheath, desmoid tumour and elastofibroma. There is no formal criterion for diagnosing a sarcoma on magnetic resonance imaging (MRI) but malignancy is strongly suspected with the presence of necrosis and vascular, bone or joint invasion. Imaging may also suggest some histological types of sarcoma such as well-differentiated liposarcoma, dedifferentiated liposarcoma, synovial sarcoma or extraskeletal osteosarcoma. Imaging is also extremely helpful in determining the appropriate kind of sampling to carry out and in guiding the performance of a microbiopsy. The appearance observed on imaging should always be taken into consideration for the interpretation of the microbiopsy by the pathologist.

  17. Histology and imaging of soft tissue sarcomas

    Energy Technology Data Exchange (ETDEWEB)

    Kind, Michele [Departement d' Imagerie Medicale, Institut Bergonie, 229 cours de l' Argonne, 33076 Bordeaux Cedex (France)], E-mail: kind@bergonie.org; Stock, Nathalie; Coindre, Jean Michel [Departement de Pathologie, Institut Bergonie, 229 cours de l' Argonne, 33076 Bordeaux Cedex (France); Universite Victor Segalen Bordeaux 2, 146 rue Leo Saignat, 33076 Bordeaux Cedex (France)

    2009-10-15

    Imaging and histology are two complementary morphological techniques which play a fundamental role in the diagnosis and management of soft tissue sarcomas. Imaging allows to identify some pseudosarcomatous benign lesions such as myositis ossificans, intramuscular hemangioma, angiomyolipoma, intramuscular lipoma, giant cell tumour of tendon sheath, desmoid tumour and elastofibroma. There is no formal criterion for diagnosing a sarcoma on magnetic resonance imaging (MRI) but malignancy is strongly suspected with the presence of necrosis and vascular, bone or joint invasion. Imaging may also suggest some histological types of sarcoma such as well-differentiated liposarcoma, dedifferentiated liposarcoma, synovial sarcoma or extraskeletal osteosarcoma. Imaging is also extremely helpful in determining the appropriate kind of sampling to carry out and in guiding the performance of a microbiopsy. The appearance observed on imaging should always be taken into consideration for the interpretation of the microbiopsy by the pathologist.

  18. New Therapeutic Targets in Soft Tissue Sarcoma

    Science.gov (United States)

    Demicco, Elizabeth G; Maki, Robert G; Lev, Dina C.; Lazar, Alexander J

    2012-01-01

    Soft tissue sarcomas are an uncommon and diverse group of more than 50 mesenchymal malignancies. The pathogenesis of many of these is poorly understood, but others have begun to reveal the secrets of their inner workings. With considerable effort over recent years, soft tissue sarcomas have increasingly been classified on the basis of underlying molecular alterations. In turn, this has allowed the development and application of targeted agents in several specific, molecularly defined, sarcoma subtypes. This review will focus the rationale for targeted therapy in sarcoma, with emphasis on the relevance of specific molecular factors and pathways in both translocation-associated sarcomas and in genetically complex tumors. In addition, we will address some of the early successes in sarcoma targeted therapy as well as a few challenges and disappointments in this field. Finally we will discuss several possible opportunities represented by poorly understood, but potentially promising new therapeutic targets, as well as several novel biologic agents currently in preclinical and early phase I/II trials. This will provide the reader with context for understanding the current state this field and a sense of where it may be headed in the coming years. PMID:22498582

  19. Results of a Qualitative Study to Develop a Patient Reported Outcome Measure for Patients with 4 Subtypes of Soft Tissue Sarcoma

    Directory of Open Access Journals (Sweden)

    Anne M. Skalicky

    2017-01-01

    Full Text Available Objective. The objective of this research was to develop a disease-specific symptom inventory for soft tissue sarcoma. Methods. Literature review and clinical expert and patient interviews were conducted to determine disease-specific symptoms important to patients with one of the four STS subtypes. Clinical experts identified the most relevant STS symptom items from the item pool developed from literature review. Concept elicitation interviews were conducted with patients to elicit their STS symptom experiences followed by a completion of the draft symptom list via web survey. A cognitive interview was conducted on the comprehension and importance of the symptom items. Results. Eighty-three symptom items were compiled and discussed with three clinical experts who identified 26 symptoms specific to the four STS subtypes. A total sample of 27 STS participants with self-reported leiomyosarcoma (74%, undifferentiated sarcoma (15%, synovial sarcoma (7%, or liposarcoma (4% diagnosis completed the web survey and 10 were interviewed. The draft 12-item STS-specific symptom inventory includes abdominal pain, pressure in abdomen, early satiety, bloating, gastrointestinal pain, muscle pain, bone pain, heavy menstrual flow, shortness of breath, chest pain, cough, and painful menstruation. Conclusion. A number of symptoms are common across STS subtypes and may form a single STS symptom inventory.

  20. Carbon ion radiotherapy in bone and soft tissue sarcomas

    International Nuclear Information System (INIS)

    Kamada, Tadashi; Imai, Reiko; Kagei, Kenji; Tsuji, Hiroshi; Yanagi, Takeshi; Ishikawa, Hitoshi; Tsujii, Hirohiko

    2006-01-01

    The Heavy Ion Medical Accelerator in Chiba (HIMAC) is the world's first heavy ion accelerator complex dedicated to medical use in a hospital environment. Heavy ions have superior depth-dose distribution and greater cell-killing capability. In June 1996, clinical research for the treatment of bone and soft tissue sarcomas was begun using carbon ions generated by the HIMAC. As of February 2006, a total of the 278 patients with bone and soft tissue sarcoma had been enrolled into the clinical trial. Most of the patients had locally advanced and/or medically inoperable tumors. The clinical trial revealed that carbon ion radiotherapy provided definite local control and offered a survival advantage without unacceptable morbidity in bone and soft tissue sarcomas that were hard to cure with other modalities. (author)

  1. Current Molecular Targeted Therapies for Bone and Soft Tissue Sarcomas

    Directory of Open Access Journals (Sweden)

    Kenji Nakano

    2018-03-01

    Full Text Available Systemic treatment options for bone and soft tissue sarcomas remained unchanged until the 2000s. These cancers presented challenges in new drug development partly because of their rarity and heterogeneity. Many new molecular targeting drugs have been tried in the 2010s, and some were approved for bone and soft tissue sarcoma. As one of the first molecular targeted drugs approved for solid malignant tumors, imatinib’s approval as a treatment for gastrointestinal stromal tumors (GISTs has been a great achievement. Following imatinib, other tyrosine kinase inhibitors (TKIs have been approved for GISTs such as sunitinib and regorafenib, and pazopanib was approved for non-GIST soft tissue sarcomas. Olaratumab, the monoclonal antibody that targets platelet-derived growth factor receptor (PDGFR-α, was shown to extend the overall survival of soft tissue sarcoma patients and was approved in 2016 in the U.S. as a breakthrough therapy. For bone tumors, new drugs are limited to denosumab, a receptor activator of nuclear factor κB ligand (RANKL inhibitor, for treating giant cell tumors of bone. In this review, we explain and summarize the current molecular targeting therapies approved and in development for bone and soft tissue sarcomas.

  2. Cellular immunotherapy for soft tissue sarcomas

    Science.gov (United States)

    Finkelstein, Steven Eric; Fishman, Mayer; Conley, Anthony P.; Gabrilovich, Dmitry; Antonia, Scott; Chiappori, Alberto

    2015-01-01

    SUMMARY Soft tissue sarcomas are rare neoplasms, with approximately 9,000 new cases in the United States every year. Unfortunately, there is little progress in the treatment of metastatic soft tissue sarcomas in the past two decades beyond the standard approaches of surgery, chemotherapy, and radiation. Immunotherapy is a modality complementary to conventional therapy,. It is appealing because functional anti-tumor activity could affect both local-regional and systemic disease and act over a prolonged period of time. In this report, we review immunotherapeutic investigative strategies being developed, including several tumor vaccine, antigen vaccine, and dendritic cell vaccine strategies. PMID:22401634

  3. Phase 1/2 study of immunotherapy with dendritic cells pulsed with autologous tumor lysate in patients with refractory bone and soft tissue sarcoma.

    Science.gov (United States)

    Miwa, Shinji; Nishida, Hideji; Tanzawa, Yoshikazu; Takeuchi, Akihiko; Hayashi, Katsuhiro; Yamamoto, Norio; Mizukoshi, Eishiro; Nakamoto, Yasunari; Kaneko, Shuichi; Tsuchiya, Hiroyuki

    2017-05-01

    There are limited options for the curative treatment of refractory bone and soft tissue sarcomas. The purpose of this phase 1/2 study was to assess the immunological and clinical effects of dendritic cells (DCs) pulsed with autologous tumor lysate (TL) in patients with advanced bone and soft tissue sarcomas. Thirty-seven patients with metastatic or recurrent sarcomas were enrolled in this study. Peripheral blood mononuclear cells obtained from the patients were suspended in media containing interleukin 4 (IL-4) and granulocyte-macrophage colony-stimulating factor. Subsequently, these cells were treated with TL, tumor necrosis factor α, and OK-432. The DCs were injected into the inguinal or axillary region. One treatment course comprised 6 weekly DC injections. The toxicity, clinical response (tumor volume, serum interferon-γ [IFN-γ], and serum IL-12), and oncological outcomes were observed. In total, 47 courses of DC therapy were performed in 37 patients. No severe adverse events or deaths associated with the DC injections were observed in the study patients. Increased serum IFN-γ and IL-12 levels were observed 1 month after the DC injection. Among the 37 patients, 35 patients were assessed for clinical responses: 28 patients showed tumor progression, 6 patients had stable disease, and 1 patient showed a partial response 8 weeks after the DC injection. The 3-year overall and progression-free survival rates of the patients were 42.3% and 2.9%, respectively. Although DC therapy appears safe and resulted in an immunological response in patients with refractory sarcoma, it resulted in an improvement of the clinical outcome in only a small number of patients. Cancer 2017;123:1576-1584. © 2017 American Cancer Society. © 2017 American Cancer Society.

  4. Osseous Kaposi sarcoma in an HIV-positive patient

    International Nuclear Information System (INIS)

    Thanos, Loukas; Mylona, Sofia; Kalioras, Vasilios; Pomoni, Maria; Batakis, Nikolaos

    2004-01-01

    A case of osseous Kaposi sarcoma in a 35-year-old man is described. The patient (HIV-positive for 8 years) suffered from cutaneous Kaposi sarcoma and presented with right-sided chest pain. He underwent a chest CT scan that revealed three osteolytic lesions involving rib and vertebra with large soft tissue masses, without cutaneous lesions at these sites. CT-guided core needle biopsy led to a histological diagnosis of Kaposi sarcoma. (orig.)

  5. Pediatric rhabdomyosarcomas and nonrhabdomyosarcoma soft tissue sarcoma

    OpenAIRE

    Agarwala Sandeep

    2006-01-01

    Tumors arising from the soft tissues are uncommon in children, accounting for about 6% of all childhood malignancies. More than half (53%) of these originate from the striated muscles and are called rhabdomyosarcomas (RMS) the remaining are nonrhabdomyosarcoma soft tissue sarcomas (NRSTS). Almost two-thirds of RMS cases are diagnosed in children < 6 years of age. They can arise at varied locations like the head and neck region, genitourinary tract, extremities, trunk and retrope...

  6. Correlation of the findings of thallium-201 chloride scans with those of other imaging modalities and histology following therapy in patients with bone and soft tissue sarcomas

    International Nuclear Information System (INIS)

    Kostakoglu, L.; Panicek, D.M.; Divgi, C.R.; Botet, J.; Healy, J.; Larson, S.M.; Abdel-Dayem, H.M.

    1995-01-01

    We performed a prospective study to evaluate the imaging potential of thallium-201 as compared with other imaging modalities in differentiating residual/recurrent tumors from post-therapy changes in patients with musculoskeletal sarcomas. 201 Tl scans, magnetic resonance imaging (17), X-ray computed tomography (6) or contrast angiography (6) studies in 29 patients previously treated for musculoskeletal sarcomas were correlated with either histopathologic findings (26 patients) or 2-year clinical follow-up (three patients). All imaging studies were acquired within 2 weeks. Ratios of 201 Tl tumor uptake to the contralateral (28 patients) or adjacent region of interest were calculated. When qualitative interpretation was in doubt, only those cases with a ratio of 1.5 or more were considered suggestive of recurrent or residual viable tumor tissue. Residual or recurrent tumor tissue was verified in 21 patients by biopsy. All had true-positive 201 Tl scans while the other imaging modalities were true-positive in 20 and equivocal in one. In eight patients, there was no evidence of viable tumor tissue as proven by biopsy in five and long-term clinical follow-up in three. 201 Tl scan was false-positive (ratio 1.5) in one patient and true-negative in seven while the other imaging modalities had four false-positives. The average 201 Tl ratios were 3.8 ± 1.1 in the true-positive cases and 1.3 ± 0.3 in the true-negative cases. The percentage sensitivities, specificities, and accuracy for 201 Tl were 100%, 87.5%, and 96.5% versus 95%, 50%, and 82.7% respectively for other imaging modalities. These results indicate that 201 Tl scintigraphy is more accurate than other imaging modalities in differentiating residual/recurrent musculoskeletal sarcomas from post-therapy changes. (orig.)

  7. Histologic response of soft tissue sarcoma to radiation therapy

    International Nuclear Information System (INIS)

    Willett, C.G.; Schiller, A.L.; Suit, H.D.; Mankin, H.J.; Rosenberg, A.

    1987-01-01

    Twenty-seven patients with soft tissue sarcoma had preoperative radiotherapy, limb-sparing marginal surgical resection and whole-mount tumor histologic analysis. Incisional biopsy specimens before radiotherapy were reviewed for tumor type, grade, and extent of necrosis. Preoperative radiotherapy was given in either of two regimens: 13 patients received a mean total dose of 5250 cGy in one daily 180 to 200 cGy fractions and 14 patients a mean total dose of 4770 cGy in two daily fractions of 180 to 200 cGy separated by 4 hours. Twenty-one specimens had at least 80% necrosis or severely altered cells, a 3+ to 4+ response. Grade and size of the tumor appeared to be indicators of response to treatment rather than histologic type. Three of five patients (60%) with Grade 1, eight of 11 patients (73%) with Grade 2 lesions, and ten of 11 patients (91%) with Grade 3 tumors had 80% or greater necrosis or severely altered cells. For tumors 10 cm or less in greatest diameter, the 3+ to 4+ histologic response was seen in 12 of 14 patients (86%) whereas for lesions greater than 10 cm, this response was observed in nine of 13 patients (69%). For patients with Grade 2 or 3 soft tissue sarcoma, 13 of 14 patients (93%) treated with two fractions per day and two of four patients (50%) receiving one fraction per day exhibited significant response. All six patients treated twice daily for lesions greater than 10 cm had 3+ to 4+ histologic response compared to three of seven (43%) patients treated once per day. Therefore, grade and size of soft tissue sarcoma are important predictors of response to radiotherapy and preoperative twice daily radiotherapy may more likely permit the conservative surgical excision of sarcomas of borderline resectability

  8. Histologic assessment of peritumoral edema in soft tissue sarcoma

    International Nuclear Information System (INIS)

    White, Lawrence M.; Wunder, Jay S.; Bell, Robert S.; O'Sullivan, Brian; Catton, Charles; Ferguson, Peter; Blackstein, Martin; Kandel, Rita A.

    2005-01-01

    Purpose: To evaluate whether satellite tumor cells can be identified histologically in the tissues surrounding a soft tissue sarcoma and whether their presence correlates with increased T 2 -weighted signal intensity on MRI. Methods and Materials: Fifteen patients with a high-grade extremity or truncal soft tissue sarcoma underwent preoperative MRI. The extent of high T 2 -weighted signal changes in the tissues surrounding tumor, thought to represent peritumoral edema/reactive changes, was determined. Twelve patients received i.v. gadolinium, and contrast enhancement was determined. All patients underwent surgical resection in the absence of preoperative chemotherapy or radiotherapy. The presence of tumor cells in the surrounding tissues was determined histologically in representative paraffin-embedded sections and correlated with the MRI findings. Results: The extent of peritumoral T 2 -weighted MRI signal changes ranged from 0 to 7.1 cm (mean, 2.5 cm); contrast enhancement ranged from 0 to 5.3 cm (mean, 1.1 cm). Sarcoma cells were identified histologically in the tissues beyond the tumor in 10 of 15 cases. In 6 cases, tumor cells were located within 1 cm of the tumor margin, and in 4 cases, malignant cells were found at a distance >1 cm and up to a maximum of 4 cm. The location of tumor cells beyond the margin did not correlate with tumor size nor did it correlate with the location or extent of peritumoral changes. Conclusion: The ability to identify tumor cells beyond the margin of a soft tissue sarcoma has important implications in planning appropriate targets for treatment. This could influence the use of new radiotherapy technologies such as intensity-modulated radiotherapy that aim to minimize treatment volumes through conformal planning

  9. Pediatric rhabdomyosarcomas and nonrhabdomyosarcoma soft tissue sarcoma

    Directory of Open Access Journals (Sweden)

    Agarwala Sandeep

    2006-01-01

    Full Text Available Tumors arising from the soft tissues are uncommon in children, accounting for about 6% of all childhood malignancies. More than half (53% of these originate from the striated muscles and are called rhabdomyosarcomas (RMS the remaining are nonrhabdomyosarcoma soft tissue sarcomas (NRSTS. Almost two-thirds of RMS cases are diagnosed in children < 6 years of age. They can arise at varied locations like the head and neck region, genitourinary tract, extremities, trunk and retroperitoneum. Pathologically RMS is now classified as superior, intermediate and poor outcome histologies. For stratification of treatment and also comparison of results the RMS are now staged both by the clinical grouping and the TNM staging systems. The ultimate outcome depends on the site, extent of disease and histology. Currently, approximately 70% of the patients survive for 5 years or more and are probably cured. This is credited to the use of multi-modal, risk-adapted therapy, refinements in tumor grouping and better supportive care which has emerged out of cooperative studies like Intergroup Rhabdomyosarcoma Study (IRS and the International Society of Pediatric Oncology studies (SIOP. The treatment involves chemotherapy, radiotherapy and organ/function preserving surgery. The gold standard chemotherapy is still vincristine, actinomycin D and cyclophosphamide (VAC regime with high doses of intensity bone marrow rescue with colony stimulating factors. The NRSTS are rare and of heterogenous histologies and so it has been difficult to arrive at a treatment strategy for these. What is definitely understood is that these are usually immature and poorly differentiated tumors that respond poorly to chemotherapy and so surgical resection forms the mainstay of treatment with adjuvant radiotherapy and chemotherapy to prevent local recurrences. In all likelihood, the molecular analysis of RMS will further refine current classification schemes and knowledge of genetic features of

  10. Indian data on bone and soft tissue sarcomas: A summary of published study results

    Directory of Open Access Journals (Sweden)

    Anant Ramaswamy

    2016-01-01

    Full Text Available Bone sarcomas are rare tumors, approximating 0.2% of all cancers, with osteosarcoma (OGS, chondrosarcoma, and Ewing sarcoma being the most common cancers in this subset. The formation of disease management groups/clinics focused on sarcomas has resulted in better understanding and management of these uncommon tumors. Multiple large-scale retrospective data from Tata Memorial Hospital (TMH and All India Institute of Medical Sciences have reported outcomes comparable to Western data in the field of OGS and Ewing sarcoma, with interesting prognostic factors identified for further evaluation. Soft tissue sarcomas are a rare heterogeneous group of tumors, more than 50 different tumor entities. The common subtypes identified in India include Ewing sarcoma and synovial sarcoma. Valuable work regarding brachytherapy has been done by radiation oncologists from the TMH, especially in pediatric patients.

  11. Intraoperative radiotherapy in the multidisciplinary management of soft tissue sarcomas

    International Nuclear Information System (INIS)

    Calvo, F.A.; Abuchaibe, O.; Escude, L.; Santos, M.; Villas, C.; Beguiristain, J.L.; Amillo, S.; Canadell, J.

    1989-01-01

    From 1984 to 1989 locally advanced stages of soft tissue sarcomas have been systematically approached with maximal surgical resection, IORT (10-20 Gy) boost to the tumor bed (high risk or residual disease areas) and conventional fractionated external beam postoperative radiotherapy. Chemotherapy was given to patients with high cellular grade sarcomas. Nineteen patients with localized soft tissue sarcomas of the extremities were treated and local control was obtained in 18/19 cases (95%). Distant metastasis developed in 2 patients and 15 patients are alive with no evidence of disease. The median follow-up time of the entire group is 27+ months (range 2+ to 43+ months). Severe IORT toxicity consisting in unreversible peripheral neuropathy has been observed in 3 patients. Another group of 24 patients had tumor located in central anatomical areas (3 head and neck, 7 trunk, 6 retroperitoneal, 3 glutens, 3 pelvis, 1 rectal, 1 bladder). Local control has been obtained in 15 patients (62%). Distant metastasis developed in 3 patients and 11 are alive and free of malignant disease. The median follow-up in this group is 14.5+ months (range from 4 to 50+ months). Severe neuropathy was observed in 1 patient. Severe neurological damage is a preoccupying finding that merits a word of caution in the development of IORT protocols, as what single dose can be selected in areas including nerves in treatment field

  12. Radiation-induced soft-tissue and bone sarcoma

    International Nuclear Information System (INIS)

    Kim, J.H.; Chu, F.C.; Woodard, H.Q.; Melamed, R.; Huvos, A.; Cantin, J.

    1978-01-01

    From the records of Memorial Hospital of the past 50 years, 47 cases with an established diagnosis of radiation-induced sarcoma were identified and divided into two groups: the first included 20 cases of soft-tissue sarcoma arising from irradiated tissues, and the second comprised 27 cases of bone sarcoma arising from normal bones in the irradiated field. Medians for the latent periods from irradiation to diagnosis of bone and soft-tissue sarcoma were 11 and 12, years, respectively. In bone sarcomas, the latent period was longer after larger radiation doses and children appeared to be more susceptible to cancer induction than adults. Criteria for establishing the diagnosis of radiation-induced sarcoma and the magnitude of the risk of bone sarcoma are discussed

  13. Patterns of Local Recurrence and Dose Fractionation of Adjuvant Radiation Therapy in 462 Patients With Soft Tissue Sarcoma of Extremity and Trunk Wall

    Energy Technology Data Exchange (ETDEWEB)

    Jebsen, Nina L., E-mail: nina.louise.jebsen@helse-bergen.no [Department of Clinical Medicine, Faculty of Medicine and Dentistry, University of Bergen, Bergen (Norway); Department of Oncology, Haukeland University Hospital, Bergen (Norway); Engellau, Jacob [Department of Oncology, Skåne University Hospital, Lund (Sweden); Engström, Katarina [Department of Oncology, Sahlgrenska University Hospital, Gothenburg (Sweden); Bauer, Henrik C. [Department of Molecular Medicine and Surgery, Section for Orthopaedics and Sports Medicine, Karolinska University Hospital, Karolinska Institute, Stockholm (Sweden); Monge, Odd R. [Department of Oncology, Haukeland University Hospital, Bergen (Norway); Muren, Ludvig P. [Department of Physics and Technology, University of Bergen, Bergen (Norway); Department of Medical Physics, Aarhus University and Aarhus University Hospital, Aarhus (Denmark); Eide, Geir E. [Centre for Clinical Research, Haukeland University Hospital, Bergen (Norway); Department of Public Health and Primary Health Care, University of Bergen, Bergen (Norway); Trovik, Clement S. [Department of Clinical Medicine, Faculty of Medicine and Dentistry, University of Bergen, Bergen (Norway); Department of Oncology, Haukeland University Hospital, Bergen (Norway); Bruland, Øyvind S. [Department of Oncology, The Norwegian Radium Hospital, Oslo University Hospital, Oslo (Norway); Institute of Clinical Medicine, University of Oslo, Oslo (Norway)

    2013-08-01

    Purpose: To study the impact of dose fractionation of adjuvant radiation therapy (RT) on local recurrence (LR) and the relation of LR to radiation fields. Methods and Materials: LR rates were analyzed in 462 adult patients with soft tissue sarcoma who underwent surgical excision and adjuvant RT at five Scandinavian sarcoma centers from 1998 to 2009. Medical records were reviewed for dose fractionation parameters and to determine the location of the LR relative to the radiation portals. Results: Fifty-five of 462 patients developed a LR (11.9%). Negative prognostic factors included intralesional surgical margin (hazard ratio [HR]: 7.83, 95% confidence interval [CI]: 3.08-20.0), high malignancy grade (HR: 5.82, 95% CI: 1.31-25.8), age at diagnosis (HR per 10 years: 1.27, 95% CI: 1.03-1.56), and malignant peripheral nerve sheath tumor histological subtype (HR: 6.66, 95% CI: 2.56-17.3). RT dose was tailored to margin status. No correlation between RT dose and LR rate was found in multiple Cox regression analysis. The majority (65%) of LRs occurred within the primary RT volume. Conclusions: No significant dose–response effect of adjuvant RT was demonstrated. Interestingly, patients given 45-Gy accelerated RT (1.8 Gy twice daily/2.5 weeks) had the best local outcome. A total dose of 50 Gy in 25 fractions seemed adequate following wide margin surgery. The risk of LR was associated with histopathologic subtype, which should be included in the treatment algorithm of adjuvant RT in soft tissue sarcoma.

  14. Patterns of Local Recurrence and Dose Fractionation of Adjuvant Radiation Therapy in 462 Patients With Soft Tissue Sarcoma of Extremity and Trunk Wall

    International Nuclear Information System (INIS)

    Jebsen, Nina L.; Engellau, Jacob; Engström, Katarina; Bauer, Henrik C.; Monge, Odd R.; Muren, Ludvig P.; Eide, Geir E.; Trovik, Clement S.; Bruland, Øyvind S.

    2013-01-01

    Purpose: To study the impact of dose fractionation of adjuvant radiation therapy (RT) on local recurrence (LR) and the relation of LR to radiation fields. Methods and Materials: LR rates were analyzed in 462 adult patients with soft tissue sarcoma who underwent surgical excision and adjuvant RT at five Scandinavian sarcoma centers from 1998 to 2009. Medical records were reviewed for dose fractionation parameters and to determine the location of the LR relative to the radiation portals. Results: Fifty-five of 462 patients developed a LR (11.9%). Negative prognostic factors included intralesional surgical margin (hazard ratio [HR]: 7.83, 95% confidence interval [CI]: 3.08-20.0), high malignancy grade (HR: 5.82, 95% CI: 1.31-25.8), age at diagnosis (HR per 10 years: 1.27, 95% CI: 1.03-1.56), and malignant peripheral nerve sheath tumor histological subtype (HR: 6.66, 95% CI: 2.56-17.3). RT dose was tailored to margin status. No correlation between RT dose and LR rate was found in multiple Cox regression analysis. The majority (65%) of LRs occurred within the primary RT volume. Conclusions: No significant dose–response effect of adjuvant RT was demonstrated. Interestingly, patients given 45-Gy accelerated RT (1.8 Gy twice daily/2.5 weeks) had the best local outcome. A total dose of 50 Gy in 25 fractions seemed adequate following wide margin surgery. The risk of LR was associated with histopathologic subtype, which should be included in the treatment algorithm of adjuvant RT in soft tissue sarcoma

  15. Adult Head and Neck Soft Tissue Sarcomas: Treatment and Outcome

    Directory of Open Access Journals (Sweden)

    Rabindra P. Singh

    2008-01-01

    Thirty-six patients were seen, of whom 24 were treated at this unit, the remainder only receiving advice. The median age of the patients was 46 years. Most of the sarcomas were deep and of high or intermediate grade with a median size of 5.5 cm. Eleven different histological subtypes were identified. Wide excision was possible only in 21% of the cases. 42% of the patients developed local recurrence and 42% developed metastatic disease usually in the lungs. Overall survival was 49% at 5 years. Tumour size was the most important prognostic factor. Adult head and neck soft tissue sarcomas have a high mortality rate with a high risk of local recurrence and metastatic disease. The rarity of the disease would suggest that centralisation of care could lead to increased expertise and better outcomes.

  16. Does an Algorithmic Approach to Using Brachytherapy and External Beam Radiation Result in Good Function, Local Control Rates, and Low Morbidity in Patients With Extremity Soft Tissue Sarcoma?

    Science.gov (United States)

    Klein, Jason; Ghasem, Alex; Huntley, Samuel; Donaldson, Nathan; Keisch, Martin; Conway, Sheila

    2018-03-01

    High-dose-rate brachytherapy (HDR-BT) and external-beam radiation therapy (EBRT) are two modalities used in the treatment of soft tissue sarcoma. Previous work at our institution showed early complications and outcomes for patients treated with HDR-BT, EBRT, or a combination of both radiation therapy modalities. As the general indications for each of these approaches to radiation therapy differ, it is important to evaluate the use of each in an algorithmic way, reflecting how they are used in contemporary practice at sites that use these treatments. QUESTION/PURPOSES: (1) To determine the proportions of intermediate- and long-term complications associated with the use of brachytherapy in the treatment of primary high-grade extremity soft tissue sarcomas; (2), to characterize the long-term morbidity of the three radiation treatment groups using the Radiation Therapy Oncology Group/ European Organization for Research and Treatment of Cancer (RTOG/EORTC) Late Radiation Morbidity Scoring Scheme; (3) to determine whether treatment with HDR-BT, EBRT, and HDR-BT+EBRT therapy, in combination with limb-salvage surgery, results in acceptable local control in this high-risk group of sarcomas. We retrospectively studied data from 171 patients with a diagnosis of high-grade extremity soft tissue sarcoma treated with limb-sparing surgery and radiation therapy between 1990 and 2012 at our institution, with a mean followup of 72 months. Of the 171 patients, 33 (20%) were treated with HDR-BT, 128 (75%) with EBRT, and 10 (6%) with HDR-BT+EBRT. We excluded 265 patients with soft tissue sarcomas owing to axial tumor location, previous radiation to the affected extremity, incomplete patient records, patients receiving primary amputation, recurrent tumors, pediatric patients, low- and intermediate-grade tumors, and rhabdoid histology. Fifteen patients (9%) were lost to followup for any reason including died of disease or other causes during the first 12 months postoperatively. This

  17. Primary Kaposi sarcoma of the subcutaneous tissue

    Directory of Open Access Journals (Sweden)

    Dezube Bruce J

    2008-09-01

    Full Text Available Abstract Background Involvement of the subcutis by Kaposi sarcoma (KS occurs primarily when cutaneous KS lesions evolve into deep penetrating nodular tumors. Primary KS of the subcutaneous tissue is an exceptional manifestation of this low-grade vascular neoplasm. Case presentation We present a unique case of acquired immune deficiency syndrome (AIDS-associated KS manifesting primarily in the subcutaneous tissue of the anterior thigh in a 43-year-old male, which occurred without overlying visible skin changes or concomitant KS disease elsewhere. Radiological imaging and tissue biopsy confirmed the diagnosis of KS. Conclusion This is the first documented case of primary subcutaneous KS occurring in the setting of AIDS. The differential diagnosis of an isolated subcutaneous lesion in an human immunodeficiency virus (HIV-infected individual is broad, and requires both imaging and a histopathological diagnosis to guide appropriate therapy.

  18. Pitfalls in soft tissue sarcoma imaging: chronic expanding hematomas.

    Science.gov (United States)

    Jahed, Kiarash; Khazai, Behnaz; Umpierrez, Monica; Subhawong, Ty K; Singer, Adam D

    2018-01-01

    Solid or nodular enhancement is typical of soft tissue sarcomas although high grade soft tissue sarcomas and those with internal hemorrhage often appear heterogeneous with areas of nonenhancement and solid or nodular enhancement. These MRI findings often prompt an orthopedic oncology referral, a biopsy or surgery. However, not all masses with these imaging findings are malignant. We report the multimodality imaging findings of two surgically proven chronic expanding hematomas (CEH) with imaging features that mimicked sarcomas. A third case of nonenhancing CEH of the lower extremity is also presented as a comparison. It is important that in the correct clinical scenario with typical imaging findings, the differential diagnosis of a chronic expanding hematoma be included in the workup of these patients. An image-guided biopsy of nodular tissue within such masses that proves to be negative for malignancy should not necessarily be considered discordant. A correct diagnosis may prevent a morbid unnecessary surgery and may indicate the need for a conservative noninvasive follow-up with imaging.

  19. Trabectedin for Soft Tissue Sarcoma: Current Status and Future Perspectives.

    Science.gov (United States)

    Gordon, Erlinda M; Sankhala, K Kumar; Chawla, Neal; Chawla, Sant P

    2016-07-01

    Trabectedin (ET743, Yondelis(®), manufactured by Baxter Oncology GmbH, Halle/Westfalen, Germany, for Janssen Products, LP, Horsham, PA), derived from the marine ascidian, Ecteinascidia turbinata, is a natural alkaloid with multiple complex mechanisms of action. On 23 October 2015, 15 years after the results of the first Phase 1 clinical trial using trabectedin for chemotherapy-resistant solid malignancies was reported, and 8 years after its approval in Europe, the United States Food and Drug Administration (USFDA) finally approved trabectedin for the treatment of unresectable or metastatic liposarcoma or leiomyosarcoma that has failed a prior anthracycline-containing regimen. Approval was based on the results of a pivotal Phase 3 trial involving a 2:1 randomization of 518 patients (who were further stratified by soft tissue sarcoma subtype), in which a significant improvement in progression-free survival was reported in the trabectedin-treated group vs. the dacarbazine-treated group (p concept. In short, trabectedin is an old new drug with proven potential to impact the lives of patients with soft tissue sarcoma and other solid malignancies. Sarcoma Oncology Center, Santa Monica, CA 90405.

  20. Oxidative stress and antioxidant status in primary bone and soft tissue sarcoma

    International Nuclear Information System (INIS)

    Nathan, Fatima M; Singh, Vivek A; Dhanoa, Amreeta; Palanisamy, Uma D

    2011-01-01

    Oxidative stress is characterised by an increased level of reactive oxygen species (ROS) that disrupts the intracellular reduction-oxidation (redox) balance and has been implicated in various diseases including cancer. Malignant tumors of connective tissue or sarcomas account for approximately 1% of all cancer diagnoses in adults and around 15% of paediatric malignancies per annum. There exists no information on the alterations of oxidant/antioxidant status of sarcoma patients in literature. This study was aimed to determine the levels of oxidative stress and antioxidant defence in patients with primary bone and soft tissue sarcoma and to investigate if there exists any significant differences in these levels between both the sarcomas. The study cohort consisted of 94 subjects; 20 soft tissue sarcoma, 27 primary bone sarcoma and 47 healthy controls. Malondialdehyde (MDA) and protein carbonyls were determined to assess their oxidative stress levels while antioxidant status was evaluated using catalase (CAT), superoxide dismutase (SOD), thiols and trolox equivalent antioxidant capacity (TEAC). Sarcoma patients showed significant increase in plasma and urinary MDA and serum protein carbonyl levels (p < 0.05) while significant decreases were noted in TEAC, thiols, CAT and SOD levels (p < 0.05). No significant difference in oxidative damage was noted between both the sarcomas (p > 0.05). In conclusion, an increase in oxidative stress and decrease in antioxidant status is observed in both primary bone and soft tissue sarcomas with a similar extent of damage. This study offers the basis for further work on whether the manipulation of redox balance in patients with sarcoma represents a useful approach in the design of future therapies for bone disease

  1. Pembrolizumab in advanced soft-tissue sarcoma and bone sarcoma (SARC028): a multicentre, two-cohort, single-arm, open-label, phase 2 trial.

    Science.gov (United States)

    Tawbi, Hussein A; Burgess, Melissa; Bolejack, Vanessa; Van Tine, Brian A; Schuetze, Scott M; Hu, James; D'Angelo, Sandra; Attia, Steven; Riedel, Richard F; Priebat, Dennis A; Movva, Sujana; Davis, Lara E; Okuno, Scott H; Reed, Damon R; Crowley, John; Butterfield, Lisa H; Salazar, Ruth; Rodriguez-Canales, Jaime; Lazar, Alexander J; Wistuba, Ignacio I; Baker, Laurence H; Maki, Robert G; Reinke, Denise; Patel, Shreyaskumar

    2017-11-01

    Patients with advanced sarcomas have a poor prognosis and few treatment options that improve overall survival. Chemotherapy and targeted therapies offer short-lived disease control. We assessed pembrolizumab, an anti-PD-1 antibody, for safety and activity in patients with advanced soft-tissue sarcoma or bone sarcoma. In this two-cohort, single-arm, open-label, phase 2 study, we enrolled patients with soft-tissue sarcoma or bone sarcoma from 12 academic centres in the USA that were members of the Sarcoma Alliance for Research through Collaboration (SARC). Patients with soft-tissue sarcoma had to be aged 18 years or older to enrol; patients with bone sarcoma could enrol if they were aged 12 years or older. Patients had histological evidence of metastatic or surgically unresectable locally advanced sarcoma, had received up to three previous lines of systemic anticancer therapy, had at least one measurable lesion according to the Response Evaluation Criteria In Solid Tumors version 1.1, and had at least one lesion accessible for biopsy. All patients were treated with 200 mg intravenous pembrolizumab every 3 weeks. The primary endpoint was investigator-assessed objective response. Patients who received at least one dose of pembrolizumab were included in the safety analysis and patients who progressed or reached at least one scan assessment were included in the activity analysis. Accrual is ongoing in some disease cohorts. This trial is registered with ClinicalTrials.gov, number NCT02301039. Between March 13, 2015, and Feb 18, 2016, we enrolled 86 patients, 84 of whom received pembrolizumab (42 in each disease cohort) and 80 of whom were evaluable for response (40 in each disease cohort). Median follow-up was 17·8 months (IQR 12·3-19·3). Seven (18%) of 40 patients with soft-tissue sarcoma had an objective response, including four (40%) of ten patients with undifferentiated pleomorphic sarcoma, two (20%) of ten patients with liposarcoma, and one (10%) of ten patients

  2. Synovial sarcoma: MR evaluation in 23 patients

    International Nuclear Information System (INIS)

    Galant, J.; Marti-Bonmati, L.; Lafuente, J.; Hernandez, L.; Soler, R.; Saez, F.

    1997-01-01

    The synovial sarcoma is one of the most common soft tissue sarcomas. MR is the technique of choice to determine to local extension of malignant soft tissue tumors. To assess the clinical and MR imaging parameters associated with synovial sarcomas that aid in establishing their diagnosis. We review the clinical findings and images of 23 histologically confirmed synovial sarcomas that were studied by MR. Synovial sarcomas usually develop in young adults as soft tissue tumors, preferentially in the deep tissues of an extremity in close proximity to a joint. They are characterized as having a lobulated contour and septa, frequently infiltrating neighboring tissues at some point, and are heterogeneous. The presence of hemorrhage, as well as infiltration of the fascia in subcutaneous tumors, suggests the diagnosis of synovial sarcoma. The development of perilesional edema is not uncommon. Although, logically, the clinical and radiological features of synovial sarcomas can overlap with those of other soft tissue tumors, the findings described here are fairly characteristic of these lesions: thus, when present, they should serve to orient the diagnostic process. (Author) 16 refs

  3. The Edinburgh experience of treating sarcomas of soft tissues and bone with neutron irradiation

    International Nuclear Information System (INIS)

    Duncan, W.; Arnott, S.J.; Jack, W.J.L.

    1986-01-01

    The experience of treating 30 patients with sarcomas of soft tissue and bone with d(15)+Be neutron irradiation is reported. The local control of measurable soft-tissue sarcomas was 38.5% (minimum follow-up 2 years), which is similar to that expected after photon therapy. The radiation morbidity was unacceptably high (50%). Bone tumours did not respond well; in only one out of nine was lasting local tumour control achieved. (author)

  4. Primary retroperitoneal soft tissue sarcoma: Imaging appearances, pitfalls and diagnostic algorithm.

    Science.gov (United States)

    Messiou, C; Moskovic, E; Vanel, D; Morosi, C; Benchimol, R; Strauss, D; Miah, A; Douis, H; van Houdt, W; Bonvalot, S

    2017-07-01

    Although retroperitoneal sarcomas are rare tumours, they can be encountered by a wide variety of clinicians as they can be incidental findings on imaging or present with non specific symptoms and signs. Surgical resection can offer hope of cure and patient outcomes are improved when patients are managed in high-volume specialist centers. Failure to recognize retroperitoneal sarcomas on imaging can lead to inappropriate management in inexperienced centers. Therefore it is critical that a diagnosis of retroperitoneal sarcoma should be considered in the differential diagnosis of a retroperitoneal mass with prompt referral to a soft tissue sarcoma unit. In particular, the most common retroperitoneal sarcoma subtypes, liposarcoma and leiomyosarcoma, have characteristic imaging appearances which are discussed. This review therefore aims to set the context and guide clinicians through a diagnostic pathway for retroperitoneal masses in adults which arise extrinsic to the solid abdominal viscera. Copyright © 2016 The Authors. Published by Elsevier Ltd.. All rights reserved.

  5. Peripheral soft tissue ewing's sarcoma: a rare case report

    Directory of Open Access Journals (Sweden)

    Farzana Shegufta

    2013-07-01

    Full Text Available A 22 years male patient presented with gradual left forearm swelling for 6 months. X ray forearm revealed large soft tissue swelling with tiny calcification and mild scalloping at inner aspect of ulna and ultrasonogram (USG revealed soft tissue mass having calcification and necrotic areas within and spectral Doppler showed arterial type of blood flow with no augmentation. Later computerized tomography (CT scan showed soft tissue mass with necrotic area and calcification with no bony involvement. Magnetic resonance imaging (MRI with contrast revealed a large heterogeneously enhancing lobulated mixed intensity lesion in antero-medial compartment of the left forearm involving flexor group of muscles causing displacement of fat plane. MRI and subsequent histopathology of the lesion revealed it as a rare soft tissue Ewing’s sarcoma / primitive neuroectodermal tumor (PNET in extremity. Ibrahim Med. Coll. J. 2013; 7(2: 43-46

  6. Radiation-induced pseudotumor following therapy for soft tissue sarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Moore, Lacey F.; Kransdorf, Mark J. [Mayo Clinic, Department of Radiology, Jacksonville, FL (United States); Buskirk, Steven J. [Mayo Clinic, Department of Radiation Oncology, Jacksonville, FL (United States); O' Connor, Mary I. [Mayo Clinic, Department of Orthopedic Surgery, Jacksonville, FL (United States); Menke, David M. [Mayo Clinic, Department of Pathology, Jacksonville, FL (United States)

    2009-06-15

    The purpose of this study was to describe the prevalence and imaging appearance of radiation induced pseudotumors in patients following radiation therapy for extremity soft tissue sarcomas. We retrospectively reviewed the serial magnetic resonance (MR) images of 24 patients following radiation therapy for extremity soft tissue sarcomas. A total of 208 exams were reviewed (mean, 8.7 exams per patient) and included all available studies following the start of radiation therapy. Exams were analyzed for the identification of focal signal abnormalities within the surgical bed suggesting local tumor recurrence. Histopathologic correlation was available in nine patients suspected of having local tumor recurrence. Additional information recorded included patient demographics, tumor type and location, radiation type, and dose. The study group consisted of 12 men and 12 women, having an average age of 63 years (range, 39-88 years). Primary tumors were malignant fibrous histiocytoma (n = 13), leiomyosarcoma (n = 6), liposarcoma (n = 3), synovial sarcoma (n = 1), and extraskeletal chondrosarcoma (n = 1). All lesions were high-grade sarcomas, except for two myxoid liposarcomas. Average patient radiation dose was 5,658 cGy (range, 4,500-8,040 cGy). Average follow-up time was 63 months (range, 3-204 months). Focal signal abnormalities suggesting local recurrence were seen in nine (38%) patients. Three of the nine patients with these signal abnormalities were surgically proven to have radiation-induced pseudotumor. The pseudotumors developed between 11 and 61 months following the initiation of radiation therapy (mean, 38 months), with an average radiation dose of 5,527 cGy (range, 5,040-6,500 cGy). MR imaging demonstrated a relatively ill-defined ovoid focus of abnormal signal and intense heterogeneous enhancement with little or no associated mass effect. MR imaging of radiation-induced pseudotumor typically demonstrates a relatively ill-defined ovoid mass-like focus of intense

  7. Light ion irradiation for unfavorable soft tissue sarcoma

    International Nuclear Information System (INIS)

    Linstadt, D.; Castro, J.R.; Phillips, T.L.; Petti, P.L.; Collier, J.M.; Daftari, I.; Schoethaler, R.; Rayner, A.

    1990-09-01

    Between 1978 and 1989, 32 patients with unfavorable soft tissue sarcoma underwent light ion (helium, neon) irradiation with curative intent at Lawrence Berkeley Laboratory. The tumors were located in the trunk in 22 patients and head and neck in 10. Macroscopic tumor was present in 22 at the time of irradiation. Two patients had tumors apparently induced by previous therapeutic irradiation. Follow-up times for surviving patients ranged from 4 to 121 months (median 27 months). The overall 3-year actuarial local control rate was 62%; the corresponding survival rate was 50%. The 3-year actuarial control rate for patients irradiated with macroscopic tumors was 48%, while none of the patients with microscopic disease developed local recurrence (100%). The corresponding 3-year actuarial survival rates were 40% (macroscopic) and 78% (microscopic). Patients with retroperitoneal sarcoma did notably well; the local control rate and survival rate were 64% and 62%, respectively. Complications were acceptable; there were no radiation related deaths, while two patients (6%) required operations to correct significant radiation-related injuries. These results appear promising compared to those achieved by low -LET irradiation, and suggest that this technique merits further investigation

  8. Synovial sarcoma

    Directory of Open Access Journals (Sweden)

    Sucari S.C. Vlok

    2014-12-01

    Full Text Available Synovial sarcoma is a malignant, predominantly juxta-articular, soft-tissue tumour representing approximately 10% of all soft-tissue sarcomas. Frequently initially incorrectly diagnosed as a benign lesion, it should be considered as a diagnosis when a young adult patient presents with a calcified juxta-articular soft-tissue mass of insidious onset.

  9. A phase IIb multicentre study comparing the efficacy of trabectedin to doxorubicin in patients with advanced or metastatic untreated soft tissue sarcoma: The TRUSTS trial

    NARCIS (Netherlands)

    Bui-Nguyen, B.; Butrynski, J.E.; Penel, N.; Blay, J.Y.; Isambert, N.; Milhem, M.; Kerst, J.M.; Reyners, A.K.; Litiere, S.; Marreaud, S.; Collin, F.; Graaf, W.T.A. van der

    2015-01-01

    PURPOSE: To evaluate whether trabectedin as first-line chemotherapy for advanced/metastatic soft tissue sarcoma prolongs progression-free survival (PFS), compared to doxorubicin and, in the phase IIb part here, to select the most appropriate trabectedin treatment schedule (3-hour or 24-hour

  10. Amputations for extremity soft tissue sarcoma in an era of limb salvage treatment : Local control and survival

    NARCIS (Netherlands)

    Stevenson, Marc G; Musters, Annelie H; Geertzen, Jan H B; van Leeuwen, Barbara L; Hoekstra, Harald J; Been, Lukas B

    2018-01-01

    BACKGROUND: Despite multimodality limb salvage treatment (LST) for locally advanced extremity soft tissue sarcoma (ESTS), some patients still need an amputation. Indications for amputation and oncological outcome for these patients are described. METHODS: Between 1996 and 2016, all patients who

  11. Characteristics of 64 sarcoma patients referred to a sarcoma center after unplanned excision.

    Science.gov (United States)

    Dyrop, Heidi Buvarp; Safwat, Akmal; Vedsted, Peter; Maretty-Kongstad, Katja; Hansen, Bjarne Hauge; Jørgensen, Peter Holmberg; Baad-Hansen, Thomas; Keller, Johnny

    2016-02-01

    Unplanned excision of sarcoma before referral to specialist centers can affect prognosis and surgical outcome. The diagnostic pathway of these patients is uncertain and needs to be reviewed. We aimed to describe patient and tumor characteristics, initial symptoms, initial and final diagnosis, and explore reasons for unplanned excision in this patient group. From a previous study on 258 sarcoma patients, we identified 64 patients referred after surgery. Medical records were reviewed. The majority were soft tissue sarcomas, most often with thoracic location. Leiomyosarcoma was the most frequent final diagnosis, lipoma, and fibroma/dermatofibroma the most frequent initial diagnoses. Fifty percent were superficial small tumors, and 60.9% had not received diagnostic imaging before surgery. Fifty percent were referred from public surgical departments, and 1/3 from private specialists. Twenty-three patients had initial presence of alarm symptoms registered before surgery, the remaining 2/3 fell outside referral criteria or alarm symptoms were not discovered. Patients referred after unplanned excision often have small superficial tumors and the majority fall outside of defined referral criteria. Referral criteria are not a guarantee for detection of all sarcomas and surgeons should always be aware of the possibility of malignancy when removing a tumor. © 2016 Wiley Periodicals, Inc.

  12. Hypoxia-Inducible Factors: Mediators of Cancer Progression; Prognostic and Therapeutic Targets in Soft Tissue Sarcomas

    International Nuclear Information System (INIS)

    Sadri, Navid; Zhang, Paul J.

    2013-01-01

    Soft-tissue sarcomas remain aggressive tumors that result in death in greater than a third of patients due to either loco-regional recurrence or distant metastasis. Surgical resection remains the main choice of treatment for soft tissue sarcomas with pre- and/or post-operational radiation and neoadjuvant chemotherapy employed in more advanced stage disease. However, in recent decades, there has been little progress in the average five-year survival for the majority of patients with high-grade soft tissue sarcomas, highlighting the need for improved targeted therapeutic agents. Clinical and preclinical studies demonstrate that tumor hypoxia and up-regulation of hypoxia-inducible factors (HIFs) is associated with decreased survival, increased metastasis, and resistance to therapy in soft tissue sarcomas. HIF-mediated gene expression regulates many critical aspects of tumor biology, including cell survival, metabolic programming, angiogenesis, metastasis, and therapy resistance. In this review, we discuss HIFs and HIF-mediated genes as potential prognostic markers and therapeutic targets in sarcomas. Many pharmacological agents targeting hypoxia-related pathways are in development that may hold therapeutic potential for treating both primary and metastatic sarcomas that demonstrate increased HIF expression

  13. Four year experience of sarcoma of soft tissues and bones in a tertiary care hospital and review of literature

    Directory of Open Access Journals (Sweden)

    Ansari Tayyaba Z

    2011-05-01

    Full Text Available Abstract Background Sarcoma encompasses an uncommon group of cancer and the data is insufficient from Pakistan. We report our four years experience of Sarcoma of soft tissues and bones. Methods This cross sectional study was carried out at Aga Khan University Hospital from 2004 to 2008. The patients were divided into two groups from the outset i.e. initially diagnosed and relapsed group and separate sub group analysis was conducted. Results Out of 93 newly diagnosed patients, 58 belonged to bone sarcoma and 35 to soft tissue sarcoma group. While for relapsed patients, 5 had soft tissue sarcoma and 9 had bone sarcoma. Mean age was 32.5 years. At presentation, approximately two third patients had localised disease while remaining one third had metastatic disease. The Kaplan Meier estimate of median recurrence free survival was 25 months, 35 months, and 44 months for Osteogenic sarcoma, Ewing's sarcoma and Chondrosarcoma respectively. For Leiomyosarcoma and Synovial sarcoma, it was 20 and 19 months respectively. The grade of the tumour (p = 0.02 and surgical margin status (p = 0.001 were statistically significant for determination of relapse of disease. Conclusion The median recurrence free survival of patients in our study was comparable to the reported literature but with significant lost to follow rate. Further large-scale, multi centre studies are needed to have a more comprehensive understanding of this heterogeneous disease in our population.

  14. Prediction of chemotherapy outcome in patients with metastatic soft tissue sarcomas based on dynamic FDG PET (dPET) and a multiparameter analysis

    International Nuclear Information System (INIS)

    Dimitrakopoulou-Strauss, Antonia; Strauss, Ludwig G.; Vasamiliette, Julie; Egerer, Gerlinde; Schmitt, Thomas; Kasper, Bernd; Haberkorn, Uwe

    2010-01-01

    Dynamic PET studies with 18 F-FDG were performed in patients with metastatic soft tissue sarcomas who received conventional chemotherapy with doxorubicin hydrochloride (Adriamycin) and ifosfamide (AI-G). The goal of the study was to evaluate the impact of full kinetic analysis and assess its value with regard to the therapy outcome based on survival data. The evaluation included 17 patients with 29 metastatic lesions of soft tissue sarcomas, who were treated with chemotherapy consisting of an AI-G regimen prior to high-dose chemotherapy and peripheral blood stem cell transplantation where applicable. Patients were examined prior to onset of therapy and after completion of the first cycle of AI-G. Restaging data (n = 17) based on Response Evaluation Criteria in Solid Tumors were available. Survival data (n = 14) served for reference. The following parameters were retrieved from the dynamic PET studies: standardized uptake value (SUV), fractal dimension, two-compartment model with computation of k1, k2, k3, k4 (unit: 1/min), the fractional blood volume and the FDG influx calculated according to Patlak. The mean SUV was 6.9 prior to therapy and 4.7 after one cycle. The mean influx was 0.066 prior to therapy in comparison to 0.058 after one cycle. We dichotomized the patients according to the median survival time of 320 days into response (n = 6) and non-response (n = 8). The mean SUV was 7.6 in the group of responders and 5.4 in the group of non-responders prior to therapy. Responders revealed a mean SUV of 3.8 after therapy as compared to 5.0 SUV for non-responders. We used discriminant analysis to classify the patients into the two response groups. The classification of the non-responders was generally higher (negative predictive value > 61%) than for the responders. Finally, the combined use of the four predictor variables, namely mean SUV and k1 of both studies led to the highest accuracy of 90% for both groups. The data demonstrate that only a multiparameter

  15. Radiological and pathological response following pre-operative radiotherapy for soft-tissue sarcoma

    International Nuclear Information System (INIS)

    Roberge, David; Skamene, Tanya; Nahal, Ayoub; Turcotte, Robert E.; Powell, Tom; Freeman, Carolyn

    2010-01-01

    Purpose: To report radiological and pathological response to neo-adjuvant radiotherapy for extremity and trunk soft-tissue sarcomas. Materials/methods: Fifty patients were identified retrospectively. All patients had MRI imaging pre and post neo-adjuvant external beam radiotherapy. Tumor volumes were measured in 3D on T1 Gadolinium enhanced sequences. Pathological treatment response was quantified in terms of percentage of treatment-related necrosis for each case. Results: Histopathologic responses to treatment varied from 0% to 100%. The median pathological treatment response was 67.5% for low-grade sarcomas and 50% for high-grade sarcomas. The median decrease in tumor volume was 13.8% for non-myxoid low-grade sarcomas, 82.1% for myxoid liposarcomas and <1% for high-grade sarcomas. A partial response on MRI (volume reduction ≥ 50%) was highly predictive of a good pathological response (p < 0.001). Patients with stable disease on imaging or volumetric progression had wide ranging pathological responses. Conclusions: Soft-tissue sarcomas show significant pathological treatment responses in the form of hyaline fibrosis, necrosis and granulation tissue. Despite this, there is minimal early volumetric response to radiation, especially for high-grade tumors. Although radiological partial response was predictive of pathological response, the significance of radiological progression was unclear. Myxoid liposarcoma tumor type was predictive of both pathological and radiological tumor response.

  16. Adherence to treatment guidelines for primary sarcomas affects patient survival: a side study of the European CONnective TIssue CAncer NETwork (CONTICANET).

    Science.gov (United States)

    Rossi, C R; Vecchiato, A; Mastrangelo, G; Montesco, M C; Russano, F; Mocellin, S; Pasquali, S; Scarzello, G; Basso, U; Frasson, A; Pilati, P; Nitti, D; Lurkin, A; Ray-Coquard, I

    2013-06-01

    The impact of adherence to clinical practice guidelines (CPGs) for loco-regional treatment (i.e. surgery and radiotherapy) and chemotherapy on local disease control and survival in sarcoma patients was investigated in a European study conducted in an Italian region (Veneto). The completeness of the adherence to the Italian CPGs for sarcomas treatment was assessed by comparing the patient's charts and the CPGs. Propensity score-adjusted multivariate survival analysis was used to assess the impact of CPGs adherence on patient clinical outcomes. A total of 151 patients were included. Adherence to CPGs for loco-regional therapy and chemotherapy was observed in 106 out of 147 (70.2%) and 129 out of 139 (85.4%) patients, respectively. Non-adherence to CPGs for loco-regional treatment was independently associated with AJCC stage III disease [odds ratio (OR) 1.77, P = 0.011] and tumor-positive excision margin (OR 3.55, P = 0.003). Patients not treated according to the CPGs were at a higher risk of local recurrence [hazard ratio (HR) 5.4, P < 0.001] and had a shorter sarcoma-specific survival (HR 4.05, P < 0.001), independently of tumor stage. Incomplete adherence to CPGs for loco-regional treatment of sarcomas was associated with worse prognosis in patients with non-metastatic tumors.

  17. Expression of multidrug resistance genes MVP, MDR1, and MRP1 determined sequentially before, during, and after hyperthermic isolated limb perfusion of soft tissue sarcoma and melanoma patients.

    Science.gov (United States)

    Stein, Ulrike; Jürchott, Karsten; Schläfke, Matthias; Hohenberger, Peter

    2002-08-01

    Isolated, hyperthermic limb perfusion (ILP) with recombinant human tumor necrosis factor alpha and melphalan is a highly effective treatment for advanced soft tissue sarcoma (STS) and locoregional metastatic malignant melanoma. Multidrug resistance (MDR)-associated genes are known to be inducible by heat and drugs; expression levels of the major vault protein (MVP), MDR1, and MDR-associated protein 1 (MRP1) were determined sequentially before, during, and after ILP of patients. Twenty-one STS or malignant melanoma patients were treated by ILP. Tumor tissue temperatures were recorded continuously and ranged from 33.4 degrees C initially to peak values of 40.4 degrees C during ILP. Serial true-cut biopsy specimens from tumor tissues were routinely microdissected. Expression analyses for MDR genes were performed by real-time reverse transcriptase polymerase chain reaction and immunohistochemistry. In 83% of the patients, MVP expression was induced during hyperthermic ILP. MVP-mRNA inductions often paralleled the increase in temperature during ILP. Increased MVP protein expressions either were observed simultaneously with the MVP-mRNA induction or were delayed until after the induction at the transcriptional level. Inductions of MDR1 and MRP1 were observed in only 13% and 27% of the specimens analyzed. Temperatures and drugs applied preferentially led to an induction of MVP and were not sufficient to induce MDR1 and MRP1 in the majority of tumors. This study is the first to analyze the expression of MDR-associated genes sequentially during ILP of patients and demonstrates that treatment might lead to increased levels of MVP, whereas enhanced levels of MDR1 and MRP1 remain rare events.

  18. Assessing the reading level of online sarcoma patient education materials.

    Science.gov (United States)

    Patel, Shaan S; Sheppard, Evan D; Siegel, Herrick J; Ponce, Brent A

    2015-01-01

    Cancer patients rely on patient education materials (PEMs) to gather information regarding their disease. Patients who are better informed about their illness have better health outcomes. The National Institutes of Health (NIH) recommends that PEMs be written at a sixth- to seventh-grade reading level. The purpose of this study was to evaluate the readability of online PEMs of bone and soft-tissue sarcomas and related conditions. We identified relevant online PEMs from the following websites: American Academy of Orthopaedic Surgeons, academic training centers, sarcoma specialists, Google search hits, Bonetumor.org, Sarcoma Alliance, Sarcoma Foundation of America, and Medscape. We used 10 different readability instruments to evaluate the reading level of each website's PEMs. In assessing 72 websites and 774 articles, we found that none of the websites had a mean readability score at or below 7 (seventh grade). Collectively, all websites had a mean readability score of 11.4, and the range of scores was grade level 8.9 to 15.5. None of the PEMs in this study of bone and soft-tissue sarcomas and related conditions met the NIH recommendation for PEM reading levels. Concerted efforts to improve the reading level of orthopedic oncologic PEMs are necessary.

  19. Therapeutic Angiotensin-(1-7) in Treating Patients With Metastatic Sarcoma That Cannot Be Removed By Surgery

    Science.gov (United States)

    2018-02-27

    Bone Cancer; Chondrosarcoma; Clear Cell Sarcoma of the Kidney; Metastatic Osteosarcoma; Ovarian Sarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Osteosarcoma; Recurrent Uterine Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage III Uterine Sarcoma; Stage IV Adult Soft Tissue Sarcoma; Stage IV Uterine Sarcoma

  20. Cytokine-induced killer cells eradicate bone and soft-tissue sarcomas.

    Science.gov (United States)

    Sangiolo, Dario; Mesiano, Giulia; Gammaitoni, Loretta; Leuci, Valeria; Todorovic, Maja; Giraudo, Lidia; Cammarata, Cristina; Dell'Aglio, Carmine; D'Ambrosio, Lorenzo; Pisacane, Alberto; Sarotto, Ivana; Miano, Sara; Ferrero, Ivana; Carnevale-Schianca, Fabrizio; Pignochino, Ymera; Sassi, Francesco; Bertotti, Andrea; Piacibello, Wanda; Fagioli, Franca; Aglietta, Massimo; Grignani, Giovanni

    2014-01-01

    Unresectable metastatic bone sarcoma and soft-tissue sarcomas (STS) are incurable due to the inability to eradicate chemoresistant cancer stem-like cells (sCSC) that are likely responsible for relapses and drug resistance. In this study, we investigated the preclinical activity of patient-derived cytokine-induced killer (CIK) cells against autologous bone sarcoma and STS, including against putative sCSCs. Tumor killing was evaluated both in vitro and within an immunodeficient mouse model of autologous sarcoma. To identify putative sCSCs, autologous bone sarcoma and STS cells were engineered with a CSC detector vector encoding eGFP under the control of the human promoter for OCT4, a stem cell gene activated in putative sCSCs. Using CIK cells expanded from 21 patients, we found that CIK cells efficiently killed allogeneic and autologous sarcoma cells in vitro. Intravenous infusion of CIK cells delayed autologous tumor growth in immunodeficient mice. Further in vivo analyses established that CIK cells could infiltrate tumors and that tumor growth inhibition occurred without an enrichment of sCSCs relative to control-treated animals. These results provide preclinical proof-of-concept for an effective strategy to attack autologous sarcomas, including putative sCSCs, supporting the clinical development of CIK cells as a novel class of immunotherapy for use in settings of untreatable metastatic disease.

  1. Surgical considerations when reporting MRI studies of soft tissue sarcoma of the limbs

    Energy Technology Data Exchange (ETDEWEB)

    De la Hoz Polo, Marcela [Kings College Hospital, Radiology Department, London, Brixton (United Kingdom); Dick, Elizabeth [St Mary' s Hospital, Imperial College Healthcare NHS Trust, Radiology Department, London (United Kingdom); Bhumbra, Rej [Newham and The Royal London Hospitals, Trauma and Orthopaedic Department, Barts Health Orthopaedic Centre, London, Whitechapel (United Kingdom); Pollock, Rob [Royal National Orthopaedic Hospital, Trauma and Orthopaedic Department, Stanmore (United Kingdom); Sandhu, Ranbir [University Hospitals Birmingham, Radiology Department, Queen Elizabeth Hospital, Birmingham (United Kingdom); Saifuddin, Asif [Royal National Orthopaedic Hospital, Radiology Department, Stanmore (United Kingdom)

    2017-12-15

    Soft tissue sarcomas (STS) are rare tumours that require prompt diagnosis and treatment at a specialist centre. Magnetic resonance imaging (MRI) has become the modality of choice for identification, characterisation, biopsy planning and staging of soft tissue masses. MRI enables both the operating surgeon and patient to be optimally prepared prior to surgery for the likelihood of margin-negative resection and to anticipate possible sacrifice of adjacent structures and consequent loss of function. The aim of this review is to aid the radiologist in performing and reporting MRI studies of soft tissue sarcomas, with particular reference to the requirements of the surgical oncologist. (orig.)

  2. Results of a phase I dose escalation study of eltrombopag in patients with advanced soft tissue sarcoma receiving doxorubicin and ifosfamide

    International Nuclear Information System (INIS)

    Chawla, Sant P; Staddon, Arthur; Hendifar, Andrew; Messam, Conrad A; Patwardhan, Rita; Kamel, Yasser Mostafa

    2013-01-01

    The objective of this Phase I dose escalation study was to explore the safety and tolerability of eltrombopag, an oral, nonpeptide, thrombopoietin receptor agonist, in patients with advanced soft tissue sarcoma (STS) and thrombocytopenia due to treatment with doxorubicin and ifosfamide (AI) combination chemotherapy. Patients aged 18 or older with histologically confirmed, locally advanced or metastatic STS were treated with 1 cycle of AI followed by AI with eltrombopag starting at Cycle 2, using 2 different dosing schedules. The study design included an eltrombopag dose escalation phase starting at 75 mg daily to determine the optimal biological dose (OBD). Eighteen patients were enrolled and 15 received at least 1 dose of chemotherapy; 3 patients withdrew prior to receiving eltrombopag. Seven, 4, and 1 patients received 75 mg, 100 mg, and 150 mg eltrombopag daily, respectively. No dose-limiting toxicities were reported. Due to slow recruitment, the study was closed prior to identifying an OBD. The most common hematologic adverse events (AEs) were thrombocytopenia (80%), neutropenia (73%), and anemia (67%). The most common nonhematologic AEs were fatigue (53%), alanine aminotransferase increased, constipation, and nausea (47% each). Eleven of 12 patients who received eltrombopag completed at least 2 chemotherapy cycles; all had increased platelet counts on Day 1 of Cycle 2 (cycle with eltrombopag) compared to Day 1 of Cycle 1 (cycle without eltrombopag). Although data are limited, safety data were consistent with the known toxicities of AI combination chemotherapy or the side effect profile of eltrombopag seen in other studies. Available data suggest a potential pre- and post-chemotherapy dosing scheme for eltrombopag when administered with AI chemotherapy, and support further investigation of eltrombopag treatment in patients with chemotherapy-induced thrombocytopenia

  3. Singapore Cancer Network (SCAN) Guidelines for the Initial Evaluation, Diagnosis, and Management of Extremity Soft Tissue Sarcoma and Osteosarcoma.

    Science.gov (United States)

    2015-10-01

    The SCAN sarcoma workgroup aimed to develop Singapore Cancer Network (SCAN) clinical practice guidelines for the initial evaluation, diagnosis, and management of extremity soft tissue sarcoma and osteosarcoma. The workgroup utilised a consensus approach to create high quality evidence-based clinical practice guidelines suited for our local setting. Various international guidelines from the fields of radiology, pathology, orthopaedic surgery, medical, radiation and paediatric oncology were reviewed, including those developed by von Mehren Metal (J Natl Compr Canc Netw 2014), the National Collaborating Centre for Cancer (2006), the European Sarcoma Network Working Group (2012) and Grimer RJ et al (Sarcoma 2008). Our clinical practice guidelines contextualised to the local patient will streamline care and improve clinical outcomes for patients with extremity soft tissue and osteosarcoma. These guidelines form the SCAN Guidelines 2015 for the initial evaluation, diagnosis, and management of extremity soft tissue sarcoma and osteosarcoma.

  4. Histology-specific therapy for advanced soft tissue sarcoma and benign connective tissue tumors.

    Science.gov (United States)

    Silk, Ann W; Schuetze, Scott M

    2012-09-01

    Molecularly targeted agents have shown activity in soft tissue sarcoma (STS) and benign connective tissue tumors over the past ten years, but response rates differ by histologic subtype. The field of molecularly targeted agents in sarcoma is increasingly complex. Often, clinicians must rely on phase II data or even case series due to the rarity of these diseases. In subtypes with a clear role of specific factors in the pathophysiology of disease, such as giant cell tumor of the bone and diffuse-type tenosynovial giant cell tumor, it is reasonable to treat with newer targeted therapies, when available, in place of chemotherapy when systemic treatment is needed to control disease. In diseases without documented implication of a pathway in disease pathogenesis (e.g. soft tissue sarcoma and vascular endothelial growth factor), clear benefit from drug treatment should be established in randomized phase III trials before implementation into routine clinical practice. Histologic subtype will continue to emerge as a critical factor in treatment selection as we learn more about the molecular drivers of tumor growth and survival in different subtypes. Many of the drugs that have been recently developed affect tumor growth more than survival, therefore progression-free survival may be a more clinically relevant intermediate endpoint than objective response rate using Response Evaluation Criteria In Solid Tumors (RECIST) in early phase sarcoma trials. Because of the rarity of disease and increasing need for multidisciplinary management, patients with connective tissue tumors should be evaluated at a center with expertise in these diseases. Participation in clinical trials, when available, is highly encouraged.

  5. Oncological outcomes of patients with Ewing's sarcoma: is there a difference between skeletal and extra-skeletal Ewing's sarcoma?

    Science.gov (United States)

    Pradhan, A; Grimer, R J; Spooner, D; Peake, D; Carter, S R; Tillman, R M; Abudu, A; Jeys, L

    2011-04-01

    The aim of this study was to identify whether there was any difference in patient, tumour, treatment or outcome characteristics between patients with skeletal or extra-skeletal Ewing's sarcoma. We identified 300 patients with new primary Ewing's sarcoma diagnosed between 1980 and 2005 from the centres' local database. There were 253 (84%) with skeletal and 47 (16%) with extra-skeletal Ewing's sarcomas. Although patients with skeletal Ewing's were younger (mean age 16.8 years) than those with extra-skeletal Ewing's sarcoma (mean age 27.5 years), there was little difference between the groups in terms of tumour stage or treatment. Nearly all the patients were treated with chemotherapy and most had surgery. There was no difference in the overall survival of patients with skeletal (64%) and extra-skeletal Ewing's sarcoma (61%) (p = 0.85), and this was also the case when both groups were split by whether they had metastases or not. This large series has shown that the oncological outcomes of Ewing's sarcoma are related to tumour characteristics and patient age, and not determined by whether they arise in bone or soft tissue.

  6. Combination of External Beam Radiotherapy (EBRT) With Intratumoral Injection of Dendritic Cells as Neo-Adjuvant Treatment of High-Risk Soft Tissue Sarcoma Patients

    Energy Technology Data Exchange (ETDEWEB)

    Finkelstein, Steven E., E-mail: steven.finkelstein@moffitt.org [H. Lee Moffitt Cancer Center, Tampa, FL (United States); Iclozan, Cristina; Bui, Marilyn M.; Cotter, Matthew J.; Ramakrishnan, Rupal; Ahmed, Jamil; Noyes, David R.; Cheong, David; Gonzalez, Ricardo J.; Heysek, Randy V.; Berman, Claudia; Lenox, Brianna C.; Janssen, William; Zager, Jonathan S.; Sondak, Vernon K.; Letson, G. Douglas; Antonia, Scott J. [H. Lee Moffitt Cancer Center, Tampa, FL (United States); Gabrilovich, Dmitry I., E-mail: dmitry.gabrilovich@moffitt.org [H. Lee Moffitt Cancer Center, Tampa, FL (United States)

    2012-02-01

    Purpose: The goal of this study was to determine the effect of combination of intratumoral administration of dendritic cells (DC) and fractionated external beam radiation (EBRT) on tumor-specific immune responses in patients with soft-tissue sarcoma (STS). Methods and Material: Seventeen patients with large (>5 cm) high-grade STS were enrolled in the study. They were treated in the neoadjuvant setting with 5,040 cGy of EBRT, split into 28 fractions and delivered 5 days per week, combined with intratumoral injection of 10{sup 7} DCs followed by complete resection. DCs were injected on the second, third, and fourth Friday of the treatment cycle. Clinical evaluation and immunological assessments were performed. Results: The treatment was well tolerated. No patient had tumor-specific immune responses before combined EBRT/DC therapy; 9 patients (52.9%) developed tumor-specific immune responses, which lasted from 11 to 42 weeks. Twelve of 17 patients (70.6%) were progression free after 1 year. Treatment caused a dramatic accumulation of T cells in the tumor. The presence of CD4{sup +} T cells in the tumor positively correlated with tumor-specific immune responses that developed following combined therapy. Accumulation of myeloid-derived suppressor cells but not regulatory T cells negatively correlated with the development of tumor-specific immune responses. Experiments with {sup 111}In labeled DCs demonstrated that these antigen presenting cells need at least 48 h to start migrating from tumor site. Conclusions: Combination of intratumoral DC administration with EBRT was safe and resulted in induction of antitumor immune responses. This suggests that this therapy is promising and needs further testing in clinical trials design to assess clinical efficacy.

  7. Phase II study of the safety and antitumor activity of the hypoxia-activated prodrug TH-302 in combination with doxorubicin in patients with advanced soft tissue sarcoma.

    Science.gov (United States)

    Chawla, Sant P; Cranmer, Lee D; Van Tine, Brian A; Reed, Damon R; Okuno, Scott H; Butrynski, James E; Adkins, Douglas R; Hendifar, Andrew E; Kroll, Stew; Ganjoo, Kristen N

    2014-10-10

    TH-302, a prodrug of the cytotoxic alkylating agent bromo-isophosphoramide mustard, is preferentially activated in hypoxic conditions. This phase II study investigated TH-302 in combination with doxorubicin, followed by single-agent TH-302 maintenance therapy in patients with first-line advanced soft tissue sarcoma (STS) to assess progression-free survival (PFS), response rate, overall survival, safety, and tolerability. In this open-label phase II study, TH-302 300 mg/m(2) was administered intravenously on days 1 and 8 with doxorubicin 75 mg/m(2) on day 1 of each 21-day cycle. After six cycles, patients with stable and/or responding disease could receive maintenance monotherapy with TH-302. Ninety-one patients initiated TH-302 plus doxorubicin induction treatment. The PFS rate at 6 months (primary efficacy measure) was 58% (95% CI, 46% to 68%). Median PFS was 6.5 months (95% CI, 5.8 to 7.7 months); median overall survival was 21.5 months (95% CI, 16.0 to 26.2 months). Best tumor responses were complete response (n = 2 [2%]) and partial response (n = 30 [34%]). During TH-302 maintenance (n = 48), five patients improved from stable disease to partial response, and one patient improved from partial to complete response. The most common adverse events during induction were fatigue, nausea, and skin and/or mucosal toxicities as well as anemia, thrombocytopenia, and neutropenia. These were less severe and less frequent during maintenance. There was no evidence of TH-302-related hepatic, renal, or cardiac toxicity. PFS, overall survival, and tumor response compared favorably with historical outcomes achieved with other first-line chemotherapies for advanced STS. A phase III study of TH-302 is ongoing (NCT01440088). © 2014 by American Society of Clinical Oncology.

  8. Genetic profiling differentiates second primary tumors from metastases in adult metachronous soft tissue sarcoma

    DEFF Research Database (Denmark)

    Fernebro, Josefin; Carneiro, Ana; Rydholm, Anders

    2008-01-01

    Purpose. Patients with soft tissue sarcomas (STS) are at increased risk of second primary malignancies, including a second STS, but distinction between metastases and a second primary STS is difficult. Patients and Methods. Array-based comparative genomic hybridization (aCGH) was applied to 30 mu...

  9. Preoperative radiotherapy for bone and soft tissue sarcoma

    International Nuclear Information System (INIS)

    Matsumoto, Seiichi; Kawaguchi, Noriyoshi; Amino, Katsuhisa; Manabe, Jun; Yamashita, Takashi; Kaneta, Kouichi; Furuya, Kohtaro; Isobe, Yasushi.

    1989-01-01

    The role of preoperative radiotherapy was evaluated in 16 cases with soft tissue sarcoma and 13 cases with osteosarcoma. Nine osteosarcoma cases underwent radiotherapy of whole lesion, and 4 cases had radiotherapy only of the surgically uncurable portion. There were no local recurrences in M0 cases, but skin necrosis occurred in the whole radiation group. As for the soft tissue sarcomas, local recurrence was not seen in virgin cases, but two cases which had received previous treatment showed local recurrence. There were no cases with severe side effects. Partial radiotherapy was effective as preoperative treatment for osteosarcoma. Preoperative radiotherapy is better than postoperative radiotherapy from many standpoints. (author)

  10. Improved survival using specialized multidisciplinary board in sarcoma patients.

    Science.gov (United States)

    Blay, J-Y; Soibinet, P; Penel, N; Bompas, E; Duffaud, F; Stoeckle, E; Mir, O; Adam, J; Chevreau, C; Bonvalot, S; Rios, M; Kerbrat, P; Cupissol, D; Anract, P; Gouin, F; Kurtz, J-E; Lebbe, C; Isambert, N; Bertucci, F; Toumonde, M; Thyss, A; Piperno-Neumann, S; Dubray-Longeras, P; Meeus, P; Ducimetière, F; Giraud, A; Coindre, J-M; Ray-Coquard, I; Italiano, A; Le Cesne, A

    2017-11-01

    Sarcomas are rare but aggressive diseases. Specialized multidisciplinary management is not implemented for all patients in most countries. We investigated the impact of a multidisciplinary tumor board (MDTB) presentation before treatment in a nationwide study over 5 years. NETSARC (netsarc.org) is a network of 26 reference sarcoma centers with specialized MDTB, funded by the French National Cancer Institute to improve the outcome of sarcoma patients. Since 2010, presentation to an MDTB and second pathological review are mandatory for sarcoma patients in France. Patients' characteristics and follow-up are collected in a database regularly monitored and updated. The management and survival of patients presented to these MDTB before versus after initial treatment were analyzed. Out of the 12 528 patients aged ≥15 years, with a first diagnosis of soft tissue and visceral sarcoma obtained between 1 January 2010 and 31 December 2014, 5281 (42.2%) and 7247 (57.8%) were presented to the MDTB before and after the initiation of treatment, respectively. The former group had generally worse prognostic characteristics. Presentation to a MDTB before treatment was associated with a better compliance to clinical practice guidelines, for example, biopsy before surgery, imaging, quality of initial surgery, and less reoperations (all P sarcoma patients are significantly better when the initial treatment is guided by a pre-therapeutic specialized MDTB. © The Author 2017. Published by Oxford University Press on behalf of the European Society for Medical Oncology. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  11. Prognostic value of {sup 18}F-FDG PET/CT in patients with soft tissue sarcoma: comparisons between metabolic parameters

    Energy Technology Data Exchange (ETDEWEB)

    Hong, Sun-pyo [Sungkyunkwan University School of Medicine, Department of Nuclear Medicine, Samsung Medical Center, Seoul (Korea, Republic of); Lee, Seung Eun; Choi, Yoon-La [Sungkyunkwan University School of Medicine, Department of Pathology, Samsung Medical Center, Seoul (Korea, Republic of); Seo, Sung Wook; Sung, Ki-Sun [Sungkyunkwan University School of Medicine, Department of Orthopedic Surgery, Samsung Medical Center, Seoul (Korea, Republic of); Koo, Hong Hoe [Sungkyunkwan University School of Medicine, Department of Pediatrics, Samsung Medical Center, Seoul (Korea, Republic of); Choi, Joon Young [Sungkyunkwan University School of Medicine, Department of Nuclear Medicine, Samsung Medical Center, Seoul (Korea, Republic of)

    2014-05-15

    To investigate the relationship between volume-based PET parameters and prognosis in patients with soft tissue sarcoma (STS). We retrospectively reviewed 55 patients with pathologically proven STS who underwent pretreatment with {sup 18} F-Fluorodeoxyglucose ({sup 18}F-FDG) PET/CT. The maximum standardized uptake value (SUV{sub max}), average SUV (SUV{sub avg}), metabolic tumor volume (MTV), and total lesion glycolysis (TLG) of primary tumors were measured using a threshold SUV as liver activity for determining the boundary of tumors. Univariate and multivariate survival analyses for overall survival were performed according to the metabolic parameters and other clinical variables. Cancer-related death occurred in 19 of 55 patients (35 %) during the follow-up period (29 ± 23 months). On univariate analysis, AJCC stage (stage IV vs. I-III, hazard ratio (HR) = 2.837, p = 0.028), necrosis (G2 vs. G0-G1, HR = 3.890, p = 0.004), SUV{sub max} (1 unit - increase, HR = 1.146, p = 0.008), SUV{sub avg} (1 unit - increase, HR = 1.469, p = 0.032) and treatment modality (non-surgical therapy vs. surgery, HR = 4.467, p = 0.002) were significant predictors for overall survival. On multivariate analyses, SUV{sub max} (HR = 1.274, p = 0.015), treatment modality (HR = 3.353, p = 0.019) and necrosis (HR = 5.985, p = 0.006) were identified as significant independent prognostic factors associated with decreased overall survival. The SUV{sub max} of the primary tumor is a significant independent metabolic prognostic factor for overall survival in patients with STS. Volume-based PET parameters may not add prognostic information outside of the SUV{sub max}. (orig.)

  12. Pretreatment tumor SUV{sub max} predicts disease-specific and overall survival in patients with head and neck soft tissue sarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Ha, Seung Cheol; Roh, Jong-Lyel; Choi, Seung-Ho; Nam, Soon Yuhl; Kim, Sang Yoon [University of Ulsan College of Medicine, Departments of Otolaryngology, Asan Medical Center, Songpa-gu, Seoul (Korea, Republic of); Oh, Jungsu S.; Moon, Hyojeong; Kim, Jae Seung [University of Ulsan College of Medicine, Departments of Nuclear Medicine, Asan Medical Center, Seoul (Korea, Republic of); Cho, Kyung-Ja [University of Ulsan College of Medicine, Departments of Pathology, Asan Medical Center, Seoul (Korea, Republic of)

    2017-01-15

    Head and neck soft tissue sarcoma (HNSTS) is a rare type of tumor with various histological presentations and clinical behaviors. {sup 18}F-FDG PET/CT is being increasingly used for staging, grading, and predicting treatment outcomes in various types of human cancers, although this modality has been rarely studied in the survival prediction of HNSTS. Here we examined the prognostic value of tumor metabolic parameters measured using {sup 18}F-FDG PET/CT in patients with HNSTS. This study included 36 consecutive patients with HNSTS who underwent {sup 18}F-FDG PET/CT scanning prior to treatment at our institution. Tumor gross total volume (GTV) was measured from pretreatment contrast-enhanced CT scans, and maximum standardized uptake value (SUV{sub max}), metabolic tumor volume (MTV), and total lesion glycolysis (TLG) were measured using pretreatment {sup 18}F-FDG PET/CT scans. Univariate and multivariate Cox proportional hazard regression analyses were used to identify associations between imaging parameters and disease-specific survival (DSS) or overall survival (OS). Univariate analyses showed that SUV{sub max}, MTV, and TLG, but not GTV, were significantly associated with DSS and OS (all P < 0.05). After controlling for clinicopathological factors, SUV{sub max}, MTV, and TLG were significantly associated with DSS and OS (all P < 0.05). Patients with a tumor SUV{sub max} value of >7.0 experienced an approximately fivefold increase in mortality in terms of DSS and OS relative to those with a tumor SUV{sub max} <7.0. Quantitative metabolic measurements on pretreatment {sup 18}F-FDG PET/CT can yield values that are significantly predictive of survival after treatment for HNSTS. (orig.)

  13. Imaging of the most frequent superficial soft-tissue sarcomas

    International Nuclear Information System (INIS)

    Morel, Melanie; Taieb, Sophie; Ceugnart, Luc; Penel, Nicolas; Mortier, Laurent; Vanseymortier, Luc; Robin, Y.M.; Gosset, Pierre; Cotten, Anne

    2011-01-01

    Superficial soft-tissue sarcomas are malignant mesenchymal tumors located within the cutaneous and/or subcutaneous layers. Most superficial soft-tissue sarcomas are low-grade tumors; yet, the risk of local recurrence is high, and initial wide surgery is the main prognostic factor. Some of these superficial sarcomas may grow, following an infiltrative pattern, and their real extent may be underestimated clinically. Imaging techniques are useful to determine precisely the real margins of the tumor, especially in cases of clinically doubtful or recurrent or large superficial lesions. Imaging tools enable one to determine the relationship with the superficial fascia separating the subcutaneous layer from the underlying muscle. In our institution ultrasonographic examination is followed by magnetic resonance (MR) imaging when the size of the lesion exceeds 3-5 cm. Imaging assessment is performed prior to biopsy, enabling optimal surgical management. Imaging features of the main superficial sarcomas are detailed in the following article, according to their major locations: those arising in the epidermis and/or dermis, which are most often diagnosed by dermatologists, and the subcutaneous sarcomas. (orig.)

  14. Imaging of the most frequent superficial soft-tissue sarcomas

    Energy Technology Data Exchange (ETDEWEB)

    Morel, Melanie; Taieb, Sophie; Ceugnart, Luc [Centre Oscar Lambret, Department of Radiology, Lille (France); Penel, Nicolas [Centre Oscar Lambret, Department of Oncology, Lille (France); Mortier, Laurent [Centre Hospitalier Universitaire de Lille, Department of Dermatology, Hopital Claude Huriez, Lille (France); Vanseymortier, Luc [Centre Oscar Lambret, Department of Surgery, Lille (France); Robin, Y.M. [Centre Oscar Lambret, Departement of Pathology, Lille (France); Gosset, Pierre [Groupement Hospitalier de l' Institut Catholique-Faculte Libre de Medecine de Lille, Department of Pathology, Hopital Saint-Philibert, Lomme (France); Cotten, Anne [Centre Hospitalier Universitaire de Lille, Department of Musculoskeletal Radiology, Centre Hopital Roger Salengro, Lille (France)

    2011-03-15

    Superficial soft-tissue sarcomas are malignant mesenchymal tumors located within the cutaneous and/or subcutaneous layers. Most superficial soft-tissue sarcomas are low-grade tumors; yet, the risk of local recurrence is high, and initial wide surgery is the main prognostic factor. Some of these superficial sarcomas may grow, following an infiltrative pattern, and their real extent may be underestimated clinically. Imaging techniques are useful to determine precisely the real margins of the tumor, especially in cases of clinically doubtful or recurrent or large superficial lesions. Imaging tools enable one to determine the relationship with the superficial fascia separating the subcutaneous layer from the underlying muscle. In our institution ultrasonographic examination is followed by magnetic resonance (MR) imaging when the size of the lesion exceeds 3-5 cm. Imaging assessment is performed prior to biopsy, enabling optimal surgical management. Imaging features of the main superficial sarcomas are detailed in the following article, according to their major locations: those arising in the epidermis and/or dermis, which are most often diagnosed by dermatologists, and the subcutaneous sarcomas. (orig.)

  15. MIB-1 expression and iododeoxyuridine labelling in soft tissue sarcomas

    DEFF Research Database (Denmark)

    Jensen, V; Høyer, M; Sørensen, Flemming Brandt

    1996-01-01

    We investigated the relationship between immunohistochemical estimates of proliferative activity and expression of bcl-2 protein and mutant p53 protein in 23 cases of soft tissue sarcoma. Furthermore, the reproducibility of estimates of proliferative activity was analysed and correlations between...

  16. High dose rate brachytherapy for the treatment of soft tissue sarcoma of the extremity

    International Nuclear Information System (INIS)

    Speight, J.L.; Streeter, O.E.; Chawla, S.; Menendez, L.E.

    1996-01-01

    Purpose: we examined the role of preoperative neoadjuvant chemoradiation and adjuvant high-dose rate brachytherapy on the management of prognostically unfavorable soft tissue sarcomas of the extremities. Our goal was to examine the effect of high dose rate interstitial brachytherapy (HDR IBT) on reducing the risk of local recurrence following limb-sparing resection, as well as shortening treatment duration. Materials and methods: eleven patients, ranging in age from 31 to 73 years old, with soft tissue sarcoma of the extremity were treated at USC/Norris Comprehensive Cancer Center during 1994 and 1995. All patients had biopsy proven soft tissue sarcoma, and all were suitable candidates for limb-sparing surgery. All lesions were greater than 5cm in size and were primarily high grade. Tumor histologies included malignant fibrous histiocytoma (45%), liposarcoma (18%) and leiomyosarcoma, synovial cell sarcoma and spindle cell sarcoma (36%). Sites of tumor origin were the lower extremity (55%), upper extremity (18%) and buttock (9%), 1 patient (9%) had lesions in both the upper and lower extremity. Patients received HDR IBT following combined chemotherapy and external beam irradiation (EBRT) and en bloc resection of the sarcoma. Neoadjuvant chemotherapy consisted of three to four cycles of either Ifosfamide/Mesna with or without Adriamycin, or Mesna, Adriamycin, Ifosfamide and Dacarbazine. One patient received Cis-platin in addition to Ifos/Adr. A minimum of two cycles of chemotherapy were administered prior to EBRT. Additional cycles of chemotherapy were completed concurrently with EBRT but prior to HDR IBT. Preoperative EBRT doses ranging from 40 to 59.4 Gy were given in daily fractions of 180 to 200cGy. Following en bloc resection, HDR IBT was administered using the Omnitron tm 2000 remote afterloading system. Doses ranging from 13 to 30 Gy were delivered to the surgical tumor bed at depths of 0.5mm to 0.75mm from the radioactive source. Results: median follow-up was

  17. Retrospective review of soft tissue sarcoma of head and neck in a ...

    African Journals Online (AJOL)

    Background: Soft tissue sarcomas like other malignancies, impact negatively on patients and their caregivers as well as pose a challenge to the managing physician with variable treatment outcomes. A review of related studies on Medline has shown the paucity of the literature on the disease in the West African sub-region.

  18. Proliferative activity (MIB-1 index) is an independent prognostic parameter in patients with high-grade soft tissue sarcomas of subtypes other than malignant fibrous histiocytomas

    DEFF Research Database (Denmark)

    Jensen, V; Sørensen, Flemming Brandt; Bentzen, S M

    1998-01-01

    . The proliferative activity was assessed by use of the monoclonal antibody MIB-1 and evaluated in multiple, random systematic sampled fields of vision. The percentage of proliferating cells (the MIB-1 index) ranged between 1% and 85% (median 12%). A significant increase in mean MIB-1 index was seen with increasing...... histological malignancy grade. Variation in the incidence of p53 accumulation and bcl-2 positivity among different histological subtypes was observed. p53 accumulation was frequent in synovial sarcomas and leiomyo- and rhabdomyosarcomas, whereas bcl-2 preferentially was expressed in synovial sarcomas....... Univariate analysis identified patient age, tumour size, histological grade of malignancy, MIB-1 index and p53 accumulation as significant prognostic parameters. Multivariate Cox analysis, including tests for interaction terms between histological subtypes and MIB-1 index, showed independent prognostic...

  19. Soft tissue sarcoma nomograms and their incorporation into practice.

    Science.gov (United States)

    Callegaro, Dario; Miceli, Rosalba; Mariani, Luigi; Raut, Chandrajit P; Gronchi, Alessandro

    2017-08-01

    The accurate prediction of prognosis in patients with soft tissue sarcoma (STS) is a challenging issue. Extreme variability in the clinical and pathological characteristics of this family of tumors hinders the simple stratification of patients into meaningful prognostic cohorts. Precision medicine tools for the prediction of prognosis, such as nomograms, enable personalized computation of outcome based on clinical and pathological characteristics of both patient and tumor. The eighth edition of the American Joint Committee on Cancer staging manual moved from a "population-based" to a "personalized" approach endorsing high-quality nomograms to improve clinician prediction ability in definite patient subgroups. The first nomogram for STS was published in 2002, and this was followed by several prognostic predictors offered to clinicians. Focusing on a specific STS subgroup or site, nomograms can take into consideration highly specific factors relevant only in that particular scenario, thereby maximizing prognostic ability. The objective of this review was to critically evaluate available nomograms for patients with STS to provide clinicians and researchers with a choice of the most optimal tool for each specific patient. Cancer 2017;123:2802-20. © 2017 American Cancer Society. © 2017 American Cancer Society.

  20. Occurrence of Comorbidities before and after Soft Tissue Sarcoma Diagnosis

    Directory of Open Access Journals (Sweden)

    Myrthe P. P. van Herk-Sukel

    2012-01-01

    Full Text Available Background. Data is limited on the burden of common comorbidities, such as cardiovascular disease (CVD, respiratory disease and diabetes, or comorbidities related to cancer and its treatment, such as anemia and depression, in patients with soft tissue sarcoma (STS. Patients and Methods. From the Dutch Pathology Registry linked to the PHARMO database (including data on drug use and hospitalizations, 533 patients with STS were selected during 2000–2007 and matched 1 : 10 to cancer-free controls. The occurrences of comorbidities were assessed in the 12 months before and after STS diagnosis. Results. STS patients were 2–4 times more likely to have comorbidities at diagnosis compared with cancer-free controls. The incidence of CVD, anemia, and depression after STS diagnosis differed significantly from cancer-free controls and decreased during followup from 40–124 per 1,000 person-years (py during the first six months to 11–38 per 1,000 py more than 12 months after diagnosis. The incidence of respiratory disease and diabetes among STS patients remained stable during followup (5–21 per 1,000 py and did not differ significantly from cancer-free controls. Conclusions. STS patients were more likely to have comorbidities before cancer diagnosis and to develop CVD, anemia, and depression after diagnosis compared to cancer-free controls.

  1. Safety considerations for transplanting cryopreserved ovarian tissue to restore fertility in female patients who have recovered from Ewing's sarcoma

    DEFF Research Database (Denmark)

    Sørensen, Stine D; Greve, Tine; Wielenga, Vera Timmermans

    2014-01-01

    ovarian tissue, potentially causing recrudescence of the original cancer after transplantation. The risk of EWS metastasizing to the ovary is probably low but has not been studied in great detail. This review describes the available evidence on the risk of malignant cell contamination in the ovaries...

  2. Nonrhabdomyosarcoma soft tissue sarcoma (NRSTS) in pediatric and young adult patients: Results from a prospective study using limited-margin radiotherapy.

    Science.gov (United States)

    Tinkle, Christopher L; Fernandez-Pineda, Israel; Sykes, April; Lu, Zhaohua; Hua, Chia-Ho; Neel, Michael D; Bahrami, Armita; Shulkin, Barry L; Kaste, Sue C; Pappo, Alberto; Spunt, Sheri L; Krasin, Matthew J

    2017-11-15

    Indications for and delivery of adjuvant therapies for pediatric nonrhabdomyosarcoma soft tissue sarcoma (NRSTS) have been derived largely from adult studies; therefore, significant concern remains regarding radiation exposure to normal tissue. The authors report long-term treatment outcomes and toxicities for pediatric and young adult patients with high-grade NRSTS who were treated on a prospective trial using limited-margin radiotherapy. Sixty-two patients (ages 3-22 years) with predominantly high-grade NRSTS requiring radiation were treated on a phase 2 institutional study of conformal external-beam radiotherapy and/or brachytherapy using a 1.5-cm to 2-cm anatomically constrained margin. The estimated cumulative incidence of local failure, Gray's method estimated cumulative incidence of local failure, Kaplan-Meier method estimated survival, competing-risk regression model determined predictors of disease outcome, and toxicity was reported according to CTCAE v2.0. At a median follow-up of 5.1 years (range, 0.2-10.9 years), 9 patients had experienced local failure. The 5-year overall cumulative incidence of local failure was 14.8% (95% confidence interval [CI], 7.2%-25%), and all but 1 local failure occurred outside the highest-dose irradiation volume. The 5-year Kaplan-Meier estimates for event-free and overall survival were 49.3% (95% CI, 36.3%-61.1%) and 67.9% (95% CI, 54.2%-78.3%), respectively. Multivariable analysis indicated that younger age was the only independent predictor of local recurrence (P = .004). The 5-year cumulative incidence of grade 3 or 4 late toxicity was 15% (95% CI, 7.2%-25.3%). The delivery of limited-margin radiotherapy using conformal external-beam radiotherapy or brachytherapy provides a high rate of local tumor control without an increase in marginal failures and with acceptable treatment-related morbidity. Cancer 2017;123:4419-29. © 2017 American Cancer Society. © 2017 American Cancer Society.

  3. MicroRNAs in the Tumor Biology of Soft Tissue Sarcomas

    NARCIS (Netherlands)

    C.M.M. Gits (Caroline)

    2013-01-01

    markdownabstract__Abstract__ Soft tissue sarcomas represent a rare, heterogeneous group of mesenchymal tumors. In sarcomas, histological classification, prediction of clinical behaviour and prognosis, and targeted treatment is often a challenge. A better understanding of the biology of soft

  4. [Demographic Analysis of Patients with Osteosarcoma, Chonddrosarcoma, Ewing's Sarcoma from one Sarcoma Center in Switzerland].

    Science.gov (United States)

    Hodel, Sandro; Seeli, Franziska; Fuchs, Bruno

    2015-06-17

    Retrospective analysis of presentation, diagnosis and outcome of patients with osteosarcoma, chondrosarcoma and Ewing's sarcoma was performed for a single Sarcoma Center in Zurich at the University Hospital Balgrist. 201 patients were included. Overall survival at five and ten years were 74 ± 6%, 69 ± 7% for osteosarcoma (n = 85, since 2000), 85 ± 7%, 80 ± 9% for Ewing's sarcoma (n = 43, since 1990) and 86 ± 5%, 78 ± 9% for chondrosarcoma (n = 73, since 2000). The here presented overall survival rates from a single Sarcoma Center in Switzerland appear to be equivalent to other large international monocenter studies. The presentation and epidemiology of these patients are in accordance with large multicenter epidemiological studies. A nationwide sarcoma database (SwissSARCOS; www.sarcoma.ch) seems indispensable for more detailed analysis and quality management in such rare diseases.

  5. Head and neck soft tissue sarcomas treated with radiation therapy

    Directory of Open Access Journals (Sweden)

    Lucas K. Vitzthum

    2016-06-01

    Full Text Available Head and neck soft tissue sarcomas (HNSTSs are rare and heterogeneous cancers in which radiation therapy (RT has an important role in local tumor control (LC. The purpose of this study was to evaluate outcomes and patterns of treatment failure in patients with HNSTS treated with RT. A retrospective review was performed of adult patients with HNSTS treated with RT from January 1, 1998, to December 31, 2012. LC, locoregional control (LRC, disease-free survival (DFS, overall survival (OS, and predictors thereof were assessed. Forty-eight patients with HNSTS were evaluated. Five-year Kaplan-Meier estimates of LC, LRC, DFS, and OS were 87, 73, 63, and 83%, respectively. Angiosarcomas were found to be associated with worse LC, LRC, DFS, and OS. Patients over the age of 60 had lower rates of DFS. HNSTSs comprise a diverse group of tumors that can be managed with various treatment regimens involving RT. Angiosarcomas have higher recurrence and mortality rates.

  6. Effectiveness of Vascular Markers (Immunohistochemical Stains) in Soft Tissue Sarcomas.

    Science.gov (United States)

    Naeem, Namra; Mushtaq, Sajid; Akhter, Noreen; Hussain, Mudassar; Hassan, Usman

    2018-05-01

    To ascertain the effectiveness of IHC markers of vascular origin like CD31, CD34, FLI1 and ERG in vascular soft tissue sarcomas including angiosarcomas, Kaposi sarcomas, epithelioid hemangioendothelioma and a non-vascular soft tissue sarcoma (Epithelioid sarcoma). Descriptive study. Shaukat Khanum Memorial Cancer Hospital and Research Centre, Lahore, from 2011 to 2017. Diagnosed cases of angiosarcomas (n=48), epithelioid hemangioendothelioma (n=9), Kaposi sarcoma (n=9) and epithelioid sarcoma (n=20) were selected. Immunohistochemical staining as performed on formalin fixed paraffin embedded sections. The sections were stained for the following markers: CD34 (VENTANA clone Q Bend 10), CD31 (Leica clone 1 A 10), FLI1 (CELL MARQUE clone MRQ-1) and ERG (CELL MARQUE clone EP111). A complete panel of CD34, CD31 and ERG was applied on 8/48 cases of angiosarcomas with triple positivity in 6 cases. Eight cases showed positivity for only CD31 and ERG and 2 cases showed positivity for only ERG. A complete panel of CD34, CD31 and ERG was applied on 3/9 cases of epithelioid hemangioendothelioma with positivity for all markers in 2 cases. Combined positivity for ERG and CD34 was seen in 2 cases and on 4 cases only CD31 immunohistochemical was solely applied with 100% positivity. FLI1 was not applied on any case. Among 9 cases of Kaposi sarcoma, ERG, CD34 and CD31 in combination were applied on only 1 case with triple positivity. Remaining cases show positivity for either CD34, CD31 or FLI1. Majority of cases of epithelioid sarcomas were diagnosed on the basis of cytokeratin and CD34 positivity with loss of INI1. The other vascular markers showed negativity in all cases. Among these four markers, ERG immunohistochemical stain is highly effective for endothelial differentiation due to its specific nuclear staining pattern in normal blood vessel endothelial cells (internal control) as well as neoplastic cells of vascular tumors and lack of background staining.

  7. Prognostic factors in adult soft tissue sarcoma treated with surgery combined with radiotherapy: a retrospective single-center study on 164 patients

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    Ling Cai

    2013-10-01

    Full Text Available The aim of the present study is to assess the disease profile, outcome and prognostic factors in patients treated with surgery combined with radiotherapy (RT, with or without chemotherapy (CXT, for soft-tissue sarcoma (STS in a multidisciplinary setting. One hundred and sixty-four patients with STS treated between 1980 and 2010 at the Centre Hospitalier Universitaire Vaudois were enrolled in this retrospective study. Seventy-six percent of patients underwent postoperative RT with (24%, or without (52% CXT, 15% preoperative RT with (5%, or without (10% CXT, surgery alone (7%, or RT alone (2% with or without CXT. The median follow-up was 60 months (range 6-292. Local failure was observed in 18%, and distant failure in 21% of the patients. Overall survival (OS, disease-free survival (DFS, local control (LC and distant metastases-free survival (DMFS were 88%, 68%, 83%, and 79% at 5 years, and 80%, 56%, 76%, and 69% at 10 years, respectively. In univariate analyses, favorable prognostic factors for OS, DFS, and DMFS were tumor size 6 cm or less, World Health Organization (WHO/Zubrod score 0, and stage 2 or less. Age and superficial tumors were favorable only for OS and DMFS respectively. STS involving the extremities had a better outcome regarding DFS and LC. Histological grade 2 or less was favorable for DFS, DMFS, and LC. Radical surgery was associated with better LC and DMFS. RT dose more than 60 Gy was favorable for OS, DFS, and LC. In multivariate analyses, independent factors were age for OS; tumor size for OS, DFS and DMFS; WHO/Zubrod score for OS, DFS and LC; hemoglobin level for DFS; site for DFS and LC; tumor depth for DMFS; histological grade for DFS and LC; surgical procedure for LC and DMFS; and RT dose for OS. This study confirms that in a multidisciplinary setting, STS have a fairly good prognosis. A number of prognostic and predictive factors, including the role of surgery combined with RT, were identified. Regarding RT, a dose of

  8. Prognostic Factors in Adult Soft Tissue Sarcoma Treated with Surgery Combined with Radiotherapy: A Retrospective Single-Center Study on 164 Patients

    Science.gov (United States)

    Cai, Ling; Mirimanoff, René-Olivier; Mouhsine, Elyazid; Guillou, Louis; Leyvraz, Pierre-Francois; Leyvraz, Serge; Gay, Beatrice; Matzinger, Oscar; Ozsahin, Mahmut; Zouhair, Abderrahim

    2013-01-01

    The aim of the present study is to assess the disease profile, outcome and prognostic factors in patients treated with surgery combined with radiotherapy (RT), with or without chemotherapy (CXT), for soft-tissue sarcoma (STS) in a multidisciplinary setting. One hundred and sixty-four patients with STS treated between 1980 and 2010 at the Centre Hospitalier Universitaire Vaudois were enrolled in this retrospective study. Seventy-six percent of patients underwent postoperative RT with (24%), or without (52%) CXT, 15% preoperative RT with (5%), or without (10%) CXT, surgery alone (7%), or RT alone (2%) with or without CXT. The median follow-up was 60 months (range 6-292). Local failure was observed in 18%, and distant failure in 21% of the patients. Overall survival (OS), diseasefree survival (DFS), local control (LC) and distant metastases-free survival (DMFS) were 88%, 68%, 83%, and 79% at 5 years, and 80%, 56%, 76%, and 69% at 10 years, respectively. In univariate analyses, favorable prognostic factors for OS, DFS, and DMFS were tumor size 6 cm or less, World Health Organization (WHO)/Zubrod score 0, and stage 2 or less. Age and superficial tumors were favorable only for OS and DMFS respectively. STS involving the extremities had a better outcome regarding DFS and LC. Histological grade 2 or less was favorable for DFS, DMFS, and LC. Radical surgery was associated with better LC and DMFS. RT dose more than 60 Gy was favorable for OS, DFS, and LC. In multivariate analyses, independent factors were age for OS; tumor size for OS, DFS and DMFS; WHO/Zubrod score for OS, DFS and LC; hemoglobin level for DFS; site for DFS and LC; tumor depth for DMFS; histological grade for DFS and LC; surgical procedure for LC and DMFS; and RT dose for OS. This study confirms that in a multidisciplinary setting, STS have a fairly good prognosis. A number of prognostic and predictive factors, including the role of surgery combined with RT, were identified. Regarding RT, a dose of more than

  9. Combining Targeted Agents With Modern Radiotherapy in Soft Tissue Sarcomas

    Science.gov (United States)

    Wong, Philip; Houghton, Peter; Kirsch, David G.; Finkelstein, Steven E.; Monjazeb, Arta M.; Xu-Welliver, Meng; Dicker, Adam P.; Ahmed, Mansoor; Vikram, Bhadrasain; Teicher, Beverly A.; Coleman, C. Norman; Machtay, Mitchell; Curran, Walter J.

    2014-01-01

    Improved understanding of soft-tissue sarcoma (STS) biology has led to better distinction and subtyping of these diseases with the hope of exploiting the molecular characteristics of each subtype to develop appropriately targeted treatment regimens. In the care of patients with extremity STS, adjunctive radiation therapy (RT) is used to facilitate limb and function, preserving surgeries while maintaining five-year local control above 85%. In contrast, for STS originating from nonextremity anatomical sites, the rate of local recurrence is much higher (five-year local control is approximately 50%) and a major cause of death and morbidity in these patients. Incorporating novel technological advancements to administer accurate RT in combination with novel radiosensitizing agents could potentially improve local control and overall survival. RT efficacy in STS can be increased by modulating biological pathways such as angiogenesis, cell cycle regulation, cell survival signaling, and cancer-host immune interactions. Previous experiences, advancements, ongoing research, and current clinical trials combining RT with agents modulating one or more of the above pathways are reviewed. The standard clinical management of patients with STS with pretreatment biopsy, neoadjuvant treatment, and primary surgery provides an opportune disease model for interrogating translational hypotheses. The purpose of this review is to outline a strategic vision for clinical translation of preclinical findings and to identify appropriate targeted agents to combine with radiotherapy in the treatment of STS from different sites and/or different histology subtypes. PMID:25326640

  10. Microscopic histological characteristics of soft tissue sarcomas: analysis of tissue features and electrical resistance.

    Science.gov (United States)

    Tosi, A L; Campana, L G; Dughiero, F; Forzan, M; Rastrelli, M; Sieni, E; Rossi, C R

    2017-07-01

    Tissue electrical conductivity is correlated with tissue characteristics. In this work, some soft tissue sarcomas (STS) excised from patients have been evaluated in terms of histological characteristics (cell size and density) and electrical resistance. The electrical resistance has been measured using the ex vivo study on soft tissue tumors electrical characteristics (ESTTE) protocol proposed by the authors in order to study electrical resistance of surgical samples excised by patients in a fixed measurement setup. The measurement setup includes a voltage pulse generator (700 V, 100 µs long at 5 kHz, period 200 µs) and an electrode with 7 needles, 20 mm-long, with the same distance arranged in a fixed hexagonal geometry. In the ESTTE protocol, the same voltage pulse sequence is applied to each different tumor mass and the corresponding resistance has been evaluated from voltage and current recorded by the equipment. For each tumor mass, a histological sample of the volume treated by means of voltage pulses has been taken for histological analysis. Each mass has been studied in order to identify the sarcoma type. For each histological sample, an image at 20× or 40× of magnification was acquired. In this work, the electrical resistance measured for each tumor has been correlated with tissue characteristics like the type, size and density of cells. This work presents a preliminary study to explore possible correlations between tissue characteristics and electrical resistance of STS. These results can be helpful to adjust the pulse voltage intensity in order to improve the electrochemotherapy efficacy on some histotype of STS.

  11. Adjuvant radiotherapy with brachytherapy boost in soft tissue sarcomas

    Directory of Open Access Journals (Sweden)

    Annalisa Cortesi

    2017-06-01

    Full Text Available Purpose: The standard primary treatment for soft tissue sarcoma (STS is a wide surgical resection, preceded or followed by radiotherapy. Purpose of this retrospective study was to assess the efficacy of perioperative brachytherapy (BRT plus postoperative external beam radiation therapy (EBRT in patients with intermediate-high risk STS. Material and methods : BRT delivered dose was 20 Gy. External beam radiation therapy was delivered with 3D-technique using multiple beams. The prescribed dose was 46 Gy to the PTV. Neoadjuvant and adjuvant chemotherapy (CHT was used in patients with potentially chemosensitive histological subtypes. The primary aim of the study was to analyze overall survival (OS and local control (LC in a large patient population treated with surgery, perioperative BRT, and adjuvant EBRT ± CHT. Secondary objective was to identify prognostic factors for patients outcome in terms of LC, disease-free survival (DFS, and OS. Results : From 2000 to 2011, 107 patients presenting 2-3 grade (FNLCC primary or recurrent STS were treated with surgery, perioperative BRT, and adjuvant EBRT ± CHT. Five-year LC and OS were 80.9% and 87.4%, respectively. At univariate analysis, a higher LC was recorded in primary vs. recurrent tumors (p = 0.015, and in lower limb tumors vs. other sites (p = 0.027. An improved DFS was recorded in patients with lower limb tumors vs. other sites (p = 0.034. Conclusions : The combination of BRT and EBRT was able to achieve satisfactory results even in a patients population with intermediate-high risk STS. Patients with recurrent or other than lower limb sited tumors show a worse LC.

  12. Adjuvant radiotherapy with brachytherapy boost in soft tissue sarcomas

    Science.gov (United States)

    Cortesi, Annalisa; Galuppi, Andrea; Arcelli, Alessandra; Romani, Fabrizio; Mattiucci, Gian Carlo; Bianchi, Giuseppe; Ferrari, Stefano; Ferraro, Andrea; Farioli, Andrea; Gambarotti, Marco; Righi, Alberto; Macchia, Gabriella; Deodato, Francesco; Cilla, Savino; Buwenge, Milly; Valentini, Vincenzo; Morganti, Alessio Giuseppe; Donati, Davide; Cammelli, Silvia

    2017-01-01

    Purpose The standard primary treatment for soft tissue sarcoma (STS) is a wide surgical resection, preceded or followed by radiotherapy. Purpose of this retrospective study was to assess the efficacy of perioperative brachytherapy (BRT) plus postoperative external beam radiation therapy (EBRT) in patients with intermediate-high risk STS. Material and methods BRT delivered dose was 20 Gy. External beam radiation therapy was delivered with 3D-technique using multiple beams. The prescribed dose was 46 Gy to the PTV. Neoadjuvant and adjuvant chemotherapy (CHT) was used in patients with potentially chemosensitive histological subtypes. The primary aim of the study was to analyze overall survival (OS) and local control (LC) in a large patient population treated with surgery, perioperative BRT, and adjuvant EBRT ± CHT. Secondary objective was to identify prognostic factors for patients outcome in terms of LC, disease-free survival (DFS), and OS. Results From 2000 to 2011, 107 patients presenting 2-3 grade (FNLCC) primary or recurrent STS were treated with surgery, perioperative BRT, and adjuvant EBRT ± CHT. Five-year LC and OS were 80.9% and 87.4%, respectively. At univariate analysis, a higher LC was recorded in primary vs. recurrent tumors (p = 0.015), and in lower limb tumors vs. other sites (p = 0.027). An improved DFS was recorded in patients with lower limb tumors vs. other sites (p = 0.034). Conclusions The combination of BRT and EBRT was able to achieve satisfactory results even in a patients population with intermediate-high risk STS. Patients with recurrent or other than lower limb sited tumors show a worse LC. PMID:28725250

  13. External-beam radiation therapy combined with limb-sparing surgery in elderly patients (>70 years) with primary soft tissue sarcomas of the extremities. A retrospective analysis

    Energy Technology Data Exchange (ETDEWEB)

    Andrae, Claudia; Rauch, Josefine; Belka, Claus [University Hospital of Munich (LMU), Department of Radiation Oncology, Munich (Germany); Klein, Alexander; Duerr, Hans Roland [University Hospital of Munich (LMU), Department of Orthopedics, Munich (Germany); Lindner, Lars Hartwin [University Hospital of Munich (LMU), Deparment of Internal Medicine, Munich (Germany); Knoesel, Thomas [University Hospital of Munich (LMU), Institute of Pathology, Munich (Germany); Angele, Martin [University Hospital of Munich (LMU), Department of Surgery, Munich (Germany); Baur-Melnyk, Andrea [University Hospital of Munich (LMU), Department of Radiology, Munich (Germany); Roeder, Falk [University Hospital of Munich (LMU), Department of Radiation Oncology, Munich (Germany); German Cancer Research Center (DKFZ), CCU Molecular Radiation Oncology, Heidelberg (Germany)

    2017-08-15

    To report our experience with EBRT combined with limb-sparing surgery in elderly patients (>70 years) with primary extremity soft tissue sarcomas (STS). Retrospectively analyzed were 35 patients (m:f 18:17, median 78 years) who all presented in primary situation without nodal/distant metastases (Charlson score 0/1 in 18 patients; ≥2 in 17 patients). Median tumor size was 10 cm, mainly located in lower limb (83%). Stage at presentation (UICC7th) was Ib:3%, 2a:20%, 2b:20%, and 3:57%. Most lesions were high grade (97%), predominantly leiomyosarcoma (26%) and undifferentiated pleomorphic/malignant fibrous histiocytoma (23%). Limb-sparing surgery was preceded (median 50 Gy) or followed (median 66 Gy) by EBRT. Median follow-up was 37 months (range 1-128 months). Margins were free in 26 patients (74%) and microscopically positive in 9 (26%). Actuarial 3- and 5-year local control rates were 88 and 81% (4 local recurrences). Corresponding rates for distant control, disease-specific survival, and overall survival were 57/52%, 76/60%, and 72/41%. The 30-day mortality was 0%. Severe postoperative complications were scored in 8 patients (23%). Severe acute radiation-related toxicity was observed in 2 patients (6%). Patients with Charlson score ≥2 had a significantly increased risk for severe postoperative complications and acute radiation-related side effects. Severe late toxicities were found in 7 patients (20%), including fractures in 3 (8.6%). Final limb preservation rate was 97%. Combination of EBRT and limb-sparing surgery is feasible in elderly patients with acceptable toxicities and encouraging but slightly inferior outcome compared to younger patients. Comorbidity correlated with postoperative complications and acute toxicities. Late fracture risk seems slightly increased. (orig.) [German] Erfahrungsbericht zur perkutanen Radiotherapie (EBRT) kombiniert mit extremitaetenerhaltender Operation bei Patienten >70 Jahre mit Weichteilsarkom der Extremitaet. Retrospektiv

  14. Adult soft tissue sarcomas: conventional therapies and molecularly targeted approaches.

    Science.gov (United States)

    Mocellin, Simone; Rossi, Carlo R; Brandes, Alba; Nitti, Donato

    2006-02-01

    The therapeutic approach to soft tissue sarcomas (STS) has evolved over the past two decades based on the results from randomized controlled trials, which are guiding physicians in the treatment decision-making process. Despite significant improvements in the control of local disease, a significant number of patients ultimately die of recurrent/metastatic disease following radical surgery due to a lack of effective adjuvant treatments. In addition, the characteristic chemoresistance of STS has compromised the therapeutic value of conventional antineoplastic agents in cases of unresectable advanced/metastatic disease. Therefore, novel therapeutic strategies are urgently needed to improve the prognosis of patients with STS. Recent advances in STS biology are paving the way to the development of molecularly targeted therapeutic strategies, the efficacy of which relies not only on the knowledge of the molecular mechanisms underlying cancer development/progression but also on the personalization of the therapeutic regimen according to the molecular features of individual tumours. In this work, we review the state-of-the-art of conventional treatments for STS and summarize the most promising findings in the development of molecularly targeted therapeutic approaches.

  15. General Information about Adult Soft Tissue Sarcoma

    Science.gov (United States)

    ... deep (in the muscle and may be in connective or subcutaneous tissue). In stage IB , the tumor is low-grade (likely to grow and spread ... deep (in the muscle and may be in connective or subcutaneous tissue). In stage IIB , the tumor is mid-grade (somewhat likely to grow and ...

  16. Stages of Adult Soft Tissue Sarcoma

    Science.gov (United States)

    ... deep (in the muscle and may be in connective or subcutaneous tissue). In stage IB , the tumor is low-grade (likely to grow and spread ... deep (in the muscle and may be in connective or subcutaneous tissue). In stage IIB , the tumor is mid-grade (somewhat likely to grow and ...

  17. Pazopanib for the treatment of soft-tissue sarcoma

    Directory of Open Access Journals (Sweden)

    Heudel P

    2012-10-01

    Full Text Available Pierre Heudel,1 Philippe Cassier,1 Olfa Derbel,1 Armelle Dufresne,1 Pierre Meeus,2 Philippe Thiesse,3 Dominique Ranchère-Vince,4 Jean Yves Blay,1 Isabelle Ray-Coquard1,51Department of Medical Oncology, 2Department of Surgical Oncology, 3Department of Radiology, 4Department of Pathology, Leon Berard Center, Lyon, 5EAM 4128 Sante-Individu-Societe, Lyon University, Lyon, FranceAbstract: Pazopanib is a multikinase inhibitor which potently inhibits the activity of major receptor tyrosine kinases, including vascular endothelial growth factor receptor-1, vascular endothelial growth factor receptor-2, vascular endothelial growth factor receptor-3, platelet-derived growth factor receptor-a, platelet-derived growth factor receptor-a, and c-Kit. Approved by the Food and Drug Administration in 2009 in the United States for the treatment of metastatic renal cell carcinoma, pazopanib has been tested in advanced or metastatic soft-tissue sarcoma. Unlike other tyrosine kinase inhibitors, a statistically significant efficacy in phase II but also in randomized phase III studies has been shown. In comparison with sunitinib or sorafenib, pazopanib has a similar toxicity profile and is generally well tolerated. This review details the development of this new therapeutic class in the treatment of metastatic soft-tissue sarcomas.Keywords: soft-tissue sarcoma, pazopanib, tyrosine kinase inhibitor

  18. PML expression in soft tissue sarcoma: prognostic and predictive value in alkylating agents/antracycline-based first line therapy.

    Science.gov (United States)

    Vincenzi, Bruno; Santini, Daniele; Schiavon, Gaia; Frezza, Anna Maria; Silletta, Marianna; Crucitti, Pierfilippo; Casali, Paolo; Dei Tos, Angelo P; Rossi, Sabrina; Rizzo, Sergio; Badalamenti, Giuseppe; Tomasino, Rosa Maria; Russo, Antonio; Butrynski, James E; Tonini, Giuseppe

    2012-04-01

    Soft tissue sarcomas are aggressive tumors representing alkylating agents/antracycline-based first line therapy. One hundred eleven patients affected by locally advanced and metastatic soft tissue sarcoma were selected. PML expression was evaluated by immunohistochemical analysis in pathological samples and in the corresponding normal tissue from each case. PML immunohistochemical results were correlated with prognosis and with radiological response to alkylating agents/antracycline-based first line therapy. PML expression was significantly reduced in synovial sarcomas (P < 0.0001), in myofibroblastic sarcomas (P < 0.0001), angiosarcomas (P < 0.0001), in leiomyosarcomas (P = 0.003), in mixoid liposarcomas (P < 0.0001), and in dedifferentiated liposarcomas (P < 0.0001). No significant difference was found for pleomorphic sarcoma [31.8 (95% CI: 16.7-41.0); P = 0.21]. and pleomorphic liposarcomas (P = 0.51). Loss of PML expression was found to be statistically correlated with TTP (P < 0.0001), median duration of response (P = 0.007), and OS (P = 0.02). No correlation was observed between PML expression and treatment efficacy. PML IHC expression is down-regulated in synovial sarcomas, myofibroblastic sarcomas, angiosarcomas, liposarcoma, and leiomyosarcomas and its expression correlated with prognosis. Copyright © 2011 Wiley Periodicals, Inc.

  19. Lymph Node Metastasis after a Soft Tissue Sarcoma of the Leg: A Case Report and a Review of the Literature

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    S. D. Nelen

    2013-01-01

    Full Text Available Introduction. Soft tissue sarcomas (STSs represent 1 percent of all adult malignancies and sarcomas only rarely spread to the regional lymph nodes. Case Presentation. We present a case of a woman with a dermatofibrosarcoma protuberans and a sarcoma not therwise specified of the lower extremity. The patient had no distant metastasis during follow-up, but did develop a regional lymph nodemetastasis (RLNM in the groin. We reviewed the literature about RLNM in STSs. Discussion. Reviewing the literature we see that within specific histological types RLNM occurs as often as distant metastasis. Furthermore RLNM occurs in over 10% for specific histological types and in 24% of all patients with a soft tissue sarcoma of the lower extremity. Except for radical lymphadenectomy with a 5-year survival rate of 46% there is no appropriate treatment. Conclusion. The risk for a RLNM in certain histological types and anatomical locations might transcend the risk for a distant lung metastasis.

  20. Synovial sarcoma of the kidney in a young patient with a review of the literature

    Directory of Open Access Journals (Sweden)

    Mahmoud Abbas

    2014-06-01

    Full Text Available Synovial sarcoma (SS is a soft tissue, generally deep seated neoplasms that occurs generally in the proximity of large joints. We report of a case of a 33-year-old man who was diagnosed with primary SS of the kidney which is an extremely rare tumor that accounts for less than 2% of malignant renal tumors. Contemporary management of renal synovial sarcoma includes surgical resection and ifosfamide-based chemotherapy and they remain the mainstay of therapy of synovial sarcoma, which is often applied, combined as part of an aggressive treatment approach. Fewer than 50 patients have been described in the English literature. Physicians should be aware of the possibility of malignancy in cystic renal masses and raise the suspicion of synovial sarcoma, especially when patients with renal masses are young adults. Along with the case report a literature review on primary synovial sarcomas of the kidney is provided with focus on the renal tumors’ differential diagnosis.

  1. Relation between pO2, 31P magnetic resonance spectroscopy parameters and treatment outcome in patients with high-grade soft tissue sarcomas treated with thermoradiotherapy

    International Nuclear Information System (INIS)

    Dewhirst, Mark W.; Poulson, Jean M.; Yu Daohai; Sanders, Linda; Lora-Michiels, Michael; Vujaskovic, Zeljko; Jones, Ellen L.; Samulski, Thaddeus V.; Powers, Barbara E.; Brizel, David M.; Prosnitz, Leonard R.; Charles, H. Cecil

    2005-01-01

    Purpose: In a prior study, the combination of 31 P magnetic resonance spectroscopy (MRS)-based intracellular pH (pHi) and T2 relaxation time was highly predictive of the pathologic complete response (pCR) rate in a small series of patients with soft tissue sarcomas (STSs) treated with thermoradiotherapy. Changes in the magnetic resonance metabolite ratios and pO 2 were related to the pCR rate. Hypoxia also correlated with a greater likelihood for the development of metastases. Because of the limited number of patients in the prior series, we initiated this study to determine whether the prior observations were repeatable and whether 31 P MRS lipid-related resonances were related to a propensity for metastasis. Methods and materials: Patients with high-grade STSs were enrolled in an institutional review board-approved Phase II thermoradiotherapy trial. All tumors received daily external beam radiotherapy (1.8-2.0 Gy, five times weekly) to a total dose of 30-50 Gy. Hyperthermia followed radiotherapy by 31 P metabolite ratios, pHi, and T2 relaxation time. The median pO 2 and hypoxic fraction were determined using pO 2 histography. Comparisons between experimental endpoints and the pCR rate and metastasis-free and overall survival were made. Results: Of 35 patients, 21 and 28 had reportable pretreatment MRS/MRI and pO 2 data, respectively. The cutpoints for a previously tested receiver operating curve for a pCR were T2 = 100 and pHi = 7.3. In the current series, few tumors fell below the cutpoints so validation was not possible. The phosphodiester (PDE)/inorganic phosphate (Pi) ratio and hypoxic fraction correlated inversely with the pCR rate in the current series (Spearman correlation coefficient -0.51, p = 0.017; odds ratio of percentage of necrosis ≥95% = 0.01 for a 1% increase in the hypoxic fraction; Wald p = 0.036). The pretreatment phosphomonoester (PME)/Pi ratio also correlated inversely with the pCR rate (odds ratio of percentage of necrosis ≥95% = 0

  2. Factors associated with reduced functional outcome and quality of life in patients having limb-sparing surgery for soft tissue sarcomas - a national multicenter study of 128 patients

    DEFF Research Database (Denmark)

    Saebye, Casper; Fugloe, Hanna M; Nymark, Tine

    2017-01-01

    from the Danish National Sarcoma Database. Wilcoxon rank-sum test and Kruskal-Wallis were used for univariable analysis. Adjusted odds ratios were estimated by using multiple logistic regression models. RESULTS: In the multiple regression analysis, it was found that female gender (p = 0.03), lower...

  3. What is current practice in soft tissue sarcoma grading?

    International Nuclear Information System (INIS)

    Golouh, R.; Bracko, M.

    2001-01-01

    Purpose. Most published grading systems of soft tissue sarcomas (STS) are somewhat subjective and it seems that there is no definite consensus among experts which of them is the most effective. The aim of this study was to collect data from practicing pathologists and to get some insight in the practice of STS grading. Subjects. A questionnaire was sent to 135 pathologists chosen randomly. Results. There were 88 responders from 30 countries from 5 continents. Most responders (85%) grade STS, more frequently in Europe than in non-European countries. Three-grade system is preferred by both European and non-European pathologists, who use it in almost 77% and 67%, respectively. They apply the criteria set by FNCLCC in 37.3%, by NCI in 24%, by Broders in 12% and by Markhede in 1.4%. In Europe, FNCLCC system is the most widely used. Beside classical histological criteria, other modern methods are applied by more than one half of the responders. Immunohistochemical evaluation of proliferation markers is the method most widely used, followed by molecular markers and DNA flow cytometry. Conclusion. The results of our study indicate that most pathologists consider histologic grade of STS as a valuable, however not completely satisfactory predictor of a patient's survival. (author)

  4. Risk assessment of radio-chemotherapy in pediatric soft tissue sarcomas

    OpenAIRE

    Abaza, A.; El-Shanshoury, H.

    2015-01-01

    Soft tissue sarcomas (STS) are a group of rare and heterogenous cancers, that diverse a wide spectrum of histology and varied clinical behavior. The aim was to study, retrospectively and prospectively the adverse effects of therapy in STS patients attending the Pediatric Oncology Clinic, National Cancer Institute (NCI), Cairo University during the last 10 years. Files of 106 STS patients were revised for history, staging, investigations, treatment modalities and side effects of therapy. Radio...

  5. The Epidemiology of Sarcoma

    Directory of Open Access Journals (Sweden)

    Burningham Zachary

    2012-10-01

    Full Text Available Abstract Sarcomas account for over 20% of all pediatric solid malignant cancers and less than 1% of all adult solid malignant cancers. The vast majority of diagnosed sarcomas will be soft tissue sarcomas, while malignant bone tumors make up just over 10% of sarcomas. The risks for sarcoma are not well-understood. We evaluated the existing literature on the epidemiology and etiology of sarcoma. Risks for sarcoma development can be divided into environmental exposures, genetic susceptibility, and an interaction between the two. HIV-positive individuals are at an increased risk for Kaposi’s sarcoma, even though HHV8 is the causative virus. Radiation exposure from radiotherapy has been strongly associated with secondary sarcoma development in certain cancer patients. In fact, the risk of malignant bone tumors increases as the cumulative dose of radiation to the bone increases (p for trend

  6. Vorinostat in refractory soft tissue sarcomas - Results of a multi-centre phase II trial of the German Soft Tissue Sarcoma and Bone Tumour Working Group (AIO).

    Science.gov (United States)

    Schmitt, Thomas; Mayer-Steinacker, Regine; Mayer, Frank; Grünwald, Viktor; Schütte, Jochen; Hartmann, Jörg T; Kasper, Bernd; Hüsing, Johannes; Hajda, Jacek; Ottawa, Gregor; Mechtersheimer, Gunhild; Mikus, Gerd; Burhenne, Jürgen; Lehmann, Lorenz; Heilig, Christoph E; Ho, Anthony D; Egerer, Gerlinde

    2016-09-01

    New treatment options for patients with metastatic Soft Tissue Sarcoma are urgently needed. Preclinical studies suggested activity of vorinostat, a histone deacetylase inhibitor. A multi-centre, open-label, non-randomised phase II trial to investigate the efficacy and safety of vorinostat in patients with locally advanced or metastatic Soft Tissue Sarcoma failing 1st-line anthracycline-based chemotherapy was initiated. Patients were treated with vorinostat 400 mg po qd for 28 d followed by a treatment-free period of 7 d, representing a treatment cycle of 5 weeks. Restaging was performed every three cycles or at clinical progression. Between 06/10 and 09/13, 40 Soft Tissue Sarcoma patients were treated with vorinostat at seven participating centres. Patients had received 1 (n=8, 20%), 2 (n=10, 25%) or ≥3 (n=22, 55%) previous lines of chemotherapy. Best response after three cycles of treatment was stable disease (n=9, 23%). Median progression-free survival and overall survival were 3.2 and 12.3 months, respectively. Six patients showed long-lasting disease stabilisation for up to ten cycles. Statistical analyses failed to identify baseline predictive markers in this subgroup. Major toxicities (grade ≥III) included haematological toxicity (n=6, 15%) gastrointestinal disorders (n=5, 13%), fatigue (n=4, 10%), musculoskeletal pain (n=4, 10%), and pneumonia (n=2, 5%). In a heavily pre-treated patient population, objective response to vorinostat was low. However, a small subgroup of patients had long-lasting disease stabilisation. Further studies aiming to identify predictive markers for treatment response as well as exploration of combination regimens are warranted. NCT00918489 (ClinicalTrials.gov) EudraCT-number: 2008-008513-19. Copyright © 2016 Elsevier Ltd. All rights reserved.

  7. Histotype-tailored neoadjuvant chemotherapy versus standard chemotherapy in patients with high-risk soft-tissue sarcomas (ISG-STS 1001): an international, open-label, randomised, controlled, phase 3, multicentre trial.

    Science.gov (United States)

    Gronchi, Alessandro; Ferrari, Stefano; Quagliuolo, Vittorio; Broto, Javier Martin; Pousa, Antonio Lopez; Grignani, Giovanni; Basso, Umberto; Blay, Jean-Yves; Tendero, Oscar; Beveridge, Robert Diaz; Ferraresi, Virginia; Lugowska, Iwona; Merlo, Domenico Franco; Fontana, Valeria; Marchesi, Emanuela; Donati, Davide Maria; Palassini, Elena; Palmerini, Emanuela; De Sanctis, Rita; Morosi, Carlo; Stacchiotti, Silvia; Bagué, Silvia; Coindre, Jean Michelle; Dei Tos, Angelo Paolo; Picci, Piero; Bruzzi, Paolo; Casali, Paolo Giovanni

    2017-06-01

    Previous trials from our group suggested an overall survival benefit with five cycles of adjuvant full-dose epirubicin plus ifosfamide in localised high-risk soft-tissue sarcoma of the extremities or trunk wall, and no difference in overall survival benefit between three cycles versus five cycles of the same neoadjuvant regimen. We aimed to show the superiority of the neoadjuvant administration of histotype-tailored regimen to standard chemotherapy. For this international, open-label, randomised, controlled, phase 3, multicentre trial, patients were enrolled from 32 hospitals in Italy, Spain, France, and Poland. Eligible patients were aged 18 years or older with localised, high-risk (high malignancy grade, 5 cm or longer in diameter, and deeply located according to the investing fascia), soft-tissue sarcoma of the extremities or trunk wall and belonging to one of five histological subtypes: high-grade myxoid liposarcoma, leiomyosarcoma, synovial sarcoma, malignant peripheral nerve sheath tumour, and undifferentiated pleomorphic sarcoma. Patients were randomly assigned (1:1) to receive three cycles of full-dose standard chemotherapy (epirubicin 60 mg/m 2 per day [short infusion, days 1 and 2] plus ifosfamide 3 g/m 2 per day [days 1, 2, and 3], repeated every 21 days) or histotype-tailored chemotherapy: for high-grade myxoid liposarcoma, trabectedin 1·3 mg/m 2 via 24-h continuous infusion, repeated every 21 days; for leiomyosarcoma, gemcitabine 1800 mg/m 2 on day 1 intravenously over 180 min plus dacarbazine 500 mg/m 2 on day 1 intravenously over 20 min, repeated every 14 days; for synovial sarcoma, high-dose ifosfamide 14 g/m 2 , given over 14 days via an external infusion pump, every 28 days; for malignant peripheral nerve sheath tumour, intravenous etoposide 150 mg/m 2 per day (days 1, 2, and 3) plus intravenous ifosfamide 3 g/m 2 per day (days 1, 2, and 3), repeated every 21 days; and for undifferentiated pleomorphic sarcoma, gemcitabine 900 mg/m 2 on days 1 and

  8. Soft-tissue sarcomas. Current aspects of diagnosis and therapy

    International Nuclear Information System (INIS)

    Hohenberger, P.

    1996-01-01

    The decisive factor for promising therapy of soft-tissue sarcomas is primary therapy provided in an experienced tumour unit. This centre must offer the entire spectrum of surgery (vascular, reconstruction and orthopaedic surgery) leading into an interdisciplinary treatment regimen. Initially, MRI would appear to be sufficient for diagnosis. On the other hand, the presence of an experienced pathologist conversant with all means of cytogenetic tumour classification is essential. For interdisciplinary therapy, a radiotherapist with hyperthermia equipment, nuclear medicine specialists and medical oncologists are indispensable. All personnel must be familiar with the special problems associated with sarcomas. The current trend is indeed toward a select number of centres where such skills are focused. (orig.) [de

  9. Response to pazopanib in two pediatric patients with pretreated relapsing synovial sarcoma.

    Science.gov (United States)

    Casanova, Michela; Basso, Eleonora; Magni, Chiara; Bergamaschi, Luca; Chiaravalli, Stefano; Carta, Roberto; Tirtei, Elisa; Massimino, Maura; Fagioli, Franca; Ferrari, Andrea

    2017-01-21

    Pazopanib is an oral multikinase inhibitor that has proved effective in adults treated for relapsing soft tissue sarcoma and synovial sarcoma in particular. Two cases are reported here of pediatric patients with pretreated relapsing synovial sarcoma whose tumors showed a prolonged response to pazopanib given on compassionate grounds. These results suggest that new agents found effective in adult patients might achieve similar results in adolescents with the same disease. Facilitating the availability of new drugs for children and adolescents is a major challenge for pediatric oncologists.

  10. A prediction model for treatment decisions in high-grade extremity soft-tissue sarcomas: Personalised sarcoma care (PERSARC).

    Science.gov (United States)

    van Praag, Veroniek M; Rueten-Budde, Anja J; Jeys, Lee M; Laitinen, Minna K; Pollock, Rob; Aston, Will; van der Hage, Jos A; Dijkstra, P D Sander; Ferguson, Peter C; Griffin, Anthony M; Willeumier, Julie J; Wunder, Jay S; van de Sande, Michiel A J; Fiocco, Marta

    2017-09-01

    To support shared decision-making, we developed the first prediction model for patients with primary soft-tissue sarcomas of the extremities (ESTS) which takes into account treatment modalities, including applied radiotherapy (RT) and achieved surgical margins. The PERsonalised SARcoma Care (PERSARC) model, predicts overall survival (OS) and the probability of local recurrence (LR) at 3, 5 and 10 years. Development and validation, by internal validation, of the PERSARC prediction model. The cohort used to develop the model consists of 766 ESTS patients who underwent surgery, between 2000 and 2014, at five specialised international sarcoma centres. To assess the effect of prognostic factors on OS and on the cumulative incidence of LR (CILR), a multivariate Cox proportional hazard regression and the Fine and Gray model were estimated. Predictive performance was investigated by using internal cross validation (CV) and calibration. The discriminative ability of the model was determined with the C-index. Multivariate Cox regression revealed that age and tumour size had a significant effect on OS. More importantly, patients who received RT showed better outcomes, in terms of OS and CILR, than those treated with surgery alone. Internal validation of the model showed good calibration and discrimination, with a C-index of 0.677 and 0.696 for OS and CILR, respectively. The PERSARC model is the first to incorporate known clinical risk factors with the use of different treatments and surgical outcome measures. The developed model is internally validated to provide a reliable prediction of post-operative OS and CILR for patients with primary high-grade ESTS. LEVEL OF SIGNIFICANCE: level III. Copyright © 2017 Elsevier Ltd. All rights reserved.

  11. Stereotactic Radiotherapy in the Treatment of Lung Metastases from Bone and Soft-tissue Sarcomas.

    Science.gov (United States)

    Frakulli, Rezarta; Salvi, Fabrizio; Balestrini, Damiano; Parisi, Alessandro; Palombarini, Marcella; Cammelli, Silvia; Rocca, Michele; Salone, Mariacristina; Longhi, Alessandra; Ferrari, Stefano; Morganti, Alessio G; Frezza, Giovanni

    2015-10-01

    The purpose of this study was to evaluate local control and toxicity in a group of patients treated with stereotactic body radiotherapy (SBRT) for lung metastases (LM) from bone and soft tissue sarcomas. From October 2010 to July 2014, patients with LM from sarcomas not suitable for surgery were treated with daily cone-beam computed tomography-guided SBRT. The dose administered ranged from 30 to 60 Gy in 3-8 fractions. Acute and late toxicity were scored according to Common Terminology Criteria for Adverse Events version 4.0. A total of 24 patients with 68 LM from sarcomas were treated with SBRT. The median follow-up after SBRT was 17 months (range=11-51 months). Two-year actuarial lesion local control and overall survival were 85.9% and 66.4%, respectively. No G3 or greater acute and late toxicities were observed. SBRT is a safe and effective treatment for LM from sarcoma and might be used as an alternative option in patients unfit for surgery. Copyright© 2015 International Institute of Anticancer Research (Dr. John G. Delinassios), All rights reserved.

  12. Soft Tissue Sarcoma, Version 2.2016, NCCN Clinical Practice Guidelines in Oncology.

    Science.gov (United States)

    von Mehren, Margaret; Randall, R Lor; Benjamin, Robert S; Boles, Sarah; Bui, Marilyn M; Conrad, Ernest U; Ganjoo, Kristen N; George, Suzanne; Gonzalez, Ricardo J; Heslin, Martin J; Kane, John M; Koon, Henry; Mayerson, Joel; McCarter, Martin; McGarry, Sean V; Meyer, Christian; O'Donnell, Richard J; Pappo, Alberto S; Paz, I Benjamin; Petersen, Ivy A; Pfeifer, John D; Riedel, Richard F; Schuetze, Scott; Schupak, Karen D; Schwartz, Herbert S; Tap, William D; Wayne, Jeffrey D; Bergman, Mary Anne; Scavone, Jillian

    2016-06-01

    Soft tissue sarcomas (STS) are rare solid tumors of mesenchymal cell origin that display a heterogenous mix of clinical and pathologic characteristics. STS can develop from fat, muscle, nerves, blood vessels, and other connective tissues. The evaluation and treatment of patients with STS requires a multidisciplinary team with demonstrated expertise in the management of these tumors. The complete NCCN Guidelines for Soft Tissue Sarcoma (available at NCCN.org) provide recommendations for the diagnosis, evaluation, and treatment of extremity/superficial trunk/head and neck STS, as well as intra-abdominal/retroperitoneal STS, gastrointestinal stromal tumor, desmoid tumors, and rhabdomyosarcoma. This manuscript discusses guiding principles for the diagnosis and staging of STS and evidence for treatment modalities that include surgery, radiation, chemoradiation, chemotherapy, and targeted therapy. Copyright © 2016 by the National Comprehensive Cancer Network.

  13. Clinical Benefit of Second-Line Palliative Chemotherapy in Advanced Soft-Tissue Sarcoma

    Directory of Open Access Journals (Sweden)

    Anna Minchom

    2010-01-01

    Full Text Available Background. This paper aimed to assess the utility of second-line chemotherapy in patients with advanced soft-tissue sarcoma. Materials and Methods. A retrospective search of a prospectively maintained database identified patients treated between 1991 and 2005. Patients with gastrointestinal stromal tumours, small round cell tumours, and Ewing's sarcoma were excluded. Response was assessed using WHO and RECIST. Patients who achieved stable disease for 6 months or more were classified as having disease control. Results. Three hundred and seventy-nine patients received second-line chemotherapy. Eighty-six (22.7% achieved disease control. Median duration of response was 11 months (95% CI: 9–13. On multivariate analysis, pathological subtype, absence of lung metastases, and the use of combination chemotherapy were independent predictors of disease control. Twenty-eight (16.1% patients who failed to respond to first-line therapy achieved disease control. Eight (2.1% patients had sufficient downstaging to enable complete surgical resection. Progression-free survival was 23% at 6 months. Median overall survival was 8 months (95% CI: 7–10 months. On multivariate analysis, synovial histology and absence of lung metastases were associated with improved survival. Conclusion. Second-line chemotherapy can provide clinical benefit in over 20% of soft-tissue sarcoma patients.

  14. Treatment of Vascular Soft Tissue Sarcomas With Razoxane, Vindesine, and Radiation

    International Nuclear Information System (INIS)

    Rhomberg, Walter; Wink, Anna; Pokrajac, Boris; Eiter, Helmut; Hackl, Arnulf; Pakisch, Brigitte; Ginestet, Angela; Lukas, Peter; Poetter, Richard Prof.

    2009-01-01

    Purpose: In previous studies, razoxane and vindesine together with radiotherapy was proved to be effective in soft tissue sarcomas (STS). Because razoxane leads to a redifferentiation of pathological tumor blood vessels, it was of particular interest to study the influence of this drug combination in vascular soft tissue sarcomas. Methods and Materials: This open multicenter Phase II study was performed by the Austrian Society of Radiooncology. Among 13 evaluable patients (10 angiosarcomas and 3 hemangio-pericytomas), 9 had unresectable measurable disease, 3 showed microscopic residuals, and 1 had a resection with clear margins. They received a basic treatment with razoxane and vindesine supported by radiation therapy. Outcome measures were objective response rates, survival time, and the incidence of distant metastases. Results: In nine patients with measurable vascular soft tissue sarcomas (eight angiosarcomas and one hemangiopericytoma), 6 complete remissions, 2 partial remissions, and 1 minor remission were achieved, corresponding to a major response rate of 89%. A maintenance therapy with razoxane and vindesine of 1 year or longer led to a suppression of distant metastases. The median survival time from the start of the treatment is 23+ months (range, 3-120+) for 12 patients with macroscopic and microscopic residual disease. The progression-free survival at 6 months was 75%. The combined treatment was associated with a low general toxicity, but attention must be given to increased normal tissue reactions. Conclusions: This trimodal treatment leads to excellent response rates, and it suppresses distant metastases when given as maintenance therapy.

  15. overall cost in recurrent soft-tissue sarcoma

    Directory of Open Access Journals (Sweden)

    Arash O. Naghavi

    2017-01-01

    Full Text Available Purpose : Adjuvant brachytherapy (AB with immediate (IR and staged reconstruction (SR are distinct treatment modalities available for patients with recurrent soft tissue sarcoma (STS. Although SR may offer local control and toxicity benefit, it requires additional upfront procedures, and there is no evidence that it improves overall survival. With the importance of value-based care, our goal is to identify which technique is more cost effective. Material and methods : A retrospective review of 22 patients with recurrent extremity STS treated with resection followed by AB alone. Hospital charges were used to compare the cost between SR and IR at the time of initial treatment, at 6-month intervals following surgery, and cumulative cost comparisons at 18 months. Results : Median follow-up was 31 months. Staged reconstruction (n = 12 was associated with an 18-month local control benefit (85% vs. 42%, p = 0.034, compared to IR (n = 10. Staged reconstruction had a longer hospital stay during initial treatment (10 vs. 3 days, p = 0.002, but at 18 months, the total hospital stay was no longer different (11 vs. 11 days. Initially, there was no difference in the cost of SR and IR. With longer follow-up, cost eventually favored SR, which was attributed primarily to the costs associated with local failure (LF. On multivariate analysis, cost of initial treatment was associated with length of hospital stay (~$4.5K per hospital day, p < 0.001, and at 18 months, the cumulative cost was ~175K lower with SR (p = 0.005 and $58K higher with LF (p = 0.02. Conclusions : In recurrent STS, SR has a longer initial hospital stay when compared to IR. At 18 months, SR had lower rates of LF, translating to lower total costs for the patient. SR is the more cost-effective brachytherapy approach in the treatment of STS, and should be considered as healthcare transitions into value-based medicine.

  16. A general aspect on soft-tissue sarcoma and c-kit expression in primitive neuroectodermal tumor and Ewings sarcoma

    International Nuclear Information System (INIS)

    Kara, Ismail O.; Sahin, B.; Gonlusen, G.; Ergin, M.; Erdogan, S.

    2005-01-01

    Within soft-tissue sarcoma, primitive neuroectodermal tumors have been shown to cover a wide spectrum of small round cell sarcomas, including Ewings sarcomas (Es) and primitive neuroectodermal tumors (PET). The role of the stem cell factor/kit pathway has been investigated in different human tumors especially in chronic metallically leukemia and gastrointestinal stromal tumor and an autocrine loop has been assumed in small cell lung carcinoma, and recently in Es and PET. Our aim is to investigate the c-kit expression in Es and PET and also to assessed if c-kit has any role in disease process. We thoroughly searched the archives of the Department of Pathology, Faculty of Medicine, Cukurova University Turkey, between 2000 and 2004; and found 14 ES and 14 PNET paraffin embedded tissues. We carried out the detection of the c-kit expression by immunohistochemical staining. The patients median age was 23.7 +/-14.6 (12 male and 16 female). Five were diagnosed as metastatic disease whereas 23 were diagnosed as non-metastatic disease at admission. The mean follow up period was 38.9 +/- 22.3 months. The main localization of the disease was lower extremity (32.1%), and others were as follows: head and neck 25%, thorax and abdomen 14.3%, pelvic and upper extremity 7.1% (11 were localized skeletal and 17 were extraskeletal). According to treatment modalities, 10 were treated with surgery alone, 11 with surgery and chemotherapy, and 7 with surgery, radiation therapy and also with chemotherapy. The primary tumor was lower than 5 cm in its dimension in 21 patients. While in 5 patients, tumor was more than 5 cm but did not exceed 10 cm, it was >10 cm in 2 patients. The c-kit expression was positive in 7 patients both cytoplasmic and membranously, whereas 8 patients were positive cytoplasmically. In 5 PNET patients, c-kit expression were stained immunohistochemically in over 50% and in 3 of ES patients. There was no significant correlation between c-kit expression and gender

  17. Anticipated Intraoperative Electron Beam Boost, External Beam Radiation Therapy, and Limb-Sparing Surgical Resection for Patients with Pediatric Soft-Tissue Sarcomas of the Extremity: A Multicentric Pooled Analysis of Long-Term Outcomes

    Energy Technology Data Exchange (ETDEWEB)

    Sole, Claudio V., E-mail: cvsole@uc.cl [Department of Oncology, Hospital General Universitario Gregorio Marañón, Madrid (Spain); School of Medicine, Complutense University, Madrid (Spain); Service of Radiation Oncology, Instituto de Radiomedicina, Santiago (Chile); Calvo, Felipe A. [Department of Oncology, Hospital General Universitario Gregorio Marañón, Madrid (Spain); School of Medicine, Complutense University, Madrid (Spain); Polo, Alfredo [Service of Radiation Oncology, Hospital Universitario Ramón y Cajal, Madrid (Spain); Cambeiro, Mauricio [Service of Radiation Oncology, Clínica Universidad de Navarra, Pamplona (Spain); Alvarez, Ana; Gonzalez, Carmen [Service of Radiation Oncology, Hospital General Universitario Gregorio Marañón, Madrid (Spain); Gonzalez, Jose [Service of Pediatric Orthopedics and Traumatology, Hospital General Universitario Gregorio Marañón, Madrid (Spain); San Julian, Mikel [Service of Orthopedics and Traumatology, Clínica Universidad de Navarra, Pamplona (Spain); Martinez-Monge, Rafael [Service of Radiation Oncology, Clínica Universidad de Navarra, Pamplona (Spain)

    2014-09-01

    Purpose: To perform a joint analysis of data from 3 contributing centers within the intraoperative electron-beam radiation therapy (IOERT)-Spanish program, to determine the potential of IOERT as an anticipated boost before external beam radiation therapy in the multidisciplinary treatment of pediatric extremity soft-tissue sarcomas. Methods and Materials: From June 1993 to May 2013, 62 patients (aged <21 years) with a histologic diagnosis of primary extremity soft-tissue sarcoma with absence of distant metastases, undergoing limb-sparing grossly resected surgery, external beam radiation therapy (median dose 40 Gy) and IOERT (median dose 10 Gy) were considered eligible for this analysis. Results: After a median follow-up of 66 months (range, 4-235 months), 10-year local control, disease-free survival, and overall survival was 85%, 76%, and 81%, respectively. In multivariate analysis after adjustment for other covariates, tumor size >5 cm (P=.04) and R1 margin status (P=.04) remained significantly associated with local relapse. In regard to overall survival only margin status (P=.04) retained association on multivariate analysis. Ten patients (16%) reported severe chronic toxicity events (all grade 3). Conclusions: An anticipated IOERT boost allowed for external beam radiation therapy dose reduction, with high local control and acceptably low toxicity rates. The combined radiosurgical approach needs to be tested in a prospective trial to confirm these results.

  18. Anticipated Intraoperative Electron Beam Boost, External Beam Radiation Therapy, and Limb-Sparing Surgical Resection for Patients with Pediatric Soft-Tissue Sarcomas of the Extremity: A Multicentric Pooled Analysis of Long-Term Outcomes

    International Nuclear Information System (INIS)

    Sole, Claudio V.; Calvo, Felipe A.; Polo, Alfredo; Cambeiro, Mauricio; Alvarez, Ana; Gonzalez, Carmen; Gonzalez, Jose; San Julian, Mikel; Martinez-Monge, Rafael

    2014-01-01

    Purpose: To perform a joint analysis of data from 3 contributing centers within the intraoperative electron-beam radiation therapy (IOERT)-Spanish program, to determine the potential of IOERT as an anticipated boost before external beam radiation therapy in the multidisciplinary treatment of pediatric extremity soft-tissue sarcomas. Methods and Materials: From June 1993 to May 2013, 62 patients (aged <21 years) with a histologic diagnosis of primary extremity soft-tissue sarcoma with absence of distant metastases, undergoing limb-sparing grossly resected surgery, external beam radiation therapy (median dose 40 Gy) and IOERT (median dose 10 Gy) were considered eligible for this analysis. Results: After a median follow-up of 66 months (range, 4-235 months), 10-year local control, disease-free survival, and overall survival was 85%, 76%, and 81%, respectively. In multivariate analysis after adjustment for other covariates, tumor size >5 cm (P=.04) and R1 margin status (P=.04) remained significantly associated with local relapse. In regard to overall survival only margin status (P=.04) retained association on multivariate analysis. Ten patients (16%) reported severe chronic toxicity events (all grade 3). Conclusions: An anticipated IOERT boost allowed for external beam radiation therapy dose reduction, with high local control and acceptably low toxicity rates. The combined radiosurgical approach needs to be tested in a prospective trial to confirm these results

  19. Role of radiotherapy and chemotherapy in management of soft tissue sarcomas

    International Nuclear Information System (INIS)

    Harmer, C.; Frampton, M.; Wiltshaw, E.

    1986-01-01

    One hundred eighty patients with soft tissue sarcoma were registered at the Royal Marsden Hospital last year, and the majority of these were seen in the Combined Sarcoma Clinic. This clinic is run by surgeons, radiotherapists, and medical oncologists who see the patients together each week. All the patients have an adequate biopsy, or if surgery has been performed at an outside hospital the previous histology is reviewed. A multidisciplinary team therefore participates in all the important decisions regarding treatment, and the patients continue to be seen in combined consultation during their physiotherapy, rehabilitation, and follow-up. By concentrating large numbers of patients with these rare tumours the scene is set for improved treatment and, hopefully, improved survival

  20. Defining the incidence and clinical significance of lymph node metastasis in soft tissue sarcoma.

    Science.gov (United States)

    Keung, Emily Z; Chiang, Yi-Ju; Voss, Rachel K; Cormier, Janice N; Torres, Keila E; Hunt, Kelly K; Feig, Barry W; Roland, Christina L

    2018-01-01

    The incidence and clinical significance of lymph node metastasis (LNM, N1) in soft tissue sarcoma (STS) is unclear. Recent studies have focused on extremity/trunk STS (ETSTS). We sought to define the subgroup of patients with LNM at sarcoma diagnosis across all disease sites and histologies. We identified and categorized 89,870 STS patients from the National Cancer Data Base (1998-2012) by nodal stage. Pathologically confirmed LNM (pN1) were identified in 1404 patients; 1750 had clinically suspicious but not pathologically confirmed LNM (cN1). Survival analyses were performed by Kaplan-Meier method. Of 3154 patients (3.5%) with pN1 or cN1 LNM at presentation, 1310 had synchronous distant metastasis (M1). LNM affected a small proportion of patients (5.8% head/neck, 5.3% intrathoracic, 5.1% intra-abdominal, 2.0% ETSTS). Angiosarcoma (6%), epithelioid (13%), clear cell (16%), and small cell sarcoma (19%) had the highest incidence of LNM, although liposarcoma, fibrous histiocytoma, and leiomyosarcoma accounted for the greatest number of LNM patients. For pN1M0 disease, median overall survival (OS) was 28.2 months, varying by histology. Among patients with pN1M0 STS, angiosarcoma, clear cell sarcoma, leiomyosarcoma, and fibrous histiocytoma were associated with worse median OS (19.4, 23.8, 27.1, and 29.3 months) compared to epithelioid sarcoma and liposarcoma (49.6 and 56.0 months, p < 0.001). Despite clinical suspicion, pathologic LN evaluation in STS is inconsistently performed. LNM occurs across anatomic disease sites and is unevenly distributed across histologies. Although M1 disease portends poor prognosis regardless of LN status, LNM predicts worse OS in a histology-dependent manner in M0 disease. Published by Elsevier Ltd.

  1. Childhood Soft Tissue Sarcoma Treatment (PDQ®)—Health Professional Version

    Science.gov (United States)

    Pediatric soft tissue sarcomas are a heterogenous group of malignant tumors that originate from primitive mesenchymal tissue and account for 7% of all childhood tumors. Get detailed information about clinical presentation, diagnosis, prognosis, and treatment of newly diagnosed and recurrent soft tissue sarcoma in this summary for clinicians.

  2. A Clinical phase I/II trial to investigate preoperative dose-escalated intensity-modulated radiation therapy (IMRT and intraoperative radiation therapy (IORT in patients with retroperitoneal soft tissue sarcoma

    Directory of Open Access Journals (Sweden)

    Roeder Falk

    2012-07-01

    Full Text Available Abstract Background Local control rates in patients with retroperitoneal soft tissue sarcoma (RSTS remain disappointing even after gross total resection, mainly because wide margins are not achievable in the majority of patients. In contrast to extremity sarcoma, postoperative radiation therapy (RT has shown limited efficacy due to its limitations in achievable dose and coverage. Although Intraoperative Radiation Therapy (IORT has been introduced in some centers to overcome the dose limitations and resulted in increased outcome, local failure rates are still high even if considerable treatment related toxicity is accepted. As postoperative administration of RT has some general disadvantages, neoadjuvant approaches could offer benefits in terms of dose escalation, target coverage and reduction of toxicity, especially if highly conformal techniques like intensity-modulated radiation therapy (IMRT are considered. Methods/design The trial is a prospective, one armed, single center phase I/II study investigating a combination of neoadjuvant dose-escalated IMRT (50–56 Gy followed by surgery and IORT (10–12 Gy in patients with at least marginally resectable RSTS. The primary objective is the local control rate after five years. Secondary endpoints are progression-free and overall survival, acute and late toxicity, surgical resectability and patterns of failure. The aim of accrual is 37 patients in the per-protocol population. Discussion The present study evaluates combined neoadjuvant dose-escalated IMRT followed by surgery and IORT concerning its value for improved local control without markedly increased toxicity. Trial registration NCT01566123

  3. A Clinical phase I/II trial to investigate preoperative dose-escalated intensity-modulated radiation therapy (IMRT) and intraoperative radiation therapy (IORT) in patients with retroperitoneal soft tissue sarcoma

    International Nuclear Information System (INIS)

    Roeder, Falk; Hensley, Frank W; Buechler, Markus W; Debus, Juergen; Koch, Moritz; Weitz, Juergen; Bischof, Marc; Schulz-Ertner, Daniela; Nikoghosyan, Anna V; Huber, Peter E; Edler, Lutz; Habl, Gregor; Krempien, Robert; Oertel, Susanne; Saleh-Ebrahimi, Ladan

    2012-01-01

    Local control rates in patients with retroperitoneal soft tissue sarcoma (RSTS) remain disappointing even after gross total resection, mainly because wide margins are not achievable in the majority of patients. In contrast to extremity sarcoma, postoperative radiation therapy (RT) has shown limited efficacy due to its limitations in achievable dose and coverage. Although Intraoperative Radiation Therapy (IORT) has been introduced in some centers to overcome the dose limitations and resulted in increased outcome, local failure rates are still high even if considerable treatment related toxicity is accepted. As postoperative administration of RT has some general disadvantages, neoadjuvant approaches could offer benefits in terms of dose escalation, target coverage and reduction of toxicity, especially if highly conformal techniques like intensity-modulated radiation therapy (IMRT) are considered. The trial is a prospective, one armed, single center phase I/II study investigating a combination of neoadjuvant dose-escalated IMRT (50–56 Gy) followed by surgery and IORT (10–12 Gy) in patients with at least marginally resectable RSTS. The primary objective is the local control rate after five years. Secondary endpoints are progression-free and overall survival, acute and late toxicity, surgical resectability and patterns of failure. The aim of accrual is 37 patients in the per-protocol population. The present study evaluates combined neoadjuvant dose-escalated IMRT followed by surgery and IORT concerning its value for improved local control without markedly increased toxicity. NCT01566123

  4. Subcentimeter Pulmonary Nodules Detected in Patients with Sarcoma

    Directory of Open Access Journals (Sweden)

    Michelle S. Ginsberg

    2000-01-01

    Full Text Available Background. Subcentimeter pulmonary nodules are being detected with increasing frequency in patients with sarcoma due to the greater use of chest CT, the advent of helical (spiral CT scanning and multidetector scanners, and the attendant decrease in image section thickness.Assessing the clinical significance of these pulmonary nodules is of particular importance in sarcoma patients, due to the frequent occurrence of pulmonary metastasis from sarcomas.

  5. Small soft tissue sarcomas do metastasize

    DEFF Research Database (Denmark)

    Styring, Emelie; Hartman, Linda; Nilbert, Mef

    2014-01-01

    had histologic high-grade tumors. RESULTS: None of the 48 patients with low-grade tumors developed metastases, whereas 24 of 181 patients with high-grade tumors (13 %) tumors did. Presence of either tumor necrosis or vascular invasion predicted development of metastases with a hazard ratio of 2.9 (95...... necrosis and vascular invasion were the major predictors of metastatic disease in this subset. Tumors with both these risk factors metastasized in 8 of 18 patients, which corresponds to a 12-fold increased risk of metastasis. These findings suggest that although small STS generally are linked to a good...... prognosis, necrosis and vascular invasion are features indicating biologically aggressive tumors for which treatment and surveillance should equal that for larger tumors....

  6. Bone and soft tissue sarcomas during pregnancy: A narrative review of the literature

    Directory of Open Access Journals (Sweden)

    George Zarkavelis

    2016-07-01

    Full Text Available Bone or soft tissue sarcomas are rarely diagnosed during pregnancy. Until today 137 well documented cases have been reported in the English literature between 1963 and 2014. Thirty-eight pregnant mothers were diagnosed with osteosarcoma, Ewing’s sarcoma or chondrosarcoma, whereas 95 other cases of soft tissue sarcomas of various types have been documented. We present the clinical picture and therapeutic management of this coexistence.

  7. Key Advances in the Systemic Therapy for Soft tissue Sarcomas: Current Status and Future Directions

    Directory of Open Access Journals (Sweden)

    Neelesh Soman

    2013-10-01

    Full Text Available Soft tissue sarcomas (STS represent a heterogeneous group of diverse neoplasms of mesenchymal origin. Once relapsed from standard therapy, STS patients have limited treatment options especially those that present with advanced or metastatic disease. In this review article, we highlight recent clinical data that led to the US Food and Drug Administration (FDA approval of pazopanib (Votrient® for STS and regorafenib (Stivarga®, BAY 73-4506 in gastrointestinal stromal tumours. We also review ongoing safety/efficacy data for trabectedin (Yondelis®, ET-743, and data from clinical studies of ridaforolimus (AP23573; MK-8669 and palifosfamide (ZIO-201. We provide a list of some promising ongoing trials in soft tissue sarcomas including first line studies of TH-302 and trabectedin. Finally, our article delves into recent advances in our understanding of the molecular pathogenesis of STS and novel therapies that might be explored as treatment options for specific STS histologies.

  8. Thallium scintigraphy used in the evaluation of soft tissue sarcoma

    International Nuclear Information System (INIS)

    Pilloni, A.; Brooks, R.

    1998-01-01

    Full text: The use of thallium scintigraphy in tumour localisation is a promising diagnostic modality. Thallium has a good affinity for numerous tumour types including sarcomas. Its properties as a potassium analogue result in active uptake via the sodium- potassium ATP-ase pump enabling its use in evaluating tumour extent, viability, staging, localisation and treatment. This case illustrates the role of thallium in tumour staging. A 67-year-old woman with a fibrous histiocytoma resected from the right foot four months previously presented with pain in the ribs, lower back and feet. A bone scan was performed to assess the possibility of metastatic disease. Several abnormal foci were seen on the bone scan suggestive of metastatic disease. A thallium scan was performed to confirm the bone scan result after a subsequent CT scan showed no evidence of this extensive disease. Static images of the whole body were acquired on an Elscinct 400AG 20 minutes post i.v. injection of 3.5 mCi of thallium. Focal areas of increased thallium uptake were seen in the 11th rib posteriorly and in the left sacroiliac region corresponding with sites on the bone scan suggesting metastases. However, an extensive area of uptake was also observed in the right inguinal region which had no corresponding abnormalities in the bone study. This was thought to be tumour metastasis in an inguinal Iymph node, which was retrospectively confirmed on an earlier CT scan of the pelvis. This study demonstrates the usefulness of thallium in the detection of soft tissue tumours and metastases. Thallium also exhibits potential in the assessment of tumour extent and viability pre- and post-treatment. This potential needs to be explored further in patient management

  9. A prognostic profile of hypoxia-induced genes for localised high-grade soft tissue sarcoma

    DEFF Research Database (Denmark)

    Aggerholm-Pedersen, Ninna; Sørensen, Brita Singers; Overgaard, Jens

    2016-01-01

    sarcoma (STS). METHODS: The hypoxia-induced gene quantification was performed by real-time quantitative PCR (RT-qPCR) of formalin-fixed, paraffin-embedded tissue samples. The gene expression cut-points were determined in a test cohort of 55 STS patients and used to allocate each patient into a more......BACKGROUND: For decades, tumour hypoxia has been pursued as a cancer treatment target. However, prognostic and predictive biomarkers are essential for the use of this target in the clinic. This study investigates the prognostic value of a hypoxia-induced gene profile in localised soft tissue...

  10. Prognostic Metabolite Biomarkers for Soft Tissue Sarcomas Discovered by Mass Spectrometry Imaging

    Science.gov (United States)

    Lou, Sha; Balluff, Benjamin; Cleven, Arjen H. G.; Bovée, Judith V. M. G.; McDonnell, Liam A.

    2017-02-01

    Metabolites can be an important read-out of disease. The identification and validation of biomarkers in the cancer metabolome that can stratify high-risk patients is one of the main current research aspects. Mass spectrometry has become the technique of choice for metabolomics studies, and mass spectrometry imaging (MSI) enables their visualization in patient tissues. In this study, we used MSI to identify prognostic metabolite biomarkers in high grade sarcomas; 33 high grade sarcoma patients, comprising osteosarcoma, leiomyosarcoma, myxofibrosarcoma, and undifferentiated pleomorphic sarcoma were analyzed. Metabolite MSI data were obtained from sections of fresh frozen tissue specimens with matrix-assisted laser/desorption ionization (MALDI) MSI in negative polarity using 9-aminoarcridine as matrix. Subsequent annotation of tumor regions by expert pathologists resulted in tumor-specific metabolite signatures, which were then tested for association with patient survival. Metabolite signals with significant clinical value were further validated and identified by high mass resolution Fourier transform ion cyclotron resonance (FTICR) MSI. Three metabolite signals were found to correlate with overall survival ( m/z 180.9436 and 241.0118) and metastasis-free survival ( m/z 160.8417). FTICR-MSI identified m/z 241.0118 as inositol cyclic phosphate and m/z 160.8417 as carnitine.

  11. A prospective study of proton reirradiation for recurrent and secondary soft tissue sarcoma.

    Science.gov (United States)

    Guttmann, David M; Frick, Melissa A; Carmona, Ruben; Deville, Curtiland; Levin, William P; Berman, Abigail T; Chinniah, Chidambaram; Hahn, Stephen M; Plastaras, John P; Simone, Charles B

    2017-08-01

    Proton reirradiation for sarcoma has not been previously described. We hypothesized that this strategy would provide favorable toxicity and survival outcomes. Patients with soft tissue sarcoma in a previously-irradiated field were enrolled on a prospective trial of proton reirradiation. The primary endpoint was provider-reported acute toxicity. Secondary endpoints included late toxicities, local control, and overall survival. 23 patients underwent proton reirradiation. Median time between radiation courses was 40.7months (range 10-272). No grade 4-5 toxicities were observed. One patient (4%) experienced acute grade 3 dysphagia. Common grade 2 acute toxicities were fatigue (26%), anorexia (17%), and urinary incontinence (13%). There were two grade 3 late wound infections (10%) and one grade 3 late wound complication (5%). Grade 2 late complications included lymphedema (10%), fracture (5%), and fibrosis (5%). At a median follow-up of 36months, the 3-year cumulative incidence of local failure was 41% (95% CI [20-63%]). Median overall survival and progression-free survival were 44 and 29months, respectively. In extremity patients, amputation was spared in 7/10 (70%). Proton reirradiation of recurrent/secondary soft tissue sarcomas is well tolerated. While longer follow-up is needed, early survival outcomes in this high-risk population are encouraging. Copyright © 2017 Elsevier B.V. All rights reserved.

  12. Multidisciplinary ''limb salvage'' treatment of soft tissue and skeletal sarcomas

    International Nuclear Information System (INIS)

    Weisenburger, T.H.; Eilber, F.R.; Grant, T.T.; Morton, D.L.; Mirra, J.J.; Steinberg, M.; Rickles, D.

    1984-01-01

    The goal of treating primary bone and soft tissue tumors, as with most other malignancies, is to control the disease locally and systematically while preserving as much function as possible. For soft tissue sarcomas the results following radical excision and post-operative radiation therapy with preservation of the limb have equaled the control rates of amputation. However, local recurrence rates of approximately 25-30% are reported for high-grade lesions of the proximal lower extremity. Amputations provides excellent local control for osteosarcoma but the functional results may be less than optimal. In an attempt to achieve limb salvage for these tumors, a multidisciplinary protocol was developed using intra-arterial doxorubicin, pre-operative radiation therapy and limb-sparing radical wide excision followed by post-operative chemotherapy for presumed micro-metastatic disease

  13. High-grade soft tissue sarcoma arising in a desmoid tumor: case report and review of the literature.

    Science.gov (United States)

    Bertucci, François; Faure, Marjorie; Ghigna, Maria-Rosa; Chetaille, Bruno; Guiramand, Jérôme; Moureau-Zabotto, Laurence; Sarran, Anthony; Perrot, Delphine

    2015-01-01

    Desmoid tumors are rare benign monoclonal fibroblastic tumors. Their aggressiveness is local with no potential for metastasis or dedifferentiation. Here we report on a 61-year-old patient who presented a locally advanced breast desmoid tumor diagnosed 20 years after post-operative radiotherapy for breast carcinoma. After 2 years of medical treatment, a high-grade undifferentiated pleomorphic soft tissue sarcoma arose within the desmoid tumor. Despite extensive surgery removing both tumors, the patient showed locoregional relapse by the sarcoma, followed by multimetastatic progression, then death 25 months after the surgery. The arising of a soft tissue sarcoma in a desmoid tumor is an exceptional event since our case is the fourth one reported so far in literature. It reinforces the need for timely and accurate diagnosis when a new mass develops in the region of a preexisting desmoid tumor, and more generally when a desmoid tumor modifies its clinical or radiological aspect.

  14. MINI REVIEW ARTICLE:Immunohistochemistry of Epithelioid Soft Tissue Sarcomas, Literature Review Based on Case Studies

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    Megha Joshi

    2012-07-01

    Full Text Available Neoplasms with epithelioid histology may be diagnostically challenging. Immunohisto chemistry (IHC can aid in confirming thedifferential diagnosis of mesotheliomas, melanomas, lymphomas, and soft tissue sarcomas, all tumors that can present with an epithelioid histology. Immunohistochemistry can also assist in confirming the type of sarcomas. Using cases diagnosed in acommunity hospital setting over a ten year period, the use of IHC in sarcomas will be illustrated.

  15. Phase II Study of Neoadjuvant Bevacizumab and Radiotherapy for Resectable Soft Tissue Sarcomas

    International Nuclear Information System (INIS)

    Yoon, Sam S.; Duda, Dan G.; Karl, Daniel L.; Kim, Tae-Min; Kambadakone, Avinash R.; Chen, Yen-Lin; Rothrock, Courtney; Rosenberg, Andrew E.; Nielsen, G. Petur; Kirsch, David G.; Choy, Edwin; Harmon, David C.; Hornicek, Francis J.; Dreyfuss, Jonathan; Ancukiewicz, Marek

    2011-01-01

    Purpose: Numerous preclinical studies have demonstrated that angiogenesis inhibitors can increase the efficacy of radiotherapy (RT). We sought to examine the safety and efficacy of bevacizumab (BV) and RT in soft tissue sarcomas and explore biomarkers to help determine the treatment response. Methods and Materials: Patients with ≥5 cm, intermediate- or high-grade soft tissue sarcomas at significant risk of local recurrence received neoadjuvant BV alone followed by BV plus RT before surgical resection. Correlative science studies included analysis of the serial blood and tumor samples and serial perfusion computed tomography scans. Results: The 20 patients had a median tumor size of 8.25 cm, with 13 extremity, 1 trunk, and 6 retroperitoneal/pelvis tumors. The neoadjuvant treatment was well tolerated, with only 4 patients having Grade 3 toxicities (hypertension, liver function test elevation). BV plus RT resulted in ≥80% pathologic necrosis in 9 (45%) of 20 tumors, more than double the historical rate seen with RT alone. Three patients had a complete pathologic response. The median microvessel density decreased 53% after BV alone (p <.05). After combination therapy, the median tumor cell proliferation decreased by 73%, apoptosis increased 10.4-fold, and the blood flow, blood volume, and permeability surface area decreased by 62–72% (p <.05). Analysis of gene expression microarrays of untreated tumors identified a 24-gene signature for treatment response. The microvessel density and circulating progenitor cells at baseline and the reduction in microvessel density and plasma soluble c-KIT with BV therapy also correlated with a good pathologic response (p <.05). After a median follow-up of 20 months, only 1 patient had developed local recurrence. Conclusions: The results from the present exploratory study indicated that BV increases the efficacy of RT against soft tissue sarcomas and might reduce the incidence of local recurrence. Thus, this regimen warrants

  16. THE POTENTIAL VALUE OF ULTRASOUND IN DIAGNOSIS OF SOFT TISSUE SARCOMA (LITERATURE REVIEW

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    I. G. Frolova

    2015-01-01

    Full Text Available Literature data on the potential value of ultrasound imaging in diagnosis of soft tissue sarcoma were analyzed. Ultrasound in B-regime was used to assess the extent of soft tissue sarcoma, Doppler ultrasonography was used to study tumor vascularization and sonoelastography was useful to differentiate benign from malignant tumors of soft tissues. The analysis of diagnostic value of ultrasound in detection of soft tissue lesions was carried out.  Criteria characterizing various histological types of tumors were identified.

  17. Effectiveness and Morbidity Associated With Reirradiation in Conservative Salvage Management of Recurrent Soft-Tissue Sarcoma

    International Nuclear Information System (INIS)

    Indelicato, Daniel J.; Meadows, Kenyon; Gibbs, Charles P.; Morris, Christopher G.; Scarborough, Mark T.; Zlotecki, Robert A.

    2009-01-01

    Purpose: The management of isolated local recurrence of soft-tissue sarcoma is therapeutically complex, and functional conservative management is preferable to radical or amputative salvage surgery. This study reviews University of Florida experience using conservative resection and reirradiation to manage isolated local recurrences of soft-tissue sarcoma. Methods and Materials: Between 1976 and 2005, a total of 14 patients who underwent primary conservative resection and irradiation developed isolated local recurrence and were managed with salvage conservative resection and reirradiation. Of the patients treated, 3 had tumors of the distal extremity, 8 had tumors of the proximal extremity, and 3 had tumors of the trunk. At the time of recurrence, 64% of tumors were greater than 5 cm and 79% were high grade. In combination with gross total resection, 13 of 14 patients received external beam radiotherapy with or without brachytherapy, and 1 patient was treated with brachytherapy alone. Two patients received chemotherapy. Results: The median follow-up was 30 months, and no living patients were lost during follow-up. From retreatment, the 5-year actuarial overall survival, cause-specific survival, and local control rates were 40%, 40%, and 18% respectively. There was a 50% incidence of serious complication requiring either reoperation or leading to permanent functional impairment. Of the 14 patients, only 1 has remained disease free and without significant complications. No treatment factors, including achieving wide surgical margins or delivering higher radiation dosages, seemed to confer an advantage in local control. Conclusions: Salvage therapy for management of locally recurrent soft-tissue sarcoma is challenging, and the effects of reoperation and reirradiation can be severe

  18. Neoadjuvant chemotherapy for high-grade soft-tissue sarcomas of the limbs

    International Nuclear Information System (INIS)

    Ramos, Pedro; Gonzalez, Manuel; Perry, Fernando; Cardona, Andres Felipe

    2005-01-01

    Background: the use of neoadjuvant chemotherapy for high-grade soft-tissue sarcomas of the limbs continues to be an area of controversy; however, the number of clinical studies favoring the use of an anthracycline and iphosphamide-based regimen is increasing steadily. This approach may provide some advantages for facilitating the surgical resection of the tumor and for local disease control. The historical 5-year survival rate of approximately 50% in this high-risk group treated with local therapy alone represents a poor standard of care; thus, there is a need to incorporate systemic therapy early in the management of these patients. Objective: to describe the role of neoadjuvant chemotherapy in the treatment of soft-tissue sarcomas. Materials and methods: the records of 42 patients who attended the national cancer institute of Colombia in search for management of primary soft-tissue sarcomas were retrospectively reviewed. Ten patients with high-grade tumors larger than 8 cm, treated from June 2000 to February 2002 with neoadjuvant chemotherapy based on an anthracycline and iphosphamide regimen, plus vincristin and cisplatinum in selected cases, followed by surgery and adjuvant therapy with chemotherapy combined with local radiotherapy, were included. Evaluations of objective tumor response, survival, and toxicity were carried out. Results: after neoadjuvant therapy, s ix patients underwent conservative and limb-salvage surgery, three required radical interventions, and one refused surgical treatment. Seven experienced an objective response: it was complete in four and partial in three; the disease kept stable in two patients, and the tumor progressed in one case. After an average 46-month follow-up, four patients were permanently free of disease. Hematological and gastrointestinal toxicity was remarkable, and no patient had a long-term morbidity related to the treatment. Conclusions: this limited retrospective review suggests an advantage for the use of

  19. Intensity of recent years in the investigation of soft tissue sarcoma.

    Science.gov (United States)

    Blay, Jean-Yves

    2017-06-01

    The European Medicines Agency's approval in 2007 of trabectedin for treatment of advanced soft tissue sarcoma (STS) - the first new agent approved in this indication for more than 30 years - coincided with the start of an exciting era in sarcoma research. Over the past 10 years, several other cytotoxic agents and targeted therapies have been approved for use in advanced STS and research efforts continue to intensify. In parallel, the virtual explosion of new evidence has presented its own set of challenges in terms of interpreting the data and understanding how it may apply to individual patients. This review provides a critical appraisal of several key studies published in the field of advanced STS in the past decade.

  20. A review of soft-tissue sarcomas: translation of biological advances into treatment measures

    Directory of Open Access Journals (Sweden)

    Hoang NT

    2018-05-01

    Full Text Available Ngoc T Hoang,* Luis A Acevedo,* Michael J Mann, Bhairavi Tolani Thoracic Oncology Program, Department of Surgery, Helen Diller Family Comprehensive Cancer Center, University of California, San Francisco, CA, USA *These authors contributed equally to this work Abstract: Soft-tissue sarcomas are rare malignant tumors arising from connective tissues and have an overall incidence of about five per 100,000 per year. While this diverse family of malignancies comprises over 100 histological subtypes and many molecular aberrations are prevalent within specific sarcomas, very few are therapeutically targeted. Instead of utilizing molecular signatures, first-line sarcoma treatment options are still limited to traditional surgery and chemotherapy, and many of the latter remain largely ineffective and are plagued by disease resistance. Currently, the mechanism of sarcoma oncogenesis remains largely unknown, thus necessitating a better understanding of pathogenesis. Although substantial progress has not occurred with molecularly targeted therapies over the past 30 years, increased knowledge about sarcoma biology could lead to new and more effective treatment strategies to move the field forward. Here, we discuss biological advances in the core molecular determinants in some of the most common soft-tissue sarcomas – liposarcoma, angiosarcoma, leiomyosarcoma, rhabdomyosarcoma, Ewing’s sarcoma, and synovial sarcoma – with an emphasis on emerging genomic and molecular pathway targets and immunotherapeutic treatment strategies to combat this confounding disease. Keywords: sarcoma, molecular pathways, immunotherapy, genomics

  1. Intraoperative extracorporeal autogenous irradiated tendon grafts for functional limb salvage surgery of soft tissue sarcomas of the wrist and hand.

    Science.gov (United States)

    Omori, Shinsuke; Hamada, Kenichiro; Outani, Hidetatsu; Oshima, Kazuya; Joyama, Susumu; Tomita, Yasuhiko; Naka, Norifumi; Araki, Nobuhito; Yoshikawa, Hideki

    2015-05-12

    In patients with soft tissue sarcoma of the wrist and hand, limb salvage operation is extremely challenging for surgeons in attempting a complete tumor resection with negative surgical margins. In this study, we report four patients with soft tissue sarcoma of the wrist and hand treated by limb salvage operation with intraoperative extracorporeal autogenous irradiated tendon grafts. The patients were all male, and the mean age at the time of surgery was 45 years. Histological diagnoses included clear cell sarcoma in two patients, synovial sarcoma in one, and angiosarcoma in one. All four patients had high grade tumors, wherein three had American Joint Committee on Cancer (AJCC) stage III disease and one with AJCC stage IV disease. The tumors were resected en bloc with involved tendons. The tendons were isolated from the resected tissues, irradiated ex vivo, and re-implanted into the host tendons. In one patient, the bone was resected additionally because of tumor invasion to the bone. Hand function was evaluated using Musculoskeletal Tumor Society (MSTS) rating system. Of the four patients, three died of distant metastatic disease. The remaining patient lives and remains disease-free. The mean follow-up period was 33 months. One patient had local recurrence outside the irradiated graft at 20 months after surgery. The functional rating was 22. Lower scores were seen in patients with reconstruction of flexor tendons than extensor tendons. Limb salvage operation with intraoperative extracorporeal autogenous irradiated tendon grafts is an acceptable method in selected patients with soft tissue sarcoma of the wrist and hand.

  2. Quantitative dynamic ¹⁸FDG-PET and tracer kinetic analysis of soft tissue sarcomas.

    Science.gov (United States)

    Rusten, Espen; Rødal, Jan; Revheim, Mona E; Skretting, Arne; Bruland, Oyvind S; Malinen, Eirik

    2013-08-01

    To study soft tissue sarcomas using dynamic positron emission tomography (PET) with the glucose analog tracer [(18)F]fluoro-2-deoxy-D-glucose ((18)FDG), to investigate correlations between derived PET image parameters and clinical characteristics, and to discuss implications of dynamic PET acquisition (D-PET). D-PET images of 11 patients with soft tissue sarcomas were analyzed voxel-by-voxel using a compartment tracer kinetic model providing estimates of transfer rates between the vascular, non-metabolized, and metabolized compartments. Furthermore, standard uptake values (SUVs) in the early (2 min p.i.; SUVE) and late (45 min p.i.; SUVL) phases of the PET acquisition were obtained. The derived transfer rates K1, k2 and k3, along with the metabolic rate of (18)FDG (MRFDG) and the vascular fraction νp, was fused with the computed tomography (CT) images for visual interpretation. Correlations between D-PET imaging parameters and clinical parameters, i.e. tumor size, grade and clinical status, were calculated with a significance level of 0.05. The temporal uptake pattern of (18)FDG in the tumor varied considerably from patient to patient. SUVE peak was higher than SUVL peak for four patients. The images of the rate constants showed a systematic pattern, often with elevated intensity in the tumors compared to surrounding tissue. Significant correlations were found between SUVE/L and some of the rate parameters. Dynamic (18)FDG-PET may provide additional valuable information on soft tissue sarcomas not obtainable from conventional (18)FDG-PET. The prognostic role of dynamic imaging should be investigated.

  3. Prognostic value of proliferation in pleomorphic soft tissue sarcomas

    DEFF Research Database (Denmark)

    Seinen, Jojanneke M; Jönsson, Mats; Bendahl, Pär-Ola O

    2012-01-01

    = 1.6-12.1), Top2a (hazard ratio = 2.2, CI = 1.2-3.5) and high S-phase fraction (hazard ratio = 1.8, CI = 1.2-3.7) significantly correlated with risk for metastasis. When combined with currently used prognostic factors, Ki-67, S-phase fraction and Top2a fraction contributed to refined identification...... of prognostic risk groups. Proliferation, as assessed by expression of Ki-67 and Top2a and evaluation of S-phase fraction and applied to statistical decision-tree models, provides prognostic information in soft tissue sarcomas of the extremity and trunk wall. Though proliferation contributes independently...... to currently applied prognosticators, its role is particularly strong when few other factors are available, which suggests a role in preoperative decision-making related to identification of high-risk individuals who would benefit from neoadjuvant therapy....

  4. Postoperative radiation boost does not improve local recurrence rates in extremity soft tissue sarcomas

    International Nuclear Information System (INIS)

    Alamanda, Vignesh K.; Schwartz, Herbert S.; Holt, Ginger E.; Song, Yanna; Shinohara, Eric

    2014-01-01

    The standard of care for extremity soft tissue sarcomas continues to be negative-margin limb salvage surgery. Radiotherapy is frequently used as an adjunct to decrease local recurrence. No differences in survival have been found between preoperative and postoperative radiotherapy regimens. However, it is uncertain if the use of a postoperative boost in addition to preoperative radiotherapy reduces local recurrence rates. This retrospective review evaluated patients who received preoperative radiotherapy (n = 49) and patients who received preoperative radiotherapy with a postoperative boost (n=45). The primary endpoint analysed was local recurrence, with distant metastasis and death due to sarcoma analysed as secondary endpoints. Wilcoxon rank-sum test and either χ 2 or Fisher's exact test were used to compare variables. Multivariable regression analyses were used to take into account potential confounders and identify variables that affected outcomes. No differences in the proportion or rate of local recurrence, distant metastasis or death due to sarcoma were observed between the two groups (P>0.05). The two groups were similarly matched with respect to demographics such as age, race and sex and tumour characteristics including excision status, tumour site, size, depth, grade, American Joint Committee on Cancer stage, chemotherapy receipt and histological subtype (P>0.05). The postoperative boost group had a larger proportion of patients with positive microscopic margins (62% vs 10%; P<0.001). No differences in rates of local recurrence, distant metastasis or death due to sarcoma were found in patients who received both pre- and postoperative radiotherapy when compared with those who received only preoperative radiotherapy.

  5. Prognostic relevance of {sup 18}F-FDG PET uptake in patients with locally advanced, extremity soft tissue sarcomas undergoing neoadjuvant isolated limb perfusion with TNF-α and melphalan

    Energy Technology Data Exchange (ETDEWEB)

    Andreou, Dimosthenis [Muenster University Hospital, Department of General Orthopedics and Tumor Orthopedics, Muenster (Germany); HELIOS Klinikum Berlin-Buch, Department of Orthopedic Oncology, Sarcoma Center Berlin-Brandenburg, Berlin (Germany); Boldt, Henrike [HELIOS Klinikum Berlin-Buch, Department of Nuclear Medicine, Berlin (Germany); Pink, Daniel [HELIOS Klinikum Bad Saarow, Department of Hematology, Oncology and Palliative Care, Sarcoma Center Berlin-Brandenburg, Bad Saarow (Germany); Jobke, Bjoern [HELIOS Klinikum Berlin-Buch, Department of Radiology, Berlin (Germany); Werner, Mathias [HELIOS Klinikum Emil von Behring, Department of Pathology, Sarcoma Center Berlin-Brandenburg, Berlin (Germany); Schuler, Markus [University Hospital Carl Gustav Carus Dresden, Department of Internal Medicine I, Dresden (Germany); Reichardt, Peter [HELIOS Klinikum Berlin-Buch, Department of Interdisciplinary Oncology, Sarcoma Center Berlin-Brandenburg, Berlin (Germany); Tunn, Per-Ulf [HELIOS Klinikum Berlin-Buch, Department of Orthopedic Oncology, Sarcoma Center Berlin-Brandenburg, Berlin (Germany)

    2014-06-15

    The objective of this study was to determine whether {sup 18}F-fluorodeoxyglucose (FDG) positron emission tomography (PET) can adequately assess the risk of systemic disease progression in patients with primary, localized, high-grade soft tissue sarcomas of the extremities undergoing neoadjuvant isolated limb perfusion (ILP) with tumour necrosis factor and melphalan. This was a retrospective analysis of the files of 35 patients who underwent a PET or PET/CT scan prior to and after ILP followed by surgical resection with curative intent between 2006 and 2012. SUV{sub max1} was defined as the maximum standardized uptake value (SUV) at diagnosis, SUV{sub max2} as the maximum SUV after ILP and ΔSUV{sub max} as the percentage difference between SUV{sub max1} and SUV{sub max2}. The median follow-up was 40 months for all patients. The median SUV{sub max1} amounted to 7.6, while the median SUV{sub max2} was 4.7. The median ΔSUV{sub max} was -44 %. Overall survival (OS) probability at 2 and 5 years amounted to 78 and 70 %, respectively, while metastasis-free survival (MFS) probability at 2 and 5 years was 67 and 64 %, respectively. Receiver-operating characteristic (ROC) curve analysis showed that both SUV{sub max2} and ΔSUV{sub max} could predict systemic disease progression, while SUV{sub max1} could not adequately identify patients who went on to develop metastatic disease. The optimal cut-off value was 6.9 for SUV{sub max2} and -31 % for ΔSUV{sub max}. Patients with an SUV{sub max2} <6.9 had a 2-year MFS of 80 %, compared to 31 % for patients with an SUV{sub max2} ≥ 6.9 (p < 0.001). Patients with a ΔSUV{sub max} < -31 %, i.e. patients with a higher metabolic response, had an MFS of 76 % at 2 years, compared to 42 % for patients with a ΔSUV{sub max} ≥ -31 % (p = 0.050). SUV{sub max} after ILP for primary, locally advanced, non-metastatic high-grade soft tissue sarcomas of the extremities appears to be significantly correlated with prognosis. Whether patients

  6. Can MRI diffusion-weighted imaging identify postoperative residual/recurrent soft-tissue sarcomas?

    Directory of Open Access Journals (Sweden)

    Mai Maher ElDaly

    2018-01-01

    Full Text Available Purpose: The aim of this study was to evaluate contrast-enhanced magnetic resonance imaging (CE-MRI and quantitative diffusion-weighted imaging (DWI with apparent diffusion coefficient (ADC mapping in the detection of recurrent/residual postoperative soft tissue sarcomas. Materials and Methods: This study included 36 patients; 27 patients had postoperative recurrent/residual soft tissue sarcomas and 9 patients had postoperative and treatment-related changes (inflammation/fibrosis. The DWI was obtained with 3 b values including 0, 400, and 800 s/mm2. Calculation of the ADC value of the lesion was done via placing the region of interest (ROI to include the largest area of the lesion. ADC values were compared to histopathology. Results: Our results showed that including CE-MRI improved the diagnostic accuracy and sensitivity in recurrence detection compared to conventional non-enhanced sequences. However, it showed low specificity (55.56% with a high false-positive rate that may lead to an unnecessary biopsy of a mass such as region of postoperative scar tissue. Conclusion: The joint use of gadolinium-enhanced MRI and quantitative DWI with ADC mapping offer added value in the detection of recurrent/residual postoperative soft tissue sarcoma. This combined use increased both the diagnostic sensitivity and specificity with a cut-off average ADC value for detecting nonmyxoid recurrent/residual lesions ≤1.3 × 10−3 mm2/s (100% specificity and 90.48% sensitivity. Our results showed limited value of DWI with ADC mapping in assessing myxoid sarcomatous tumor recurrences.

  7. Detection of Human Herpes Virus 8 in Kaposi's sarcoma tissues at ...

    African Journals Online (AJOL)

    Introduction: Human herpes virus-8, a γ2-herpes virus, is the aetiological agent of Kaposi sarcoma. Recently, Kaposi's sarcoma cases have increased in Zambia. However, the diagnosis of this disease is based on morphological appearance of affected tissues using histological techniques, and the association with its ...

  8. Feasibility of combined modality therapy for localized high-grade soft tissue sarcomas in adults

    International Nuclear Information System (INIS)

    Blum, R.H.; Greenberger, J.S.; Wilson, R.E.; Corson, J.M.

    1979-01-01

    Seventeen consecutive patients with localized, high grade soft tissue sarcomas had resection of their primary tumor, radiation therapy and chemotherapy. The soft tissue sarcoma was primary in 14 patients and regionally recurrent in 3 patients. Chemotherapy consisted of cyclophosphamide 500 mg/M 2 day 1, Adriamycin (ADR) 60 mg/M 2 day 2, and DTIC 400 mg/M 2 days 1 and 2, given every 21 days to a maximum ADR dose of 450 mg/M 2 . Cyclophosphamide and DTIC were then given to a total duration of 1 year. Radiation therapy consisted of 4000 to 5000 rad by megavoltage photons in 5 weeks, and in selected cases, an additional 1500 to 2000 rad by electron beam boost in the tumor bed delivered over 2 additional weeks. Following surgery, 12 patients were treated sequentially with an interval of chemotherapy, radiation therapy and then the completion of chemotherapy. The added morbidity of this sequential approach is minimal: one patient of 12 had delayed primary healing of her wound, 1 of 10 patients required a break in radiation therapy because of skin erythema. Four patients were treated with intensive pre-chemotherapy radiation therapy because of inadequate surgical margins. The median time on study was 18 months from onset of treatment (range, 8 to 41 months). Although there have been no local, regional or distant recurrences, the follow-up time is inadequate to assess the therapeutic benefit of this combined modality treatment

  9. Three-dimensional volumetric MRI with isotropic resolution: improved speed of acquisition, spatial resolution and assessment of lesion conspicuity in patients with recurrent soft tissue sarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Ahlawat, Shivani [The Johns Hopkins Medical Institutions, The Russell H. Morgan Department of Radiology and Radiological Science, Baltimore, MD (United States); Morris, Carol [The Johns Hopkins Medical Institutions, Department of Orthopedic Surgery, Baltimore, MD (United States); The Johns Hopkins Medical Institutions, Department of Oncology, Baltimore, MD (United States); Fayad, Laura M. [The Johns Hopkins Medical Institutions, The Russell H. Morgan Department of Radiology and Radiological Science, Baltimore, MD (United States); The Johns Hopkins Medical Institutions, Department of Orthopedic Surgery, Baltimore, MD (United States); The Johns Hopkins Medical Institutions, Department of Oncology, Baltimore, MD (United States)

    2016-05-15

    To assess the acquisition speed, lesion conspicuity, and inter-observer agreement associated with volumetric T{sub 1}-weighted MR sequences with isotropic resolution for detecting recurrent soft-tissue sarcoma (STS). Fifteen subjects with histologically proven recurrent STS underwent MRI, including axial and coronal T{sub 1}-weighted spin echo (T{sub 1}-WSE) (5-mm slice thickness) and coronal 3D volumetric T{sub 1}-weighted (fat-suppressed, volume-interpolated, breath-hold examination; repetition time/echo time, 3.7/1.4 ms; flip angle, 9.5 ; 1-mm slice thickness) sequences before and after intravenous contrast administration. Subtraction imaging and multiplanar reformations (MPRs) were performed. Acquisition times for T{sub 1}-WSE in two planes and 3D sequences were reported. Two radiologists reviewed images for quality (>50 % artifacts, 25-50 % artifacts, <25 % artifacts, and no substantial artifacts), lesion conspicuity, contrast-to-noise ratio (CNR{sub muscle}), recurrence size, and recurrence-to-joint distance. Descriptive and intraclass correlation (ICC) statistics are given. Mean acquisition times were significantly less for 3D imaging compared with 2-plane T{sub 1}-WSE (183.6 vs 342.6 s; P = 0.012). Image quality was rated as having no substantial artifacts in 13/15 and <25 % artifacts in 2/15. Lesion conspicuity was significantly improved for subtracted versus unsubtracted images (CNR{sub muscle}, 100 ± 138 vs 181 ± 199; P = 0.05). Mean recurrent lesion size was 2.5 cm (range, 0.7-5.7 cm), and measurements on 3D sequences offered excellent interobserver agreement (ICC, 0.98 for lesion size and 0.96 for recurrence-to-joint distance with MPR views). Three-dimensional volumetric sequences offer faster acquisition times, higher spatial resolution, and MPR capability compared with 2D T{sub 1}-WSE for postcontrast imaging. Subtraction imaging provides higher lesion conspicuity for detecting recurrent STS in skeletal muscle, with excellent interobserver

  10. Trabectedin and Eribulin: Where Do They Fit in the Management of Soft Tissue Sarcoma?

    Science.gov (United States)

    Ratan, Ravin; Patel, Shreyaskumar R

    2017-06-01

    Trabectedin and eribulin are two agents that have been recently approved for the treatment of specific soft tissue sarcoma subtypes. They have proved to be a much-needed line of additional treatment for patients with these rare tumors, but their activity remains admittedly modest in most cases. Further exploitation of these novel agents is likely to require a more granular understanding of the salient mechanisms of action. For example, if as some studies suggest, eribulin derives its benefit from restructuring of tumor vasculature to improve efficacy of subsequent lines of therapy, then patients may benefit from its use earlier in the treatment pathway. The sequencing of trabectedin with other agents is also worth examining. In a disease like myxoid liposarcoma, consideration should be given to using trabectedin before other salvage regimens like gemcitabine and docetaxel, given its tolerability and excellent efficacy against this sarcoma subtype. Also, to be further investigated is the use of trabectedin in sarcoma subtypes which were excluded from the phase III study, but in which activity has been documented in earlier trials and subsequent reports. Combinations of trabectedin with other agents, particularly doxorubicin, have been explored, but the data to date do not support the routine use of these regimens.

  11. Benign mural nodules within fluid collections at MRI after soft-tissue sarcoma resection.

    Science.gov (United States)

    Lantos, Joshua E; Hwang, Sinchun; Panicek, David M

    2014-06-01

    The purpose of this study was to determine the prevalence and clinical significance of nodules within fluid collections on MRI after surgical resection of soft-tissue sarcoma. This retrospective study included 175 patients who underwent resection of primary soft-tissue sarcoma and whose postoperative MRI reports mentioned fluid. Images were reviewed to determine the presence of fluid collections of 1 cm or greater in diameter in the surgical bed and any nodule (measuring ≥ 0.7 cm) within the collection. Signal intensity and characteristics of each collection and rim and presence of septa or blood products were recorded. Size, signal intensity, and contrast enhancement of nodules were reviewed. Nodules were classified as benign or malignant on the basis of histologic results or clinical or MRI follow-up. Fluid collections were present in 75 patients. Of those, 45 collections (60%) showed homogeneous fluid signal intensity and 30 (40%) were heterogeneous; septa were present in 45 (60%) and blood products in 12 (16%). Most collections showed a thin rim (59%) and rim enhancement (88%). Nodules were present along the inner wall of six (8%) collections. Four (66%) nodules enhanced and two (33%) were T1 hyperintense. At follow-up MRI, two nodules were stable in size, one decreased, and three resolved. Nodules in three patients were biopsied; all were benign. Two other patients had no recurrence at follow-up, and another died at 3 months. A nodule within a postoperative fluid collection at MRI after soft-tissue sarcoma resection generally does not represent tumor recurrence; short-interval follow-up MRI is recommended rather than immediate biopsy.

  12. A novel splice variant of the stem cell marker LGR5/GPR49 is correlated with the risk of tumor-related death in soft-tissue sarcoma patients

    International Nuclear Information System (INIS)

    Rot, Swetlana; Taubert, Helge; Bache, Matthias; Greither, Thomas; Würl, Peter; Eckert, Alexander W; Schubert, Johannes; Vordermark, Dirk; Kappler, Matthias

    2011-01-01

    The human leucine-rich, repeat-containing G protein-coupled receptor (LGR) 5, also called GPR49, is a marker of stem cells in adult intestinal epithelium, stomach and hair follicles. LGR5/GPR49 is overexpressed in tumors of the colon, ovary and liver and in basal cell carcinomas. Moreover, an expression in skeletal muscle tissues was also detected. However, there has been no investigation regarding the expression and function of LGR5/GPR49 in soft-tissue sarcomas (STS) yet. Seventy-seven frozen tumor samples from adult STS patients were studied using quantitative real-time TaqMan™ PCR analysis. The mRNA levels of wild type LGR5/GPR49 and a newly identified splice variant of LGR5/GPR49 lacking exon 5 (that we called GPR49Δ5) were quantified. A low mRNA expression level of GPR49Δ5, but not wild type LGR5/GPR49, was significantly correlated with a poor prognosis for the disease-associated survival of STS patients (RR = 2.6; P = 0.026; multivariate Cox's regression hazard analysis). Furthermore, a low mRNA expression level of GPR49Δ5 was associated with a shorter recurrence-free survival (P = 0.043). However, tumor onset in patients with a lower expression level of GPR49Δ5 mRNA occurred 7.5 years later (P = 0.04) than in patients with a higher tumor level of GPR49Δ5 mRNA. An attenuated mRNA level of the newly identified transcript variant GPR49Δ5 is a negative prognostic marker for disease-associated and recurrence-free survival in STS patients. Additionally, a lower GPR49Δ5 mRNA level is associated with a later age of tumor onset. A putative role of GPR49Δ5 expression in tumorigenesis and tumor progression of soft tissue sarcomas is suggested

  13. Local control of Ewing's sarcoma: an analysis of 67 patients

    International Nuclear Information System (INIS)

    Brown, A.P.; Fixsen, J.A.; Plowman, P.N.

    1987-01-01

    Local control of Ewing's sarcoma was analysed in a series of 67 patients treated by surgery and/or radiotherapy as well as combination chemotherapy. Radiotherapy was employed with or without surgery in 60 patients and produced an overall local control rate of 55%; complete excision of the primary lesion seemed to be beneficial. There was a marked variation in control rates depending on the site of the primary lesion: limb 85%, rib 53%, pelvis 31% and other sites 33%.Primary tumours greater than 10 cm in diameter were significantly less likely to be controlled. Using daily fractions of approximately 180 cGy, total doses in excess of 6000 cGy seem more likely to produce serious late morbidity amd may not increase the local control rate. No cases of second malignancy arising in irradiated tissue have been observed to date, but one patient developed acute lymphoblastic leukaemia. (author)

  14. Physiotherapy management of patients with HIV-associated Kaposi's sarcoma.

    Science.gov (United States)

    Harris-Love, Michael O; Shrader, Joseph A

    2004-01-01

    Kaposi's sarcoma is the most common form of cancer in patients with human immunodeficiency virus (HIV) infection. Although Kaposi sarcoma lesions may contribute to significant physical impairments, there is a lack of scientific literature detailing the role of physiotherapy in the treatment of HIV-associated Kaposi's sarcoma. The present Case Report includes two males, aged 36 and 39 years, seropositive for HIV with invasive Kaposi's sarcoma. Patient A was evaluated for bilateral foot pain caused by plantar surface Kaposi s sarcoma lesions that rendered him unable to walk. He progressed to walking 400feet after a treatment regimen of gait training with the use of custom plastazote sandals. Patient B was evaluated for right lower extremity lymphoedema secondary to invasive Kaposi's sarcoma. He experienced an 18% reduction in limb volume, a 38% reduction in pain and a 20 degrees increase in terminal knee flexion after therapeutic exercise and the use of compressive bandaging and garments. This Case Report suggests that physiotherapy interventions may be valuable in the conservative management of patients with HIV-associated Kaposi s sarcoma.

  15. Neoadjuvant chemoradiation (modified Eilber protocol) versus adjuvant radiotherapy in the treatment of extremity soft tissue sarcoma

    International Nuclear Information System (INIS)

    Lehane, Chris; Parasyn, Andrew; Ho, Frederick; Thompson, Stephen R.; Smee, Robert; Links, David; Crowe, Phil; Lewis, Craig; Friedlander, Michael; Williams, Janet

    2016-01-01

    Local control for extremity soft tissue sarcomas (STS) requires surgery combined with radiotherapy, usually given pre-operatively or post-operatively. The modified Eilber protocol, a neoadjuvant chemoradiation regimen, has been reported with excellent local control rates. This retrospective single-centre study compared outcomes for patients treated with the modified Eilber protocol with those treated with standard adjuvant radiotherapy. Twenty-nine patients were treated with modified Eilber protocol. Thirty-four patients received adjuvant radiotherapy. Three patients (10%) in the Eilber group and five patients (15%) in the Adjuvant group developed local recurrence (P = 0.87). Major acute wound complications were noted in four patients in each group (P = 0.55). One patient (3.4%) in the Eilber group developed Grade 3 or 4 late toxicities after 1 year compared with nine patients (27%) in the Adjuvant group (P = 0.02). Patients with a diagnosis of extremity STS were retrospectively reviewed from the Prince of Wales Hospital Sarcoma Database from 1995 to 2012. Sixty-three patients underwent curative surgery with either neoadjuvant Eilber chemoradiotherapy (Eilber) or adjuvant radiotherapy (Adjuvant). Neoadjuvant chemoradiation (Eilber protocol) provided similar rates of local control when compared with adjuvant radiotherapy. Acute wound complication rates were similar but there was less severe late toxicity in the Eilber group.

  16. Radiosensitivity and repair capacity of two xenografted human soft tissue sarcomas to photons and fast neutrons

    International Nuclear Information System (INIS)

    Budach, V.; Stuschke, M.; Budach, W.; Krause, U.; Streffer, C.; Sack, H.

    1989-01-01

    The radiation response, the relative biological effectiveness (RBE) and sublethal damage repair of two xenografted human soft tissue sarcomas after single doses and fractionated irradiation with 60 Co and 5.8 MeV fast neutrons are presented. (author)

  17. Adjuvant Radiotherapy for Pediatric and Young Adult Nonrhabdomyosarcoma Soft-Tissue Sarcoma

    International Nuclear Information System (INIS)

    Smith, Kristy B.; Indelicato, Daniel J.; Knapik, Jacquelyn A.; Lagmay, Joanne P.; Morris, Christopher; Kirwan, Jessica M.; Zlotecki, Robert A.; Scarborough, Mark T.; Gibbs, C. Parker; Marcus, Robert B.

    2011-01-01

    Purpose: To evaluate the prognostic factors, outcomes, and complications in patients aged ≤30 years with resectable nonrhabdomyosarcoma soft-tissue sarcoma treated at the University of Florida with radiotherapy (RT) during a 34-year period. Methods and Materials: A total of 95 pediatric or young adult patients with nonrhabdomyosarcoma soft-tissue sarcoma were treated with curative intent with surgery and RT at the University of Florida between 1973 and 2007. The most common histologic tumor subtypes were synovial sarcoma in 22 patients, malignant fibrous histiocytoma in 19, and malignant peripheral nerve sheath tumor in 11 patients. The mean age at RT was 22 years (range, 6-30). Of the 95 patients, 73 had high-grade tumors; 45 had undergone preoperative RT and 50 postoperative RT. The prognostic factors for survival, local recurrence, and distant recurrence were analyzed. Results: The median follow-up was 7.2 years (range, 0.4-30.5). The actuarial 5-year local control rate was 88%. A microscopically negative margin was associated with superior local control. Although 83% of local recurrence cases initially developed in the absence of metastases, all patients with local failure ultimately died of their disease. The actuarial estimate of 5-year overall survival and disease-free survival was 65% and 63%, respectively. Of all the deaths, 92% were disease related. An early American Joint Committee on Cancer stage, tumor <8 cm, and the absence of neurovascular invasion were associated with superior disease-free survival. The National Cancer Institute Common Toxicity Criteria, version 3, Grade 3-4 treatment complication rate was 9%. No secondary malignancies were observed. Conclusion: In the present large single-institution study, we found positive margins and locally advanced features to be poor prognostic factors for both local progression and survival. The results from the present study have helped to characterize the therapeutic ratio of RT in pediatric and young

  18. Statistics of bone sarcoma in Japan: Report from the Bone and Soft Tissue Tumor Registry in Japan.

    Science.gov (United States)

    Ogura, Koichi; Higashi, Takahiro; Kawai, Akira

    2017-01-01

    No previous reports to date have characterized the national profiles of bone sarcoma overall. In the present study, we aimed to describe the nationwide statistics of bone sarcoma in Japan by analyzing data from the Bone and Soft Tissue Tumor (BSTT) Registry in Japan, which is a nationwide organ-specific cancer registry for bone and soft tissue tumor. We identified 2773 patients with bone sarcomas using the BSTT Registry during 2006-2012. We extracted the data regarding patient demographics, treatment, and prognosis at the last follow-up for each patient. There was a slight male preponderance. The age distribution had 2 peaks overall: one in the second decade and the other in the sixth to seventh decade with the proportion of the elderly patients over 60 years approximately 30%. The most frequent tumor locations were the lower extremity (N = 1342; 48.4%) and the trunk (N = 1038; 37.4%). We also showed the significant association between disease-specific survival and patient's age, histologic grade and subtype, tumor size and location, and limb salvage status based on 1401 patients with bone sarcoma, and demonstrated the worst disease-specific survival in the elderly patients. The present study is the first study to have analyzed data from the BSTT Registry and has provided an overview of the epidemiology, clinical features, treatment, prognosis, and significant factors affecting prognosis of patients with bone sarcoma in Japan based on cases assumed to have received relatively uniform treatment strategies. It is essential to document our data regarding the outcomes of elderly patients so that other countries showing similar population aging trends can learn from our experiences. Copyright © 2016 The Japanese Orthopaedic Association. Published by Elsevier B.V. All rights reserved.

  19. External-beam radiation therapy combined with limb-sparing surgery in elderly patients (>70 years) with primary soft tissue sarcomas of the extremities : A retrospective analysis.

    Science.gov (United States)

    Andrä, Claudia; Klein, Alexander; Dürr, Hans Roland; Rauch, Josefine; Lindner, Lars Hartwin; Knoesel, Thomas; Angele, Martin; Baur-Melnyk, Andrea; Belka, Claus; Roeder, Falk

    2017-08-01

    To report our experience with EBRT combined with limb-sparing surgery in elderly patients (>70 years) with primary extremity soft tissue sarcomas (STS). Retrospectively analyzed were 35 patients (m:f 18:17, median 78 years) who all presented in primary situation without nodal/distant metastases (Charlson score 0/1 in 18 patients; ≥2 in 17 patients). Median tumor size was 10 cm, mainly located in lower limb (83%). Stage at presentation (UICC7th) was Ib:3%, 2a:20%, 2b:20%, and 3:57%. Most lesions were high grade (97%), predominantly leiomyosarcoma (26%) and undifferentiated pleomorphic/malignant fibrous histiocytoma (23%). Limb-sparing surgery was preceded (median 50 Gy) or followed (median 66 Gy) by EBRT. Median follow-up was 37 months (range 1-128 months). Margins were free in 26 patients (74%) and microscopically positive in 9 (26%). Actuarial 3‑ and 5‑year local control rates were 88 and 81% (4 local recurrences). Corresponding rates for distant control, disease-specific survival, and overall survival were 57/52%, 76/60%, and 72/41%. The 30-day mortality was 0%. Severe postoperative complications were scored in 8 patients (23%). Severe acute radiation-related toxicity was observed in 2 patients (6%). Patients with Charlson score ≥2 had a significantly increased risk for severe postoperative complications and acute radiation-related side effects. Severe late toxicities were found in 7 patients (20%), including fractures in 3 (8.6%). Final limb preservation rate was 97%. Combination of EBRT and limb-sparing surgery is feasible in elderly patients with acceptable toxicities and encouraging but slightly inferior outcome compared to younger patients. Comorbidity correlated with postoperative complications and acute toxicities. Late fracture risk seems slightly increased.

  20. Epidemic Kaposi Sarcoma

    Science.gov (United States)

    ... Sarcoma Treatment Childhood Vascular Tumors Treatment Research Kaposi Sarcoma Treatment (PDQ®)–Patient Version General Information About Kaposi Sarcoma Go to Health Professional Version Key Points Kaposi ...

  1. Classic Kaposi Sarcoma

    Science.gov (United States)

    ... Sarcoma Treatment Childhood Vascular Tumors Treatment Research Kaposi Sarcoma Treatment (PDQ®)–Patient Version General Information About Kaposi Sarcoma Go to Health Professional Version Key Points Kaposi ...

  2. Immunohistochemical expression of promyelocytic leukemia body in soft tissue sarcomas

    Directory of Open Access Journals (Sweden)

    Yasunaga Yuji

    2008-11-01

    Full Text Available Abstract Background The function of promyelocytic leukemia (PML bodies is not well known but plays an important role in controlling cell proliferation, apoptosis and senescence. This study was undertaken to analyze the clinical significance of PML body expression in primary tumor samples from malignant fibrous histiocytoma (MFH and liposarcoma patients. Methods We studied MFH and liposarcoma samples from 55 patients for PML bodies. Fluorescent immunostaining of PML bodies was performed in the paraffin-embedded tumor sections. Results PML body immunostaining was identified in 63.9% of MFH and 63.2% of liposarcoma samples. PML body expression rates of all sarcoma cells were 1.5 ± 1.8% (range: 0–7.0 in MFH and 1.3 ± 1.4% (0–5.2 in liposarcoma samples. PML body expression (p = 0.0053 and a high rate of PML body expression (p = 0.0012 were significantly greater prognostic risk factors for death than the other clinical factors in MFH patients. All liposarcoma patients without expression of PML were disease free at the end of the study. Conclusion Our study suggests that the presence of PML bodies may indicate a poor prognosis for MFH and liposarcoma patients.

  3. Analysis of Dietary Intake during Consecutive-Day Chemotherapy for Bone and Soft-Tissue Sarcomas

    Directory of Open Access Journals (Sweden)

    Yuta Hori

    2018-01-01

    Full Text Available BackgroundBone and soft tissue sarcomas are commonly treated with consecutive-day chemotherapy regimens consisting of multiple anticancer agents. Chemotherapy-induced nausea and vomiting (CINV is a serious adverse effect of these regimens and may result in decreased energy intake during chemotherapy. Decreased energy intake may lead to undernutrition and may cause adverse effects on patient quality of life and survival.MethodsPatients with bone and soft tissue sarcomas who received consecutive-day chemotherapy were retrospectively evaluated. CINV and dietary energy intake were assessed, as well as the occurrences of hiccups and constipation during chemotherapy.ResultsA total of 13 patients, 10 males and 3 females, with a total 16 chemotherapy courses were included in the study. All patients received antiemetic prophylaxis. The CINV control rate, defined as no emesis and no rescue therapy, gradually decreased from chemotherapy day 1 (94% to day 5 (75%. Four patients experienced emesis, two of whom had been treated with a cisplatin-containing regimen. Decreased dietary energy intake was possibly associated with CINV during chemotherapy. Anorexia was grade 2 except for one case of grade 3. The incidences of hiccups and constipation were high on days 3–5.ConclusionAntiemetic prophylaxis treatment did not prevent emesis due to consecutive-day chemotherapy, especially with cisplatin-containing regimens, in patients with bone and soft-tissue tumors. Dietary energy intake decreased during chemotherapy, and this appeared to be associated with CINV. In addition, the incidence of hiccups and constipation increased during the course of consecutive-day chemotherapy regimens. Although these results are based on a small number of patients, it may be important to observe nutritional status during chemotherapy, as this may reflect a patient’s general condition. Nutritional counseling might be useful in supporting nutritional status in patients undergoing

  4. Epidemiological study of soft-tissue sarcomas in Ireland.

    LENUS (Irish Health Repository)

    Bhatt, Nikita

    2015-11-21

    Soft-tissue sarcomas (STS) account for 1% of adult and 7% of pediatric malignancies. Histopathology and classification of these rare tumors requires further refinements. The aim of this paper is to describe the current incidence and survival of STS from 1994 to 2012 in Ireland and compare these with comparably coded international published reports. This is a retrospective, population study based on the data from the National Cancer Registry of Ireland (NCRI). Incidence and relative survival rates for STS in Ireland were generated. Incidence of STS based on gender, age and anatomical location was examined. Annual mean incidence rate (European Age Standardized) in Ireland between 1994 and 2012 was 4.48 ± 0.15 per 100,000 person-years. The overall relative 5-year survival rate of STS for the period 1994-2011 in Ireland was 56%, which was similar to that reported in the U.K. but lower than in most of Europe and U.S.A. Survival rate fluctuated over the period examined, declining slightly in females but showing an increase in males. STS incidence trends in Ireland were comparable to international reports. Survival trends of STS were significantly different between Ireland and other European countries, requiring further study to understand causation.

  5. [Evaluation of the management of soft tissue sarcomas in Franche-Comté since the establishment of a multidisciplinary meeting at University Hospital. About 47 cases].

    Science.gov (United States)

    Haddad, J; Kalbacher, E; Piccard, M; Aubry, S; Chaigneau, L; Pauchot, J

    2017-02-01

    A multidisciplinary meeting (RCP) dedicated to the treatment of sarcoma was established in Franche-Comte in 2010. The goals of the study are: (a) To evaluate the treatment of sarcomas by confrontation with the existing literature; (b) To evaluate the influence of the multidisciplinary meeting on the management of sarcomas by hospitals at the regional level. This is a retrospective single center study from 2010 to 2015 on patients with sarcoma and peripheral soft tissue drawn from a Netsarc database (National Network of sarcomas) and communicating cancer record. A database Cleanweb especially dedicated is created. Forty-seven patients were included: ten sarcomas at the upper member 26 to the lower limbs, 11 on the trunk. Forty patients were operated on: ten out of the university hospital, 28 at the university hospital and two in a coordinating center. Ninety percent of patients treated at the university hospital were in accordance with the recommandations. None of the patients operated out of the university hospital benefited from medical care in accordance to the recommendations. There is an increase in the number of files sent by the hospitals out of the university hospital discussed in multidisciplinary meeting, before treatment. The creation of a dedicated multidisciplinary meeting sarcoma improves the medical management of these tumors and decreases inappropriate medical managements thanks to a better education of the regional physicians. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

  6. Lymphangiectatic Kaposi's sarcoma in a patient with AIDS Sarcoma de Kaposi linfangiectásico em paciente com Aids

    Directory of Open Access Journals (Sweden)

    Mônica Santos

    2013-04-01

    Full Text Available Kaposi's sarcoma is a malignant disease that originates in the lymphatic endothelium. It has a broad spectrum of clinical manifestations. Its four distinct clinical forms are: classic, endemic, iatrogenic and epidemic Kaposi's sarcoma. In non-HIV-associated Kaposi's sarcoma, the disease is typically limited to the lower extremities, but in immunodeficient patients, it is a multifocal systemic disease. The clinical course of the disease differs among patients, ranging from a single or a few indolent lesions to an aggressive diffuse disease. Advanced Kaposi's sarcoma lesions, typically those on the lower extremities, are often associated with lymphedema. In this paper, we report a case of a patient with a rare form of AIDS-associated Kaposi sarcoma called lymphangiectatic Kaposis's sarcoma.O sarcoma de Kaposi é uma neoplasia originária do endotélio linfatico, que apresenta um amplo espectro de manifestações, com quatro formas clínicas: sarcoma de Kaposi clássico, endêmico, iatrogêncio e epidêmico ou associado ao HIV. Em pacientes imunocompetentes, a doença é tipicamente limitada às extremidades. Porém em pacientes imunideprimidos, o sarcoma de Kaposi é uma doença sistêmica multifocal. Apresenta cursos clínicos diferentes, desde simples lesões cutâneas isoladas até lesões agressivas e difusas, com ou sem envolvimento sistêmico. Lesões avançadas de sarcoma de Kaposi, principalmente as localizadas nas extremidades, podem apresentar linfedema. Neste trabalho, reportamos caso de paciente com forma rara de Sarcoma de Kaposi associado a Aids, chamada de sarcoma de Kaposi linfangiectásico.

  7. Predictive and prognostic factors associated with soft tissue sarcoma response to chemotherapy

    DEFF Research Database (Denmark)

    Young, Robin J; Litière, Saskia; Lia, Michela

    2017-01-01

    BACKGROUND: The European Organization for Research and Treatment of Cancer (EORTC) 62012 study was a Phase III trial of doxorubicin versus doxorubicin-ifosfamide chemotherapy in 455 patients with advanced soft tissue sarcoma (STS). Analysis of the main study showed that combination chemotherapy...... improved tumor response and progression-free survival, but differences in overall survival (OS) were not statistically significant. We analyzed factors prognostic for tumor response and OS, and assessed histological subgroup and tumor grade as predictive factors to identify patients more likely to benefit...... patients had improved tumor response compared to other histological subgroups, whilst patients with metastases other than lung, liver or bone had a poorer response [odds ratio (OR) 0.42, 95% confidence interval (CI) 0.23-0.78; p = 0.006]. Patients with bone metastases had reduced OS [hazard ratio (HR) 1...

  8. Soft tissue sarcoma after treatment for breast cancer

    International Nuclear Information System (INIS)

    Karlsson, Per; Holmberg, Erik; Johansson, Karl-Axel; Kindblom, Lars-Gunnar; Carstensen, John; Wallgren, Arne

    1996-01-01

    In a register study all women in the West of Sweden Health Care Region with a breast cancer diagnosed between 1960 and 1980 (n = 13 490) were followed up in the Swedish Cancer Register to the end of 1988 for later occurrence of a soft tissue sarcoma (STS). Nineteen sarcomas were reported, whereas 8.7 were expected and the relative risk (RR) was 2.2 (CI 95% 1.3-3.4). The absolute risk was (1.7(10 4 )) person years (PY) in comparison with 0.8 expected. To obtain a more detailed analysis of the associations between arm lymphoedema, radiotherapy and STS development, and to control the quality of the register data, a case control study was also performed. Clinical records from the different hospitals in the region were collected for all the 19 cases as well as for three selected controls per case. The histopathology of the cases were reviewed, and one of the cases was reclassified as a malignant melanoma and excluded from further analysis. Thirteen of the cases were clustered around the treated breast area. To quantify the exposure to radiotherapy, the integral dose was estimated. The presence of lymphedema was included as a binary variable in the analysis. The exact conditional randomisation test indicated a significant correlation between the integral dose and the development of an STS (p = 0.008) and this association was still significant after stratification for arm oedema. A conditional logistic regression analysis with STS as the dependent variable and the integral dose as the explanatory variable gave an odds ratio (OR) of(5.2(100)) J (CI 95% 1.3-21.2), and if this regression was restricted only to the STS developing in the radiation fields the OR was(3.2(100)) J (CI 95% 0.8-12.9). Thus, the excess of STS in this breast cancer cohort was very low ((0.9(10 4 )) PY). However the integral dose correlates well to the development of STS and can be useful in quantifying even small risks of secondary malignancies in the breast cancer population

  9. The relationship between tumor oxygenation and cell proliferation in human soft tissue sarcomas

    International Nuclear Information System (INIS)

    Nordsmark, Marianne; Hoeyer, Morten; Keller, Johnny; Nielsen, Ole Steen; Jensen, Oluf Myhre; Overgaard, Jens

    1996-01-01

    Purpose: In malignant tumors the oxygenation status and tumor cell proliferation are known to influence local tumor control after radiotherapy. However, the relationship between oxygenation status and tumor cell kinetics in human tumors has not yet been described. Newly developed clinically applicable techniques such as oxygen electrode measurements and assessment of tumor cell proliferation rates have been suggested as promising predictive assays. The purpose of the present study was to characterize tumor oxygenation status in soft tissue sarcomas and to compare this with tumor cell kinetics and clinical parameters. Methods and Materials: Pretreatment tumor oxygenation status was measured by polarographic oxygen needle electrodes and evaluated as the median pO 2 and the percentage of pO 2 values ≤ 5 mmHg and ≤ 2.5 mmHg in 22 patients with primary soft tissue sarcomas. All tumors were characterized by histology, grade of malignancy, the level of microscopic necrosis, the level of effective hemoglobin, and magnetic resonance imaging estimation of tumor volume. The tumor cell potential doubling time and labeling index were measured by flow cytometric and immunohistochemical analysis of tumor biopsy specimens after in vivo incorporation of iododeoxyuridine. Results: There was a significant correlation between the median pO 2 and the tumor cell potential doubling time (p = 0.041), whereas no correlation was found between the level of hypoxia expressed by the percentage of pO 2 values ≤ 2.5 and ≤ 5 mmHg, respectively, and tumor cell potential doubling time. Furthermore, no correlation was found between either of the three tumor oxygenation parameters and labeling index. The material represented large intertumor heterogeneity in oxygenation status, cell kinetics, and tumor volume, and no correlation was found between oxygenation status and either volume, histopathology, grade of malignancy, or effective hemoglobin. Conclusion: This report is the first to suggest

  10. Expression of neural cell adhesion molecules and neurofilament protein isoforms in Ewing's sarcoma of bone and soft tissue sarcomas of other than rhabdomyosarcoma

    NARCIS (Netherlands)

    Molenaar, W.M.; Muntinghe, F.L.H.

    1999-01-01

    In a previous study, it was shown that rhabdomyosarcomas widely express "neural" markers, such as neural cell adhesion molecules (N-CAM) and neurofilament protein isoforms, In the current study, a series of Ewing's sarcomas of bone and soft tissue sarcomas other than rhabdomyosarcoma was probed for

  11. Adult soft tissue sarcomas of the head and neck. Treatment outcome after combined surgery and irradiation or radiotherapy alone

    International Nuclear Information System (INIS)

    Willers, H.; Spiro, I.J.; Efird, J.T.; Rosenberg, A.E.; Wang, C.C.

    1997-01-01

    To analyse the experience treating soft tissue sarcomas of the head and neck at the Massachusetts General Hospital, Boston. Detailed results have been published previously. After 5 years, patients with angiosarcomas (n=11) and patients with other tumor types (n=46) had locoregional control rates of 24% and 69%, distant failure rates of 58% and 17%, and overall survival rates of 31% and 74%, respectively (p [de

  12. A Rare Case of Pure Erythroid Sarcoma in a Pediatric Patient: Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Pablo Manresa

    2017-12-01

    Full Text Available We describe an exceptional case of erythroid sarcoma in a pediatric patient as a growing orbital mass with no evidence of morphologic bone marrow involvement, who was finally diagnosed of pure erythroid sarcoma based on histopathology and flow cytometry criteria. We discuss the contribution of standardized eight-color flow cytometry as a rapid and reliable diagnostic method. The use of normal bone marrow databases allowed us to identify small aberrant populations in bone marrow and later confirm the diagnosis in the neoplastic tissue.

  13. SOFT TISSUE SARCOMAS – A CROS S SECTIONAL STUDY AT MNJIO

    Directory of Open Access Journals (Sweden)

    Ramesh

    2015-07-01

    Full Text Available BACKGROUND: Sarcomas are an uncommon group of cancers with a wide distribution in the various anatomical regions of the body and even wider and confusing range of histopathological diagnosis. AIMS: To study the mode of presentation, histology, treatment modalities use d and outcomes both in terms of function and disease free survivals of all patients treated in the Department of surgical oncology in our Institute. MATERIALS & METHODS: All patients of soft tissue sarcoma presenting to out department were studied and data collected in a proforma. Histology was established by Trucut/open biopsy. Patients after clinical examination were evaluated f or distant disease in lungs by x - ray chest followed by CT scan . RESULTS: Symptomatology and anatomical location in our study was similar to world literature but stage of presentation and tumour size is advanced in our study. CONCLUSIONS: Patients in our study presented late, probably due to lack of access to medical care, delay in diagnosis and delay in referral. Hence higher incidence of amputations and also higher incidence of need for reconstruction. Successful utilization of multi - modality treatment and availability of good reconstruction ensured good functional outcome.

  14. Brachytherapy in the conservative treatment of soft tissue sarcomas extending to neurovascular structures: an analysis of 38 cases

    International Nuclear Information System (INIS)

    Thomas, L.; Delannes, M.; Stoeckle, E.; Martel, P.; Pigneux, J.; Daly-Schveitzer, N.; Bui, B.N.; Chevreau, C.; Kantor, G.

    1996-01-01

    To evaluate the tolerance of neurovascular structures to brachytherapy, a retrospective review of our series was undertaken. Between May 1986 and January 1994, 85 patients with soft tissue sarcomas underwent conservative surgery and low-dose rate interstitial irradiation. Thirty-eight patients had tumors extending to neurovascular structures. Brachytherapy was part of initial treatment in 30 patients and was done in 7 cases for recurrent sarcomas. Afterloading catethers for brachytherapy were inserted intraoperatively and placed direct upon or under the neurovascular structures in the tumor bed. A mean dose of 20 Gy was delivered to the target volume. Thirty patients received 45 to 50 Gy of postoperative external irradiation. With a median follow-up of 39 months, the 3-year actuarial survival was 82.9%, the 3-year disease-free survival was 71.9% and the 3-year actuarial local control was 91%. The 3-year actuarial incidence of distant metastase was 28%. Acute side effects occurred in 12 patients requiring conservative surgical procedures in 6 cases. Significant late toxicity occurred in 8 patients : 2 lymphoedemas interfering with normal activity, 1 partial artery stenosis, 5 peripheral neuropathy (2 grade 2, 3 grade 3). Late toxicity has led to significant impairment of mobility in 4 patients. Limb preservation was achieved in every patient, no amputation was required. We conclude that integration of brachytherapy in the conservative treatment of soft tissue sarcomas extending to neurovascular structures can provide excellent local control with an acceptable level of toxicity

  15. Diagnostic Study of Tumor Characteristics in Patients With Ewing's Sarcoma

    Science.gov (United States)

    2013-06-20

    Localized Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor

  16. Soft Tissue Sarcoma, Version 2.2018, NCCN Clinical Practice Guidelines in Oncology.

    Science.gov (United States)

    von Mehren, Margaret; Randall, R Lor; Benjamin, Robert S; Boles, Sarah; Bui, Marilyn M; Ganjoo, Kristen N; George, Suzanne; Gonzalez, Ricardo J; Heslin, Martin J; Kane, John M; Keedy, Vicki; Kim, Edward; Koon, Henry; Mayerson, Joel; McCarter, Martin; McGarry, Sean V; Meyer, Christian; Morris, Zachary S; O'Donnell, Richard J; Pappo, Alberto S; Paz, I Benjamin; Petersen, Ivy A; Pfeifer, John D; Riedel, Richard F; Ruo, Bernice; Schuetze, Scott; Tap, William D; Wayne, Jeffrey D; Bergman, Mary Anne; Scavone, Jillian L

    2018-05-01

    Soft tissue sarcomas (STS) are rare solid tumors of mesenchymal cell origin that display a heterogenous mix of clinical and pathologic characteristics. STS can develop from fat, muscle, nerves, blood vessels, and other connective tissues. The evaluation and treatment of patients with STS requires a multidisciplinary team with demonstrated expertise in the management of these tumors. The complete NCCN Guidelines for STS provide recommendations for the diagnosis, evaluation, and treatment of extremity/superficial trunk/head and neck STS, as well as intra-abdominal/retroperitoneal STS, gastrointestinal stromal tumors, desmoid tumors, and rhabdomyosarcoma. This portion of the NCCN Guidelines discusses general principles for the diagnosis, staging, and treatment of STS of the extremities, superficial trunk, or head and neck; outlines treatment recommendations by disease stage; and reviews the evidence to support the guidelines recommendations. Copyright © 2018 by the National Comprehensive Cancer Network.

  17. Automated planning volume definition in soft-tissue sarcoma adjuvant brachytherapy

    International Nuclear Information System (INIS)

    Lee, Eva K.; Fung, Albert Y.C.; Zaider, Marco; Brooks, J. Paul

    2002-01-01

    soft-tissue sarcoma tumour beds within various anatomical structures. For each of 15 patient cases considered, the algorithm takes approximately 2 min to generate the planning volume. Although the tumour shapes are rather different, the algorithm consistently generates planning volumes that visually demonstrate smooth curves compactly encapsulating the circles. This general-purpose contouring algorithm works well whether the catheters are all close together, spread far apart in the plane or arranged in a convoluted way. The automatic contouring algorithm significantly reduces labour time and provides a consistent and objective method for determining planning volumes for soft-tissue sarcoma. Further studies are needed to validate the significance of the resulting planning volumes in designing treatment plans and the role that sophisticated brachytherapy treatment planning optimization may have in producing good plans. (author)

  18. Automated planning volume definition in soft-tissue sarcoma adjuvant brachytherapy

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Eva K. [Department of Radiation Oncology, Emory University School of Medicine, Atlanta, GA (United States); School of Industrial and Systems Engineering, Georgia Institute of Technology, Atlanta, GA (United States); Fung, Albert Y.C.; Zaider, Marco [Department of Medical Physics, Memorial Sloan Kettering Cancer Center, New York, NY (United States); Brooks, J. Paul [School of Industrial and Systems Engineering, Georgia Institute of Technology, Atlanta, GA (United States)

    2002-06-07

    soft-tissue sarcoma tumour beds within various anatomical structures. For each of 15 patient cases considered, the algorithm takes approximately 2 min to generate the planning volume. Although the tumour shapes are rather different, the algorithm consistently generates planning volumes that visually demonstrate smooth curves compactly encapsulating the circles. This general-purpose contouring algorithm works well whether the catheters are all close together, spread far apart in the plane or arranged in a convoluted way. The automatic contouring algorithm significantly reduces labour time and provides a consistent and objective method for determining planning volumes for soft-tissue sarcoma. Further studies are needed to validate the significance of the resulting planning volumes in designing treatment plans and the role that sophisticated brachytherapy treatment planning optimization may have in producing good plans. (author)

  19. Synovial sarcoma in children and adolescents: the European Pediatric Soft Tissue Sarcoma Study Group prospective trial (EpSSG NRSTS 2005)

    NARCIS (Netherlands)

    Ferrari, A.; de Salvo, G. L.; Brennan, B.; van Noesel, M. M.; de Paoli, A.; Casanova, M.; Francotte, N.; Kelsey, A.; Alaggio, R.; Oberlin, O.; Carli, M.; Ben-Arush, M.; Bergeron, C.; Merks, J. H. M.; Jenney, M.; Stevens, M. C.; Bisogno, G.; Orbach, D.

    2015-01-01

    To report the results of the first European prospective nonrandomized trial dedicated to pediatric synovial sarcoma. From August 2005 to August 2012, 138 patients <21 years old with nonmetastatic synovial sarcoma were registered in 9 different countries (and 60 centers). Patients were treated with a

  20. Preoperative Intensity-Modulated Radiotherapy Combined with Temozolomide for Locally Advanced Soft-Tissue Sarcoma

    International Nuclear Information System (INIS)

    Jakob, Jens; Wenz, Frederik; Dinter, Dietmar J.; Stroebel, Philipp; Hohenberger, Peter

    2009-01-01

    Purpose: To evaluate the toxicity and efficacy of preoperative intensity-modulated radiotherapy (IMRT) combined with temozolomide to improve local tumor control in soft-tissue sarcoma (STS). Patients and Methods: A cohort of 15 consecutive patients with nonmetastasized, primary high-grade or locally recurrent Stage III (n = 14) or IIb (n = 1) STS not amenable to surgical resection without significant organ or extremity function loss was prospectively investigated. Median tumor size was 9.8 cm, and most tumors were non-extremity sarcomas. Patients preoperatively received 50 mg/m 2 of temozolomide during IMRT (50.4 Gy). Resection was intended 6 weeks thereafter. Toxicity was assessed by the National Cancer Institute Common Terminology Criteria for Adverse Events version 3.0, and response was assessed by Response Evaluation Criteria in Solid Tumors. Results: Of 15 patients, 14 completed preoperative treatment. No Grade 4 toxicities occurred. Nausea and vomiting were the most frequent Grade 3 toxicities. The most frequent toxicities of any grade were dermatologic, gastrointestinal, and hematologic. Response was partial response in 5, stable disease in 7, and progressive disease in 2 patients. Ten patients underwent surgery: 7 were resected with clear margins (R0), and 2 patients had an R1 resection; in 1 patient the tumor was not resectable. Postoperative complications occurred in 4 patients. Five patients did not undergo surgery because of intercurrent metastatic disease, unresectable disease, or refusal. Conclusions: Preoperative chemoradiation with temozolomide and IMRT can be administered safely and with promising efficacy in patients with locally advanced STS.

  1. Epirubicin is not Superior to Doxorubicin in the Treatment of Advanced Soft Tissue Sarcomas.The Experience of the EORTC Soft Tissue and Bone Sarcoma Group

    DEFF Research Database (Denmark)

    Nielsen, Ole Steen; Dombernowsky, Per; Mouridsen, Henning T

    2000-01-01

    studies the EORTC STBSG tested whether epirubicin (epi) is an alternative to standard dose dox in the treatment of chemonaive patients with advanced soft tissue sarcoma. The present report gives the final results of these studies.Patients/Methods. In the first study 210 patients were randomized to receive...... either dox or epi both at a dose of 75 mg/m(2) given as bolus injection at 3-week intervals. In the second study 334 patients were randomized to dox 75 mg/m(2), epi 150 mg/m(2) or epi 50 mg/m(2) days 1-3, all given as bolus injection at 3-week intervals.Results. In the first study no differences...... in median survival and duration of response were found. Of 167 evaluable patients the response rate was slightly in favour of dox (23% vs 18%) but at the expense of more toxicity.These data could suggest that increasing the epi dose may lead to a greater antineoplastic effect with acceptable toxicity...

  2. Outcome and Toxicity of an Ifosfamide-Based Soft Tissue Sarcoma Treatment Protocol in Children. The Importance of Local Therapy

    Directory of Open Access Journals (Sweden)

    S. Murray Yule

    1998-01-01

    Full Text Available Background. Although the survival of children with soft tissue sarcoma (STS has improved considerably, the outcome of patients with metastatic disease, and those with primary tumours of the extremities or parameningeal sites remains disappointing. We describe the clinical outcome of an ifosfamide-based regimen with local therapy directed only to children who failed to achieve a complete response to initial chemotherapy.

  3. Granulocytic sarcoma of the ovary in a nonleukemic patient.

    Science.gov (United States)

    Aguiar, R C; Pozzi, D H; Chamone, D A

    1993-01-01

    We report a case of granulocytic sarcoma of the ovary preceding acute myeloid leukemia by twelve months, with no evidence of any hematological involvement at the time of first diagnosis. The patient was initially treated with surgery and chemotherapy for undifferentiated lymphoma and, although this aggressive protocol resulted in a complete response, granulocytic sarcoma recurred as extramedullary disease, followed by the appearance of acute myeloid leukemia. We discuss the clinical, histopathological and immunohistochemical features of the disease, the differential diagnosis and, in particular, the role of early aggressive treatment on the outcome of the patient.

  4. Prognostic Value of External Beam Radiation Therapy in Patients Treated With Surgical Resection and Intraoperative Electron Beam Radiation Therapy for Locally Recurrent Soft Tissue Sarcoma: A Multicentric Long-Term Outcome Analysis

    Energy Technology Data Exchange (ETDEWEB)

    Calvo, Felipe A. [Department of Oncology, Hospital General Universitario Gregorio Marañón, Madrid (Spain); School of Medicine, Complutense University, Madrid (Spain); Sole, Claudio V., E-mail: cvsole@uc.cl [Department of Oncology, Hospital General Universitario Gregorio Marañón, Madrid (Spain); School of Medicine, Complutense University, Madrid (Spain); Service of Radiation Oncology, Instituto de Radiomedicina, Santiago (Chile); Cambeiro, Mauricio [Service of Radiation Oncology, Clínica Universitaria, Universidad de Navarra, Pamplona (Spain); Montero, Angel; Polo, Alfredo [Service of Radiation Oncology, Hospital Universitario Ramón y Cajal, Universidad de Alcala, Madrid (Spain); Gonzalez, Carmen [School of Medicine, Complutense University, Madrid (Spain); Service of Radiation Oncology, Instituto de Radiomedicina, Santiago (Chile); Service of Radiation Oncology, Clínica Universitaria, Universidad de Navarra, Pamplona (Spain); Service of Radiation Oncology, Hospital Universitario Ramón y Cajal, Universidad de Alcala, Madrid (Spain); Service of Radiation Oncology, Hospital General Universitario Gregorio Marañón, Madrid (Spain); Cuervo, Miguel [Service of Orthopedics and Traumatology, Hospital General Universitario Gregorio Marañón, Madrid (Spain); San Julian, Mikel [Service of Orthopedics and Traumatology, Clínica Universitaria, Universidad de Navarra, Pamplona (Spain); and others

    2014-01-01

    Background: A joint analysis of data from centers involved in the Spanish Cooperative Initiative for Intraoperative Electron Radiotherapy was performed to investigate long-term outcomes of locally recurrent soft tissue sarcoma (LR-STS) patients treated with a multidisciplinary approach. Methods and Materials: Patients with a histologic diagnosis of LR-STS (extremity, 43%; trunk wall, 24%; retroperitoneum, 33%) and no distant metastases who underwent radical surgery and intraoperative electron radiation therapy (IOERT; median dose, 12.5 Gy) were considered eligible for participation in this study. In addition, 62% received external beam radiation therapy (EBRT; median dose, 50 Gy). Results: From 1986 to 2012, a total of 103 patients from 3 Spanish expert IOERT institutions were analyzed. With a median follow-up of 57 months (range, 2-311 months), 5-year local control (LC) was 60%. The 5-year IORT in-field control, disease-free survival (DFS), and overall survival were 73%, 43%, and 52%, respectively. In the multivariate analysis, no EBRT to treat the LR-STS (P=.02) and microscopically involved margin resection status (P=.04) retained significance in relation to LC. With regard to IORT in-field control, only not delivering EBRT to the LR-STS retained significance in the multivariate analysis (P=.03). Conclusion: This joint analysis revealed that surgical margin and EBRT affect LC but that, given the high risk of distant metastases, DFS remains modest. Intensified local treatment needs to be further tested in the context of more efficient concurrent, neoadjuvant, and adjuvant systemic therapy.

  5. Prognostic Value of External Beam Radiation Therapy in Patients Treated With Surgical Resection and Intraoperative Electron Beam Radiation Therapy for Locally Recurrent Soft Tissue Sarcoma: A Multicentric Long-Term Outcome Analysis

    International Nuclear Information System (INIS)

    Calvo, Felipe A.; Sole, Claudio V.; Cambeiro, Mauricio; Montero, Angel; Polo, Alfredo; Gonzalez, Carmen; Cuervo, Miguel; San Julian, Mikel

    2014-01-01

    Background: A joint analysis of data from centers involved in the Spanish Cooperative Initiative for Intraoperative Electron Radiotherapy was performed to investigate long-term outcomes of locally recurrent soft tissue sarcoma (LR-STS) patients treated with a multidisciplinary approach. Methods and Materials: Patients with a histologic diagnosis of LR-STS (extremity, 43%; trunk wall, 24%; retroperitoneum, 33%) and no distant metastases who underwent radical surgery and intraoperative electron radiation therapy (IOERT; median dose, 12.5 Gy) were considered eligible for participation in this study. In addition, 62% received external beam radiation therapy (EBRT; median dose, 50 Gy). Results: From 1986 to 2012, a total of 103 patients from 3 Spanish expert IOERT institutions were analyzed. With a median follow-up of 57 months (range, 2-311 months), 5-year local control (LC) was 60%. The 5-year IORT in-field control, disease-free survival (DFS), and overall survival were 73%, 43%, and 52%, respectively. In the multivariate analysis, no EBRT to treat the LR-STS (P=.02) and microscopically involved margin resection status (P=.04) retained significance in relation to LC. With regard to IORT in-field control, only not delivering EBRT to the LR-STS retained significance in the multivariate analysis (P=.03). Conclusion: This joint analysis revealed that surgical margin and EBRT affect LC but that, given the high risk of distant metastases, DFS remains modest. Intensified local treatment needs to be further tested in the context of more efficient concurrent, neoadjuvant, and adjuvant systemic therapy

  6. Advantages of 18F FDG-PET/CT over Conventional Staging for Sarcoma Patients.

    Science.gov (United States)

    Németh, Zsuzsanna; Boér, Katalin; Borbély, Katalin

    2017-10-09

    The effective management of patients with sarcomas requires accurate diagnosis and staging. Imaging, such as ultrasonography (US), computed tomography (CT), magnetic resonance imaging (MRI) are the most freqently used methods for the detection of the lesion location, size, morphology and structural changes to adjacent tissues; however, these modalities provide little information about tumour biology. MRI is a robust and useful modality in tumour staging of sarcomas, however metabolic-fluorodeoxyglucose positron emission tomography/ computer tomography ( 18 F-FDG PET/CT) provides greater accuracy to overall staging in combination with MRI [1]. The advantages of 18 F-FDG PET/CT method compared with CT and MRI is that it provides a whole body imaging, maps the viability of the tumour or the metabolic activity of the tissue. Additionally, PET detects the most agressive part of the tumour, demonstrates the biological behaviour of the tumour and therefore has a predictive value. Little data ara available on the role of 18 F-FDG PET/CT in the management of sarcomas. The present manuscript aims to provide a review of the major indications of 18 F-FDG PET/CT for diagnosis, staging, restaging and monitoring response to therapy and to compare its usefulness with the conventional imaging modalities in the management of patients with sarcomas.

  7. Fast neutron radiotherapy for soft tissue and cartilaginous sarcomas at high risk for local recurrence

    International Nuclear Information System (INIS)

    Schwartz, David L.; Einck, John; Bellon, Jennifer; Laramore, George E.

    2001-01-01

    Purpose: The practice policy at the University of Washington has been to employ fast neutron radiotherapy for soft tissue sarcoma lesions with prognostic features predictive for poor local control. These include gross residual disease/inoperable disease, recurrent disease, and contaminated surgical margins. Cartilaginous sarcomas have also been included in this high-risk group. This report updates and expands our previously described experience with this approach. Methods and Materials: Eighty-nine soft tissue sarcoma lesions in 72 patients were treated with neutron radiotherapy in our department between 1984 and 1996. Six patients, each with solitary lesions, were excluded from analysis due to lack of follow-up. Seventy-three percent were treated with fast neutron radiation alone, the rest with a combination of neutrons and photons. Median neutron dose was 18.3 nGy (range 4.8-22). Forty-two patients with solitary lesions were treated with curative intent. Thirty-one patients (including 7 previously treated with neutrons) with 41 lesions were treated with the goal of local palliation. Tumors were predominantly located in the extremity and torso. Thirty of 35 (85%) of curative group patients treated postoperatively had close or positive surgical margins. Thirty-four (82%) lesions treated for palliation were unresectable. Thirty-five patients (53%) were treated at the time of recurrence. Median tumor size at initial presentation was 8.0 cm (range 0.6-29), median treated gross disease size was 5.0 cm (range 1-22), and 46/69 evaluable lesions (67%) were judged to be of intermediate to high histologic grade. Fourteen patients (21%) had chondrosarcomas. Results: Median follow-up was 6 months (range 2-47) and 38 months (range 2-175) for the palliative and curative groups, respectively. Kaplan-Meier estimates were obtained for probability of local relapse-free survival (68%), distant disease-free survival (59%), cause-specific survival (68%), and overall survival (66%) at

  8. A rheumatoid nodule mimicking soft tissue sarcoma on MR: a case report

    International Nuclear Information System (INIS)

    Choi, Joon Il; Kong, Keun Young; Kang, Heung Sik

    2000-01-01

    We report a case of rheumatoid nodule of the right knee in a 55-year-old woman which on the basis of MR images was misdiagnosed as soft tissue sarcoma. Reumatoid nodules occur in up to 20% of adult patients with rheumatoid arthritis and most patients with rheumatoid nodules are rheumatoid factor positive. The nodules are most commonly located between the skin and an underlying bony prominence. In this case, the patient had a history of rheumatoid arthritis and a soft tissue mass was present in the lateral aspect of the right knee. T1-weighted images revealed a mass of similar signal intensity to that of muscle, and on T2-weighted images, a central portion of high signal intensity was noted. Enhanced T1-weighted images demonstrated peripheral rim enhancement. The mass extended to subcutaneous tissue, with penetration of lateral patellar retinaculum. Excisional biopsy was performed, and the pathologic diagnosis was rheumatoid nodule. Although its radiologic findings are non-specific, rheumatoid nodule should -in appropriate clinical setting- be included in the differential diagnosis of a predominantly solid or centrally necrotic periarticular soft tissue. (author)

  9. Successful collection of peripheral blood stem cells upon VIDE chemomobilization in sarcoma patients.

    Science.gov (United States)

    Kriegsmann, Katharina; Heilig, Christoph; Cremer, Martin; Novotny, Philipp; Kriegsmann, Mark; Bruckner, Thomas; Müller-Tidow, Carsten; Egerer, Gerlinde; Wuchter, Patrick

    2017-11-01

    In patients with Ewing sarcoma and some distinct subgroups of soft tissue sarcoma (STS), a quantitatively sufficient autologous peripheral blood stem cell (PBSC) collection for stem cell support might facilitate treatment continuation, dose-intensification, and high-dose chemotherapy. Here, we provide a detailed evaluation of PBSC collection upon vincristine, ifosfamide, doxorubicin, and etoposide (VIDE) chemomobilization. Mobilization and collection parameters of 42 sarcoma patients (Ewing sarcoma n = 35, other STS n = 7) were analyzed retrospectively. Data were evaluated with regard to the number of previous VIDE therapy cycles. All patients reached the collection goal of ≥2.0 × 10 6 CD34 + cells/kg body weight (bw) upon VIDE/G-CSF mobilization, in the majority of cases with one single leukapheresis (LP) session (n = 29, 69%). No significant differences were identified with regard to mobilization and collection variables or the number of previous induction VIDE therapy cycles. However, upon 5 cycles of VIDE, we found the highest relative proportion of patients who required two or three LP sessions. Our data demonstrate the feasibility of successful PBSC collection upon VIDE chemomobilization even after up to five cycles of induction therapy, while at the same time the increasing risk of bone marrow exhaustion with every consecutive cycle is outlined. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  10. The financial burden of reexcising incompletely excised soft tissue sarcomas: a cost analysis.

    Science.gov (United States)

    Alamanda, Vignesh K; Delisca, Gadini O; Mathis, Shannon L; Archer, Kristin R; Ehrenfeld, Jesse M; Miller, Mark W; Homlar, Kelly C; Halpern, Jennifer L; Schwartz, Herbert S; Holt, Ginger E

    2013-09-01

    Although survival outcomes have been evaluated between those undergoing a planned primary excision and those undergoing a reexcision following an unplanned resection, the financial implications associated with a reexcision have yet to be elucidated. A query for financial data (professional, technical, indirect charges) for soft tissue sarcoma excisions from 2005 to 2008 was performed. A total of 304 patients (200 primary excisions and 104 reexcisions) were identified. Wilcoxon rank sum tests and χ2 or Fisher's exact tests were used to compare differences in demographics and tumor characteristics. Multivariable linear regression analyses were performed with bootstrapping techniques. The average professional charge for a primary excision was $9,694 and $12,896 for a reexcision (pfinancial burden nearly doubles.

  11. Preoperative chemotherapy of bone and soft tissue sarcomas. Evaluation with dynamic MR imaging

    International Nuclear Information System (INIS)

    Ando, Yoko; Fukatsu, Hiroshi; Isomura, Takayuki; Itoh, Shigeki; Ishigaki, Takeo; Yamamura, Shigeki; Sugiura, Hideshi; Satoh, Keiji.

    1995-01-01

    Dynamic MR imaging and conventional angiography were performed in eleven patients with musculoskeletal malignant tumors before and after preoperative chemotherapy in order to evaluate its effect. Dynamic MRI was obtained with GRASS (TR/TE/FA=50/10-13/30) or SE (TR/TE=150-350/20). Although resected specimen in one case of osteosarcoma had the necrotic ratio of more than 90%, it had marked early enhancement in dynamic MRI, and microscopic examination revealed fibrotic necrosis with many capillaries. In soft tissue sarcomas with hemorrhage and/or cystic change, dynamic MRI findings did not necessarily correlate with the chemotherapy effect. Dynamic MRI was more useful than angiography because of its ability to show tumor vascularity and of its non-invasiveness. (author)

  12. Preoperative vs. postoperative radiotherapy in the treatment of soft tissue sarcomas: a matter of presentation

    International Nuclear Information System (INIS)

    Pollack, Alan; Zagars, Gunar K.; Goswitz, Mary S.; Pollock, Raphael A.; Feig, Barry W.; Pisters, Peter W.T.

    1998-01-01

    Purpose: Radiotherapy for soft tissue sarcoma is typically preoperative or postoperative, with advocates of each. In this study, the relationship of the sequencing of radiotherapy and surgery to local control was examined. Methods and Materials: The cohort consisted of 453 patients with Grade 2-3 malignant fibrous histiocytoma, synovial sarcoma, or liposarcoma treated from 1965-1992. Retroperitoneal sarcomas were excluded. Median follow-up was 97 months. There were 3 groups of patients that were classified by the treatment administered at our institution: preoperative radiotherapy to a median dose of 50 Gy given before excision at MDACC (Preop; n = 128); postoperative radiotherapy to a median dose of 64 Gy given after excision at MDACC (Postop; n = 165); and radiotherapy to a median dose of 65 Gy without excision at MDACC (RT Alone; n = 160). Those in the RT Alone Group had gross total excision at an outside center prior to referral. Results: Histological classification, whether locally recurrent at referral, and final MDACC margins were independent determinants of local control in Cox proportional hazards multivariate analysis using the entire cohort. The type of treatment was not significant; however, tumor status at presentation (gross disease vs. excised) affected these findings greatly. Gross disease treated with Preop was controlled locally in 88% at 10 years, as compared to 67% with Postop (p = 0.01). This association was independently significant for patients treated primarily (not for recurrence). In contrast, for those presenting after excision elsewhere, 10-year local control was better with Postop (88% vs. 73%, p = 0.07), particularly for patients treated primarily (91% vs. 72%, p 0.02 in univariate analysis; p = 0.06 in multivariate analysis). Re-excision at MDACC (Postop) resulted in enhanced 10-year local control over that with RT Alone (88% vs. 75%, p = 0.06), and was confirmed to be an independent predictor in multivariate analysis (p = 0

  13. Improved Survival With Radiation Therapy in High-Grade Soft Tissue Sarcomas of the Extremities: A SEER Analysis

    International Nuclear Information System (INIS)

    Koshy, Matthew; Rich, Shayna E.; Mohiuddin, Majid M.

    2010-01-01

    Purpose: The benefit of radiation therapy in extremity soft tissue sarcomas remains controversial. The purpose of this study was to determine the effect of radiation therapy on overall survival among patients with primary soft tissue sarcomas of the extremity who underwent limb-sparing surgery. Methods and Materials: A retrospective study from the Surveillance, Epidemiology, and End Results (SEER) database that included data from January 1, 1988, to December 31, 2005. A total of 6,960 patients constituted the study population. Overall survival curves were constructed using the Kaplan-Meir method and for patients with low- and high-grade tumors. Hazard ratios were calculated based on multivariable Cox proportional hazards models. Results: Of the cohort, 47% received radiation therapy. There was no significant difference in overall survival among patients with low-grade tumors by radiation therapy. In high-grade tumors, the 3-year overall survival was 73% in patients who received radiation therapy vs. 63% for those who did not receive radiation therapy (p < 0.001). On multivariate analysis, patients with high-grade tumors who received radiation therapy had an improved overall survival (hazard ratio 0.67, 95% confidence interval 0.57-0.79). In patients receiving radiation therapy, 13.5% received it in a neoadjuvant setting. The incidence of patients receiving neoadjuvant radiation did not change significantly between 1988 and 2005. Conclusions: To our knowledge, this is the largest population-based study reported in patients undergoing limb-sparing surgery for soft tissue sarcomas of the extremities. It reports that radiation was associated with improved survival in patients with high-grade tumors.

  14. Therapeutic options and postoperative wound complications after extremity soft tissue sarcoma resection and postoperative external beam radiotherapy.

    Science.gov (United States)

    Abouarab, Mohamed H; Salem, Iman L; Degheidy, Magdy M; Henn, Dominic; Hirche, Christoph; Eweida, Ahmad; Uhl, Matthias; Kneser, Ulrich; Kremer, Thomas

    2018-02-01

    Soft tissue sarcomas occur most commonly in the lower and upper extremities. The standard treatment is limb salvage surgery combined with radiotherapy. Postoperative radiotherapy is associated with wound complications. This systematic review aims to summarise the available evidence and review the literature of the last 10 years regarding postoperative wound complications in patients who had limb salvage surgical excision followed by direct closure vs flap coverage together with postoperative radiotherapy and to define the optimal timeframe for adjuvant radiotherapy after soft tissue sarcomas resection and flap reconstruction. A literature search was performed using PubMed. The following keywords were searched: limb salvage, limb-sparing, flaps, radiation therapy, radiation, irradiation, adjuvant radiotherapy, postoperative radiotherapy, radiation effects, wound healing, surgical wound infection, surgical wound dehiscence, wound healing, soft tissue sarcoma and neoplasms. In total, 1045 papers were retrieved. Thirty-seven articles were finally selected after screening of abstracts and applying dates and language filters and inclusion and exclusion criteria. Plastic surgery provides a vast number of reconstructive flap procedures that are directly linked to decreasing wound complications, especially with the expectant postoperative radiotherapy. This adjuvant radiotherapy is better administered in the first 3-6 weeks after reconstruction to allow timely wound healing and avoid local recurrence. © 2017 Medicalhelplines.com Inc and John Wiley & Sons Ltd.

  15. Durable clinical activity of single-agent bevacizumab in a nonagenarian patient with metastatic alveolar soft part sarcoma.

    Science.gov (United States)

    Mir, Olivier; Boudou-Rouquette, Pascaline; Larousserie, Frédérique; Blanchet, Benoit; Babinet, Antoine; Anract, Philippe; Goldwasser, François

    2012-08-01

    Alveolar soft part sarcoma is a rare malignancy usually considered resistant to conventional chemotherapy, but recent data suggest that the multikinase inhibitors sunitinib and cediranib could be active in this setting. A 90-year-old lady with alveolar soft part sarcoma of the leg and lung metastases was started on sunitinib 37.5 mg daily. The treatment was poorly tolerated with grade 3 hypertension and grade 3 thrombocytopenia, which persisted after dose reduction to 25 mg daily. The patient was subsequently started on bevacizumab 10 mg/kg every 2 weeks, resulting in a marked improvement in pain and a partial response on lung metastases for 16 months and ongoing. Agents targeting the vascular endothelial growth factor-signalling pathway seem to exert clinically relevant and prolonged activity against alveolar soft part sarcoma and deserve further evaluation in the treatment of this rare soft tissue sarcoma.

  16. Secondary Leukemia in a non-Hodgkin's Lymphoma Patient Presenting as Myeloid Sarcoma of the Breast

    Directory of Open Access Journals (Sweden)

    Vincenzo Pitini

    2011-01-01

    Full Text Available As defined by the World Health Organization classification of tumors of hematopoietic and lymphoid tissue, myeloid sarcoma (MS is a tumor mass of myeloblasts or immature myeloid cells that can arise before, concurrent with, or following acute myeloid leukaemia. We describe a case of secondary leukemia presenting itself as MS of the breast in a patient previously treated for a non-Hodgkin's Lymphoma.

  17. Survival of patients with Ewing's sarcoma in Yazd-Iran.

    Science.gov (United States)

    Akhavan, Ali; Binesh, Fariba; Shamshiri, Hadi; Ghanadi, Fazllolah

    2014-01-01

    The Ewing's sarcoma family is a group of small round cell tumors which accounts for 10-15% of all primary bone neoplasms. The aim of this study was to evaluate the survival of Ewing's sarcoma patients in our province and to determine of influencing factors. All patients with documented Ewing's sarcoma/ primitive neuroectodermal tumor(PNET) family pathology were enrolled in this study during a period of eight years. For all of them local and systemic therapy were carried out. Overall and event free survival and prognostic factors were evaluated. Thirty two patients were enrolled in the study. The median age was 17.5 years. Twenty (65.2%) were male and 9 (28.1%) were aged 14 years or less. Mean disease free survival was 26.8 (95%CI; 13.8-39.9) months and five year disease free survival was 26%. Mean overall survival was 38.7 months (95%CI; 25.9-50.6) and median overall survival was 24 months. Five year overall survival was 25%. From the variables evaluated , only presence of metastatic disease at presentation (p value=0. 028) and complete response (p value =0. 006) had significant relations to overall survival. Survival of Ewing's sarcoma in our province is disappointing. It seems to be mostly due to less effective treatment. Administration of adequate chemotherapy dosage, resection of tumor with negative margins and precise assessment of irradiation volume may prove helpful.

  18. Inter- and intra-observer variation in soft-tissue sarcoma target definition.

    Science.gov (United States)

    Roberge, D; Skamene, T; Turcotte, R E; Powell, T; Saran, N; Freeman, C

    2011-08-01

    To evaluate inter- and intra-observer variability in gross tumor volume definition for adult limb/trunk soft tissue sarcomas. Imaging studies of 15 patients previously treated with preoperative radiation were used in this study. Five physicians (radiation oncologists, orthopedic surgeons and a musculoskeletal radiologist) were asked to contour each of the 15 tumors on T1-weighted, gadolinium-enhanced magnetic resonance images. These contours were drawn twice by each physician. The volume and center of mass coordinates for each gross tumor volume were extracted and a Boolean analysis was performed to measure the degree of volume overlap. The median standard deviation in gross tumor volumes across observers was 6.1% of the average volume (range: 1.8%-24.9%). There was remarkably little variation in the 3D position of the gross tumor volume center of mass. For the 15 patients, the standard deviation of the 3D distance between centers of mass ranged from 0.06 mm to 1.7 mm (median 0.1mm). Boolean analysis demonstrated that 53% to 90% of the gross tumor volume was common to all observers (median overlap: 79%). The standard deviation in gross tumor volumes on repeat contouring was 4.8% (range: 0.1-14.4%) with a standard deviation change in the position of the center of mass of 0.4mm (range: 0mm-2.6mm) and a median overlap of 93% (range: 73%-98%). Although significant inter-observer differences were seen in gross tumor volume definition of adult soft-tissue sarcoma, the center of mass of these volumes was remarkably consistent. Variations in volume definition did not correlate with tumor size. Radiation oncologists should not hesitate to review their contours with a colleague (surgeon, radiologist or fellow radiation oncologist) to ensure that they are not outliers in sarcoma gross tumor volume definition. Protocols should take into account variations in volume definition when considering tighter clinical target volumes. Copyright © 2011 Société française de radioth

  19. Roentgenologic examination in Kaposi's sarcoma

    International Nuclear Information System (INIS)

    Kossovoj, A.L.

    1990-01-01

    Review of roentgenologic investigations into Kaposi's sarcoma is presented. It is shown that Kaposi's sarcoma is a disease injuring skin, osteal system, lungs and mediastinum, gastroeuteric tract and lymphatic nodes. Roentgenologic changes of soft tissues of limbs, osteal system, chest and gastroenteric tract organs are described. Manifestations of a tumor of any localization are quite different which makes it more difficult to perform roentgenologic diagnosis. An increase of Kaposi's sarcoma occurrence in patients suffering from aids as the disease increases is indicated

  20. Results of total lung irradiation and chemotherapy in comparison with partial lung irradiation in metastatic undifferentiated soft tissue sarcomas

    Energy Technology Data Exchange (ETDEWEB)

    Zamboglou, N.; Fuerst, G.; Pape, H.; Bannach, B.; Schmitt, G.; Molls, M.

    1988-07-01

    The poor prognosis of patients with unresectable pulmonary metastases of soft tissue sarcoma is well known. In order to evaluate the beneficial effect of radiotherapy, we have treated 44 patients with pulmonary metastases of grade 3 soft tissue sarcoma from 1980 to 1986. In 36 patients the treatment volume was restricted to the single metastases up to a dose of 50 to 60 (9 to 10 Gy/week). The survival rate at one year was 18% and at two years 6%. Eight patients were treated with a combined regimen, consisting of cisplatin and ifosfamide with simultaneous whole lung irradiation. Irradiation was performed with 8 or 16 MV photons at a hyperfractionation of 2x0,8 Gy/day (8 Gy/week). After a dose of 12 Gy, the single metastases were boosted up to 50 to 60 Gy, with a second course of chemotherapy. In six of eight patients complete remissions were achieved, one patient showed a partial remission. The survival rate at 27 months was 50%. The patients with partial remission died from pulmonary progression at 23 months. One patient died after twelve months from a loco-regional recurrence in the tonsillar fossa without evidence of pulmonary disease. Side effects included alopecia and moderate bone marrow suppression approximately twelve days after each chemotherapy cycle. Pulmonary fibrosis was observed only at the high dose volume without impairment of respiratory function. From these observations the conclusion is drawn that whole lung irradiation simultaneously with cisplatin and ifosfamide chemotherapy provides good palliative results without relevant morbidity in patients with high grade unresectable pulmonary metastases of soft tissue sarcomas.

  1. Association Between Treatment at High-Volume Facilities and Improved Overall Survival in Soft Tissue Sarcomas.

    Science.gov (United States)

    Venigalla, Sriram; Nead, Kevin T; Sebro, Ronnie; Guttmann, David M; Sharma, Sonam; Simone, Charles B; Levin, William P; Wilson, Robert J; Weber, Kristy L; Shabason, Jacob E

    2018-03-15

    Soft tissue sarcomas (STS) are rare malignancies that require complex multidisciplinary management. Therefore, facilities with high sarcoma case volume may demonstrate superior outcomes. We hypothesized that STS treatment at high-volume (HV) facilities would be associated with improved overall survival (OS). Patients aged ≥18 years with nonmetastatic STS treated with surgery and radiation therapy at a single facility from 2004 through 2013 were identified from the National Cancer Database. Facilities were dichotomized into HV and low-volume (LV) cohorts based on total case volume over the study period. OS was assessed using multivariable Cox regression with propensity score-matching. Patterns of care were assessed using multivariable logistic regression analysis. Of 9025 total patients, 1578 (17%) and 7447 (83%) were treated at HV and LV facilities, respectively. On multivariable analysis, high educational attainment, larger tumor size, higher grade, and negative surgical margins were statistically significantly associated with treatment at HV facilities; conversely, black race and non-metropolitan residence were negative predictors of treatment at HV facilities. On propensity score-matched multivariable analysis, treatment at HV facilities versus LV facilities was associated with improved OS (hazard ratio, 0.87, 95% confidence interval, 0.80-0.95; P = .001). Older age, lack of insurance, greater comorbidity, larger tumor size, higher tumor grade, and positive surgical margins were associated with statistically significantly worse OS. In this observational cohort study using the National Cancer Database, receipt of surgery and radiation therapy at HV facilities was associated with improved OS in patients with STS. Potential sociodemographic disparities limit access to care at HV facilities for certain populations. Our findings highlight the importance of receipt of care at HV facilities for patients with STS and warrant further study into improving access to

  2. Evaluation of Soft Tissue Sarcoma Tumors Electrical Conductivity Anisotropy Using Diffusion Tensor Imaging for Numerical Modeling on Electroporation

    Directory of Open Access Journals (Sweden)

    Ghazikhanlou-sani K.

    2016-06-01

    Full Text Available Introduction: There is many ways to assessing the electrical conductivity anisotropy of a tumor. Applying the values of tissue electrical conductivity anisotropy is crucial in numerical modeling of the electric and thermal field distribution in electroporation treatments. This study aims to calculate the tissues electrical conductivity anisotropy in patients with sarcoma tumors using diffusion tensor imaging technique. Materials and Method: A total of 3 subjects were involved in this study. All of patients had clinically apparent sarcoma tumors at the extremities. The T1, T2 and DTI images were performed using a 3-Tesla multi-coil, multi-channel MRI system. The fractional anisotropy (FA maps were performed using the FSL (FMRI software library software regarding the DTI images. The 3D matrix of the FA maps of each area (tumor, normal soft tissue and bone/s was reconstructed and the anisotropy matrix was calculated regarding to the FA values. Result: The mean FA values in direction of main axis in sarcoma tumors were ranged between 0.475–0.690. With assumption of isotropy of the electrical conductivity, the FA value of electrical conductivity at each X, Y and Z coordinate axes would be equal to 0.577. The gathered results showed that there is a mean error band of 20% in electrical conductivity, if the electrical conductivity anisotropy not concluded at the calculations. The comparison of FA values showed that there is a significant statistical difference between the mean FA value of tumor and normal soft tissues (P<0.05. Conclusion: DTI is a feasible technique for the assessment of electrical conductivity anisotropy of tissues. It is crucial to quantify the electrical conductivity anisotropy data of tissues for numerical modeling of electroporation treatments.

  3. Latent and lytic HHV-8 mRNA expression in PBMCs and Kaposi's sarcoma skin biopsies of AIDS Kaposi's sarcoma patients

    NARCIS (Netherlands)

    Polstra, Abeltje M.; Goudsmit, Jaap; Cornelissen, Marion

    2003-01-01

    Human herpes virus 8 (HHV-8) is associated with all clinical forms of Kaposi's sarcoma. HHV-8 DNA is present in Kaposi's sarcoma biopsies and is observed regularly in saliva and less consistently in blood of Kaposi's sarcoma patients. The expression pattern of latent (ORF 73) and lytic (vGCR,

  4. Adult Soft Tissue Sarcoma Treatment (PDQ®)—Health Professional Version

    Science.gov (United States)

    Soft tissue sarcomas (STS) arise in any of the extremities, trunk, retroperitoneum, or head and neck. Treatment is determined by the tumor grade and options include surgery, radiation therapy, and chemotherapy. Get comprehensive information for STS and treatment in this clinician summary.

  5. HEMIPELVECTOMY AND INTRAOPERATIVE RADIOTHERAPY FOR BONE AND SOFT-TISSUE SARCOMAS OF THE PELVIC GIRDLE

    NARCIS (Netherlands)

    HOEKSTRA, HJ; SINDELAR, WF; SZABO, BG; KINSELLA, TJ

    1995-01-01

    Current treatment of locally advanced bone and soft tissue sarcomas of the pelvic girdle are associated with a high local and distant failure rate, and local tumor control after hemipelvectomy can be a significant problem. IORT has been used in conjunction with hemipelvectomy, both conventional

  6. Development of Soft Tissue Sarcomas in Ribosomal Proteins L5 and S24 Heterozygous Mice

    Czech Academy of Sciences Publication Activity Database

    Kazerounian, S.; Ciarlini, P.D.S.C.; Yuan, D.; Ghazvinian, R.; Alberich-Jorda, Meritxell; Joshi, M.; Zhang, H.; Beggs, A.H.; Gazda, H.T.

    2016-01-01

    Roč. 7, č. 1 (2016), s. 32-36 ISSN 1837-9664 R&D Projects: GA MŠk LK21307 Institutional support: RVO:68378050 Keywords : Ribosomal proteins RPL5 and RPS24 * Diamond-Blackfan anemia * Soft tissue sarcoma Subject RIV: EB - Genetics ; Molecular Biology Impact factor: 2.916, year: 2016

  7. Tissue dose in thorotrast patients

    International Nuclear Information System (INIS)

    Kaul, A.; Noffz, W.

    1978-01-01

    Absorbed doses to the liver, spleen, red marrow, lungs, kidneys, and to various parts of bone tissue were calculated for long-term burdens of intravascularly injected Thorotrast. The estimates were performed for typical injection levels of 10, 30, 50 and 100 ml, based upon best estimates of 232 Th tissue distribution, and steady state activity ratios between the subsequent daughters. Correcting for the α-particle self absorption within Thorotrast aggregates, the mean α-dose to a standard 70-kg man at 30 yr after the injection 0f 25 ml of Thorotrast is 750 rad to the liver, 2100 rad to the spleen, 270 rad to the red marrow, 60-620 rad in various parts of the lung, and 13 rad to the kidneys. Dose rates to various parts of bone tissue (bone surface, compact, and cancellous bone) were estimated by applying the ICRP model on alkaline earth metabolism to the continuous translocation of thorium daughters to bone and to the formation of thorium daughters by decay within bone tissue. The average dose to calcified bone from translocated 224 Ra with its daughters is 18 rad at 30 yr after the injection of 25 ml of Thorotrast. Considering the Spiess-Mays risk coefficient of 0.9-1.7% bone sarcoma/ 100 rad of average skeletal dose from 224 Ra and its daughters, the induction of 1.6-3.1 bone sarcomas per 1000 Thorotrast patients is predicted. (author)

  8. General Information about Kaposi Sarcoma

    Science.gov (United States)

    ... Sarcoma Treatment Childhood Vascular Tumors Treatment Research Kaposi Sarcoma Treatment (PDQ®)–Patient Version General Information About Kaposi Sarcoma Go to Health Professional Version Key Points Kaposi ...

  9. Agreement Among RTOG Sarcoma Radiation Oncologists in Contouring Suspicious Peritumoral Edema for Preoperative Radiation Therapy of Soft Tissue Sarcoma of the Extremity

    Energy Technology Data Exchange (ETDEWEB)

    Bahig, Houda [Department of Radiation Oncology, Centre Hospitalier de l' Université de Montréal, Montreal, QC (Canada); Roberge, David, E-mail: david.roberge.chum@ssss.gouv.qc.ca [Department of Radiation Oncology, Centre Hospitalier de l' Université de Montréal, Montreal, QC (Canada); Bosch, Walter [Department of Radiation Oncology, Washington University, St Louis, Missouri (United States); Levin, William [Department of Radiation Oncology, University of Pennsylvania, Philadelphia, Pennsylvania (United States); Petersen, Ivy; Haddock, Michael [Department of Radiation Oncology, Mayo Clinic, Rochester, Minnesota (United States); Freeman, Carolyn [Division of Radiation Oncology, McGill University Health Centre, Montreal, QC (Canada); DeLaney, Thomas F. [Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts (United States); Abrams, Ross A. [Department of Radiation Oncology, Rush University Medical Center, Chicago, Illinois (United States); Indelicato, Danny J. [Department of Radiation Oncology, University of Florida Medical Center, Jacksonville, Florida (United States); Baldini, Elizabeth H. [Department of Radiation Oncology, Brigham and Women' s Hospital and Dana-Farber Cancer Institute, Boston, Massachusetts (United States); Hitchcock, Ying [Department of Radiation Oncology, University of Utah Medical Center, Salt Lake City, Utah (United States); Kirsch, David G. [Department of Radiation Oncology, Duke University Medical Center, Durham, North Carolina (United States); Kozak, Kevin R. [Department of Human Oncology, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin (United States); Wolfson, Aaron [Department of Radiation Oncology, University of Miami Miller School of Medicine, Miami, Florida (United States); and others

    2013-06-01

    Purpose: Peritumoral edema may harbor sarcoma cells. The extent of suspicious edema (SE) included in the treatment volume is subject to clinical judgment, balancing the risk of missing tumor cells with excess toxicity. Our goal was to determine variability in SE delineation by sarcoma radiation oncologists (RO). Methods and Materials: Twelve expert ROs were provided with T1 gadolinium and T2-weighted MR images of 10 patients with high-grade extremity soft-tissue sarcoma. Gross tumor volume, clinical target volume (CTV)3cm (3 cm longitudinal and 1.5 cm radial margin), and CTV2cm (2 cm longitudinal and 1 cm radial margin) were contoured by a single observer. Suspicious peritumoral edema, defined as abnormal signal on T2 images, was independently delineated by all 12 ROs. Contouring agreement was analyzed using the simultaneous truth and performance level estimation (STAPLE) algorithm and kappa statistics. Results: The mean volumes of GTV, CTV2cm, and CTV3cm were, respectively, 130 cm{sup 3} (7-413 cm{sup 3}), 280 cm{sup 3} and 360 cm{sup 3}. The mean consensus volume computed using the STAPLE algorithm at 95% confidence interval was 188 cm{sup 3} (24-565 cm{sup 3}) with a substantial overall agreement corrected for chance (mean kappa = 0.71; range: 0.32-0.87). The minimum, maximum, and mean volume of SE (excluding the GTV) were 4, 182, and 58 cm{sup 3} (representing a median of 29% of the GTV volume). The median volume of SE not included in the CTV2cm and in the CTV3cm was 5 and 0.3 cm{sup 3}, respectively. There were 3 large tumors with >30 cm{sup 3} of SE not included in the CTV3cm volume. Conclusion: Despite the fact that SE would empirically seem to be a more subjective volume, a substantial or near-perfect interobserver agreement was observed in SE delineation in most cases with high-grade soft-tissue sarcomas of the extremity. A median of 97% of the consensus SE is within the CTV2cm (99.8% within the CTV3cm). In a minority of cases, however, significant

  10. Agreement among RTOG sarcoma radiation oncologists in contouring suspicious peritumoral edema for preoperative radiation therapy of soft tissue sarcoma of the extremity.

    Science.gov (United States)

    Bahig, Houda; Roberge, David; Bosch, Walter; Levin, William; Petersen, Ivy; Haddock, Michael; Freeman, Carolyn; Delaney, Thomas F; Abrams, Ross A; Indelicato, Danny J; Baldini, Elizabeth H; Hitchcock, Ying; Kirsch, David G; Kozak, Kevin R; Wolfson, Aaron; Wang, Dian

    2013-06-01

    Peritumoral edema may harbor sarcoma cells. The extent of suspicious edema (SE) included in the treatment volume is subject to clinical judgment, balancing the risk of missing tumor cells with excess toxicity. Our goal was to determine variability in SE delineation by sarcoma radiation oncologists (RO). Twelve expert ROs were provided with T1 gadolinium and T2-weighted MR images of 10 patients with high-grade extremity soft-tissue sarcoma. Gross tumor volume, clinical target volume (CTV)3cm (3 cm longitudinal and 1.5 cm radial margin), and CTV2cm (2 cm longitudinal and 1 cm radial margin) were contoured by a single observer. Suspicious peritumoral edema, defined as abnormal signal on T2 images, was independently delineated by all 12 ROs. Contouring agreement was analyzed using the simultaneous truth and performance level estimation (STAPLE) algorithm and kappa statistics. The mean volumes of GTV, CTV2cm, and CTV3cm were, respectively, 130 cm(3) (7-413 cm(3)), 280 cm(3) and 360 cm(3). The mean consensus volume computed using the STAPLE algorithm at 95% confidence interval was 188 cm(3) (24-565 cm(3)) with a substantial overall agreement corrected for chance (mean kappa = 0.71; range: 0.32-0.87). The minimum, maximum, and mean volume of SE (excluding the GTV) were 4, 182, and 58 cm(3) (representing a median of 29% of the GTV volume). The median volume of SE not included in the CTV2cm and in the CTV3cm was 5 and 0.3 cm(3), respectively. There were 3 large tumors with >30 cm(3) of SE not included in the CTV3cm volume. Despite the fact that SE would empirically seem to be a more subjective volume, a substantial or near-perfect interobserver agreement was observed in SE delineation in most cases with high-grade soft-tissue sarcomas of the extremity. A median of 97% of the consensus SE is within the CTV2cm (99.8% within the CTV3cm). In a minority of cases, however, significant expansion of the CTVs is required to cover SE. Copyright © 2013 Elsevier Inc. All rights

  11. Agreement Among RTOG Sarcoma Radiation Oncologists in Contouring Suspicious Peritumoral Edema for Preoperative Radiation Therapy of Soft Tissue Sarcoma of the Extremity

    International Nuclear Information System (INIS)

    Bahig, Houda; Roberge, David; Bosch, Walter; Levin, William; Petersen, Ivy; Haddock, Michael; Freeman, Carolyn; DeLaney, Thomas F.; Abrams, Ross A.; Indelicato, Danny J.; Baldini, Elizabeth H.; Hitchcock, Ying; Kirsch, David G.; Kozak, Kevin R.; Wolfson, Aaron

    2013-01-01

    Purpose: Peritumoral edema may harbor sarcoma cells. The extent of suspicious edema (SE) included in the treatment volume is subject to clinical judgment, balancing the risk of missing tumor cells with excess toxicity. Our goal was to determine variability in SE delineation by sarcoma radiation oncologists (RO). Methods and Materials: Twelve expert ROs were provided with T1 gadolinium and T2-weighted MR images of 10 patients with high-grade extremity soft-tissue sarcoma. Gross tumor volume, clinical target volume (CTV)3cm (3 cm longitudinal and 1.5 cm radial margin), and CTV2cm (2 cm longitudinal and 1 cm radial margin) were contoured by a single observer. Suspicious peritumoral edema, defined as abnormal signal on T2 images, was independently delineated by all 12 ROs. Contouring agreement was analyzed using the simultaneous truth and performance level estimation (STAPLE) algorithm and kappa statistics. Results: The mean volumes of GTV, CTV2cm, and CTV3cm were, respectively, 130 cm 3 (7-413 cm 3 ), 280 cm 3 and 360 cm 3 . The mean consensus volume computed using the STAPLE algorithm at 95% confidence interval was 188 cm 3 (24-565 cm 3 ) with a substantial overall agreement corrected for chance (mean kappa = 0.71; range: 0.32-0.87). The minimum, maximum, and mean volume of SE (excluding the GTV) were 4, 182, and 58 cm 3 (representing a median of 29% of the GTV volume). The median volume of SE not included in the CTV2cm and in the CTV3cm was 5 and 0.3 cm 3 , respectively. There were 3 large tumors with >30 cm 3 of SE not included in the CTV3cm volume. Conclusion: Despite the fact that SE would empirically seem to be a more subjective volume, a substantial or near-perfect interobserver agreement was observed in SE delineation in most cases with high-grade soft-tissue sarcomas of the extremity. A median of 97% of the consensus SE is within the CTV2cm (99.8% within the CTV3cm). In a minority of cases, however, significant expansion of the CTVs is required to cover SE

  12. Kaposi’s sarcoma in an HIV-negative patient

    Directory of Open Access Journals (Sweden)

    Clara Georgina Garcia Lahera

    2015-06-01

    Full Text Available Kaposi’s sarcoma is a vascular tumour of the skin, most frequently seen in men over 50 years of age, of long-term progress and low mortality. It is related to the infection caused by the human immunodeficiency virus (HIV; it appears in the advanced stages of the disease and with immunosuppression, affecting approximately 20 % of the people with HIV who do not take antiretroviral drugs. This is a case of an urban female patient who did not have a history of disease and who began to have squamous erythematous lesions on the right foot and on the thighs. A skin biopsy was performed and the patient was diagnosed with a Kaposi’s sarcoma. The patient was HIV-negative. This case is presented as it is a rare condition in an HIV-negative patient.

  13. SU-G-JeP4-14: Assessment of Inter- and Intra-Fractional Motion for Extremity Soft Tissue Sarcoma Patients by Using In-House Real-Time Optical Image-Based Monitoring System

    Energy Technology Data Exchange (ETDEWEB)

    Kim, H [Interdisciplinary Program in Radiation Applied Life Science, College of Medicine, Seoul National University, Seoul (Korea, Republic of); Kim, I [Dept. of Radiation Oncology, Seoul National University Hospital, Seoul (Korea, Republic of); Ye, S [Dept. of Radiation Oncology, Seoul National University Hospital, Seoul (Korea, Republic of); Program in Biomedical Radiation Sciences, Graduate School of Convergence Science and Technology, Seoul National University, Seoul (Korea, Republic of)

    2016-06-15

    Purpose: This study aimed to assess inter- and intra-fractional motion for extremity Soft Tissue Sarcoma (STS) patients, by using in-house real-time optical image-based monitoring system (ROIMS) with infra-red (IR) external markers. Methods: Inter- and intra-fractional motions for five extremity (1 upper, 4 lower) STS patients received postoperative 3D conformal radiotherapy (3D-CRT) were measured by registering the image acquired by ROIMS with the planning CT image (REG-ROIMS). To compare with the X-ray image-based monitoring, pre- and post-treatment cone beam computed tomography (CBCT) scans were performed once per week and registered with planning CT image as well (REG-CBCT). If the CBCT scan is not feasible due to the large couch shift, AP and LR on-board imager (OBI) images were acquired. The comparison was done by calculating mutual information (MI) of those registered images. Results: The standard deviation (SD) of the inter-fractional motion was 2.6 mm LR, 2.8 mm SI, and 2.0 mm AP, and the SD of the intra-fractional motion was 1.4 mm, 2.1 mm, and 1.3 mm in each axis, respectively. The SD of rotational inter-fractional motion was 0.6° pitch, 0.9° yaw, and 0.8° roll and the SD of rotational intra-fractional motion was 0.4° pitch, 0.9° yaw, and 0.7° roll. The derived averaged MI values were 0.83, 0.92 for REG-CBCT without rotation and REG-ROIMS with rotation, respectively. Conclusion: The in-house real-time optical image-based monitoring system was implemented clinically and confirmed the feasibility to assess inter- and intra-fractional motion for extremity STS patients while the daily basis and real-time CBCT scan is not feasible in clinic.

  14. SU-G-JeP4-14: Assessment of Inter- and Intra-Fractional Motion for Extremity Soft Tissue Sarcoma Patients by Using In-House Real-Time Optical Image-Based Monitoring System

    International Nuclear Information System (INIS)

    Kim, H; Kim, I; Ye, S

    2016-01-01

    Purpose: This study aimed to assess inter- and intra-fractional motion for extremity Soft Tissue Sarcoma (STS) patients, by using in-house real-time optical image-based monitoring system (ROIMS) with infra-red (IR) external markers. Methods: Inter- and intra-fractional motions for five extremity (1 upper, 4 lower) STS patients received postoperative 3D conformal radiotherapy (3D-CRT) were measured by registering the image acquired by ROIMS with the planning CT image (REG-ROIMS). To compare with the X-ray image-based monitoring, pre- and post-treatment cone beam computed tomography (CBCT) scans were performed once per week and registered with planning CT image as well (REG-CBCT). If the CBCT scan is not feasible due to the large couch shift, AP and LR on-board imager (OBI) images were acquired. The comparison was done by calculating mutual information (MI) of those registered images. Results: The standard deviation (SD) of the inter-fractional motion was 2.6 mm LR, 2.8 mm SI, and 2.0 mm AP, and the SD of the intra-fractional motion was 1.4 mm, 2.1 mm, and 1.3 mm in each axis, respectively. The SD of rotational inter-fractional motion was 0.6° pitch, 0.9° yaw, and 0.8° roll and the SD of rotational intra-fractional motion was 0.4° pitch, 0.9° yaw, and 0.7° roll. The derived averaged MI values were 0.83, 0.92 for REG-CBCT without rotation and REG-ROIMS with rotation, respectively. Conclusion: The in-house real-time optical image-based monitoring system was implemented clinically and confirmed the feasibility to assess inter- and intra-fractional motion for extremity STS patients while the daily basis and real-time CBCT scan is not feasible in clinic.

  15. Calcific haemorrhagic bursitis anterior to the knee mimicking a soft tissue sarcoma: report of two cases

    Energy Technology Data Exchange (ETDEWEB)

    Stahnke, M.; Davies, A.M. [Department of Radiology, Royal Orthopaedic Hospital, Woodlands, B31 2AP, Northfield, Birmingham (United Kingdom); Mangham, D.C. [Department of Pathology, Royal Orthopaedic Hospital, Woodlands, B31 2AP, Northfield, Birmingham (United Kingdom)

    2004-06-01

    We describe the radiological and pathological findings of two cases of calcific haemorrhagic bursitis, one involving the superficial infrapatellar bursa and the other the prepatellar bursa. It was the presence of dystrophic calcification within the lesion that suggested a mineralizing soft tissue sarcoma such as synovial sarcoma. As the radiographic and MR features of the two conditions can be similar but the appropriate management very different, rare calcifying haemorrhagic bursitis needs to be included in the differential diagnosis of masses adjacent to the knee joint showing calcification. (orig.)

  16. Calcific haemorrhagic bursitis anterior to the knee mimicking a soft tissue sarcoma: report of two cases

    International Nuclear Information System (INIS)

    Stahnke, M.; Davies, A.M.; Mangham, D.C.

    2004-01-01

    We describe the radiological and pathological findings of two cases of calcific haemorrhagic bursitis, one involving the superficial infrapatellar bursa and the other the prepatellar bursa. It was the presence of dystrophic calcification within the lesion that suggested a mineralizing soft tissue sarcoma such as synovial sarcoma. As the radiographic and MR features of the two conditions can be similar but the appropriate management very different, rare calcifying haemorrhagic bursitis needs to be included in the differential diagnosis of masses adjacent to the knee joint showing calcification. (orig.)

  17. A Phase I/II Clinical Trial of Belinostat (PXD101) in Combination with Doxorubicin in Patients with Soft Tissue Sarcomas

    DEFF Research Database (Denmark)

    Vitfell-Rasmussen, Joanna; Judson, Ian; Safwat, Akmal Ahmed

    2016-01-01

    ) and 75 mg/m(2) Dox. Results. 41 patients were included (25 in phase I, 16 in phase II). Adverse events were fatigue (95%), nausea (76%), and alopecia (63%). There was one DLT, grade 3 rash/hand and foot syndrome. MTD was Bel 1000 mg/m(2)/d and Dox 75 mg/m(2). Four responses were seen: 2 PR in phase I, RR...

  18. Isolated limb perfusion with tumor necrosis factor alpha and melphalan for locally advanced soft tissue sarcoma : Three time periods at risk for amputation

    NARCIS (Netherlands)

    van Ginkel, Robert J.; Thijssens, Katja M. J.; Pras, Elisabeth; van der Graaf, Winette T. A.; Suurmeijer, Albert J. H.; Hoekstra, Harald J.

    Background: The aim of this study was to investigate the long-term limb salvage rate and overall survival after isolated limb perfusion (ILP) with tumor necrosis factor alpha and melphalan for locally advanced soft tissue sarcoma (STS). Methods: From 1991 to 2003, 73 patients (36 men, 37 women,

  19. Isolated limb perfusion with tumor necrosis factor alpha and melphalan for locally advanced soft tissue sarcoma : The value of adjuvant radiotherapy

    NARCIS (Netherlands)

    Thijssens, KMJ; van Ginkel, RJ; Pras, E; Suurmeijer, AJH; Hoekstra, HJ

    Background: The aim was to investigate the value of adjuvant radiotherapy for locally advanced soft tissue sarcoma after hyperthermic isolated limb perfusion (ILP) with tumor necrosis factor alpha and melphalan followed by limb-saving surgery. Methods: From 1991 to 2003, 73 patients (median age, 54

  20. Histologic Appearance After Preoperative Radiation Therapy for Soft Tissue Sarcoma: Assessment of the European Organization for Research and Treatment of Cancer–Soft Tissue and Bone Sarcoma Group Response Score

    International Nuclear Information System (INIS)

    Schaefer, Inga-Marie; Hornick, Jason L.; Barysauskas, Constance M.; Raut, Chandrajit P.; Patel, Sagar A.; Royce, Trevor J.; Fletcher, Christopher D.M.; Baldini, Elizabeth H.

    2017-01-01

    Purpose: To critically assess the prognostic value of the European Organization for Research and Treatment of Cancer–Soft Tissue and Bone Sarcoma Group (EORTC-STBSG) response score and define histologic appearance after preoperative radiation therapy (RT) for soft tissue sarcoma (STS). Methods and Materials: For a cohort of 100 patients with STS of the extremity/trunk treated at our institution with preoperative RT followed by resection, 2 expert sarcoma pathologists evaluated the resected specimens for percent residual viable cells, necrosis, hyalinization/fibrosis, and infarction. The EORTC response score and other predictors of recurrence-free survival (RFS) and overall survival (OS) were assessed by Kaplan-Meier and proportional hazard models. Results: Median tumor size was 7.5 cm; 92% were intermediate or high grade. Most common histologies were unclassified sarcoma (34%) and myxofibrosarcoma (25%). Median follow-up was 60 months. The 5-year local recurrence rate was 5%, 5-year RFS was 68%, and 5-year OS was 75%. Distribution of cases according to EORTC response score tiers was as follows: no residual viable tumor for 9 cases (9% pathologic complete response); <1% viable tumor for 0, ≥1% to <10% for 9, ≥10% to <50% for 44, and ≥50% for 38. There was no association between EORTC-STBSG response score and RFS or OS. Conversely, hyalinization/fibrosis was a significant independent favorable predictor for RFS (hazard ratio 0.49, P=.007) and OS (hazard ratio 0.36, P=.02). Conclusion: Histologic evaluation after preoperative RT for STS showed a 9% pathologic complete response rate. The EORTC-STBSG response score and percent viable cells were not prognostic. Hyalinization/fibrosis was associated with favorable outcome, and if validated, may become a valid endpoint for neoadjuvant trials.

  1. Histologic Appearance After Preoperative Radiation Therapy for Soft Tissue Sarcoma: Assessment of the European Organization for Research and Treatment of Cancer–Soft Tissue and Bone Sarcoma Group Response Score

    Energy Technology Data Exchange (ETDEWEB)

    Schaefer, Inga-Marie; Hornick, Jason L. [Department of Pathology, Brigham and Women' s Hospital, Harvard Medical School, Boston, Massachusetts (United States); Barysauskas, Constance M. [Department of Biostatistics and Computational Biology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, Massachusetts (United States); Raut, Chandrajit P. [Division of Surgical Oncology, Brigham and Women' s Hospital, Harvard Medical School, Boston, Massachusetts (United States); Center for Sarcoma and Bone Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, Massachusetts (United States); Patel, Sagar A.; Royce, Trevor J. [Department of Radiation Oncology, Brigham and Women' s Hospital, Harvard Medical School, Boston, Massachusetts (United States); Fletcher, Christopher D.M. [Department of Pathology, Brigham and Women' s Hospital, Harvard Medical School, Boston, Massachusetts (United States); Baldini, Elizabeth H., E-mail: ebaldini@partners.org [Center for Sarcoma and Bone Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, Massachusetts (United States); Department of Radiation Oncology, Brigham and Women' s Hospital, Harvard Medical School, Boston, Massachusetts (United States)

    2017-06-01

    Purpose: To critically assess the prognostic value of the European Organization for Research and Treatment of Cancer–Soft Tissue and Bone Sarcoma Group (EORTC-STBSG) response score and define histologic appearance after preoperative radiation therapy (RT) for soft tissue sarcoma (STS). Methods and Materials: For a cohort of 100 patients with STS of the extremity/trunk treated at our institution with preoperative RT followed by resection, 2 expert sarcoma pathologists evaluated the resected specimens for percent residual viable cells, necrosis, hyalinization/fibrosis, and infarction. The EORTC response score and other predictors of recurrence-free survival (RFS) and overall survival (OS) were assessed by Kaplan-Meier and proportional hazard models. Results: Median tumor size was 7.5 cm; 92% were intermediate or high grade. Most common histologies were unclassified sarcoma (34%) and myxofibrosarcoma (25%). Median follow-up was 60 months. The 5-year local recurrence rate was 5%, 5-year RFS was 68%, and 5-year OS was 75%. Distribution of cases according to EORTC response score tiers was as follows: no residual viable tumor for 9 cases (9% pathologic complete response); <1% viable tumor for 0, ≥1% to <10% for 9, ≥10% to <50% for 44, and ≥50% for 38. There was no association between EORTC-STBSG response score and RFS or OS. Conversely, hyalinization/fibrosis was a significant independent favorable predictor for RFS (hazard ratio 0.49, P=.007) and OS (hazard ratio 0.36, P=.02). Conclusion: Histologic evaluation after preoperative RT for STS showed a 9% pathologic complete response rate. The EORTC-STBSG response score and percent viable cells were not prognostic. Hyalinization/fibrosis was associated with favorable outcome, and if validated, may become a valid endpoint for neoadjuvant trials.

  2. Histologic Appearance After Preoperative Radiation Therapy for Soft Tissue Sarcoma: Assessment of the European Organization for Research and Treatment of Cancer-Soft Tissue and Bone Sarcoma Group Response Score.

    Science.gov (United States)

    Schaefer, Inga-Marie; Hornick, Jason L; Barysauskas, Constance M; Raut, Chandrajit P; Patel, Sagar A; Royce, Trevor J; Fletcher, Christopher D M; Baldini, Elizabeth H

    2017-06-01

    To critically assess the prognostic value of the European Organization for Research and Treatment of Cancer-Soft Tissue and Bone Sarcoma Group (EORTC-STBSG) response score and define histologic appearance after preoperative radiation therapy (RT) for soft tissue sarcoma (STS). For a cohort of 100 patients with STS of the extremity/trunk treated at our institution with preoperative RT followed by resection, 2 expert sarcoma pathologists evaluated the resected specimens for percent residual viable cells, necrosis, hyalinization/fibrosis, and infarction. The EORTC response score and other predictors of recurrence-free survival (RFS) and overall survival (OS) were assessed by Kaplan-Meier and proportional hazard models. Median tumor size was 7.5 cm; 92% were intermediate or high grade. Most common histologies were unclassified sarcoma (34%) and myxofibrosarcoma (25%). Median follow-up was 60 months. The 5-year local recurrence rate was 5%, 5-year RFS was 68%, and 5-year OS was 75%. Distribution of cases according to EORTC response score tiers was as follows: no residual viable tumor for 9 cases (9% pathologic complete response); <1% viable tumor for 0, ≥1% to <10% for 9, ≥10% to <50% for 44, and ≥50% for 38. There was no association between EORTC-STBSG response score and RFS or OS. Conversely, hyalinization/fibrosis was a significant independent favorable predictor for RFS (hazard ratio 0.49, P=.007) and OS (hazard ratio 0.36, P=.02). Histologic evaluation after preoperative RT for STS showed a 9% pathologic complete response rate. The EORTC-STBSG response score and percent viable cells were not prognostic. Hyalinization/fibrosis was associated with favorable outcome, and if validated, may become a valid endpoint for neoadjuvant trials. Copyright © 2017 Elsevier Inc. All rights reserved.

  3. Soft tissue sarcoma: how can posttreatment alterations be distinguished from recurrences?; Weichteilsarkome: Wie lassen sich posttherapeutische Veraenderungen von Rezidiven unterscheiden

    Energy Technology Data Exchange (ETDEWEB)

    Noebauer-Huhmann, I.M. [Universitaetsklinik fuer Radiologie und Nuklearmedizin, Abteilung fuer Neuroradiologie und Muskuloskeletale Radiologie, Wien (Austria); Grieser, T. [Klinikum Augsburg, Klinik fuer Diagnostische und Interventionelle Radiologie und Neuroradiologie, Augsburg (Germany)

    2017-11-15

    The recognition of recurrent soft tissue sarcomas and the differentiation from post-treatment alterations is complex. This article aims to assist the clinical radiologist in the systematic evaluation of local follow-up imaging in soft tissue sarcoma patients. Soft tissue sarcomas encompass multiple entities with different recurrence rates and follow-up intervals. Approved and up to date recommendations are provided, including imaging techniques. The past medical history of the patient, the clinical situation and previous therapies should be known in detail, including surgery, radiation therapy and chemotherapy. Previous imaging results should be consulted, if available. This article describes the time-dependent imaging spectrum of local post-therapeutic as well as local treatment-related complications. These include early complications, such as seromas, hematomas and infections, as well as late complications, including edema, fibrosis and joint stiffness, and also inflammatory pseudotumors, which may occur after variable time intervals. The imaging appearance of local recurrent and radiation-associated sarcoma are elucidated. In particular, magnetic resonance imaging (MRI) criteria are provided, which may help in differentiating post-therapeutic alterations from recurrent soft tissue sarcomas. (orig.) [German] Die Erkennung von Weichteilsarkomrezidiven und ihre Differenzierung von posttherapeutischen Veraenderungen sind komplex. Der Beitrag soll dem klinisch taetigen Radiologen helfen, lokale Nachkontrollen systematisch zu beurteilen. Voraussetzungen sind einerseits die genaue Kenntnis der klinischen und therapeutischen Anamnese und der aktuellen klinischen Situation sowie andererseits eine adaequate Untersuchungstechnik, fuer die Empfehlungen gegeben werden, und ein Vergleich mit der initialen Bildgebung. Der Beitrag gibt einen Ueberblick ueber Rezidivhaeufigkeiten und Therapiemodalitaeten wie der Operation, Bestrahlung und Chemotherapie. Er beschreibt das MR

  4. Wound Complications in Preoperatively Irradiated Soft-Tissue Sarcomas of the Extremities

    International Nuclear Information System (INIS)

    Rosenberg, Lewis A.; Esther, Robert J.; Erfanian, Kamil; Green, Rebecca; Kim, Hong Jin; Sweeting, Raeshell; Tepper, Joel E.

    2013-01-01

    Purpose: To determine whether the involvement of plastic surgery and the use of vascularized tissue flaps reduces the frequency of major wound complications after radiation therapy for soft-tissue sarcomas (STS) of the extremities. Methods and Materials: This retrospective study evaluated patients with STS of the extremities who underwent radiation therapy before surgery. Major complications were defined as secondary operations with anesthesia, seroma/hematoma aspirations, readmission for wound complications, or persistent deep packing. Results: Between 1996 and 2010, 73 patients with extremity STS were preoperatively irradiated. Major wound complications occurred in 32% and secondary operations in 16% of patients. Plastic surgery closed 63% of the wounds, and vascularized tissue flaps were used in 22% of closures. When plastic surgery performed closure the frequency of secondary operations trended lower (11% vs 26%; P=.093), but the frequency of major wound complications was not different (28% vs 38%; P=.43). The use of a vascularized tissue flap seemed to have no effect on the frequency of complications. The occurrence of a major wound complication did not affect disease recurrence or survival. For all patients, 3-year local control was 94%, and overall survival was 72%. Conclusions: The rates of wound complications and secondary operations in this study were very similar to previously published results. We were not able to demonstrate a significant relationship between the involvement of plastic surgery and the rate of wound complications, although there was a trend toward reduced secondary operations when plastic surgery was involved in the initial operation. Wound complications were manageable and did not compromise outcomes.

  5. Wound Complications in Preoperatively Irradiated Soft-Tissue Sarcomas of the Extremities

    Energy Technology Data Exchange (ETDEWEB)

    Rosenberg, Lewis A. [Department of Radiation Oncology, University of North Carolina, Chapel Hill, North Carolina (United States); Esther, Robert J. [Department of Orthopedics, University of North Carolina, Chapel Hill, North Carolina (United States); Erfanian, Kamil [Department of Surgery, University of North Carolina, Chapel Hill, North Carolina (United States); Green, Rebecca [Department of Radiation Oncology, University of North Carolina, Chapel Hill, North Carolina (United States); Kim, Hong Jin; Sweeting, Raeshell [Department of Surgery, University of North Carolina, Chapel Hill, North Carolina (United States); Tepper, Joel E., E-mail: tepper@med.unc.edu [Department of Radiation Oncology, University of North Carolina, Chapel Hill, North Carolina (United States)

    2013-02-01

    Purpose: To determine whether the involvement of plastic surgery and the use of vascularized tissue flaps reduces the frequency of major wound complications after radiation therapy for soft-tissue sarcomas (STS) of the extremities. Methods and Materials: This retrospective study evaluated patients with STS of the extremities who underwent radiation therapy before surgery. Major complications were defined as secondary operations with anesthesia, seroma/hematoma aspirations, readmission for wound complications, or persistent deep packing. Results: Between 1996 and 2010, 73 patients with extremity STS were preoperatively irradiated. Major wound complications occurred in 32% and secondary operations in 16% of patients. Plastic surgery closed 63% of the wounds, and vascularized tissue flaps were used in 22% of closures. When plastic surgery performed closure the frequency of secondary operations trended lower (11% vs 26%; P=.093), but the frequency of major wound complications was not different (28% vs 38%; P=.43). The use of a vascularized tissue flap seemed to have no effect on the frequency of complications. The occurrence of a major wound complication did not affect disease recurrence or survival. For all patients, 3-year local control was 94%, and overall survival was 72%. Conclusions: The rates of wound complications and secondary operations in this study were very similar to previously published results. We were not able to demonstrate a significant relationship between the involvement of plastic surgery and the rate of wound complications, although there was a trend toward reduced secondary operations when plastic surgery was involved in the initial operation. Wound complications were manageable and did not compromise outcomes.

  6. Treatment of soft-tissue sarcomas of children through brachytherapy

    International Nuclear Information System (INIS)

    Ladeia, F.T.; Novaes, P.E.R.S.; Pereira, A.J.; Peres, O.; Camargo, B.; Bianchi, A.

    1988-01-01

    Twelve children were treated from January 1979 to June 1986 and the age range was three months to 14 years. Ten patients had implanted sources in the tumour tissue and two had a surface radioactive applicator. Eleven children had local control of disease, four with long term survival (longer than 50 months), good cosmetic and functional results and seven with shorter follow-up (minimal 17 months). Only one local relapse occurred in the irradiated area, five months after treatment. Brachytherapy may be an useful modality of treatment in pediatric oncology making possible the reduction of external therapy dose, minimizing the late effects of treatment, with better survival. (author)

  7. Preclinical evaluation of oncolytic vaccinia virus for therapy of canine soft tissue sarcoma.

    Directory of Open Access Journals (Sweden)

    Ivaylo Gentschev

    Full Text Available Virotherapy using oncolytic vaccinia virus (VACV strains is one promising new strategy for canine cancer therapy. In this study we describe the establishment of an in vivo model of canine soft tissue sarcoma (CSTS using the new isolated cell line STSA-1 and the analysis of the virus-mediated oncolytic and immunological effects of two different Lister VACV LIVP1.1.1 and GLV-1h68 strains against CSTS. Cell culture data demonstrated that both tested VACV strains efficiently infected and destroyed cells of the canine soft tissue sarcoma line STSA-1. In addition, in our new canine sarcoma tumor xenograft mouse model, systemic administration of LIVP1.1.1 or GLV-1h68 viruses led to significant inhibition of tumor growth compared to control mice. Furthermore, LIVP1.1.1 mediated therapy resulted in almost complete tumor regression and resulted in long-term survival of sarcoma-bearing mice. The replication of the tested VACV strains in tumor tissues led to strong oncolytic effects accompanied by an intense intratumoral infiltration of host immune cells, mainly neutrophils. These findings suggest that the direct viral oncolysis of tumor cells and the virus-dependent activation of tumor-associated host immune cells could be crucial parts of anti-tumor mechanism in STSA-1 xenografts. In summary, the data showed that both tested vaccinia virus strains and especially LIVP1.1.1 have great potential for effective treatment of CSTS.

  8. Doxorubicin alone versus intensified doxorubicin plus ifosfamide for first-line treatment of advanced or metastatic soft-tissue sarcoma

    DEFF Research Database (Denmark)

    Judson, Ian; Verweij, Jaap; Gelderblom, Hans

    2014-01-01

    BACKGROUND: Effective targeted treatment is unavailable for most sarcomas and doxorubicin and ifosfamide-which have been used to treat soft-tissue sarcoma for more than 30 years-still have an important role. Whether doxorubicin alone or the combination of doxorubicin and ifosfamide should be used...

  9. LONG-TERM TREATMENT RESULTS OF BONE SARCOMA PATIENTS WITH CONSIDERATION OF SERUM METALLOPROTEINASE LEVELS

    Directory of Open Access Journals (Sweden)

    I. V. Babkina

    2015-01-01

    Full Text Available Background: Bone sarcomas are extremely malignant prone to rapid hematogenic metastasing. Evaluation of biological marker expression by the tumor is important not only for the search of new potential chemotherapy targets, but for the assessment of the disease prognosis.Aim: A comparative evaluation of matrix metalloproteinases (MMP-2, -7, -9 and tissue inhibitor of metalloproteinase-1 (TIMP-1 in the serum of patients with primary bone tumors and in healthy people to identify their potential association with the histological characteristics of the tumor and the disease prognosis.Materials and methods: A comparative study of serum MMP-2, -7, -9, and TIMP-1 levels was performed in 54 patients with primary bone tumors (malignant, 45 patients, including central osteosarcoma in 21, periosteal osteosarcoma in 4, Ewing's sarcoma in 11, primary chondrosarcoma in 6, undifferentiated pleomorphic sarcoma in 3, and borderline giant cell tumors in 9 and in 26 healthy individuals with the use of the immunoenzyme technique (Biosource, USA, for TIMP-1 and R&D, USA, for MMP-2, -7, and -9. Results: The TIMP-1 levels in the serum of patients with central and periosteal osteosarcomas were significantly higher than in the serum of healthy controls (р = 0.038 and p = 0.007, respectively. The MMP-9 levels in patients with bone malignancies were significantly lower than that in the normal controls (p < 0.05. There was a positive correlation between serum TIMP-1 and MMP-9 levels in patients with central, periosteal and Ewing's sarcomas (r = 0.37, p = 0.024. No significant differences in the 5-year survival rates related to serum TIMP-1, MMP-2, -7, -9 levels were found in patients with bone sarcomas. However, in those with osteosarcoma and serum MMP-2 > 160 ng/ml, the overall 5-year survival rate was 1.6-fold higher than in those with lower MMP-2 levels, and in those with ММP-9 levels < 377 ng/ml, the 5-year survival rate was 1.4-fold higher than in patients with

  10. Efficacy and safety of pharmacological interventions in second- or later-line treatment of patients with advanced soft tissue sarcoma: a systematic review

    International Nuclear Information System (INIS)

    Sharma, Sheetal; Takyar, Shweta; Manson, Stephanie C; Powell, Sarah; Penel, Nicolas

    2013-01-01

    Current guidelines recommend anthracycline-based chemotherapy primarily with doxorubicin either as monotherapy or in combination with ifosfamide as the first-line treatment for most advanced STS subtypes. Therapeutic options after failure of doxorubicin and/or ifosfamide are limited. This study aimed to comprehensively review available data on the activity and safety of interventions in second- or later-line treatment of advanced STS. Electronic literature databases (Embase ® , MEDLINE ® , MEDLINE ® In-Process, Cochrane Central Register of Controlled Trials, and Cochrane Database of Systematic Reviews) were searched from 1980 to 01 March 2012 to identify randomised controlled trials (RCTs) and non-randomised studies (both prospective and retrospective) evaluating pharmacological interventions in patients with advanced STS pre-treated with anthracycline- and/or ifosfamide-based therapy. The review identified six RCTs (one phase III and five phase II trials) and 94 non-randomised studies. Based on the primary trial endpoints, RCTs demonstrated favourable efficacy for pazopanib over placebo (PFS: 4.6 months vs. 1.6 months), gemcitabine plus dacarbazine over dacarbazine monotherapy (3-month PFS rate: 54.2% vs. 35.2%), and trabectedin 3-weekly schedule over weekly schedule (TTP: 3.7 months vs. 2.3 months. The non-randomised studies demonstrated heterogeneity in efficacy and safety results. Across the RCTs, pazopanib over placebo, gemcitabine-dacarbazine over dacarbazine, and trabectedin 3-weekly over weekly regimen clearly demonstrated a PFS advantage in the second- and later-line treatment of advanced STS. With only one phase III trial in this setting, there is a clear need for additional comparative trials to better understand the risk: benefit ratios of available agents and combinations

  11. Prognostic factors in adult soft-tissue sarcomas of the head and neck

    International Nuclear Information System (INIS)

    Le, Quynh-Thu X.; Fu, Karen K.; Kroll, Stew; Fitts, Linda; Massullo, Vincent; Ferrell, Linda; Kaplan, Michael J.; Phillips, Theodore L.

    1997-01-01

    Purpose: The main objectives of this study were (a) to review the treatment results of primary head and neck soft-tissue sarcoma at our institution, (b) to identify important prognostic factors in local control and survival, and (c) to assess the efficacy of salvage therapy. Methods and Materials: Sixty-five patients were treated at the University of California, San Francisco, between 1961 and 1993. Seventeen patients (27%) had low-grade, 10 (15%) had intermediate-grade, and 38 (58%) had high-grade sarcomas. Tumors were > 5 cm in 35 patients. Local management consisted of surgery alone in 14 patients (22%), surgery and radiotherapy in 40 (61%), and radiotherapy alone in 11 (17%) patients. The median follow-up was 64 months. Results: The 5-year actuarial local control rate of the entire group was 66%. Tumor size and grade were important predictors for local control on multivariate analysis. The actuarial local control rate at 5 years was 92% for T1 vs. 40% for T2 primaries (p = 0.004), and 80% for Grade 1-2 vs. 48% for Grade 3 tumors (p 0.01). None of the patients treated with radiotherapy alone with a dose of 50-65 Gy were controlled locally. Combined radiotherapy and surgery appeared to yield superior local control compared to surgery alone (77% vs. 59%); however, the difference was not statistically significant. The 5-year actuarial overall and cause-specific survivals were 56% and 60%, respectively. Unfavorable prognostic factors for cause-specific survival on multivariate analysis were age > 55 (p = 0.009), high tumor grade (p 0.0002), inadequate surgery (p = 0.008), and positive surgical margins (p 0.0009). In patients who underwent salvage therapy for treatment failure, the 5-year actuarial survival after salvage treatment was 26%. Conclusion: Tumor size and grade were important predictors for local control. Age, grade, adequacy of surgery, and status of surgical margins were significant prognostic factors for survival. There was a trend of improved local

  12. Identification of heterogeneity among soft tissue sarcomas by gene expression profiles from different tumors

    Directory of Open Access Journals (Sweden)

    Skubitz Amy PN

    2008-05-01

    Full Text Available Abstract The heterogeneity that soft tissue sarcomas (STS exhibit in their clinical behavior, even within histological subtypes, complicates patient care. Histological appearance is determined by gene expression. Morphologic features are generally good predictors of biologic behavior, however, metastatic propensity, tumor growth, and response to chemotherapy may be determined by gene expression patterns that do not correlate well with morphology. One approach to identify heterogeneity is to search for genetic markers that correlate with differences in tumor behavior. Alternatively, subsets may be identified based on gene expression patterns alone, independent of knowledge of clinical outcome. We have reported gene expression patterns that distinguish two subgroups of clear cell renal carcinoma (ccRCC, and other gene expression patterns that distinguish heterogeneity of serous ovarian carcinoma (OVCA and aggressive fibromatosis (AF. In this study, gene expression in 53 samples of STS and AF [including 16 malignant fibrous histiocytoma (MFH, 9 leiomyosarcoma, 12 liposarcoma, 4 synovial sarcoma, and 12 samples of AF] was determined at Gene Logic Inc. (Gaithersburg, MD using Affymetrix GeneChip® U_133 arrays containing approximately 40,000 genes/ESTs. Gene expression analysis was performed with the Gene Logic Genesis Enterprise System® Software and Expressionist software. Hierarchical clustering of the STS using our three previously reported gene sets, each generated subgroups within the STS that for some subtypes correlated with histology, and also suggested the existence of subsets of MFH. All three gene sets also recognized the same two subsets of the fibromatosis samples that we had found in our earlier study of AF. These results suggest that these subgroups may have biological significance, and that these gene sets may be useful for sub-classification of STS. In addition, several genes that are targets of some anti-tumor drugs were found to

  13. Ewing`s Sarcoma

    Directory of Open Access Journals (Sweden)

    Agnieszka Budny

    2017-06-01

    Full Text Available Ewing's sarcoma is a small round-cell tumor typically arising in the bones, rarely in soft tissues, of children and adolescents. Clinical presentation is usually dominated by local bone pain and a mass. Magnetic resonance best defines the extent of the lesion. Patients diagnosed with Ewing's sarcoma within  last years show a improving  survival rate . Rehabilitation seems to be a crucial part of multimodal therapy.

  14. Radiation Therapy for Control of Soft-Tissue Sarcomas Resected With Positive Margins

    International Nuclear Information System (INIS)

    DeLaney, Thomas F.; Kepka, Lucyna; Goldberg, Saveli I.; Hornicek, Francis J.; Gebhardt, Mark C.; Yoon, Sam S.; Springfield, Dempsey S.; Raskin, Kevin A.; Harmon, David C.; Kirsch, David G.; Mankin, Henry J.; Rosenberg, Andrew E.; Nielsen, G. Petur; Suit, Herman D.

    2007-01-01

    Purpose: Positive margins (PM) remain after surgery in some soft-tissue sarcoma (STS) patients. We investigated the efficacy of radiation therapy (RT) in STS patients with PM. Methods and Materials: A retrospective chart review was performed on 154 patients with STS at various anatomic sites with PM, defined as tumor on ink, who underwent RT with curative intent between 1970 and 2001. Local control (LC), disease-free survival (DFS), and overall survival (OS) rates were evaluated by univariate (log-rank) and multivariate analysis of prognostic and treatment factors. Results: At 5 years, actuarial LC, DFS, and OS rates were: 76%, 46.7%, and 65.2%, respectively. LC was highest with extremity lesions (p 64 Gy (p 64 Gy had higher 5-year LC, DFS, and OS rates of 85%, 52.1%, and 67.8% vs. 66.1%, 41.8%, and 62.9% if ≤64 Gy, p 50), also significantly influenced OS. By multivariate analysis, the best predictors of LC were site (extremity vs. other), p 64 vs. ≤64 Gy), p 64 Gy, superficial location, and extremity site are associated with improved LC. OS is worse in patients with tumors with lesions >5 cm, grossly positive margins, and after local failure

  15. Expression of Kirsten murine sarcoma virus sequences in Beagle dog tissues

    Energy Technology Data Exchange (ETDEWEB)

    Kerkof, P R; Kelly, G

    1988-12-01

    Labeled cDNA synthesized from RNA extracted from {sup 238}PuO{sub 2}-, {sup 239}PuO{sub 2}-, and {sup 90}Sr-induced lung tumors in Beagle dogs, from nontumor tissue from {sup 239}PuO{sub 2}-exposed dogs, and from unexposed dog lung and liver tissue produces strong hybridization signals with a plasmid (pKSma) that contains Kirsten murine sarcoma virus (KMSV) sequences. At least 90 percent of the KMSV sequences are expressed in these dog tissues, including sequences corresponding to p21 K-ras, qp70 envelope glycoprotein, and at least one other proviral sequence. The expression of Kirsten ras and other sarcoma virus sequences may have important implications for the interpretation of carcinogenesis studies in these dogs. (author)

  16. Expression of Kirsten murine sarcoma virus sequences in Beagle dog tissues

    International Nuclear Information System (INIS)

    Kerkof, P.R.; Kelly, G.

    1988-01-01

    Labeled cDNA synthesized from RNA extracted from 238 PuO 2 -, 239 PuO 2 -, and 90 Sr-induced lung tumors in Beagle dogs, from nontumor tissue from 239 PuO 2 -exposed dogs, and from unexposed dog lung and liver tissue produces strong hybridization signals with a plasmid (pKSma) that contains Kirsten murine sarcoma virus (KMSV) sequences. At least 90 percent of the KMSV sequences are expressed in these dog tissues, including sequences corresponding to p21 K-ras, qp70 envelope glycoprotein, and at least one other proviral sequence. The expression of Kirsten ras and other sarcoma virus sequences may have important implications for the interpretation of carcinogenesis studies in these dogs. (author)

  17. Genetic susceptibility to bone and soft tissue sarcomas: a field synopsis and meta-analysis.

    Science.gov (United States)

    Benna, Clara; Simioni, Andrea; Pasquali, Sandro; De Boni, Davide; Rajendran, Senthilkumar; Spiro, Giovanna; Colombo, Chiara; Virgone, Calogero; DuBois, Steven G; Gronchi, Alessandro; Rossi, Carlo Riccardo; Mocellin, Simone

    2018-04-06

    The genetic architecture of bone and soft tissue sarcomas susceptibility is yet to be elucidated. We aimed to comprehensively collect and meta-analyze the current knowledge on genetic susceptibility in these rare tumors. We conducted a systematic review and meta-analysis of the evidence on the association between DNA variation and risk of developing sarcomas through searching PubMed, The Cochrane Library, Scopus and Web of Science databases. To evaluate result credibility, summary evidence was graded according to the Venice criteria and false positive report probability (FPRP) was calculated to further validate result noteworthiness. Integrative analysis of genetic and eQTL (expression quantitative trait locus) data was coupled with network and pathway analysis to explore the hypothesis that specific cell functions are involved in sarcoma predisposition. We retrieved 90 eligible studies comprising 47,796 subjects (cases: 14,358, 30%) and investigating 1,126 polymorphisms involving 320 distinct genes. Meta-analysis identified 55 single nucleotide polymorphisms (SNPs) significantly associated with disease risk with a high (N=9), moderate (N=38) and low (N=8) level of evidence, findings being classified as noteworthy basically only when the level of evidence was high. The estimated joint population attributable risk for three independent SNPs (rs11599754 of ZNF365/EGR2 , rs231775 of CTLA4 , and rs454006 of PRKCG ) was 37.2%. We also identified 53 SNPs significantly associated with sarcoma risk based on single studies.Pathway analysis enabled us to propose that sarcoma predisposition might be linked especially to germline variation of genes whose products are involved in the function of the DNA repair machinery. We built the first knowledgebase on the evidence linking DNA variation to sarcomas susceptibility, which can be used to generate mechanistic hypotheses and inform future studies in this field of oncology.

  18. Soft Tissue Sarcoma: A Prognostic Variables and Treatment Outcomes after Chemo - Radiotherapy and Conservative Surgery

    International Nuclear Information System (INIS)

    Abaza, A.; El-Shanshoury, H.

    2015-01-01

    Soft tissue sarcomas (STS) constitute a group of rare and heterogeneous cancers, that diverse a wide spectrum of histology and varied clinical behavior. The aim was to study, retrospectively the clinico-epidemiologic manifestation, response to therapy and prognostic factors of retroperitoneal (RPS), extremity and other STS patients treated with conservative surgery plus chemo - radiotherapy (CRT) attending the Pediatric Oncology Clinic, National Cancer Institute (NCI), Cairo University during 6 years period (inclusive). Files of 66 STS patients were revised for history, staging, investigations, treatment modalities and outcome of therapy. Their ages ranged from 6 months to 21 years. The male to female ratio was 1.87:1. Most of the patients belong to stage 3 and 1. Radiotherapy (RTH) and surgery remains the backbone of the multi - modality treatment plan. Complete remission (CR) was obtained in 80.3% of STS patients. Meanwhile, 6% of cases had disease progression and 6% died. The 2 - years overall survival rate (OS) and Disease - free survivals (DFS) we re 74.2% and 57.6% respectively. However, 20.8% of patients develop relapse>2 years after the end of treatment. Age at diagnosis, stage of the disease, tumor mass, pathological subtype and the RTH used were statistically significant prognostic variables affecting survival. Finally, the use of RTH in the treatment of STS gives positive impact on the survival of patients. Recommendation regarding further studies would be required to identify methods of increasing the radiation dose selectively. Also, using the newly developed radiation technologies to make dose escalation and sparing normal tissue

  19. Retroperitoneal soft tissue sarcoma: An analysis of radiation and surgical treatment

    International Nuclear Information System (INIS)

    Ballo, Matthew T.; Zagars, Gunar K.; Pollock, Raphael E.; Benjamin, Robert S.; Feig, Barry W.; Cormier, Janice N.; Hunt, Kelly K.; Patel, Shreyaskumar R.; Trent, Jonathan C.; Beddar, Sam; Pisters, Peter W.T.

    2007-01-01

    Purpose: To evaluate the clinical outcomes of patients with localized retroperitoneal soft tissue sarcoma (STS) treated with complete surgical resection and radiation. Methods and Materials: The medical records of 83 patients were reviewed retrospectively. Sixty patients presented with primary disease and the remaining 23 had recurrence after previous surgical resection. Results: With a median follow-up of 47 months, the actuarial overall disease-specific survival (DSS), distant metastasis-free survival, and local control (LC) rates were 44%, 67%, and 40%, respectively. Of the 38 patients dying of disease, local disease progression was the sole site of recurrence for 16 patients and was a component of progression for another 11 patients. Multivariate analysis indicated that histologic grade was associated with the 5-year rates of DSS (low-grade, 92%; intermediate-grade, 51%; and high-grade, 41%, p = 0.006). Multivariate analysis also indicated an inferior 5-year LC rate for patients presenting with recurrent disease, positive or uncertain resection margins, and age greater than 65 years. The data did not suggest an improved local control with higher doses of external-beam radiation (EBRT) or with the specific use of intraoperative radiotherapy (IORT). Radiation-related complications (10% at 5 years) developed in 5 patients; all had received their EBRT postoperatively. Conclusions: Although preoperative radiation therapy and aggressive surgical resection is well tolerated in patients, local disease progression continues to be a significant component of disease death. In this small cohort of patients, the use of higher doses of EBRT or IORT did not result in clinically apparent improvements in outcomes

  20. Management of locally recurrent soft-tissue sarcoma after prior surgery and radiation therapy

    International Nuclear Information System (INIS)

    Torres, Mylin A.; Ballo, Matthew T.; Butler, Charles E.; Feig, Barry W.; Cormier, Janice N.; Lewis, Valerae O.; Pollock, Raphael E.; Pisters, Peter W.; Zagars, Gunar K.

    2007-01-01

    Purpose: The aim of this study was to evaluate outcome and treatment toxicity after wide local re-excision (WLE), with or without additional radiation therapy, for patients with isolated first local recurrence of soft-tissue sarcoma arising within a previously irradiated field. Methods: A retrospective review was performed of 62 consecutive patients. All patients underwent prior resection and external beam radiation. For recurrent disease, 25 patients were treated with WLE alone, and 37 patients were treated with WLE and additional radiation (45- 64 Gy). In 33 patients, the radiation was delivered via an afterloaded brachytherapy, single-plane implant. Results: The 5-year disease specific and distant metastasis-free survival rates were 65% and 73%, respectively. Local control (LC) at 5 years was 51%, and on multivariate analysis, a positive surgical resection margin (p< 0.001) was associated with a lower rate of LC. Reirradiation was not associated with improved LC; however complications requiring outpatient or surgical management were more common in patients who had undergone reirradiation (80% vs. 17%, p < 0.001). Amputation was also more common in the subgroup of patients who underwent extremity reirradiation (35% with radiation vs. 11% without, p = 0.05), although only one amputation was performed to resolve a treatment complication. Conclusion: Conservative surgery alone results in LC in a minority of patients who have failed locally after previous excision and external beam radiation. Although selection biases and small patient numbers confound the analysis, local treatment intensification with additional radiation does not clearly improve outcome after surgical excision alone, and is associated with an increase in complications

  1. Radiotherapy of epidemic Kaposi's sarcoma in patients with AIDS

    International Nuclear Information System (INIS)

    Westermann, V.A.; Mueller, R.P.; Adler, M.; Bendick, C.; Rasokat, H.

    1990-01-01

    From August 1986 to May 1989, 15 patients suffering from Kaposi's sarcoma and serologically proven HIV infections were treated in the Department of Radiotherapy, University of Cologne, Medical Hospital. All patients were male and homosexual. Therapeutic objectives were palliation of pain and functional impairment as well as elimination of the cosmetically disturbing Kaposi's sarcoma. 68 localizations (facial skin, torso, extremities, sole of the foot, penis, oral mucosa and oropharynx) were irradiated. Depending on the individual therapy regimen, photons or high-energy electrons up to a total dose of 26 to 40 Gy, with single doses of 1.8 to 2.5 Gy were applied four to five times a week. In 66% of the cases, complete remission was achieved within the area of irradiation at the dermal or mucosal level, with at most a discrete residual pigmentation of the cluster remaining. Partial remission with at least 50% regression or a distinctive residual pigmentation was achieved in 31%. In 3% of the cases, a less than 50% regression of the Kaposi's lesions were achieved after radiotherapy. There were five local recurrences. Treatment with radiation is an effective local therapy in epidemic Kaposi's sarcoma and yields good functional and cosmetic results and also provides relief from pain. (orig.) [de

  2. Dermatomyositis with Kaposi’s Sarcoma in a Patient without Human Immunodeficiency Virus-1 Infection

    Directory of Open Access Journals (Sweden)

    Dana Liang

    1991-01-01

    Full Text Available The first case of dermatomyositis complicating cutaneous and visceral Kaposi’s sarcoma is presented in a 75-year-old man without human immunodeficiency virus infection. Dermatomyositis preceded a definitive diagnosis of Kaposi’s sarcoma by six months, although in retrospect unrecognized lesions may have presented simultaneously. He was treated with prednisone and azathioprine, thus raising the possibility of the role of immunosuppression in promoting progression of the sarcoma. It is suggested that although the association between dermatomyositis and Kaposi’s sarcoma occurs rarely, dermatomyositis should be considered a paraneoplastic syndrome of Kaposi’s sarcoma. Further, the finding of cutaneous lesions of Kaposi’s sarcoma could predict gastrointestinal involvement when dermatomyositis and Kaposi’s sarcoma occur in the same patient.

  3. Near-infrared intraoperative imaging during resection of an anterior mediastinal soft tissue sarcoma.

    Science.gov (United States)

    Predina, Jarrod D; Newton, Andrew D; Desphande, Charuhas; Singhal, Sunil

    2018-01-01

    Sarcomas are rare malignancies that are generally treated with multimodal therapy protocols incorporating complete local resection, chemotherapy and radiation. Unfortunately, even with this aggressive approach, local recurrences are common. Near-infrared intraoperative imaging is a novel technology that provides real-time visual feedback that can improve identification of disease during resection. The presented study describes utilization of a near-infrared agent (indocyanine green) during resection of an anterior mediastinal sarcoma. Real-time fluorescent feedback provided visual information that helped the surgeon during tumor localization, margin assessment and dissection from mediastinal structures. This rapidly evolving technology may prove useful in patients with primary sarcomas arising from other locations or with other mediastinal neoplasms.

  4. Osteogenic sarcoma of the skull. A clinicopathologic study of 19 patients

    International Nuclear Information System (INIS)

    Huvos, A.G.; Sundaresan, N.; Bretsky, S.S.; Butler, A.

    1985-01-01

    The authors studied 19 patients with well documented osteogenic sarcomas arising in the skull, which represent 1.6% of all osteogenic sarcomas registered during a 60-year period (1921-1981). Ten sarcomas were primary, de novo tumors. Nine others developed secondary osteogenic sarcomas; among these, six arose as a complication of Paget's disease, two followed irradiation, and one was associated with pre-existent fibrous dysplasia. The sarcomas arose in equal proportion in both sexes with the men being much older (mean age, 44 years) as compared to the women (mean age, 31 years). Patients with de novo osteogenic sarcomas were considerably younger than those with secondary lesions. Osteoblastic osteogenic sarcoma was by far the most common histologic variant in both the primary and the Paget's sarcomas. None of the patients with Paget's sarcoma lived longer than 1 year; the median survival here was 4 months. Patients with de novo osteogenic sarcomas fared much better and there are four long-term survivors (longer than 3 years) who are currently disease-free

  5. Volume fractions of DCE-MRI parameter as early predictor of histologic response in soft tissue sarcoma: A feasibility study.

    Science.gov (United States)

    Xia, Wei; Yan, Zhuangzhi; Gao, Xin

    2017-10-01

    To find early predictors of histologic response in soft tissue sarcoma through volume transfer constant (K trans ) analysis based on dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI). 11 Patients with soft tissue sarcoma of the lower extremity that underwent preoperative chemoradiotherapy followed by limb salvage surgery were included in this retrospective study. For each patient, DCE-MRI data sets were collected before and two weeks after therapy initiation, and histologic tumor cell necrosis rate (TCNR) was reported at surgery. The DCE-MRI volumes were aligned by registration. Then, the aligned volumes were used to obtain the K trans variation map. Accordingly, three sub-volumes (with increased, decreased or unchanged K trans ) were defined and identified, and fractions of the sub-volumes, denoted as F + , F - and F 0 , respectively, were calculated. The predictive ability of volume fractions was determined by using area under a receiver operating characteristic curve (AUC). Linear regression analysis was performed to investigate the relationship between TCNR and volume fractions. In addition, the K trans values of the sub-volumes were compared. The AUC for F - (0.896) and F 0 (0.833) were larger than that for change of tumor longest diameter ΔD (0.625) and the change of mean K trans ΔK trans ¯ (0.792). Moreover, the regression results indicated that TCNR was directly proportional to F 0 (R 2 =0.75, P=0.0003), while it was inversely proportional to F - (R 2 =0.77, P=0.0002). However, TCNR had relatively weak linear relationship with ΔK trans ¯ (R 2 =0.64, P=0.0018). Additionally, TCNR did not have linear relationship with DD (R 2 =0.16, P=0.1246). The volume fraction F - and F 0 have potential as early predictors of soft tissue sarcoma histologic response. Copyright © 2017 Elsevier B.V. All rights reserved.

  6. Cell-surface glycoproteins of human sarcomas: differential expression in normal and malignant tissues and cultured cells

    International Nuclear Information System (INIS)

    Rettig, W.F.; Garin-Chesa, P.; Beresford, H.R.; Oettgen, H.F.; Melamed, M.R.; Old, L.J.

    1988-01-01

    Normal differentiation and malignant transformation of human cells are characterized by specific changes in surface antigen phenotype. In the present study, the authors have defined six cell-surface antigens of human sarcomas and normal mesenchymal cells, by using mixed hemadsorption assays and immunochemical methods for the analysis of cultured cells and immunohistochemical staining for the analysis of normal tissues and > 200 tumor specimens. Differential patterns of F19, F24, G171, G253, S5, and Thy-1 antigen expression were found to characterize (i) subsets of cultured sarcoma cell lines, (ii) cultured fibroblasts derived from various organs, (iii) normal resting and activated mesenchymal tissues, and (iv) sarcoma and nonmesenchymal tumor tissues. These results provide a basic surface antigenic map for cultured mesenchymal cells and mesenchymal tissues and permit the classification of human sarcomas according to their antigenic phenotypes

  7. Bone sarcomas

    International Nuclear Information System (INIS)

    Mudry, P.

    2008-01-01

    Bone sarcomas are malignancies with peak incidence in adolescents and young adults. The most frequent are osteosarcoma and Ewing sarcoma/PNET, in an older adults are seen chondrosarcomas, other ones are rare. In general, biology of sarcomas is closely related to pediatric malignancies with fast growth, local aggressiveness, tendency to early hematogenic dissemination and chemo sensitivity. Diagnostics and treatment of bone sarcomas should be done in well experienced centres due to low incidence and broad issue of this topic. An interdisciplinary approach and staff education is essential in due care of patients with bone sarcoma. If these criteria are achieved, the cure rate is contemporary at 65 - 70 %, while some subpopulation of patients has chance for cure up to 90 %. Osteosarcoma and Ewing sarcoma/PNET are discussed below as types of most frequent bone sarcoma. (author)

  8. Detection of soft-tissue sarcoma recurrence: added value of functional MR imaging techniques at 3.0 T.

    Science.gov (United States)

    Del Grande, Filippo; Subhawong, Ty; Weber, Kristy; Aro, Michael; Mugera, Charles; Fayad, Laura M

    2014-05-01

    To determine the added value of functional magnetic resonance (MR) sequences (dynamic contrast material-enhanced [DCE] and quantitative diffusion-weighted [DW] imaging with apparent diffusion coefficient [ADC] mapping) for the detection of recurrent soft-tissue sarcomas following surgical resection. This retrospective study was approved by the institutional review board. The requirement to obtain informed consent was waived. Thirty-seven patients referred for postoperative surveillance after resection of soft-tissue sarcoma (35 with high-grade sarcoma) were studied. Imaging at 3.0 T included conventional (T1-weighted, fluid-sensitive, and contrast-enhanced T1-weighted imaging) and functional (DCE MR imaging, DW imaging with ADC mapping) sequences. Recurrences were confirmed with biopsy or resection. A disease-free state was determined with at least 6 months of follow-up. Two readers independently recorded the signal and morphologic characteristics with conventional sequences, the presence or absence of arterial enhancement at DCE MR imaging, and ADCs of the surgical bed. The accuracy of conventional MR imaging in the detection of recurrence was compared with that with the addition of functional sequences. The Fisher exact and Wilcoxon rank sum tests were used to define the accuracy of imaging features, the Cohen κ and Lin interclass correlation were used to define interobserver variability, and receiver operating characteristic analysis was used to define a threshold to detect recurrence and assess reader confidence after the addition of functional imaging to conventional sequences. There were six histologically proved recurrences in 37 patients. Sensitivity and specificity of MR imaging in the detection of tumor recurrence were 100% (six of six patients) and 52% (16 of 31 patients), respectively, with conventional sequences, 100% (six of six patients) and 97% (30 of 31 patients) with the addition of DCE MR imaging, and 60% (three of five patients) and 97% (30 of

  9. Developments in the management of advanced soft-tissue sarcoma – olaratumab in context

    Directory of Open Access Journals (Sweden)

    Moroncini G

    2018-02-01

    Full Text Available Gianluca Moroncini,1,* Elena Maccaroni,2,* Ilaria Fiordoliva,2 Chiara Pellei,2 Armando Gabrielli,1 Rossana Berardi2 1Department of Clinical and Molecular Sciences, Università Politecnica delle Marche, Ancona, Italy; 2Medical Oncology Unit, Università Politecnica delle Marche, Azienda Ospedaliero-Universitaria Ospedali Riuniti Umberto I, GM Lancisi, G Salesi, Ancona, Italy *These authors contributed equally to this work Abstract: Lartruvo® (olaratumab is a fully human immunoglobulin G subclass 1 (IgG1 monoclonal antibody that inhibits platelet-derived growth factor receptor alpha (PDGFRα. The antitumor activity of olaratumab has been tested in vitro and in vivo, and inhibition of tumor growth has been observed in cancer cell lines, including glioblastoma and leiomyosarcoma cells. It represents the first-in-class antibody to be approved by regulatory authorities for the treatment of advanced soft-tissue sarcomas (STSs in combination with doxorubicin, based on the results of the Phase Ib/II trial by Tap et al. The median progression-free survival (PFS, which was the primary end point of the study, was improved for patients treated with olaratumab plus doxorubicin compared to those treated with doxorubicin monotherapy (6.6 vs 4.1 months, respectively; HR 0.672, 95% CI 0.442–1.021, p=0.0615. Moreover, final analysis of overall survival (OS showed a median OS of 26.5 months with olaratumab plus doxorubicin vs 14.7 months with doxorubicin, with a gain of 11.8 months (HR 0.46, 95% CI 0.30–0.71, p=0.0003. In October 2016, olaratumab was admitted in the Accelerated Approval Program by the US Food and Drug Administration (FDA for use in combination with doxorubicin for the treatment of adult patients with STSs. In November 2016, the European Medicines Agency (EMA granted conditional approval for olaratumab in the same indication under its Accelerated Assessment Program. A double-blind, placebo-controlled, randomized Phase III study (ANNOUNCE

  10. Procholecystokinin as marker of human Ewing sarcomas

    DEFF Research Database (Denmark)

    Reubi, Jean Claude; Koefoed, Pernille; Hansen, Thomas von O

    2004-01-01

    PURPOSE: Ewing sarcoma is a rapidly growing mesenchymal tumor in young adults. Although it was shown previously to express the cholecystokinin (CCK) gene, it is unknown whether CCK gene expression is detectable at protein level in Ewing sarcoma tumor cell lines, in tumor tissue, and in plasma from...... Ewing sarcoma patients, and, if so, whether CCK peptides might play a role as tumor markers. EXPERIMENTAL DESIGN: CCK gene expression was evaluated with in situ hybridization or reverse transcription-PCR in tumor tissue. CCK precursors and bioactive CCK were measured with specific RIAs in tumor tissue......, in cell culture medium, and in plasma of Ewing sarcoma patients before and after chemotherapy as well as after tumor recurrence. RESULTS: CCK mRNA was identified in 12 Ewing sarcoma biopsies sampled in two series and in four Ewing sarcoma cell lines but not in unrelated neoplasia. Immunoreactive pro...

  11. Safety of concurrent adjuvant radiotherapy and chemotherapy for locally advanced soft tissue sarcoma.

    Science.gov (United States)

    Greto, Daniela; Loi, Mauro; Saieva, Calogero; Muntoni, Cristina; Delli Paoli, Camilla; Becherini, Carlotta; Ciabatti, Cinzia; Perna, Marco; Campanacci, Domenico; Terziani, Francesca; Beltrami, Giovanni; Scoccianti, Guido; Bonomo, Pierluigi; Meattini, Icro; Desideri, Isacco; Simontacchi, Gabriele; Mangoni, Monica; Livi, Lorenzo

    2018-04-01

    This retrospective study analyzes the safety and feasibility of concurrent chemoradiotherapy (CRT) in adjuvant treatment of soft tissue sarcoma (STS). A total of 158 patients with STS were retrospectively analyzed. Anthracycline-based computed tomography was performed in high-risk patients. Acute radiotherapy toxicity and chemotherapy-related toxicity were assessed according to the Common Terminology Criteria for Adverse Events 4.0; late radiotherapy toxicity was recorded according to Radiation Therapy Oncology Group/European Organization for Research and Treatment of Cancer criteria. Fifty-four (34.2%) patients received CRT. Mean follow up was 5.4 years (range .2-21.1 years). Local DFS-recurrence-free survival, distant DFS-relapse-free survival, and overall survival were 79.1%, 76.4%, and 64.6%, respectively, at last follow-up. Leukopenia occurred in 11.4% of patients. Skin acute toxicity developed in 60.1% of patients and determined interruption of radiotherapy treatment in 19 (12%) patients. Nineteen patients (12%) experienced moderate fibrosis (grade 2). Mild and moderate joint stiffness was recorded in 16 (10.1%) patients. Size ≥5 cm was the only predictor of local recurrence at multivariate analysis (hazard ratio [HR] 9.65, 95% confidence interval [CI] 1.28-72.83, p = .028). Age and stage resulted as independent distant relapse predictors (HR 4.77, 95% CI 1.81-12.58, p = .002 and HR 4.83, CI 1.41-16.57, p = .012, respectively). At Cox regression univariate analysis, Karnofsky Performance Status, size, and stage were significant survival predictors (HR 2.23, 95% CI 1.02-4.87, p = .045; HR 2.88, 95% CI 1.10-7.52, p = .031; HR 2.59, 95% CI 1.11-6.04, p = .028). Concurrent CRT is a well-tolerated treatment option with no additional toxicity compared to exclusive radiotherapy or sequential CRT.

  12. Treatment Outcome Following Transarterial Chemoembolization in Advanced Bone and Soft Tissue Sarcomas

    Energy Technology Data Exchange (ETDEWEB)

    Jiang, Chunyu; Wang, Jianbo, E-mail: a602131499@163.com [Shanghai Jiaotong University Affiliated Sixth People’s Hospital, Department of Radiology (China); Wang, Yonggang [Shanghai Jiaotong University Affiliated Sixth People’s Hospital, Department of Oncology (China); Zhao, Jungong; Zhu, Yueqi; Ma, Xu; Zhou, Jia [Shanghai Jiaotong University Affiliated Sixth People’s Hospital, Department of Radiology (China); Yan, Xuebing [Shanghai Jiaotong University Affiliated Sixth People’s Hospital, Department of General Surgery (China)

    2016-10-15

    PurposeTransarterial chemoembolization (TACE) is used to treat unresectable bone and soft tissue sarcoma (STS) and as a pre-surgical adjuvant treatment. However, its efficiency for advanced STS is undetermined. This study evaluated TACE’s efficiency in treating advanced STS and prognostic factors for patient survival.Materials and MethodsWe enrolled 39 patients with unresectable STS who underwent TACE as an alternative treatment during 2010–2014, with overall survival (OS) as the primary end point. Cancer pain was evaluated by visual analogue scores (VAS) before and after TACE procedures. Factors that affect survival were evaluated by multivariate analyses (Cox proportional hazard model).ResultsMean OS after TACE was 23.7 ± 2.1 months, with 1-year OS 71.5 %, 2-year OS 45.8 %, and 3-year OS 32.5 %. Lesion number and tumor stage were key predictors of survival. TACE was found to decrease cancer pain VAS and increase relapse interval. Size of polyvinyl alcohol (PVA) particle diameter (P = 0.03) and imaging response (P = 0.044) were also found to affect relapse interval.ConclusionTACE was an effective treatment for advanced STS, with a 32.5 % 3-year OS rate, and led to lower cancer pain VAS and longer relapse intervals than chemoinfusion only. Smaller PVA particles are preferable during the TACE procedure.

  13. Tumor type resulting in upgrade: An analysis based on 333 low grade soft tissue sarcoma

    Directory of Open Access Journals (Sweden)

    Langer, Stefan

    2014-11-01

    Full Text Available [english] Introduction: Soft tissue sarcomas (STS are rare tumors. Based on histopathological criteria, three grades are distinguished from low (G1 to intermediate (G2 and high grade (G3. After complete initial surgical resection, some G1 STS recur as lesions with an upgrade of a previous G1 STS to a recurrent G2 STS. This upgrade indicates higher malignancy of the STS. Our aim was to find possible risk factors for these upgrades including age, localization of tumor and tumor type. Methods: This retrospective case-control study evaluated 333 patients. Of these 333, 54.7% were male and 45.3% female. All patients underwent R0 resections and among these, 10% subsequently upgraded. The processed data include age, gender, tumor type, tumor localization, local recurrence and upgrade. Results: Patients with upgrades have a higher mean age of 5.5 years than our reference collective. The tumor type has a significant effect on upgrades. Patients with fibrosarcomas are at a threefold risk of an upgrade compared to patients with other G1 STS.Conclusion: Our results indicate that age and tumor type play a key role in upgrades in G1 STS. Patients, age 60 and above and diagnosed with G1 fibrosarcomas, are three times as likely to upgrade compared to patients younger than 60 with other G1 STS. We discuss the significance of these risk factors and whether aside from complete tumor resection, additional therapies (e.g. irradiation may be applied to improve therapeutic outcome.

  14. Endosialin and Associated Protein Expression in Soft Tissue Sarcomas: A Potential Target for Anti-Endosialin Therapeutic Strategies

    Directory of Open Access Journals (Sweden)

    Daniel J. O’Shannessy

    2016-01-01

    Full Text Available Endosialin (CD248, TEM-1 is expressed in pericytes, tumor vasculature, tumor fibroblasts, and some tumor cells, including sarcomas, with limited normal tissue expression, and appears to play a key role in tumor-stromal interactions, including angiogenesis. Monoclonal antibodies targeting endosialin have entered clinical trials, including soft tissue sarcomas. We evaluated a cohort of 94 soft tissue sarcoma samples to assess the correlation between gene expression and protein expression by immunohistochemistry for endosialin and PDGFR-β, a reported interacting protein, across available diagnoses. Correlations between the expression of endosialin and 13 other genes of interest were also examined. Within cohorts of soft tissue diagnoses assembled by tissue type (liposarcoma, leiomyosarcoma, undifferentiated sarcoma, and other, endosialin expression was significantly correlated with a better outcome. Endosialin expression was highest in liposarcomas and lowest in leiomyosarcomas. A robust correlation between protein and gene expression data for both endosialin and PDGFR-β was observed. Endosialin expression positively correlated with PDGFR-β and heparin sulphate proteoglycan 2 and negatively correlated with carbonic anhydrase IX. Endosialin likely interacts with a network of extracellular and hypoxia activated proteins in sarcomas and other tumor types. Since expression does vary across histologic groups, endosialin may represent a selective target in soft tissue sarcomas.

  15. Sarcoma de Ewing en paciente masculino Ewing's sarcoma in a male patient

    Directory of Open Access Journals (Sweden)

    Eddy Millán Escalona

    2010-09-01

    Full Text Available El sarcoma de Ewing es un tumor óseo maligno de células redondas pequeñas. Es el segundo tumor óseo maligno más frecuente en la infancia, y se puede presentar en cualquier momento durante la niñez y comienzos de la edad adulta. El tumor puede originarse en cualquier parte del cuerpo, generalmente en los huesos largos de las extremidades, la pelvis o el tórax, al igual que en el cráneo o en los huesos planos del tronco. Se trata de un paciente masculino, mestizo, de 38 años de edad, que acude a consulta externa en el hospital de Nickerie, Suriname, por presentar aumento de volumen en la región lateral derecha del abdomen. Refiere que ha presentado pérdida de peso, decaimiento marcado y anorexia. Estos síntomas se iniciaron 3 meses antes, y se han incrementado paulatina y progresivamente. Tiene también dificultad para deambular y dolor en la pierna derecha.The Ewing's sarcoma is a malignant bone tumor of round cells. The second malignant bone tumor more frequent in childhood and may to appear in any moment during this life stage and at onset of adulthood. Tumor may to originate in any place of body, generally in limbs long bones, pelvis or thorax as well as in skull or in trunk flat bones. This case is a black male patient aged 38 seen in external consultation in the hospital of Nickerie, Surinam presenting with a volume increase in the right lateral region of abdomen. He refers a weight loss, a marked weakness and anorexia. These symptoms appeared three months before increasing in a gradual and progressively way. He has difficulty to walk and pain in the right leg.

  16. Intraoperative Radiation Therapy for Locally Advanced and Recurrent Soft-Tissue Sarcomas in Adults

    International Nuclear Information System (INIS)

    Tran, Phuoc T.; Hara, Wendy; Su Zheng; Lin, H. Jill; Bendapudi, Pavan K.; Norton, Jeffrey; Teng, Nelson; King, Christopher R.; Kapp, Daniel S.

    2008-01-01

    Purpose: To analyze the outcomes of and identify prognostic factors for patients treated with surgery and intraoperative radiotherapy (IORT) for locally advanced and recurrent soft-tissue sarcoma in adults from a single institution. Methods and Materials: We retrospectively reviewed 50 consecutive patients treated with IORT to 62 sites of disease. Primary sites included retroperitoneum-pelvis (78%), extremity (8%), and other (14%). Seventy percent of patients had recurrent disease failing prior surgery (70%) and/or radiation (32%). Mean disease-free interval (DFI) before IORT was 1.9 years (range, 2 weeks-5.4 years). The IORT was delivered with orthovoltage X-rays using individually sized beveled cone applicators. Clinical characteristics were as follows: mean tumor size, 10 cm (range, 1-25 cm); high-grade histologic subtype (72%); and mean dose, 1,159 cGy (range, 600-1,600 cGy). Postoperative radiation or chemotherapy was administered to 37% of IORT Sites and 32% of patients, respectively. Outcomes measured were infield control (IFC), locoregional control (LRC), distant metastasis-free survival (DMFS), disease-specific survival (DSS), and treatment-related complications. Mean and median follow-up of alive patients were 59 and 35 months, respectively. Results: Kaplan-Meier 5-year IFC, LRC, DMFS, and DSS probabilities for the entire group were 55%, 26%, 51%, and 25%, respectively. Prognostic factors found to be significant (p < 0.05) on multivariate analysis were prior DFI and tumor size for LRC, extremity location and leiomyosarcoma histologic subtype for DMFS, and prior DFI for DSS. Our cohort had five Grade 3/4 complications associated with treatment or a 5-year Kaplan-Meier Grade 3/4 complication-free survival rate of 85%. Conclusions: IORT after tumor reductive surgery is well tolerated and seems to confer IFC in carefully selected patients

  17. Local changes in bone marrow at MRI after treatment of extremity soft tissue sarcoma

    International Nuclear Information System (INIS)

    Hwang, Sinchun; Lefkowitz, Robert; Landa, Jonathan; Akin, Oguz; Schwartz, Lawrence H.; Cassie, Conrad; Panicek, David M.; Healey, John H.; Alektiar, Kaled M.

    2009-01-01

    To determine the prevalence and appearance of magnetic resonance imaging (MRI) signal changes that occur in local bone marrow after radiation therapy (RT) and/or chemotherapy for extremity soft tissue sarcoma (STS). Seventy patients with primary STS at the level of a long bone who also had undergone pretreatment MRI and at least one post-treatment MRI of the tumor bed were identified. MRIs of each patient were retrospectively reviewed for new changes in marrow signal in the region of the tumor bed and for the morphology, relative signal intensities, heterogeneity, and progression or regression of changes over time. Focal signal changes in marrow were observed in 26/70 patients (37%) at a median of 9.5 months after RT and/or chemotherapy and diffuse changes in seven (10%) at a median of 8 months. Patients who received neither RT nor chemotherapy did not develop marrow changes. Mean RT doses in patients with changes and those without were 5,867 and 6,076 cGy, respectively. In most patients with focal changes, changes were seen in all sequences and were linear-curvilinear, patchy, or mixed at the level of the tumor bed. Predominant signal intensity of changes was between muscle and fat at T1WI and between muscle and fluid at fat-saturated T2WI or short tau inversion recovery. Most focal changes enhanced heterogeneously and increased or fluctuated in size over time. Changes in MRI appearance of long bone marrow frequently are evident after combined RT and chemotherapy for STS and most commonly increase or fluctuate in size over time. These changes have various non-mass-like configurations and often show signal intensities similar to those of red marrow and thus should not be mistaken for metastases. The marrow changes might represent an early stage of gelatinous transformation of marrow. (orig.)

  18. Limb-sparing management with surgical resection, external-beam and intraoperative electron-beam radiation therapy boost for patients with primary soft tissue sarcoma of the extremity. A multicentric pooled analysis of long-term outcomes

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    Calvo, Felipe A. [Hospital General Universitario Gregorio Maranon, Department of Oncology, Madrid (Spain); Complutense University, School of Medicine, Madrid (Spain); Sole, Claudio V. [Hospital General Universitario Gregorio Maranon, Department of Oncology, Madrid (Spain); Complutense University, School of Medicine, Madrid (Spain); Instituto de Radiomedicina, Service of Radiation Oncology, Santiago (Chile); Polo, Alfredo; Montero, Angel [Hospital Universitario Ramon y Cajal, Service of Radiation Oncology, Madrid (Spain); Cambeiro, Mauricio; Martinez-Monge, Rafael [Clinica Universidad de Navarra, Service of Radiation Oncology, Pamplona (Spain); Alvarez, Ana [Hospital General Universitario Gregorio Maranon, Service of Radiation Oncology, Madrid (Spain); Cuervo, Miguel [Hospital General Universitario Gregorio Maranon, Service of Orthopedics and Traumatology, Madrid (Spain); Julian, Mikel San [Clinica Universidad de Navarra, Service of Orthopedics and Traumatology, Pamplona (Spain)

    2014-10-15

    A joint analysis of data from three contributing centres within the intraoperative electron-beam radiation therapy (IOERT) Spanish program was performed to investigate the main contributions of IORT to the multidisciplinary treatment of high-risk extremity soft tissue sarcoma (STS). Patients with an histologic diagnosis of primary extremity STS, with absence of distant metastases, undergoing limb-sparing surgery with radical intent, external beam radiotherapy (median dose 45 Gy) and IOERT (median dose 12.5 Gy) were considered eligible for participation in this study. From 1986-2012, a total of 159 patients were analysed in the study from three Spanish institutions. With a median follow-up time of 53 months (range 4-316 years), 5-year local control (LC) was 82 %. The 5-year IOERT in-field control, disease-free survival (DFS) and overall survival (OS) were 86, 62 and 72 %, respectively. On multivariate analysis, only microscopically involved margin (R1) resection status retained significance in relation to LC (HR 5.20, p < 0.001). With regard to IOERT in-field control, incomplete resection (HR 4.88, p = 0.001) and higher IOERT dose (≥ 12.5 Gy; HR 0.32, p = 0.02) retained a significant association in multivariate analysis. From this joint analysis emerges the fact that an IOERT dose ≥ 12.5 Gy increases the rate of IOERT in-field control, but DFS remains modest, given the high risk of distant metastases. Intensified local treatment needs to be tested in the context of more efficient concurrent, neo- and adjuvant systemic therapy. (orig.) [German] Um den therapeutischen Beitrag einer intraoperativen Bestrahlung mit Elektronen (IOERT) als Teil eines multidisziplinaeren Behandlungskonzepts von Weichteilsarkomen (STS) im Extremitaetenbereich mit hohem Risikoprofil evaluieren zu koennen, wurde anhand des spanischen IOERT-Programms eine gepoolte Datenanalyse von drei teilnehmenden Zentren vorgenommen. Eingeschlossen in diese Studie wurden Patienten mit histologisch

  19. Outcome of primary soft tissue sarcoma of the knee and elbow

    International Nuclear Information System (INIS)

    Alektiar, Kaled M.; McKee, Andrea B.; Jacobs, Jordan M.; McKee, Brady J.; Healey, John H.; Brennan, Murray F.

    2002-01-01

    Purpose: Concern is frequently raised regarding the tolerance of irradiation over a joint space. The purpose of this study was to determine the outcome in terms of relapse and potential complications in patients with knee and elbow soft tissue sarcoma treated with limb-sparing surgery with or without adjuvant radiotherapy (RT). Methods and Materials: A review of our prospective database between June 1982 and December 1999 identified 86 adult patients with primary soft tissue sarcoma arising from the knee (n=65; 76%) or elbow (n=21; 24%) treated with limb-sparing surgery. Tumors had high-grade histologic features in 72% and were >5 cm in 48% of patients. Adjuvant RT was given to 46 (54%) of 86 patients. The type of RT was postoperative external beam RT in 63% and brachytherapy in 37%. Of the 46 patients who received RT, 85% (n=39) had deep, 78% (n=36) high-grade, and 54% (n=25) >5-cm tumors. Complications were assessed in terms of wound complications requiring repeated surgery, bone fracture, nerve damage, and joint stiffness. Results: With a median follow-up of 48 months (range 4-175), the 5-year actuarial rate of local control, distant control, and overall survival was 75% (95% confidence interval [CI] 64-85%), 82% (95% CI 73-91%), and 81% (95% CI 71-91%), respectively. The 5-year local control rate for patients who received RT was 80% vs. 71% for those who did not (p=0.3). The type of RT did not significantly influence the local control rate. Patients treated with external beam RT had a 5-year local control rate of 84% compared with 73% for those treated with brachytherapy (p=0.4). On multivariate analysis, tumor size >5 cm retained its significance as an independent predictor of poor local control (p=0.04; relative risk 3; 95% CI 1-6). In addition, high-grade histologic features emerged as an independent predictor of local recurrence (p=0.02; relative risk 4; 95% CI 1-20). No statistically significant difference was found between the RT and no-RT group in terms

  20. Prediction of treatment response and metastatic disease in soft tissue sarcoma

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    Farhidzadeh, Hamidreza; Zhou, Mu; Goldgof, Dmitry B.; Hall, Lawrence O.; Raghavan, Meera.; Gatenby, Robert A.

    2014-03-01

    Soft tissue sarcomas (STS) are a heterogenous group of malignant tumors comprised of more than 50 histologic subtypes. Based on spatial variations of the tumor, predictions of the development of necrosis in response to therapy as well as eventual progression to metastatic disease are made. Optimization of treatment, as well as management of therapy-related side effects, may be improved using progression information earlier in the course of therapy. Multimodality pre- and post-gadolinium enhanced magnetic resonance images (MRI) were taken before and after treatment for 30 patients. Regional variations in the tumor bed were measured quantitatively. The voxel values from the tumor region were used as features and a fuzzy clustering algorithm was used to segment the tumor into three spatial regions. The regions were given labels of high, intermediate and low based on the average signal intensity of pixels from the post-contrast T1 modality. These spatially distinct regions were viewed as essential meta-features to predict the response of the tumor to therapy based on necrosis (dead tissue in tumor bed) and metastatic disease (spread of tumor to sites other than primary). The best feature was the difference in the number of pixels in the highest intensity regions of tumors before and after treatment. This enabled prediction of patients with metastatic disease and lack of positive treatment response (i.e. less necrosis). The best accuracy, 73.33%, was achieved by a Support Vector Machine in a leave-one-out cross validation on 30 cases predicting necrosis treatment and metastasis.

  1. Kaposi sarcoma

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    ... please enable JavaScript. Kaposi sarcoma is a cancerous tumor of the connective tissue, and is often associated with HIV/AIDS . Causes Before the HIV/AIDS epidemic, Kaposi sarcoma was seen mainly in older Italian ... this group, the tumors developed slowly. In people with HIV/AIDS, the ...

  2. Sarcoma de Kaposi em paciente transplantada renal em uso de Fk-506 Kaposi's Sarcoma in a renal transplant patient receiving Fk-506

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    Jorge David Rocha Zanol

    2002-12-01

    Full Text Available O Sarcoma de Kaposi (SK é neoplasia maligna multicêntrica, cutânea e extracutânea, que tem sido descrita em pacientes transplantados renais que recebem terapia imunossupressora clássica. Este estudo descreve um caso de sarcoma de Kaposi em paciente transplantada renal recebendo FK-506, que surgiu 10 meses após o transplante.Kaposi's sarcoma is a cutaneous and extra cutaneous multicentric malignancy that has been widely described in renal-transplant patients under classic immunosuppressive therapy. This study describes a renal-transplant patient under immunosuppressive therapy with FK-506 who presented Kaposi's sarcoma 10 months after the transplantation.

  3. Epidemiology and therapies for metastatic sarcoma

    Science.gov (United States)

    Amankwah, Ernest K; Conley, Anthony P; Reed, Damon R

    2013-01-01

    Sarcomas are cancers arising from the mesenchymal layer that affect children, adolescents, young adults, and adults. Although most sarcomas are localized, many display a remarkable predilection for metastasis to the lungs, liver, bones, subcutaneous tissue, and lymph nodes. Additionally, many sarcoma patients presenting initially with localized disease may relapse at metastatic sites. While localized sarcomas can often be cured through surgery and often radiation, controversies exist over optimal management of patients with metastatic sarcoma. Combinations of chemotherapy are the most effective in many settings, and many promising new agents are under active investigation or are being explored in preclinical models. Metastatic sarcomas are excellent candidates for novel approaches with additional agents as they have demonstrated chemosensitivity and affect a portion of the population that is motivated toward curative therapy. In this paper, we provide an overview on the common sarcomas of childhood (rhabdomyosarcoma), adolescence, and young adults (osteosarcoma, Ewing sarcoma, synovial sarcoma, and malignant peripheral nerve sheath tumor) and older adults (leiomyosarcoma, liposarcoma, and undifferentiated high grade sarcoma) in terms of the epidemiology, current therapy, promising therapeutic directions and outcome with a focus on metastatic disease. Potential advances in terms of promising therapy and biologic insights may lead to more effective and safer therapies; however, more clinical trials and research are needed for patients with metastatic sarcoma. PMID:23700373

  4. Epidemiology and therapies for metastatic sarcoma

    Directory of Open Access Journals (Sweden)

    Amankwah EK

    2013-05-01

    Full Text Available Ernest K Amankwah,1 Anthony P Conley,2 Damon R Reed2 1Department of Cancer Epidemiology, H Lee Moffitt Cancer Center and Research Institute, Tampa, FL, USA; 2Sarcoma Department, H Lee Moffitt Cancer Center and Research Institute, Tampa, FL, USA Abstract: Sarcomas are cancers arising from the mesenchymal layer that affect children, adolescents, young adults, and adults. Although most sarcomas are localized, many display a remarkable predilection for metastasis to the lungs, liver, bones, subcutaneous tissue, and lymph nodes. Additionally, many sarcoma patients presenting initially with localized disease may relapse at metastatic sites. While localized sarcomas can often be cured through surgery and often radiation, controversies exist over optimal management of patients with metastatic sarcoma. Combinations of chemotherapy are the most effective in many settings, and many promising new agents are under active investigation or are being explored in preclinical models. Metastatic sarcomas are excellent candidates for novel approaches with additional agents as they have demonstrated chemosensitivity and affect a portion of the population that is motivated toward curative therapy. In this paper, we provide an overview on the common sarcomas of childhood (rhabdomyosarcoma, adolescence, and young adults (osteosarcoma, Ewing sarcoma, synovial sarcoma, and malignant peripheral nerve sheath tumor and older adults (leiomyosarcoma, liposarcoma, and undifferentiated high grade sarcoma in terms of the epidemiology, current therapy, promising therapeutic directions and outcome with a focus on metastatic disease. Potential advances in terms of promising therapy and biologic insights may lead to more effective and safer therapies; however, more clinical trials and research are needed for patients with metastatic sarcoma. Keywords: chemotherapy, pediatric sarcoma, rhabdomyosarcoma, osteosarcoma, Ewing sarcoma, synovial sarcoma

  5. Primary renal synovial sarcoma

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    Girish D. Bakhshi

    2012-03-01

    Full Text Available Primary Renal Sarcoma is rare tumor comprising only 1% of all renal tumours. Synovial sarcomas are generally deep-seated tumors arising in the proximity of large joints of adolescents and young adults and account for 5-10% of all soft tissue tumours. Primary synovial sarcoma of kidney is rare and has poor prognosis. It can only be diagnosed by immunohistochemistry. It should be considered as a differential in sarcomatoid and spindle cell tumours. We present a case of 33-year-old female, who underwent left sided radical nephrectomy for renal tumour. Histopathology and genetic analysis diagnosed it to be primary renal synovial sarcoma. Patient underwent radiation therapy and 2 years follow up is uneventful. A brief case report with review of literature is presented.

  6. Current concepts in non-gastrointestinal stromal tumor soft tissue sarcomas: A primer for radiologists

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    Baheti, Akahay D. [Dept. of Radiology, Tata Memorial Centre, Mumbai (India); Tirumani, Harika [Dept. of Radiology, University of Arkansas for Medical Sciences, Little Rock (United States); O' Neill, Alibhe; Jagannathan, Jyothi P. [Dept. of Imaging, Dana-Farber Cancer Institute, Boston (United States)

    2017-01-15

    Non-gastrointestinal stromal tumor (GIST) soft tissue sarcomas (STSs) are a heterogeneous group of neoplasms whose classification and management continues to evolve with better understanding of their biologic behavior. The 2013 World Health Organization (WHO) has revised their classification based on new immunohistochemical and cytogenetic data. In this article, we will provide a brief overview of the revised WHO classification of soft tissue tumors, discuss in detail the radiology and management of the two most common adult non-GIST STS, namely liposarcoma and leiomyosarcoma, and review some of the emerging histology-driven targeted therapies in non-GIST STS, focusing on the role of the radiologist.

  7. Current concepts in non-gastrointestinal stromal tumor soft tissue sarcomas: A primer for radiologists

    International Nuclear Information System (INIS)

    Baheti, Akahay D.; Tirumani, Harika; O'Neill, Alibhe; Jagannathan, Jyothi P.

    2017-01-01

    Non-gastrointestinal stromal tumor (GIST) soft tissue sarcomas (STSs) are a heterogeneous group of neoplasms whose classification and management continues to evolve with better understanding of their biologic behavior. The 2013 World Health Organization (WHO) has revised their classification based on new immunohistochemical and cytogenetic data. In this article, we will provide a brief overview of the revised WHO classification of soft tissue tumors, discuss in detail the radiology and management of the two most common adult non-GIST STS, namely liposarcoma and leiomyosarcoma, and review some of the emerging histology-driven targeted therapies in non-GIST STS, focusing on the role of the radiologist

  8. Phase I vaccination trial of SYT-SSX junction peptide in patients with disseminated synovial sarcoma

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    Asanuma Hiroko

    2005-01-01

    Full Text Available Abstract Background Synovial sarcoma is a high-grade malignant tumor of soft tissue, characterized by the specific chromosomal translocation t(X;18, and its resultant SYT-SSX fusion gene. Despite intensive multimodality therapy, the majority of metastatic or relapsed diseases still remain incurable, thus suggesting a need for new therapeutic options. We previously demonstrated the antigenicity of SYT-SSX gene-derived peptides by in vitro analyses. The present study was designed to evaluate in vivo immunological property of a SYT-SSX junction peptide in selected patients with synovial sarcoma. Methods A 9-mer peptide (SYT-SSX B: GYDQIMPKK spanning the SYT-SSX fusion region was synthesized. Eligible patients were those (i who have histologically and genetically confirmed, unresectable synovial sarcoma (SYT-SSX1 or SYT-SSX2 positive, (ii HLA-A*2402 positive, (iii between 20 and 70 years old, (iv ECOG performance status between 0 and 3, and (v who gave informed consent. Vaccinations with SYT-SSX B peptide (0.1 mg or 1.0 mg were given subcutaneously six times at 14-day intervals. These patients were evaluated for DTH skin test, adverse events, tumor size, tetramer staining, and peptide-specific CTL induction. Results A total of 16 vaccinations were carried out in six patients. The results were (i no serious adverse effects or DTH reactions, (ii suppression of tumor progression in one patient, (iii increases in the frequency of peptide-specific CTLs in three patients and a decrease in one patient, and (iv successful induction of peptide-specific CTLs from four patients. Conclusions Our findings indicate the safety of the SYT-SSX junction peptide in the use of vaccination and also give support to the property of the peptide to evoke in vivo immunological responses. Modification of both the peptide itself and the related protocol is required to further improve the therapeutic efficacy.

  9. The efficacy of postoperative radiotherapy in localized primary soft tissue sarcoma treated with conservative surgery

    International Nuclear Information System (INIS)

    Zhao, Ru-Ping; Yu, Xiao-Li; Zhang, Zhen; Jia, Li-Juan; Feng, Yan; Yang, Zhao-Zhi; Chen, Xing-Xing; Wang, Jian; Ma, Sheng-Lin; Guo, Xiao-Mao

    2016-01-01

    To evaluate the efficacy of postoperative radiotherapy (RT) on local failure-free survival (LFFS), distant metastasis-free survival (DMFS) and overall survival (OS) in patients with localized primary soft tissue sarcoma (STS) and to identify prognostic factors. Between January 2000 and July 2010, 220 consecutive patients with localized primary STS, who received conservative surgery with or without postoperative RT, were enrolled in the study. Survival curves were constructed by the Kaplan-Meier method and log-rank test was used to assess statistical significance. Multivariate analysis was applied to identify the prognostic factors. After a median follow-up of 68 months (range, 5–127 months), the 5-year LFFS, DMFS and OS were 70.0, 78.2 and 71.2 %, respectively. Tumor size, histological subtypes, margin status and postoperative RT were independent predictors for OS. Postoperative RT was associated with a significant reduced local recurrence risk versus surgery alone (hazard ratio [HR] = 0.408, 95 % confidence interval [CI] 0.235–0.707, P = 0.001), with 5-year LFFS of 81.1 and 63.6 %, respectively (log-rank, P = 0.004). The log-rank test showed that postoperative RT had a tendency of improving OS compared with surgery alone, with 5-year OS of 74.8 and 65.0 %, respectively (P = 0.089). Multivariate analysis demonstrated that postoperative RT significantly reduced mortality rate compared with surgery alone (HR = 0.512, 95 % CI 0.296–0.886, p = 0.017), especially in patients with liposarcoma (p = 0.034). Postoperative radiotherapy reduce both local recurrence and STS mortality in patients with localized primary STS. The efficacy of RT on survival warrants further prospective study. The online version of this article (doi:10.1186/s13014-016-0605-y) contains supplementary material, which is available to authorized users

  10. Post-relapse survival in patients with Ewing sarcoma.

    Science.gov (United States)

    Ferrari, Stefano; Luksch, Roberto; Hall, Kirsten Sundby; Fagioli, Franca; Prete, Arcangelo; Tamburini, Angela; Tienghi, Amelia; DiGirolamo, Stefania; Paioli, Anna; Abate, Massimo Eraldo; Podda, Marta; Cammelli, Silvia; Eriksson, Mikael; Brach del Prever, Adalberto

    2015-06-01

    Post-relapse survival (PRS) was evaluated in patients with Ewing sarcoma (EWS) enrolled in chemotherapy protocols based on the use of high-dose chemotherapy with busulfan and melfalan (HDT) as a first-line consolidation treatment in high-risk patients. EWS patients enrolled in ISG/SSG III and IV trials who relapsed after complete remission were included in the analysis. At recurrence, chemotherapy based on high-dose ifosfamide was foreseen, and patients who responded but had not received HDT underwent consolidation therapy with HDT. Data from 107 EWS patients were included in the analysis. Median time to recurrence (RFI) was 18 months, and 45 (42%) patients had multiple sites of recurrence. Patients who had previously been treated with HDT had a significantly (P = 0.02) shorter RFI and were less likely to achieve a second complete remission (CR2). CR2 status was achieved by 42 (39%) patients. Fifty patients received high-dose IFO (20 went to consolidation HDT). The 5-year PRS was 19% (95% CI 11 to 27%). With CR2, the 5-year PRS was 48% (95% CI 31 to 64%). Without CR2, median time to death was six months (range 1-45 months). According to the multivariate analysis, patients younger than 15 years, recurrence to the lung only, and RFI longer than 24 months significantly influenced the probability of PRS. Age, pattern of recurrence, RFI, and response to second-line chemotherapy influence post-relapse survival in patients with recurrent Ewing sarcoma. No survival advantage was observed from chemotherapy consolidation with HDT. © 2015 Wiley Periodicals, Inc.

  11. Efficacy of triplet regimen antiemetic therapy for chemotherapy-induced nausea and vomiting (CINV) in bone and soft tissue sarcoma patients receiving highly emetogenic chemotherapy, and an efficacy comparison of single-shot palonosetron and consecutive-day granisetron for CINV in a randomized, single-blinded crossover study

    International Nuclear Information System (INIS)

    Kimura, Hiroaki; Yamamoto, Norio; Shirai, Toshiharu; Nishida, Hideji; Hayashi, Katsuhiro; Tanzawa, Yoshikazu; Takeuchi, Akihiko; Igarashi, Kentaro; Inatani, Hiroyuki; Shimozaki, Shingo; Kato, Takashi; Aoki, Yu; Higuchi, Takashi; Tsuchiya, Hiroyuki

    2015-01-01

    The first aim of this study was to evaluate combination antiemetic therapy consisting of 5-HT 3 receptor antagonists, neurokinin-1 receptor antagonists (NK-1RAs), and dexamethasone for multiple high emetogenic risk (HER) anticancer agents in bone and soft tissue sarcoma. The second aim was to compare the effectiveness of single-shot palonosetron and consecutive-day granisetron in a randomized, single-blinded crossover study. A single randomization method was used to assign eligible patients to the palonosetron or granisetron arm. Patients in the palonosetron arm received a palonosetron regimen during the first and third chemotherapy courses and a granisetron regimen during the second and fourth courses. All patients received NK-1RA and dexamethasone. Patients receiving the palonosetron regimen were administered 0.75 mg palonosetron on day 1, and patients receiving the granisetron regimen were administered 3 mg granisetron twice daily on days 1 through 5. All 24 patients in this study received at least 4 chemotherapy courses. A total of 96 courses of antiemetic therapy were evaluated. Overall, the complete response CR rate (no emetic episodes and no rescue medication use) was 34%, while the total control rate (a CR plus no nausea) was 7%. No significant differences were observed between single-shot palonosetron and consecutive-day granisetron. Antiemetic therapy with a 3-drug combination was not sufficient to control chemotherapy-induced nausea and vomiting (CINV) during chemotherapy with multiple HER agents for bone and soft tissue sarcoma. This study also demonstrated that consecutive-day granisetron was not inferior to single-shot palonosetron for treating CINV

  12. Efficacy of triplet regimen antiemetic therapy for chemotherapy-induced nausea and vomiting (CINV) in bone and soft tissue sarcoma patients receiving highly emetogenic chemotherapy, and an efficacy comparison of single-shot palonosetron and consecutive-day granisetron for CINV in a randomized, single-blinded crossover study.

    Science.gov (United States)

    Kimura, Hiroaki; Yamamoto, Norio; Shirai, Toshiharu; Nishida, Hideji; Hayashi, Katsuhiro; Tanzawa, Yoshikazu; Takeuchi, Akihiko; Igarashi, Kentaro; Inatani, Hiroyuki; Shimozaki, Shingo; Kato, Takashi; Aoki, Yu; Higuchi, Takashi; Tsuchiya, Hiroyuki

    2015-03-01

    The first aim of this study was to evaluate combination antiemetic therapy consisting of 5-HT3 receptor antagonists, neurokinin-1 receptor antagonists (NK-1RAs), and dexamethasone for multiple high emetogenic risk (HER) anticancer agents in bone and soft tissue sarcoma. The second aim was to compare the effectiveness of single-shot palonosetron and consecutive-day granisetron in a randomized, single-blinded crossover study. A single randomization method was used to assign eligible patients to the palonosetron or granisetron arm. Patients in the palonosetron arm received a palonosetron regimen during the first and third chemotherapy courses and a granisetron regimen during the second and fourth courses. All patients received NK-1RA and dexamethasone. Patients receiving the palonosetron regimen were administered 0.75 mg palonosetron on day 1, and patients receiving the granisetron regimen were administered 3 mg granisetron twice daily on days 1 through 5. All 24 patients in this study received at least 4 chemotherapy courses. A total of 96 courses of antiemetic therapy were evaluated. Overall, the complete response CR rate (no emetic episodes and no rescue medication use) was 34%, while the total control rate (a CR plus no nausea) was 7%. No significant differences were observed between single-shot palonosetron and consecutive-day granisetron. Antiemetic therapy with a 3-drug combination was not sufficient to control chemotherapy-induced nausea and vomiting (CINV) during chemotherapy with multiple HER agents for bone and soft tissue sarcoma. This study also demonstrated that consecutive-day granisetron was not inferior to single-shot palonosetron for treating CINV. © 2014 The Authors. Cancer Medicine published by John Wiley & Sons Ltd.

  13. A Systematic Overview of Radiation Therapy Effects in Soft Tissue Sarcomas

    International Nuclear Information System (INIS)

    Strander, Hans; Turesson, Ingela; Cavallin-Staahl, Eva

    2003-01-01

    A systematic review of radiation therapy trials in several tumour types was performed by The Swedish Council of Technology Assessment in Health Care (SBU). The procedures for evaluation of the scientific literature are described separately. This synthesis of the literature on radiation therapy for soft tissue sarcomas (STS) is based on data from five randomized trials. Moreover, data from 6 prospective studies, 25 retrospective studies and 3 other articles were used. In total, 39 scientific articles are included, involving 4,579 patients. The results were compared with those of a similar overview from 1996 which included 3,344 patients. The conclusions reached can be summarized as follows: The well-established prognostic factors for tumour-related death from STS--histological grade, tumour size and age--are well documented. The importance of superficial versus deep site as well as the anatomic site is also reaffirmed to some extent. There is strong evidence that adjuvant radiotherapy improves the local control rate in combination with conservative surgery in the treatment of STS of extremities and trunk in patients with negative, marginal or minimal microscopic positive surgical margins. A local control rate of 90% has been achieved. Improvement is obtained with radiotherapy added in the case of intralesional surgery, but the local control rate is somewhat lower. More studies are needed on this issue. For STS in other anatomic sites, retroperitoneum, head and neck, breast and uterus, there is only weak indication of a benefit for the local control rate, with the use of adjuvant radiotherapy. There is still insufficient data to establish that preoperative radiotherapy is favourable compared to postoperative radiotherapy for local control in patients presenting primarily with large tumours. One small study has shown a possible survival benefit for preoperative radiotherapy. There is fairly good evidence to suggest that the preoperative setting results in more wound

  14. Can Bone Tissue Engineering Contribute to Therapy Concepts after Resection of Musculoskeletal Sarcoma?

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    Boris Michael Holzapfel

    2013-01-01

    Full Text Available Resection of musculoskeletal sarcoma can result in large bone defects where regeneration is needed in a quantity far beyond the normal potential of self-healing. In many cases, these defects exhibit a limited intrinsic regenerative potential due to an adjuvant therapeutic regimen, seroma, or infection. Therefore, reconstruction of these defects is still one of the most demanding procedures in orthopaedic surgery. The constraints of common treatment strategies have triggered a need for new therapeutic concepts to design and engineer unparalleled structural and functioning bone grafts. To satisfy the need for long-term repair and good clinical outcome, a paradigm shift is needed from methods to replace tissues with inert medical devices to more biological approaches that focus on the repair and reconstruction of tissue structure and function. It is within this context that the field of bone tissue engineering can offer solutions to be implemented into surgical therapy concepts after resection of bone and soft tissue sarcoma. In this paper we will discuss the implementation of tissue engineering concepts into the clinical field of orthopaedic oncology.

  15. Clinical implications of determination of safe surgical margins by using a combination of CT and 18FDG-positron emission tomography in soft tissue sarcoma

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    Yoshioka Takako

    2011-07-01

    Full Text Available Abstract Background To determine safe surgical margins for soft tissue sarcoma, it is essential to perform a general evaluation of the extent of tumor, responses to auxiliary therapy, and other factors preoperatively using multiple types of diagnostic imaging. 18F-fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT is a tool for diagnostic imaging that has recently spread rapidly in clinical use. At present, the roles played by FDG-PET/CT in determination of margins for surgical resection of sarcoma are unclear. The present study was undertaken to explore the roles of FDG-PET/CT in determination of surgical margins for soft tissue sarcoma and to examine whether PET can serve as a standard means for setting the margins of surgical resection during reduced surgery. Methods The study involved 7 patients with sarcoma who underwent surgery in our department and in whom evaluation with FDG-PET/CT was possible. Sarcoma was histologically rated as MFH in 6 cases and leiomyosarcoma in 1 case. In all cases, sarcoma was superficial (T1a or T2a. The tumor border was defined by contrast-enhanced MRI, and SUVs were measured at intervals of 1 cm over a 5-cm long area from the tumor border. Mapping of viable tumor cells was carried out on whole-mount sections of resected tissue, and SUVs were compared with histopathological findings. Results Preoperative maximum SUVs (SUV-max of the tumor averaged 11.7 (range: 3.8-22.1. Mean SUV-max was 2.2 (range: 0.3-3.8 at 1 cm from the tumor border, 1.1 (0.85-1.47 at 2 cm, 0.83 (0.65-1.15 at 3 cm, 0.7 (0.42-0.95 at 4 cm, and 0.64 (0.45-0.82 at 5 cm. When resected tissue was mapped, tumor cells were absent in the areas where SUV-max was below 1.0. Conclusions Our findings suggest that a safe surgical margin free of viable tumor cells can be ensured if the SUV cut-off level is set at 1.0. FDG-PET/CT is promising as a diagnostic imaging technique for setting of safe minimal margins for surgical

  16. Granulocytic sarcoma in a patient with chronic myeloid leukaemia in complete haematological, cytogenetic and molecular remission.

    Science.gov (United States)

    Kittai, Adam; Yu, Eun-Mi; Tabbara, Imad

    2014-12-23

    Granulocytic sarcoma, also known as myeloid sarcoma, is an extramedullary tumour composed of immature myeloid cells. Granulocytic sarcoma is typically found in patients with acute myeloid leukaemia, accelerated phase or blast crisis of chronic myeloid leukaemia, myelodysplastic syndrome, or as an isolated event without bone marrow involvement. We present a case of granulocytic sarcoma in a patient with chronic myeloid leukaemia in the setting of complete haematological, molecular and cytogenetic remission. Our patient was first treated with imatinib for chronic-phase chronic myeloid leukaemia. After maintaining remission for 42 months, he developed a granulocytic sarcoma in his spine. In this case report, we describe our case, along with the three other cases reported in the literature. In addition to being a rare diagnosis, this case demonstrates the importance of being vigilant in diagnosing the cause of back pain and atypical symptoms in patients with a history of leukaemia. 2014 BMJ Publishing Group Ltd.

  17. Down-regulated E-cadherin expression is associated with poor five-year overall survival in bone and soft tissue sarcoma: results of a meta-analysis.

    Directory of Open Access Journals (Sweden)

    Ning Wang

    Full Text Available To conduct a meta-analysis to evaluate the prognostic role of E-cadherin expression in bone and soft tissue sarcomas.The PubMed, EMBASE, and Web of Science databases were searched using terms related to E-cadherin, sarcoma, and prognosis for all articles published in English before March 2014. Pooled effect was calculated from the available data to evaluate the association between negative E-cadherin expression and 5-year overall survival and tumor clinicopathological features in sarcoma patients. Pooled odds ratios (OR and risk ratios (RR with 95% confidence intervals (CI were calculated using a fixed-effects model.Eight studies met the selection criteria and reported on 812 subjects. A total of 496 subjects showed positive E-cadherin expression (59.9%. Negative E-cadherin expression in bone and soft tissue sarcomas was correlated with lower 5-year overall survival (OR = 3.831; 95% CI: 2.246-6.534, and was associated with higher clinical stage (RR = 1.446; 95% CI: 1.030-2.028 and with male sex (RR = 0.678; 95% CI: 0.493-0.933.In the E-cadherin negative group, 5-year overall survival was significantly worse than in the E-cadherin positive group. However, further studies are required to confirm these results.

  18. The effect of delayed postoperative irradiation on local control of soft tissue sarcomas of the extremity and torso

    International Nuclear Information System (INIS)

    Schwartz, David L.; Einck, John; Hunt, Karen; Bruckner, James; Conrad, Ernest; Koh, W.-J.; Laramore, George E.

    2002-01-01

    Purpose: The impact of delayed adjuvant radiotherapy in patients treated by surgical resection for peripheral or torso soft tissue sarcoma has not been well characterized. We retrospectively examined this issue in an institutional patient cohort. Methods and Materials: One hundred two adult patients were treated at the University of Washington Medical Center between 1981 and 1998 with postoperative radiotherapy for cure of a newly diagnosed soft tissue sarcoma. Of this group, 58 patients had primary intermediate- or high-grade disease of the extremity or torso (50 extremity/8 torso). Tumor histology was predominantly malignant fibrohistiocytoma, synovial cell sarcoma, and leiomyosarcoma. The group was dichotomized according to time interval from definitive resection to the start of adjuvant radiation. Twenty-six patients had a short delay, defined as <4 months, and 32 patients had a long delay of ≥4 months. Both groups were balanced with regard to site, size, margin status, and tumor depth; however, the long-delay group had a larger proportion of high histologic grade lesions and was treated more frequently with chemotherapy (31/32 [97%] for long-delay patients vs. 14/26 [54%] for short-delay patients). Median follow-up was 49.5 months (range: 7-113 months). Median follow-up for patients still alive was 54 months (range: 9-113 months). Survival outcomes were estimated by the Kaplan-Meier method. Results: Overall local relapse-free survival at 5 years from the time of definitive resection was 74%. On univariate analysis, estimated 5-year local relapse-free survival was significantly improved in the short-delay group (88% vs. 62% for the long-delay group, p = 0.048 by log rank). Overall distant relapse-free survival, disease-free survival, and overall survival at 5 years were 77%, 68%, and 86%, respectively. These survival outcomes were statistically equivalent in both radiation delay groups. There was no evidence to suggest that delaying adjuvant systemic therapy

  19. Immunosuppressive Therapy-Related Kaposi Sarcoma

    Science.gov (United States)

    ... Sarcoma Treatment Childhood Vascular Tumors Treatment Research Kaposi Sarcoma Treatment (PDQ®)–Patient Version General Information About Kaposi Sarcoma Go to Health Professional Version Key Points Kaposi ...

  20. Kaposi's sarcoma

    International Nuclear Information System (INIS)

    Kirova, Y.M.; Belembaogo, E.; Frikha, H.; Yu, S.J.; Le Bourgeois, J.P.

    1997-01-01

    Moriz Kaposi was the first who, in 1872, described five patients presenting with 'sarcoma idiopathicum multiple hemorrhagicum'. In 1912 Sternberg termed this disease Kaposi's sarcoma. Since then various forms of this rare disease have been observed. In 1914 Hallenberg described the first cases of African or endemic Kaposi's sarcoma. In the 1960's the first reports discussing Kaposi's sarcoma following organ transplantation and immunosuppressive therapy were published. After 1981, the epidemic form associated with the acquired immunodeficiency syndrome (AIDS) was described. All these forms, their history, treatment methods and the role of radiation therapy in the management of this rare malignancy are discussed, and the literature is reviewed. (authors)

  1. Intraoperative Radiotherapy in the Management of Locally Recurrent Extremity Soft Tissue Sarcoma

    Directory of Open Access Journals (Sweden)

    Christopher L. Tinkle

    2015-01-01

    Full Text Available Purpose. To investigate the efficacy and morbidity of limb-sparing surgery with intraoperative radiotherapy (IORT for patients with locally recurrent extremity soft tissue sarcoma (ESTS. Methods and Materials. Twenty-six consecutively treated patients were identified in a single institution retrospective analysis of patients with locally recurrent ESTS treated with IORT following salvage limb-sparing resection from May 2000 to July 2011. Fifteen (58% patients received external beam radiotherapy (EBRT prior to recurrence (median dose 63 Gy, while 11 (42% patients received EBRT following IORT (median dose 52 Gy. The Kaplan-Meier product limit method was used to estimate disease control and survival and subsets were compared using a log rank statistic, Cox’s regression model was used to determine independent predictors of disease outcome, and toxicity was reported according to CTCAE v4.0 guidelines. Results. With a median duration of follow-up from surgery and IORT of 34.9 months (range: 4 to 139 mos., 10 patients developed a local recurrence with 4 subsequently undergoing amputation. The 5-year estimate for local control (LC was 58% (95% CI: 36–75%, for amputation-free was 81% (95% CI: 57–93%, for metastasis-free control (MFC was 56% (95% CI: 31–75%, for disease-free survival (DFS was 35% (95% CI: 17–54%, and for overall survival (OS was 50% (95% CI: 24–71%. Prior EBRT did not appear to influence disease control (LC, p=0.74; MFC, p=0.66 or survival (DFS, p=0.16; OS, p=0.58. Grade 3 or higher acute and late toxicities were reported for 6 (23% and 8 (31% patients, respectively. The frequency of both acute and late grade 3 or higher toxicities occurred equally between patients who received EBRT prior to or after IORT. Conclusions. IORT in combination with oncologic resection of recurrent ESTS yields good rates of local control and limb-salvage with acceptable morbidity. Within the limitations of small subsets, these data suggest

  2. Low-dose-rate intraoperative brachytherapy combined with external beam irradiation in the conservative treatment of soft tissue sarcoma

    International Nuclear Information System (INIS)

    Delannes, M.; Thomas, L.; Martel, P.; Bonnevialle, P.; Stoeckle, E.; Chevreau, Ch.; Bui, B.N.; Daly-Schveitzer, N.; Pigneux, J.; Kantor, G.

    2000-01-01

    Purpose: Conservative treatment of soft tissue sarcomas most often implies combination of surgical resection and irradiation. The aim of this study was to evaluate low-dose-rate intraoperative brachytherapy, delivered as a boost, in the local control of primary tumors, with special concern about treatment complications. Methods and Materials: Between 1986 and 1995, 112 patients underwent intraoperative implant. This report focuses on the group of 58 patients with primary sarcomas treated by combination of conservative surgery, intraoperative brachytherapy, and external irradiation. Most of the tumors were located in the lower limbs (46/58--79%). Median size of the tumor was 10 cm, most of the lesions being T2-T3 (51/58--88%), Grade 2 or 3 (48/58--83%). The mean brachytherapy dose was 20 Gy and external beam irradiation dose 45 Gy. In 36/58 cases, iridium wires had to be placed on contact with neurovascular structures. Results: With a median follow-up of 54 months, the 5-year actuarial survival was 64.9%, with a 5-year actuarial local control of 89%. Of the 6 patients with local relapse, 3 were salvaged. Acute side effects, essentially wound healing problems, occurred in 20/58 patients, late side effects in 16/58 patients (7 neuropathies G2 to G4). No amputation was required. The only significant factor correlated with early side effects was the location of the tumor in the lower limb (p = 0.003), and with late side effects the vicinity of the tumor with neurovascular structures (p = 0.009). Conclusion: Brachytherapy allows early delivery of a boost dose in a reduced volume of tissue, precisely mapped by the intraoperative procedure. Combined with external beam irradiation, it is a safe and efficient treatment technique leading to high local control rates and limited functional impairment

  3. Primary Ewing's Sarcoma of Frontoparietal Bone with Major Soft Tissue Extension: An Unusual Presentation and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Anshu Gupta

    2012-01-01

    Full Text Available An 11-year-old girl presented with progressively increasing swelling in scalp of 8-month duration with no neurological deficit. Local examination showed a hard swelling that seemed to be arising from frontal bone. General and systemic examination was normal. MRI revealed a well-defined lytic lesion in left frontoparietal bone with a subgaleal component. The patient was operated upon and excision of tumor with reconstruction of skull was done. Histopathological examination showed a monomorphic small round cell tumor of bone infiltrating into the subcutaneous tissue. Immunohistochemical stain showed diffuse immunopositivity for MIC-2 in tumor cells, thus final diagnosis of Ewing’s sarcoma was made. The patient was kept for follow up for 3 months and had no symptoms.

  4. Orbital Epithelioid Sarcoma: A Case Report

    NARCIS (Netherlands)

    Jurdy, Lama L.; Blank, Leo E.; Bras, Johannes; Saeed, Peerooz

    2016-01-01

    Epithelioid sarcoma is a rare but often aggressive malignancy of soft tissue that usually occurs in young adults as a superficial lesion in the distal upper limbs. To date, there are only 4 case reports of epithelioid sarcoma primarily occurring in the orbit. Two of these patients were treated with

  5. Dose optimization of intra-operative high dose rate interstitial brachytherapy implants for soft tissue sarcoma

    Directory of Open Access Journals (Sweden)

    Jamema Swamidas

    2009-01-01

    Full Text Available Objective : A three dimensional (3D image-based dosimetric study to quantitatively compare geometric vs. dose-point optimization in combination with graphical optimization for interstitial brachytherapy of soft tissue sarcoma (STS. Materials and Methods : Fifteen consecutive STS patients, treated with intra-operative, interstitial Brachytherapy, were enrolled in this dosimetric study. Treatment plans were generated using dose points situated at the "central plane between the catheters", "between the catheters throughout the implanted volume", at "distances perpendicular to the implant axis" and "on the surface of the target volume" Geometrically optimized plans had dose points defined between the catheters, while dose-point optimized plans had dose points defined at a plane perpendicular to the implant axis and on the target surface. Each plan was graphically optimized and compared using dose volume indices. Results : Target coverage was suboptimal with coverage index (CI = 0.67 when dose points were defined at the central plane while it was superior when the dose points were defined at the target surface (CI=0.93. The coverage of graphically optimized plans (GrO was similar to non-GrO with dose points defined on surface or perpendicular to the implant axis. A similar pattern was noticed with conformity index (0.61 vs. 0.82. GrO were more conformal and less homogeneous compared to non-GrO. Sum index was superior for dose points defined on the surface of the target and relatively inferior for plans with dose points at other locations (1.35 vs. 1.27. Conclusions : Optimization with dose points defined away from the implant plane and on target results in superior target coverage with optimal values of other indices. GrO offer better target coverage for implants with non-uniform geometry and target volume.

  6. Cost-Effectiveness Analysis of Preoperative Versus Postoperative Radiation Therapy in Extremity Soft Tissue Sarcoma

    International Nuclear Information System (INIS)

    Qu, Xuanlu M.; Louie, Alexander V.; Ashman, Jonathan; Wasif, Nabil

    2017-01-01

    Purpose: Surgery combined with radiation therapy (RT) is the cornerstone of multidisciplinary management of extremity soft tissue sarcoma (STS). Although RT can be given in either the preoperative or the postoperative setting with similar local recurrence and survival outcomes, the side effect profiles, costs, and long-term functional outcomes are different. The aim of this study was to use decision analysis to determine optimal sequencing of RT with surgery in patients with extremity STS. Methods and Materials: A cost-effectiveness analysis was conducted using a state transition Markov model, with quality-adjusted life years (QALYs) as the primary outcome. A time horizon of 5 years, a cycle length of 3 months, and a willingness-to-pay threshold of $50,000/QALY was used. One-way deterministic sensitivity analyses were performed to determine the thresholds at which each strategy would be preferred. The robustness of the model was assessed by probabilistic sensitivity analysis. Results: Preoperative RT is a more cost-effective strategy ($26,633/3.00 QALYs) than postoperative RT ($28,028/2.86 QALYs) in our base case scenario. Preoperative RT is the superior strategy with either 3-dimensional conformal RT or intensity-modulated RT. One-way sensitivity analyses identified the relative risk of chronic adverse events as having the greatest influence on the preferred timing of RT. The likelihood of preoperative RT being the preferred strategy was 82% on probabilistic sensitivity analysis. Conclusions: Preoperative RT is more cost effective than postoperative RT in the management of resectable extremity STS, primarily because of the higher incidence of chronic adverse events with RT in the postoperative setting.

  7. Gemcitabine treatment of rat soft tissue sarcoma with phosphatidyldiglycerol-based thermosensitive liposomes.

    Science.gov (United States)

    Limmer, Simone; Hahn, Jasmin; Schmidt, Rebecca; Wachholz, Kirsten; Zengerle, Anja; Lechner, Katharina; Eibl, Hansjörg; Issels, Rolf D; Hossann, Martin; Lindner, Lars H

    2014-09-01

    The pyrimidine analogue gemcitabine (dFdC) is frequently used in the treatment of patients with solid tumors. However, after i.v. application dFdC is rapidly inactivated by metabolization. Here, the potential of thermosensitive liposomes based on 1,2-dipalmitoyl-sn-glycero-3-phosphodiglycerol (DPPG2-TSL) were investigated as carrier and targeting system for delivery of dFdC in combination with local hyperthermia (HT). DPPG2-TSL were prepared by the lipid film hydration and extrusion method and characterized by dynamic light scattering, thin layer chromatography, phosphate assay and HPLC. In vivo experiments were performed in Brown Norway rats with a syngeneic soft tissue sarcoma. Local HT treatment was performed by light exposure. DPPG2-TSL were stable at 37°C in serum and showed a temperature dependent dFdC release >40°C. Plasma half-life of dFdC was strongly increased from 0.07 h (non-liposomal) to 0.53 h (liposomal, vesicle size 105 nm) or 2.59 h (liposomal, 129 nm). Therapy of BN175 tumors with dFdC encapsulated in DPPG2-TSL + HT showed significant improvement in tumor growth delay compared to non-liposomal dFdC without HT (p < 0.05), non-liposomal dFdC with HT (p < 0.01), and liposomal dFdC without HT (p < 0.05), respectively. Gemcitabine encapsulated in DPPG2-TSL in combination with local HT is a promising tool for the treatment of solid tumors. Therefore, these encouraging results ask for further investigation and evaluation.

  8. Cost-Effectiveness Analysis of Preoperative Versus Postoperative Radiation Therapy in Extremity Soft Tissue Sarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Qu, Xuanlu M. [Department of Oncology, Queen' s University, Kingston, Ontario (Canada); Louie, Alexander V. [Department of Radiation Oncology, London Health Sciences Center, London, Ontario (Canada); Ashman, Jonathan [Department of Radiation Oncology, Mayo Clinic Arizona, Phoenix, Arizona (United States); Wasif, Nabil, E-mail: wasif.nabil@mayo.edu [Department of General Surgery, Mayo Clinic Arizona, Phoenix, Arizona (United States)

    2017-02-01

    Purpose: Surgery combined with radiation therapy (RT) is the cornerstone of multidisciplinary management of extremity soft tissue sarcoma (STS). Although RT can be given in either the preoperative or the postoperative setting with similar local recurrence and survival outcomes, the side effect profiles, costs, and long-term functional outcomes are different. The aim of this study was to use decision analysis to determine optimal sequencing of RT with surgery in patients with extremity STS. Methods and Materials: A cost-effectiveness analysis was conducted using a state transition Markov model, with quality-adjusted life years (QALYs) as the primary outcome. A time horizon of 5 years, a cycle length of 3 months, and a willingness-to-pay threshold of $50,000/QALY was used. One-way deterministic sensitivity analyses were performed to determine the thresholds at which each strategy would be preferred. The robustness of the model was assessed by probabilistic sensitivity analysis. Results: Preoperative RT is a more cost-effective strategy ($26,633/3.00 QALYs) than postoperative RT ($28,028/2.86 QALYs) in our base case scenario. Preoperative RT is the superior strategy with either 3-dimensional conformal RT or intensity-modulated RT. One-way sensitivity analyses identified the relative risk of chronic adverse events as having the greatest influence on the preferred timing of RT. The likelihood of preoperative RT being the preferred strategy was 82% on probabilistic sensitivity analysis. Conclusions: Preoperative RT is more cost effective than postoperative RT in the management of resectable extremity STS, primarily because of the higher incidence of chronic adverse events with RT in the postoperative setting.

  9. Radiation planning comparison for superficial tissue avoidance in radiotherapy for soft tissue sarcoma of the lower extremity

    International Nuclear Information System (INIS)

    Griffin, Anthony M.; Euler, Colleen I.; Sharpe, Michael B.; Ferguson, Peter C.; Wunder, Jay S.; Bell, Robert S.; Chung, Peter W.M.; Catton, Charles N.; O'Sullivan, Brian

    2007-01-01

    Purpose: Three types of preoperative radiotherapy (RT) plans for extremity soft tissue sarcoma were compared to determine the amount of dose reduction possible to the planned surgical skin flaps required for tumor resection and wound closure, without compromising target coverage. Methods and Materials: Twenty-four untreated patients with large, deep, lower extremity STS treated with preoperative RT and limb salvage surgery had their original conventional treatment plans re-created. The same clinical target volume was used for all three plans. The future surgical skin flaps were created virtually through contouring by the treating surgeon and regarded as an organ at risk. The original, conformal, and intensity-modulated RT (IMRT) plans were created to deliver 50 Gy in 25 fractions to the clinical target volume. Clinical target volume and organ-at-risk dose-volume histograms were calculated and the plans compared for conformality, target coverage, and dose sparing. Results: The mean dose to the planned skin flaps was 42.62 Gy (range, 30.24-48.65 Gy) for the original plans compared with 40.12 Gy (range, 24.24-47.26 Gy) for the conformal plans and 26.71 Gy (range, 22.31-31.91 Gy) for the IMRT plans (p = 0.0008). An average of 86.4% (range, 53.2-97.4%) of the planned skin flaps received ≥30 Gy in the original plans compared with 83.4% (range, 36.2-96.2%) in the conformal plans and only 34.0% (range, 22.5-53.3%) in the IMRT plans (p = 0.0001). IMRT improved target conformality compared with the original and conformal plans (1.27, 2.34, and 1.76, respectively, p = 0.0001). Conclusion: In a retrospective review, preoperative IMRT substantially lowered the dose to the future surgical skin flaps, sparing a greater percentage of this structure's volume without compromising target (tumor) coverage

  10. Acute and long-term adverse effects of postoperative irradiation on limb function of combined modality limb sparing therapy for extermity of soft tissue sarcoma

    International Nuclear Information System (INIS)

    Glinski, B.; Dymek, P.; Walasek, T.; Pecak, M.; Rogowska, M.

    2008-01-01

    The aim of this study is to focus on the aspect of functional outcome for early and late normal tissues damage for patients undergoing postoperative radiotherapy after limb preservation surgery for extremity soft tissues sarcoma (ESTS). The review of the literature suggests that approximately 50% of patients with ESTS have significant impairments, but synthesis of the results is difficult because of the heterogeneity of outcomes used to evaluate function. Future studies require the use of standardized definitions, and reliable and functional outcome measures. (author)

  11. MRI guided needle localization in a patient with recurrence pleomorphic sarcoma and post-operative scarring

    Energy Technology Data Exchange (ETDEWEB)

    Chang, Ching-Di [Chang Gung University College of Medicine, Department of Radiology, Kaohsiung Chang Gung Memorial Hospital, Kaohsiung (China); Harvard Medical School, Department of Radiology, Beth Israel Deaconess Medical Center, Boston, MA (United States); Wei, Jesse; Wu, Jim S. [Harvard Medical School, Department of Radiology, Beth Israel Deaconess Medical Center, Boston, MA (United States); Goldsmith, Jeffrey D. [Harvard Medical School, Department of Pathology, Beth Israel Deaconess Medical Center, Boston, MA (United States); Gebhardt, Mark C. [Harvard Medical School, Department of Orthopedic Surgery, Beth Israel Deaconess Medical Center, Boston, MA (United States)

    2017-07-15

    MRI-guided wire localization is commonly used for surgical localization of breast lesions. Here we introduce an alternative use of this technique to help with surgical resection of a recurrent pleomorphic sarcoma embedded in extensive post-treatment scar tissue. We describe a case of recurrent pleomorphic soft tissue sarcoma in the thigh after treatment with neoadjuvant therapy, surgery, and radiation. Due to the distortion of the normal tissue architecture and formation of extensive scar tissue from prior treatment, wire localization under MRI was successfully used to assist the surgeon in identifying the recurrent tumor for removal. (orig.)

  12. Influence of site on the therapeutic ratio of adjuvant radiotherapy in soft-tissue sarcoma of the extremity

    International Nuclear Information System (INIS)

    Alektiar, Kaled M.; Brennan, Murray F.; Singer, Samuel

    2005-01-01

    Purpose: The ultimate goal of adjuvant radiotherapy (RT) in soft-tissue sarcoma of the extremity is to improve the therapeutic ratio by increasing local control while minimizing morbidity. Most efforts in trying to improve this ratio have focused on the sequencing of RT and surgery, with little attention to the potential influence of the tumor site. The purpose of this study was to determine the influence of tumor site on local control and complications in a group of patients with primary high-grade soft-tissue sarcoma of the extremity treated at a single institution with postoperative RT. Methods and Materials: Between July 1982 and December 2000, 369 adult patients with primary high-grade soft-tissue sarcoma of the extremity were treated with limb-sparing surgery and postoperative RT. Patients who underwent surgery or RT outside our institution were excluded. The tumor site was the upper extremity (UE) in 103 (28%) and the lower extremity (LE) in 266 (72%). The tumor was ≤5 cm in 98 patients (27%), and the microscopic margins were positive in 44 (12%). Of the 369 patients, 104 (28%) underwent postoperative external beam RT (EBRT), 233 (63%) postoperative brachytherapy (BRT), and 32 underwent a combination (9%); 325 (88%) received a 'conventional' radiation dose, defined as 60-70 Gy for EBRT, 45 Gy for BRT, and 45-50 Gy plus 15-20 Gy for EBRT plus BRT. Complications were assessed in terms of wound complications requiring repeat surgery, fracture, joint stiffness, edema, and Grade 3 or worse peripheral nerve damage. Results: The UE and LE groups were balanced with regard to age, depth, margin status, and type of RT (EBRT vs. BRT ± EBRT). However, more patients in the UE group had tumors ≤5 cm and more received a conventional radiation dose (p = 0.01 and P = 0.03, respectively). With a median follow-up of 50 months, the 5-year actuarial rate of local control, distant relapse-free survival, and overall survival for the whole population was 82% (95% confidence

  13. [Soft tissue sarcomas: the role of histology and molecular pathology for differential diagnosis].

    Science.gov (United States)

    Poremba, C

    2006-01-01

    Soft tissue sarcomas include a wide spectrum of different entities. The so-called small round blue cell tumors and spindle cell tumors are difficult to classify based solely on conventional histology. To identify different subtypes of tumors special histochemical and immunohistochemical techniques are necessary. Analysis of protein expression by immunohistochemistry provides a helpful tool to investigate the histogenesis of tumors. A basic spectrum of antibodies should be included to study these tumors: Desmin and myogenin (or MyoD1) for skeletal differentiation; S-100, NSE, CD56, and synaptophysin for neural/neuroendocrine differentiation; CD3, CD20, and CD79 alpha for malignant lymphomas; CD34, sm-actin, and beta-catenin for spindle cell tumors; additional antigens, e. g. Ki-67 and p 53, for estimation of proliferation and tumor suppressor gene malfunctions. Nevertheless, the molecular analysis of soft tissue sarcomas is necessary for demonstration of specific translocations or gene defects to specify and proof a diagnosis. For this purpose, RT-PCR for RNA expression analysis of gene fusion transcripts and multi-color FISH for analysis of chromosomal rearrangements are used. Further investigations, using DNA microrrays may help to subclassify such tumors, with respect to prognosis or prediction of therapeutic response.

  14. A Review: Molecular Aberrations within Hippo Signaling in Bone and Soft Tissue Sarcomas

    Directory of Open Access Journals (Sweden)

    Michael D Deel

    2015-09-01

    Full Text Available The Hippo signaling pathway is an evolutionarily conserved developmental network vital for the regulation of organ size, tissue homeostasis, repair and regeneration, and cell fate. The Hippo pathway has also been shown to have tumor suppressor properties. Hippo transduction involves a series of kinases and scaffolding proteins that are intricately connected to proteins in developmental cascades and in the tissue microenvironment. This network governs the downstream Hippo transcriptional coactivators YAP and TAZ, which bind to and activate the output of TEADs, as well as other transcription factors responsible for cellular proliferation, self-renewal, differentiation, and survival. Surprisingly, there are few oncogenic mutations within the core components of the Hippo pathway. Instead, dysregulated Hippo signaling is a versatile accomplice to commonly mutated cancer pathways. For example, YAP and TAZ can be activated by oncogenic signaling from other pathways, or serve as coactivators for classical oncogenes. Emerging evidence suggests that Hippo signaling couples cell density and cytoskeletal structural changes to morphogenic signals, and conveys a mesenchymal phenotype. While much of Hippo biology has been described in epithelial cell systems, it is clear that dysregulated Hippo signaling also contributes to malignancies of mesenchymal origin. This review will summarize the known molecular alterations within the Hippo pathway in sarcomas, and highlight how several pharmacologic compounds have shown activity in modulating Hippo components, providing proof-of-principle that Hippo signaling may be harnessed for therapeutic application in sarcomas.

  15. Kaposi's sarcoma involving the thyroid in a patient with AIDS

    International Nuclear Information System (INIS)

    Krauth, P.H.; Katz, J.F.

    1987-01-01

    A 30-year-old man with acquired immune deficiency syndrome (AIDS) and Kaposi's sarcoma had a palpable thyroid mass and cervical lymphadenopathy. Nuclear medicine and ultrasound scans revealed multiple thyroid nodules. Results of biopsy showed Kaposi's sarcoma metastatic to the thyroid

  16. Ten-Year Follow-Up of a Patient with Metastatic Ewing's Sarcoma of the Pelvis

    Directory of Open Access Journals (Sweden)

    Robert U. Ashford

    2002-01-01

    Full Text Available Patient: We report a 32-year-old women with a pelvic Ewing's sarcoma, who developed skeletal metastases within 20 months of diagnosis but following treatment remains disease-free at 10 years.

  17. Primary intracranial soft tissue sarcomas in children, adolescents, and young adults: single institution experience and review of the literature.

    Science.gov (United States)

    Maher, Ossama M; Khatua, Soumen; Mukherjee, Devashis; Olar, Adriana; Lazar, Alexander; Luthra, Raja; Liu, Diane; Wu, Jimin; Ketonen, Leena; Zaky, Wafik

    2016-03-01

    There is a paucity of literature reporting the outcome of intracranial sarcomas (IS) in children, adolescents, and young adults (CAYA). A multimodal therapeutic approach is commonly used, with no well-established treatment consensus. We conducted a retrospective review of CAYA with IS, treated at our institution, to determine their clinical findings, treatments, and outcomes. Immunohistochemistry (PDGFRA and EGFR) and DNA sequencing were performed on 5 tumor samples. A literature review of IS was also conducted. We reviewed 13 patients (median age, 7 years) with a primary diagnosis of IS between 1990 and 2015. Diagnoses included unclassified sarcoma (n = 9), chondrosarcoma (n = 2), and rhabdomyosarcoma (n = 2). Five patients underwent upfront gross total resection (GTR) of the tumor. The 5-drug regimen (vincristine, doxorubicin, cyclophosphamide, etoposide, and ifosfamide) was the most common treatment used. Nine patients died due to progression or recurrence (n = 8) or secondary malignancy (n = 1). The median follow-up period of the 4 surviving patients was 1.69 years (range 1.44-5.17 years). The 5-year progression-free survival and overall survival rates were 21 and 44 %, respectively. BRAF, TP53, KRAS, KIT, ERBB2, MET, RET, ATM, and EGFR mutations were detected in 4 of the 5 tissue samples. All 5 samples were immunopositive for PDGFRA, and only 2 were positive for EGFR. IS remain a therapeutic challenge due to high progression and recurrence rates. Collaborative multi-institutional studies are warranted to delineate a treatment consensus and investigate tumor biology to improve the disease outcome.

  18. Phase III trial of two investigational schedules of ifosfamide compared with standard-dose doxorubicin in advanced or metastatic soft tissue sarcoma: a European Organisation for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group Study

    NARCIS (Netherlands)

    Lorigan, Paul; Verweij, Jaap; Papai, Zsuzsa; Rodenhuis, Sjoerd; Le Cesne, Axel; Leahy, Michael G.; Radford, John A.; van Glabbeke, Martine M.; Kirkpatrick, Anne; Hogendoorn, Pancras C. W.; Blay, Jean-Yves

    2007-01-01

    PURPOSE: Single-agent doxorubicin remains the standard treatment for advanced soft tissue sarcomas. Combining doxorubicin with standard-dose ifosfamide has not been shown to improve survival and is associated with a significantly increased toxicity; it is not known whether higher dose single-agent

  19. Angiography of histopathologic variants of synovial sarcoma

    International Nuclear Information System (INIS)

    Lois, J.F.; Fischer, H.J.; Mirra, J.M.; Gomes, A.S.; California Univ., Los Angeles

    1986-01-01

    Synovial sarcomas are rare soft tissue tumors which histopathologically can be divided into monophasic, biphasic and mixed variants. As part of a protocol for intra-arterial chemotherapy 12 patients with biopsy proven synovial sarcoma underwent angiography. The angiograms on these patients were reviewed to determine whether synovial sarcomas and their variants demonstrated a characteristic angiographic appearance. Synovial sarcomas appeared angiographically as soft tissue masses which showed a fine network of tumor vessels with an inhomogeneous capillary blush. Their degree of vascularity varied according to their histopathology. Monophasic synovial sarcomas demonstrated in general a higher degree of neovascularity than the biphasic form. This finding was also suggested by histopathologic analysis of the vessels in the tumor. Although angiography did not show a distinctive vascular pattern it may be useful to evaluate tumor size and vascularity. (orig.)

  20. Radiographic appearance of Ewing sarcoma of the hands and feet: report from the Intergroup Ewing Sarcoma Study

    International Nuclear Information System (INIS)

    Reinus, W.R.; Gilula, L.A.; Shirley, S.K.; Askin, F.B.; Siegal, G.P.

    1985-01-01

    Review of current data from the Intergroup Ewing Sarcoma Study (IESS) shows that Ewing sarcoma is rare in bones of the hands and feet. The 12 patients from the IESS protocols with hand or foot Ewing sarcoma are comparable to those already reported in the literature. With the exception of lesions in the calcaneus, the prognosis for disease-free survival is excellent. The radiographic features of hand and foot Ewing sarcoma are generally those of classic Ewing sarcoma: permeation, soft-tissue mass, and often, associated sclerotic reaction. However, with the exception of sclerosis, features suggesting bone reaction and slow tumor growth in these patients were distinctly uncommon compared with Ewing sarcoma in general. Apparently location of the lesion is important, since in the reported cases in the literature and in this series, lesions of the calcaneus fared poorly. The importance of this set of patients therefore relates to awareness and early recognition of an unusual appearance and location of Ewing sarcoma

  1. Diagnostic value of 18F-FDG-PET/CT for the follow-up and restaging of soft tissue sarcomas in adults.

    Science.gov (United States)

    Kassem, T W; Abdelaziz, O; Emad-Eldin, S

    2017-10-01

    The purpose of this study was to evaluate the clinical utility of 2-[ 18 F] fluoro-2-deoxy-D-glucose ( 18 FDG) positron emission tomography (PET)/computed tomography (CT) ( 18 F-FDG-PET/CT) in the follow-up of adult patients with soft tissue sarcomas. We prospectively evaluated 37 consecutive patients with known soft tissue sarcoma with 18 F-FDG-PET/CT examination for suspected recurrence of disease. They were 21 men and 16 women with a mean age of 49.6±10.6 (SD) years (range, 34-75years). The sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) and accuracy of 18 F-FDG-PET/CT examination were calculated on a per patient basis. 18 F-FDG-PET/CT showed an overall diagnostic accuracy of 91.8%, sensitivity of 90% and a specificity of 100%. The positive predictive value and negative predictive value were 100 and 70%, respectively. The 18 F-FDG-PET/CT interpretations were correct in 34/37 patients (91.8%). Incorrect interpretations occurred in three patients (8.1%). Reasons for false negative findings were low 18 F-FDG uptake of local recurrence in one patient and low 18 F-FDG uptake of subcentimetric inguinal lymph node metastases. 18 F-FDG-PET/CT has a high diagnostic value in the follow-up of patients with soft tissue sarcoma. Copyright © 2017 Editions françaises de radiologie. Published by Elsevier Masson SAS. All rights reserved.

  2. Primary clear cell sarcoma of rib

    International Nuclear Information System (INIS)

    Hersekli, Murat Ali; Ozkoc, Gurkan; Akpinar, Sercan; Ozalay, Metin; Tandogan, Reha N.; Bircan, Sema; Tuncer, Ilhan

    2005-01-01

    Clear cell sarcoma (malignant melanoma of soft tissues) is a very rare soft tissue neoplasm. It generally arises in tendons and aponeuroses. Although metastasis of malignant melanoma to bone is not uncommon, primary clear cell sarcoma of bone is an extremely rare neoplasm. To our knowledge five cases have been reported in the English literature. We present a case of primary clear cell sarcoma of bone in a 28-year-old woman arising in the left ninth rib. We treated the patient with total excision of the mass and postoperative radiotherapy. The patient is alive and well without local recurrence or distant metastasis at 33 months after surgery. (orig.)

  3. Hypoxia-inducible factor 1α predicts recurrence in high-grade soft tissue sarcoma of extremities and trunk wall

    DEFF Research Database (Denmark)

    Nyström, H; Jönsson, M; Werner-Hartman, L

    2017-01-01

    BACKGROUND AND AIM: Sarcomas are of mesenchymal origin and typically show abundant tumour stroma and presence of necrosis. In search for novel biomarkers for personalised therapy, we determined the prognostic impact of stromal markers, hypoxia and neovascularity in high-grade soft tissue leiomyos...

  4. Recursive partitioning analysis (RPA) classification predicts survival in patients with brain metastases from sarcoma.

    Science.gov (United States)

    Grossman, Rachel; Ram, Zvi

    2014-12-01

    Sarcoma rarely metastasizes to the brain, and there are no specific treatment guidelines for these tumors. The recursive partitioning analysis (RPA) classification is a well-established prognostic scale used in many malignancies. In this study we assessed the clinical characteristics of metastatic sarcoma to the brain and the validity of the RPA classification system in a subset of 21 patients who underwent surgical resection of metastatic sarcoma to the brain We retrospectively analyzed the medical, radiological, surgical, pathological, and follow-up clinical records of 21 patients who were operated for metastatic sarcoma to the brain between 1996 and 2012. Gliosarcomas, sarcomas of the head and neck with local extension into the brain, and metastatic sarcomas to the spine were excluded from this reported series. The patients' mean age was 49.6 ± 14.2 years (range, 25-75 years) at the time of diagnosis. Sixteen patients had a known history of systemic sarcoma, mostly in the extremities, and had previously received systemic chemotherapy and radiation therapy for their primary tumor. The mean maximal tumor diameter in the brain was 4.9 ± 1.7 cm (range 1.7-7.2 cm). The group's median preoperative Karnofsky Performance Scale was 80, with 14 patients presenting with Karnofsky Performance Scale of 70 or greater. The median overall survival was 7 months (range 0.2-204 months). The median survival time stratified by the Radiation Therapy Oncology Group RPA classes were 31, 7, and 2 months for RPA class I, II, and III, respectively (P = 0.0001). This analysis is the first to support the prognostic utility of the Radiation Therapy Oncology Group RPA classification for sarcoma brain metastases and may be used as a treatment guideline tool in this rare disease. Copyright © 2014 Elsevier Inc. All rights reserved.

  5. Treatment Option Overview (Ewing Sarcoma)

    Science.gov (United States)

    ... Ewing Sarcoma Treatment Osteosarcoma Treatment Research Ewing Sarcoma Treatment (PDQ®)–Patient Version General Information About Ewing Sarcoma ... started or in another part of the body. Treatment Option Overview Key Points There are different types ...

  6. Preoperative evaluation and monitoring chemotherapy in patients with high-grade osteogenic and Ewing's sarcoma: review of current imaging modalities

    International Nuclear Information System (INIS)

    Woude, H.-J. van der; Bloem, J.L.; Hogendoorn, P.C.W.

    1998-01-01

    Diagnostic imaging is pivotal in the initial detection, characterization, staging and post-treatment follow-up of patients with high-grade osteogenic and Ewing's sarcoma. In the present review article, conventional and new imaging modalities are discussed with regard to the monitoring of the effect of neoadjuvant chemotherapy in such patients. Presurgical monitoring of response to chemotherapy may have an impact on modification of neoadjuvant treatment protocols, on patient selection for the performance and timing of limb-salvage surgery and on planning of radiation therapy (in non-operated Ewing's sarcomas) and selection of postoperative chemotherapy regimens. Dynamic contrast-enhanced MR imaging, as part of a routine MR protocol, assists in the detection of the most viable parts of the tumour and serves as an initial standard for follow-up of the metabolic activity of the tumour during and after chemotherapy, both in small intraosseous tumours and in tumours with an associated soft tissue mass. In combination with selected morphological features, dynamic imaging parameters are therefore advocated for monitoring the effect of neoadjuvant chemotherapy in patients with osteogenic and Ewing's sarcoma. (orig.)

  7. ALK-negative anaplastic large cell lymphoma mimicking a soft tissue sarcoma

    Directory of Open Access Journals (Sweden)

    Rachel Hudacko

    2011-01-01

    Full Text Available Anaplastic lymphoma kinase protein (ALK-negative anaplastic large cell lymphoma (ALCL has a vast morphologic spectrum and may mimic many other types of malignancies both cytologically and histologically. There are only a few published case reports/series describing the cytomorphologic features of ALCL on fine-needle aspiration (FNA biopsy specimens. We describe a case of ALK-negative ALCL mimicking a high-grade soft tissue sarcoma of the thigh in a 62-year-old man. The characteristic morphologic findings on FNA and core biopsy along with the immunophenotypic profile are described and reviewed. The diagnosis of ALCL on FNA biopsy may be difficult, but can be done successfully with the use of ancillary tests. Therefore, it must be considered in the differential diagnosis of lesions with pleomorphism, anaplasia, and wreath-like or horseshoe-shaped nuclei to ensure that adequate material is obtained for ancillary studies.

  8. Soft tissue sarcomas in the precision medicine era: new advances in clinical practice and future perspectives.

    Science.gov (United States)

    Badalamenti, Giuseppe; Messina, Carlo; De Luca, Ida; Musso, Emmanuela; Casarin, Alessandra; Incorvaia, Lorena

    2018-04-04

    Soft tissue sarcomas (STSs) represent a rare and heterogeneous group of solid tumours derived from mesenchymal progenitors and account for 1% of all adult malignancies. Although in the last decade anthracycline-based chemotherapy single agent or in combinations has been able to improve clinical benefits, prognosis is still poor and STSs represent an important unmet medical need. Continuous advances in cancer genetics and genomics have contributed to change management paradigms of STSs as it occurred for other solid tumours. Several treatments have been recently developed with the specific aim of targeting different cell pathways and immune-checkpoints that have been recognized to drive tumour progression. The following attempts to provide a review of literature focusing on the available data concerning novel treatments and future prospective for the management of metastatic STSs.

  9. Aleukemic granulocytic sarcoma presenting at multiple sites: ovary, breast and soft tissue

    Directory of Open Access Journals (Sweden)

    Jitendra Singh Nigam

    2012-06-01

    Full Text Available An 18 year old female presented with the history of pain in abdomen, breast engorgement, swelling over both legs and breathlessness for three month. On clinical examination diagnosis of fibroadenoma breast was made. Ultrasonography of abdomen showed bilateral ovarian mass. Bilateral salpingo-ophrectomy was done and specimen was sent for histological examination. Two lobulated solid masses of tissues the larger one measuring 13x8x5 cm and smaller one measuring 10x7x5 cm in size received. Microscopic examination showed monomorphic population of discohesive, hyperchromatic small round cells had high N:C ratio, coarse chromatin, conspicuous nucleoli and scant to moderate amount of basophilic cytoplasm, lying in sheets and separated by fibrous strands and diffusely infiltrating the ovarian stroma. Fine needle aspiration from breast lump and leg swelling showed predominant population of blast cells. Myeloperoxidase was strongly positive and diagnosis of granulocytic sarcoma was confirmed.

  10. Children’s Oncology Group’s 2013 Blueprint for Research: Soft Tissue Sarcomas

    Science.gov (United States)

    Hawkins, Douglas S.; Spunt, Sheri L.; Skapek, Stephen X.

    2013-01-01

    In the US, approximately 850-900 children are diagnosed each year with soft tissue sarcomas (STS). Key findings from recent clinical trials include safe reduction in therapy for low risk rhabdomyosarcoma (RMS), validation of FOXO1 fusion as a prognostic factor, a modest improvement in outcome for high-risk RMS, and a biologically-designed non-cytotoxic therapy for pediatric desmoid tumor. Planned Phase 2 trials include targeted agents for VEGF/PDGF, mTOR, and IGF-1R for children with RMS and VEGF for children with non-RMS STS (NRSTS). For RMS, COG Phase 3 trials potentially will explore VEGF/mTOR inhibition or chemotherapy interval compression. For NRSTS, a COG Phase 3 trial will explore VEGF inhibition. PMID:23255356

  11. Gross tumor volume and clinical target volume: soft-tissue sarcoma of the extremities

    International Nuclear Information System (INIS)

    Lartigau, E.; Kantor, G.; Lagarde, P.; Taieb, S.; Ceugnart, L.; Vilain, M.O.; Penel, N.; Depadt, G.

    2001-01-01

    Soft tissue sarcomas of the extremities are currently treated with more conservative and functional approaches, combining surgery, radiotherapy and chemotherapy. The role of external beam radiotherapy and brachytherapy has been defined through randomized studies performed in the 80's and 90's. However, the ubiquity of tumour location for these tumours makes difficult a systematic definition of local treatments. Tumour volume definition is based on pre and post surgical imaging (MRI) and on described pathological report. The clinical target volume will take into account quality of the resection and anatomical barriers and will be based on an anatomy and not only on safety margins around the tumour bed. General rules for this irradiation (doses, volumes) and principal results will be presented. (authors)

  12. Particle Therapy Using Protons or Carbon Ions for Unresectable or Incompletely Resected Bone and Soft Tissue Sarcomas of the Pelvis

    Energy Technology Data Exchange (ETDEWEB)

    Demizu, Yusuke, E-mail: y_demizu@nifty.com [Department of Radiology, Hyogo Ion Beam Medical Center, Tatsuno, Hyogo (Japan); Jin, Dongcun; Sulaiman, Nor Shazrina; Nagano, Fumiko; Terashima, Kazuki; Tokumaru, Sunao [Department of Radiology, Hyogo Ion Beam Medical Center, Tatsuno, Hyogo (Japan); Akagi, Takashi [Department of Radiation Physics, Hyogo Ion Beam Medical Center, Tatsuno, Hyogo (Japan); Fujii, Osamu [Department of Radiation Oncology, Hakodate Goryokaku Hospital, Hakodate, Hokkaido (Japan); Daimon, Takashi [Department of Biostatistics, Hyogo College of Medicine, Nishinomiya, Hyogo (Japan); Sasaki, Ryohei [Division of Radiation Oncology, Kobe University Graduate School of Medicine, Kobe, Hyogo (Japan); Fuwa, Nobukazu [Department of Radiation Oncology, Ise Red Cross Hospital, Ise, Mie (Japan); Okimoto, Tomoaki [Department of Radiology, Hyogo Ion Beam Medical Center, Tatsuno, Hyogo (Japan)

    2017-06-01

    Purpose: To retrospectively analyze the treatment outcomes of particle therapy using protons or carbon ions for unresectable or incompletely resected bone and soft tissue sarcomas (BSTSs) of the pelvis. Methods and Materials: From May 2005 to December 2014, 91 patients with nonmetastatic histologically proven unresectable or incompletely resected pelvic BSTSs underwent particle therapy with curative intent. The particle therapy used protons (52 patients) or carbon ions (39 patients). All patients received a dose of 70.4 Gy (relative biologic effectiveness) in 32 fractions (55 patients) or 16 fractions (36 patients). Results: The median patient age was 67 years (range 18-87). The median planning target volume (PTV) was 455 cm{sup 3} (range 108-1984). The histologic type was chordoma in 53 patients, chondrosarcoma in 14, osteosarcoma in 10, malignant fibrous histiocytoma/undifferentiated pleomorphic sarcoma in 5, and other in 9 patients. Of the 91 patients, 82 had a primary tumor and 9 a recurrent tumor. The median follow-up period was 32 months (range 3-112). The 3-year rate of overall survival (OS), progression-free survival (PFS), and local control was 83%, 72%, and 92%, respectively. A Cox proportional hazards model revealed that chordoma histologic features and a PTV of ≤500 cm{sup 3} were significantly associated with better OS, and a primary tumor and PTV of ≤500 cm{sup 3} were significantly associated with better PFS. Ion type and number of fractions were not significantly associated with OS, PFS, or local control. Late grade ≥3 toxicities were observed in 23 patients. Compared with the 32-fraction protocol, the 16-fraction protocol was associated with significantly more frequent late grade ≥3 toxicities (18 of 36 vs 5 of 55; P<.001). Conclusions: Particle therapy using protons or carbon ions was effective for unresectable or incompletely resected pelvic BSTS, and the 32-fraction protocol was effective and relatively less toxic. Nevertheless, a

  13. Rare Cause of Stricture Esophagus—Sarcoma: A Case Report and Review of the literature

    Directory of Open Access Journals (Sweden)

    S. Patricia

    2011-01-01

    Full Text Available Adenocarcinoma and squamous cell carcinoma account for the vast majority of oesophageal malignancies. Other malignancies known to occur in the oesophagus include melanoma, sarcoma, and lymphoma. Among the sarcomas, carcinosarcoma is the commonest with both carcinomatous and sarcomatous elements followed by leiomyosarcoma of mesenchymal origin. Other sarcomas reported in the literature are liposarcoma, synovial sarcoma, myxofibrosarcoma, Ewing's sarcoma, granulocytic sarcoma, histiocytic sarcoma, schwannoma rhabdomyosarcoma, and epithelioid sarcoma. We report a case of malignant spindle cell tumour of oesophagus. Sarcomas of esophagus present as a polypoid exophytic soft tissue mass. Our patient presented with a stricture which is a rare presentation. Locally aggressive treatment with surgery is beneficial, and local palliative treatment including radiotherapy is worthwhile.

  14. Analyzing the "CareGap": assessing gaps in adherence to clinical guidelines in adult soft tissue sarcoma.

    Science.gov (United States)

    Waks, Zeev; Goldbraich, Esther; Farkash, Ariel; Torresani, Michele; Bertulli, Rossella; Restifo, Nicola; Locatelli, Paolo; Casali, Paolo; Carmeli, Boaz

    2013-01-01

    Clinical decision support systems (CDSSs) are gaining popularity as tools that assist physicians in optimizing medical care. These systems typically comply with evidence-based medicine and are designed with input from domain experts. Nonetheless, deviations from CDSS recommendations are abundant across a broad spectrum of disorders, raising the question as to why this phenomenon exists. Here, we analyze this gap in adherence to a clinical guidelines-based CDSS by examining the physician treatment decisions for 1329 adult soft tissue sarcoma patients in northern Italy using patient-specific parameters. Dubbing this analysis "CareGap", we find that deviations correlate strongly with certain disease features such as local versus metastatic clinical presentation. We also notice that deviations from the guideline-based CDSS suggestions occur more frequently for patients with shorter survival time. Such observations can direct physicians' attention to distinct patient cohorts that are prone to higher deviation levels from clinical practice guidelines. This illustrates the value of CareGap analysis in assessing quality of care for subsets of patients within a larger pathology.

  15. Identification of tissue sites for increased albumin degradation in sarcoma-bearing mice

    International Nuclear Information System (INIS)

    Andersson, C.; Iresjoe, B.M.L.; Lundholm, K.

    1991-01-01

    Plasma albumin concentration declines in both experimental and clinical cancer. Previous investigations have demonstrated that this is partly explained by increased breakdown of albumin. The present study has identified the tissue sites for increased albumin degradation in a nonmetastasizing sarcoma mouse (C57/BL6J) model. Results have been compared to nontumor-bearing animals either freely fed or food restricted (pair-weighed) so that their body composition was similar to tumor-bearing animals. Tumor-bearing mice had increased albumin degradation (0.13 +/- 0.02 mg/hr/g bw) compared to both freely fed (0.09 +/- 0.007) and pair-weighed control animals (0.05 +/- 0.008). Radioactivity from circulating [3H]raffine aldehyde labeled albumin appeared with maximum peak values in lysosomes isolated from both tumor and nontumor tissues at 48 hr following iv injection. The intralysosomal accumulation of radioactivity was two- to threefold higher in tumor tissue compared to liver tissue, although the specific activity of protease(s) for albumin degradation measured in vitro was not higher in tumor tissue (30.4 +/- 3.6 mg/hr/g tissue) compared to normal liver tissue (36.9 +/- 1.7). Accounting for the entire tumor the proteolytic capacity for albumin breakdown was however much larger in the tumor (161.6 +/- 32.6 mg/organ) compared to both normal liver (37.5 +/- 2.3) and tumor-host liver (56.4 +/- 2.8). Pepstatin inhibited 78 +/- 6% of the proteolytic activity in the tumor measured by 125I-labeled undenatured mouse albumin as the substrate. Leupeptin inhibited 49 +/- 6%. There was a significantly decreased breakdown of albumin in both skeletal muscles and the gastrointestinal tract from tumor-bearing animals

  16. Optically-tracked handheld fluorescence imaging platform for monitoring skin response in the management of soft tissue sarcoma

    Science.gov (United States)

    Chamma, Emilie; Qiu, Jimmy; Lindvere-Teene, Liis; Blackmore, Kristina M.; Majeed, Safa; Weersink, Robert; Dickie, Colleen I.; Griffin, Anthony M.; Wunder, Jay S.; Ferguson, Peter C.; DaCosta, Ralph S.

    2015-07-01

    Standard clinical management of extremity soft tissue sarcomas includes surgery with radiation therapy. Wound complications (WCs) arising from treatment may occur due to bacterial infection and tissue breakdown. The ability to detect changes in these parameters during treatment may lead to earlier interventions that mitigate WCs. We describe the use of a new system composed of an autofluorescence imaging device and an optical three-dimensional tracking system to detect and coregister the presence of bacteria with radiation doses. The imaging device visualized erythema using white light and detected bacterial autofluorescence using 405-nm excitation light. Its position was tracked relative to the patient using IR reflective spheres and registration to the computed tomography coordinates. Image coregistration software was developed to spatially overlay radiation treatment plans and dose distributions on the white light and autofluorescence images of the surgical site. We describe the technology, its use in the operating room, and standard operating procedures, as well as demonstrate technical feasibility and safety intraoperatively. This new clinical tool may help identify patients at greater risk of developing WCs and investigate correlations between radiation dose, skin response, and changes in bacterial load as biomarkers associated with WCs.

  17. The Prognostic Value of Serum Biomarkers in Localized Bone Sarcoma

    DEFF Research Database (Denmark)

    Aggerholm-Pedersen, Ninna; Maretty-Kongstad, Katja; Keller, Johnny

    2016-01-01

    sarcoma were included. Of these patients, 63 were diagnosed with chondrosarcoma and 109 patients with Ewing/osteosarcoma. The median age was 55 years for chondrosarcoma and 19 years for Ewing/osteosarcoma patients. The overall 5-year mortality was 31% [95% confidence interval (CI): 21-44] and 41% (95% CI......OBJECTIVE: Certain biomarkers such as the C-reactive protein, serum albumin, and the neutrophils to lymphocyte ratio are of prognostic significance regarding survival in different types of cancers. Data from sarcoma patients are sparse and mainly derived from soft tissue sarcoma and/or metastatic...... with localized bone sarcomas and to adjust for potential confounders. MATERIAL AND METHODS: All patients diagnosed with localized intermediate and high-grade bone sarcoma during 1994 to 2008 were extracted from the Aarhus Sarcoma Registry. The serum levels of albumin, C-reactive protein, hemoglobin, neutrophils...

  18. Complex surgery for locally advanced bone and soft tissue sarcomas of the shoulder girdle.

    Science.gov (United States)

    Lesenský, Jan; Mavrogenis, Andreas F; Igoumenou, Vasilios G; Matejovsky, Zdenek; Nemec, Karel; Papagelopoulos, Panayiotis J; Fabbri, Nicola

    2017-08-01

    Surgical management of primary musculoskeletal tumors of the shoulder girdle is cognitively and technically demanding. Over the last decades, advances in the medical treatments, imaging and surgical techniques have fostered limb salvage surgery and reduced the need for amputation. Despite well-accepted general principles, an individualized approach is often necessary to accommodate tumor extension, anatomical challenges and patient characteristics. A combination of techniques is often required to achieve optimal oncologic and durable functional outcome. Goal of this article is to review approach and management of patients with locally advanced sarcomas of the shoulder girdle requiring major tumor surgery, to illustrate principles of surgical strategy, outcome and complications, and to provide useful guidelines for the treating physicians.

  19. Non-Rhabdomyosarcoma Soft Tissue Sarcomas in Children: A Surveillance, Epidemiology, and End Results Analysis Validating COG Risk Stratifications

    Energy Technology Data Exchange (ETDEWEB)

    Waxweiler, Timothy V., E-mail: timothy.waxweiler@ucdenver.edu [Department of Radiation Oncology, University of Colorado School of Medicine, Aurora, Colorado (United States); Rusthoven, Chad G. [Department of Radiation Oncology, University of Colorado School of Medicine, Aurora, Colorado (United States); Proper, Michelle S. [Department of Radiation Oncology, Billings Clinic, Billings, Montana (United States); Cost, Carrye R. [Division of Hematology and Oncology, Department of Pediatrics, University of Colorado Denver School of Medicine, Aurora, Colorado (United States); Cost, Nicholas G. [Division of Urology, Department of Surgery, University of Colorado Denver School of Medicine, Aurora, Colorado (United States); Donaldson, Nathan [Department of Orthopedics, University of Colorado Denver School of Medicine, Aurora, Colorado (United States); Garrington, Timothy; Greffe, Brian S. [Division of Hematology and Oncology, Department of Pediatrics, University of Colorado Denver School of Medicine, Aurora, Colorado (United States); Heare, Travis [Department of Orthopedics, University of Colorado Denver School of Medicine, Aurora, Colorado (United States); Macy, Margaret E. [Division of Hematology and Oncology, Department of Pediatrics, University of Colorado Denver School of Medicine, Aurora, Colorado (United States); Liu, Arthur K. [Department of Radiation Oncology, University of Colorado School of Medicine, Aurora, Colorado (United States)

    2015-06-01

    Purpose: Non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) are a heterogeneous group of sarcomas that encompass over 35 histologies. With an incidence of ∼500 cases per year in the United States in those <20 years of age, NRSTS are rare and therefore difficult to study in pediatric populations. We used the large Surveillance, Epidemiology, and End Results (SEER) database to validate the prognostic ability of the Children's Oncology Group (COG) risk classification system and to define patient, tumor, and treatment characteristics. Methods and Materials: From SEER data from 1988 to 2007, we identified patients ≤18 years of age with NRSTS. Data for age, sex, year of diagnosis, race, registry, histology, grade, primary size, primary site, stage, radiation therapy, and survival outcomes were analyzed. Patients with nonmetastatic grossly resected low-grade tumors of any size or high-grade tumors ≤5 cm were considered low risk. Cases of nonmetastatic tumors that were high grade, >5 cm, or unresectable were considered intermediate risk. Patients with nodal or distant metastases were considered high risk. Results: A total of 941 patients met the review criteria. On univariate analysis, black race, malignant peripheral nerve sheath (MPNST) histology, tumors >5 cm, nonextremity primary, lymph node involvement, radiation therapy, and higher risk group were associated with significantly worse overall survival (OS) and cancer-specific survival (CSS). On multivariate analysis, MPNST histology, chemotherapy-resistant histology, and higher risk group were significantly poor prognostic factors for OS and CSS. Compared to low-risk patients, intermediate patients showed poorer OS (hazard ratio [HR]: 6.08, 95% confidence interval [CI]: 3.53-10.47, P<.001) and CSS (HR: 6.27; 95% CI: 3.44-11.43, P<.001), and high-risk patients had the worst OS (HR: 13.35, 95% CI: 8.18-21.76, P<.001) and CSS (HR: 14.65, 95% CI: 8.49-25.28, P<.001). Conclusions: The current COG risk group

  20. Tissue characteristics of high- and low-incidence plutonium-induced osteogenic sarcoma sites in life-span beagles

    International Nuclear Information System (INIS)

    Miller, S.C.; Jee, W.S.S.; Smith, J.M.; Wronski, T.J.

    1986-01-01

    On the basis of information gathered from the 239 Pu life-span study in beagles at the University of Utah, the tissue features were found to be characteristic of high-incidence bone-tumor sites compared to low-incidence sites included more hematopoietic tissues in the bone marrow; greater trabecular bone mass; greater bone remodeling rates; greater mineral apposition rates; greater density and activity of bone surface cells; greater density of putative bone-cell precursors; greater initial uptake of plutonium on bone surfaces; and greater marrow vascular volumes and a venous sinusoidal bed. Although most of these studies are not yet complete, the information being collected should contribute to our understanding of the mechanisms of radiation-induced osteogenic sarcomas. This should aid in predicting the types and characteristics of osseous tissues where radiation-induced osteogenic sarcomas may arise in humans. 25 refs., 4 figs., 3 tabs

  1. MRI findings associated with microscopic residual tumor following unplanned excision of soft tissue sarcomas in the extremities

    Energy Technology Data Exchange (ETDEWEB)

    Wang, Lee; Chelala, Lydia; Jose, Jean; Subhawong, Ty K. [University of Miami Miller School of Medicine/Jackson Memorial Hospital, Department of Radiology, Miami, FL (United States); Pretell-Mazzini, Juan [University of Miami Miller School of Medicine, Department of Orthopaedic Surgery, Division of Musculoskeletal Oncology, Miami, FL (United States); Kerr, Darcy A. [University of Miami Miller School of Medicine, Department of Pathology, Miami, FL (United States); Yang, Xuan [University of Miami Miller School of Medicine, Department of Public Health Sciences, Miami, FL (United States)

    2018-02-15

    MRI is often used to determine the presence of residual disease following unplanned excisions (UPE) of soft tissue sarcomas (STS). We sought to identify MRI features associated with histologic evidence of residual disease after TBE. This was an IRB-approved retrospective review of 27 patients with R1-type UPE of STS over a 32-month period, with subsequent MRI and TBE. MRI studies were retrospectively evaluated to determine depth of tissue involvement, presence of nodular enhancement, and maximum length of soft tissue edema normalized to extremity size. MRI findings were correlated with histology from unplanned excision and TBE. Among the 21 subjects, there were 13 males and 8 females, mean age 58. Eighteen of 21 STS were grade 2 or 3. Deep compartments were involved in 5/21 cases. Original margins were positive in 17/21 UPE, with inadequate margin assessment in the remaining 4 cases. Residual tumor was present at TBE in 11/21 cases; it was found in 4/6 cases with nodular enhancement and 7/15 cases without nodular enhancement (sensitivity = 0.36; specificity = 0.80; PPV = 0.67; NPV = 0.53). Increased extent of soft tissue edema increased the likelihood of residual tumor at TBE (OR = 35.0; 95% CI = 1.6 to 752.7; p = 0.023). Nodular enhancement is neither sensitive nor specific in predicting residual microscopic tumor in TBE following UPE. Extensive soft tissue edema on MRI after UPE increases the likelihood of finding a residual microscopic tumor, justifying ample margins at TBE and consideration of adjuvant therapy. (orig.)

  2. Three-Dimensional Patient-Derived In Vitro Sarcoma Models: Promising Tools for Improving Clinical Tumor Management

    Directory of Open Access Journals (Sweden)

    Manuela Gaebler

    2017-09-01

    Full Text Available Over the past decade, the development of new targeted therapeutics directed against specific molecular pathways involved in tumor cell proliferation and survival has allowed an essential improvement in carcinoma treatment. Unfortunately, the scenario is different for sarcomas, a group of malignant neoplasms originating from mesenchymal cells, for which the main therapeutic approach still consists in the combination of surgery, chemotherapy, and radiation therapy. The lack of innovative approaches in sarcoma treatment stems from the high degree of heterogeneity of this tumor type, with more that 70 different histopathological subtypes, and the limited knowledge of the molecular drivers of tumor development and progression. Currently, molecular therapies are available mainly for the treatment of gastrointestinal stromal tumor, a soft-tissue malignancy characterized by an activating mutation of the tyrosine kinase KIT. Since the first application of this approach, a strong effort has been made to understand sarcoma molecular alterations that can be potential targets for therapy. The low incidence combined with the high level of histopathological heterogeneity makes the development of clinical trials for sarcomas very challenging. For this reason, preclinical studies are needed to better understand tumor biology with the aim to develop new targeted therapeutics. Currently, these studies are mainly based on in vitro testing, since cell lines, and in particular patient-derived models, represent a reliable and easy to handle tool for investigation. In the present review, we summarize the most important models currently available in the field, focusing in particular on the three-dimensional spheroid/organoid model. This innovative approach for studying tumor biology better represents tissue architecture and cell–cell as well as cell–microenvironment crosstalk, which are fundamental steps for tumor cell proliferation and survival.

  3. [Histopathological diagnostic concordance in bone and soft tissue sarcomas between two comprehensive cancer centers from eastern and western Europe: a collaborative experience].

    Science.gov (United States)

    Somcutian, Oana; Buiga, Rares; Galatir, Mihaela; Tudor Eniu, Dan; Rachieru, Claudiu; Coza, Daniela; Terrier, Philippe

    2015-01-01

    This study aims to assess the degree of concordance of histological diagnosis of bone and soft tissue sarcomas between a Comprehensive Cancer Center (CCC) of Eastern Europe - not specialized in this area of pathology - and an important CCC of Western Europe, which is one of the coordinators of a clinical reference network in sarcoma pathology. The goal is to have an overview of the sarcomatous pathology in a region of Eastern Europe and to discover diagnostic discrepancies between the two centers, while determining their cause. The initial diagnosis was compared with the revised diagnosis on 110 specimens from 88 patients with bone or soft tissue sarcomas from East-European CCC, in a one-year period of time. Complete diagnostic agreement was observed in 55 cases (62.5%), a partial agreement in 23 cases (26.1%) and a major disagreement in 10 cases (11.4%). Major discrepancies of the histological type was observed in only 3 cases (3.4%): one case of discordance benign/malignant and 2 cases of discordance mesenchymal/non mesenchymal. Minor histological discrepancies - not affecting the management of the patient - were observed in 18 cases (20.4%). A major discordance in grading - potentially changing the management of the patient - was noted in 7 cases (7.9%), and a minor discrepancy in 5 cases (5.7%). Some histological types were clearly overdiagnosed, like "adult fibrosarcomas" and "malignant peripheral nerve sheet tumors" (MPNST), mostly converted after the audit into "undifferentiated spindle cell sarcomas" or other types of sarcomas. Some "unclassified" sarcomas and "undifferentiated pleomorphic sarcomas" could be re-classified with the aid of an extensive panel of antibodies. Overall, immunohistochemistry was responsible, but not in exclusivity, for half of the minor discrepancies, and for 2 out of 3 cases of major histological discrepancies. Otherwise, the main cause of discrepancies was the difficulties in the interpretation of the morphology. Molecular

  4. High expression of CD109 antigen regulates the phenotype of cancer stem-like cells/cancer-initiating cells in the novel epithelioid sarcoma cell line ESX and is related to poor prognosis of soft tissue sarcoma.

    Directory of Open Access Journals (Sweden)

    Makoto Emori

    Full Text Available Epithelioid sarcoma (ES is a relatively rare, highly malignant soft tissue sarcoma. The mainstay of treatment is resection or amputation. Currently other therapeutic options available for this disease are limited. Therefore, a novel therapeutic option needs to be developed. In the present study, we established a new human ES cell line (ESX and analyzed the characteristics of its cancer stem-like cells/cancer-initiating cells (CSCs/CICs based on ALDH1 activity. We demonstrated that a subpopulation of ESX cells with high ALDH1 activity (ALDH(high cells correlated with enhanced clonogenic ability, sphere-formation ability, and invasiveness in vitro and showed higher tumorigenicity in vivo. Next, using gene expression profiling, we identified CD109, a GPI-anchored protein upregulated in the ALDH(high cells. CD109 mRNA was highly expressed in various sarcoma cell lines, but weakly expressed in normal adult tissues. CD109-positive cells in ESX predominantly formed spheres in culture, whereas siCD109 reduced ALDH1 expression and inhibited the cell proliferation in vitro. Subsequently, we evaluated the expression of CD109 protein in 80 clinical specimens of soft tissue sarcoma. We found a strong correlation between CD109 protein expression and the prognosis (P = 0.009. In conclusion, CD109 might be a CSC/CIC marker in epithelioid sarcoma. Moreover, CD109 is a promising prognostic biomarker and a molecular target of cancer therapy for sarcomas including ES.

  5. Interleukin-6 concentrations in the serum of patients with AIDS-associated Kaposi's sarcoma during treatment with interferon-alpha

    NARCIS (Netherlands)

    de Wit, R.; Raasveld, M. H.; ten Berge, R. J.; van der Wouw, P. A.; Bakker, P. J.; Veenhof, C. H.

    1991-01-01

    Interleukin-6 (IL-6) levels were determined in the serum of 14 HIV-1-infected patients with Kaposi's sarcoma, 10 HIV-1-infected patients without symptoms, and 10 healthy male subjects. IL-6 levels were also determined in the serum of the 14 patients with Kaposi's sarcoma during treatment with

  6. Ewing's sarcoma, a rare but dangerous tumor

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    Theophilus Maksha Dabkana

    2015-01-01

    Full Text Available Ewing's sarcoma or Ewing tumor is a rare primary bone tumor that affects mainly children and adolescents. It belongs to a group of cancers known collectively as Ewing sarcoma family tumors or Ewing family tumors. By the time, the patients present and diagnosis is made, the disease is usually advanced. We reviewed the case files of two patients managed in our hospital within one (2013. Fine-needle aspirations for cytology (FNAC and tissue biopsy were used for diagnosis in the two patients we had. The two patients, both males aged 20 years and 38 years presented late and FNAC and tissue biopsy revealed Ewing's sarcoma. They were referred for radio- and chemotherapy. However, due to poor socioeconomic status, they died of their primary disease. Unless diagnosed early, and in the absence of a multidisciplinary approach, Ewing's sarcoma is a fatal disease.

  7. Blue Cell Tumour at Unusual Site: Retropritoneal Ewings Sarcoma

    OpenAIRE

    Javalgi, Anita P; Karigoudar, Mahesh H; Palur, Katyayani

    2016-01-01

    Ewing’s sarcoma is a highly malignant tumour of osseous or non-osseous origin, tremed as extra-skeletal Ewings sarcoma if arising from soft tissue. It is rare occurrence tumor most commonly occurring in paravertebral area, chest wall, head & neck and retroperitoneum. Reporting an interesting case of retroperitoneal Ewing’s sarcoma in 39 years old female. Patient had complains of abdominal discomfort & vague pain since 2 months, following weakness in lower limb and loss of weight. On detail hi...

  8. Limb sparing approach: Adjuvant radiation therapy in adults with intermediate or high-grade limb soft tissue sarcoma

    International Nuclear Information System (INIS)

    Merimsky, Ofer; Soyfer, Vjacheslav; Kovner, Felix; Bickels, Jacob; Issakov, Josephine; Flusser, Gideon; Meller, Isaac; Ofer, Oded; Kollender, Yehuda

    2005-01-01

    Background: Limb soft tissue sarcomas (STS) are currently treated with limb sparing surgery (LSS) followed by radiation therapy (RT). Patients and methods: Between October 1994 and October 2002, 133 adult patients with intermediate or high-grade limb STS were approached by LSS+RT. Results: RT related toxicity was manageable, with a low rate of severe effects. At 4-year median follow-up, there were 48 recurrences of any type, 23 of isolated local failure, and 35 of systemic spread w/o local failure. DFS and OS were influenced by disease stage II vs I, primary site in the upper limb vs lower limb, MPNST vs other types, induction therapy vs no induction, adequate resection vs marginal resection or involved margins, and good response to induction therapy vs bad response. DFS and OS were Patient's age and sex, tumor depth, acute or late toxicity of RT, or the interval of time between the date of definitive surgery and the start of RT did not affect DFS and or OS. Conclusions: The RT protocol is applicable in the era of complicated, expensive and time-consuming 3D therapy. Our results of LSS+RT in adults with limb HG STS are satisfactory

  9. Maxillary Sinus Kaposi Sarcoma: Case Report in an HIV-Negative Patient with Thymoma

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    Bernardo Carvalho Araújo

    2017-01-01

    Full Text Available Introduction. Kaposi sarcoma is an angioproliferative disorder that requires infection with human herpesvirus 8 (HHV-8 for its development. The majority of cases are associated with HIV infection or other immunocompromising conditions. Thymomas are occasionally associated to cytopenia, which may alter the patients’ immune responses. Methods. Case report using clinical records. Results. Case report of a 46-year-old male patient diagnosed with thymoma and myasthenia gravis. The patient was referred to an otolaryngology consultation with complaints of facial pain in the right malar region, interpreted as an acute sinusitis. Following examination, an expansive maxillary sinus mass was found, and endoscopic surgery was undertaken. After careful investigation, it was diagnosed as a Kaposi sarcoma. Conclusions. It is thought to be the first described case of a maxillary sinus Kaposi sarcoma in an HIV-negative patient. Thus, this entity has to be considered in the differential diagnosis of sinus masses, even in non-HIV patients.

  10. Preoperative therapy with pazopanib in high-risk soft tissue sarcoma: a phase II window-of-opportunity study by the German Interdisciplinary Sarcoma Group (GISG-04/NOPASS).

    Science.gov (United States)

    Ronellenfitsch, Ulrich; Dimitrakopoulou-Strauss, Antonia; Jakob, Jens; Kasper, Bernd; Nowak, Kai; Pilz, Lothar R; Attenberger, Ulrike; Gaiser, Timo; Egerer, Gerlinde; Fröhling, Stefan; Derigs, Hans-Günter; Schwarzbach, Matthias; Hohenberger, Peter

    2016-01-06

    For resectable soft tissue sarcoma (STS), radical surgery, usually combined with radiotherapy, is the mainstay of treatment and the only potentially curative modality. Since surgery is often complicated by large tumour size and extensive tumour vasculature, preoperative treatment strategies with the aim of devitalising the tumour are being explored. One option is treatment with antiangiogenic drugs. The multikinase inhibitor pazopanib, which possesses pronounced antiangiogenic effects, has shown activity in metastatic and unresectable STS, but has so far not been tested in the preoperative setting. This open-label, multicentre phase II window-of-opportunity trial assesses pazopanib as preoperative treatment of resectable STS. Participants receive a 21-day course of pazopanib 800 mg daily during wait time for surgery. Major eligibility criteria are resectable, high-risk adult STS of any location, or metachronous solitary STS metastasis for which resection is planned, and adequate organ function and performance status. The trial uses an exact single-stage design. The primary end point is metabolic response rate (MRR), that is, the proportion of patients with >50% reduction of the mean standardised uptake value (SUVmean) in post-treatment compared to pre-treatment fluorodeoxyglucose positron emission tomography CT. The MRR below which the treatment is considered ineffective is 0.2. The MRR above which the treatment warrants further exploration is 0.4. With a type I error of 5% and a power of 80%, the sample size is 35 evaluable patients, with 12 or more responders as threshold. Main secondary end points are histopathological and MRI response, resectability, toxicity, recurrence-free and overall survival. In a translational substudy, endothelial progenitor cells and vascular epithelial growth factor receptor are analysed as potential prognostic and predictive markers. Approval by the ethics committee II, University of Heidelberg, Germany (2012-019F-MA), German Federal

  11. No evidence of increased risk of soft tissue sarcomas in the neighborhood of a steel foundry in Verona

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    Marta Benedetti

    2010-12-01

    Full Text Available The aim of the present study was to evaluate the possible association between occurrence of soft tissue sarcomas in Verona (Italy and residence near a steel foundry, whose emissions of dioxin-like compounds may be relevant. Exposure to total suspended particulate (TSP emitted from the plant as estimated by ADMS-urban dispersion model was used as an indirect index of exposure to dioxin-like compounds. Verona municipality was divided in six subareas according to the decreasing levels of estimated TSP exposure, and soft tissue sarcomas cases were mapped according to residence at time of diagnosis. Standardized incidence ratios were computed. No statistically significant departures between observed and expected cases were detected as illustrated by trend-test results.

  12. Comparison of MRI and PET-CT in detecting the loco-regional recurrence of soft tissue sarcomas during surveillance

    Energy Technology Data Exchange (ETDEWEB)

    Park, Sun-Young [University of Ulsan College of Medicine, Asan Medical Center, Department of Radiology and Research Institute of Radiology, 88, Olympic-ro 43-gil, Songpa-gu, Seoul (Korea, Republic of); Hallym University Sacred Heart Hospital, Department of Radiology, Anyang-si, Gyeonggi-do (Korea, Republic of); Chung, Hye Won [University of Ulsan College of Medicine, Asan Medical Center, Department of Radiology and Research Institute of Radiology, 88, Olympic-ro 43-gil, Songpa-gu, Seoul (Korea, Republic of); Chae, Sun Young [University of Ulsan College of Medicine, Asan Medical Center, Department of Nuclear Medicine, 88, Olympic-ro 43-gil, Songpa-gu, Seoul (Korea, Republic of); Lee, Jong-Seok [University of Ulsan College of Medicine, Asan Medical Center, Department of Orthopedic Surgery, 88, Olympic-ro 43-gil, Songpa-gu, Seoul (Korea, Republic of)

    2016-10-15

    To investigate the diagnostic performance of MRI and PET-CT for the detection of loco-regional recurrences after soft tissue sarcoma (STS) excision. From Dec 2003 to Aug 2014, 394 patients with STSs, who were included in the electronic patient registry for initial or repeated surgery at our hospital, were retrospectively reviewed. We identified 152 patients who underwent regular postoperative follow-ups with both MRI and PET-CT, obtained within a 3 month period of each other. We analyzed differences in the performance of MRI and PET-CT for the diagnosis of loco-regional recurrences using McNemar's test. The receiver-operating characteristic curves and calculations of the area under the curve were used. Twenty patients were found to have a loco-regional recurrence after tumor excision. For MRI and PET-CT, the sensitivities were 90.0 and 95.0 %, and the specificities 97.7 and 95.5 %, respectively, with positive predictive values of 85.7 and 76.0 % and negative predictive values of 98.5 and 99.2 %, respectively. No significant difference was detected between the sensitivities of MRI and PET-CT (p = 0.125). The area under the receiver-operating characteristic curve for PET-CT (0.952) was not significantly greater than that for MRI (0.939; p = 0.6). MRI of the area of interest is recommended for evaluation of tumor recurrence after surgical excision of STS. PET-CT was shown to be effective for detection of STS recurrence, and comparable to MRI. However, if PET-CT or MRI findings are inconclusive, the other modality may be helpful in differentiating tumor recurrence from post-therapeutic tissue change. (orig.)

  13. Predictive factors for complete removal in soft tissue sarcomas: a retrospective analysis in a series of 592 cases.

    Science.gov (United States)

    Sastre-Garau, X; Coindre, J M; Leroyer, A; Terrier, P; Ollivier, L; Stöckle, E; Bonichon, F; Collin, F; Le Doussal, V; Contesso, G; Vilain, M O; Jacquemier, J; Nguyen, B B

    1997-07-01

    In order to specify the indications for conservative surgery and preoperative therapeutic approaches of soft tissues sarcomas (STS), we looked for the clinico-pathological parameters associated with the failure to obtain a complete removal (CRm) of the tumor. We retrospectively analyzed a series of 592 cases of primary non-metastatic STS. Surgery was performed in 495 cases as a primary treatment and in 88 cases after chemo- or radiotherapy. Nine patients were treated by chemotherapy-radiotherapy. In a univariate analysis, 20 parameters were tested for their association with CRm. A multivariate analysis was then used to define the independent parameters linked to the achievement of a CRm. In the univariate analysis, 15 parameters were found to be linked to the achievement of a CRm. Three of them proved to be independent in the multivariate analysis: T in the TNM classification, tumor location, and tumor necrosis. By the combination of these risk factors, four groups of patients were defined, with respective rates of CRm of 97% (no factor), 95% (one factor), 70% (two factors), and 48% (three factors). The achievement of a CRm after surgery of STS depends not only on the accessibility of the lesion, but also on tumor aggressiveness, a reflection of which is necrosis. The detection of necrosis by imaging procedures may thus help predicting the resectability of tumors and defining the indications for neoadjuvant therapies, likely to broaden the use of conservative surgery.

  14. Limb sparing surgery and boost with high dose rate interstitial brachytherapy in treatment of soft tissue sarcoma

    International Nuclear Information System (INIS)

    Koike, P.; Miziara, M.; Soares, C.; Fogaroli, R.; Baraldi, H.; Pellizoni, A.; Borba, G.

    2003-01-01

    Soft tissue sarcoma, a rare neoplasia with high aggressively, accounts for approximately 0,7% of the malignant tumors and occurs more often with youngest. Because of the potential risk of local recurrence, theoretically surgical resection, encompassing macroscopic tumor with a margin of macroscopically noninvolved tissue is the right way to perform, with a wide 'en bloc' resection, amputation, with bad functional results. The aim of conservative treatment is combined modality therapy as surgical resection and irradiation to obtain a local control rate as high as possible while preserving functional results. A retrospective review of 31 patients treated with high dose rate (HDR) Brachytherapy in the Radiotherapy Department Arnaldo Vieira de Carvalho Cancer Institute. Methods: Between April 1995 and August 1999, 31 patients who underwent a combine therapy; examined the results on multivariate analysis of conservative surgery and brachytherapy follow/or not by external beam radiation (EBRT). The 31 patients treated, 17 ( 54,8%) females and 14(45,2%) males have a median age of 48 years ( range,19 to 77 years). Most of the tumors was located in the lower limb (17/31 - 54,8%) . The other sites were the upper limb (10/31-32,3%), thoracic wall and abdomen (3/31-9,7%).Classification of the tumors, according to the International Union Against Cancer (UICC) staging was T1 5 patients (16%), T2 (24/31-77%). Median size of the tumors was 9,2cm ( ranged, 2,5 to 24cm). Most of the tumors being malignant fibrous histiocytomas (9/31-29%) and the histological grade II (14/31-45%). Twenty-two (71%) patients had intraoperative implants and the insertion of the radioactive source was delayed 24 to 120 hours. Eight patients (25,8%) had postoperative and received HDRB 45 to 60 days after the surgery . Guide needles were placed in the tumor bed, perpendicular to the scar, systematically in a single plane, the implant volume being defined by radiotherapist . A minimum safety margin of 2 cm

  15. Quality of Life Following Amputation or Limb Preservation in Patients with Lower Extremity Bone Sarcoma

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    Gary E Mason

    2013-08-01

    Full Text Available PURPOSE: Although functional differences have been described between patients with lower extremity bone sarcoma with amputation and limb preservation surgery, differences have not clearly been shown between the two groups related to quality of life. The aim of the study was to determine if there is a difference in overall quality of life in lower extremity bone sarcoma survivors related to whether they had an amputation or a limb preservation procedure. PATIENTS AND METHODS: Eighty-two long-term survivors of lower extremity bone sarcoma were studied to make a comparison of the overall quality of life, pain assessment and psychological evaluations in limb preservation and amputation patients. Forty-eight patients with limb preservation and thirty-four patients with amputations were enrolled in the study. Validated psychometric measures including the Quality of Life Questionnaire, the Minnesota Multiphasic Personality Inventory and visual analog scales were utilized.RESULTS: The overall quality of life of patients with limb preservation was significantly higher than patients with amputation (p-value < 0.01. Significant differences were noted in the categories of material well being, job satisfiers and occupational relations. CONCLUSION: The overall quality of life of patients with limb preservation appears to be better than for those patients with amputation based on the quality of life questionnaire in patients surviving lower extremity bone sarcoma. Further analysis needs to verify the results and focus on the categories that significantly affect the overall quality of life.

  16. Late effects after treatment of twenty children with soft tissue sarcomas of the head and neck. Experience at a single institution with a review of the literature

    International Nuclear Information System (INIS)

    Fromm, M.; Littman, P.; Raney, R.B.; Nelson, L.; Handler, S.; Diamond, G.; Stanley, C.

    1986-01-01

    Twenty children with soft tissue sarcomas of the head and neck, treated at the Children's Hospital of Philadelphia and the Hospital of the University of Pennsylvania from 1972 to 1981, were evaluated for the late deleterious effects of treatment. All patients received radiation therapy and combination chemotherapy with vincristine, dactinomycin, and cyclophosphamide; certain patients also received Adriamycin (doxorubicin). All had ophthalmologic, otologic, growth, and cosmetic evaluations; 15 also had dental and maxillofacial examinations. The median age at diagnosis was 6 years (range, 7 months-13 years). Median follow-up from time of diagnosis was 5.5 years with a minimum of 3 years in all but four patients. The major problems encountered were related to the eyes (xerophthalmia and cataracts), ears (hearing loss), teeth (maleruption and caries), glandular structures (xerostomia, hypopituitarism), and development (craniofacial deformity). It is concluded that children treated for soft tissue sarcomas of the head and neck with combined modality therapy, including radiation enhancers, may show a variety of late treatment-related adversities. These children require close multidisciplinary follow-up for detection of late effects in order that appropriate prophylactic or symptomatic treatment can be instituted to minimize their consequences

  17. Combined zidovudine and interferon-alpha treatment in patients with AIDS-associated Kaposi's sarcoma

    NARCIS (Netherlands)

    de Wit, R.; Danner, S. A.; Bakker, P. J.; Lange, J. M.; Eeftinck Schattenkerk, J. K.; Veenhof, C. H.

    1991-01-01

    The effectiveness of addition of interferon-alpha (IFN-alpha) to zidovudine in patients with AIDS-associated Kaposi's sarcoma was assessed in a non-randomized, phase II clinical trial. Twenty-one patients were treated with oral zidovudine (600 mg daily) and IFN-alpha was increased to 18 MU daily for

  18. The value of local treatment in patients with primary, disseminated, multifocal Ewing sarcoma (PDMES)

    NARCIS (Netherlands)

    Haeusler, Julia; Ranft, Andreas; Boelling, Tobias; Gosheger, Georg; Braun-Munzinger, Gabriele; Vieth, Volker; Burdach, Stefan; van den Berg, Henk; Juergens, Heribert; Dirksen, Uta

    2010-01-01

    The value of local treatment in patients with primary, disseminated, multifocal Ewing sarcoma (PDMES) was investigated. We analyzed 120 patients registered into the European Ewing Tumor Working Initiative of National Groups (EURO-E.W.I.N.G. 99) trial at the trial center of Muenster from 1998 to

  19. Nonmetastatic Ewing’s Sarcoma of the Lumbar Spine in an Adult Patient

    Directory of Open Access Journals (Sweden)

    Maurizio Iacoangeli

    2012-01-01

    Full Text Available Although the spine is frequently involved in metastatic Ewing's sarcoma, primary involvement of the spine, beside sacrum, is much less frequent, especially in adult patients. Because of the low incidence of these tumors, there are currently no clinical guidelines outlining their management and a multitude of therapeutic strategies have been employed with varying success. The definitive management of Ewing's sarcoma of the spine, as in other locations, could include the combination of three main modalities: aggressive surgery, radiotherapy, and combined chemotherapy. Whenever possible, en bloc spondylectomy or extralesional resection is preferable, providing a better oncological result with a longer survival and a better preservation of the spine biomechanics. This is the lesson we learned about the case, we present here, of nonmetastatic lumbar localization by Ewing’s sarcoma in as adult patient.

  20. Conservative treatment of soft tissue sarcomas of the extremities. Functional evaluation with LENT-SOMA scales and the Enneking score

    International Nuclear Information System (INIS)

    Tawfiq, N.; Lagarde, P.; Thomas, L.; Kantor, G.; Stockle, E.; Bui, B.N.

    2000-01-01

    Objective. - The aim of this prospective study is the feasibility of late effects assessment by LENT-SOMA scales after conservative treatment of soft tissue sarcomas of the extremities and a comparison with the functional evaluation by the Enneking score. Patients and methods. - During the systematic follow-up consultations, a series of 32 consecutive patients was evaluated in terms of late effects by LENT SOMA scales and functional results by the Enneking score. The median time after treatment was 65 months. The treatment consisted of conservative surgery (all cases) followed by radiation therapy (29 cases), often combined with adjuvant therapy (12 concomitant radio-chemotherapy association cases out of 14). The assessment of the toxicity was retrospective for acute effects and prospective for the following late tissue damage: skin/subcutaneous tissues, muscles/soft tissues and peripheral nerves. Results. -According to the Enneking score, the global score for the overall series was high (24/30) despite four the scores zero for the psychological acceptance. According to LENT SOMA scales, a low rate of severe sequelae (grade 3-4) was observed. The occurrence of high-grade sequelae and their functional consequences were not correlated with quality of exeresis, dose of radiotherapy or use of concomitant chemotherapy. A complementarity was observed between certain factors of the Enneking score and some criteria of the LENTSOMA scales, especially of muscles/soft tissues. Conclusion. -The good quality of functional results was confirmed by the two mean scoring systems for late normal tissue damage. The routine use of LENT-SOMA seems to be more time consuming than the Enneking score (mean time of scoring: 1 3 versus five minutes). The LENT-SOMA scales are aimed at a detailed description of late toxicity and sequelae while the Enneking score provides a more global evaluation, including the psychological acceptance of treatment. The late effects assessment by the LENT

  1. Ewing's sarcoma of the head and neck

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    Adriano Santana Fonseca

    2000-11-01

    Full Text Available CONTEXT: Ewing's sarcoma is a rare neoplasm, which usually arises in long bones of the limbs and in flat bones of the pelvis, with the involvement of head and neck bones being very unusual. CASE REPORT: a case of Ewing's sarcoma occurring in the mandible of a 35-year-old female. Pain and swelling of the tumor were the main complaints. The early hypothesis was an undifferentiated malignant neoplasm, possibly a sarcoma. The CT scan depicted an expansive lesion, encapsulated, with septa and characteristics of soft tissue, involving the left side of the mandible and extending to the surrounding tissues. The patient underwent surgical excision of the lesion, the definitive diagnosis of Ewing's sarcoma was established, and the patient commenced on radiotherapy.

  2. Utility Values for Advanced Soft Tissue Sarcoma Health States from the General Public in the United Kingdom

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    Julian F. Guest

    2013-01-01

    Full Text Available Soft tissue sarcomas are a rare type of cancer generally treated with palliative chemotherapy when in the advanced stage. There is a lack of published health utility data for locally advanced “inoperable”/metastatic disease (ASTS, essential for calculating the cost-effectiveness of current and future treatments. This study estimated time trade-off (TTO and standard gamble (SG preference values associated with four ASTS health states (progressive disease, stable disease, partial response, complete response among members of the general public in the UK (n=207. The four health states were associated with decreases in preference values from full health. Complete response was the most preferred health state (mean utility of 0.60 using TTO. The second most preferred health state was partial response followed by stable disease (mean utilities were 0.51 and 0.43, respectively, using TTO. The least preferred health state was progressive disease (mean utility of 0.30 using TTO. The utility value for each state was significantly different from one another (P<0.001. This study demonstrated and quantified the impact that different treatment responses may have on the health-related quality of life of patients with ASTS.

  3. Elevated HERV-K Expression in Soft Tissue Sarcoma Is Associated with Worsened Relapse-Free Survival.

    Science.gov (United States)

    Giebler, Maria; Staege, Martin S; Blauschmidt, Sindy; Ohm, Lea I; Kraus, Matthias; Würl, Peter; Taubert, Helge; Greither, Thomas

    2018-01-01

    A wide variety of endogenous retroviral sequences has been demonstrated in the human genome so far, divided into several different families according to the sequence homology to viral strains. While increased expression of human endogenous retrovirus (HERV) elements has already been linked to unfavorable prognosis in hepatocellular carcinoma, breast cancer, and ovarian carcinoma yet less is known about the impact of the expression of different HERV elements on sarcomagenesis in general as well as the outcome of soft tissue sarcoma (STS) patients. Therefore, in this study the association between expression of HERV-K and HERV-F and the clinicopathological characteristics in a cohort of STSs as well as the patients' prognosis was evaluated. HERV-K and HERV-F expression was assessed by quantitative real-time PCR in 120 patient specimens. HERV-K and HERV-F expression was significantly correlated ( r S = 0.5; p = 6.4 × 10 -9 ; Spearman's rank bivariate correlation). Also, tumor diameter exhibited a significant negative association to HERV-K and HERV-F expression. Levels of several hypoxia-related RNAs like HIF-1α and miR-210 showed a significant positive correlation with both HERV-K and HERV-F expression. Although in survival analyses no impact of HERV expression on disease-specific survival could be detected, patients with elevated HERV-K expression had a significantly shorter relapse-free survival ( p = 0.014, log-rank analysis). In conclusion, we provide evidence for the first time that the increased expression of HERV-K in tumors is associated with STS patients' prognosis.

  4. Ewing's Sarcoma: An Analysis of miRNA Expression Profiles and Target Genes in Paraffin-Embedded Primary Tumor Tissue.

    Science.gov (United States)

    Parafioriti, Antonina; Bason, Caterina; Armiraglio, Elisabetta; Calciano, Lucia; Daolio, Primo Andrea; Berardocco, Martina; Di Bernardo, Andrea; Colosimo, Alessia; Luksch, Roberto; Berardi, Anna C

    2016-04-30

    The molecular mechanism responsible for Ewing's Sarcoma (ES) remains largely unknown. MicroRNAs (miRNAs), a class of small non-coding RNAs able to regulate gene expression, are deregulated in tumors and may serve as a tool for diagnosis and prediction. However, the status of miRNAs in ES has not yet been thoroughly investigated. This study compared global miRNAs expression in paraffin-embedded tumor tissue samples from 20 ES patients, affected by primary untreated tumors, with miRNAs expressed in normal human mesenchymal stromal cells (MSCs) by microarray analysis. A miRTarBase database was used to identify the predicted target genes for differentially expressed miRNAs. The miRNAs microarray analysis revealed distinct patterns of miRNAs expression between ES samples and normal MSCs. 58 of the 954 analyzed miRNAs were significantly differentially expressed in ES samples compared to MSCs. Moreover, the qRT-PCR analysis carried out on three selected miRNAs showed that miR-181b, miR-1915 and miR-1275 were significantly aberrantly regulated, confirming the microarray results. Bio-database analysis identified BCL-2 as a bona fide target gene of the miR-21, miR-181a, miR-181b, miR-29a, miR-29b, miR-497, miR-195, miR-let-7a, miR-34a and miR-1915. Using paraffin-embedded tissues from ES patients, this study has identified several potential target miRNAs and one gene that might be considered a novel critical biomarker for ES pathogenesis.

  5. Long-term follow-up of 15 patients with non-metastatic Ewing's sarcoma and a skip lesion

    NARCIS (Netherlands)

    Jiya, T.U.; Wuisman, P.I.J.M.

    2005-01-01

    Background: Skip lesions in Ewing's sarcoma of the bone seem to be rare; to our knowledge only 7 cases have been published in the English medical literature. Methods: We retrospectively reviewed imaging and histological data relating to 235 patients with non-metastatic Ewing's sarcoma of the bone

  6. Ewing's sarcoma. Radiographic pattern of healing and bony complications in patients with long-term survival

    International Nuclear Information System (INIS)

    Ehara, S.; Kattapuram, S.V.; Egglin, T.K.

    1991-01-01

    The radiographic appearance of Ewing's sarcoma was studied retrospectively in 22 patients who survived 5 years or longer after diagnosis and treatment. Expected changes from treatment, including regression of the extraosseous soft tissue mass, periostitis, and reconstitution of the cortex, occurred in all patients. Local recurrence occurred in one patient 10 years after complete remission whereas secondary osteosarcoma occurred more than 5 years after complete remission in two other cases. Both recurrent and secondary tumors presented as new lytic foci at the site of the original primary lesion. Lytic changes from radiation (radiation osteitis) may develop more than 2 years after treatment and in this sample; such findings were widely distributed in the radiation port. The authors conclude that bone remodeling and postradiation changes occur slowly over 2 years after treatment, and that any localized lysis at the primary site is suspicious for recurrence or secondary neoplasm. Knowledge of the expected changes and patterns of local recurrence and secondary neoplasms helps one to detect any significant change in its early phase

  7. "In situ preparation": new surgical procedure indicated for soft-tissue sarcoma of a lower limb in close proximity to major neurovascular structures.

    Science.gov (United States)

    Matsumoto, Seiichi; Kawaguchi, Noriyoshi; Manabe, Jun; Matsushita, Yasushi

    2002-02-01

    When soft-tissue sarcomas occur near neurovascular structures, preoperative images cannot always reveal the accurate relationship between the tumor and these structures. Therefore, in some patients, neurovascular structures are sacrificed unnecessarily. In other patients, neurovascular structures are preserved with an inappropriate margin, followed by local recurrence. The objective of this study was to evaluate a new surgical method, "in situ preparation" (ISP), which enables the preparation of neurovascular bundles and the intraoperative evaluation of the surgical margin without contamination by tumor cells. With this method, additional procedures, including pasteurization, alcohol soaking, and distilled water soaking of the preserved neurovascular bundle can also be performed to preserve the continuity of vessels. Between April 1992 and December 1998, 18 patients with soft-tissue sarcoma were operated on using ISP. The tumor and neurovascular structure were lifted en bloc from the surgical bed and separated from the field by the use of a vinyl sheet. The consistency of the neurovascular structures was preserved. The tissue block could be freely turned around and the neurovascular structure was separated from the block through the nearest approach. The margin between the tumor and neurovascular structure was evaluated, and an additional procedure, such as pasteurization, alcohol soaking or distilled water soaking, was performed, according to the safety of the surgical margin. Only one patient showed recurrence after ISP. Complications after ISP were arterial occlusion in two patients and nerve palsy in three patients. The main cause of these complications was the long period of pasteurization; modified additional procedures could prevent such complications. ISP is a useful method with which to ensure a safe surgical margin and good functional results.

  8. Immunotherapy of childhood Sarcomas

    Directory of Open Access Journals (Sweden)

    Stephen S Roberts

    2015-08-01

    Full Text Available Pediatric sarcomas are a heterogeneous group of malignant tumors of bone and soft tissue origin. Although more than 100 different histologic subtypes have been described, the majority of pediatric cases belong to the Ewing’s family of tumors, rhabdomyosarcoma and osteosarcoma. Most patients that present with localized stage are curable with surgery and/or chemotherapy; however, those with metastatic disease at diagnosis or those who experience a relapse continue to have a very poor prognosis. New therapies for these patients are urgently needed. Immunotherapy is an established treatment modality for both liquid and solid tumors, and in pediatrics, most notably for neuroblastoma and osteosarcoma. In the past, immunomodulatory agents such as interferon, interleukin-2, and Liposomal-muramyl  tripeptide phosphatidyl-ethanolamine (L-MTP have been tried, with some activity seen in subsets of patients; additionally, various cancer vaccines have been studied with possible benefit. Monoclonal antibody therapies against tumor antigens such as disialoganglioside GD2 or immune checkpoint targets such as CTLA4 and PD-1 are being actively explored in pediatric sarcomas. Building on the success of adoptive T cell therapy for EBV-related lymphoma, strategies to redirect T cells using chimeric antigen receptors and bispecific antibodies are rapidly evolving with potential for the treatment of sarcomas. This review will focus on recent preclinical and clinical developments in targeted agents for pediatric sarcomas with emphasis on the immunobiology of immune checkpoints, immunoediting, tumor microenvironment, antibody engineering, cell engineering, and tumor vaccines. The future integration of antibody based and cell based therapies into an overall treatment strategy of sarcoma will be discussed.

  9. Working to improve the management of sarcoma patients across Europe: a policy checklist.

    Science.gov (United States)

    Kasper, Bernd; Lecointe-Artzner, Estelle; Wait, Suzanne; Boldon, Shannon; Wilson, Roger; Gronchi, Alessandro; Valverde, Claudia; Eriksson, Mikael; Dumont, Sarah; Drove, Nora; Kanli, Athanasia; Wartenberg, Markus

    2018-04-16

    The Sarcoma Policy Checklist was created by a multidisciplinary expert group to provide policymakers with priority areas to improve care for sarcoma patients. This paper draws on this research, by looking more closely at how France, Germany, Italy, Spain, Sweden and the United Kingdom are addressing each of these priority areas. It aims to highlight key gaps in research, policy and practice, as well as ongoing initiatives that may impact the future care of sarcoma patients in different European countries. A pragmatic review of the published and web-based literature was undertaken. Telephone interviews were conducted in each country with clinical and patient experts to substantiate findings. Research findings were discussed within the expert group and developed into five core policy recommendations. The five identified priority areas were: the development of designated and accredited centres of reference; more professional training; multidisciplinary care; greater incentives for research and innovation; and more rapid access to effective treatments. Most of the countries studied have ongoing initiatives addressing many of these priorities; however, many are in early stages of development, or require additional funding and resources. Gaps in access to quality care are particularly concerning in many of Europe's lower-resourced countries. Equitable access to information, clinical trials, innovative treatments and quality specialist care should be available to all sarcoma patients. Achieving this across Europe will require close collaboration between all stakeholders at both the national and European level.

  10. Conditional survival is greater than overall survival at diagnosis in patients with osteosarcoma and Ewing's sarcoma.

    Science.gov (United States)

    Miller, Benjamin J; Lynch, Charles F; Buckwalter, Joseph A

    2013-11-01

    Conditional survival is a measure of the risk of mortality given that a patient has survived a defined period of time. These estimates are clinically helpful, but have not been reported previously for osteosarcoma or Ewing's sarcoma. We determined the conditional survival of patients with osteosarcoma and Ewing's sarcoma given survival of 1 or more years. We used the Surveillance, Epidemiology, and End Results (SEER) Program database to investigate cases of osteosarcoma and Ewing's sarcoma in patients younger than 40 years from 1973 to 2009. The SEER Program is managed by the National Cancer Institute and provides survival data gathered from population-based cancer registries. We used an actuarial life table analysis to determine any cancer cause-specific 5-year survival estimates conditional on 1 to 5 years of survival after diagnosis. We performed a similar analysis to determine 20-year survival from the time of diagnosis. The estimated 5-year survival improved each year after diagnosis. For local/regional osteosarcoma, the 5-year survival improved from 74.8% at baseline to 91.4% at 5 years-meaning that if a patient with localized osteosarcoma lives for 5 years, the chance of living for another 5 years is 91.4%. Similarly, the 5-year survivals for local/regional Ewing's sarcoma improved from 72.9% at baseline to 92.5% at 5 years, for metastatic osteosarcoma 35.5% at baseline to 85.4% at 5 years, and for metastatic Ewing's sarcoma 31.7% at baseline to 83.6% at 5 years. The likelihood of 20-year cause-specific survival from the time of diagnosis in osteosarcoma and Ewing's sarcoma was almost 90% or greater after 10 years of survival, suggesting that while most patients will remain disease-free indefinitely, some experience cancer-related complications years after presumed eradication. The 5-year survival estimates of osteosarcoma and Ewing's sarcoma improve with each additional year of patient survival. Knowledge of a changing risk profile is useful in counseling

  11. Diagnosis of pulmonary Kaposi's sarcoma in AIDS patients.

    Science.gov (United States)

    Jeyapalan, M; Steffenson, S

    1997-02-01

    Pulmonary Kaposi's sarcoma (KS) is one of the many manifestations of AIDS. There are no specific tests for its early diagnosis. Because its symptoms may be similar to tuberculosis, it may be diagnosed incorrectly and treated as such. Consequently, by the time of the correct diagnosis, valuable time will have been lost for effective medical care that could positively impact prognosis. The discussion in this case study is focused on pulmonary KS with an interest in improving premorbid diagnosis that may lead to an earlier recognition and better treatment of the disease.

  12. Single block orbitectomy in a patient with neurogenic orbital sarcoma

    OpenAIRE

    Abreu Perdomo, Franklyn Alain; Cruz García, Orlando; Ortíz Ramos, Datia Liset; Santos Silva, Dania; Sánchez Wals, Lenia; Lamadrid García, Janet

    2016-01-01

    El sarcoma neurogénico es un tumor maligno que se origina en las células de Schwann de la vaina del revestimiento de los nervios periféricos y son poco frecuentes en la órbita. Se presenta un paciente de 23 años de edad, masculino, blanco, con antecedentes de neurofibromatosis tipo I, con desplazamiento anteroinferior del globo ocular izquierdo, dolor intenso y pérdida de la visión de 4 meses de evolución. Al examen oftalmológico se constató proptosis severa con descenso del globo ocular izqu...

  13. Efficacy of Sunitinib and Radiotherapy in Genetically Engineered Mouse Model of Soft-Tissue Sarcoma

    International Nuclear Information System (INIS)

    Yoon, Sam S.; Stangenberg, Lars; Lee, Yoon-Jin; Rothrock, Courtney; Dreyfuss, Jonathan M.; Baek, Kwan-Hyuck; Waterman, Peter R.; Nielsen, G. Petur; Weissleder, Ralph; Mahmood, Umar; Park, Peter J.; Jacks, Tyler

    2009-01-01

    Purpose: Sunitinib (SU) is a multitargeted receptor tyrosine kinase inhibitor of the vascular endothelial growth factor and platelet-derived growth factor receptors. The present study examined SU and radiotherapy (RT) in a genetically engineered mouse model of soft tissue sarcoma (STS). Methods and Materials: Primary extremity STSs were generated in genetically engineered mice. The mice were randomized to treatment with SU, RT (10 Gy x 2), or both (SU+RT). Changes in the tumor vasculature before and after treatment were assessed in vivo using fluorescence-mediated tomography. The control and treated tumors were harvested and extensively analyzed. Results: The mean fluorescence in the tumors was not decreased by RT but decreased 38-44% in tumors treated with SU or SU+RT. The control tumors grew to a mean of 1378 mm 3 after 12 days. SU alone or RT alone delayed tumor growth by 56% and 41%, respectively, but maximal growth inhibition (71%) was observed with the combination therapy. SU target effects were confirmed by loss of target receptor phosphorylation and alterations in SU-related gene expression. Cancer cell proliferation was decreased and apoptosis increased in the SU and RT groups, with a synergistic effect on apoptosis observed in the SU+RT group. RT had a minimal effect on the tumor microvessel density and endothelial cell-specific apoptosis, but SU alone or SU+RT decreased the microvessel density by >66% and induced significant endothelial cell apoptosis. Conclusion: SU inhibited STS growth by effects on both cancer cells and tumor vasculature. SU also augmented the efficacy of RT, suggesting that this combination strategy could improve local control of STS.

  14. Targeted therapy for sarcomas

    Directory of Open Access Journals (Sweden)

    Forscher C

    2014-03-01

    Full Text Available Charles Forscher,1 Monica Mita,2 Robert Figlin3 1Sarcoma Program, Samuel Oschin Comprehensive Cancer Institute, Cedars-Sinai Medical Center, Los Angeles, CA, USA; 2Experimental Therapeutics Program, Samuel Oschin Comprehensive Cancer Institute, Cedars-Sinai Medical Center, Los Angeles, CA, USA; 3Academic Development Program, Samuel Oschin Comprehensive Cancer Institute, and Division of Hematology/Oncology, Cedars-Sinai Medical Center, Los Angeles, CA, USA Abstract: Sarcomas are tumors of mesenchymal origin that make up approximately 1% of human cancers. They may arise as primary tumors in either bone or soft tissue, with approximately 11,280 soft tissue tumors and 2,650 bone tumors diagnosed each year in the United States. There are at least 50 different subtypes of soft tissue sarcoma, with new ones described with ever-increasing frequency. One way to look at sarcomas is to divide them into categories on the basis of their genetic make-up. One group of sarcomas has an identifiable, relatively simple genetic signature, such as the X:18 translocation seen in synovial sarcoma or the 11:22 translocation seen in Ewing's sarcoma. These specific abnormalities often lead to the presence of fusion proteins, such as EWS-FLI1 in Ewing's sarcoma, which are helpful as diagnostic tools and may become therapeutic targets in the future. Another group of sarcomas is characterized by complex genetic abnormalities as seen in leiomyosarcoma, osteosarcoma, and undifferentiated sarcoma. It is important to keep these distinctions in mind when contemplating the development of targeted agents for sarcomas. Different abnormalities in sarcoma could be divided by tumor subtype or by the molecular or pathway abnormality. However, some existing drugs or drugs in development may interfere with or alter more than one of the presented pathways. Keywords: sarcoma, targeted agents, tyrosine kinase inhibitors, mTor inhibition

  15. The Molecular Biology of Soft-Tissue Sarcomas and Current Trends in Therapy

    Directory of Open Access Journals (Sweden)

    Jorge Quesada

    2012-01-01

    Full Text Available Basic research in sarcoma models has been fundamental in the discovery of scientific milestones leading to a better understanding of the molecular biology of cancer. Yet, clinical research in sarcoma has lagged behind other cancers because of the multiple clinical and pathological entities that characterize sarcomas and their rarity. Sarcomas encompass a very heterogeneous group of tumors with diverse pathological and clinical overlapping characteristics. Molecular testing has been fundamental in the identification and better definition of more specific entities among this vast array of malignancies. A group of sarcomas are distinguished by specific molecular aberrations such as somatic mutations, intergene deletions, gene amplifications, reciprocal translocations, and complex karyotypes. These and other discoveries have led to a better understanding of the growth signals and the molecular pathways involved in the development of these tumors. These findings are leading to treatment strategies currently under intense investigation. Disruption of the growth signals is being targeted with antagonistic antibodies, tyrosine kinase inhibitors, and inhibitors of several downstream molecules in diverse molecular pathways. Preliminary clinical trials, supported by solid basic research and strong preclinical evidence, promises a new era in the clinical management of these broad spectrum of malignant tumors.

  16. Epithelioid inflammatory myofibroblastic sarcoma responsive to surgery and an ALK inhibitor in a patient with panhypopituitarism.

    Science.gov (United States)

    Kurihara-Hosokawa, Kotomi; Kawasaki, Isao; Tamai, Anna; Yoshida, Yoko; Yakushiji, Yosuke; Ueno, Hiroki; Fukumoto, Mariko; Fukushima, Hiroko; Inoue, Takeshi; Hosoi, Masayuki

    2014-01-01

    We encountered a case of epithelioid inflammatory myofibroblastic sarcoma (EIMS) originating from an abdominal organ that rapidly regrew twice. The patient underwent two surgeries. Large tumors grew within three months after the second surgery. The patient subsequently received chemotherapy with an anaplastic lymphoma kinase (ALK) inhibitor. Although EIMS has a poor prognosis, the patient continues to be alive with disease 14 months after surgical treatment and the administration of the ALK inhibitor.

  17. Prognostic factors for survival among patients with primary bone sarcomas of small bones

    Directory of Open Access Journals (Sweden)

    Wang Z

    2018-05-01

    Full Text Available Zhan Wang,1,* Shu Li,2,* Yong Li,1 Nong Lin,1 Xin Huang,1 Meng Liu,1 Weibo Pan,1 Xiaobo Yan,1 Lingling Sun,1 Hengyuan, Li,1 Binghao Li,1 Hao Qu,1 Yan Wu,1 Peng Lin,1 Zhaoming Ye1 1Department of Orthopaedics, The Second Affiliated Hospital of Zhejiang University School of Medicine, Centre for Orthopaedic Research, Orthopedics Research Institute of Zhejiang University, Hangzhou, China; 2Key Laboratory of Cancer Prevention and Intervention, Key Laboratory of Molecular Biology in Medical Sciences, National Ministry of Education, Department of Hematology, The Second Affiliated Hospital, Zhejiang University School of Medicine, Cancer Institute, Hangzhou, China *These authors contributed equally to this work Background: Primary bone sarcomas of the hands or feet are rare lesions and poorly documented. Moreover, the prognostic determinants of bone sarcomas of the hands or feet have not been reported. Materials and methods: The Surveillance, Epidemiology, and End Results (SEER program database was used to screen patients with bone sarcomas of the hands or feet from 1973 to 2013, with attention paid to chondrosarcoma, Ewing sarcoma, and osteosarcoma. The prognostic values of overall survival (OS and cancer-specific survival (CSS were assessed using Cox proportional hazards regression model with univariate and multivariate analyses. The Kaplan–Meier method was used to obtain OS and CSS curves. Results: A total of 457 cases were selected from the SEER database. Chondrosarcoma was the most common form of lesion in hands or feet or both, followed by Ewing sarcoma and osteosarcoma. The 5- and 10-year OS rates of the entire group were 75.7% and 66.1%, respectively. The 5- and 10-year CSS rates were 78.7% and 73.7%, respectively. Multivariate analysis revealed that age under 40 years, localized stage, low grade, surgical treatment, and first primary tumor were associated with improved OS, and decade of diagnosis, stage, grade, and surgery were independent

  18. Adult soft tissue sarcomas of the head and neck treated by radiation and surgery or radiation alone: patterns of failure and prognostic factors

    International Nuclear Information System (INIS)

    Willers, Henning; Hug, Eugen G.; Spiro, Ira J.; Efird, Jimmy T.; Rosenberg, Andrew E.; Wang, C. C.

    1995-01-01

    Purpose: To analyze our experience treating soft tissue sarcomas of the head and neck in adults, and to identify patterns of failure and prognostic factors. Methods and Materials: The records of 57 patients with Stage M0 disease treated by radiation with or without surgery between 1972 and 1993 were reviewed. Median follow-up time was 4.3 years (range, 1.1-16.8 years). A group of potential prognostic factors was evaluated, including age at diagnosis, sex, initial tumor presentation (primary vs. recurrent), grade, T-stage, direct tumor extension, tumor depth, duration of treatment, and radiation dose. Results: The subset of angiosarcomas (11 out of 57 patients) had a considerably adverse effect on treatment outcome for the total group of sarcomas, with actuarial 5-year overall survival (OS), locoregional control (LRC), and freedom from distant metastasis (FDM) rates being 31%, 24%, and 42%, respectively. In contrast, for the remaining 46 patients with other histopathological tumor types, OS, LRC, and FDM rates were significantly higher (74%, 69%, and 83%, respectively). For this group of significant prognostic factors identified by uni- and multivariate analysis included tumor grade as a predictor of OS and T-stage as a predictor of LRC (p ≤ 0.050). Those patients who experienced a locoregional recurrence were at a significantly increased risk of dying (p = 0.004 in a multivariate model). All 17 patients without direct tumor extension to neurovascular structures, bone, contiguous organs, or skin remained free from distant failure. In contrast, 27% of 29 patients with direct extension had developed distant metastases at 5 years. In multivariate analysis, the absence of direct extension was a positive predictor of FDM (p = 0.007) and of OS (p = 0.034). Conclusions: 1) Angiosarcomas of the head and neck have a considerably poorer prognosis than other soft tissue sarcomas of this site. 2) In addition to tumor grade and size, direct tumor extension may be a useful

  19. Postradiation sarcoma involving the spine

    International Nuclear Information System (INIS)

    Sundaresan, N.; Huvos, A.G.; Krol, G.; Hughes, J.E.; Cahan, W.G.

    1986-01-01

    Postradiation sarcomas arising many years after treatment of cancer are long term sequelae of therapy. We describe the clinical features, radiographic findings, and results of treatment in 13 patients with such sarcomas encountered over a 6-year period. Of these patients, 9 had bone sarcomas and the remaining 4 had paraspinal tumors arising from adjacent soft tissue and nerve. The primary cancer for which radiation was given included Hodgkin's disease (4 patients), breast cancer (2 patients), cervix cancer (2 patients), and a variety of others (5 patients). The latent interval to the occurrence of the second neoplasm varied from 6 to 30 years (median, 10 years) after treatment of the original tumor. Despite aggressive treatment, the overall prognosis was poor. The median survival was 8 months, with only 3 surviving more than 2 years. Although rare, postradiation sarcoma should be considered in the differential diagnosis of patients presenting with late onset of spinal pain or neurological symptoms after clinical remission of an original cancer

  20. Evolution of Randomized Trials in Advanced/Metastatic Soft Tissue Sarcoma: End Point Selection, Surrogacy, and Quality of Reporting.

    Science.gov (United States)

    Zer, Alona; Prince, Rebecca M; Amir, Eitan; Abdul Razak, Albiruni

    2016-05-01

    Randomized controlled trials (RCTs) in soft tissue sarcoma (STS) have used varying end points. The surrogacy of intermediate end points, such as progression-free survival (PFS), response rate (RR), and 3-month and 6-month PFS (3moPFS and 6moPFS) with overall survival (OS), remains unknown. The quality of efficacy and toxicity reporting in these studies is also uncertain. A systematic review of systemic therapy RCTs in STS was performed. Surrogacy between intermediate end points and OS was explored using weighted linear regression for the hazard ratio for OS with the hazard ratio for PFS or the odds ratio for RR, 3moPFS, and 6moPFS. The quality of reporting for efficacy and toxicity was also evaluated. Fifty-two RCTs published between 1974 and 2014, comprising 9,762 patients, met the inclusion criteria. There were significant correlations between PFS and OS (R = 0.61) and between RR and OS (R = 0.51). Conversely, there were nonsignificant correlations between 3moPFS and 6moPFS with OS. A reduction in the use of RR as the primary end point was observed over time, favoring time-based events (P for trend = .02). In 14% of RCTs, the primary end point was not met, but the study was reported as being positive. Toxicity was comprehensively reported in 47% of RCTs, whereas 14% inadequately reported toxicity. In advanced STS, PFS and RR seem to be appropriate surrogates for OS. There is poor correlation between OS and both 3moPFS and 6moPFS. As such, caution is urged with the use of these as primary end points in randomized STS trials. The quality of toxicity reporting and interpretation of results is suboptimal. © 2016 by American Society of Clinical Oncology.

  1. Synovial Sarcoma of the Buccal Mucosa: A Rare Case Report

    Directory of Open Access Journals (Sweden)

    Kumar T. S. Mahesh

    2013-01-01

    Full Text Available Synovial sarcoma (SS is a rare malignant neoplasm that arises most commonly in joint capsules and articular tendons, but its relationship to the synovium is not always obvious. Synovial sarcoma is a malignant soft tissue tumor representing 5.6% to 10% of all soft tissue sarcomas. They are termed SS because of their histologic resemblance to the synovium, but they rarely involve a synovial structure and are thought to arise from pluripotential mesenchymal cells. The tumor usually occurs in close association with tendon sheaths, bursae, and joint capsules, primarily in the para-articular regions of the extremities, with approximately 9% occurring in the head and neck region. Synovial sarcoma has been reported rarely in the oral cavity. We report a very rare case of Synovial sarcoma of the buccal mucosa in a 24-year-old male patient.

  2. Retrospective review of soft tissue sarcoma of head and neck in a ...

    African Journals Online (AJOL)

    Ayotunde James Fasunla

    2012-10-10

    Oct 10, 2012 ... Conclusion: Head and neck sarcomas are rare in West Africa. .... sive dysphagia, change in voice and cervical lymphadenopathy. .... graphic (CT) scan and magnetic resonance imaging (MRI), ... or bleeding occurring within the tumour mass. .... Irregular and inconsistent irradiation therapy received by the.

  3. Maturation toward neuronal tissue in a Ewing sarcoma of bone after chemotherapy

    NARCIS (Netherlands)

    Salet, Maria Carolina Wilhelmina; Vogels, Rob; Brons, Paul P. T.; Schreuder, Bart; Flucke, Uta

    2016-01-01

    Background: Ewing sarcoma is the second most common bone tumor, occurring mainly in children and young adults. It shows a typical primitive, small round cell morphology and a characteristic fusion oncogene involving EWSR1 and members of the ETS family in most of the cases. Neuronal maturation after

  4. Efficacy of PET/CT to exclude leiomyoma in patients with lesions suspicious for uterine sarcoma on MRI

    Directory of Open Access Journals (Sweden)

    Soshi Kusunoki

    2017-08-01

    Conclusions: In patients with MRI findings consistent with either uterine sarcoma or leiomyoma, PET/CT can decrease the false-positive rate by setting an optimal cutoff SUVmax of 7.5. Using this cutoff can avoid unnecessary surgery.

  5. Postradiation sarcomas: importance of surgery

    International Nuclear Information System (INIS)

    Lagrange, J.L.; Ramaioli, A.; Chateau, M.C.; Pignol, J.P.; Marchal, C.; Resbeut, M.; Richaud, P.; Rambert, P.; Tortechaux, J.; Seng, S.H.; La Fontan, B. de; Reme-Saumon, M.; Roullet, B.; Bof, J.; Coindre, J.M.

    1997-01-01

    Purpose: To evaluate the role of surgery in the treatment of Post-radiation sarcomas Materials. Post-radiation sarcomas is a rare entity and large series have rarely been reported. In order to improve knowledge about this entity the Radiotherapist group of the French Cancer Centres (FNCLCC) decided to collect retrospectively the cases treated in their institutions. In order to be sure of the histology, all the cases were reviewed by a panel of pathologists of the FNCLCC Pathologist group. A total of 129 cases of sarcomas, and 108 were reviewed; analysis of 8 is in progress, and no material was obtained in the other 11 cases. The diagnosis of sarcomas was confirmed in 80 cases. All patients (60 F, 20 M) have received radiation therapy (median dose 50 Gy; 9-110 Gy) for the treatment of the primary tumor. At this time the age was 44 years (6-83 y). Diagnoses included: breast C. 42%, Lymphomas 11.5%, gynaecological C. 10% benign lesions 5% miscellaneous. Sarcomas developed after a mean interval of 12 years (3-64 y), in bone in 30% of the cases and in soft tissue in 70%. The majority of lesions (90%) developed in the irradiated field (dose received was between 50 Gy and 60 Gy). Histologically there were 29% Malignant HistiocytofibroSarcomas, 19% OsteoSarcomas, 15% FibroSarcomas, 9% LipoSarcomas, 6% LeiomyoSarcomas, miscellaneous sarcomas 22%. Treatment included: Surgery 28 cases, Surgery+Chemotherapy 17 cases, Chemotherapy only 16 cases, Radiation therapy only 1 case, surgery + Radiation therapy 5 cases, Radiation therapy +chemotherapy 6 cases, Surgery + Radiation therapy + Chemotherapy 7 cases, no treatment 5 cases. Results. The outcome is known for all but 3 patients. 51 patients have died (44 of their sarcoma, 4 of the primary tumour, 2 of other cause and 1 iatrogenic). Median survival is 23 months (95% confidence interval 16-29 mo) but 9 patients survived 5 yr or more. Median survival was 43 mo for patients treated by surgery (28p), 6 mo for chemotherapy group (16 p

  6. Long-term outcome of combined modality therapy in retroperitoneal and deep-trunk soft-tissue sarcoma: analysis of prognostic factors

    International Nuclear Information System (INIS)

    Youssef, Emad; Fontanesi, James; Mott, Michael; Kraut, Michael; Lucas, David; Mekhael, Hany; Ben-Josef, Edgar

    2002-01-01

    Purpose: To evaluate the long-term outcome of surgery and postoperative radiotherapy (RT) in retroperitoneal and deep-trunk soft-tissue sarcoma, and to identify the prognostic factors for local control, disease-free survival, and overall survival. Methods and Materials: Between January 1980 and December 1998, 60 patients with nonmetastatic retroperitoneal and deep-trunk soft-tissue sarcoma were treated at Wayne State University using combined surgery and RT. The location was retroperitoneal in 38 patients (63%) and deep trunk in 22 (27%). Forty-six patients (76%) were treated for primary disease and 14 (24%) for recurrent disease. The resection margins were negative in 24 patients (40%), close in 3 (5%), and positive in 33 (55%; 18 microscopic and 15 macroscopic). The median tumor size was 8.6 cm (range 2-55). External beam RT (EBRT; median dose 5220 cGy) was given to 44 patients (73%) and combined EBRT (median dose 4200 cGy) and brachytherapy (median dose 1600 cGy) to 16 patients (27%). Univariate and multivariate Cox regression analyses were conducted to identify the possible associations between patient age, race, gender, tumor site, histologic features, grade, size, stage, surgical margin, RT dose, modality (EBRT vs. EBRT plus brachytherapy), and presentation (primary vs. recurrent) and disease control. Results: The actuarial 5- and 10-year disease-free survival rate was 53% and 44%, respectively. Disease-free survival was significantly associated with female gender on univariate analysis (67% for female patients and 37% for male patients at 5 years, p=0.05). On multivariate analysis, both gender and surgical margin had borderline significance (p=0.06). The actuarial local control rate was 71% and 54% at 5 and 10 years, respectively. The median time to local relapse was 10.2 months, with 75% of all failures occurring within 29 months. The surgical margin status was significantly associated with local control (78% for patients with negative or close margins vs

  7. Predictive and prognostic factors associated with soft tissue sarcoma response to chemotherapy: a subgroup analysis of the European Organisation for Research and Treatment of Cancer 62012 study.

    Science.gov (United States)

    Young, Robin J; Litière, Saskia; Lia, Michela; Hogendoorn, Pancras C W; Fisher, Cyril; Mechtersheimer, Gunhild; Daugaard, Søren; Sciot, Raf; Collin, Françoise; Messiou, Christina; Grünwald, Viktor; Gronchi, Alessandro; van der Graaf, Winette; Wardelmann, Eva; Judson, Ian

    2017-07-01

    The European Organization for Research and Treatment of Cancer (EORTC) 62012 study was a Phase III trial of doxorubicin versus doxorubicin-ifosfamide chemotherapy in 455 patients with advanced soft tissue sarcoma (STS). Analysis of the main study showed that combination chemotherapy improved tumor response and progression-free survival, but differences in overall survival (OS) were not statistically significant. We analyzed factors prognostic for tumor response and OS, and assessed histological subgroup and tumor grade as predictive factors to identify patients more likely to benefit from combination chemotherapy. Central pathology review was performed by six reference pathologists. Gender, age, performance status, time from first presentation with sarcoma to starting palliative chemotherapy, tumor grade, histological subgroup, primary tumor site involvement, and sites of metastases were assessed as prognostic factors. Three hundred and ten patients were included in this study. Discordance between local and central pathology opinion of tumor histology and tumor grade was observed in 98 (32%) and 122 (39%) cases, respectively. In multivariate analysis, liposarcoma patients had improved tumor response compared to other histological subgroups, whilst patients with metastases other than lung, liver or bone had a poorer response [odds ratio (OR) 0.42, 95% confidence interval (CI) 0.23-0.78; p = 0.006]. Patients with bone metastases had reduced OS [hazard ratio (HR) 1.56, 95% CI 1.16-2.09; p = 0.003]. By central pathology review, patients with undifferentiated pleomorphic sarcoma (UPS) had improved tumor response and OS with doxorubicin-ifosfamide compared to single-agent doxorubicin (OR 9.90, 95% CI 1.93-50.7 and HR 0.44, 95% CI 0.26-0.79, respectively). Grade III tumors had improved response with combination chemotherapy but there was no interaction between chemotherapy and grade on OS. Prospective central pathology review of tumor histology should be

  8. Ewing sarcoma

    International Nuclear Information System (INIS)

    Hamanoue, Satoshi; Makimoto, Atsushi

    2007-01-01

    Ewing sarcoma is the second most frequent primary bone cancer affecting children or young adults. Advances in molecular biology have revealed common chromosomal translocations such as EWS-FLI1 among Ewing sarcoma and related diseases such as primitive neuroectodermal tumor (PNET), so these are considered as Ewing sarcoma family tumor (ESFT). Although fewer than 10% of patients with ESFT survived before establishment of modern multiagent chemotherapy, the multimodal therapeutic regimens including combination chemotherapy, radiotherapy, and surgery can cure 60% of patients with localized disease, due to the collaborative research in European-American or the international trials. The standard chemotherapy for localized ESFT now comprises vincristine, actinomycin D, cyclophosphamide and doxorubicin (VACD) in Europe or vincristine, doxorubicin, cyclophosphamide, ifosfamide and etoposide (VDC-IE) in North America. Meanwhile, those with metastatic disease have a much worse outcome with an approximately 10-30% 5-year event-free survival rate. New American-European collaborative trials such as EURO-E.W.I.N.G. 99 are in progress for further improvement of the cure rate in localized and metastatic ESFT. In Japan, Japan Ewing Sarcoma Study Group (JESS) phase II clinical trial for localized ESFT, and some clinical trials including new drugs are ongoing and waiting for results. (author)

  9. Volume-Based F-18 FDG PET/CT Imaging Markers Provide Supplemental Prognostic Information to Histologic Grading in Patients With High-Grade Bone or Soft Tissue Sarcoma

    DEFF Research Database (Denmark)

    Andersen, Kim Francis; Fuglo, Hanna Maria; Rasmussen, Sine Hvid

    2015-01-01

    analysis. Kaplan-Meier survival estimates and log-rank test were used to compare the degree of equality of survival distributions. Prognostic variables with related hazard ratios (HR) were assessed using Cox proportional hazards regression analysis.Forty-one of 92 patients died during follow-up (45%; 12 BS.......05, HR 3.37 [95% CI 1.02-11.11]). No significant results were demonstrated for MTV40%.Volume-based F-18 FDG PET/CT imaging markers in terms of pretreatment estimation of TLG provide supplemental prognostic information to histologic grading, with significant independent properties for prediction...

  10. Tumor hypoxia adversely affects the prognosis of carcinoma of the head and neck and soft tissue sarcoma

    International Nuclear Information System (INIS)

    Brizel, David M.; Dewhirst, Mark W.; Sibley, Gregory S.; Scully, Sean P.; Harrelson, John M.; Scher, Richard L.; Prosnitz, Leonard R.

    1996-01-01

    Purpose The development of modern polarographic electrode technology has led to a resurgence in the clinical assessment of tumor oxygenation. Tumor hypoxia adversely affects short term clinical radiation response of head and neck cancer lymph node metastases and long term disease-free survival (DFS) in cervix carcinoma. This study was performed to evaluate the influence that tumor hypoxia exerts on the DFS of patients with soft tissue sarcoma (STS) and squamous carcinoma of the head and neck (SCCHN). Methods and Materials Pretreatment tumor pO 2 was assessed in STS and SCCHN patients using the Eppendorf polarographic electrode system. pO 2 probe placement was performed under CT scan guidance to ensure that measurements were obtained only from the tumor and in order to avoid measurement through areas of overt necrosis. A minimum of 3 measurement tracks that ranged in length from 10-35 mm was obtained from each tumor. A minimum of 100 measured points was taken from each tumor. All SCCHN patients had pO 2 measurements taken from locally advanced primaries (T3 or T4) or neck nodes ≥ 1.5 cm diameter. Treatment consisted of once or twice daily irradiation (70-75 Gy) +/- planned neck dissection (for ≥N2A disease) according to institutional treatment protocols. All STS patients had high grade tumors and received preoperative irradiation (2 Gy/day to 50 Gy) + hyperthermia followed by resection. DFS was measured by the Kaplan-Meier product limit method. The log rank technique was used to compare the DFS of different groups of patients. Single factor analysis of variance was used to compare tumor median pO 2 and tumor volume (STS) or stage (SCCHN) in those patients who relapsed versus those who did not. Correlation coefficients were calculated to examine the relationship between tumor volume and tumor oxygenation. Results Since November, 1992, 28 SCCHN and 33 STS patients have undergone tumor pO 2 measurement and have minimum followup of 6 months. The actuarial DFS at 12

  11. Synovial sarcoma: a rare presentation of parapharyngeal mass.

    Science.gov (United States)

    Shaariyah, Mohd Mokhtar; Mazita, Ami; Masaany, Mansor; Razif, Mohd Yunus; Isa, Mohamed Rose; Asma, Abdullah

    2010-06-01

    Synovial sarcoma is a rare soft tissue sarcoma of the head and neck region involving the parapharyngeal space. The diagnosis of synovial sarcoma can be very challenging to the pathologists. We present a rare case of parapharyngeal synovial sarcoma in a young female patient who had a two-month history of left cervical intumescent mass at level II. The fine needle aspiration cytology of the mass was proved inconclusive. Transcervical excision of the mass was performed and the first case of parapharyngeal sarcoma was identified in our center by fluorescence in situ hybridization (FISH) technique. Repeat imaging revealed residual tumor. The patient successfully underwent a second excision of the residual tumor and received adjuvant radiotherapy.

  12. Primary pleuropulmonary synovial sarcoma with brain metastases in a paediatric patient: an unusual presentation.

    Science.gov (United States)

    Chirmade, Pushpak Chandrakant; Parikh, Sonia; Anand, Asha; Panchal, Harsha; Patel, Apurva; Shah, Sandip

    2017-01-01

    Primary lung neoplasms are rare in children. The most common primary lung malignancies in children are pleuropulmonary blastoma and carcinoid tumour. Synovial sarcoma (SS) accounts for approximately 1% of all childhood malignancies. In absolute terms, the SS of the lungs and pleura are extremely rare and pose a diagnostic difficulty. Soft tissue sarcomas usually have a high potential for metastases, however, metastasis to the brain is rare, even in widely disseminated disease, and it has been described only in 3 case reports previously. Primary pleuropulmonary SS with brain metastases is even rarer. Here we present a case of an 11-year-old boy who presented with respiratory complaints, viz. fever and cough for 20 days. Initial impression was lung abscess, however, on histopathological, immunohistochemical and molecular study, the disorder was diagnosed as synovial sarcoma. After a week from the first consult, the child developed neurological symptoms, viz., an episode of convulsion and gradually worsening power of the lower limb. Computed tomography scan and Magnetic Resonance Spectroscopy was suggestive of brain metastases. Given the rarity of primary lung neoplasms in children, clinical detection remains a challenge. Delayed diagnoses are common as respiratory symptoms may be attributed to inflammatory or infective processes. Primary pleuropulmonary synovial sarcoma is a rare tumour and it is not known to commonly metastasise to the brain. Though rare, primary pleuropulmonary SS should be considered an important differential among peadiatric primary lung neoplasms due to its potential for curability if detected early, and more aggressive metastatic pattern, e.g. brain metastases making early detection imperative.

  13. Characterization and management of patients with retroperitoneal sarcoma, Hospital San Juan de Dios 2007-2012

    International Nuclear Information System (INIS)

    Batista Rodriguez, Gabriela

    2013-01-01

    Patients diagnosed with retroperitoneal sarcoma at the Hospital San Juan de Dios were characterized during the years 2007 to 2012. The population framework was taken from the hospital's Pathology service database. A total of 15 patients older than 12 years with a diagnosis of sarcoma or malignant fibrous histiocytoma and that the site of origin of the biopsy was the retroperitoneum were selected. The data of the selected patients were collected through a data collection form. The variables included were: age, sex, origin, dates of importance (income, discharge, surgery, complication), presence and type of complications, pathological staging, type of surgery, comorbidities, presence and date of recurrence, treatment with chemotherapy and radiotherapy, death and causes of death. A descriptive analysis with the calculation of frequency measurements (absolute and relative) was carried out to describe the occurrence of the event. The analysis of proportional risks was performed considering the relationship between death and time, the possible relationship with the service of approach, clinical stage and resection status was also assessed. Information on diagnosis, evaluation, staging and management of retroperitoneal sarcomas is cited in the theoretical framework [es

  14. Evaluation of c-kit expression in classic Kaposi's sarcoma in a cohort of Egyptian patients

    International Nuclear Information System (INIS)

    Hussein, T.M.; El-Sabaa, B.M.; Hanafy, N.F.

    2012-01-01

    Kaposis sarcoma (KS) is in angio proliferative disorder associated with human herpes virus 8 infection. Classic Ks is the most prevalent type of KS in countries of the Mediterranean basin including Egypt. Several in vitro studies have detected C-kit expression in AIDS related-KS however, only a few studies addressed this Issue In the classic type with no data on the ethnicity of studied cases. The prospect of installing targeted anti-c-kit treatment to KS patients presents a promising avenue in KS therapeutics. Aim: To elucidate the expression of c-kit in classic KS cases and study possible relations with expression of HHV8 latency-associated nuclear antigen-1 (LANA-1) and other clinico pathological parameters. Methods: Twenty four cases of classic KS of the plaque and nodular stages in the lower limb were studied. Immunohistochemical detection of HHV8-LANA-1 and c-kit was carried out on archival paraffin embedded tissue, possession of the pathology and dermatology Departments, Alexandria School Of Medicine, Egypt. Statistical analysis of possible reltions between both antigen and clinico pathological parameters (patient age and gender and histological stage) was performed. Results: HHV8 expression was detected in 100% of cases while c-kit immunoreactivity was found in 54.2% of cases. There was no correlation between c-kit and HHV8 immunoreactivity or any of the studied clinico pathological parameters. Conclusions: This is the first report of c-kit expression in classic KS in an ethnically homogeneous cohort of arabs of the Mediterranean region. We detected c-kit expression in about half the cases with no relationship to HHV8 LANA expression or clinico pathological parameters

  15. Treatment Option Overview (Kaposi Sarcoma)

    Science.gov (United States)

    ... Treatment Childhood Vascular Tumors Treatment Research Kaposi Sarcoma Treatment (PDQ®)–Patient Version General Information About Kaposi Sarcoma ... Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery ) and treatment ...

  16. Oncolytic Maraba Virus MG1 as a Treatment for Sarcoma.

    Science.gov (United States)

    Le Boeuf, Fabrice; Selman, Mohammed; Son, Hwan Hee; Bergeron, Anabel; Chen, Andrew; Tsang, Jovian; Butterwick, Derek; Arulanandam, Rozanne; Forbes, Nicole E; Tzelepis, Fanny; Bell, John C; Werier, Joel; Abdelbary, Hesham; Diallo, Jean-Simon

    2017-09-15

    The poor prognosis of patients with advanced bone and soft-tissue sarcoma has not changed in the past several decades, highlighting the necessity for new therapeutic approaches. Immunotherapies, including oncolytic viral (OV) therapy, have shown great promise in a number of clinical trials for a variety of tumor types. However, the effective application of OV in treating sarcoma still remains to be demonstrated. Although few pre-clinical studies using distinct OVs have been performed and demonstrated therapeutic benefit in sarcoma models, a side-by-side comparison of clinically relevant OV platforms has not been performed. Four clinically relevant OV platforms (Reovirus, Vaccinia virus, Herpes-simplex virus and Rhabdovirus) were screened for their ability to infect and kill human and canine sarcoma cell lines in vitro, and human sarcoma specimens ex vivo. In vivo treatment efficacy was tested in a murine model. The rhabdovirus MG1 demonstrated the highest potency in vitro. Ex vivo, MG1 productively infected more than 80% of human sarcoma tissues tested, and treatment in vivo led to a significant increase in long-lasting cures in sarcoma-bearing mice. Importantly, MG1 treatment induced the generation of memory immune response that provided protection against a subsequent tumor challenge. This study opens the door for the use of MG1-based oncolytic immunotherapy strategies as treatment for sarcoma or as a component of a combined therapy. © 2017 UICC.

  17. A case of clear cell sarcoma

    DEFF Research Database (Denmark)

    Juel, Jacob; Ibrahim, Rami Mossad

    2017-01-01

    INTRODUCTION: Clear cell sarcoma (CCS) is a rare tumour of the soft tissue often misdiagnosed, as it shares characteristics with malignant melanoma (MM). Previously, CCS has been characterised, as malignant melanoma of the soft tissue, contemporary immunohistochemical techniques, however, have made...... this designation obsolete. The true incidence remains unknown, but CCS is believed to represent less than one percent of all sarcomas. PRESENTATION OF CASE: A 22-year-old patient presented with a mass sized 2.6×2.7×2.7cm of the left gluteal region, pain, and malaise. Initially, the symptoms were interpreted...

  18. Primary Pulmonary Ewing's Sarcoma: Rare Cause of Superior Vena Cava Syndrome in Children.

    Science.gov (United States)

    Mehra, Shibani; Atwal, Swapndeep Singh; Garga, Umesh Chandra

    2014-08-01

    Ewing's sarcoma is a common malignant bone tumour presenting in children and young adults. Rarely extra- skeletal soft tissues and visceral organs can also be the site of origin of Ewing's sarcoma. Primary pulmonary Ewing's sarcoma is an extremely rare malignancy which occurs in the paediatric population. We report an unusual case of primary pulmonary Ewing's sarcoma in a nine year old girl who presented with features of superior vena cava syndrome in the emergency department. The diagnosis was confirmed pathologically both by light microscopy and immunohistochemistry. The patient was put on chemotherapy and surgery was planned but the patient expired within three days of starting chemotherapy.

  19. The Danish Sarcoma Database

    DEFF Research Database (Denmark)

    Jørgensen, Peter Holmberg; Lausten, Gunnar Schwarz; Pedersen, Alma B

    2016-01-01

    AIM: The aim of the database is to gather information about sarcomas treated in Denmark in order to continuously monitor and improve the quality of sarcoma treatment in a local, a national, and an international perspective. STUDY POPULATION: Patients in Denmark diagnosed with a sarcoma, both...... skeletal and ekstraskeletal, are to be registered since 2009. MAIN VARIABLES: The database contains information about appearance of symptoms; date of receiving referral to a sarcoma center; date of first visit; whether surgery has been performed elsewhere before referral, diagnosis, and treatment; tumor...... of Diseases - tenth edition codes and TNM Classification of Malignant Tumours, and date of death (after yearly coupling to the Danish Civil Registration System). Data quality and completeness are currently secured. CONCLUSION: The Danish Sarcoma Database is population based and includes sarcomas occurring...

  20. Utility of whole-body (head-to-toe) PET/CT in the evaluation of melanoma and sarcoma patients.

    Science.gov (United States)

    Webb, Heather R; Latifi, Hamid R; Griffeth, Landis K

    2018-01-01

    The aim of this study was to assess the added benefit of whole-body (head-to-toes) PET/CT versus routine 'eyes-to-thighs' PET/CT of melanoma and sarcoma patients. We performed a retrospective review of consecutive whole-body PET/CT scans from January 2006 through December 2010 in patients with melanoma or sarcoma. PET abnormalities in the brain, distal thighs, and legs were recorded and clinical significance was assessed on the basis of pathology, imaging studies, and clinical follow-up. Patients with known primary lesions distal to the proximal femora were excluded as these patients would routinely undergo 'head-to-toe' PET/CT. We reviewed reports from 352 PET/CT examinations in 194 patients with melanoma and 75 PET/CT examinations in 44 patients with sarcoma. Melanoma: 13 patients had brain metastases on PET. In five of these patients, lesions were unknown, but all were in the setting of other metastatic disease. Twenty-seven patients had lower extremity metastases, all in the setting of other metastatic disease. No lower extremity metastases were found in the remaining 167 patients. Sarcoma: one patient had an isolated, unexpected brain metastasis. Six patients had leg metastases, but none were isolated. No lower extremity metastases were found in the remaining 38 patients. In patients with melanoma and sarcoma, inclusion of entire lower extremities adds little additional clinical value as detection of isolated, unexpected metastasis is rare. Brain imaging may add value as the presence of brain metastases alters clinical management. Overall, in patients with melanoma or sarcoma, whole-brain PET/CT imaging may be of value, but routine inclusion of the entire lower extremities adds little additional value.

  1. Magnetic resonance imaging features of extremity sarcomas of uncertain differentiation

    International Nuclear Information System (INIS)

    Stacy, G.S.; Nair, L.

    2007-01-01

    The purpose of this review is to illustrate the pertinent clinical and imaging features of extremity sarcomas of uncertain differentiation, including synovial sarcoma, epithelioid sarcoma, clear-cell sarcoma, and alveolar soft part sarcoma. These tumours should be considered in the differential diagnosis when a soft-tissue mass is encountered in the extremity of an adolescent or young adult

  2. Neo-adjuvant chemotherapy alone or with regional hyperthermia for localised high-risk soft-tissue sarcoma: a randomised phase 3 multicentre study

    DEFF Research Database (Denmark)

    Issels, Rolf D; Lindner, Lars H; Verweij, Jaap

    2010-01-01

    The optimum treatment for high-risk soft-tissue sarcoma (STS) in adults is unclear. Regional hyperthermia concentrates the action of chemotherapy within the heated tumour region. Phase 2 studies have shown that chemotherapy with regional hyperthermia improves local control compared with chemother...

  3. A prognostic model for soft tissue sarcoma of the extremities and trunk wall based on size, vascular invasion, necrosis, and growth pattern

    DEFF Research Database (Denmark)

    Carneiro, Ana; Bendahl, Par-Ola; Engellau, Jacob

    2011-01-01

    type, necrosis, and grade. METHODS:: Whole-tumor sections from 239 soft tissue sarcomas of the extremities were reviewed for the following prognostic factors: size, vascular invasion, necrosis, and growth pattern. A new prognostic model, referred to as SING (Size, Invasion, Necrosis, Growth...

  4. The irony of highly-effective bacterial therapy of a patient-derived orthotopic xenograft (PDOX) model of Ewing's sarcoma, which was blocked by Ewing himself 80 years ago.

    Science.gov (United States)

    Murakami, Takashi; Kiyuna, Tasuku; Kawaguchi, Kei; Igarashi, Kentaro; Singh, Arun S; Hiroshima, Yukihiko; Zhang, Yong; Zhao, Ming; Miyake, Kentaro; Nelson, Scott D; Dry, Sarah M; Li, Yunfeng; DeLong, Jonathan C; Lwin, Thinzar M; Chishima, Takashi; Tanaka, Kuniya; Bouvet, Michael; Endo, Itaru; Eilber, Fritz C; Hoffman, Robert M

    2017-06-03

    William B. Coley developed bacterial therapy of cancer more than 100 years ago and had clinical success. James Ewing, a very famous cancer pathologist for whom the Ewing sarcoma is named, was Coley's boss at Memorial Hospital in New York and terminated Coley's bacterial therapy of cancer. A tumor from a patient with soft-tissue Ewing's sarcoma, who failed doxorubicin (DOX) therapy, was previously implanted in nude mice to establish a patient-derived orthotopic xenograft (PDOX) model. In the present study, the Ewing's sarcoma PDOX was treated with tumor-targeting S. typhimurium A1-R expressing green fluorescent (GFP), alone and in combination with DOX. S. typhimurium A1-R-GFP was detected in the tumors after intratumor (i.t.) or intravenous (i.v.) injection. The combination of S. typhimurium A1-R and DOX significantly reduced tumor weight (37.8 ± 15.6 mg) compared to the untreated control (73.8 ± 10.1 mg, P Ewing's sarcoma had in the patient. S. typhimurium A1-R converted the Ewing's sarcoma from DOX resistant to sensitive. One can only wonder how bacterial therapy and immunotherapy of cancer would have developed over the past 80 years if Ewing did not stop Coley.

  5. Soft tissue sarcomas of the extremities: survival and patterns of failure with conservative surgery and postoperative irradiation compared to surgery alone

    International Nuclear Information System (INIS)

    Leibel, S.A.; Tranbaugh, R.F.; Wara, W.M.; Beckstead, J.H.; Bovill, E.G.; Phillips, T.L.

    1982-01-01

    Between 1960 and 1978, 81 patients received their primary treatment for localized soft tissue sarcomas of the extremities, buttock and shoulder at the University of California, San Francisco. Initial treatment consisted of surgery alone in 47 patients, planned conservative surgery followed by radiation therapy in 29 patients, and irradiation alone in five patients. The two- and five-year determinate survival for all cases was 86% and 73%, respectively. The local control rate achieved with surgery alone was related to the extent of surgery. Eighty-seven percent (14/16) of the patients undergoing amputation were locally controlled. Seventy-two percent (8/11) were treated with wide en bloc resection and had local tumor control while only 30% (6/20) having simple excision were controlled. The local control rate with surgery and postoperative irradiation was 90% (26/29). No patients treated with irradiation therapy alone were controlled. This review suggests that local tumor control achieved with limb preserving conservative surgery and postoperative irradiation is superior to limited surgery alone. The survival and patterns of failure of patients undergoing radical surgery is comparable to combined treatment with the risk-benefit ratio favoring the latter

  6. Synovial sarcoma of the foot

    International Nuclear Information System (INIS)

    Beus, J.; Kreitner, K.F.; Rompe, J.D.; Riehle, H.M.

    1996-01-01

    The case of a 29 year-old female patient who had experienced pain in the right midfoot for 5 years which was diagnosed as a degenerative or rheumatic change and treated by physiotherapy and medication. By means of magnetic resonance imaging we identified a soft-tissue tumor of the midfoot. Histology provided the findings of a monophasic fibrous synovial sarcoma. The case history is reported together with a presentation of the disease and its radiological diagnosis. (orig.) [de

  7. Cirurgia conservadora, radioterapia externa e reforço de dose com braquiterapia de alta taxa de dose: uma nova perspectiva no tratamento de sarcomas de partes moles do adulto Limb-sparing surgery, external beam radiotherapy and boost with high-dose rate brachytherapy: a new perspective for the treatment of soft tissue sarcomas in adults

    Directory of Open Access Journals (Sweden)

    Antonio Cássio Assis Pellizzon

    2002-03-01

    Full Text Available OBJETIVO: Avaliar a influência no controle local de pacientes adultos e portadores de sarcoma de partes moles em extremidades e submetidos a cirurgia conservadora do membro, com braquiterapia de alta taxa de dose (BATD como reforço para a radioterapia externa (RT. MATERIAL E MÉTODOS: Foram avaliados 16 pacientes tratados, de 1993 até 1999. A RT foi utilizada com finalidade pré ou pós-operatória (30--55 Gy e BATD com dose de 18--36 Gy (fx 3--6 Gy BID. Com base no modelo linear quadrático calculou-se a dose efetiva biológica ("biological effective dose" - BED para o tumor e comparou-se seu valor a dados da literatura internacional, que utiliza tratamentos com RT e braquiterapia de baixa taxa de dose (BBTD. RESULTADOS: Os valores médios e medianos da BED para os sarcomas de partes moles foram de 78,5 Gy7 e 80 Gy7. A análise univariada mostrou que a BED para o tumor, quando utilizada BATD, era semelhante ao valor de 83 Gy7 quando utilizada BBTD (p = 0,008. As taxas de controle local, sobrevida livre de doença e sobrevida global atuarial em cinco anos foram de 83,2%, 75% e 93,7%, respectivamente. CONCLUSÕES: A BATD, quando utilizada como método complementar no reforço de dose da RT no tratamento conservador dos sarcomas de partes moles, apresenta taxas de controle local equiparáveis às da literatura internacional; no entanto, estudos com número maior de pacientes e período maior de seguimento são ainda necessários para determinar o verdadeiro potencial da BATD em substituir a BBTD.PURPOSE: To evaluate the influence on local control in adult patients with soft tissue sarcomas of the limbs that underwent limb-sparing surgery and high-dose rate brachytherapy (HDRB in association with teletherapy. MATERIAL AND METHODS: Sixteen patients treated from 1993 to 1999 were reviewed. Teletherapy was used pre- or postoperatively (30--55 Gy in association with HDRB in a dose range of 18--36 Gy (fx 3--6 Gy BID. The linear quadratic model was

  8. Safety, pharmacokinetics, and preliminary activity of the anti-IGF-1R antibody figitumumab (CP-751,871) in patients with sarcoma and Ewing's sarcoma: a phase 1 expansion cohort study.

    Science.gov (United States)

    Olmos, David; Postel-Vinay, Sophie; Molife, L Rhoda; Okuno, Scott H; Schuetze, Scott M; Paccagnella, M Luisa; Batzel, Gretchen N; Yin, Donghua; Pritchard-Jones, Kathryn; Judson, Ian; Worden, Francis P; Gualberto, Antonio; Scurr, Michelle; de Bono, Johann S; Haluska, Paul

    2010-02-01

    Figitumumab is a fully human IgG2 monoclonal antibody targeting the insulin-like growth-factor-1 receptor (IGF-1R). Preclinical data suggest a dependence on insulin-like growth-factor signalling for sarcoma subtypes, including Ewing's sarcoma, and early reports show antitumour activity of IGF-1R-targeting drugs in these diseases. Between January, 2006, and August, 2008, patients with refractory, advanced sarcomas received figitumumab (20 mg/kg) in two single-stage expansion cohorts within a solid-tumour phase 1 trial. The first cohort (n=15) included patients with multiple sarcoma subtypes, age 18 years or older, and the second cohort (n=14) consisted of patients with refractory Ewing's sarcoma, age 9 years or older. The primary endpoint was to assess the safety and tolerability of figitumumab. Secondary endpoints included pharmacokinetic profiling and preliminary antitumour activity (best response by Response Evaluation Criteria in Solid Tumours [RECIST]) in evaluable patients who received at least one dose of medication. This study is registered with ClinicalTrials.gov, number NCT00474760. 29 patients, 16 of whom had Ewing's sarcoma, were enrolled and received a total of 177 cycles of treatment (median 2, mean 6.1, range 1-24). Grade 3 deep venous thrombosis, grade 3 back pain, and grade 3 vomiting were each noted once in individual patients; one patient had grade 3 increases in aspartate aminotransferase and gammaglutamyltransferase concentrations. This patient also had grade 4 increases in alanine aminotransferase concentrations. The only other grade 4 adverse event was raised concentrations of uric acid, noted in one patient. Pharmacokinetics were comparable between patients with sarcoma and those with other solid tumours. 28 patients were assessed for response; two patients, both with Ewing's sarcoma, had objective responses (one complete response and one partial response) and eight patients had disease stabilisation (six with Ewing's sarcoma, one with

  9. Analysis of gene expression profiles of soft tissue sarcoma using a combination of knowledge-based filtering with integration of multiple statistics.

    Directory of Open Access Journals (Sweden)

    Anna Takahashi

    Full Text Available The diagnosis and treatment of soft tissue sarcomas (STS have been difficult. Of the diverse histological subtypes, undifferentiated pleomorphic sarcoma (UPS is particularly difficult to diagnose accurately, and its classification per se is still controversial. Recent advances in genomic technologies provide an excellent way to address such problems. However, it is often difficult, if not impossible, to identify definitive disease-associated genes using genome-wide analysis alone, primarily because of multiple testing problems. In the present study, we analyzed microarray data from 88 STS patients using a combination method that used knowledge-based filtering and a simulation based on the integration of multiple statistics to reduce multiple testing problems. We identified 25 genes, including hypoxia-related genes (e.g., MIF, SCD1, P4HA1, ENO1, and STAT1 and cell cycle- and DNA repair-related genes (e.g., TACC3, PRDX1, PRKDC, and H2AFY. These genes showed significant differential expression among histological subtypes, including UPS, and showed associations with overall survival. STAT1 showed a strong association with overall survival in UPS patients (logrank p = 1.84 × 10(-6 and adjusted p value 2.99 × 10(-3 after the permutation test. According to the literature, the 25 genes selected are useful not only as markers of differential diagnosis but also as prognostic/predictive markers and/or therapeutic targets for STS. Our combination method can identify genes that are potential prognostic/predictive factors and/or therapeutic targets in STS and possibly in other cancers. These disease-associated genes deserve further preclinical and clinical validation.

  10. Current State of Pediatric Sarcoma Biology and Opportunities for Future Discovery: A Report from the Sarcoma Translational Research Workshop

    Science.gov (United States)

    Hingorani, Pooja; Janeway, Katherine; Crompton, Brian D.; Kadoch, Cigall; Mackall, Crystal L.; Khan, Javed; Shern, Jack F.; Schiffman, Joshua; Mirabello, Lisa; Savage, Sharon A.; Ladanyi, Marc; Meltzer, Paul; Bult, Carol J.; Adamson, Peter C.; Lupo, Philip J.; Mody, Rajen; DuBois, Steven G.; Parsons, D. Williams; Khanna, Chand; Lau, Ching; Hawkins, Douglas S.; Randall, R. Lor; Smith, Malcolm; Sorensen, Poul H.; Plon, Sharon E.; Skapek, Stephen X.; Lessnick, Stephen; Gorlick, Richard; Reed, Damon R.

    2017-01-01

    Sarcomas are a rare subgroup of pediatric cancers comprised of a variety of bone and soft-tissue tumors. While significant advances have been made in improving outcomes of patients with localized pediatric sarcomas since the addition of systemic chemotherapy to local control many decades ago, outcomes for patients with metastatic and relapsed sarcoma remain poor with few novel therapeutics identified to date. With the advent of new technologies to study cancer genomes, transcriptomes and epigenomes, our understanding of sarcoma biology has improved tremendously in a relatively short period of time. However, much remains to be accomplished in this arena especially with regard to translating all of this new knowledge to the bedside. To this end, a meeting was convened in Philadelphia, PA on April 18, 2015 sponsored by the QuadW foundation, Children’s Oncology Group and CureSearch for Children’s Cancer that brought together sarcoma clinicians and scientists from North America to review the current state of pediatric sarcoma biology and ongoing/planned genomics based clinical trials in an effort to identify and bridge knowledge gaps that continue to exist at the current time. At the conclusion of the workshop, three key objectives that would significantly further our understanding of sarcoma were identified and a proposal was put forward to develop an all-encompassing pediatric sarcoma biology protocol that would address these specific needs. This review summarizes the proceedings of the workshop. PMID:27132463

  11. Whole-tumor apparent diffusion coefficient (ADC) histogram analysis to differentiate benign peripheral neurogenic tumors from soft tissue sarcomas.

    Science.gov (United States)

    Nakajo, Masanori; Fukukura, Yoshihiko; Hakamada, Hiroto; Yoneyama, Tomohide; Kamimura, Kiyohisa; Nagano, Satoshi; Nakajo, Masayuki; Yoshiura, Takashi

    2018-02-22

    Apparent diffusion coefficient (ADC) histogram analyses have been used to differentiate tumor grades and predict therapeutic responses in various anatomic sites with moderate success. To determine the ability of diffusion-weighted imaging (DWI) with a whole-tumor ADC histogram analysis to differentiate benign peripheral neurogenic tumors (BPNTs) from soft tissue sarcomas (STSs). Retrospective study, single institution. In all, 25 BPNTs and 31 STSs. Two-b value DWI (b-values = 0, 1000s/mm 2 ) was at 3.0T. The histogram parameters of whole-tumor for ADC were calculated by two radiologists and compared between BPNTs and STSs. Nonparametric tests were performed for comparisons between BPNTs and STSs. P histogram parameters except kurtosis and entropy differed significantly between BPNTs and STSs. 3 Technical Efficacy: Stage 2 J. Magn. Reson. Imaging 2018. © 2018 International Society for Magnetic Resonance in Medicine.

  12. Clinicians' adherence versus non adherence to practice guidelines in the management of patients with sarcoma: a cost-effectiveness assessment in two European regions

    Directory of Open Access Journals (Sweden)

    Perrier Lionel

    2012-03-01

    Full Text Available Abstract Background Although the management of sarcoma is improving, non adherence to clinical practice guidelines (CPGs remains high, mainly because of the low incidence of the disease and the variety of histological subtypes. Since little is known about the health economics of sarcoma, we undertook a cost-effectiveness analysis (within the CONnective TIssue CAncer NETwork, CONTICANET comparing costs and outcomes when clinicians adhered to CPGs and when they did not. Methods Patients studied had a histological diagnosis of sarcoma, were older than 15 years, and had been treated in the Rhône-Alpes region of France (in 2005/2006 or in the Veneto region of Italy (in 2007. Data collected retrospectively for the three years after diagnosis were used to determine relapse free survival and health costs (adopting the hospital's perspective and a microcosting approach. All costs were expressed in euros (€ at their 2009 value. A 4% annual discount rate was applied to both costs and effects. The incremental cost-effectiveness ratio (ICER was expressed as cost per relapse-free year gained when management was compliant with CPGs compared with when it was not. To capture uncertainty surrounding ICER, a probabilistic sensitivity analysis was performed based on a non-parametric bootstrap method. Results A total of 219 patients were included in the study. Compliance with CPGs was observed for 118 patients (54%. Average total costs reached 23,571 euros when treatment was in accordance with CPGs and 27,313 euros when it was not. In relation to relapse-free survival, compliance with CPGs strictly dominates non compliance, i.e. it is both less costly and more effective. Taking uncertainty into account, the probability that compliance with CPGs still strictly dominates was 75%. Conclusions Our findings should encourage physicians to increase their compliance with CPGs and healthcare administrators to invest in the implementation of CPGs in the management of

  13. [{sup 18}F]FMISO and [{sup 18}F]FDG PET imaging in soft tissue sarcomas: correlation of hypoxia, metabolism and VEGF expression

    Energy Technology Data Exchange (ETDEWEB)

    Rajendran, J.G.; Peterson, L.M.; Grierson, J.R.; Eary, J.F. [Division of Nuclear Medicine, Department of Radiology, University of Washington Medical Center, Box 356113, WA 98195, Seattle (United States); Wilson, D.C. [Radiation Oncology, British Columbia Cancer Control Agency, Vancouver, BC (Canada); Conrad, E.U.; Bruckner, J.D. [Department of Orthopedic Surgery, University of Washington Medical Center, Seattle, Washington (United States); Rasey, J.S.; Chin, L.K.; Hofstrand, P.D. [Department of Radiation Oncology, University of Washington Medical Center, Seattle, Washington (United States); Krohn, K.A. [Division of Nuclear Medicine, Department of Radiology, University of Washington Medical Center, Box 356113, WA 98195, Seattle (United States); Department of Radiation Oncology, University of Washington Medical Center, Seattle, Washington (United States)

    2003-05-01

    Hypoxia imparts resistance to radiotherapy and chemotherapy and also promotes a variety of changes in tumor biology through inducible promoters. The purpose of this study was to evaluate the use of positron emission tomography (PET) imaging with fluorine-18 fluoromisonidazole (FMISO) in soft tissue sarcomas (STS) as a measure of hypoxia and to compare the results with those obtained using [{sup 18}F]fluorodeoxyglucose (FDG) and other known biologic correlates. FDG evaluates energy metabolism in tumors while FMISO uptake is proportional to tissue hypoxia. FMISO uptake was compared with FDG uptake. Vascular endothelial growth factor (VEGF) expression was also compared with FMISO uptake. Nineteen patients with STS underwent PET scanning with quantitative determination of FMISO and FDG uptake prior to therapy (neo-adjuvant chemotherapy or surgery alone). Ten patients receiving neo-adjuvant chemotherapy were also imaged after chemotherapy but prior to surgical resection. Standardized uptake value (SUV) was used to describe FDG uptake; regional tissue to blood ratio ({>=}1.2 was considered significant) was used for FMISO uptake. Significant hypoxia was found in 76% of tumors imaged prior to therapy. No correlation was identified between pretherapy hypoxic volume (HV) and tumor grade (r=0.15) or tumor volume (r=0.03). The correlation of HV with VEGF expression was 0.39. Individual tumors showed marked heterogeneity in regional VEGF expression. The mean pixel-by-pixel correlation between FMISO and FDG uptake was 0.49 (range 0.09-0.79) pretreatment and 0.32 (range -0.46-0.72) after treatment. Most tumors showed evidence of reduced uptake of both FMISO and FDG following chemotherapy. FMISO PET demonstrates areas of significant and heterogeneous hypoxia in soft tissue sarcomas. The significant discrepancy between FDG and FMISO uptake seen in this study indicates that regional hypoxia and glucose metabolism do not always correlate. Similarly, we did not find any relationship

  14. Chemokines in Ewing sarcoma

    NARCIS (Netherlands)

    Sand, L.G.L.

    2016-01-01

    Ewing sarcoma is an aggressive primary malignant bone tumor with high degree of tumor vascularization and is the second most common sarcoma of bone in children and young adults. Patients with disseminated disease at diagnosis or early relapse have a poor prognosis. To identify novel therapies and

  15. Ewing sarcoma versus osteomyelitis: differential diagnosis with magnetic resonance imaging

    International Nuclear Information System (INIS)

    Henninger, B.; Glodny, B.; Rudisch, A.; Trieb, T.; Loizides, A.; Judmaier, W.; Schocke, M.F.; Putzer, D.

    2013-01-01

    To find and evaluate characteristic magnetic resonance imaging (MRI) patterns for the differentiation between Ewing sarcoma and osteomyelitis. We identified 28 consecutive patients referred to our department for MRI (1.5 T) of an unclear bone lesion with clinical symptoms suggestive of Ewing sarcoma or osteomyelitis. MRI scans were re-evaluated by two experienced radiologists, typical MR imaging features were documented and a diagnostic decision between Ewing sarcoma and osteomyelitis was made. Statistical significance of the association between MRI features and the biopsy-based diagnosis was assessed using Fisher's exact test. The most clear-cut pattern for determining the correct diagnosis was the presence of a sharp and defined margin of the bone lesion, which was found in all patients with Ewing sarcoma, but in none of the patients with osteomyelitis (P < 0.0001). Contrast enhancing soft tissue was present in all cases with Ewing sarcoma and absent in 4 patients with osteomyelitis (P = 0.0103). Cortical destruction was found in all patients with Ewing sarcoma, 4 patients with osteomyelitis did not present any cortical reaction (P = 0.0103). Cystic or necrotic areas were identified in 13 patients with Ewing sarcoma and in 1 patient with osteomyelitis (P = 0.004). Interobserver reliability was very good (kappa = 1) in Ewing sarcoma and moderate (kappa = 0.6) in patients with osteomyelitis. A sharp and defined margin, optimally visualized on T1-weighted images in comparison to short tau inversion recovery (STIR) images, is the most significant feature of Ewing sarcoma in differentiating from osteomyelitis. (orig.)

  16. Prevalence and clinical impact of anaplasia in childhood rhabdomyosarcoma : a report from the Soft Tissue Sarcoma Committee of the Children's Oncology Group.

    Science.gov (United States)

    Qualman, Stephen; Lynch, James; Bridge, Julia; Parham, David; Teot, Lisa; Meyer, William; Pappo, Alberto

    2008-12-01

    Anapalsia is rare in childhood rhabdomyosarcoma and has not been included in the International Classification of Rhabdomyosarcoma (ICR). A recent review of cases from the Soft Tissue Sarcoma Committee of the Children's Oncology Group (COG) suggests that anaplasia might be more common than previously reported and may impact clinical outcome. The prevalence of anaplasia (focal or diffuse) was prospectively assessed in 546 eligible cases who were registered in an Intergroup Rhabdomyosarcoma Study Group (IRSG) or COG therapeutic trial from 1995 through 1998. The incidence of anaplasia in tumor samples and its impact in predicting clinical outcome was assessed. Overall, 71 (13%) of all samples analyzed had anaplasia. Anaplasia was more common in patients with tumors in favorable sites and was less commonly observed in younger patients and in those with stage II, III, or clinical group III disease. Regardless of its distribution (focal or diffuse), on univariate analysis the presence of anaplasia negatively influenced the failure-free survival rate (63% vs 77% at 5 years) and overall survival (68% vs 82% at 5 years) rates in patients with embryonal rhabdomyosarcoma. This effect was most pronounced in children with intermediate-risk tumors. Anaplasia did not affect outcome in patients with alveolar tumors. The incidence of anaplasia in patients with rhabdomyosarcoma is higher than previously described and may be of prognostic significance in children with intermediate-risk embryonal rhabdomyosarcoma. (c) 2008 American Cancer Society

  17. Age dependency of primary tumor sites and metastases in patients with Ewing sarcoma.

    Science.gov (United States)

    Worch, Jennifer; Ranft, Andreas; DuBois, Steven G; Paulussen, Michael; Juergens, Heribert; Dirksen, Uta

    2018-06-01

    The median age of patients with Ewing sarcoma (EwS) at diagnosis is around 14-15 years. Older age is associated with a worse outcome. The correlation of age at diagnosis on sites of disease has not been fully described. The goal of this study was to evaluate the differences in sites of primary tumor and metastatic tumor involvement according to age groups. EwS data from the Gesellschaft für Pädiatrische Onkologie und Hämatology (GPOH) database of the Cooperative Ewing Sarcoma Study (CESS) 81/86 and the European Intergroup Cooperative Ewing's Sarcoma Study EICESS 92 and the EUROpean Ewing tumor Working Initiative of National Groups-99-Protocol (EURO-E.W.I.N.G.-99) study were analyzed. Patient and tumor characteristics were evaluated statistically using chi square tests. The study population included 2,635 patients with bone EwS. Sites of primary and metastatic tumors differed according to the age groups of young children (0-9 years), early adolescence (10-14 years), late adolescence (15-19 years), young adults (20-24 years), and adults (more than 24 years). Young children demonstrated the most striking differences in site of disease with a lower proportion of pelvic primary and axial tumors. They presented less often with metastatic disease at diagnosis. Site of primary and metastatic tumor involvement in EwS differs according to patient age. The biological and developmental etiology for these differences requires further investigations. © 2018 Wiley Periodicals, Inc.

  18. Detection of SYT-SSX mutant transcripts in formalin-fixed paraffin-embedded sarcoma tissues using one-step reverse transcriptase real-time PCR.

    Science.gov (United States)

    Norlelawati, A T; Mohd Danial, G; Nora, H; Nadia, O; Zatur Rawihah, K; Nor Zamzila, A; Naznin, M

    2016-04-01

    Synovial sarcoma (SS) is a rare cancer and accounts for 5-10% of adult soft tissue sarcomas. Making an accurate diagnosis is difficult due to the overlapping histological features of SS with other types of sarcomas and the non-specific immunohistochemistry profile findings. Molecular testing is thus considered necessary to confirm the diagnosis since more than 90% of SS cases carry the transcript of t(X;18)(p11.2;q11.2). The purpose of this study is to diagnose SS at molecular level by testing for t(X;18) fusion-transcript expression through One-step reverse transcriptase real-time Polymerase Chain Reaction (PCR). Formalin-fixed paraffin-embedded tissue blocks of 23 cases of soft tissue sarcomas, which included 5 and 8 cases reported as SS as the primary diagnosis and differential diagnosis respectively, were retrieved from the Department of Pathology, Tengku Ampuan Afzan Hospital, Kuantan, Pahang. RNA was purified from the tissue block sections and then subjected to One-step reverse transcriptase real-time PCR using sequence specific hydrolysis probes for simultaneous detection of either SYT-SSX1 or SYT-SSX2 fusion transcript. Of the 23 cases, 4 cases were found to be positive for SYT-SSX fusion transcript in which 2 were diagnosed as SS whereas in the 2 other cases, SS was the differential diagnosis. Three cases were excluded due to failure of both amplification assays SYT-SSX and control β-2-microglobulin. The remaining 16 cases were negative for the fusion transcript. This study has shown that the application of One-Step reverse transcriptase real time PCR for the detection SYT-SSX transcript is feasible as an aid in confirming the diagnosis of synovial sarcoma.

  19. Prognostic factors for survival in patients with Ewing's sarcoma using the surveillance, epidemiology, and end results (SEER) program database.

    Science.gov (United States)

    Duchman, Kyle R; Gao, Yubo; Miller, Benjamin J

    2015-04-01

    The current study aims to determine cause-specific survival in patients with Ewing's sarcoma while reporting clinical risk factors for survival. The Surveillance, Epidemiology, and End Results (SEER) Program database was used to identify patients with osseous Ewing's sarcoma from 1991 to 2010. Patient, tumor, and socioeconomic variables were analyzed to determine prognostic factors for survival. There were 1163 patients with Ewing's sarcoma identified in the SEER Program database. The 10-year cause-specific survival for patients with non-metastatic disease at diagnosis was 66.8% and 28.1% for patients with metastatic disease. Black patients demonstrated reduced survival at 10 years with an increased frequency of metastatic disease at diagnosis as compared to patients of other race, while Hispanic patients more frequently presented with tumor size>10cm. Univariate analysis revealed that metastatic disease at presentation, tumor size>10cm, axial tumor location, patient age≥20 years, black race, and male sex were associated with decreased cause-specific survival at 10 years. Metastatic disease at presentation, axial tumor location, tumor size>10cm, and age≥20 years remained significant in the multivariate analysis. Patients with Ewing's sarcoma have decreased cause-specific survival at 10 years when metastatic at presentation, axial tumor location, tumor size>10cm, and patient age≥20 years. Copyright © 2015 Elsevier Ltd. All rights reserved.

  20. Myeloid Sarcoma after Allogenic Stem Cell Transplantation for Acute Myeloid Leukemia: Successful Consolidation Treatment Approaches in Two Patients

    Directory of Open Access Journals (Sweden)

    Silje Johansen

    2018-01-01

    Full Text Available Myeloid sarcoma is an extramedullary (EM manifestation (i.e., manifestation outside the bone marrow of acute myeloid leukemia (AML; it is assumed to be relatively uncommon and can be the only manifestation of leukemia relapse after allogenic stem cell transplantation (allo-SCT. An EM sarcoma can manifest in any part of the body, although preferentially manifesting in immunological sanctuary sites as a single or multiple tumors. The development of myeloid sarcoma after allo-SCT is associated with certain cytogenetic abnormalities, developing of graft versus host disease (GVHD, and treatment with donor lymphocytes infusion (DLI. It is believed that posttransplant myeloid sarcomas develop because the EM sites evade immune surveillance. We present two patients with EM myeloid sarcoma in the breast and epipharynx, respectively, as the only manifestation of leukemia relapse. Both patients were treated with a combination of local and systemic therapy, with successfully longtime disease-free survival. Based on these two case reports, we give an updated review of the literature and discuss the pathogenesis, diagnosis, and treatment of EM sarcoma as the only manifestation of AML relapse after allo-SCT. There are no standard guidelines for the treatment of myeloid sarcomas in allotransplant recipients. In our opinion, the treatment of these patients needs to be individualized and should include local treatment (i.e., radiotherapy combined with systemic therapy (i.e., chemotherapy, immunotherapy, DLI, or retransplantation. The treatment has to consider both the need for sufficient antileukemic efficiency versus the risk of severe complications due to cumulative toxicity.

  1. Preoperative Radiotherapy and Wide Resection for Soft Tissue Sarcomas: Achieving a Low Rate of Major Wound Complications with the Use of Flaps. Results of a Single Surgical Team

    Directory of Open Access Journals (Sweden)

    Lester Wai Mon Chan

    2018-01-01

    Full Text Available BackgroundSurgery in combination with radiotherapy (RT has become the standard of care for most soft tissue sarcomas. The choice between pre- and postoperative RT is controversial. Preoperative RT is associated with a 32–35% rate of major wound complications (MWC and 16–25% rate of reoperation. The role of vascularized soft tissue “flaps” in reducing complications is unclear. We report the outcomes of patients treated with preoperative RT, resection, and flap reconstruction.Patients and methods122 treatment episodes involving 117 patients were retrospectively reviewed. All patients were treated with 50.4 Gy of external beam radiation. Surgery was performed at 4–8 weeks after completion of RT by the same combination of orthopedic oncology and plastic reconstructive surgeon. Defects were reconstructed with 64 free and 59 pedicled/local flaps.Results30 (25% patients experienced a MWC and 17 (14% required further surgery. 20% of complications were exclusively related to the donor site. There was complete or partial loss of three flaps. There was no difference in the rate of MWC or reoperation for complications with respect to age, sex, tumor site, previous unplanned excision, tumor grade, depth, and type of flap. Tumor size ≥8 cm was associated with a higher rate of reoperation (11/44 vs 6/78; P = 0.008 but the rate of MWC was not significant (16/44 vs 14/78; P = 0.066.ConclusionThe use of soft tissue flaps is associated with a low rate of MWC and reoperation. Our results suggest that a high rate of flap usage may be required to observe a reduction in complication rates.

  2. The reverse protraction factor in the induction of bone sarcomas in radium-224 patients

    International Nuclear Information System (INIS)

    Chmelevsky, D.; Spiess, H.; Mays, C.W.; Kellerer, A.M.

    1990-01-01

    More than 50 bone sarcomas have occurred among a collective of about 800 patients who had been injected in Germany after World War II with large activities of radium-224 for the intended treatment of bone tuberculosis and ankylosing spondylitis. In an earlier analysis it was concluded that, at equal mean absorbed doses in the skeleton, patients with longer exposure time had a higher incidence of bone sarcomas. The previous analysis was based on approximations; in particular, it did not account for the varying times at risk of the individual patients. In view of the implications of a reverse protraction factor for basic considerations in radiation protection, the need was therefore felt to reevaluate the data from the continued follow-up by more rigorous statistical methods. A first step of the analysis demonstrates the existence of the reverse dose-rate effect in terms of a suitably constructed rank-order test. In a second step of the analysis it is concluded that the data are consistent with a linear no-threshold dose dependence under the condition of constant exposure time, while there is a steeper than linear dependence on dose when the exposure times increase proportionally to dose. A maximum likelihood fit of the data is then performed in terms of a proportional hazards model that includes the individual parameters, dose, treatment duration, and age at treatment. The fit indicates proportionality of the tumor rates to mean skeletal dose with an added factor (1 + 0.18.tau), where tau is the treatment time in months. This indicates that a protraction of the injections over 15 months instead of 5 months doubles the risk of bone sarcoma

  3. Epidemiological Evaluation of Head and Neck Sarcomas in Iran (the Study of 105 Cases Over 13 Years).

    Science.gov (United States)

    Alishahi, Batoul; Kargahi, Neda; Homayouni, Solmaz

    2015-08-01

    Head and neck sarcomas are exceedingly rare and they include 4% - 10% of all sarcomas and less than 1% of all neoplasm of head and neck. The aim of this study is to evaluate the epidemiological characteristics of head and neck sarcomas of patients in Isfahan, Iran. In this retrospective study, from the 16000 patients whose files were evaluated, the total number of 105 head and neck sarcomas were collected. They were evaluated with due attention to age, gender of the patients and the most common location of the lesion. From the total number of 105 (0.6%) patients with sarcomas, 56 were men (53.33%) and 49 women (46.66%). The most common head and neck sarcomas among this population were Osteosarcoma (32 cases, 30.47%), Chondrosarcoma (14 cases, 13.33%), and Ewing sarcoma (11 cases, 10.47%).The most common soft tissue sarcoma was Rabdomiosarcoma. Mandible was the most common location for these lesions. In this study, the hard tissue sarcomas were more prevalent than soft tissue ones. Hence, special attention should be paid to the patients when being diagnosed.

  4. Uterine sarcoma - current perspectives.

    Science.gov (United States)

    Benson, Charlotte; Miah, Aisha B

    2017-01-01

    Uterine sarcomas comprise a group of rare tumors with differing tumor biology, natural history and response to treatment. Diagnosis is often made following surgery for presumed benign disease. Currently, preoperative imaging does not reliably distinguish between benign leiomyomas and other malignant pathology. Uterine leiomyosarcoma is the most common sarcoma, but other subtypes include endometrial stromal sarcoma (low grade and high grade), undifferentiated uterine sarcoma and adenosarcoma. Clinical trials have shown no definite survival benefit of adjuvant radiotherapy or chemotherapy and have been hampered by the rarity and heterogeneity of these disease types. There is a role of adjuvant treatment in carefully selected cases following multidisciplinary discussion at sarcoma reference centers. In patients with metastatic disease, systemic chemotherapy can then be considered. There is activity of a number of agents, including doxorubicin, trabectedin, gemcitabine-based chemotherapy, eribulin and pazopanib. Patients should be considered for clinical trial entry where possible. Close international collaboration is important to allow progress in this group of diseases.

  5. Long-term results of preoperative intra-arterial doxorubicin combined with neoadjuvant radiotherapy, followed by extensive surgical resection for locally advanced soft tissue sarcomas of the extremities

    International Nuclear Information System (INIS)

    Nijhuis, P.H.A.; Pras, E.; Sleijfer, D.T.; Molenaar, W.M.; Schraffordt Koops, H.; Hoekstra, H.J.

    1999-01-01

    Background and purpose: In the 1980s a combined modality therapy of intraarterial doxorubicin, neoadjuvant radiotherapy and surgery was initiated at the Groningen University Hospital as a limb-saving treatment for locally advanced, primarily irresectable high-grade soft tissue sarcomas (STS) of the extremities. This study presents the short- and long-term results.Patients and methods: Between 1983 and 1987, 11 patients were treated with intraarterial doxorubicin, preoperative radiotherapy (10x3.5 Gy) and surgical resection. Non-radical resections received additional postoperative radiotherapy of 20-30 Gy.Results: The limb-salvage rate was 91%, without local recurrences during a median hollow-up of 84 months. Six patients died (55%); five from metastatic disease (45%). There were five long-term survivors with a median follow-up of 10 years. Three patients (60%) suffered serious late complications, resulting in disabilitating limb function. Conclusion: Although this approach is feasible as a limb-saving treatment for these unfavorable STS, long-term morbidity is high. (Copyright (c) 1999 Elsevier Science B.V., Amsterdam. All rights reserved.)

  6. Implication of chemo-resistant memory T cells for immune surveillance in patients with sarcoma receiving chemotherapy.

    Science.gov (United States)

    Shibayama, Yuji; Tsukahara, Tomohide; Emori, Makoto; Murata, Kenji; Mizushima, Emi; Hirohashi, Yoshihiko; Kanaseki, Takayuki; Nakatsugawa, Munehide; Kubo, Terufumi; Yamashita, Toshihiko; Sato, Noriyuki; Torigoe, Toshihiko

    2017-09-01

    Chemotherapy has improved the prognosis of patients with sarcomas. However, it may suppress anti-tumor immunity. Recently, we reported a novel CD8 + memory T cell population with a chemo-resistance property, "young memory" T (T YM ) cells. In this study, we investigated the proportion and function of T YM cells in peripheral blood of healthy donors and sarcoma patients who received chemotherapy and those who did not