WorldWideScience

Sample records for sarcoma patient remained

  1. 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.

  2. 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.

  3. 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.

  4. Osseous Kaposi sarcoma in an HIV-positive patient

    Energy Technology Data Exchange (ETDEWEB)

    Thanos, Loukas; Mylona, Sofia; Kalioras, Vasilios; Pomoni, Maria; Batakis, Nikolaos [Radiology Department, ' ' Korgialeneio-Benakeio' ' , Red Cross Hospital of Athens, 1 Athanasaki Street, 11526, Athens (Greece)

    2004-04-01

    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. 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.

  6. 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.)

  7. Collecting and Storing Biological Samples From Patients With Ewing Sarcoma

    Science.gov (United States)

    2016-11-21

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

  8. 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

  9. Follicular cell dendritic sarcoma of cervical nodes in a patient with significant WTC exposure.

    Science.gov (United States)

    Shemen, Larry; Kaplan, Barry; Sussman, Louis

    2015-01-07

    A patient who worked at the World Trade Center (WTC) site presented with right cervical lymphadenopathy. He underwent right neck dissection. The final pathology showed follicular dendritic cell sarcoma. He was treated with radiation and chemotherapy and remained free of disease initially. He then presented with left cervical lymphadenopathy 2.5 years later and underwent a left neck dissection with similar pathology. A discussion of the disease process and WTC exposure is presented. Clinicians should be cognisant of this disease process and the latency between WTC exposure and the onset of sarcomas. 2015 BMJ Publishing Group Ltd.

  10. The Prevalence of Pain in Patients Attending Sarcoma Outpatient Clinics

    Directory of Open Access Journals (Sweden)

    P. Y. Kuo

    2011-01-01

    Full Text Available The prevalence of pain in patients with sarcoma is not well documented. We investigated this in outpatients at a tertiary cancer referral centre, assessing the adequacy of pain control and for risk factors leading to higher prevalence and severity of pain. 149 patients were surveyed. Patients with pain within the previous 7 days completed pain assessment tools (BPI, S-LANSS, PMI. 53% of patients had pain within the previous 7 days, and 25% had significant pain. Of those with pain, 63% was inadequately controlled and neuropathic pain was identified in 36%. Age, gender, tumour type, and the type of cancer treatment were not significant predictors of the prevalence or severity of the pain. Based on our results, patients with sarcoma should be actively screened for pain and have regular reviews of their analgesic requirements.

  11. Sorafenib in Treating Patients With Soft Tissue Sarcomas (Extremity Sarcoma Closed to Entry as of 5/30/07)

    Science.gov (United States)

    2014-04-01

    Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Osteosarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Osteosarcoma; Stage I Adult Soft Tissue Sarcoma; Stage II Adult Soft Tissue Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma

  12. Disseminated Kaposi sarcoma in a HIV negative patient.

    Science.gov (United States)

    Lin, Guoshu; Wang, Hongyan; Fan, Xing; Li, Hui; Wang, Zaixing; Lin, Da; Yang, Sen; Zhang, Xuejun

    2015-01-01

    Kaposi sarcoma (KS) is a neoplasm of the endothelial cells. It often manifests with multiple vascular nodules on the skin and other organs. It is a systemic, malignant and multifactor disease and has a variable course. We describe an elderly Chinese man who had a rapidly growing maroon nodule on his right foot, both arms and cheekbones. KS in HIV-negative patients has only been reported sporadically.

  13. Tonsillar Kaposi sarcoma in a patient with membranous glomerulonephritis on immunosuppressive therapy.

    Science.gov (United States)

    Al-Brahim, Nabeel; Zaki, Ashraf H; El-Merhi, Khaled; Ahmad, Mahmoud S

    2013-07-01

    Kaposi sarcoma is a malignant vascular neoplasm uncommonly seen in immunosuppressed patients. Herein we report an unusual case of tonsillar Kaposi sarcoma in a patient with membranous glomerulonephritis treated with prednisolone and cyclosporine. The patient presented after 10 months of starting the treatment with a tonsillar mass. Histological examination was typical of monomorphic spindle cell proliferation with slit-like vascular channels. The tumor cells expressed CD34, D2-40 and positive nuclear stain for HHV-8. Kaposi sarcoma is associated with immunosuppression and rarely occurs in the tonsil. Clinicians should be aware of this rare presentation of Kaposi sarcoma.

  14. 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.

  15. Mycosis fungoides and Kaposi’s sarcoma association in an HIV-negative patient*

    Science.gov (United States)

    Bariani, Maria Carolina Prado Fleury; Fleury Júnior, Luiz Fernando Fróes; Ribeiro, Ana Maria Quinteiro; Carneiro, Siderley de Souza; Pereira, Tiago Arantes

    2016-01-01

    The association of mycosis fungoides and kaposi’s sarcoma in HIV-negative patients is a rare phenomenon. The presence of human herpesvirus 8 (HHV-8) – associated with all forms of Kaposi’s sarcoma – has also been recently identified in mycosis fungoides lesions. However, a causal association between HHV-8 and the onset of mycosis fungoides has not been established yet. The present case reports a patient who developed Kaposi’s sarcoma lesions after a two-year UVB phototherapy to treat a mycosis fungoides. Negative immunohistochemistry staining for Kaposi’s sarcoma-associated herpesvirus in the initial mycosis fungoides lesions strengthens the absence of a link between Kaposi’s sarcoma-associated herpesvirus and mycosis fungoides. Immunosuppression caused by the lymphoma and prolonged phototherapy were probably the contribut ing factors for the onset of Kaposi’s sarcoma.

  16. Isolated Kaposi sarcoma of the finger pulp in an AIDS patient.

    Science.gov (United States)

    Aïm, F; Rosier, L; Dumontier, C

    2012-02-01

    A 63-year-old woman with long-standing AIDS and previous Kaposi sarcomas of the lower limb presented to our consultation complaining of a painful left ring finger with pulp enlargement. X-rays revealed an osteolytic lesion of the distal phalanx. We suspected an isolated osseous Kaposi sarcoma and at surgery we found a hemorrhagic lesion with bone extension into the phalanx. Bone involvement is rare in Kaposi sarcoma and even rarer in patients without a cutaneous location.

  17. Peginterferon alfa-2a for AIDS-associated Kaposi sarcoma: experience with 10 patients.

    Science.gov (United States)

    Rokx, Casper; van der Ende, Marchina E; Verbon, Annelies; Rijnders, Bart J A

    2013-11-01

    In this observational cohort study, 10 patients with extensive or treatment-refractory AIDS-associated Kaposi sarcoma were treated with peginterferon alfa-2a. Tumor responses were observed in 9 patients with a median progression-free survival of 645 days. Peginterferon alfa-2a could be an effective therapy for extensive or treatment-resistant Kaposi sarcoma.

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

    Directory of Open Access Journals (Sweden)

    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.

  19. Spinal epidural granulocytic sarcoma in non-leukemic patient.

    Science.gov (United States)

    Antic, Darko; Verstovsek, Srdan; Elezovic, Ivo; Grujicic, Dana; Gotic, Mirjana; Bila, Jelena; Perunicic, Maja; Jakovic, Ljubomir

    2009-01-01

    A previously healthy 24-year-old male presented with a 3-month history of progressive backache and weakness in both legs. Magnetic resonance imaging of the spine showed a large soft tissue mass infiltrating paraspinal musculature of lumbosacral area, sacral laminas, last lumbar and all sacral vertebra, protruding into the spinal canal, and with propagation into pelvis. Baseline laboratory data were normal. Decompressive laminectomy and tumor removal were performed resulting in neurological improvement. Histological examination identified granulocytic sarcoma (GS). Bone marrow biopsy showed normal findings. The patient underwent adjuvant chemotherapy and radiotherapy, resulting in the elimination of residual lesion, followed by autologous transplant. Immediate diagnosis and adequate systematic treatment are essential to achieve optimal results in patients with isolated GS. The patient is alive and free of the disease 14 months from the diagnosis.

  20. Pulmonary involvement of Kaposi sarcoma in an AIDS patient: radiologic and pathologic findings

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Seung Ho; Goo, Jin Mo; Lee, Jun Woo; Chung, Myung Jin; Lee, Yu Jin; Im, Jung Gi [College of Medicine, Seoul National Univ., Seoul (Korea, Republic of)

    2002-01-01

    Kaposi sarcoma is the most common malignancy in aquired immunodeficiency syndrome (AIDS), and when disseminated may involve any organ system. Pulmonary involvement of Kaposi sarcoma in AIDS patients has not been previously reported in Korea and we report one such case, confirmed pathologically at autopsy.

  1. Risk of Kaposi's sarcoma and of other cancers in Italian renal transplant patients

    Science.gov (United States)

    Serraino, D; Piselli, P; Angeletti, C; Minetti, E; Pozzetto, A; Civati, G; Bellelli, S; Farchi, F; Citterio, F; Rezza, G; Franceschi, S; Busnach, G

    2005-01-01

    A follow-up study of 1844 renal transplant patients in Italy showed a 113-fold increased risk for Kaposi's sarcoma. Kaposi's sarcoma risk was higher in persons born in southern than in northern Italy. Significant increases were also observed for cancers of the lip, liver, kidney and for non-Hodgkin's lymphoma. PMID:15668710

  2. [Sarcoma of the breast].

    Science.gov (United States)

    Haberthür, F; Almendral, A C; Feichter, G; Torhorst, J K

    1992-05-01

    Sarcoma of the breast represents only 0.2-1% of all mammary malignancies. This study reports 5 such cases, including 2 osteosarcomas, 1 fibro-, 1 lipo-, and 1 malignant fibrous sarcoma. The treatment used was mastectomy in 3 cases with excision of axillary lymph nodes. The remaining 2 patients were treated by simple mastectomy whereby 1 of these received a immediate reconstruction with a prosthesis. 1 patient demonstrated local recurrence and died. The remaining 4 patients did not develop neither metastases nor local recurrence and are still alive after an observing period between 12 months up to 17 years. Today, first-line treatment is wide local excision or simple mastectomy. Excision of the axillary lymphatics, adjuvant radiotherapy, and chemotherapy have been disappointing in the treatment of breast sarcoma.

  3. Myasthenia gravis developing in an HIV-negative patient with Kaposi's sarcoma.

    Science.gov (United States)

    Mantero, Vittorio; Mascolo, Maria; Bandettini di Poggio, Monica; Caponnetto, Claudia; Pardini, Matteo

    2013-07-01

    Myasthenia gravis is a disorder of neuromuscular transmission caused by autoimmune mechanisms. We reported a possible association between seropositive myasthenia gravis and Kaposi's sarcoma in a HIV-negative subject and the observed interactions between the treatment regimen for these two conditions. A 62-year-old man came to our attention for ocular myasthenia gravis. He suffered from a classic form of Kaposi's sarcoma since about 1 year. When myasthenic symptoms worsened, the patient was started on prednisone and azathioprine. The patient had a significant worsening of Kaposi's sarcoma, so prednisone and azathioprine were reduced and he was treated with vinblastine, with improvement both in dermatologic than in neurological symptomatology. We propose some considerations: the potential correlation between Kaposi's sarcoma and myasthenia gravis through immunological mechanism; myasthenia gravis as a paraneoplastic manifestation of Kaposi's sarcoma, and the role of an antitumoral agent as a treatment for both the conditions.

  4. 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.

  5. 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.

  6. Systemic treatment options for patients with refractory adult-type sarcoma beyond anthracyclines.

    Science.gov (United States)

    Hartmann, Jörg T

    2007-03-01

    For the subgroup of patients with inoperable gastrointestinal stromal tumors, progress has been made by the rapid development and approval of the targeted therapy imatinib mesylate. Small round cell sarcoma, such as Ewing/PNET, desmoplastic small round cell sarcoma and rhabdomyosarcoma, are chemotherapy-sensitive and potentially curable malignancies, which are treated with multimodality, dose-intensitive and neoadjuvant protocols regardless of size or overt metastatic disease. A limited number of effective agents available for the treatment of patients with metastatic adult soft-tissue sarcoma exists, which have failed anthracyline and ifosfamide-based chemotherapy. Most other high-grade (grading >I) so-called adult-type soft-tissue sarcomas such as fibro, lipo, pleomorphic and synovial sarcoma are treated with a anthracycline-based regimen with or without ifosfamide as front-line therapy. In this review, the therapeutic activities of drugs currently available as second-line treatment in patients with metastatic soft tissue sarcoma are summarized, providing an overview of contentious or emerging treatment issues. In relapsed 'adult-type' soft-tissue sarcomas trofosfamide, gemcitabine and ecteinascidin (ET-743) appear to be drugs associated with moderate activity and an acceptable toxicity profile. An interesting finding to be noted is that the different drugs have particular effects in distinct subtypes of soft-tissue sarcoma; however, it has to be taken into account that the number of patients included in those phase II trials are limited. The role of the newer agents (e.g. patupilone derivates, brostallicin) is currently not definable. The so-called selective therapy targeting vascular endothelial growth factor (receptor), epidermal growth factor receptor, c-kit, Raf kinase or platelet-derived growth factor receptor and bcl-2 antisensing, proteasome, protein kinase C/B, and mammalian target of rabamycin inhibition will continue to be tested in gastrointestinal

  7. A longitudinal study on emotional adjustment of sarcoma patients: the determinant role of demographic, clinical and coping variables.

    Science.gov (United States)

    Paredes, T; Pereira, M; Simões, M R; Canavarro, M C

    2012-01-01

    The present study examined change on emotional distress of sarcoma patients from the diagnostic to treatment phases, the distinct trajectories of adjustment and the influence of demographic, clinical and coping variables on anxiety and depression. Thirty-six sarcoma patients completed questionnaires on emotional distress (Hospital Anxiety and Depression Scale) and coping strategies (Brief Cope) at time of diagnosis, and again during treatment. No significant change in emotional distress levels was found from diagnostic to treatment phase, with mean anxiety and depression scores remaining below the clinical range. Over time, 52.8% and 66.7% of patients maintained non-clinical anxious and depressive symptoms respectively, and 25% and 11.1% remained with clinical anxiety and depression. Living with partner, less use of humour and more denial were associated with high emotional distress at time of diagnosis and during treatments, and high levels of distress at baseline were predictive of poorer emotional adjustment during treatments. Although sarcoma patients, in general, seem to exhibit good psychological adjustment, there is a significant minority that requires mental health services in order to help decrease their emotional distress following the diagnosis, and prevent psychological difficulties during treatments. Our findings are an important contribution to understanding the psychological adjustment of patients with a specific and rare type of cancer.

  8. Endometrial stromal sarcoma together with leiomyosarcoma in a single patient: a rare case report

    Directory of Open Access Journals (Sweden)

    Reema Jain

    2016-04-01

    Full Text Available Uterine sarcomas are relatively rare tumors of mesodermal origin. The three most common histological variants of uterine sarcoma are Leiomyosarcomas, Endometrial stromal sarcomas, and Carcinosarcomas. It is extremely uncommon to find the 2 variants in a single patient. Given the rarity of these tumors there are limited reports in the literature referring to the clinical management and final outcome of these cases. Our patient was a 60 year old post-menopausal women presented in the opd with post-menopausal bleeding. Ultrasound report showed a fibroid of around 8 by 8 cm fundal fibroid. Endometrial biopsy report showed low grade endometrial sarcoma while the CECT report showed leiomyosarcoma creating a dilemma in the diagnosis. Patient was then taken up for surgical exploration. The biopsy report confirmed the existence of both the types of tumor, a very rare finding. [Int J Reprod Contracept Obstet Gynecol 2016; 5(4.000: 1239-1241

  9. Multicentric Castleman's disease and Kaposi's sarcoma in a cyclosporin treated, HIV-1 negative patient: case report

    Directory of Open Access Journals (Sweden)

    van Oers MHJ

    2003-12-01

    Full Text Available Abstract Background Multicentric Castleman's disease (MCD is a rare disease, but is more frequent in AIDS patients. MCD has only been reported twice before in patients receiving immunosuppressive therapy after renal transplantation, and never in patients receiving immunosuppressive therapy without transplantation. About half of the cases of MCD are human herpesvirus 8 (HHV8 – related, in contrast to Kaposi's sarcoma, a more common complication arising after immunosuppression, where the virus is found in virtually all cases. Case presentation We report a HIV-1 negative, non-transplant patient who developed HHV8-associated multicentric Castleman's disease and Kaposi's sarcoma after 17 years of immunosuppressive treatment with cyclosporin A for a minimal change nephropathy. Chemotherapy with liposomal doxorubicin resolved both symptoms of multicentric Castleman's disease and Kaposi's sarcoma in this patient. A concomitant decline in the HHV8 viral load in serum/plasma, as determined by a quantitative real-time PCR assay, was observed. Conclusions Multicentric Castleman's disease can be a complication of cyclosporin A treatment. Both multicentric Castleman's disease and Kaposi's sarcoma in this patient were responsive to liposomal doxorubicin, the treatment of choice for Kaposi's sarcoma at the moment, again suggesting a common mechanism linking both disorders, at least for HHV8-positive multicentric Castleman's disease and Kaposi's sarcoma. HHV8 viral load measurements can be used to monitor effectiveness of therapy.

  10. Kaposi sarcoma: review and medical management update.

    Science.gov (United States)

    Fatahzadeh, Mahnaz

    2012-01-01

    Despite recent advances in our understanding of pathogenic mechanisms involved, the true nature of Kaposi sarcoma remains an enigma. Four clinical variants have been described for the disease, differing in natural history, site of predilection, and prognosis. All forms of Kaposi sarcoma may manifest in the oral cavity and Kaposi sarcoma-associated virus appears essential to development of all clinical variants. The spectrum of therapeutic strategies is broad and selection of appropriate intervention mandates a thorough understanding of disease spread and the patient's symptomatology, as well as risks and benefits of therapy. This article provides an overview of epidemiology, subtypes, clinical course, pathogenesis, and management strategies for Kaposi sarcoma.

  11. Adjuvant chemotherapy for primary cardiac sarcomas: the IGR experience.

    OpenAIRE

    Llombart-Cussac, A.; Pivot, X; Contesso, G; Rhor-Alvarado, A.; Delord, J P; Spielmann, M.; Türsz, T.; Le Cesne, A.

    1998-01-01

    The effect of additional treatments after surgery in patients with primary cardiac sarcoma (PCS) remains unknown. The present study aims to evaluate the benefit of chemotherapy in patients with non-metastatic cardiac sarcomas after optimal resection. Between October 1979 and December 1995, 15 patients with a median age of 45 (range 16-66) and a resected primary cardiac sarcoma [angiosarcoma (six), malignant fibrous histiocytoma (three), leiomyosarcoma (two), rhabdomyosarcoma (two), liposarcom...

  12. Surgical treatment of pelvic sarcoma in children: outcomes for twenty six patients.

    Science.gov (United States)

    Kadhim, Muayad; Womer, Richard B; Dormans, John P

    2017-07-27

    Pelvic sarcoma is uncommon in children and challenging to treat. This study examined different surgical approaches to treat pelvic sarcoma with the aim of assessing the oncologic, and functional outcomes. We retrospectively examined the medical records of patients younger than 21 years of age who underwent surgery for pelvic sarcoma at our institution from 1992 to 2010. The functional status of the patients was examined after a minimum follow-up of two years. Twenty-six patients were included in the analysis. Nineteen (73%) patients were male and seven (27%) were female. Mean age at presentation was 12.0 ± 3.9 years. Nineteen patients had Ewing sarcoma (73%), five had osteosarcoma (19%), one had chondrosarcoma (4%) and one had rhabdomyosarcoma (4%). Iliac wing resection with no reconstruction was done in three patients. Reconstruction with free fibular graft A-frame was performed in four patients, saddle endoproshtesis in five patients, iliac autoclave in one patient, and internal hemipelvectomy in nine patients. Hindquarter amputation was performed in five patients. Median follow-up was 4.6 years (range, 2.6-16 years). Nineteen patients were alive (73%); of those, 13 were known to be without disease, three were with disease and three did not have known tumor status. Six patients were reported deceased, three had osteosarcoma and three had Ewing sarcoma. Function was assessed in 17 patients; 64% were asymptomatic and ambulatory and 36% were symptomatic and ambulatory. Salvage reconstruction for pelvic sarcoma can be performed through various procedures on the extent of necessary bony resection. Survival rate and functional outcomes were promising in the performed study.

  13. Intensive combined modality therapy including low-dose TBI in high-risk Ewing's sarcoma patients

    Energy Technology Data Exchange (ETDEWEB)

    Kinsella, T.J.; Glaubiger, D.; Diesseroth, A.; Makuch, R.; Waller, B.; Pizzo, P.; Glatstein, E.

    1983-12-01

    Twenty-four high-risk Ewing's sarcoma patients were treated on an intensive combined modality protocol including low-dose fractionated total body irradiaiton (TBI) and autologous bone marrow infusion (ABMI). Twenty patients (83%) achieved a complete clinical response to the primary and/or metastatic sites following induction therapy. The median disease-free interval was 18 months, and nine patients remain disease-free with a follow-up of 22 to 72 months. Local failure as a manifestation of initial relapse occurred in only three patients (15%), each having synchronous distant failure. Eight patients failed initially with only distant metastases, usually within 1-2 years following a complete clinical response. Two patterns of granulocyte recovery following consolidative therapy (including TBI and ABMI) were recognized. The time to platelet recovery was different for the groups with early and late granulocyte recovery. Patients with late recovery did not tolerate maintenance chemotherapy. However, there was no difference in disease-free and overall survival, when comparing the groups with early and late granulocyte recovery. It is concluded that these high-risk Ewing's sarcoma patients remain a poor-prognosis group in spite of intensive combined modality therapy including low-dose TBI. The control of microscopic systemic disease remains the major challenge to improving the cure rate. A new combined modality protocol with high-dose 'therapeutic' TBI (800 rad/2 fractions) is being used and the protocol design is outlined.

  14. Therapeutic Trial for Patients With Ewing Sarcoma Family of Tumor and Desmoplastic Small Round Cell Tumors

    Science.gov (United States)

    2016-08-25

    Desmoplastic Small Round Cell Tumor; Ewing Sarcoma of Bone or Soft Tissue; Localized Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor

  15. 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

  16. CA-125 IN SERUM AND TUMOR FROM PATIENTS WITH UTERINE SARCOMA

    NARCIS (Netherlands)

    DUK, JM; BOUMA, J; BURGER, GTN; DEBRUIJN, HWA

    1994-01-01

    Serial serum samples of 33 patients with primary sarcoma of the uterus were analyzed for CA 125 and frozen tissue sections of tumor from 23 patients were tested for this antigen. Before treatment, 12 of 30 evaluable patients showed serum CA 125 levels >16 Uml-1 (40%). There was no relationship betwe

  17. Molecularly targeted therapy for Kaposi's sarcoma in a kidney transplant patient: case report, "what worked and what did not"

    Directory of Open Access Journals (Sweden)

    Correa-Rotter Ricardo

    2007-03-01

    Full Text Available Abstract Background Imatinib is a tyrosine-kinase inhibitor; for which there is limited information regarding its effects on AIDS Kaposi's sarcoma and none in patients with transplant-associated Kaposi's sarcoma. Sirolimus, an immunosuppressive drug used for kidney transplant, exhibits antiangiogenic activity related to impaired production of VEGF (vascular endothelial growth factor, clinical benefit has been reported in Kaposi's sarcoma associated with renal graft. Case Presentation Here we report a case of an 80 year old male, who developed Kaposi's Sarcoma nine months after receiving a living non-related donor kidney transplant at age 74. Three years after treatment with different chemotherapeutic agents for progressive cutaneous Kaposi's Sarcoma with no visceral involvement, he was prescribed Imatinib (200 mg/day for two weeks followed by 400 mg/day after four weeks of treatment he developed anasarca, further progression of KS and agranulocytosis. Imatinib was discontinued and there was significant clinical recovery. One year later his immunosuppressive therapy was changed to Sirolimus and regression of the Kaposi's sarcoma occurred. Conclusion The lack of benefit and severe toxicity associated with the use of Imatinib in this patient should alert clinicians of potentially adverse consequence of its use in patients with transplant associated Kaposi's sarcoma. On the other hand the positive response seen in this patient to Sirolimus even after a long evolution of Kaposi's sarcoma, multiple chemotherapy regimens and extensive cutaneous disease further suggest it therapeutical utility for transplant associated Kaposi's sarcoma.

  18. Quality of Life Following Amputation or Limb Preservation in Patients with Lower Extremity Bone Sarcoma

    Directory of Open Access Journals (Sweden)

    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.

  19. Bilateral tonsillar and esophageal Kaposi sarcoma in an HIV-negative patient.

    Science.gov (United States)

    Ozbudak, Irem Hicran; Guney, Kenan; Mutlu, Derya; Gelen, Tekinalp; Ozbilim, Gulay

    2011-07-01

    Tonsillar involvement in Kaposi sarcoma is extremely rare, as only a few such cases have been reported; all but 1 of these previously reported cases occurred in patients with human immunodeficiency virus (HIV) infection. We describe what to the best of our knowledge is the first reported case of concurrent bilateral tonsillar and esophageal Kaposi sarcoma in an HIV-negative patient. A 68-year-old man presented with sore throat and dysphagia. Clinical examination revealed the presence of bilateral and asymmetrical tonsillar masses, as well as generalized lymphadenopathy in the cervical chain. The masses were resected, and findings on histopathologic analysis were consistent with Kaposi sarcoma. In addition, human herpesvirus 8 was demonstrated on a tonsil specimen by polymerase chain reaction, and microinvasive squamous cell carcinoma was also detected. Later, another Kaposi sarcoma lesion was detected in the lower third of the esophagus. We recommend that clinicians not discount the possibility of oral classic Kaposi sarcoma in the workup of an immunocompetent patient with oral vascular lesions.

  20. Molecular biology of sarcomas.

    Science.gov (United States)

    Gebhardt, M C

    1996-07-01

    There has been a virtual explosion of information relating to the biology of sarcomas with which we as orthopaedists deal. Much more is yet to be learned. These findings will teach us more about the etiology of these tumors. More important, the findings will alter the way in which these tumors are treated. It is unlikely that we will continue to treat osteosarcoma or Ewing's sarcoma patients with currently available drug regimens and surgery or make treatment decisions based on the histologic classification of tumors we know today. If we are to remain active in the management of these patients we must be aware of the findings as they occur. That will ensure both that we remain the primary caretakers of these patients, and that we will continue to be stimulated intellectually by these intriguing scientific investigations.

  1. [Production of interleukin-2 by peripheral blood lymphocytes from patients with soft tissue sarcomas].

    Science.gov (United States)

    Berezhnaia, N M; Goretskiĭ, B A; Konovalenko, V F; Palivets, A Iu; Tolstopiatov, B A

    1987-01-01

    Interleukin-2 (IL-2) production of phytohemagglutinin-stimulated peripheral blood lymphocytes (PBL) was studied in 9 healthy subjects and 19 patients with soft tissue sarcomas. Mean IL-2 production by PBL in 19 patients was significantly diminished as compared with the control. Surgery leads to an increase of IL-2 production, however, the levels observed in the control do not restore completely.

  2. Classic Kaposi's sarcoma treated with elastic stockings and outpatient follow-up of a 90-year-old patient.

    Science.gov (United States)

    Trevisan, Flavia; Cunha, Paulo Rowilson; Pinto, Clovis Antonio Lopes; Alves, Celia Antonia Xavier de Moraes

    2013-01-01

    Kaposi's sarcoma is a multifactorial angioproliferative disorder. The herpes virus 8 human contributes to its pathogenesis, but it is uncertain whether these lesions are only reactive hyperplasia to the virus or neoplasia. Four clinical types are described: classic, endemic, iatrogenic and HIV-associated. Classic Kaposi's sarcoma has no standard staging or treatment protocols. Some studies have shown the use of compression stockings in the treatment of lymphedema associated with Kaposi's sarcoma. We report the case of a 90 year-old patient with classic Kaposi's sarcoma treated with compression stockings who showed a satisfactory response.

  3. Classic Kaposi's sarcoma treated with elastic stockings and outpatient follow-up of a 90-year-old patient*

    Science.gov (United States)

    Trevisan, Flavia; Cunha, Paulo Rowilson; Pinto, Clovis Antonio Lopes; Alves, Celia Antonia Xavier de Moraes

    2013-01-01

    Kaposi's sarcoma is a multifactorial angioproliferative disorder. The herpes virus 8 human contributes to its pathogenesis, but it is uncertain whether these lesions are only reactive hyperplasia to the virus or neoplasia. Four clinical types are described: classic, endemic, iatrogenic and HIV-associated. Classic Kaposi's sarcoma has no standard staging or treatment protocols. Some studies have shown the use of compression stockings in the treatment of lymphedema associated with Kaposi's sarcoma. We report the case of a 90 year-old patient with classic Kaposi's sarcoma treated with compression stockings who showed a satisfactory response. PMID:24346919

  4. Nonmetastatic Ewing's Sarcoma of the Lumbar Spine in an Adult Patient

    Science.gov (United States)

    Iacoangeli, Maurizio; Dobran, Mauro; Di Rienzo, Alessandro; di Somma, Lucia Giovanna Maria; Alvaro, Lorenzo; Moriconi, Elisa; Nocchi, Niccolò; Gladi, Maurizio; Scerrati, Massimo

    2012-01-01

    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. PMID:23133768

  5. 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.

  6. Evaluation of minimal disseminated disease in cryopreserved ovarian tissue from bone and soft tissue sarcoma patients.

    Science.gov (United States)

    Dolmans, M M; Iwahara, Y; Donnez, J; Soares, M; Vaerman, J L; Amorim, C A; Poirel, H

    2016-10-01

    What is the risk of finding malignant cells in cryopreserved ovarian tissue from sarcoma patients? Minimal disseminated disease (MDD) was not detected in frozen-thawed ovarian tissue from 26 patients by any of the sensitive methods applied. In case of leukemia, the risk of malignant cell transmission through the graft is well known and widely documented. However, for bone cancer, like Ewing sarcoma or osteosarcoma, only a small number of case reports, have been published. These cancers often affect prepubertal girls, in whom ovarian tissue cryopreservation and transplantation is the only option to preserve fertility. The presence of malignant cells in cryopreserved ovarian tissue from patients with bone/soft tissue sarcoma was investigated with disease-specific markers for each patient, using immunohistochemistry (IHC), FISH and real-time quantitative RT-PCR (qPCR), with the original tumor serving as a positive control. Forty-eight sarcoma patients were enrolled in the study, 12 of whom subsequently died. In each case, tissue from the primary tumor was investigated in order to identify markers (immunohistochemical and/or molecular) to analyze the ovarian tissue case by case. Ovarian tissue from osteosarcoma (n = 15), liposarcoma (n = 1) and undifferentiated sarcoma (n = 5) patients could not be evaluated, as no specific markers were detected by FISH or sensitive IHC in any of their primary tumoral tissue. One patient with Li-Fraumeni syndrome was also excluded from the study. IHC analyses were therefore performed on ovarian tissue from 26 patients and qPCR on 19. The primary tumors involved were Ewing sarcoma family of tumors (n = 14), rhabdomyosarcoma (n = 7), synovial sarcoma (n = 2), clear cell sarcoma (n = 2) and a malignant peripheral nerve sheath tumor (n = 1). MDD was not detected in any of the 26 analyzed samples using sensitive techniques in this largest reported series, even from patients who subsequently died and/or those who presented

  7. Kaposi's sarcoma with HHV8 infection and ANCA-associated vasculitis in a hemodialysis patient.

    Science.gov (United States)

    Fatma, Lilia Ben; Rais, Lamia; Mebazza, Amel; Azzouz, Haifa; Beji, Somaya; Krid, Madiha; Smaoui, Wided; Maiz, Hedi Ben; Zouaghi, Karim; Zitouna, Moncef; Osmane, Amel Ben; Moussa, Fatma Ben

    2013-11-01

    The association between Kaposi's sarcoma (KS) and human herpes virus eight (HHV-8) infection is rarely reported in hemodialysis (HD) patients. We report here the rare association of KS, HHV-8 and hepatitis C virus (HCV) infection as well as syphilis in a HD patient. We report the case of a 72-year-old woman who presented with microscopic polyangiitis with alveolar hemorrhage and pauci-immune necrosing and crescentic glomerulonephritis as well as renal failure requiring HD. Biological tests showed positive HCV and syphilis tests. The patient was treated by HD and intravenous pulse, followed by oral corticosteroids and six cyclophosphamide monthly pulses with remission of the alveolar hemorrhage, but without renal functional recovery as the patient remained HD dependent. Five months after the first treatment administration, she developed extensive purpuric lesions on her lower limbs, abdomen face and neck. A skin biopsy showed KS. The HHV-8 test was positive, with positive polymerase chain reaction-HHV8 in the serum and skin. After immunosuppression withdrawal, the KS skin lesions regressed rapidly without relapse after 12 months of follow-up, but alveolar hemorrhage relapsed after 16 months of follow-up. Our case showed that the immunosuppressed state related to multiple factors such as aging, vasculitis, HHV-8, HCV, syphilis, immunosuppressive therapy and HD may all have contributed to the development of KS in our patient.

  8. SYNOVIAL CELL SARCOMA

    Directory of Open Access Journals (Sweden)

    M. Farzan

    1997-06-01

    Full Text Available Ten cases of synovial cell sarcoma are reported. The youngest patient was a 2'A years old boy with synovial cell sarcoma of the knee and the oldest one was a man with synovial cell sarcoma of the elbow.

  9. Hypercalcemia and diffuse osteolytic lesions in a 45-year-old patient with myeloid sarcoma with megakaryocytic differentiation

    Directory of Open Access Journals (Sweden)

    Aditya Goud

    2016-04-01

    Full Text Available Acute megakaryocytic leukemia is a rare form of acute myeloid leukemia that carries a poor prognosis. As most cases of osteolytic lesions are due to plasma cell and myeloid malignancies, maintaining a broad differential directly influences clinical course. We document a 45-year-old patient with progressive constitutional symptoms, osteolytic bone lesions in the setting of hypercalcemia, who developed acutely worsening pancytopenia. The diagnosis of myeloid sarcoma with megakaryocytic differentiation was made after obtaining tissue from osteolytic bone that stained strong for CD34. Immunohistochemical testing underscores the importance of how serologic and urine testing remains limited and can delay early diagnosis in this disease.

  10. Allografts about the Knee in Young Patients with High-Grade Sarcoma.

    Science.gov (United States)

    Brigman, Brian E; Hornicek, Francis J; Gebhardt, Mark C; Mankin, Henry J

    2004-04-01

    Reconstruction after resections for high-grade sarcomas about the knee in children and adolescents is a challenging problem because of the large soft tissue and skeletal defects, the effects of adjuvant therapy, and the potential for long-term use of the limb. One hundred sixteen patients, all 18 years or younger, with osteosarcoma or Ewing's sarcoma located between the middle femur and middle tibia, were treated with chemotherapy, resection, and allograft reconstruction. One hundred three patients with osteosarcoma and 13 patients with Ewing's sarcoma had 105 Stage II and 11 Stage III tumors. There were 72 osteoarticular grafts (39 femur, 33 tibia), 28 intercalary grafts (19 femur), seven allograft-prosthetic composites (all femur,) and nine allograft-arthrodeses (seven femur, two tibia). At latest followup, 49% of all of the allograft reconstructions were rated good or excellent, 14% were rated as fair, and 37% were failures. Sixteen percent had an infection develop. Twenty-seven percent of patients had a fracture, 34% had a nonunion, and 14 patients eventually required amputation. Reconstruction of large bone defects about the knee in young patients who are being treated with chemotherapy is difficult. Although complications significantly affect outcome, allografts are a viable option for reconstruction in children with high-grade sarcomas about the knee.

  11. Ecteinascidin-743: evidence of activity in advanced, pretreated soft tissue and bone sarcoma patients

    National Research Council Canada - National Science Library

    Huygh, G; Clement, Paul M J; Dumez, H; Schöffski, P; Wildiers, H; Selleslach, J; Jimeno, J M; Wever, I De; Sciot, R; Duck, L; Van Oosterom, A T

    2006-01-01

    .... The objective response rate was 6.7% and the clinical benefit rate at 3 and 6 months was 37.7% and 23.4%, respectively. Responses were noted in patients with lipo-, leiomyo-, osteo-, and myogenic sarcoma, with a median duration...

  12. Ecteinascidin-743: Evidence of Activity in Advanced, Pretreated Soft Tissue and Bone Sarcoma Patients

    National Research Council Canada - National Science Library

    Huygh, G; Clement, Paul M. J; Dumez, H; Schöffski, P; Wildiers, H; Selleslach, J; Jimeno, J. M; De Wever, I; Sciot, R; Duck, L; Van Oosterom, A. T

    2006-01-01

    .... The objective response rate was 6.7% and the clinical benefit rate at 3 and 6 months was 37.7% and 23.4%, respectively. Responses were noted in patients with lipo-, leiomyo-, osteo-, and myogenic sarcoma, with a median duration...

  13. Evaluation of Novel Targeted Therapies in Aggressive Biology Sarcoma Patients after progression from US FDA approved Therapies

    Science.gov (United States)

    Subbiah, Vivek; Hess, Kenneth R.; Khawaja, Muhammad Rizwan; Wagner, Michael J.; Tang, Chad; Naing, Aung; Fu, Siqing; Janku, Filip; Piha-Paul, Sarina; Tsimberidou, Apostolia M.; Herzog, Cynthia E.; Ludwig, Joseph A.; Patel, Shreyaskumar; Ravi, Vinod; Benjamin, Robert S.; Meric-Bernstam, Funda; Hong, David S.

    2016-01-01

    Prognosis of patients with advanced sarcoma after progression from FDA approved therapies remains grim. In this study, clinical outcomes of 100 patients with advanced sarcoma who received treatment on novel targeted therapy trials were evaluated. Outcomes of interest included best response, clinical benefit rate, progression-free survival (PFS) and overall survival (OS). Median patient age was 48 years (range 14–80). Patients had received a median of 2 prior lines of systemic treatment. Phase I treatments were anti-VEGF–based (n = 45), mTOR inhibitor–based (n = 15), and anti-VEGF + mTOR inhibitor–based (n = 17) or involved other targets (n = 23). Best responses included partial response (n = 4) and stable disease (n = 57). Clinical benefit rate was 36% (95% confidence interval 27–46%). Median OS was 9.6 months (95% Confidence Interval 8.1–14.2); median PFS was 3.5 months (95% Confidence Interval 2.4–4.7). RMH prognostic score of 2 or 3 was associated with lower median OS (log-rank p-value < 0.0001) and PFS (log-rank p-value 0.0081). Receiving cytotoxic chemotherapy as part of phase I trial was also associated with shorter median OS (log-rank p-value 0.039). Patients with advanced sarcoma treated on phase I clinical trials had a clinical benefit rate of 36% and RMH score predicted survival. PMID:27748430

  14. ESHAP therapy effective in a patient with Langerhans cell sarcoma.

    Science.gov (United States)

    Yoshimi, Akihide; Kumano, Keiki; Motokura, Toru; Takazawa, Yutaka; Oota, Satoshi; Chiba, Shigeru; Takahashi, Tsuyoshi; Fukayama, Masashi; Kurokawa, Mineo

    2008-06-01

    We describe the rare case of a 53-year-old woman with systemic involvement of Langerhans cell sarcoma (LCS) who had undergone living-related liver transplantation. We chose the CHOP regimen as first-line chemotherapy, and clinical improvement of LCS was obtained. Intensive care was necessary due to the systemic involvement of LCS and severe infectious diseases. After the third cycle of CHOP therapy, however, disease progression was observed, and we administrated a modified ESHAP regimen (etoposide, carboplatin, cytarabine, methylprednisolone) as second-line therapy. A marked response was obtained after four cycles of this combination chemotherapy. Modified ESHAP may be a very effective combination chemotherapy regimen for LCS.

  15. Ribociclib and Doxorubicin in Treating Patients With Metastatic or Advanced Soft Tissue Sarcomas That Cannot Be Removed by Surgery

    Science.gov (United States)

    2017-04-10

    Metastatic Angiosarcoma; Metastatic Epithelioid Sarcoma; Metastatic Fibrosarcoma; Metastatic Leiomyosarcoma; Metastatic Liposarcoma; Metastatic Malignant Peripheral Nerve Sheath Tumor; Metastatic Synovial Sarcoma; Metastatic Undifferentiated Pleomorphic Sarcoma; Myxofibrosarcoma; Pleomorphic Rhabdomyosarcoma; Stage III Soft Tissue Sarcoma; Stage IV Soft Tissue Sarcoma; Undifferentiated (Embryonal) Sarcoma

  16. Ifosfamide plus etoposide combined with regional hyperthermia in patients with locally advanced sarcomas: a phase II study.

    Science.gov (United States)

    Issels, R D; Prenninger, S W; Nagele, A; Boehm, E; Sauer, H; Jauch, K W; Denecke, H; Berger, H; Peter, K; Wilmanns, W

    1990-11-01

    From July 1986 to July 1989, 40 patients (92% pretreated) with deep-seated, advanced soft tissue sarcomas (STS, 25 patients), Ewing's sarcomas (ES, eight patients), osteosarcomas (OS, three patients) and chondrosarcomas (ChS, four patients) were treated at the University of Munich in a protocol involving regional hyperthermia (RHT) combined with ifosfamide plus etoposide. A total of 265 RHT treatments (mean, 6.6 RHT per patient) were applied including 33 pelvic, four extremity, and three abdominal sites. The mean tumor volume was 537 cc (range, 50 to 2,980 cc). For systemic chemotherapy, all patients received ifosfamide (1.5 g/m2, days 1 to 5), etoposide (100 mg/m2, days 1, 3, and 5), and mesna (300 mg/m2 x 4, days 1 to 5) with RHT given only on days 1 and 5 in repeated cycles every 4 weeks. Acute toxicity consisted primarily of pain (57%) combined with local discomfort within the annular phased array applicator (AA) of the BSD hyperthermia system (BSD Medical Corp, Salt Lake City, UT). The average maximum systemic temperature was 37.4 +/- 0.5 degrees C, and there was no indication of enhanced bone marrow toxicity due to the addition of RHT to the systemic chemotherapy. Detailed thermal mapping by invasive thermometry was performed in all patients. In 38 assessable patients, the overall objective response rate was 37%: six complete responses (CRs), four partial responses (PRs), and four favorable histologic responses (FHRs) (95% confidence limits, 22% to 54%). Complete responders are alive and disease-free at 40, 35, 23, 19, 19, and 8 months. Of patients with PR and FHR, two died from metastatic disease after 4 and 17 months and one died from other disease after 27 months. The remaining five patients are stable at 37, 25, 21, 13, and 8 months. Eleven patients showed no change (NC), and 13 patients showed local tumor progression (PD). The mean observation time for all patients was 11.6 months. The time-averaged temperatures (Ts) of all RHT treatments calculated as

  17. Histiocytic sarcoma

    Directory of Open Access Journals (Sweden)

    Eduardo Silva Machado

    2011-01-01

    Full Text Available A 59-year-old white woman, SC, after being treated for pneumonia, presented with an increase in the size of lymph nodes. The immunohistochemical examination diagnosed histiocytic sarcoma. Relapse occurred 12 months after starting chemotherapy. The patient evolved with febrile neutropenia, septic shock and death.

  18. Cabozantinib-s-malate in Treating Patients With Relapsed Osteosarcoma or Ewing Sarcoma

    Science.gov (United States)

    2017-09-04

    Metastatic Ewing Sarcoma; Metastatic Osteosarcoma; Recurrent Ewing Sarcoma; Recurrent Osteosarcoma; Stage III Osteosarcoma AJCC v7; Stage IV Osteosarcoma AJCC v7; Stage IVA Osteosarcoma AJCC v7; Stage IVB Osteosarcoma AJCC v7; Unresectable Ewing Sarcoma; Unresectable Osteosarcoma

  19. 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.

  20. 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 (TYM ) cells. In this study, we investigated the proportion and function of TYM cells in peripheral blood of healthy donors and sarcoma patients who received chemotherapy and those who did not. The proportion of TYM cells was significantly decreased in patients compared with that in healthy donors. In healthy donors, anti-EBV CTLs were induced using mixed lymphocyte peptide culture, from not only TYM cells but also TCM and TEM cells. No CTLs directed to tumor-associated antigens were induced. In sarcoma patients who did not receive chemotherapy, in addition to anti-EBV CTLs, CTLs directed to the tumor-associated antigen PBF were induced from TYM , TCM and TEM cells. In sarcoma patients who received chemotherapy, EBV-specific CTLs were induced from TYM cells but were hardly induced from TEM cells. Interestingly, CTLs directed to the anti-tumor-associated antigen PBF were induced from TYM cells but not from the TCM and TEM cells in sarcoma patients who received chemotherapy. The findings suggest that TYM cells are resistant to chemotherapy and can firstly recover from the nadir. TYM cells might be important for immunological memory, especially in sarcoma patients receiving chemotherapy. © 2017 The Authors. Cancer Science published by John Wiley & Sons Australia, Ltd on behalf of Japanese Cancer Association.

  1. Isolated penile Kaposi's sarcoma in a HIV-positive patient stable on treatment for three years.

    Science.gov (United States)

    Lebari, Dornubari; Gohil, Jesal; Patnaik, Lipsita; Wasef, Wafaa

    2014-07-01

    Kaposi's sarcoma (KS) is an AIDS-defining condition. Typically, KS affects the skin with or without visceral involvement. The extensive use of antiretroviral therapy (ART) has decreased the incidence of KS amongst the HIV-positive population. We report a case of a 40-year-old man with HIV-1 infection with CD4 count of 551 cells/mm(3)and an undetectable viral load who presented with two skin-coloured KS lesions on the prepuce of the penis. Diagnosis was confirmed by histopathology. He had been commenced on ART three years earlier with a nadir CD4 count of 255 cells/mm(3) He had achieved and maintained viral suppression since commencing ART. The patient was initially treated with cryotherapy and 5% imiquimod as the lesions were presumed to be warts. The lack of response to treatment prompted further investigation. We carried out a literature search of published cases of penile KS over the past 10 years. The majority of articles regarding penile KS were published in the pre-ART era and involved patients with AIDS. Over the past 10 years, published cases of penile KS have almost exclusively been in HIV-negative men. We found 10 published cases of penile KS in HIV-negative men and only one other published case of penile KS in a HIV-positive man, who had severe immune suppression with CD4 count below 200 cells/mm(3) This is the first case report to describe a HIV-positive patient stable on ART with a CD4 count above 200 cells/mm(3)and suppressed HIV-1 viral load, to develop two KS lesions on the penis. Clinicians have to remain suspicious of penile lesions and appreciate the crucial role a biopsy with histopathological analysis plays in confirming a diagnosis. In addition, this case illustrates that unusual presentations of KS can still occur in treated HIV-positive patients with sustained immune recovery.

  2. Endoscopic Appearance of Oropharyngeal and Upper GI Kaposi's Sarcoma in an Immunocompromised Patient

    Science.gov (United States)

    Renno, Anas; Khan, Zubair; Alkully, Turki; Kamal, Sehrish; Nawras, Ali

    2017-01-01

    Introduction. Kaposi's sarcoma (KS) usually manifests as a cutaneous disease but GI manifestation is often rare. It is associated with human herpes virus-8 (HHV-8) and seen in immunocompromised patients. In the USA, use of highly active antiretroviral therapy (HAART) has drastically reduced incidence of KS in HIV patients. Case Presentation. A 65-year-old male with human immunodeficiency virus (HIV) was admitted to the intensive care unit (ICU) with cardiopulmonary arrest secondary to hyperkalemia of 7.5 meq/L. Following placement of orogastric and endotracheal tube (ETT), a significant amount of blood was noticed in the ETT. Hemoglobin trended down from 9.6 mg/dL to 6.7 mg/dL over five days. Stool guaiac was positive. Esophagogastroduodenoscopy (EGD) was performed and revealed multiple large hypervascularized violaceous submucosal nodular lesions with stigmata of bleeding seen on the soft palate and pharynx and within the cricopharyngeal area close to the vocal cords. Biopsy of the soft palate lesions showed proliferation of neoplastic spindle shaped cells arranged in bundles with slit-like capillary spaces containing erythrocytes consistent with Kaposi's sarcoma. Biopsy was positive for HHV-8. Colonoscopy was unremarkable. There were no cutaneous manifestations of the disease. Conclusion. GI involvement of Kaposi's sarcoma must be considered in immunocompromised patients and can be confirmed by endoscopic methods.

  3. Phase II Trial of Angiotensin-(1-7 for the Treatment of Patients with Metastatic Sarcoma

    Directory of Open Access Journals (Sweden)

    Paul D. Savage

    2016-01-01

    Full Text Available Background. Angiotensin-(1-7 [Ang-(1-7] is an endogenous antiangiogenic hormone with anticancer activity. In a phase I study of Ang-(1-7, two of three patients with metastatic sarcoma experienced disease stabilization. This phase II study examined clinical and biomarker outcomes for patients with metastatic sarcoma. Methods. Ang-(1-7 was administered by subcutaneous injection at a dose of 20 mg daily. If excessive toxicities occurred in the first cohort, a dose deescalation cohort was allowed. Blood samples were obtained to measure changes in biomarkers. Results. Treatment was well-tolerated and the dose deescalation cohort was not required. Plasma PlGF concentrations following treatment were not statistically significantly changed. A significant increase in plasma Ang-(1-7 was observed at 4 hours after injection. The median progression-free survival was 2.7 months (95% CI; 1.4 to 4.1 months, and the median overall survival was 10.2 months (95% CI; 5.3 to 18.3 months. Two patients with vascular sarcomas demonstrated prolonged disease stabilization of 10 months (hemangiopericytoma and 19 months (epithelioid hemangioendothelioma. Conclusions. Ang-(1-7 at a dose of 20 mg daily was well-tolerated. This prospective phase II study failed to confirm the PlGF biomarker effect identified in the prior phase I study. Prolonged disease stabilization in hemangiopericytoma and epithelioid hemangioendothelioma may warrant further investigation.

  4. Ecteinascidin-743: Evidence of Activity in Advanced, Pretreated Soft Tissue and Bone Sarcoma Patients

    OpenAIRE

    Huygh, G.; Clement, Paul M. J.; Dumez, H; Schöffski, P; Wildiers, H.; Selleslach, J.; Jimeno, J. M.; I. De Wever; Sciot, R; Duck, L.; Van Oosterom, A. T.

    2006-01-01

    Purpose. To evaluate the activity and safety of ecteinascidin (ET-743) in pretreated patients with advanced or metastatic soft tissue and bone sarcoma. Patients or subjects. Eighty-nine patients received ET-743 as a 24-hour continuous infusion at a dose of 900-1500 μg/ m2 every 3 weeks. Results. We observed one complete remission, 5 partial remissions, one minimal response, and 16 patients with a disease stabilization of 6 months or more. The objective response rate was 6.7% and the clinical ...

  5. One-year mortality in patients with bone and soft tissue sarcomas as an indicator of delay in presentation.

    Science.gov (United States)

    Nandra, R; Hwang, N; Matharu, G S; Reddy, K; Grimer, R

    2015-09-01

    For many cancers, one-year mortality following diagnosis is a reflection of either advanced stage at diagnosis, multiple co-morbidities and/or complications of treatment. One-year mortality has not been reported for soft tissue or bone sarcomas. This study reports 1-year sarcoma mortality data over a 25-year period, investigates prognostic factors and considers whether a delay in presentation affects 1-year mortality. A total of 4,945 newly diagnosed bone sarcoma and soft tissue sarcoma patients were identified from a prospectively maintained, single institution oncology database. Of these, 595 (12%) died within 1 year of diagnosis. Both patient factors and tumour characteristics available at diagnosis were analysed for effect. There was significant variation in one-year mortality between different histological subtypes. There has been no significant change in mortality rate during the last 25 years (mean: 11.7%, standard deviation: 2.8 percentage points). Soft tissue sarcoma patients who survived over one year reported a longer duration of symptoms preceding diagnosis than those who died (median: 26 vs 20 weeks, pyear mortality in bone and soft tissue sarcoma patients is easy to measure, and could be a proxy for late presentation and therefore a potential performance indicator, similar to other cancers. It is possible to predict the risk of one-year mortality using factors available at diagnosis. Death within one year does not correlate with a long history but is associated with advanced disease at diagnosis.

  6. Combination Chemotherapy in Treating Patients With Non-Metastatic Extracranial Ewing Sarcoma

    Science.gov (United States)

    2017-02-08

    Adult Supratentorial Primitive Neuroectodermal Tumor (PNET); Childhood Supratentorial Primitive Neuroectodermal Tumor; Ewing Sarcoma of Bone; Extraosseous Ewing Sarcoma; Extraosseous Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Localized Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Peripheral Primitive Neuroectodermal Tumor of the Kidney; Untreated Childhood Supratentorial Primitive Neuroectodermal Tumor

  7. 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 ...

  8. Granulocytic sarcoma of the femur in a patient with acute megakaryoblastic leukaemia

    Directory of Open Access Journals (Sweden)

    Čolović Milica

    2011-01-01

    Full Text Available Introduction. Granulocytic sarcoma, chloroma or myeloblastoma are observed in 3% to7% of acute myeloid leukaemia and represents localized tumour composed of collection of immature leukaemic cells. It appears most frequently in patients with M2, M4 and M5 subtypes of acute myeloid leukaemia Case Outline. A 58-year-old female presented with pain and oedema of the right upper limb in November 2009. After two months the patinet had fracture dislocation and numerous osteolytic lesions of the right femur. Immunohistochemistry of tumour biopsy showed megakaryoblastic granulocytic sarcoma which was CD31++, F-XIII++, CD34-, FVIII+++, S100-, aktin-, EMA++, Bcl2++, CD43++, with positive proliferative marker measured with Ki-67 positivity in more of 50% of cells. Aspirate of bone marrow and immunophenotyping with flowcytometry revealed diagnosis of acute megakaryoblastic leukaemia. The course of the disease was rapid and the patient died before commencing chemotherapy, five months after first complaints. Conclusion. Granulocytic sarcoma is extramedullary localization of collection of leukaemia cells which can proceed, to arise concomitantly with leukaemia, or may be the only manifestation of the disease. The diagnosis can be established only with immunohystochemistry.

  9. Chemotherapy in Ewing′s sarcoma

    Directory of Open Access Journals (Sweden)

    Jain Sandeep

    2010-01-01

    Full Text Available Ewing′s sarcoma constitutes three per cent of all pediatric malignancies. Ewing′s sarcoma has generally been more responsive to chemotherapy than adult-type sarcomas, and chemotherapy is now recommended for all patients with this disease. It is essential to integrate local control measures in the form of surgery and/or radiotherapy at the appropriate time, along with chemotherapy to eradicate the disease. This approach has improved the survival substantially to the tune of 70% in localized disease, although outcome for metastatic disease remains dismal. Newer therapeutic approaches are required to improve outcome for metastatic and recurrent or refractory Ewing′s sarcoma in organized co-operative group trials.

  10. 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...... analogue 18F-fluoro-deoxyglucose (FDG). We investigated clinically relevant tumor volumes and evaluated the relations to skeletal periosteum and nerve bundles. The patient scans suggest that FDG PET/MRI improved the edge detection, and invasion of tumor tissue into important adjacent anatomical structures...... planning, including radiation therapy planning in patients with STS....

  11. Sarcoma Immunotherapy

    Energy Technology Data Exchange (ETDEWEB)

    Gouw, Launce G., E-mail: launce.gouw@hsc.utah.edu [Departments of Oncology, Huntsman Cancer Institute at the University of Utah, 2000 Circle of Hope, Salt Lake City, UT 84112 (United States); Jones, Kevin B. [Departments of Orthopaedic Surgery, Huntsman Cancer Institute at the University of Utah, 2000 Circle of Hope, Salt Lake City, UT 84112 (United States); Sharma, Sunil [Departments of Oncology, Huntsman Cancer Institute at the University of Utah, 2000 Circle of Hope, Salt Lake City, UT 84112 (United States); Randall, R. Lor [Departments of Orthopaedic Surgery, Huntsman Cancer Institute at the University of Utah, 2000 Circle of Hope, Salt Lake City, UT 84112 (United States)

    2011-11-10

    Much of our knowledge regarding cancer immunotherapy has been derived from sarcoma models. However, translation of preclinical findings to bedside success has been limited in this disease, though several intriguing clinical studies hint at the potential efficacy of this treatment modality. The rarity and heterogeneity of tumors of mesenchymal origin continues to be a challenge from a therapeutic standpoint. Nonetheless, sarcomas remain attractive targets for immunotherapy, as they can be characterized by specific epitopes, either from their mesenchymal origins or specific alterations in gene products. To date, standard vaccine trials have proven disappointing, likely due to mechanisms by which tumors equilibrate with and ultimately escape immune surveillance. More sophisticated approaches will likely require multimodal techniques, both by enhancing immunity, but also geared towards overcoming innate mechanisms of immunosuppression that favor tumorigenesis.

  12. 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...

  13. 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...... modification. The main toxicity was haematotoxicity with grade 3/4 neutropenia in 49%, grade 3/4 thrombocytopenia in 23%, and grade 3/4 anaemia in 15% of patients, respectively. Non-haematological toxicity consisted mainly of grade 2/3 dyspnoea in 36% of patients and grade 2/3 fatigue in 28%. One treatment...

  14. Vismodegib and Gamma-Secretase/Notch Signalling Pathway Inhibitor RO4929097 in Treating Patients With Advanced or Metastatic Sarcoma

    Science.gov (United States)

    2016-06-09

    Adult Alveolar Soft Part Sarcoma; Adult Angiosarcoma; Adult Desmoplastic Small Round Cell Tumor; Adult Epithelioid Hemangioendothelioma; Adult Epithelioid Sarcoma; Adult Extraskeletal Myxoid Chondrosarcoma; Adult Extraskeletal Osteosarcoma; Adult Fibrosarcoma; Adult Leiomyosarcoma; Adult Liposarcoma; Adult Malignant Mesenchymoma; Adult Malignant Peripheral Nerve Sheath Tumor; Adult Rhabdomyosarcoma; Adult Synovial Sarcoma; Adult Unclassified Pleomorphic Sarcoma; Chondrosarcoma; Clear Cell Sarcoma of the Kidney; Conjunctival Kaposi Sarcoma; Dermatofibrosarcoma Protuberans; Gastrointestinal Stromal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Osteosarcoma; Ovarian Sarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Adult Unclassified Pleomorphic Sarcoma of Bone; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Kaposi Sarcoma; Recurrent Osteosarcoma; Recurrent Uterine Corpus Sarcoma; Small Intestine Leiomyosarcoma; Stage III Adult Soft Tissue Sarcoma; Stage III Uterine Sarcoma; Stage IV Adult Soft Tissue Sarcoma; Stage IV Uterine Sarcoma; Unclassified Pleomorphic Sarcoma of Bone

  15. Management of sporadic desmoid-type fibromatosis: a European consensus approach based on patients' and professionals' expertise - a sarcoma patients EuroNet and European Organisation for Research and Treatment of Cancer/Soft Tissue and Bone Sarcoma Group initiative.

    Science.gov (United States)

    Kasper, B; Baumgarten, C; Bonvalot, S; Haas, R; Haller, F; Hohenberger, P; Moreau, G; van der Graaf, W T A; Gronchi, A

    2015-01-01

    Desmoid-type fibromatosis (DF) is a rare monoclonal, fibroblastic proliferation characterised by a variable and often unpredictable clinical course. It may affect nearly all parts of the body including extremities, trunk and abdomen. Considering the variable clinical presentations, anatomic locations and biological behaviours, an individualised treatment approach is required. No established or evidence-based approach for the treatment of this neoplasm is available as of today. Therefore, we propose a consensus treatment algorithm based on a round table meeting bringing together sarcoma experts from the European Organisation for Research and Treatment of Cancer (EORTC) Soft Tissue and Bone Sarcoma Group (STBSG) with patient advocates from Sarcoma Patients EuroNet (SPAEN). The aim of the meeting was to develop - for the first time ever - a consensus approach based on professionals' AND patients' expertise. As a fundamental prerequisite, all patients should be discussed in a multidisciplinary setting in centres or professional networks with a specific expertise in the disease.

  16. Ecteinascidin-743: Evidence of Activity in Advanced, Pretreated Soft Tissue and Bone Sarcoma Patients

    Directory of Open Access Journals (Sweden)

    G. Huygh

    2006-01-01

    Full Text Available Purpose. To evaluate the activity and safety of ecteinascidin (ET-743 in pretreated patients with advanced or metastatic soft tissue and bone sarcoma. Patients or subjects. Eighty-nine patients received ET-743 as a 24-hour continuous infusion at a dose of 900–1500 μg/m2 every 3 weeks. Results. We observed one complete remission, 5 partial remissions, one minimal response, and 16 patients with a disease stabilization of 6 months or more. The objective response rate was 6.7% and the clinical benefit rate at 3 and 6 months was 37.7% and 23.4%, respectively. Responses were noted in patients with lipo-, leiomyo-, osteo-, and myogenic sarcoma, with a median duration of 9.85 months. Toxicity mainly involved an asymptomatic elevation of transaminases and neutropenia. Estimated 1- and 2-year survival rates were 39.4% and 15.8%. Median overall survival was 8.25 months. Discussion. This retrospective analysis confirms that ET-743 induces objective responses and progression arrest in a clinically relevant proportion of patients.

  17. Ecteinascidin-743: evidence of activity in advanced, pretreated soft tissue and bone sarcoma patients.

    Science.gov (United States)

    Huygh, G; Clement, Paul M J; Dumez, H; Schöffski, P; Wildiers, H; Selleslach, J; Jimeno, J M; Wever, I De; Sciot, R; Duck, L; Van Oosterom, A T

    2006-01-01

    Purpose. To evaluate the activity and safety of ecteinascidin (ET-743) in pretreated patients with advanced or metastatic soft tissue and bone sarcoma. Patients or subjects. Eighty-nine patients received ET-743 as a 24-hour continuous infusion at a dose of 900-1500 mug/m(2) every 3 weeks. Results. We observed one complete remission, 5 partial remissions, one minimal response, and 16 patients with a disease stabilization of 6 months or more. The objective response rate was 6.7% and the clinical benefit rate at 3 and 6 months was 37.7% and 23.4%, respectively. Responses were noted in patients with lipo-, leiomyo-, osteo-, and myogenic sarcoma, with a median duration of 9.85 months. Toxicity mainly involved an asymptomatic elevation of transaminases and neutropenia. Estimated 1- and 2-year survival rates were 39.4% and 15.8%. Median overall survival was 8.25 months. Discussion. This retrospective analysis confirms that ET-743 induces objective responses and progression arrest in a clinically relevant proportion of patients.

  18. Recent advances in targeted therapy for Ewing sarcoma [version 1; referees: 2 approved

    Directory of Open Access Journals (Sweden)

    Kathleen I. Pishas

    2016-08-01

    Full Text Available Ewing sarcoma is an aggressive, poorly differentiated neoplasm of solid bone that disproportionally afflicts the young. Despite intensive multi-modal therapy and valiant efforts, 70% of patients with relapsed and metastatic Ewing sarcoma will succumb to their disease. The persistent failure to improve overall survival for this subset of patients highlights the urgent need for rapid translation of novel therapeutic strategies. As Ewing sarcoma is associated with a paucity of mutations in readily targetable signal transduction pathways, targeting the key genetic aberration and master regulator of Ewing sarcoma, the EWS/ETS fusion, remains an important goal.

  19. [Modifying effect of the blood serum from patients with soft-tissue sarcomas on interleukin-2 production and activity].

    Science.gov (United States)

    Goretskiĭ, B A; Berezhnaia, N M; Palivets, A Iu; Konovalenko, V F

    1989-01-01

    Interleukin-2 (IL-2) production by lymphocytes of a peripheral blood (LPB) in patients with soft tissue sarcomas is considerably lower in comparison with that in donors. Autological serum had either suppressive or stimulative effect on LPB IL-2 production in the observed patients. The investigated serum affected LPB of donors in the same way. Serum of patients with soft tissue sarcomas had the same dual effect on the ability of the active drug IL-2 to proliferate human T-lymphoblasts. Moreover serum-inhibitors of production acted as IL-2 activity stimulators, while serum-stimulators of IL-2 production inhibited the mediator effects.

  20. Evaluation of 14 patients performed radiotherapy due to Kaposi sarcoma

    Directory of Open Access Journals (Sweden)

    Fatma Teke

    2015-09-01

    Full Text Available Methods: The patients undergoing radiotherapy (RT because of the KS between the years 2005-2012 in Radiation Oncology Department of Dicle University Hospital were included. All patients underwent RT with different dose-fractionation schemes to increase quality of life and to palliate the symptoms. Patients with lesions in multiple regions underwent RT in the same or different dates. Responses to radiotherapy were recorded as complete or partial response. Results: Fourteen patients received radiotherapy because of f KS were evaluated retrospectively. Twenty two different regions of 14 patients underwent RT . Only one patient (4.5% was performed RT to glans penis as a third region while performed to the two regions in six patients (27.3%. At irradiations, 6 MV and 10 MV photon energies with 6 MeV, 9 MeV and 12 MeV electron energy were used. Water phantom or bolus material was used to obtain a homogeneous dose distribution in the photon irradiation. RT dose administered to a total of 22 different regional was median 800 cGy (Range: 800-3000 cGy. Median number of RT fractions was 1 (Range: 1-10. When treatment response were evaluated stable disease was present in the 4 (18.1% regions. Partial response was achieved in eight (36.4% regions, complete response in 10 (45.5%. RT-related common lymphedema in the feet and legs was observed in the four (57.3% regions in the acute period. Complication of pain was present in two (28.7% regions. Conclusion: RT is an appropriate and effective treatment regimen in the palliative treatment of KS lesions. Excellent response rates of skin lesions may be obtained by RT. Lesions and symptoms such as itching may be lost after RT. Side effects such as edema and pain may be relieved by supportive treatment.

  1. A case of pulmonary Kaposi`s sarcoma in patient with renal transplantation : high resolution CT findings

    Energy Technology Data Exchange (ETDEWEB)

    Park, Hye Seong; Kim, Hak Hee; Choi, Yeong Jin; Kim, Young Ok; Shinn, Kyung Sub [Catholic Univ., Seoul (Korea, Republic of). Coll. of Medicine

    1998-03-01

    Kaposi`s sarcoma accounts for more than 3% of neoplasms occurring in patients who have undergone a transplant. An epidemiologic study showed that in renal transplanted patients, the incidence of Kaposi`s sarcoma was 400 to 500 times higher than in controls of the same ethnic origin. We report a case of Kaposi`s sarcoma involving the lung and skin after immunosuppressive therapy in a patient with renal transplant. A plain chest radiograph showed diffusely increased interstital opacity with multiple, ill-defined small nodules in both lung fields. HRCT revealed multiple small nodules, predominantly in the peribronchovascular regions, and ill-defined areas of ground-glass opacity and consolidation in both lungs. (author). 10 refs., 1 fig.

  2. Extraosseous Osteogenic Sarcoma

    Directory of Open Access Journals (Sweden)

    Shao-Ying Lin

    2005-11-01

    Full Text Available Extraosseous osteogenic sarcoma is a very rare malignant neoplasm. Out of the more than 400 cases of soft tissue sarcomas on file in our hospital, only 2 were extraosseous osteogenic sarcomas. Both were situated in the thigh. The first case was initially diagnosed as a hematoma and treated by marginal excision. The diagnosis of high-grade osteosarcoma primarily arising in soft tissue was made from histopathologic examination. Radiotherapy of 60 Gy in 30 fractions was given postoperatively. The second patient, primarily diagnosed as having a soft tissue sarcoma, was treated by wide excision. The final pathologic report was high-grade extraosseous osteogenic sarcoma. Adjuvant chemotherapy was given postoperatively. Both patients are alive without local recurrence and distant metastasis at postoperative 90-month and 107-month follow-up, respectively.

  3. Social support and adjustment in patients with sarcoma: the moderator effect of the disease phase.

    Science.gov (United States)

    Paredes, Tiago F; Canavarro, Maria C; Simões, Mário R

    2012-01-01

    This study examined the association between different types of perceived social support and adjustment of patients with sarcoma, and if these relationships would differ depending on the outcome measure and phase of disease. Forty-nine patients in the diagnostic phase, 43 in the treatment phase, and 59 in the follow-up phase were recruited. Participants completed the Medical Outcomes Study Social Support Survey Questionnaire, the Hospital Anxiety and Depression Scale, and World Health Organization Quality of Life Assessment. Positive social interaction, emotional/informational, affectionate, and tangible supports were significantly associated with depression scores, but not with anxiety. Except for affectionate support, all the associations with overall quality of life were significant. A moderating effect of the phase of the disease was also found in the association between tangible support and anxiety, and between affectionate support, depression, and overall quality of life. In clinical practice it is important to implement phase-specific psychosocial interventions and to take into consideration other factors beyond perceived social support while handling patients with sarcoma.

  4. 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...

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

    Science.gov (United States)

    2017-09-26

    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

  6. Patient experience after lower extremity amputation for sarcoma in England: a national survey.

    Science.gov (United States)

    Furtado, Sherron; Briggs, Tim; Fulton, Jennifer; Russell, Lin; Grimer, Rob; Wren, Vicky; Cool, Paul; Grant, Kristin; Gerrand, Craig

    2017-06-01

    After amputation, rehabilitation and limb fitting services are critically important to optimise outcomes. We investigated the reported patient experience and variation in limb fitting services after amputation for musculoskeletal tumours in England. A postal survey instrument was developed following literature review, patient and clinician consultation and piloting. The survey was sent from each of the five bone tumour surgical centres in England. One hundred and five responses were received from 250 patients (42%). The number of limb fitting centres accessed by each surgical centre varied from 2 to 28. Many patients reported care falling short of national standards in areas including pre-amputation counselling, information provision, meeting someone with a similar amputation before surgery, psychological support and falls management. Patients were seen sooner where limb fitting services were on site. Many patients rely on being driven, ambulance and public transport to access services. This study demonstrates variation in the reported experience of limb fitting services by sarcoma patients. Areas for improvement include information provision, pre-amputation counselling, psychological support and falls management. Clinicians should be aware services are highly variable, and this may impact on outcomes. Patients treated in sarcoma centres with limb fitting services on site may experience better care. Implications for Rehabilitation Rehabilitation services should strive to meet agreed national standards consistently. Where preamputation counselling involving meeting someone with a similar amputation is not possible, good information including video could be helpful. Services should support rehabilitation in the form of early walking aids and efficient prosthetic repair and maintenance. Psychological support, occupational therapy and physiotherapy support must be provided in the acute and chronic phases, including access to long-term rehabilitation care

  7. Therapeutic strategies for epidemic Kaposi's sarcoma

    NARCIS (Netherlands)

    Aldenhoven, M.; Barlo, N. P.; Sanders, C. J. G.

    2006-01-01

    Kaposi's sarcoma (KS) remains the most commonly diagnosed malignancy in HIV-infected patients, and is one of the AIDS-defining diagnoses. Several different therapeutic options are available, but the optimal therapy is still unclear. The incidence of KS has sharply declined since highly active antire

  8. Assessment of Objective Ambulation in Lower Extremity Sarcoma Patients with a Continuous Activity Monitor: Rationale and Validation

    Directory of Open Access Journals (Sweden)

    Kenneth R. Gundle

    2014-01-01

    Full Text Available In addition to patient reported outcome measures, accelerometers may provide useful information on the outcome of sarcoma patients treated with limb salvage. The StepWatch (SW Activity Monitor (SAM is a two-dimensional accelerometer worn on the ankle that records an objective measure of walking performance. The purpose of this study was to validate the SW in a cross-sectional population of adult patients with lower extremity sarcoma treated with limb salvage. The main outcome was correlation of total steps with the Toronto Extremity Salvage Score (TESS. In a sample of 29 patients, a mean of 12 days of SW data was collected per patient (range 6–16, with 2767 average total steps (S.D. 1867; range 406–7437. There was a moderate positive correlation between total steps and TESS (r=0.56,  P=0.002. Patients with osseous tumors walked significantly less than those with soft tissue sarcoma (1882 versus 3715, P<0.01. This study supports the validity of the SAM as an activity monitor for the objective assessment of real world physical function in sarcoma patients.

  9. Kaposi′s sarcoma with HHV8 infection and ANCA-associated vasculitis in a hemodialysis patient

    Directory of Open Access Journals (Sweden)

    Lilia Ben Fatma

    2013-01-01

    Full Text Available The association between Kaposi′s sarcoma (KS and human herpes virus eight (HHV-8 infection is rarely reported in hemodialysis (HD patients. We report here the rare association of KS, HHV-8 and hepatitis C virus (HCV infection as well as syphilis in a HD patient. We report the case of a 72-year-old woman who presented with microscopic polyangiitis with alveolar hemorrhage and pauci-immune necrosing and crescentic glomerulonephritis as well as renal failure requiring HD. Biological tests showed positive HCV and syphilis tests. The patient was treated by HD and intravenous pulse, followed by oral corticosteroids and six cyclophosphamide monthly pulses with remission of the alveolar hemorrhage, but without renal functional recovery as the patient remained HD dependent. Five months after the first treatment administration, she developed extensive purpuric lesions on her lower limbs, abdomen face and neck. A skin biopsy showed KS. The HHV-8 test was positive, with positive polymerase chain reaction-HHV8 in the serum and skin. After immunosuppression withdrawal, the KS skin lesions regressed rapidly without relapse after 12 months of follow-up, but alveolar hemorrhage relapsed after 16 months of follow-up. Our case showed that the immunosuppressed state related to multiple factors such as aging, vasculitis, HHV-8, HCV, syphilis, immunosuppressive therapy and HD may all have contributed to the development of KS in our patient.

  10. Distal lower extremity sarcomas: frequency of occurrence and patient survival rate.

    Science.gov (United States)

    Zeytoonjian, Trisha; Mankin, Henry J; Gebhardt, Mark C; Hornicek, Francis J

    2004-05-01

    Primary sarcomas in the distal leg, tibia, fibula, ankle, and foot are uncommon and are believed to be less malignant than those that arise in other sites, but only limited information is available to support this contention. Using a computerized system containing extensive information regarding over 14,000 patients, mostly with tumors treated by our center over a 25-year period, 175 sarcomatous lesions with MSTS stage I, II, and III were located in the distal lower extremity. These were compared with 2367 lesions of similar diagnoses in other body parts. The principal studies included diagnostic distribution and outcome (recorded as death as a result of disease). Data were compared for diagnosis, gender, age, Musculoskeletal Tumor Society (MSTS) stage, anatomic site, and treatment methods and evaluated statistically by chi-square methods. The most frequent distal lower limb tumors were synovial cell sarcoma, osteosarcoma, and Ewing's tumor and the percentage distribution of the various tumors for that site as compared with the rest of the body was quite different. Of even more importance was the remarkable difference in outcome with the death rate for the lower limb tumors set at 10%, while the same tumors at other sites had a death rate of 27% (p <.000002). Furthermore, gender, stage, age, and the type of operative procedure showed highly significant differences between the tumors of the lower leg and those of the remainder of the body. On the basis of these data, it is evident that far fewer sarcomas occur in the lower leg, tibia, fibula, ankle, and foot than in other body sites and that their diagnostic distribution is different. Of perhaps greater interest is the fact that the rate of metastasis and death is markedly reduced for this site as compared with others. The authors speculate on the causes of this remarkable alteration in numbers and outcome.

  11. Supraglottic Kaposi's Sarcoma in HIV-Negative Patients: Case Report and Literature Review

    Science.gov (United States)

    Server, Ela A.; Durna, Yusuf M.; Yigit, Ozgur; Bozkurt, Erol R.

    2016-01-01

    This paper presents a case report of an HIV-negative, supraglottic Kaposi's sarcoma patient. The 80-year-old male patient was admitted with complaints of hoarseness, difficulty in swallowing, and a stinging sensation in his throat for approximately six months. The endoscopic larynx examination revealed a lesion which had completely infiltrated the epiglottis, reached right aryepiglottic fold, was vegetating, pink and purple in color, multilobular, fragile, and shaped like a bunch of grapes, and partially blocked the bleeding airway passage. The case was discussed by the hospital's head-neck cancer committee and a surgery decision was made. A tracheotomy was performed under local anesthesia before the operation due to respiratory distress and endotracheal intubation difficulty. Direct laryngoscopy showed that the mass was limited in the supraglottic area, had invaded the entire left aryepiglottic fold and one-third of the front right aryepiglottic fold, and completely covered epiglottis. It should be remembered that although rare, Kaposi's sarcoma may be encountered in larynx malignancy cases. Disease-free survival may be achieved through local excision and postoperative radiotherapy. PMID:27375914

  12. Supraglottic Kaposi’s Sarcoma in HIV-Negative Patients: Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Ela A. Server

    2016-01-01

    Full Text Available This paper presents a case report of an HIV-negative, supraglottic Kaposi’s sarcoma patient. The 80-year-old male patient was admitted with complaints of hoarseness, difficulty in swallowing, and a stinging sensation in his throat for approximately six months. The endoscopic larynx examination revealed a lesion which had completely infiltrated the epiglottis, reached right aryepiglottic fold, was vegetating, pink and purple in color, multilobular, fragile, and shaped like a bunch of grapes, and partially blocked the bleeding airway passage. The case was discussed by the hospital’s head-neck cancer committee and a surgery decision was made. A tracheotomy was performed under local anesthesia before the operation due to respiratory distress and endotracheal intubation difficulty. Direct laryngoscopy showed that the mass was limited in the supraglottic area, had invaded the entire left aryepiglottic fold and one-third of the front right aryepiglottic fold, and completely covered epiglottis. It should be remembered that although rare, Kaposi’s sarcoma may be encountered in larynx malignancy cases. Disease-free survival may be achieved through local excision and postoperative radiotherapy.

  13. Quality of life assessment of patients in extremity sarcoma clinical trials.

    Science.gov (United States)

    Sugarbaker, P H; Barofsky, I; Rosenberg, S A; Gianola, F J

    1982-01-01

    Twenty-six patients with soft tissue sarcoma who participated in a randomized controlled clinical trial were studied to compare the impact of treatments on quality of life. Patients were treated with amputation plus chemotherapy or with limb-sparing surgery plus radiation therapy plus chemotherapy. After completion of treatments, when the patients' physical status had stabilized, a "Quality of Life Assessment" was administered. It consisted of the Psychosocial Adjustment to Illness Scale, the Sickness Impact Profile, the Barthel Function Scale, the Katz Activities of Daily Living Scale, nd an economic assessment. In addition, clinical assessments of mobility, pain, sexual relationships, and treatment trauma were obtained. Analysis of these assessments indicated that our hypothesis that limb-sparing surgery plus irradiation would provide improved quality of life when compared to amputation was not substantiated.

  14. Ewing sarcoma

    Science.gov (United States)

    Bone cancer - Ewing sarcoma; Ewing family of tumors; Primitive neuroectodermal tumors (PNET); Bone neoplasm - Ewing sarcoma ... this tissue to help determine how aggressive the cancer is and what treatment may be best.

  15. The Use of Positron Emission Tomography in Soft Tissue Sarcoma Patients under Therapy with Trabectedin

    Directory of Open Access Journals (Sweden)

    Gerlinde Egerer

    2009-07-01

    Full Text Available Background: We used 2-deoxy-2-[18F] fluoro-D-glucose (FDG positron emission tomography (PET to evaluate the FDG uptake in patients with advanced and/or metastatic soft tissue sarcoma (STS undergoing therapy with Ecteinascidin-743 (ET-743, Trabectedin, YondelisTM. Patients and Methods: The pilot study included nine patients with metastatic STS receiving a minimum of one cycle of treatment with trabectedin. Patients were examined using PET prior to onset of therapy and after completion of one or three cycles of trabectedin. Restaging according to Response Evaluation Criteria in Solid Tumours (RECIST was performed in parallel using computed tomography (CT and/or magnetic resonance imaging (MRI and served for reference. Results: Clinical outcome of nine evaluable patients was as follows: one patient with partial remission (PR, three patients with stable disease (SD, and five patients with progressive disease (PD. A more than 40% decrease of the standardized uptake value (SUV of sequential PET examination could be demonstrated for the responding patient (PR, whereas patients with SD or PD showed a stable SUV, but no increase in SUV. Conclusion: To our knowledge, this is the first small series of patients being treated with trabectedin and monitored using sequential PET imaging demonstrating SUV stabilization in nearly all monitored patients.

  16. Kaposi's Sarcoma-Associated Herpesvirus Infection of Neurons in HIV-Positive Patients.

    Science.gov (United States)

    Tso, For Yue; Sawyer, Ashley; Kwon, Eun Hee; Mudenda, Victor; Langford, Dianne; Zhou, You; West, John; Wood, Charles

    2017-06-15

    Kaposi's sarcoma-associated herpesvirus (KSHV) is the causative agent of Kaposi sarcoma (KS), one of the leading cancers in human immunodeficiency virus (HIV)-infected patients in Zambia. KSHV was detected in the human central nervous system (CNS) by polymerase chain reaction (PCR) analysis, but tissue location and cell tropism for KSHV infection has not been established. Given the neurotropism exhibited by other herpesviruses and the frequent coinfection of HIV-positive individuals by KSHV, we sought to determine whether the central nervous system (CNS) can be infected by KSHV in HIV-positive Zambian individuals. Postmortem brain tissue specimens were collected from individuals coinfected with KSHV and HIV. PCR and Southern blots were performed on DNA extracted from the brain tissue specimens to verify KSHV infection. Immunohistochemical analysis and immunofluorescent microscopy were used to localize and identify KSHV-infected cells. Tropism was further established by in vitro infection of primary human neurons with rKSHV.219. KSHV DNA was detected in the CNS from 4 of 11 HIV-positive individuals. Immunohistochemical analysis and immunofluorescent microscopy demonstrated that KSHV infected neurons and oligodendrocytes in parenchymal brain tissues. KSHV infection of neurons was confirmed by in vitro infection of primary human neurons with rKSHV.219. Our study showed that KSHV infects human CNS-resident cells, primarily neurons, in HIV-positive Zambian individuals.

  17. Early decrements in bone density after completion of neoadjuvant chemotherapy in pediatric bone sarcoma patients

    Directory of Open Access Journals (Sweden)

    Hardes Jendrik

    2010-12-01

    Full Text Available Abstract Background Bone mineral density (BMD accrual during childhood and adolescence is important for attaining peak bone mass. BMD decrements have been reported in survivors of childhood bone sarcomas. However, little is known about the onset and development of bone loss during cancer treatment. The objective of this cross-sectional study was to evaluate BMD in newly diagnosed Ewing's and osteosarcoma patients by means of dual-energy x-ray absorptiometry (DXA after completion of neoadjuvant chemotherapy. Methods DXA measurements of the lumbar spine (L2-4, both femora and calcanei were performed perioperatively in 46 children and adolescents (mean age: 14.3 years, range: 8.6-21.5 years. Mean Z-scores, areal BMD (g/cm2, calculated volumetric BMD (g/cm3 and bone mineral content (BMC, g were determined. Results Lumbar spine mean Z-score was -0.14 (95% CI: -0.46 to 0.18, areal BMD was 1.016 g/cm2 (95% CI: 0.950 to 1.082 and volumetric BMD was 0.330 g/cm3 (95% CI: 0.314 to 0.347 which is comparable to healthy peers. For patients with a lower extremity tumor (n = 36, the difference between the affected and non-affected femoral neck was 12.1% (95% CI: -16.3 to -7.9 in areal BMD. The reduction of BMD was more pronounced in the calcaneus with a difference between the affected and contralateral side of 21.7% (95% CI: -29.3 to -14.0 for areal BMD. Furthermore, significant correlations for femoral and calcaneal DXA measurements were found with Spearman-rho coefficients ranging from ρ = 0.55 to ρ = 0.80. Conclusions The tumor disease located in the lower extremity in combination with offloading recommendations induced diminished BMD values, indicating local osteopenia conditions. However, the results revealed no significant decrements of lumbar spine BMD in pediatric sarcoma patients after completion of neoadjuvant chemotherapy. Nevertheless, it has to be taken into account that bone tumor patients may experience BMD decrements or secondary osteoporosis

  18. Kaposi’s sarcoma: An unusual penile lesion in a HIV negative patient

    Directory of Open Access Journals (Sweden)

    Aldo Franco De Rose

    2017-06-01

    Full Text Available Kaposi's sarcoma (KS of the penis is a very rare lesion and it is usually observed in HIV-infected patients. We introduce a case of KS of the penis in a 75 years old HIV negative patient with a peripheral T-cell lymphoma. He came to our attention with a painful ulcerated red lesion on the glans that stretched from the urethral meatus to the coronal skin. This lesion was found to be a KS balanopreputial in the classical variant. Penile KS must be included in the differential diagnosis of genital diseases especially when the clinical features of the lesion are aspecific and diagnosis can be made histologically by performing a biopsy.

  19. Chylothorax in a patient with metastatic Kaposi sarcoma: Differential diagnostic considerations

    OpenAIRE

    Ryan Alexander, DO; Magda Rizer, DO; William Burke, MD; Lawrence Ciment, MD

    2015-01-01

    Kaposi sarcoma (KS) is a low-grade mesenchymal tumor involving blood and lymphatic vessels. There are four types, based on clinical presentation: classic, endemic (Africana), iatrogenic (typically, involving renal allograft recipients), and AIDS-associated (epidemic). Kaposi's sarcoma-associated herpes virus infection has been linked along with other factors to the development of KS. The Kaposi's sarcoma-associated herpes virus interacts and encodes for numerous molecular proteins that play a...

  20. The Danish Sarcoma Database

    Directory of Open Access Journals (Sweden)

    Jorgensen PH

    2016-10-01

    Full Text Available Peter Holmberg Jørgensen,1 Gunnar Schwarz Lausten,2 Alma B Pedersen3 1Tumor Section, Department of Orthopedic Surgery, Aarhus University Hospital, Aarhus, 2Tumor Section, Department of Orthopedic Surgery, Rigshospitalet, Copenhagen, 3Department of Clinical Epidemiology, Aarhus University Hospital, Aarhus, Denmark 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 characteristics such as location, size, malignancy grade, and growth pattern; details on treatment (kind of surgery, amount of radiation therapy, type and duration of chemotherapy; complications of treatment; local recurrence and metastases; and comorbidity. In addition, several quality indicators are registered in order to measure the quality of care provided by the hospitals and make comparisons between hospitals and with international standards. Descriptive data: Demographic patient-specific data such as age, sex, region of living, comorbidity, World Health Organization's International Classification 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 in Denmark since 2009. It is a valuable tool for monitoring sarcoma incidence and quality of treatment and its improvement, postoperative

  1. Phase II Study of Bevacizumab in Patients With HIV-Associated Kaposi's Sarcoma Receiving Antiretroviral Therapy

    Science.gov (United States)

    Uldrick, Thomas S.; Wyvill, Kathleen M.; Kumar, Pallavi; O'Mahony, Deirdre; Bernstein, Wendy; Aleman, Karen; Polizzotto, Mark N.; Steinberg, Seth M.; Pittaluga, Stefania; Marshall, Vickie; Whitby, Denise; Little, Richard F.; Yarchoan, Robert

    2012-01-01

    Purpose Alternatives to cytotoxic agents are desirable for patients with HIV-associated Kaposi's sarcoma (KS). Vascular endothelial growth factor-A (VEGF-A) contributes to KS pathogenesis. We evaluated the humanized anti–VEGF-A monoclonal antibody, bevacizumab, in patients with HIV-KS. Patients and Methods Patients with HIV-KS who either experienced progression while receiving highly active antiretroviral therapy (HAART) for at least 1 month or did not regress despite HAART for at least 4 months were administered bevacizumab 15 mg/kg intravenously on days 1 and 8 and then every 3 weeks. The primary objective was assessment of antitumor activity using modified AIDS Clinical Trial Group (ACTG) criteria for HIV-KS. HIV-uninfected patients were also eligible and observed separately. Results Seventeen HIV-infected patients were enrolled. Fourteen patients had been receiving effective HAART for at least 6 months (median, 1 year). Thirteen patients had advanced disease (ACTG T1), 13 patients had received prior chemotherapy for KS, and seven patients had CD4 count less than 200 cells/μL. Median number of cycles was 10 (range, 1 to 37 cycles); median follow-up was 8.3 months (range, 3 to 36 months). Of 16 assessable patients, best tumor responses observed were complete response (CR) in three patients (19%), partial response (PR) in two patients (12%), stable disease in nine patients (56%), and progressive disease in two patients (12%). Overall response rate (CR + PR) was 31% (95% CI, 11% to 58.7%). Four of five responders had received prior chemotherapy for KS. Over 202 cycles, grade 3 to 4 adverse events at least possibly attributed to therapy included hypertension (n = 7), neutropenia (n = 5), cellulitis (n = 3), and headache (n = 2). Conclusion Bevacizumab is tolerated in patients with HIV-KS and has activity in a subset of patients. PMID:22430271

  2. Incidentally Detected Kaposi Sarcoma of Adrenal Gland with Anaplastic Features in an HIV Negative Patient

    Directory of Open Access Journals (Sweden)

    Zeliha Esin Celik

    2016-01-01

    Full Text Available Kaposi sarcoma (KS, a vascular tumor caused by infection with human herpesvirus 8 (HHV8, is a systemic disease that can present with cutaneous lesions with or without visceral involvement. Very few cases of KS, most of which were associated with AIDS, have been reported in the adrenal gland. Anaplastic transformation of KS is a rare clinical presentation known as an aggressive disease with local recurrence and metastatic potential. We report here a 47-year-old HIV negative male presented with extra-adrenal symptoms and had an incidentally detected anaplastic adrenal KS exhibited aggressive clinical course. To the best of our knowledge, this is the first case of anaplastic primary adrenal KS without mucocutaneous involvement but subsequently developed other side adrenal metastases in an HIV negative patient.

  3. Incidentally Detected Kaposi Sarcoma of Adrenal Gland with Anaplastic Features in an HIV Negative Patient

    Science.gov (United States)

    Celik, Murat; Sen, Erdem; Cebeci, Hakan; Ata, Ozlem; Yavas, Cagdas

    2016-01-01

    Kaposi sarcoma (KS), a vascular tumor caused by infection with human herpesvirus 8 (HHV8), is a systemic disease that can present with cutaneous lesions with or without visceral involvement. Very few cases of KS, most of which were associated with AIDS, have been reported in the adrenal gland. Anaplastic transformation of KS is a rare clinical presentation known as an aggressive disease with local recurrence and metastatic potential. We report here a 47-year-old HIV negative male presented with extra-adrenal symptoms and had an incidentally detected anaplastic adrenal KS exhibited aggressive clinical course. To the best of our knowledge, this is the first case of anaplastic primary adrenal KS without mucocutaneous involvement but subsequently developed other side adrenal metastases in an HIV negative patient.

  4. NG2 expression predicts the metastasis formation in soft-tissue sarcoma patients.

    Science.gov (United States)

    Benassi, Maria Serena; Pazzaglia, Laura; Chiechi, Antonella; Alberghini, Marco; Conti, Amalia; Cattaruzza, Sabrina; Wassermann, Bruna; Picci, Piero; Perris, Roberto

    2009-01-01

    Enhanced expression levels of NG2 proteoglycan in presurgical original lesions of soft-tissue sarcoma (STS) patients defines with 55% probability the immediate (i.e., within 12 months postsurgery) risk in these individuals to develop postsurgical secondary lesions, independently of any other clinical trait. It, therefore, provides a molecular factor that alone prospects a particularly unfavorable clinical outcome in such patients. Evaluation of the timing of metastasis formation in patients with high and low levels of NG2 in their primitive lesions further stratified the patients in subsets with diverse lag phases in the occurrence of metastatic disease. In our cohort of high-grade STS cases, transcription of NG2 also showed a 81-fold amplification in metastatic lesions, when compared to primitive ones, and this gene overexpression was accompanied by an abundant but nonuniform in situ expression of its product. In a similar manner as seen in primitive lesions, patients with higher levels of metastatic NG2 encountered a significantly more dismal clinical course. Multivariate analysis asserted that in these individuals upregulation of NG2 represented an absolute independent prognostic parameter. Therefore, minimally invasive assessment of the transcription levels of the NG2 gene represents a parameter capable of predicting the arising of metastatic disease within a definite postsurgery time interval, and affords in adjunct in the definition of life expectance in STS patients.

  5. [Kaposi sarcoma and HHV-8: a model of cutaneous cancer in immunosuppressed patients].

    Science.gov (United States)

    Dupin, Nicolas; Deleuze, Jean

    2014-03-01

    The virus HHV-8 will celebrate its twentieth birthday by the end of this year and its relationships with Kaposi sarcoma are not completely elucidated. HHV-8 is an enigmatic virus, with an inhomogeneous distribution, a salivary transmission while it is not an ubiquitous virus, at least in western countries. However, HHV-8 has a unique genetic equipment rending is role in Kaposi sarcoma more than plausible. While the virus is necessary, it appears that it is not sufficient as the development of Kaposi sarcoma is frequently associated with immunosuppression whatever the cause (iatrogenic, viral, age-related). Kaposi sarcoma should be more considered as an opportunistic tumour than a viral-induced cancer and the best treatment for Kaposi sarcoma is immune restoration at least when it is possible.

  6. Treatment Options by Stage (Uterine Sarcoma)

    Science.gov (United States)

    ... Cancer Prevention Endometrial Cancer Screening Research Uterine Sarcoma Treatment (PDQ®)–Patient Version General Information About Uterine Sarcoma ... Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery ) and treatment ...

  7. PD-L1 Expression Is Associated with FOXP3+ Regulatory T-Cell Infiltration of Soft Tissue Sarcoma and Poor Patient Prognosis.

    Science.gov (United States)

    Que, Yi; Xiao, Wei; Guan, Yuan-Xiang; Liang, Yao; Yan, Shu-Mei; Chen, Huo-Ying; Li, Qiao-Qiao; Xu, Bu-Shu; Zhou, Zhi-Wei; Zhang, Xing

    2017-01-01

    Background: Programmed death ligand-1(PD-L1) functions as a negative mediator of immune response through different pathways in anti-tumor immunity. Recent studies have reported that PD-L1 plays a pivotal role in the function of regulatory T-cells (Tregs). Although increases in FOXP3+ Tregs infiltration and PD-L1 expression have been revealed in several cancers, their correlation with soft tissue sarcoma remains unknown. Methods: We included 163 cases of soft tissue sarcoma who were diagnosed and underwent extensive and radical resection at the Sun Yat-sen University Cancer Center, Guangzhou, China, from 2000-2010. PD-L1 and FOXP3 expression was evaluated by immunohistochemistry. Correlation between their expressions and associations with clinicopathological features were studied. Results: Among 163 STS samples, 19 (11.7%) exhibited PD-L1 positivity, and 41 (25.2%) cases expressed high FOXP3+ Treg infiltration. Significant correlation between PD-L1 expression and FOXP3+Treg infiltration in STS was identified (r=0.450, pTregs was significantly associated with the age of patients, high tumor stage, higher tumor grade and tumor depth. Multivariate analysis revealed PD-L1 and FOXP3 as independent prognostic indicators significantly associated with OS and DFS. Conclusions: Our study revealed that PD-L1 and FOXP3+Tregs may work synergistically in promoting immune evasion of the tumors in soft tissue sarcoma. A combined strategy to block PD-L1/PD-1 with simultaneous depletion of Tregs may show promise in enhancing the therapeutic efficacy of these patients.

  8. Primary renal synovial sarcoma

    Directory of Open Access Journals (Sweden)

    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.

  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. PET surveillance of patients with ewing sarcomas of the trunk. Must the lower legs be included?

    Energy Technology Data Exchange (ETDEWEB)

    Vrachimis, A.; Stegger, L.; Weckesser, M. [Klinik und Poliklinik fuer Nuklearmedizin, Univ. Muenster (Germany); Dirksen, U.; Juergens, H. [Klinik und Poliklinik fuer Kinder- und Jugendmedizin - Paediatrische Haematologie und Onkologie, Univ. Muenster (Germany); Wessling, J. [Inst. fuer Klinische Radiologie, Univ. Muenster (Germany); Wenning, C.; Schober, O. [Klinik und Poliklinik fuer Nuklearmedizin, Univ. Muenster (Germany); European Inst. of Molecular Imaging, Westfaelische Wilhelms Univ. Muenster (Germany); Franzius, C. [MR- und PET/CT-Zentrum Bremen Mitte, Bremen (Germany)

    2010-07-01

    Aim: FDG-PET(/CT) is frequently used in surveillance of Ewing sarcoma (ES) patients. Since ES and PNET (primitive neuroectodermal tumours) may cause peripheral metastases some centers routinely recommend whole body PET acquisition from head to toe what may necessitate repositioning of the patient and thus extending examination time. It is not clear yet whether inclusion of lower leg adds to the diagnostic accuracy of PET scanning, especially in primary tumors of the trunk. Patients, method: 40 patients with ES and PNET of the trunk who were referred for surveillance after primary therapy with complete remission, were evaluated retrospectively: 27 men, 13 women; mean age at diagnosis 16.3 (3-35) years. At the time of diagnosis 28 patients had localized and 12 metastatic disease. Almost all of the patients had undergone a combined chemotherapy with surgery or/and radiotherapy. 156 follow-up PET scans of the legs of these patients were evaluated retrospectively. Results: Only in three (1.9%) of 156 scans a pathologic FDG accumulation was attributed to metastatic disease of the lower extremities. In these cases the observation of metastatic disease in the legs did not alter therapy, since in all three cases a multifocal disease progression was observed. Conclusion: Scanning of the lower legs may be omitted during follow-up in patients in whom the primary tumor was located in the trunk and in whom no clinical signs pointing to metastases in the lower legs are present. This provides a sufficient diagnostic power and a shorter examination time, thus increasing patient comfort and scanner availability. (orig.)

  11. Complete oral rehabilitation of a deep bite patient with Ewing’s sarcoma; surgical and prosthetic phases: A clinical report

    Directory of Open Access Journals (Sweden)

    Mehran Bahrami

    2017-01-01

    Full Text Available Introduction: Ewings sarcoma (ES is an unusual, aggressive malignant neoplasm occurring primarily in the long bones of the lower and upper extremities followed by pelvis, ribs, vertebrae, skull, and jaws-bones. Patients with ES tumor need long-term complete oral reconstruction. The location of the primary tumor in the face is predominantly in the mandible, and usually in the posterior mandible. Case Report: Here, both surgical procedures and prosthetic reconstruction in three stages of a 10-year-old childs life presented. Definitive prosthetic reconstruction is extremely challenging in such patients after previous excessive surgical resection, especially when the patient has complex dental abnormality. Fixed dental prostheses and implant-supported fixed restorations have been selected as the preferred treatment option in this case after increasing occlusal vertical dimension (OVD to create sufficient interocclusal space (IOS. Discussion: In this case, ES patient treated in three phases. In the first-stage, only surgical resection of tumor and bone graft reconstruction with titanium plate fixation was performed. In the second-stage, Lefort I surgery for maxilla with dental implants insertion in mandible was done simultaneously. For definitive restorations fabrication, it was mandatory to increase occlusal vertical dimension (OVD to create sufficient inter-occlusal space (IOS for the restorations. OVD has been altered to achieve more stable relationship of mandibular teeth with maxillary teeth, and to obtain an optimum esthetic and functional result. Several factors should be considered as determinants for increasing the OVD such as remaining tooth structure, the space available for the restorations such as the current situation, occlusal variables, and esthetics.

  12. HHV-8 infection in patients with AIDS-related Kaposi's sarcoma in Brazil

    Directory of Open Access Journals (Sweden)

    Keller R.

    2001-01-01

    Full Text Available The aims of the present study were to determine the prevalence of human herpesvirus type 8 (HHV-8 in HIV-positive Brazilian patients with (HIV+/KS+ and without Kaposi's sarcoma (HIV+/KS- using PCR and immunofluorescence assays, to assess its association with KS disease, to evaluate the performance of these tests in detecting HHV-8 infection, and to investigate the association between anti-HHV-8 antibody titers, CD4 counts and staging of KS disease. Blood samples from 66 patients, 39 HIV+/KS+ and 27 HIV+/KS-, were analyzed for HHV-8 viremia in peripheral blood mononuclear cells by PCR and HHV-8 antigenemia for latent and lytic infection by immunofluorescence assay. Positive samples for latent nuclear HHV-8 antigen (LNA antibodies were titrated out from 1/100 to 1/409,600 dilution. Clinical information was collected from medical records and risk behavior was assessed through an interview. HHV-8 DNA sequences were detected by PCR in 74.3% of KS+ patients and in 3.7% of KS- patients. Serological assays were similar in detecting anti-LNA antibodies and anti-lytic antigens in sera from KS+ patients (79.5% and KS- patients (18.5%. HHV-8 was associated with KS whatever the method used, i.e., PCR (odds ratio (OR = 7.4, 95% confidence interval (CI = 2.16-25.61 or anti-LNA and anti-lytic antibodies (OR = 17.0, 95%CI = 4.91-59.14. Among KS+ patients, HHV-8 titration levels correlated positively with CD4 counts (rho 0.48, P = 0.02, but not with KS staging. HHV-8 is involved in the development of KS in different geographic areas worldwide, as it is in Brazil, where HHV-8 is more frequent among HIV+ patients. KS severity was associated with immunodeficiency, but no correlation was found between HHV-8 antibody titers and KS staging.

  13. Should Aggressive Surgical Local Control Be Attempted in All Patients with Metastatic or Pelvic Ewing's Sarcoma?

    Directory of Open Access Journals (Sweden)

    Steven W. Thorpe

    2012-01-01

    Full Text Available In previous reports, patients with Ewing's sarcoma received radiation therapy (XRT for definitive local control because metastatic disease and pelvic location were thought to preclude aggressive local treatment. We sought to determine if single-site metastatic disease should be treated differently from multicentric-metastatic disease. We also wanted to reinvestigate the impact of XRT, pelvic location, and local recurrence on outcomes. Our results demonstrated a significant difference in overall survival (OS between patients with either localized disease or a single-metastatic site and patients with multicentric-metastatic disease (=0.004. Local control was also found to be an independent predictor of outcomes as demonstrated by a significant difference in OS between those with and without local recurrence (=0.001. Axial and pelvic location did not predict a decreased OS. Based on these results, we concluded that pelvic location and the diagnosis of metastatic disease at diagnosis should not preclude aggressive local control, except in cases of multicentric-metastatic disease.

  14. Retroperitoneal Sarcomas.

    Science.gov (United States)

    Porpiglia, Andrea S; Reddy, Sanjay S; Farma, Jeffrey M

    2016-10-01

    Retroperitoneal sarcomas are rare tumors, representing only 15% of all sarcomas. The mainstay of therapy is surgical resection with negative margins. However, this is challenging because of the late presentation of many of these tumors and involvement with adjacent structures. Decisions on radiation therapy and chemotherapy should be made in a multidisciplinary setting at a tertiary referral center.

  15. Combination Chemotherapy With or Without Ganitumab in Treating Patients With Newly Diagnosed Metastatic Ewing Sarcoma

    Science.gov (United States)

    2017-03-27

    Metastatic Ewing Sarcoma; Metastatic Malignant Neoplasm in the Bone; Metastatic Malignant Neoplasm in the Bone Marrow; Metastatic Malignant Neoplasm in the Lung; Metastatic Peripheral Primitive Neuroectodermal Tumor of Bone; Peripheral Primitive Neuroectodermal Tumor of Soft Tissues

  16. Primary Pericardial Sarcoma with Right Atrial Invasion and Multiple Bilateral Pulmonary Metastases in a Patient with Hereditary Nonpolyposis Colorectal Cancer

    Directory of Open Access Journals (Sweden)

    Eugene Wong

    2016-01-01

    Full Text Available Primary tumours originating from the pericardium are extremely rare. Previous studies have reported that these tumours account for only 6.7–12.8% of all mediastinal tumours with an overall prevalence of 0.001% to 0.007%. The majority of these tumours are benign lipomas or pericardial cysts. The most common pericardial malignancy is mesothelioma. Sarcomas are soft-tissue mesenchymal malignancies originating from various parts of the body but are extremely rare in this area. We report a case of a 52-year-old female who was diagnosed with a primary sarcoma with rhabdoid differentiation originating from the pericardium. The patient presented to her GP with a four-week history of progressive dyspnea and chest pain on exertion. Chest X-Ray demonstrated a prominent pericardial effusion and suspicious chest and pericardial lesions. Biopsies of the effusion and primary tumour identified on FDG/PET scans revealed the diagnosis of primary undifferentiated sarcoma. On thoracotomy, it was noted that the tumour had invaded the right atrium; therefore, pericardial window was aborted and a drain inserted instead. The patient was then started on chemotherapy; however, progression soon occurred and the patient died within 4 months, suggesting there is urgent need for efficacious treatments for sarcomatous lesions.

  17. Histiocytic Sarcoma in a Kidney Transplant Patient: A Case Report and Review of the Literature

    Science.gov (United States)

    Pollen, Maressa; El Jamal, Siraj; Lewin, Jack

    2016-01-01

    Objective. Histiocytic sarcoma (HS) is an aggressive neoplasm with only limited number of reported series of cases and rare case reports of occurrence as a posttransplant neoplastic disorder. The etiology and pathogenesis of the disease is unknown and the optimal treatment is still under investigation. We describe an unusual case of HS in a patient with a remote history of kidney transplant. Method and Results. A 54-year-old male with a remote history of renal transplantation under maintenance immunosuppression presented with features of sepsis. CT abdomen revealed multiple heterogeneous masses in bilateral native kidneys and liver and enlarged abdominal and retroperitoneal lymph nodes. Viral serology work-up was negative. Needle core biopsy revealed a highly undifferentiated neoplasm comprised of highly atypical large cells with eosinophilic to vacuolated cytoplasm and hemophagocytosis. Extended panel of immunohistochemistry proved histiocytic lineage for the tumor cells. The patient expired 2 weeks following the diagnosis. Conclusion. Our case along with three previously published case reports raised the possibility of HS as a treatment-related neoplasm or a posttransplantation neoplastic disorder in solid organ transplant recipients.

  18. Histiocytic Sarcoma in a Kidney Transplant Patient: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Maressa Pollen

    2016-01-01

    Full Text Available Objective. Histiocytic sarcoma (HS is an aggressive neoplasm with only limited number of reported series of cases and rare case reports of occurrence as a posttransplant neoplastic disorder. The etiology and pathogenesis of the disease is unknown and the optimal treatment is still under investigation. We describe an unusual case of HS in a patient with a remote history of kidney transplant. Method and Results. A 54-year-old male with a remote history of renal transplantation under maintenance immunosuppression presented with features of sepsis. CT abdomen revealed multiple heterogeneous masses in bilateral native kidneys and liver and enlarged abdominal and retroperitoneal lymph nodes. Viral serology work-up was negative. Needle core biopsy revealed a highly undifferentiated neoplasm comprised of highly atypical large cells with eosinophilic to vacuolated cytoplasm and hemophagocytosis. Extended panel of immunohistochemistry proved histiocytic lineage for the tumor cells. The patient expired 2 weeks following the diagnosis. Conclusion. Our case along with three previously published case reports raised the possibility of HS as a treatment-related neoplasm or a posttransplantation neoplastic disorder in solid organ transplant recipients.

  19. Combined Modality Management of Retroperitoneal Sarcomas: A Single-Institution Series of 121 Patients

    Energy Technology Data Exchange (ETDEWEB)

    Bishop, Andrew J.; Zagars, Gunar K. [Department of Radiation Oncology, MD Anderson Cancer Center, Houston, Texas (United States); Torres, Keila E.; Hunt, Kelly K.; Cormier, Janice N.; Feig, Barry W. [Department of Surgical Oncology, MD Anderson Cancer Center, Houston, Texas (United States); Guadagnolo, B. Ashleigh, E-mail: aguadagn@mdanderson.org [Department of Radiation Oncology, MD Anderson Cancer Center, Houston, Texas (United States); Department of Health Services Research, MD Anderson Cancer Center, Houston, Texas (United States)

    2015-09-01

    Purpose: The purpose of this study was to investigate local control, survival outcomes, and complication rates of patients treated with aggressive surgery and radiation therapy (RT) for retroperitoneal sarcomas (RPS). Methods and Materials: We reviewed the medical records of 121 consecutive patients treated for RPS with surgery and RT between 1965 and 2012. The most common histology was liposarcoma (n=42; 35%). The median follow-up was 100 months (range: 20-467 months). Eighty-six patients (71%) were treated for initial presentation of RPS, and 35 patients (29%) presented with and were treated for RPS recurrence. RT was preoperative in 88 patients (73%; median dose: 50.4 Gy) and postoperative in 33 patients (27%; median dose: 55 Gy). Results: Five-year local control and overall survival rates were 56% and 57%, respectively. Two factors were associated with higher risk of any intra-abdominal recurrence at 5 years: positive or uncertain margins (58% vs 30% for negative margins, P<.001; hazard ratio [HR]: 2.7; 95% confidence interval [CI]: 1.6-4.8) and disease recurrence after previous resection (76% vs 31% for de novo RPS, P<.001; HR: 4.4; 95% CI: 2.5-7.5). The 10-year complication rate was 5%, and RT-related complications were associated with postoperative RT (P<.001) and RT dose of ≥60 Gy (P<.001). Conclusions: Intra-abdominal RPS recurrence continues to be a significant challenge despite the use of aggressive surgery and radiation therapy. Given the complications associated with postoperative radiation therapy, we recommend that preoperative radiation therapy is the preferred strategy when combined modality therapy is recommended.

  20. Granulocytic Sarcoma in a Nonleukemic Patient: Place of Radiotherapy and Systemic Therapies

    Directory of Open Access Journals (Sweden)

    C. Chargari

    2011-01-01

    Full Text Available Granulocytic sarcoma is a rare extramedullary tumour, which most often occurs in the course of an acute or chronic leukaemia or myeloproliferative disorders. Rarely it is found before peripheral blood or bone marrow evidence of leukemia is present. We report an unusual case of acute paraplegia at first presentation of a spinal epidural granulocytic sarcoma without any haematological disorder. Therapeutic strategies are discussed in the light of the literature.

  1. Predictors of patients remaining anovulatory during clomiphene citrate induction of ovulation in normogonadotropic oligoamenorrheic infertility

    NARCIS (Netherlands)

    B. Imani (Babak); M.J.C. Eijkemans (René); E.R. te Velde (Egbert); J.D.F. Habbema (Dik); B.C.J.M. Fauser (Bart)

    1998-01-01

    textabstractThe diagnostic criteria used to identify patients suffering from polycystic ovary syndrome remain controversial. The present prospective longitudinal follow-up study was designed to identify whether certain criteria assessed during standardized initial scree

  2. Predictors of patients remaining anovulatory during clomiphene citrate induction of ovulation in normogonadotropic oligoamenorrheic infertility

    NARCIS (Netherlands)

    B. Imani (Babak); M.J.C. Eijkemans (René); E.R. te Velde (Egbert); J.D.F. Habbema (Dik); B.C.J.M. Fauser (Bart)

    1998-01-01

    textabstractThe diagnostic criteria used to identify patients suffering from polycystic ovary syndrome remain controversial. The present prospective longitudinal follow-up study was designed to identify whether certain criteria assessed during standardized initial

  3. Vascularized fibula grafts for reconstruction of bone defects after resection of bone sarcomas

    DEFF Research Database (Denmark)

    Petersen, Michael Mørk; Hovgaard, Dorrit; Elberg, Jens Jørgen

    2010-01-01

    We evaluated the results of limb-sparing surgery and reconstruction of bone defects with vascularized fibula grafts in 8 consecutive patients (mean age at operation 13.6 years (range 4.1-24.2 years), female/male = 6/2) with bone sarcomas (BS) (osteosarcoma/Ewing's sarcoma/chondrosarcoma= 4......'s sarcoma had an early hip disarticulation, developed multiple metastases, and died 9 months after the operation. The remaining patients (n = 7) are all alive 50 months (range 26-75 months) after surgery. During the follow-up the following major complications were seen: 1-2 fractures (n = 4), pseudarthrosis...

  4. Pazopanib in advanced vascular sarcomas: an EORTC Soft Tissue and Bone Sarcoma Group (STBSG) retrospective analysis

    NARCIS (Netherlands)

    Kollar, A.; Jones, R.L.; Stacchiotti, S.; Gelderblom, H.; Guida, M.; Grignani, G.; Steeghs, N.; Safwat, A.; Katz, D.; Duffaud, F.; Sleijfer, S.; Graaf, W.T. van der; Touati, N.; Litiere, S.; Marreaud, S.; Gronchi, A.; Kasper, B.

    2017-01-01

    BACKGROUND: Pazopanib is a multitargeted tyrosine kinase inhibitor approved for the treatment of patients with selective subtypes of advanced soft tissue sarcoma (STS) who have previously received standard chemotherapy including anthracyclines. Data on the efficacy in vascular sarcomas are limited.

  5. What Is Kaposi Sarcoma?

    Science.gov (United States)

    ... Treatment? Kaposi Sarcoma About Kaposi Sarcoma What Is Kaposi Sarcoma? Cancer starts when cells in the body begin ... the lungs may cause trouble breathing. Types of Kaposi sarcoma The different types of KS are defined by ...

  6. The role of {sup 18}F-FDG PET/CT as a prognostic factor in patients with synovial sarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Chang, Kyoung Jin; Lim, Il Han; Park, Joon Yeun [Korea Institute of Radiological and Medical Sciences, Seoul (Korea, Republic of)

    2015-03-15

    This research aims to investigate the potential of 18F-fluorodeoxyglucose positron emission tomography/computed tomography (FDG PET) to predict pathologic response after neoadjuvant chemotherapy (NAC) and overall survival (OS) of patients with synovial sarcoma in Korea. Twenty patients with synovial sarcoma from January 2001 to December 2011 were reviewed retrospectively. All patients underwent pre-treatment FDG PET and tumor removal. Patients were classified with the maximum SUV (SUVmax), metabolic tumor volume (MTV), total lesion glycolysis (TLG), age, sex, histologic subtype, tumor size, NAC, resection margin, and metastasis at diagnosis. Pathologic response was assessed using the French Federation of Cancer Centers system. Statistical analyses were analyzed using the Kaplan-Meier method, log-rank test, Cox proportional hazards regression model, and Mann-Whitney test. Nine patients (45 %) showed pathologic response, and ten patients survived. Higher SUVmax, higher MTV, higher TLG, monophasic epithelial type, and metastasis at diagnosis were significantly related to poorer OS (p = 0.047, 0.016, 0.016, 0.045, and 0.018, respectively). By multivariate analysis, metastasis at diagnosis was significantly related to poorer OS (p = 0.012/HR = 5.9, 95 % CI 1.47 to 24.1). The SUVmax, MTV, and TLG of the non-responder group were significantly higher than those of the responder group (p = 0.020, 0.020, and 0.020, respectively). There was no significant difference in size between the two groups (p = 0.062). A higher SUVmax on the pre-treatment scan, monophasic epithelial type, and metastasis at diagnosis were significantly associated with a poorer OS, and pathologic responders showed a higher SUVmax before NAC. The PET parameters can be used to predict OS and pathologic response in patients with synovial sarcomas before NAC.

  7. Immunotherapeutic Intervention against Sarcomas

    Directory of Open Access Journals (Sweden)

    Paolo Pedrazzoli, Simona Secondino, Vittorio Perfetti, Patrizia Comoli, Daniela Montagna

    2011-01-01

    Full Text Available Advances in systemic therapy for sarcoma have produced, over the last two decades, relatively short-term benefits for the majority of patient. Among the novel biologic therapeutics that will likely increase our ability to cure human cancer in the years to come, immunotherapy is one of the most promising approaches. While past attempts to use immunotherapy have failed to dramatically shift the paradigm of care for the treatment of patients with sarcoma, major advances in basic and translational research have resulted, in more recent years, in clinical trial activity that is now beginning to generate promising results. However, to move from “proof of principle” to large scale clinical applicability, we need well-designed, multi-institutional clinical trials, along with continuous laboratory research to explore further the immunological characteristics of individual sarcoma subtypes and the consequent tailoring of therapy.

  8. Characterization of Human Mesenchymal Stem Cells from Ewing Sarcoma Patients. Pathogenetic Implications

    Science.gov (United States)

    Amaral, Ana Teresa; Manara, Maria Cristina; Berghuis, Dagmar; Ordóñez, José Luis; Biscuola, Michele; Lopez-García, Maria Angeles; Osuna, Daniel; Lucarelli, Enrico; Alviano, Francesco; Lankester, Arjan; Scotlandi, Katia; de Álava, Enrique

    2014-01-01

    Background Ewing Sarcoma (EWS) is a mesenchymal-derived tumor that generally arises in bone and soft tissue. Intensive research regarding the pathogenesis of EWS has been insufficient to pinpoint the early events of Ewing sarcomagenesis. However, the Mesenchymal Stem Cell (MSC) is currently accepted as the most probable cell of origin. Materials and Methods In an initial study regarding a deep characterization of MSC obtained specifically from EWS patients (MSC-P), we compared them with MSC derived from healthy donors (MSC-HD) and EWS cell lines. We evaluated the presence of the EWS-FLI1 gene fusion and EWSR1 gene rearrangements in MSC-P. The presence of the EWS transcript was confirmed by q-RT-PCR. In order to determine early events possibly involved in malignant transformation, we used a multiparameter quantitative strategy that included both MSC immunophenotypic negative/positive markers, and EWS intrinsic phenotypical features. Markers CD105, CD90, CD34 and CD45 were confirmed in EWS samples. Results We determined that MSC-P lack the most prevalent gene fusion, EWSR1-FLI1 as well as EWSR1 gene rearrangements. Our study also revealed that MSC-P are more alike to MSC-HD than to EWS cells. Nonetheless, we also observed that EWS cells had a few overlapping features with MSC. As a relevant example, also MSC showed CD99 expression, hallmark of EWS diagnosis. However, we observed that, in contrast to EWS cells, MSC were not sensitive to the inhibition of CD99. Conclusions In conclusion, our results suggest that MSC from EWS patients behave like MSC-HD and are phenotypically different from EWS cells, thus raising important questions regarding MSC role in sarcomagenesis. PMID:24498265

  9. Characterization of FGFR signaling pathway as therapeutic targets for sarcoma patients.

    Science.gov (United States)

    Zhou, Wen-Ya; Zheng, Hong; Du, Xiao-Ling; Yang, Ji-Long

    2016-06-01

    The fibroblast growth factor receptor (FGFR) family plays important roles in regulating cell growth, proliferation, survival, differentiation and angiogenesis. Deregulation of the FGF/FGFR signaling pathway has been associated with multiple development syndromes and cancers, and thus therapeutic strategies targeting FGFs and FGFR in human cancer are currently being explored. However, few studies on the FGF/FGFR pathway have been conducted in sarcoma, which has a poor outcome with traditional treatments such as surgery, chemotherapy, and radiotherapy. Hence, in the present review, we provide an overview of the role of the FGF/FGFR pathway signal in sarcoma and FGFR inhibitors, which might be new targets for the treatment of sarcomas according to recent research.

  10. Characterization of FGFR signaling pathway as therapeutic targets for sarcoma patients

    Institute of Scientific and Technical Information of China (English)

    Wen-Ya Zhou; Hong Zheng; Xiao-Ling Du; Ji-Long Yang

    2016-01-01

    The fibroblast growth factor receptor (FGFR) family plays important roles in regulating cell growth, proliferation, survival, differentiation and angiogenesis. Deregulation of the FGF/FGFR signaling pathway has been associated with multiple development syndromes and cancers, and thus therapeutic strategies targeting FGFs and FGFR in human cancer are currently being explored. However, few studies on the FGF/FGFR pathway have been conducted in sarcoma, which has a poor outcome with traditional treatments such as surgery, chemotherapy, and radiotherapy. Hence, in the present review, we provide an overview of the role of the FGF/FGFR pathway signal in sarcoma and FGFR inhibitors, which might be new targets for the treatment of sarcomas according to recent research.

  11. Kaposi’s sarcoma in Brazilian AIDS patients: a study of 144 cases Sarcoma de Kaposi em pacientes com AIDS: estudo de 144 casos

    Directory of Open Access Journals (Sweden)

    Esther G. BIRMAN

    2000-12-01

    Full Text Available One hundred and forty-four Brazilian AIDS patients presenting with Kaposi’s sarcoma (KS were evaluated with respect to the frequency of oral neoplasms and their clinical features. The majority of the patients were young male adults (age range: 21-40 years old, from which 11.1 % presented with oral KS (OKS exclusively. Oral and skin lesions were associated in 25% of the cases, while only four patients showed association between oral and visceral KS; 49.3% of the cases were exclusively dermatological. The hard palate was the main site affected, followed by the oropharynx. The localization of KS was found to be similarly frequent in the tongue, gingiva and other sites of the oral mucosa. Candidosis was the prevailing fungal disease; in 20% of the cases it was restricted to the oral mucosa and in 80% it was systemic. No high frequency of paracoccidioidomicosis and cryptococcosis was detected. The prevailing bacterial disease was Tuberculosis and there was only one case of syphilis. Among the viral diseases, the most frequently detected was herpes simplex, followed by molusco contagiosum, condiloma acuminatum and cytomegaloviroses at lower frequencies. Pneumonia caused by Pneumocystes carinii and toxoplasmosis were also identified. The authors emphasise the importance of oral examination in HIV-infected patients bearing in mind several aspects related especially to KS, and stress the need for an interdisciplinary team in the management of these patients, in order to provide better quality of life as well as rapid diagnosis and treatment.Foram estudados pacientes brasileiros portadores de SIDA apresentando sarcoma de Kaposi (SK. O perfil de idade mostrou um grupo com média de idade entre 21 e 40 anos, sendo que 11,1% da amostra apresentava SK exclusivamente na cavidade bucal, observando-se em 25% da amostra uma associação de lesões bucais e na pele. Somente quatro pacientes apresentaram associação de lesões bucais e viscerais, enquanto 49

  12. Impact of surgery, radiation and systemic therapy on the outcomes of patients with dendritic cell and histiocytic sarcomas

    Science.gov (United States)

    Gounder, Mrinal; Desai, Ved; Kuk, Deborah; Agaram, Narasimhan; Arcila, Maria; Durham, Benjamin; Keohan, Mary L.; Dickson, Mark A.; D’Angelo, Sandra P.; Shukla, Neerav; Moskowitz, Craig; Noy, Ariela; Maki, Robert G.; Herrera, Diego Adrianzen; Sanchez, Armando; Krishnan, Anita; Pourmoussa, Andrew; Qin, Li-Xuan; Tap, William D.

    2016-01-01

    Background Neoplasms of histiocytic and dendritic cell origin, including follicular dendritic cell sarcoma (FDCS), histiocytic sarcoma (HS) and interdigitating dendritic cell sarcoma (IDCS), are extremely rare, and data on their natural history and treatment outcomes are sparse. We evaluated the impact of surgery, radiation and systemic therapies on overall survival (OS). Methods We conducted a retrospective chart review of patients with FDCS, IDCS and HS treated at Memorial Sloan Kettering Cancer Center between 1995 and 2014. Results We identified 31, 15 and 7 patients with FDCS, HS and IDCS, respectively. Median age was 48.7, 42.3 and 58.8 years for FDCS, HS and IDCS, respectively. Only a slight disparity in gender distribution existed for FDCS and HS; however, IDCS predominantly affected males (6:1). The most common sites of presentation were abdomen and pelvis (42%), extremities (33%) and head and neck (57%) for FDCS, HS and IDCS, respectively. At diagnosis, 74%, 40% and 86% of patients presented with localised disease in FDCS, HS and IDCS, respectively. Patients with localised disease had significantly improved OS than those with metastatic disease in FDCS (P = 0.04) and IDCS (P = 0.014) but not in HS (P = 0.95). In FDCS and HS, adjuvant or neo-adjuvant therapy was not associated with improved OS compared with observation. In IDCS, surgery alone provided a 5-year overall survival rate of 71%. Conclusions Adjuvant or neo-adjuvant treatment in FDCS and HS did not affect OS. Patients with IDCS had an excellent outcome with surgery. In the metastatic setting, chemotherapy and small molecule inhibitors may provide benefit. PMID:26298731

  13. Identifying actionable variants using next generation sequencing in patients with a historical diagnosis of undifferentiated pleomorphic sarcoma.

    Science.gov (United States)

    Lewin, Jeremy; Garg, Swati; Lau, Beatrice Y; Dickson, Brendan C; Traub, Frank; Gokgoz, Nalan; Griffin, Anthony M; Ferguson, Peter C; Andrulis, Irene L; Sim, Hao-Wen; Kamel-Reid, Suzanne; Stockley, Tracy L; Siu, Lillian L; Wunder, Jay S; Razak, Albiruni R A

    2017-09-10

    There are limited data regarding the molecular characterization of undifferentiated pleomorphic sarcomas (UPS; formerly malignant fibrous histiocytoma). This study aimed to investigate the utility of next generation sequencing (NGS) in UPS to identify subsets of patients who harbour actionable mutations. Patients diagnosed with UPS underwent pathological re-evaluation by a pathologist specializing in sarcoma. Tumor DNA was isolated from archived fresh frozen tissue samples and genotyped using NGS with the Illumina MiSeq TruSeq Amplicon Cancer Panel (48 genes, 212 amplicons). In total, 95 patients initially classified with UPS were identified. Following pathology re-review the histological subtypes were reclassified to include: Myxofibrosarcoma (MFS, N = 44); UPS(N = 18); and Others (N = 27; including undifferentiated spindle cell sarcoma (N = 15) and dedifferentiated liposarcoma (N = 6)). Seven cases were excluded from further analysis for other reasons. Baseline demographics of the finalized cohort (N = 88) showed a median age of 66 years (32-95), primarily with stage I-III disease (92%) and high-grade (86%) lesions. Somatic mutations were identified in 31 cases (35%)(Total mutations = 36: solitary mutation(n = 27); two mutations( =n = 3); three mutations(n = 1)). The most commonly identified mutations were in TP53 (n = 24), ATM (n = 3) and PIK3CA (n = 2). Three of 43 patients with MFS and one of 18 patients with UPS had clinically relevant mutations, mainly related to biomarkers of prediction of response; however few had targetable driver mutations. Somatic mutation status did not influence disease free or overall survival. Based on the small number of clinically relevant mutations, these data do not support the routine use of targeted NGS panels outside of research protocols in UPS. © 2017 UICC.

  14. 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.)

  15. 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

  16. Kaposi’s Sarcoma of the Rectum in a Homosexual Male with HIV-AIDS

    Science.gov (United States)

    Nautsch, Deborah

    2016-01-01

    Visceral involvement in AIDS-related Kaposi’s sarcoma is common, but it is rarely seen in the absence of cutaneous disease. Most patients with gastrointestinal tract Kaposi’s sarcoma remain asymptomatic and are often diagnosed on endoscopy or autopsy. We report a case of a 24-year-old homosexual man who presented with rectal pain and bleeding and was found to have skin-sparing, disseminated Kaposi’s sarcoma with rectum, liver, lungs, and lymph node involvement. Despite treatment with highly active anti-retroviral therapy, he developed multiorgan failure resulting in death. PMID:28119943

  17. Xenograft and genetically engineered mouse model systems of osteosarcoma and Ewing's sarcoma: tumor models for cancer drug discovery

    Science.gov (United States)

    Sampson, Valerie B; Kamara, Davida F; Kolb, E Anders

    2014-01-01

    Introduction There are > 75 histological types of solid tumors that are classified into two major groups: bone and soft-tissue sarcomas. These diseases are more prevalent in children, and pediatric sarcomas tend to be highly aggressive and rapidly progressive. Sarcomas in adults may follow a more indolent course, but aggressive tumors are also common. Sarcomas that are metastatic at diagnosis, or recurrent following therapy, remain refractory to current treatment options with dismal overall survival rates. A major focus of clinical trials, for patients with sarcoma, is to identify novel and more effective therapeutic strategies targeted to genomic or proteomic aberrations specific to the malignant cells. Critical to the understanding of the potential for targeted therapies are models of disease that are representative of clinical disease and predictive of relevant clinical responses. Areas covered In this article, the authors discuss the use of mouse xenograft models and genetically engineered mice in cancer drug discovery. The authors provide a special focus on models for the two most common bone sarcomas: osteosarcoma (OS) and Ewing's sarcoma (ES). Expert opinion Predicting whether a new anticancer agent will have a positive therapeutic index in patients with OS and ES remains a challenge. The use of mouse sarcoma models for understanding the mechanisms involved in the response of tumors to new treatments is an important step in the process of drug discovery and the development of clinically relevant therapeutic strategies for these diseases. PMID:23844615

  18. Plantar pressures in diabetic patients with foot ulcers which have remained healed

    NARCIS (Netherlands)

    T.M. Owings; J. Apelqvist; A. Stenstrom; M. Becker; S.A. Bus; A. Kalpen; J.S. Ulbrecht; P.R. Cavanagh

    2009-01-01

    Aims The recurrence of foot ulcers is a significant problem in people with diabetic neuropathy. The purpose of this study was to measure in-shoe plantar pressures and other characteristics in a group of neuropathic patients with diabetes who had prior foot ulcers which had remained healed. Methods T

  19. High prevalence of antibodies to human herpesvirus 8 in relatives of patients with classic Kaposi's sarcoma from Sardinia

    OpenAIRE

    Angeloni, Antonio; Heston, Lee; Uccini, Stefania; Sirianni, Maria Caterina; Cottoni, Francesca Maria Giovanna; Masala, Maria Vittoria; Cerimele, Decio; Lin, Su-Fang; Sun, Ren; Rigsby, Michael; Faggioni, Alberto; Miller, George

    1998-01-01

    A survey for antibodies to a recombinant small viral capsid antigen (sVCA) of human herpesvirus type 8 (HHV‐8) was conducted in Sardinia, one of the world's highest incidence areas for classic Kaposi's sarcoma (KS). Prevalence of antibodies to HHV‐8 sVCA was greatest in patients with KS (95%), followed by family members (39%) and a Sardinian control population age‐ and sex‐matched to the relatives (11%). Within families, prevalence of antibodies was about equal among spouses, children, and si...

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

    Energy Technology Data Exchange (ETDEWEB)

    Tagliafico, Alberto [University of Genoa, Institute of Anatomy, Department of Experimental Medicine (DIMES), Genoa (Italy); IRCCS AOU San Martino-IST, Department of Radiology, Genoa (Italy); University of Genoa, Department of Experimental Medicine -DIMES, Genoa (Italy); Truini, Mauro; Spina, Bruno [Istituto di Ricovero e Cura a Carattere Scientitifico Azienda Ospedaliera Universitaria San Martino-Istituto Nazionale per la Ricerca sul Cancro (IRCCS AOU San Martino-IST), Department of Pathology, Genova (Italy); Cambiaso, Paolo; Zaottini, Federico [School of Medicine, Genova (Italy); Bignotti, Bianca; Derchi, Lorenzo E.; Martinoli, Carlo [University of Genoa, Department of Health Sciences (DISSAL), Genoa (Italy); Calabrese, Massimo [IRCCS AOU San Martino-IST, Department of Radiology, Genoa (Italy)

    2015-09-15

    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.)

  1. Advances and controversies in the management of soft tissue sarcomas.

    Science.gov (United States)

    Demetri, George D; Blay, Jean-Yves; Casali, Paolo G

    2017-01-01

    Intensive clinical research in the sarcoma field has provided insight into the histopathological diversity of soft tissue sarcomas (STS) and led to the introduction of many new agents that promise to play an important role in the management of patients with STS. While an increasing body of scientific data has advanced our knowledge of this complex family of mesenchymal diseases, several controversies remain to be resolved: Is doxorubicin-based therapy still the definitive standard first-line treatment for all patients with unresectable and/or metastatic STS of all subtypes? Is histology-driven therapy beyond gastrointestinal stromal tumors a reality or are we pursuing an unachievable objective? Are we making practical headway in the establishment of sarcoma reference centers? Is it clearly established which is the best parameter to evaluate the efficacy of a new agent in STS?

  2. Management of Bone Sarcoma.

    Science.gov (United States)

    Gutowski, Christina J; Basu-Mallick, Atrayee; Abraham, John A

    2016-10-01

    Treatment of bone sarcoma requires careful planning and involvement of an experienced multidisciplinary team. Significant advancements in systemic therapy, radiation, and surgery in recent years have contributed to improved functional and survival outcomes for patients with these difficult tumors, and emerging technologies hold promise for further advancement. Copyright © 2016 Elsevier Inc. All rights reserved.

  3. The Epidemiology of Sarcoma

    OpenAIRE

    Burningham Zachary; Hashibe Mia; Spector Logan; Schiffman Joshua D

    2012-01-01

    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, a...

  4. Rehabilitation considerations for a patient with external hemipelvectomy and hemisacrectomy for recurrent soft tissue pelvic sarcoma: a case report.

    Science.gov (United States)

    Schaal Wilson, Rachel E

    2015-01-01

    External hemipelvectomy (EHP) is an uncommon procedure involving surgical amputation of the lower extremity and hemipelvis. Soft tissue sarcomas of the pelvis are rare and accompanied by a high risk of disease progression after surgical intervention. The purpose of this case report is to describe the rehabilitation considerations and importance of interdisciplinary collaboration to achieve functional goals to allow discharge with improved independence and decreased caregiver burden. A 58-year-old Caucasian male patient underwent a hemipelvectomy and hemisacrectomy for treatment of a recurrent pelvic soft tissue sarcoma following 4 months of bed confinement secondary to pain and weakness of the involved lower extremity. This report outlines the physical therapy interventions during 3 weeks of inpatient rehabilitation. This case demonstrates that continuous interdisciplinary care of a complex patient with EHP and hemisacrectomy contributes to significant improvement in functional mobility exceeding prior level of function. This case is particularly interesting because in addition to having a rare diagnosis and undergoing an uncommon surgical procedure, this patient was confined to bed for 4 months prior to surgical intervention, and thus began rehabilitation with a baseline of limited functional mobility and high caregiver burden.

  5. Stereotactic Body Radiotherapy for Pulmonary Metastases From Soft-Tissue Sarcomas: Excellent Local Lesion Control and Improved Patient Survival

    Energy Technology Data Exchange (ETDEWEB)

    Dhakal, Sughosh [Department of Radiation Oncology, University of Rochester, Rochester, NY (United States); Corbin, Kimberly S. [Department of Radiation and Cellular Oncology, University of Chicago, Chicago, IL (United States); Milano, Michael T.; Philip, Abraham [Department of Radiation Oncology, University of Rochester, Rochester, NY (United States); Sahasrabudhe, Deepak [Department of Medicine, Division of Hematology and Oncology, University of Rochester, Rochester, NY (United States); Jones, Carolyn [Department of Surgery, Division of Thoracic/Foregut, University of Rochester, Rochester, NY (United States); Constine, Louis S., E-mail: louis_constine@urmc.rochester.edu [Department of Radiation Oncology, University of Rochester, Rochester, NY (United States)

    2012-02-01

    Purpose: Patients with pulmonary metastases (PM) from soft-tissue sarcomas (STS) have historically been treated with surgery and/or chemotherapy. Since 2001, we have treated PM with stereotactic body radiation therapy (SBRT). We postulated that SBRT for PM from STS would yield excellent local control (LC) and overall survival (OS). Methods and Materials: Fifty-two patients with PM from STS, diagnosed between 1990 and 2006 at University of Rochester, were retrospectively reviewed. Most patients received multimodality treatment comprising of surgery, chemotherapy, and/or radiation. SBRT used the Novalis ExacTrac patient positioning platform, vacuum bag immobilization, and relaxed end-expiratory breath hold techniques. Results: Leiomyosarcoma (23%), malignant fibrous histiocytoma (19%), and synovial sarcoma (15%) were the most common histologies. Forty-eight percent initially presented with PM, whereas 52% developed PM at a median of 0.7 (0.3-7.3) years after initial diagnosis. Median follow-up from diagnosis of PM was 0.9 (0.3-7.3) years. Fifteen patients underwent SBRT to 74 lesions. Median number of lesions treated was 4 (1-16) per patient and 3.5 (1-6) per session. Preferred dose and fractionation was 50 Gy in 5 Gy fractions. Three-year LC was 82%. No patients experienced Grade {>=}3 toxicity. Median OS was 2.1 (0.8-11.5) years for patients treated with SBRT, and 0.6 (0.1-7.8) years for those who never received SBRT (p = 0.002). Conclusions: SBRT provides excellent LC of PM and may extend OS. SBRT should be considered for all patients with PM from STS, particularly those who are not surgical candidates. Further investigation is warranted to establish criteria for the use of SBRT for STS patients with PM.

  6. Acute Pulmonary Artery Obstruction as the Primary Manifestation of a Rapidly Growing Intimal Sarcoma in a 54-Year-Old Patient.

    Science.gov (United States)

    Westhofen, Sumi; Kugler, Christian; Reichenspurner, Hermann; Deuse, Tobias

    2016-12-01

    Pulmonary artery sarcoma is a rare malignant neoplasm that is often misdiagnosed and most often only recognized postmortem during the autopsy. We present the case of a male patient with a rapidly progressive pulmonary tumor who underwent urgent pneumonectomy for increasing symptoms of chest pain and septic clinical picture. Histological analysis revealed the diagnosis of a pulmonary artery sarcoma. Despite an R1-resection and adjuvant chemotherapy, the patient is in good clinical health and free of tumor relapse 1 year after the surgery.

  7. Human herpes virus-8 DNA in bronchoalveolar lavage samples from patients with AIDS-associated pulmonary Kaposi's sarcoma

    DEFF Research Database (Denmark)

    Benfield, T L; Dodt, K K; Lundgren, Jens Dilling

    1997-01-01

    Kaposi's sarcoma (KS) is the most frequent AIDS-associated neoplasm, and often disseminates to visceral organs, including the lungs. An ante-mortem diagnosis of pulmonary KS is difficult. Recently, DNA sequences resembling a new human herpes virus (HHV-8), have been identified in various forms...... of KS. We hypothesized that these sequences are present in samples obtained by bronchoalveolar lavage (BAL) in patients with pulmonary KS. Utilizing a nested polymerase chain reaction (PCR), 7/12 BAL cell samples from HIV-infected patients with endobronchial KS were positive for HHV-8 DNA. In contrast......, only 2/39 samples from HIV-infected patients without evidence of KS were positive (p = 0.007). Detection of HHV-8 in BAL cells of patients with pulmonary KS was highly specific (95%), with a sensitivity of 58% and a positive predictive value of 78%. In conclusion, HHV-8 is associated with pulmonary KS...

  8. Cystic synovial sarcomas: imaging features with clinical and histopathologic correlation

    Energy Technology Data Exchange (ETDEWEB)

    Nakanishi, Hirofumi; Araki, Nobuhito [Department of Orthopedic Surgery, Osaka Medical Center for Cancer and Cardiovascular Diseases, 1-3-3, Nakamichi, Higashinari-Ku, 537-8511, Osaka (Japan); Sawai, Yuka [Department of Radiology, Osaka Medical Center for Cancer and Cardiovascular Diseases, Osaka (Japan); Kudawara, Ikuo [Department of Orthopedic Surgery, Osaka National Hospital, Osaka (Japan); Mano, Masayuki; Ishiguro, Shingo [Department of Pathology, Osaka Medical Center for Cancer and Cardiovascular Diseases, Osaka (Japan); Ueda, Takafumi; Yoshikawa, Hideki [Department of Orthopedic Surgery, Osaka University Graduate School of Medicine, Suita, Osaka (Japan)

    2003-12-01

    To characterize the radiological and clinicopathologic features of cystic synovial sarcoma. Seven patients with primary cystic synovial sarcoma were evaluated. Computed tomography (CT) and magnetic resonance (MR) imaging were undertaken at the first presentation. The diagnosis of synovial sarcoma was made on the basis of histological examinations followed by molecular analysis. Radiological and clinicopathologic findings were reviewed. CT showed well-defined soft tissue mass without cortical bone erosion and invasion. Calcification was seen at the periphery of the mass in three cases. T2-weighted MR images showed multilocular inhomogeneous intensity mass in all cases, five of which showed fluid-fluid levels. On gross appearance, old and/or fresh hematomas were detected in six cases. In the one remaining case, microscopic hemorrhage in the cystic lumen was proven. Four cases had poorly differentiated areas. In five cases prominent hemangiopericytomatous vasculature was observed. Histologic grade was intermediate in one tumor and high in six. One case had a history of misdiagnosis for tarsal tunnel syndrome, one for lymphadenopathy, two for sciatica and two for hematoma. All cystic synovial sarcomas demonstrated multilocularity with well-circumscribed walls and internal septae. Synovial sarcoma should be taken into consideration in patients with deeply situated multicystic mass with triple signal intensity on T2-weighted MR imaging. (orig.)

  9. 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.

  10. Chylothorax in a patient with metastatic Kaposi sarcoma: Differential diagnostic considerations

    Directory of Open Access Journals (Sweden)

    Ryan Alexander, DO

    2015-01-01

    Full Text Available Kaposi sarcoma (KS is a low-grade mesenchymal tumor involving blood and lymphatic vessels. There are four types, based on clinical presentation: classic, endemic (Africana, iatrogenic (typically, involving renal allograft recipients, and AIDS-associated (epidemic. Kaposi's sarcoma-associated herpes virus infection has been linked along with other factors to the development of KS. The Kaposi's sarcoma-associated herpes virus interacts and encodes for numerous molecular proteins that play a role in the pathogenesis of KS, including latency-associated nuclear antigen, viral G protein-coupled receptor, viral FLICE inhibitory protein, and viral IL-6. KS primarily affects the skin and causes disseminated disease in a variety of organs. Involvement of visceral organs other than the lining of the alimentary tract is extremely rare. While the chylous pleural effusions of KS may resemble other pulmonary diseases (including lymphangioma, lymphangectasis, and lymphangioleiomyomatosis with chylous effusions at thoracic CT, differentiating features may allow for more prompt diagnosis and treatment. The presumptive diagnosis of AIDS-related pulmonary KS is often clinical. A tissue diagnosis is not required to establish the diagnosis of pulmonary KS. There are a variety of causes of chylothorax. The primary finding is a near-water-attenuating pleural effusion. The secondary findings of chylothorax can help differentiate the etiology.

  11. Multimodality Local Therapy for Retroperitoneal Sarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Paryani, Nitesh N.; Zlotecki, Robert A.; Swanson, Erika L.; Morris, Christopher G. [Department of Radiation Oncology, University of Florida College of Medicine, Gainesville, FL (United States); Grobmyer, Stephen R.; Hochwald, Steven N. [Department of General Surgery, University of Florida College of Medicine, Gainesville, FL (United States); Marcus, Robert B. [Department of Radiation Oncology, University of Florida College of Medicine, Gainesville, FL (United States); University of Florida Proton Therapy Institute, Jacksonville, FL (United States); Indelicato, Daniel J., E-mail: dindelicato@floridaproton.org [Department of Radiation Oncology, University of Florida College of Medicine, Gainesville, FL (United States); University of Florida Proton Therapy Institute, Jacksonville, FL (United States)

    2012-03-01

    Purpose: Soft-tissue sarcomas of the retroperitoneum are rare tumors comprising less than 1% of all malignancies. Although surgery continues as the mainstay of treatment, the large size of these tumors coupled with their proximity to critical structures make resection with wide margins difficult to achieve. The role and timing of radiotherapy are controversial. This study updates our institutional experience using multimodality local therapy for resectable retroperitoneal sarcoma and identifies prognostic factors impacting disease control and survival. Methods and Materials: Between 1974 and 2007, 58 patients with nonmetastatic retroperitoneal sarcoma were treated with surgery and radiation at University of Florida. The median age at radiotherapy was 57 years old (range, 18-80 years). Forty-two patients received preoperative radiotherapy and 16 received postoperative radiotherapy. Nineteen patients received 1.8 Gy once daily and 39 patients received 1.2 Gy twice daily. Variables analyzed for prognostic value included age, grade, kidney involvement, histology, de novo versus recurrent presentation, tumor diameter, margin status, radiotherapy sequencing (preoperative vs. postoperative), total radiation dose, fractionation scheme, and treatment era. Results: The 5-year overall survival, cause-specific survival, and local control rates were 49%, 58%, and 62%, respectively. Nearly two-thirds of disease failures involved a component of local progression. On multivariate analysis, only margin status was significantly associated with improved 5-year local control (85%, negative margins; 63%, microscopic positive margins; 0%, gross positive margins; p < 0.0001) and 5-year overall survival (64%, negative margins; 56%, microscopic positive margins; 13%, gross positive margins; p = 0.0012). Thirty-one Grade 3 or greater toxicities were observed in 22 patients, including two treatment-related deaths (3%). Conclusion: For retroperitoneal sarcoma, local control remains a

  12. Sarcoma Immunotherapy: Past Approaches and Future Directions

    Directory of Open Access Journals (Sweden)

    S. P. D'Angelo

    2014-01-01

    Full Text Available Sarcomas are heterogeneous malignant tumors of mesenchymal origin characterized by more than 100 distinct subtypes. Unfortunately, 25–50% of patients treated with initial curative intent will develop metastatic disease. In the metastatic setting, chemotherapy rarely leads to complete and durable responses; therefore, there is a dire need for more effective therapies. Exploring immunotherapeutic strategies may be warranted. In the past, agents that stimulate the immune system such as interferon and interleukin-2 have been explored and there has been evidence of some clinical activity in selected patients. In addition, many cancer vaccines have been explored with suggestion of benefit in some patients. Building on the advancements made in other solid tumors as well as a better understanding of cancer immunology provides hope for the development of new and exciting therapies in the treatment of sarcoma. There remains promise with immunologic checkpoint blockade antibodies. Further, building on the success of autologous cell transfer in hematologic malignancies, designing chimeric antigen receptors that target antigens that are over-expressed in sarcoma provides a great deal of optimism. Exploring these avenues has the potential to make immunotherapy a real therapeutic option in this orphan disease.

  13. Microenvironmental targets in sarcoma

    Directory of Open Access Journals (Sweden)

    Monika eEhnman

    2015-11-01

    Full Text Available Sarcomas are rare malignant tumors affecting all age groups. They are typically classified according to their resemblance to corresponding normal tissue. Their heterogeneous features, for example in terms of disease-driving genetic aberrations and body location, complicate both disease classification and development of novel treatment regimens. Many years of failure of improved patient outcome in clinical trials has lead to the conclusion that novel targeted therapies are likely needed in combination with current multimodality regimens. Sarcomas have not, in contrast to the common carcinomas, been the subject for larger systematic studies on how tumor behavior relates to characteristics of the tumor microenvironment. There is consequently an urgent need for identifying suitable molecular targets, not only in tumor cells, but also in the tumor microenvironment. This review discusses preclinical and clinical data about potential molecular targets in sarcomas. Studies on targeted therapies involving the tumor microenvironment are prioritized. A greater understanding of the biological context is expected to facilitate more successful design of future clinical trials in sarcoma.

  14. Levels of activated platelet-derived microvesicles in patients with soft tissue sarcoma correlate with an increased risk of venous thromboembolism.

    Science.gov (United States)

    Fricke, A; Ullrich, P V; Cimniak, A F V; Becherer, C; Follo, M; Heinz, J; Scholber, J; Herget, G W; Hauschild, O; Wittel, U A; Stark, G B; Bannasch, H; Braig, D; Eisenhardt, S U

    2017-08-07

    Microvesicles are small vesicles expressing specific antigens from their cells of origin. Elevated levels of microvesicles have been shown to be associated with coagulation disorders as well as with different types of malignancies. This study aims to evaluate a possible correlation of different microvesicle subpopulations with a positive history of venous thromboembolism (VTE) in patients with soft tissue sarcoma. Annexin V - positive microvesicles, leukocyte (CD45-positive), platelet (CD61-positive), activated platelet (CD62P-, CD63-positive), endothelium-derived (CD62E-positive) and tissue-factor (CD142-positive) microvesicles were identified in the peripheral blood of patients with soft tissue sarcoma (n = 39) and healthy controls (n = 17) using fluorescence-activated cell sorting (FACS). Both the total amount of Annexin V-positive microvesicles and levels of endothelium-derived (CD62E-positive) microvesicles were shown to decrease significantly after tumor resection (n = 18, p = 0.0395 and p = 0.0109, respectively). Furthermore, the total amount of Annexin V - positive microvesicles as well as leukocyte (CD45-positive) and endothelium-derived (CD62E-positive) microvesicles were significantly higher in patients with grade 3 (G3) soft tissue sarcoma (n = 9) compared to healthy controls (n = 17) (p = 0.0304, p = 0.0254 and p = 0.0357, respectively). Moreover, patients with G3 soft tissue sarcoma (n = 9) presented higher levels of Annexin V-positive and endothelium-derived (CD62E-positive) microvesicles compared to patients with grade 2 (G2) soft tissue sarcoma (n = 8) (p = 0.0483 and p = 0.0045). Patients with grade 1 (G1) soft tissue sarcoma (n = 3) presented with significantly lower levels of platelet (CD61-positive) microvesicles than patients with G3 soft tissue sarcoma (n = 9) (p = 0.0150). In patients with a positive history of VTE (n = 11), significantly higher levels of activated platelet (CD62P- and CD63-positive

  15. Isolated limb perfusion with tumor necrosis factor and melphalan for limb salvage in 186 patients with locally advanced soft tissue extremity sarcomas. The cumulative multicenter European experience

    NARCIS (Netherlands)

    A.M.M. Eggermont (Alexander); P.M. Schlag; D. Lienard; A.N. van Geel (Albert); F.J. Lejeune; H.J. Hoekstra; I. Meller; J.C. Pector; O.E. Nieweg (Omgo); G. Ben-Ari; C. Kettelhack; H. Schraffordt Koops; J.M. Klausner; B.B. Kroon

    1996-01-01

    textabstractOBJECTIVE: The objective of the study was to achieve limb salvage in patients with locally advanced soft tissue sarcomas that can only be treated by amputation or functionally mutilating surgery by performing an isolated limb perfusion (ILP) with tumor necro

  16. High prevalence of antibodies to human herpesvirus 8 in relatives of patients with classic Kaposi's sarcoma from Sardinia.

    Science.gov (United States)

    Angeloni, A; Heston, L; Uccini, S; Sirianni, M C; Cottoni, F; Masala, M V; Cerimele, D; Lin, S F; Sun, R; Rigsby, M; Faggioni, A; Miller, G

    1998-06-01

    A survey for antibodies to a recombinant small viral capsid antigen (sVCA) of human herpesvirus type 8 (HHV-8) was conducted in Sardinia, one of the world's highest incidence areas for classic Kaposi's sarcoma (KS). Prevalence of antibodies to HHV-8 sVCA was greatest in patients with KS (95%), followed by family members (39%) and a Sardinian control population age- and sex-matched to the relatives (11%). Within families, prevalence of antibodies was about equal among spouses, children, and siblings of KS patients, a finding that raises the possibilities of intrafamilial person-to-person or vertical transmission. Antibodies were detected 2-3 times more frequently in males than in females. The data show that prevalence of antibodies to HHV-8 sVCA correlates with the distribution of classic KS in a high- incidence area. Clustering of seroprevalence within some families suggests the presence of familial risk factors for active HHV-8 infection.

  17. 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

    potentially preserve fertility. When the patient has been cured and primary ovarian insufficiency has developed, transplantation of frozen/thawed ovarian tissue can restore ovarian function. The tissue is usually collected before chemotherapy is initiated, and malignant cells may contaminate the stored......Ewing's sarcoma (EWS) is a highly malignant cancer in children, adolescents and young adults. The chemotherapy required to treat female EWS patients may cause primary ovarian insufficiency and infertility as a side effect. Cryopreservation of ovarian tissue before the start of chemotherapy can...... 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...

  18. Myeloid Sarcoma in the Orbit.

    Science.gov (United States)

    Qian, Xiaoxiao; Gigantelli, James W; Abromowitch, Minnie; Morgan, Linda A; Suh, Donny W

    2016-12-08

    The authors describe a case of myeloid sarcoma of the orbit in a pediatric patient. An 8-month-old male infant presented to the ophthalmology clinic with a left orbital mass, which had been increasing in size over the previous 2 months. The mass was initially diagnosed at another clinic as an infantile hemangioma, and had been treated with a topical formulation of timolol. In the ophthalmology clinic, orbital magnetic resonance imaging showed a solid enhancing mass. A biopsy was performed, and histopathology revealed myeloid sarcoma. The disease responded well to a standard chemotherapy regimen. Myeloid sarcoma is a rare, extra-medullary presentation that can occur as an isolated tumor, concurrently with or at relapse of acute myeloid leukemia. Because few cases of myeloid sarcoma in the orbit have been reported, this case report aids in the management of myeloid sarcoma in pediatric patients. The report describes an 8-month-old male infant, the youngest patient to develop myeloid sarcoma without preexisting acute myeloid leukemia. [J Pediatr Ophthalmol Strabismus. 2016;53:e64-e68.].

  19. Latency-associated nuclear antigen of Kaposi sarcoma-associated herpesvirus promotes angiogenesis through targeting notch signaling effector Hey1.

    Science.gov (United States)

    Wang, Xing; He, Zhiheng; Xia, Tian; Li, Xiaofan; Liang, Deguang; Lin, Xianzhi; Wen, Hao; Lan, Ke

    2014-04-01

    Notch signaling has been implicated in the pathogenesis of Kaposi sarcoma. Kaposi sarcoma is an angioproliferative neoplasm that originates from Kaposi sarcoma-associated herpesvirus (KSHV) infection. Previously, we showed that the KSHV LANA protein can stabilize intracellular Notch in KSHV-infected tumor cells and promote cell proliferation. However, whether Notch signaling functions in pathologic angiogenesis of Kaposi sarcoma remains largely unknown. Hey1, an essential downstream effector of the Notch signaling pathway, has been demonstrated to play a fundamental role in vascular development. In the present study, we performed whole transcriptome, paired-end sequencing on three patient-matched clinical Kaposi sarcoma specimens and their corresponding adjacent stroma samples, with an average depth of 42 million reads per sample. Dll4, Hey1, and HeyL displayed significant upregulation in Kaposi sarcoma. Further verification based on immunohistochemistry analysis demonstrated that Hey1 was indeed highly expressed in Kaposi sarcoma lesions. Using the Matrigel plug assay, we showed that downregulation of Hey1 and γ-secretase inhibitor treatment caused dramatic reduction in the formation of new blood vessels in mice. Interestingly, LANA was responsible for the elevated level of Hey1 through inhibition of its degradation. Importantly, Hey1 stabilized by LANA promoted the neoplastic vasculature. Taken together, our data suggest that hijacking of the proangiogenic property of Hey1 by LANA is an important strategy utilized by KSHV to achieve pathologic angiogenesis and that Hey1 is a potential therapeutic target in Kaposi sarcoma.

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

    Energy Technology Data Exchange (ETDEWEB)

    Sadri, Navid; Zhang, Paul J., E-mail: pjz@mail.med.upenn.edu [Anatomic Pathology, Department of Pathology and Laboratory Medicine, Hospital of the University of Pennsylvania, 3400 Spruce Street, 6th Floor Founders Building, Philadelphia, PA 19104 (United States)

    2013-04-02

    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.

  1. Acquired hemophagocytic syndrome in a patient with synovial sarcoma: a case report

    Science.gov (United States)

    Ciccarese, Chiara; Ferrara, Roberto; Fantinel, Emanuela; Zecchetto, Camilla; Simionato, Francesca; Grego, Elisabetta; Ortolani, Silvia; Caccese, Mario; Bimbatti, Davide; Cingarlini, Sara; Brunelli, Matteo; Andreini, Angelo; Tortora, Giampaolo; Massari, Francesco

    2015-01-01

    Hemophagocytic lymphohistiocytosis (HLH) is a syndrome characterized by severe hyperinflammation due to an overwhelming ineffective immune response to different triggers. Most important symptoms are fever, hepatosplenomegaly and cytopenias. Biochemical signs include elevated ferritin, hypertriglyceridemia and low fibrinogen. Hemophagocytosis in the bone marrow is a hallmark of this syndrome. Based on the pathogenetic mechanism, it can be classified into primary (inherited) or secondary (acquired) HLH. We report, to our knowledge, the first case of acquired hemophagocytic syndrome that arose in a 20-year-old man affected by synovial sarcoma as a complication during chemotherapy. PMID:28031902

  2. Kaposi sarcoma in a fingolimod-treated patient with multiple sclerosis.

    Science.gov (United States)

    Walker, Susan; Brew, Bruce

    2016-09-01

    Kaposi sarcoma (KS) is a vascular tumour of endothelial cell origin, associated with human herpes virus 8. It develops in one of four clinical settings, one of which is iatrogenic immunosuppression. We present the case of a 46year-old man with relapsing-remitting multiple sclerosis who developed KS in the context of fingolimod use. To our knowledge, this is the second reported case of KS in a fingolimod-treated individual. This case highlights potential risks associated with immunosuppression with this medicine and ongoing need for vigilance in assessing for such complications.

  3. Epithelioid sarcoma : Still an only surgically curable disease

    NARCIS (Netherlands)

    de Visscher, Sebastiaan A. H. J.; van Ginkel, Robbert J.; Wobbes, Theo; Veth, Rene P. H.; ten Heuvel, Suzanne E.; Suurmeijer, Albert J. H.; Hoekstra, Harad J.

    2006-01-01

    BACKGROUND. Epithelioid sarcoma is a rare soft tissue sarcoma with a known high propensity for locoregional recurrence and distant metastases. The clinical behavior and prognostic factors that influence the survival of patients with epithelioid sarcoma were studied. METHODS. Twenty-three patients,

  4. Potential Therapeutic Targets in Uterine Sarcomas

    OpenAIRE

    2015-01-01

    Uterine sarcomas are rare tumors accounting for 3,4% of all uterine cancers. Even after radical hysterectomy, most patients relapse or present with distant metastases. The very limited clinical benefit of adjuvant cytotoxic treatments is reflected by high mortality rates, emphasizing the need for new treatment strategies. This review summarizes rising potential targets in four distinct subtypes of uterine sarcomas: leiomyosarcoma, low-grade and high-grade endometrial stromal sarcoma, and undi...

  5. The Cancer-Associated Virus Landscape in HIV Patients with Oral Hairy Leukoplakia, Kaposi's Sarcoma, and Non-Hodgkin Lymphoma

    Directory of Open Access Journals (Sweden)

    Peter D. Burbelo

    2012-01-01

    Full Text Available Although HIV-positive patients are at higher risk for developing a variety of infection-related cancers, the prevalence of infections with the seven known cancer-associated viruses has not been studied. Luciferase immunoprecipitation systems were used to evaluate antiviral antibodies in four 23-person groups: healthy blood donors and HIV-infected patients with oral hairy leukoplakia (OLP, Kaposi's sarcoma (KS, or non-Hodgkin lymphoma (NHL. Antibody profiling revealed that all HIV-positive individuals were strongly seropositive for anti-gp41 and antireverse transcriptase antibodies. However, anti-p24 HIV antibody levels were highly variable and some OLP and KS patients demonstrated weak or negative responses. Profiling two EBV antigens revealed no statistical difference in antibody levels among the three HIV-infected groups. A high frequency of KSHV infection was detected in HIV patients including 100% of KS, 78% of OLP, and 57% of NHL patients. Most HIV-infected subjects (84% showed anti-HBV core antibodies, but only a few showed antibodies against HCV. MCV seropositivity was also common (94% in the HIV-infected individuals and KS patients showed statistically higher antibody levels compared to the OLP and NHL patients. Overall, 68% of the HIV-infected patients showed seropositivity with at least four cancer-associated viruses. Antibody profiles against these and other infectious agents could be useful for enhancing the clinical management of HIV patients.

  6. BEZ235 (PIK3/mTOR inhibitor Overcomes Pazopanib Resistance in Patient-Derived Refractory Soft Tissue Sarcoma Cells

    Directory of Open Access Journals (Sweden)

    Hee Kyung Kim

    2016-06-01

    Full Text Available BACKGROUND: Although pazopanib treatment has become the standard chemotherapy in salvage setting for metastatic sarcoma patients, most patients progress after pazopanib treatment in 4 to 6 months. After failure to pazopanib, patients have limited options for treatment. Therefore, subsequent therapy in patients who failed to pazopanib is urgently needed and the use of patient derived cells or patient derived tumors for accompanying testing with various pharmacological inhibitors could offer additional treatment options for these patients. METHODS: Patient derived tumor cells were collected from ascites at the time of progression to pazopanib and a 13-drug panel was tested for drug sensitivity. We confirmed the results using in vitro cell viability assay and immunoblot assay. We also performed the genomic profiling of PDX model. RESULTS: The growth of patient derived tumor cells was significantly reduced by exposure to 1.0 μM AZD2014 compared with control (control versus AZD2014, mean growth = 100.0% vs 16.04%, difference = 83.96%, 95% CI = 70.01% to 97.92%, P = .0435. Similarly, 1.0 μM BEZ235 profoundly inhibited tumor cell growth in vitro when compared to control (control versus BEZ235, mean growth = 100.0% vs 7.308%, difference = 92.69%, 95% CI = 78.87% to 106.5%, P < .0001. Despite the presence of CDK4 amplification in the patient-derived tumor cells, LEE011 did not considerably inhibit cell proliferation when compared with control (control vs LEE011, mean growth = 100.0% vs 80.23%, difference = 19.77%, 95% CI = 1.828% to 37.72%, P = .0377. The immunoblot analysis showed that BEZ235 treatment decreased pAKT, pmTOR and pERK whereas AZD2014 decreased only pmTOR. CONCLUSION: Taken together, upregulation of mTOR/AKT pathway in sarcoma patient derived cells was considerably inhibited by the treatment of AZD2014 and BEZ235 with downregulation of AKT pathway (greater extent for BEZ235. These molecules may be considered as treatment option in STS

  7. Incidental Benign Metastasizing Leiomyoma in a Patient with Bone Sarcoma: A Case Report

    Directory of Open Access Journals (Sweden)

    Zanndor Jacob del Real-Romo

    2014-01-01

    Full Text Available Background. The benign metastasizing leiomyoma is an exceptionally rare entity; it presents with ectopic leiomyoma nodules with a benign pattern. Symptoms vary according to the anatomic location. The diagnosis is histopathological, usually in patients with history of hysterectomy. Case Presentation. A 36-year-old female with 2-month history of left knee pain was diagnosed with bone fibrosarcoma. A CT scan showed pulmonary nodules. The patient started neoadjuvant chemotherapy. Conservative surgery of pelvic limb was achieved. A new CT scan reported pulmonary nodules that remained in relation to the previous CT. A nodule resection by thoracotomy and TOB (transoperative biopsy was performed. The final pathology report described benign proliferative lesions consistent with benign metastatic leiomyoma. Conclusions. Benign metastatic leiomyoma is a rare condition presenting with uterine and extrauterine nodules most commonly in the lung. The diagnosis is histopathological. The surgical procedure must be reserved for selected patients.

  8. Candida tropicalis arthritis of the elbow in a patient with Ewing’s sarcoma that successfully responded to itraconazole

    Directory of Open Access Journals (Sweden)

    Seung Youn Kim

    2011-09-01

    Full Text Available Fungal infections are rarely responsible for arthritis. Few cases of fungal arthritis have been reported, even in immunocompromised hosts susceptible to low-virulence organisms. Herein, the authors report the first case of Candida tropicalis arthritis in a child with a solid tumor. A 13-year-old boy with Ewing’s sarcoma developed arthritis in his elbow during the neutropenic period after chemotherapy. Despite treatment with broad-spectrum antibiotics, his condition did not improve and serial blood cultures failed to reveal any causative organisms. After surgical drainage, culture of the joint fluid revealed the presence of C. tropicalis . Itraconazole treatment was started and after 3 months of therapy, the patient completely recovered full elbow function.

  9. Front-line window therapy with cisplatin in patients with primary disseminated Ewing sarcoma: A study by the Associazione Italiana di Ematologia ed Oncologia Pediatrica and Italian Sarcoma Group.

    Science.gov (United States)

    Luksch, Roberto; Grignani, Giovanni; D'Angelo, Paolo; Prete, Arcangelo; Puma, Nadia; Podda, Marta; Casanova, Michela; Ferrari, Andrea; Morosi, Carlo; Fagioli, Franca; Aglietta, Massimo; Ferrari, Stefano; Picci, Piero; Massimino, Maura

    2017-06-14

    The aim was to assess the activity of cisplatin (CDDP) in Ewing sarcoma (ES). The study consisted of front-line window therapy with CDDP 120 mg/sqm every 3 weeks for two courses in children and young adults with primary disseminated ES. Response was assessed using the Response Evaluation Criteria in Solid Tumours criteria, and Simon's two-stage design was applied. Twelve consecutive patients were enrolled in stage 1. Only one objective response was observed. Since the target response rate was not achieved, accrual was stopped and CDDP as a single agent in ES was judged unworthy of further assessment. © 2017 Wiley Periodicals, Inc.

  10. Prognostic Factors and Patterns of Relapse in Ewing Sarcoma Patients Treated With Chemotherapy and R0 Resection

    Energy Technology Data Exchange (ETDEWEB)

    Pan, Hubert Y. [Department of Radiation Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States); Morani, Ajaykumar [Department of Radiology, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States); Wang, Wei-Lien [Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States); Hess, Kenneth R. [Department of Biostatistics, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States); Paulino, Arnold C. [Department of Radiation Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States); Ludwig, Joseph A. [Department of Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States); Lin, Patrick P. [Department of Orthopedic Surgery, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States); Daw, Najat C. [Department of Pediatrics, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States); Mahajan, Anita, E-mail: amahajan@mdanderson.org [Department of Radiation Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States)

    2015-06-01

    Purpose: To identify prognostic factors and patterns of relapse for patients with Ewing sarcoma who underwent chemotherapy and R0 resection without radiation therapy (RT). Methods and Materials: We reviewed the medical records of patients who underwent surgical resection at our institution between 2000 and 2013 for an initial diagnosis of Ewing sarcoma. The associations of demographic and clinical factors with local control (LC) and patient outcome were determined by Cox regression. Time to events was measured from the time of surgery. Survival curves were estimated by the Kaplan-Meier method and compared by the log-rank test. Results: A total of 66 patients (median age 19 years, range 4-55 years) met the study criteria. The median follow-up was 5.6 years for living patients. In 43 patients (65%) for whom imaging studies were available, the median tumor volume reduction was 73%, and at least partial response by Response Evaluation Criteria in Solid Tumors was achieved in 17 patients (40%). At 5 years, LC was 78%, progression-free survival (PFS) was 59%, and overall survival (OS) was 65%. Poor histologic response (necrosis ≤95%) was an independent predictor of LC (hazard ratio [HR] 6.8, P=.004), PFS (HR 5.2, P=.008), and OS (HR 5.0, P=.008). Metastasis on presentation was also an independent predictor of LC (HR 6.3, P=.011), PFS (HR 6.8, P=.002), and OS (HR 6.7, P=.002). Radiologic partial response was a predictor of PFS (HR 0.26, P=.012), and postchemotherapy tumor volume was associated with OS (HR 1.06, P=.015). All deaths were preceded by distant relapse. Of the 8 initial local-only relapses, 5 (63%) were soon followed by distant relapse. Predictors of poor postrecurrence survival were time to recurrence <1 year (HR 11.5, P=.002) and simultaneous local and distant relapse (HR 16.8, P=.001). Conclusions: Histologic and radiologic response to chemotherapy were independent predictors of outcome. Additional study is needed to determine the role of adjuvant

  11. 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.

  12. Clinicopathological study of patients with head and neck sarcomas Avaliação clínico-patológica de pacientes com sarcomas em cabeça e pescoço

    Directory of Open Access Journals (Sweden)

    Isabela Alves Pacheco

    2011-06-01

    Full Text Available Sarcomas are rare tumors, mainly stemming from the embryonic mesoderm, with a high grade of morbidity and mortality. OBJECTIVE: To carry out a retrospective study of head and neck sarcoma cases between 1999 and 2008 in three specialized centers in the city of Fortaleza. MATERIALS AND METHODS: Data collection was based on the charts of the patients in the study. For statistical analysis purposes we used the chi-square associations and the z test for proportions. RESULTS: We found records of 36 patients, and the most affected ones were adult brown males, in the age range between 20 and 59 years - mean age of 39.7. The man/woman ratio was 1.76:1. The most prevalent histological type was the rhabdomyosarcoma and their most common locations were the face and the neck. Most of the sample was made up of live patients without evidence of the disease in the last visit - 41.6% of the cases. The most common treatment modes were the combination of surgery + radiotherapy + chemotherapy and surgery + radiotherapy, with 27.8% of the cases each. CONCLUSION: Sarcomas have a great histological variability and may have numerous locations. Since these are rare and not well-known lesions, new epidemiological studies must be carried out in order to enhance our understanding of the disease.Sarcomas são tumores raros originados predominantemente do mesoderma embrionário e que apresentam elevado grau de morbidade e mortalidade. OBJETIVO: Realizar estudo retrospectivo dos casos de sarcomas em cabeça e pescoço no período de 1999 a 2008 em três centros especializados. MATERIAL E MÉTODO: A coleta de dados teve base nos prontuários dos pacientes estudados. Para análise estatística, foram utilizados os testes de associações qui-quadrado e o teste z para proporções. RESULTADO: Foram encontrados registros de 36 pacientes, sendo os indivíduos mais acometidos homens adultos, da raça parda, na faixa etária de 20 a 59 anos, com idade média de 39,7. A rela

  13. Iatrogenic colorectal Kaposi sarcoma complicating a refractory ulcerative colitis in a human immunodeficiency negative-virus patient.

    Science.gov (United States)

    Hamzaoui, Lamine; Kilani, Houda; Bouassida, Mahdi; Mahmoudi, Moufida; Chalbi, Emna; Siai, Karima; Ezzine, Heykel; Touinsi, Hassen; Azzouz, Mohamed M'saddak; Sassi, Sadok

    2013-01-01

    Kaposi sarcoma is a mesenchymal tumor associated to a human herpes virus-8. It often occurs in human immunodeficiency virus-positive subjects. Colorectal localization is rare. We report the case of a colorectal Kaposi sarcoma complicating a refractory ulcerative colitis treated with surgery after the failure of immunomodulator therapy in a human immunodeficiency virus-negative heterosexual man.

  14. Tracing partners of patients with syphilis infection remains challenging: experience of Geneva Hospital.

    Science.gov (United States)

    de Lorenzi, Caroline; Angèle, Gayet-Ageron; Martine, Girard-Strohbach; Laurence, Toutous Trellu

    2017-01-01

    Syphilis has been reinstated on the list of notifiable diseases in Switzerland since 2006 and the active management of sexual partners is encouraged to avoid reinfection. However, contact tracing has yielded unsatisfactory results and the incidence of syphilis remains important, especially in high-risk populations. The aim of this study was to compare the proportions of notified sexual partners of patients diagnosed with syphilis by the laboratories of Geneva University Hospitals (HUG) with those diagnosed in private laboratories (non-HUG) and to assess the risk factors for no notification to sexual partners. All syphilis cases notified to the Office of the Surgeon General in Geneva (Switzerland) between 1 January 2011 and 31 December 2013 were analysed. The proportions of partner notification (PN) between HUG and non-HUG laboratories were compared by Chi square test and the main risk factors for no notification to sexual partners were assessed by binomial log-linear regression. Among a total of 720 notifications reported, 244 cases were diagnosed with contagious syphilis stages and 263 with non-contagious stages (i.e. successfully treated patients with or late latent cases). Overall, PN was higher among contagious than non-contagious cases (58.4% versus 31.0%; p = 0.030) and it was significantly higher in the non-HUG compared to the HUG group (75.9% versus 50.0%, respectively; p 45 years (RR 1.36; 95% CI: 1.05-1.76) and if the patient had received treatment for syphilis (RR 1.91; 95% CI: 1.38-2.66). Our results illustrate the difficulty of contact tracing in syphilis infection and the necessity to improve this crucial part of sexually transmitted infection management.

  15. Delays in the management of retroperitoneal sarcomas

    DEFF Research Database (Denmark)

    Seinen, Jojanneke; Almquist, Martin; Styring, Emelie

    2010-01-01

    at the general practitioner, 36 days at local hospitals, and 55 days at the sarcoma centre. Conclusion. Centralization per se is not sufficient for optimized and efficient management. Our findings suggest that delays can be minimized by direct referral of patients from primary health care to sarcoma centers...... sarcoma in the southern Sweden health care region 2003-2009 were eligible for the study. Data on referrals and diagnostic investigations were collected from clinical files from primary health care, local hospitals, and from the sarcoma centre. Lead times were divided into patient delays and health care...... delays caused by primary health care, local hospitals, or procedures at the sarcoma centre. Results. Complete data were available from 33 patients and demonstrated a median patient delay of 23 days (0-17 months) and median health care delay of 94 days (1-40 months) with delays of median 15 days...

  16. Delays in the management of retroperitoneal sarcomas

    DEFF Research Database (Denmark)

    Seinen, Jojanneke; Almquist, Martin; Styring, Emelie

    2010-01-01

    at the general practitioner, 36¿days at local hospitals, and 55¿days at the sarcoma centre. Conclusion. Centralization per se is not sufficient for optimized and efficient management. Our findings suggest that delays can be minimized by direct referral of patients from primary health care to sarcoma centers...... sarcoma in the southern Sweden health care region 2003-2009 were eligible for the study. Data on referrals and diagnostic investigations were collected from clinical files from primary health care, local hospitals, and from the sarcoma centre. Lead times were divided into patient delays and health care...... delays caused by primary health care, local hospitals, or procedures at the sarcoma centre. Results. Complete data were available from 33 patients and demonstrated a median patient delay of 23¿days (0-17¿months) and median health care delay of 94¿days (1-40¿months) with delays of median 15¿days...

  17. The role of FDG PET/CT in patients treated with neoadjuvant chemotherapy for localized bone sarcomas

    Energy Technology Data Exchange (ETDEWEB)

    Palmerini, Emanuela; Marchesi, Emanuela; Paioli, Anna; Ferrari, Stefano [Istituto Ortopedico Rizzoli, Chemotherapy, Bologna (Italy); Colangeli, Marco; Donati, Davide; Cevolani, Luca; De Paolis, Massimiliano [Istituto Ortopedico Rizzoli, Orthopaedic Surgery, Bologna (Italy); Nanni, Cristina; Fanti, Stefano; Cambioli, Silvia [Sant' Orsola Hospital, Nuclear Medicine, Bologna (Italy); Picci, Piero [Istituto Ortopedico Rizzoli, Research Laboratory, Bologna (Italy); Gambarotti, Marco [Istituto Ortopedico Rizzoli, Surgical Pathology, Bologna (Italy); Istituto Ortopedico Rizzoli, Radiology, Musculoskeletal Oncology Department, Bologna (Italy)

    2017-02-15

    The histological response to neoadjuvant chemotherapy is an important prognostic factor in patients with osteosarcoma (OS) and Ewing sarcoma (EWS). The aim of this study was to assess baseline primary tumour FDG uptake on PET/CT, and serum values of alkaline phosphatase (ALP) and lactate dehydrogenase (LDH), to establish whether these factors are correlated with tumour necrosis and prognosis. Patients treated between 2009 and 2014 for localized EWS and OS, who underwent FDG PET/CT as part of their staging work-up, were included. The relationships between primary tumour SUVmax at baseline (SUV1), SUVmax after induction chemotherapy (SUV2), metabolic response calculated as [(SUV1 - SUV2)/(SUV1)] x 100, LDH and ALP and tumour response/survival were analysed. A good response (GR) was defined as tumour necrosis >90 % in patients with OS, and grade II-III Picci necrosis (persistence of microscopic foci only or no viable tumor) in patients with Ewing sarcoma. The study included 77 patients, 45 with EWS and 32 with OS. A good histological response was achieved in 53 % of EWS patients, and 41 % of OS patients. The 3-year event-free survival (EFS) was 57 % in EWS patients and 48 % OS patients. The median SUV1 was 5.6 (range 0 - 17) in EWS patients and 7.9 (range 0 - 24) in OS patients (p = 0.006). In EWS patients the GR rate was 30 % in those with a high SUV1 (≥6) and 72 % in those with a lower SUV1 (p = 0.0004), and in OS patients the GR rate was 29 % in those with SUV1 ≥6 and 64 % in those with a lower SUV1 (p = 0.05). In the univariate analysis the 3-year EFS was significantly better in patients with a low ALP level (59 %) than in those with a high ALP level (22 %, p = 0.02) and in patients with a low LDH level (62 %) than in those with a high LDH level (37 %, p = 0.004). In EWS patients the 3-year EFS was 37 % in those with a high SUV1 and 75 % in those with a low SUV1 (p = 0.004), and in OS patients the 3-year EFS was 32 % in those with a high SUV1 and 66 % in those

  18. 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

    PURPOSE: The purpose of this study was to evaluate local control, survival and complication rate after treatment of soft tissue sarcoma (STS) with limb-sparing surgery combined with pulsed-dose rate (PDR) interstitial brachytherapy (BRT) and external beam radiotherapy (EBRT). PATIENTS AND METHODS...... 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......PURPOSE: The purpose of this study was to evaluate local control, survival and complication rate after treatment of soft tissue sarcoma (STS) with limb-sparing surgery combined with pulsed-dose rate (PDR) interstitial brachytherapy (BRT) and external beam radiotherapy (EBRT). PATIENTS AND METHODS......: A retrospective review of 39 adult patients (female/male=25/14, mean age 51(range 21-78) years) with STS who underwent primary limb-sparing surgery combined with PDR BRT (20Gy) and additional post-operative EBRT (50Gy) during the years 1995-2004. RESULTS: Five patients developed local recurrence after a mean...

  19. Reliability of Margin Assessment after Surgery for Extremity Soft Tissue Sarcoma: The SSG Experience

    Directory of Open Access Journals (Sweden)

    Clement S. Trovik

    2012-01-01

    Full Text Available Surgery remains the mainstay of soft tissue sarcoma (STS treatment and has been the primary treatment for the majority of patients in Scandinavia during the last 30 years although the use of adjuvant radiotherapy has increased. Patient and treatment characteristics have been recorded in the Scandinavian Sarcoma Group (SSG Register since 1987. When the effect of new radiotherapy guidelines from 1998 was evaluated, the reliability of surgical margin assessments among different Scandinavian institutions was investigated. Margins were reevaluated by a panel of sarcoma surgeons, studying pathology and surgical reports from 117 patients, randomly selected among 470 recorded patients treated between 1998–2003. In 80% of cases, the panel agreed with the original classification. Disagreement was most frequent when addressing the distinction between marginal and wide margins. Considered the element of judgment inherent in all margin assessment, we find this reliability acceptable for using the Register for studies of local control of STS.

  20. Functional Reconstruction of Sarcoma Defects Utilising Innervated Free Flaps

    Directory of Open Access Journals (Sweden)

    Damien Grinsell

    2012-01-01

    Full Text Available Soft-tissue reconstruction following preoperative radiotherapy and wide resection of soft tissue sarcoma remains a challenge. Pedicled and free tissue transfers are an essential part of limb sparing surgery. We report 22 cases of sarcoma treated with radiotherapy and wide excision followed by one-stage innervated free or pedicled musculocutaneous flap transfers. The resection involved the upper limb in 3 cases, the lower limb in 17, and the abdominal wall in 2. The flaps used for the reconstruction were mainly latissimus dorsi and gracilis. The range of motion was restored fully in 14 patients. The muscle strength of the compartment reconstructed was of grades 4 and 5 in all patients except one. The overall function was excellent in all the cases with functional scores of 71.2% in the upper limb and 84% in the lower limb. The only 2 major complications were flap necrosis, both revised with another flap, one of which was innervated with restoration of function. Innervated flaps are valuable alternatives for reconstruction after sarcoma resection in the extremity and in the abdominal wall. The excellent functional results are encouraging, and we believe that innervated muscle reconstruction should be encouraged in the treatment of sarcoma after radiotherapy and wide resection.

  1. T-cell infiltration and clonality correlate with programmed cell death protein 1 and programmed death-ligand 1 expression in patients with soft tissue sarcomas.

    Science.gov (United States)

    Pollack, Seth M; He, Qianchuan; Yearley, Jennifer H; Emerson, Ryan; Vignali, Marissa; Zhang, Yuzheng; Redman, Mary W; Baker, Kelsey K; Cooper, Sara; Donahue, Bailey; Loggers, Elizabeth T; Cranmer, Lee D; Spraker, Matthew B; Seo, Y David; Pillarisetty, Venu G; Ricciotti, Robert W; Hoch, Benjamin L; McClanahan, Terrill K; Murphy, Erin; Blumenschein, Wendy M; Townson, Steven M; Benzeno, Sharon; Riddell, Stanley R; Jones, Robin L

    2017-09-01

    Patients with metastatic sarcomas have poor outcomes and although the disease may be amenable to immunotherapies, information regarding the immunologic profiles of soft tissue sarcoma (STS) subtypes is limited. The authors identified patients with the common STS subtypes: leiomyosarcoma, undifferentiated pleomorphic sarcoma (UPS), synovial sarcoma (SS), well-differentiated/dedifferentiated liposarcoma, and myxoid/round cell liposarcoma. Gene expression, immunohistochemistry for programmed cell death protein (PD-1) and programmed death-ligand 1 (PD-L1), and T-cell receptor Vβ gene sequencing were performed on formalin-fixed, paraffin-embedded tumors from 81 patients. Differences in liposarcoma subsets also were evaluated. UPS and leiomyosarcoma had high expression levels of genes related to antigen presentation and T-cell infiltration. UPS were found to have higher levels of PD-L1 (P≤.001) and PD-1 (P≤.05) on immunohistochemistry and had the highest T-cell infiltration based on T-cell receptor sequencing, significantly more than SS, which had the lowest (P≤.05). T-cell infiltrates in UPS also were more oligoclonal compared with SS and liposarcoma (P≤.05). A model adjusted for STS histologic subtype found that for all sarcomas, T-cell infiltration and clonality were highly correlated with PD-1 and PD-L1 expression levels (P≤.01). In the current study, the authors provide the most detailed overview of the immune microenvironment in sarcoma subtypes to date. UPS, which is a more highly mutated STS subtype, provokes a substantial immune response, suggesting that it may be well suited to treatment with immune checkpoint inhibitors. The SS and liposarcoma subsets are less mutated but do express immunogenic self-antigens, and therefore strategies to improve antigen presentation and T-cell infiltration may allow for successful immunotherapy in patients with these diagnoses. Cancer 2017;123:3291-304. © 2017 The Authors. Cancer published by Wiley Periodicals, Inc

  2. Chemotherapy for Soft Tissue Sarcomas

    Science.gov (United States)

    ... Stage Soft Tissue Sarcoma Treating Soft Tissue Sarcomas Chemotherapy for Soft Tissue Sarcomas Chemotherapy (chemo) is the use of drugs given into ... Depending on the type and stage of sarcoma, chemotherapy may be given as the main treatment or ...

  3. 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.

  4. A phase I study of temsirolimus and bryostatin-1 in patients with metastatic renal cell carcinoma and soft tissue sarcoma.

    Science.gov (United States)

    Plimack, Elizabeth R; Tan, Tingting; Wong, Yu-Ning; von Mehren, Margaret M; Malizzia, Lois; Roethke, Susan K; Litwin, Samuel; Li, Tianyu; Hudes, Gary R; Haas, Naomi B

    2014-04-01

    Temsirolimus, an inhibitor of mammalian target of rapamycin (mTOR) complex 1, is approved for the treatment of metastatic renal cell carcinoma (RCC). Bryostatin-1 inhibits protein kinase C, a downstream effector of mTOR complex 2. We observed antitumor effects with the combination of temsirolimus and bryostatin-1 in RCC cell lines. METHODS. Four cohorts of patients received weekly bryostatin-1 (20 μg/m²) with temsirolimus (10, 15, 25, or 37.5 mg) in 28-day cycles. Thirty patients received a total of 138 cycles across four dose levels. Twenty-five patients had RCC (17 clear cell, 7 papillary, and 1 unclassified). Two sarcoma patients with prior cytotoxic therapy experienced dose-limiting toxicity at 15 mg of temsirolimus (grade 3 neutropenia and grade 3 hypophosphatemia). Subsequently, patients with prior cytotoxic therapy were excluded. Two additional dose-limiting toxicities were noted with 37.5 mg of temsirolimus (grade 3 neutropenia and grade 3 creatinine elevation). Consequently, the maximum tolerated dose was defined as temsirolimus at 25 mg and bryostatin-1 at 20 μg/m² every 28 days. Of the 25 RCC patients, 3 patients had partial responses that lasted for 14 months, 28 months, and ≥ 80 months, respectively. Partial responses were seen in both clear cell and papillary histology. This combination of 37.5 mg of temsirolimus with 20 μg/m² of bryostatin-1 was reasonably safe and well tolerated. Durable responses were observed in 3 of 25 patients with RCC.

  5. Ewing's sarcoma. Radiographic pattern of healing and bony complications in patients with long-term survival

    Energy Technology Data Exchange (ETDEWEB)

    Ehara, S.; Kattapuram, S.V.; Egglin, T.K. (Department of Radiology, Massachusetts General Hospital, Harvard Medical School, Boston (United States))

    1991-10-01

    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.

  6. Kaposi Sarcoma among HIV Infected Patients in Lagos University Teaching Hospital, Nigeria: A 14-Year Retrospective Clinicopathological Study

    Science.gov (United States)

    Akinde, Olakanmi; Adeyemo, Titilope; Omoseebi, Oladipo; Ikeri, Nzechukwu; Okonkwo, Ikechukwu; Afolayan, Olatunji

    2016-01-01

    Background. Despite the increased incidence of Kaposi sarcoma (KS) resulting from the Human Immunodeficiency Virus/Acquired Immunodeficiency Syndrome (HIV/AIDS) pandemic, there is still significant underreporting of KS in this environment. Objectives. This study was aimed at determining the incidence and clinicopathologic patterns of KS among HIV infected patients in Lagos University Teaching Hospital (LUTH), Nigeria, over a 14-year period: January 2000 to December 2013. Methodology. The materials for this study included patients' hospital clinical files, duplicate copies of histopathologic reports, and tissue blocks and corresponding archival slides in the Anatomic and Molecular Pathology Department and the HIV/AIDS unit of the Department of Haematology. Results. Within the study period, 182 cases of KS were diagnosed, accounting for 1.2% of all patients managed for HIV/AIDS and 2.99% of solid malignant tumours. The male-to-female ratio and modal age group were 1 : 1.3 and 5th decade, respectively. Most cases (90%) had purely mucocutaneous involvement with the lower limb being the commonest site (65.8%). The majority of lesions were plaques (65.8%). Vascular formation was the predominant histologic type seen (43.5%). Conclusion. KS in Lagos followed the same epidemiologic trend as other centers in Nigeria, with an increasing incidence in this era of HIV/AIDS. PMID:27034839

  7. Chemotherapy-related toxicity in patients with non-metastatic Ewing sarcoma: influence of sex and age.

    Science.gov (United States)

    Paioli, Anna; Luksch, Roberto; Fagioli, Franca; Tamburini, Angela; Cesari, Marilena; Palmerini, Emanuela; Abate, Massimo Eraldo; Marchesi, Emanuela; Balladelli, Alba; Pratelli, Loredana; Ferrari, Stefano

    2014-02-01

    Influence of age and sex on chemotherapy-related toxicity was evaluated in children (3-9 years), adolescents (10-17 years), and adults (up to 40 years) with localized Ewing sarcoma (ES) enrolled in the ISG/SSG III protocol. Treatment was based on vincristine, doxorubicin, cyclophosphamide, ifosfamide, dactinomycin, and etoposide. High-dose chemotherapy with busulfan and melphalan was given in poor responder patients. The analysis was based on 2191 courses of standard chemotherapy and 230 patients. A lower risk of G4 leukopenia and thrombocytopenia, hospitalization, febrile neutropenia, and red blood cell (RBC) transfusions was observed in males. Use of granulocyte colony-stimulating factor (G-CSF) was more frequent in adults, while children more often received RBC transfusions. A significant correlation between sex and chemotherapy-related toxicity was observed in the study, whereas no significant differences in terms of bone marrow toxicity can be expected according to patient age. Further studies should analyse the role of pharmacokinetics, pharmacogenomics, and clinical characteristics.

  8. Preoperative evaluation and monitoring chemotherapy in patients with high-grade osteogenic and Ewing`s sarcoma: review of current imaging modalities

    Energy Technology Data Exchange (ETDEWEB)

    Woude, H.-J. van der; Bloem, J.L. [Department of Radiology, Leiden University Medical Centre, C2-S, P.O. Box 9600, NL-2300 RC Leiden (Netherlands); Hogendoorn, P.C.W. [Department of Pathology, Leiden University Medical Centre, Leiden (Netherlands)

    1998-02-01

    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.) With 9 figs., 2 tabs., 62 refs.

  9. EWS/FLI1 Target Genes and Therapeutic Opportunities in Ewing Sarcoma

    Science.gov (United States)

    Cidre-Aranaz, Florencia; Alonso, Javier

    2015-01-01

    Ewing sarcoma is an aggressive bone malignancy that affect children and young adults. Ewing sarcoma is the second most common primary bone malignancy in pediatric patients. Although significant progress has been made in the treatment of Ewing sarcoma since it was first described in the 1920s, in the last decade survival rates have remained unacceptably invariable, thus pointing to the need for new approaches centered in the molecular basis of the disease. Ewing sarcoma driving mutation, EWS–FLI1, which results from a chromosomal translocation, encodes an aberrant transcription factor. Since its first characterization in 1990s, many molecular targets have been described to be regulated by this chimeric transcription factor. Their contribution to orchestrate Ewing sarcoma phenotype has been reported over the last decades. In this work, we will focus on the description of a selection of EWS/FLI1 targets, their functional role, and their potential clinical relevance. We will also discuss their role in other types of cancer as well as the need for further studies to be performed in order to achieve a broader understanding of their particular contribution to Ewing sarcoma development. PMID:26258070

  10. Primary Right Atrial Sarcoma Presenting with Cardiac Tamponade and Massive Pleural Effusion

    Directory of Open Access Journals (Sweden)

    Shahram Momtahen

    2016-07-01

    Full Text Available Primary cardiac sarcomas are very rare and there is no consensus on management. Clinical presentation is usually late. Despite newer diagnostic technology, prognosis remains dismal. We report a case of right atrial sarcoma in a 28-year-old man who presented with acute cardiac tamponade. Emergency subxiphoid pericardial drainage stabilized the patient's critical condition. The lesion was advanced. Therefore, we only performed a suboptimal surgical resection. Despite planning for radiation, the patient's status deteriorated. Only palliative measures continued during the next four months before his death due to disseminated metastasis and progressive cardiopulmonary failure.

  11. Do We Know What Causes Kaposi Sarcoma?

    Science.gov (United States)

    ... and Prevention Do We Know What Causes Kaposi Sarcoma? Kaposi sarcoma (KS) is caused by infection with a ... Sarcoma? Can Kaposi Sarcoma Be Prevented? More In Kaposi Sarcoma About Kaposi Sarcoma Causes, Risk Factors, and Prevention ...

  12. 18F-FDG PET/CT for Detection Sarcoma of the Aorta in a Patient with Takayasu Arteritis

    Energy Technology Data Exchange (ETDEWEB)

    Yakahashi, Tomoko; Watanabe, Naoto; Wakasa Minoru; Kajinami, Kouji; Tonami, Hisao [Kazazawa Medical Univ., Ishikawa (Japan)

    2016-06-15

    Sarcoma of the aorta is extremely rare; however, 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) imaging is a useful modality for detecting malignant tumors, including various sarcomas. We report on a case of sarcoma of the aorta associated concomitantly with Takayasu arteritis. The 18F-FDG PET/CT detected an abnormal increased up take in an aortic mass of the descending thoracic aorta, thoracic vertebra, and ilium. The standardized uptake value (SUV) of 18F-FDG in the aortic mass was 21.7, suggesting that 18F-FDG PET/CT imaging may be useful for detecting sarcoma of the aorta associated with Takayasu arteritis and bone metatases during treatment.

  13. Clinical efficacy of eribulin mesylate for the treatment of metastatic soft tissue sarcoma.

    Science.gov (United States)

    Emambux, Sheik; Italiano, Antoine

    2017-06-01

    Metastatic soft tissue sarcoma, a devastating disease, has a median overall survival of only 12-18 months. Treatment options remain scarce. However, eribulin mesylate, a first-in-class halichondrin B-based microtubule dynamics inhibitor, has recently been approved for the management of patients with advanced liposarcoma. Areas covered: Based on a review of the literature between 2005 and 2017, we present a summary of eribulin mesylate's mechanism of action and the studies showing its clinical efficacy in locally advanced or metastatic sarcomas. Expert commentary: Future development includes the definition of a biomarker signature related to patient outcome with eribulin. Further investigation via controlled clinical trials is needed to identify combination regimens that can optimize the efficacy of eribulin while providing an acceptable safety profile in sarcoma patients.

  14. Kaposi Sarcoma among HIV Infected Patients in Lagos University Teaching Hospital, Nigeria: A 14-Year Retrospective Clinicopathological Study

    Directory of Open Access Journals (Sweden)

    Olakanmi Akinde

    2016-01-01

    Full Text Available Background. Despite the increased incidence of Kaposi sarcoma (KS resulting from the Human Immunodeficiency Virus/Acquired Immunodeficiency Syndrome (HIV/AIDS pandemic, there is still significant underreporting of KS in this environment. Objectives. This study was aimed at determining the incidence and clinicopathologic patterns of KS among HIV infected patients in Lagos University Teaching Hospital (LUTH, Nigeria, over a 14-year period: January 2000 to December 2013. Methodology. The materials for this study included patients’ hospital clinical files, duplicate copies of histopathologic reports, and tissue blocks and corresponding archival slides in the Anatomic and Molecular Pathology Department and the HIV/AIDS unit of the Department of Haematology. Results. Within the study period, 182 cases of KS were diagnosed, accounting for 1.2% of all patients managed for HIV/AIDS and 2.99% of solid malignant tumours. The male-to-female ratio and modal age group were 1 : 1.3 and 5th decade, respectively. Most cases (90% had purely mucocutaneous involvement with the lower limb being the commonest site (65.8%. The majority of lesions were plaques (65.8%. Vascular formation was the predominant histologic type seen (43.5%. Conclusion. KS in Lagos followed the same epidemiologic trend as other centers in Nigeria, with an increasing incidence in this era of HIV/AIDS.

  15. Concomitant occurrence of EGFR (epidermal growth factor receptor) and KRAS (V-Ki-ras2 Kirsten rat sarcoma viral oncogene homolog) mutations in an ALK (anaplastic lymphoma kinase)-positive lung adenocarcinoma patient with acquired resistance to crizotinib

    DEFF Research Database (Denmark)

    Rossing, Henrik H; Grauslund, Morten; Urbanska, Edyta M;

    2013-01-01

    , the events behind crizotinib-resistance currently remain largely uncharacterized. Thus, we report on an anaplastic lymphoma kinase-positive non-small cell lung carcinoma patient with concomitant occurrence of epidermal growth factor receptor and V-Ki-ras2 Kirsten rat sarcoma viral oncogene homolog mutations......Anaplastic lymphoma kinase-positive non-small cell lung carcinoma patients are generally highly responsive to the dual anaplastic lymphoma kinase and MET tyrosine kinase inhibitor crizotinib. However, they eventually acquire resistance to this drug, preventing the anaplastic lymphoma kinase...... inhibitors from having a prolonged beneficial effect. The molecular mechanisms responsible for crizotinib resistance are beginning to emerge, e.g., in some anaplastic lymphoma kinase-positive non-small cell lung carcinomas the development of secondary mutations in this gene has been described. However...

  16. Whole-Body Radiation Therapy, Systemic Chemotherapy, and High-Dose Chemotherapy Followed By Stem Cell Rescue in Treating Patients With Poor-Risk Ewing Sarcoma

    Science.gov (United States)

    2015-01-07

    Adult Supratentorial Primitive Neuroectodermal Tumor (PNET); Ewing Sarcoma of Bone; Extraosseous Ewing Sarcoma; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Childhood Supratentorial Primitive Neuroectodermal Tumor; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Untreated Childhood Supratentorial Primitive Neuroectodermal Tumor

  17. A preliminary study of cyclophosphamide (NSC-26271), adriamycin (NSC-123127), imidazole carboxamide (NSC-45388), and actinomycin D (NSC-3053) with or without MER-BCG in patients with advanced sarcomas.

    Science.gov (United States)

    Creagan, E T; Edmonson, J H; Hahn, R G; Ahmann, D L; Bisel, H F; Eagan, R T

    1978-01-01

    Polychemotherapy for soft tissue sarcomas has been reported to produce response rates ranging from 24--60% (1, 2). Immunotherapy has reportedly prolonged survival after surgery for some tumors and enhanced the effectiveness of chemotherapy (3, 4). This report summarizes our preliminary experience with the combination of cyclophosphamide, adriamycin, imidazole carboxamide (DTIC), and actinomycin D (CAIA) with or without methanol extraction residue of BCG (MER) in patients with advanced sarcomas.

  18. Trabectedin in Soft Tissue Sarcomas

    Directory of Open Access Journals (Sweden)

    Bradley J. Petek

    2015-02-01

    Full Text Available Soft tissue sarcomas are a group of rare tumors derived from mesenchymal tissue, accounting for about 1% of adult cancers. There are over 60 different histological subtypes, each with their own unique biological behavior and response to systemic therapy. The outcome for patients with metastatic soft tissue sarcoma is poor with few available systemic treatment options. For decades, the mainstay of management has consisted of doxorubicin with or without ifosfamide. Trabectedin is a synthetic agent derived from the Caribbean tunicate, Ecteinascidia turbinata. This drug has a number of potential mechanisms of action, including binding the DNA minor groove, interfering with DNA repair pathways and the cell cycle, as well as interacting with transcription factors. Several phase II trials have shown that trabectedin has activity in anthracycline and alkylating agent-resistant soft tissue sarcoma and suggest use in the second- and third-line setting. More recently, trabectedin has shown similar progression-free survival to doxorubicin in the first-line setting and significant activity in liposarcoma and leiomyosarcoma subtypes. Trabectedin has shown a favorable toxicity profile and has been approved in over 70 countries for the treatment of metastatic soft tissue sarcoma. This manuscript will review the development of trabectedin in soft tissue sarcomas.

  19. Gastrointestinal Kaposi sarcoma with appendiceal involvement.

    Science.gov (United States)

    Egwuonwu, Steve; Gatto-Weis, Cara; Miranda, Roberto; Casas, Luis De Las

    2011-04-01

    Kaposi sarcoma is a vascular tumor manifesting as nodular lesions on skin, mucous membranes, or internal organs. This is a case of a 42-year-old human immunodeficiency virus- (HIV) positive bisexual male, not on highly active antiretroviral therapy (HAART) since diagnosis four years ago. He presented with a three-day history of abdominal pains, fever, vomiting, and a one-week history of melena stools. Endoscopy revealed Kaposi sarcoma in the stomach and duodenum. Postendoscopy, he developed acute abdomen. Exploratory laparotomy revealed extensive Kaposi sarcoma of the gastrointestinal tract with appendiceal involvement. The patient underwent appendectomy and had an uneventful recovery. A review of the literature discusses appendiceal Kaposi sarcoma with appendicitis, a rare but critical manifestation of gastrointestinal Kaposi sarcoma.

  20. [Synovial sarcoma. Case report].

    Science.gov (United States)

    Deme, Dániel; Abdulfatah, Bishr; Telekes, András

    2016-02-07

    In 2013 there were 94,770 new cancer patients reported in Hungary. Synovial sarcoma accounts for 0.05-0.1% of all cancers and, therefore its incidence is predicted to be 47-94 patients/year in Hungary. The authors report the history of a 18-year-old man who was operated on a right upper abdominal wall tumor with R1 resection. During the next 5 months the tumor grew up to 8 cm in largest diameter. Histology revealed monophasic synovial sarcoma. Immunohistochemistry showed bcl2, focal CD99 and high molecular weight cytokeratin positivity, while smooth muscle actin, S100 and CD34 immunostainings were negative. Becose of this reoperation was not possible, curative six cycles of doxorubicine and ifosfamide with granulocyte colony stimulating factor support and 60 Gy radiotherapy was given to the tumor bed. After these treatments computed tomography scan was negative and the patient attended regular imaging every 3 months. At the age of 20 years the patient developed two neoplastic lesions in the surgical scar measuring 10 mm and 45 × 10 mm in size. R0 resection, partial rib resection and abdominal wall reconstruction were performed. Histology confirmed residual monophasic synovial sarcoma. Radiotherapy was not given because of a risk of intestinal wall perforation. Staging positron emission tomography-computed tomography proved to be negative. At the age of 22 years magnetic resonance imaging scans indicated no tumor recurrence, but after one month a rapidly growing tumorous lesion was found on ultrasound in the surgical scar measuring 20 × 20 × 12 mm in size. Cytology confirmed local recurrence and fluorescence in situ hibridization indicated t(x;18). R0 exstirpation and partial mesh resection were performed and histology showed the same monophasic synovial sarcoma. Because of the presence of vascular invasion and a close resection margin (1 mm) the patient underwent 3 cycles of adjuvant chemotherapy (doxorubicine and ifosfamide) with granulocyte colony stimulating

  1. Type 1 (11; 22)(q24: q12) translocation is common in Ewing's sarcoma/peripheral neuroectodermal tumour in south Indian patients

    Indian Academy of Sciences (India)

    T Parija; S Shirley; S Uma; K R Rajalekshmy; S Ayyappan; T Rajkumar

    2005-06-01

    The Ewing’s sarcoma family can present diagnostic difficulties. In the past the basis of diagnosis has been a exclusion. Identification of a specific translocation especially t(11; 22) (EWS-FLI 1 fusion gene), which is seen in nearly 85% of Ewing’s sarcoma cases can help in precise diagnosis. We have carried out a study on twenty patient samples diagnosed to have Ewing’s sarcoma/peripheral neuroectodermal tumour (PNET)/small round cell malignant tumour. The study involved RT-PCR analysis for the fusion transcript, followed by sequencing to identify the specific type of fusion. Ninety percent (18/20) of the samples tested were found to be t(11; 22) translocations involving EWS-FLI 1 genes. Sixty-one percent (11/18) were found to be type 1 fusion and seven were type 2 (39%). This is the first study in India with quantitative information about the types of EWS-FLI 1 translocations present in Ewing’s family of tumours in south Indian patients.

  2. Kaposi's sarcoma following immune suppressive therapy for Wegener's granulomatosis.

    Science.gov (United States)

    Deschênes, Isabelle; Dion, Louise; Beauchesne, Claude; de Brum-Fernandes, Artur

    2003-03-01

    The association between Kaposi's sarcoma and infection with human herpesvirus 8 is now well recognized. Immunologic impairment is associated with 2 forms of Kaposi's sarcoma, epidemic [associated with human immunodeficiency virus (HIV) infection] and iatrogenic (associated with immunosuppressive treatment); both forms have become more common during the last decade. We describe an HIV negative 54-year-old man who developed Kaposi's sarcoma 2 months after the beginning of immuno-suppressive therapy for Wegener's granulomatosis (WG). With tapering of medication, complete remission of Kaposi's sarcoma was achieved in one year. To our knowledge, this is the second reported case of iatrogenic Kaposi's sarcoma in a patient with WG.

  3. Primary mediastinal giant synovial sarcoma: A rare case report

    Directory of Open Access Journals (Sweden)

    Gaetano Rea

    2015-03-01

    Full Text Available Synovial sarcoma has been defined by the World Health Organization (WHO in 2002 as a type of mesenchymal tissue cell tumor that exhibits epithelial differentiation and represents the third most common soft-tissue sarcoma in adults, accounting for approximately 10% of soft-tissue sarcomas. To date, only few reports have focused on mediastinal synovial sarcoma imaging findings. Herein, we report a case of a 13 cm primary mediastinal giant synovial sarcoma, diagnosed in a 56-year-old patient admitted in our Department of Radiology with a six-month history of dyspnea and back pain.

  4. Multicentric Castleman’s disease and Kaposi’s sarcoma in a HIV-positive patient on highly active antiretroviral therapy

    Directory of Open Access Journals (Sweden)

    Lauro Ortega

    2014-08-01

    Full Text Available Castleman’s disease is a group of rare lymphoproliferative disorders. The plasmablastic multicentric Castleman’s disease is frequently discovered in HIV-infected individuals in association with Kaposi sarcoma (HHV-8. Thirty-five year old male presented to our care with the main compliant of severe back pain for one week. His past medical problems include acquired immune deficiency syndrome diagnosed 12 years prior and Kaposi sarcoma, currently on highly active antiretroviral therapy (HAART. Radiographic imaging revealed hepatomegaly and diffuse lymphadenopathy. The HIV viral load was <20 polymerase chain reaction copies/mL, absolute CD4 count was 453 cells/mcL (490-1740 cells/mcL and CD8 count was 4142 cells/mcL (180-1170 cells/ mcL. Excisional biopsy of the left supraclavicular lymph node was performed with pathological findings of HHV8+ Kaposi sarcoma in the background of multicentric Castleman’s disease (plasmacytic variant. No evidence of transformation into large B-cell or plasmablastic lymphoma was noted. He was discharged on HAART and follow up to receive chemotherapy with cyclophosphamide, adriamycin, vin- cristine plus prednisone was started and rituximab plus prophylaxis for pneumocystis carinii. Multicentric Castleman’s disease has become more relevant in recent years due to its association with HIV and HHV-8 (Kaposi sarcoma and its potential to progress into plasmablastic B-cell lymphoma. The progression of MCD to B-cell lymphoma is a concern, especially in patients with HIV infection because it precludes the worst outcome and a high mortality, despite treatment. The most intriguing part of this case is that MCD occurred in a HIV-positive on HAART. This case signals a warning that a high suspicion for MCD can be justified even in those HIV-positive patients on HAART because the possibly of progression to plasmablastic B-cell lymphoma.

  5. Skin Ultrasound in Kaposi Sarcoma.

    Science.gov (United States)

    Carrascosa, R; Alfageme, F; Roustán, G; Suarez, M D

    2016-05-01

    The use of ultrasound imaging has recently been increasing in numerous dermatologic diseases. This noninvasive technique provides additional details on the structure and vascularization of skin lesions. Kaposi sarcoma is a vascular tumor that typically arises in the skin and mucosas. It can spread to lymph nodes and internal organs. We performed B-mode and color Doppler ultrasound studies in 3 patients with a clinical diagnosis of Kaposi sarcoma confirmed by histological examination. We found differences in the ultrasound pattern between nodular and plaque lesions, in both B-mode and color Doppler. We believe that skin ultrasound imaging could be a useful technique for studying cutaneous Kaposi sarcoma, providing additional information on the structural and vascular characteristics of the lesion.

  6. False-Negative Results of Endoscopic Biopsy in the Diagnosis of Gastrointestinal Kaposi's Sarcoma in HIV-Infected Patients.

    Science.gov (United States)

    Nagata, Naoyoshi; Sekine, Katsunori; Igari, Toru; Hamada, Yohei; Yazaki, Hirohisa; Ohmagari, Norio; Akiyama, Junichi; Shimbo, Takuro; Teruya, Katsuji; Oka, Shinichi; Uemura, Naomi

    2012-01-01

    Kaposi's sarcoma (KS) is a rare endothelial neoplasm mainly involving the skin, but it is often associated with AIDS. Diagnosis of gastrointestinal (GI) tract KS, a common site of visceral involvement in AIDS, is important, but endoscopic biopsy carries a risk of false-negative results (FNRs) due to its submucosal appearance. This study sought to determine the rate and causes of FNR for endoscopic biopsy of GI-KS lesions. Endoscopic biopsy samples of 116 GI-KS lesions were reviewed retrospectively. All GI-KS lesions were confirmed to be resolved following KS therapy. FNRs were yielded for 41 of the lesions (35.3%). Among upper and lower GI sites, the esophagus was the only site significantly associated with FNRs (P FNRs (P FNRs were found in 35.3% of GI-KS lesions and were especially related to the site of the esophagus and endoscopic early stage (small size or patch appearance). An SMT with ulceration may be relatively easy to diagnose on endoscopic biopsy. Caution should be exercised when performing endoscopic biopsy of these lesions in AIDS patients and evaluating the histological features.

  7. Solitary Kaposi's sarcoma in retromolar region of an HIV positive patient: case report Sarcoma de Kaposi em região retromolar de um paciente HIV positivo: relato de caso

    Directory of Open Access Journals (Sweden)

    Maiara de Moraes

    2012-02-01

    Full Text Available Kaposi's sarcoma is a malignant neoplasm of vascular origin. It occurs mainly among immune deficient individuals, thus it is the most common neoplasm among HIV- positive patients. Its pathogenesis is complex and has not been fully clarified. This case arouses particular interest due to its anatomic location in the retromolar region of a 39-year-old male HIV- positive patient, who presented low white blood cell count and was not undergoing antiretroviral therapy. The emergence of this lesion may be associated with highly active antiretroviral therapy (HAART discontinuation and leukopenia. Hence, the reestablishment of therapy allows a suitable therapeutic approach and contributes to prognosis and survival rates.Sarcoma de Kaposi é uma neoplasia maligna de origem vascular que ocorre principalmente em indivíduos com deficiência imunológica, sendo a neoplasia mais comum em pacientes HIV positivos. Sua patogênese é complexa e não está bem estabelecida. Este caso é de interesse pela localização anatômica em região retromolar de paciente soropositivo, que apresentou baixa contagem de células brancas do sangue e que não realizava terapia antirretroviral. O surgimento da lesão pode estar associado à interrupção da terapia antirretroviral altamente ativa (HAART e à baixa contagem leucocitária. Assim, o restabelecimento da terapia pode permitir a abordagem terapêutica e contribuir para o prognóstico e a sobrevida.

  8. Sentinel node biopsy as an adjunct to limb salvage surgery for epithelioid sarcoma of the hand

    Directory of Open Access Journals (Sweden)

    Hammond Alex

    2005-06-01

    Full Text Available Abstract Background Epithelioid sarcomas of the hand are rare, high-grade tumors with a propensity for regional lymphatic spread approaching 40%. Case presentation A 54-year-old male with an epithelioid sarcoma of the palm was treated with neoadjuvant radiation, wide excision, and two-stage reconstruction. Sentinel lymph node biopsy was used to stage the patient's axilla. Sentinel node biopsy results were negative. The patient has remained free of local, regional and distant disease for the follow-up time of 16 months. Conclusion The rarity of this tumor makes definitive conclusions difficult but SLN biopsy appears to be a useful adjunct in the treatment of these sarcomas.

  9. Successful use of dydrogesterone as maintenance therapy in recurrent endometrial stromal sarcoma: a case report.

    Science.gov (United States)

    Akashi, Daisuke; Todo, Yukiharu; Shimada, Chisa; Okamoto, Kazuhira; Minobe, Shinichiro; Kato, Hidenori

    2013-11-01

    Endometrial stromal sarcoma is known to be a hormone-dependent tumor. Efficacy of hormonal therapy including high-dose progestins, aromatase inhibitors or gonadotropin-releasing hormone analogs has been reported. We report a case of recurrent endometrial stromal sarcoma, the tumor cells of which were strongly positive for CD10, estrogen and progesterone receptors. Although almost all of the pelvic tumors infiltrating the rectum or pelvic side wall remained, the patient is alive with slight disease 9 years and 6 months after the initial failure. During the treatment period of 4 years and 3 months, the patient was treated exclusively with dydrogesterone at a daily dose of 10 mg and the tumor clinically disappeared. Dydrogesterone at a daily dose of 10 mg may be effective in treating low-grade endometrial stromal sarcoma.

  10. Sequential Dose-Dense Doxorubicin and Ifosfamide in Advanced Soft-Tissue Sarcoma Patients in an Out-Patient-Basis Schedule

    Directory of Open Access Journals (Sweden)

    G. F. G. Almeida

    2011-01-01

    Full Text Available Aims. This phase II study explored activity/safety of front-line dose-dense chemotherapy in high-grade STS (soft tissue sarcoma patients and tested ezrin as prognostic factor. Patients and Methods. The protocol consisted of three cycles of doxorubicin (DOXO 30 mg/m2 on days 1–3 every 2 weeks, followed by three cycles of ifosfamide (IFO 2.5 g/m2 two hours a day on days 1–5 every 3 weeks, with GCSF support. Ezrin was assessed immunohistochemically. Results. Twenty patients, 13 metastatic and 7 locally advanced, were enrolled. Median age was 39 years (25–60. Median dose intensities were 42 mg/m2/week and 3.6 g/m2/week for DOXO and IFO, respectively. Grade 3/4 toxicities occurred in 18 patients. Response rate was 15% (3 of 20 by RECIST. Patients younger than 45 years with locally advanced disease and synovial histology presented longer survival. A trend towards longer survival was observed among ezrin-positive patients. Conclusions. This dose-dense schedule should not be routinely used due to its high frequency of toxic events; however, a sequential strategy with DOXO and IFO may benefit selected patients and should be further explored with lower doses. The role of ezrin as a prognostic marker should be confirmed in a larger group of patients.

  11. Study on osteogenic sarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Park, Eung Chun; Kim, Young Il; Choi, Won Jae; Kim, Young Jin [Dept. of Dentomaxillofacial Radiology, College of Dentistry, Chosun University, Kwangju (Korea, Republic of)

    1993-02-15

    The author observed a case of osteogenic sarcoma in a 11-year-old female with complaint of painful swelling on face in right side. The observed results were as follows: 1. Large hematoma was observed, and patient complained painfull swelling on c/c site. 2. Predisposing factor of osteogenic sarcoma was not clear, but patient had history of extraction before patient visiting infirmary of our dental collage. 3. Serologic findings were not specific, and serum aldaline level was normal. 4. Radiographic findings were as follows: a. Diffuse faint radiopacit in the lesion. b. Bony destruction and increased radiopacity in right antrum. c. Displacement of multiple teeth on involved area (i. e no 12, 15, 55, 16, 17, 18) d. Increased periodontal space in single tooth (no 13) e. Destruction of bony crypt on involved teeth (no 13, 14, 15, 17, 18) f. Loss of lamina dura of three teeth in involved area (no 11, 12, 16) 5. Computed tomographic findings were as follows: a. Large calcific and heterogenous component mass in the Rt. maxillary sinus, and this mass extending to Rt. maxilla, alveolar bone, ethmoid sinus. b. Soft tissue bulging in to Rt. side nasal cavity and oral cavity. c. Bone destruction of maxillary sinus wall and Rt. alveolar bone.

  12. Pregnancy and genital sarcoma: a systematic review of the literature.

    Science.gov (United States)

    Matsuo, Koji; Eno, Michele L; Im, Dwight D; Rosenshein, Neil B

    2009-08-01

    We conducted a literature review to determine the clinical characteristics of genital sarcoma during pregnancy. The systematic literature search was conducted using the search engines PubMed and MEDLINE with keywords "sarcoma" and "pregnancy" and was limited to female genital organs such as ovary, uterus, cervix, vagina, vulva, and retroperitoneal sarcoma. Kaposi's sarcoma, metastatic sarcoma, history of sarcoma, bone sarcoma located in pelvis, and fetal sarcoma were excluded in this study. There were 40 cases of genital sarcoma during pregnancy between 1955 and 2007. The majority of the cases were uterine sarcoma (37.5%), followed by retroperitoneal sarcoma (27.5%), vulvar sarcoma (22.5%), and vaginal sarcoma (12.5%). Mean age of the patient was 27.8 +/- 7.0. The distribution in the onset of symptoms had two peaks: first trimester (27.5%) and third trimester (50.0%). Growing mass (42.5%), abdominal pain (30.0%), and vaginal bleeding (22.5%) were the three most common symptoms. Incidental diagnosis was made in 22.5% and included during cesarean section (12.5%) and routine pelvic exam (7.5%). The cases initially not suspicious for malignancy were 42.5%. Thirty-three (82.5%) cases had live-born infants with term delivery in 55.2%. Mean birth weight was 2843 +/- 791 g, and male infants were more common (66.7%). Intrauterine growth retardation was seen in 12.5% of cases. Preterm labor was a common complication. Median survival period was 2.5 years (95% confidence, 1.9 to 3.1). The 2-, 3-, and 5-year cumulative survival rates were 60%, 38%, and 17%, respectively. Genital sarcomas in pregnancy are rare. There is a delay in diagnosis due to low index of suspicion. A majority had live births, and the 5-year survival is similar to that of advanced-stage sarcoma in nonpregnant women.

  13. 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...

  14. Treatment Option Overview (Adult Soft Tissue Sarcoma)

    Science.gov (United States)

    ... Vascular Tumors Treatment Research Adult Soft Tissue Sarcoma Treatment (PDQ®)–Patient Version General Information About Adult Soft ... dye reacts to the light. Certain factors affect treatment options and prognosis (chance of recovery). The treatment ...

  15. Treatment Options for Adult Soft Tissue Sarcoma

    Science.gov (United States)

    ... Vascular Tumors Treatment Research Adult Soft Tissue Sarcoma Treatment (PDQ®)–Patient Version General Information About Adult Soft ... dye reacts to the light. Certain factors affect treatment options and prognosis (chance of recovery). The treatment ...

  16. Classic Kaposi’s sarcoma: The clinical, demographic and teratment characteristics of seventy-four patients

    Directory of Open Access Journals (Sweden)

    Beril Gülüş Demirel

    2016-12-01

    Full Text Available Background and Design: Classic Kaposi's sarkoma (CKS is a rare disease, generally seen across Mediterranean and the Middle East region. It's an angioproliferative disorder associated with human herpes virus-8 infection. There is a few data on epidemiology and clinical characteristics among Turkish patients with CKS. This study aims to evaluate epidemiologic, clinical characteristics and treatment results in patients with the diagnosis of CKS in Zonguldak. Materials and Methods: We retrospectively evaluated the hospital records of patients with CKS who attended the dermatological and venereal diseases department between 2003 and 2014. Seventy-four patients were included in this study. Demographic and clinical characteristics, applied treatments and responses to treatments were evaluated. Results: During the eleven year examination period, 74 CKS patients have been diagnosed in the dermatology clinic. The prevalence of CKS among dermatologic patients was found to be 0.02%. Patient age at diagnosis ranged from 33 to 90 years (mean: 70.2±11.7. Fifty-two patients were male (70.3% and 22 patients were female (29.7%. Multiple nodules were the most frequently seen clinical forms and the distal lower extremity was the most common site of involvement (80.6%. According to the CKS staging system, it was observed that 47 patients (62.7% were at stage 1, 11 patients (15.49% at stage 2, eight patients (10.7% at stage 3, and six patients (8% were at stage 4. Treatment options were excision for 35.1% of patients (n=26, radiotherapy for 25.7% of patients (n=19, cryosurgery for 14.9% of patients (n=11, and chemotherapy for 10.8% of patients (n=8. Relapse was found to occur most commonly after excision (58.3%. Conclusion: Larger, multicenter studies are needed in order to determine the prevalence of CKS and characteristics of patients with CKS in our country.

  17. Reasons for schizophrenia patients remaining out of treatment: results from a prospective study in a rural south indian community

    Directory of Open Access Journals (Sweden)

    Channaveerachari Naveen Kumar

    2016-01-01

    Full Text Available Background: A few studies have examined the factors associated with schizophrenia patients remaining untreated in India. Materials and Methods: We identified 184 schizophrenia patients in a rural community, offered the treatment with antipsychotics and followed them up in their Primary Health Centers for 1-year. Twenty-nine (15.8% patients remained untreated at both the baseline and 1-year follow-up despite our best attempts to keep them under the treatment umbrella. They were interviewed in detail regarding the reasons for remaining untreated. This group was compared with another group of patients (n = 69 who had stopped the treatment at baseline but were successfully brought under the treatment umbrella throughout the 1-year follow-up period. Results: The reasons for remaining untreated were (n; %: (a Unsatisfactory improvement with previous treatment attempts (19; 65.5%, (b poor bond between the patients and the families (6; 20.7%, (c active symptoms not allowing any treatment efforts from the family members (6; 20.7%, (d magico-religious beliefs about the illness and its treatment (4; 13.8%, (e poor social support (3; 10.3%, (f adverse effects of the medications (2; 6.9%, and (g perception of recovery and cure (1; 3.4%. For many patients, a constellation of these reasons was responsible for them remaining untreated. In contrast, the common reasons for those who restarted medications to have stopped the treatment at some time were the lack of awareness, the need to continue medications (47; 68.1%, and the financial constraints (28; 40.6%. Conclusion: The predominant reason for schizophrenia patients not remaining on the treatment in this rural community was the families′ lack of faith in antipsychotic treatment. Provision of comprehensive treatment package including medical, psychosocial and rehabilitative services, and sensitizing the community about benefits of the treatment may help in ensuring that all patients with psychosis receive

  18. When the patient remains in danger: psychotherapy in U.S. combat zones.

    Science.gov (United States)

    Roach, Christopher

    2008-03-01

    Much of the literature on the psychological treatment of victims of violence focuses on demographic populations that do not represent the majority of victims. Part of the origin of this problem is that the prototypical victim and the prototypical perpetrator are often the same. Young, poor, minority males often constitute both the victim and the perpetrator class in America. As a group, these individuals, while disproportionately thought of as suitable for incarceration, are not thought of as good candidates for psychological treatment. However, their suffering is real, unrelenting and often not addressed. Therapists face significant barriers, in the patient, in society and in themselves, in trying to help these individuals. This article examines the issues of racism, drugs, ongoing danger, class inequalities, misogyny and the blended victim/perpetrator, all of which complicate engagement and understanding between therapist and patient.

  19. Interval compressed vincristine, doxorubicin, cyclophosphamide alternating with ifosfamide, etoposide in patients with advanced Ewing’s and other Small Round Cell Sarcomas

    Directory of Open Access Journals (Sweden)

    Whelan Jeremy

    2012-09-01

    Full Text Available Abstract Background To evaluate tolerability and maintenance of dose intensity of 2 weekly treatment with vincristine, doxorubicin, cyclophosphamide alternating with ifosfamide, etoposide (VDC/IE in patients with advanced small round cell sarcomas including Ewing family tumours (EFT, desmoplastic small round cell tumours (DSRCT and undifferentiated high grade round cell sarcomas (UHGRCS. Methods Retrospective review of 16 patients treated at a single centre with VDC/IE. Dose received, treatment delay, toxicity and clinical outcome were recorded for each cycle up to a maximum of 14 cycles. Results A total 193 cycles of VDC/IE were administered to 10 patients with EFT, 4 with DSRCT and 2 with UHGRCS. Median age was 22 years with 75% over 18 years. Metastases were present in 14 patients. The mean duration of each cycle was 16.7 days. Febrile neutropenia occurred in 14 % of cycles, and grade 3/4 haematologic toxicity including anaemia and thrombocytopenia in 16 % and 11 % of cycles respectively. Seven patients had a dose reduction. Five patients discontinued VDC/IE early due to toxicity. Conclusions This schedule of VDC/IE is feasible in patients with EFT and DSRCT including adults and those with metastases. Its comparison with other standard regimens for these diseases is justified.

  20. Perioperative Outcomes Following Partial Nephrectomy Performed on Patients Remaining on Antiplatelet Therapy.

    Science.gov (United States)

    Ito, Timothy; Derweesh, Ithaar H; Ginzburg, Serge; Abbosh, Philip H; Raheem, Omer A; Mirheydar, Hossein; Hamilton, Zachary; Chen, David Y T; Smaldone, Marc C; Greenberg, Richard E; Viterbo, Rosalia; Kutikov, Alexander; Uzzo, Robert G

    2017-01-01

    We evaluated the risk of bleeding complications in patients undergoing partial nephrectomy in whom perioperative antiplatelet therapy was continued, as antiplatelet therapy is increasingly used and hemorrhage is a significant concern in partial nephrectomy. In this 2-center retrospective analysis 1,097 patients underwent partial nephrectomy between 2000 and 2014. The cohort was split into 3 groups of perioperative continuation of antiplatelet therapy (group 1-67), antiplatelet therapy stopped preoperatively (group 2-254) and no chronic antiplatelet therapy (group 3-776). Bleeding complications were defined as any transfusion, or any hospital readmission or secondary procedure performed for hemorrhage. Multivariable analysis was performed to elucidate independent risk factors for bleeding complications. Patients in group 1 were older (median age 66 years vs 64 and 57 years in groups 2/3, p antiplatelet therapy was an independent predictor of bleeding complications (OR 2.19, 95% CI 1.06-4.51, p=0.03). These findings appear attributable to intraoperative clopidogrel use. On multivariable analysis the use of aspirin alone was not associated with bleeding complications (OR 1.64, 95% CI 0.72-3.75, p=0.24). The risk of bleeding complications due to antiplatelet therapy use at partial nephrectomy may be due to clopidogrel. The need to continue perioperative aspirin alone does not appear to be a contraindication to the safe performance of partial nephrectomy. Copyright © 2017 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.

  1. Efficacy and safety of omalizumab in patients with chronic idiopathic/spontaneous urticaria who remain symptomatic on h1 antihistamines

    DEFF Research Database (Denmark)

    Saini, Sarbjit S; Bindslev-Jensen, Carsten; Maurer, Marcus;

    2015-01-01

    ASTERIA I was a 40-week, randomized, double-blind, placebo-controlled study to evaluate the efficacy and safety of subcutaneous omalizumab as add-on therapy for 24 weeks in patients with chronic idiopathic urticaria/spontaneous urticaria (CIU/CSU) who remained symptomatic despite H1 antihistamine...... treatment at licensed doses. Patients aged 12-75 years with CIU/CSU who remained symptomatic despite treatment with approved doses of H1 antihistamines were randomized (1:1:1:1) in a double-blind manner to subcutaneous omalizumab 75 mg, 150 mg, or 300 mg or placebo every 4 weeks for 24 weeks followed by 16...... event. Omalizumab 300 mg administered subcutaneously every 4 weeks reduced weekly ISS and other symptom scores versus placebo in CIU/CSU patients who remained symptomatic despite treatment with approved doses of H1 antihistamines....

  2. Retroperitoneal sarcomas: A single center experience; Sarcomes retroperitoneaux: experience d'un centre

    Energy Technology Data Exchange (ETDEWEB)

    Ben Hassouna, J.; Slimane, M.; Damak, T.; Chargui, R.; Ben Bachouche, W.; Oukad, M.; Bouzaine, H.; Bouzid, T.; Mtallah, M.; Khomsi, F.; Gamoudi, A.; Hechiche, M.; Dhiab, T.; Rahal, K. [Institut Salah Azaiz, Service de Chirurgie Carcinologique, Tunis (Tunisia)

    2008-09-15

    Purpose: To analyse the management and clinical outcome of patients treated for retroperitoneal soft tissue sarcoma and to identify prognosis factors. Patients and method: This is a retrospective study of 20 adults; 11 women and nine men treated between 1980 and 2000 in our institution. Results: There were seven lipo sarcomas and 13 leiomyo-sarcomas. The mean tumor size was 26 cm. Three patients underwent a complete resection (R0), 14 patients an incomplete resection (R1/R2) with microscopic or macroscopic tumour remained after surgery. Only three patients had a surgical biopsy. Median follow-up was 18 months. Overall survival rates was 45%, 10% at two and five years, respectively. Among the following factors, sex, age, symptom duration, tumor size, histologic type, histologic grade, resection type, mitotic index, necrosis extension, histologic differentiation, and A.J.C.C. stage, only mitotic index factor (scale 1,2 versus scale 3) significantly influenced patient survival (p = 0.02). Conclusion: This study re-emphasizes the poor outcome of patients with retroperitoneal sarcoma. Survival rates appeared low when compared with other studies. These results incite for a more aggressive approach. (authors)

  3. Genetic evolution of HIV in patients remaining on a stable HAART regimen despite insufficient viral suppression

    DEFF Research Database (Denmark)

    Kristiansen, Thomas B; Pedersen, Anders; Eugen-Olsen, Jesper;

    2005-01-01

    consistent HIV-RNA levels above 200 copies/ml were included in the study. The study period spanned at least 12 months and included 47 plasma samples from 17 patients that were sequenced and analysed with respect to evolutionary changes. At inclusion, the median CD4 count was 300 cells/ml (inter......-quartile range (IQR): 231-380) and the median HIV-RNA was 2000 copies/ml (IQR: 1301-6090). Reverse transcription inhibitor (RTI) mutations increased 0.5 mutations per y (STD = 0.8 mutations per y), while major protease inhibitor (PI) resistance mutations increased at a rate of 0.2 mutations per y (STD = 0.......8 mutations per y) and minor PI resistance mutations increased at a rate of 0.3 mutations per y (STD = 0.7 mutations per y). The rate at which RTI mutations accumulated decreased during the study period (p = 0.035). Interestingly, the rate of mutation accumulation was not associated with HIV-RNA level...

  4. Prognostic and predictive factors for outcome to first-line ifosfamide-containing chemotherapy for adult patients with advanced soft tissue sarcomas An exploratory, retrospective analysis on large series from the European Organization for Research and Treatment of Cancer-Soft Tissue and Bone Sarcoma Group (EORTC-STBSG)

    DEFF Research Database (Denmark)

    Sleiffer, S.; Ouali, M.; van Glabbeke, M.;

    2010-01-01

    Background: Adult patients with advanced soft tissue sarcomas (STS) are generally treated similarly, regardless of great differences between STS subtypes, disease presentation and patients' characteristics. As ifosfamide is frequently applied in first line systemic therapy, we aimed to establish...... prognostic and predictive factors for outcome to ifosfamide-based therapy. Methods: A retrospective, exploratory analysis was performed on data from 1337 advanced STS patients who received first-time ifosfamide-containing chemotherapy. For predictive factor analysis, 660 patients treated with doxorubicin...

  5. Comprehensive analysis of published phase I/II clinical trials between 1990-2010 in osteosarcoma and Ewing sarcoma confirms limited outcomes and need for translational investment

    Directory of Open Access Journals (Sweden)

    van Maldegem Annemiek M

    2012-01-01

    Full Text Available Abstract Background High grade primary bone sarcomas are rare cancers that affect mostly children and young adults. Osteosarcoma and Ewing sarcoma are the most common histological subtypes in this age group, with current multimodality treatment strategies achieving 55-70% overall survival. As there remains an urgent need to develop new therapeutic interventions, we have reviewed published phase I/II trials that have been reported for osteosarcoma and Ewing sarcoma in the last twenty years. Results We conducted a literature search for clinical trials between 1990 and 2010, either for trials enrolling bone sarcoma patients as part of a general sarcoma indication or trials specifically in osteosarcoma and Ewing sarcoma. We identified 42 clinical trials that fulfilled our search criteria for general sarcoma that enrolled these patient groups, and eight and twenty specific trials for Ewing and osteosarcoma patients, respectively. For the phase I trials which enrolled different tumour types our results were incomplete, because the sarcoma patients were not mentioned in the PubMed abstract. A total of 3,736 sarcoma patients were included in these trials over this period, 1,114 for osteosarcoma and 1,263 for Ewing sarcoma. As a proportion of the worldwide disease burden over this period, these numbers reflect a very small percentage of the potential patient recruitment, approximately 0.6% for Ewing sarcoma and 0.2% for osteosarcoma. However, these data show an increase in recent activity overall and suggest there is still much room for improvement in the current trial development structures. Conclusion Lack of resources and commercial investment will inevitably limit opportunity to develop sufficiently rapid improvements in clinical outcomes. International collaboration exists in many well founded co-operative groups for phase III trials, but progress may be more effective if there were also more investment of molecular and translational research into

  6. Semi-Quantitative Calculations of Primary Tumor Metabolic Activity Using F-18 FDG PET/CT as a Predictor of Survival in 92 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

    To assess the prognostic value of primary tumor metabolic activity in patients with high-grade bone sarcomas (BS) or soft tissue sarcomas (STS) using F-18 FDG PET/CT. A single-site, retrospective study including 92 patients with high-grade BS or STS. Pretreatment F-18 FDG PET/CT scan was performed...... metabolic activity with pretherapeutic SUVmax using F-18 FDG PET/CT demonstrates independent properties beyond histologic grading for prediction of survival in patients with high-grade STS, but not with high-grade BS....

  7. Human herpes virus-8 DNA in bronchoalveolar lavage samples from patients with AIDS-associated pulmonary Kaposi's sarcoma

    DEFF Research Database (Denmark)

    Benfield, T L; Dodt, K K; Lundgren, Jens Dilling

    1997-01-01

    Kaposi's sarcoma (KS) is the most frequent AIDS-associated neoplasm, and often disseminates to visceral organs, including the lungs. An ante-mortem diagnosis of pulmonary KS is difficult. Recently, DNA sequences resembling a new human herpes virus (HHV-8), have been identified in various forms...

  8. Neurogenic sarcomas of the neck in neurofibromatosis.

    Science.gov (United States)

    Martin, G; Kleinsasser, O

    1981-01-01

    Based on two observations and a review of the literature, the pathological and clinical findings in sarcomas of the neck in patients with neurofibromatosis are described. Histologically these neurogenic tumours show a manifold picture; in addition to spindle-cell sarcomas pleomorphic structures are to be found, which can be similar to rhabdomyo-, lipo-, chondro-, angio-, or osteogenic sarcomas so that a histological diagnosis of a neurogenic sarcoma cannot always be made without clinical details. Up to the present surgical treatment is preferred; the value of cytostatic therapy and irradiation is controversial. The results of treating these tumours are unsatisfactory. Of 29 cases reported in the literature, only two could be found in which the patient survived without a recurrence for more than five years.

  9. 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

  10. Salivary production of IgA and IgG to human herpes virus 8 latent and lytic antigens by patients in whom Kaposi's sarcoma has regressed.

    Science.gov (United States)

    Mbopi-Keou, Francois-Xavier; Legoff, Jerome; Piketty, Christophe; Hocini, Hakim; Malkin, Jean-Elie; Inoue, Naoki; Scully, Crispian M; Porter, Stephen R; Teo, Chong-Gee; Belec, Laurent

    2004-01-23

    IgG and IgA antibodies with specificities to a latent and a lytic antigen of human herpes virus 8 (HHV-8) were detectable in the saliva and serum of eight patients whose Kaposi's sarcoma had regressed, seven of whom were HIV-1 infected. The measurement of antibody-specific activity and secretion rate, and the detection of secretory IgA all indicate anti-HHV-8 antibody activity in saliva. The specific humoral responses possibly influence mucosal replication of HHV-8, and in turn, that of HIV.

  11. 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;

    2016-01-01

    Background. Belinostat is a novel histone deacetylase inhibitor. Primary Objectives. Maximum tolerated dose (MTD) and dose limiting toxicities (DLTs) of belinostat (Bel) in combination with doxorubicin (Dox) in solid tumours (phase I) and response rate (RR) in soft tissue sarcomas (phase II......) 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...

  12. The rewarming benefit of anterior torso heat pad application in mildly hypothermic conscious adult trauma patients remains inconclusive

    Directory of Open Access Journals (Sweden)

    Ting Joseph

    2012-03-01

    Full Text Available Abstract The rewarming benefit of anterior torso heat pad application in mildly hypothermic conscious adult trauma patients remains inconclusive in this randomized comparative clinical trial. There was no between-group rewarming gain in ear canal temperature when an anterior torso chemical heat pad was compared with blankets. Patient awareness, and favorable perception of, being administered the active intervention (heat pad could explain the significant improvement in patient-rated cold discomfort discerned with the heat pad. In the context of marginal demonstrated benefit, it would have been informative to ascertain adverse effects related to the heat pad, including burn injury to the chest wall.

  13. Classic Kaposi's sarcoma presenting in the oral cavity of two HIV-negative Quechua patients.

    Science.gov (United States)

    Mohanna, Salim; Bravo, Francisco; Ferrufino, Juan Carlos; Sanchez, Juvenal; Gotuzzo, Eduardo

    2007-09-01

    Traditionally, classic KS lesions have a general distribution, often involving the skin of the feet and legs, and to a lesser extent, that of the hands, arms, and trunk. Oral involvement is a rare manifestation. Initial oral involvement is an even rarer occurrence. We report two unusual cases of classic KS presenting in the oral cavity of two patients from indigenous origin; the first patient with primary oral KS lesion on the hard palate, with no other signs of the condition in any other region of the body; the second patient with generalized dermal KS lesions with lymph node and lower lip involvement. In conclusion, clinicians and pathologists should be aware of the typical clinical, gross, and histologic features of KS. Moreover, we would like to emphasize that oral KS may affect patients without AIDS or exposure to immunosuppression. The awareness of oral classic KS as a diagnostic possibility is important in the work-up of vascular lesions in the oral cavity of non-immunosuppressed individuals.

  14. The International Scoring System (ISS) for multiple myeloma remains a robust prognostic tool independently of patients' renal function.

    Science.gov (United States)

    Dimopoulos, M A; Kastritis, E; Michalis, E; Tsatalas, C; Michael, M; Pouli, A; Kartasis, Z; Delimpasi, S; Gika, D; Zomas, A; Roussou, M; Konstantopoulos, K; Parcharidou, A; Zervas, K; Terpos, E

    2012-03-01

    The International Staging System (ISS) is the most widely used staging system for patients with multiple myeloma (MM). However, serum β2-microglobulin increases in renal impairment (RI) and there have been concerns that ISS-3 stage may include 'up-staged' MM patients in whom elevated β2-microglobulin reflects the degree of renal dysfunction rather than tumor load. In order to assess the impact of RI on the prognostic value of ISS, we analyzed 1516 patients with symptomatic MM and the degree of RI was classified according to the Kidney Disease Outcomes Quality Initiative-Chronic Kidney Disease (CKD) criteria. Forty-eight percent patients had stages 3-5 CKD while 29% of patients had ISS-1, 38% had ISS-2 and 33% ISS-3. The frequency and severity of RI were more common in ISS-3 patients. RI was associated with inferior survival in univariate but not in multivariate analysis. When analyzed separately, ISS-1 and ISS-2 patients with RI had inferior survival in univariate but not in multivariate analysis. In ISS-3 MM patients, RI had no prognostic impact either in univariate or multivariate analysis. Results were similar, when we analyzed only patients with Bence-Jones >200 mg/day. ISS remains unaffected by the degree of RI, even in patients with ISS-3, which includes most patients with renal dysfunction.

  15. Postoperative intensity-modulated radiation therapy provides favorable local control and low toxicities in patients with soft tissue sarcomas in the extremities and trunk wall

    Directory of Open Access Journals (Sweden)

    Wang JY

    2015-10-01

    Full Text Available Jianyang Wang, Shulian Wang, Yongwen Song, Xinfan Liu, Jing Jin, Weihu Wang, Zihao Yu, Yueping Liu, Yexiong Li Department of Radiation Oncology, Cancer Hospital and Institute, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, People’s Republic of China Purpose: To investigate the efficacy and toxicity of postoperative intensity-modulated radiation therapy (IMRT for patients with soft tissue sarcomas (STSs in the extremities and trunk wall. Patients and methods: Eighty patients with localized STSs in the extremities and trunk wall treated with function-conserving surgery and postoperative IMRT were analyzed. The primary locations were in the extremities in 51 patients and in the trunk wall in 29 patients. The margins were positive in nine patients and negative in 71 patients. The median dose of IMRT was 64 Gy. Results: At a median follow-up time of 38 months, eight patients developed local recurrences. The 5-year local control (LC rate was 88.1%. The patients with negative margins exhibited much better LC than did those with positive margins (90% vs 64.8%, P=0.023. Multivariate analysis revealed that positive margin was an independent risk factor for LC. The 5-year distant metastasis-free survival, disease-free survival, and overall survival rates were 75.2%, 72.6%, and 83.6%, respectively. Large tumor size (>5 cm was significantly associated with poor overall survival. Edema and joint stiffness were observed in 17.6% and 9.8% of patients with extremity STSs, respectively. Conclusion: IMRT provides excellent LC and low toxicity for patients with STSs in the extremities and trunk wall. Keywords: soft tissue sarcoma, extremities and trunk wall, intensity-modulated radiation therapy, local control, toxicitiess

  16. 18F-FLT Positron Emission Tomography and Diffusion-Weighted Magnetic Resonance Imaging in Planning Surgery and Radiation Therapy and Measuring Response in Patients With Newly Diagnosed Ewing Sarcoma

    Science.gov (United States)

    2016-03-15

    Adult Supratentorial Primitive Neuroectodermal Tumor (PNET); Ewing Sarcoma of Bone; Extraosseous Ewing Sarcoma; Localized Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Untreated Childhood Supratentorial Primitive Neuroectodermal Tumor

  17. Potential Therapeutic Targets in Uterine Sarcomas

    Directory of Open Access Journals (Sweden)

    Tine Cuppens

    2015-01-01

    Full Text Available Uterine sarcomas are rare tumors accounting for 3,4% of all uterine cancers. Even after radical hysterectomy, most patients relapse or present with distant metastases. The very limited clinical benefit of adjuvant cytotoxic treatments is reflected by high mortality rates, emphasizing the need for new treatment strategies. This review summarizes rising potential targets in four distinct subtypes of uterine sarcomas: leiomyosarcoma, low-grade and high-grade endometrial stromal sarcoma, and undifferentiated uterine sarcoma. Based on clinical reports, promising approaches for uterine leiomyosarcoma patients include inhibition of VEGF and mTOR signaling, preferably in combination with other targeted or cytotoxic compounds. Currently, the only targeted therapy approved in leiomyosarcoma patients is pazopanib, a multitargeted inhibitor blocking VEGFR, PDGFR, FGFR, and c-KIT. Additionally, preclinical evidence suggests effect of the inhibition of histone deacetylases, tyrosine kinase receptors, and the mitotic checkpoint protein aurora kinase A. In low-grade endometrial stromal sarcomas, antihormonal therapies including aromatase inhibitors and progestins have proven activity. Other potential targets are PDGFR, VEGFR, and histone deacetylases. In high-grade ESS that carry the YWHAE/FAM22A/B fusion gene, the generated 14-3-3 oncoprotein is a putative target, next to c-KIT and the Wnt pathway. The observation of heterogeneity within uterine sarcoma subtypes warrants a personalized treatment approach.

  18. Potential Therapeutic Targets in Uterine Sarcomas

    Science.gov (United States)

    Cuppens, Tine; Tuyaerts, Sandra; Amant, Frédéric

    2015-01-01

    Uterine sarcomas are rare tumors accounting for 3,4% of all uterine cancers. Even after radical hysterectomy, most patients relapse or present with distant metastases. The very limited clinical benefit of adjuvant cytotoxic treatments is reflected by high mortality rates, emphasizing the need for new treatment strategies. This review summarizes rising potential targets in four distinct subtypes of uterine sarcomas: leiomyosarcoma, low-grade and high-grade endometrial stromal sarcoma, and undifferentiated uterine sarcoma. Based on clinical reports, promising approaches for uterine leiomyosarcoma patients include inhibition of VEGF and mTOR signaling, preferably in combination with other targeted or cytotoxic compounds. Currently, the only targeted therapy approved in leiomyosarcoma patients is pazopanib, a multitargeted inhibitor blocking VEGFR, PDGFR, FGFR, and c-KIT. Additionally, preclinical evidence suggests effect of the inhibition of histone deacetylases, tyrosine kinase receptors, and the mitotic checkpoint protein aurora kinase A. In low-grade endometrial stromal sarcomas, antihormonal therapies including aromatase inhibitors and progestins have proven activity. Other potential targets are PDGFR, VEGFR, and histone deacetylases. In high-grade ESS that carry the YWHAE/FAM22A/B fusion gene, the generated 14-3-3 oncoprotein is a putative target, next to c-KIT and the Wnt pathway. The observation of heterogeneity within uterine sarcoma subtypes warrants a personalized treatment approach. PMID:26576131

  19. 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.

  20. Evaluation of a Water-based Bolus Device for Radiotherapy to the Extremities in Kaposi's Sarcoma Patients

    Energy Technology Data Exchange (ETDEWEB)

    Ahn, Seung Kwon; Kim, Yong Bae; Lee, Ik Jae [Yonsei University College of Medicine, Seoul (Korea, Republic of)] (and others)

    2008-09-15

    We designed a water-based bolus device for radiation therapy in Kaposi's sarcoma. This study evaluated the usefulness of this new device and compared it with the currently used rice-based bolus. Materials and Methods: We fashioned a polystyrene box and cut a hole in order to insert patient's extremities while the patient was in the supine position. We used a vacuum-vinyl based polymer to reduce water leakage. Next, we eliminated air using a vacuum pump and a vacuum valve to reduce the air gap between the water and extremities in the vacuum-vinyl box. We performed CT scans to evaluate the density difference of the fabricated water-based bolus device when the device in which the rice-based bolus was placed directly, the rice-based bolus with polymer-vinyl packed rice, and the water were all put in. We analyzed the density change with the air gap volume using a planning system. In addition, we measured the homogeneity and dose in the low-extremities phantom, attached to six TLD, and wrapped film exposed in parallel-opposite fields with the LINAC under the same conditions as the set-up of the CT-simulator. Results: The density value of the rice-based bolus with the rice put in directly was 14% lower than that of the water-based bolus. Moreover, the value of the other experiments in the rice-based bolus with the polymer-vinyl packed rice showed an 18% reduction in density. The analysis of the EDR2 film revealed that the water-based bolus shows a more homogeneous dose plan, which was superior by 4.0-4.4% to the rice-base bolus. The mean TLD readings of the rice-based bolus, with the rice put directly into the polystyrene box had a 3.4% higher density value. Moreover, the density value in the case of the rice-based bolus with polymer-vinyl packed rice had a 4.3% higher reading compared to the water-based bolus. Conclusion: Our custom-made water-based bolus device increases the accuracy of the set-up by confirming the treatment field. It also improves the

  1. Long-term treatment with the oncolytic ECHO-7 virus Rigvir of a melanoma stage IV M1c patient, a small cell lung cancer stage IIIA patient, and a histiocytic sarcoma stage IV patient-three case reports.

    Science.gov (United States)

    Alberts, Pēteris; Olmane, Evija; Brokāne, Linda; Krastiņa, Zanda; Romanovska, Māra; Kupčs, Kārlis; Isajevs, Sergejs; Proboka, Guna; Erdmanis, Romualds; Nazarovs, Jurijs; Venskus, Dite

    2016-10-01

    Oncolytic virotherapy is a recent addition to cancer treatment. Here, we describe positive treatment outcomes in three patients using Rigvir virotherapy. One of the patients is diagnosed with melanoma stage IV M1c, one with small cell lung cancer stage IIIA, and one with histiocytic sarcoma stage IV. The diagnoses of all patients are verified by histology or cytology. All patients started Rigvir treatment within a few months after being diagnosed and are currently continuing Rigvir treatment. The degree of regression of the disease has been determined by computed tomography. Safety assessment of adverse events graded according to NCI CTCAE did not show any value above grade 1 during Rigvir(®) treatment. Using current standard treatments, the survival of patients with the present diagnoses is low. In contrast, the patients described here were diagnosed 3.5, 7.0, and 6.6 years ago, and their condition has improved and been stabile for over 1.5, 6.5, and 4 years, respectively. These observations suggest that virotherapy using Rigvir can successfully be used in long-term treatment of patients with melanoma stage IV M1c, small cell lung cancer stage IIIA, and histiocytic sarcoma stage IV and therefore could be included in prospective clinical studies.

  2. Kaposi`s sarcoma; Sarcome de Kaposi

    Energy Technology Data Exchange (ETDEWEB)

    Kirova, Y.M.; Belembaogo, E.; Frikha, H.; Yu, S.J.; Le Bourgeois, J.P. [Hopital Henri-Mondor, 94 - Creteil (France)

    1997-09-01

    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)

  3. Pulmonary Artery Intimal Sarcoma: A Case Report

    Directory of Open Access Journals (Sweden)

    Joseph P. Kriz

    2016-04-01

    Full Text Available Pulmonary artery intimal sarcomas are rare and lethal malignant tumors that typically affect larger vessels: the aorta, inferior vena cava, and pulmonary arteries. Since symptoms and imaging of pulmonary arterial intimal sarcomas mimic pulmonary thromboembolism, the differential diagnosis of a patient presenting with chest pain, dyspnea, and filling defect within the pulmonary arteries should include intimal sarcoma. Often right ventricular failure is observed due to pulmonary hypertension caused by the obstructive effect of the tumor and concomitant chronic thromboembolism. We report the case of a 72-year-old African-American male with arterial intimal sarcoma of the left and right pulmonary artery with extension through the right artery into the bronchus and right lung.

  4. CLINICAL ANALYSIS AND SURGICAL RESULTS IN SARCOMA

    Directory of Open Access Journals (Sweden)

    Basavaraju

    2016-02-01

    Full Text Available INTRODUCTION Sarcomas are quite rare with only 15,000 new cases per year in the United States. Sarcomas therefore represent about one percent of the 1.5 million new cancer diagnoses in that country each year. Sarcoma can be defined as cancer whose cells originate from the cells of mesenchymal origin. The bones, cartilages, muscles are a few examples to be mentioned. This is in contrast to a malignant tumour originating from epithelial cells, which are termed carcinoma. AIMS AND OBJECTIVES 1. To clinically analyze the sarcomas. 2. To analyze the surgical outcome of this disease. The survival of the patient depends on the extent of metastasis and the primary identification. The study forms a base for further studies. So atleast it could be diagnosed earlier and treated to the full extent.

  5. Myeloid sarcoma of submandibular salivary gland

    Directory of Open Access Journals (Sweden)

    Federico Dagna

    2016-01-01

    Full Text Available Objective: To report a rare case of a myeloid sarcoma of submandibular salivary gland. Methods: A 65-year-old woman with a history of successfully treated myelodysplastic syndrome, presenting with periodic painful swelling of her right submandibular area. Results: Physical evaluation, ultrasound and CT scan revealed the presence of a 3-cm mass contiguous to the submandibular salivary gland. A core needle biopsy confirmed the diagnosis of myeloid sarcoma. Bone marrow biopsy was still showing complete remission and the submandibular gland was the only extramedullary site involved. The patient was submitted to chemotherapy. Conclusion: Myeloid sarcoma is a rare extramedullary neoplasm. It can virtually involve any anatomic site, but it usually involves lymph nodes, paranasal sinuses, skin, soft tissue and periostium. Myeloid sarcomas of salivary glands are very rare and ENTs should be aware of this disease in order to include it in the differential diagnosis of a solitary neck mass.

  6. 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 fro...... in human cancer; Ewing sarcomas synthesize and secrete proCCK that can be identified in plasma as circulating tumor marker....... 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...

  7. Transarterial chemoembolization in soft-tissue sarcoma metastases to the liver – The use of imaging biomarkers as predictors of patient survival

    Energy Technology Data Exchange (ETDEWEB)

    Chapiro, Julius [Russell H. Morgan Department of Radiology and Radiological Science, Division of Vascular and Interventional Radiology, The Johns Hopkins Hospital, Sheikh Zayed Tower, Suite 7203, 1800 Orleans St, Baltimore, MD, USA 21287 (United States); Department of Diagnostic and Interventional Radiology, Charite Universitätsmedizin, Berlin (Germany); Duran, Rafael [Russell H. Morgan Department of Radiology and Radiological Science, Division of Vascular and Interventional Radiology, The Johns Hopkins Hospital, Sheikh Zayed Tower, Suite 7203, 1800 Orleans St, Baltimore, MD, USA 21287 (United States); Lin, MingDe [U/S Imaging and Interventions (UII), Philips Research North America, Briarcliff Manor, NY (United States); Mungo, Benedetto [Department of Surgery, The Johns Hopkins Hospital, Baltimore, MD (United States); Schlachter, Todd; Schernthaner, Rüdiger [Russell H. Morgan Department of Radiology and Radiological Science, Division of Vascular and Interventional Radiology, The Johns Hopkins Hospital, Sheikh Zayed Tower, Suite 7203, 1800 Orleans St, Baltimore, MD, USA 21287 (United States); Gorodetski, Boris [Russell H. Morgan Department of Radiology and Radiological Science, Division of Vascular and Interventional Radiology, The Johns Hopkins Hospital, Sheikh Zayed Tower, Suite 7203, 1800 Orleans St, Baltimore, MD, USA 21287 (United States); Department of Diagnostic and Interventional Radiology, Charite Universitätsmedizin, Berlin (Germany); Wang, Zhijun [Russell H. Morgan Department of Radiology and Radiological Science, Division of Vascular and Interventional Radiology, The Johns Hopkins Hospital, Sheikh Zayed Tower, Suite 7203, 1800 Orleans St, Baltimore, MD, USA 21287 (United States); and others

    2015-03-15

    Highlights: •TACE is effective in achieving tumor response in sarcoma liver metastases. •Enhancement-based response criteria are beneficial as compared to RECIST. •3D quantitative image response assessment is the best predictor of survival. -- Abstract: Background: The clinical management of patients with metastatic soft-tissue sarcoma of the liver is complicated by the paucity of reliable clinical data. This study evaluated the safety profile, survival outcome as well as the role of imaging biomarkers of tumor response in metastatic soft-tissue sarcoma (mSTS) of the liver treated with conventional transarterial chemoembolization (cTACE). Materials/methods: This retrospective analysis included 30 patients with mSTS of the liver treated with cTACE. The safety profile, overall survival (OS) and progression-free survival (PFS) after the procedure were evaluated. Tumor response in each patient was assessed using RECIST, modified (m) RECIST and EASL guidelines. In addition, a 3D quantification of the enhancing tumor volume (quantitative [q] EASL) was performed. For each method, patients were classified as responders (R) and non-responders (NR), and evaluated using Kaplan-Meier and multivariate Cox proportional hazard ratio (HR) analysis. Results: No Grade III or IV toxicities were reported in a total of 77 procedures (mean, 2.6/patient). Median OS was 21.2 months (95% CI, 13.4–28.9) and PFS was 6.3 months (95% CI, 4.4–8.2). The enhancement-based techniques identified 11 (44%), 12 (48%) and 12 (48%) patients as R according to EASL, mRECIST and qEASL, respectively. No stratification was achieved with RECIST. Multivariate analysis identified tumor response according to mRECIST and qEASL as reliable predictors of improved patient survival (P = 0.019; HR 0.3 [0.1–0.8] and P = 0.006; HR 0.2 [0.1–0.6], respectively). Conclusion: This study confirmed the role of cTACE as a safe salvage therapy option in patients with mSTS of the liver. The demonstrated advantages

  8. Treatment response and mortality among patients starting antiretroviral therapy with and without Kaposi sarcoma: a cohort study.

    Directory of Open Access Journals (Sweden)

    Mhairi Maskew

    Full Text Available BACKGROUND: Improved survival among HIV-infected individuals on antiretroviral therapy (ART has focused attention on AIDS-related cancers including Kaposi sarcoma (KS. However, the effect of KS on response to ART is not well-described in Southern Africa. We assessed the effect of KS on survival and immunologic and virologic treatment responses at 6- and 12-months after initiation of ART. METHODS: We analyzed prospectively collected data from a cohort of HIV-infected adults initiating ART in South Africa. Differences in mortality between those with and without KS at ART initiation were estimated with Cox proportional hazard models. Log-binomial models were used to assess differences in CD4 count response and HIV virologic suppression within a year of initiating treatment. RESULTS: Between January 2001-January 2008, 13,847 HIV-infected adults initiated ART at the study clinics. Those with KS at ART initiation (n = 247, 2% were similar to those without KS (n = 13600,98% with respect to age (35 vs. 35yrs, presenting CD4 count (74 vs. 85cells/mm³ and proportion on TB treatment (37% vs. 30%. In models adjusted for sex, baseline CD4 count, age, treatment site, tuberculosis and year of ART initiation, KS patients were over three times more likely to have died at any time after ART initiation (hazard ratio[HR]: 3.62; 95% CI: 2.71-4.84 than those without KS. The increased risk was highest within the first year on ART (HR: 4.05; 95% CI: 2.95-5.55 and attenuated thereafter (HR: 2.30; 95% CI: 1.08-4.89. Those with KS also gained, on average, 29 fewer CD4 cells (95% CI: 7-52cells/mm³ and were less likely to increase their CD4 count by 50 cells from baseline (RR: 1.43; 95% CI: 0.99-2.06 within the first 6-months of treatment. CONCLUSIONS: HIV-infected adults presenting with KS have increased risk of mortality even after initiation of ART with the greatest risk in the first year. Among those who survive the first year on therapy, subjects with KS

  9. Dermoscopy of Kaposi's sarcoma: areas exhibiting the multicoloured 'rainbow pattern'.

    Science.gov (United States)

    Hu, S C-S; Ke, C-L K; Lee, C-H; Wu, C-S; Chen, G-S; Cheng, S-T

    2009-10-01

    Kaposi's sarcoma is a vascular tumour characterized by a proliferation of spindle cells and endothelial cells to form closely arranged slit-like vascular spaces. Currently, the definitive diagnosis of Kaposi's sarcoma relies on histology. The dermoscopic features of Kaposi's sarcoma are not clearly defined in the scientific literature. We seek to evaluate the dermoscopic features of Kaposi's sarcoma and compare them with other vascular tumours. One hundred forty-one lesions from seven patients with histologically proven Kaposi's sarcoma were evaluated using polarized light dermoscopy for the presence of various dermoscopic features. Twenty patients with other vascular tumours were also examined. Dermoscopic examination revealed bluish-reddish coloration (84% of lesions), multicoloured areas showing various colours of the rainbow spectrum (36%), scaly surface (29%), and small brown globules (15%). The 'rainbow pattern' was found in six out of seven patients with Kaposi's sarcoma and was not observed in other vascular tumours. In addition, there was an absence of dermoscopic features specific for other vascular and non-vascular skin tumours, such as well-defined lacunae or structured vascular pattern, in most of the Kaposi's sarcoma lesions. The most frequent dermoscopic patterns in Kaposi's sarcoma were found to be bluish-reddish coloration, the 'rainbow pattern', and scaly surface. The rainbow pattern is a dermoscopic feature which has not been previously described. We propose that dermoscopy, as an adjunct to clinical examination, may enhance accuracy in the preoperative diagnosis of Kaposi's sarcoma.

  10. Candida tropicalis arthritis of the elbow in a patient with Ewing's sarcoma that successfully responded to itraconazole

    OpenAIRE

    Seung Youn Kim; Jung Sub Lim; Dong Hwan Kim; Hyeon Jeong Lee; Joon Bum Cho; Jn Ah Lee; Dong Ho Kim

    2011-01-01

    Fungal infections are rarely responsible for arthritis. Few cases of fungal arthritis have been reported, even in immunocompromised hosts susceptible to low-virulence organisms. Herein, the authors report the first case of Candida tropicalis arthritis in a child with a solid tumor. A 13-year-old boy with Ewing’s sarcoma developed arthritis in his elbow during the neutropenic period after chemotherapy. Despite treatment with broad-spectrum antibiotics, his condition did not improve and serial ...

  11. Imaging of the optic disk in caring for patients with glaucoma: ophthalmoscopy and photography remain the gold standard.

    Science.gov (United States)

    Spaeth, George L; Reddy, Swathi C

    2014-01-01

    Optic disk imaging is integral to the diagnosis and treatment of patients with glaucoma. We discuss the various forms of imaging the optic nerve, including ophthalmoscopy, photography, and newer imaging modalities, including optical coherence tomography (OCT), confocal scanning laser ophthalmoscopy (HRT), and scanning laser polarimetry (GDx), specifically highlighting their benefits and disadvantages. We argue that ophthalmoscopy and photography remain the gold standard of imaging due to portability, ease of interpretation, and the presence of a large database of images for comparison.

  12. Uterine sarcoma Part II—Uterine endometrial stromal sarcoma: The TAG systematic review

    Directory of Open Access Journals (Sweden)

    Huann-Cheng Horng

    2016-08-01

    Full Text Available Endometrial stromal tumors are rare uterine tumors (<1%. Four main categories include endometrial stromal nodule, low-grade endometrial stromal sarcoma (LG-ESS, high-grade endometrial stromal sarcoma (HG-ESS, and uterine undifferentiated sarcoma (UUS. This review is a series of articles discussing the uterine sarcomas. LG-ESS, a hormone-dependent tumor harboring chromosomal rearrangement, is an indolent tumor with a favorable prognosis, but characterized by late recurrences even in patients with Stage I disease, suggesting the requirement of a long-term follow-up. Patients with HG-ESS, based on the identification of YWHAE-NUTM2A/B (YWHAE-FAM22A/B gene fusion, typically present with advanced stage diseases and frequently have recurrences, usually within a few years after initial surgery. UUS is, a high-grade sarcoma, extremely rare, lacking a specific line of differentiation, which is a diagnosis of exclusion (the wastebasket category, which fails to fulfill the morphological and immunohistochemical criteria of translocation-positive ESS. Surgery is the main strategy in the management of uterine sarcoma. Due to rarity, complex biological characteristics, and unknown etiology and risk factors of uterine sarcomas, the role of adjuvant therapy is not clear. Only LG-ESS might respond to progestins or aromatase inhibitors.

  13. Primary extraosseous Ewing sarcoma of the orbit.

    Science.gov (United States)

    Alio, Jorge L; Sales-Sanz, Marco; Vaz, Maria A; Barrancos, Constanza; Reguero, Maria E; Diamantopoulus, Jorge; Poveda, Pedro

    2013-01-01

    A 40-year-old man presented with painless, progressive vision loss and mild proptosis of the OD. CT revealed a right intraconal mass with slight penetration of the optic canal not contiguous with any bony structure. Incisional biopsy through a transfrontal orbitotomy revealed a diffuse growth of homogeneous, small, round cells. Immunohistochemical stains were positive for vimentin and MIC2 (CD99), and the translocation at EWS gene (22q12) was detected. Metastatic workup and a full-body bone scan were negative, confirming primary orbital extraosseous Ewing sarcoma. The patient received neoadjuvant chemotherapy and an orbital exenteration with preservation of eyelids and conjunctiva. He also received adjuvant chemotherapy and local radiotherapy, and he has remained disease-free for almost 3 years.

  14. Results of the Scandinavian Sarcoma Group XIV protocol for classical osteosarcoma: 63 patients with a minimum follow-up of 4 years.

    Science.gov (United States)

    Smeland, Sigbjørn; Bruland, Oyvind S; Hjorth, Lars; Brosjö, Otte; Bjerkehagen, Bodil; Osterlundh, Gustaf; Jakobson, Ake; Hall, Kirsten Sundby; Monge, Odd R; Björk, Olle; Alvegaard, Thor A

    2011-04-01

    The Scandinavian Sarcoma Group (SSG) XIV protocol is based on experience from previous SSG trials and other osteosarcoma intergroup trials, and has been considered the best standard of care for patients with extremity localized, non-metastatic osteosarcoma. We analyzed the outcome in 63 consecutive patients. Patients and methods From 2001 through 2005, 63 patients recruited from centers in Sweden, Norway, and Finland were included. They received preoperative chemotherapy consisting of 2 cycles of paired methotrexate (12 g/m²), cisplatin (90 mg/m²), and doxorubicin (75 mg/m²). 3 cycles were administered postoperatively, and poor histological responders were given 3 additional cycles of ifosfamide (10-12 g/m²) as a salvage strategy. With a median follow-up of 77 months for survivors, the estimated metastasis-free and sarcoma-related survival at 5 years was 70% and 76%, respectively. 53 patients were treated with limb salvage surgery or rotationplasty and 2 patients experienced a local recurrence. 3 toxic deaths were recorded, all related to acute toxicity from chemotherapy. The 5-year metastasis-free survival of poor histological responders receiving add-on treatment with ifosfamide was 47%, as compared to 89% for good histological responders. Outcome from the SSG XIV protocol compares favorably with the results of previous SSG trials and other published osteosarcoma trials. However, salvage therapy given to poor responders did not improve outcome to a similar degree as for good responders. In a multi-institutional setting, more than four-fifths of the patients were operated with limb salvage surgery or rotationplasty, with few local recurrences.

  15. Implicações sociais enfrentadas pelas famílias que possuem pacientes com sarcoma ósseo Social implications faced by bone sarcoma patients' families

    Directory of Open Access Journals (Sweden)

    Virginia Pinheiro Stolagli

    2008-01-01

    Full Text Available O grupo de oncologia ortopédica proporciona atendimento médico e psicossocial aos pacientes internados e em tratamento ambulatorial, portadores de tumores músculos-esqueléticos. Nesta pesquisa, nosso objetivo foi conhecer o perfil socioeconômico dos paciente-familiares e as mudanças ocorridas após a constatação do diagnóstico. A amostra foi composta por 25 famílias de pacientes com osteossarcoma de membro inferior. A pesquisa ocorreu no período de setembro a outubro/2005, através de formulários com questões fechadas e abertas, utilizando para análise qualitativa o discurso do sujeito coletivo. O estudo foi prospectivo, descritivo, com abordagem quanti-qualitativa. Dos entrevistados, 68% eram do sexo feminino, 44% destes eram genitoras; 76% estavam trabalhando e 28% possuíam vínculo empregatício. 60% possuía renda entre 2 a 5 salários mínimos. Após o diagnóstico, 92% tiveram aumento de gastos; 80% apresentaram dificuldade com a quimioterapia; 56% relacionam o transporte como dificultador para aderir ao tratamento. 100% desesperaram-se diante da descoberta do câncer. O câncer ocasiona mudanças nos papéis dos membros da família. Existe o receio da recidiva da doença que leva a família a ter medo da morte, fazendo-se necessário um trabalho em conjunto com a equipe multiprofissional.The orthopaedic oncology group provides medical and psychosocial care to patients, both in hospital and outpatient facilities, with musculoskeletal tumors. With this research we aimed to know the socioeconomical profile of the patients/ families, as well as the changes occurred after such diagnosis is received. The sample was constituted of 25 families of patients with osteosarcoma on lower limbs. The study was conducted between September and October 2005 by means of forms containing open and closed questions, using the collective subject speech for qualitative analysis. The study had a prospective, descriptive design with quanti

  16. Stages of Ewing Sarcoma

    Science.gov (United States)

    ... tumor that forms from a certain kind of cell in bone or soft tissue . Ewing sarcoma may be found in the bones of the legs, arms, feet, hands, chest , pelvis , spine , or skull . Ewing sarcoma also may be found in the soft tissue of the trunk, arms, legs, head and neck, abdominal cavity , or ...

  17. Breast sarcomas. Literature review

    Directory of Open Access Journals (Sweden)

    D. A. Ryabchikov

    2014-01-01

    Full Text Available The article presents an overview of the literature about breast sarcomas (nonepithelial malignances. Primary sarcomas are extremely rare, with less than 1 % of all malignant tumors of the breast. Breast carcinomas cause an increased interest of the scientists due to their unique clinical and pathological features and unpredictable prognosis.

  18. Patterns of care and outcomes of patients with METAstatic soft tissue SARComa in a real-life setting: the METASARC observational study.

    Science.gov (United States)

    Savina, Marion; Le Cesne, Axel; Blay, Jean-Yves; Ray-Coquard, Isabelle; Mir, Olivier; Toulmonde, Maud; Cousin, Sophie; Terrier, Philippe; Ranchere-Vince, Dominique; Meeus, Pierre; Stoeckle, Eberhard; Honoré, Charles; Sargos, Paul; Sunyach, Marie-Pierre; Le Péchoux, Cécile; Giraud, Antoine; Bellera, Carine; Le Loarer, François; Italiano, Antoine

    2017-04-10

    Well-designed observational studies of individuals with rare tumors are needed to improve patient care, clinical investigations, and the education of healthcare professionals. The patterns of care, outcomes, and prognostic factors of a cohort of 2225 patients with metastatic soft tissue sarcomas who were diagnosed between 1990 and 2013 and documented in the prospectively maintained database of the French Sarcoma Group were analyzed. The median number of systemic treatments was 3 (range, 1-6); 27% of the patients did not receive any systemic treatment and 1054 (49%) patients underwent locoregional treatment of the metastasis. Half of the patients who underwent chemotherapy (n = 810) received an off-label drug. Leiomyosarcoma was associated with a significantly better outcome than the other histological subtypes. With the exception of leiomyosarcomas, the benefit of a greater than third-line regimen was very limited, with a median time to next treatment (TNT) and overall survival (OS) ranging between 2.3 and 3.7 months and 5.4 and 8.5 months, respectively. The TNT was highly correlated with OS. Female sex, leiomyosarcoma histology, locoregional treatment of metastases, inclusion in a clinical trial, and treatment with first-line polychemotherapy were significantly associated with improved OS in the multivariate analysis. The combination of doxorubicin with a second drug, such as ifosfamide, represents a valid option, particularly when tumor shrinkage is expected to provide clinical benefits. After failure of the second-line therapy, best supportive care should be considered, particularly in patients with non-leiomyosarcoma histology who are not eligible to participate in a clinical trial. Locoregional treatment of metastasis should always be included in the therapeutic strategy when feasible. TNT may represent a useful surrogate endpoint for OS in clinical studies.

  19. Sarcoma sinovial anorretal: relato de caso

    Directory of Open Access Journals (Sweden)

    Hernán Augusto Centurión Sobral

    2006-03-01

    Full Text Available Os sarcomas são neoplasias que se originam das células mesenquimais primitivas, sendo raros na região anorretal. O objetivo é relatar um caso de sarcoma sinovial anorretal, neoplasia extremamente rara nesta localização. É descrito o caso de uma paciente de 77 anos que apresentava nodulação anal dolorosa e sangrante às evacuações, associada a puxo, tenesmo e perda ponderal. A lesão foi biopsiada e o estudo imunohistoquímico evidenciou sarcoma sinovial anorretal. A paciente foi submetida a amputação abdomino-perineal do reto, encontra-se assintomática, sem sinais de recidiva e em seguimento ambulatorial.Originated from mesenchymal cells, the sarcoma is rare in the anorectal area. The authors report a case of anorectal sinovial sarcoma, extremely rare in this location with no previous reports on literature. It's described a case of a 77 years old patient presenting an anal tumor, associated to pain, bleeding, tenesmus and weight loss. A synovial sarcoma was diagnosed after biopsy and imunohistochemical study. The pacient was submitted to a Miles procedure being assymptomatic and without any signs of disease in the last seven months.

  20. Immunotherapy for Bone and Soft Tissue Sarcomas

    Directory of Open Access Journals (Sweden)

    Takenori Uehara

    2015-01-01

    Full Text Available Although multimodal therapies including surgery, chemotherapy, and radiotherapy have improved clinical outcomes of patients with bone and soft tissue sarcomas, the prognosis of patients has plateaued over these 20 years. Immunotherapies have shown the effectiveness for several types of advanced tumors. Immunotherapies, such as cytokine therapies, vaccinations, and adoptive cell transfers, have also been investigated for bone and soft tissue sarcomas. Cytokine therapies with interleukin-2 or interferons have limited efficacy because of their cytotoxicities. Liposomal muramyl tripeptide phosphatidylethanolamine (L-MTP-PE, an activator of the innate immune system, has been approved as adjuvant therapeutics in combination with conventional chemotherapy in Europe, which has improved the 5-year overall survival of patients. Vaccinations and transfer of T cells transduced to express chimeric antigen receptors have shown some efficacy for sarcomas. Ipilimumab and nivolumab are monoclonal antibodies designed to inhibit immune checkpoint mechanisms. These antibodies have recently been shown to be effective for patients with melanoma and also investigated for patients with sarcomas. In this review, we provide an overview of various trials of immunotherapies for bone and soft tissue sarcomas, and discuss their potential as adjuvant therapies in combination with conventional therapies.

  1. Postradiation sarcoma of bone: review of 78 Mayo Clinic cases

    Energy Technology Data Exchange (ETDEWEB)

    Weatherby, R.P.; Dahlin, D.C.; Ivins, J.C.

    1981-05-01

    Postradiation sarcoma of bone is an uncommon but serious sequela of radiation therapy. Seventy-eight Mayo Clinic patients have been treated for sarcomas arising in irradiated bones. They received their initial radiotherapy for a wide variety of nonneoplastic and neoplastic conditions, both benign and malignant. Thirty-five sarcomas arose in bone that was normal at the time of radiotherapy, and 43 arose in irradiated preexisting osseous lesions. The latent period between radiotherapy and diagnosis of sarcoma averaged 14.3 years. Ninety percent of the postradiation sarcomas were either osteosarcomas or fibrosarcomas; chondrosarcoma, malignant (fibrous) histiocytoma, malignant lymphoma, Ewing's tumor, and metastasizing chondroblastoma also occurred. Prompt radical surgery, when feasible, is usually the treatment of choice for the sarcoma. About 30% of patients with sarcomas of the extremities or craniofacial bones survived 5 years without recurrence; there were no disease-free survivors among patients with tumors of the vertebral column, pelvis, or shoulder girdle. The low risk of sarcoma following radiotherapy for the treatment of cancer should not be a contraindication to its use in these patients; however, radiation therapy for benign bone tumors should be reserved for lesions that are not amenable to surgical treatment. An unusual case is also reported herein in which a fibrosarcoma was discovered in the humerus of a patient who had received radiotherapy 55 years previously for a verified osteosarcoma in the same site.

  2. Trial of Dasatinib in Advanced Sarcomas

    Science.gov (United States)

    2016-10-12

    Rhabdomyosarcoma; Malignant Peripheral Nerve Sheath Tumors; Chondrosarcoma; Sarcoma, Ewing's; Sarcoma, Alveolar Soft Part; Chordoma; Epithelioid Sarcoma; Giant Cell Tumor of Bone; Hemangiopericytoma; Gastrointestinal Stromal Tumor (GIST)

  3. Proton Radiotherapy for Pediatric Sarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Ladra, Matthew M.; Yock, Torunn I., E-mail: tyock@partners.org [Department of Radiation Oncology, Massachusetts General Hospital, Boston, MA 02114 (United States)

    2014-01-14

    Pediatric sarcomas represent a distinct group of pathologies, with approximately 900 new cases per year in the United States alone. Radiotherapy plays an integral role in the local control of these tumors, which often arise adjacent to critical structures and growing organs. The physical properties of proton beam radiotherapy provide a distinct advantage over standard photon radiation by eliminating excess dose deposited beyond the target volume, thereby reducing both the dose of radiation delivered to non-target structures as well as the total radiation dose delivered to a patient. Dosimetric studies comparing proton plans to IMRT and 3D conformal radiation have demonstrated the superiority of protons in numerous pediatric malignancies and data on long-term clinical outcomes and toxicity is emerging. In this article, we review the existing clinical and dosimetric data regarding the use of proton beam radiation in malignant bone and soft tissue sarcomas.

  4. Primary epithelioid sarcoma of scalp

    Directory of Open Access Journals (Sweden)

    Sushma G Gurwale

    2017-01-01

    Full Text Available Epithelioid sarcoma (ES is a rare mesenchymal tumor of unknown histogenesis which displays multidirectional differentiation, predominantly epithelial. They have no normal cellular counterpart and differ from both synovial sarcoma and other carcinomas. It mainly affects young adults. It has two variants, classic type and proximal type. The more common classic type presents as a slowly growing painless nodule or plaque on the distal extremities. It is rare in children and older individuals. There is male predominance. The size varies from few millimeters to several centimeters. Central deeply seated lesions in pelvis and genital tract are termed as proximal ES. It has a multinodular growth pattern and usually occurs in older patients. These are comparatively more aggressive and metastasize early. On histopathological examination, these lesions need to be distinguished from other tumors showing epithelioid morphology. Primary ES of scalp is an exceedingly rare tumor. We present a case of nodular tumor on the scalp with cervical lymph node metastasis.

  5. Olaratumab for soft tissue sarcoma.

    Science.gov (United States)

    Teyssonneau, Diego; Italiano, Antoine

    2017-08-01

    Soft tissue sarcomas (STS) are rare malignant tumors. Unfortunately, the first-line doxorubicin-based treatment has not been improved since the 1970s. Platelet-derived growth factor (PDGF) receptor alpha (PDGFR-α) and its ligands are co-expressed in many types of cancer, including sarcomas. They are involved in stimulating growth and regulating stromal-derived fibroblasts and angiogenesis. PDGFR-α and its ligand may play an important role in tumorigenesis and be a potential target in the treatment of sarcomas. Olaratumab is a fully human IgG1-type anti-PDGFR-α monoclonal antibody with a high affinity and a low 50% inhibitory concentration (IC50). Areas covered: The authors review the role of olaratumab in the treatment of STS by focusing on the recent, randomized Phase II JDGD trial that challenged patients with unresectable or metastatic STS with doxorubicin in the presence or absence of olaratumab. This trial showed a great improvement in overall survival (OS), with an increase in survival from 14.7 months to 26.5 months for patients in the experimental arm and showed acceptable toxicity. Expert opinion: Results seem promising. However, it must be qualified, as the study includes several uncertainties. These uncertainties should be addressed by the ongoing Phase 3 JGDJ confirmatory trial, for which the final efficacy analysis is expected by 2019.

  6. Synovial sarcoma presenting as iliotibial band friction syndrome.

    Science.gov (United States)

    Mesiha, Mena; Bauer, Thomas; Andrish, Jack

    2009-10-01

    Iliotibial band friction syndrome is a common entity that is often quickly diagnosed in orthopedic clinics. However, synovial sarcoma is an elusive clinical entity that appears around many joints with variable presentations. This case report is an example of a patient with a classic presentation of iliotibial band friction syndrome that was diagnosed as a synovial sarcoma on further investigation.

  7. Intimal sarcoma of the pulmonary artery--diagnostic challenge.

    Science.gov (United States)

    Fukuda, Wakako; Morohashi, Satoko; Fukuda, Ikuo

    2011-08-01

    Pulmonary artery intimal sarcoma is a rare tumour and the diagnosis is often delayed. We report the case of a woman with a primary pulmonary artery intimal sarcoma who presented with massive pulmonary embolism. The definitive diagnosis was elucidated after the patient's death by autopsy specimen. We discuss the diagnosis and lessons learned from this case.

  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...... multiple STS of the extremities and the trunk wall from 13 patients. Different histotypes were present with malignant fibrous histiocytomas/undifferentiated pleomorphic sarcomas being the predominant subtype. Results. aCGH profiling revealed genetic complexity with multiple gains and losses in all tumors....... In an unsupervised hierarchical cluster analysis, similar genomic profiles and close clustering between the first and subsequent STS were identified in 5 cases, suggesting metastatic disease, whereas the tumors from the remaining 8 patients did not cluster and showed only weak pairwise correlation, suggesting...

  9. Does the Prevalence of Spondylolysis and Spina Bifida Occulta Observed in Pediatric Patients Remain Stable in Adults?

    Science.gov (United States)

    Urrutia, Julio; Zamora, Tomas; Cuellar, Jorge

    2017-10-01

    Cross-sectional study. To compare the prevalence of lumbar spondylolysis and spina bifida occulta (SBO) in pediatric and adult populations. The prevalence of spondylolysis reported from radiograph-based studies in children had been questioned in computed tomography (CT)-based studies in adults; however, a recent CT-based study in pediatric patients has confirmed the previously reported data in pediatric populations. SBO, which has been associated with spondylolysis, has demonstrated a decreasing prevalence with increasing age during childhood and adolescence. No studies have compared the prevalence of spondylolysis and SBO in pediatric and adult patients using CT as a screening method. We studied 228 pediatric patients (4-15 y old) and 235 adults (30-45 y old) who underwent abdominal and pelvic CT scans for reasons not related to the spine. The entire lumbosacral spine was evaluated to detect the presence of spondylolysis and SBO. We compared the prevalence of spondylolysis and SBO in pediatric patients and adults. The prevalence of spondylolysis in pediatric patients was 3.5% (1.1%-5.9%), and in adults, it was 3.8% (1.7%-6.8%), P=1. The prevalence of SBO in pediatric subjects was 41.2% (34.8%-59.2%) and dropped to 7.7% (4.3%-11.5%) in adults, P<0.01. The male prevalence of SBO in pediatric patients was 51.4%, whereas it was 32.2% in females (P<0.01); this sex difference was not significant in the adult population (P=0.8). The prevalence of lumbar spondylolysis remained constant from pediatric age through adulthood. The prevalence of SBO decreased from 41.2% in children to 7.7% in adults; this finding suggests that closure of the vertebral arch may not be completed in early childhood in a large percentage of subjects.

  10. Lumbar Lordosis Minus Thoracic Kyphosis: Remain Constant in Adolescent Idiopathic Scoliosis Patients Before and After Correction Surgery.

    Science.gov (United States)

    Yang, Mingyuan; Yang, Changwei; Chen, Ziqiang; Wei, Xianzhao; Chen, Yuanyuan; Zhao, Jian; Shao, Jie; Zhu, Xiaodong; Li, Ming

    2016-03-01

    A retrospective study. To explore the relationship between the change of lumbar lordosis (LL) and thoracic kyphosis (TK) in AIS patients after correction surgery. TK tends to decrease in Lenke 1 and Lenke 2 AIS patients after correction surgery using pedicle screws, with the compensation of LL decrease. We hypothesize that lumbar lordosis minus thoracic kyphosis (LL-TK) remains constant after correction surgery to achieve the sagittal balance in AIS patients. Medical records of Lenke 1 or Lenke 2 AIS patients who received posterior correction surgery using pedicle screws in our hospital from January 2010 to January 2013 were reviewed. General characters of patients and radiological parameters were evaluated before the surgery and at two years' follow-up. Correlation analysis between TK and LL was conducted. LL-TK and the change of LL and TK were analyzed at preoperation and final follow-up. A total of 76 Lenke 1 and Lenke 2 AIS patients were included. Both TK and LL decreased significantly after correction surgery (P = 0.019 and P = 0.040, respectively). There were significant correlations between TK and LL before and after surgery, respectively (preoperative: r = 0.234, P = 0.042; postoperative: r = 0.310, P = 0.006). Preoperative and postoperative LL-TK was 23.80° and 25.09°, respectively, and no significant difference of LL-TK was observed (P = 0.372). The same tendency was observed in the change of LL and TK, and significant correlation was also found between the change of TK and LL (r = 0.626, P = 0.002). The same change of LL and TK and no significant difference in LL-TK indicated that LL-TK might be an important compensatory mechanism in keeping sagittal balance.

  11. Implementation of patient blood management remains extremely variable in Europe and Canada: the NATA benchmark project: An observational study.

    Science.gov (United States)

    Van der Linden, Philippe; Hardy, Jean-François

    2016-12-01

    Preoperative anaemia is associated with increased postoperative morbidity and mortality. Patient blood management (PBM) is advocated to improve patient outcomes. NATA, the 'Network for the advancement of patient blood management, haemostasis and thrombosis', initiated a benchmark project with the aim of providing the basis for educational strategies to implement optimal PBM in participating centres. Prospective, observational study with online data collection in 11 secondary and tertiary care institutions interested in developing PBM. Ten European centres (Austria, Spain, England, Denmark, Belgium, Netherlands, Romania, Greece, France, and Germany) and one Canadian centre participated between January 2010 and June 2011. A total of 2470 patients undergoing total hip (THR) or knee replacement, or coronary artery bypass grafting (CABG), were registered in the study. Data from 2431 records were included in the final analysis. Primary outcome measures were the incidence and volume of red blood cells (RBC) transfused. Logistic regression analysis identified variables independently associated with RBC transfusions. The incidence of transfusion was significantly different between centres for THR (range 7 to 95%), total knee replacement (range 3 to 100%) and CABG (range 20 to 95%). The volume of RBC transfused was significantly different between centres for THR and CABG. The incidence of preoperative anaemia ranged between 3 and 40% and its treatment between 0 and 40%, the latter not being related to the former. Patient characteristics, evolution of haemoglobin concentrations and blood losses were also different between centres. Variables independently associated with RBC transfusion were preoperative haemoglobin concentration, lost volume of RBC and female sex. Implementation of PBM remains extremely variable across centres. The relative importance of factors explaining RBC transfusion differs across institutions, some being patient related whereas others are related to

  12. AIDS-associated Kaposi's sarcoma is linked to advanced disease and high mortality in a primary care HIV programme in South Africa

    Directory of Open Access Journals (Sweden)

    Chu Kathryn M

    2010-07-01

    Full Text Available Abstract Background AIDS-associated Kaposi's sarcoma is an important, life-threatening opportunistic infection among people living with HIV/AIDS in resource-limited settings. In western countries, the introduction of combination antiretroviral therapy (cART and new chemotherapeutic agents has resulted in decreased incidence and improved prognosis of AIDS-associated Kaposi's sarcoma. In African cohorts, however, mortality remains high. In this study, we describe disease characteristics and risk factors for mortality in a public sector HIV programme in South Africa. Methods We analysed data from an observational cohort study of HIV-infected adults with AIDS-associated Kaposi's sarcoma, enrolled between May 2001 and January 2007 in three primary care clinics. Paper records from primary care and tertiary hospital oncology clinics were reviewed to determine the site of Kaposi's sarcoma lesions, immune reconstitution inflammatory syndrome stage, and treatment. Baseline characteristics, cART use and survival outcomes were extracted from an electronic database maintained for routine monitoring and evaluation. Cox regression was used to model associations with mortality. Results Of 6292 patients, 215 (3.4% had AIDS-associated Kaposi's sarcoma. Lesions were most commonly oral (65% and on the lower extremities (56%. One quarter of patients did not receive cART. The mortality and lost-to-follow-up rates were, respectively, 25 (95% CI 19-32 and eight (95% CI 5-13 per 100 person years for patients who received cART, and 70 (95% CI 42-117 and 119 (80-176 per 100 person years for patients who did not receive cART. Advanced T stage (adjusted HR, AHR = 5.3, p Patients with AIDS-associated Kaposi's sarcoma presented with advanced disease and high rates of mortality and loss to follow up. Risk factors for mortality included advanced Kaposi's sarcoma disease and lack of chemotherapy use. Contributing factors to the high mortality for patients with AIDS

  13. Hidden Pictures of Kaposi's Sarcoma in Psoriatic Lesions: A Diagnostic Challenge

    Science.gov (United States)

    Yoo, Jisook; Jo, Mingyul; Kim, Min-Soo; Choi, Kwang-Hyun; Park, Hyang-Joon

    2016-01-01

    Kaposi's sarcoma is a multifocal proliferative vascular tumor involving the skin and other organ and psoriasis is a chronic cutaneous disease with papules and plaques with white scale. Development of Kaposi's sarcoma in psoriasis patients has been reported rarely. A 71-year-old man presented with multiple brownish to violaceous plaques on both feet and arms which were found 4 months ago. The biopsy confirmed Kaposi's sarcoma. The patient was diagnosed with psoriasis vulgaris 10 years ago and Kaposi's sarcoma lesions developed between psoriatic plaques. We herein report a rare case of simultaneous occurrence of Kaposi's sarcoma and psoriasis vulgaris which need quite different treatment. PMID:27904275

  14. Full-term newborn after repeated ovarian tissue transplants in a patient treated for Ewing sarcoma by sterilizing pelvic irradiation and chemotherapy

    Science.gov (United States)

    Rodriguez-Wallberg, Kenny A; Karlström, Per-Olof; Rezapour, Masoumeh; Castellanos, Enrique; Hreinsson, Julius; Rasmussen, Carsten; Sheikhi, Mona; Ouvrier, Bettina; Bozóky, Béla; Olofsson, Jan I; Lundqvist, Monalill; Hovatta, Outi

    2015-01-01

    We report the first successful transplantation of cryopreserved ovarian cortical tissue into heavily irradiated tissues in a patient who had received sterilizing pelvic radiotherapy (54 Gy) and 40 weeks of intensive high-dose chemotherapy for the treatment of Ewing’s sarcoma 14 years earlier. Repeated transplantation procedures were required to obtain fully functional follicular development. Enlargement of the transplants over time and increase of the size of the uterus were demonstrated on sequential ultrasonographic examinations. Eggs of good quality that could be fertilized in vitro were obtained only after a substantial incremental increase of the amount of ovarian tissue transplanted. Single embryo replacement resulted in a normal pregnancy and the birth of a healthy child by cesarean section at full-term. No neonatal or maternal postoperative complications occurred. Women facing high-dose pelvic radiotherapy should not be systematically excluded from fertility preservation options, as is currently the trend. PMID:25545009

  15. Constrictive Pericarditis in the Presence of Remaining Remnants of a Left Ventricular Assist Device in a Heart Transplanted Patient

    Directory of Open Access Journals (Sweden)

    R. Rivinius

    2015-01-01

    Full Text Available Constrictive pericarditis (CP is a severe subform of pericarditis with various causes and clinical findings. Here, we present the unique case of CP in the presence of remaining remnants of a left ventricular assist device (LVAD in a heart transplanted patient. A 63-year-old man presented at the Heidelberg Heart Center outpatient clinic with progressive dyspnea, fatigue, and loss of physical capacity. Heart transplantation (HTX was performed at another heart center four years ago and postoperative clinical course was unremarkable so far. Pharmacological cardiac magnetic resonance imaging (MRI stress test was performed to exclude coronary ischemia. The test was negative but, accidentally, a foreign body located in the epicardial adipose tissue was found. The foreign body was identified as the inflow pump connection of an LVAD which was left behind after HTX. Echocardiography and cardiac catheterization confirmed the diagnosis of CP. Surgical removal was performed and the epicardial tubular structure with a diameter of 30 mm was carefully removed accompanied by pericardiectomy. No postoperative complications occurred and the patient recovered uneventfully with a rapid improvement of symptoms. On follow-up 3 and 6 months later, the patient reported about a stable clinical course with improved physical capacity and absence of dyspnea.

  16. 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.

  17. Combination Therapy for Advanced Kaposi Sarcoma

    Science.gov (United States)

    In this clinical trial, adult patients with any form of advanced Kaposi sarcoma will be treated with liposomal doxorubicin and bevacizumab every 3 weeks for a maximum of six treatments.  Patients who respond to this therapy or have stable disease will rec

  18. Soft tissue sarcoma : why not treated?

    NARCIS (Netherlands)

    Farshadpour, F; Schaapveld, M; Suurmeijer, AJH; Wymenga, ANM; Otter, R; Hoekstra, HJ

    2005-01-01

    Background : Soft tissue sarcomas (STS) are uncommon malignancies and elderly STS patients have been reported to receive less definitive treatment compared to young STS patients. The present study was performed to investigate whether withholding treatment was based on disease specific aspects, patie

  19. Pulmonary Kaposi Sarcoma with Osseous Metastases in an Human Immunodeficiency Virus (HIV) Patient: A Remarkable Response to Highly Active Antiretroviral Therapy

    Science.gov (United States)

    Dirweesh, Ahmed; Khan, Muhammad Yasir; Hamiz, Shaikh Fawad; Karabulut, Nigahus

    2017-01-01

    Patient: Male, 34 Final Diagnosis: Pulmonary Kaposi’s sarcoma with bony metastatses Symptoms: Cough • weight loss Medication: — Clinical Procedure: — Specialty: Infectious Diseases Objective: Rare disease Background: Kaposi sarcoma (KS) is known to involve the mucocutaneous tissues and the aero-digestive tracts. In acquired immune deficiency syndrome (AIDS) patients, KS has an aggressive course and carries poor prognosis. We present a case of pulmonary KS with osseous metastases as the first presentation of human immunodeficiency virus (HIV) infection in a young male. The lesions impressively decreased in size and numbers following initiation of highly active antiretroviral therapy (HAART). Case Report: A 34-year-old heterosexual male presented with a one month history of cough and 15–20 pound weight loss within six months. Examination revealed oral thrush, decreased breath sounds and crackles on the right lower lung base. Imaging showed a large right perihilar mass with multiple lytic lesions involving thoracic and lumber vertebrae, ribs, sternum, and clavicles. Blood and sputum cultures, smears for acid fast bacilli, and a QUANTIferon gold test were all negative. He tested positive for HIV and his CD4 count was 7 cells/uL. Bronchoscopy with biopsy was unrevealing. Pathology of the right hilar mass was diagnostic of KS. Following initiation of antiretroviral therapy his condition dramatically improved; repeat chest CT scan showed marked regression of the bony and pulmonary lesions. Conclusions: The dual action of HAART on the recovery of the immune system and against human herpes virus 8 (HHV-8) may essentially cause regression of KS lesions. PMID:28216610

  20. BCOR-CCNB3 (Ewing-like) sarcoma: a clinicopathologic analysis of 10 cases, in comparison with conventional Ewing sarcoma.

    Science.gov (United States)

    Puls, Florian; Niblett, Angela; Marland, Gillian; Gaston, Czar Louie L; Douis, Hassan; Mangham, D Chas; Sumathi, Vaiyapuri P; Kindblom, Lars-Gunnar

    2014-10-01

    BCOR-CCNB3 fusion transcripts resulting from an X-chromosomal paracentric inversion were recently identified in a series of unclassifiable soft tissue and bone sarcomas with Ewing sarcoma-like morphology. The morphologic and clinical features of these sarcomas are, as yet, not well characterized. Here we describe the clinicopathologic features of 10 cases of BCOR-CCNB3 sarcoma and compare their clinical course with typical Ewing sarcoma. Nine of 10 patients were male, and all were 11 to 18 years of age. Seven tumors were located in the bone and 3 in the deep soft tissues. The histomorphologic spectrum was quite wide, with 7 tumors predominately showing small primitive cell morphology with angulated nuclei simulating so-called atypical Ewing sarcoma and 3 predominately showing spindle cell morphology. Recurrent and metastatic lesions showed increased cellularity and marked pleomorphism. Immunohistochemistry showed expression of CCNB3 (100%), bcl2 (90%), CD99 (60%), and CD117 (60%). Reverse transcription polymerase chain reaction for BCOR-CCNB3 fusion transcripts was positive in all 9 cases, which yielded sufficient extracted RNA. Five- and 10-year survival rates were 75% and 56%, respectively. BCOR-CCNB3 sarcomas located in axial skeleton and soft tissues showed a significantly shorter survival. The Ewing sarcoma overall survival was not statistically different, although there was a trend for longer survival of patients with BCOR-CCNB3 sarcomas in the extremities. In conclusion, this study provides a detailed description of the histologic spectrum, immunohistochemical features, and clinical characteristic of BCOR-CCNB3 sarcoma justifying distinction from Ewing sarcoma with its typical EWS/FUS-ETS translocations. Ideally immunohistochemistry is used in combination with reverse transcription polymerase chain reaction for definitive diagnosis.

  1. Primary Breast Sarcoma. A Case Report

    Directory of Open Access Journals (Sweden)

    Lidia Torres Ajá

    2013-06-01

    Full Text Available Primary breast sarcoma is the least frequent non-epithelial malignant tumour, representing less than 1 % of all breast cancers. It has a dismal prognosis with the presence of early metastases, mainly in the lungs and bones. Survival is very poor at 5 years. A case of a 79 year-old female patient with a large ulcerated sarcoma in the left breast is presented. The patient was examined in an interdisciplinary consultation, and the presence of a stromal sarcoma of the breast without apparent visceral or bone metastases was confirmed by an aspiration biopsy with thick needle, excisional biopsy by paraffin and by immunohistochemical studies. The publication of this report has clinical interest for health professionals due to the rarity of the disease.

  2. Remaining visual field and preserved subjective visual functioning prevent mental distress in patients with visual field defects

    Directory of Open Access Journals (Sweden)

    Carolin eGall

    2013-09-01

    Full Text Available Background: Patients with visual field defects after visual pathway lesion may experience reduced vision-related quality of life (vrQoL. It has not been clarified how vrQoL impairments contribute to vision-related mental distress.Methods: 108 subjects with visual field defects caused by optic neuropathies (age M=57.6; SD=13.7 years answered the National Eye Institute Visual-Functioning Questionnaire (NEI-VFQ for vrQoL and the SF-12 Short Form Health Survey for health-related quality of life (hrQoL. A ten item composite of NEI-VFQ visual functioning and five items of mental health symptoms due to vision problems were subjected to Rasch analysis. The test battery comprised static and High Resolution Perimetry (HRP. Regression and path analysis were used to investigate associations between QoL, mental distress and perimetry results.Results: A higher level of visual functioning was associated with monocular impairment and a larger remaining visual field compared to binocular impairment. Subjective visual functioning but not visual field parameters predicted mental health symptoms due to vision problems which was the only variable associated with the SF-12 mental component score. The SF-12 physical component score was less strongly associated with mental health symptoms due to vision problems. Here, reaction time in HRP and mean threshold in perimetry were additional significant variables. Path analysis revealed a significant path of remaining visual field via visual functioning on mental health. Conclusions: Subjective consequences of visual impairments in everyday life impact mental health rather than objective visual function loss as measured by perimetry. Since a higher extent of vrQoL was related to lower levels of mental distress, the maintenance of vrQoL could reduce and prevent mental distress due to vision problems. Patients with persisting visual field defects may benefit from neuropsychological rehabilitation and supportive therapies.

  3. Ewing sarcoma versus osteomyelitis: differential diagnosis with magnetic resonance imaging

    Energy Technology Data Exchange (ETDEWEB)

    Henninger, B.; Glodny, B.; Rudisch, A.; Trieb, T.; Loizides, A.; Judmaier, W.; Schocke, M.F. [Innsbruck Medical University, Department of Radiology, Innsbruck (Austria); Putzer, D. [Innsbruck Medical University, Department of Nuclear Medicine, Innsbruck (Austria)

    2013-08-15

    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.)

  4. What Are the Key Statistics about Kaposi Sarcoma?

    Science.gov (United States)

    ... Kaposi Sarcoma What Are the Key Statistics About Kaposi Sarcoma? Before the AIDS epidemic, Kaposi sarcoma (KS) was ... in Kaposi Sarcoma Research and Treatment? More In Kaposi Sarcoma About Kaposi Sarcoma Causes, Risk Factors, and Prevention ...

  5. What's New in Kaposi Sarcoma Research and Treatment?

    Science.gov (United States)

    ... Kaposi Sarcoma About Kaposi Sarcoma What’s New in Kaposi Sarcoma Research and Treatment? A great deal of research ... in Kaposi Sarcoma Research and Treatment? More In Kaposi Sarcoma About Kaposi Sarcoma Causes, Risk Factors, and Prevention ...

  6. (111)In-trastuzumab scintigraphy in HER2-positive metastatic breast cancer patients remains feasible during trastuzumab treatment.

    Science.gov (United States)

    Gaykema, Sietske B M; de Jong, Johan R; Perik, Patrick J; Brouwers, Adrienne H; Schröder, Carolien P; Oude Munnink, Thijs H; Bongaerts, Alphons H H; de Vries, Elisabeth G E; Lub-de Hooge, Marjolijn N

    2014-01-01

    Human epidermal growth factor receptor (HER)2 imaging with radiolabeled trastuzumab might support HER2-targeted therapy. It is, however, frequently questioned whether HER2 imaging is also possible during trastuzumab treatment as the receptor might be saturated. We studied the effect of trastuzumab treatment on 111In-trastuzumab uptake. Patients received trastuzumab weekly and paclitaxel once every 3 weeks. 111In-trastuzumab was injected on day 1 of cycle 1 and day 15 of cycle 4. Whole-body planar scintigraphy was acquired at different time points postinjection. Tumor uptake and organ distribution between the first and repeated scan series were calculated via residence times. Twenty-five tumor lesions in 12 patients were visualized on both scintigraphy series. Tumor uptake decreased (19.6%; p  =  .03). The residence times of normal organs remained similar except for the cardiac blood pool (+ 16.3%; p  =  .014). Trastuzumab treatment decreases tumor 111In-trastuzumab uptake around 20%. HER2 imaging is feasible during trastuzumab treatment.

  7. Primary Mediastinal Synovial Sarcoma Presenting as Superior Vena Cava Syndrome: A Rare Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Irappa Madabhavi

    2015-01-01

    Full Text Available Primary mediastinal sarcomas are aggressive tumors with a very rare incidence. This report describes the case of a 35-year-old male patient who presented with acute symptoms of dyspnoea, facial puffiness, voice-hoarseness, and engorged neck veins. With the clinical picture consistent with the superior vena cava (SVC syndrome, the patient was investigated with computed tomography of the chest. This revealed a large soft tissue density mass lesion compressing the SVC along with other critical superior mediastinal structures. Histopathological evaluation of the mass revealed features consistent with a soft tissue sarcoma and positive staining was observed for vimentin and S-100. Cytogenetic analysis by fluorescent in situ hybridisation (FISH demonstrated the t(X:18 translocation. Thus diagnosis was established as primary mediastinal synovial sarcoma. Patient was treated with three cycles of neoadjuvant chemotherapy, to which there was a partial response as per the RECIST criteria. Surgical excision of the mediastinal mass was performed, and further postoperative treatment with adjuvant chemoradiotherapy was provided. Patient currently is free of disease. This is to the best of our knowledge the first report in the world literature of a successfully treated case of “primary mediastinal sarcomas presenting as SVC syndrome.” Patient is under regular surveillance at our clinic and remains free of recurrence one year after treatment completion.

  8. [Historical Review of Kaposi sarcoma in pre-HAART era: evolution with different chemotherapy schedules and remission with ganciclovir use].

    Science.gov (United States)

    Volkow, Patricia; Jacquemin, Benedicte; Zinser, Juan W; Pérez-Padilla, Rogelio

    2016-10-01

    Ganciclovir has shown in vitro anti-human herpesvirus-8 activity, Kaposi sarcoma agent. We analyzed all Kaposi sarcoma patients from 1985 to 1996 pre-HAART era and identified Kaposi sarcoma/AIDS patients who achieved complete remission prior to HAART use.

  9. The roles and implications of exosomes in sarcoma.

    Science.gov (United States)

    Min, Li; Shen, Jacson; Tu, Chongqi; Hornicek, Francis; Duan, Zhenfeng

    2016-09-01

    Better diagnostic biomarkers and therapeutic options are still necessary for patients with sarcomas due to the current limitations of diagnosis and treatment. Exosomes are small extracellular membrane vesicles that are released by various cells and are found in most body fluids. Tumor-derived exosomes have been proven to mediate tumorigenesis, intercellular communication, microenvironment modulation, and metastasis in different cancers, including in sarcomas. Recently, exosomes have been considered as potential biomarkers for sarcoma diagnosis and prognosis, and as possible targets for sarcoma therapy. Moreover, due to their specific cell tropism and bioavailability, exosomes can also be engineered as vehicles for drug delivery. In this review, we discuss recent advances in the roles of tumor-derived exosomes in sarcoma and their potential clinical applications.

  10. 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

    The aim of the study is to assess the prognostic value of different volume-based calculations of tumor metabolic activity in the initial assessment of patients with high-grade bone sarcomas (BS) and soft tissue sarcomas (STS) using F-18 FDG PET/CT.A single-site, retrospective study from 2002...... to 2012 including 92 patients with histologically verified high-grade BS (N = 37) or STS (N = 55). All patients underwent a pretreatment F-18 FDG PET/CT scan. Clinical data were registered. Measurements of the accuracy of metabolic tumor volume with a preset threshold of 40% of the maximum standardized.......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...

  11. The Value of Surgery for Retroperitoneal Sarcoma

    Science.gov (United States)

    Gholami, Sepideh; Jacobs, Charlotte D.; Kapp, Daniel S.; Parast, Layla M.; Norton, Jeffrey A.

    2009-01-01

    Introduction. Retroperitoneal sarcomas are uncommon large malignant tumors. Methods. Forty-one consecutive patients with localized retroperitoneal sarcoma were retrospectively studied. Results. Median age was 58 years (range 20–91 years). Median tumor size was 17.5 cm (range 4–41 cm). Only 2 tumors were <5 cm. Most were liposarcoma (44%) and high-grade (59%). 59% were stage 3 and the rest was stage 1. Median followup was 10 months (range 1–106 months). Thirty-eight patients had an initial complete resection; 15 (37%) developed recurrent sarcoma and 12 (80%) had a second complete resection. Patients with an initial complete resection had a 5-year survival of 46%. For all patients, tumor grade affected overall survival (P = .006). Complete surgical resection improved overall survival for high-grade tumors (P = .03). Conclusions. Tumor grade/stage and complete surgical resection for high-grade tumors are important prognostic variables. Radiation therapy or chemotherapy had no significant impact on overall or recurrence-free survival. Complete surgical resection is the treatment of choice for patients with initial and locally recurrent retroperitoneal sarcoma. PMID:19826633

  12. The Value of Surgery for Retroperitoneal Sarcoma

    Directory of Open Access Journals (Sweden)

    Sepideh Gholami

    2009-01-01

    Full Text Available Introduction. Retroperitoneal sarcomas are uncommon large malignant tumors. Methods. Forty-one consecutive patients with localized retroperitoneal sarcoma were retrospectively studied. Results. Median age was 58 years (range 20–91 years. Median tumor size was 17.5 cm (range 4–41 cm. Only 2 tumors were <5 cm. Most were liposarcoma (44% and high-grade (59%. 59% were stage 3 and the rest was stage 1. Median followup was 10 months (range 1–106 months. Thirty-eight patients had an initial complete resection; 15 (37% developed recurrent sarcoma and 12 (80% had a second complete resection. Patients with an initial complete resection had a 5-year survival of 46%. For all patients, tumor grade affected overall survival (=.006. Complete surgical resection improved overall survival for high-grade tumors (=.03. Conclusions. Tumor grade/stage and complete surgical resection for high-grade tumors are important prognostic variables. Radiation therapy or chemotherapy had no significant impact on overall or recurrence-free survival. Complete surgical resection is the treatment of choice for patients with initial and locally recurrent retroperitoneal sarcoma.

  13. Generalized intramuscular granulocytic sarcoma mimicking polymyositis

    Energy Technology Data Exchange (ETDEWEB)

    Fritz, Jan; Claussen, Claus D.; Pereira, Philippe L.; Horger, Marius S. [Eberhard-Karls-University, Department of Diagnostic Radiology, Tuebingen (Germany); Vogel, Wichard [Eberhard-Karls-University, Department of Internal Medicine-Oncology, Tuebingen (Germany); Wehrmann, Martin [Eberhard-Karls-University, Department of Pathology, Tuebingen (Germany)

    2007-10-15

    We report a case of granulocytic sarcoma exclusively manifesting as diffuse intramuscular infiltration of the proximal upper and lower limb girdle and the torso muscles in a patient with previous history of acute myelogenous leukemia 5a. Whole-body CT showed widespread distribution of ill-defined intramuscular, homogeneously enhancing lesions. On whole-body MRI, lesions were homogeneously hyperintense on fat saturated T2-weighted images, isointense on T1-weighted images and strongly enhancing after intravenous gadolinium contrast administration. Histopathology revealed muscular infiltration of blast cells with identical immunochemistry to the initial manifestation of leukemia, diagnostic for an extramedullary relapse manifesting as granulocytic sarcoma. CT and MRI characteristics of this previously undocumented manifestation of granulocytic sarcoma should assist in the identification of such cases. (orig.)

  14. Comparison of clinical features and outcomes in patients with extraskeletal versus skeletal localized Ewing sarcoma: A report from the Children's Oncology Group.

    Science.gov (United States)

    Cash, Thomas; McIlvaine, Elizabeth; Krailo, Mark D; Lessnick, Stephen L; Lawlor, Elizabeth R; Laack, Nadia; Sorger, Joel; Marina, Neyssa; Grier, Holcombe E; Granowetter, Linda; Womer, Richard B; DuBois, Steven G

    2016-10-01

    The prognostic significance of having extraskeletal (EES) versus skeletal Ewing sarcoma (ES) in the setting of modern chemotherapy protocols is unknown. The purpose of this study was to compare the clinical characteristics, biologic features, and outcomes for patients with EES and skeletal ES. Patients had localized ES and were treated on two consecutive protocols using five-drug chemotherapy (INT-0154 and AEWS0031). Patients were analyzed based on having an extraskeletal (n = 213) or skeletal (n = 826) site of tumor origin. Event-free survival (EFS) was estimated using the Kaplan-Meier method, compared using the log-rank test, and modeled using Cox multivariate regression. Patients with extraskeletal ES (EES) were more likely to have axial tumors (72% vs. 55%; P 8 cm (9% vs. 17%; P rate were independently associated with inferior EFS. Clinical characteristics, but not key tumor genomic features, differ between EES and skeletal ES. Extraskeletal origin is a favorable prognostic factor, independent of age, race, and primary site. © 2016 Wiley Periodicals, Inc.

  15. T4/T8 ratio and absolute T4 cell numbers in different clinical stages of Kaposi's sarcoma in AIDS.

    Science.gov (United States)

    Spornraft, P; Fröschl, M; Ring, J; Meurer, M; Goebel, F D; Ziegler-Heitbrock, H W; Riethmüller, G; Braun-Falco, O

    1988-07-01

    Thirty-seven men (36 homosexual or bisexual and one heterosexual) with epidemic Kaposi's sarcoma and underlying HIV infection were followed up over a period of up to 32 months. Fourteen patients (38%) died, with a median survival time of 7.2 months after the diagnosis of AIDS. Seventeen patients (46%) presented with one or more opportunistic infections, mostly Pneumocystis carinii pneumonia. Eighteen patients (49%) had lymphadenopathy syndrome according to the definition of the CDC. Using the Laubenstein-classification of Kaposi's sarcoma, all patients either remained stable or deteriorated, improvement was never observed. Absolute T4 lymphocyte counts and the T4/T8 ratio were not related to the disease stage. With the onset of B symptoms (systemic symptoms), however, the absolute T4 numbers and the T4/T8 ratio markedly decreased. Delayed type hypersensitivity also showed no relationship to the clinical stages of Kaposi's sarcoma. Thus, the clinical progression of Kaposi's sarcoma lesions seems to be largely independent of the immunological parameters investigated. However, the onset of B symptoms was observed to be related to changes in immune status.

  16. THE RESPONSE OF DISSEMINATED RETICULUM CELL SARCOMA TO THE INTRAVENOUS INJECTION OF COLLOIDAL RADIOACTIVE GOLD

    Energy Technology Data Exchange (ETDEWEB)

    Rubin, Philip; Levitt, Seymour H.

    1963-06-15

    Case histories of two patients treated with colloidal radiogold for diffuse reticulum cell sarcoma are presented. Further analysis of the method is suggested by the unusually long survival time of one of the patients. It was concluded that, although external radiotherapy remains the treatment of choice in localized reticulum cell sarcoma, intravenous colloidal radiogold may be a useful agent in lymphosarcomas with diffuse minute neoplastic liver and spleen involvements. Intravenous colloidal radiogold can produce bone marrow depression and thrombocytopenia which can lead to death. This factor tends to argue against therapeutic use of the agent. It is suggested that no more than 50 mC Au/sup 198/ intravenously should be used for treatment of this disease. (R.M.G.)

  17. AIDS-related primary Kaposi sarcoma of the nasopharynx.

    Science.gov (United States)

    Çelenk, Fatih; Yilmaz, Metin; Asal, Korhan; Ekinci, Özgür; Tokgöz, Nil

    2011-06-01

    Primary nasopharyngeal Kaposi sarcoma is extremely rare, as only 1 case has been previously reported in the literature. We report a new case, which occurred in a 37-year-old man with a known history of acquired immune deficiency syndrome (AIDS). The patient presented with complaints of recurrent epistaxis and postnasal hemorrhage. Endoscopic examination detected a bluish, smooth, firm, nonpulsatile mass in the nasopharyngeal wall. Histopathologic findings on biopsy were consistent with Kaposi sarcoma. The tumor was successfully treated with radiotherapy. Kaposi sarcoma should be considered in the differential diagnosis of any AIDS patient who presents with recurrent unilateral nasal bleeding.

  18. Pseudo-Kaposi sarcoma (acroangiodermatitis): occurring after bullous erysipelas.

    Science.gov (United States)

    Kutlubay, Zekayi; Yardimci, Gürkan; Engin, Burhan; Demirkesen, Cuyan; Aydin, Övgü; Khatib, Rashid; Tuzun, Yalçın

    2015-05-18

    Pseudo-Kaposi sarcoma is a benign reactive vascular proliferative disorder, which can be seen at any age. It occurs when the chronic venous pressure changes result in vascular proliferation in the upper and mid dermis. This disease is divided into two subtypes: the most frequent subtype is the Mali type and seen in early ages. The Mali type is seen in chronic venous insufficiency and in those patients with arteriovenous shunts. The rare subtype is the Stewart-Bluefarb type. This disease must be distinguished from Kaposi sarcoma because of their clinical resemblance. Herein, we present a patient with pseudo-Kaposi sarcoma, which developed after bullous erysipelas.

  19. Treatment of classical Kaposi's sarcoma with gemcitabine

    NARCIS (Netherlands)

    Brambilla, L; Labianca, R; Ferrucci, SM; Taglioni, M; Boneschi, [No Value

    2001-01-01

    Background: Several drugs are active in aggressive classical Kaposi's sarcoma (CKS); chemotherapeutic agents with fewer side-effects, more rapid response and able to overcome resistance to previous treatment are advisable when treating patients in a second line. Gemcitabine, an analogue of deoxycyti

  20. Resistance and perspectives in soft tissue sarcomas

    NARCIS (Netherlands)

    Komdeur, Rudy

    2003-01-01

    Soft tissue sarcomas are rare malignancies originating from mesenchymal origin. They may occur at any age, but the incidence increases with age: about 50% of the patients are over 60 years of age. A distinct peak incidence is made up by embryonal rhabdomyosarcomas that mostly afflict children at age

  1. The role of the CXCL12-CXCR4/CXCR7 axis in the progression and metastasis of bone sarcomas (Review).

    Science.gov (United States)

    Liao, Yu-Xin; Zhou, Cheng-Hao; Zeng, Hui; Zuo, Dong-Qing; Wang, Zhuo-Ying; Yin, Fei; Hua, Ying-Qing; Cai, Zheng-Dong

    2013-12-01

    Bone sarcomas, which comprise less than 1% of all human malignancies, are a group of relatively rare mesenchymal-derived tumors. They are mainly composed of osteosarcoma, chondrosarcoma and Ewing's sarcoma. In spite of advances in adjuvant chemotherapy and wide surgical resection, prognosis remains poor due to the high propensity for lung metastasis, which is the leading cause of mortality in patients with bone sarcomas. Chemokines are a superfamily of small pro-inflammatory chemoattractant cytokines which can bind to specific G protein-coupled seven-span transmembrane receptors. Chemokine 12 (CXCL12), also designated as stromal cell-derived factor-1 (SDF-1), is able to bind to its cognate receptors, chemokine receptor 4 (CXCR4) and chemokine receptor 7 (CXCR7), with high affinity. The binding of CXCL12 to CXCR4/CXCR7 stimulates the activation of several downstream signaling pathways that regulate tumor progression and metastasis. In this review, the structure and function of CXCL12 and its receptors, CXCR4 and CXCR7, as well as many factors affecting their expression are discussed. Phosphoinositide 3-kinase (PI3K) and mitogen-activated protein kinase (MAPK) pathways are the two most important downstream pathways regulated by the CXCL12-CXCR4/CXCR7 interaction. CXCR4 expression in bone sarcomas, including tumor cells and samples and the correlation between CXCR4/CXCR7 expression and the survival of patients with bone sarcomas are also discussed. In addition, we review the involvement of the CXCL12‑CXCR4/CXCR7 axis in the growth and metastasis of bone sarcomas and the targeting of this axis in preclinical studies.

  2. Genetic Profiling Differentiates Second Primary Tumors from Metastases in Adult Metachronous Soft Tissue Sarcoma

    Directory of Open Access Journals (Sweden)

    Josefin Fernebro

    2008-01-01

    Full Text Available 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 multiple STS of the extremities and the trunk wall from 13 patients. Different histotypes were present with malignant fibrous histiocytomas/undifferentiated pleomorphic sarcomas being the predominant subtype. Results. aCGH profiling revealed genetic complexity with multiple gains and losses in all tumors. In an unsupervised hierarchical cluster analysis, similar genomic profiles and close clustering between the first and subsequent STS were identified in 5 cases, suggesting metastatic disease, whereas the tumors from the remaining 8 patients did not cluster and showed only weak pairwise correlation, suggesting development of second primary STS. Discussion. The similarities and dissimilarities identified in the first and second STS suggest that genetic profiles can be used to distinguish soft tissue metastases from second primary STS. The demonstration of genetically different soft tissue sarcomas in the same patient suggests independent tumor origin and serves as a reminder to consider development of second primary STS, which has prognostic and therapeutic implications.

  3. Management of high-grade bone sarcomas over two decades: The Norwegian Radium Hospital experience

    Energy Technology Data Exchange (ETDEWEB)

    Hege Aksnes, Liv; Sundby Hall, Kirsten; Folleraas, Gunnar; Stenwig, Anna Elisabeth; Bjerkehagen, Bodil; Taksdal, Ingeborg; Winderen, Mette; Bruland, Oeyvind Sverre; Saeter, Gunnar [The Norwegian Radium Hospital, Montebello, Oslo (Norway)

    2006-02-15

    All cases of high-grade osteosarcoma (OS) (n=196) and Ewing's sarcoma of bone (ES) (n=56) treated at the Norwegian Radium Hospital in the period 1980-1999 were analyzed retrospectively. They were allocated to consecutive ten-year periods by their time of diagnosis. Patient and tumour characteristics have been relatively stable. Eighty percent of all patients received surgical treatment and the amputation rate decreased from 64% to 23%. The percentage of patients receiving chemotherapy has remained around 80%. The use of radiotherapy in primary treatment decreased gradually from 33% to 18%. Sarcoma specific survival (SSS) at five years for all patients increased significantly from 39% to 53%. Similar trends for improvement were seen for both OS and ES. In multivariate analysis, independent prognostic factors for improved SSS were non-metastatic disease at diagnosis, age under 40, extremity tumours, small tumours and treatment from 1995 onwards. No major new treatment options have emerged over these 20 years. The improved outcome appears partly to be due to refinements in the use of existing modalities and improved quality and integration of multidisciplinary approaches. Improved formalized organisation of the sarcoma group and annual audited reports of its patient and research activity may also have contributed to improved focus and performance.

  4. Myeloid sarcoma of the rib: An atypical isolated chest finding

    Directory of Open Access Journals (Sweden)

    Antonio Raucci

    2015-03-01

    Systemic treatment was administered and currently neither systemic nor local relapse has been identified. Our experience suggests surgical resection could be a valid treatment in isolated myeloid sarcoma patients.

  5. Radiation Therapy for Chloroma (Granulocytic Sarcoma)

    Energy Technology Data Exchange (ETDEWEB)

    Bakst, Richard; Wolden, Suzanne [Department of Radiation Oncology, Memorial Sloan-Kettering Cancer Center, New York, NY (United States); Yahalom, Joachim, E-mail: yahalomj@mskcc.org [Department of Radiation Oncology, Memorial Sloan-Kettering Cancer Center, New York, NY (United States)

    2012-04-01

    Objectives: Chloroma (granulocytic sarcoma) is a rare, extramedullary tumor of immature myeloid cells related to acute nonlymphocytic leukemia or myelodysplastic syndrome. Radiation therapy (RT) is often used in the treatment of chloromas; however, modern studies of RT are lacking. We reviewed our experience to analyze treatment response, disease control, and toxicity associated with RT to develop treatment algorithm recommendations for patients with chloroma. Patients and Methods: Thirty-eight patients who underwent treatment for chloromas at our institution between February 1990 and June 2010 were identified and their medical records were reviewed and analyzed. Results: The majority of patients that presented with chloroma at the time of initial leukemia diagnosis (78%) have not received RT because it regressed after initial chemotherapy. Yet most patients that relapsed or remained with chloroma after chemotherapy are in the RT cohort (90%). Thirty-three courses of RT were administered to 22 patients. Radiation subsite breakdown was: 39% head and neck, 24% extremity, 9% spine, 9% brain, 6% genitourinary, 6% breast, 3% pelvis, and 3% genitourinary. Median dose was 20 (6-36) Gy. Kaplan-Meier estimates of progression-free survival and overall survival in the RT cohort were 39% and 43%, respectively, at 5 years. At a median follow-up of 11 months since RT, only 1 patient developed progressive disease at the irradiated site and 4 patients developed chloromas at other sites. RT was well tolerated without significant acute or late effects and provided symptom relief in 95% of cases. Conclusions: The majority of patients with chloromas were referred for RT when there was extramedullary progression, marrow relapse, or rapid symptom relief required. RT resulted in excellent local disease control and palliation of symptoms without significant toxicity. We recommend irradiating chloromas to at least 20 Gy, and propose 24 Gy in 12 fractions as an appropriate regimen.

  6. 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.

  7. [Moritz Kaposi and his sarcoma].

    Science.gov (United States)

    van Kessel, Anne; Quint, Koen D

    2011-01-01

    Nowadays, Kaposi sarcoma is a multidisciplinary condition, not only observed by dermatologists. Since the HIV epidemic in the 80s and 90s of the last century, more insight into the aetiology of Kaposi sarcoma has been acquired. However, this sarcoma had already been described in 1872 by a Hungarian dermatologist named Moritz Kaposi (1832-1902). Kaposi described the entity as 'idiopathic multiple pigmented sarcoma of the skin'. This entity was an extraordinary diagnosis at that time, mostly observed in Jewish or Mediterranean men. In 1912, 10 years after the death of Moritz Kaposi, the entity name was changed to Kaposi sarcoma.

  8. Radiation therapy of epidemic kaposi sarcoma: the Henri-Mondor hospital experience (643 patients); Radiotherapie du sarcome de kaposi epidemique: l`experience de l`hopital Henri-Mondor (643 patients)

    Energy Technology Data Exchange (ETDEWEB)

    Belembaogo, E.; Kirova, Y.; Frikha, H.; Yu, S.; Piedbois, P.; Le Bourgeois, J.P. [Hopital Henri-Mondor, 94 - Creteil (France). Dept. de cancerologie

    1998-01-01

    From June 1986 to December 1996, 643 patients presenting with acquired immunodeficiency syndrome (AIDS)-related epidemic Kaposi`s sarcoma were treated with irradiation at the Oncology Department of Henri Mondor University Hospital. Three-hundred eighty-seven patients (60 %) had previously received a treatment with interferon (259 patients, 40.2 %), vinblastine (225 patients, 34.5 %), doxorubicin (22 patients, 3.4 %), bleomycin (19 patients, 2.9 %), and/or antiviral treatment (216, 33.5 %). The radiotherapy was delivered by 4 MeV OR 8 MeV electron beam for extended cutaneous fields and 45-100 kV x-ray for localized fields. The delivered dose was 20 Gy in 2 weeks (2.5 Gy/fraction, 4 fractions/week) followed by 2 weeks rest and second series of 10 Gy in 1 week. For oral cavity lesions, we used a series of 15.2 Gy was delivered (1.9 Gy/fraction, 4 fractions/week), followed for three patients by a 3 week rest and by a similar second series of 15.2 Gy.Six-hundred and twenty-one patients were evaluable and the objective response rate was 92 %, with e complete regression of clinical and functional symptoms for all patients. The skin tolerance was good, with 7.3 % grade I reactions, 69.3 % of grade II reactions, and 23.4 % grade III reactions. There was a correlation between recurrence rate and the occurrence of opportunistic infections. This analysis shows the efficacy of dose radiotherapy for treatment of epidemic Kaposi sarcoma. (author)

  9. A Decade in Banking Ewing Sarcoma: A Report from the Children's Oncology Group.

    Science.gov (United States)

    Borinstein, Scott C; Beeler, Natalie; Block, John J; Gorlick, Richard; Grohar, Patrick; Jedlicka, Paul; Krailo, Mark; Morris, Carol; Phillips, Sharon; Siegal, Gene P; Lawlor, Elizabeth R; Lessnick, Stephen L

    2013-01-01

    Outcomes for patients with metastatic and recurrent Ewing sarcoma remain poor and a better understanding of the biology of this malignancy is critical to the development of prognostic biomarkers and novel therapies. Therefore, the Children's Oncology Group (COG) has created tissue banking protocols designed to collect high quality, clinically annotated, tumor specimens that can be distributed to researchers to perform basic science and correlative investigation. Data from the COG Ewing sarcoma tissue banking protocols AEWS02B1 and its successor study AEWS07B1 were reviewed in this study. Six-hundred and thirty five patients were enrolled on AEWS02B1 and 396 patients have had tissue submitted to AEWS07B1. The average age of participation was 13.2 years. About 86% were less than 19 years old and only 6% were greater than 21 years of age at diagnosis. When compared to SEER data, approximately 18% of all cases and only 8% of all patients >20 years old diagnosed with Ewing sarcoma annually in the United States have had tumor banked. The majority of participants submitted formalin fixed, paraffin embedded, primary tumor and blood samples. In total, fresh frozen tissue was submitted for only 29% of cases. Only seven metastatic tumor samples have been collected. Although the COG has been successful in collecting tumor samples from patients newly diagnosed with Ewing sarcoma, fresh frozen tumor specimens from primary and metastatic disease are critically needed, especially from young adult patients, in order to conduct high quality basic science and translational research investigation with a goal of developing better treatments.

  10. A Decade in Banking Ewing Sarcoma: A Report from the Children’s Oncology Group

    Science.gov (United States)

    Borinstein, Scott C.; Beeler, Natalie; Block, John J.; Gorlick, Richard; Grohar, Patrick; Jedlicka, Paul; Krailo, Mark; Morris, Carol; Phillips, Sharon; Siegal, Gene P.; Lawlor, Elizabeth R.; Lessnick, Stephen L.

    2013-01-01

    Outcomes for patients with metastatic and recurrent Ewing sarcoma remain poor and a better understanding of the biology of this malignancy is critical to the development of prognostic biomarkers and novel therapies. Therefore, the Children’s Oncology Group (COG) has created tissue banking protocols designed to collect high quality, clinically annotated, tumor specimens that can be distributed to researchers to perform basic science and correlative investigation. Data from the COG Ewing sarcoma tissue banking protocols AEWS02B1 and its successor study AEWS07B1 were reviewed in this study. Six-hundred and thirty five patients were enrolled on AEWS02B1 and 396 patients have had tissue submitted to AEWS07B1. The average age of participation was 13.2 years. About 86% were less than 19 years old and only 6% were greater than 21 years of age at diagnosis. When compared to SEER data, approximately 18% of all cases and only 8% of all patients >20 years old diagnosed with Ewing sarcoma annually in the United States have had tumor banked. The majority of participants submitted formalin fixed, paraffin embedded, primary tumor and blood samples. In total, fresh frozen tissue was submitted for only 29% of cases. Only seven metastatic tumor samples have been collected. Although the COG has been successful in collecting tumor samples from patients newly diagnosed with Ewing sarcoma, fresh frozen tumor specimens from primary and metastatic disease are critically needed, especially from young adult patients, in order to conduct high quality basic science and translational research investigation with a goal of developing better treatments. PMID:23519678

  11. [Primary pulmonary artery sarcoma in 36-year-old women: 3-years follow-up after partial resection and radiotherapy].

    Science.gov (United States)

    Drożdż, Jarosław; Warchoł, Ewa; Fijuth, Jacek; Filipiak, Krzysztof; Spych, Michał; Maciejewski, Marek; Piestrzeniewicz, Katarzyna; Ludomir, Stafańczyk; Janaszek-Sitkowska, Hanna; Januszewicz, Andrzej; Zembala, Marian

    2013-01-01

    Intimal sarcoma of the heart and pulmonary artery is a very rare, malignant, primary tumour. The prognosis in patients with primary sarcoma of the pulmonary artery, including intimal sarcoma, is poor. We present the case and 3-years follow-up of 36-year-old woman who was successfully treated with surgical, partial resection of the tumour followed by radiotherapy.

  12. 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.

  13. Intracranial CNS Manifestations of Myeloid Sarcoma in Patients with Acute Myeloid Leukemia: Review of the Literature and Three Case Reports from the Author’s Institution

    Directory of Open Access Journals (Sweden)

    Gustavo M. Cervantes

    2015-05-01

    Full Text Available Myeloid sarcoma (MS of the central nervous system (CNS is a rare presentation of leukemic mass infiltration outside of the bone marrow. It may involve the subperiosteum and dura mater and, on rare occasions, can also invade the brain parenchyma. The disease is most commonly seen in children or young adults; however, it has been described in multiple age groups. MS can be seen in patients with acute myeloid leukemia (AML, chronic myeloid leukemia and other myeloproliferative disorders. This entity has the potential to be underdiagnosed if the MS appearance precedes the first diagnosis of leukemia. The main reason is that their appearance on CT and MRI has a broad differential diagnosis, and proper diagnosis of MS can only be made if the imaging findings are correlated with the clinical history and laboratory findings. Herein, we describe the intracranial CNS manifestations of MS in patients with AML on CT and MRI involving the brain and/or meninges. This study is based on a systematic review of the literature. In addition, three case reports from the author’s institution with AML and intracranial involvement of MS are included. Our aim is to enhance the awareness of this entity among both clinicians and radiologists.

  14. Role of radiotherapy in local control of non-AIDS associated Kaposi's sarcoma patients in Korea: a single institution experience

    Energy Technology Data Exchange (ETDEWEB)

    Chang, Ji Hyun; Kim, Il Han [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    2012-12-15

    There has been no definite consensus on standard treatment, either local or systemic, for the Kaposi's sarcoma (KS). Radiotherapy (RT) can be a good local therapeutic choice especially in non-AIDS associated KS (NAKS) for its indolent behavior. Medical records of 17 KS patients treated with RT at the Seoul National University Hospital from February 1998 to January 2012 were retrospectively reviewed. One human immunodeficiency virus (HIV)+ patient with 3 lesions was excluded. The total number of the lesion was 23 among the 16 patients. The median follow-up period was 27.9 months. Correlation between response and variables was analyzed using the logistic regression model. Median age of the patients was 75 years. All the 23 lesions were located at the extremities. Fourteen (61%) of those had pain or local swelling as the initial presentation. Ten patients had possible causes of immunodeficiency and were regarded as iatrogenic, and other 6 were classic KS. Median dose of RT was 36 Gy. No KS-related death was observed. Excluding 2 with short-term follow-up only, complete response and partial response were obtained in 2 (9%) and 19 (73%) lesions, respectively. Of those, 3 lesions underwent local progression. Six had out-of-field recurrence after RT. Symptom improvement was achieved in 13 (93%) of 14 patients. Grade 2 skin toxicities were found in 9 lesions but all got improvement after treatment. When divided into responsive and progressive group, free from progression was not related to any of the possible variables. RT is effective in local control of NAKS resulting great response rate.

  15. Diagnostic accuracy of {sup 18}F-FDG-PET and PET/CT in patients with Ewing sarcoma family tumours: a systematic review and a meta-analysis

    Energy Technology Data Exchange (ETDEWEB)

    Treglia, Giorgio [Institute of Nuclear Medicine, Catholic University of the Sacred Heart, Department of Bioimaging and Radiological Sciences, Rome (Italy); Institute of Nuclear Medicine, Positron Emission Tomography Centre, Catholic University of the Sacred Heart, Department of Bioimaging and Radiological Sciences, Rome (Italy); Salsano, Marco; Stefanelli, Antonella; Mattoli, Maria Vittoria; Giordano, Alessandro [Institute of Nuclear Medicine, Catholic University of the Sacred Heart, Department of Bioimaging and Radiological Sciences, Rome (Italy); Bonomo, Lorenzo [Institute of Radiology, Catholic University of the Sacred Heart, Department of Bioimaging and Radiological Sciences, Rome (Italy)

    2012-03-15

    To systematically review and meta-analyse literature data on the diagnostic performance of fluorine-18-fluorodeoxyglucose (FDG) positron emission tomography (PET) and positron emission tomography/computed tomography (PET/CT) in patients with Ewing sarcoma family tumours (ESFT). PubMed/MEDLINE, Embase and Scopus databases were searched for articles that evaluated FDG-PET and PET/CT in patients with ESFT from inception to 31 May 2011. Studies that fulfilled the three following criteria were included in the systematic review: FDG-PET or PET/CT performed in patients with ESFT; articles about the diagnostic accuracy of FDG-PET and PET/CT; sample size of at least 10 patients with ESFT were included. Studies in which there were sufficient data to reassess sensitivity and specificity of FDG-PET or PET/CT in ESFT were included in the meta-analysis, excluding duplicate publications. Finally, pooled sensitivity, pooled specificity and area under the receiver operating characteristic (ROC) curve of FDG-PET or PET/CT in ESFT were calculated. We found 13 studies comprising a total of 342 patients with ESFT. The main findings of the studies included are presented. The meta-analysis of five selected studies provided these results about FDG-PET and PET/CT in ESFT: pooled sensitivity: 96% (95% confidence interval [CI] 91-99%); pooled specificity: 92% (95% CI 87-96%); area under the ROC curve: 0.97. With regard to the staging and restaging of patients with ESFT, the sensitivity, specificity and accuracy of FDG-PET and PET/CT are high; the combination of FDG-PET or PET/CT with conventional imaging is a valuable tool for the staging and restaging of ESFT and has a relevant impact on the treatment strategy plan. (orig.)

  16. HIV-associated cutaneous Kaposi`s sarcoma - palliative local treatment by radiotherapy

    Energy Technology Data Exchange (ETDEWEB)

    Saran, F.H. [Dept. of Radiotherapy and Oncology, Univ. Hospital, Frankfurt Univ. (Germany); Adamietz, I.A. [Dept. of Radiotherapy and Oncology, Univ. Hospital, Frankfurt Univ. (Germany); Thilmann, C. [Dept. of Radiotherapy and Oncology, Univ. Hospital, Frankfurt Univ. (Germany); Mose, S. [Dept. of Radiotherapy and Oncology, Univ. Hospital, Frankfurt Univ. (Germany); Boettcher, H.D. [Dept. of Radiotherapy and Oncology, Univ. Hospital, Frankfurt Univ. (Germany)

    1997-07-01

    The increasing number of HIV-infected patients makes palliative treatment of HIV-associated Kaposi`s sarcoma more common. We retrospectively evaluated a reduced fractionated radiotherapy with 20 Gy in respect to response rates and acute side-effects. From January 1992 to January 1995, 52 patients with HIV-associated Kaposi`s sarcoma were treated with 133 single portals. Six weeks after the end of radiotherapy 42 patients with 124 portals were evaluable with respect to response rates and side-effects. Of the treated portals 32% were judged as complete responses (CR), 55% as partial responses (PR) and 12% as no change (NC). Skin reactions RTOG, grade 1 were seen in 74% of the patients. Compared with literature data the reduced overall dose of 20 Gy in 10 fractions led to a reduction of CRs by approximately 50% while the overall response rate remained equal. The success of radiotherapy for the nodular component of Kaposi`s sarcoma can be improved, if a dose exceeding 20 Gy in 10 fractions is applied but at the cost of increasing side-effects in case that non-conventional fractionation schemes are used. (orig.).

  17. Vascularized Fibula Grafts for Reconstruction of Bone Defects after Resection of Bone Sarcomas

    Science.gov (United States)

    Petersen, Michael Mørk; Hovgaard, Dorrit; Elberg, Jens Jørgen; Rechnitzer, Catherine; Daugaard, Søren; Muhic, Aida

    2010-01-01

    We evaluated the results of limb-sparing surgery and reconstruction of bone defects with vascularized fibula grafts in 8 consecutive patients (mean age at operation 13.6 years (range 4.1–24.2 years), female/male = 6/2) with bone sarcomas (BS) (osteosarcoma/Ewing's sarcoma/chondrosarcoma= 4/3/1) operated on form 2000 to 2006. The bone defects reconstructed were proximal femoral diaphysis and epiphysis (n = 2), humeral diaphysis (n = 2), humeral proximal diaphysis and epiphysis (n = 1), femoral diaphysis (n = 1), ulnar diaphysis (n = 1), and tibial diaphysis (n = 1). One patient with Ewing's sarcoma had an early hip disarticulation, developed multiple metastases, and died 9 months after the operation. The remaining patients (n = 7) are all alive 50 months (range 26–75 months) after surgery. During the follow-up the following major complications were seen: 1-2 fractures (n = 4), pseudarthrosis (n = 2), and hip dislocation (n = 1). Limb-sparing surgery with reconstruction of bone defects using vascularized fibular grafts in BS cases is feasible with acceptable clinical results, but fractures should be expected in many patients. PMID:20490263

  18. Intraoperative Electron-Beam Radiation Therapy for Pediatric Ewing Sarcomas and Rhabdomyosarcomas: Long-Term Outcomes

    Energy Technology Data Exchange (ETDEWEB)

    Sole, Claudio V., E-mail: csole@iram.cl [Department of Radiation Oncology, Instituto de Radiomedicina, Santiago (Chile); School of Medicine, Complutense University, Madrid (Spain); Calvo, Felipe A. [School of Medicine, Complutense University, Madrid (Spain); Department of Oncology, Hospital General Universitario Gregorio Marañón, 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); Gonzalez, Carmen [School of Medicine, Complutense University, Madrid (Spain); Service of Radiation Oncology, Hospital General Universitario Gregorio Marañón, Madrid (Spain); Desco, Manuel [School of Medicine, Complutense University, Madrid (Spain); Department of Experimental Surgery and Medicine, Hospital General Universitario Gregorio Marañón, Madrid (Spain); Martinez-Monge, Rafael [Service of Radiation Oncology, Clínica Universidad de Navarra, Pamplona (Spain)

    2015-08-01

    Purpose: To assess long-term outcomes and toxicity of intraoperative electron-beam radiation therapy (IOERT) in the management of pediatric patients with Ewing sarcomas (EWS) and rhabdomyosarcomas (RMS). Methods and Materials: Seventy-one sarcoma (EWS n=37, 52%; RMS n=34, 48%) patients underwent IOERT for primary (n=46, 65%) or locally recurrent sarcomas (n=25, 35%) from May 1983 to November 2012. Local control (LC), overall survival (OS), and disease-free survival were estimated using Kaplan-Meier methods. For survival outcomes, potential associations were assessed in univariate and multivariate analyses using the Cox proportional hazards model. Results: After a median follow-up of 72 months (range, 4-310 months), 10-year LC, disease-free survival, and OS was 74%, 57%, and 68%, respectively. In multivariate analysis after adjustment for other covariates, disease status (P=.04 and P=.05) and R1 margin status (P<.01 and P=.04) remained significantly associated with LC and OS. Nine patients (13%) reported severe chronic toxicity events (all grade 3). Conclusions: A multimodal IOERT-containing approach is a well-tolerated component of treatment for pediatric EWS and RMS patients, allowing reduction or substitution of external beam radiation exposure while maintaining high local control rates.

  19. A Cases of AIDS Patient Related Kaposi' s Sarcoma%艾滋病相关型Kaposi肉瘤1例

    Institute of Scientific and Technical Information of China (English)

    付学敬; 徐彦春; 杜华; 许旭峰

    2012-01-01

    患者男,32岁.口腔上颚出现褐色增生物、面颈部及阴茎出现褐色斑块和结节4个月,周身乏力3个月.皮损组织病理示:真皮血管扩张,充血明显,部分呈出芽状生长,间质内可见小灶性梭型细胞.血清抗HIV抗体阳性;RPR(-);球蛋白52.2g/L;免疫全项:CD4:12细胞/μL; CD8:430细胞/μL; CD4/CD8:0.03;β微球蛋白6.4mg/L; A/G:0.7;WBC:1.4× 109/L,L:0.5×109/L.诊断:艾滋病相关型Kaposi肉瘤.%A 32-year-old male presented with brown lump on his Palate, and brown plaques , nodules on his face and penis about 4 months, and also felt lack power for 3 months. Histopathological examination showed that there were lots of vascular dilatation and congestion in the dermis, part of them presented budding shaped growth. There were small spindle cells gather together in the interstice. HIV (+); RPR(-); Globulins : 52.2 g/L; Immunonhistochemical examination showed: CD4: 12 cells /μL; CD8: 430 cells /μL; CD4/ CD8: 0.03; Beta microglobulin: 6.4mg/L; A/G 0.7; WBC 1.4×109/L; L 0.5×109/L. Diagnosis: AIDS related Kaposi's sarcoma.

  20. The diagnostic and prognostic value of ¹⁸F-FDG PET/CT in the initial assessment of high-grade bone and soft tissue sarcoma. A retrospective study of 89 patients

    DEFF Research Database (Denmark)

    Fuglø, Hanna Maria; Jørgensen, Simon Møller; Loft, Annika

    2012-01-01

    To evaluate the feasibility of (18)F-FDG PET/CT for initial assessment in high-grade bone sarcomas (BS) and soft tissue sarcomas (STS).......To evaluate the feasibility of (18)F-FDG PET/CT for initial assessment in high-grade bone sarcomas (BS) and soft tissue sarcomas (STS)....

  1. Postoperative External Beam Radiotherapy for Retroperitoneal Soft Tissue Sarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Jang, Na Yong; Kim, Il Han; Choi, Jin Hwa; Park, Charn Il [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    2008-09-15

    To evaluate the clinical outcomes and prognostic factors in retroperitoneal soft tissue sarcomas treated by postoperative radiotherapy. The records of 23 patients with retroperitoneal soft tissue sarcomas, who underwent postoperative radiotherapy between 1985 and 2003, were analyzed. The median follow-up period was 77 months (range, 8-240 months). A total of 21 patients presented with primary disease, and two patients presented with recurrent disease. Liposarcomas and leiomyosarcomas represented 78% of the diagnosed tumor cases. Moreover, 17 cases were of high grade (grade 2 or 3). The median tumor size was 13 cm (range, 3-50 cm). Complete excision was achieved in 65% of patients. The median radiation dose was 50.4 Gy (range, 45.0 to 59.4 Gy), with conventional fractionation. The 5-year overall, local recurrence-free, and distant metastasis-free survival rates were 68%, 58%, and 71%, respectively. Eleven patients experienced local recurrence, while 9 patients experienced distant metastasis. The most common site for distant metastasis was the liver. A univariate analysis revealed that adjacent organ invasion and age (>60 years) as the significant risk factors contributing to the prediction of poor overall survival. Moreover, multivariate analyses indicated that adjacent organ invasion remained significantly associated with a higher risk of death. In addition, patient age (>60 years) was the other identified risk factor for local recurrence by univariate and multivariate analyses. Except for one case of grade 3 diarrhea, no patient suffered grade 3 or higher complications. Our results were comparable to previous reports in that adjacent organ invasion and patient age (>60 years) were significant predictors of poor survival and tumor recurrence, respectively.

  2. Ewing Sarcoma of the Posterior Fossa in an Adolescent Girl

    Directory of Open Access Journals (Sweden)

    Andreas M. Stark

    2014-01-01

    Full Text Available Medulloblastoma, astrocytoma, and ependymoma represent the most common infratentorial tumors in childhood, while Ewing sarcomas in that localization are extremely rare. A large left infratentorial space-occupying lesion was diagnosed in a 12-year-old girl with signs of increased intracranial pressure. Following total tumor resection, histological and molecular examination revealed Ewing sarcoma with rearranged EWSR-1 gene. The patient achieved complete remission following adjuvant chemotherapy and radiotherapy according to Euro-EWING 2008 treatment protocol. Intracranial Ewing sarcoma, although rare, should be an important differential diagnosis of intracranial tumors in childhood which requires aggressive multimodal treatment.

  3. Osteogenic sarcoma with skeletal muscle metastases

    Energy Technology Data Exchange (ETDEWEB)

    Peh, W.C.G. [Department of Diagnostic Radiology, The University of Hong Kong, Queen Mary Hospital (Hong Kong); Shek, T.W.H. [Department of Pathology, The University of Hong Kong, Queen Mary Hospital (Hong Kong); Wang Shihchang [Department of Diagnostic Imaging, National University of Singapore, National University Hospital (Singapore); Wong, J.W.K.; Chien, E.P. [Department of Orthopaedic Surgery, The University of Hong Kong, Queen Mary Hospital (Hong Kong)

    1999-05-01

    Two cases of osteogenic sarcoma with skeletal muscle metastases are described. A 40-year-old woman presented with progressive swelling of both calves and a soft tissue back lump. She had been diagnosed with mandibular chondroblastic osteogenic sarcoma 6 years earlier. Radiographs showed calcified masses. MRI scans and bone scintigraphy revealed multiple soft tissue masses in both calves. Bone scintigraphy also showed uptake in the back lump, right thigh and left lung base. Biopsy confirmed metastatic chondroblastic osteogenic sarcoma, which initially responded well to chemotherapy. However, the metastatic disease subsequently progressed rapidly and she died 21 months after presentation. The second case concerns a 20-year-old man who presented with a pathologic fracture of the humerus, which was found to be due to osteoblastic osteogenic sarcoma. He developed cerebral metastases 17 months later, followed by metastases at other sites. Calcified masses were subsequently seen on radiographs of the abdomen and chest. CT scans confirmed the presence of densely calcified muscle metastases in the abdominal wall, erector spinae and gluteal muscles. The patient`s disease progressed rapidly and he died 30 months after presentation. (orig.) With 6 figs., 29 refs.

  4. 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.

  5. Impact of a New Nasal Pillows Mask on Patients' Acceptance, Compliance, and Willingness to Remain on CPAP Therapy.

    Science.gov (United States)

    Wimms, Alison; Ketheeswaran, Sahisha; Ziegenbein, Claus; Jennings, Laura; Woehrle, Holger

    2016-01-01

    Aim. Continuous positive airway pressure (CPAP) masks are a key factor in patient compliance. This program assessed the performance of a new nasal pillows mask (NPM) on a variety of new and established obstructive sleep apnea (OSA) patients using CPAP therapy. Methods. Five programs were developed to assess the new NPM [AirFit P10, ResMed] on naïve patients; patients established on another NPM; patients using a nasal mask; patients with low CPAP compliance; and patients who wished to stop using CPAP therapy. Results. A total of 212 patients were included. In naïve patients, CPAP usage after 3 months was 5.9 ± 1.7 hours/night, compared with the control group at 4.6 ± 2.4 hours/night (p CPAP, 60% continued with therapy using the new NPM. Conclusion. The new NPM mask performed well in a variety of clinical groups of OSA patients receiving CPAP therapy and shows that technical advances in CPAP masks can improve patient compliance.

  6. Extremity perfusion for sarcoma

    NARCIS (Netherlands)

    Hoekstra, Harald Joan

    2008-01-01

    For more than 50 years, the technique of extremity perfusion has been explored in the limb salvage treatment of local, recurrent, and multifocal sarcomas. The "discovery" of tumor necrosis factor-or. in combination with melphalan was a real breakthrough in the treatment of primarily irresectable ext

  7. Sarcoma Foundation of America

    Science.gov (United States)

    ... Google+ Twitter LinkedIn YouTube © 2017 Sarcoma Foundation of America | All Rights Reserved. | Terms of Use | Privacy Policy Website Design & Hosting by 270net Technologies, Inc. X - Enter Your Location - - or - Get your current location Home About Us History People Public Filings News & Media SFA in the ...

  8. Epidemic Kaposi Sarcoma

    Science.gov (United States)

    ... and its treatment, see the AIDSinfo website . Nonepidemic Gay-related Kaposi Sarcoma There is a type of ... better than another. Trials are based on past studies and what has been learned ... by their creator. In such cases, it is necessary to contact the writer, artist, ...

  9. Classic Kaposi Sarcoma

    Science.gov (United States)

    ... and its treatment, see the AIDSinfo website . Nonepidemic Gay-related Kaposi Sarcoma There is a type of ... better than another. Trials are based on past studies and what has been learned ... by their creator. In such cases, it is necessary to contact the writer, artist, ...

  10. Extremity perfusion for sarcoma

    NARCIS (Netherlands)

    Hoekstra, Harald Joan

    2008-01-01

    For more than 50 years, the technique of extremity perfusion has been explored in the limb salvage treatment of local, recurrent, and multifocal sarcomas. The "discovery" of tumor necrosis factor-or. in combination with melphalan was a real breakthrough in the treatment of primarily irresectable

  11. Synovial sarcoma mechanisms

    DEFF Research Database (Denmark)

    Svejstrup, Jesper Q

    2013-01-01

    Human synovial sarcoma is caused by a chromosome translocation, which fuses DNA encoding SSX to that encoding the SS18 protein. Kadoch and Crabtree now show that the resulting cellular transformation stems from disruption of the normal architecture and function of the human SWI/SNF (BAF) complex....

  12. Leukosis/Sarcoma Group

    Science.gov (United States)

    The leukosis/sarcoma (L/S) group of diseases designates a variety of transmissible benign and malignant neoplasms of chickens caused by members that belong to the family Retroviridae. Because the expansion of the literature on this disease, it is no longer feasible to cite all relevant publications ...

  13. Soft tissue sarcomas in adolescents and young adults: a comparison with their paediatric and adult counterparts.

    Science.gov (United States)

    van der Graaf, Winette T A; Orbach, Daniel; Judson, Ian R; Ferrari, Andrea

    2017-03-01

    Survival outcomes for adolescent and young adult patients with soft tissue sarcomas lag behind those of children diagnosed with histologically similar tumours. To help understand these differences in outcomes, we discuss the following issues with regard to the management of these patients with soft tissue sarcomas: delays in diagnosis, trial availability and participation, aspects of the organisation of care (with an emphasis on age-specific needs), national centralisation of sarcoma care, international consortia, and factors related to tumour biology. Improved understanding of the causes of the survival gap between adolescents and young adults with sarcomas will help drive new initiatives to improve final health outcomes in these populations. In this Review, we specifically focus on embryonal and alveolar rhabdomyosarcoma, synovial sarcoma, and adult soft tissue sarcomas diagnosed in adolescents and young adults, and discuss the age-specific needs of these patients. Copyright © 2017 Elsevier Ltd. All rights reserved.

  14. 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.

  15. Genotypic analysis on the ORF-K1 gene of human herpesvirus 8 from patients with Kaposi's sarcoma in Xinjiang, China

    Institute of Scientific and Technical Information of China (English)

    Dezhi Zhang; Xiongming Pu; Weidong Wu; Ying Jin; Xiujuan Wu

    2008-01-01

    Human herpesvirus 8 (HH'V-8) is thought to be essential for the development of all forms of Kaposi's sarcoma (KS). HHV-8 DNA is present virtually in all KS tumor biopsy samples. Genes at both ends of the HHV-8 genome have been shown to vary considerably. Seve nmajor molecular subtypes of HHV-8 were defined based on the amino acid sequence of the open reading frame K1 (ORF-Kl), generally known as A, B, C, D, E, E and Z. Most strains collected worldwide were clustered into two subtypes (A and C). Here, the Kl/VRl region of HHV-8 was amplified by nested PCR in 22 (81.48%) of 27 cases from Xinjiang Uygur Autonomous Region, a province in northwest-ern China. Phylogenetic analysis on the basis of the KI/VRI amino acid sequence indicated that the majority of these KS patients were infected by subtype C HHV-8 (n = 18, including 15 belonging to the C2 group), and several by subtype A (n = 4, including 3 being the Al group). This is the fast report of subtype A HHV-g in China. Furthermore, the correlations between different forms and lesions of KS and different subtypes of HHV-8 were analyzed. The findings showed that subtype A HHV-8 resulted in significantly more frequent mucosal KS lesions than subtype C. However, there was no obvious correlation between different forms of KS and different subtypes of HHV-8.

  16. False-Negative Results of Endoscopic Biopsy in the Diagnosis of Gastrointestinal Kaposi’s Sarcoma in HIV-Infected Patients

    Directory of Open Access Journals (Sweden)

    Naoyoshi Nagata

    2012-01-01

    Full Text Available Kaposi’s sarcoma (KS is a rare endothelial neoplasm mainly involving the skin, but it is often associated with AIDS. Diagnosis of gastrointestinal (GI tract KS, a common site of visceral involvement in AIDS, is important, but endoscopic biopsy carries a risk of false-negative results (FNRs due to its submucosal appearance. This study sought to determine the rate and causes of FNR for endoscopic biopsy of GI-KS lesions. Endoscopic biopsy samples of 116 GI-KS lesions were reviewed retrospectively. All GI-KS lesions were confirmed to be resolved following KS therapy. FNRs were yielded for 41 of the lesions (35.3%. Among upper and lower GI sites, the esophagus was the only site significantly associated with FNRs (P<0.01. Small size (<10 mm and patches found on endoscopy were significantly associated with FNRs (P<0.05. Findings of submucosal tumor (SMT with ulceration were significantly associated with true-positive results (P<0.05. In conclusion, FNRs were found in 35.3% of GI-KS lesions and were especially related to the site of the esophagus and endoscopic early stage (small size or patch appearance. An SMT with ulceration may be relatively easy to diagnose on endoscopic biopsy. Caution should be exercised when performing endoscopic biopsy of these lesions in AIDS patients and evaluating the histological features.

  17. Pulmonary Kaposi's sarcoma after heart transplantation: a case report

    Directory of Open Access Journals (Sweden)

    Kristiansen Glen

    2010-07-01

    Full Text Available Abstract Introduction Kaposi's sarcomas have been associated with different conditions of immunosuppression and are also known to be a typical complication of solid organ transplantations. Case presentation We report the case of a 65-year-old Turkish man with a history of heart transplantation 10 months ago who presented for clarification of his dyspnea. The patient had a known history of chronic obstructive pulmonary disease and a smoking history of 40 pack years. Radiologically, three progressively growing intra-pulmonary nodules were detected. The histology was diagnostic for a Kaposi's sarcoma. Visceral and especially primary intra-pulmonary Kaposi's sarcomas are very rare and have been described to have a rather unfavorable prognosis. Conclusions Even with a history suggestive for conventional lung cancer, Kaposi's sarcomas should be considered in patients after transplantation of solid organs. It should be noted that in a minority of cases this tumor exists in the absence of the typical cutaneous lesions.

  18. Treatment Outcomes and Prognostic Factors of Pulmonary Metastasectomy for Bone and Soft Tissue Sarcoma: a High Volume Academic Institution Experience

    Directory of Open Access Journals (Sweden)

    Xiaozheng KANG

    2016-05-01

    Full Text Available Background and objective The bone and soft tissue sarcoma can metastasize to distant sites, most commonly the lungs. Some cases can be cured by radical metastasectomy, but its role, indication and prognostic factors remains controversial. The rarity of the disease combined with the diverse number of subtypes can make bone and soft tissue sarcomas very difficult to study. There are few randomized control studies or international high volume results, and such reports in China are seldom seen. The aim of this study is to investigate surgical treatment outcomes and prognostic factors of pulmonary metastatic bone and soft tissue sarcoma patients. Methods From January 2007 to December 2015, patients with bone and soft tissue sarcoma who underwent multimodality therapy including definitive surgery for the primary lesion and at least one pulmonary metastasectomy were enrolled in the retrospective study. All the relevant clinical variables were collected, and then statistically analyzed and interpreted with the aid of univariate and multivariate Cox proportional hazard regression method. Results Totally 155 pulmonary metastasectomies in 144 patients were analyzed. Incomplete R0 resection, a less than 1-year interval from a previous surgery, more than three detected nodules; and the summed maximum diameter of more than 45 mm for pulmonary metastases were independent prognostic indicators by multivariate analysis. Conclusion We suggest that metastatic bone and soft tissue sarcoma patients can benefit most from aggressive surgical intervention of pulmonary metastasectomy. Its prognostic factors include R0 resection, a longer interval from a previous surgery, smaller total number and total size of pulmonary metastases.

  19. Evolution of drug resistance in HIV infected patients remaining on a virologically failing cART regimen

    DEFF Research Database (Denmark)

    Cozzi-Lepri, A; Phillips, AN; Ruiz, L

    2007-01-01

    OBJECTIVE: To estimate the extent of drug resistance accumulation in patients kept on a virologically failing regimen and its determinants in the clinical setting. DESIGN: The study focused on 110 patients of EuroSIDA on an unchanged regimen who had two genotypic tests performed at two time point...

  20. Fatores de risco de complicações pulmonares em pacientes com sarcoma após toracotomia para a ressecção de nódulos pulmonares Risk factors for pulmonary complications in patients with sarcoma after the resection of pulmonary nodules by thoracotomy

    Directory of Open Access Journals (Sweden)

    Rogério Santos Silva

    2010-12-01

    Full Text Available OBJETIVO: Identificar os fatores de risco para complicações pulmonares em pacientes com sarcoma após serem submetidos a toracotomia para a ressecção de nódulos pulmonares. MÉTODOS: Estudo de coorte retrospectivo com 68 pacientes consecutivos com diagnóstico de sarcomas e submetidos a 174 toracotomias para a ressecção de nódulos pulmonares. A variável dependente foi definida como a ocorrência de qualquer complicação pulmonar pós-operatória. As variáveis independentes foram relacionadas com o paciente, o diagnóstico de base e o tipo de procedimento cirúrgico. Os dados foram analisados segundo um modelo multivariado de estimação de equações generalizadas, com uma função de ligação logística e uma estrutura de correlação simétrica. RESULTADOS: Houve 24 complicações (13,8%; IC95%: 9,0-19,8, incluindo um óbito. Os pacientes que apresentaram complicações pós-operatórias tiveram um tempo médio de internação duas vezes superior àqueles sem complicações (18,8 ± 10,0 dias vs. 8,6 ± 6,0 dias; p OBJECTIVE: To identify the risk factors for pulmonary complications after thoracotomy for the resection of pulmonary nodules in patients with sarcoma. METHODS: A retrospective cohort study involving 68 consecutive patients diagnosed with sarcoma and submitted to a total of 174 thoracotomies for the resection of pulmonary nodules. The dependent variable was defined as the occurrence of any postoperative pulmonary complications. The independent variables were related to the patient, underlying diagnosis, and type of surgical procedure. We analyzed the data using a multivariate generalized estimating equations model with logistic link function and a symmetric correlation structure. RESULTS: Complications were observed in 24 patients (13.8%, 95% CI: 9.0-19.8, and there was one death. The mean length of hospital stay was twice as long in the patients with postoperative complications as in those without (18.8 ± 10.0 days vs

  1. Kaposi's Sarcoma: clinical and pathological aspects in patients seen at the Hospital Universitário Cassiano Antônio Moraes - Vitória - Espírito Santo - Brazil Sarcoma de Kaposi: achados clínico-patológicos nos pacientes atendidos no Hospital Universitário Cassiano Antônio Moraes - Vitória - Espírito Santo - Brazil

    Directory of Open Access Journals (Sweden)

    Ricardo Montibeler Tiussi

    2012-04-01

    Full Text Available BACKGROUND: Kaposi's sarcoma is a neoplasm of endothelial origin that is divided into four distinct types according to the clinical characteristics and the affected population: Classic (in elder men of Jewish or Mediterranean origin; Epidemic (in patients affected by AIDS; Endemic (in black African men and Iatrogenic (in patients under immunosuppressive regimens. Human herpesvirus 8 infection is essential but not sufficient for the sarcoma development. OBJECTIVE: To describe the epidemiological, clinical and histopathological aspects of patients with KS seen at the Dermatology Clinic -Cassiano Antônio Moraes University Hospital - Federal University of Espirito Santo, Vitória - ES. METHODS: A descriptive and retrospective study based on clinical charts of patients with KS seen at the Dermatology Clinic from 1986 to 2009. RESULTS: The majority of the 15 cases were male patients (93,3% and white (60%. Epidemic Kaposi's sarcoma occurred in 80%, and the Classic form in 20%, with no cases in the Endemic or Iatrogenic groups. All the histopatho logical exams of the cutaneous lesions were reviewed and a proliferation of fusiform cells, extravasated erythrocytes and vascular rifts among the largest vessels, assuming the "vessels in vessels" typical aspect, were seen. CONCLUSION: The number of cases of Kaposi's Sarcoma was linear throughout the years of the study, especially of the epidemic form, although the incidence and prevalence of AIDS increased in the state of Espírito Santo. Therefore, if we consider the relation between KS and AIDS, a decreasing line of Kaposi's sarcoma could be seen, especially after the introduction of HAART.FUNDAMENTOS: O Sarcoma de Kaposi é neoplasia de origem endotelial, dividida em quatro formas clínicas: clássica (homens idosos de origem judaica e mediterrânea, epidêmica (associada ao HIV, endêmica (negros africanos e iatrogênica (relacionada à imunossupressão. A infecção pelo herpes vírus humano tipo 8 (HHV

  2. MRI of perineural extramedullary granulocytic sarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Graham, A. [Rehabilitation Medicine, Hunters Moor Neurological Rehabilitation Centre, Newcastle-Upon-Tyne (United Kingdom); Hodgson, T. [Neuroradiology Dept., Royal Hallamshire Hospital, Sheffield (United Kingdom); Jacubowski, J. [Neurosurgical Dept., Royal Hallamshire Hospital, Sheffield (United Kingdom); Norfolk, D. [Haematology Department, Leeds General Infirmary, Leeds LS1 3EX (United Kingdom); Smith, C. [Pathology Dept., Royal Hallamshire Hospital, Sheffield (United Kingdom)

    2001-06-01

    Granulocytic sarcoma is an extramedullary solid tumour consisting of myelogenous leukaemic blast cells, usually seen in acute myeloid leukaemia and less commonly in patients with chronic myeloid leukaemia or myeloproliferative disorders. Blast cells have a predilection for periosteal and perineural regions and rarely precede evidence of systemic disease. We present two patients, aleukaemic on peripheral blood counts, both at presentation and during subsequent treatment. We present the MRI features of this rare but important condition. (orig.)

  3. Radiation Therapy for Soft Tissue Sarcomas

    Science.gov (United States)

    ... Stage Soft Tissue Sarcoma Treating Soft Tissue Sarcomas Radiation Therapy for Soft Tissue Sarcomas Radiation therapy uses ... spread. This is called palliative treatment . Types of radiation therapy External beam radiation therapy: For this treatment, ...

  4. Recent Progress in the Management of Retroperitoneal Sarcoma

    Directory of Open Access Journals (Sweden)

    Rona Cheifetz

    2001-01-01

    Full Text Available Retroperitoneal sarcomas (RPS are rare tumours that typically present late and carry a poor prognosis even following grossly complete resection. In an attempt to improve the outlook for patients with RPS, sarcoma specialists have employed various adjuvant therapies, including extermal beam radiation, intraoperative radiation, brachyradiation and systemic chemotherapy. This article reviews the presentation and prognosis of RPS, and focuses on the results of new treatment strategies compared with conventional management.

  5. Undifferentiated Pleomorphic Sarcoma in Mandible.

    Science.gov (United States)

    Kim, Chul-Hwan; Jang, Jong-Won; Kim, Moon-Young; Kim, Yong-Hwan; Kim, Hang-Gul; Kim, Joo-Hwan

    2014-11-01

    Undifferentiated pleomorphic sarcoma (UPS), previously known as malignant fibrous histiocytoma, occurs commonly in the soft tissues in adult, but is rare in the maxillofacial region. It consists of undifferentiated mesenchymal tumor cells resembling histiocytes and fibroblasts. The purpose of this article is to report a case of UPS in the mandible. A 44-year-old patient presented with a painful growing mass in the mandible of two months' duration. Computed tomography and positron emission tomography-computed tomography revealed an ill-defined heterogenous, hypermetabolic mass about 4 cm in size in the left mandible invading adjacent soft tissues. A left mandiblulectomy and reconstruction with a fibular free flap were performed. Immunohistochemical study gave a diagnosis of UPS. The patient was referred for adjuvant chemotherapy after surgical removal of the tumor.

  6. 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.)

  7. Immunostaining for SYT protein discriminates synovial sarcoma from other soft tissue tumors: analysis of 146 cases.

    Science.gov (United States)

    He, Rui; Patel, Rajiv M; Alkan, Serhan; Hammadeh, Rasheed; Weiss, Sharon W; Goldblum, John R; Venkataraman, Girish; Baila, Horea

    2007-05-01

    Synovial sarcoma in its classic biphasic form can be distinguished readily from other soft tissue lesions; however, monophasic and poorly differentiated forms are diagnostically more problematic. For this reason, we assessed the efficacy of immunostaining for SYT and SSX1 proteins, the gene products resulting from unique synovial sarcoma translocation, to distinguish synovial sarcoma from other soft tissue lesions. A total number of 146 cases were analyzed, including 47 synovial sarcoma cases (all of which were verified by FISH to have t(X; 18) translocation and SYT-SSX fusion gene) and 99 soft tissue tumors of various types. A polyclonal IgG antibody against SYT was used to stain formalin-fixed paraffin embedded tissues. Forty-one out of 47 (87%) synovial sarcoma displayed strong positive nuclear staining (ranging from 80 to 90% of the tumor cells) for SYT antibody. Nineteen of 99 (19%) non-synovial sarcoma cases showed variable nuclear and cytoplasmic staining with SYT, which ranged from 20 to 60% of tumor nuclei, and included malignant peripheral nerve sheath tumor (5/25), solitary fibrous tumor (2/14), Ewing sarcoma (2/6), low grade fibromyxoid tumor (2/4), extraskeletal mesenchymal chondrosarcoma (2/6), gastrointestinal tumor (4/17), epithelioid sarcoma (2/2). The remaining non-synovial sarcomas were negative. This is the first study demonstrating SYT protein expression in tissue sections of synovial sarcoma. This method could provide an easy, rapid and widely applicable means of assisting in the diagnosis of synovial sarcoma, particularly when material and/or resources are unavailable for PCR or FISH-based testing. However, as variable weak staining for SYT may be encountered in a small percentage of non-synovial sarcoma sarcomas, a positive interpretation should be made only when the staining is strong, nuclear and present in the majority of cells.

  8. Multimodality Treatment in Adult Patients with High-risk Soft-tissue Sarcomas%成人高风险性软组织肉瘤的多形性治疗

    Institute of Scientific and Technical Information of China (English)

    2006-01-01

    Objective: We enrolled retrospective data to determine the efficacy of combined chemotherapy and surgery for local tumour control and survival in patients with high-risk soft-tissue sarcomas. Methods: We collected data from 25 patients with high-risk soft-tissue sarcomas treated with four cycles of etoposide, ifosfamide and doxorubicin (EIA) followed by definitive surgery with or without postoperative radiotherapy and adjuvant chemotherapy. 21 patients received chemotherapy in a neoadjuvant/adjuvant clinical setting;eighteen of them completed adjuvant chemotherapy. Four patients received chemotherapy in an adjuvant setting only. Results: The objective response rate of neoadjuvant chemotherapy assessable in 21 patients was 43%. Including NED (n=7) and partial remissions (n=3), the radiographic response rate was 47.6% with additional 42.9% stable diseases (n=9). Surgery was performed in two patients before completing four neoadjuvant chemotherapy cycles because of disease progression. Median overall survival for all patients was 21.6+ months. After completion of chemotherapy, in 62% of patients R0-resection could be performed. Conclusion: High proportion of R0-resections supports the idea of tumour down-staging after neoadjuvant treatment. Response to neoadjuvant chemotherapy is predictive for improved local tumour control resulting in long-term survival benefit.

  9. Primary hepatic sarcomas: CT findings

    Energy Technology Data Exchange (ETDEWEB)

    Yu, Ri-Sheng; Chen, Ying; Jiang, Biao; Wang, Liu-Hong [Zhejiang University School of Medicine, Department of Radiology, Hangzhou (China); Xu, Xiu-Fang [Zhejiang Medical College, Teaching and Research Group of Radiology, Hangzhou (China)

    2008-10-15

    Primary hepatic sarcomas are rare tumors that are difficult to diagnose clinically. Different primary hepatic sarcomas may have different clinical, morphologic, and radiological features. In this pictorial review, we summarized computed tomography (CT) findings of some relatively common types of hepatic sarcomas, including angiosarcoma, epithelioid hemangioendothelioma (EHE), liposarcoma, undifferentiated embryonal sarcoma (UES), leiomyosarcoma, malignant fibrous histiocytoma (MFH), and carcinosarcoma (including cystadenocarcinosarcoma). To our knowledge, hepatic cystadenocarcinosarcoma has not been described in the English literature. The CT findings in our case are similar to that of cystadenocarcinoma, a huge, multilocular cystic mass with a large mural nodule and solid portion. The advent of CT has allowed earlier detection of primary hepatic sarcomas as well as more accurate diagnosis and characterization. In addition, we briefly discuss the MRI findings and diagnostic value of primary hepatic sarcomas. (orig.)

  10. Clinicopathological features and prognosis assessment of extranodal follicular dendritic cell sarcoma

    Institute of Scientific and Technical Information of China (English)

    2010-01-01

    AIM: To establish a model for prognosis assessment of extranodal follicular dendritic cell (FDC) sarcoma.METHODS: Nine lesions were examined by routine and molecular approaches.Clinicopathological factors from the new cases and 97 reported cases were analyzed for their prognostic values.RESULTS: The current lesions were found in f ive male and four female patients,located mainly in the head and neck area and averaging 7.2 cm in size.Six patients had recurrence or metastasis and three remained free of diseas...

  11. Perigastric extraskeletal Ewing's sarcoma: A case report

    Institute of Scientific and Technical Information of China (English)

    Radoje B Colovic; Nikica M Grubor; Marjan T Micev; Slavko V Matic; Henry Dushan Edward Atkinson; Stojan M Latincic

    2009-01-01

    Ewing's sarcoma (ES) is a neoplasm of undifferentiated small round cells, which occurs in the bones and deep soft tissues of children and adolescents. We present a rare case of a 44-year-old woman with gastric ES presenting with epigastric pain and weight loss. Ultrasound and computed tomography scans indicated a solid/cystic mass in the pancreatic tail. At laparotomy, the tumor was found attached to the posterior surface of the stomach, completely free from the pancreas, with no lymphadenopathy or local metastases. The polynodal, partly pseudocystic, dark-red soft tumor was excised. Histopathology revealed an anaplastic small-round-cell tumor with strong membranous CD99 immunoexpression. Additionally, there was patchy immunostaining for S-100 protein, vimentin, protein gene product (PGP) 9.5 and neuron-specific enolase, and weak focal CD117 cytoplasmic immunoreactivity. The patient had no adjuvant chemotherapy; her postoperative recovery was uneventful, and she remains symptom-free, and without any sign of recurrence at 20 mo. To the best of our knowledge, this is only the third ever case of gastric ES.

  12. Hormonal treatments in metastatic endometrial stromal sarcomas: the 10-year experience of the sarcoma unit of Royal Marsden Hospital

    OpenAIRE

    2015-01-01

    Background Hormonal manipulation is sometimes recommended in the treatment of metastatic endometrial stromal sarcoma, but there are few data assessing the efficacy of endocrine therapies in this subtype of uterine sarcomas. Methods We performed a retrospective electronic medical record review of patients with metastatic ESS treated with a hormonal agent at Royal Marsden Hospital between 1999 and 2011. We assessed progression-free survival (PFS), objective response and toxicity profile among p...

  13. Evolution of drug resistance in HIV infected patients remaining on a virologically failing cART regimen

    DEFF Research Database (Denmark)

    Cozzi-Lepri, A; Phillips, AN; Ruiz, L;

    2007-01-01

    OBJECTIVE: To estimate the extent of drug resistance accumulation in patients kept on a virologically failing regimen and its determinants in the clinical setting. DESIGN: The study focused on 110 patients of EuroSIDA on an unchanged regimen who had two genotypic tests performed at two time points.......08 [95% confidence interval (CI), -2.13 to -0.03; P = 0.04] in those with GSS_f-t0 of 0.5-1.5 and -1.24 (95% CI, -2.44 to -0.04; P = 0.04) in those with GSS_f-t0 >or= 2. CONCLUSIONS: In patients kept on the same virologically failing cART regimen for a median of 6 months, there was considerable...

  14. An orthotopic xenograft model with survival hindlimb amputation allows investigation of the effect of tumor microenvironment on sarcoma metastasis.

    Science.gov (United States)

    Goldstein, Seth D; Hayashi, Masanori; Albert, Catherine M; Jackson, Kyle W; Loeb, David M

    2015-10-01

    Overall survival rates for pediatric high-grade sarcoma have improved greatly in the past few decades, but prevention and treatment of distant metastasis remain the most compelling problems facing these patients. Traditional preclinical mouse models have not proven adequate to study the biology and treatment of spontaneous distant sarcoma metastasis. To address this deficit, we developed an orthotopic implantation/amputation model in which patient-derived sarcoma xenografts are surgically implanted into mouse hindlimbs, allowed to grow, then subsequently amputated and the animals observed for development of metastases. NOD/SCID/IL-2Rγ-null mice were implanted with either histologically intact high grade sarcoma patient-derived xenografts or cell lines in the pretibial space and affected limbs were amputated after tumor growth. In contrast to subcutaneous flank tumors, we were able to consistently detect spontaneous distant spread of the tumors using our model. Metastases were seen in 27-90 % of animals, depending on the xenograft, and were repeatable and predictable. We also demonstrate the utility of this model for studying the biology of metastasis and present preliminary new insights suggesting the role of arginine metabolism and macrophage phenotype polarization in creating a tumor microenvironment that facilitates metastasis. Subcutaneous tumors express more arginase than inducible nitric oxide synthase and demonstrate significant macrophage infiltration, whereas orthotopic tumors express similar amounts of inducible nitric oxide synthase and arginase and have only a scant macrophage infiltrate. Thus, we present a model of spontaneous distant sarcoma metastasis that mimics the clinical situation and is amenable to studying the biology of the entire metastatic cascade.

  15. Ewing sarcomas of the sino-nasal tract and maxillary bone

    Directory of Open Access Journals (Sweden)

    Jbali Souheil

    2016-11-01

    Full Text Available Ewing’s sarcoma is a malignant tumor belonging to the group of small round cells tumors. Histologically similar to soft tissue neoplasms originally described as primitive neuro-ectodermal tumors (PNET, in the WHO classification, Ewing’s sarcoma and PNET are labeled together under the rubric of EWS/PNET. Rarely located in the nasal cavity and the para-nasal sinuses, we report three cases of Ewing’s sarcoma of maxillary bone and sinus. Our patients, 2 males and one female, were aged 20, 16 and 13 years respectively. The chief complaint was a painful face swelling. The diagnosis was retained on histologic and immuno-histochemical results. In two cases, surgery was performed as primary treatment modality followed by chemo-radiotherapy, which was the only therapeutic modality in the remaining case. After a follow-up of 2, 3 and 8 years (for each patient, we did not report local or distant failures.

  16. Primary Thyroid Sarcoma: A Systematic Review.

    Science.gov (United States)

    Surov, Alexey; Gottschling, Sebastian; Wienke, Andreas; Meyer, Hans Jonas; Spielmann, Rolf Peter; Dralle, Henning

    2015-10-01

    Different types of malignant tumors can occur within the thyroid. Primary cancer is the most common type of thyroid malignancy. Non-epithelial malignancies can also arise within the thyroid. The aim of the present study was to analyze clinical and radiological characteristics of reported primary thyroid sarcomas (PTS), based on a large sample of cases. The PubMed database was screened for articles from between 1990 and 2014. Overall, 86 articles with 142 patients were identified. Ultrasound evaluation was reported for 36 patients. Data regarding computed tomography of the neck were available for 29 cases. Magnetic resonance imaging was performed for eight patients. The following data were retrieved for the identified sarcomas: localization, size, homogeneity, internal texture, and margin characteristics. In most cases, PTS occurred in patients over 40 years of age, with a peak incidence for the group aged 60-79 years. Angiosarcoma was diagnosed in 29 cases (20.4%), followed by malignant hemangioendothelioma (n=23, 16.3%), malignant fibrous histiocytoma (n=20, 14.1%), leiomyosarcoma (n=16, 11.3%), and fibrosarcoma (n=13, 9.2%). In most patients (n=113, 79.6%), PTS manifested clinically as a painless goiter. On ultrasound, PTS were predominantly mixed hypo-to-hyperechoic in comparison to the normal thyroid tissue. On non-contrast computed tomography, most sarcomas were inhomogeneous hypo-to-hyperdense. On post-contrast magnetic resonance images, most sarcomas showed marked non-homogenous enhancement. In 26.8%, infiltration of the adjacent organs was seen. The trachea or esophagus was affected more frequently in patients with malignant histiocytoma and liposarcoma. Different strategies were used in the treatment of PTS. Our analysis provides clinical and radiological characteristics of PTS. The described features should be taken into consideration in the differential diagnosis of thyroid tumors.

  17. Clinicopathologic study of 6 patients with myeloid sarcoma%6例髓系肉瘤的临床病理分析

    Institute of Scientific and Technical Information of China (English)

    姜青明; 卢萍; 周文文; 叶学正; 李进

    2015-01-01

    目的:探讨髓系肉瘤( MS)的临床病理学特征、免疫学表型、鉴别诊断、治疗及预后。方法收集6例MS患者临床病理资料,对标本采用常规石蜡切片行HE染色,EnVision法免疫组化染色,骨髓涂片检查,并对生存进行随访。结果6例MS中男女各3例,年龄范围23~66岁;发生部位为小肠1例、上腭1例、手掌1例、结肠1例、肺1例、宫颈1例。细胞形态学特点为肿瘤细胞弥漫浸润,散在分布,互不粘附;肿瘤细胞中等大小,形态较单一,细胞核大,呈圆形或卵圆形,胞浆少,染色质细腻而均匀分布,2例核仁清晰,大小不一,部分可见嗜酸性粒细胞。免疫组化标记不同程度表达MPO、CD43、CD117、CD68⁃KP1、CD13、CD15、Lysozyme,结合病理组织学形态及免疫组化,6例均符合粒细胞肉瘤。随访期内3例伴急性髓系白血病确诊后分别于5、6、11个月死亡,其余3例仍存活。结论 MS为一种罕见以髓系细胞形态学和免疫表型特征的恶性肿瘤,需在形态学的基础上辅以免疫组织化学进行诊断。%Objective To investigate the clinicopathologic features, immunophenotyping, differential diagnoses, treatment and prognosis of myeloid sarcoma( MS) . Methods The clinical and pathologic data of 6 cases of MS were reviewed. HE stain,immu⁃nohistochemistry stain by EnVision method and bone marrow slides examination were carried out. The followed up information was avail⁃able in all patients. Results There were 3 males and 3 females. The age ranged from 23 to 66 years. The sites of involvement included small intestine, palate, palm of the hand, colon, lung and cervix.The tumor cells were widespread infiltration,diffused distribution with no adhesion each other;tumor cells were middle size, little cytoplasm and large nucleolus. Nucleolus were round and orbicular⁃ovate. Chromatin was exquisite and uniform distribution; nucleolus was clear with

  18. Evolution of drug resistance in HIV-infected patients remaining on a virologically failing combination antiretroviral therapy regimen

    DEFF Research Database (Denmark)

    Cozzi-Lepri, Alessandro; Phillips, Andrew N; Ruiz, Lidia

    2007-01-01

    .08 [95% confidence interval (CI), -2.13 to -0.03; P = 0.04] in those with GSS_f-t0 of 0.5-1.5 and -1.24 (95% CI, -2.44 to -0.04; P = 0.04) in those with GSS_f-t0 >or= 2. CONCLUSIONS: In patients kept on the same virologically failing cART regimen for a median of 6 months, there was considerable...

  19. Imaging of Kaposi sarcoma in a transplanted liver: A rare case report

    Directory of Open Access Journals (Sweden)

    Saumya Gupta

    2015-06-01

    Full Text Available In post-transplant patients, de novo malignancies such as post-transplant lymphoproliferative disease (PTLD, lung carcinoma, renal cell carcinoma, cutaneous malignancies, and Kaposi sarcoma are now seen. The immunotherapy used to prevent graft failure indirectly increases their risk. We present a rare case of visceral Kaposi sarcoma in a patient with orthotopic liver transplant.

  20. 人疱疹病毒8型ORF75基因亚型与Kaposi肉瘤的相关性研究%Study on the ORF75 subtypes of human herpesvirus-8 in patients with Kaposi's sarcoma

    Institute of Scientific and Technical Information of China (English)

    马春雷; 普雄明; 吴卫东; 张德志; 吴秀娟

    2009-01-01

    Objective To profile the subtypes of open reading frame 75(ORF75)of human herpesvirus 8(HHV-8)in patients with Kaposi's sarcoma,and to evaluate their relationship with clinical phenotypes and invasiveness of Kaposi's sarcoma.Methods Twenty-five paraffin-embeded tissue specimens of Kaposi's sarcoma were collected in the Department of Dermatology.People's Hospiml of Xinjiang Uygur Autonomous Region.DNA was extracted from these specimens.and nested-PCR was performed to amplify HHV-8 DNA followed bv bi-directional sequencing.Phylogenetic analysis was carried out by using the software DNASTAR,Clustal W program and PHYLIP package so as to identify the ORF75 subtyoe of HHV-8.Results HHV-8 DNA was detected in 21(84%)out of the 25 samples,and 7 cases of AIDS-associated Kaposi's sarcoma were all positive for HHV-8.Among the 21 patients carrying HHV-8 DNA,18 were positive for subtype A ORF75.3 for subtype C ORF75.The ORF75 subtypes had no significant correlation with the presence of mucosal lesions or clinical phenotypes of Kaposi's sarcoma.Conclusions The majority of patients with Kaposi's sarcoma in Xinjiang are infected with HHV-8 of ORF 75 subtype A and C.The ORF75 subtypes of HHV-8 have no correlation with the presence of mucosal lesions or clinical phenotypes of Kaposi's sarcoma.%目的 探讨人类疱疹病毒8型(HHV-8)ORF75基因亚型,与Kaposi肉瘤不同临床分型及侵袭性的相关性.方法 对25例新疆Kaposi肉瘤石蜡包埋组织进行HHV-8 DNA抽提、扩增及双向测序,使用Clustal W软件和PHYLIP软件包对测序结果进行发生学分析,从而确定HHV-8 ORF75基因哑型.结果 25例Kaposi肉瘤中,21例HHV-8阳性,阳性率为84%,其中7例AIDS相关型Kaposi肉瘤患者HHV-8均阳性.21例HHV-8阳性患者中,18例为HHV-8 ORF75 A亚型,3例为C亚型;不同亚型间Kaposi肉瘤患者有无黏膜损害及临床分型的分布差异均无统计学意义(P>0.05).结论 新疆Kaposi肉瘤患者感染HHV-8 ORF75

  1. Pazopanib in advanced vascular sarcomas: an EORTC Soft Tissue and Bone Sarcoma Group (STBSG) retrospective analysis.

    Science.gov (United States)

    Kollár, A; Jones, R L; Stacchiotti, S; Gelderblom, H; Guida, M; Grignani, G; Steeghs, N; Safwat, A; Katz, D; Duffaud, F; Sleijfer, S; van der Graaf, W T; Touati, N; Litière, S; Marreaud, S; Gronchi, A; Kasper, B

    2017-01-01

    Pazopanib is a multitargeted tyrosine kinase inhibitor approved for the treatment of patients with selective subtypes of advanced soft tissue sarcoma (STS) who have previously received standard chemotherapy including anthracyclines. Data on the efficacy in vascular sarcomas are limited. The main objective of this study was to investigate the activity of pazopanib in vascular sarcomas. A retrospective study of patients with advanced vascular sarcomas, including angiosarcoma (AS), epithelioid hemangioendothelioma (HE) and intimal sarcoma (IS) treated with pazopanib in real life practice at EORTC centers as well as patients treated within the EORTC phase II and III clinical trials (62043/62072) was performed. Patient and tumor characteristics were collected. Response was assessed according to RECIST 1.1. and survival analysis was performed. Fifty-two patients were identified, 40 (76.9%), 10 (19.2%) and two (3.8%) with AS, HE and IS, respectively. The response rate was eight (20%), two (20%) and two (100%) in the AS, HE and IS subtypes, respectively. There was no significant difference in response rate between cutaneous and non-cutaneous AS and similarly between radiation-associated and non-radiation-associated AS. Median progression-free survival (PFS) and median overall survival (OS; from commencing pazopanib) were three months (95% CI 2.1-4.4) and 9.9 months (95% CI 6.5-11.3) in AS, respectively. The activity of pazopanib in AS is comparable to its reported activity in other STS subtypes. In this study, the activity of pazopanib was similar in cutaneous/non-cutaneous and in radiation/non-radiation-associated AS. In addition, pazopanib showed promising activity in HE and IS, worthy of further evaluation.

  2. Myofibroblastic sarcomas: a clinicopathological study of 20 cases

    Institute of Scientific and Technical Information of China (English)

    MENG Guo-zhao; ZHANG Hong-ying; BU Hong; ZHANG Xian-liang; PANG Zong-guo; KE Qi; LIU Xi; YANG Guo

    2007-01-01

    Background Myofibroblastic sarcoma was used to be a controversial neoplasm. This study investigated the clinicopathological features of 20 cases of myofibroblastic sarcoma arising in different locations.Methods The paraffin-embedded tissue samples from 20 cases of patients with myofibroblastic sarcoma were stained immunohistochemically, and 5 cases examined by electron microscopy. Student's t test was used to analyze the difference of Ki-67 labeling index between grade 1 and grade 2 myofibroblastic sarcomas.Results Histologically, the tumors were composed of slender spindle cells with eosinophilic cytoplasm, and fusiform,tapering, wavy, or plump ovoid; vesicular nuclei and a small central eosinophilic nucleoli. Immunohistochemically, the tumor cells expressed smooth muscle actin (18/20), muscle specific actin (16/20), fibronectin (20/20) and desmin (2/20).Ultrastructurally, the tumor cells revealed abundant rough endoplasmic reticulum and longitudinally arranged fine filaments with focal densities in the cytoplasm. A clinical follow-up of 19 patients showed that 2 cases experienced local recurrence and distant metastasis 6 months to 4 years after the initial operation. Nine cases recurred locally 17 to 46 months after the initial excision, and 9 cases were alive with no evidence of disease.Conclusions Myofibroblastic sarcomas, which exhibit diverse histological appearance, can easily be misdiagnosed as benign tumors. Myofibroblastic sarcomas are local destructive lesions with frequent recurrence, and may metastase distantly.

  3. Human herpesvirus 8 open reading frame 26 and open reading frame 65 sequences from multiple myeloma patients: a shared pattern not found in Kaposi's sarcoma or primary effusion lymphoma.

    Science.gov (United States)

    Ma, H J; Sjak-Shie, N N; Vescio, R A; Kaminsky, M; Mikail, A; Pold, M; Parker, K; Beksac, M; Belson, D; Moss, T J; Wu, C H; Zhou, J; Zhang, L; Chen, G; Said, J W; Berenson, J R

    2000-11-01

    Human herpesvirus 8 (HHV-8), also known as Kaposi's sarcoma-associated herpesvirus, has been implicated in the pathogenesis of Kaposi's sarcoma (KS), primary effusion lymphoma (PEL), multicentric Castleman's disease, and recently multiple myeloma (MM). DNA sequence analyses of HHV-8 suggest that multiple HHV-8 strains exist. We extracted DNA from 24 patients with MM and 3 patients with monoclonal gammopathy of undetermined significance and compared HHV-8 open reading frames (ORFs) 26 and 65 sequences with those derived from patients with KS, PEL, and two HHV-8-positive PEL cell lines KS-1 and BC-1. ORF26 sequence data suggest that MM patients are consistently carriers of HHV-8 strain subtype C3. All MM patients also consistently revealed either a single bp deletion or substitution at position 112197 in ORF65. This unique alteration is not present in patients with KS or PEL or in PEL cell lines. It occurs in the portion of ORF65 that is known to be responsible for a serological response to HHV-8.

  4. Sarcomas arising after radiotherapy for peptic ulcer disease

    Energy Technology Data Exchange (ETDEWEB)

    Lieber, M.R.; Winans, C.S.; Griem, M.L.; Moossa, R.; Elner, V.M.; Franklin, W.A.

    1985-06-01

    Therapeutic gastric irradiation has been used to reduce peptic juice secretion in patients with peptic ulcer disease. Between 1937 and 1968 a total of 2049 patients received such therapy at the University of Chicago. Three of these patients are known to have developed sarcomas in the field of radiation. Two gastric leiomyosarcomas of the stomach were diagnosed 26 and 14 years after treatment and a malignant fibrous histiocytoma of the anterior chest wall was removed six years after gastric irradiation. Of 743 peptic ulcer patients treated without irradiation and constituted as a control group for the study of therapeutic gastric radiation, none is known to have developed sarcoma. As the incidence of sarcoma in these patient groups is known only from the tumor registry of the University of Chicago, other cases of sarcoma may exist in the groups. While an increased incidence of sarcoma has not been proven to occur in patients who received therapeutic gastric irradiation for peptic ulcer disease, the possibility of such a risk should be borne in mind by physicians caring for such patients.

  5. [Soft tissue sarcoma in children and adolescents: experiences of the cooperative Soft Tissue Sarcoma Group Studies (CWS-81 - 96)].

    Science.gov (United States)

    Brecht, I B; Treuner, J

    2004-10-01

    The very heterogeneous group of paediatric soft tissue sarcomas account for approximately 7 % of all malignant childhood tumours. More than one half of all cases are rhabdomyosarcomas, some of the over 20 entities are very rare. The prognosis and biology of soft tissue sarcomas in children and adolescents vary greatly depending on histological subtype, the age of the patient, the primary site, the tumour size, tumour invasiveness and the extent of disease at diagnosis. Since 1981, 2918 children and adolescents with soft tissue sarcomas were treated prospectively according to the common treatment protocols of the Cooperative Soft Tissue Sarcoma Study Group (CWS-81 - 96). The known prognostic factors were used to develop a more and more detailed risk stratification. The multimodal treatment includes the use of surgery, chemotherapy and radiotherapy and should be planned by a multidisciplinary team. That way, an overall survival of nearly 70 % over all risk groups could be achieved.

  6. Preoperative, intraoperative, and postoperative radiation in the treatment of primary soft tissue sarcoma.

    Science.gov (United States)

    Suit, H D; Mankin, H J; Wood, W C; Proppe, K H

    1985-06-01

    The rationale for combining radiation with conservative surgery in the treatment of sarcoma of soft tissue is discussed, as well as the advantages for performing the radiation preoperatively on the one hand and postoperatively on the other. The results of treatment of soft tissue sarcoma by radical resectional surgery or amputation in 464 patients at four centers and by conservative surgery, and postoperative radiation in 416 patients at three centers, have been reviewed. The local failure rates were 18.1% and 18.3%, respectively. The results obtained by radiation administered postoperatively (110 patients) or preoperatively (60 patients) at the Massachusetts General Hospital during the period September 1971 to August 1982 are analyzed and discussed with reference to 5-year actuarial local control and survival results as well as causes of failure with respect to AJC stage, histologic type, anatomic site, and size of tumor. The results which have been obtained by the preoperative approach are judged to be superior, particularly for the larger lesions and higher grades that predominated in that group. Of a total of 170 patients, there were 19 local failures; 13 of these were diagnosed at the time when metastatic disease was not evident. Of those 13, 12 have been subjected to salvage surgery and 7 remain with no evidence of disease at 1 to 3.5 years after the salvage procedure. A major problem in the management of these patients remains the occult metastatic disease.

  7. Prognostic and predictive factors for outcome to first-line ifosfamide-containing chemotherapy for adult patients with advanced soft tissue sarcomas An exploratory, retrospective analysis on large series from the European Organization for Research and Treatment of Cancer-Soft Tissue and Bone

    DEFF Research Database (Denmark)

    Sleiffer, S.; Ouali, M.; van Glabbeke, M.;

    2010-01-01

    Background: Adult patients with advanced soft tissue sarcomas (STS) are generally treated similarly, regardless of great differences between STS subtypes, disease presentation and patients' characteristics. As ifosfamide is frequently applied in first line systemic therapy, we aimed to establish ...... contribute to further treatment individualisation of advanced STS patients. (C) 2009 Elsevier Ltd. All rights reserved...

  8. Evolution of drug resistance in HIV infected patients remaining on a virologically failing cART regimen

    DEFF Research Database (Denmark)

    Cozzi-Lepri, A; Phillips, AN; Ruiz, L;

    2007-01-01

    (t0 and t1) when viral load was > 400 copies/ml. METHODS: Accumulation of resistance between t0 and t1 was measured using genotypic susceptibility scores (GSS) obtained by counting the total number of active drugs (according to the Rega system v6.4.1) among all licensed antiretrovirals as of 1...... to the failing regimen were still receiving benefit from treatment. An overall 6-monthly increase of 1.96 (SD, 2.23) International Aids Society-mutations and an average loss of 1.25 (SD, 1.81) active drugs were estimated. In comparison with patients with GSS_f-t0 = 0, the number of active drugs lost was -1...

  9. Sonographic Findings of Uterine Endometrial Stromal Sarcoma

    OpenAIRE

    Kim, Jeong-Ah; Lee, Myung Sook; Choi, Jong-Sun

    2006-01-01

    Objective The study was performed to present the sonographic findings of uterine endometrial stromal sarcoma (ESS). Materials and Methods We conducted a retrospective review of sonographic findings of 10 cases that were diagnosed as uterine ESS. The patients' ages ranged from 25 to 51 years (mean age: 36.1 years). The reviews focused on the location, margin, size, number and echotexture of the lesions. Hysterectomy (n = 9) and myomectomy (n = 1) were performed and a pathologic diagnosis was o...

  10. ECCO Essential Requirements for Quality Cancer Care: Soft Tissue Sarcoma in Adults and Bone Sarcoma. A critical review.

    Science.gov (United States)

    Andritsch, Elisabeth; Beishon, Marc; Bielack, Stefan; Bonvalot, Sylvie; Casali, Paolo; Crul, Mirjam; Bolton, Roberto Delgado-; Donati, Davide Maria; Douis, Hassan; Haas, Rick; Hogendoorn, Pancras; Kozhaeva, Olga; Lavender, Verna; Lovey, Jozsef; Negrouk, Anastassia; Pereira, Philippe; Roca, Pierre; de Lempdes, Godelieve Rochette; Saarto, Tiina; van Berck, Bert; Vassal, Gilles; Wartenberg, Markus; Yared, Wendy; Costa, Alberto; Naredi, Peter

    2017-02-01

    ECCO essential requirements for quality cancer care (ERQCC) are checklists and explanations of organisation and actions that are necessary to give high-quality care to patients who have a specific tumour type. They are written by European experts representing all disciplines involved in cancer care. ERQCC papers give oncology teams, patients, policymakers and managers an overview of the elements needed in any healthcare system to provide high quality of care throughout the patient journey. References are made to clinical guidelines and other resources where appropriate, and the focus is on care in Europe. Sarcoma: essential requirements for quality care • Sarcomas - which can be classified into soft tissue and bone sarcomas - are rare, but all rare cancers make up more than 20% of cancers in Europe, and there are substantial inequalities in access to high-quality care. Sarcomas, of which there are many subtypes, comprise a particularly complex and demanding challenge for healthcare systems and providers. This paper presents essential requirements for quality cancer care of soft tissue sarcomas in adults and bone sarcomas. • High-quality care must only be carried out in specialised sarcoma centres (including paediatric cancer centres) which have both a core multidisciplinary team and an extended team of allied professionals, and which are subject to quality and audit procedures. Access to such units is far from universal in all European countries. • It is essential that, to meet European aspirations for high-quality comprehensive cancer control, healthcare organisations implement the requirements in this paper, paying particular attention to multidisciplinarity and patient-centred pathways from diagnosis and follow-up, to treatment, to improve survival and quality of life for patients. Taken together, the information presented in this paper provides a comprehensive description of the essential requirements for establishing a high-quality service for soft

  11. Kaposi's sarcoma in an elderly man with Wegener's granulomatosis treated with cyclophosphamide and corticosteroids.

    Science.gov (United States)

    Erban, S B; Sokas, R K

    1988-05-01

    The association of Kaposi's sarcoma with malignant lymphoreticular diseases and immunosuppressive therapy is well documented. This report describes an elderly man who presented with fulminant Wegener's granulomatosis that responded to treatment with cyclophosphamide and corticosteroids. Rapidly progressing cutaneous Kaposi's sarcoma developed ten weeks after the start of immunosuppressive therapy yet regressed on discontinuation of the corticosteroid therapy, despite continuation of cyclophosphamide therapy. To our knowledge, this is the first reported case of Kaposi's sarcoma occurring in association with Wegener's granulomatosis. The literature on Kaposi's sarcoma in immunosuppressed patients is reviewed.

  12. Long-Term Survival of a Patient with Pulmonary Artery Intimal Sarcoma after Sequential Metastasectomies of the Thyroid and Adrenal Glands

    Directory of Open Access Journals (Sweden)

    Yun Mi Choi

    2013-03-01

    Full Text Available Cancer metastases to the thyroid or adrenal gland are uncommon. Furthermore, cases showing long-term survival after surgical resection of those metastatic tumors are rare. We report a case of pulmonary artery intimal sarcoma with metastases to the thyroid and adrenal glands sequentially that was successfully treated with sequential metastasectomies. A 62-year-old woman presented with a 4-week history of dyspnea on exertion and facial edema in November 1999. Echocardiography and chest computed tomography (CT revealed an embolism-like mass in the pulmonary trunk. Pulmonary artery endarterectomy with pulmonary valve replacement was performed, and histopathology revealed pulmonary artery intimal sarcoma. A thyroid nodule was found by chest CT in November 2001 (2 years after initial surgery. During follow-up, this lesion showed no change, but we decided to obtain fine needle aspiration cytology (FNAC in August 2004 (4.7 years after initial surgery. FNAC revealed atypical spindle cells suggestive of metastatic intimal sarcoma. She underwent total thyroidectomy. During follow-up, a right adrenal gland mass was detected by chest CT in March 2006 (6.3 years after initial surgery, and adrenalectomy was done, which also revealed metastatic sarcoma. She has been followed up without any evidence of recurrent disease until May 2012 (12.5 years after initial surgery.

  13. 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. L.; Litiere, S.; Marreaud, S.; Collin, F.; van der Graaf, W. T. A.

    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

  14. 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

  15. Changing patterns of Kaposi's sarcoma in Danish acquired immunodeficiency syndrome patients with complete follow-up. The Danish Study Group for HIV Infection (DASHI)

    DEFF Research Database (Denmark)

    Lundgren, Jens Dilling; Melbye, M; Pedersen, C

    1995-01-01

    clinically and with consecutive CD4 cell count measurement from time of AIDS-defining illness to date of death or censoring date, whichever came first. The proportion of homo-/bisexual men (n = 520) with Kaposi's sarcoma (n = 100) at AIDS diagnosis declined from 31% before 1985 to 13% in 1990, whereas...

  16. 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. L.; Litiere, S.; Marreaud, S.; Collin, F.; van der Graaf, W. T. A.

    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 infusion

  17. 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 infusion

  18. Changing patterns of Kaposi's sarcoma in Danish acquired immunodeficiency syndrome patients with complete follow-up. The Danish Study Group for HIV Infection (DASHI)

    DEFF Research Database (Denmark)

    Lundgren, Jens Dilling; Melbye, M; Pedersen, C

    1995-01-01

    clinically and with consecutive CD4 cell count measurement from time of AIDS-defining illness to date of death or censoring date, whichever came first. The proportion of homo-/bisexual men (n = 520) with Kaposi's sarcoma (n = 100) at AIDS diagnosis declined from 31% before 1985 to 13% in 1990, whereas...

  19. General Information about Ewing Sarcoma

    Science.gov (United States)

    ... tumor that forms from a certain kind of cell in bone or soft tissue . Ewing sarcoma may be found in the bones of the legs, arms, feet, hands, chest , pelvis , spine , or skull . Ewing sarcoma also may be found in the soft tissue of the trunk, arms, legs, head and neck, abdominal cavity , or ...

  20. Treatment Option Overview (Ewing Sarcoma)

    Science.gov (United States)

    ... tumor that forms from a certain kind of cell in bone or soft tissue . Ewing sarcoma may be found in the bones of the legs, arms, feet, hands, chest , pelvis , spine , or skull . Ewing sarcoma also may be found in the soft tissue of the trunk, arms, legs, head and neck, abdominal cavity , or ...

  1. Differential diagnosis of sarcomatoid mesothelioma from true sarcoma and sarcomatoid carcinoma using immunohistochemistry.

    Science.gov (United States)

    Kushitani, Kei; Takeshima, Yukio; Amatya, Vishwa Jeet; Furonaka, Osamu; Sakatani, Akio; Inai, Kouki

    2008-02-01

    Differentiation of sarcomatoid mesothelioma from other sarcomatoid tumors involving the pleura and other structures by light microscopy remains an important diagnostic challenge for surgical pathologists. The purpose of the present study was to investigate the utility of diagnostic immunohistochemistry for differentiating sarcomatoid mesothelioma from its histological mimics: true sarcoma and pulmonary sarcomatoid carcinoma. A total of 39 specimens of mesotheliomas with sarcomatoid components, 43 specimens of true sarcomas, and nine specimens of pulmonary sarcomatoid carcinomas were obtained from Japanese patients and examined using a 10-antibody panel (calretinin, WT1, AE1/AE3, CAM5.2, epithelial membrane antigen, desmin, alpha-smooth muscle actin, S-100 protein, CD34, and CD68). CAM5.2 had the highest sensitivity and specificity for differentiating sarcomatoid mesothelioma from true sarcoma. The combination of CAM5.2, WT1, and AE1/AE3 is recommended for routine pathological diagnosis. Accurate clinical information is necessary for differentiating sarcomatoid mesothelioma from sarcomatoid carcinoma.

  2. Lymphangioma-Like Kaposi’s Sarcoma Presenting as Gangrene

    Directory of Open Access Journals (Sweden)

    Eitan R. Friedman

    2013-01-01

    Full Text Available Kaposi’s sarcoma (KS is a multicentric vascular neoplasm associated with the Kaposi’s sarcoma-associated herpes virus (KSHV. KS can occur in immunocompromised patients as well as certain populations in Africa or in the Mediterranean. Less than 5% of KS cases can present with lymphangioma-like kaposi sarcoma (LLKS, which can occur in all KS variants. KS presents with characteristic skin lesions that appear as brown, red, blue, or purple plaques and nodules. The lesions are initially flat and if untreated will become raised. LLKS presents similarly to KS but is associated with severe lymphedema and soft tissue swelling as well as bulla-like vascular lesions. We present the case of an 85-year-old Lebanese, HIV negative, man who presented with a swollen and painful right lower extremity accompanied by necrotic lesions. Wound cultures were positive, and we began the work-up for secondarily infected gangrene. However, skin biopsy results revealed that he in fact had lymphangioma-like Kaposi sarcoma, which allowed us to shift our management. Advanced Kaposi’s sarcoma can present similar to gangrene. It is important to recognize the typical skin lesions of KS and not to overlook Kaposi’s sarcoma or LLKS within the differential.

  3. Sarcoma risk after radiation exposure

    Directory of Open Access Journals (Sweden)

    Berrington de Gonzalez Amy

    2012-10-01

    Full Text Available Abstract Sarcomas were one of the first solid cancers to be linked to ionizing radiation exposure. We reviewed the current evidence on this relationship, focusing particularly on the studies that had individual estimates of radiation doses. There is clear evidence of an increased risk of both bone and soft tissue sarcomas after high-dose fractionated radiation exposure (10 + Gy in childhood, and the risk increases approximately linearly in dose, at least up to 40 Gy. There are few studies available of sarcoma after radiotherapy in adulthood for cancer, but data from cancer registries and studies of treatment for benign conditions confirm that the risk of sarcoma is also increased in this age-group after fractionated high-dose exposure. New findings from the long-term follow-up of the Japanese atomic bomb survivors suggest, for the first time, that sarcomas can be induced by acute lower-doses of radiation (

  4. Proactive coping and spirituality among patients who left or remained in antiretroviral treatment in St Petersburg, Russian Federation.

    Science.gov (United States)

    Pecoraro, Anna; Pacciolla, Aureliano; O'Cleirigh, Conall; Mimiaga, Matthew; Kwiatek, Piotr; Blokhina, Elena; Verbitskaya, Elena; Krupitsky, Evgeny; Woody, George E

    2016-01-01

    Positive Psychology, the study of "positive" factors or strengths and evidence-based interventions to increase them, is a rapidly developing field that is beginning to be applied to HIV care. Proactive coping and spirituality are two positive characteristics that have been examined in multiple chronic serious health conditions. In the present study, lost-to-care (LTCs; did not attend treatment for ≥12 months; n = 120) and engaged-in-care HIV clinic patients (EICs; attended treatment for ≥12 months and adherent with antiretrovirals; n = 120) in Leningrad Oblast, Russian Federation were compared on the Proactive Coping Inventory and View of God Scale. EICs had higher scores in proactive coping [t(229) = 3.69; p = .001] and instrumental [t(232) = 2.17; p = .03] and emotional [t(233) = 2.33; p = .02] support, indicating that they engage in autonomous goal setting and self-regulate their thoughts and behaviors; obtain advice and support from their social network; and cope with emotional distress by turning to others. LTCs had higher scores in avoidance coping [t(236) = -2.31; p = .02]. More EICs were spiritual, religious, or both [ χ(2)(1, N = 239) = 7.49, p = .006]. EICs were more likely to believe in God/Higher Power [χ(2)(1, N = 239 = 8.89, p = .002] and an afterlife [ χ(2)(1, N = 236) = 5.11, p = .024]; have a relationship with God/Higher Power [ χ(2)(1, N = 237) = 12.76, p = .000]; and call on God/Higher Power for help, healing, or protection [ χ(2)(1, N = 239) = 9.61]. EICs had more positive [t(238) = 2.78; p = .006] and less negative [t(236) = -2.38; p = .002] views of God. Similar proportions, but slightly more EICs than LTCs were members of a faith community; members of a12-step group; or attended religious or spiritual services, meetings, or activities. More EICs than LTCs engaged in private spiritual or religious activities, such as

  5. Primary Occipital Ewing’s Sarcoma with Subsequent Spinal Seeding

    Directory of Open Access Journals (Sweden)

    Ali Alqahtani

    2017-01-01

    Full Text Available Ewing’s sarcoma is a primary bone cancer that mainly affects the long bones. This malignancy is particularly common in pediatric patients. Primary cranial involvement accounts for 1% of cases, with occipital involvement considered extremely rare. In this case study, primary occipital Ewing’s sarcoma with a posterior fossa mass and subsequent relapse resulting in spinal seeding is reported. A 3-year-old patient presented with a 1-year history of left-sided headaches, localized over the occipital bone with progressive torticollis. Computed tomography (CT imaging showed a mass in the left posterior fossa compressing the brainstem. The patient then underwent surgical excision followed by adjuvant chemoradiation therapy. Two years later, the patient presented with severe lower back pain and urinary incontinence. Whole-spine magnetic resonance imaging (MRI showed cerebrospinal fluid (CSF seeding from the L5 to the S4 vertebrae. Primary cranial Ewing’s sarcoma is considered in the differential diagnosis of children with extra-axial posterior fossa mass associated with destructive permeative bone lesions. Although primary cranial Ewing’s sarcoma typically has good prognosis, our patient developed metastasis in the lower spine. Therefore, with CNS Ewing’s sarcoma, screening of the entire neural axis should be taken into consideration for early detection of CSF seeding metastasis in order to decrease the associated morbidity and mortality.

  6. The Danish experience with trabectedin treatment for metastatic sarcoma: Importance of hyponatremia

    DEFF Research Database (Denmark)

    Schack, Line M Hybel; Mouritsen, Lene S; Krarup-Hansen, Anders

    2015-01-01

    centers in Denmark. Known prognostic factors such as age, gender and performance status, the histopathology as well as other new factors such as response to previous chemotherapy and hyponatremia were tested. RESULTS: Median age was 59 years. Lipo- and leiomysosarcomas (L-sarcomas) represented 43......BACKGROUND: Trabectedin was in Europe approved for treatment of metastatic soft tissue sarcoma (STS) in 2007 based on results of a phase II study with relatively few patients. The purpose of this nationwide retrospective study was to assess efficacy and safety of using trabectedin in the entire...... unselected cohort of patients with metastatic sarcoma and to test known, as well as new prognostic factors that may affect overall survival (OS). PATIENTS AND METHODS: Between January 2008 and April 2013, 117 patients were treated with trabectedin for metastatic sarcoma in the three specialized sarcoma...

  7. Alveolar Soft Part Sarcoma: A Single-Center 26-Patient Case Series and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Koichi Ogura

    2012-01-01

    Methods. A retrospective review of 26 consecutive ASPS patients (12 male, 14 female; mean age of 27 years treated at our institution over 30 years (mean followup; 71 months was performed. Results. The primary tumor developed in the lower extremity (12, trunk (8, and upper extremity (6, with an average size of 7.2 cm (range, 2–14 cm. The AJCC stage at presentation was IIA (7, III (3, and IV (16. Surgical excision was performed in 20 patients (R0 18, R1 plus radiotherapy 2 without local recurrence. Six patients (stage IIA 3/7, stage III 3/3 later developed metastases after an average period of 28.7 months. The median survival of the 26 patients was 90 months, with overall 5/10-year survival rates of 64%/48%. AJCC stage and tumor size were significant prognostic factors. Significant palliation and slowing of metastasis progression were achieved with gamma knife radiotherapy. Nine patients receiving chemotherapy showed no objective response. Conclusions. ASPS is indolent but has a high propensity for metastasis. Early diagnosis and complete excision of the small primary tumor are essential in the treatment of ASPS.

  8. Psychometric properties of the World Health Organization Quality of Life Questionnaire (WHOQOL-100) in Portuguese patients with sarcoma.

    Science.gov (United States)

    Paredes, T; Simões, M R; Canavarro, M C

    2010-08-01

    The purpose of this article is to report on the study of the psychometric properties of the European Portuguese version of the World Health Organization Quality of Life Questionnaire--WHOQOL-100, in a clinical sample of patients with bone and soft tissue cancer. Eighty-one participants, in different phases of the disease, were recruited from the Department of Orthopaedics of the Coimbra University Hospitals. After the informed consent was obtained, all patients responded to the European Portuguese version of WHOQOL-100 and the Portuguese versions of Beck Depression Inventory (BDI) and Brief Symptoms Inventory (BSI). The results show an acceptable internal consistency for the set of facets, domains, and 100 questions (alphas from 0.73 to 0.96) and across domains (alphas from 0.82 to 0.94), as well as an acceptable test-retest reliability (test-retest correlations from 0.71 to 0.88) of WHOQOL-100. Construct validity was demonstrated by moderate correlations between domains (r from 0.23 to 0.70) and with the general facet (r from 0.32 to 0.57), concurrent validity by its correlation with the total scores of BDI and Global Severity Index of BSI, and discriminant validity by its ability to discriminate between healthy individuals and cancer patients. In general, the European Portuguese version of WHOQOL-100 is a reliable and valid instrument for the assessment of quality of life in patients with bone and soft tissue cancer.

  9. Immediate versus Delayed Sarcoma Reconstruction: Impact on Outcomes

    Directory of Open Access Journals (Sweden)

    Kyle J. Sanniec

    2016-01-01

    Full Text Available Background. Sarcoma is a rare malignancy, and more recent management algorithms emphasize a multidisciplinary approach and limb salvage, which has resulted in an increase in overall survival and limb preservation. However, limb salvage has resulted in a higher rate of wound complications. Objective. To compare the complications between immediate and delayed (>three weeks reconstruction in the multidisciplinary limb salvage sarcoma patient population. Methods. A ten-year retrospective review of patients who underwent sarcoma resection was performed. The outcome of interest was wound complication in the postoperative period based on timing of reconstruction. We defined infection as any infection requiring intravenous antibiotics, partial flap failure as any flap requiring a debridement or revision, hematoma/seroma as any hematoma/seroma requiring drainage, and wound dehiscence as a wound that was not completely intact by three weeks postoperatively. Results. 70 (17 delayed, 53 immediate patients who underwent sarcoma resection and reconstruction met the inclusion criteria. Delayed reconstruction significantly increased the incidence of postoperative wound infection and wound dehiscence. There was no difference in partial or total flap loss, hematoma, or seroma between the two groups. Discussion and Conclusion. Immediate reconstruction results in decreased wound complications may reduce the morbidity associated with multidisciplinary treatment in the limb salvage sarcoma patient.

  10. 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

  11. Retroperitoneal Sarcoma Target Volume and Organ at Risk Contour Delineation Agreement Among NRG Sarcoma Radiation Oncologists

    Energy Technology Data Exchange (ETDEWEB)

    Baldini, Elizabeth H., E-mail: ebaldini@partners.org [Department of Radiation Oncology, Dana-Farber Cancer Institute, Brigham and Women' s Hospital, Boston, Massachusetts (United States); Abrams, Ross A. [Department of Radiation Oncology, Rush University Medical Center, Chicago, Illinois (United States); Bosch, Walter [Department of Radiation Oncology, Washington University, St. Louis, Missouri (United States); Roberge, David [Department of Radiation Oncology, Centre Hospitalier de l' Universite de Montreal, Montreal, Quebec (Canada); Haas, Rick L.M. [Department of Radiotherapy, Netherlands Cancer Institute, Amsterdam (Netherlands); Catton, Charles N. [Department of Radiation Oncology, Princess Margaret Cancer Centre, Toronto, Ontario (Canada); Indelicato, Daniel J. [Department of Radiation Oncology, University of Florida Medical Center, Jacksonville, Florida (United States); Olsen, Jeffrey R. [Department of Radiation Oncology, Washington University, St. Louis, Missouri (United States); Deville, Curtiland [Department of Radiation Oncology, University of Pennsylvania, Philadelphia, Pennsylvania (United States); Chen, Yen-Lin [Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts (United States); Finkelstein, Steven E. [Translational Research Consortium, 21st Century Oncology, Scottsdale, Arizona (United States); DeLaney, Thomas F. [Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts (United States); Wang, Dian [Department of Radiation Oncology, Rush University Medical Center, Chicago, Illinois (United States)

    2015-08-01

    Purpose: The purpose of this study was to evaluate the variability in target volume and organ at risk (OAR) contour delineation for retroperitoneal sarcoma (RPS) among 12 sarcoma radiation oncologists. Methods and Materials: Radiation planning computed tomography (CT) scans for 2 cases of RPS were distributed among 12 sarcoma radiation oncologists with instructions for contouring gross tumor volume (GTV), clinical target volume (CTV), high-risk CTV (HR CTV: area judged to be at high risk of resulting in positive margins after resection), and OARs: bowel bag, small bowel, colon, stomach, and duodenum. Analysis of contour agreement was performed using the simultaneous truth and performance level estimation (STAPLE) algorithm and kappa statistics. Results: Ten radiation oncologists contoured both RPS cases, 1 contoured only RPS1, and 1 contoured only RPS2 such that each case was contoured by 11 radiation oncologists. The first case (RPS 1) was a patient with a de-differentiated (DD) liposarcoma (LPS) with a predominant well-differentiated (WD) component, and the second case (RPS 2) was a patient with DD LPS made up almost entirely of a DD component. Contouring agreement for GTV and CTV contours was high. However, the agreement for HR CTVs was only moderate. For OARs, agreement for stomach, bowel bag, small bowel, and colon was high, but agreement for duodenum (distorted by tumor in one of these cases) was fair to moderate. Conclusions: For preoperative treatment of RPS, sarcoma radiation oncologists contoured GTV, CTV, and most OARs with a high level of agreement. HR CTV contours were more variable. Further clarification of this volume with the help of sarcoma surgical oncologists is necessary to reach consensus. More attention to delineation of the duodenum is also needed.

  12. The multidisciplinary management of bone and soft tissue sarcoma: an essential organizational framework

    Directory of Open Access Journals (Sweden)

    Siegel GW

    2015-02-01

    Full Text Available Geoffrey W Siegel,1 J Sybil Biermann,1 Rashmi Chugh,2 Jon A Jacobson,3 David Lucas,4 Mary Feng,5 Andrew C Chang,6 Sean R Smith,7 Sandra Wong,6 Jill L Hasen1 1Department of Orthopedics, 2Department of Medical Oncology, 3Department of Radiology, 4Department of Pathology, 5Department of Radiation, 6Department of Surgery, 7Department of Physical Medicine and Rehabilitation, University of Michigan, Ann Arbor, MI, USA Abstract: The rarity of bone and soft tissue sarcoma, the difficulty in interpretation of imaging and histology, the plethora of treatment modalities, and the complexity and intensity of the treatment contribute to the need for systematic multidisciplinary team management of patients with these diseases. An integrated multidisciplinary clinic and team with a structured sarcoma tumor board facilitate team coordination and communication. This paper reviews the rationale for multidisciplinary management of sarcoma and details the operational structure of the Multidisciplinary Sarcoma Clinic and Sarcoma Tumor Board. The structured Multidisciplinary Sarcoma Tumor Board provides opportunity for improvement in logistics, teaching, quality, and enrollment in clinical trials. Keywords: sarcoma, sarcoma care, sarcoma tumor board, collaborative approach

  13. Vascularized fibula grafts for reconstruction of bone defects after resection of bone sarcomas

    DEFF Research Database (Denmark)

    Petersen, Michael Mørk; Hovgaard, Dorrit; Elberg, Jens Jørgen

    2010-01-01

    We evaluated the results of limb-sparing surgery and reconstruction of bone defects with vascularized fibula grafts in 8 consecutive patients (mean age at operation 13.6 years (range 4.1-24.2 years), female/male = 6/2) with bone sarcomas (BS) (osteosarcoma/Ewing's sarcoma/chondrosarcoma= 4...

  14. Postirradiation Sarcoma: Clinicopathologic Features and Role of Chemotherapy in the Treatment Strategy

    Directory of Open Access Journals (Sweden)

    Gaetan des Guetz

    2009-01-01

    Full Text Available Purpose. An analysis of the clinicopathologic features and treatment of patients was performed to guide evaluation and management of postirradiation sarcoma. Patients and Methods. Between 1994 and 2001, 25 patients with postirradiation sarcoma were treated in one center with different chemotherapy, mainly in neoadjuvant setting (19. Tumors for which these patients received radiotherapy initially were mainly breast carcinoma (for 15 patients. The postirradiation sarcomas were of different histopathologic forms, most frequently osteosarcoma, leiomyosarcoma, and angiosarcoma. Results. Of the 25 patients, 19 were initially treated with chemotherapy. Nine of 19 pretreated patients achieved clinical partial response (RP = 47%. Leiomyosarcomas were good responders (3/4 and undifferentiated sarcoma (3/5. Responders were more often treated with MAID (6/8. Eight of the 9 responders underwent surgery. Two patients achieved complete histological response. Seven of the 9 good responders are alive with a median follow up of 24 months. For all treated patients, median follow up 24 months (6–84 months, overall survival and disease free survival were, respectively, 17/25 (68%, and 14/25 (56%. Conclusion. From our data, postirradiation sarcoma should not be managed differently from primary sarcoma. Chemotherapy has to be included in the treatment plan of postirradiation sarcoma, in future studies.

  15. Delayed phlegmon with gallstone fragments masquerading as soft tissue sarcoma

    Science.gov (United States)

    Goodman, Laura F.; Bateni, Cyrus P.; Bishop, John W.; Canter, Robert J.

    2016-01-01

    Complications from lost gallstones after cholecystectomy are rare but varied from simple perihepatic abscess to empyema and expectoration of gallstones. Gallstone complications have been reported in nearly every organ system, although reports of malignant masquerade of retained gallstones are few. We present the case of an 87-year-old woman with a flank soft tissue tumor 4 years after laparoscopic cholecystectomy. The initial clinical, radiographic and biopsy findings were consistent with soft tissue sarcoma (STS), but careful review of her case in multidisciplinary conference raised the suspicion for retained gallstones rather than STS. The patient was treated with incisional biopsy/drainage of the mass, and gallstones were retrieved. The patient recovered completely without an extensive resectional procedure, emphasizing the importance of multidisciplinary sarcoma care to optimize outcomes for potential sarcoma patients. PMID:27333918

  16. The clinical use of biomarkers as prognostic factors in Ewing sarcoma

    Directory of Open Access Journals (Sweden)

    van Maldegem Annmeik M

    2012-02-01

    Full Text Available Abstract Ewing Sarcoma is the second most common primary bone sarcoma with 900 new diagnoses per year in Europe (EU27. It has a poor survival rate in the face of metastatic disease, with no more than 10% survival of the 35% who develop recurrence. Despite the remaining majority having localised disease, approximately 30% still relapse and die despite salvage therapies. Prognostic factors may identify patients at higher risk that might require differential therapeutic interventions. Aside from phenotypic features, quantitative biomarkers based on biological measurements may help identify tumours that are more aggressive. We audited the research which has been done to identify prognostic biomarkers for Ewing sarcoma in the past 15 years. We identified 86 articles were identified using defined search criteria. A total of 11,625 patients were reported, although this number reflects reanalysis of several cohorts. For phenotypic markers, independent reports suggest that tumour size > 8 cm and the presence of metastasis appeared strong predictors of negative outcome. Good histological response (necrosis > 90% after treatment appeared a significant predictor for a positive outcome. However, data proposing biological biomarkers for practical clinical use remain un-validated with only one secondary report published. Our recommendation is that we can stratify patients according to their stage and using the phenotypic features of metastases, tumour size and histological response. For biological biomarkers, we suggest a number of validating studies including markers for 9p21 locus, heat shock proteins, telomerase related markers, interleukins, tumour necrosis factors, VEGF pathway, lymphocyte count, and a number of other markers including Ki-67.

  17. Serologic association of human herpesvirus eight with posttransplant Kaposi's sarcoma in Saudi Arabia.

    Science.gov (United States)

    Qunibi, W; Al-Furayh, O; Almeshari, K; Lin, S F; Sun, R; Heston, L; Ross, D; Rigsby, M; Miller, G

    1998-02-27

    In Saudi Arabia, Kaposi's sarcoma occurs in 4.1% of renal transplant recipients and accounts for 70% of malignancies in this group. Human herpes virus 8 (HHV8) has been identified in the DNA of many of these patients. The association between HHV8 and Kaposi's sarcoma was investigated further in post-renal transplant Kaposi's sarcoma patients from a tertiary care hospital (King Faisal Specialist Hospital and Research Center) in Riyadh, Saudi Arabia (n = 14), and non-Kaposi's sarcoma controls with renal transplant (n = 18), chronic renal failure (n = 14), other cancers that did not affect renal function (n = 15), and healthy volunteers (n = 15). The median time from transplant to Kaposi's sarcoma was 13 months. A serum sample was assumed to have antibodies to HHV8 if antibody to either p40 or sVCA was detected. The prevalence of HHV8 seroreactivity was 13/14 (93%) in cases, 5/18 (28%) in renal transplants without Kaposi's sarcoma, and 11/62 (18%) in the aggregate control group. HHV8 seroreactivity was significantly more common (p 0.001) among transplant patients with Kaposi's sarcoma than those without this cancer (odds ratio, 33.80; 95% confidence interval, 2.96-904). These findings suggest an etiologic link between HHV8 and Kaposi's sarcoma presumably due to immunologic or cellular factors that influence host-virus interactions.

  18. Lymphangioma-like Kaposi sarcoma: case report.

    Science.gov (United States)

    Posada García, Celia; García-Cruz, Aranzazu; García-Doval, Ignacio; De La Torre, Carlos; Cruces, Manuel José

    2009-09-15

    Kaposi sarcoma (KS) is a multifocal vascular disease with uncertain histogenesis. It is characterized by clinical and histologic polymorphism. The "lymphangioma-like" variant is very uncommon, accounting for less than 5% of all cases. We report the case of a 76-year-old woman, HIV negative, with a 4-year history of classic Kaposi sarcoma treated with cryotherapy who developed new bullous lesions on her lower extremities. Biopsy revealed histologic findings of lymphangioma-like KS (LLKS), together with areas of classic KS; HHV-8 staining was positive. Diagnosis of LLKS was made and the patient was proposed for radiotherapy. The lymphangioma-like Kaposi sarcoma is a rare morphologic expression of KS characterized by dilated and bizarrely shaped vascular channels lined by flattened endothelium permeating the dermis. "Bulla-like" lesions have been considered as a clinical hallmark of this variant. Its histologic appearance suggests a lymphatic origin of KS and it may resemble other vascular tumors. Findings of areas of typical KS and positive staining for HHV-8 may help to make a definitive diagnosis.

  19. FDG PET/CT imaging in primary osseous and soft tissue sarcomas: a retrospective review of 212 cases

    Energy Technology Data Exchange (ETDEWEB)

    Charest, Mathieu [Lakeshore General Hospital, Service of Nuclear Medicine, Pointe-Claire (Canada); Hickeson, Marc; Lisbona, Robert; Novales-Diaz, Javier A.; Derbekyan, Vilma [McGill University Health Center, Department of Nuclear Medicine, Montreal (Canada); Turcotte, Robert E. [McGill University Health Center, Department of Orthopedic Surgery, Montreal (Canada)

    2009-12-15

    The aims of this study are to evaluate the sensitivity of FDG PET/CT for detection of soft tissue and osseous sarcomas on the basis of FDG avidity. We retrospectively evaluated 212 consecutive patients with known soft tissue or osseous sarcoma who had undergone a FDG PET/CT study for the initial staging or assessment of recurrence of disease. The maximum standardized uptake value (SUV{sub max}) of each primary and/or most intense metastatic lesion was measured and compared with the histological data provided in the final pathological reports. An SUV{sub max} of 2.5 or greater was considered positive for our analysis. Sufficient histopathological data were available for 160 soft tissue sarcomas and 52 osseous sarcomas. FDG PET/CT detected 93.9% of all sarcomas with a sensitivity of 93.7% for soft tissue sarcomas and 94.6% for osseous sarcomas. The sensitivities of the most common sarcoma histologies were 100% for leiomyosarcomas, 94.7% for osteosarcomas, 100% for Ewing's sarcomas, 88.9% for liposarcomas, 80.0% for synovial sarcomas, 100% for gastrointestinal stromal tumors, 87.5% for malignant peripheral nerve sheath tumors, 100% for fibroblastic and myoblastic sarcomas, and 100% for malignant fibrohistiocytic tumors. The receiver-operating characteristic curve revealed an area under the curve of 94% for the discrimination of low-grade and high-grade sarcomas imaged for initial staging by FDG PET/CT. The combined metabolic and morphological information of FDG PET/CT imaging allows high sensitivity for the detection of various sarcomas and accurate discrimination between newly diagnosed low-grade and high-grade sarcomas. (orig.)

  20. Efficacy of ATR inhibitors as single agents in Ewing sarcoma

    DEFF Research Database (Denmark)

    Nieto-Soler, Maria; Morgado-Palacin, Isabel; Lafarga, Vanesa

    2016-01-01

    Ewing sarcomas (ES) are pediatric bone tumors that arise from a driver translocation, most frequently EWS/FLI1. Current ES treatment involves DNA damaging agents, yet the basis for the sensitivity to these therapies remains unknown. Oncogene-induced replication stress (RS) is a known source of en...

  1. 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.

  2. What Should You Ask Your Doctor about Kaposi Sarcoma?

    Science.gov (United States)

    ... What Should You Ask Your Doctor About Kaposi Sarcoma? Kaposi Sarcoma Early Detection, Diagnosis, and Staging What Should You Ask Your Doctor About Kaposi Sarcoma? As you cope with Kaposi sarcoma (KS) and ...

  3. Diagnostic Performance of Endoscopic and Microscopic Procedures for Identifying Different Middle Ear Structures and Remaining Disease in Patients with Chronic Otitis Media: A Prospective Cohort Study.

    Directory of Open Access Journals (Sweden)

    Farhad Farahani

    Full Text Available The diagnostic performance of endoscopic and microscopic procedures for detecting diseases of the middle ear in patients with chronic otitis media (COM has rarely been investigated. This study was conducted to compare the performance of these procedures for identifying middle ear structures and their associated diseases in COM patients.In this prospective cohort study, 58 patients with chronic COM, who were candidates for tympanoplasty with or without a mastoidectomy, were enrolled. Before the surgical intervention, the middle ear was examined via an operating microscope and then through an endoscope to identify the middle ear structures as well as diseases associated with the middle ear.The patients were 15 years of age or older. The anatomical parts of the middle ear - the epitympanic, posterior mesotympanic, and hypotympanic structures - were more visible through an endoscope than through a microscope. In addition, the various segments of the mesotympanum, oval window, round window, and Eustachian tube were more visible via endoscopy. The post-operative endoscopic reevaluation of the middle ear revealed that a cholesteatoma had remained in four of 13 patients after surgery.According to the results of this study, in cases in which there is poor visibility with the operating microscope or the surgeon suspects remaining disease within the middle ear, endoscopy could be utilized to improve the evaluation of more hidden middle ear pits and structures, particularly if there is a potentially recrudescent pathology.

  4. Targeting the p53 Pathway in Ewing Sarcoma

    Directory of Open Access Journals (Sweden)

    Paul M. Neilsen

    2011-01-01

    Full Text Available The p53 tumour suppressor plays a pivotal role in the prevention of oncogenic transformation. Cancers frequently evade the potent antitumour surveillance mechanisms of p53 through mutation of the TP53 gene, with approximately 50% of all human malignancies expressing dysfunctional, mutated p53 proteins. Interestingly, genetic lesions in the TP53 gene are only observed in 10% of Ewing Sarcomas, with the majority of these sarcomas expressing a functional wild-type p53. In addition, the p53 downstream signaling pathways and DNA-damage cell cycle checkpoints remain functionally intact in these sarcomas. This paper summarizes recent insights into the functional capabilities and regulation of p53 in Ewing Sarcoma, with a particular focus on the cross-talk between p53 and the EWS-FLI1 gene rearrangement frequently associated with this disease. The development of several activators of p53 is discussed, with recent evidence demonstrating the potential of small molecule p53 activators as a promising systemic therapeutic approach for the treatment of Ewing Sarcomas with wild-type p53.

  5. [Malignant fibrous histiocytoma: pleomorphic sarcoma NOS or pleomorphic fibrosarcoma].

    Science.gov (United States)

    Meister, P

    2005-03-01

    The entity and nosology of pleomorphic malignant fibrous histiocytoma (MFH) is still ambiguous. The actual WHO-Classification uses pleomorphic malignant fibrous histiocytoma (MFH) and pleomorphic sarcoma NOS (not otherwise specified) synonymously. On the other hand text and illustrations convey the impression, that these tumors also could be pleomorphic lipo-, leio- or rhabdomyosarcomas etc. It would have been more informative to emphasize, that with the above mentioned specific sarcoma types MFH-like appearance may occur. Furthermore it would have been more up to date to consider pleomorphic sarcomas NOS as pleomorphic fibrosarcomas and include them in the chapter of fibroblastic and myofibroblastic tumors. This concept already has been carried out for the former myxoid variant of MFH, nowadays preferentially called myxofibrosarcoma. There is controversial discussion about the clinical significance of exact typing of pleomorphic sarcomas. Problems may also occur due to the lack of standards, which degree of desmin expression signifies leiomyosarcoma or just indicates myofibroblasts in MFH. The requirement of exclusion of other tumor-types before diagnosing pleomorphic fibrosarcoma still remains obligatory. After verification of the diagnosis pleomorphic sarcoma NOS or pleomorphic fibrosarcoma, grading e.g. according to criteria of the FFCCS can be carried out. Most cases of pleomorphic fibrosarcoma will qualify as high grade malignant.

  6. [Fast neutrons in the treatment of soft tissue sarcomas].

    Science.gov (United States)

    Chernichenko, V A; Tolstopiatov, B A; Monich, A Iu; Konovalenko, V F; Galakhin, K A; Palivets, A Iu; Vorona, A M

    1990-01-01

    Results of treatment of 101 cases of soft tissue sarcoma are presented in the paper. Preoperative irradiation technique and radical program of treatment are described. Combined radiation and surgical treatment was given to 45 patients whereas conservative--to 56. Sixty-three cases received adjuvant combination chemotherapy. Response and three-year survival rates were compared to those in control group treated by photons. The results observed in patients of combined and conservative treatment groups who had been irradiated with fast neutrons proved significantly better than in controls. These data suggest vistas in application of fast neutron irradiation for the treatment of soft tissue sarcomas.

  7. Ewing′s sarcoma in mandibular similar to dental abscess

    Directory of Open Access Journals (Sweden)

    Forouz Keshani

    2014-01-01

    This case report deals with a 16-year-old patient wrongly diagnosed with odontogenic infection and abscess, and hospitalized. As the symptoms did not remit, biopsy was carried out and the patient was operated on with Ewing′s sarcoma diagnosis.

  8. Uterine sarcomas-Recent progress and future challenges

    Energy Technology Data Exchange (ETDEWEB)

    Seddon, Beatrice M., E-mail: beatrice.seddon@uclh.nhs.uk [London Sarcoma Service, Department of Oncology, University College Hospital, 1st Floor Central, 250 Euston Road, London, NW1 2PG (United Kingdom); Davda, Reena [London Sarcoma Service, Department of Oncology, University College Hospital, 1st Floor Central, 250 Euston Road, London, NW1 2PG (United Kingdom)

    2011-04-15

    Uterine sarcomas are a group of rare tumours that provide considerable challenges in their treatment. Radiological diagnosis prior to hysterectomy is difficult, with the diagnosis frequently made post-operatively. Current staging systems have been unsatisfactory, although a new FIGO staging system specifically for uterine sarcomas has now been introduced, and may allow better grouping of patients according to expected prognosis. While the mainstay of treatment of early disease is a total abdominal hysterectomy, it is less clear whether routine oophorectomy or lymphadenectomy is necessary. Adjuvant pelvic radiotherapy may improve local tumour control in high risk patients, but is not associated with an overall survival benefit. Similarly there is no good evidence for the routine use of adjuvant chemotherapy. For advanced leiomyosarcoma, newer chemotherapy agents including gemcitabine and docetaxel, and trabectedin, offer some promise, while hormonal therapies appear to be more useful in endometrial stromal sarcoma. Novel targeted agents are now being introduced for sarcomas, and uterine sarcomas, and show some indications of activity. Non-pharmacological treatments, including surgical metastatectomy, radiofrequency ablation, and CyberKnife radiotherapy, are important additions to systemic therapy for advanced metastatic disease.

  9. RADIOGRAPHIC IMAGING OF BIPHASIC SYNOVIAL SARCOMA ON LEFT ELBOW

    Directory of Open Access Journals (Sweden)

    Sitanggang Firman P

    2013-04-01

    Full Text Available Synovial sarcoma is the fourth most common type of sarcoma. It represents between 5% -10% of all soft tissue sarcomas and most prevalent in aged between 15 - 40 years. Synovial sarcoma is the most commonly misdiagnosed soft tissue malignancy, initially as an inflammation process,often because it may be slow-growing, have a benign appearance on imaging studies, may vary in size, and may have pain similar to that associated with trauma.    A rare case is presented of 17 years old women with a synovial sarcoma biphasic. The primary tumor originated in the left elbow since 2004. Since then, the patient has had repeated passive or active left elbow pain and tenderness. No history of trauma. She has not developed metastases of the lung. The patient refused to have surgery and others medical procedures that already planned and explained to her. The conclusion of this case report point that radiology is important to diagnosis and planning for further management

  10. Pulmonary involvement in Kaposi sarcoma: correlation between imaging and pathology.

    Science.gov (United States)

    Gasparetto, Taisa Davaus; Marchiori, Edson; Lourenço, Sílvia; Zanetti, Gláucia; Vianna, Alberto Domingues; Santos, Alair A S M D; Nobre, Luiz Felipe

    2009-07-14

    Kaposi sarcoma is a low-grade mesenchymal tumor involving blood and lymphatic vessels. There are four variants of this disease, each presenting a different clinical manifestation: classic or sporadic, African or endemic, organ transplant-related or iatrogenic, and AIDS-related or epidemic. Kaposi sarcoma is the most common tumor among patients with HIV infection, occurring predominantly in homosexual or bisexual men. The pulmonary involvement in Kaposi sarcoma occurs commonly in critically immunosupressed patients who commonly have had preceding mucocutaneous or digestive involvement.The etiology of Kaposi sarcoma is not precisely established; genetic, hormonal, and immune factors, as well as infectious agents, have all been implicated. There is evidence from epidemiologic, serologic, and molecular studies that Kaposi sarcoma is associated with human herpes virus type 8 infection. The disease starts as a reactive polyclonal angioproliferative response towards this virus, in which polyclonal cells change to form oligoclonal cell populations that expand and undergo malignant transformation.The diagnosis of pulmonary involvement in Kaposi sarcoma usually can be made by a combination of clinical, radiographic, and laboratory findings, together with the results of bronchoscopy and transbronchial biopsy. Chest high-resolution computed tomography scans commonly reveal peribronchovascular and interlobular septal thickening, bilateral and symmetric ill-defined nodules in a peribronchovascular distribution, fissural nodularity, mediastinal adenopathies, and pleural effusions. Correlation between the high-resolution computed tomography findings and the pathology revealed by histopathological analysis demonstrate that the areas of central peribronchovascular infiltration represent tumor growth involving the bronchovascular bundles, with nodules corresponding to proliferations of neoplastic cells into the pulmonary parenchyma. The interlobular septal thickening may represent

  11. Pulmonary involvement in Kaposi sarcoma: correlation between imaging and pathology

    Directory of Open Access Journals (Sweden)

    Vianna Alberto

    2009-07-01

    Full Text Available Abstract Kaposi sarcoma is a low-grade mesenchymal tumor involving blood and lymphatic vessels. There are four variants of this disease, each presenting a different clinical manifestation: classic or sporadic, African or endemic, organ transplant-related or iatrogenic, and AIDS-related or epidemic. Kaposi sarcoma is the most common tumor among patients with HIV infection, occurring predominantly in homosexual or bisexual men. The pulmonary involvement in Kaposi sarcoma occurs commonly in critically immunosupressed patients who commonly have had preceding mucocutaneous or digestive involvement. The etiology of Kaposi sarcoma is not precisely established; genetic, hormonal, and immune factors, as well as infectious agents, have all been implicated. There is evidence from epidemiologic, serologic, and molecular studies that Kaposi sarcoma is associated with human herpes virus type 8 infection. The disease starts as a reactive polyclonal angioproliferative response towards this virus, in which polyclonal cells change to form oligoclonal cell populations that expand and undergo malignant transformation. The diagnosis of pulmonary involvement in Kaposi sarcoma usually can be made by a combination of clinical, radiographic, and laboratory findings, together with the results of bronchoscopy and transbronchial biopsy. Chest high-resolution computed tomography scans commonly reveal peribronchovascular and interlobular septal thickening, bilateral and symmetric ill-defined nodules in a peribronchovascular distribution, fissural nodularity, mediastinal adenopathies, and pleural effusions. Correlation between the high-resolution computed tomography findings and the pathology revealed by histopathological analysis demonstrate that the areas of central peribronchovascular infiltration represent tumor growth involving the bronchovascular bundles, with nodules corresponding to proliferations of neoplastic cells into the pulmonary parenchyma. The interlobular

  12. Drugs Approved for Kaposi Sarcoma

    Science.gov (United States)

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

  13. SYNOVIAL SARCOMA IN CHILDHOOD: CLINICAL AND RADIOLOGICAL FINDINGS

    Institute of Scientific and Technical Information of China (English)

    Xu Deyong; Zhan Alai; Luan Hongmei; Feng Weihua; Sun Xihe; Yang Zuwen

    1998-01-01

    Objective: To study the clinical characteristics and radiological features of synovial sarcoma in childhood and its relation to the diagnosis and treatment. Methods:The clinical radiological features of 15 children with synovial sarcoma proved surgically and pathologically were analyzed. Results: In children, the tumor boundaries are poorly defined due to paucity of fat, and metastasis usually occurs early. Eight patients in this series had bone involvement, including: direct erosion by tumor causing cortical destruction, indirect pressure defect with sharp margin and reactive bone sclerosis and bone destruction of the primary intraosseous synovial sarcoma.Conclusion: The tumor is often misdiagnosed, the final confirmed diagnosis must be made by histological examination with imaging findings. It is emphasized that the patients should be treated with radiotherapy and chemotherapy preoperatively and postoperatively.

  14. Kaposi´s sarcoma, epidemic type. Case presentation

    Directory of Open Access Journals (Sweden)

    Gilberto Serrano Ocaña

    2009-05-01

    Full Text Available Before the AIDS epidemic, Kaposi's sarcoma was found mainly in elderly men of Mediterranean coast, eastern European background and Jewish ancestry (rarely in older women and is a slow growing skin tumor. In AIDS patients, the KS tends to develop more rapidly compromising the skin, lungs, gastrointestinal tract and other organs. In people with AIDS, Kaposi's sarcoma is caused by an interaction between HIV, a weakened immune system and human herpes virus 8. It affects approximately 20% of people with HIV that don’t take antiretroviral drugs. It is more common in homosexual’s patients, but may appear in any HIV positive individual, in Africa where heterosexual HIV transmission route is the most important can also be found in children and women. We are presenting a case of Kaposi sarcoma in a young female admitted at the Internal Medicine Department of Dora Nginza Hospital.

  15. [Oral lesions in Kaposi sarcoma: clinical and radiotherapeutic considerations].

    Science.gov (United States)

    Barberis, M; Brenna Betti, N; Lauritano, D; Sangiani, L; Spadari, F; Villa, S

    1996-01-01

    The epidemic form of Kaposi's sarcoma is the most frequent tumor in sieropositive patients. Every part of the body including oral cavity is affected by these lesions. According to modern acknowledgement in treating oropharynge carcinoma, radiotherapy is used for management of oral Kaposi's sarcoma. This paper reports a study of 10 patients suffering from Kaposi's sarcoma correlated to AIDS (EKS) treated with radiotherapy and chemiotherapy, achieving good results, at the Istituto Nazionale per lo Studio e la Cura dei Tumori of Milan (Divisone di Radioterapia C) from 1988 to 1992. Treatment has been performed using linear accelerator (6 Mev) or Co 60 unity in order to reach the deepest layer of mucosa lesions. Radiotherapy schedule consisted of 150-200 cGy daily fractions given 5 times/week (w) for 4-5 w in split-course.

  16. Two cases with fatal outcome following total lung irradiation for metastatic bone sarcoma

    OpenAIRE

    Lia, K.; Bruland, Ø.S.; Randem, H.L.; Aksnes, L.H.; Poulsen, J P; Taksdal, I.; Sundby Hall, K.

    2013-01-01

    We report a single institution experience with total lung irradiation in 53 metastatic bone sarcoma patients in the context of two young female patients who died from treatment-induced pulmonary toxicity. A radiation dose of 19.5?Gy in 1.5?Gy daily fractions was given as two opposing fields with a conventional technique. Both patients succumbed within 3 months following radiotherapy. One patient had osteosarcoma whereas the other advanced Ewing's sarcoma; both with widespread metastases to th...

  17. The diagnostic and prognostic value of {sup 18}F-FDG PET/CT in the initial assessment of high-grade bone and soft tissue sarcoma. A retrospective study of 89 patients

    Energy Technology Data Exchange (ETDEWEB)

    Fugloe, Hanna Maria; Hovgaard, Dorrit; Petersen, Michael M. [Copenhagen University Hospital, Department of Orthopaedic Surgery, Rigshospitalet, Copenhagen Oe (Denmark); Joergensen, Simon Moeller; Loft, Annika [Copenhagen University Hospital, Department of Clinical Physiology, Nuclear Medicine and PET, Rigshospitalet, Copenhagen (Denmark)

    2012-09-15

    To evaluate the feasibility of {sup 18}F-FDG PET/CT for initial assessment in high-grade bone sarcomas (BS) and soft tissue sarcomas (STS). During the years 2001-2010, 89 patients (30 BS, 59 STS) referred for further evaluation and surgical treatment of a high-grade BS or STS also had a PET/CT scan performed for staging preoperatively (n = 68) or within 1 month of surgery (n = 21). Metastatic lesions suggested on the PET/CT scan were confirmed or rejected by histological evaluation, by additional imaging or by follow-up. In 68 patients (28 BS, 40 STS) the relationship between the maximal standardized uptake value (SUVmax) of the primary tumour and survival was examined. The PET/CT scan suggested the presence of 13 metastatic lesions in BS patients (5 lymph node, 8 distant) and 21 metastatic lesions (6 lymph node, 15 distant) in STS patients. The calculated sensitivity (SE) and specificity (SP) were 95 % and 96 % for detection of distant metastases, and the predictive value (PV) of a positive or a negative test was 87 % and 98 %, respectively. SE and SP were 100 % and 90 % for detection of lymph node metastases, and the PV of a positive or a negative test was 27 % and 100 %, respectively. The 5-year survival was 81 % among patients with SUVmax below the median value ({<=}10), but was 33 % among those with SUVmax >10. FDG PET/CT for the initial assessment of patients with high-grade BS or STS was feasible with high SE and SP, but in those with lymph node metastases the PV of a positive test was low. The SUVmax of the primary tumour was a strong prognostic factor for survival. (orig.)

  18. Should sulfonylureas remain an acceptable first-line add-on to metformin therapy in patients with type 2 diabetes? Yes, they continue to serve us well!

    Science.gov (United States)

    Abrahamson, Martin J

    2015-01-01

    Since their introduction to clinical practice in the 1950s, sulfonylureas have been widely prescribed for use in patients with type 2 diabetes. Of all the other medications currently available for clinical use, only metformin has been used more frequently. However, several new drug classes have emerged that are reported to have equal glucose-lowering efficacy and greater safety when added to treatment of patients in whom metformin monotherapy is no longer sufficient. Moreover, current arguments also suggest that the alternative drugs may be superior to sulfonylureas with regard to the risk of cardiovascular complications. Thus, while there is universal agreement that metformin should remain the first-line pharmacologic therapy for those in whom lifestyle modification is insufficient to control hyperglycemia, there is no consensus as to which drug should be added to metformin. Therefore, given the current controversy, we provide a Point-Counterpoint on this issue. In the point narrative presented below, Dr. Abrahamson provides his argument suggesting that avoiding use of sulfonylureas as a class of medication as an add-on to metformin is not appropriate as there are many patients whose glycemic control would improve with use of these drugs with minimal risk of adverse events. In the following counterpoint narrative, Dr. Genuth suggests there is no longer a need for sulfonylureas to remain a first-line addition to metformin for those patients whose clinical characteristics are appropriate and whose health insurance and/or financial resources make an alternative drug affordable.

  19. Should sulfonylureas remain an acceptable first-line add-on to metformin therapy in patients with type 2 diabetes? No, it's time to move on!

    Science.gov (United States)

    Genuth, Saul

    2015-01-01

    Since their introduction to clinical practice in the 1950s, sulfonylureas have been widely prescribed for use in patients with type 2 diabetes. Of all the other medications currently available for clinical use, only metformin has been used more frequently. However, several new drug classes have emerged that are reported to have equal glucose-lowering efficacy and greater safety when added to treatment of patients in whom metformin monotherapy is no longer sufficient. Moreover, current arguments also suggest that the alternative drugs may be superior to sulfonylureas with regard to the risk of cardiovascular complications. Thus, while there is universal agreement that metformin should remain the first-line pharmacologic therapy for those in whom lifestyle modification is insufficient to control hyperglycemia, there is no consensus as to which drug should be added to metformin. Therefore, given the current controversy, we provide a Point-Counterpoint on this issue. In the preceding point narrative, Dr. Abrahamson provides his argument suggesting that avoiding use of sulfonylureas as a class of medication as an add-on to metformin is not appropriate as there are many patients whose glycemic control would improve with use of these drugs with minimal risk of adverse events. In the counterpoint narrative below, Dr. Genuth suggests there is no longer a need for sulfonylureas to remain a first-line addition to metformin for those patients whose clinical characteristics are appropriate and whose health insurance and/or financial resources make an alternative drug affordable.

  20. Testing Current and Developing Novel Therapies for NF1-Mutant Sarcomas in a Genetically Engineered Mouse Model

    Science.gov (United States)

    2015-04-01

    1   AWARD NUMBER: W81XWH-14-1-0067 TITLE: Testing Current and Developing Novel Therapies for NF1 -Mutant Sarcomas in a Genetically Engineered...Mar 2014 - 14 Mar 2015 4. TITLE AND SUBTITLE Testing Current and Developing Novel Therapies for NF1 - Mutant Sarcomas in a Genetically Engineered...Patients with Neurofibromatosis type 1 ( NF1 ) are at increased risk for developing malignant tumors of the connective tissue called soft-tissue sarcomas

  1. Primary sarcoma of the lung: A very rare diagnosis and poor prognosis

    Directory of Open Access Journals (Sweden)

    Rhizlane Belbaraka

    2014-01-01

    Full Text Available Primary lung sarcoma is a very rare malignant tumor, accounting for less than 0.5% of all lung tumors. We describe the case of a 50-year-old man who presented with primary pulmonary sarcoma. X-ray chest and thoracic computed tomography (CT revealed a 40 mm Χ 60 mm mass in the right upper lobe. Biopsy was reported as fusiform sarcoma. Extensive clinical examination, followed by full body CT scan was performed to exclude primary synovial sarcoma located peripherally and distant metastases. Hence, surgical excision was planned after three cycles of chemotherapy, but the patient died after the second cycle. Because a sarcoma of the lung is rare, data regarding its natural history and published cases are limited. Further investigation and data collection are required to optimize the treatment of this group of rare and aggressive tumors.

  2. Diagnostic confusion resulting from CD56 expression by cutaneous myeloid sarcoma

    Directory of Open Access Journals (Sweden)

    Sheeja T. Pullarkat

    2009-12-01

    Full Text Available Myeloid sarcomas are tumor masses composed of aggregates of malignant myeloid precursors in extramedullary sites including the skin. We report a case of myeloid sarcoma in a patient who presented with an ear lobe mass and facial nerve paralysis. Expression of CD56 by the malignant cells led to an initial misdiagnosis as Merkel cell tumor. Comprehensive pathological evaluation confirmed the diagnosis of myeloid sarcoma with aberrant expression of CD56 and carrying the translocation t(8;21 (q22;q22. Aberrant antigen expression by cutaneous myeloid sarcomas can cause diagnostic confusion with other cutaneous neoplasms. This is especially relevant when myeloid sarcoma is the sole manifestation of acute myeloid leukemia.

  3. Retroperitoneal Pleomorphic Lipo sarcoma Mimicking Adrenal Cancer in F-18 FDG PET/CT

    Energy Technology Data Exchange (ETDEWEB)

    Yoon, Minki; Kim, Seogjoon [Good Samaritan Hospital, Pohang (Korea, Republic of)

    2010-09-15

    Lipo sarcoma is the second most common type of soft tissue sarcoma, but pleomorphic lipo sarcoma is the least common subtype. We present the case of a 42-year-old man who had experienced intermittent left flank pain for a month. A large soft-tissue mass was detected by ultrasonography in a local clinic, and he was referred for further evaluation. Positron emission tomography/computed tomography (PET/CT) with F-18 fluoro-2-deoxy-D-glucose (F-18 FDG) showed intense uptake in the retroperitoneal mass, which mimicked an adrenal cancer. The patient underwent left radical nephroadrenalectomy, and the tumor was revealed to be a pleomorphic lipo sarcoma upon pathological examination. When there is a large retroperitoneal mass with intense F-18 FDG activity, the possibility of a pleomorphic lipo sarcoma should be considered.

  4. Nursing for one patient with classical Kaposi's sarcoma%1例经典型Kaposi肉瘤患者的护理

    Institute of Scientific and Technical Information of China (English)

    张昕婷

    2007-01-01

    @@ Kaposi肉瘤(Kaposi's sarcoma)是一种极其罕见的发生在皮肤、黏膜、淋巴结、内脏的恶性肿瘤,在临床上分为经典型、非洲地方型、免疫抑制/器官移植相关型和艾滋病相关型[3,4].我院2006年3-8月收治了1例经典型Kaposi肉瘤患者,现报道如下.

  5. Granulocytic sarcoma masquerading as Ewing′s sarcoma: A diagnostic dilemma

    Directory of Open Access Journals (Sweden)

    Haresh Kunhi

    2008-01-01

    Full Text Available An eleven-year-old boy presented with a swelling in his left elbow. Radiologically the features were that of an Ewing′s sarcoma involving the ulna. Histopathology showed small round cell tumor strongly positive for Monoclonal Imperial Cancer research fund 2 (MIC2 antigen. Similar cells in the bone marrow were involved with MIC2 positivity. The patient developed skin lesions, which on biopsy were found to be chloromas. The initial biopsies were reevaluated with special stains revealing granulocytic sarcomas in acute myeloid leukemia masquerading as Ewing′s due to its MIC2 positivity. The possibility of myeloid neoplasms should be considered routinely with known MIC2 positive round cell tumors.

  6. How Are Soft Tissue Sarcomas Diagnosed?

    Science.gov (United States)

    ... type of cancer or a benign disease. Several types of biopsies are used to diagnose sarcomas. Doctors experienced with ... But if FNA results suggest a sarcoma, another type of biopsy will usually be done to remove enough tissue ...

  7. General Information about Childhood Soft Tissue Sarcoma

    Science.gov (United States)

    ... forms in soft tissues of the body, including muscle, tendons, fat, blood vessels, lymph vessels, nerves, and tissue around joints. Soft tissue sarcoma occurs in children and adults. Soft ... disorders can increase the risk of childhood soft tissue sarcoma. Anything ...

  8. Stages of Childhood Soft Tissue Sarcoma

    Science.gov (United States)

    ... forms in soft tissues of the body, including muscle, tendons, fat, blood vessels, lymph vessels, nerves, and tissue around joints. Soft tissue sarcoma occurs in children and adults. Soft ... disorders can increase the risk of childhood soft tissue sarcoma. Anything ...

  9. Treatment Options for Childhood Soft Tissue Sarcoma

    Science.gov (United States)

    ... forms in soft tissues of the body, including muscle, tendons, fat, blood vessels, lymph vessels, nerves, and tissue around joints. Soft tissue sarcoma occurs in children and adults. Soft ... disorders can increase the risk of childhood soft tissue sarcoma. Anything ...

  10. Treatment Option Overview (Childhood Soft Tissue Sarcoma)

    Science.gov (United States)

    ... forms in soft tissues of the body, including muscle, tendons, fat, blood vessels, lymph vessels, nerves, and tissue around joints. Soft tissue sarcoma occurs in children and adults. Soft ... disorders can increase the risk of childhood soft tissue sarcoma. Anything ...

  11. Epithelioid Sarcoma: Opportunities for Biology-driven Targeted Therapy

    Directory of Open Access Journals (Sweden)

    Jonathan eNoujaim

    2015-08-01

    Full Text Available Epithelioid sarcoma is a soft tissue sarcoma of children and young adults for which the preferred treatment for localised disease is wide surgical resection. Medical management is to a great extent undefined, and therefore for patients with regional and distal metastases, the development of targeted therapies is greatly desired. In this review we will summarize clinically-relevant biomarkers (e.g., SMARCB1, CA125, dysadherin and others with respect to targeted therapeutic opportunities. We will also examine the role of EGFR, mTOR and polykinase inhibitors (e.g., sunitinib in the management of local and disseminated disease. Towards building a consortium of pharmaceutical, academic and non-profit collaborators, we will discuss the state of resources for investigating epithelioid sarcoma with respect to cell line resources, tissue banks, and registries so that a roadmap can be developed towards effective biology-driven therapies.

  12. Simultaneous lymph node involvement by Castleman disease and Kaposi sarcoma

    Directory of Open Access Journals (Sweden)

    Luciana Wernersbach Pinto

    2011-02-01

    Full Text Available Both multicentric Castleman disease and Kaposi sarcoma are more frequently observed in HIV infected patients. The coexistence of these Human herpesvirus 8 related lesions, in the same tissue, has been observed, but literature reports are scant. On the other hand, the expression of HHV-8-LANA-1 is easily demonstrable by immunohistochemistry. This has been shown to be a powerful tool for the diagnosis of these entities. The aim of this report is to communicate our experience with a case of multicentric Castleman disease occurring in the setting of HIV infection, which demonstrated microscopic Kaposi sarcoma in the same lymph node during the pathological work-up

  13. SARC: Development and Support of a Sarcoma Research Consortium Infrastructure

    Energy Technology Data Exchange (ETDEWEB)

    Arkison, Jim

    2007-10-29

    SARC is a non-for-profit organization whose mission and vision is to advocate for the collaboration on the design of clinical trials on sarcoma, to further the knowledge regarding the diagnosis and treatment of sarcoma and provide accurate and up to date information to physicians, patients and families. The objectives are to assist in the development of the infrastructure for the continued growth and spectrum of clinical research, to facilitate biannual meeting of investigators, and to develop a preclinical research base that would design and conduct research that would improve the process of drug treatments selected for clinical research trials.

  14. Ewing sarcoma in adolescents and young adults: diagnosis and treatment

    Directory of Open Access Journals (Sweden)

    Davis LE

    2014-09-01

    Full Text Available Lara E Davis,1,2 Suman Malempati2 1Department of Medicine, 2Department of Pediatrics, Oregon Health and Science University, Portland, OR, USA Abstract: Ewing sarcoma (ES is a rare tumor that most often occurs in adolescents and young adults. This review discusses the diagnosis, prognosis, and treatment of localized and metastatic ES, with an emphasis on the care of adolescent and young adult patients. The pathobiology is reviewed. Particular attention is given to recent and current clinical trials, and an introduction to future directions for therapy of ES is provided. Keywords: Ewing, sarcoma, primitive neuroectodermal tumor, PNET

  15. Clear cell sarcoma: A case mimicking primary cutaneous malignant melanoma

    Directory of Open Access Journals (Sweden)

    Rodriguez-Martin M

    2009-01-01

    Full Text Available Clear cell sarcoma (CCS is a recently described variant of sarcoma characterized by prominent clear cells showing features similar to clear cell melanoma. This neoplasm was first described by Dr. Franz M. Erzinger. Primary CCS usually arises in deeper soft tissues, in association with fascia, tendons, or aponeuroses. Characteristic translocation t(12;22 (q13;q12 has been considered pathognomonic for CCS. Prognosis is related to tumor size. An early recognition and initial radical surgery is the key to a favourable outcome. We present a patient with an unusual neoplasm that resembled malignant melanoma.

  16. Reasons Why Individuals Remain Uninsured Under the Affordable Care Act: Experiences of Patients at a Student-Run Free Clinic in Michigan, a Medicaid Expansion State.

    Science.gov (United States)

    Desmond, Brian S; Laux, Molly A; Levin, Carolyn C; Huang, Jiaxin; Williams, Brent C

    2016-04-01

    Since the enactment of the Affordable Care Act (ACA), many people remain uninsured. While studies have examined this population, few have explored patients' experiences seeking insurance. To elucidate these individuals' perspectives, we surveyed patients (n = 80) at the University of Michigan Student-Run Free Clinic concerning their activity accessing insurance. Over half of respondents had sought insurance in the past 6 months; 57 % of respondents qualified for Medicaid by self-reported income (≤138 % FPL) and all but one were eligible for tax credits on the Health Insurance Marketplace. However, only 35 % of apparently Medicaid-eligible respondents had recently applied. There was no significant difference in the rate of applying for those above the income cutoff (p = 0.901). Perceived expense of plans and belief of ineligibility for Medicaid discouraged respondents from actively seeking insurance. Personalized outreach emphasizing new Medicaid eligibility requirements and tax credits may be needed to facilitate Medicaid enrollment for some uninsured persons under the ACA.

  17. The prevalence of human herpesvirus 8 genotypes in Kaposi\\\\\\'s sarcoma in Iran by using molecular technique

    Directory of Open Access Journals (Sweden)

    Reza Shah Siah

    2013-10-01

    Full Text Available Background: In the Mediterranean region , Kaposi's sarcoma (KS has a high prevalence especially in patients with AIDS. Iran is located close to the Mediterranean region and the HIV prevalence is increasing in our country . In some stages, Kaposi's sarcoma is morphologically similar to other vascular tumors. Owing to the presence of human herpesvirus 8 (HHV-8 in all cases of Kaposi's sarcoma , detection of virus DNA by PCR method can help in the identification of non-diagnostic cases. Moreover, the prevalence of HHV-8 genotypes is different in various regions of the world and in different races. There are limited studies performed on the HHV-8 genotypes in Iranian population. Methods: Patients with Kaposi's sarcoma from 2001 to 2011 who refer to Tehran Razi Hospital were enrolled in this study. HHV-8 DNA was extracted from paraffin blocks and amplification of the virus genome was performed by PCR method . Finally, the target DNA fragment was used for sequencing and genotype determination. Results: PCR was performed on 53 cases. In 8 cases with suspicious morphology, PCR was negative and they were excluded from study. Of remaining 45 cases, 35 had positive PCR results, 7 had negative results and 3 had low PCR product. Samples from 28 cases that had positive PCR results, which were acceptable for genotyping, were chosen for sequencing. Twenty cases had genotype C, 7 cases had genotype A and one case was negative. The results are consistent with other studies in our geographical area. No correlation was found between the different microscopic stages and HHV-8 Genotypes. Conclusion: Since the HHV-8 is obtained in almost 100% of KS lesions and PCR s ensitivity in detection of the virus is close to 100 %, KS diagnosis can be confirmed in suspicious cases by detection of HHV-8 DNA on paraffin blocks. Moreover the prevalence of HHV-8 genotype was determined in Iran.

  18. Kaposi′s sarcoma: HIV-negative man with isolated penile localization

    Directory of Open Access Journals (Sweden)

    Soufiane Mellas

    2010-07-01

    Full Text Available Kaposi′s sarcoma is the malignant proliferation of the endothelial cell vessels. Its genesis is still unclear; however, it seems to be related to the herpes virus infection (HHV-8. This neoplasia usually affects the lower limbs and the affected persons are mostly from the Mediterranean region. The exclusive penile localization of the Kaposi′s sarcoma in a patient with a negative HIV serologia is exceptional. Our case is of a 73-year old patient with a negative HIV serology presenting an exclusive penile localization of the Kaposi′s sarcoma treated by radiotherapy.

  19. Features at diagnosis of 324 patients with acromegaly did not change from 1981 to 2006; Acromegaly remains under-recognized and under-diagnosed

    Science.gov (United States)

    Reid, Tirissa J.; Post, Kalmon D.; Bruce, Jeffrey N.; Kanibir, M. Nabi; Reyes-Vidal, Carlos M.; Freda, Pamela U.

    2009-01-01

    BACKGROUND Traditionally, acromegaly evaded diagnosis until in its clinically obvious later stages when treatment is more difficult. Over the last 25 years diagnostic tests have improved, but whether clinical disease detection also improved was unknown so we tested if disease severity at diagnosis had changed from 1981 to 2006. METHODS Data on 324 consecutive acromegaly patients presenting from 1981–2006 at two New York City hospitals were collected by retrospective review (n=324) and by interview (n=200). The main complaint, acromegaly-associated co-morbidities, signs, symptoms, healthcare providers visited, pre-operative growth hormone (GH) and insulin-like growth factor I (IGF-I) levels and pituitary tumor size at diagnosis were compared in patients presenting in the earlier vs. later halves of the time period. RESULTS Times from symptom onset to diagnosis were 5.9 yr. (early) vs. 5.2 yr. (late)(p=ns). At diagnosis, 96% of early and late groups had facial feature changes and/or hand/foot enlargement. Co-morbidities included hypertension (HTN) 37 % (early) vs. 36% (late), carpal tunnel syndrome (24 vs. 24%), sleep apnea (13 vs. 29%)(p acromegaly patients were unchanged from 1981–2006. Most patients still have marked manifestations of acromegaly at diagnosis suggesting that acromegaly remains clinically under-recognized. Healthcare professionals should more commonly consider acromegaly, which can lead to earlier diagnosis and better treatment outcome. PMID:19473180

  20. Comportamiento Inusual de Sarcoma de alto grado de malignidad en abdomen. Reporte de un caso

    Directory of Open Access Journals (Sweden)

    Maryem Seco Meza

    2014-03-01

    . The most aggressive and with major metastatic capacity are high-grade sarcomas, while low-grade ones are aggressive but of local form. In this regard, the case of a 52-year-old female patient, from a rural area, with 3 months of onset of symptoms, is presented. The patient showed a progressive increase in abdominal volume, sensation of weight and palpable mass, concomitant vomiting, weakness, weight loss and lower limb edema. Abdominal ultrasound was performed which showed a sonographically mixed tumor of 16 x 20 cm in the midline. A CT scan showed a heterogeneous space-occupying lesion, oval, with irregular borders of 15 x 10 cm that displaces thin bowel loops. The medial laparotomy showed no peritoneal implants and carcinomatosis; the tumor was highly vascularized, encapsulated, of smooth contexture, firm, and with defined edges of 15 x 20 cm. After resection, no lymphadenopathy or local and distant metastases were observed. The pathology report identified a high-grade sarcoma. After two years of surgery, no metastasis was found. In contrast to what is reported in the literature, the sarcoma remained "benign" despite its histology, which is unusual considering the natural history of the disease

  1. Regional prevalence and transmission route of Kaposi's sarcoma-associated herpes virus in Zhejiang, China

    Institute of Scientific and Technical Information of China (English)

    JU Hong-zhen; ZHU Biao; WANG Ying-jie; SHENG Zi-ke; SHENG Ji-fang

    2012-01-01

    Background The infection of Kaposi's sarcoma-associated herpes virus (KSHV) is most likely the cause of clinical Kaposi's sarcoma,primary effusion lymphoma,and multi-center Castleman's disease.KSHV infection has very limited epidemiological survey data in China,and its definite mode of transmission remains controversial.This study aimed to determine the infection status and the main transmission route of KSHV in Chinese population.Methods An enzyme-linked immunosorbent assay (ELISA) utilizing KSHV ORF65 recombinant protein was employed to analyze the antibody response to KSHV ORF65 in sera from 122 healthy physical examination people,107intravenous drug users,135 non-intravenous drug users,211 hepatitis B (HBV) patients infected via blood transmission,107 kidney transplant recipients,and 72 female sex workers in Zhejiang Province in Southeast China.Results KSHV infection occurred relatively common (13.1%) in healthy population in Zhejiang,China.Infection rate was 16.7% in female sex workers,but significantly elevated in intravenous drug addicts (58.9%),blood-transmitted HBV patients (28.0%) and kidney transplant patients (41.1%).Conclusion Blood borne transmission of KSHV is probably the main route of infection in Zhejiang Province.

  2. Physical Function and Spinal Mobility Remain Stable Despite Radiographic Spinal Progression in Patients with Ankylosing Spondylitis Treated with TNF-α Inhibitors for Up to 10 Years.

    Science.gov (United States)

    Poddubnyy, Denis; Fedorova, Aleksandra; Listing, Joachim; Haibel, Hildrun; Baraliakos, Xenofon; Braun, Jürgen; Sieper, Joachim

    2016-12-01

    The aim of the study was to investigate the effect of radiographic spinal progression and disease activity on function and spinal mobility in patients with ankylosing spondylitis (AS) treated with tumor necrosis factor-α (TNF-α) inhibitors for up to 10 years. Patients with AS who participated in 2 longterm open-label extensions of clinical trials with TNF-α inhibitors (43 receiving infliximab and 17 receiving etanercept) were included in this analysis based on the availability of spinal radiographs performed at baseline and at a later timepoint (yr 2, 4, 6, 8, and 10) during followup. Spinal radiographs were scored according to the modified Stoke Ankylosing Spondylitis Spine Score (mSASSS). Function was assessed by the Bath Ankylosing Spondylitis Functional Index (BASFI), spinal mobility by the Bath Ankylosing Spondylitis Metrology Index (BASMI), and disease activity by the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI). After the initial improvement, BASFI and BASMI remained remarkably stable at low levels over up to 10 years despite radiographic spinal progression. In the generalized mixed effects model analysis, no association between the mSASSS and the BASFI change (β = 0.0, 95% CI -0.03 to 0.03) was found, while there was some effect of mSASSS changes on BASMI changes over time (β = 0.05, 95% CI 0.01-0.09). BASDAI showed a strong association with function (β = 0.64, 95% CI 0.54-0.73) and to a lesser extent, with spinal mobility (β = 0.14, 95% CI 0.01-0.26). Functional status and spinal mobility of patients with established AS remained stable during longterm anti-TNF-α therapy despite radiographic progression. This indicates that reduction and continuous control of inflammation might be able to outweigh the functional effect of structural damage progression in AS.

  3. 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

  4. Metastatic endometrial stromal sarcoma: a case report

    Directory of Open Access Journals (Sweden)

    Shobha S. Pillai

    2014-06-01

    Full Text Available Endometrial Stromal Sarcoma (ESS is a rare slow growing tumour of mesodermal origin arising from the stroma of the endometrium and accounting for less than 1% of all uterine cancers. It is characterized by late recurrences and distant metastases. This report presents a case of ESS in a 40 year old nulliparous woman who had a myomectomy for a clinically suspected Leiomyoma uterus in a local hospital. The histopathological examination of the specimen revealed ESS and the patient was referred to our tertiary institute. Here after investigations including a CT scan which also revealed pulmonary metastases, patient underwent Modified Radical Hysterectomy with Bilateral Salpingo-oophorectomy with pelvic lymph node sampling. Histopathological Examination of the uterine specimen confirmed the diagnosis. The patient was given the option of referral to a thoracic surgeon for resection of the isolated lung metastasis, but she refused this and opted instead for hormone therapy which she is presently undergoing. ESS is a very rare tumour often presenting with clinical and examination findings suggestive of leiomyoma of the uterus and hence misdiagnosed. In cases of rapidly growing tumours and suspicious radiological features, suspect sarcoma and initiate timely diagnosis and proper treatment. Recommended long-term follow up in view of late recurrences. [Int J Reprod Contracept Obstet Gynecol 2014; 3(3.000: 812-815

  5. Primary fibro sarcoma of the heart.

    Science.gov (United States)

    Kabashi, Serbeze; Hoxha, Naim; Gashi, Shkelzen; Ahmegjekaj, Ilir; Bejta, Ilir; Sadiku, Muharrem; Ymeri, Halit; Kabashi, Antigona; Bicaj, Xhavit; Mucaj, Sefedin

    2013-01-01

    Primary malignant heart tumors represent rare entities where fibro sarcoma represents about 3% of all. Introducing the patient: A 15 years old patient with cardiac insufficiency (heart failure) symptoms, such as weakness, cyanosis, palpitations and breathing difficulties; enlargement of upper mediastinum and pleural effusion. Through echocardiography a pericardial effusion and intracavitary thrombus in atrium was diagnosed. With computed tomography is diagnosed a tumoral mass in right atrium which is also spread in the right ventricle of the heart. Tumor is completely removed; pat histology result showed primary fibro sarcoma of the heart. At that time no metastasis was found. Conclusion. Primary malignant heart tumors may manifest like cardiac insufficiency or like systemic diseases. Fibrosarcomas are rare and have bad prognosis. On average patients can live around six months after initial symptoms appeared and diagnosis of the tumor was done. In the case of cardiac insufficiency with differential diagnosis we should also think of heart tumors, which could certainly be proved for or eliminated by echocardiography.

  6. Dosimetric comparison between VMAT with different dose calculation algorithms and protons for soft-tissue sarcoma radiotherapy

    Energy Technology Data Exchange (ETDEWEB)

    Fogliata, Antonella [Oncology Inst. of Southern Switzerland, Medical Physics Unit, Bellinzona (Switzerland)], e-mail: Antonella.Fogliata-Cozzi@eoc.ch; Scorsetti, Marta; Navarria, Piera [IRCCS Instituto Clinico Humanitas, Radiation Oncology, Rozzano, Milan (Italy)] [and others

    2013-04-15

    Background: To appraise the potential of volumetric modulated arc therapy (VMAT, RapidArc) and proton beams to simultaneously achieve target coverage and enhanced sparing of bone tissue in the treatment of soft-tissue sarcoma with adequate target coverage. Material and methods: Ten patients presenting with soft-tissue sarcoma of the leg were collected for the study. Dose was prescribed to 66.5 Gy in 25 fractions to the planning target volume (PTV) while significant maximum dose to the bone was constrained to 50 Gy. Plans were optimised according to the RapidArc technique with 6 MV photon beams or for intensity modulated protons. RapidArc photon plans were computed with: 1) AAA; 2) Acuros XB as dose to medium; and 3) Acuros XB as dose to water. Results: All plans acceptably met the criteria of target coverage (V{sub 95%} >90-95%) and bone sparing (D{sub 1cm}{sup 3} <50 Gy). Significantly higher PTV dose homogeneity was found for proton plans. Near-to-maximum dose to bone was similar for RapidArc and protons, while volume receiving medium/low dose levels was minimised with protons. Similar results were obtained for the remaining normal tissue. Dose distributions calculated with the dose to water option resulted 5% higher than corresponding ones computed as dose to medium. Conclusion: High plan quality was demonstrated for both VMAT and proton techniques when applied to soft-tissue sarcoma.

  7. Blood vessel growth blocker may treat AIDS-related Kaposi’s sarcoma

    Science.gov (United States)

    Patients with an AIDS-associated cancer, Kaposi's sarcoma (KS), showed improvement after receiving the combination of bevacizumab, a cancer drug that blocks the growth of new blood vessels, and highly active antiretroviral therapy (HAART).

  8. Evaluation of the Mesiodistal Crown Sizes of the Remaining Dentition in Patients with Hypodontia, between 12 and 16 Years of Age

    Directory of Open Access Journals (Sweden)

    Albena Reshitaj

    2013-09-01

    Full Text Available Objective: This study evaluated the mesiodistal crown sizes of the remaining dentition in patients affected with hypodontia, compared with the control group possessing complete dentition. Methods: Panoramic radiographs of the patients treated in the Department of Orthodontics, Dental School - branch of Medical Faculty – University of Pristina, were reviewed to select a sample of 22 cases with agenesis of one or more permanent teeth, except the third molars (hypodontia group. A control group included 22 patients with complete dentition. Dental casts were measured for both groups. The patients ranged in age from 12 to 16 years. Mesiodistal crown dimensions were recorded by measuring all erupted teeth on study models with a manual caliper. Statistical calculation was performed using SPSS 15.0. Paired samples t-test was used to detect the statistical differences in tooth width measurements between the two groups. The significance level was predetermined at α = 0.05.Results: The mean age of the patients in the hypodontia group was 14.55±1.57 years, while that of the control group was 14.23±1.11. The most common congenital missing teeth were the upper lateral incisors (46.16% followed by the upper first premolars (11.54%. We found significant differences between the groups in tooth size for the maxillary arch central incisors and the maxillary arch molar right quadrant, while in the left quadrant differences between the two groups were observed in the central incisor (P<0.05. However, we did not observe any significant differences between the groups with respect to tooth size for the lower jaw. Conclusion: The reduced tooth size was evident except in the posterior segment right molar in the hypodontia group. This should be taken into consideration during treatment planning and deciding upon the treatment mechanics in order to achieve functional occlusion and an esthetic dentition at the culmination of the orthodontic treatment.

  9. Kaposi sarcoma related to acquired immunodeficiency syndrome: hepatic findings on computed tomography and magnetic resonance imaging

    Energy Technology Data Exchange (ETDEWEB)

    Costa, Daniel Nobrega da; Viana, Publio Cesar Cavalcante; Maciel, Rosangela Pereira; Rocha, Manoel de Souza; Gebrim, Eloisa Maria Mello Santiago [Universidade de Sao Paulo (USP), SP (Brazil). Hospital das Clinicas. Inst. de Radiologia]. E-mail: dnobrega@gmail.com

    2008-03-15

    Kaposi sarcoma is a neoplasm associated with immunosuppressive conditions, and involving blood and lymphatic vessels. It is the most frequent intrahepatic neoplasm in patients with acquired immunodeficiency syndrome. Computed tomography and magnetic resonance imaging demonstrate multiple small nodules, prominence and contrast-enhancement of periportal branches due to the presence of the neoplastic tissue. The authors report a case of a 47-year-old male patient with acquired immunodeficiency syndrome presenting disseminated Kaposi sarcoma. (author)

  10. Avaliação funcional dos pacientes portadores de sarcomas ósseos submetidos à tratamento cirúrgico utilizando a endoprótese total ou parcial, na substituição da extremidade distal do fêmur Functional assessment of patients with bone sarcomas submitted to surgical treatment using total or partial prosthesis in replacement of the distal femoral end

    Directory of Open Access Journals (Sweden)

    Sandra Maria Holanda de Mendonça

    2008-01-01

    Full Text Available OBJETIVO: O Osteosarcoma e o Sarcoma de Ewing são as principais neoplasias malignas primárias ósseas, que acometem indivíduos menores de 15 anos. O objetivo deste estudo é comparar, retrospectivamente, os resultados funcionais dos pacientes submetidos à ressecção da extremidade distal do fêmur e à reconstrução com endoprótese não convencional, total ou parcial, do joelho. MÉTODOS: Foram analisados 26 pacientes portadores de sarcomas ósseos da extremidade distal do fêmur, acompanhados no Centro Infantil Boldrini, no período de 1990 a 2003. Vinte e quatro eram portadores de Osteossarcoma e 2 de Sarcoma de Ewing. O sistema de avaliação foi o proposto por Enneking (1987, preconizado pela Musculoskeletal Tumor Society. Para a comparação das médias entre cada critério e também entre os escores finais, utilizou-se o teste de Wilcoxon, com erro alfa de 5%. RESULTADOS: A idade variou de 5 a 17 anos, média=11,9 anos. A predominância foi no sexo feminino (61,5%. Na avaliação funcional, a comparação entre as médias de cada critério, foi encontrada diferença estatisticamente significativa somente relacionada ao item estabilidade (p=0,0037. Nos demais critérios, não foi observado diferença estatisticamente significativa: movimento (p=0,7546, dor (p=0,4848, deformidade (p=0,8695, força (p=1,0000, atividade funcional (p=0,9127 e resultado funcional (p=0,5866. CONCLUSÕES: O escore final global da avaliação funcional não apresentou diferença estatisticamente significativa (p=0,6027. O tipo de endoprótese utilizado para reconstrução do fêmur não interferiu nos resultados funcionais dos pacientes.OBJECTIVES: Osteosarcoma and Ewing's sarcoma are the most common malignant primary bone tumors in individuals under the age of 15 years. The purpose of the study is to retrospectively compare functional outcomes of patients submitted to resection of the distal femoral end and to reconstruction with total or partial non

  11. Mast cell sarcoma: clinical management.

    Science.gov (United States)

    Weiler, Catherine R; Butterfield, Joseph

    2014-05-01

    Mast cell sarcoma is a disorder that results in abnormal mast cells as identified by morphology, special stains, and in some publications, c-kit mutation analysis. It affects animal species such as canines more commonly than humans. In humans it is a very rare condition, with variable clinical presentation. There is no standard therapy for the disorder. It can affect any age group. It is occasionally associated with systemic mastocytosis and/or urticaria pigmentosa. The prognosis of mast cell sarcoma in published literature is very poor in humans.

  12. Myofibroblastic sarcoma of the base of tongue. Case report and review of the literature

    Energy Technology Data Exchange (ETDEWEB)

    Takacsi-Nagy, Zoltan; Fodor, Janos [National Institute of Oncology, Budapest (Hungary). Dept. of Radiotherapy; Murakoezy, Gyoergyi [Szent Imre Hospital, Budapest (Hungary). Dept. of Otorhinolaryngology; Pogany, Peter [Szent Imre Hospital, Budapest (Hungary). Dept. of Pathology; Orosz, Zsolt [National Institute of Oncology, Budapest (Hungary). Dept. of Pathology

    2009-03-15

    Background: Mesenchymal malignancies with myofibroblastic differentiation exhibit a spectrum from low-grade myofibroblastic sarcoma mimicking fibromatosis to pleomorphic high-grade sarcoma. Low-grade myofibroblastic sarcoma shows a wide anatomic distribution with a predilection for the head-and-neck region; however, intermediate- and high-grade myofibroblastic sarcomas in this localization are exceptional. Case Report: A 56-year-old woman with intermediate-grade myofibroblastic sarcoma of the base of tongue is presented. She was treated with surgical excision, but computed tomography proved local residual tumor. Reexcision and chemotherapy were refused by the patient. Irradiation was given to a total dose of 66 Gy. Result: 50 months after completion of radiotherapy, the patient is in good health without any evidence of disease. According to the review of the literature, base of tongue as the primary site of myofibroblastic sarcoma has not been published so far. Conclusion: Similarly to the low-grade form, intermediate- and high-grade myofibroblastic sarcomas may also occur in the head-and-neck region. In case of incomplete excision, radiotherapy may be an effective treatment. (orig.)

  13. 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.

  14. Limb salvage treatment vs. amputation in sarcoma

    Directory of Open Access Journals (Sweden)

    Motamedi M

    1993-05-01

    Full Text Available Many years ago the treatment of sarcoma was radiotherapy up to 2000-4000 rad. This treatment was very complicated, due to producing neoplasm after radiotherapy. By this method of treatment of osteosarcoma, the rate of survival became about 20% (two years. The second method of treatment was chemotherapy for a period of 2-5 weeks that amputation was performed afterwards. By chemotherapy, the rate of being alive reached up to 25-27% (five years. Right now, the best treatment for sarcoma is limb salvage. In our report, the chance of being alive in chondrosarcoma was about four years. This was nearly the same as that of the other institutes in the world especially in America, Europe, and Japan. The rate of recurrence was also more than that from different parts of the world. The survival rate in osteosarcomatic patients was about two years less for males the females, and it was more in tall people than short ones. The survival rate of the patients with giant cell tumor was more than osteosarcoma up to five years, and it has no recurrence or metastasis

  15. Multidisciplinary Management of Soft Tissue Sarcoma

    Directory of Open Access Journals (Sweden)

    Lukas M. Nystrom

    2013-01-01

    Full Text Available Soft tissue sarcoma is a rare malignancy, with approximately 11,000 cases per year encountered in the United States. It is primarily encountered in adults but can affect patients of any age. There are many histologic subtypes and the malignancy can be low or high grade. Appropriate staging work up includes a physical exam, advanced imaging, and a carefully planned biopsy. This information is then used to guide the discussion of definitive treatment of the tumor which typically involves surgical resection with a negative margin in addition to neoadjuvant or adjuvant external beam radiation. Advances in imaging and radiation therapy have made limb salvage surgery the standard of care, with local control rates greater than 90% in most modern series. Currently, the role of chemotherapy is not well defined and this treatment is typically reserved for patients with metastatic or recurrent disease and for certain histologic subtypes. The goal of this paper is to review the current state of the art in multidisciplinary management of soft tissue sarcoma.

  16. Sarcoma indiferenciado primário no sistema nervoso central Primary undifferentiated sarcoma of the central nervous system

    Directory of Open Access Journals (Sweden)

    Milton Marcio Machota Junior

    2012-04-01

    Full Text Available INTRODUÇÃO: O sarcoma de sistema nervoso central (SNC é uma neoplasia rara, com incidência de 0,1% a 4,3% dos tumores intracranianos. São tumores agressivos com prognóstico reservado e a maioria é tratada com ressecção radical. RELATO: Homem, 29 anos, com episódios de crises convulsivas e diagnóstico de hemorragia intraparenquimatosa. Durante a cirurgia, foi identificada lesão bem delimitada. A histologia demonstrou neoplasia fusocelular com atipias e numerosas mitoses. Os únicos marcadores imuno-histoquímicos positivos foram vimentina e S-100. O diagnóstico foi de sarcoma indiferenciado de alto grau. CONCLUSÃO: No diagnóstico diferencial de sarcomas de SNC, devem-se excluir lesões metastáticas e gliossarcoma.INTRODUCTION: The central nervous system (CNS sarcoma is a rare neoplasm with an incidence of 0.1% to 4.3% in intracranial tumors. They are aggressive with poor prognosis, and mostly treated with radical resection. REPORT: 29 year-old male patient with episodes of seizures and diagnosed with intraparenchymal hemorrhage. During the surgery a well-defined lesion was identified. Histology showed a spindle cell neoplasm with atypia and numerous mitoses. The immunohistochemical markers were positive only for vimentin and S-100. The diagnosis was high-grade undifferentiated sarcoma. CONCLUSION: Metastatic lesions and gliosarcoma should be excluded in the differential diagnosis of CNS sarcomas.

  17. 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

    Energy Technology Data Exchange (ETDEWEB)

    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

  18. Stem-Like Cells in Bone Sarcomas: Implications for Tumorigenesis

    Directory of Open Access Journals (Sweden)

    C. Parker Gibbs

    2005-11-01

    Full Text Available Bone sarcomas are a clinically and molecularly heterogeneous group of malignancies characterized by varying degrees of mesenchymal differentiation. Despite advances in medical and surgical management, survival rates for high-grade tumors have remained static at 50% to 70%. Tumor stem cells have been recently implicated in the pathogenesis of other heterogeneous, highly malignant tumors. We demonstrate here the existence of a small subpopulation of self-renewing bone sarcoma cells that are capable of forming suspended spherical, clonal colonies, also called “sarcospheres,” in anchorage-independent, serum-starved conditions. These bone sarcoma cells as well as tissue specimens express activated STAT3 and the marker genes of pluripotent embryonic stem (ES cells, Oct 3/4 and Nanog. Expression levels of Oct 3/4 and Nanog are greater in sarcospheres than in adherent cultures. A subset of bone sarcoma cells displays several surface markers of mesenchymal stem cells (Stro-1, CD105, and CD44 as well as attributes of mesodermal, ectodermal, and endodermal differentiation. Although previously documented in brain and breast tumors, our results support the extension of the cancer stem cell hypothesis to include tumors of mesenchymal lineage. Furthermore, they suggest the participation of ES cell homeobox proteins in non-germ cell tumorigenesis.

  19. Copy Number Alterations and Methylation in Ewing's Sarcoma

    Directory of Open Access Journals (Sweden)

    Mona S. Jahromi

    2011-01-01

    Full Text Available Ewing's sarcoma is the second most common bone malignancy affecting children and young adults. The prognosis is especially poor in metastatic or relapsed disease. The cell of origin remains elusive, but the EWS-FLI1 fusion oncoprotein is present in the majority of cases. The understanding of the molecular basis of Ewing's sarcoma continues to progress slowly. EWS-FLI1 affects gene expression, but other factors must also be at work such as mutations, gene copy number alterations, and promoter methylation. This paper explores in depth two molecular aspects of Ewing's sarcoma: copy number alterations (CNAs and methylation. While CNAs consistently have been reported in Ewing's sarcoma, their clinical significance has been variable, most likely due to small sample size and tumor heterogeneity. Methylation is thought to be important in oncogenesis and balanced karyotype cancers such as Ewing's, yet it has received only minimal attention in prior studies. Future CNA and methylation studies will help to understand the molecular basis of this disease.

  20. Undifferentiated sarcoma of the liver: a case study of an erythropoietin-secreting tumor.

    Science.gov (United States)

    Lin, JoAnn M; Heath, Jonathon E; Twaddell, William S; Castellani, Rudy J

    2014-09-01

    Undifferentiated embryonal sarcoma of the liver (UESL) is an uncommon hepatic tumor usually found in children, with rare cases reported in adults. We present a case of a 53-year-old woman with an undifferentiated sarcoma of the liver (USL), which resembles UESL, who initially presented with a markedly elevated hematocrit (61.2%). Cytogenetic studies for polycythemia vera were negative, but the patient's erythropoietin (EPO) was elevated. A computed tomography scan and subsequent partial hepatectomy revealed a well-circumscribed, partially cystic mass in the right lobe of the liver measuring 34 cm. Following surgery, the patient's EPO level and hematocrit dropped to within normal range and remained so for 1 year, at which point it rose again. A subsequent magnetic resonance imaging scan showed a liver mass at the previous resection margin, consistent with a recurrence. In this case study, we describe the first reported USL resembling an UESL that secretes EPO, which was a useful marker of tumor recurrence. © The Author(s) 2013.

  1. MR imaging of myxofibrosarcoma and undifferentiated sarcoma with emphasis on tail sign; diagnostic and prognostic value

    Energy Technology Data Exchange (ETDEWEB)

    Yoo, Hye Jin; Hong, Sung Hwan; Kang, Yusuhn; Choi, Ja-Young; Yi, Minkyong [Seoul National University College of Medicine, Seoul National University Hospital, Department of Radiology, Seoul (Korea, Republic of); Moon, Kyung Chul [Seoul National University College of Medicine, Seoul National University Hospital, Department of Pathology, Seoul (Korea, Republic of); Kim, Han-Soo; Han, Ilkyu [Seoul National University College of Medicine, Seoul National University Hospital, Department of Orthopedic Surgery, Seoul (Korea, Republic of); Kang, Heung Sik [Seoul National University Bundang Hospital, Department of Radiology, Seongnam-City, Gyeongi-Do (Korea, Republic of)

    2014-08-15

    To assess the prevalence of the tail sign in soft tissue sarcomas and determine whether the local recurrence rate differed based on the presence of the tail sign. In our retrospective study, myxofibrosarcoma (MFS, n = 25) and undifferentiated sarcoma (US, n = 38) comprised group 1, and the remaining tumours (n = 115) were assigned to group 2. Location, size, and imaging features of the tumours were assessed on MRI. The radiological-pathological correlation of the tail sign was analysed. The tail sign, thick fascial enhancement extending from the tumour margin, was more common and significantly thicker in group 1. In the subgroup analysis between MFS and US, there was no significant difference in the presence of a tail sign. Histological examination revealed extensive tumour cell infiltrations along the deep fascia from the main mass. Patients with a tail sign had a worse local recurrence-free survival than patients without it, not only in all tumours (p < 0.01), but also in group 1 (p = 0.019) The tail sign was a common MRI feature of both MFS and US, and was also associated with worse local recurrence-free survival. Radiologists should be aware of these MRI findings and inform the surgeon preoperatively in order to obtain a sufficient surgical margin to minimise the risk of local tumour recurrence. (orig.)

  2. Radiation-induced Sarcomas Occurring in Desmoid-type Fibromatosis Are Not Always Derived From the Primary Tumor.

    Science.gov (United States)

    Verschoor, Arie J; Cleton-Jansen, Anne-Marie; Wijers-Koster, Pauline; Coffin, Cheryl M; Lazar, Alexander J; Nout, Remi A; Rubin, Brian P; Gelderblom, Hans; Bovée, Judith V M G

    2015-12-01

    Desmoid-type fibromatosis is a rare, highly infiltrative, locally destructive neoplasm that does not metastasize, but recurs often after primary surgery. Activation of the Wnt/β-catenin pathway is the pathogenic mechanism, caused by an activating mutation in exon 3 of CTNNB1 (85% of the sporadic patients). Radiotherapy is a frequent treatment modality with a local control rate of approximately 80%. In very rare cases, this may result in the development of radiation-induced sarcoma. It is unclear whether these sarcomas develop from the primary tumor or arise de novo in normal tissue. In 4 tertiary referral centers for sarcoma, 6 cases of desmoid-type fibromatosis that subsequently developed sarcoma after radiotherapy were collected. The DNA sequence of CTNNB1 exon 3 in the desmoid-type fibromatosis and the subsequent postradiation sarcoma was determined. Sarcomas developed 5 to 21 years after the diagnosis of desmoid-type fibromatosis and included 2 osteosarcomas, 2 high-grade undifferentiated pleomorphic sarcomas, 1 fibrosarcoma, and 1 undifferentiated spindle cell sarcoma. Three patients showed a CTNNB1 hotspot mutation (T41A, S45F, or S45N) in both the desmoid-type fibromatosis and the radiation-induced sarcoma. The other 3 patients showed a CTNNB1 mutation in the original desmoid-type fibromatosis (2 with a T41A and 1 with an S45F mutation), which was absent in the sarcoma. In conclusion, postradiation sarcomas that occur in the treatment area of desmoid-type fibromatosis are extremely rare and can arise through malignant transformation of CTNNB1-mutated desmoid fibromatosis cells, but may also originate from CTNNB1 wild-type normal cells lying in the radiation field.

  3. Extensive primary Ewings' sarcoma in the greater wing of the sphenoid bone.

    Science.gov (United States)

    Apostolopoulos, Kostas; Ferekidis, Eleftherios

    2003-01-01

    We describe a rare case of an extensive primary cranial Ewing's sarcoma located in the greater wing of the sphenoid bone with extension to the orbit, the endocranium, the parapharyngeal and infratemporal space. The patient presented with diplopia, anosmia and prolapse of the left eye. He was given chemo- and radiotherapy and was free of symptoms on re-examination 1.5 years later. The prognosis of Ewing's sarcoma in the absence of surgery is uncertain, but prompt treatment appears to have a satisfactory therapeutic outcome. In the future, more cases should be studied in order to investigate the biological behaviour of a primary cranial Ewing's sarcoma.

  4. [Radiotherapy for sarcoma: hadrontherapy, for whom and what for?].

    Science.gov (United States)

    Pommier, P; Sunyach, M-P; Hu, Yi; Amsalem, E; Claude, L; Moncort-Boulch, D; Toutenu, P; Balosso, J

    2010-06-01

    The radiobiological properties of the hadrons (neutrons, protons, carbon ions) led to their therapeutic use in sarcomas, as a referent therapy or as an alternative to photon therapy. An extensive review of the literature has been conducted to assess the present indications and the perspectives for hadrontherapy. Compared to photons, neutrons are characterized by a higher biological efficiency that is on particular importance for these tumours usually considered as radio-resistant. Neutrons have been considered as a standard therapy for sarcoma' patients, contra-indicated for surgery or with a definitive R2 resection, but their indications and use have been restricted due to the occurrence of late severe toxicities related to their poor ballistic' properties. Thanks to their physical properties (Bragg Peak), protons are characterized by a higher conformity index compared to photons (and neutrons) with optimal organs at risk preservation that permits a dose escalation. Protontherapy is to date the standard of care for base of skull, spinal and paraspinal sarcomas. Carbon ions combined both advantages from protons and neutrons. Literature data permits to consider this radiation modality as a referent therapy for unresectable sarcomas. The ongoing diffusions of protons and carbon ions radiotherapy facilities will permit to offer these therapies to more patients and to conduct studies that are warranted to determine their indications and their results.

  5. 病毒巨噬细胞炎症蛋白vMIP-Ⅰ和vMIP-Ⅱ在Kaposi's肉瘤患者中的表达及意义%The expression and clinical significance of viral macrophage inflammatory protein vMIP-Ⅰ and vMIP-Ⅱ in Kapsi's sarcoma patients

    Institute of Scientific and Technical Information of China (English)

    王晓东; 姜涵; 侯平; 多兰·达力汗; 罗浩杰; 马萍; 帕丽达·阿布利孜

    2015-01-01

    目的 初步探明KSHV编码的病毒巨噬细胞炎症蛋白vMIP-Ⅰ和vMIP-Ⅱ在新疆经典型Kaposi's sarcoma(KS)患者的肉瘤组织、血液、唾液和尿液中的表达情况.方法 对8例新疆经典KS患者的新鲜冰冻肉瘤组织、血液、唾液和尿液标本进行基因组DNA提取,应用PCR扩增各标本中的vMIP-Ⅰ和vMIP-Ⅱ基因.结果 8例新疆经典型KS患者的肉瘤组织、血液、唾液和尿液中均获得较好的基因组DNA;同时8例组织标本全部扩增出vMIP-Ⅰ、vMIP-Ⅱ基因,但是8例患者的血液、唾液和尿液标本都未出现vMIP-Ⅰ和vMIP-Ⅱ基因表达.结论 新疆经典型KS患者肉瘤组织中均检测到vMIP-Ⅰ和vMIP-Ⅱ基因片段的表达,为深入研究卡波氏肉瘤的血管形成机制提供初步的依据.%Objective To observe the expression in sarcoma tissue,blood,salivaand urine of KSHV-encoded virus macrophage inflammatory protein vMIP-Ⅰ,vMIP-Ⅱ.Methods Genomic DNA was extracted from samples in 8 classic Kapsi' s sarcoma patients of Xinjiang,vMIP-Ⅰ and vMIP-Ⅱ gene were amplified by PCR.Results Genomic DNA were detected from sarcoma tissue,blood,saliva and urine of 8 classic Kapsi' s sarcoma patients,while vMIP-Ⅰ and vMIP-Ⅱ were amplified from all tissue samples,but the expression of vMIP-Ⅰ and vMIP-Ⅱ in blood,saliva and urine was negtive.Conclusions There is special expression of vMIP-Ⅰ and vMIP-Ⅱ in Kapsi' s sarcoma tissue.It provides a preliminary basis for further study of the mechanism of angiogenesis Kaposi's sarcoma.

  6. Fish remains and humankind

    Directory of Open Access Journals (Sweden)

    Andrew K G Jones

    1997-08-01

    Full Text Available The four papers in this issue represent a trawl of the reports presented to the Fourth meeting of the International Council for Archaeozoology (ICAZ Fish Remains Working Group, which met at the University of York in 1987. The conference discussed material from many parts of the world - from Australasia to the north-west coast of America - and many eras, ranging in date from the early Pleistocene to the 1980s. It demonstrated both the variety of work being carried out and the growing interest in ancient fish remains. Internet Archaeology plans to publish other batches of papers from this conference. These reports will demonstrate the effort being made to distinguish between assemblages of fish remains which have been deposited by people and those which occur in ancient deposits as a result of the action of other agents. To investigate this area, experiments with modern material and observations of naturally occurring fish bone assemblages are supplemented with detailed analysis of ancient and modern fish remains. The papers published here illustrate the breadth of research into osteology, biogeography, documentary research, and the practicalities of recovering fish remains. Read, digest and enjoy them! Using the Internet for publishing research papers is not only ecologically sound (saving paper, etc. it disseminates scholarship to anyone anywhere on the planet with access to what is gradually becoming necessary technology in the late 20th century. Hopefully, future groups of papers will include video and audio material recorded at the conference, and so enable those who could not attend to gain further insights into the meeting and the scholarship underpinning this area of research.

  7. A Comprehensive Single Institutional Review of 2 Years in a Designated Fast-Track Sarcoma Diagnostic Clinic Linked with a Sarcoma Specialist Advisory Group: Meeting the Target but Failing the Task?

    Directory of Open Access Journals (Sweden)

    Zoltan Szucs

    2016-01-01

    Full Text Available Background. National guidelines prompted the implementation of a designated two-week wait referral pathway to facilitate the early diagnosis of sarcomas, to improve treatment outcomes. Methods. Patients referred to the Cambridge Sarcoma Diagnostic Clinic between January 2013 and December 2014 were identified through the electronic appointments system. Information was retrospectively retrieved about patient characteristics and details of the diagnostic pathway. Results. 17.3% of patients referred (69/397 were diagnosed with a malignancy. Of these, 59.3% (41/69 had primary sarcomas, 17.4% (12/69 had metastatic cancer, and 23.2% (16/69 had a different primary malignancy. 15% of the 41 sarcomas were 10 cm. Sarcomas diagnosed through this clinic represented 13% (41/315 of sarcomas managed at the centre during the same 2 years. Conclusion. While we achieved the target of 10% (41/397 sarcoma diagnosis rate in the rapid access clinic, only 15% of these were <5 cm better prognosis lesions. This calls into question the “real world” impact of such diagnostic clinics on early diagnosis of sarcomas. In order to enhance generic cancer diagnostic skills, training in these diagnostic clinics could be usefully integrated into national training curricula for both surgical and nonsurgical oncologists.

  8. Primary sarcoma of the ovary: clinicopathological characteristics, prognostic factors and evaluation of therapy

    Institute of Scientific and Technical Information of China (English)

    DAI Yi; SHEN Keng; LANG Jing-he; HUANG Hui-fang; PAN Ling-ya; WU Ming; YANG Jia-xin; ZHONG Ding-rong

    2011-01-01

    Background The primary ovarian sarcoma is a very rare malignancy. The objective of this study was to further investigate the clinicopathologic features and outcome in patients with primary sarcoma of the ovary.Methods Between 1988 and 2007, 24 patients with primary ovarian sarcoma who underwent treatment at Peking Union Medical Hospital were reviewed retrospectively. Response to treatment, progression and overall survival were analyzed.Results Patients with ovarian sarcoma had a mean age of (54.3±10.3) years, and 16 of them were postmenopausal.The most common symptom was abdominal pain, present in 14 patients. Of the 24 patients, 16 patients were pathologically diagnosed as carcinosarcoma (known as malignant mixed mesodermal tumor (MMMT)), 2 as ovarian leiomyosarcoma (LS) and 6 patients as ovarian endometrial stromal sarcoma (ESS). The patients in optimal debulking group had a median survival period of 28 months and 1-year survival rate of 71%. The patients in suboptimal debulking group had a significantly lower median survival of 6 months (P=-0.02) and 1-year survival rate of 29%. Among the patients,23 patients received chemotherapy and most of regimens were based on platinum, 3 patients received chemoradiation.The mean number of courses of combined chemotherapy was 6.6±5.0, and the response was unsatisfactory. The median survival for the entire group was 18.7 months. The one-year survival rate was 58%, and two-year survival rate only 29%.Conclusions Ovarian primary sarcoma has a poor overall prognosis. Optimal debulking surgery appears to be of prognostic significance. There is a clear need for further study to explore the role and the regimen of platinum-based chemotherapy in primary ovarian sarcoma.

  9. Treatment Options for Kaposi Sarcoma

    Science.gov (United States)

    ... and its treatment, see the AIDSinfo website . Nonepidemic Gay-related Kaposi Sarcoma There is a type of ... better than another. Trials are based on past studies and what has been learned ... by their creator. In such cases, it is necessary to contact the writer, artist, ...

  10. General Information about Kaposi Sarcoma

    Science.gov (United States)

    ... and its treatment, see the AIDSinfo website . Nonepidemic Gay-related Kaposi Sarcoma There is a type of ... better than another. Trials are based on past studies and what has been learned ... by their creator. In such cases, it is necessary to contact the writer, artist, ...

  11. Kaposi sarcoma associated with lipoedema.

    Science.gov (United States)

    Ekmekci, T R; Ayabakan, O; Sakiz, D; Koslu, A

    2005-05-01

    Lipoedema is a form of lipodistrophy, which consists of abnormal accumulation of fat in subcutaneous tissue of the lower limbs. It does not cause any disease and it has not been reported association with malignity. We describe a 63-year-old woman occurring of Kaposi sarcoma on the lipoedema base.

  12. [PALEOPATHOLOGY OF HUMAN REMAINS].

    Science.gov (United States)

    Minozzi, Simona; Fornaciari, Gino

    2015-01-01

    Many diseases induce alterations in the human skeleton, leaving traces of their presence in ancient remains. Paleopathological examination of human remains not only allows the study of the history and evolution of the disease, but also the reconstruction of health conditions in the past populations. This paper describes the most interesting diseases observed in skeletal samples from the Roman Imperial Age necropoles found in urban and suburban areas of Rome during archaeological excavations in the last decades. The diseases observed were grouped into the following categories: articular diseases, traumas, infections, metabolic or nutritional diseases, congenital diseases and tumours, and some examples are reported for each group. Although extensive epidemiological investigation in ancient skeletal records is impossible, the palaeopathological study allowed to highlight the spread of numerous illnesses, many of which can be related to the life and health conditions of the Roman population.

  13. Understanding sarcomas and other rare tumors: an interview with Robin L Jones.

    Science.gov (United States)

    Jones, Robin L

    2017-02-01

    Robin L Jones speaks to Jade Parker, Commissioning Editor: Robin Jones is a medical oncologist specializing in the treatment of bone and soft tissue sarcomas and Head of the Sarcoma Unit at The Royal Marsden. He completed his medical training at Guy's and St Thomas' Hospital, and his oncology training at The Royal Marsden. His postgraduate research degree, with Professor Dowsett at the Institute of Cancer Research (ICR), evaluated potential predictive and prognostic factors in breast cancer patients treated with neoadjuvant chemotherapy. In January 2010 he was appointed Associate Professor and Director of the Sarcoma Program at the University of Washington and Fred Hutchinson Cancer Research Center in Seattle. He led a successful, grant funded program and continued his translational and clinical trial-based research. The laboratory work with Dr Seth Pollack evaluated novel immunotherapeutic targets in bone and soft tissue sarcomas, and has led to a number of early-phase immunotherapeutic clinical trials in sarcoma. He returned to The Royal Marsden and Institute of Cancer Research in December 2014, as Sarcoma Clinical Trials Team Leader and Consultant Medical Oncologist. He has experience in conducting Phase I, II and III trials, as well as translational studies in sarcoma. He is continuing a number of trials of investigational agents as well as laboratory-based immunotherapy studies.

  14. Uterine sarcoma part III—Targeted therapy: The Taiwan Association of Gynecology (TAG systematic review

    Directory of Open Access Journals (Sweden)

    Ming-Shyen Yen

    2016-10-01

    Full Text Available Uterine sarcoma is a very aggressive and highly lethal disease. Even after a comprehensive staging surgery or en block cytoreduction surgery followed by multimodality therapy (often chemotherapy and/or radiation therapy, many patients relapse or present with distant metastases, and finally die of diseases. The worst outcome of uterine sarcomas is partly because of their rarity, unknown etiology, and highly divergent genetic aberration. Uterine sarcomas are often classified into four distinct subtypes, including uterine leiomyosarcoma, low-grade uterine endometrial stromal sarcoma, high-grade uterine endometrial stromal sarcoma, and undifferentiated uterine sarcoma. Currently, evidence from tumor biology found that these tumors showed alternation and/or mutation of genomes and the intracellular signal pathway. In addition, some preclinical studies showed promising results for targeting receptor tyrosine kinase signaling, phosphatidylinositol 3-kinase/AKT/mammalian target of rapamycin pathway, various kinds of growth factor pathways, Wnt/beta-catenin signaling pathway, transforming growth factor β/bone morphogenetic protein signal pathway, aurora kinase A, MDM2 proto-oncogene, histone deacetylases, sex hormone receptors, certain types of oncoproteins, and/or loss of tumor suppressor genes. The current review is attempted to summarize the recurrent advance of targeted therapy for uterine sarcomas.

  15. Individualised risk assessment for local recurrence and distant metastases in a retrospective transatlantic cohort of 687 patients with high-grade soft tissue sarcomas of the extremities: a multistate model

    Science.gov (United States)

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

    2017-01-01

    Objectives This study investigates the effect of surgical margins and radiotherapy, in the presence of individual baseline characteristics, on survival in a large population of high-grade soft tissue sarcoma of the extremities using a multistate model. Design A retrospective multicentre cohort study. Setting 4 tertiary referral centres for orthopaedic oncology. Participants 687 patients with primary, non-disseminated, high-grade sarcoma only, receiving surgical treatment with curative intent between 2000 and 2010 were included. Main outcome measures The risk to progress from ‘alive without disease’ (ANED) after surgery to ‘local recurrence’ (LR) or ‘distant metastasis (DM)/death’. The effect of surgical margins and (neo)adjuvant radiotherapy on LR and overall survival was evaluated taking patients' and tumour characteristics into account. Results The multistate model underlined that wide surgical margins and the use of neoadjuvant radiotherapy decreased the risk of LR but have little effect on survival. The main prognostic risk factors for transition ANED to LR are tumour size (HR 1.06; 95% CI 1.01 to 1.11 (size in cm)) and (neo)adjuvant radiotherapy. The HRs for patients treated with adjuvant or no radiotherapy compared with neoadjuvant radiotherapy are equal to 4.36 (95% CI 1.34 to 14.24) and 14.20 (95% CI 4.14 to 48.75), respectively. Surgical resection margins had a protective effect for the occurrence of LR with HRs equal to 0.61 (95% CI 0.33 to 1.12), and 0.16 (95% CI 0.07 to 0.41) for margins between 0 and 2 mm and wider than 2 mm, respectively. For transition ANED to distant metastases/Death, age (HR 1.64 (95% CI 0.95 to 2.85) and 1.90 (95% CI 1.09 to 3.29) for 25–50 years and >50 years, respectively) and tumour size (1.06 (95% CI 1.04 to 1.08)) were prognostic factors. Conclusions This paper underlined the alternating effect of surgical margins and the use of neoadjuvant radiotherapy on oncological outcomes between patients with

  16. First-in-Human Treatment With a Dendritic Cell-targeting Lentiviral Vector-expressing NY-ESO-1, LV305, Induces Deep, Durable Response in Refractory Metastatic Synovial Sarcoma Patient.

    Science.gov (United States)

    Pollack, Seth M; Lu, Hailing; Gnjatic, Sacha; Somaiah, Neeta; O'Malley, Ryan B; Jones, Robin L; Hsu, Frank J; Ter Meulen, Jan

    2017-10-01

    Effective induction of antitumor T cells is a pivotal goal of cancer immunotherapy. To this end, lentiviral vectors (LV) are uniquely poised to directly prime CD8 T-cell responses via transduction of dendritic cells in vivo and have shown promise as active cancer therapeutics in preclinical tumor models. However, until now, significant barriers related to production and regulation have prevented their widespread use in the clinic. We developed LV305, a dendritic cell-targeting, integration-deficient, replication incompetent LV from the ZVex platform, encoding the full-length cancer-testis antigen NY-ESO-1. LV305 is currently being evaluated in phase 1 and 2 trials in metastatic recurrent cancer patients with NY-ESO-1 positive solid tumors as a single agent and in combination with anti-PD-L1. Here we report on the first patient treated with LV305, a young woman with metastatic, recurrent, therapy-refractive NY-ESO-1 synovial sarcoma. The patient developed a robust NY-ESO-1-specific CD4 and CD8 T-cell response after 3 intradermal injections with LV305, and subsequently over 85% disease regression that is continuing for >2.5 years posttherapy. No adverse events >grade 2 occurred. This case demonstrates that LV305 can be safely administered and has the potential to induce a significant clinical benefit and immunologic response in a patient with advanced stage cancer.

  17. Histiocytic sarcoma that mimics benign histiocytosis.

    Science.gov (United States)

    Boisseau-Garsaud, A M; Vergier, B; Beylot-Barry, M; Nastasel-Menini, F; Dubus, P; de Mascarel, A; Eghbali, H; Beylot, C

    1996-06-01

    A 28-year-old man presented with a histiocytic sarcoma of a very uncommon origin, as it had developed for several years like a benign cutaneous histiocytosis resembling generalized eruptive histiocytoma before becoming acute, with nodal and massive pulmonary involvement. Despite various chemotherapies, the patient died within 8 months. Skin biopsies showed histiocytic proliferation in the dermis and node biopsies showed histiocytic proliferation with a sinusoidal pattern. Immunohistochemical analysis, performed on paraffin-embedded sections, demonstrated strong labeling of tumoral cells for CD68 and moderate labeling for CD3 and CD4. CD30 labeling was negative. S-100 protein was positive on a Langerhans' cell reactive subpopulation. Electron microscopy confirmed the histiocytic nature of malignant cells and showed cytoplasmic inclusions such as regularly laminated bodies, dense bodies and pleomorphic inclusions. No Birbeck granules were seen. A gene rearrangement study of T-cell receptor gamma and immunoglobulin heavy chain genes showed a germline configuration. Histiocytic sarcoma is an extremely rare true histiocytic malignancy, the existence of which has been recently debated since it has often been mistaken in the past for large cell lymphomas. Such a deceptive onset as benign cutaneous histiocytosis has not been described in the literature to our knowledge.

  18. Sonographic Findings of Uterine Endometrial Stromal Sarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Jeong Ah; Lee, Myung Sook; Choi, Jong Sun [Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of)

    2006-12-15

    The study was performed to present the sonographic findings of uterine endometrial stromal sarcoma (ESS). We conducted a retrospective review of sonographic findings of 10 cases that were diagnosed as uterine ESS. The patients ages ranged from 25 to 51 years (mean age: 36.1 years). The reviews focused on the location, margin, size, number and echotexture of the lesions. Hysterectomy (n = 9) and myomectomy (n = 1) were performed and a pathologic diagnosis was obtained in all cases. The masses were located in the uterine wall (n = 6), or they presented as a polypoid mass protruding into the endometrial cavity from the myometrium (n = 3) or as a central cavity mass (n = 1). The lesion margins were smooth (n = 5), ill defined (n = 2), or smooth with partially nodular extensions (n = 3). The maximal mass length was 38 mm to 160 mm with a mean mass length of 83.5 mm. There were single lesions in eight cases and multiple lesions in two cases. The lesion echotextures were hypoechoic solid (n = 3), heterogeneously intermediate echoic (n = 5), diffuse myometrial thickening with heterogeneous echogenicity (n = 1) and septated cystic (n = 1). Endometrial stromal sarcoma presents with four patterns of its sonographic appearance; a polypoid mass with nodular myometrial extension, an intramural mass with an ill defined margin and heterogeneous echogenicity, an ill defined large central cavity mass or, diffuse myometrial thickening.

  19. Primary intravascular synovial sarcoma: case report.

    Science.gov (United States)

    Tuncer, Osman Nuri; Erbasan, Ozan; Golbasi, Ilhan

    2012-10-01

    Synovial sarcoma (SS), a mesenchymal spindle cell tumor, displays variable epithelial differentiation, including glandular formation, and features a specific chromosomal translocation, t(X;18)(p11;q11). SS accounts for 5% to 10% of soft-tissue sarcomas. These tumors occur mostly in the joints, especially near the knee, but they also occur in other locations. Primary intravascular SS (IVSS) are extremely rare; only 6 well-documented cases have been reported in the English literature. We describe a new case of primary IVSS of the superior vena cava (SVC) in a 16-year-old boy. A transthoracic echocardiogram confirmed a large (4.8 × 4.6 cm) circumscribed mass filling the right atrium, as well as a moderate pericardial effusion. The mass extended from the SVC to the tricuspid valve but did not prevent valve coaptation. Surgery via a transatrial approach revealed a huge mass (8 to 12 cm) attached to the SVC via a 5-mm pedicle. The tumor was excised, and the patient experienced an uneventful postoperative course. Fluorescence in situ hybridization analysis revealed the presence of the SS-specific translocation.

  20. Primary Ewing's Sarcoma of the Kidney in a 73-Year-Old Man.

    Science.gov (United States)

    Wedde, T B; Lobmaier, I V K; Brennhovd, B; Lohne, F; Hall, K S

    2011-01-01

    Objective. Ewing's sarcoma of the kidney is rare and is usually found in young adults. We present here a single case study of Ewing's sarcoma found in an elderly man. Material and methods. A 73-year-old man underwent routine surgery for hydrocoele of the testis. He developed urinary obstruction symptoms, and radiological examinations revealed a tumour in the right kidney. Results. Microscopical, immunohistochemical, and molecular pathological analysis of the tumour was consistent with Ewing's sarcoma. FISH showed rearrangement of chromosomes 22q12 (EWSR1). The patient subsequently underwent nephrectomy followed by 6 adjuvant chemotherapy cycles. Follow-up after 7 months shows no recurrence. Conclusion. This case report presents not only the rare finding of Ewing's sarcoma in the kidney, but also the occurrence of this tumour entity in an elderly patient. Treatment options for the different types of renal tumours are vastly different and the need for a correct diagnosis is, therefore, vital.

  1. Ewing’s sarcoma of ilium, a diagnostic dilemma - case report with review of literature

    Directory of Open Access Journals (Sweden)

    Usama Saleh Alshaya

    2016-01-01

    Full Text Available Ewing’s sarcoma is a highly malignant tumor of bone and is more common in children in the age group of 10 to 20 years. Sometimes the classic clinical and radiological presentation of Ewing’s sarcoma may not be the norm and patient may have an atypical presentation leading to diagnostic confusion. This is especially true for Ewing’s sarcoma involving iliac bone. We present such a case of Ewing’s sarcoma involving the right ilium in a patient presenting as right lower quadrant pain and non-specific radiological changes. To the best of our knowledge, this scenario has not been reported in literature. We recommend early magnetic resonance imaging and computed tomography to diagnose the disease early when there is slightest suspicion of the disease.

  2. Primary Ewing’s Sarcoma of the Kidney in a 73-Year-Old Man

    Directory of Open Access Journals (Sweden)

    T. B. Wedde

    2011-01-01

    Full Text Available Objective. Ewing’s sarcoma of the kidney is rare and is usually found in young adults. We present here a single case study of Ewing's sarcoma found in an elderly man. Material and methods. A 73-year-old man underwent routine surgery for hydrocoele of the testis. He developed urinary obstruction symptoms, and radiological examinations revealed a tumour in the right kidney. Results. Microscopical, immunohistochemical, and molecular pathological analysis of the tumour was consistent with Ewing's sarcoma. FISH showed rearrangement of chromosomes 22q12 (EWSR1. The patient subsequently underwent nephrectomy followed by 6 adjuvant chemotherapy cycles. Follow-up after 7 months shows no recurrence. Conclusion. This case report presents not only the rare finding of Ewing's sarcoma in the kidney, but also the occurrence of this tumour entity in an elderly patient. Treatment options for the different types of renal tumours are vastly different and the need for a correct diagnosis is, therefore, vital.

  3. O uso da ventilação mecânica não-invasiva nos cuidados paliativos de paciente com sarcoma torácico metastático: relato de caso The use of noninvasive mechanical ventilation in the palliative care of a patient with metastatic thoracic sarcoma: case report

    Directory of Open Access Journals (Sweden)

    Mariana Almada Bassani

    2008-06-01

    Full Text Available JUSTIFICATIVA E OBJETIVOS: Os cuidados paliativos se baseiam na prevenção e alívio do sofrimento, identificando, avaliando e tratando a dor e outros problemas físicos, psicossociais e espirituais. Quadros súbitos de dispnéia são freqüentes em pacientes oncológicos em fase terminal. Nestes casos, a ventilação mecânica não-invasiva pode ser uma boa opção no manuseio da dispnéia, promovendo conforto e permitindo o contato do paciente com seus familiares. O objetivo deste estudo foi apresentar os benefícios obtidos com a ventilação mecânica não-invasiva no paciente sob cuidados paliativos. RELATO DO CASO: Paciente com 29 anos, em pós-operatório imediato de cesariana, admitida na unidade de terapia intensiva (UTI para tratamento de insuficiência respiratória aguda. À radiografia de tórax visualizava-se massa pulmonar a direita. Após investigação clínica e de imagem foi diagnosticado sarcoma torácico metastático em fase avançada, sendo indicadas pela equipe de Oncologia medidas e cuidados paliativos. Para alivio da dispnéia, a equipe multidisciplinar da UTI optou pelo uso de ventilação mecânica não-invasiva (modalidade CPAP + PSV, permitindo a interação da mãe com o bebê e familiares. CONCLUSÕES: No contexto de cuidados paliativos, a VMNI mostrou-se ser um método capaz de contribuir para o controle da dispnéia provendo conforto e alívio ao paciente.BACKGROUND AND OBJECTIVES: Palliative care is based on prevention and relief of suffering, identifying, assessing and treating pain and other physical, psychological, social and spiritual problems. Sudden dyspnea is frequently observed in terminal oncologic patients. In these cases, noninvasive ventilation can be an adequate option to control dyspnea promoting comfort and allowing patient interaction with their relatives. The aim of this article was to present the benefits of noninvasive ventilation in the palliative care setting. CASE REPORT: The case of a 29

  4. 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.

  5. A Systematic Review of the Recent Quality of Life Studies in Adult Extremity Sarcoma Survivors

    Directory of Open Access Journals (Sweden)

    Melissa H. Tang

    2012-01-01

    Full Text Available Background. Extremity sarcoma represents a heterogeneous group of rare cancers that carries a relatively high morbidity with regards to physical function. Quality of Life (QoL as an outcome is an important consideration in this cohort. We aimed to identify the correlates of QoL in extremity sarcoma cohorts. Methods. A systematic review of the literature on extremity sarcoma in adults from five databases over the last ten years was undertaken. Results. Twelve articles were chosen and assessed for quality. Physical and social function of extremity sarcoma survivors is below that of the general population. Overall QoL scores of these patients are comparable to those of the general population. Studies that used more recently treated cohorts found that patients who had limb sparing surgery displayed superior functional outcomes over those that underwent amputations. Pain and perceiving that the cancer negatively influenced opportunities was associated with poor outcomes. Conclusion. The available literature regarding QoL in extremity sarcoma patients is heterogeneous in terms of aims and assessment tools. Results need to be interpreted in light of the improved management of extremity sarcoma in more recent patient cohorts.

  6. Contemporary Management of Retroperitoneal Soft Tissue Sarcomas.

    Science.gov (United States)

    Olimpiadi, Yuliya; Song, Suisui; Hu, James S; Matcuk, George R; Chopra, Shefali; Eisenberg, Burton L; Sener, Stephen F; Tseng, William W

    2015-08-01

    Management of retroperitoneal soft tissue sarcomas (RP STS) can be very challenging. In contrast to the more common extremity STS, the two predominant histologic subtypes encountered in the retroperitoneum are well-differentiated/dedifferentiated liposarcoma and leiomyosarcoma. Surgery remains the mainstay of treatment for RP STS. Preoperative planning and anticipation of the need for resection of adjacent organs/structures are critical. The extent of surgery, including the role of compartmental resection, is still controversial. Radiation therapy may be an important adjunct to surgery to provide locoregional disease control; this is currently being evaluated in the preoperative setting in the EORTC STRASS trial. Systemic therapy, tailored to the specific histologic subtype, may also be of benefit for the management of RP STS. Further investigation of novel therapies (e.g., targeted therapies, immunotherapy) is needed. Overall, multi-institutional collaboration is important moving forward, to continue to better understand and optimize management of this disease.

  7. Epithelioid sarcoma of the upper extremity with cerebral metastases.

    Directory of Open Access Journals (Sweden)

    Rege A

    1992-10-01

    Full Text Available A forty year old male patient presented with swollen, deformed right hand with multiple irregular ulcers. The axillary lymph nodes were enlarged, firm and tender. The biopsy of nodules present on the hand revealed areas of haemorrhage and necrosis. The histopathological examination confirmed the clinical diagnosis of epithelioid sarcoma. Following below-elbow amputation, patient failed to regain consciousness and expired. On autopsy, distant metastases were found in the regional lymph nodes, pleura, kidney and cerebrum.

  8. Liposomal doxorubicin (Doxil): an effective new treatment for Kaposi's sarcoma in AIDS.

    Science.gov (United States)

    James, N D; Coker, R J; Tomlinson, D; Harris, J R; Gompels, M; Pinching, A J; Stewart, J S

    1994-01-01

    The objective of this study was to assess the efficacy and toxicity of a novel Stealth liposomal encapsulated formulation of doxorubicin (Doxil). A Phase I/II dose escalation study was carried out in a specialist HIV oncology unit in a teaching hospital (predominantly in an outpatient department). Fifteen patients with HIV related, biopsy confirmed, cutaneous Kaposi's sarcoma, with or without visceral involvement of sufficient severity to require systemic chemotherapy, were treated. Most patients had poor prognosis disease as assessed by the Tumour/Immune status/Systemic symptoms (TIS) system and Karnofsky indices; six patients had previously received combination chemotherapy. Primary treatment consisted of a dose of Doxil 10 mg/m2, repeated after 2 weeks. If the Kaposi's sarcoma (KS) responded and the treatment was tolerated, the patient began maintenance therapy at the same dose every 2 weeks. If there was no clinical response, the dose was increased to 20 mg/m2 for the further two cycles, before proceeding to maintenance therapy. Treatment continued until other intercurrent disease, lack of further response, patient preference, or toxicity precluded further treatment. Tumour response was assessed 2 weeks after completion of at least two cycles of chemotherapy. Toxicity was assessed for each cycle. Doxil was well tolerated, and toxicity was manageable, the principal toxicity being haematological. A partial response rate of 11/15 (73%) was achieved, with disease stabilization in the remaining patients. We conclude that Doxil is an effective palliative treatment for epidemic KS in a patient group with a poor predicted outcome.(ABSTRACT TRUNCATED AT 250 WORDS)

  9. Clear Cell Sarcoma (Malignant Melanoma of Soft Parts: A Clinicopathologic Study of 52 Cases

    Directory of Open Access Journals (Sweden)

    O. Hocar

    2012-01-01

    Full Text Available Clear cell sarcomas are aggressive, rare soft tissue tumors and their classification among melanoma or sarcoma is still undetermined due to their clinical, pathologic, and molecular properties found in both types of tumors. This is a retrospective study of 52 patients with CCS seen between April 1979 and April 2005 in two institutions. The EWS-ATF-1 fusion transcript was studied in 31 patients and an activating mutation of the BRAF or NRAS gene was researched in 22 patients. 30 men and 22 women, with a mean age of 33 were studied. Forty-three tumors (82.69% were located in the extremities, specially the foot (19 tumors. Median initial tumor size was 4.8 cm (1 to 15 cm. Necrosis involving more than 50% of the tumor cells was found in 14 cases (26.92%. High mitotic rate (>10 was found in 25 cases (48.07%. The EWS/ATF-1 translocation was found in 28 (53.84% of 31 patients studied, and mutation of BRAF or NRAS was found in only 2 of 22 patients analyzed cases (3.84%. Among the tumor-associated parameters, only tumor size (>4 cm emerged as a significant prognostic factor. Forty-nine patients had a localized disease at diagnosis (94.23% and underwent surgical resection immediately (90% or after neoadjuvant chemotherapy (CT (10%. Various CT regimens were used in 37 patients (71.15% with no significant efficacy. The 5- and 10-year OS rates were 59% and 41%, respectively. Tumor size was the only emerging prognosis factor in our series. Complete surgical resection remains the optimal treatment for this aggressive chemoresistant tumor.

  10. One Case about Primary Mediastinal Primary Mediastinal Tumor 
with Mediastinal Sarcoma and Literature Review

    Directory of Open Access Journals (Sweden)

    Jinchen DU

    2016-09-01

    Full Text Available Primary mediastinal seminoma is a kind of germ cell malignancy outside the gonads, and it’s rarer with sarcoma component. This disease which has no special clinical manifestations and imaging characteristics is difficult to identify with other mediastinal tumors and mediastinal type lung cancer. This paper reported a case of primary mediastinal seminoma with mediastinal sarcoma. Through the analysis of the diagnosis and treatment process in this patient, we will make a comprehensive review of the disease.

  11. Stereotactic Body Radiotherapy for Metastatic and Recurrent Ewing Sarcoma and Osteosarcoma

    OpenAIRE

    Brown, Lindsay C.; Lester, Rachael A.; Grams, Michael P.; Haddock, Michael G.; Olivier, Kenneth R.; Arndt, Carola A S; Rose, Peter S.; Laack, Nadia N.

    2014-01-01

    Background. Radiotherapy has been utilized for metastatic and recurrent osteosarcoma and Ewing sarcoma (ES), in order to provide palliation and possibly prolong overall or progression-free survival. Stereotactic body radiotherapy (SBRT) is convenient for patients and offers the possibility of increased efficacy. We report our early institutional experience using SBRT for recurrent and metastatic osteosarcoma and Ewing sarcoma. Methods. We reviewed all cases of osteosarcoma or ES treated with ...

  12. MR findings of primary Ewing's sarcoma of greater wing of sphenoid.

    Science.gov (United States)

    Singh, Paramjeet; Jain, Manoj; Singh, D P; Kalra, Naveen; Khandelwal, N; Suri, S

    2002-12-01

    Primary Ewing's sarcoma of the skull is a very rare entity. We report MRI findings in a case of Ewing's sarcoma of the greater wing of sphenoid in a 4-year-old patient. Magnetic resonance imaging showed markedly heterogenous signal intensity with areas of haemorrhage and necrosis. It also demonstrated the exact extent of tumour due to its multiplanar capabilities and was, therefore, helpful in planning surgery.

  13. NY-ESO-1 expression in synovial sarcoma and other mesenchymal tumors: significance for NY-ESO-1-based targeted therapy and differential diagnosis.

    Science.gov (United States)

    Lai, Jin-Ping; Robbins, Paul F; Raffeld, Mark; Aung, Phyu Phyu; Tsokos, Maria; Rosenberg, Steven A; Miettinen, Markku M; Lee, Chyi-Chia Richard

    2012-06-01

    A promising targeted therapy against NY-ESO-1 (CTAG 1B) using genetically modified T-cells in synovial sarcomas was recently demonstrated in a clinical trial at the NCI. To investigate the role of NY-ESO-1 immunohistochemistry in patient selection and gain better insight into the incidence of NY-ESO-1 expression in synovial sarcomas and other mesenchymal tumors, we evaluated NY-ESO-1 expression by immunohistochemistry in 417 tumors. This collection of samples included: 50 SS18/SSX1/2 fusion positive synovial sarcomas, 155 gastrointestinal stromal tumors (GIST), 135 other spindle cell sarcomas as well as 77 other sarcomas (chondrosarcoma, osteosarcoma, dedifferentiated liposarcoma, alveolar soft part sarcoma, rhabdomyosarcoma, angiosarcoma, malignant mesothelioma, and Ewing's sarcoma). We report that 76% of synovial sarcomas expressed NY-ESO-1 in a strong and diffuse pattern (2-3+, >50-70% of tumor cells). In contrast, only rare cases of other spindle cell mesenchymal tumor expressed NY-ESO-1 (GIST (2/155), malignant peripheral nerve sheath tumors (1/34), and dermatofibrosarcoma protuberans (2/20)). Individual cases of other sarcomas (angiosarcoma, malignant mesothelioma, chondrosarcoma, osteosarcoma, dedifferentiated liposarcoma, alveolar soft part sarcoma, and Ewing's sarcoma) were positive for NY-ESO-1. However, no positive cases were identified amongst our cohort of leiomyosarcomas (0/24), hemangiopericytoma/solitary fibrous tumors (0/40), and cellular schwannomas (0/17). In summary, we find that NY-ESO-1 is strongly and diffusely expressed in a majority of synovial sarcomas, but only rarely in other mesenchymal lesions. Beyond its role in patient selection for targeted therapy, immunohistochemistry for NY-ESO-1 may be diagnostically useful for the distinction of synovial sarcoma from other spindle cell neoplasms.

  14. Alveolar soft-part sarcoma of the tongue in a 17-month-old.

    Science.gov (United States)

    Hillyer, Shahab; Vicens, Jose C; Levinson, Howard; Bhayani, Rajendra; Mesea, Lilia; Chaudhry, Rashid; Fayans, Edgar; Fogler, Richard

    2009-10-01

    Alveolar soft-part sarcoma (ASPS) is a rare and often fatal tumor. Overall survival rates have been reported to be 62% after 5 years, 42% after 10 years, and 18% after 20 years. ASPS accounts for 5% of all pediatric soft-tissue sarcomas other than rhabdomyosarcomas. In children, ASPS rarely occurs in the oral cavity, and to the best of our knowledge, only 12 cases of ASPS of the tongue occurring during the first decade of life have been previously reported in the literature. Because of the rarity of lingual ASPS in children, no specific treatment protocols have been developed, which makes its management difficult. We report a new case of lingual ASPS in a young child. Our patient was a 17-month-old girl whose tumor was located at the base of the tongue. She was successfully treated with surgical excision without adjuvant radiation or chemotherapy. Postoperatively, she has remained disease-free during 4 years of follow-up.

  15. Nursing care of 9 patients with Kaposi sarcoma accepting radiotherapy%9例卡波西肉瘤病人放射治疗的护理

    Institute of Scientific and Technical Information of China (English)

    蔺波; 蔡蕊

    2010-01-01

    @@ 卡波西肉瘤(Kaposi's sarcoma,KS)是匈牙利皮肤学家Moritz Kaposi于1872年首次发现的一种罕见的肿瘤性疾病[1].又称多发性、特发性出血性肉瘤.本病分为经典型KS、艾滋病(AIDS)相关型KS、地方性KS、医源性或移植后KS四种类型[2].按照Kaposi的描述,肿瘤多中心发生,全身皮肤及血管广泛受累,表现为紫色结节性皮肤损害,被称为经典的卡波西肉瘤.经典型KS有明显的种族和地理差异,好发于中欧犹太人、波兰人、俄罗斯人和意大利人.在我国主要分布于新疆地区的维吾尔族和哈萨克族人[3].此种病例极为罕见.我科于2007年1月-2008年9月共收治经典型卡波西肉瘤病人9例,给予放射治疗后效果满意.现将临床特征及护理介绍如下.

  16. Primary Histiocytic Sarcoma of the Central Nervous System

    Science.gov (United States)

    So, Hoonsub; Kim, Sun A; Yoon, Dok Hyun; Khang, Shin Kwang; Hwang, Jihye; Suh, Chong Hyun; Suh, Cheolwon

    2015-01-01

    Histiocytic sarcoma is a type of lymphoma that rarely involves the central nervous system (CNS). Its rarity can easily lead to a misdiagnosis. We describe a patient with primary CNS histocytic sarcoma involving the cerebral hemisphere and spinal cord, who had been initially misdiagnosed as demyelinating disease. Two biopsies were necessary before a correct diagnosis was made. A histologic examination showed bizarre shaped histiocytes with larger nuclei and nuclear atypia. The cells were positive for CD68, CD163, and S-100 protein. As a resection was not feasible due to multifocality, he was treated with highdose methotrexate, but showed no response. As a result, he was switched to high dose cytarabine; but again, showed no response. The patient died 2 months from the start of chemotherapy and 8 months from the onset of symptoms. Since few patients with this condition have been described and histopathology is difficult to diagnose, suspicion of the disease is essential. PMID:25345462

  17. Soft tissue reconstruction after lower extremity limb-sparing pediatric sarcoma resection

    Institute of Scientific and Technical Information of China (English)

    Kevin Shultz; Nicholas Webster; Miguel A Soto-Miranda; Anas Eid; Jon P Ver Halen

    2016-01-01

    Aim:Limb salvage is the treatment of choice for lower extremity bone sarcomas in children. To date, peers have not described algorithms for soft tissue reconstruction of these defects. This paper is to report a large single center series of lower extremity salvage after sarcoma treatment, with algorithm. Methods:The authors performed a retrospective review of patients undergoing resection of lower extremity bone sarcomas at a single center over 12 years. Results:In total, 65 children (29 girls, 36 boys) with a mean age of 13 years (range 2.9-23.3 years) underwent resection of a lower leg sarcoma with limb-salvage. Tumors types included 50 osteosarcomas, and 15 Ewing sarcoma family of tumors. The types of reconstruction utilized included:34 primary closures, 22 gastrocnemius and soleus lfaps, 3 bipedicled lfaps, 2 sural artery lfaps, 1 pedicled anterolateral thigh lfap, 3 pedicled posterior thigh lfaps for subsequent above-knee amputations. No free lfap based reconstructions were performed. An algorithm for reconstruction of leg defects in the setting of limb-salvage surgery is presented. Successful limb salvage rate was 95.4%. Limb salvage failed in 3 patients and they required amputation. Finally, 56 patients were able to ambulate without assistance at last follow-up. Conclusion:The authors present an algorithm for the reconstruction soft tissue after resection of lower extremity bone sarcomas. The use of these techniques helps to decrease complications and maximize function in children with these tumors.

  18. Imaging features of radiation-induced sarcoma in patients with nasopharyngeal carcinoma%鼻咽癌放疗后继发肉瘤的影像特征分析

    Institute of Scientific and Technical Information of China (English)

    何明燕; 蔡培强; 班晓华; 刘学文; 谢传淼; 张嵘

    2014-01-01

    目的 总结鼻咽癌放疗后继发肉瘤的CT和MRI影像特征.方法 回顾性分析鼻咽癌放疗后确诊为继发肉瘤73例患者的临床特征、CT及MRI表现.73例患者中,43例行CT检查,24例行MR检查,6例同时行CT和MR检查.结果 继发肉瘤中前3位的病理类型依次为纤维肉瘤45.3% (33/73)、骨肉瘤31.5% (23/73)和恶性纤维组织细胞瘤9.6%(7/73).好发部位前3位依次为上颌窦26.7% (20/73)、颈部软组织17.8%(13/73)及下颌骨13.7%(10/73).鼻咽癌放疗继发肉瘤在CT和MRI上均以形成密度或信号不均匀软组织肿块为主要表现.肿块形态不规则,与周围组织分界不清78.1%(57/73);肿块呈类圆形或圆形,与周围组织分界清楚21.9%(16/73).CT平扫呈等或混杂密度,平扫T2 WI呈稍高信号,T1WI呈等信号.增强扫描明显均匀或不均匀强化65.8%(48/73),中度均匀或不均匀强化23.2%(17/73),轻度均匀或不均匀强化11.0%(8/73).骨肉瘤中,78.3%(18/23)可见新生瘤骨.结论 鼻咽癌放疗继发肉瘤的影像表现有一定特征,结合放疗病史及发病部位,借助于放疗后严格、系统的影像复查能够及早做出正确的诊断.%Objective To summarize the CT and MRI features of radiation-induced sarcoma (RIS) after radiotherapy in patients with nasopharyngeal carcinoma (NPC).Methods From January 1997 to October 2012,a total of 73 NPC patients with RIS after radiotherapy were confirmed by pathology.The clinical data and imaging findings (CT and MRI findings) were retrospectively reviewed.Of the 73 patients,43 underwent CT examination,24 underwent MRI,and the remaining 6 underwent both CT and MRI scans.Results Fibrosarcoma [45.3% (33/73)] was the most frequently histologic type,followed by osteosarcoma[31.5% (23/73)] and malignant fibrous histiocytoma [9.6% (7/73)].The top three common sites were maxillary sinus [26.7% (20/73)],followed by the neck soft tissue [17.8% (13/73)] and mandible[13.7% (10

  19. 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

    Purpose. Doxorubicin (dox) still appears to be one of the most active drugs in the treatment of soft tissue sarcomas. However, treatment duration is limited due to cumulative cardiotoxicity. A number of small studies from single institutions have suggested activity of other analogues. In two...... 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......, epi is not superior to dox in the treatment of patients with advanced soft tissue sarcomas. In addition, the results illustrate that the data from small studies of single institutions should always be confirmed by large multi-institutional studies before being taken for granted....

  20. Head and Neck Soft Tissue Sarcoma

    OpenAIRE

    Aljabab, A. S.; Nason, R. W.; Kazi, R; Pathak, K. A.

    2011-01-01

    Sarcomas are malignant neoplasms originating from mesodermal tissues and constitute less than 1% of body’s tumors, including those of the head and neck region. 5–15% of adult sarcomas are in the head and neck region (20% from bones and cartilages and 80% in soft tissues). Commonly encountered sarcomas in the head and neck region are - osteosarcoma, rhabdomyosarcoma, malignant fibrous histiocytoma, fibrosarcoma and angiosarcoma. This article reviews the available literature on head and neck sa...