WorldWideScience

Sample records for bone sarcoma group

  1. 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 ...

  2. 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.

  3. 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....

  4. 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.

  5. 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...

  6. 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.

  7. 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.

  8. 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.

  9. 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.

  10. Limitations of Single Slice Dynamic Contrast Enhanced MR in Pharmacokinetic Modeling of Bone Sarcomas

    Energy Technology Data Exchange (ETDEWEB)

    Toms, Andoni P. (Dept. of Radiology, The Norfolk and Norwich Univ. Hospital, Norwich, Norfolk (United Kingdom)); White, Lawrence M.; Bleakney, Robert R. (Dept. of Medical Imaging, Mount Sinai Hospital, Toronto, ON (Canada)); Kandel, Rita (Dept. of Pathology and Laboratory Medicine, Mount Sinai Hospital, Toronto, ON (Canada)); Noseworthy, Michael (Health Sciences Centre, Faculty of Health Sciences, McMaster Univ., Hamilton, ON (Canada)); Lee, Shepstone (Institute of Health, Univ. of East Anglia, Norwich, Norfolk (United Kingdom)); Blackstein, Martin E. (Dept. of Oncology, Mount Sinai Hospital, Toronto, ON (Canada)); Wunder, Jay (Musculoskeletal Oncology Unit, Mount Sinai Hospital, Toronto, ON (Canada))

    2009-06-15

    Background: Single slice dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI) appears to provide perfusion data about sarcomas in vivo that correlate with tumor necrosis on equivalent pathological sections. However, sarcomas are heterogeneous and therefore single slice DCE-MRI may not correlate with total tumor necrosis. Purpose: To determine whether changes in pharmacokinetic modeling of DCE-MRI, during chemotherapy for primary bone sarcomas correlated with histological measures of total tumor necrosis. Material and Methods: Twelve patients with appendicular primary bone sarcomas were included in the study. Each patient had DCE-MRI before, and after completion, of pre-operative chemotherapy. The mean arterial slope (A), endothelial permeability coefficient (Ktrans), and extravascular extracellular volume (Ve) were derived from each data set using a modified two compartment pharmacokinetic model. Total tumor necrosis rates were compared with changes in A, Ktrans, and Ve. Results: Six patients had total tumor necrosis of =90% and six had a measure of <90%. The median percentage changes in A, Ktrans, and Ve for the =90% necrosis group were -52.5% (-83 to 6), -66% (-82 to 26), and 23.5% (-26 to 40), respectively. For the <90% necrosis group, A = - 35% (-75 to 132), Ktrans= - 53 (-66 to 149) and Ve= - 14.5% (-42 to 40). One patient with >90% necrosis had increases in all three measures. Comparison of the two groups generated P-values of 0.699 for A, 0.18 for Ktrans, and 0.31 for Ve. Conclusion: There was no statistically significant correlation between changes in pharmacokinetic perfusion parameters and total tumor necrosis. When using single slice DCE-MRI heterogeneous histology of primary bone sarcomas and repair mediated angiogenesis might both be confounding factors

  11. Late sarcoma development after curettage and bone grafting of benign bone tumors

    Energy Technology Data Exchange (ETDEWEB)

    Picci, Piero, E-mail: piero.picci@ior.it [Bone Tumor Center, Istituto Ortopedico Rizzoli, Bologna (Italy); Sieberova, Gabriela [Dept. of Pathology, National Cancer Institute, Bratislava (Slovakia); Alberghini, Marco; Balladelli, Alba; Vanel, Daniel [Bone Tumor Center, Istituto Ortopedico Rizzoli, Bologna (Italy); Hogendoorn, Pancras C.W. [Dept. of Pathology, Leiden University Medical Center, Leiden (Netherlands); Mercuri, Mario [Bone Tumor Center, Istituto Ortopedico Rizzoli, Bologna (Italy)

    2011-01-15

    Background and aim: Rarely sarcomas develop in previous benign lesions, after a long term disease free interval. We report the experience on these rare cases observed at a single Institution. Patients and methods: 12 cases curetted and grafted, without radiotherapy developed sarcomas, between 1970 and 2005, 6.5-28 years from curettage (median 18, average 19). Age ranged from 13 to 55 years (median 30, average 32) at first diagnosis; tumors were located in the extremities (9 GCT, benign fibrous histiocytoma, ABC, and solitary bone cyst). Radiographic and clinic documentation, for the benign and malignant lesions, were available. Histology was available for 7 benign and all malignant lesions. Results: To fill cavities, autogenous bone was used in 4 cases, allograft in 2, allograft and tricalcium-phosphate/hydroxyapatite in 1, autogenous/allograft in 1, heterogenous in 1. For 3 cases the origin was not reported. Secondary sarcomas, all high grade, were 8 osteosarcoma, 3 malignant fibrous histiocytoma, and 1 fibrosarcoma. Conclusions: Recurrences with progression from benign tumors are possible, but the very long intervals here reported suggest a different cancerogenesis for these sarcomas. This condition is extremely rare accounting for only 0.26% of all malignant bone sarcomas treated in the years 1970-2005 and represents only 8.76% of all secondary bone sarcomas treated in the same years. This incidence is the same as that of sarcomas arising on fibrous dysplasia, and is lower than those arising on bone infarcts or on Paget's disease. This possible event must be considered during follow-up of benign lesions.

  12. 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...... (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....

  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....../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...

  14. Theory of the induction of bone sarcoma by bone-seeking alpha emitters and its application to risk assessment

    Energy Technology Data Exchange (ETDEWEB)

    Petojan, I.M.

    1992-06-01

    This work discusses the theory of bone sarcoma induction by bone seeking alpha emitters, which is based strictly on biological considerations relative to the mechanism of radiation-induced carcinogenesis, identification of cells at risk and their location in bone, bone tissue renewal processes and bone cell kinetics with or without radiation exposure. The model is consistent with the data on bone sarcoma incidence human with incorporated long-lived isotopes Ra-226 + Ra-228. Extrapolation of these data to a low intake region of the basis of the developed theoretical approach suggests that the linear ICRP-UNSCEAR model overestimates carciogenic risk at low doses, possibly by a factor of 2-4. The model suggests a linear response of target cells to the initiation effects of alpha irradiation. The non-linear (linear-quadratic) initial part of dose-response curve for osteosarcoma induction is explained quantitatively by a model based on a promoter effect of regenerative hyperplasia resulting from invitation effects of alpha radiation. The maximum overestimation inherent to the model of the low-level risk due to the the dose-dependent promotion factor is estimated using bo{sup +1}/bo, where bo is a model parameter which is proportional to the normal division rate of osteogenic cells in vivo and which can be estimated within the framework of the model. The model provides confirming evidence that, for radiation protection purposes, endosteal cells may be considered the only group of cells at risk of sarcoma induction by low doses of bone-seeking alpha emitters, whereas the role of marrow stromal (osteogenic) cells as target cells is much more significant with increasing intakes, and can become dominating if intake is high enough.

  15. 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.

  16. 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

  17. Routine bone scintigraphy in primary staging of soft tissue sarcoma - Is it worthwhile?

    NARCIS (Netherlands)

    Jager, PL; Hoekstra, HJ; Leeuw, JA; van der Graaf, WTA; de Vries, EGE; Piers, DA

    2000-01-01

    BACKGROUND. The incidence of bone metastases in soft tissue sarcoma (STS) patients seems to be low but has not been studied separately. In this study, the authors aimed to determine the value of routine radionuclide bone scanning in preoperative staging of STS patients. METHODS. Preoperative bone sc

  18. Primary Ewing's sarcoma of the greater wing of the sphenoid bone.

    Science.gov (United States)

    Sharma, R R; Netalkar, A; Lad, S D

    2000-02-01

    Primary Ewing's sarcoma is an uncommon lethal tumour of the long bones and pelvic girdle mainly affecting children and young adults. An origin in the cranial bones is extremely rare. We report a unique case of primary involvement of the greater wing of sphenoid bone in a 16-year-old patient. Aggressive management using microsurgical resection, radiotherapy and chemotherapy was curative. Localized, primary Ewing's sarcoma of the cranial bones should be considered as a distinct clinicopathological entity with an extremely low rate of dural penetration and metastases, and with a relatively better prognosis as compared with those of long bones and pelvic girdle. In neurosurgical practice, primary Ewing's sarcoma of the cranial bones requires early aggressive management to achieve adequate long-term prognosis and cure.

  19. 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

  20. Risk of second bone sarcoma following childhood cancer: role of radiation therapy treatment.

    Science.gov (United States)

    Schwartz, Boris; Benadjaoud, Mohamed Amine; Cléro, Enora; Haddy, Nadia; El-Fayech, Chiraz; Guibout, Catherine; Teinturier, Cécile; Oberlin, Odile; Veres, Cristina; Pacquement, Hélène; Munzer, Martine; N'guyen, Tan Dat; Bondiau, Pierre-Yves; Berchery, Delphine; Laprie, Anne; Hawkins, Mike; Winter, David; Lefkopoulos, Dimitri; Chavaudra, Jean; Rubino, Carole; Diallo, Ibrahima; Bénichou, Jacques; de Vathaire, Florent

    2014-05-01

    Bone sarcoma as a second malignancy is rare but highly fatal. The present knowledge about radiation-absorbed organ dose-response is insufficient to predict the risks induced by radiation therapy techniques. The objective of the present study was to assess the treatment-induced risk for bone sarcoma following a childhood cancer and particularly the related risk of radiotherapy. Therefore, a retrospective cohort of 4,171 survivors of a solid childhood cancer treated between 1942 and 1986 in France and Britain has been followed prospectively. We collected detailed information on treatments received during childhood cancer. Additionally, an innovative methodology has been developed to evaluate the dose-response relationship between bone sarcoma and radiation dose throughout this cohort. The median follow-up was 26 years, and 39 patients had developed bone sarcoma. It was found that the overall incidence was 45-fold higher [standardized incidence ratio 44.8, 95 % confidence interval (CI) 31.0-59.8] than expected from the general population, and the absolute excess risk was 35.1 per 100,000 person-years (95 % CI 24.0-47.1). The risk of bone sarcoma increased slowly up to a cumulative radiation organ absorbed dose of 15 Gy [hazard ratio (HR) = 8.2, 95 % CI 1.6-42.9] and then strongly increased for higher radiation doses (HR for 30 Gy or more 117.9, 95 % CI 36.5-380.6), compared with patients not treated with radiotherapy. A linear model with an excess relative risk per Gy of 1.77 (95 % CI 0.6213-5.935) provided a close fit to the data. These findings have important therapeutic implications: Lowering the radiation dose to the bones should reduce the incidence of secondary bone sarcomas. Other therapeutic solutions should be preferred to radiotherapy in bone sarcoma-sensitive areas.

  1. 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.

  2. [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.

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

    NARCIS (Netherlands)

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

    1999-01-01

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

  4. Workshop Report on the European Bone Sarcoma Networking Meeting: Integration of Clinical Trials with Tumor Biology.

    Science.gov (United States)

    Thomas, David M; Wilhelm, Miriam; Cleton-Jansen, Anne-Marie; Dirksen, Uta; Entz-Werlé, Natacha; Gelderblom, Hans; Hassan, Bass; Jürgens, Heribert; Koster, Jan; Kovar, Heinrich; Lankester, Arjan C; Lewis, Ian J; Myklebost, Ola; Nathrath, Michaela H M; Picci, Piero; Whelan, Jeremy S; Hogendoorn, Pancras C W; Bielack, Stefan S

    2011-09-01

    A key workshop was held in The Netherlands in June 2011, hosted by several European bone sarcoma networks and with a broad range of stakeholders from Europe and Australia. The purpose of the meeting was to identify the strengths and weaknesses in current clinical trials for bone sarcomas and to make recommendations as to how to accelerate progress in this field. Two areas of particular interest were discussed. First, all participants agreed upon the importance of tumor biology to understanding clinical responses for all types of bone sarcoma. Various barriers to biobanking tumor and germline specimens were canvassed and are outlined in this paper. Second, there was consideration of the particular challenges of dealing with adolescent and young adult cancers, exemplified by bone sarcomas. Participants recommended greater engagement of both pediatric and adult sarcoma trial organizations to address this issue. Specific opportunities were identified to develop biological sub-studies within osteosarcoma, focused on understanding germ line risk and pharmacogenomics defining toxicity and biological responses. In Ewing sarcoma, it was harder to define opportunities for biological insights. There was agreement that the results for insulin-like growth factor pathway inhibition in Ewing family tumors were disappointing, but represented a clear indication of the need for companion biologic studies to develop predictive biomarkers. The meeting ended with broad commitment to working together to make progress in this rare but important subgroup of cancers.

  5. 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

  6. 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.

  7. 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

  8. The ENCCA-WP7/EuroSarc/EEC/PROVABES/EURAMOS 3rd European Bone Sarcoma Networking Meeting/Joint Workshop of EU Bone Sarcoma Translational Research Networks; Vienna, Austria, September 24-25, 2015. Workshop Report

    NARCIS (Netherlands)

    Kager, L.; Whelan, J.; Dirksen, U.; Hassan, B.; Anninga, J.; Bennister, L.; Bovee, J.V.; Brennan, B.; Broto, J.M.; Brugieres, L.; Cleton-Jansen, A.M.; Copland, C.; Dutour, A.; Fagioli, F.; Ferrari, S.; Fiocco, M.; Fleuren, E.D.; Gaspar, N.; Gelderblom, H.; Gerrand, C.; Gerss, J.; Gonzato, O.; Graaf, W. van der; Hecker-Nolting, S.; Herrero-Martin, D.; Klco-Brosius, S.; Kovar, H.; Ladenstein, R.; Lancia, C.; Ledeley, M.C.; McCabe, M.G.; Metzler, M.; Myklebost, O.; Nathrath, M.; Picci, P.; Potratz, J.; Redini, F.; Richter, G.H.; Reinke, D.; Rutkowski, P.; Scotlandi, K.; Strauss, S.; Thomas, D; Tirado, O.M.; Tirode, F.; Vassal, G.; Bielack, S.S.

    2016-01-01

    This report summarizes the results of the 3rd Joint ENCCA-WP7, EuroSarc, EEC, PROVABES, and EURAMOS European Bone Sarcoma Network Meeting, which was held at the Children's Cancer Research Institute in Vienna, Austria on September 24-25, 2015. The joint bone sarcoma network meetings bring together Eu

  9. Occupational factors and risk of adult bone sarcomas: a multicentric case-control study in Europe.

    Science.gov (United States)

    Merletti, Franco; Richiardi, Lorenzo; Bertoni, Franco; Ahrens, Wolfgang; Buemi, Antoine; Costa-Santos, Cristina; Eriksson, Mikael; Guénel, Pascal; Kaerlev, Linda; Jöckel, Karl-Heinz; Llopis-Gonzalez, Agustin; Merler, Enzo; Miranda, Ana; Morales-Suárez-Varela, Maria M; Olsson, Håkan; Fletcher, Tony; Olsen, Jorn

    2006-02-01

    We investigated the association between occupational factors and risk of bone sarcoma, a rare tumor with a largely unknown aetiology. A multicentric case-control study was conducted in 7 European countries in 1995-97. Ninety-six cases aged 35-69 years with a centrally reviewed diagnosis of bone sarcoma (68 chondrosarcomas and 28 osteosarcomas) were compared to 2,632 population (68%) or colon cancer (32%) controls. Subjects were interviewed to obtain information on occupational, medical and reproductive history, smoking and alcohol consumption and selected exposures including use of pesticides. Response proportions were 90% among cases and 66% among controls. Odds ratios (OR) and 95% confidence intervals (CI) were estimated for selected categories of job titles and branches of industry and for use of pesticides. We found an increased OR for bone sarcoma among blacksmiths, toolmakers, machine-tool operators (OR = 2.14, 95% CI 1.08-4.26), woodworkers (OR = 2.68, 95% CI 1.36-5.29) and construction workers (OR = 1.62, 95% CI 0.92-2.87). Ever users of pesticide had an OR of 2.33 (95% CI 1.31-4.13), with similar risks for exposure to insecticides and exposure to herbicides. Neither duration of employment in any of the analyzed occupational categories nor duration of use of pesticides showed an increasing trend in the risk of bone sarcoma. ORs of bone sarcoma were 1.03 (95% CI 0.23-4.57), 3.13 (95% CI 1.26-7.76) and 1.44 (95% CI 0.43-4.85) for the first, second and third tertile of days of use of pesticides. Our study suggests that novel and previously reported (woodworking) occupational factors play a role in the aetiology of bone sarcomas.

  10. Usefulness of F-18 FDG PET/CT in a case of Kaposi sarcoma with an unexpected bone lesion.

    Science.gov (United States)

    Morooka, Miyako; Ito, Kimiteru; Kubota, Kazuo; Yanagisawa, Kunio; Teruya, Katsuji; Hasuo, Kahehiro; Shida, Yoshitaka; Minamimoto, Rhogo; Kikuchi, Yoshimi; Oka, Shinichi

    2011-03-01

    Bone lesions of Kaposi sarcoma are rare. A 56-year-old man who was HIV positive and was diagnosed with Kaposi sarcoma on the basis of the results of a biopsy of skin lesions, underwent F-18 FDG PET/CT scan for detecting Kaposi sarcoma lesions and other AIDS-related diseases. An abnormal uptake was observed in the lumbar spine. MRI showed a diffuse enhanced spine lesion, and Ga-67 and ²⁰¹Tl scanning were negative. As a result, the lesion was considered to be a Kaposi sarcoma, and the shrinkage of the lesion was noted after the therapy for Kaposi sarcoma.

  11. Radioisotopic methods for the study of bone sarcoma and soft tissue neoplasms

    Energy Technology Data Exchange (ETDEWEB)

    Gongora, R.

    1988-01-01

    Radioisotopic methods are widely applied to investigations of bone sarcoma and soft tissue neoplasms. We have at our disposal molecules with osseous, tumoral or vascular tropism. Their use, as single agents or combination, is helpful in positive and differential diagnosis and provides nosological informations. They are also useful in treatment monitoring and in long-term follow-up.

  12. Alpha radiation risk coefficients for liver cancer, bone sarcomas, and leukemia

    Energy Technology Data Exchange (ETDEWEB)

    Hunacek, M.M.; Kathren, R.L. [Washington State Univ., Richland WA (United States)

    1995-01-01

    This study compares published risk coefficients with those determined from dose rates established by postmortem radiochemical analysis of tissues from two whole body donors to the U.S. Transuranium and Uranium Registries, both of whom had been injected with Thorotrast approximately four decades prior to death. The dose data from these cases were used in combination with published latent periods and epidemiologic study results to calculate the following risk coefficients: 0.020 liver cancers Gy{sup -1}, 0.002 bone sarcomas Gy{sup -1}, and 0.032 leukemias Gy{sup -1}. These compare with the ranges of 0.60 to 0.074 liver cancers Gy{sup -1}, 0.0016 to 0.0120 bone sarcomas Gy{sup -1}, and 0.005 to 0.060 leukemias Gy{sup -1} reported in the literature. The results of this study are generally consistent with previously reported values with two exceptions: the values for bone sarcomas fall below the range given by BEIR IV and the values for leukemia are a factor of 6 greater than those reported by BEIR IV. This suggests that the BEIR IV risk coefficient for bone sarcomas may be too high, and that for leukemia may be too low. 46 refs., 5 tabs.

  13. The Prognostic Value of Serum Biomarkers in Localized Bone Sarcoma

    DEFF Research Database (Denmark)

    Aggerholm-Pedersen, Ninna; Nielsen, Katja Maretty; Keller, Johnny;

    2016-01-01

    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...... cases. Adjusting for confounders such as comorbidity and age is an essential safeguard against erroneous conclusions regarding the possible prognostic value of these biomarkers. The aim of this study was to assess the prognostic value of a battery of pretreatment biomarkers in the serum of patients......, lymphocytes, and sodium were collected from the patient records. The prognostic values of overall and disease-specific mortality were tested for each individual biomarker as well as for the Glasgow prognostic score (GPS) and for a new composite score incorporating five biomarkers (Aarhus composite biomarker...

  14. 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)

  15. A RARE CASE OF ATYPICAL PRIMARY EWING’S SARCOMA OF OCCIPITAL BONE

    Directory of Open Access Journals (Sweden)

    K. Srihari

    2016-07-01

    Full Text Available BACKGROUND Ewing’s Sarcoma is an aggressive malignant neoplasm most frequently manifesting in the second decade of life and accounting for 4% of childhood and adolescent malignancies. These tumours were first described by James Ewing in 1921 as tumours that arise from bone. These osseous lesions have since become infamous for their highly aggressive course with 20% to 30% of patients having evidence of metastasis at the time of diagnosis and an estimated 10-year survival rate of 50%. Metastases to the CNS have most recently been estimated to occur in less than 5% of cases and are usually due to direct extension of an osseous lesion into the extradural space or more rarely through haematogenous spread. CASE REPORT In this article, we report a case of 17-year-old boy who presented to the radiology department with complaints of recurrent episodes of headache and vomiting for the past 3 days. On radiological investigation, there was a large well-defined, lobulated, extra-axial mass lesion measuring 3.6 X 5.7 X 5.9 cm noted in the supratentorial left occipital region which was fairly enhancing after contrast administration. The mass was causing permeative type of destruction of the left occipital bone and extending into extracranial soft tissue. Final diagnosis was done by biopsy and histopathology which showed “Atypical Ewing’s Sarcoma” of the left occipital bone. Considering its unusual site and soft tissue extension, we report this case of Primary Atypical Ewing’s sarcoma of occipital bone. CONCLUSION Primary cranial Ewing's sarcoma is to be considered in the differential diagnosis in children with a tumour involving the skull with destruction of the bone and presence of extra-axial soft tissue swelling. CT is the excellent modality for demonstration of bone destruction while MRI depicts soft tissue extension and metastasis if any.

  16. 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)....

  17. Giant myxoinflammatory fibroblastic sarcoma with bone invasion:a very rare clinical entity and literature review

    Institute of Scientific and Technical Information of China (English)

    Guray Togral; Murat Arikan; Elif Aktas; Safak Gungor

    2014-01-01

    Myxoinflammatory fibroblastic sarcoma (MIFS) is a rare low-grade, malignant soft tissue tumor that is usually observed in the extremities of adult patients. Magnetic resonance imaging findings for this tumor type have rarely been reported. We report a case involving the distal left femur of a middle-aged man and tumoral invasion of the bone, which, to our knowledge, has been previously described only once. He was treated with distal femoral tumor resection and reconstruction with a modular prosthesis. Histopathologic diagnosis confirmed MIFS. We reviewed literature of the diagnostic imaging and bone invasion findings associated with this tumor type.

  18. Ewings sarcoma of 4th metacarpal bone of right hand, a rare case report

    Directory of Open Access Journals (Sweden)

    Basharat Mubeen

    2014-05-01

    Full Text Available Ewings sarcoma of the hand is relatively rare. Ewings sarcoma can present with minimal pain and swelling of the affected digit. The Erythrocyte sedimentation rate and C-reactive protein may be high. Because of these features, this can be confused with an inflammatory lesion. This leads to starting of inappropriate treatment and delay in diagnosis. Radiologically, Ewings sarcoma can present with a plethora of features from permeative bone destruction to expansile lesions with or without periosteal reaction. We present a case of 10 year old male, with complaints of pain and swelling in right hand, which on X-ray showed periosteal reaction, giving a sun burst appearance. So the provisional diagnosis of osteosarcoma was made. The diagnosis of ewings sarcoma was made on FNAC [fine needle aspiration cytology] which was later on confirmed on histopathology. We intend to report this case, because it is very rare location and the radiological features can mimic other lesions which commonly occur in this location like chronic osteomyelitis, osteosarcoma so it can be easily missed especially at preliminary evaluations.

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

    Directory of Open Access Journals (Sweden)

    Ning Wang

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

  20. CNS Metastases from Bone and Soft Tissue Sarcomas in Children, Adolescents, and Young Adults: Are They Really So Rare?

    Science.gov (United States)

    Duczkowska, Agnieszka; Duczkowski, Marek; Bragoszewska, Hanna; Romaniuk-Doroszewska, Anna; Iwanowska, Beata; Szkudlinska-Pawlak, Sylwia; Madzik, Jaroslaw; Bilska, Katarzyna; Raciborska, Anna

    2017-01-01

    Purpose. To check whether primary involvement of brain/spinal cord by bone/soft tissue sarcomas' metastases in children is as rare as described and to present various morphological forms of bone/soft tissue sarcomas' CNS metastases. Methods. Patients with first diagnosis in 1999–2014 treated at single center were included with whole course of disease evaluation. Brain/spinal canal magnetic resonance imaging (MRI)/computed tomography were performed in cases suspicious for CNS metastases. Extension from skull/vertebral column metastases was excluded. Results. 550 patients were included. MRI revealed CNS metastases in 19 patients (incidence 3.45%), 14 boys, aged 5–22 years. There were 12/250 osteosarcoma cases, 2/200 Ewing's sarcoma, 1/50 chondrosarcoma, 3/49 rhabdomyosarcoma (RMS), and 1/1 malignant mesenchymoma. There were 10 single metastases and 7 cases of multiple ones; in 2 RMS cases only leptomeningeal spread in brain and spinal cord was found. Calcified metastases were found in 3 patients and hemorrhagic in 4. In one RMS patient there were numerous solid, cystic, hemorrhagic lesions and leptomeningeal spread. Conclusions. CNS metastases are rare and late in children with bone/soft tissue sarcomas, although in our material more frequent (3.45%) than in other reports (0.7%). Hematogenous spread to brain and hemorrhagic and calcified lesions dominated in osteosarcoma. Ewing sarcoma tended to metastasize to skull bones. Soft tissue sarcomas presented various morphological forms.

  1. A reappraisal of hemangiopericytoma of bone; analysis of cases reclassified as synovial sarcoma and solitary fibrous tumor of bone

    DEFF Research Database (Denmark)

    Verbeke, Sofie L J; Fletcher, Christopher D M; Alberghini, Marco;

    2010-01-01

    21 and 73 years. All tumors were located within bone, either sited within spine or extremities. All tumors showed thin-walled branching vessels surrounded by undifferentiated spindle or round cells. These cells showed variation in their morphologic pattern: 6 tumors showed a pattern-less architecture...... are characterized by distinct morphology and immunohistochemical profile. SFT of bone is located within spine and has a better prognosis, whereas SS of bone is located within long bones having a poor prognosis.......Hemangiopericytoma (HPC) was first described as a neoplasm with distinct morphologic features, presumably composed of pericytes. In soft tissue, it is accepted that most such lesions are solitary fibrous tumors (SFTs), monophasic synovial sarcomas (SSs), or myofibromatoses. It is unclear whether...

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

    Directory of Open Access Journals (Sweden)

    Boris Michael Holzapfel

    2013-01-01

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

  3. 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...

  4. 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

  5. CNS Metastases from Bone and Soft Tissue Sarcomas in Children, Adolescents, and Young Adults: Are They Really So Rare?

    Directory of Open Access Journals (Sweden)

    Monika Bekiesinska-Figatowska

    2017-01-01

    Full Text Available Purpose. To check whether primary involvement of brain/spinal cord by bone/soft tissue sarcomas’ metastases in children is as rare as described and to present various morphological forms of bone/soft tissue sarcomas’ CNS metastases. Methods. Patients with first diagnosis in 1999–2014 treated at single center were included with whole course of disease evaluation. Brain/spinal canal magnetic resonance imaging (MRI/computed tomography were performed in cases suspicious for CNS metastases. Extension from skull/vertebral column metastases was excluded. Results. 550 patients were included. MRI revealed CNS metastases in 19 patients (incidence 3.45%, 14 boys, aged 5–22 years. There were 12/250 osteosarcoma cases, 2/200 Ewing’s sarcoma, 1/50 chondrosarcoma, 3/49 rhabdomyosarcoma (RMS, and 1/1 malignant mesenchymoma. There were 10 single metastases and 7 cases of multiple ones; in 2 RMS cases only leptomeningeal spread in brain and spinal cord was found. Calcified metastases were found in 3 patients and hemorrhagic in 4. In one RMS patient there were numerous solid, cystic, hemorrhagic lesions and leptomeningeal spread. Conclusions. CNS metastases are rare and late in children with bone/soft tissue sarcomas, although in our material more frequent (3.45% than in other reports (0.7%. Hematogenous spread to brain and hemorrhagic and calcified lesions dominated in osteosarcoma. Ewing sarcoma tended to metastasize to skull bones. Soft tissue sarcomas presented various morphological forms.

  6. Disseminated bone lesions in AIDS-associated Kaposi sarcoma, a bad prognosis? About four cases

    Directory of Open Access Journals (Sweden)

    N Wassilew

    2012-11-01

    Full Text Available Kaposi sarcoma (KS can present with a wide range of clinical features ranging from minimal cutaneous disease to a rapidly progressing neoplasm. Bone lesions are most often discovered accidently in the context of radiological investigations done for the screening of KS-visceral involvement [1]. Little is known on clinical outcome and response to antiretroviral therapy (ART and/or chemotherapy of these lytic osseous lesions. We report four cases with bone involvement in the context of systemic KS and aim at describing the long-term clinical outcome in two of these patients. Cases of AIDS-associated KS with disseminated bone lesions were collected in the HIV Unit, University Hospital Geneva, Switzerland. Patients were compared on clinical, biological and radiological features and therapeutic responses. Between 2002 and 2012, four HIV1-infected patients with T1 stage of KS presented disseminated osseous lesions (Table 1. Mean age was 43 years (range 39 - 47 years, mean time of follow up until our analysis was 48.5 months (SD 53.8, and mean CD4 count at KS diagnosis was 190.5 c/mL (SD 202.8. All patients showed hypodense bone lesions predominating the axial skeleton (figure 1, but no radiological imaging was performed to search for peripheral bone lesions.No patient reported pain or experienced pathological fractures. In one patient a dual-energy X-ray absorptiometry (DXA showed a bone mineral density within normal range after 10 years of KS diagnosis with disseminated bone lesions. No radiological change was observed in that patient despite stable KS disease after 13 cycles of liposomal doxorubicin and ART (figure 1. We describe a well-documented long-term follow up of disseminated osseous AIDS-associated KS disease. In our four cases, lytic bone lesions were asymptomatic and were not associated with bone fragility. We even could confirm the KS nature of the lesions by bone biopsy in patient B (3 months after KS diagnosis, as the differential

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

    Science.gov (United States)

    2016-09-23

    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

  8. Development of a preclinical orthotopic xenograft model of ewing sarcoma and other human malignant bone disease using advanced in vivo imaging.

    Directory of Open Access Journals (Sweden)

    Britta Vormoor

    Full Text Available Ewing sarcoma and osteosarcoma represent the two most common primary bone tumours in childhood and adolescence, with bone metastases being the most adverse prognostic factor. In prostate cancer, osseous metastasis poses a major clinical challenge. We developed a preclinical orthotopic model of Ewing sarcoma, reflecting the biology of the tumour-bone interactions in human disease and allowing in vivo monitoring of disease progression, and compared this with models of osteosarcoma and prostate carcinoma. Human tumour cell lines were transplanted into non-obese diabetic/severe combined immunodeficient (NSG and Rag2(-/-/γc(-/- mice by intrafemoral injection. For Ewing sarcoma, minimal cell numbers (1000-5000 injected in small volumes were able to induce orthotopic tumour growth. Tumour progression was studied using positron emission tomography, computed tomography, magnetic resonance imaging and bioluminescent imaging. Tumours and their interactions with bones were examined by histology. Each tumour induced bone destruction and outgrowth of extramedullary tumour masses, together with characteristic changes in bone that were well visualised by computed tomography, which correlated with post-mortem histology. Ewing sarcoma and, to a lesser extent, osteosarcoma cells induced prominent reactive new bone formation. Osteosarcoma cells produced osteoid and mineralised "malignant" bone within the tumour mass itself. Injection of prostate carcinoma cells led to osteoclast-driven osteolytic lesions. Bioluminescent imaging of Ewing sarcoma xenografts allowed easy and rapid monitoring of tumour growth and detection of tumour dissemination to lungs, liver and bone. Magnetic resonance imaging proved useful for monitoring soft tissue tumour growth and volume. Positron emission tomography proved to be of limited use in this model. Overall, we have developed an orthotopic in vivo model for Ewing sarcoma and other primary and secondary human bone malignancies, which

  9. 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

  10. Combination of Trabectedin and Gemcitabine for Advanced Soft Tissue Sarcomas: Results of a Phase I Dose Escalating Trial of the German Interdisciplinary Sarcoma Group (GISG

    Directory of Open Access Journals (Sweden)

    Bernd Kasper

    2015-01-01

    Full Text Available Background: Evaluation of the potential efficacy and safety of combination therapies for advanced soft tissue sarcomas (STS has increased substantially after approval of trabectedin and pazopanib. Trabectedin's introduction in Europe in 2007 depended mainly on its activity in so-called L-sarcomas (liposarcoma and leiomyosarcoma; combination of trabectedin with other chemotherapies used in STS seems of particular interest. Methods: We initiated within the German Interdisciplinary Sarcoma Group (GISG a phase I dose escalating trial evaluating the combination of trabectedin and gemcitabine in patients with advanced and/or metastatic L-sarcomas (GISG-02; ClinicalTrials.gov NCT01426633. Patients were treated with increasing doses of trabectedin and gemcitabine. The primary endpoint was to determine the maximum tolerated dose. Results: Five patients were included in the study. Two patients were treated on dose level 1 comprising trabectedin 0.9 mg/m2 on day 1 and gemcitabine 700 mg/m2 on days 1 + 8, every 3 weeks. Due to dose-limiting toxicity (DLT in both patients (elevated transaminases and thrombocytopenia, an additional three patients were treated on dose level −1 with trabectedin 0.7 mg/m2 plus gemcitabine 700 mg/m2. Of these three patients, two demonstrated another DLT; therefore, the trial was stopped and none of the dose levels could be recommended for phase II testing. Conclusion: The GISG-02 phase I study was stopped with the conclusion that the combination of gemcitabine and trabectedin is generally not recommended for the treatment of patients with advanced and/or metastatic leiomyosarcoma or liposarcoma. Also, this phase I study strongly supports the necessity for careful evaluation of combination therapies.

  11. Ewing Sarcoma of the Bone in Children under 6 Years of Age

    Science.gov (United States)

    De Ioris, Maria Antonietta; Prete, Arcangelo; Cozza, Raffaele; Podda, Marta; Manzitti, Carla; Pession, Andrea; Schiavello, Elisabetta; Contoli, Benedetta; Balter, Rita; Fagioli, Franca; Bisogno, Gianni; Amoroso, Loredana

    2013-01-01

    Background Ewing Sarcoma Family Tumours (ESFT) are rare in early childhood. The aim of this study was to report the clinical characteristics and outcome of children under 6 years of age affected by ESFT of the bone in Italy. Methods The records of all the children diagnosed with osseous ESFT in centres members of the Associazione Italiana di Ematologia ed Oncologia Pediatrica (AIEOP) from 1990 to 2008 were reviewed. The Kaplan–Meier method was used for estimating overall and progression-free survival (OS, PFS) curves; multivariate analyses were performed using Cox proportional hazards regression model. Results This study includes 62 patients. An axial primary localization was present in 66% of patients, with the primary site in the chest wall in 34%. Fourteen (23%) patients presented metastatic disease. The 5-year OS and PFS were 73% (95% confidence interval, CI, 58–83%) and 72% (95% CI 57–83%) for patients with localized disease and 38% (95% CI 17–60%) and 21% (95% CI 5–45%) for patients with metastatic disease. Metastatic spread, skull/pelvis/spine primary localization, progression during treatment and no surgery predicted worse survival (P<0.01), while patients treated in the last decade had better survival (P  = 0.002). In fact, the 5-year OS and PFS for patients diagnosed in the period 2000–2008 were 89% (95% CI 71–96%) and 86% (95% CI 66–94%), respectively. Conclusion The axial localization is the most common site of ESFT in pre-scholar children. Patients treated in the most recent period have an excellent outcome. PMID:23382839

  12. Ewing sarcoma of the bone in children under 6 years of age.

    Directory of Open Access Journals (Sweden)

    Maria Antonietta De Ioris

    Full Text Available BACKGROUND: Ewing Sarcoma Family Tumours (ESFT are rare in early childhood. The aim of this study was to report the clinical characteristics and outcome of children under 6 years of age affected by ESFT of the bone in Italy. METHODS: The records of all the children diagnosed with osseous ESFT in centres members of the Associazione Italiana di Ematologia ed Oncologia Pediatrica (AIEOP from 1990 to 2008 were reviewed. The Kaplan-Meier method was used for estimating overall and progression-free survival (OS, PFS curves; multivariate analyses were performed using Cox proportional hazards regression model. RESULTS: This study includes 62 patients. An axial primary localization was present in 66% of patients, with the primary site in the chest wall in 34%. Fourteen (23% patients presented metastatic disease. The 5-year OS and PFS were 73% (95% confidence interval, CI, 58-83% and 72% (95% CI 57-83% for patients with localized disease and 38% (95% CI 17-60% and 21% (95% CI 5-45% for patients with metastatic disease. Metastatic spread, skull/pelvis/spine primary localization, progression during treatment and no surgery predicted worse survival (P<0.01, while patients treated in the last decade had better survival (P = 0.002. In fact, the 5-year OS and PFS for patients diagnosed in the period 2000-2008 were 89% (95% CI 71-96% and 86% (95% CI 66-94%, respectively. CONCLUSION: The axial localization is the most common site of ESFT in pre-scholar children. Patients treated in the most recent period have an excellent outcome.

  13. Assessment of histological response of paediatric bone sarcomas using FDG PET in comparison to morphological volume measurement and standardized MRI parameters

    Energy Technology Data Exchange (ETDEWEB)

    Denecke, Timm; Misch, Daniel; Steffen, Ingo G.; Plotkin, Michail; Stoever, Brigitte [Charite - Universitaetsmedizin Berlin, Klinik fuer Strahlenheilkunde, Campus Virchow-Klinikum, Berlin (Germany); Hundsdoerfer, Patrick; Henze, Guenter [Charite - Universitaetsmedizin Berlin, Klinik fuer Paediatrie m.S. Onkologie und Haematologie, Otto-Heubner-Zentrum, Campus Virchow-Klinikum, Berlin (Germany); Schoenberger, Stefan [Universitaetsklinikum der Heinrich-Heine-Universitaet Duesseldorf, Klinik fuer Kinder-Onkologie, -Haematologie und -Immunologie, Duesseldorf (Germany); Furth, Christian; Ruf, Juri [Otto-von-Guericke-Universitaet Magdeburg, Klinik fuer Radiologie und Nuklearmedizin, Universitaetsklinikum Magdeburg A.oe.R., Magdeburg (Germany); Hautzel, Hubertus [Universitaetsklinikum der Heinrich Heine Universitaet Duesseldorf, Nuklearmedizinische Klinik, Duesseldorf (Germany); Kluge, Regine [Universitaetsklinikum Leipzig A.oe.R., Klinik und Poliklinik fuer Nuklearmedizin, Leipzig (Germany); Bierbach, Uta [Universitaetsklinikum Leipzig A.oe.R., Abteilung fuer Kinder-Haematologie, -Onkologie und -Haemostaseologie, Leipzig (Germany); Otto, Sylke [Universitaetsklinikum Greifswald, Institut fuer Diagnostische Radiologie und Neuroradiologie, Greifswald (Germany); Beck, James F. [Universitaetsklinikum Greifswald, Abteilung fuer Paediatrische Haematologie und Onkologie, Greifswald (Germany); Franzius, Christiane [MR- und PET/CT-Zentrum, Bremen-Mitte (Germany); Universitaetsklinikum Muenster, Klinik und Poliklinik fuer Nuklearmedizin, Muenster (Germany); Amthauer, Holger [Charite - Universitaetsmedizin Berlin, Klinik fuer Strahlenheilkunde, Campus Virchow-Klinikum, Berlin (Germany); Otto-von-Guericke-Universitaet Magdeburg, Klinik fuer Radiologie und Nuklearmedizin, Universitaetsklinikum Magdeburg A.oe.R., Magdeburg (Germany)

    2010-10-15

    The objective of this study was to evaluate positron emission tomography (PET) using {sup 18}F-fluoro-2-deoxy-D-glucose (FDG) in comparison to volumetry and standardized magnetic resonance imaging (MRI) parameters for the assessment of histological response in paediatric bone sarcoma patients. FDG PET and local MRI were performed in 27 paediatric sarcoma patients [Ewing sarcoma family of tumours (EWS), n = 16; osteosarcoma (OS), n = 11] prior to and after neoadjuvant chemotherapy before local tumour resection. Several parameters for assessment of response of the primary tumour to therapy by FDG PET and MRI were evaluated and compared with histopathological regression of the resected tumour as defined by Salzer-Kuntschik. FDG PET significantly discriminated responders from non-responders using the standardized uptake value (SUV) reduction and the absolute post-therapeutic SUV (SUV2) in the entire patient population ({nabla}SUV, p = 0.005; SUV2, p = 0.011) as well as in the subgroup of OS patients ({nabla}SUV, p = 0.009; SUV2, p = 0.028), but not in the EWS subgroup. The volume reduction measured by MRI/CT did not significantly discriminate responders from non-responders either in the entire population (p = 0.170) or in both subgroups (EWS, p = 0.950; OS, p = 1.000). The other MRI parameters alone or in combination were unreliable and did not improve the results. Comparing diagnostic parameters of FDG PET and local MRI, metabolic imaging showed high superiority in the subgroup of OS patients, while similar results were observed in the population of EWS. FDG PET appears to be a useful tool for non-invasive response assessment in the group of OS patients and is superior to MRI. In EWS patients, however, neither FDG PET nor volumetry or standardized MRI criteria enabled a reliable response assessment to be made after neoadjuvant treatment. (orig.)

  14. 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.

  15. Uma nova abordagem para as endopróteses parciais de joelho em sarcomas primários ósseos A new approach to partial knee endoprosthesis in primary bone sarcomas

    Directory of Open Access Journals (Sweden)

    Valter Penna

    2009-02-01

    Full Text Available OBJETIVO: As endopróteses parciais de joelho para as ressecções em sarcomas ósseos demonstram serem boa solução para o tratamento de pacientes com imaturidade esquelética. O objetivo deste estudo é avaliar o escore funcional, as vantagens, as desvantagens e indicações para esta técnica cirúrgica em quatorze pacientes em um protocolo brasileiro de osteossarcoma e sarcoma de Ewing. MÉTODOS: Análise retrospectiva realizada para identificar a evolução funcional e as possíveis complicações do procedimento. 14 pacientes com idade entre 10 e 22 anos avaliados funcionalmente pelos critérios de Enneking/ISOLS (International Society of Limb Salvage, sendo todos operados na mesma Instituição e pelo mesmo cirurgião. Foram utilizadas endopróteses parciais das extremidades distal do fêmur e proximal da tíbia com reconstrução ligamentar. ReSULTADOS: A análise do escore funcional de Enneking/ISOLS demonstrou 78,6 % de excelentes resultados e 21,4% de bons. Dos 14 pacientes, todos portadores de tumores primitivos ósseos em protocolo de quimioterapia, nove não apresentaram nenhum tipo de complicação e cinco indivíduos evoluíram com complicações relacionadas ao procedimento, sendo que houve relação estatística positiva entre os maus resultados e a presença de complicações (p=0,027. CONCLUSÃO: As endopróteses parciais de joelhos são menos prejudiciais ao estoque ósseo de pacientes com esqueleto imaturo. As críticas sobre os maus resultados funcionais estão sendo suplantadas pelas novas técnicas de reconstrução, corretos protocolos de reabilitação, qualidade e tecnologia dos implantes, e o aumento da curva de aprendizado. Essa opção de tratamento per-mite a preservação do estoque ósseo e a possibilidade de revisão da artroplastia não convencional de modo menos agressivo.OBJECTIVE: Partial knee endoprosthesis to bone sarcomas resections seems to be a good solution to treat this immature skeletal patients

  16. A reappraisal of hemangiopericytoma of bone; analysis of cases reclassified as synovial sarcoma and solitary fibrous tumor of bone

    DEFF Research Database (Denmark)

    Verbeke, Sofie L J; Fletcher, Christopher D M; Alberghini, Marco

    2010-01-01

    Hemangiopericytoma (HPC) was first described as a neoplasm with distinct morphologic features, presumably composed of pericytes. In soft tissue, it is accepted that most such lesions are solitary fibrous tumors (SFTs), monophasic synovial sarcomas (SSs), or myofibromatoses. It is unclear whether ...

  17. Uncommon bone tumors of the skull: Ewing's sarcoma and Triton's tumor; Tumores osseos raros da calota craniana: sarcoma de Ewing e tumor de Triton

    Energy Technology Data Exchange (ETDEWEB)

    Rosa, Ana Claudia Ferreira; Carvalho, Claudio Sobral de [Hospital Sirio-Libanes, Sao Paulo, SP (Brazil). Dept. de Radiologia; Machado, Marcio Martins [Sao Paulo Univ., SP (Brazil). Faculdade de Medicina. Dept. de Radiologia; Figueiredo, Marco Antonio Junqueira; Albertotti, Cesar Jose [Hospital Sirio-Libanes, Sao Paulo, SP (Brazil). Servico de Tomografia Computadorizada e Ressonancia Magnetica; Cerri, Giovanni Guido [Hospital Sirio-Libanes, Sao Paulo, SP (Brazil). Centro de Diagnostico]. E-mail: giovanni.cerri@hcnet.usp.br

    2002-04-01

    Ewing's sarcoma and Triton's tumor are two uncommon bone tumors of the skull that have nonspecific clinical and imaging features. However, imaging methods are important in the detection of the lesions during the diagnostic investigation in order to evaluate the extent of the bone lesions, involvement of the soft tissues and brain, and to determine the presence of local recurrence and metastases. The confirmatory diagnosis relies on histological studies and immunohistochemistry. The authors report two cases of patients with these tumors and present the radiological findings. (author)

  18. High-grade endometrial stromal sarcomas: a clinicopathologic study of a group of tumors with heterogenous morphologic and genetic features.

    Science.gov (United States)

    Sciallis, Andrew P; Bedroske, Patrick P; Schoolmeester, John K; Sukov, William R; Keeney, Gary L; Hodge, Jennelle C; Bell, Debra A

    2014-09-01

    The existence of a "high-grade endometrial stromal sarcoma" category of tumors has been a controversial subject owing to, among other things, the difficulty in establishing consistent diagnostic criteria. Currently, the recommended classification for such tumors is undifferentiated uterine/endometrial sarcoma. Interest in this subject has recently increased markedly with the identification of recurrent molecular genetic abnormalities. At Mayo Clinic, a group of neoplasms has been observed that morphologically resemble, either cytologically or architecturally, classic "low-grade" endometrial stromal sarcoma but feature obvious deviations, specifically, 17 tumors with unequivocally high-grade morphology. These high-grade tumors displayed 3 morphologic themes: (1) tumors with a component that is identical to low-grade ESS that transitions abruptly into an obviously higher-grade component; (2) tumors composed exclusively of high-grade cells with uniform nuclear features but with a permeative pattern of infiltration; (3) tumors similar to the second group but with a different, yet characteristic, cytomorphology featuring enlarged round to ovoid cells (larger than those found in low-grade ESS) with smooth nuclear membranes and distinct chromatin clearing but lacking prominent nucleoli. We collected clinicopathologic data, applied immunohistochemical studies, and also tested tumors by fluorescence in situ hybridization for abnormalities in JAZF1, PHF1, YWHAE, and CCND1. Tumors from these 3 groups were found to be immunohistochemically and genetically distinct from one another. Most notable was the fact that category 3 contained all the cases that tested positive for YWHAE rearrangement, did not show any classic translocations for JAZF1, PHF1, or CCND1, often presented at a high stage, and behaved aggressively. This study demonstrates the morphologic, immunophenotypic, and molecular genetic heterogeneity that exists within "undifferentiated endometrial sarcomas" as

  19. Aggressive Ewing's sarcoma appearing as a cold lesion on bone scan; Sarcome d'Ewing agressif apparaissant comme une lesion froide sur la scintigraphie osseuse

    Energy Technology Data Exchange (ETDEWEB)

    Chatti, K.; Guezguez, M.; Maha Ben Fredj, M.; Sfar, R.; Essabbah, H. [Hopital Universitaire de Sahloul, Dept. de Medecine Nucleaire, Sahloul (Tunisia); Mtaoumi, M. [Hopital Universitaire de Sahloul, Dept. d' Orthopedie, Sousse (Tunisia); Chatti, K. [Faculte de Medecine de Monastir, Lab. de Biophysique, Monastir (Tunisia)

    2009-10-15

    Ewing's sarcoma classically presents as a hot spot on bone scan as a result of increased vascularity of the tumor and new bone formation. Purpose We report and analyze an uncommon pattern of a 'cold' lesion in Ewing's sarcoma on bone scan and its pathophysiologic significance. Case report A 15-year-old boy complaining of thigh pain. CT scan evoked Ewing's sarcoma or osteitis. MRI evoked chronic osteitis. Scintigraphy showed a fairly intense and heterogeneous uptake on the femoral lesion and no abnormal uptake elsewhere. Biopsy showed none pathologic pattern. Three months later, a second biopsy concluded to Ewing's sarcoma. Bone scan showed a larger lesion with peripheral intense uptake centered by enlarged 'cold' area in the left femoral diaphysis and no evident bone metastasis. The patient underwent chemotherapy and surgery. Three months later, bone scan showed extensive skeletal metastasis. Conclusion Ewing's sarcoma appears usually as an intense lesion on bone scan. Nevertheless, decreased radiopharmaceutical uptake or 'cold' lesion may be seen in aggressive Ewing's sarcoma with lytic tumor, growth of which is very rapid and bony reaction is minimal. (authors)

  20. 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

  1. Limb salvage with microvascular free fibula following primary bone sarcoma resection

    Directory of Open Access Journals (Sweden)

    Sahasrabudhe Parag

    2016-01-01

    Full Text Available Background: Extremity sarcomas are challenging to manage. Total eradication of tumour has to be balanced with restoration of limb function to prevent mortality and morbidity. Disease-free survival with maximum limb function is the ultimate goal in these patients. Materials and Methods: We present a series of ten cases of extremity malignancies, where limb salvage was attempted with microvascular free fibula for limb reconstruction from the period of 2008 to 2015. Results: Of the ten cases in the study, there were two females and eight males. There were nine patients with lower limb malignancies and one patient with upper limb malignancy. There were four patients with Ewing's sarcoma of femur, five patients with osteosarcoma of femur and one patient with chondrosarcoma of the humerus. The follow-up period ranged from 1.2 to 6.2 years with mean follow-up of 3.1 years. There were two deaths during follow-up, both were due to distant metastasis. The assessment of the function was done on the basis of Musculoskeletal Tumour Society functional score. Maximum score was 30 and minimum score was 24, the average score being 26. Of the eight surviving patients, three patients had full weightbearing, four patients had partial weightbearing at end of 2 years and one patient of upper limb reconstruction had complete upper limb function. None of the patients had to undergo limb amputation. Conclusion: Limb salvage with vascularised fibula graft offers good functional outcome along with good disease-free survival rates.

  2. 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.

  3. Utility of opposed-phase magnetic resonance imaging in differentiating sarcoma from benign bone lesions

    Directory of Open Access Journals (Sweden)

    Barry E. Kenneally

    2015-12-01

    Conclusion: Opposed-phase imaging is helpful in differentiating benign from malignant lesions in bone. Confidence in diagnosis rose for both the attending and the resident as result of the inclusion of OP sequences.

  4. Two Cases of Sarcoma Arising in Giant Cell Tumor of Bone Treated with Denosumab

    Directory of Open Access Journals (Sweden)

    Cory Julian Broehm

    2015-01-01

    Full Text Available Giant cell tumor (GCT of bone is a generally benign, but often locally aggressive, neoplasm of bone, with a propensity for recurrence. Sarcomatous transformation is rare and typically occurs with a history of recurrences and radiation treatment. Denosumab, an inhibitor of the RANK ligand involved in bone resorption in GCT, is increasingly used in treatment of recurrent or unresectable giant cell tumor of bone. We report two cases of sarcomatous transformation of GCT to osteosarcoma in patients receiving denosumab. One was a 59-year-old male with a 12-year history of GCT and multiple recurrences taking denosumab for 2.5 years. The second case was in a 56-year-old male with a seven-year history of GCT taking denosumab for six months. Review of the literature shows one case report of malignant transformation of GCT in a patient being treated with denosumab. As the use of denosumab for treatment of GCT will likely increase, larger, controlled studies are needed to ascertain whether denosumab may play a role in malignant transformation of giant cell tumor of bone.

  5. 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.

  6. 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

  7. 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

  8. Carbon Ion Radiation Therapy Improves the Prognosis of Unresectable Adult Bone and Soft-Tissue Sarcoma of the Head and Neck

    Energy Technology Data Exchange (ETDEWEB)

    Jingu, Keiichi [Research Center for Charged Particle Therapy, National Institute of Radiological Sciences (NIRS), Chiba (Japan); Department of Radiation Oncology, Tohoku University School of Medicine, Sendai (Japan); Tsujii, Hirohiko, E-mail: tsujii@nirs.go.jp [Research Center for Charged Particle Therapy, National Institute of Radiological Sciences (NIRS), Chiba (Japan); Mizoe, Jun-Etsu; Hasegawa, Azusa; Bessho, Hiroki; Takagi, Ryo; Morikawa, Takamichi [Research Center for Charged Particle Therapy, National Institute of Radiological Sciences (NIRS), Chiba (Japan); Tonogi, Morio [Department of Oral Medicine, Tokyo Dental College, Ichihara (Japan); Tsuji, Hiroshi; Kamada, Tadashi [Research Center for Charged Particle Therapy, National Institute of Radiological Sciences (NIRS), Chiba (Japan); Yamada, Shogo [Department of Radiation Oncology, Tohoku University School of Medicine, Sendai (Japan)

    2012-04-01

    Purpose: To evaluate the safety and efficacy of carbon ion radiotherapy (C-ion RT) with 70.4 GyE for unresectable bone and soft-tissue sarcoma of the adult head and neck. Methods and Materials: Twenty-seven patients (mean age, 46.2 years) were enrolled in this prospective study on C-ion RT with 70.4 GyE/16 fractions (fr) between April 2001 and February 2008. The primary end points were acute and late reactions of normal tissues, local control rate, and overall survival rate. The secondary end point was efficacy of the treatment in comparison to historical results with 57.6 or 64.0 GyE/16 fr. Results: The 3-year local control rate and overall survival rate for all patients were 91.8% (95% confidence interval [CI] = 81.0-100%) and 74.1% (95% CI = 57.5-90.6%), respectively. Acute reaction of Grade 3 or more was observed in only 1 patient. With regard to late reactions, visual loss was observed in 1 patient and a Grade 3 reaction of the maxillary bone was observed in 4 patients. A comparison with historical results revealed that the local control rate with 70.4 GyE was significantly higher than that with 57.6 or 64.0 GyE (3-year, 91.8% vs. 23.6%, p < 0.0001). Furthermore, the overall survival with 70.4 GyE tended to be higher than that with 57.6 or 64.0 GyE (3-year, 74.1% vs. 42.9%, p = 0.09). Conclusion: C-ion RT with 70.4 GyE/16 fr for bone and soft-tissue sarcoma of the adult head and neck appears to be effective with acceptable toxicities in comparison to conventional RT and C-ion RT with lower doses.

  9. 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.

  10. 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.

  11. 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....

  12. Granulocytic Sarcoma by AML M4eo (inv16 after Allogeneic Stem Cell Transplantation without Bone Marrow Involvement

    Directory of Open Access Journals (Sweden)

    Stephan Zaenker

    2011-01-01

    Full Text Available Granulocytic sarcoma (GS represents a rare type of extramedullar manifestation from the acute myeloid leukaemia (AML. We report the case of a patient with recurrences of AML M4eo leukaemia in the uterus and the small intestine at 3 and 5 years, respectively, after matched related peripheral blood stem cell transplantation (PBSCT. The patient underwent the withdrawal of immunosuppression, hysterectomy, and local irradiation at first relapse, as well as systemic chemotherapy and donor lymphocyte infusions at second recurrence, inducing a second and third complete remission, respectively. At year six after transplantation, the patient experienced disease progression by meningeosis leukaemia to which she succumbed despite intrathecal chemotherapy. Following allogeneic stem cell transplantation, awareness for atypical manifestations of granulocytic sarcoma appears prudent, the cellular immunotherapy should aim at immunological disease control.

  13. 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

  14. 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 ...

  15. 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

  16. Ewing's sarcoma, fibrogenic tumors, giant cell tumor, hemangioma of bone. Radiology and pathology; Ewing-Sarkom, fibrogene Tumoren, Riesenzelltumor, Haemangiom des Skeletts. Radiologie und Pathologie

    Energy Technology Data Exchange (ETDEWEB)

    Freyschmidt, J. [Beratungsstelle und Referenzzentrum fuer Osteoradiologie, Bremen (Germany); Ostertag, H. [Klinikum Region Hannover GmbH, Pathologisches Institut, Hannover (Germany)

    2016-06-15

    Radiological imaging only reflects the anatomy and its pathological abnormalities. Therefore, the radiologist should be able to recognize the basic features of the pathological anatomy of bone tumors. This can only be learned working closely with a pathologist who is experienced in this field. On the other hand, the pathologist needs from the radiologist their diagnostic assessment with information on size, location, aggressiveness and the existence of a bone tumor's matrix, of the whole lesion, because he usually only receives a small part for examination in the form of a biopsy. In this article, the features and fundamentals (standards) of radiological-pathological cooperation as the mainstay for a precise diagnosis in bone tumors are outlined. The radiological appearance and the histopathological features behind it are presented for Ewing's sarcoma, fibrogenic tumors, giant cell tumor, and hemangioma of the bone. (orig.) [German] Radiologische Bilder spiegeln nichts anderes als die Anatomie und ihre pathologischen Abweichungen wider. Deshalb sollte der Radiologe die Grundzuege der pathologischen Anatomie auch von Knochentumoren kennen. Das kann er nur durch eine enge Zusammenarbeit mit einem auf diesem Gebiet erfahrenen Pathologen erlernen. Andererseits braucht der Pathologe vom Radiologen dessen diagnostische Einschaetzung mit Informationen ueber die Groesse, Lage, Aggressivitaet und das Vorhandensein einer Matrix eines Knochentumors und zwar von der gesamten Laesion, denn er bekommt inform einer Biopsie i. d. R. nur einen mehr oder weniger kleinen Teil zur Untersuchung. In diesem Beitrag werden die Grundzuege und Standards der radiologisch-pathologischen Zusammenarbeit aufgezeigt, auf denen eine praezise Diagnosestellung beruht. Radiologisches Erscheinungsbild und die dahintersteckenden - und erklaerenden - histopathologischen Merkmale werden fuer das Ewing-Sarkom, fuer fibrogene Tumoren, den Riesenzelltumor und das Haemangiom des Knochens

  17. Metastatic Bone Disease

    Science.gov (United States)

    ... begin in bone are much less common in adults older than 45 years. Other diseases, such as Paget’s sarcoma, post-radiation sarcoma, hyperparathyroidism, and fractures due to osteoporosis, are also possibilities. Additional tests will likely be ...

  18. Recurrence of a t(8;21-Positive Acute Myeloid Leukemia in the Form of a Granulocytic Sarcoma Involving Cranial Bones: A Diagnostic and Therapeutic Challenge

    Directory of Open Access Journals (Sweden)

    Ambra Di Veroli

    2013-01-01

    Full Text Available Granulocytic sarcoma (GS is a rare extramedullary solid tumor defined as an accumulation of myeloblasts or immature myeloid cells. It can cooccur with or precede the acute myeloid leukemia (AML as well as following treated AML. The incidence of GS in AML patients is 3–8% but it significantly rises in M2 FAB subtype AML. This variety of AML harbors t(8;21 in up to 20–25% of cases (especially in children and black ones of African origin and, at a molecular level, it is characterized by the generation of a fusion gene known as RUNX1-RUNX1T1. Approximately 10% of M2 AML patients will develop GS, as a consequence, the t(8;21 and the relative transcript represent the most common cytogenetic and molecular abnormalities in GS. FLT3-ITD mutation was rarely described in AML patients presenting with GS. FLT3 ITD is generally strongly associated with poor prognosis in AML, and is rarely reported in patients with t(8;21. GS presentation is extremely variable depending on organs involved; in general, cranial bones and sinus are very rarely affected sites. We report a rare case of GS occurring as a recurrence of a previously treated t(8;21, FLT3-ITD positive AML, involving mastoid bones and paravertebral tissues.

  19. MR criteria for differentiation of 'pseudo tumourous' lesions from bone sarcomas of the extremities. MRT-Kriterien zur Differenzierung 'pseudotumoroeser' Laesionen von Knochensarkomen der Extremitaeten

    Energy Technology Data Exchange (ETDEWEB)

    Lehner, K. (Technische Univ. Muenchen, Klinikum rechts der Isar, Inst. fuer Roentgendiagnostik (Germany)); Rechl, H. (Technische Univ. Muenchen, Klinikum rechts der Isar, Orthopaedische Klinik (Germany)); Daschner, H. (Technische Univ. Muenchen, Klinikum rechts der Isar, Inst. fuer Roentgendiagnostik (Germany)); Kutschker, C. (Technische Univ. Muenchen, Klinikum rechts der Isar, Inst. fuer Roentgendiagnostik (Germany))

    1993-05-01

    The MRT scans of 57 patients with Ewing or osteosarcomas and 34 patients with haematogenous osteomyelitis or periostitis/stress fractures were examined in order to determine whether a distinction between benign or malignant lesions is possible. Four criteria were evaluated: The margin of the bone marrow component; intensity and homogeneity of the T[sub 1]-weighted signal in the bone marrow; presence of an extraosseus structured soft tissue mass and/or soft tissue edema. It was found that central osteosarcomas and Ewing's sarcomas reduced signal intensity of the marrow to become muscle-isointense with a well defined margin. In acute haematogenous osteomyelitis and periostitis/stress fracture the marrow lesion was not sharply demarcated. In contrast to patients with bone sarcomas only one case of osteomyelitis showed an extrosseus structured soft tissue mass. On the basis of these findings we believe that acute haematogenous osteomyelitis can be distinguished with high degree of accuracy from Ewing's sarcoma and central osteosarcomas. (orig.)

  20. 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.

  1. Managing a Bone Healthy Lifestyle After Attending Multifaceted Group Education

    DEFF Research Database (Denmark)

    Jensen, Annesofie Lunde; Lomborg, Kirsten; Langdahl, Bente Lomholt

    2016-01-01

    We examined patients with osteoporosis implementation of recommendations regarding a bone healthy lifestyle after the patients attended multifaceted osteoporosis group education (GE). Our findings suggest that GE can support and influence patients’ transfer of preventive actions. Still patients...... diagnosed with osteoporosis who attended multifaceted GE at a Danish hospital participated. Data consisted of field work and individual interviews in the participants’ everyday environment after completion of GE. After attending multifaceted GE, participants experienced increased attention to and reflected....... On the contrary, attending GE was in some cases not sufficient to overcome social and physical concerns, or to eliminate uncertainty about recommendations or to make participants identify with the osteoporosis diagnosis, which thus impeded implementation of a bone healthy lifestyle. Attending multifaceted GE can...

  2. 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.

  3. Pediatric mast cell sarcoma of temporal bone with novel L799F (2395 C>T) KIT mutation, mimicking histiocytic neoplasm.

    Science.gov (United States)

    Kim, Young S; Wu, Huiqing; Pawlowska, Anna B; Bautista-Quach, Marnelli A; Huang, Qin; Gaal, Karl; Chang, Karen L

    2013-03-01

    Mast cell sarcoma (MCS) is an extremely rare neoplasm with a clinically aggressive course. Because of its rarity, its morphologic and molecular characteristics are still not well defined. We report a case of a 15-year-old girl with MCS of the temporal bone extending into the posterior fossa creating a mass effect. The lesion mimicked a histiocytic neoplasm morphologically, but showed a novel KIT missense mutation, L799F (2395 C>T). The KIT D816V mutation is frequently found in systemic mastocytosis, but it has not been documented in the few reported human MCS cases. However, 1 reported case of MCS has shown a different alteration in the KIT gene. Our case is the first MCS case with L799F mutation, located between the catalytic loop (790 to 797) and the activation loop (810 to 837) of the KIT gene, and only the second case of MCS with KIT mutation documented in the literature. Proximity of the L799F mutation to the enzymatic region of the KIT tyrosine kinase domain may induce resistance to tyrosine kinase inhibitors.

  4. Administration of a tropomyosin receptor kinase inhibitor attenuates sarcoma-induced nerve sprouting, neuroma formation and bone cancer pain

    Directory of Open Access Journals (Sweden)

    Bloom Aaron P

    2010-12-01

    Full Text Available Abstract Pain often accompanies cancer and most current therapies for treating cancer pain have significant unwanted side effects. Targeting nerve growth factor (NGF or its cognate receptor tropomyosin receptor kinase A (TrkA has become an attractive target for attenuating chronic pain. In the present report, we use a mouse model of bone cancer pain and examine whether oral administration of a selective small molecule Trk inhibitor (ARRY-470, which blocks TrkA, TrkB and TrkC kinase activity at low nm concentrations has a significant effect on cancer-induced pain behaviors, tumor-induced remodeling of sensory nerve fibers, tumor growth and tumor-induced bone remodeling. Early/sustained (initiated day 6 post cancer cell injection, but not late/acute (initiated day 18 post cancer cell injection administration of ARRY-470 markedly attenuated bone cancer pain and significantly blocked the ectopic sprouting of sensory nerve fibers and the formation of neuroma-like structures in the tumor bearing bone, but did not have a significant effect on tumor growth or bone remodeling. These data suggest that, like therapies that target the cancer itself, the earlier that the blockade of TrkA occurs, the more effective the control of cancer pain and the tumor-induced remodeling of sensory nerve fibers. Developing targeted therapies that relieve cancer pain without the side effects of current analgesics has the potential to significantly improve the quality of life and functional status of cancer patients.

  5. 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.

  6. 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...

  7. 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.

  8. Bone Mineral Density Evaluation in Four Different Occupational Groups

    Directory of Open Access Journals (Sweden)

    Mustafa Turgut Yıldızgören

    2015-04-01

    Full Text Available Objective: The aim of this study was to evaluate bone mineral density (BMD and to discuss the potential risk factors for osteoporosis in four different occupational groups. Materials and Methods: In this study, 100 males who were admitted to our clinics for their periodic occupational controls and 40 healthy subjects were included. Demographic features of the participants were recorded. BMD was evaluated by Dual-energy x-ray absorptiometry (DXA from lumbar vertebrae and proximal femur. Results: Participants were mainly from the following four occupational groups; accumulator manufacturers (n=30, 21.4%, painting workers (n=30, 21.4%, welders (n=15, 10.7%, and quartz miners (n=25, 17.9% sectors. In addition, there were 40 healthy subjects (28.6%. Compared with the control group, femoral neck T-scores (p=0.023 and Z-scores (p=0.031 were significantly lower in miners. L2-L4 BMD values were significantly lower in accumulator manufacturers (p=0.041 and quartz miners (p=0.022 as compared with the control group. Conclusion: Workers in the accumulator and mining sectors had lower BMD than control subjects. Clinicians should keep in mind occupational risk factors related with chemical exposure for osteoporosis while questioning osteoporosis risk factors. (Turkish Journal of Osteoporosis 2015;21: 19-22

  9. Primary granulocytic sarcoma of the ovary.

    Science.gov (United States)

    Sreejith, G; Gangadharan, V P; Elizabath, K A; Preetha, S; Chithrathara, K

    2000-06-01

    Granulocytic sarcomas are rare extramedullary tumors of malignant myeloid precursor cells. Exceedingly rare in childhood, it commonly involves skin, lymph nodes, bone, and the spine. Ovarian involvement is rare. It can arise de novo, precede the development of acute nonlymphocytic leukemia, or be the sole manifestation of relapse. We describe a 26-year-old woman with granulocytic sarcoma of the ovary without any hematologic disorder.

  10. 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

  11. Primary intracranial soft tissue sarcoma in children and adolescents: a cooperative analysis of the European CWS and HIT study groups.

    Science.gov (United States)

    Benesch, Martin; von Bueren, André O; Dantonello, Tobias; von Hoff, Katja; Pietsch, Torsten; Leuschner, Ivo; Claviez, Alexander; Bierbach, Uta; Kropshofer, Gabriele; Korinthenberg, Rudolf; Graf, Norbert; Suttorp, Meinolf; Kortmann, Rolf Dieter; Friedrich, Carsten; von der Weid, Nicolas; Kaatsch, Peter; Klingebiel, Thomas; Koscielniak, Ewa; Rutkowski, Stefan

    2013-02-01

    Purely intracranial soft tissue sarcomas (ISTS) are very rare among children. A retrospective database analysis of the Cooperative Weichteilsarkom Studiengruppe (CWS) and brain tumor (HIT) registries was conducted to describe treatment and long-term outcome of children and adolescents with ISTS. Nineteen patients from Germany, Austria and Switzerland were reported between 1988 and 2009. Median age at diagnosis was 9.7 years (range, 0.5-17.8). Central pathological review was performed in 17 patients. Eleven patients underwent a total and five a subtotal tumor resection. A biopsy was done in one patient. In two patients no data concerning extent of initial resection was available. Radiotherapy was performed in 15 patients (first-line, n = 11; following progression, n = 4). All but one patient received chemotherapy (first-line, n = 7, following progression, n = 5; first-line and following progression, n = 6). With a median follow-up of 5.8 years (range, 0.6-19.8) ten patients were alive in either first or second complete remission. Seven patients died due to relapse or progression and two were alive with progressive disease. Estimated progression-free and overall survival at 5 years were 47 % (±12 %) and 74 % (±10 %), respectively. About 50 % of patients with ISTS remain relapse-free after 5 years. Multimodality treatment including complete tumor resection and radio-/chemotherapy is required to achieve sustained tumor control in patients with ISTS. Early initiation of postoperative non-surgical treatment seems to be important to prevent recurrence. Due to the intracranial localization local therapy should follow the recommendations used in brain tumors rather than in soft tissue sarcomas, whereas chemotherapy should be guided by histological subtype.

  12. The value of FDG-PET in the detection, grading and response to therapy of soft tissue and bone sarcomas; a systematic review and meta-analysis

    NARCIS (Netherlands)

    Bastiaannet, E; Groen, H; Jager, PL; Cobben, DCP; van der Graaf, WTA; Vaalburg, W; Hoekstra, HJ

    2004-01-01

    Background: Sarcomas represent a significant diagnostic and therapeutic challenge that requires techniques to provide better assessment of the disease than provided by traditional means. FDG-PET depicts the increased metabolism in abnormal tissues, enabling visualisation and quantification in vivo.

  13. In vitro expanded bone marrow-derived murine (C57Bl/KaLwRij) mesenchymal stem cells can acquire CD34 expression and induce sarcoma formation in vivo

    Energy Technology Data Exchange (ETDEWEB)

    Xu, Song [Department of Lung Cancer Surgery, Lung Cancer Institute, Tianjin Medical University General Hospital, 300052 Tianjin (China); Stem Cell Laboratory-Division Clinical Hematology, Universitair Ziekenhuis Brussel (UZ Brussel), Laarbeeklaan 101, 1090 Brussels (Belgium); Department of Hematology and Immunology, Vrije Universiteit Brussel (VUB)-Myeloma Center, Laarbeeklaan 103, 1090 Brussels (Belgium); De Becker, Ann [Stem Cell Laboratory-Division Clinical Hematology, Universitair Ziekenhuis Brussel (UZ Brussel), Laarbeeklaan 101, 1090 Brussels (Belgium); De Raeve, Hendrik [Department of Anatomopathology, Universitair Ziekenhuis Brussel (UZ Brussel), Laarbeeklaan 101, 1090 Brussels (Belgium); Van Camp, Ben; Vanderkerken, Karin [Department of Hematology and Immunology, Vrije Universiteit Brussel (VUB)-Myeloma Center, Laarbeeklaan 103, 1090 Brussels (Belgium); Van Riet, Ivan, E-mail: ivan.vanriet@uzbrussel.be [Stem Cell Laboratory-Division Clinical Hematology, Universitair Ziekenhuis Brussel (UZ Brussel), Laarbeeklaan 101, 1090 Brussels (Belgium); Department of Hematology and Immunology, Vrije Universiteit Brussel (VUB)-Myeloma Center, Laarbeeklaan 103, 1090 Brussels (Belgium)

    2012-08-03

    Highlights: Black-Right-Pointing-Pointer Murine MSCs can undergo spontaneously malignant transformation and form sarcoma. Black-Right-Pointing-Pointer Acquisition of CD34 is a transformation type for MSCs into sarcoma. Black-Right-Pointing-Pointer Notch/Hh/Wnt pathways are related to the malignant phenotype of transformed MSCs. -- Abstract: Mesenchymal stem cells (MSCs) have currently generated numerous interests in pre-clinical and clinical applications due to their multiple lineages differentiation potential and immunomodulary effects. However, accumulating evidence indicates that MSCs, especially murine MSCs (mMSCs), can undergo spontaneous transformation after long-term in vitro culturing, which might reduce the therapeutic application possibilities of these stem cells. In the present study, we observed that in vitro expanded bone marrow (BM) derived mMSCs from the C57Bl/KaLwRij mouse strain can lose their specific stem cells markers (CD90 and CD105) and acquire CD34 expression, accompanied with an altered morphology and an impaired tri-lineages differentiation capacity. Compared to normal mMSCs, these transformed mMSCs exhibited an increased proliferation rate, an enhanced colony formation and migration ability as well as a higher sensitivity to anti-tumor drugs. Transformed mMSCs were highly tumorigenic in vivo, resulting in aggressive sarcoma formation when transplanted in non-immunocompromised mice. Furthermore, we found that Notch signaling downstream genes (hey1, hey2 and heyL) were significantly upregulated in transformed mMSCs, while Hedgehog signaling downstream genes Gli1 and Ptch1 and the Wnt signaling downstream gene beta-catenin were all decreased. Taken together, we observed that murine in vitro expanded BM-MSCs can transform into CD34 expressing cells that induce sarcoma formation in vivo. We assume that dysregulation of the Notch(+)/Hh(-)/Wnt(-) signaling pathway is associated with the malignant phenotype of the transformed mMSCs.

  14. 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

  15. 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 ...

  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. Soft tissue sarcoma with metastasis to the stomach: A case report

    Institute of Scientific and Technical Information of China (English)

    Lemuel; Leon; Dent; Cesar; Yamil; Cardona; Michael; Clause; Buchholz; Roosevelt; Peebles; Julie; Denise; Scott; Derrick; Jerome; Beech; Billy; Ray; Ballard

    2010-01-01

    Soft tissue sarcomas are unusual malignancies comprising 1% of cancer diagnoses in the United States. Undifferentiated pleomorphic sarcoma accounts for approximately 5% of sarcomas occurring in adults. The most common site of metastasis is the lung, with other sites being bone, the brain, and the liver. Metastasis to the gastrointestinal tract has rarely been documented. We present an unusual case of high-grade pleomorphic sarcoma with metastasis to the stomach, complicated by upper gastrointestinal bleedin...

  18. 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.

  19. 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.

  20. 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 ...

  1. 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.

  2. Treatment of undifferentiated high grade pleomorphic sarcoma of bone%骨未分化高级别多形性肉瘤的治疗探讨

    Institute of Scientific and Technical Information of China (English)

    吴志圣; 李菊明; 韦永中

    2015-01-01

    Objective To investigate the clinical characteristic, treatment and prognosis of undifferentiated high grade pleomorphic sarcoma of bone( BUPS) . Methods Seven cases of BUPS were analyzed retrospectively, including sites of tumor, image findings, pathology, surgical staging of tumor, treatment and results of follow-up, and the related lit-erature was reviewed. The preoperative traditional X-ray, CT, MRI, ECT and PET/CT were made. All cases were performed operation when BUPS was confirmed by puncturing biopsy or open biopsy. Results Tumors involved in distal femur in 2 cases, proximal femur in 3 cases, the proximal tibia and ilium each in 1 case. Radiologic findings of BUPS were nonspecific, mainly with a patchy or motheaten pattern of bone destruction. There were 2 cases in stage ofⅡA, 4 ⅡB and 1 Ⅲ. Hip disarticulation was performed in 3 cases, limb salvage was performed in 4 cases, and the chemotherapy was performed in all cases. The time of follow-up was 12~102 (45 ± 30) months, 3 cases developed distant metastasis and all of them with pathologic fracture, 2 cases died of lung metastasis two years after operation, the rest 4 cases were disease-free survival. Conclusions Surgical treatment is the primary method for BUPS and standard chemotherapy can improve survival rate. Pathologic fracture is associated with a poor prognosis.%目的:探讨骨未分化高级别多形性肉瘤( BUPS)的临床特征、治疗方法及预后。方法回顾分析7例BUPS患者的发病部位、影像学资料、病理、肿瘤的外科分期、治疗方法及随访结果等资料,并复习相关文献。患者术前行X线、CT、MRI、ECT或PET/CT检查,影像学表现缺乏特异性,主要以虫噬状或斑片状溶骨性骨破坏为主。穿刺活检或切开活检确诊为BUPS后均行手术治疗。结果病变累及股骨远端2例,股骨近端3例,胫骨近端及髂骨各1例,其中3例合并有病理性骨折。 Enneking分期:ⅡA期2例,ⅡB期4例,Ⅲ期1例。3

  3. Granulocytic Sarcoma of the Stomach Presenting as Dysphagia during Pregnancy

    Directory of Open Access Journals (Sweden)

    Anuradha Sekaran

    2011-01-01

    Full Text Available Granulocytic sarcoma also known as extramedullary myeloid sarcoma or chloroma is an uncommon manifestation of leukemia and presents as a deposit of leukemic cells outside the bone marrow. We report a case of a twenty-five-year-old pregnant woman who presented with progressive dysphagia and recurrent postprandial vomiting. Upper GI endoscopy had shown large flat laterally spread nodular lesions in the cardia and proximal body of stomach. Biopsies from the gastric lesion showed granulocytic sarcoma of the stomach. Concurrent peripheral and bone marrow picture was suggestive of acute myeloid leukemia (AML–M4. There is limited reported literature on granulocytic sarcoma of the stomach. Concurrent gastric granulocytic sarcoma involving cardia and AML in pregnancy has not been reported till date.

  4. 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.

  5. 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.

  6. 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.

  7. Primary Malignant Tumours of Bone Following Previous Malignancy

    Science.gov (United States)

    Patton, J. T.; Sommerville, S. M. M.; Grimer, R. J.

    2008-01-01

    Destructive bone lesions occurring in patients who have previously had a malignancy are generally assumed to be a metastasis from that malignancy. We reviewed 60 patients with a previous history of malignancy, who presented with a solitary bone lesion that was subsequently found to be a new and different primary sarcoma of bone. These second malignancies occurred in three distinct groups of patients: (1) patients with original tumours well known to be associated with second malignancies (5%); (2) patients whose second malignancies were likely to be due to the previous treatment of their primary malignancy (40%); (3) patients in whom there was no clearly defined association between malignancies (55%). The purpose of this study is to emphasise the necessity for caution in assuming the diagnosis of a metastasis when a solitary bone lesion is identified following a prior malignancy. Inappropriate biopsy and treatment of primary bone sarcomas compromises limb salvage surgery and can affect patient mortality. PMID:18414590

  8. Primary Malignant Tumours of Bone Following Previous Malignancy

    Directory of Open Access Journals (Sweden)

    R. J. Grimer

    2008-04-01

    Full Text Available Destructive bone lesions occurring in patients who have previously had a malignancy are generally assumed to be a metastasis from that malignancy. We reviewed 60 patients with a previous history of malignancy, who presented with a solitary bone lesion that was subsequently found to be a new and different primary sarcoma of bone. These second malignancies occurred in three distinct groups of patients: (1 patients with original tumours well known to be associated with second malignancies (5%; (2 patients whose second malignancies were likely to be due to the previous treatment of their primary malignancy (40%; (3 patients in whom there was no clearly defined association between malignancies (55%. The purpose of this study is to emphasise the necessity for caution in assuming the diagnosis of a metastasis when a solitary bone lesion is identified following a prior malignancy. Inappropriate biopsy and treatment of primary bone sarcomas compromises limb salvage surgery and can affect patient mortality.

  9. 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.

  10. The Challenges of Detecting Circulating Tumor Cells in Sarcoma

    Science.gov (United States)

    Tellez-Gabriel, Marta; Brown, Hannah K.; Young, Robin; Heymann, Marie-Françoise; Heymann, Dominique

    2016-01-01

    Sarcomas are a heterogeneous group of malignant neoplasms of mesenchymal origin, many of which have a propensity to develop distant metastases. Cancer cells that have escaped from the primary tumor are able to invade into surrounding tissues, to intravasate into the bloodstream to become circulating tumor cells (CTCs), and are responsible for the generation of distant metastases. Due to the rarity of these tumors and the absence of specific markers expressed by sarcoma tumor cells, the characterization of sarcoma CTCs has to date been relatively limited. Current techniques for isolating sarcoma CTCs are based on size criteria, the identification of circulating cells that express either common mesenchymal markers, sarcoma-specific markers, such as CD99, CD81, or PAX3, and chromosomal translocations found in certain sarcoma subtypes, such as EWS-FLI1 in Ewing’s sarcoma, detection of osteoblast-related genes, or measurement of the activity of specific metabolic enzymes. Further studies are needed to improve the isolation and characterization of sarcoma CTCs, to demonstrate their clinical significance as predictive and/or prognostic biomarkers, and to utilize CTCs as a tool for investigating the metastatic process in sarcoma and to identify novel therapeutic targets. The present review provides a short overview of the most recent literature on CTCs in sarcoma. PMID:27656422

  11. 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...

  12. Uncommon sarcomas of the uterine cervix: a review of selected entities

    Directory of Open Access Journals (Sweden)

    Fadare Oluwole

    2006-09-01

    Full Text Available Abstract Sarcomas constitute less than 1% of all cervical malignancies. With over 150 reported cases, rhabdomyosarcomas represent the most commonly reported sarcoma at this location. In this report, a select group of the more uncommon sarcomas of the uterine cervix are reviewed, including all previously reported examples of leiomyosarcoma, liposarcoma, alveolar soft part sarcoma, Ewing sarcoma/primitive neuroectodermal tumor, undifferentiated endocervical sarcoma, and malignant peripheral nerve sheath tumor (MPNST. Emphasis is placed on any distinctive clinicopathologic features of these entities at this unusual location.

  13. Sarcoma derived from cultured mesenchymal stem cells.

    Science.gov (United States)

    Tolar, Jakub; Nauta, Alma J; Osborn, Mark J; Panoskaltsis Mortari, Angela; McElmurry, Ron T; Bell, Scott; Xia, Lily; Zhou, Ning; Riddle, Megan; Schroeder, Tania M; Westendorf, Jennifer J; McIvor, R Scott; Hogendoorn, Pancras C W; Szuhai, Karoly; Oseth, Leann; Hirsch, Betsy; Yant, Stephen R; Kay, Mark A; Peister, Alexandra; Prockop, Darwin J; Fibbe, Willem E; Blazar, Bruce R

    2007-02-01

    To study the biodistribution of MSCs, we labeled adult murine C57BL/6 MSCs with firefly luciferase and DsRed2 fluorescent protein using nonviral Sleeping Beauty transposons and coinfused labeled MSCs with bone marrow into irradiated allogeneic recipients. Using in vivo whole-body imaging, luciferase signals were shown to be increased between weeks 3 and 12. Unexpectedly, some mice with the highest luciferase signals died and all surviving mice developed foci of sarcoma in their lungs. Two mice also developed sarcomas in their extremities. Common cytogenetic abnormalities were identified in tumor cells isolated from different animals. Original MSC cultures not labeled with transposons, as well as independently isolated cultured MSCs, were found to be cytogenetically abnormal. Moreover, primary MSCs derived from the bone marrow of both BALB/c and C57BL/6 mice showed cytogenetic aberrations after several passages in vitro, showing that transformation was not a strain-specific nor rare event. Clonal evolution was observed in vivo, suggesting that the critical transformation event(s) occurred before infusion. Mapping of the transposition insertion sites did not identify an obvious transposon-related genetic abnormality, and p53 was not overexpressed. Infusion of MSC-derived sarcoma cells resulted in malignant lesions in secondary recipients. This new sarcoma cell line, S1, is unique in having a cytogenetic profile similar to human sarcoma and contains bioluminescent and fluorescent genes, making it useful for investigations of cellular biodistribution and tumor response to therapy in vivo. More importantly, our study indicates that sarcoma can evolve from MSC cultures.

  14. Adult Intramedullary Ewing Sarcoma of the Proximal Hip

    Directory of Open Access Journals (Sweden)

    Preetam Gongidi

    2014-01-01

    Full Text Available Ewing sarcoma of bone is classically a permeative lesion in the diaphysis of long bones in children. While they occur primarily in children and adolescents, they can be seen in young adults in their 20s, but these are typically seen in flat bones. The permeative nature of the lesion can elicit new bone formation creating a partially sclerotic appearance, cortical expansion presenting as a “Codman triangle,” or have an “onion-skin” type of aggressive periosteal reaction/periostitis. Ewing sarcoma is rarely seen without an associated soft-tissue mass and is even rarer to just have benign-appearing periostitis (e.g., thick, uniform, or wavy cortex. We present such a case of Ewing sarcoma in a young adult confined to just the medullary metadiaphysis without cortical erosion or soft-tissue mass. To the best of our knowledge, this is the first case to be reported in the radiology literature.

  15. Ancient Human Bone Microstructure in Medieval England: Comparisons between Two Socio-Economic Groups

    OpenAIRE

    Miszkiewicz, Justyna J.; Mahoney, Patrick

    2016-01-01

    Understanding the links between bone microstructure and human lifestyle is critical for clinical and anthropological research into skeletal\\ud growth and adaptation. The present study is the first to report correspondence between socio-economic status and variation in bone microstructure\\ud in ancient humans. Products of femoral cortical remodeling were assessed using histological methods in a large human medieval\\ud sample (N:450) which represented two distinct socio-economic groups. Osteona...

  16. 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.)

  17. 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.

  18. 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.

  19. Synchrotron-based XRD from rat bone of different age groups.

    Science.gov (United States)

    Rao, D V; Gigante, G E; Cesareo, R; Brunetti, A; Schiavon, N; Akatsuka, T; Yuasa, T; Takeda, T

    2017-05-01

    Synchrotron-based XRD spectra from rat bone of different age groups (w, 56 w and 78w), lumber vertebra at early stages of bone formation, Calcium hydroxyapatite (HAp) [Ca10(PO4)6(OH)2] bone fill with varying composition (60% and 70%) and bone cream (35-48%), has been acquired with 15keV synchrotron X-rays. Experiments were performed at Desy, Hamburg, Germany, utilizing the Resonant and Diffraction beamline (P9), with 15keV X-rays (λ=0.82666 A(0)). Diffraction data were quantitatively analyzed using the Rietveld refinement approach, which allowed us to characterize the structure of these samples in their early stages. Hydroxyapatite, received considerable attention in medical and materials sciences, since these materials are the hard tissues, such as bone and teeth. Higher bioactivity of these samples gained reasonable interest for biological application and for bone tissue repair in oral surgery and orthopedics. The results obtained from these samples, such as phase data, crystalline size of the phases, as well as the degree of crystallinity, confirm the apatite family crystallizing in a hexagonal system, space group P63/m with the lattice parameters of a=9.4328Å and c=6.8842Å (JCPDS card #09-0432). Synchrotron-based XRD patterns are relatively sharp and well resolved and can be attributed to the hexagonal crystal form of hydroxyapatite. All the samples were examined with scanning electron microscope at an accelerating voltage of 15kV. The presence of large globules of different sizes is observed, in small age groups of the rat bone (8w) and lumber vertebra (LV), as distinguished from, large age groups (56 and 78w) in all samples with different magnification, reflects an amorphous phase without significant traces of crystalline phases. Scanning electron microscopy (SEM) was used to characterize the morphology and crystalline properties of Hap, for all the samples, from 2 to 100μm resolution.

  20. Clinical and biochemical function of polymorphic NR0B1 GGAA-microsatellites in Ewing sarcoma: a report from the Children's Oncology Group.

    Directory of Open Access Journals (Sweden)

    Michael J Monument

    Full Text Available The genetics involved in Ewing sarcoma susceptibility and prognosis are poorly understood. EWS/FLI and related EWS/ETS chimeras upregulate numerous gene targets via promoter-based GGAA-microsatellite response elements. These microsatellites are highly polymorphic in humans, and preliminary evidence suggests EWS/FLI-mediated gene expression is highly dependent on the number of GGAA motifs within the microsatellite.Here we sought to examine the polymorphic spectrum of a GGAA-microsatellite within the NR0B1 promoter (a critical EWS/FLI target in primary Ewing sarcoma tumors, and characterize how this polymorphism influences gene expression and clinical outcomes.A complex, bimodal pattern of EWS/FLI-mediated gene expression was observed across a wide range of GGAA motifs, with maximal expression observed in constructs containing 20-26 GGAA motifs. Relative to white European and African controls, the NR0B1 GGAA-microsatellite in tumor cells demonstrated a strong bias for haplotypes containing 21-25 GGAA motifs suggesting a relationship between microsatellite function and disease susceptibility. This selection bias was not a product of microsatellite instability in tumor samples, nor was there a correlation between NR0B1 GGAA-microsatellite polymorphisms and survival outcomes.These data suggest that GGAA-microsatellite polymorphisms observed in human populations modulate EWS/FLI-mediated gene expression and may influence disease susceptibility in Ewing sarcoma.

  1. 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.)

  2. Sarcomas cutâneos primários Primary cutaneous sarcomas

    Directory of Open Access Journals (Sweden)

    Luiz Fernando Fróes Fleury Jr

    2006-06-01

    Full Text Available Os sarcomas com apresentação cutânea primária são tumores raros e de grande heterogeneidade histológica. Com a evolução da oncologia cutânea e da cirurgia dermatológica, os dermatologistas têm sido cada vez mais requisitados para o diagnóstico e orientação terapêutica de tumores menos freqüentes. Este artigo de revisão analisa os sarcomas cutâneos primários observando suas características clínicas, etiopatogênicas e histológicas, bem como aspectos do tratamento e evolução. Enfatiza os sarcomas de maior relevância para o dermatologista, como angiossarcoma, dermatofibrossarcoma protuberans, fibroxantoma atípico, leiomiossarcoma, lipossarcoma, tumor maligno de bainha de nervo periférico e sarcoma epitelióide. O sarcoma de Kaposi não é abordado devido a suas características individuais específicas.Soft tissue tumors represent a heterogeneous group of mesenchymal and neural lesions. The cutaneous presentation of these tumours is rare. With the evolution of dermatologic surgery and cutaneous oncology, dermatologists have emerged as specialists for skin cancer management. This article reviews primary cutaneous sarcomas with particular emphasis on the epidemiologic, clinical, and histological features of diagnosis, as well as treatment modalities and prognosis. The most frequent cutaneous sarcomas were reviewed, including angiosarcoma, dermatofibrosarcoma protuberans, atypical fibroxanthoma, leiomyosarcoma, liposarcoma, malignant nerve sheath tumor, and epithelioid sarcoma. Kaposi's sarcoma, due to specific characteristics, was omitted from this review.

  3. Designing novel therapies against sarcomas in the era of personalized medicine and economic crisis.

    Science.gov (United States)

    Manara, Maria Cristina; Garofalo, Cecilia; Ferrari, Stefano; Belfiore, Antonino; Scotlandi, Katia

    2013-01-01

    Drug "repurposing" is the process of finding new therapeutic indications for existing drugs, and can be considered as a more efficient and realistic strategy for the design of therapies against rare diseases than the current efforts to develop targeted-drugs. In this review, we explore the difficulties related to the identification and development of tailored therapies for individual patients with sarcomas, which are relatively rare diseases characterized by an extreme genetic and histologic variability. Overall, sarcomas comprise about 1% of all adult tumors and 10% of pediatric cancers. They are conventionally divided in bone and soft-tissue sarcomas, considering their site of origin. However, each group is highly heterogeneous and recent global characterization of their genetic alterations has clearly identified the existence of peculiarities that render these group of tumors even more "orphan" for pharmaceutical companies to develop and market specific- targeted drugs. The present review highlights key examples of molecular targets identification in bone sarcomas, reexamining the history of insulin-like growth factor receptor (IGF-IR) and its role in physiology and in cancer as well as developments regarding phase I to II clinical trials of agents directed against this receptor. The IGF system is quite complex, with many players in the field. Insulin receptor function in cancer cells has certainly been underestimated, but also little attention was paid to the type of ligands that are mainly involved in each tumor type. Strategies considering the system in its complex are encouraged and, in this context, drugs aimed at reducing circulating insulin levels, such as metformin, should receive attention as potential anti-cancer agents.

  4. Surgical therapy strategy of soft tissue sarcomas with juxta-articular bone involvement%侵犯关节周围骨组织的软组织肉瘤外科治疗策略

    Institute of Scientific and Technical Information of China (English)

    燕太强; 梁伟民; 郭卫; 杨荣利; 董森; 周文灏

    2012-01-01

    Objective To explore the surgical treating methods of extremity soft tissue sarcomas with juxta-articular bone involvement, and to analyze the postoperative complications, limb function and survival status of the patients. Methods 30 patients of soft tissue sarcomas with local juxta-articular bone involvement were adopted in our center from May 2004 to October 2011, whose clinical data were retrospectively analyzed. There were 14 males and 16 females, with a mean age of 51 years old (range; 17-75 years). There were 12 cases of malignant fibrous histiocytoma (MFH), 8 cases of liposarcoma, 4 cases of primitive neuroectodermal tumors (PNET), and 2 cases of synovial sarcoma, alveolar soft part sarcoma and malignant peripheral nerve sheath tumors (MPNST) respectively. Among them, 10 patients had tumors in the proximal femur, 9 in the distal femur, 8 in the proximal humerus, 2 in the proximal tibia and 1 in the total femur. Bone defect reconstruction using tumor prostheses was performed on all the patients after the wide excision of tumors and bone tissues involved. The periodic reviews of limb function, X-ray images, pulmonary CT scans and so on were carried out postoperatively. All patients were followed up regularly, including the occurrence of postoperative complications, recovery condition of limb function, oncology and survival status and so on. Results The mean follow-up period was 25 months (range; 3-84 months). 1 patient had temporary peroneal nerve palsy. 3 patients had poor wound healing, and then underwent debridement, 1 of whom underwent amputation due to deep infection. Implant fractures leading to additional revisions occurred in 2 cases. 4 patients had local tumor recurrence, and the recurrence rate was 13.3%, 1 of whom underwent amputation. 15 patients had lung metastases, and 11 patients died of disseminated metastases, including 3 patients with bone and lymph node metastases. In the latest follow-up, 14 patients survived tumor free, and 5 were alive

  5. 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

  6. 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.

  7. Growth, bone maturation, and biochemical changes in Brazilian children from two different socioeconomic groups.

    Science.gov (United States)

    Linhares, E D; Round, J M; Jones, D A

    1986-10-01

    A study of blood biochemistry related to skeletal growth in 900 Brazilian children aged 7 to 17 yr is reported. Two groups were studied, a privileged and underprivileged sample. Anthropometry and measures of bone maturation in the control group were comparable with American and British standards. Underprivileged children showed growth impairment and delay in bone maturation. No signs of rickets were found in either group. Plasma calcium, magnesium, vitamin D, and total protein did not differ in the two groups of Brazilian children. Plasma alkaline phosphatase and inorganic phosphorus were abnormal in the underprivileged children. Alkaline-phosphatase activity and phosphorus levels did not fall towards adult levels after the predicted age of the adolescent spurt for underprivileged children. Menarche was delayed in the underprivileged girls.

  8. Soft Tissue Sarcoma

    Science.gov (United States)

    ... other body structures. The soft tissues include muscle, fat, blood vessels, nerves, tendons and the lining of your joints. Many types of soft tissue sarcoma exist. Some types are more likely to ...

  9. 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...... 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...

  10. 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.

  11. 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.

  12. 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 ...

  13. 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 ...

  14. 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

  15. Treatment Option Overview (Kaposi Sarcoma)

    Science.gov (United States)

    ... Treatment Childhood Vascular Tumors Treatment Research Kaposi Sarcoma Treatment (PDQ®)–Patient Version General Information About Kaposi Sarcoma ... Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery ) and treatment ...

  16. Osteogenic Sarcoma: A 21st Century Review.

    Science.gov (United States)

    Osasan, Stephen; Zhang, Mingyong; Shen, Fan; Paul, Paulose J; Persad, Sujata; Sergi, Consolato

    2016-09-01

    Compared to other bone tumors, bone osteogenic sarcoma (BOS) continues to confer a much grimmer prognosis as the survival benefit of traditional chemotherapy treatment regimens is still unsatisfactory. Chemotherapy was demonstrated to be effective in eradicating both primary tumor and pulmonary metastases in the last century, with effective agents used in various combination regimens having changed the survival rate from less than 10% to 75%. The most common primary bone cancer, BOS is conventionally a primary intramedullary high-grade malignant tumor characterized by malignant cells forming immature bone or osteoid. BOS is a disease with diverse morphological presentations. The treatment of all morphological variants seem to have been the same for over 30 years. The introduction of antiproliferative agents such as insulin growth factor-binding protein 3 hold promise of a potentially veritable therapeutic target. In this review, we highlight recent data on osteosarcoma to consolidate a platform able to connect bench and bedside.

  17. Ancient Human Bone Microstructure in Medieval England: Comparisons between Two Socio-Economic Groups.

    Science.gov (United States)

    Miszkiewicz, Justyna J; Mahoney, Patrick

    2016-01-01

    Understanding the links between bone microstructure and human lifestyle is critical for clinical and anthropological research into skeletal growth and adaptation. The present study is the first to report correspondence between socio-economic status and variation in bone microstructure in ancient humans. Products of femoral cortical remodeling were assessed using histological methods in a large human medieval sample (N = 450) which represented two distinct socio-economic groups. Osteonal parameters were recorded in posterior midshaft femoral sections from adult males (N = 233) and females (N = 217). Using univariate and multivariate statistics, intact, fragmentary, and osteon population densities, Haversian canal area and diameter, and osteon area were compared between the two groups, accounting for sex, age, and estimated femoral robusticity. The size of osteons and their Haversian canals, as well as osteon density, varied significantly between the socio-economic groups, although minor inconsistencies were observed in females. Variation in microstructure was consistent with historical textual evidence that describes differences in mechanical loading and nutrition between the two groups. Results demonstrate that aspects of ancient human lifestyle can be inferred from bone microstructure.

  18. Bone

    Science.gov (United States)

    Helmberger, Thomas K.; Hoffmann, Ralf-Thorsten

    The typical clinical signs in bone tumours are pain, destruction and destabilization, immobilization, neurologic deficits, and finally functional impairment. Primary malignant bone tumours are a rare entity, accounting for about 0.2% of all malignancies. Also benign primary bone tumours are in total rare and mostly asymptomatic. The most common symptomatic benign bone tumour is osteoid osteoma with an incidence of 1:2000.

  19. [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.

  20. International Myeloma Working Group Recommendations for the Treatment of Multiple Myeloma–Related Bone Disease

    Science.gov (United States)

    Terpos, Evangelos; Morgan, Gareth; Dimopoulos, Meletios A.; Drake, Matthew T.; Lentzsch, Suzanne; Raje, Noopur; Sezer, Orhan; García-Sanz, Ramón; Shimizu, Kazuyuki; Turesson, Ingemar; Reiman, Tony; Jurczyszyn, Artur; Merlini, Giampaolo; Spencer, Andrew; Leleu, Xavier; Cavo, Michele; Munshi, Nikhil; Rajkumar, S. Vincent; Durie, Brian G.M.; Roodman, G. David

    2013-01-01

    Purpose The aim of the International Myeloma Working Group was to develop practice recommendations for the management of multiple myeloma (MM) –related bone disease. Methodology An interdisciplinary panel of clinical experts on MM and myeloma bone disease developed recommendations based on published data through August 2012. Expert consensus was used to propose additional recommendations in situations where there were insufficient published data. Levels of evidence and grades of recommendations were assigned and approved by panel members. Recommendations Bisphosphonates (BPs) should be considered in all patients with MM receiving first-line antimyeloma therapy, regardless of presence of osteolytic bone lesions on conventional radiography. However, it is unknown if BPs offer any advantage in patients with no bone disease assessed by magnetic resonance imaging or positron emission tomography/computed tomography. Intravenous (IV) zoledronic acid (ZOL) or pamidronate (PAM) is recommended for preventing skeletal-related events in patients with MM. ZOL is preferred over oral clodronate in newly diagnosed patients with MM because of its potential antimyeloma effects and survival benefits. BPs should be administered every 3 to 4 weeks IV during initial therapy. ZOL or PAM should be continued in patients with active disease and should be resumed after disease relapse, if discontinued in patients achieving complete or very good partial response. BPs are well tolerated, but preventive strategies must be instituted to avoid renal toxicity or osteonecrosis of the jaw. Kyphoplasty should be considered for symptomatic vertebral compression fractures. Low-dose radiation therapy can be used for palliation of uncontrolled pain, impending pathologic fracture, or spinal cord compression. Orthopedic consultation should be sought for long-bone fractures, spinal cord compression, and vertebral column instability. PMID:23690408

  1. Assessment of changes in vascularity and blood volume in canine sarcomas and squamous cell carcinomas during fractionated radiation therapy using quantified contrast-enhanced power Doppler ultrasonography: a preliminary study.

    Science.gov (United States)

    Ohlerth, Stefanie; Bley, Carla Rohrer; Laluhová, Dagmar; Roos, Malgorzata; Kaser-Hotz, Barbara

    2010-10-01

    Radiation therapy does not only target tumour cells but also affects tumour vascularity. In the present study, changes in tumour vascularity and blood volume were investigated in five grade 1 oral fibrosarcomas, eight other sarcomas (non-oral soft tissue and bone sarcomas) and 12 squamous cell carcinomas in dogs during fractionated radiation therapy (total dose, 45-56 Gy). Contrast-enhanced power Doppler ultrasound was performed before fraction 1, 3, 6, 8, 10, 12, 14 and 15 or 16 (sarcomas) or 17 (squamous cell carcinomas). Prior to treatment, median vascularity and blood volume were significantly higher in squamous cell carcinomas (P=0.0005 and 0.001), whereas measurements did not differ between oral fibrosarcomas and other sarcomas (P=0.88 and 0.999). During the course of radiation therapy, only small, non-significant changes in vascularity and blood volume were observed in all three tumour histology groups (P=0.08 and P=0.213), whereas median tumour volume significantly decreased until the end of treatment (P=0.04 for fibrosarcomas and other sarcomas, P=0.008 for squamous cell carcinomas). It appeared that there was a proportional decrease in tumour volume, vascularity and blood volume. Doppler measurements did not predict progression free interval or survival in any of the three tumour groups (P=0.06-0.86). However, the number of tumours investigated was small and therefore, the results can only be considered preliminary.

  2. Low body mass index is an important risk factor for low bone mass and increased bone loss in early postmenopausal women. Early Postmenopausal Intervention Cohort (EPIC) study group

    DEFF Research Database (Denmark)

    Ravn, Pernille; Cizza, G; Bjarnason, N H;

    1999-01-01

    Thinness (low percentage of body fat, low body mass index [BMI], or low body weight) was evaluated as a risk factor for low bone mineral density (BMD) or increased bone loss in a randomized trial of alendronate for prevention of osteoporosis in recently postmenopausal women with normal bone mass (n...... of fat mass parameters, prevention of postmenopausal osteoporosis can be equally achieved in thinner and heavier women....... (r = -0.12 to -0.15, p treatment effect of alendronate was dependent on these risk factors, the group treated with 5 mg of alendronate was included (n = 403). There were no associations between fat mass parameters and response to alendronate treatment, which...

  3. 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

  4. Usefulness of postoperative hip irradiation in the prevention of heterotopic bone formation in a high risk group of patients

    Energy Technology Data Exchange (ETDEWEB)

    MacLennan, I.; Keys, H.M.; Evarts, C.M.; Rubin, P.

    1984-01-01

    Heterotopic ossification is a complication of total hip arthroplasty in 14 to 30% of patients. Significant functional impairment will occur in up to 28% of patients with ectopic bone. The high risk group includes those with preexisting heterotopic bone in either hip, those suffering from hypertrophic osteoarthritis or ankylosing spondylitis and patients who have had multiple procedures on the hip. Fifty-eight patients (67 hips) were irradiated after surgical removal of ectopic bone (53 hips) or received radiation prophylaxis of heterotopic ossification (14 hips). Ninety-five percent of patients had either no bone visible or insignificant amounts of ectopic bone visible on postoperative hip X-rays. Only 5% of patients showed significant persistence of ectopic bone. Postoperative hip function was dramatically improved compared to preoperative function in all patients treated. The importance of early commencement of irradiation is emphasized.

  5. 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....

  6. 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, ...

  7. 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, ...

  8. 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

  9. 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 ...

  10. Kaposi sarcoma incidence in Mozambique: national and regional estimates.

    Science.gov (United States)

    Meireles, Paula; Albuquerque, Gabriela; Vieira, Mariana; Foia, Severiano; Ferro, Josefo; Carrilho, Carla; Lunet, Nuno

    2015-11-01

    Kaposi sarcoma is expressed in four clinical variants, all associated with human herpes virus type 8 infection, namely, classic, endemic, immunosuppression-related and AIDS-related. The latter currently accounts for most of the burden of Kaposi sarcoma in sub-Saharan Africa, reflecting the frequency of HIV infection and its management. We aimed to estimate the incidence of Kaposi sarcoma in Mozambique and in its provinces. We estimated the number of incident cases of Kaposi sarcoma by adding up the expected number of endemic and AIDS-related cases. The former were estimated from the rates observed in Kyandondo, Uganda (1960-1971). The latter were computed from the number of AIDS-related deaths in each region, assuming that the ratio between the AIDS-related Kaposi sarcoma incident cases and the number of AIDS-related deaths observed in the city of Beira applies to all regions. A total of 3862 Kaposi sarcoma cases were estimated to have occurred in Mozambique in 2007, mostly AIDS-related, in the age group 25-49 years, and in provinces from South/Centre. The age-standardized incidence rates were 36.1/100 000 in men and 11.5/100 000 in women, with a more than three-fold variation across provinces. We estimated a high incidence of Kaposi sarcoma in Mozambique, along with large regional differences. These results can be used to improve disease management and to sustain political decisions on health policies.

  11. Sarcoma of the breast: an update on a rare entity.

    Science.gov (United States)

    Lim, Sue Zann; Ong, Kong Wee; Tan, Benita Kiat Tee; Selvarajan, Sathiyamoorthy; Tan, Puay Hoon

    2016-05-01

    Breast sarcoma is a rare condition. It consists of a heterogeneous group of non-epithelial tumours arising from the mesenchymal tissue of the breast. It has a distinctly different natural history, treatment response and prognosis as compared with carcinoma of the breast. A different diagnostic approach and treatment strategy have to be defined for this group of tumours. Due to its rarity, the current understanding on breast sarcoma is limited and is mostly based on small retrospective case series or case reports. Hence, the management generally follows the algorithms derived from randomised control trials of soft tissue sarcomas in the extremities and chest wall. Through this review, we discuss the results of major retrospective studies on breast sarcomas including data on epidemiology, aetiology, diagnostic approach, treatment strategies and outcomes of this challenging and potentially aggressive condition.

  12. 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...... efficacy in ES xenografts as single agents. Expression of EWS/FLI1 or EWS/ERG oncogenic translocations sensitizes non-ES cells to ATR inhibitors. Our data shed light onto the sensitivity of ES to genotoxic agents, and identify ATR inhibitors as a potential therapy for Ewing Sarcomas....

  13. 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, ...

  14. 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.)

  15. A case of primary mediastinal Ewing′s sarcoma /primitive neuroectodermal tumor presenting with initial compression of superior vena cava

    Directory of Open Access Journals (Sweden)

    Alessia Reali

    2013-01-01

    Full Text Available Ewing′s sarcomas and peripheral primitive neuroectodermal tumors (ES/PNETs are high grade malignant neoplasms. These malignancies are characterized by a chromosome 22 rearrangement, arise from bone or soft tissue, predominantly affect children and young adults, and are grouped in the Ewing family of tumors. Multimodality treatment programs are the treatment of choice. Primary localization of ES/PNET in the mediastinum is extremely rare. We describe a case of ES/PNET presenting as a mediastinal mass with tracheal compression and initial signs of superior vena cava in a 66-year-old woman.

  16. 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.

  17. Impact of tumor volume doubling time on post-metastatic survival in bone or soft-tissue sarcoma patients treated with metastasectomy and/or radiofrequency ablation of the lung

    Science.gov (United States)

    Nakamura, Tomoki; Matsumine, Akihiko; Takao, Motoshi; Nakatsuka, Atsuhiro; Matsubara, Takao; Asanuma, Kunihiro; Sudo, Akihiro

    2017-01-01

    Metastasectomy represents the standard treatment for improving survival in patients with lung metastases (LMs) from bone (BS) or soft-tissue sarcoma (STS). Recently, radiofrequency ablation (RFA) of the LMs has been proved to be a useful option which can promise the similar effect to metastasectomy. The aim of this study was to determine prognostic factors, including tumor volume doubling time (TVDT), for post-metastatic survival in BS and STS patients treated with metastasectomy and/or RFA of the lung. Forty-eight patients with LMs were retrospectively reviewed. The mean age of the patients at the time of LMs was 56 years. The cohort comprised 27 male and 21 female patients. Eight of the 48 patients had LMs at the point of initial presentation. The mean follow-up period after commencing the treatment for LMs was 37 months. The mean maximum diameter of the initial LMs was 11 mm. The mean number of LMs was 4. The TVDT was calculated using a method originally described by Schwartz. At last follow-up, 5 patients had no evidence of disease, 3 patients were still alive with disease, and 32 patients had died of disease. The 3-year and 5-year post-metastatic survival rates were 32% and 16.8%, respectively. In a Cox univariate analysis, the size (P=0.04) and number of LMs (P<0.001), disease-free interval (P=0.04), curability of the initial LMs (P<0.001), and TVDT (P<0.001) were significantly identified as factors which affect prognosis. In the multivariate analysis, TVDT (P<0.001) and curability of the initial LMs (P<0.001) were confirmed as independent predictors of survival. There was a significant association between the number and curability of the initial LMs (P<0.001). In conclusion, metastasectomy and/or RFA of LMs is recommended for improving survival. However, TVDT and the curability of the LMs should be taken into consideration. PMID:28203089

  18. GLUT1 Expression in Synovial Sarcomas

    Directory of Open Access Journals (Sweden)

    Gülşah KAYGUSUZ

    2009-09-01

    Full Text Available Objective: In this study, the role of GLUT1 expression in synovial sarcomas and its association with disease pathogenesis were examined.Materials and Methods: Twenty two cases of synovial sarcoma were included in this study. The clinicopathological features of the cases, such as age, sex, localization of tumor, information of primary or metastatic tumor, histopathological type were recorded. The tissue microarray paraffin block containing tumor tissues was built by using tissue microarrayer. GLUT1 expression was analyzed on tissue sections by immunohistochemistry.Results: A total of 22 cases (mean age 36 years; range 14-54 years were analyzed. All cases except one were primary tumors. The tumors showed monophasic histological type in 13 cases and biphasic type in 9 cases. GLUT1 expression was found in 3 cases with biphasic type (14%. The cytoplasmic and incomplete membranous GLUT1 expression was seen in the tumor cells showing epithelial-glandular differentiation, whereas spindled cells were negative.Conclusion: Although GLUT1 expression is a diagnostic marker for juvenile capillary hemangioma and perineural tumors, both of which included in the group of mesenchymal tumors, it can be seen in a subset of synovial sarcomas. In our series, the observation of GLUT1 expression especially in the epithelial component of biphasic synovial sarcomas suggests that; i GLUT1 may be relatively used by tumoral cells composing epithelial component of the tumor, and ii the spindle cell component of the tumor would have been positive for other glucose transporters. The finding of uncommon GLUT1 expression in synovial sarcomas is indirectly consistent with the reported results of decreased standardized uptake value by Positron emission tomography with 2-[18F]fluoro-2-deoxy-D-glucose method in the literature.

  19. 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.)

  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. 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.

  2. MLN0128, an ATP-Competitive mTOR Kinase Inhibitor, with Potent In vitro and In vivo Antitumor Activity as Potential Therapy for Bone and Soft-Tissue Sarcoma

    Science.gov (United States)

    Slotkin, Emily K.; Patwardhan, Parag P.; Vasudeva, Shyamprasad Deraje; de Stanchina, Elisa; Tap, William D.; Schwartz, Gary K.

    2014-01-01

    The mammalian target of rapamycin (mTOR) is a serine/threonine protein kinase that exists in two complexes (mTORC1 and mTORC2) and integrates extracellular and intracellular signals to act as a master regulator of cell growth, survival, and metabolism. The PI3K/AKT/mTOR pro-survival pathway is often dysregulated in multiple sarcoma subtypes. First-generation allosteric inhibitors of mTORC1 (rapalogues) have been extensively tested with great pre-clinical promise, but have had limited clinical utility. Here we report that MLN0128, a second-generation, ATP-competitive, pan-mTOR kinase inhibitor, acts on both mTORC1 and mTORC2, and has potent in vitro and in vivo anti-tumor activity in multiple sarcoma subtypes. In vitro, MLN0128 inhibits mTORC1/2 targets in a concentration dependent fashion, and shows striking anti-proliferative effect in rhabdomyosarcoma (RMS), Ewing sarcoma (ES), malignant peripheral nerve sheath tumor, synovial sarcoma, osteosarcoma, and liposarcoma. Unlike rapamycin, MLN0128 inhibits phosphorylation of 4EBP1 and NDRG1 as well as prevents the reactivation of pAKT that occurs via negative feedback release with mTORC1 inhibition alone. In xenograft models, MLN0128 treatment results in suppression of tumor growth with two dosing schedules (1 mg/kg daily and 3 mg/kg BID TIW). At the 3 mg/kg dosing schedule, MLN0128 treatment results in significantly better tumor growth suppression than rapamycin in RMS and ES models. Additionally, MLN0128 induces apoptosis in models of RMS both in vitro and in vivo. Results from our study strongly suggest that MLN0128 treatment should be explored further as potential therapy for sarcoma. PMID:25519700

  3. 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

  4. 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

  5. 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

  6. [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

  7. Myeloid sarcoma: a report of four cases at unusual sites

    Directory of Open Access Journals (Sweden)

    Siraj, Fouzia

    2017-02-01

    Full Text Available Background: Myeloid sarcoma (MS or granulocytic sarcoma is a rare tumor consisting of myeloid blasts with or without maturation occurring at anatomic sites other than the bone marrow. MS can involve any organ system but shows a predilection for skin, bone, and soft tissues of head and neck region.Case report: We report four cases of MS occurring at unusual sites, out of which three were de novo and one was associated with acute myeloid leukemia (AML.Conclusion: Although MS is associated with AML, it can rarely present without any existent hematologic disease. Differential diagnosis of a soft tissue mass should include MS even in the absence of leukemia. Establishment of the correct diagnosis depends on morphologic, histochemical, and immunohistochemical examination.

  8. Variable expression of PIK3R3 and PTEN in Ewing Sarcoma impacts oncogenic phenotypes.

    Directory of Open Access Journals (Sweden)

    Brian F Niemeyer

    Full Text Available Ewing Sarcoma is an aggressive malignancy of bone and soft tissue affecting children and young adults. Ewing Sarcoma is driven by EWS/Ets fusion oncoproteins, which cause widespread alterations in gene expression in the cell. Dysregulation of receptor tyrosine kinase signaling, particularly involving IGF-1R, also plays an important role in Ewing Sarcoma pathogenesis. However, the basis of this dysregulation, including the relative contribution of EWS/Ets-dependent and independent mechanisms, is not well understood. In the present study, we identify variable expression of two modifiers of PI3K signaling activity, PIK3R3 and PTEN, in Ewing Sarcoma, and examine the consequences of this on PI3K pathway regulation and oncogenic phenotypes. Our findings indicate that PIK3R3 plays a growth-promotional role in Ewing Sarcoma, but suggest that this role is not strictly dependent on regulation of PI3K pathway activity. We further show that expression of PTEN, a well-established, potent tumor suppressor, is lost in a subset of Ewing Sarcomas, and that this loss strongly correlates with high baseline PI3K pathway activity in cell lines. In support of functional importance of PTEN loss in Ewing Sarcoma, we show that re-introduction of PTEN into two different PTEN-negative Ewing Sarcoma cell lines results in downregulation of PI3K pathway activity, and sensitization to the IGF-1R small molecule inhibitor OSI-906. Our findings also suggest that PTEN levels may contribute to sensitivity of Ewing Sarcoma cells to the microtubule inhibitor vincristine, a relevant chemotherapeutic agent in this cancer. Our studies thus identify PIK3R3 and PTEN as modifiers of oncogenic phenotypes in Ewing Sarcoma, with potential clinical implications.

  9. Primary alveolar soft part sarcoma of fibula demonstrating ASPL-TFE3 fusion: a case report and review of the literature

    Energy Technology Data Exchange (ETDEWEB)

    Aisner, Seena C.; Mirani, Neena; Hameed, Meera [University Hospital, Department of Pathology, Newark, NJ (United States); New Jersey Medical School, Newark (United States); Beebe, Kathleen [University Hospital, Department of Orthopedic Surgery, Newark, NJ (United States); New Jersey Medical School, Newark (United States); Blacksin, Marcia [University Hospital, Department of Radiology, Newark, NJ (United States); New Jersey Medical School, Newark (United States)

    2008-11-15

    Alveolar soft part sarcoma is a rare soft tissue tumor typically affecting young adults. These tumors are most often seen in the deep soft tissues of the extremities and patients generally present with advanced disease. Primary bone involvement is extremely rare and has only been reported in seven cases. This is the first case of alveolar soft part sarcoma in bone documenting the ASPL-TFE3 gene product. Herein, we report a rare presentation of alveolar soft part sarcoma presenting as a primary bone neoplasm involving the proximal fibula in a 41-year-old woman. (orig.)

  10. 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.

  11. Intracranial Dural Metastasis of Ewing's Sarcoma: a Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Eung Yeop; Lee, Seung Koo; Kim, Dong Joon; Kim, Jin Na; Lee, Kyu Sung; Jung, Woo Hee; Kim, Dong Ik [Yonsei University College of Medicine, Seoul (Korea, Republic of)

    2008-02-15

    Ewing's sarcoma is a malignant bone tumor that can occur anywhere in the body, but it is most commonly observed in the long bones of the arms and legs, the pelvis and in the chest. The predominant sites of metastasis include the lung (38%), bone (including the spine; 31%), and the bone marrow (11%). Metastasis of Ewing's sarcoma to the central nervous system (CNS) is relatively rare, and most of the previous reports have demonstrated involvement of the bony calvarium or brain parenchyma. We describe here the imaging findings of dural metastasis of Ewing's sarcoma, and these imaging findings have not been previously reported on in the medical literature. In conclusion, dural metastasis of Ewing's sarcoma is very rare and its imaging characteristics are similar to those of a primary tumor, which mimic the findings of a schwannoma or meningioma. Despite its rarity, secondary Ewing's sarcoma may be included in the differential diagnosis of extra-axial dural masses.

  12. 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.

  13. Primary sarcomas of the central nervous system: UCSF experience (1985-2005

    Directory of Open Access Journals (Sweden)

    Tarık TİHAN

    2007-01-01

    Full Text Available Sarcomas constitute less than 2% of all cancers, and are a highly diverse group of neoplasms. Primary sarcomas of the central nervous system (CNS are even less common, and our experience is limited by lack of studies with sufficient size that can address the challenges in predicting behavior or management. It is critical to recognize the characteristics of these uncommon neoplasms and to develop better predictors for prognosis and behavior.We have conducted a search of the UCSF Department of Pathology and UCSF Cancer Center Registry databases for all primary CNS sarcomas that were diagnosed and treated between 1985 and 2005. Hemangiopericytomas were included, so were the solitary fibrous tumors due to their close association with the former. We excluded all cases of metastatic sarcomas, chordomas, sarcomatoid variants of all neuroepithelial neoplasms, Ewing’s sarcomas and other embryonal tumors. In addition, we have identified all soft tissue sarcomas diagnosed and treated during the same period. There were 43 primary CNS neoplasms that fulfilled the inclusion criteria. At the same time, we identified 1706 sarcomas primary to the soft tissue. Primary CNS sarcomas included 16 hemangiopericytomas, 15 chondrosarcomas, 3 solitary fibrous tumors, 3 osteosarcomas, 2 leiomyosarcomas, 2 undifferentiated sarcomas, 1 histiocytic sarcoma, and 1 fibrosarcoma. There was a distinctly higher frequency of hemangiopericytoma in the CNS compared to soft tissue. In addition, a group of low grade, parasagittal chondrosarcomas were noted for their highly indolent biological behavior. Unlike some previous series, our cohort was devoid of angiosarcoma and malignant fibrous histiocytoma. This study underscores the limitations of single institutional series, and highlights the value of multi-institutional studies to understand and better treat primary CNS sarcomas.

  14. 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 ...

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

    Science.gov (United States)

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

    2015-03-01

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

  16. [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.

  17. 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.

  18. 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

  19. 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.

  20. 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.

  1. Bone marrow transplantation in childhood: a report from the AIEOP-BMT group registry.

    Science.gov (United States)

    Paolucci, P; Dini, G; Dallorso, S; Meloni, G; Arcese, W; Porta, F; Bonetti, F; Uderzo, C; Locasciulli, A; Andolina, M

    1991-01-01

    A multicenter cooperative group has been activated in Italy in 1986, with the aim of focusing on the following issues: to standardize perspectively the approach to bone marrow transplantation (BMT) in pediatric centers granting the recommendations of the Associazione Italiana di Ematologia e Oncologia Pediatrica (AIEOP); to study effects and toxicity of preparative regimens to BMT; to evaluate the special features of BMT in children, such as clinical management, care and late effects, as peculiar issues of a pediatric setting. Indeed, one of the major aims of the group is to attempt to address in the most appropriate ways, such as in children only and at a nation-wide level, the most crucial questions about the real role of BMT, taking advantage from the fact that most of the children with cancer undergoing BMT in Italy have been treated homogeneously before transplant, i.e. according to AIEOP protocols (70%). Accordingly, specific information were recorded by means of problem oriented forms, aimed to generate a full registry. The registry has been collecting the experiences of 10 pediatric hematology and oncology centers where children eligible for BMT were registered and/or where BMT was performed. This paper was meant both to summarize the 4 years activity of the registry and to analyze what we have learnt so far, as well as what may be suggested to design our future strategies by presently available although retrospective data. It is concluded that the pediatric BMT registry we set up appears useful and of growing interest and potential relevance.

  2. Synovial Sarcoma Associated With Indwelling Intramedullary Pin in a Peach-Faced Lovebird (Agapornis roseicollis).

    Science.gov (United States)

    Nakano, Yumiko; Une, Yumi

    2016-03-01

    Sarcoma developing in association with a metallic orthopedic procedure is an uncommon but well-recognized complication in mammals. We report on a synovial sarcoma that developed at the site of an intramedullary pin after surgery to treat a bone fracture. A 17-year-old female peach-faced lovebird (Agapornis roseicollis) developed a spherical mass on the distal right dorsal wing at a site that was previously fractured and surgically repaired with an indwelling intramedullary pin. The right wing was amputated at the scapulohumeral joint. One year later, the bird died. Postmortem examination revealed metastases in the right lung, left thoracic wall, and proventricular serosa. Histologically, the tumor had a characteristic biphasic pattern. The tumor was immunohistologically and ultrastructurally identified as a synovial sarcoma. This is the first report of a suspected fracture-associated sarcoma in a bird.

  3. Extraskeletal Ewing’s Sarcoma Arising from the Sciatic Nerve: A Diagnostic Challenge

    Directory of Open Access Journals (Sweden)

    Aadhar Sharma

    2015-01-01

    Full Text Available Ewing’s sarcoma is a common bone tumour of childhood but is a rare occurrence in individuals over 20 years of age. Few cases are reported as originating from peripheral nerves. We present an unusual case of extraosseous Ewing’s sarcoma originating from the sciatic nerve in a 66-year-old patient which had the clinical hallmarks of a benign nerve sheath tumour. Following discussion at a multidisciplinary meeting, excision biopsy of the suspected benign nerve sheath tumour was planned. At operation, the mass had malignant features. Histology confirmed the presence of Ewing’s sarcoma. Due to the morbidity of nerve resection, radiotherapy and chemotherapy were commenced. Ewing’s sarcoma is known to mimic benign pathologies. In this case there were subtle signs of a malignant process in the form of unremitting pain. It is vital to keep in mind the less common tumours that can affect the peripheral nervous system in such cases.

  4. Epigenetic remodeling of chromatin architecture: exploring tumor differentiation therapies in mesenchymal stem cells and sarcomas.

    Science.gov (United States)

    Siddiqi, Sara; Mills, Joslyn; Matushansky, Igor

    2010-03-01

    Sarcomas are the mesenchymal-derived malignant tumors of connective tissues (e.g., fat, bone, and cartilage) presumed to arise from aberrant development or differentiation of mesenchymal stem cells (MSCs). Appropriate control of stem cell maintenance versus differentiation allows for normal connective tissue development. Current theories suggest that loss of this control--through accumulation of genetic lesions in MSCs at various points in the differentiation process--leads to development of sarcomas, including undifferentiated, high grade sarcoma tumors. The initiation of stem cell differentiation is highly associated with alteration of gene expression, which depends on chromatin remodeling. Epigenetic chromatin modifying agents have been shown to induce cancer cell differentiation and are currently being used clinically to treat cancer. This review will focus on the importance of epigenetic chromatin remodeling in the context of mesenchymal stem cells, sarcoma tumorigenesis and differentiation therapy.

  5. 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.

  6. 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.].

  7. Cytogenetics Findings in a Histiocytic Sarcoma Case

    Science.gov (United States)

    Alonso-Dominguez, J. M.; Calbacho, M.; Talavera, M.; Villalon, C.; Abalo, L.; Garcia-Gutierrez, J. V.; Lozano, S.; Tenorio, M.; Villarrubia, J.; Lopez-Jimenez, J.; Ferro, M. T.

    2012-01-01

    Histiocytic sarcoma (HS) is a neoplasm derived from histiocytes. Its diagnosis was not clear until its immunohistochemistry profile was correctly established. Not much is known about its genetic properties. We report a case of a 48-year-old male patient whose bone marrow was almost completely occupied by monomorphic medium size neoplastic cellularity. Its immunohistochemical profile was CD68+, CD4+, CD45+ with negativity of other dendritic cells, and other lineage markers. Cytogenetic study showed 4 related clones: one with trisomy 8 and extra material on the short arms of chromosome 4; a second line with tetrasomy of chromosome 8, add(4)(p16); the third clone had the same alterations as the previous and deletion of chromosome 3 at q11; the fourth line had tetrasomy 8 and translocation t(3;5)(q25;q35). To our knowledge this is the first HS case showing chromosome 8 trisomy and tetrasomy and the other described alterations. PMID:22937328

  8. Cytogenetics Findings in a Histiocytic Sarcoma Case

    Directory of Open Access Journals (Sweden)

    J. M. Alonso-Dominguez

    2012-01-01

    Full Text Available Histiocytic sarcoma (HS is a neoplasm derived from histiocytes. Its diagnosis was not clear until its immunohistochemistry profile was correctly established. Not much is known about its genetic properties. We report a case of a 48-year-old male patient whose bone marrow was almost completely occupied by monomorphic medium size neoplastic cellularity. Its immunohistochemical profile was CD68+, CD4+, CD45+ with negativity of other dendritic cells, and other lineage markers. Cytogenetic study showed 4 related clones: one with trisomy 8 and extra material on the short arms of chromosome 4; a second line with tetrasomy of chromosome 8, add(4(p16; the third clone had the same alterations as the previous and deletion of chromosome 3 at q11; the fourth line had tetrasomy 8 and translocation t(3;5(q25;q35. To our knowledge this is the first HS case showing chromosome 8 trisomy and tetrasomy and the other described alterations.

  9. 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.

  10. Bone status of Indian women from a low-income group and its relationship to the nutritional status.

    Science.gov (United States)

    Shatrugna, Veena; Kulkarni, Bharati; Kumar, P Ajay; Rani, K Usha; Balakrishna, N

    2005-12-01

    Indian women from low-income groups consume diets that have inadequate calcium coupled with too few calories, proteins and micronutrients. Hospital-based data suggest that these women have osteoporotic hip fractures at a much earlier age than Western women. Studies reporting bone parameters of the Indian population involving large sample sizes are not available. This study was therefore carried out with 289 women in the 30-60-year age group to estimate the prevalence of osteoporosis and measure the bone parameters by dual energy X-ray absorptiometry (DXA). Their mean (+/- SD) age was 41.0+/-8.60 years. Their mean (+/- SD) height, weight and body mass index (BMI) were 149.1+/-5.49 cm, 49.2+/-9.85 kg and 22.1+/-3.99, respectively. Dietary intake of calcium was estimated to be 270+/-57 mg/day. The prevalence of osteoporosis at the femoral neck was around 29%. Bone mineral density (BMD) and T scores at all the skeletal sites were much lower than the values reported from the developed countries and were indicative of a high prevalence of osteopenia and osteoporosis. BMD showed a decline after the age of 35 years in cases of the lumbar spine and femoral neck. This was largely due to a decrease of bone mineral content (BMC). The nutritional status of women appears to be an important determinant of bone parameters. BMD and BMC at all the skeletal sites and whole body increased significantly with increasing body weight and BMI of women (Pmenopause and calcium intake were the other important determinants of BMD (Pnutritional status, dietary calcium intake and thus the bone health of this population.

  11. 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.

  12. 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.

  13. 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 ...

  14. 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.

  15. Clear cell sarcoma of the kidney: A case report

    Directory of Open Access Journals (Sweden)

    Dipanwita Nag

    2014-01-01

    Full Text Available Clear cell sarcoma of the kidney is a rare malignant neoplasm of childhood, known for its aggressiveness, its tendency for recurrence, and to metastasize to bone. We report the observation of 8-month-old child presenting with a large abdominal mass. Clinically, it was diagnosed as Wilm′s tumor, and left nephrectomy was done. Grossly, 10 cm × 8 cm × 3.5 cm globular, white, encapsulated, smooth mass uniformly involving the whole kidney was noted. Histologically, the tumor was diagnosed as clear cell sarcoma with renal vein showing presence of tumor embolus in lumen. The tumor was given stage-II (NWTS-5 protocol. Immunohistochemistry showed vimentin positive and cytokeratin negative tumor cells. The child is currently undergoing chemotherapy and has not yet shown any sign of bony metastasis.

  16. Extraosseous Ewing's sarcoma: review of a case. Sarcoma de Ewing extraoseo: revision a proposito de un caso

    Energy Technology Data Exchange (ETDEWEB)

    Quevedo Moreno, P.; Hernandez Moreno, L.; Perez Diaz, M.; Lafuente Martinez, J.L.; Arozamena Laso, M.

    1994-01-01

    Extraosseous Ewing's sarcoma (EES) is an uncommon lesion included in the group of soft tissue tumors. We present a case in a 19-year-old woman in which the diagnosis was not initially suspected because of the absence of clinical and radiological evidence. (Author)

  17. 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.

  18. Bone shape difference between control and osteochondral defect groups of the ankle joint

    NARCIS (Netherlands)

    Tümer, N; Blankevoort, L; van de Giessen, M; Terra, M P; de Jong, P.A.; Weinans, H; Tuijthof, G J M; Zadpoor, A A

    2016-01-01

    OBJECTIVE: The etiology of osteochondral defects (OCDs), for which the ankle (talocrural) joint is one of the common sites, is not yet fully understood. In this study, we hypothesized that bone shape plays a role in development of OCDs. Therefore, we quantitatively compared the morphology of the tal

  19. Tooth counts do not predict bone mineral density in early postmenopausal Caucasian women. EPIC study group

    DEFF Research Database (Denmark)

    Earnshaw, S A; Keating, N; Hosking, D J;

    1998-01-01

    BACKGROUND: It has been suggested that poor dental status may be a suitable criterion for bone densitometry referral in early postmenopausal women. We evaluated this hypothesis in a cohort of 1365 Caucasian women aged between 45 and 59 years, who were enrolled into an international multi-centre t...... loss. Tooth counts therefore cannot be used to identify individuals at risk of osteoporosis....

  20. Vitamin B12: a novel indicator of bone health in vulnerable groups

    NARCIS (Netherlands)

    Dhonukshe-Rutten, R.A.M.

    2004-01-01

    Background: A number of modifiable predictors for osteoporosis and fractures have been identified, including nutritional factors, such as vitamin D deficiency and low calcium intake. Cobalamin deficiency has been suggested to affect bone metabolism. Pernicious anaemia, which can result in cobalamin

  1. The clinical diagnosis of Osteoporosis: A position statement from the National Bone Health Alliance working group

    Science.gov (United States)

    Osteoporosis is a common disorder of reduced bone strength that predisposes to an increased risk for fractures in older individuals. In the United States, the standard criterion for the diagnosis of osteoporosis in postmenopausal women and older men is a T-score less than or equal to-2.5 at the lum...

  2. Results of a phase II trial with second-line cystemustine at 60 mg/m{sup 2} in advanced soft tissue sarcoma: A trial of the EORTC early clinical studies group

    Energy Technology Data Exchange (ETDEWEB)

    Chollet, P. [Centre Jean Perrin and Inserm U71, 58 rue Montalembert, B.P. 392, 63011 Clermont-Ferrand Cedex 1 (France); Fumoleau, P. [Centre Rene Gauducheau, Site Hospitalier Nord, Boulevard Jacques Monod, 44805 Saint-Herblain Cedex (France); Lentz, M.A. [EORTC Data Center, Avenue E. Mounier, 83-B. 11, 1200 Brussels (Belgium); Chevallier, B. [Centre Henri Becquerel, Rue d' Amiens, 76038 Rouen Cedex (France); Roche, H. [Centre Claudius Regaud, 20-24 rue du Pont-Saint-Pierre, 31052 Toulouse Cedex (France); Kerbrat, P. [Centre Eugene Marquis, Rue de la bataille Flandre-Dunkerque, B.P. 6279, 35062 Rennes Cedex (France); Tubiana, N. [C.H.U. Dupuytren, 2 avenue Martin Luther King, 87042 Limoges Cedex (France); Adenis, A. [Centre Oscar Lambret, Rue Frederic Combemale, B.P. 307, 59020 Lille Cedex (France); Krakowski, I. [Centre Alexis Vautrin, Avenue de Bourgogne, 54511 Vandoeuvre-les-Nancy Cedex (France); Cure, H. [Centre Jean Perrin and Inserm U71, 58 rue Montalembert, B.P. 392, 63011 Clermont-Ferrand Cedex 1 (France)

    1998-02-01

    The aim of this phase II trial was to examine the efficacy of a new nitrosourea, cystemustine, in soft tissue sarcoma. Between January 1990 and March 1991, 32 pretreated patients with advanced soft tissue sarcoma were enrolled. Cystemustine was given every 2 weeks at 60 mg/m{sup 2} via a 15-min i.v. infusion. All eligible patients were considered evaluable for response and toxicity (WHO criteria). Of the 32 enrolled patients, 4 were ineligible, leaving 28 evaluable patients. All but 1 had been pretreated: 6 with adjuvant chemotherapy, 18 patients with first-line palliative chemotherapy without nitrosourea, 3 with both treatments, and 18 had received radiotherapy. Median age was 54 years (range 20-73) and median performance status was 1 (0-2). One partial response (PR, duration 12 weeks), 2 stable disease and 25 progressions were observed, giving an overall response rate of 3.57% (confidence interval: 0.1-18.4%). Toxicity was mild, and was mainly neutropenia (no grade 3 or 4), thrombocytopenia (3.57% grade 3 and grade 4) and nausea-vomiting (no grade 3 or 4). It should be noted that the treatment for the patient who obtained a PR was third line with no previous response. Cystemustine with this schedule appears to have a low clinical activity and toxicity in advanced soft tissue sarcoma. (Copyright (c) 1998 Elsevier Science B.V., Amsterdam. All rights reserved.)

  3. Recurrent, multiple, calcified soft tissue metastases from osteogenic sarcoma without pulmonary involvement

    NARCIS (Netherlands)

    Wolf, R; Wolf, RFE; Hoekstra, HJ

    1999-01-01

    Osteosarcoma (osteogenic sarcoma) metastasizes primarily to the lung. With the introduction of neoadjuvant chemotherapy as part of the treatment, the overall and disease-free survival rates have dramatically improved. In this case report, a young man with multiple soft tissue and bone metastases, in

  4. Cerebellar, Pancreatic, and Paraspinal Metastases in Soft Tissue Sarcomas: Unusual Sites or Changing Patterns?

    Directory of Open Access Journals (Sweden)

    Girish Bedre

    2007-07-01

    Full Text Available Context Soft tissue sarcomas generally first metastasize to the lungs followed by the involvement of other sites such as lymph nodes and bones as part of the disseminated disease. Cerebellar and pancreatic metastases from tumors of mesenchymal origin such as soft tissue sarcomas are exceptional, more so in the absence of pulmonary metastases. Case report A previously treated case of chest wall sarcoma presented with the sudden onset of neurological symptoms. An MRI brain scan was suggestive of a solitary cerebellar metastasis. A CT scan of the thorax and abdomen showed no evidence of disease. A metastasectomy of the solitary brain lesion confirmed a deposit from a previously treated sarcoma. Within two months he presented with central abdominal pain and low backache radiating down both lower limbs. FDG-PET and CT scans revealed a large pancreatic and left paraspinal mass with intense tracer uptake suggestive of metastatic involvement. There was no evidence of pulmonary metastases. A CT-guided biopsy was suggestive of high-grade sarcoma. He was treated with palliative radiotherapy with good symptomatic relief. Conclusion Cerebellar, pancreatic, and paraspinal metastases from soft tissue sarcomas are rare, especially in the absence of pulmonary metastases. A high index of suspicion is necessary, and appropriate imaging should be considered for symptomatic patients.

  5. Spinal high-mobility group box 1 contributes to mechanical allodynia in a rat model of bone cancer pain

    Energy Technology Data Exchange (ETDEWEB)

    Tong, Wei [Department of Out-Patient, Xijing Hospital, Fourth Military Medical University, Xi' an 710032 (China); Wang, Wei; Huang, Jing [Department of Anatomy and K. K. Leung Brain Research Centre, Fourth Military Medical University, Xi' an 710032 (China); Ren, Ning [Comprehensive Diagnostic and Therapeutic Center, Xijing Hospital, Fourth Military Medical University, Xi' an 710032 (China); Wu, Sheng-Xi, E-mail: shengxi@fmmu.edu.cn [Department of Anatomy and K. K. Leung Brain Research Centre, Fourth Military Medical University, Xi' an 710032 (China); Li, Yong-Qi, E-mail: devneuro@fmmu.edu.cn [Comprehensive Diagnostic and Therapeutic Center, Xijing Hospital, Fourth Military Medical University, Xi' an 710032 (China)

    2010-05-14

    Mechanisms underlying bone cancer-induced pain are largely unknown. Previous studies indicate that neuroinflammation in the spinal dorsal horn is especially involved. Being first reported as a nonhistone chromosomal protein, high-mobility group box 1 (HMGB1) is now implicated as a mediator of inflammation. We hypothesized that HMGB1 could trigger the release of cytokines in the spinal dorsal horn and contribute to bone cancer pain. To test this hypothesis, we first built a bone cancer pain model induced by intratibal injection of Walker 256 mammary gland carcinoma cells. The structural damage to the tibia was monitored by radiological analysis. The mechanical allodynia was measured and the expression of spinal HMGB1 and IL-1{beta} was evaluated. We observed that inoculation of cancer cells, but not heat-killed cells, induced progressive bone destruction from 9 d to 21 d post inoculation. Behavioral tests demonstrated that the significant nociceptive response in the cancer cells-injected rats emerged on day 9 and this kind of mechanical allodynia lasted at least 21 d following inoculation. Tumor cells inoculation significantly increased HMGB1 expression in the spinal dorsal horn, while intrathecal injecting a neutralizing antibody against HMGB1 showed an effective and reliable anti-allodynia effect with a dose-dependent manner. IL-1{beta} was significantly increased in caner pain rats while intrathecally administration of anti-HMGB1 could decrease IL-1{beta}. Together with previous reports, we predict that bone cancer induces HMGB1 production, enhancing spinal IL-1{beta} expression and thus modulating spinal excitatory synaptic transmission and pain response.

  6. Pseudoanaplastic tumors of bone

    Energy Technology Data Exchange (ETDEWEB)

    Bahk, Won-Jong [Uijongbu St. Mary Hospital, The Catholic University of Korea, Department of Orthopaedic Surgery, Gyunggido, 480-821 (Korea); Mirra, Joseph M. [Orthopaedic Hospital, Orthopedic Oncology, Los Angeles, California (United States)

    2004-11-01

    To discuss the concept of pseudoanaplastic tumors of bone, which pathologically show hyperchromatism and marked pleomorphism with quite enlarged, pleomorphic nuclei, but with no to extremely rare, typical mitoses, and to propose guidelines for their diagnosis. From a database of 4,262 bone tumors covering from 1971 to 2001, 15 cases of pseudoanaplastic bone tumors (0.35% of total) were retrieved for clinical, radiographic and pathologic review. Postoperative follow-up after surgical treatment was at least 3 years and a maximum of 7 years. There were eight male and seven female patients. Their ages ranged from 10 to 64 years with average of 29.7 years. Pathologic diagnoses of pseudoanaplastic variants of benign bone tumors included: osteoblastoma (4 cases), giant cell tumor (4 cases), chondromyxoid fibroma (3 cases), fibrous dysplasia (2 cases), fibrous cortical defect (1 case) and aneurysmal bone cyst (1 case). Radiography of all cases showed features of a benign bone lesion. Six cases, one case each of osteoblastoma, fibrous dysplasia, aneurysmal bone cyst, chondromyxoid fibroma, giant cell tumor and osteoblastoma, were initially misdiagnosed as osteosarcoma. The remaining cases were referred for a second opinion to rule out sarcoma. Despite the presence of significant cytologic aberrations, none of our cases showed malignant behavior following simple curettage or removal of bony lesions. Our observation justifies the concept of pseudoanaplasia in some benign bone tumors as in benign soft tissue tumors, especially in their late evolutionary stage when bizarre cytologic alterations strongly mimic a sarcoma. (orig.)

  7. 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.

  8. 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, ...

  9. 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, ...

  10. Treatment Option Overview (Osteosarcoma and Malignant Fibrous Histiocytoma of Bone)

    Science.gov (United States)

    ... Research Osteosarcoma and Malignant Fibrous Histiocytoma of Bone Treatment (PDQ®)–Patient Version General Information About Osteosarcoma and ... Ewing Sarcoma Treatment for more information. Having past treatment with radiation can increase the risk of osteosarcoma. ...

  11. Epithelioid sarcoma mimicking a primary osseous multifocal scapula lesion

    Energy Technology Data Exchange (ETDEWEB)

    Nakashima, Hiroatsu; Sugiura, Hideshi; Nishida, Yoshihiro; Yamada, Yoshihisa [Department of Orthopaedic Surgery, Nagoya University School of Medicine (Japan); Katagiri, Hirohisa [Department of Orthopaedic Surgery, Nagoya Memorial Hospital, Nagoya (Japan); Yonekawa, Masahiro [Department of Orthopaedic Surgery, Aichi Prefectural Hospital, Nagoya (Japan)

    2002-07-01

    This report describes a case of bone involvement by epithelioid sarcoma, which on imaging had the appearance of a primary intraosseous lesion of the scapula. The tumor initially presented as a subcutaneous nodule which was mistakenly diagnosed as ''fibrosis'' following initial resection. The lesion recurred locally and after several resections presented with several ulcerated subcutaneous nodules, at which time all imaging studies were performed. The patient was treated with en bloc upper humeral interscapulothoracic resection and shoulder reconstruction. Two years after the operation the patient is alive without local recurrence or metastasis. (orig.)

  12. Management of chronic kidney disease–mineral and bone disorder: Korean working group recommendations

    Directory of Open Access Journals (Sweden)

    Eunah Hwang

    2015-03-01

    Full Text Available For Korean dialysis patients, chronic kidney disease–mineral bone disorder is a serious burden because of cardiovascular calcification and mortality. However, recent epidemiologic data have demonstrated that many patients undergoing maintenance hemodialysis are out of the target ranges of serum calcium, phosphorus, and intact parathyroid hormone. Thus, we felt the necessity for the development of practical recommendations to treat abnormal serum phosphorus, calcium, and iPTH in dialysis patients. In this paper, we briefly comment on the measurement of serum calcium, phosphorus, iPTH, dialysate calcium concentration, dietary phosphorus restriction, use of phosphate binders, and medical and surgical options to correct secondary hyperparathyroidism. In particular, for the optimal management of secondary hyperparathyroidism, we suggest a simplified medication adjustment according to certain ranges of serum phosphorus and calcium. Large-scale, well-designed clinical studies are required to support our strategies to control chronic kidney disease–mineral bone disorder in this country. Based on such data, our practice guidelines could be established and better long-term outcomes should be anticipated in our dialysis patients.

  13. Bone cysts: unicameral and aneurysmal bone cyst.

    Science.gov (United States)

    Mascard, E; Gomez-Brouchet, A; Lambot, K

    2015-02-01

    Simple and aneurysmal bone cysts are benign lytic bone lesions, usually encountered in children and adolescents. Simple bone cyst is a cystic, fluid-filled lesion, which may be unicameral (UBC) or partially separated. UBC can involve all bones, but usually the long bone metaphysis and otherwise primarily the proximal humerus and proximal femur. The classic aneurysmal bone cyst (ABC) is an expansive and hemorrhagic tumor, usually showing characteristic translocation. About 30% of ABCs are secondary, without translocation; they occur in reaction to another, usually benign, bone lesion. ABCs are metaphyseal, excentric, bulging, fluid-filled and multicameral, and may develop in all bones of the skeleton. On MRI, the fluid level is evocative. It is mandatory to distinguish ABC from UBC, as prognosis and treatment are different. UBCs resolve spontaneously between adolescence and adulthood; the main concern is the risk of pathologic fracture. Treatment in non-threatening forms consists in intracystic injection of methylprednisolone. When there is a risk of fracture, especially of the femoral neck, surgery with curettage, filling with bone substitute or graft and osteosynthesis may be required. ABCs are potentially more aggressive, with a risk of bone destruction. Diagnosis must systematically be confirmed by biopsy, identifying soft-tissue parts, as telangiectatic sarcoma can mimic ABC. Intra-lesional sclerotherapy with alcohol is an effective treatment. In spinal ABC and in aggressive lesions with a risk of fracture, surgical treatment should be preferred, possibly after preoperative embolization. The risk of malignant transformation is very low, except in case of radiation therapy.

  14. 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.

  15. 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...

  16. Carotenoid derivatives inhibit nuclear factor kappa B activity in bone and cancer cells by targeting key thiol groups.

    Science.gov (United States)

    Linnewiel-Hermoni, Karin; Motro, Yair; Miller, Yifat; Levy, Joseph; Sharoni, Yoav

    2014-10-01

    Aberrant activation of the nuclear factor kappa B (NFkB) transcription system contributes to cancer progression, and has a harmful effect on bone health. Several major components of the NFkB pathway such as IkB Kinase (IKK) and the NFkB subunits contain cysteine residues that are critical for their activity. The interaction of electrophiles with these cysteine residues results in NFkB inhibition. Carotenoids, hydrophobic plant pigments, are devoid of electrophilic groups, and we have previously demonstrated that carotenoid derivatives, but not the native compounds activate the Nrf2 transcription system. The aim of the current study was to examine whether carotenoid derivatives inhibit NFkB, and, if so, to determine the molecular mechanism underpinning the inhibitory action. We report in the present study that a mixture of oxidized derivatives, prepared by ethanol extraction from partially oxidized lycopene preparation, inhibited NFkB reporter gene activity. In contrast, the intact carotenoid was inactive. A series of synthetic dialdehyde carotenoid derivatives inhibited reporter activity as well as several stages of the NFkB pathway in both cancer and bone cells. The activity of the carotenoid derivatives depended on the reactivity of the electrophilic groups in reactions such as Michael addition to sulfhydryl groups of proteins. Specifically, carotenoid derivatives directly interacted with two key proteins of the NFkB pathway: the IKKβ and the p65 subunit. Direct interaction with IKKβ was found in an in vitro kinase assay with a recombinant enzyme. The inhibition by carotenoid derivatives of p65 transcriptional activity was observed in a reporter gene assay performed in the presence of excess p65. This inhibition action resulted, at least in part, from direct interaction of the carotenoid derivative with p65 leading to reduced binding of the protein to DNA as evidenced by electrophoretic mobility shift assay (EMSA) experiments. Importantly, we found by using

  17. 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.

  18. Primary bone tumours in infants

    Energy Technology Data Exchange (ETDEWEB)

    Kozlowski, K.; Beluffi, G.; Cohen, D.H.; Padovani, J.; Tamaela, L.; Azouz, M.; Bale, P.; Martin, H.C.; Nayanar, V.V.; Arico, M.

    1985-09-01

    Ten cases of primary bone tumours in infants (1 osteosarcoma, 3 Ewing's sarcoma, 1 chondroblastoma and 5 angiomastosis) are reported. All cases of angiomatosis showed characteristic radiographic findings. In all the other tumours the X-ray appearances were different from those usually seen in older children and adolescents. In the auhtors' opinion the precise diagnosis of malignant bone tumours in infancy is very difficult as no characteristic X-ray features are present in this age period.

  19. Relationship between body mass index, bone mineral density, and oral hygiene with periodontal disease in a Mexican elderly group.

    Directory of Open Access Journals (Sweden)

    José Francisco Murrieta

    2016-05-01

    Full Text Available The aim of this study was to evaluate the relationship of body mass index (BMI, bone mineral density (BMD, and oral hygiene with periodontal disease (PD in a group of elderly adults in Mexico City. Material and Methods: A cross-sectional study with a convenience sample of 151 elderly adults was conducted. Before applying the epidemiological survey, each subject was asked to sign an informed consent. Standardization for measuring Ramfjord’s Periodontal Disease Index (PDI, BMI, and Green and Vermilion’s OHI-S was carried out. Descriptive statistics and linear regression models were performed. Results: The 93.4% of the group had PD, 33.8% showed severe gingivitis and 20.5% mild gingivitis. A 28.5% five percent of the group had osteopenia and 18.5% had osteoporosis, being more common in people over 69 years. The 38.4% percent of the group was underweight and 53.0% had poor oral hygiene. Oral hygiene accounted for 63.1% of the PD variance (p=0.0001, figure that did not increase considerably by adding BMD and BMI variables to the regression model. Conclusion: The frequency of PD in this group of elderly adults was high and significantly associated with BMD, BMI, and mainly oral hygiene.

  20. 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.

  1. The challenges of detecting circulating tumour cells in sarcoma

    OpenAIRE

    Tellez-Gabriel, M.; Brown, H K; Young, R.; Heymann, M. F.; Heymann, D

    2016-01-01

    International audience; Sarcomas are a heterogenous group of malignant neoplasms of mesenchymal origin, many of which have a propensity to develop distant metastases. Cancer cells that have escaped from the primary tumour are able to invade into surrounding tissues, to intravasate into the bloodstream to become Circulating Tumour Cells (CTCs), and are responsible for the generation of distant metastases. Due to the rarity of these tumours and the absence of specific markers expressed by sarco...

  2. Prostatic sarcoma of the Ewing family in a 33-year-old male – A case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Lukas Esch

    2016-04-01

    Full Text Available Ewing sarcoma is the second most common primary bone tumor seen in children and adolescents, typically presenting between 10 and 20 years of age. Extraosseous sarcomas of the Ewing family in adults are rare. We report a manifestation of this tumor entity in the periprostatic tissue of a 33-year-old male and discuss our treatment approach. Transrectal biopsy is a feasible and simple diagnostic tool for unclear pelvic masses. Multi-modal therapy and central registries are needed to gain knowledge of rare pelvic tumors like Ewing sarcoma.

  3. Langerhan’s cell sarcoma: two case reports

    Directory of Open Access Journals (Sweden)

    Tasneem A. Kaleem

    2016-04-01

    Full Text Available Langerhan’s cell sarcoma (LCS is a rare neoplasm with a poor prognosis. To our knowledge, only sixty-six cases have been published. We discuss two patients who presented very differently with LCS, as well as a recently published review of all sixty-six cases. Our first case had a complicated history of metastatic, high-grade myxofibrosarcomas and presented with a single skin lesion of LCS which was treated with resection to a positive margin and adjuvant radiotherapy. The LCS recurred locoregionally and was again resected. The patient is alive two years after initial diagnosis. The second case presented with bone marrow and splenic involvement, leukocytosis, and thrombocytopenia. This patient had an excellent response to etoposide, prednisone, oncovorin, cyclophosphamide, and adriamycin, with normalization of the complete blood count, negative bone marrow biopsy at follow up, and splenectomy without viable neoplasm. This patient is alive without signs of disease at 16 months after initial diagnosis.

  4. Evaluation of temporal bone pneumatization on high resolution CT (HRCT) measurements of the temporal bone in normal and otitis media group and their correlation to measurements of internal auditory meatus, vestibular or cochlear aqueduct

    Energy Technology Data Exchange (ETDEWEB)

    Nakamura, Miyako

    1988-07-01

    High resolution CT axial scans were made at the three levels of the temoral bone 91 cases. These cases consisted of 109 sides of normal pneumatization (NR group) and 73 of poor pneumatization resulted by chronic otitis (OM group). NR group included sensorineural hearing loss cases and/or sudden deafness on the side. Three levels of continuous slicing were chosen at the internal auditory meatus, the vestibular and the cochlear aqueduct, respectively. In each slice two sagittal and two horizontal measurements were done on the outer contour of the temporal bone. At the proper level, diameter as well as length of the internal acoustic meatus, the vestibular or the cochlear aqueduct were measured. Measurements of the temporal bone showed statistically significant difference between NR and OM groups. Correlation of both diameter and length of the internal auditory meatus to the temporal bone measurements were statistically significant. Neither of measurements on the vestibular or the cochlear aqueduct showed any significant correlation to that of the temporal bone.

  5. The Findings of {sup 99m}Tc-MDP Bone Scan in Primary malignant Bone Tumors

    Energy Technology Data Exchange (ETDEWEB)

    Hyun, In Young; Lee, Kung Han; Lee, Dong Soo; Chung, June Key; Lee, Myung Chul; Koh, Chang Soon; Kang, Heung Sik; Lee, Sang Hoon; Lee, Han Koo [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    1995-03-15

    Tc-99m-MDP bone scan was performed in 31 patients with primary malignant bone tumors, 22 patients with osteogenic sarcoma, 5 patients with chondrosarcoma and 4 patients with Ewing's sarcoma. The findings were classified by isotope intensity of accumulation in tumor as grade 1 to 3, overall pattern of isotope distribution in tumor as grade 1 to 3, and distortion of bony outline as grade 1 to 3. Histologic classifications were correlated with scan findings in 22 patients with osteogenic sarcoma. The results were as follows. 1) In 22 patients with osteogenic sarcoma, markedly increased isotope intensity higher than sacroiliac joint with patchy areas of decreased intensity and severe bony distortion were found in 16 patients. The correlations between histologic classification and scan findings were not discovered. 2) In 5 patients with chondrosarcoma, mildly increased isotope intensity with patchy areas of increased intensity and mild bony distortion were found in 4 patients. 3) In 4 patients with Ewing's sarcoma, markedly increased homogenous intensity with moderate bony distortion were found in 3 patients. Conclusively there were common findings in each 3 primary malignant bone tumors and Tc-99m-MDP bone scan was complemented with radiologic studies in differentiating primary malignant bone tumors.

  6. The management of clear cell sarcoma

    NARCIS (Netherlands)

    Kuiper, DR; Hoekstra, HJ; Veth, RPH; Wobbes, T

    2003-01-01

    Clear cell sarcoma is a rare soft tissue tumour, constituting approximately 1% of all soft tissue sarcomas. Prognosis is reported to be poor due to the great propensity to metastasise regionally and distantly. In this paper, we report the surgical experience of two university hospitals. Both disease

  7. Massive Pleural Effusion in an 18-Year-Old Girl with Ewing Sarcoma

    Directory of Open Access Journals (Sweden)

    Cengiz Özge

    2004-01-01

    Full Text Available Ewing sarcoma is a bone tumour that commonly appears between ages five and 10 in the diaphysis of the long bones and predominantly presents with pain and swelling. The case of an 18-year-old girl who presented with back pain, cough, dyspnea, weakness and fever is described. Chest radiograph showed a homogenous density in the middle and inferior zones of the right hemithorax. Thoracic computed tomography revealed a diffuse pleural effusion and a 6.99 cmx4.45 cm solid mass composed of lobulated, small cystic lesions and calcifications in the right hemithorax. Biochemical analysis of pleural fluid showed hemorrhagic effusion and exudate. A pleural needle biopsy demonstrated solid uniform tumour cells with narrowed cytoplasm, round nuclei and uncertain nucleoli. All of the tumour cell cytoplasms stained with CD99. The pathological examination supported Ewing sarcoma. Three-phase Tc-99m methylene diphosphonate scintigraphy of the whole body showed pathological tracer uptake in a broad area of the eighth costal bone and in smaller areas of the ninth and 10th costal bones. This case is reported because Ewing sarcoma is a rare cause of pleural effusion in clinical practice among younger adults.

  8. Development of genetically flexible mouse models of sarcoma using RCAS-TVA mediated gene delivery.

    Directory of Open Access Journals (Sweden)

    Leah Kabaroff

    Full Text Available Sarcomas are a heterogeneous group of mesenchymal malignancies and unfortunately there are limited functional genomics platforms to assess the molecular pathways contributing to sarcomagenesis. Thus, novel model systems are needed to validate which genes should be targeted for therapeutic intervention. We hypothesized that delivery of oncogenes into mouse skeletal muscle using a retroviral (RCAS-TVA system would result in sarcomagenesis. We also sought to determine if the cell type transformed (mesenchymal progenitors vs. terminally differentiated tissues would influence sarcoma biology. Cells transduced with RCAS vectors directing the expression of oncoproteins KrasG12D, c-Myc and/or Igf2 were injected into the hindlimbs of mice that expressed the retroviral TVA receptor in neural/mesenchymal progenitors, skeletal/cardiac muscle or ubiquitously (N-tva, AKE and BKE strains respectively. Disrupting the G1 checkpoint CDKN2 (p16/p19-/- resulted in sarcoma in 30% of p16/p19-/- xN-tva mice with a median latency of 23 weeks (range 8-40 weeks. A similar incidence occurred in p16/p19-/- xBKE mice (32%, however, a shorter median latency (10.4 weeks was observed. p16/p19-/- xAKE mice also developed sarcomas (24% incidence; median 9 weeks yet 31% of mice also developed lung sarcomas. Gene-anchored PCR demonstrated retroviral DNA integration in 86% of N-tva, 93% of BKE and 88% of AKE tumors. KrasG12D was the most frequent oncogene isolated. Oncogene delivery by the RCAS-TVA system can generate sarcomas in mice with a defective cell cycle checkpoint. Sarcoma biology differed between the different RCAS models we created, likely due to the cell population being transformed. This genetically flexible system will be a valuable tool for sarcoma research.

  9. 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.

  10. Penile epithelioid sarcoma: MR imaging findings

    Energy Technology Data Exchange (ETDEWEB)

    Sirikci, A.; Bayram, M.; Demirci, M. [Department of Radiology, Faculty of Medicine, Gaziantep University, Kolejtepe, Gaziantep (Turkey); Bakir, K. [Department of Pathology, Faculty of Medicine, Gaziantep University, Kolejtepe, Gaziantep (Turkey); Sarica, K. [Department of Urology, Faculty of Medicine, Gaziantep University, Kolejtepe, Gaziantep (Turkey)

    1999-10-01

    Magnetic resonance imaging findings of a 38-year-old man with epithelioid sarcoma of the penis is presented. It started as a firm, painless and slowly growing nodule at the base of his penis 6 months previously which caused pain radiating to the testis during coitus. It has been well known that sarcomas may well mimic reactive processes. Initial presentation of epithelioid sarcoma may provoke considerable diagnostic difficulty, and its differentiation from benign lesions, such as Peyronie`s disease and chronic inflammation, may be a clinical problem. In our present report the MR findings are compared with those of the epithelioid sarcomas of various locations reported in the literature and differential diagnosis of the entity is discussed. To our knowledge, this is the first report regarding the MR findings of the epithelioid sarcoma of penis. (orig.) With 3 figs., 16 refs.

  11. 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.

  12. Bone marrow oedema associated with benign and malignant bone tumours

    Energy Technology Data Exchange (ETDEWEB)

    James, S.L.J. [Department of Radiology, Royal Orthopaedic Hospital, Birmingham, B31 2AP (United Kingdom)], E-mail: steven.james@roh.nhs.uk; Panicek, D.M. [Department of Radiology, Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, New York, NY 10021 (United States); Davies, A.M. [Department of Radiology, Royal Orthopaedic Hospital, Birmingham, B31 2AP (United Kingdom)

    2008-07-15

    Bone marrow oedema is associated with a wide variety of pathological processes including both benign and malignant bone tumours. This imaging finding in relation to intraosseous tumours can aid in providing a more focused differential diagnosis. In this review, we will discuss the MR imaging of bone marrow oedema surrounding intraosseous neoplasms. The different pulse sequences used in differentiating underlying tumour from surrounding oedema are discussed along with the role of dynamic contrast enhanced MRI. Benign lesions commonly associated with bone marrow oedema include osteoid osteoma, osteoblastoma, chondroblastoma and Langerhan's cell histiocytosis. Metastases and malignant primary bone tumours such as osteosarcoma, Ewing's sarcoma and chondrosarcoma may also be surrounded by bone marrow oedema. The imaging findings of these conditions are reviewed and illustrated. Finally, the importance of bone marrow oedema in assessment of post chemotherapeutic response is addressed.

  13. Synovial Sarcoma in the Rectovesical Space: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Kil, Min Chul; Cho, Bum Sang; Han, Gi Seok; Park, Kil Sun; Kim, Sung Jin; Cha, Sang Hoon; Lee, Seung Young; Kang, MIn Ho [Dept. of Radiology, Chungbuk National University Hospital, Chunju (Korea, Republic of); Lee, Ok Jun [Dept. of Pathology, Chungbuk National University Hospital, Chunju (Korea, Republic of)

    2011-08-15

    Synovial sarcoma is an uncommon soft tissue malignancy usually arising in the extremities of young adults. Synovial sarcomas at unusual anatomic locations have been reported; however, to the best of our knowledge, there are no reports on primary synovial sarcoma in the rectovesical space. Here, we describe the radiologic findings of primary synovial sarcoma in the rectovesical space and review relevant literature.

  14. Ewing's Sarcoma and Second Malignancies

    Directory of Open Access Journals (Sweden)

    Joshua D. Schiffman

    2011-01-01

    Full Text Available Ewing's sarcoma (ES is a rare tumor that is most common in children and young adults. Late effects of ES therapy include second cancers, a tragic outcome for survivors of such a young age. This paper will explore the frequencies and types of malignancies that occur after ES. Additionally, it will review how second malignancies have changed with the shift in treatment from high-dose radiation to chemotherapy regimens including alkylators and epipodophyllotoxins. The risk of additional cancers in ES survivors will also be compared to survivors of other childhood cancers. Finally, the possible genetic contribution to ES and second malignancies will be discussed.

  15. 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.

  16. Primary osteogenic sarcoma of the breast

    Directory of Open Access Journals (Sweden)

    Akang Effiong E

    2006-12-01

    Full Text Available Abstract Background Primary extra-osseous osteogenic sarcomas have been reported in many tissues of the body but their occurrence in the breast is extremely rare. It can arise as a result of osseous metaplasia in a pre-existing benign or malignant neoplasm of the breast or as non-phylloides sarcoma from the soft tissue of a previously normal breast. Case presentation A 40 year-old Nigerian woman was clinically diagnosed to have carcinoma of the left breast. The histology report of core-needle biopsy of the mass showed a malignant neoplasm comprising islands of chondroblastic and osteoblastic stromal cells. This report changed the diagnosis from carcinoma to osteogenic sarcoma of the breast. She had a left modified radical mastectomy, however there was significant post surgery skin deficit. A latissimus dorsi musculocutaneous flap was used to cover the anterior chest wall defect. Sections from the mastectomy specimen confirmed the diagnosis of osteogenic sarcoma. She died six months after mastectomy. Conclusion A diagnosis of osteogenic sarcoma of the breast was made based on histology report and after excluding an osteogenic sarcoma arising from underlying ribs and sternum. This is the second documented case of primary osteogenic sarcoma of the breast coming from Nigeria

  17. (18)F-FDG PET/CT findings in a case with HIV (-) Kaposi sarcoma.

    Science.gov (United States)

    Ozdemir, E; Poyraz, N Y; Keskin, M; Kandemir, Z; Turkolmez, S

    2014-01-01

    Although mucocutaneous sites are the most frequently encountered sites of involvement, Kaposi Sarcoma (KS) may also occasionally involve the breast and the skeletal, endocrine, urinary and nervous systems.. Various imaging modalities may be used to delineate the extent of the disease by detecting unexpected sites of involvement. Herein, we report a case of classical type KS, in whom staging with (18)F-FDG PET/CT imaging disclosed widespread disease and unexpected findings of bone and salivary gland involvement.

  18. Pediatric Sarcoma in Central America: Outcomes, Challenges and Plans for Improvement

    Science.gov (United States)

    Friedrich, Paola; Ortiz, Roberta; Strait, Kelly; Fuentes, Soad; Gamboa, Yéssica; Arambú, Ingrid; Ah-Chu-Sanchez, María; London, Wendy; Rodríguez-Galindo, Carlos; Antillón-Klussmann, Federico; Báez, Fulgencio

    2012-01-01

    Background Children with cancer in middle-income countries have inferior outcomes to those in high-income countries. The magnitude and drivers for this survival gap are not well understood. We sought to describe patterns of clinical presentation, magnitude of treatment abandonment, and survival in children with sarcoma in Central America. Methods Retrospective review of hospital-based registries from national pediatric oncology referral centers. Patients with newly diagnosed osteosarcoma, Ewing sarcoma (Ewing), rhabdomyosarcoma (RMS), and soft tissue sarcomas (STS) between 1/1/00-12/31/09 were included. Survival analysis was performed using standard definitions of overall and event-free survival (OS and EFS) and with abandonment included as an event (AOS and AEFS). Results A total of 785 new cases of pediatric sarcoma were reported (264 osteosarcoma, 175 Ewing, 240 RMS, and 106 STS). Metastatic disease at presentation was high (osteosarcoma 38%, Ewing 39%, RMS 29% and STS 21%). Treatment abandonment rate was high, particularly among patients with extremity bone sarcomas (osteosarcoma 30%, Ewing 15%, RMS 25% and STS 15%). Of 559 patients experiencing a first event, 59% had either relapse or progressive disease. The 4-year OS was 40% (SE±3%) and EFS was 30% (SE±2%), but further decreased to 31% (SE±2%) and 24% (SE±2%), when abandonment was taken into account. Conclusion High rate of metastases and treatment abandonment, and difficulty with upfront treatment effectiveness are important contributors to poor survival of children with pediatric sarcomas in Central America. Initiatives for early diagnosis, psychosocial support, quality improvement, and multidisciplinary care are warranted to improve outcomes. PMID:22972687

  19. Isolated Granulocytic Sarcoma of the Breast after Allogeneic Stem Cell Transplantation: A Rare Involvement Also Detected by 18FDG-PET/CT

    Directory of Open Access Journals (Sweden)

    Eren Gündüz

    2014-03-01

    Full Text Available Granulocytic sarcoma is a tumor consisting of myeloid blasts with or without maturation that occurs at an anatomical site other than bone marrow. Most frequently affected sites are skin, lymph nodes, gastrointestinal tract, bone, soft tissue and testes. AML may manifest as granulocytic sarcoma at diagnosis or relapse. Although it has been considered to be rare relapse as granulocytic sarcoma after stem cell transplantation is being increasingly reported. However it is rare without bone marrow involvement and in AML M6 subtype. Breast is also a rare involvement. We report a 30-year-old woman with AML M6 relapsed 16 months after allogeneic stem cell transplantation as a granulocytic sarcoma in right breast without bone marrow involvement. She was treated with systemic chemotherapy but died of sepsis. 18FDG-PET/CT images were also obtained and detected lesions other than detected by breast ultrasound. The incidence of granulocytic sarcoma may increase if suspected or new diagnostic modalities are performed.

  20. Diagnostic dilemma in Kaposi′s sarcoma

    Directory of Open Access Journals (Sweden)

    Rao Satish

    2006-01-01

    Full Text Available Kaposi′s sarcoma is described as cutaneous and extracutaneous neoplasm predominantly affecting older individuals. Though earlier uncommon and endemic to certain African areas, its incidence is on a rise due to infections with human immunodeficiency virus and also due to transplant-associated immunosuppression. Further, certain benign conditions like Pseudo Kaposi′s sarcoma, certain infective conditions like bacillary angiomatosis of acquired immunodeficiency syndrome can mimic Kaposi′s sarcoma both clinically and histologically leading to a diagnostic dilemma. We report such a case here.

  1. [Systemic therapy and hyperthermia for locally advanced soft tissue sarcoma].

    Science.gov (United States)

    Lindner, L H; Angele, M; Dürr, H R; Rauch, J; Bruns, C

    2014-05-01

    Patients with high-risk soft tissue sarcomas (FNCLCC grades 2-3, > 5 cm and deep lying) are at a high risk of local recurrence or distant metastases despite optimal surgical tumor resection. Therefore, multimodal treatment should be considered for this difficult to treat patient group. Besides surgery, radiation therapy and chemotherapy, hyperthermia has become a valid, complementary treatment option within multimodal treatment concepts. Hyperthermia in this context means the selective heating of the tumor region to temperatures of 40-43 °C for 60 min by microwave radiation in addition to simultaneous chemotherapy or radiation therapy. A randomized phase III study demonstrated that the addition of hyperthermia to neoadjuvant chemotherapy improved tumor response and was associated with a minimal risk of early disease progression as compared to chemotherapy alone. The addition of hyperthermia to a multimodal treatment regimen for high-risk soft tissue sarcoma consisting of surgery, radiation therapy and chemotherapy, either in the neoadjuvant or adjuvant setting after incomplete or marginal tumor resection, significantly improved local progression-free and disease-free survival. Based on these results and due to the generally good tolerability of hyperthermia, this treatment method in combination with chemotherapy should be considered as a standard treatment option within multimodal treatment approaches for locally advanced high-risk soft tissue sarcoma.

  2. Intravenous Autologous Bone Marrow Mononuclear Cell Transplantation for Stroke: Phase1/2a Clinical Trial in a Homogeneous Group of Stroke Patients.

    Science.gov (United States)

    Taguchi, Akihiko; Sakai, Chiaki; Soma, Toshihiro; Kasahara, Yukiko; Stern, David M; Kajimoto, Katsufumi; Ihara, Masafumi; Daimon, Takashi; Yamahara, Kenichi; Doi, Kaori; Kohara, Nobuo; Nishimura, Hiroyuki; Matsuyama, Tomohiro; Naritomi, Hiroaki; Sakai, Nobuyuki; Nagatsuka, Kazuyuki

    2015-10-01

    The goal of this clinical trial was to assess the feasibility and safety of transplanting autologous bone marrow mononuclear cells into patients suffering severe embolic stroke. Major inclusion criteria included patients with cerebral embolism, age 20-75 years, National Institute of Health Stroke Scale (NIHSS) score displaying improvement of ≤ 5 points during the first 7 days after stroke, and NIHSS score of ≥ 10 on day 7 after stroke. Bone marrow aspiration (25 or 50 mL; N = 6 patients in each case) was performed 7-10 days poststroke, and bone marrow mononuclear cells were administrated intravenously. Mean total transplanted cell numbers were 2.5 × 10(8) and 3.4 × 10(8) cells in the lower and higher dose groups, respectively. No apparent adverse effects of administering bone marrow cells were observed. Compared with the lower dose, patients receiving the higher dose of bone marrow cells displayed a trend toward improved neurologic outcomes. Compared with 1 month after treatment, patients receiving cell therapy displayed a trend toward improved cerebral blood flow and metabolic rate of oxygen consumption 6 months after treatment. In comparison with historical controls, patients receiving cell therapy had significantly better neurologic outcomes. Our results indicated that intravenous transplantation of autologous bone marrow mononuclear cells is safe and feasible. Positive results and trends favoring neurologic recovery and improvement in cerebral blood flow and metabolism by cell therapy underscore the relevance of larger scale randomized controlled trials using this approach.

  3. Myeloid sarcoma presenting as a colon polyp and harbinger of chronic myelogenous leukemia

    Institute of Scientific and Technical Information of China (English)

    Robert Rogers; Mark Ettel; Margaret Cho; Alexander Chan; Xiao-Jun Wu; Antonio G Neto

    2016-01-01

    Myeloid sarcoma, also known as granulocytic sarcoma or chloroma is an unusual accumulation of malignant myeloid precursor cells in an extramedullary site, which disrupts the normal architecture of the involved tissue. It is known to occur more commonly in patients with acute myelogenous leukemia and less commonly in those with myelodysplastic syndrome and myeloproliferative neoplasm, such as chronic myelogenous leukemia. The most common sites of involvement include bone, skin and lymph nodes. However, rare cases have been reported in the gastrointestinal tract, genitourinary tract, or breast. Most commonly, a neoplastic extramedullary proliferation of myeloid precursors in a patient would have systemic involvement of a myeloid neoplasm, including in the bone marrow and peripheral blood. Infrequently, extramedullary disease may be the only site of involvement. It may also occur as a localized antecedent to more generalized disease or as a site of recurrence. Herein, we present the first case in the English literature of a patient presenting with an isolated site of myeloid sarcoma arising in the form of a colonic polyp which, after subsequent bone marrow biopsy, was found to be a harbinger of chronic myelogenous leukemia.

  4. Actuarial risk of isolated CNS involvement in Ewing's sarcoma following prophylactic cranial irradiation and intrathecal methotrexate

    Energy Technology Data Exchange (ETDEWEB)

    Trigg, M.E.; Makuch, R.; Glaubiger, D.

    1985-04-01

    Records of 154 patients with Ewing's sarcoma treated at the National Cancer Institute were reviewed to assess the incidence and risk of developing isolated central nervous system (CNS) Ewing's sarcoma. Sixty-two of the 154 patients had received CNS irradiation and intrathecal (i.t.) methotrexate as part of their initial therapy to prevent the occurrence of isolated CNS Ewing's sarcoma. The risk of developing isolate CNS Ewing's sarcoma was greatest within the first two years after diagnosis and was approximately 10%. The overall risk of CNS recurrence in the group of patients receiving DNS treatment was similar to the group receiving no therapy directed to the CNS. The occurrence of isolated CNS involvement was not prevented by the use of CNS irradiation and i.t. methotrexate. Because of a lack of efficacy to the CNS irradiation regimen, current treatment regimens do not include therapy directed to CNS.

  5. The role of FLI-1-EWS, a fusion gene reciprocal to EWS-FLI-1, in Ewing sarcoma

    OpenAIRE

    Elzi, David J.; Song, Meihua; Houghton, Peter J.; Chen, Yidong; Shiio, Yuzuru

    2015-01-01

    Ewing sarcoma is a cancer of bone and soft tissue in children that is characterized by a chromosomal translocation involving EWS and an Ets family transcription factor, most commonly FLI-1. The EWS-FLI-1 fusion oncogene is widely believed to play a central role in Ewing sarcoma. The EWS-FLI-1 gene product regulates the expression of a number of genes important for cancer progression, can transform mouse cells such as NIH3T3 and C3H10T1/2, and is necessary for proliferation and tumorigenicity ...

  6. The Molecular Biology of Soft-Tissue Sarcomas and Current Trends in Therapy

    Directory of Open Access Journals (Sweden)

    Jorge Quesada

    2012-01-01

    Full Text Available Basic research in sarcoma models has been fundamental in the discovery of scientific milestones leading to a better understanding of the molecular biology of cancer. Yet, clinical research in sarcoma has lagged behind other cancers because of the multiple clinical and pathological entities that characterize sarcomas and their rarity. Sarcomas encompass a very heterogeneous group of tumors with diverse pathological and clinical overlapping characteristics. Molecular testing has been fundamental in the identification and better definition of more specific entities among this vast array of malignancies. A group of sarcomas are distinguished by specific molecular aberrations such as somatic mutations, intergene deletions, gene amplifications, reciprocal translocations, and complex karyotypes. These and other discoveries have led to a better understanding of the growth signals and the molecular pathways involved in the development of these tumors. These findings are leading to treatment strategies currently under intense investigation. Disruption of the growth signals is being targeted with antagonistic antibodies, tyrosine kinase inhibitors, and inhibitors of several downstream molecules in diverse molecular pathways. Preliminary clinical trials, supported by solid basic research and strong preclinical evidence, promises a new era in the clinical management of these broad spectrum of malignant tumors.

  7. Synovial sarcoma with relevant immunocytochemistry and special emphasis on the monophasic fibrous variant

    Directory of Open Access Journals (Sweden)

    Kottu Radhika

    2010-01-01

    Full Text Available Background: Monophasic fibrous synovial sarcoma (SS is the most common variant of SS. Only a few cytological studies are available on this entity. Bcl-2 protein expression has been described as a characteristic marker of SS and is useful for its differentiation from other sarcomas. Cytokeratin and CD99 are also used in detecting SS. Aims: To evaluate synovial sarcoma and its variants cytomorphologically. Materials and Methods: During a period of 10 years 7 months, i.e. from January 1998 to July 2008, 12 cytologic specimens diagnosed as synovial sarcoma were reviewed. Ten cases were diagnosed as SS on aspiration alone but two cases required ancillary technique i.e., immunocytochemistry staining with bcl-2 and cytokeratin. The smears were stained with Papanicolaou and May-Grόnwald-Giemsa stains. Results: All cytologic specimens in our study had similar appearance. Most smears were highly cellular and were made up of densely packed tri-dimensional groups and singly scattered round to oval cells. Cellular monomorphism and vascular channels within the cell groups were the remarkable findings. Only one case showed cytologic evidence of epithelial differentiation. Bcl-2, cytokeratin, CD99 positivity was seen on immunohistochemistry staining. Results were categorized according to age, sex and morphologic variants. Conclusions: Although cytomorphologic features of synovial sarcomas are characteristic enough to permit its recognition, clinical correlation is necessary for accurate diagnosis. Monophasic variant is the most common entity observed in the present study.

  8. Ewing’s sarcoma of the maxillary sinus

    Directory of Open Access Journals (Sweden)

    Firas Nasser

    2015-07-01

    Full Text Available Ewing’s sarcoma is typically an aggressive, poorly differentiated tumor affecting children and young adults, it accounts for 4–6% of all primary bone tumors and facial primary localizations occur in only 1–4% of all cases, mostly in the mandible and calvaria. Paranasal sinus involvement is rare. A 22-year-old female was reviewed in Oral & Cranio Maxillofacial Surgery Department. She complained of swelling of the right paranasal area, of one-month duration, progressively increasing in size and associated with pain. The medical history was unremarkable, Contrast Enhanced Computed Tomography scan showed a destructive lesion of the anterior wall of the right maxillary sinus reaching up to the medial wall of the maxillary sinus, other paranasal sinus appearance was normal. Incisional biopsy proved it to be Ewing’s Sarcoma. She was treated by chemotherapy using Vincristine, Adriamycin, and Cyclophosphamide alternating with Etoposide & Ifosfamide and Radiotherapy, and this resulted in complete regression of the tumor. Repeated PET scans every 6 months did not suggest any recurrence of the right maxillary sinus tumor. We concluded that treatment by induction chemotherapy followed by radiation therapy leads to a favorable outcome in the above described case, avoiding the morbidity that can result from surgical options.

  9. 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.

  10. 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)

  11. 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 ...

  12. 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 ...

  13. Drugs Approved for Soft Tissue Sarcoma

    Science.gov (United States)

    ... 2015 2014 2013 2012 Media Resources Media Contacts Multicultural Media ... This page lists cancer drugs approved by the Food and Drug Administration (FDA) for soft tissue sarcoma. The list includes ...

  14. Acroangiodermatitis (Pseudo-Kaposi sarcoma

    Directory of Open Access Journals (Sweden)

    Satyendra Kumar Singh

    2014-01-01

    Full Text Available Acroangiodermatitis or Pseudo-Kaposi sarcoma is a rare angioproliferative entity, related to chronic venous insufficiency or certain other vascular anomalies. It is often associated with chronic venous insufficiency, arteriovenous malformation of the legs, chronic renal failure treated with dialysis, paralyzed legs and amputation stumps. We hereby describe a case of 45 year old female presenting with pitting pedal edema, multiple ulcers over bilateral lower limbs with irregular margins with erythema and hyperpigmentation of the surrounding skin. Color Doppler study of bilateral lower limbs was normal. Histopathological examination from one of the lesions showed hyperplastic epidermis, proliferation of capillaries in dermis, hemosiderin deposits and lymphocytic infiltrate. These features thus confirmed the diagnosis of Acroangiodermatitis.

  15. 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.

  16. Primary synovial sarcoma of lung

    Directory of Open Access Journals (Sweden)

    Devleena

    2014-01-01

    Full Text Available A synovial sarcoma (SS is a rare form of cancer which usually occurs near the joints of the arm, neck, or leg, but has been documented in most human tissues and organs, including the brain, prostate, and heart. Primary pulmonary SS is an extremely rare tumor. We report a case of primary SS of lung who presented with severe chest pain and a large right lung mass with right-sided pleural effusion in computed tomography (CT scan of thorax. The diagnosis was made on the basis of CT-guided core biopsy and immunohistochemistry. On immunohistochemistry, tumor cell expressed epithelial membrane antigen, bcl 2, Vimentin and smooth muscle actin and were immunonegative for S100 and cytokeratin. So, the final diagnosis was primary SS.

  17. Oncological outcome and prognostic factors in the therapy of soft tissue sarcoma of the extremities

    Directory of Open Access Journals (Sweden)

    Ingmar Ipach

    2012-11-01

    Full Text Available Uniform conclusions about therapeutic concepts and survival time of bone and soft tissue sarcoma patients are difficult due to the heterogeneity of histological subtypes as well as the different responses to neoadjuvant therapy. The subject of this retrospective study was the analysis of tumour free survival, risk and prognostic factors of sarcoma patients treated by limb sparing techniques or amputation. We included 118 patients with soft tissue sarcoma of the extremities treated primarily or secondarily at our institution between 1990 and 2008 with a minimum follow-up of 12 months. Data about the tumour free survival time, operative techniques and potential prognostic factors were analysed. The tumour-specific and overall survival were significantly influenced by two factors: the grading and distant metastases present at time of diagnosis. Optimal multimodal therapeutic concepts at a specialized Cancer Center decreased the risk of local recurrence. The importance of optimal preoperative and surgical course concerning the oncological long term outcome was investigated. The decrease in local recurrence as a result of multimodal therapeutic concepts at a specialized Cancer Center was confirmed. To evaluate the individual prognosis of a patient, multiple factors have to be considered. Factors for a poor prognosis are primary metastasis, high-grade tumours and several histological entities (e.g. synovial sarcoma, not other specified.

  18. Hypertrophic osteopathy in a cat with a concurrent injection-site sarcoma

    Directory of Open Access Journals (Sweden)

    Raquel Salgüero

    2015-07-01

    Full Text Available Case summary An 11-year old neutered female domestic shorthair cat presented for investigation of a large, partially ulcerated skin mass in the area of the left scapula. The cat had been vaccinated 6 weeks previously in the same area. Haematology showed a marked neutrophilia and monocytosis. Tru-cut biopsies were taken and histopathology was consistent with a high-grade soft tissue sarcoma. Thoracic radiographs and abdominal ultrasound revealed no abnormalities. Moderate mixed (palisading, brush border and smooth periosteal reaction was seen on the diaphysis of long bones at the time of the radiographic examination. Magnetic resonance imaging of the mass showed infiltration within deeper tissues and the owners elected euthanasia. Post-mortem examination confirmed the presence of hypertrophic osteopathy with a concurrent injection-site sarcoma. No evidence of intra-thoracic or intra-abdominal disease was found. Relevance and novel information To our knowledge, this is the first report where hypertrophic osteopathy has been described in a cat with a soft tissue sarcoma, most likely an injection-site sarcoma.

  19. 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

  20. 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.

  1. Renal Clear Cell Sarcoma - Anaplastic Variant: A Rare Entity.

    Science.gov (United States)

    Walke, Vaishali Atmaram; Shende, Nitin Y; Kumbhalkar, D T

    2017-01-01

    Clear Cell Sarcoma of Kidney (CCSK) is known for its morphologic diversity, aggressive behaviour, tendency to recur and metastasis to bone. Amongst the various morphologic subtypes, anaplastic CCSK is associated with worse prognosis. Here, we report a case of this rare variant of CCSK. A five-year-old boy presented with history of lump and pain in abdomen since one week. The Computed Tomography (CT) scan revealed a large mass occupying the middle and inferior pole of right kidney. The clinical impression was Wilms tumour. Nephrectomy specimen was received and the diagnosis of CCSK anaplastic variant was offered only after excluding the differentials and after performing ancillary tests such as Immunohistochemistry (IHC). Thus, this case emphasizes the diagnostic challenges on morphology and the essential role of IHC in arriving at a definitive diagnosis, because failure to do so may deprive the child from optimal treatment.

  2. Targeting the DNA repair pathway in Ewing sarcoma.

    Science.gov (United States)

    Stewart, Elizabeth; Goshorn, Ross; Bradley, Cori; Griffiths, Lyra M; Benavente, Claudia; Twarog, Nathaniel R; Miller, Gregory M; Caufield, William; Freeman, Burgess B; Bahrami, Armita; Pappo, Alberto; Wu, Jianrong; Loh, Amos; Karlström, Åsa; Calabrese, Chris; Gordon, Brittney; Tsurkan, Lyudmila; Hatfield, M Jason; Potter, Philip M; Snyder, Scott E; Thiagarajan, Suresh; Shirinifard, Abbas; Sablauer, Andras; Shelat, Anang A; Dyer, Michael A

    2014-11-06

    Ewing sarcoma (EWS) is a tumor of the bone and soft tissue that primarily affects adolescents and young adults. With current therapies, 70% of patients with localized disease survive, but patients with metastatic or recurrent disease have a poor outcome. We found that EWS cell lines are defective in DNA break repair and are sensitive to PARP inhibitors (PARPis). PARPi-induced cytotoxicity in EWS cells was 10- to 1,000-fold higher after administration of the DNA-damaging agents irinotecan or temozolomide. We developed an orthotopic EWS mouse model and performed pharmacokinetic and pharmacodynamic studies using three different PARPis that are in clinical development for pediatric cancer. Irinotecan administered on a low-dose, protracted schedule previously optimized for pediatric patients was an effective DNA-damaging agent when combined with PARPis; it was also better tolerated than combinations with temozolomide. Combining PARPis with irinotecan and temozolomide gave complete and durable responses in more than 80% of the mice.

  3. Prevention of bone loss with alendronate in postmenopausal women under 60 years of age. Early Postmenopausal Intervention Cohort Study Group

    DEFF Research Database (Denmark)

    Hosking, D; Chilvers, C E; Christiansen, C

    1998-01-01

    BACKGROUND: Estrogen-replacement therapy prevents osteoporosis in postmenopausal women by inhibiting bone resorption, but the balance between its long-term risks and benefits remains unclear. Whether other antiresorptive therapies can prevent osteoporosis in these women is also not clear. METHODS......: We studied the effect of 2.5 mg or 5 mg of alendronate per day or placebo on bone mineral density in 1174 postmenopausal women under 60 years of age. An additional 435 women who were prepared to receive a combination of estrogen and progestin were randomly assigned to one of the above treatments...... or open-label estrogen-progestin. The main outcome measure was the change in bone mineral density of the lumbar spine, hip, distal forearm, and total body measured annually for two years by dual-energy x-ray absorptiometry. RESULTS: The women who received placebo lost bone mineral density at all measured...

  4. 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.)

  5. MDM2 antagonist Nutlin-3a potentiates antitumour activity of cytotoxic drugs in sarcoma cell lines

    Directory of Open Access Journals (Sweden)

    Lothe Ragnhild A

    2011-05-01

    Full Text Available Abstract Background Frequent failure and severe side effects of current sarcoma therapy warrants new therapeutic approaches. The small-molecule MDM2 antagonist Nutlin-3a activates the p53 pathway and efficiently induces apoptosis in tumours with amplified MDM2 gene and overexpression of MDM2 protein. However, the majority of human sarcomas have normal level of MDM2 and the therapeutic potential of MDM2 antagonists in this group is still unclear. We have investigated if Nutlin-3a could be employed to augment the response to traditional therapy and/or reduce the genotoxic burden of chemotherapy. Methods A panel of sarcoma cell lines with different TP53 and MDM2 status were treated with Nutlin-3a combined with Doxorubicin, Methotrexate or Cisplatin, and their combination index determined. Results Clear synergism was observed when Doxorubicin and Nutlin-3a were combined in cell lines with wild-type TP53 and amplified MDM2, or with Methotrexate in both MDM2 normal and amplified sarcoma cell lines, allowing for up to tenfold reduction of cytotoxic drug dose. Interestingly, Nutlin-3a seemed to potentiate the effect of classical drugs as Doxorubicin and Cisplatin in cell lines with mutated TP53, but inhibited the effect of Methotrexate. Conclusion The use of Nutlin in combination with classical sarcoma chemotherapy shows promising preclinical potential, but since clear biomarkers are still lacking, clinical trials should be followed up with detailed tumour profiling.

  6. Genetics Home Reference: Ewing sarcoma

    Science.gov (United States)

    ... and young adults. Affected individuals usually feel stiffness, pain, swelling, or tenderness of the bone ... of genetic change, called a somatic mutation, is not inherited. The protein produced from ...

  7. 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.

  8. What Are the Risk Factors for Soft Tissue Sarcoma?

    Science.gov (United States)

    ... not been proven to cause soft tissue sarcomas. Arsenic has also been linked to a type of ... Tissue Sarcoma Causes, Risk Factors, and Prevention Early Detection, Diagnosis, and Staging Treatment After Treatment Back To ...

  9. Cutaneous myeloid sarcoma: natural history and biology of an uncommon manifestation of acute myeloid leukemia.

    Science.gov (United States)

    Hurley, M Yadira; Ghahramani, Grant K; Frisch, Stephanie; Armbrecht, Eric S; Lind, Anne C; Nguyen, Tudung T; Hassan, Anjum; Kreisel, Friederike H; Frater, John L

    2013-05-01

    We conducted a retrospective study of patients with cutaneous myeloid sarcoma, from 2 tertiary care institutions. Eighty-three patients presented, with a mean age of 52 years. Diagnosis of myeloid sarcoma in the skin was difficult due to the low frequency of myeloperoxidase and/or CD34+ cases (56% and 19% of tested cases, respectively). Seventy-one of the 83 patients (86%) had ≥ 1 bone marrow biopsy. Twenty-eight (39%) had acute myeloid leukemia with monocytic differentiation. Twenty-three had other de novo acute myeloid leukemia subtypes. Thirteen patients had other myeloid neoplasms, of which 4 ultimately progressed to an acute myeloid leukemia. Seven had no bone marrow malignancy. Ninety-eight percent of the patients received chemotherapy, and approximately 89% died of causes related to their disease. Cutaneous myeloid sarcoma in most cases represents an aggressive manifestation of acute myeloid leukemia. Diagnosis can be challenging due to lack of myeloblast-associated antigen expression in many cases, and difficulty in distinguishing monocyte-lineage blasts from neoplastic and non-neoplastic mature monocytes.

  10. Promoter Methylation Analysis Reveals that KCNA5 Ion Channel Silencing Supports Ewing Sarcoma Cell Proliferation

    Science.gov (United States)

    Ryland, Katherine E; Hawkins, Allegra G.; Weisenberger, Daniel J.; Punj, Vasu; Borinstein, Scott C.; Laird, Peter W.; Martens, Jeffrey R.; Lawlor, Elizabeth R.

    2015-01-01

    Polycomb proteins are essential regulators of gene expression in stem cells and development. They function to reversibly repress gene transcription via post-translational modification of histones and chromatin compaction. In many human cancers, genes that are repressed by polycomb in stem cells are subject to more stable silencing via DNA methylation of promoter CpG islands. Ewing sarcoma is an aggressive bone and soft tissue tumor that is characterized by over-expression of polycomb proteins. This study investigates the DNA methylation status of polycomb target gene promoters in Ewing sarcoma tumors and cell lines and observes that the promoters of differentiation genes are frequent targets of CpG-island DNA methylation. In addition, the promoters of ion channel genes are highly differentially methylated in Ewing sarcoma compared to non-malignant adult tissues. Ion channels regulate a variety of biological processes, including proliferation, and dysfunction of these channels contributes to tumor pathogenesis. In particular, reduced expression of the voltage-gated Kv1.5 channel has been implicated in tumor progression. These data show that DNA methylation of the KCNA5 promoter contributes to stable epigenetic silencing of Kv1.5 channel. This epigenetic repression is reversed by exposure to the DNA methylation inhibitor decitabine, which inhibits Ewing sarcoma cell proliferation through mechanisms that include restoration of Kv1.5 channel function. Implications This study demonstrates that promoters of ion channels are aberrantly methylated in Ewing sarcoma and that epigenetic silencing of KCNA5 contributes to tumor cell proliferation, thus providing further evidence of the importance of ion channel dyregulation to tumorigenesis. PMID:26573141

  11. Ewing sarcoma EWS protein regulates midzone formation by recruiting Aurora B kinase to the midzone.

    Science.gov (United States)

    Park, Hyewon; Turkalo, Timothy K; Nelson, Kayla; Folmsbee, Stephen Sai; Robb, Caroline; Roper, Brittany; Azuma, Mizuki

    2014-01-01

    Ewing sarcoma is a malignant bone cancer that primarily occurs in children and adolescents. Eighty-five percent of Ewing sarcoma is characterized by the presence of the aberrant chimeric EWS/FLI1 fusion gene. Previously, we demonstrated that an interaction between EWS/FLI1 and wild-type EWS led to the inhibition of EWS activity and mitotic dysfunction. Although defective mitosis is considered to be a critical step in cancer initiation, it is unknown how interference with EWS contributes to Ewing sarcoma formation. Here, we demonstrate that EWS/FLI1- and EWS-knockdown cells display a high incidence of defects in the midzone, a midline structure located between segregating chromatids during anaphase. Defects in the midzone can lead to the failure of cytokinesis and can result in the induction of aneuploidy. The similarity among the phenotypes of EWS/FLI1- and EWS siRNA-transfected HeLa cells points to the inhibition of EWS as the key mechanism for the induction of midzone defects. Supporting this observation, the ectopic expression of EWS rescues the high incidence of midzone defects observed in Ewing sarcoma A673 cells. We discovered that EWS interacts with Aurora B kinase, and that EWS is also required for recruiting Aurora B to the midzone. A domain analysis revealed that the R565 in the RGG3 domain of EWS is essential for both Aurora B interaction and the recruitment of Aurora B to the midzone. Here, we propose that the impairment of EWS-dependent midzone formation via the recruitment of Aurora B is a potential mechanism of Ewing sarcoma development.

  12. Isolated limb perfusion of soft tissue sarcomas : A comprehensive review of literature

    NARCIS (Netherlands)

    Seinen, Jojanneke M.; Hoekstra, Harald J.

    2013-01-01

    Patients with primary irresectable, locally advanced soft tissue sarcomas of the limbs form a challenging group for the treating physician. Multimodality treatment is necessary to guarantee optimal limb salvage and survival rates. Since the introduction of isolated limb perfusion in the late fifties

  13. 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, i

  14. Kaposi sarcoma following postmastectomy lymphedema.

    Science.gov (United States)

    Montero Pérez, Iria; Rodríguez-Pazos, Laura; Álvarez-Pérez, Adriana; Ferreirós, M Mercedes Pereiro; Aliste, Carlos; Suarez-Peñaranda, Jose Manuel; Toribio, Jaime

    2015-11-01

    Classical Kaposi sarcoma (KS) usually appears on lower extremities accompanied or preceded by local lymphedema. However, the development in areas of chronic lymphedema of the arms following mastectomy, mimicking a Stewart-Treves syndrome, has rarely been described. We report an 81-year-old woman who developed multiple, erythematous to purple tumors, located on areas of post mastectomy lymphedema. Histopathological examination evidenced several dermal nodules formed by spindle-shaped cells that delimitated slit-like vascular spaces with some red cell extravasation. Immunohistochemically, the human herpesvirus type 8 (HHV-8) latent nuclear antigen-1 was detected in the nuclei of most tumoral cells confirming the diagnosis of KS. Lymphedema could promote the development of certain tumors by altering immunocompetence. Although angiosarcoma (AS) is the most frequent neoplasia arising in the setting of chronic lymphedema, other tumors such as benign lymphangiomatous papules (BLAP) or KS can also develop in lymphedematous limbs. It is important to establish the difference between AS and KS because their prognosis and treatment are very different. Identification by immunohistochemistry of HHV-8 is useful for the distinction between KS and AS or BLAP.

  15. 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.)

  16. Gender and age groups interactions in the quantification of bone marrow fat content in lumbar spine using 3T MR spectroscopy: A multivariate analysis of covariance (Mancova)

    Energy Technology Data Exchange (ETDEWEB)

    Roldan-Valadez, Ernesto, E-mail: ernest.roldan@usa.net [Magnetic Resonance Unit, Medica Sur Clinic and Foundation, Mexico City (Mexico); Piña-Jimenez, Carlos [Magnetic Resonance Unit, Medica Sur Clinic and Foundation, Mexico City (Mexico); Favila, Rafael [GE Healthcare, Mexico City (Mexico); Rios, Camilo [Neurochemistry Department, Mexican National Institute of Neurology and Neurosurgery, Mexico City (Mexico)

    2013-11-01

    Introduction: There is an age-related conversion of red to yellow bone marrow in the axial skeleton, with a gender-related difference less well established. Our purpose was to clarify the variability of bone marrow fat fraction (FF) in the lumbar spine due to the interaction of gender and age groups. Methods: 44 healthy volunteers (20 males, 30–65 years old and 24 females, 30–69 years old) underwent 3T magnetic resonance spectroscopy (MRS) and conventional MRI examination of the lumbar spine; single-voxel spectrum was acquired for each vertebral body (VB). After controlling body mass index (BMI), a two-way between-groups multivariate analysis of covariance (MANCOVA) assessed the gender and age group differences in FF quantification for each lumbar VB. Results: There was a significant interaction between gender and age group, p = .017, with a large effect size (partial η{sup 2} = .330). However the interaction explained only 33% of the observed variance. Main effects were not statistically significant. BMI was non-significantly related to FF quantification. Conclusions: Young males showed a high FF content, which declined in the 4th decade, then increased the next 3 decades to reach a FF content just below the initial FF means. Females’ FF were low in the 3rd decade, depicted an accelerated increase in the 4th decade, then a gradual increase the next 3 decades to reach a FF content similar to males’ values. Our findings suggest that quantification of bone marrow FF using MRS might be used as a surrogate biomarker of bone marrow activity in clinical settings.

  17. 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.

  18. Sarcomas mandibulares: experiencia quirúrgica en los últimos 10 años Mandibular sarcomas: surgical experience over the past 10 years

    Directory of Open Access Journals (Sweden)

    Javier Gutiérrez Santamaría

    2012-09-01

    Full Text Available Introducción: Los sarcomas mandibulares representan una entidad de difícil estudio por su escasa incidencia e histopatología. Pacientes y métodos: Presentamos la experiencia del servicio de Cirugía Oral y Maxilofacial del Hospital Vall d'Hebron de Barcelona en los últimos 10 años (2001-2010 en el manejo de los sarcomas mandibulares, realizando una revisión retrospectiva de 12 casos de pacientes afectos por este tipo de tumor. Resultados: La técnica más utilizada para la reconstrucción fue el colgajo microvascularizado (hueso peroné: 8/12, recibiendo tratamiento adyuvante (quimioterapia y/o radioterapia el 82% de los pacientes. Cinco pacientes fallecieron (42%, 2 se encuentran con progresión de la enfermedad (16% y 5 sobreviven libres de enfermedad (42% hasta la finalización del seguimiento. Conclusiones: Los casos descritos representan una serie singular debido a la localización mandibular, no antes publicadas en la literatura. Aún así, los resultados obtenidos en términos de supervivencia y factores pronóstico son similares a los descritos para los sarcomas de cabeza y cuello. La consecución de márgenes libres con la cirugía es la clave del tratamiento, siendo necesario el tratamiento complementario para mejorar el pronóstico.Introduction: Sarcomas located in the mandible are difficult to study due to their relatively rare appearance and histology. Patients and Methods: We present the experience of the Oral and Maxillofacial Surgery Department of the Vall d'Hebron Hospital in Barcelona over the last 10 years (2001-2010 in the management of jaw sarcomas, performing a retrospective review of 12 cases of patients affected by this type of tumour. Results: The technique mostly used for the reconstruction was the microvascularised bone graft (fibula: 8/12, with 82% of the patients receiving adjuvant therapy (chemotherapy and radiotherapy. Five of the patients died (42%, two were found with disease progression (16%, and 5

  19. Bone tumor

    Science.gov (United States)

    Tumor - bone; Bone cancer; Primary bone tumor; Secondary bone tumor; Bone tumor - benign ... The cause of bone tumors is unknown. They often occur in areas of the bone that grow rapidly. Possible causes include: Genetic defects ...

  20. 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...

  1. Clinical Pathological Analysis of Synovial Sarcoma

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    OBJECTIVE To investigate the clinical diagnosis and differential diagnosis of synovial sarcoma (SS).METHODS A total of 41 paraffin-embedded synovial sarcoma samples were examined by H&E staining, immunohistochemistry staining and the reverse transcriptase polymerase chain reaction (RT-PCR), in order to provide a scientific bases for diagnosis and differential diagnosis.RESULTS Twelve cases were a biphasic type, 22 cases were a monophasic fibrous type, and 7 cases were a poorly differentiated type. Thirty-six cases were both CK (and/or EMA) and Vim positive. Five cases were only Vim positive. A SYT-SSX fusion gene was detected in 18 cases by RT-PCR.CONCLUSION By observation of the histomorphology, immunohistochemistry markers and detection of a SYT-SSX fusion gene, we can make a clinical pathological diagnosis of synovial sarcoma.

  2. 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.)

  3. The association of dietary quality and food group intake patterns with bone health status among Korean postmenopausal women: a study using the 2010 Korean National Health and Nutrition Examination Survey Data

    OpenAIRE

    Go, Gyeongah; Tserendejid, Zuunnast; Lim, Youngsook; Jung, Soyeon; Min, Younghee; Park, Haeryun

    2014-01-01

    BACKGROUND/OBJECTIVES Osteoporosis and osteoporosis-related disease are drawing a lot of attention in Korea as one of the serious health problems. Bone health status may be influenced by the general dietary quality and dietary pattern. SUBJECTS/METHODS To determine the relationship between dietary quality and intake patterns and bone health status, the %RNI, NAR, DDS, and food group intake patterns were assessed according to their bone health status for 847 postmenopausal women using the 2010...

  4. 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.

  5. 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.

  6. Primary Renal Synovial Sarcoma: An Oncologic Surprise

    Directory of Open Access Journals (Sweden)

    H. Krishna Moorthy

    2014-09-01

    Full Text Available Primary renal synovial sarcoma is a rare tumor having a specific chromosomal translocation t(X; 18 (p11.2; q11.2. The clinical features of this tumor and radiologic appearances are quite similar to those of renal cell carcinoma. Confirmatory diagnosis requires fluorescent in situ hybridization or reverse transcriptase polymerase chain reaction validation for differentiating the tumors from sarcomatoid renal cell carcinoma. We present a case of primary renal synovial sarcoma that was diagnosed in a middle-aged man.

  7. Amputation for histiocytic sarcoma in a cat.

    Science.gov (United States)

    Teshima, Takahiro; Hata, Takashi; Nezu, Yoko; Michishita, Masaki; Matsumoto, Hirotaka; Mizutani, Hisashi; Takahashi, Kimimasa; Koyama, Hidekazu

    2012-02-01

    A 9-year-old spayed female domestic shorthair cat presented with a skin lesion of the left tarsus. The lesion was biopsied and, based on the microscopic appearance and immunohistochemical characteristics, histiocytic sarcoma was diagnosed. Amputation was performed with improved demeanor seen postoperatively. However, between 44 and 60 days following the surgery, relapse of skin lesions appeared in multiple locations, including at the previous amputation site, and euthanasia was elected. This is the first report of a histiocytic sarcoma treated with amputation in a cat.

  8. 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.

  9. Molecular piracy of Kaposi's sarcoma associated herpesvirus.

    Science.gov (United States)

    Choi, J; Means, R E; Damania, B; Jung, J U

    2001-01-01

    Kaposi's Sarcoma associated Herpesvirus (KSHV) is the most recently discovered human tumor virus and is associated with the pathogenesis of Kaposi's sarcoma, primary effusion lymphoma, and Multicentric Casttleman's disease. KSHV contains numerous open reading frames with striking homology to cellular genes. These viral gene products play a variety of roles in KSHV-associated pathogenesis by disrupting cellular signal transduction pathways, which include interferon-mediated anti-viral responses, cytokine-regulated cell growth, apoptosis, and cell cycle control. In this review, we will attempt to cover our understanding of how viral proteins deregulate cellular signaling pathways, which ultimately contribute to the conversion of normal cells to cancerous cells.

  10. MAPPING OF NATURAL KAPOSI SARCOMA INHIBITOR USING NETWORK BIOLOGY APPROACH

    Directory of Open Access Journals (Sweden)

    Jayadeepa R. M.

    2012-03-01

    Full Text Available Identification of protein-ligand interaction networks on a proteome scale is crucial to address a wide range of biological problems such as correlating molecular functions to physiological processes and designing safe and efficient therapeutics. In this study we have developed a novel computational strategy to identify ligand binding profiles of proteins across gene families and applied it to predicting protein functions, elucidating molecular mechanisms of drug adverse effects, and repositioning safe pharmaceuticals to treat different diseases The resultant network is then extrapolated to proteomics level to sort out the genes only expressed in the specific cancer types. The network is statistically analyzed and represented by the graphical interpretation to encounter the hub nodes. The objective of developing a biological networking is for the evaluation and validation of cancer drugs and their targets. In the field of cancer biology, the drug and their targets holds a role of paramount importance. With the work conducted here it shows the study of relation between drug target networks. Kaposi’s sarcoma (KS is a systemic disease which can present with cutaneous lesions with or without internal involvement. Genes belonging to the group of proto-oncogenes and tumor suppressors are best targeted for cancer studies. Biological networks like gene regulatory networks, protein interaction network is usually created to simplify the studies. In the present study, 26 proteins as receptor were selected for the study; all the receptors were responsible for the cause of Kaposi’s sarcoma. Also, 121 natural anti-Kaposi Sarcoma compounds were selected from different sources the natural components were the best component for blocking of abnormal activity.

  11. The role of imaging for translational research in bone tumors

    Energy Technology Data Exchange (ETDEWEB)

    Benassi, Maria Serena, E-mail: mariaserena.benassi@ior.it [Laboratory of Experimental Oncology, Istituto Ortopedico Rizzoli, Via di Barbiano 1/10, 40136 Bologna (Italy); Rimondi, Eugenio, E-mail: eugenio.rimondi@ior.it [Radiology, Istituto Ortopedico Rizzoli, Bologna (Italy); Balladelli, Alba, E-mail: alba.balladelli@ior.it [Laboratory of Experimental Oncology, Istituto Ortopedico Rizzoli, Via di Barbiano 1/10, 40136 Bologna (Italy); Ghinelli, Cristina, E-mail: cristina.ghinelli@ior.it [Laboratory of Experimental Oncology, Istituto Ortopedico Rizzoli, Via di Barbiano 1/10, 40136 Bologna (Italy); Magagnoli, Giovanna, E-mail: giovanna.magagnoli@ior.it [Laboratory of Experimental Oncology, Istituto Ortopedico Rizzoli, Via di Barbiano 1/10, 40136 Bologna (Italy); Vanel, Daniel, E-mail: daniel.vanel@ior.it [Bone Tumor Center, Istituto Ortopedico Rizzoli, Bologna (Italy)

    2013-12-01

    Sarcomas are a heterogeneous group of rare connective tissue tumors, representing 1% of adult and 15% of childhood cancers for which biological and pathological information is still incomplete. In bone tumors patients with metastatic disease at onset, those who relapse and those with post-surgical secondary lesions still have a dismal outcome because of poor response to current therapies. Different molecular biology approaches have identified activated cell signalling pathways or specific molecular endpoints that may be considered potential drug targets or markers useful for diagnosis/prognosis in musculoskeletal pathology. Recently, advances in the field of molecular imaging allow visualization of cell and metabolic functions with the use of targets that include cell membrane receptors, enzymes of intracellular transport. Moreover advanced non-invasive newer imaging techniques like 18-FDG PET, quantitative dynamic-contrast MR imaging, diffusion weighted imaging have all shown a potential in distinguish malignant from benign lesions, in revealing the efficacy of therapy in tumors, the onset of recurrence and a good reliability in reckoning the percentage of necrosis in Ewing sarcoma and osteosarcoma. Thus, in vivo detection of imaging cancer biomarkers may be useful to better characterize those complex pathologic processes, such as apoptosis, proliferation and angiogenesis that determine tumor aggressiveness, providing not only complementary information of prognostic metabolic indicators, but also data in real-time on the efficacy of the treatment through the modulation of the cell metabolism.

  12. Primary Ewing's sarcoma of the vertebral column

    Energy Technology Data Exchange (ETDEWEB)

    Ilaslan, Hakan; Sundaram, Murali [Department of Radiology, Mayo Clinic, Ch2-290 200 First Street, SW, Rochester, 55905, MN (United States); Unni, K.Krishnan [Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 First Street, SW, 55905, Rochester, MN (United States); Dekutoski, Mark B. [Department of Orthopedic Surgery, Mayo Clinic, 200 First Street, SW, 55905, Rochester, MN (United States)

    2004-09-01

    To determine the demographics, imaging findings, clinical symptoms, and prognosis of primary vertebral Ewing's sarcoma (PVES). A retrospective review of medical records and radiological studies of patients diagnosed with PVES from 1936 through 2001 in our institution and Department of Pathology consultation files was undertaken. Metastatic and soft tissue Ewing's sarcoma cases were excluded. From a total of 1,277 cases of Ewing's sarcoma, 125 (9.8%) had a primary vertebral origin. There were 48 females and 76 males. Patient ages ranged from 4 to 54 (mean 19.3, standard deviation 10.7, median 16) years. Vertebral column distribution was four cervical (3.2%), 13 thoracic (10.5%), 31 lumbar (25%), and 67 sacrum (53.2%). More than one vertebral segment was involved in ten cases (8%). Satisfactory imaging studies were available in 51 patients: 49 radiographs, 27 computerized tomography (CT), and 23 magnetic resonance imaging (MRI) studies. The majority of tumors were lytic (93%). Three cases were mixed lytic and sclerotic (6%) and one sclerotic. In the nonsacral spine, the majority of lesions (12/20) involved the posterior elements with extension into the vertebral body. Five cases were centered in the vertebral body with extension into the posterior elements. Two cases were limited to the posterior elements, and one case solely involved the vertebral body. Ala was the most frequently affected site in the sacrum (18/26). Spinal canal invasion was frequent (91%). Detailed clinical information was available in 53 patients. Duration of symptoms ranged from 1 to 30 (mean 7) months. Local pain was the first symptom and seen in all cases. Neurological deficits were present in 21 (40%) cases. All patients received radiation in various dosages; 70% additionally received chemotherapy. Twenty-five patients had surgery, and two patients received bone marrow transplantation. Forty-five patients had follow-up; the five-year disease-free survival probability is 0

  13. Treatment Option Overview (Childhood Soft Tissue Sarcoma)

    Science.gov (United States)

    ... first formed. This summary is about the following types of soft tissue sarcoma: Fat tissue tumors Liposarcoma . This is a rare cancer of the fat cells. Liposarcoma usually forms in the fat layer just under the skin. In ... types of liposarcoma. Myxoid liposarcoma is usually low grade ...

  14. General Information about Childhood Soft Tissue Sarcoma

    Science.gov (United States)

    ... first formed. This summary is about the following types of soft tissue sarcoma: Fat tissue tumors Liposarcoma . This is a rare cancer of the fat cells. Liposarcoma usually forms in the fat layer just under the skin. In ... types of liposarcoma. Myxoid liposarcoma is usually low grade ...

  15. Treatment Options for Childhood Soft Tissue Sarcoma

    Science.gov (United States)

    ... first formed. This summary is about the following types of soft tissue sarcoma: Fat tissue tumors Liposarcoma . This is a rare cancer of the fat cells. Liposarcoma usually forms in the fat layer just under the skin. In ... types of liposarcoma. Myxoid liposarcoma is usually low grade ...

  16. Stages of Childhood Soft Tissue Sarcoma

    Science.gov (United States)

    ... first formed. This summary is about the following types of soft tissue sarcoma: Fat tissue tumors Liposarcoma . This is a rare cancer of the fat cells. Liposarcoma usually forms in the fat layer just under the skin. In ... types of liposarcoma. Myxoid liposarcoma is usually low grade ...

  17. 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

  18. 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

  19. Extraosseus Ewing sarcoma: An uncommon periclavicular location

    Directory of Open Access Journals (Sweden)

    Fabrizio Albarello, MD

    2015-01-01

    Full Text Available A rapidly enlarging right sternoclavicular mass in a young male was labeled as a nonspecific mass. MRI played a crucial role in characterizing the lesion, helping to define the possible mesenchymal origin and the relative involvement of the surrounding structures. We also discuss the differential diagnosis of an extraosseus Ewing sarcoma (ES, with its imaging findings.

  20. 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.

  1. Primary pleomorphic sarcoma of the liver

    Energy Technology Data Exchange (ETDEWEB)

    Mani, S.; Naik, L.; Shet, S.; Vora, I.M.; Rananavare, R. [BYL Nail Hospital, Bombay (India). Departments of Radiology and Pathology

    1998-02-01

    A 35-year-old woman presented with abdominal distension and a palpable liver mass. Ultrasonography and computed tomography revealed a large well-delineated liver mass with bilobar involvement. Based on autopsy and immunohistochemical findings, a final diagnosis of primary pleomorphic liver sarcoma with myogenic differentiation W established. Copyright (1998) Blackwell Science Pty Ltd 8 refs., 5 figs.

  2. 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

  3. 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

  4. 3 cases of radiation-induced sarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Shiba, K.; Fukuma, H.; Beppu, Y.; Hirota, T. (National Cancer Center, Tokyo (Japan). Hospital); Shinohara, N.

    1982-03-01

    Criteria for the diagnosis of radiation-induced sarcoma have been previously described. All cases must have a history of irradiation and the second neoplasm must have arisen in the area of the radiation field. A latent period of several years must have elapsed after irradiation before clinical evidence of a second malignant neoplasm. Most important thing is that, all suspected cases must have been proved histologically. We have experienced 3 cases of radiation-induced sarcoma, they were 42-years-old man who developed an osteosarcoma of the lumbar spine at the field of postoperative irradiation for seminoma 7 years previously, 69-years-old woman who developed a malignant fibrous histiocytoma of the buttock at the field of radical radiation for uterine carcinoma 7 years previously and 59-years-old woman who developed an extraskeletal osteosarcoma of the abdominal wall at the field of postoperative irradiation for uterine sarcoma 7 years previously. The last case is very rare and only 8 cases of radiation-induced extraskeletal osteosarcoma have been reported. Since there has been a definite trend in the treatment of cancer toward employing radiation for more favorable cases, in addition to technical improvements in the administration of radiotherapy and more modern equipment, survival data may have been altered considerably in many malignant tumors. Accordingly, more radiation-induced tumors may be encountered in the future. The clinical presentation and histopathology of these radiation-induced sarcomas are presented with a review of the literature.

  5. Immunosuppressive Therapy-Related 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, ...

  6. Feline postvaccinal sarcoma: 20 years later.

    Science.gov (United States)

    Wilcock, Brian; Wilcock, Anne; Bottoms, Katherine

    2012-04-01

    Comparison of the annual prevalence of feline postvaccinal sarcomas among 11 609 feline skin mass submissions from 1992 to 2010 revealed no decrease in disease prevalence or increase in the age of affected cats in response to changes in vaccine formulation or recommended changes in feline vaccination protocols.

  7. Feline postvaccinal sarcoma: 20 years later

    OpenAIRE

    Wilcock, Brian; Wilcock, Anne; Bottoms, Katherine

    2012-01-01

    Comparison of the annual prevalence of feline postvaccinal sarcomas among 11 609 feline skin mass submissions from 1992 to 2010 revealed no decrease in disease prevalence or increase in the age of affected cats in response to changes in vaccine formulation or recommended changes in feline vaccination protocols.

  8. 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

  9. Intra-articular epithelioid sarcoma showing mixed classic and proximal-type features: report of 2 cases, with immunohistochemical and molecular cytogenetic INI-1 study.

    Science.gov (United States)

    Kosemehmetoglu, Kemal; Kaygusuz, Gulsah; Bahrami, Armita; Raimondi, Susana C; Kilicarslan, Kasim; Yildiz, Yusuf; Folpe, Andrew L

    2011-06-01

    Epithelioid sarcoma, a rare sarcoma with epithelial differentiation, most often occurs in the distal extremities; however, it may occur in essentially any location. With the recent recognition that the loss of expression of the tumor-suppressor gene INI-1 may be associated with epithelioid sarcoma, it has become clear that epithelioid sarcoma may occur in previously unsuspected locations such as bone. Only 2 cases of intra-articular epithelioid sarcoma have been previously reported. We retrieved 2 intra-articular cases coded as epithelioid sarcoma from our archives. Both expressed cytokeratins (AE1/AE3 and OSCAR), CD34, vimentin, and epithelial membrane antigen, and showed complete loss of expression of INI-1. Fluorescence in situ hybridization was performed on formalin-fixed, paraffin-embedded sections by using a laboratory-developed dual-color probe containing INI1 (CTD-2511E13 and CTD-2034E7) (22q11.2) (OR) and PANX2 (RPCI3-402G11) (22q13.33) (GR) probes as control. Both cases occurred in a clearly intra-articular location in the knee. Case 1 was that of a 19-year-old man with a long-standing history of pain and limited joint function. This patient was disease free after amputation. Case 2 was that of a 60-year-old woman. Follow-up information available for this patient showed bilateral subpleural metastases. Morphologically, case 1 showed features of proximal-type epithelioid sarcoma, whereas case 2 showed mixed features of classic and proximal-type epithelioid sarcoma. Immunohistochemistry showed complete loss of INI-1 protein in both cases; fluorescence in situ hybridization analyses were negative for INI-1 gene deletion. Herein, we have reported 2 cases of intra-articular epithelioid sarcoma, showing morphologic and immunohistochemical features identical to those of epithelioid sarcoma in conventional locations, including loss of INI-1 expression. Intra-articular epithelioid sarcoma should be distinguished from malignant pigmented villonodular synovitis and

  10. Case Report: Myelodysplastic syndrome- associated myeloid sarcoma: an unusual clinical presentation of a rare disease.

    Science.gov (United States)

    Horvath, Emoke; Demian, Smaranda; Nagy, Elod

    2016-01-01

    Myeloid sarcoma results from the extramedullary homing and proliferation of immature myeloid precursors. We present the timeline, events and diagnostic pitfalls related to a 66 year-old male patient's case, admitted to the Hematology Clinic for pancytopenia, fever, weight loss and fatigue. The severe cytopenia and the few blasts observed in his blood smear indicated a bone marrow biopsy. The bone marrow showed hypercellularity and multilineage dysplasia with the presence of 15% myeloblasts. After the biopsy, he promptly developed paraplegia and nuclear magnetic resonance revealed an epidural tumour which was then resected.In the epidural tumour mass blast-like, round cells were observed with a complex immunophenotype, characterized by myeloperoxidase, CD117, CD15, CD99, leucocyte common antigen positivity and a high Ki-67 proliferation index. Considering the main differential diagnostic issues, the final diagnosis was stated as myelodysplastic syndrome-associated myeloid sarcoma. The prognosis was unfavourable, the bone marrow was quickly invaded by proliferating blast cells, and despite chemotherapy attempts, the patient died.

  11. 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.

  12. Primary Vaginal Myeloid Sarcoma: A Rare Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Gaurang Modi

    2015-01-01

    Full Text Available Myeloid sarcoma (chloroma, granulocytic sarcoma, or extramedullary myeloid tumour is an extramedullary mass forming neoplasm composed of myeloid precursor cells. It is usually associated with myeloproliferative disorders but very rarely may precede the onset of leukemia. Here, we are presenting a rare case of primary vaginal myeloid sarcoma in a geriatric female patient without initial presentation of acute myeloid leukemia (AML. A 68-year-old female patient with ECOG Performance Score of 1 presented with pervaginal bleeding for 20 days. On colposcopic examination, she was found to have mass in the anterior fornix of vagina. A punch biopsy specimen revealed chloromatous infiltration of the vagina. LCA (leukocyte common antigen, MPO (myeloperoxidase, and c-kit were strongly positive on IHC (immunohistochemistry. The patient’s routine blood investigations were normal including peripheral smear, lactose dehydrogenase, uric acid, 2D echocardiography, conventional cytogenetics, bone marrow aspiration, and biopsy. The patient was given 4 cycles of decitabine (Decitex, manufactured by Sun Pharmaceutical Industries Limited, India, 20 mg/m2 for 5 days at an interval of 28 days. There was a partial response to decitabine according to RECIST criteria. As decitabine therapy was well tolerated, we are continuing in the same way until disease progression without any complications. The patient is undergoing regular follow-up at our centre.

  13. [Clear-cell sarcoma of the kidney: about a paediatric case].

    Science.gov (United States)

    Namaoui, R Y; Castex, M P; Vial, J; Galinier, P; Rubie, H; Laprie Mazieres, A; Le Mandat, A; Brousset, P; Delsol-Tahou, M

    2010-06-01

    Clear-cell sarcoma of the kidney (CCSK) is a rare malignant tumor of childhood, known for its aggressiveness, its tendency to recurrence and to metastasis to bone. We report an observation of a child of 48 months carrying a large abdominal mass. The diagnosis of the SCCR was made on biopsy, since imaging remained uncertain as to the renal origin of the mass. Indeed, our observation underlines the difficulty of its diagnosis. Excepting the morphological aspect, there is no criterion for its recognition. Its prognosis has been improved by the new treatments.

  14. 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

  15. Index of CD34+ Cells and Mononuclear Cells in the Bone Marrow of Spinal Cord Injury Patients of Different Age Groups: A Comparative Analysis

    Directory of Open Access Journals (Sweden)

    Vidyasagar Devaprasad Dedeepiya

    2012-01-01

    Full Text Available Introduction. Recent evidence of safety and efficacy of Bone Marrow Mononuclear Cells (BMMNC in spinal cord injury makes the Bone Marrow (BM CD34+ percentage and the BMMNC count gain significance. The indices of BM that change with body mass index and aging in general population have been reported but seldom in Spinal Cord Injury (SCI victims, whose parameters of relevance differ from general population. Herein, we report the indices of BMMNC in SCI victims. Materials and Methods. BMMNCs of 332 SCI patients were isolated under GMP protocols. Cell count by Trypan blue method and CD34+ cells by flow cytometry were documented and analysed across ages and gender. Results. The average BMMNC per ml in the age groups 0–20, 21–40, 41–60, and 61–80 years were 4.71, 4.03, 3.67, and 3.02 million and the CD34+ were 1.05%, 1.04%, 0.94%, and 0.93% respectively. The decline in CD34+ was sharp between 20–40 and 40–60 age groups. Females of reproductive age group had lesser CD34+. Conclusion. The BMMNC and CD34+ percentages decline with aging in SCI victims. Their lower values in females during reproductive age should be analysed for relevance to hormonal influence. This study offers reference values of BMMNC and CD34+ of SCI victims for successful clinical application.

  16. Different subtypes of epithelioid sarcoma and their clinical implication: long-term multi-institutional experience with a rare sarcoma.

    Science.gov (United States)

    Kim, Chan; Yoo, Kwai Han; Kim, Min Hwan; Chon, Hong Jae; Lee, Soon Il; Lee, Hyo Jin; Koh, Sujin; Lee, Ha Yeon; Lee, Hye Ran; Kim, Kyung Sik; Choi, Young Deuk; Rha, Sun Young; Lee, Su Jin; Kim, Hyo Song

    2017-03-01

    Epithelioid sarcoma (ES) is a very rare soft-tissue sarcoma with a high tendency of recurrence and metastasis. We analyzed clinical features of ES and aimed to identify the potential role of radio- and chemotherapy in ES. Fifty-five patients diagnosed with ES between 1997 and 2014 were enrolled from seven tertiary hospitals in Korean Cancer Research Group. The clinical variables were retrospectively reviewed and analyzed. Forty-six (84%) patients underwent surgical resection of ES, and among them, 27 experienced recurrence. In these patients, resection margin status and adjuvant radiotherapy were independent prognostic factors for longer recurrence-free survival (RFS), while adjuvant chemotherapy did not influence RFS. Twenty-two (40%) patients received palliative chemotherapy for metastatic or recurrent ES, and in these patients, palliative chemotherapy was the only independent prognostic factor for longer overall survival. Intriguingly, the clinical benefit of radio- and chemotherapy was observable only in proximal ES, but not in extremity ES, indicating that subtypes of ES might respond to radio- or chemotherapy differently. Proximal ES seems to benefits more from active anticancer treatment than conventional extremity ES. The aggressive characteristics of proximal ES could be overcome with an optimal multimodal treatment.

  17. 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.

  18. Mediastinal nonleukemic granulocytic sarcoma with cardiac infiltration Sarcoma granulocítico mediastinal não associado à leucemia com infiltração cardíaca

    Directory of Open Access Journals (Sweden)

    Gabrielle G. Lima

    2008-08-01

    Full Text Available We report on a case of mediastinal granulocytic sarcoma with cardiac infiltration in a young man with no evidence of leukemia involving the bone marrow or peripheral blood. Diagnosis was accomplished by immuno-histochemistry with expressions of myeloperoxidase and CD99 antigens. The patient achieved clinical remission, but evolved with febrile neutropenia during chemotherapy and died. Although subclinical cardiac infiltrations are commonly found at autopsy in patients with acute non-lymphoblastic leukemia, only one case of involvement of the heart with granulocytic sarcoma in the absence of bone marrow disease has been published in the literature. A diagnosis of granulocytic sarcoma should not be excluded when the biopsy of the bone marrow does not show any evidence of leukemic infiltration.Relata-se o caso de um adulto jovem com sarcoma granulocítico (SG mediastinal com infiltração cardíaca sem evidência de leucemia envolvendo medula óssea ou sangue periférico. O diagnóstico foi revelado pela imuno-histoquímica com positividade para mieloperoxidase e CD99. O paciente apresentou remissão clínica, porém evoluiu com neutropenia febril durante a quimioterapia e foi a óbito. Embora infiltrados cardíacos subclínicos sejam comumente detectados na autópsia em pacientes com leucemia aguda nãolinfoblástica, somente um caso de SG com envolvimento cardíaco na ausência de doença na medula óssea foi descrito na literatura. Um diagnóstico de SG não deve ser excluída quando a biópsia da medula óssea não mostrar nenhuma evidência de infiltração leucêmica.

  19. Cranial bones and atlas of titanosaurs (Dinosauria, Sauropoda) from Late Cretaceous (Bauru Group) of Uberaba, Minas Gerais State, Brazil

    Science.gov (United States)

    Martinelli, Agustín G.; Marinho, Thiago da Silva; Filippi, Leonardo S.; Ribeiro, Luiz Carlos Borges; Ferraz, Mara Lúcia da Fonseca; Cavellani, Camila Lourencini; Teixeira, Vicente de Paula Antunes

    2015-08-01

    Isolated left prefrontal, left squamosal and atlas of titanosaur dinosaurs are described and compared. They come from the Late Cretaceous Serra da Galga Member of the Marília Formation at the Serra do Veadinho region, Peirópolis (Uberaba County, Minas Gerais State, Brazil). Due to the sparse cranial elements of titanosaurs already known from Brazil, these specimens are noticeable to be presented. In addition, the atlas vertebra is described for the first time for Brazilian titanosaurs. The morphology of the cranial bones closely resembles lithostratian titanosaurs, such as Rapetosaurus, rather than basal titanosaurs. The atlas is similar to that of other titanosaurs, suggesting that the anatomy of this element seems to be more conservative than other vertebral elements, in which vertebral laminae play an important rule in titanosaur taxonomy.

  20. 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...

  1. Radiation-induced prostatic sarcoma: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Scully, J.M.; Uno, J.M.; McIntyre, M.; Mosely, S. (Bay Area Hospital, Coos Bay, OR (USA))

    1990-09-01

    A 64-year-old man had a prostatic sarcoma 8 years after transurethral prostatectomy and radical bilateral pelvic lymph node dissection with insertion of 125-iodine implants for stage B1N carcinoma of the prostate. Therapy for the sarcoma consisted of isolated pelvic perfusion and then pelvic exenteration with creation of an ileal conduit and colostomy. The pathology report showed well encapsulated grade 2 spindle cell sarcoma of the prostate. Multiple small metallic particles were embedded in the tumor specimen.

  2. 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.)

  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. Effects of inhaled plutonium nitrate on bone and liver in dogs

    Energy Technology Data Exchange (ETDEWEB)

    Dagle, G.E.; Weller, R.E.; Watson, C.R.; Buschbom, R.L. [Pacific Northwest Lab., Richland, WA (United States). Biology and Chemistry Dept.

    1994-04-01

    The life-span biological effects of inhaled soluble, alpha-emitting radionuclides deposited in the skeleton and liver were studied in 5 groups of 20 beagles exposed to initial lung depositions ranging from 0.48 to 518 Bq/g of lung. Average plutonium amounts in the lungs decreased to approximately 1% of the final body deposition in dogs surviving 5 years or more; more than 90% of the final depositions accumulated in the liver and skeleton. The liver-to-skeletal ratio of deposited plutonium was 0.83. The incidence of bone tumors, primarily osteogenic sarcomas causing early mortality, at final group average skeletal depositions of 15.8, 2.1, and 0.5 Bq/g was, respectively, 85%, 50%, and 5%; there were no bone tumors in exposure groups with mean average depositions lower than 0.5 Bq/g. Elevated serum liver enzyme levels were observed in exposure groups down to 1.3 Bq/g. The incidence of liver tumors at final group average liver depositions of 6.9, 1.3, 0.2, and 0.1 Bq/g, was, respectively, 25%, 15%, 15%, and 15%; one hepatoma occurred among 40 control dogs. The risk of the liver cancer produced by inhaled plutonium nitrate was difficult to assess due to the competing risks of life shortening from lung and bone tumors.

  5. Analysis of multiple sarcoma expression datasets: implications for classification, oncogenic pathway activation and chemotherapy resistance.

    Directory of Open Access Journals (Sweden)

    Panagiotis A Konstantinopoulos

    Full Text Available BACKGROUND: Diagnosis of soft tissue sarcomas (STS is challenging. Many remain unclassified (not-otherwise-specified, NOS or grouped in controversial categories such as malignant fibrous histiocytoma (MFH, with unclear therapeutic value. We analyzed several independent microarray datasets, to identify a predictor, use it to classify unclassifiable sarcomas, and assess oncogenic pathway activation and chemotherapy response. METHODOLOGY/PRINCIPAL FINDINGS: We analyzed 5 independent datasets (325 tumor arrays. We developed and validated a predictor, which was used to reclassify MFH and NOS sarcomas. The molecular "match" between MFH and their predicted subtypes was assessed using genome-wide hierarchical clustering and Subclass-Mapping. Findings were validated in 15 paraffin samples profiled on the DASL platform. Bayesian models of oncogenic pathway activation and chemotherapy response were applied to individual STS samples. A 170-gene predictor was developed and independently validated (80-85% accuracy in all datasets. Most MFH and NOS tumors were reclassified as leiomyosarcomas, liposarcomas and fibrosarcomas. "Molecular match" between MFH and their predicted STS subtypes was confirmed both within and across datasets. This classification revealed previously unrecognized tissue differentiation lines (adipocyte, fibroblastic, smooth-muscle and was reproduced in paraffin specimens. Different sarcoma subtypes demonstrated distinct oncogenic pathway activation patterns, and reclassified MFH tumors shared oncogenic pathway activation patterns with their predicted subtypes. These patterns were associated with predicted resistance to chemotherapeutic agents commonly used in sarcomas. CONCLUSIONS/SIGNIFICANCE: STS profiling can aid in diagnosis through a predictor tracking distinct tissue differentiation in unclassified tumors, and in therapeutic management via oncogenic pathway activation and chemotherapy response assessment.

  6. Prophylactic Antitumor Effect of Mixed Heat Shock Proteins/Peptides in Mouse Sarcoma

    Institute of Scientific and Technical Information of China (English)

    Yu Wang; Shu-Yun Liu; Mei Yuan; Yu Tang; Quan-Yi Guo; Xue-Mei Cui; Xiang Sui

    2015-01-01

    Background:To develop a vaccine-based immunotherapy for sarcoma,we evaluated a mixture of heat shock proteins (mHSPs) as a vaccine for sarcoma treatment in a mouse model.Heat shock protein/peptides (HSP/Ps) are autoimmune factors that can induce both adaptive and innate immune responses;HSP/Ps isolated from tumors can induce antitumor immune activity when used as vaccines.Methods:In this study,we evaluated the effects of mHSP/Ps on prophylactic antitumor immunity.We extracted mHSP/Ps,including HSP60,HSP70,GP96,and HSP l 10,from the mouse sarcoma cell lines S 180 and MCA207 using chromatography.The immunity induced by mHSP/Ps was assessed using flow cytometry,ELISPOT,lactate dehydrogenase release,and enzyme-linked immunosorbent assay.Results:Of S180 sarcoma-beating mice immunized with mHSP/Ps isolated from S180 cells,41.2% showed tumor regression and long-term survival,with a tumor growth inhibition rate of 82.3% at 30 days.Of MCA207 sarcoma-bearing mice immunized with mHSP/Ps isolated from MCA207 cells,50% showed tumor regression and long-term survival with a tumor growth inhibition rate of 79.3%.All control mice died within 40 days.The proportions of natural killer cells,CD8+,and interferon-γ-secreting cells and tumor-specific cytotoxic T-lymphocyte activity were increased in the immunized group.Conclusions:Vaccination with a polyvalent mHSP/P cancer vaccine can induce an immunological response and a marked antitumor response to autologous tumors.This mHSP/P vaccine exerted greater antitumor effects than did HSPT0,HSP60,or tumor lysates alone.

  7. Endometrial stromal sarcoma: a rare tumour

    Directory of Open Access Journals (Sweden)

    Amrit Pal Kaur

    2014-02-01

    Full Text Available Endometrial stromal sarcomas (ESS are rare endometrial tumours arising from stroma of endometrium i.e. connective tissue of endometrium rather than glands. Usually a pre-operative diagnosis is difficult. Total abdominal hysterectomy with bilateral salpingo-oophorectomy is main line of treatment. Adjuvant hormone therapy in the form of progesterones, GnRH analogues, aromatase inhibitors are effective for prevention of recurrences as these tumours are invariably positive for oestrogen & progesterone receptors. Surgical excision, radiotherapy, hormone therapy are recommended for recurrences. We report a 52 yrs widow with undifferentiated endometrial stromal sarcoma weighing 3.75 kg with a short history of 3 months diagnosed only after histopathology. [Int J Reprod Contracept Obstet Gynecol 2014; 3(1.000: 276-278

  8. Radiological diagnostic in cat stratch disease and bone lesions - a case report; Doenca da arranhadura do gato e lesoes osseas - relato de um caso

    Energy Technology Data Exchange (ETDEWEB)

    Abreu, Marcelo Rodrigues de [Rio Grande do Sul Univ., Porto Alegre, RS (Brazil). Faculdade de Medicina; Abreu, Armando de; Santos, Leandro Durval dos [Hospital Mae de Deus, Porto Alegre, RS (Brazil). Servico de Radiologia; Scharnovski, Alvaro [Hospital de Taquara, RS (Brazil)

    1999-02-01

    Cat scratch disease is not a common disease in immunocompetent patients. Its association with bone lesions is rare. A patient with bone complain and radiologic alterations of bone lesion must be investigated for this disease. A simple story can make the differential diagnosis with more complex disease like Ewing sarcoma or eosinophilic granuloma. (author)

  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. 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.)

  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. 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.

  13. Bone Biopsy

    Science.gov (United States)

    ... News Physician Resources Professions Site Index A-Z Bone Biopsy Bone biopsy uses a needle and imaging ... the limitations of Bone Biopsy? What is a Bone Biopsy? A bone biopsy is an image-guided ...

  14. 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.

  15. 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.

  16. Implantação intracerebral experimental de sarcomas Experimental intracerebral implantation of sarcomas

    Directory of Open Access Journals (Sweden)

    Alexandre Alencar

    1972-01-01

    Full Text Available Estudo histopatológico da implantação intracerebral de sarcomas, realizado em ratos (fibrosarcoma e em camundongos (sarcoma 180. A implantação intracerebral de fibrosarcoma desenvolveu, em cerca de 45 dias, neoplasia relativamente bem delimitada, com pequena infiltração do parênquima nervoso adjacente, nunca se propagando a maiores distãncias devido a forte coesão entre as suas células, com grande diferenciação de fibrilas reticulares e de colágeno. Ao contrário, a inoculação do sarcoma 180, principalmente sob a forma ascítica, levou rapidamente a um quadro de forte hipertensão intracraniana, com disseminação das células neoplásticas pelos espaços subaracnoideanos e intraventriculares. Ambas as neoplasias propagavam-se pelos espaços subaracnoideanos e intraventriculares. Ambas as neoplasias propagavam-se pelos espaços perivasculares, porém o faziam de maneira diversa; o sarcoma 180 tinha uma disseminação muito intensa e rápida, que se fazia a longas distâncias, enquanto que o fibrossarcoma somente se disseminava nos vasos próximos à neoplasia em desenvolvimento. Tomando por base observações próprias e outras colhidas na bibliografia especializada, conclui-se que os agentes etiológicos destes tumores exercem sua ação sobre tipos celulares diferentes.Histopathological study of intracerebral implantation of sarcomas, performed in rats (fibrosarcomas and mice (sarcoma 180. The intracerebral implantation of fibrosarcoma has developed in about 45 days a well limited tumor, with little infiltration of the adjacent nervous parenchyma, never streading to longer distances because the strong cohesion between its cells, with great differentiation of reticular fibers and collagen. On the contrary, the innoculation of sarcoma 180, chiefly of the ascitical form, has rapidly lead to a strong intracranial hipertension, with dissemination of neoplastic cells through the subarachnoid and intraventricular spaces. Both

  17. The role of Kaposi sarcoma-associated herpesvirus in the pathogenesis of Kaposi sarcoma.

    Science.gov (United States)

    Gramolelli, Silvia; Schulz, Thomas F

    2015-01-01

    Kaposi sarcoma (KS) is an unusual vascular tumour caused by an oncogenic-herpesvirus, Kaposi sarcoma-associated herpesvirus (KSHV), also known as human herpesvirus 8 (HHV 8). KS lesions are characterized by an abundant inflammatory infiltrate, the presence of KSHV-infected endothelial cells that show signs of aberrant differentiation, as well as faulty angiogenesis/ vascularization. Here we discuss the molecular mechanisms that lead to the development of these histological features of KS, with an emphasis on the viral proteins that are responsible for their development.

  18. FOXM1 is an oncogenic mediator in Ewing Sarcoma.

    Directory of Open Access Journals (Sweden)

    Laura Christensen

    Full Text Available Ewing Family Tumors (Ewing Sarcoma and peripheral Primitive Neuroectodermal Tumor are common bone and soft tissue malignancies of childhood, adolescence and young adulthood. Chromosomal translocation in these tumors produces fusion oncogenes of the EWS/ETS class, with EWS/FLI1 being by far the most common. EWS/ETS chimera are the only well established driver mutations in these tumors and they function as aberrant transcription factors. Understanding the downstream genes whose expression is modified has been a central approach to the study of these tumors. FOXM1 is a proliferation associated transcription factor which has increasingly been found to play a role in the pathogenesis of a wide range of human cancers. Here we demonstrate that FOXM1 is expressed in Ewing primary tumors and cell lines. Reduction in FOXM1 expression in Ewing cell lines results in diminished potential for anchorage independent growth. FOXM1 expression is enhanced by EWS/FLI1, though, unlike other tumor systems, it is not driven by expression of the EWS/FLI1 target GLI1. Thiostrepton is a compound known to inhibit FOXM1 by direct binding. We show that Thiostrepton diminishes FOXM1 expression in Ewing cell lines and this reduction reduces cell viability through an apoptotic mechanism. FOXM1 is involved in Ewing tumor pathogenesis and may prove to be a useful therapeutic target in Ewing tumors.

  19. Targeting the DNA Repair Pathway in Ewing Sarcoma

    Directory of Open Access Journals (Sweden)

    Elizabeth Stewart

    2014-11-01

    Full Text Available Ewing sarcoma (EWS is a tumor of the bone and soft tissue that primarily affects adolescents and young adults. With current therapies, 70% of patients with localized disease survive, but patients with metastatic or recurrent disease have a poor outcome. We found that EWS cell lines are defective in DNA break repair and are sensitive to PARP inhibitors (PARPis. PARPi-induced cytotoxicity in EWS cells was 10- to 1,000-fold higher after administration of the DNA-damaging agents irinotecan or temozolomide. We developed an orthotopic EWS mouse model and performed pharmacokinetic and pharmacodynamic studies using three different PARPis that are in clinical development for pediatric cancer. Irinotecan administered on a low-dose, protracted schedule previously optimized for pediatric patients was an effective DNA-damaging agent when combined with PARPis; it was also better tolerated than combinations with temozolomide. Combining PARPis with irinotecan and temozolomide gave complete and durable responses in more than 80% of the mice.

  20. Bone Densitometry (Bone Density Scan)

    Science.gov (United States)

    ... News Physician Resources Professions Site Index A-Z Bone Densitometry (DEXA) Bone densitometry, also called dual-energy ... limitations of DEXA Bone Densitometry? What is a Bone Density Scan (DEXA)? Bone density scanning, also called ...

  1. Gracile bone dysplasias

    Energy Technology Data Exchange (ETDEWEB)

    Kozlowski, Kazimierz [Department of Medical Imaging, The Children' s Hospital at Westmead, Locked Bag 4001, Westmead 2145, NSW (Australia); Masel, John [Department of Radiology, Royal Children' s Hospital, Brisbane (Australia); Sillence, David O. [Department of Paediatrics and Child Health, The University of Sydney (Australia); Arbuckle, Susan [Department of Anatomical Pathology, The Children' s Hospital at Westmead, NSW (Australia); Juttnerova, Vera [Oddeleni Lekarske Genetiky, Hradec Kralove (Czech Republic)

    2002-09-01

    Gracile bone dysplasias constitute a group of disorders characterised by extremely slender bones with or without fractures. We report four newborns, two of whom showed multiple fractures. Two babies had osteocraniostenosis and one had features of oligohydramnios sequence. The diagnosis in the fourth newborn, which showed thin long bones and clavicles and extremely thin, poorly ossified ribs, is uncertain. Exact diagnosis of a gracile bone dysplasia is important for genetic counselling and medico-legal reasons. (orig.)

  2. The clone wars - revenge of the metastatic rogue state: the sarcoma paradigm

    Directory of Open Access Journals (Sweden)

    Holly Lynn Spraker

    2012-01-01

    Full Text Available Ewing sarcoma (ES is the second most common bone tumor affecting primarily adolescents and young adults. Despite recent advances in biological understanding, intensification of chemotherapeutic treatments, and progress in local control with surgery and/or radiation therapy, patients with metastatic or recurrent ES continue to have a dismal prognosis with less than 20% overall survival. All ES likely is metastatic at diagnosis although our methods of detection and classification may not account for this. Progressive disease may arise via a combination of: 1 selection of chemotherapy-resistant clones, 2 signaling from bone or lung microenvironments that may attract tumor cells to distant locations, and/or 3 genetic changes within the ES cells themselves due to a combination of therapy-related selection and DNA-damaging chemotherapeutic agents. These possibilities and the evidence base to support them are explored.

  3. A zebrafish transgenic model of Ewing’s sarcoma reveals conserved mediators of EWS-FLI1 tumorigenesis

    Directory of Open Access Journals (Sweden)

    Stefanie W. Leacock

    2012-01-01

    Ewing’s sarcoma, a malignant bone tumor of children and young adults, is a member of the small-round-blue-cell tumor family. Ewing’s sarcoma family tumors (ESFTs, which include peripheral primitive neuroectodermal tumors (PNETs, are characterized by chromosomal translocations that generate fusions between the EWS gene and ETS-family transcription factors, most commonly FLI1. The EWS-FLI1 fusion oncoprotein represents an attractive therapeutic target for treatment of Ewing’s sarcoma. The cell of origin of ESFT and the molecular mechanisms by which EWS-FLI1 mediates tumorigenesis remain unknown, and few animal models of Ewing’s sarcoma exist. Here, we report the use of zebrafish as a vertebrate model of EWS-FLI1 function and tumorigenesis. Mosaic expression of the human EWS-FLI1 fusion protein in zebrafish caused the development of tumors with histology strongly resembling that of human Ewing’s sarcoma. The incidence of tumors increased in a p53 mutant background, suggesting that the p53 pathway suppresses EWS-FLI1-driven tumorigenesis. Gene expression profiling of the zebrafish tumors defined a set of genes that might be regulated by EWS-FLI1, including the zebrafish ortholog of a crucial EWS-FLI1 target gene in humans. Stable zebrafish transgenic lines expressing EWS-FLI1 under the control of the heat-shock promoter exhibit altered embryonic development and defective convergence and extension, suggesting that EWS-FLI1 interacts with conserved developmental pathways. These results indicate that functional targets of EWS-FLI1 that mediate tumorigenesis are conserved from zebrafish to human and provide a novel context in which to study the function of this fusion oncogene.

  4. Serotype chimeric oncolytic adenovirus coding for GM-CSF for treatment of sarcoma in rodents and humans.

    Science.gov (United States)

    Bramante, Simona; Koski, Anniina; Kipar, Anja; Diaconu, Iulia; Liikanen, Ilkka; Hemminki, Otto; Vassilev, Lotta; Parviainen, Suvi; Cerullo, Vincenzo; Pesonen, Saila K; Oksanen, Minna; Heiskanen, Raita; Rouvinen-Lagerström, Noora; Merisalo-Soikkeli, Maiju; Hakonen, Tiina; Joensuu, Timo; Kanerva, Anna; Pesonen, Sari; Hemminki, Akseli

    2014-08-01

    Sarcomas are a relatively rare cancer, but often incurable at the late metastatic stage. Oncolytic immunotherapy has gained attention over the past years, and a wide range of oncolytic viruses have been delivered via intratumoral injection with positive safety and promising efficacy data. Here, we report preclinical and clinical results from treatment of sarcoma with oncolytic adenovirus Ad5/3-D24-GMCSF (CGTG-102). Ad5/3-D24-GMCSF is a serotype chimeric oncolytic adenovirus coding for human granulocyte-macrophage colony-stimulating factor (GM-CSF). The efficacy of Ad5/3-D24-GMCSF was evaluated on a panel of soft-tissue sarcoma (STS) cell lines and in two animal models. Sarcoma specific human data were also collected from the Advanced Therapy Access Program (ATAP), in preparation for further clinical development. Efficacy was seen in both in vitro and in vivo STS models. Fifteen patients with treatment-refractory STS (13/15) or primary bone sarcoma (2/15) were treated in ATAP, and treatments appeared safe and well-tolerated. A total of 12 radiological RECIST response evaluations were performed, and two cases of minor response, six cases of stable disease and four cases of progressive disease were detected in patients progressing prior to virus treatment. Overall, the median survival time post treatment was 170 days. One patient is still alive at 1,459 days post virus treatment. In summary, Ad5/3-D24-GMCSF appears promising for the treatment of advanced STS; a clinical trial for treatment of refractory injectable solid tumors including STS is ongoing.

  5. Body protein status in Fischer 344 rats bearing the MCA sarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Radcliffe, J.D.

    1986-03-05

    The effect of a methylcholanthrene (MCA) sarcoma on the protein status of adult, male, Fischer-344 rats was investigated. Three groups of ten rats were used. One group (TB) was inoculated with MCA sarcoma tissue, control (C) and pair-fed (PF) groups received saline only. Rats were fed a purified (20% casein, 20% fat, 45% sucrose) diet from day 7 post-transplant, when tumors became palpable. Food intake of TB was depressed relative to C at day 15 PT. Animals were killed at day 22 PT. In comparison to C, tumor growth was associated with a decrease in gastrocnemius muscle protein content and an increase in spleen weight and protein content. There was no effect of tumor growth on the weight or protein content of liver, heart, or kidneys. Data from PF animals suggested that decreased gastrocnemius muscle protein content was partly attributable to decreased food intake and partly to tumor growth; the increased spleen protein was due to tumor growth per se. Thus, growth of the MCA sarcoma affects host protein status. Some of these effects are caused partly by hypophagia and partly by tumor growth and others are due to tumor growth per se.

  6. Postradiation sarcomas of the pelvis after treatment for uterine cervical cancer: review of the CT and MR findings of five cases

    Energy Technology Data Exchange (ETDEWEB)

    Nakanishi, Katsuyuki [Osaka Seamen' s Insurance Hospital (Japan). Dept. of Radiology; Yoshikawa, Hideki [Osaka Univ. Medical School (Japan). Dept. of Orthopaedic Surgery; Ueda, Takafumi; Araki, Nobuhito [Osaka Medical Center for Cancer and Cardiovascular Diseases (Japan). Dept. of Orthopaedic Surgery; Tanaka, Hisashi; Nakamura, Hironobu [Osaka Univ. Medical School (Japan). Dept. of Radiology; Aozasa, Katsuyuki [Osaka Medical School (Japan). Dept. of Pathology

    2001-03-01

    Objective. To characterize the radiologic features of postradiation sarcomas arising in the pelvic bones following treatment for uterine cervical carcinoma. Design and patients. Five patients who developed postradiation sarcomas in the pelvic bones following radiation therapy for carcinoma of the uterine cervix within the irradiated field were evaluated. Pelvic radiographs, computed tomography (CT) and magnetic resonance (MR) imaging were undertaken in all patients. Histologic confirmation of the tumor type was obtained. Results. Three patients whose tumors were characterized as an osteosarcoma, an angiosarcoma and a malignant fibrous histiocytoma (MFH) showed a large round or oval mass mainly in the sacroiliac joint which extended into the posterior gluteal soft tissues. In a fourth patient an osteosarcoma developed in the central ilium extending widely into the soft tissues both anteriorly and posteriorly, with calcified areas within the extraosseous mass. The fifth patient had a MFH which showed osteolytic destruction of the cortex of the acetabulum, and minimal soft tissue extension. There were no specific features or signal intensity changes on MR imaging to differentiate these cases from primary sarcomas. Conclusion. Postradiation sarcoma must be considered in patients with uterine carcinoma when a soft tissue mass is seen in the previously irradiated field, especially if the mass is posterior to the sacroiliac joint and the latent period is more than 5 years. (orig.)

  7. 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.

  8. 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.

  9. Is There a Predisposition Gene for Ewing's Sarcoma?

    Directory of Open Access Journals (Sweden)

    R. L. Randall

    2010-01-01

    Full Text Available Ewing's sarcoma is a highly malignant tumor of children and young adults. The molecular mechanisms that underlie Ewing's Sarcoma development are beginning to be understood. For example, most cases of this disease harbor somatic chromosomal translocations that fuse the EWSR1 gene on chromosome 22 with members of the ETS family. While some cooperative genetic events have been identified, such as mutations in TP53 or deletions of the CDKN2A locus, these appear to be absent in the vast majority of cases. It is therefore uncertain whether EWS/ETS translocations are the only consistently present alteration in this tumor, or whether there are other recurrent abnormalities yet to be discovered. One method to discover such mutations is to identify familial cases of Ewing's sarcoma and to then map the susceptibility locus using traditional genetic mapping techniques. Although cases of sibling pairs with Ewing's sarcoma exist, familial cases of Ewing's sarcoma have not been reported. While Ewing's sarcoma has been reported as a 2nd malignancy after retinoblastoma, significant associations of Ewing's sarcoma with classic tumor susceptibility syndromes have not been identified. We will review the current evidence, or lack thereof, regarding the potential of a heritable condition predisposing to Ewing's sarcoma.

  10. 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.

  11. Two Cases of Ectopic Hamartomatous Thymoma Masquerading as Sarcoma

    Science.gov (United States)

    Sato, Yukiko; Tanaka, Hiroko; Sasaki, Toru; Kawabata, Kazuyoshi; Mitani, Hiroki; Yonekawa, Hiroyuki; Fukushima, Hirofumi; Shimbashi, Wataru

    2017-01-01

    Ectopic hamartomatous thymoma (EHT) is an extremely rare benign tumor. EHTs are difficult to differentiate from sarcomas, especially synovial sarcomas. We encountered two cases of EHT that were referred from other hospitals because sarcoma was suspected. In these cases, fusion gene detection via polymerase chain reaction or fluorescence in situ hybridization was useful for differentiating EHT from synovial sarcoma. EHT requires accurate diagnosis before surgery to avoid excessive treatment. Both tumor location and the presence of fat inside the tumor are important imaging findings for EHT, and confirmation of spindle cells, epithelial cells, and mature adipose cells in the tumor is an important pathological finding. It is important to exclude synovial sarcoma from the differential diagnosis via fusion gene analysis. PMID:28168073

  12. Plasmacytoid dendritic cells in skin lesions of classic Kaposi's sarcoma.

    Science.gov (United States)

    Karouni, Mirna; Kurban, Mazen; Abbas, Ossama

    2016-09-01

    Plasmacytoid dendritic cells (pDCs) are the most potent producers of type I interferons (IFNs), which allows them to provide anti-viral resistance and to link the innate and adaptive immunity by controlling the function of myeloid DCs, lymphocytes, and natural killer cells. pDCs are involved in the pathogenesis of several infectious [especially viral, such as Molluscum contagiosum (MC)], inflammatory/autoimmune, and neoplastic entities. Kaposi's sarcoma (KS) is a multifocal, systemic lympho-angioproliferative tumor associated with Kaposi's sarcoma-associated herpesvirus (KSHV) infection. Microscopy typically exhibits a chronic inflammatory lymphoplasmacytic infiltrate in addition to the vascular changes and spindle cell proliferation. Despite the extensive research done on the immune evasion strategies employed by KSHV, pDCs role in relation to KS has only rarely been investigated. Given this, we intend to investigate pDC occurrence and activity in the skin lesions of KS. Immunohistochemical staining for BDCA-2 (specific pDC marker) and MxA (surrogate marker for local type I IFN production) was performed on classic KS (n = 20) with the control group comprising inflamed MC (n = 20). As expected, BDCA-2+ pDCs were present in abundance with diffuse and intense MxA expression (indicative of local type I IFN production) in all inflamed MC cases (20 of 20, 100 %). Though present in all the KS cases, pDCs were significantly less abundant in KS than in inflamed MC cases, and MxA expression was patchy/weak in most KS cases. In summary, pDCs are part of the inflammatory host response in KS; however, they were generally low in number with decreased type I IFN production which is probably related to KSHV's ability to evade the immune system through the production of different viral proteins capable of suppressing IFN production as well as pDC function.

  13. Splenotoxicity associated with splenic sarcomas in rats fed high doses of D & C Red No. 9 or aniline hydrochloride.

    Science.gov (United States)

    Weinberger, M A; Albert, R H; Montgomery, S B

    1985-10-01

    A histopathologic review of F344 rat spleens from the National Toxicology Program-National Cancer Institute bioassays of barium salt of 5-chloro-2-(2-hydroxy-1-naphthalenyl)-azo-4-methylbenzenesulfonic acid [(D & C Red No. 9) CAS: 516-00-21] and aniline HCI (CAS: 142-04-1) was conducted to assess splenotoxic changes associated with splenic sarcomas induced by these aromatic amines. Four splenic changes--fatty metamorphosis (FM), splenic fibrosis (FIB), capsule hyperplasia (CH), and hemorrhage--were markedly increased in incidence and severity in males treated with high doses of either D & C Red No. 9 or aniline HCI. Females treated with high doses of either of these compounds showed similar but less severe changes. FIB and FM showed strong group correlations with tumor incidence (r greater than or equal to 0.87). All groups that demonstrated FM also demonstrated splenic sarcomas; groups without the FM lesions did not exhibit splenic tumors. The morphologic similarity of the FIB and CH lesions to the induced splenic sarcomas suggests that these lesions are preneoplastic. Moreover, the treatment-related splenic lesions appear to be precursors of the induced splenic sarcomas. Carcinogenicity studies with serial sacrifices at varying intervals will be required for experimental verification of these conclusions. A schema, based on the findings of the study, suggests a hypothetical pathway for the progression of the treatment-related splenic lesions from onset to tumor formation.

  14. 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.

  15. Development of a Food Group-Based Diet Score and Its Association with Bone Mineral Density in the Elderly: The Rotterdam Study

    Directory of Open Access Journals (Sweden)

    Ester A.L. de Jonge

    2015-08-01

    Full Text Available No diet score exists that summarizes the features of a diet that is optimal for bone mineral density (BMD in the elderly. Our aims were (a to develop a BMD-Diet Score reflecting a diet that may be beneficial for BMD based on the existing literature, and (b to examine the association of the BMD-Diet Score and the Healthy Diet Indicator, a score based on guidelines of the World Health Organization, with BMD in Dutch elderly participating in a prospective cohort study, the Rotterdam Study (n = 5144. Baseline dietary intake, assessed using a food frequency questionnaire, was categorized into food groups. Food groups that were consistently associated with BMD in the literature were included in the BMD-Diet Score. BMD was measured repeatedly and was assessed using dual energy X-ray absorptiometry. The BMD-Diet Score considered intake of vegetables, fruits, fish, whole grains, legumes/beans and dairy products as “high-BMD” components and meat and confectionary as “low-BMD” components. After adjustment, the BMD-Diet Score was positively associated with BMD (β (95% confidence interval = 0.009 (0.005, 0.012 g/cm2 per standard deviation. This effect size was approximately three times as large as has been observed for the Healthy Diet Indicator. The food groups included in our BMD-Diet Score could be considered in the development of future dietary guidelines for healthy ageing.

  16. Acroangiodermatite (pseudo-sarcoma de Kaposi

    Directory of Open Access Journals (Sweden)

    Azulay Rubem David

    2004-01-01

    Full Text Available Acroangiodermatite é enfermidade rara, caracterizada por lesões eritêmato-violáceas bem delimitadas que acometem pernas e pés com aspecto semelhante ao do sarcoma de Kaposi. É relatado o caso de paciente do sexo feminino, de 57 anos, com início súbito de lesões eritêmato-violáceas nas pernas sem outras alterações. O caso acrescenta aprendizado por sua dificuldade diagnóstica e reafirma a importância da imuno-histoquímica. Trata-se da publicação do primeiro caso brasileiro.

  17. Histological variants of cutaneous Kaposi sarcoma

    Directory of Open Access Journals (Sweden)

    Pantanowitz Liron

    2008-07-01

    Full Text Available Abstract This review provides a comprehensive overview of the broad clinicopathologic spectrum of cutaneous Kaposi sarcoma (KS lesions. Variants discussed include: usual KS lesions associated with disease progression (i.e. patch, plaque and nodular stage; morphologic subtypes alluded to in the older literature such as anaplastic and telangiectatic KS, as well as several lymphedematous variants; and numerous recently described variants including hyperkeratotic, keloidal, micronodular, pyogenic granuloma-like, ecchymotic, and intravascular KS. Involuting lesions as a result of treatment related regression are also presented.

  18. Bone within a bone

    Energy Technology Data Exchange (ETDEWEB)

    Williams, H.J.; Davies, A.M. E-mail: wendy.turner@roh.nhs.uk; Chapman, S

    2004-02-01

    The 'bone within a bone' appearance is a well-recognized radiological term with a variety of causes. It is important to recognize this appearance and also to be aware of the differential diagnosis. A number of common conditions infrequently cause this appearance. Other causes are rare and some remain primarily of historical interest, as they are no longer encountered in clinical practice. In this review we illustrate some of the conditions that can give the bone within a bone appearance and discuss the physiological and pathological aetiology of each where known.

  19. Ex Vivo Behaviour of Human Bone Tumor Endothelial Cells

    Energy Technology Data Exchange (ETDEWEB)

    Infante, Teresa [SDN-Foundation, Institute of Diagnostic and Nuclear Development, IRCCS, 80143 Naples (Italy); Cesario, Elena [Department of Biochemistry and Biophysics, Second University of Naples, 80138 Naples (Italy); Gallo, Michele; Fazioli, Flavio [Division of Skeletal Muscles Oncology Surgery, National Cancer Institute, Pascale Foundation, 80131 Naples (Italy); De Chiara, Annarosaria [Anatomic Pathology Unit, National Cancer Institute, Pascale Foundation, 80131 Naples (Italy); Tutucci, Cristina; Apice, Gaetano [Medical Oncology of Bone and Soft Sarcoma tissues Unit, National Cancer Institute, Pascale Foundation, 80131 Naples (Italy); Nigris, Filomena de, E-mail: filomena.denigris@unina2.it [Department of Biochemistry and Biophysics, Second University of Naples, 80138 Naples (Italy)

    2013-04-11

    Cooperation between endothelial cells and bone in bone remodelling is well established. In contrast, bone microvasculature supporting the growth of primary tumors and metastasis is poorly understood. Several antiangiogenic agents have recently been undergoing trials, although an extensive body of clinical data and experimental research have proved that angiogenic pathways differ in each tumor type and stage. Here, for the first time, we characterize at the molecular and functional level tumor endothelial cells from human bone sarcomas at different stages of disease and with different histotypes. We selected a CD31{sup +} subpopulation from biopsies that displayed the capability to grow as adherent cell lines without vascular endothelial growth factor (VEGF). Our findings show the existence in human primary bone sarcomas of highly proliferative endothelial cells expressing CD31, CD44, CD105, CD146 and CD90 markers. These cells are committed to develop capillary-like structures and colony formation units, and to produce nitric oxide. We believe that a better understanding of tumor vasculature could be a valid tool for the design of an efficacious antiangiogenic therapy as adjuvant treatment of sarcomas.

  20. 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.

  1. Primary Synovial Sarcoma of the Kidney

    Directory of Open Access Journals (Sweden)

    Takashi Kawahara

    2009-10-01

    Full Text Available The case was a 40-year-old female. She visited a local doctor with a chief complaint of right side abdominal pain. A right kidney tumor measuring 10 cm in diameter was observed in an abdominal Computed Tomography (CT scan. Based on the CT image, the possibility of angiomiolipoma (AML could not be ruled out, but a high maximum standardized uptake value (SUVmax of 7.8 was observed in a Positron Emission Tomography CT (PET-CT scan and there was a possibility of malignancy. We therefore performed a transperitoneal right radial nephrectomy. Although adhesion of the tumor to the duodenum and the inferior vena cava was observed, it was possible to perform an excision. The tumor accounted for a large proportion of the excised kidney; the surrounding areas had taken on a cyst-like structure, and the interior comprised grayish brittle tissue exhibiting solid growth. Histologically, gland-like and cyst-like structures composed of cylindrical cuboidal cells and mainly characterized by the solid growth of short fusiform-shaped and oval-shaped basophilic cells were observed, and we believed it was a synovial sarcoma. There were no malignant findings in the adrenal gland. There have been approximately 30 reported cases around the world of synovial sarcoma that developed in the kidney, and we herein report this case with bibliographic considerations.

  2. Pulmonary Kaposi sarcoma in six children

    Energy Technology Data Exchange (ETDEWEB)

    Theron, Salomine [University of Stellenbosch, Department of Radiology, Tygerberg Academic Hospital, Cape Town (South Africa); University of Stellenbosch, Department of Radiology, Tygerberg Hospital, Cape Town (South Africa); Andronikou, Savvas; Plessis, Jaco du; George, Reena; Mapukata, Ayanda; Grobbelaar, Marie; Hayes, Murray [University of Stellenbosch, Department of Radiology, Tygerberg Academic Hospital, Cape Town (South Africa); Goussard, Pierre [University of Stellenbosch, Department of Child Health, Tygerberg Academic Hospital, Cape Town (South Africa); Wieselthaler, Nicky [University of Cape Town, Department of Radiology, Red Cross Children' s Hospital, Cape Town (South Africa); Davidson, Alan [University of Cape Town, Department of Oncology, Red Cross Children' s Hospital, Cape Town (South Africa)

    2007-12-15

    Pulmonary involvement in Kaposi sarcoma is rare in children and can be difficult to distinguish from other pathology. To describe the radiological findings in paediatric pulmonary Kaposi sarcoma. Sequential chest radiographs of six children and CT scans of four of these children were evaluated retrospectively. Their ages ranged from 18 months to 10 years; four were male and two were female. All six children were HIV-positive. The observers were two radiologists. Chest radiographs revealed air-space (100%) and reticular (83%) opacification in the mid- and lower lung zones; pleural effusions were present in 83% of the children. All the children showed progressive air-space opacification on follow-up radiography. CT demonstrated bilateral air-space opacification in a perihilar distribution in all the children; reticular opacification was seen in 75%. All the children had mediastinal and axillary lymphadenopathy; 75% had bilateral hilar lymphadenopathy. In both adults and children, chest radiography demonstrates perihilar and lower zone involvement. Pleural effusions are more common on radiographs in children. Air-space disease and lymphadenopathy are much more common on CT in children than adults. (orig.)

  3. 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

  4. 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.

  5. 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.

  6. 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.

  7. 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.

  8. Improved repair of bone defects with prevascularized tissue-engineered bones constructed in a perfusion bioreactor.

    Science.gov (United States)

    Li, De-Qiang; Li, Ming; Liu, Pei-Lai; Zhang, Yuan-Kai; Lu, Jian-Xi; Li, Jian-Min

    2014-10-01

    Vascularization of tissue-engineered bones is critical to achieving satisfactory repair of bone defects. The authors investigated the use of prevascularized tissue-engineered bone for repairing bone defects. The new bone was greater in the prevascularized group than in the non-vascularized group, indicating that prevascularized tissue-engineered bone improves the repair of bone defects. [Orthopedics. 2014; 37(10):685-690.].

  9. [The combination treatment of malignant bone tumors using fast neutrons].

    Science.gov (United States)

    Chernichenko, V A; Tolstopiatov, B A; Konovalenko, V F; Monich, A Iu; Palivets, A Iu

    1990-01-01

    The study deals with results of a clinical trial evaluating treatment efficacy of a 6 MeV neutron beam produced by Y-120 cyclotron (Kiev). Procedures of preoperative radiotherapy and radical treatment are discussed. Radiotherapy was administered to 52 patients suffering chondrosarcoma (30 cases), osteogenic sarcoma (15) or chordoma (7). Combined treatment (radiation + surgery) was given to 22 patients whereas neutron beam therapy--to 30. All patients with osteogenic sarcoma received adjuvant combination chemotherapy. Three-year survival rate was compared to that observed in controls in whom combined treatment had included gamma-therapy. A significant increase in three-year survival rate was observed for osteogenic sarcoma and chordoma whereas for chondrosarcoma the improvement in survival proved insignificant. The use of fast neutrons in combined treatment of bone tumors was considered promising.

  10. Primary Ewing's Sarcoma of the Spine in a Two-Year-Old Boy

    Science.gov (United States)

    Electricwala, Jaffer T.

    2016-01-01

    Ewing's Sarcoma (ES) is a highly malignant bone tumour. It may involve any part of the skeleton but the most frequent parts are the ilium and diaphysis of femur and tibia (Alfeeli et al., 2005; Zhu et al., 2012). Primary ES of the spine is extremely rare (Yan et al., 2011). It accounts for only 3.5 to 14.9 percent of all primary bone sarcomas. The age of presentation ranges from 12 to 24 years (median 21 years) (Ferguson, 1999; Sharafuddin et al., 1992; Klimo Jr. et al., 2009). We report an unusual case of primary ES of the spine in a two-year-old boy, who presented to us with paraparesis and features of cauda equina syndrome. MRI scan showed a tumour mass arising from the pedicle of L4 vertebra invading the spinal canal. Tc-99 bone scan showed increased tracer uptake in L4 vertebra and normal tracer uptake elsewhere in the skeleton. After reaching the diagnosis of a space occupying lesion invading the lumber spinal canal, we performed a decompressive laminectomy and a biopsy was sent which confirmed the diagnosis of ES. Immunohistochemistry showed tumour cells staining positive for CD-99 (specific stain for ES). Gene testing showed an EWS-FLI 1 chimera. Surgery was followed by good improvement in motor signs. The child was then referred to a specialized oncotherapy centre for further treatment, radiation, and chemotherapy. To the best of our knowledge, we are the first to report primary ES of the spine at the age of two years. PMID:27895949

  11. Primary Ewing’s Sarcoma of the Spine in a Two-Year-Old Boy

    Directory of Open Access Journals (Sweden)

    Ali J. Electricwala

    2016-01-01

    Full Text Available Ewing’s Sarcoma (ES is a highly malignant bone tumour. It may involve any part of the skeleton but the most frequent parts are the ilium and diaphysis of femur and tibia (Alfeeli et al., 2005; Zhu et al., 2012. Primary ES of the spine is extremely rare (Yan et al., 2011. It accounts for only 3.5 to 14.9 percent of all primary bone sarcomas. The age of presentation ranges from 12 to 24 years (median 21 years (Ferguson, 1999; Sharafuddin et al., 1992; Klimo Jr. et al., 2009. We report an unusual case of primary ES of the spine in a two-year-old boy, who presented to us with paraparesis and features of cauda equina syndrome. MRI scan showed a tumour mass arising from the pedicle of L4 vertebra invading the spinal canal. Tc-99 bone scan showed increased tracer uptake in L4 vertebra and normal tracer uptake elsewhere in the skeleton. After reaching the diagnosis of a space occupying lesion invading the lumber spinal canal, we performed a decompressive laminectomy and a biopsy was sent which confirmed the diagnosis of ES. Immunohistochemistry showed tumour cells staining positive for CD-99 (specific stain for ES. Gene testing showed an EWS-FLI 1 chimera. Surgery was followed by good improvement in motor signs. The child was then referred to a specialized oncotherapy centre for further treatment, radiation, and chemotherapy. To the best of our knowledge, we are the first to report primary ES of the spine at the age of two years.

  12. Alendronate prevents postmenopausal bone loss in women without osteoporosis. A double-blind, randomized, controlled trial. Alendronate Osteoporosis Prevention Study Group

    DEFF Research Database (Denmark)

    McClung, M; Clemmesen, B; Daifotis, A

    1998-01-01

    BACKGROUND: Preventing bone loss associated with menopause and aging and maintaining the normal micro-architecture of bone provide important opportunities for the prevention of osteoporosis and fractures. OBJECTIVE: To determine the safety and efficacy of alendronate, an aminobisphosphonate......, for preventing postmenopausal bone loss. DESIGN: 3-year double-blind, randomized, placebo-controlled trial. SETTING: 15 osteoporosis centers throughout the world. PARTICIPANTS: 447 women who had recently experienced menopause (6 to 36 months before study entry). INTERVENTION: Participants were randomly assigned...

  13. 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.

  14. Ewing sarcoma ewsa protein regulates chondrogenesis of Meckel's cartilage through modulation of Sox9 in zebrafish.

    Directory of Open Access Journals (Sweden)

    Chris Merkes

    Full Text Available Ewing sarcoma is the second most common skeletal (bone and cartilage cancer in adolescents, and it is characterized by the expression of the aberrant chimeric fusion gene EWS/FLI1. Wild-type EWS has been proposed to play a role in mitosis, splicing and transcription. We have previously shown that EWS/FLI1 interacts with EWS, and it inhibits EWS activity in a dominant manner. Ewing sarcoma is a cancer that specifically develops in skeletal tissues, and although the above data suggests the significance of EWS, its role in chondrogenesis/skeletogenesis is not understood. To elucidate the function of EWS in skeletal development, we generated and analyzed a maternal zygotic (MZ ewsa/ewsa line because the ewsa/wt and ewsa/ewsa zebrafish appeared to be normal and fertile. Compared with wt/wt, the Meckel's cartilage of MZ ewsa/ewsa mutants had a higher number of craniofacial prehypertrophic chondrocytes that failed to mature into hypertrophic chondrocytes at 4 days post-fertilization (dpf. Ewsa interacted with Sox9, which is the master transcription factor for chondrogenesis. Sox9 target genes were either upregulated (ctgfa, ctgfb, col2a1a, and col2a1b or downregulated (sox5, nog1, nog2, and bmp4 in MZ ewsa/ewsa embryos compared with the wt/wt zebrafish embryos. Among these Sox9 target genes, the chromatin immunoprecipitation (ChIP experiment demonstrated that Ewsa directly binds to ctgfa and ctgfb loci. Consistently, immunohistochemistry showed that the Ctgf protein is upregulated in the Meckel's cartilage of MZ ewsa/ewsa mutants. Together, we propose that Ewsa promotes the differentiation from prehypertrophic chondrocytes to hypertrophic chondrocytes of Meckel's cartilage through inhibiting Sox9 binding site of the ctgf gene promoter. Because Ewing sarcoma specifically develops in skeletal tissue that is originating from chondrocytes, this new role of EWS may provide a potential molecular basis of its pathogenesis.

  15. High dose rate interstitial brachytherapy in soft tissue sarcomas: technical aspect

    Energy Technology Data Exchange (ETDEWEB)

    Chun, Mi Son; Kang, Seung Hee; Kim, Byoung Suck; Oh, Young Taek [College of Medicine, Ajou Univ., Suwon (Korea, Republic of)

    1999-03-01

    To discuss the technical aspect of interstitial brachytherapy including method of implant, insertion time of radioactive source, total radiation dose, and complication, we reviewed patients who had diagnoses of soft tissue sarcoma and were treated by conservative surgery, interstitial implant and external beam radiation therapy. Between May 1995 and Dec. 1997, the patients with primary or recurrent soft tissue sarcoma underwent surgical resection (wide margin excision) and received radiotherapy including interstitial brachytherapy. Catheters were placed with regular intervals of 1-1.5 cm immediately after tumor removal and covering the critical structures, such as neurovascular bundle or bone, with gelform, muscle, or tissue expander in the cases where the tumors were close to those structures. Brachytherapy consisted of source axis with 2-2.5 Gy/fraction, twice a day, starting on 6th day after the surgery. Within one month after the surgery, total dose of 50-55 Gy was delivered to the tumor bed with wide margin by the external beam radiotherapy. All patients completed planned interstitial brachytherapy without acute side effects directly related with catheter implantation such as infection or bleeding. With median follow up duration of 25 months (range 12-41 months), no local recurrences were observed. And there was no severe form of chromic complication (RTOG/EORTC grade 3 or 4). The high dose rate interstitial brachytherapy is easy and safe way to minimize the radiation dose delivered to the adjacent normal tissue and to decrease radiation induced chronic morbidity such as fibrosis by reducing the total dose of external radiotherapy in the management of soft tissue sarcoma with conservative surgery.

  16. 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.

  17. 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.

  18. 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.

  19. Myeloid Sarcoma Presenting as Multiple Lymphadenopathy: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Hong, Sun Hwa; Suh, Sang Il; Seol, Hae Young; Cho, Jea Gu; Shin, Bong Kyung [Guro Hospital, Korea University College of Medicine, Seoul (Korea, Republic of)

    2010-10-15

    Myeloid sarcoma manifesting as multiple lymphadenopathy is quite rare. We present here a case of myeloid sarcoma that first presented with palpable bilateral neck masses. A 53-year-old woman complained about repetitive swelling in the right infraauricular and submental areas for 3 years. The results of computed tomography showed multiple lymphadenopathy in both areas of the neck as well as other parts of the body. So, the presumptive diagnosis was lymphoma, but the result of the excisional biopsy of the neck mass confirmed it to be a myeloid sarcoma.

  20. Extremely underwound chromosomal DNA in nucleoids of mouse sarcoma cells.

    Science.gov (United States)

    Hartwig, M; Matthes, E; Arnold, W

    1981-07-01

    The superhelical properties of chromosomal DNA from cells of a mouse sarcoma were investigated in neutral sucrose gradients containing ethidium bromide. Removal of negative supercoiling from the DNA of the sarcoma cells required a substantially higher dye concentration than was necessary in the case of DNA from cultured mouse fibroblasts. The calculated value of the mean superhelical density in malignant cells (sigma = -0.14) appears abnormally high compared with the value (sigma = -0.09) obtained for DNA of mouse fibroblasts. Chromosomal DNA from mouse sarcoma cells is therefore concluded to be highly deficient in helical turns.

  1. Ewing sarcoma cells secrete EWS/Fli-1 fusion mRNA via microvesicles.

    Directory of Open Access Journals (Sweden)

    Masanori Tsugita

    Full Text Available Tumours defined as Ewing sarcoma (ES constitute a group of highly malignant neoplasms that most often affect children and young adults in the first 2 decades of life. The EWS/Fli-1 fusion gene, a product of the translocation t(11;22 (q24; 12, is detected in 95% of ES patients. Recently, it was validated that cells emit a heterogeneous mixture of vesicular, organelle-like structures (microvesicles, MVs into their surroundings including blood and body fluids, and that these MVs contain a selected set of tumor-related proteins and high levels of mRNAs and miRNAs. In this present study, we detected the Ewing sarcoma-specific EWS/Fli-1 mRNA in MVs from the culture medium of ES cell lines carrying t(11;22 (q24; 12. Also, we detected this fusion gene in approximately 40% of the blood samples from mice inoculated with xenografts of TC135 or A673 cells. These findings indicate the EWS/Fli-1 mRNA in MVs might be a new non-invasive diagnostic marker for specific cases of Ewing sarcoma.

  2. Primary bone tumors of the spine.

    Science.gov (United States)

    Cañete, A Navas; Bloem, H L; Kroon, H M

    2016-04-01

    Primary bone tumors of the spine are less common than metastases or multiple myeloma. Based on the patient's age and the radiologic pattern and topography of the tumor, a very approximate differential diagnosis can be established for an osseous vertebral lesion. This article shows the radiologic manifestations of the principal primary bone tumors of the spine from a practical point of view, based on our personal experience and a review of the literature. If bone metastases, multiple myeloma, lymphomas, hemangiomas, and enostoses are excluded, only eight types of tumors account for 80% of all vertebral tumors. These are chordomas, osteoblastomas, chondrosarcomas, giant-cell tumors, osteoid osteomas, Ewing's sarcomas, osteosarcomas, and aneurysmal bone cysts.

  3. Case Report: Myelodysplastic syndrome- associated myeloid sarcoma: an unusual clinical presentation of a rare disease [version 1; referees: 2 approved

    Directory of Open Access Journals (Sweden)

    Emoke Horvath

    2016-02-01

    Full Text Available Myeloid sarcoma results from the extramedullary homing and proliferation of immature myeloid precursors. We present the timeline, events and diagnostic pitfalls related to a 66 year-old male patient’s case, admitted to the Hematology Clinic for pancytopenia, fever, weight loss and fatigue. The severe cytopenia and the few blasts observed in his blood smear indicated a bone marrow biopsy. The bone marrow showed hypercellularity and multilineage dysplasia with the presence of 15% myeloblasts. After the biopsy, he promptly developed paraplegia and nuclear magnetic resonance revealed an epidural tumour which was then resected.In the epidural tumour mass blast-like, round cells were observed with a complex immunophenotype, characterized by myeloperoxidase, CD117, CD15, CD99, leucocyte common antigen positivity and a high Ki-67 proliferation index. Considering the main differential diagnostic issues, the final diagnosis was stated as myelodysplastic syndrome-associated myeloid sarcoma. The prognosis was unfavourable, the bone marrow was quickly invaded by proliferating blast cells, and despite chemotherapy attempts, the patient died.

  4. Radioimmunoimaging of osteogenic sarcoma xenografts in nude mice using monoclonal antibodies to osteogenic sarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Sakahara, H.; Endo, K.; Nakashima, T.; Koizumi, M.; Ohta, H.; Kunimatsu, M.; Torizuka, K.; Nakamura, T.; Tanaka, H.; Kotoura, Y.

    1985-05-01

    The authors have developed several monoclonal antibodies against human osteogenic sarcoma, one of which; OST7 (IgGl) selectively localized in osteogenic sarcoma xenografts in nude mice. In the present study, F(ab')/sub 2/ fragment was compared with whole IgG and those labeled with In-111 as well as I-131 were used as a radiotracer for the scintigraphic imaging of tumors. IgC and F(ab')/sub 2/ were labeled with I-131 using chloramine-T method and injected into nude mice bearing human osteogenic sarcoma. Scintigrams at day 2 clearly delineated the site of tumors with almost no radioactivity in other organs with F(ab')/sub 2/, which yielded much better images than whole IgG. Tumor-to-blood ratio of 6.09-27.87 was obtained at day 2 using F(ab')/sub 2/, whereas it was 0.76-1.12 at day 2 and 2.05-3.27 at day 7 with IgG. I-131 labeled nonspecific F(ab')/sub 2/ or IgG resulted in no or very low tumor uptake with tumor-to-blood ratio of 0.94-1.18 at day 2 for F(ab')/sub 2/ and 0.67-0.76 at day 7 for IgG, respectively. In-111 labeled F(ab')/sub 2/ fragment of OST7, which was prepared using DTPA as a bifunctional chelate, also showed a high tumor accumulation with tumor-to-blood ratio of 11.67-17.54 at day 2, but higher background activity in the liver and kidney was observed than I-131 labeled one. These results indicate that F(ab')/sub 2/ fragment of OST7 labeled with either I-131 or In-111, has a great potential for the radioimmunoimaging of osteogenic sarcoma.

  5. Myxoinflammatory fibroblastic sarcoma: spectrum of disease and imaging presentation

    Energy Technology Data Exchange (ETDEWEB)

    Gaetke-Udager, Kara; Yablon, Corrie M.; Morag, Yoav [University of Michigan Health System, Department of Radiology, Ann Arbor, MI (United States); Lucas, David R. [University of Michigan Health System, Department of Pathology, Ann Arbor, MI (United States)

    2016-03-15

    To describe the imaging findings of a series of myxoinflammatory fibroblastic sarcomas (MFSs) from our institution, including a case of dedifferentiated MFS and two cases with areas of high-grade tumor, in addition to typical cases of low-grade tumor. To correlate the imaging findings with the pathologic features of these tumors. IRB approval was obtained. Retrospective search of the pathology database at our institution from 2000 to 2015 identified seven cases of MFS with available imaging. Imaging, pathology, and clinical data were reviewed. Unlike the majority of well-differentiated tumors in our series (four cases), one tumor showed dedifferentiation and two cases had areas of high-grade tumor. The dedifferentiated tumor showed peripheral post-contrast enhancement. One case with a substantial high-grade component showed osseous destruction and peripheral enhancement in the high-grade area, while the low-grade component enhanced diffusely. The second case had a small high-grade area and showed diffuse enhancement. All three of these cases had non-acral locations and lacked association with a tendon. The four cases of low-grade MFS demonstrated diffuse enhancement, were located in the distal extremities, and were associated with a tendon. The imaging findings of dedifferentiated and high-grade MFS differ from the more typical low-grade tumors in that they have nonenhancing areas, a non-acral location, lack association with a tendon, and may involve bone. The radiologist should be aware that MFS represents a spectrum that includes low-grade tumors, tumors with high-grade areas, and tumors with dedifferentiation and that this spectrum presents with differing imaging features. (orig.)

  6. 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.

  7. Radiation therapy for Kaposi's sarcoma associated with acquired immunodeficiency syndrome. Tokyo Metropolitan Komagome Hospital experience

    Energy Technology Data Exchange (ETDEWEB)

    Ebara, Takeshi [Municipal Kanbara General Hospital, Fujikawa, Shizuoka (Japan); Karasawa, Katsuyuki; Maebayashi, Katsuya; Kurosaki, Hiromasa; Ishikawa, Hitoshi; Kaizu, Toshihide; Tanaka, Yoshiaki; Akagi, Kumiko; Masuda, Gota

    2000-12-01

    Kaposi's sarcoma is frequently found in association with acquired immunodeficiency syndrome (AIDS). We report on radiotherapy for patients with AIDS-related Kaposi's sarcoma at Tokyo Metropolitan Komagome Hospital. Between April 1991 and May 1997, radiotherapy was given to 11 lesions in eight men with AIDS-related Kaposi's sarcoma to relieve their symptoms. The lesions involved the head and neck region, the legs, and the gastrointestinal tract. Radiotherapy was carried out with 4-MV photon through parallel opposed field or high energy electrons. Total doses ranged from 20 to 38 Gy, with a median of 30 Gy, delivered in 2- to 3-Gy fractions. Four patients were given other treatments prior to the radiotherapy. Acute reaction was evaluated according to the modified acute radiation morbidity scoring criteria of the Radiation Therapy Oncology Group (RTOG). Radiotherapy had relieved the symptoms in all patients at completion of this therapy. Lesions that involved the hard palate and vocal cords had completely disappeared. The lesions that received radiotherapy were controlled without symptoms until the patients died. Patients who had the head and neck region treated exhibited severe acute mucosal reaction (at a dose of 30 Gy, there was grade 2 morbidity by modified RTOG criteria, in two patients, and grade 3 in three patients) although the radiation therapy was completed for these patients. Radiotherapy promises a favorable outcome for symptom relief in AIDS-related Kaposi's sarcoma. (author)

  8. Generalized Lymphadenopathy as the First Presentation of Granulocytic Sarcoma: A Diagnostic Challenge

    Directory of Open Access Journals (Sweden)

    Ghaleb Elyamany

    2013-01-01

    Full Text Available Introduction. Granulocytic sarcoma (GS, also known as chloroma or extramedullary myeloblastoma, is a solid tumor composed of primitive precursors of the granulocytic series that include myeloblasts, promyelocytes, and myelocytes. Granulocytic sarcoma is a rare tumor that may develop during acute myeloid leukemia (AML but less frequently may precede its presentation. Although generalized lymph node enlargement is a presentation for malignant lymphoma, it can also rarely be the early presenting sign of GS. Methods. We present a case of GS mimicking lymphoma in a 45-year-old male. The patient presented with bilateral neck masses and had widespread, prominent lymphadenopathy secondary to AML as the first presenting manifestation of GS for the last 4 months with concurrent marrow AML. Result. A clinical diagnosis of lymphoma was suspected; fine needle aspiration cytology findings were also suggestive of lymphoma. However, peripheral blood and bone marrow examination reported as acute myeloid leukemia with monocytic differentiation and histopathology of excised lymph node confirmed it to be a GS not lymphoma. Conclusion. GS is often misdiagnosed as malignant lymphoma because of cytomorphologic and histologic similarities of the blasts to large cell lymphoma. A careful search for immature myeloid is a useful clue to the diagnosis accompanied with appropriate immunophenotyping.

  9. Case report 423: Clear-cell sarcoma plantar aspect of right foot

    Energy Technology Data Exchange (ETDEWEB)

    Sartoris, D.J.; Resnick, D.; Haghighi, P.

    1987-06-01

    The case is presented of a 65-year-old woman who developed a painful swelling of the plantar aspect of the right foot over a 3-year period. Conventional roentgenograms of the foot showed a large, soft tissue mass on the plantar aspect, with some suggestion of erosion of the adjacent metatarsals. CT studies, however, failed to confirm the presence of any bony abnormalities. At the time of the initial presentation, a biopsy was performed and approximately 3 months later the patient was treated definitely with wide excision of the mass. Histological sections showed the presence of a clear-cell sarcoma - an uncommon lesion mainly confined to the lower extremities and originating either in tendons or aponeurosis. The differential diagnosis, radiologically and clinically was described, and similarly, the differential diagnosis pathologically was also considered. The clinical, pathological and radiological aspects of clear-cell sarcoma of tendon sheath or aponeurosis were discussed in depth and the treatment and prognosis were considered. The authors stress that the importance of the case from a diagnostic imaging perspective lies in the successful use of CT in defining such a soft tissue neoplasm. The ability of CT to determine the extent of involvement of soft tissue and bone by such a lesion is important. Cross-sectional techniques, including magnetic resonance as well as CT, must be the procedure of choice in the definitive diagnosis and treatment of such lesions.

  10. In vitro secretion of TNF-{alpha} from bone marrow mononuclear cells incubated on amino group modified TiO{sub 2} nano-composite under ultrasound irradiation

    Energy Technology Data Exchange (ETDEWEB)

    Furuzono, T., E-mail: furuzono@ri.ncvc.go.jp [Department of Bioengineering, Advanced Medical Engineering Center, National Cardiovascular Center Research Institute, 5-7-1 Fujishiro-dai, Suita, Osaka 565-8565 (Japan); Masuda, M. [Department of Bioengineering, Advanced Medical Engineering Center, National Cardiovascular Center Research Institute, 5-7-1 Fujishiro-dai, Suita, Osaka 565-8565 (Japan); Nitta, N.; Kaya, A.; Yamane, T. [Institute for Human Science and Biomedical Engineering, National Institute of Advanced Industrial Science and Technology, 1-2-1 Namiki, Tsukuba, Ibaraki, 305-8564 (Japan); Okada, M. [Department of Bioengineering, Advanced Medical Engineering Center, National Cardiovascular Center Research Institute, 5-7-1 Fujishiro-dai, Suita, Osaka 565-8565 (Japan)

    2010-10-15

    It is recently known that titanium dioxide (TiO{sub 2}) can be excited by ultrasound and release of OH radicals on the surface. In this study, secretion of an indirect angiogenic factor, tumor necrosis factor-{alpha} (TNF-{alpha}), from bone marrow mononuclear cells (BM-MNC) incubated on amino group modified TiO{sub 2} nano-particles covalently coated on polyester fabric (TiO{sub 2}/PET) under ultrasonic irradiation was examined in vitro. The cell viability and TNF-{alpha} secretion were measured under ultrasound irradiation condition with 255 mW/cm{sup 2} of intensity, which is below the highest output (1 W/cm{sup 2}) specified in the safety standard for a medical ultrasonic diagnostic apparatus. The living cell number on the TiO{sub 2}/PET and original PET with/without continuous ultrasound irradiation was unchanged statistically by ANOVA test. TNF-{alpha} secretion level from BM-MNC remarkably increased on the TiO{sub 2}/PET under ultrasonic irradiation without cell damage. It was, therefore, thought that the high level of TNF-{alpha} secretion on the TiO{sub 2} nano-composite by ultrasound irradiation was due to oxidative stress induced from OH radicals on TiO{sub 2}.

  11. 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.

  12. Fine needle cytology of Kaposi's sarcoma in heterosexual male

    Directory of Open Access Journals (Sweden)

    Anjali R. Dhote

    2014-04-01

    Full Text Available Kaposi's sarcomas the most common malignancy associated with Human Herpesvirus-8 (HHV8 infection. Though name is sarcoma but it is low grade vascular neoplasm. It is the tumour which arises from endothelial lining of vessels as well as lymphatic channels. So it involved all sites such as skin, Gastro intestine, lungs along with lymph nodes. We are presenting one such case of 65 year immunocompromised Indian male presented with multiple non blanching reddish bluish nodules on all extremities, chest, back with submandibular and cervical lymphadenopathy. Fine needle aspiration cytology (FNAC was performed and diagnosis was given low grade spindle cell neoplasm consistent with Kaposi's sarcoma which was confirmed on histopathology as Kaposi's sarcoma. [Int J Res Med Sci 2014; 2(2.000: 789-791

  13. Dasatinib and Doxorubicin Treatment of Sarcoma Initiating Cells

    DEFF Research Database (Denmark)

    Aggerholm-Pedersen, Ninna; Demuth, Christina; Safwat, Akmal;

    2016-01-01

    Background. One of the major challenges affecting sarcoma treatment outcome, particularly that of metastatic disease, is resistance to chemotherapy. Cancer-initiating cells are considered a major contributor to this resistance. Methods. An immortalised nontransformed human stromal (mesenchymal) s...

  14. Radiation-induced spindle cell sarcoma: A rare case report

    Directory of Open Access Journals (Sweden)

    Khan Mubeen

    2009-01-01

    Full Text Available Ionizing radiation has been known to induce malignant transformation in human beings. Radiation-induced sarcomas are a late sequel of radiation therapy. Most sarcomas have been reported to occur after exposure to a radiation dose of 55 Gray (Gy and above, with a dose ranging from 16 to 112 Gys. Spindle cell sarcomas, arising after radiotherapy given to treat the carcinoma of head and neck region is a very uncommon sequel. This is a rare case report of spindle cell sarcoma of left maxilla, in a 24-year-old male, occurring as a late complication of radiotherapy with Cobalt-60 given for the treatment of retinoblastoma of the left eye 21 years back.

  15. 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.

  16. Breast sarcoma. A case report and review of literature

    Directory of Open Access Journals (Sweden)

    Nuria Li

    2016-01-01

    Conclusion: Surgery represents the only potentially curative therapy for breast sarcoma. Tumor size and adequate resection margin are the most important prognostic factors. Approximately 80% of recurrences appear in the first two years.

  17. Biomechanics Significance of Femoral Head and Neck of Grafting-bone Group After Femoral Neck Fracture-healing%股骨颈骨折愈合后钉道植骨的生物力学意义

    Institute of Scientific and Technical Information of China (English)

    唐洪涛; 仝允辉; 杨茹萍; 朱太永; 张美超; 赵卫东

    2011-01-01

    Objective To study biomechanical performance of fracture-healing femoral head and neck after the hollow compressionb screws are taken out in ungrafting-bone group, grafting-bone group, normal group, and provide theoretical basis for the treatment of necrosis of the femoral head by bone grafting in the channel of screws. Methods Nine femurs fixed and preserved by formalin were used in this test. They were randomly divided into three groups: normal group, ungrafting-bone group, grafting-bone group. According to inversed-triangle, at the range of physiological load, it tested the defixed bias-value of three group femoral head at different load. The data were collected and analyzed. The three-dimension finite element model of the femoral head and neck was calculated. Nodal solution of stress value and max-offset was calculated at area of femoral head and neck, then the data were compared and analyzed.Results Compared with the mean of load-offset about the femoral head and neck, biomechanical capability of ungrafting-bone group was lowest with regard to other two groups , the influence was very hard, P <0.05. But there was no singnificant difference between normal group and graflng-bone group, P >0.05. Analysis of the three-dimension finite element model showed that the massive stress was concentrated at weight loading region of ungrafting-bone group's femoral head, it was an dangerous chance to lead to femoral head collapse. Conclusion For the treatment of femur neck fractures with cannulated compression screws, bone grafting in the channel of screw should be used to improve biomechanical integrity after fracture healed and cannulated compression screws are taken out. This is of benefit to loading in early stage and to preventing the collapse of femoral head, etc.%目的 探讨股骨颈骨折愈合后取出空心加压螺钉遗留钉道不植骨、植骨及正常的股骨头颈部的生物力学特性,为临床提供实验依据.方法 收集9付股骨标本测量

  18. The second European interdisciplinary Ewing sarcoma research summit – A joint effort to deconstructing the multiple layers of a complex disease

    Science.gov (United States)

    2016-01-01

    Despite multimodal treatment, long term outcome for patients with Ewing sarcoma is still poor. The second “European interdisciplinary Ewing sarcoma research summit” assembled a large group of scientific experts in the field to discuss their latest unpublished findings on the way to the identification of novel therapeutic targets and strategies. Ewing sarcoma is characterized by a quiet genome with presence of an EWSR1-ETS gene rearrangement as the only and defining genetic aberration. RNA-sequencing of recently described Ewing-like sarcomas with variant translocations identified them as biologically distinct diseases. Various presentations adressed mechanisms of EWS-ETS fusion protein activities with a focus on EWS-FLI1. Data were presented shedding light on the molecular underpinnings of genetic permissiveness to this disease uncovering interaction of EWS-FLI1 with recently discovered susceptibility loci. Epigenetic context as a consequence of the interaction between the oncoprotein, cell type, developmental stage, and tissue microenvironment emerged as dominant theme in the discussion of the molecular pathogenesis and inter- and intra-tumor heterogeneity of Ewing sarcoma, and the difficulty to generate animal models faithfully recapitulating the human disease. The problem of preclinical development of biologically targeted therapeutics was discussed and promising perspectives were offered from the study of novel in vitro models. Finally, it was concluded that in order to facilitate rapid pre-clinical and clinical development of novel therapies in Ewing sarcoma, the community needs a platform to maintain knowledge of unpublished results, systems and models used in drug testing and to continue the open dialogue initiated at the first two Ewing sarcoma summits. PMID:26802024

  19. Sarcoma cutáneo mixto radioinducido Radiation-induced mixed cutaneous sarcoma

    Directory of Open Access Journals (Sweden)

    Isidoro Rubio-Correa

    2012-06-01

    Full Text Available Introducción: Los sarcomas son tumores malignos poco frecuentes, siendo raros en cabeza y cuello. En su etiología se involucran factores como agentes químicos, radiación, inmunosupresión y síndromes y anomalías genéticas. Caso clínico: Varón de 64 años, que presenta lesión en piel de mejilla derecha de un año de evolución, localización en la que presentó hace veinte años un carcinoma basocelular tratado con radioterapia. Tras descartar existencia de metástasis, se realizó exéresis de la lesión con márgenes de seguridad y reconstrucción con colgajo de Mustardé. Se complementó el tratamiento con radioterapia. Discusión: El diagnóstico es anatomopatológico, siendo fundamental descartar afectación metastásica. Para mejorar la supervivencia y disminuir su elevada tasa de recidiva, deberían tratarse de forma multidisciplinar (cirugía, radioterapia y/o quimioterapia. Conclusión: A pesar de su baja frecuencia, los sarcomas deben estar presentes en el diagnóstico diferencial de toda lesión que aparezca en zonas radiadas previamente, especialmente en la piel facial.Introduction: Sarcomas are malignant tumors that are infrequent, being rare in the head and neck. Factors such as chemical agents, radiation, immunosuppression, and genetic syndromes and abnormalities are involved in their etiology. Case report: A 64-year-old man developed a skin lesion on the right cheek one year earlier at the site where he had presented a basal cell carcinoma 20 years earlier that was treated with radiation therapy. After ruling out the existence of metastasis, the lesion was treated by surgical resection with safety margins and reconstruction with the Mustardé flap. Treatment was supplemented with radiation therapy. Discussion: The diagnosis of sarcomas is histopathologic and it is essential to rule out metastasis. To improve survival and reduce the high rate of recurrence, a multidisciplinary approach to treatment should be used (surgery

  20. 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.

  1. Hepatic involvement of histiocytic sarcoma: CT and MRI findings

    Energy Technology Data Exchange (ETDEWEB)

    Kubo, Takatosh; Ohtomo, Kuni [Graduate School of Medicine, University of Tokyo, Tokyo (Japan); Kiryu, Shigeru; Akai, Hiroyuki; Ora, Yasunori; Tojo, Arinobu; Yoshida, Hideo; Kato, Naoya; Nakano, Yoshiyasu [Institute of Medical Science, University of Tokyo, Tokyo (Japan)

    2016-09-15

    Histiocytic sarcoma in the liver is an extremely rare hematological malignancy. Herein, we reported the case of a 68-year-old woman who presented with characteristic wedge-shaped abnormality bounded by hepatic veins on computed tomography and magnetic resonance imaging of the liver. In the wedge-shaped area, decreased portal flow and the deposition of iron were observed. These imaging findings are consistent with intrasinusoidal tumor cell infiltration. A liver biopsy was performed, and histiocytic sarcoma was confirmed histopathologically.

  2. Hepatic Involvement of Histiocytic Sarcoma: CT and MRI Findings

    Energy Technology Data Exchange (ETDEWEB)

    Kubo, Takatoshi [Department of Radiology, Graduate School of Medicine, University of Tokyo, Tokyo 113-8654 (Japan); Kiryu, Shigeru; Akai, Hiroyuki [Department of Radiology, Institute of Medical Science, University of Tokyo, Tokyo 108-8639 (Japan); Ota, Yasunori [Department of Pathology, Institute of Medical Science, University of Tokyo, Tokyo 108-8639 (Japan); Tojo, Arinobu [Department of Hematology and Oncology, Institute of Medical Science, University of Tokyo, Tokyo 108-8639 (Japan); Yoshida, Hideo [Department of Gastroenterology, Japanese Red Cross Medical Center, Tokyo 150-8935 (Japan); Kato, Naoya [Advanced Medical Science, Institute of Medical Science, University of Tokyo, Tokyo 108-8639 (Japan); Nakano, Yoshiyasu [Department of Radiology, Institute of Medical Science, University of Tokyo, Tokyo 108-8639 (Japan); Ohtomo, Kuni [Department of Radiology, Graduate School of Medicine, University of Tokyo, Tokyo 113-8654 (Japan)

    2016-11-01

    Histiocytic sarcoma in the liver is an extremely rare hematological malignancy. Herein, we reported the case of a 68-year-old woman who presented with characteristic wedge-shaped abnormality bounded by hepatic veins on computed tomography and magnetic resonance imaging of the liver. In the wedge-shaped area, decreased portal flow and the deposition of iron were observed. These imaging findings are consistent with intrasinusoidal tumor cell infiltration. A liver biopsy was performed, and histiocytic sarcoma was confirmed histopathologically.

  3. Myxoinflammatory fibroblastic sarcoma: An uncommon tumour at an unusual site

    Directory of Open Access Journals (Sweden)

    Varuna Mallya

    2014-01-01

    Full Text Available Myxoinflammatory fibroblastic sarcoma is a low grade sarcoma that is composed of a mixed inflammatory infiltrate along with spindled, epithelioid and bizarre appearing cells in a background of hyaline and myxoid zones. Seen affecting the distal extremities commonly, with an equal sex predilection, these tumors are rare and require an extensive immunohistochemical work up for proper diagnosis. They have a tendency to recur.

  4. Targeting the p53 Pathway in Ewing Sarcoma

    OpenAIRE

    Neilsen, Paul M.; Pishas, Kathleen I.; Callen, David F; Thomas, David M.

    2011-01-01

    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 downs...

  5. Primary Kaposi sarcoma of the subcutaneous tissue

    Directory of Open Access Journals (Sweden)

    Dezube Bruce J

    2008-09-01

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

  6. Imaging features of alveolar soft part sarcoma

    Institute of Scientific and Technical Information of China (English)

    Teng Jin; Ping Zhang Co-first author; Xiaoming Li

    2015-01-01

    Objective The aim of this study was to analyze the imaging features of alveolar soft part sarcoma (ASPS). Methods The imaging features of 11 cases with ASPS were retrospectively analyzed. Results ASPS mainly exhibited an isointense or slightly high signal intensity on T1-weighted imaging (T1WI), and a mixed high signal on T2-weighted imaging (T2WI). ASPS was partial, with rich tortuous flow voids, or “line-like” low signal septa. The essence of the mass was heterogeneous enhancement. The 1H-MRS showed a slight choline peak at 3.2 ppm. Conclusion The wel-circumscribed mass and blood voids, combined with “line-like” low signals play a significant role in diagnosis. The choline peak and the other signs may be auxiliary diagnoses.

  7. Myxoinflammatory fibroblastic sarcoma of the face.

    Science.gov (United States)

    Gómez Martín, Cristina; Ortega, María I; Aramburu, José A; Fernández-Cañamaque, José L

    2012-08-01

    Myxoinflammatory fibroblastic sarcoma (MIFS) is a rare low-grade tumor of modified fibroblasts, with tendency to local recurrence. This unusual entity typically presents as a slow-growing painless mass in the distal extremities of middle-aged subjects. A 48-year-old woman presented to our clinic with a painless subcutaneous mass in the right temporal region. Excisional biopsy made the rare diagnosis of MIFS. Histologic examination showed the unique features that characterize this lesion: a myxoid component with a superimposed inflammatory infiltrate and the presence of distinctive, large, and bizarre Reed-Stemberg-like cells. A second wide tumor bed resection was performed, achieving clear margins. No adjuvant therapy was administered, and the patient is free of disease at 18 months postoperatively. To the best of our knowledge, this is the first reported case of MIFS presenting in the face. This adds another possibility for differential diagnoses of soft tissue tumors of the face.

  8. Epithelioid sarcoma with unusual radiological findings

    Energy Technology Data Exchange (ETDEWEB)

    Yamato, M.; Nishimura, G. [Department of Radiology, Dokkyo University School of Medicine, Tochigi (Japan); Yamaguchi, Takehiko [Department of Pathology, Dokkyo University School of Medicine, Tochigi (Japan); Tamai, Kazuya; Saotome, Koichi [Department of Orthopaedic Surgery, Dokkyo University School of Medicine, Tochigi (Japan)

    1997-10-01

    The case of a patient with epithelioid sarcoma in the right arm is reported. The diagnosis was delayed because of misinterpretation arising from complexity in the MR findings, including a honeycomb pattern in the subcutaneous fat simulating lymphedema, and an intramuscular diffuse high signal intensity on T2-weighted images without a discrete mass lesion. The histological findings revealed that the diffuse muscular abnormality mainly resulted from denervation of the muscles due to perineural invasion by the tumor, and subcutaneous edema from lymphedema secondary to lymphatic tumor spread concurrent with lymphatic fibrosis. Multiple foci of cortical erosions in the humerus, a rare manifestation of this tumor, were detected 6 months later. (orig.) 11 refs.

  9. 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.

  10. Endpoint comparison for bone mineral density measurements in North Central Cancer Treatment Group cancer clinical trials N02C1 and N03CC (Alliance)

    Science.gov (United States)

    Singh, J.; Atherton, P.; Liu, H.; Novotny, P.; Hines, S.; Loprinzi, C. L.; Perez, E. A.; Tan, A.; Burger, K.; Zhao, X.; Diekmann, B.; Sloan, J. A.

    2015-01-01

    Summary Bone mineral density (BMD) measurement can vary depending upon anatomical site, machine, and normative values used. This analysis compared different BMD endpoints in two clinical trials. Trial results differed across endpoints. Future clinical trials should consider inclusion of multiple endpoints in sensitivity analysis to ensure sound overall study conclusions. Introduction Methodological issues hamper efficacy assessment of osteoporosis prevention agents in cancer survivors. Osteoporosis diagnosis can vary depending upon which bone mineral density (BMD) anatomical site and machine is used and which set of normative values are applied. This analysis compared different endpoints for osteoporosis treatment efficacy assessment in two clinical studies. Methods Data from North Central Cancer Treatment Group phase III clinical trials N02C1 and N03CC (Alliance) were employed involving 774 patients each comparing two treatments for osteoporosis prevention. Endpoints for three anatomical sites included raw BMD score (RawBMD); raw machine-based, sample-standardized, and reference population-standardized T scores (RawT, TSamp, TRef); and standard normal percentile corresponding to the reference population-standardized T score (TPerc). For each, treatment arm comparison was carried out using three statistical tests using change and percentage change from baseline (CB, %CB) at 1 year. Results Baseline correlations among endpoints ranged from 0.79 to 1.00. RawBMD and TPerc produced more statistically significant results (14 and 19 each out of 36 tests) compared to RawT (11/36), TSamp (8/36), and TRef (7/36). Spine produced the most statistically significant results (26/60) relative to femoral neck (20/60) and total hip (13/60). Lastly, CB resulted in 44 statistically significant results out of 90 tests, whereas %CB resulted in only 15 significant results. Conclusions Treatment comparisons and interpretations were different across endpoints and anatomical sites

  11. Myeloid Sarcoma and Acute Myelomonocytic Leukemia in an Adolescent with Tetrasomy 8: Staging With {sup 18}F-FDG PET/CT

    Energy Technology Data Exchange (ETDEWEB)

    Makis, William [Brandon Regional Health Centre, Brandon (Canada); Rakheja, Rajan; Lavoie, Josee; Marc Hickeson [McGill Univ. Health Centre, Brandon (Canada)

    2012-06-15

    Tetrasomy 8 is a relatively rare chromosomal abnormality that has been reported in only 33 cases in hematologic disorders, It is known for its association with aggressive acute myeloid leukemia (AML) and myeloid sarcoma and is considered a very poor prognostic factor. Myeloid sarcoma is a rare hematologic malignancy characterized by tumor masses consisting of immature myeloid cells, presenting at an extramedullary site. We present a case of a 17-year-old boy referred for an {sup 18}F-FDG PET/CT for the evaluation of pleural masses and spinal bone lesions seen on CT, after presenting with a 4 month history of chest pain. The PET/CT revealed extensive FDG-avid extrame-dullary disease in the soft tissues of the chest, abdomen, and pelvis, which were biopsy-proven to be myeloid sarcoma, as well as extensive intramedullary disease biopsy proven to be AML. This is the first report of the use of {sup 18}F-FDG PET/CT to stage a subset of aggressive AML and myeloid sarcoma in a patient with an associated chromosomal abnormality (tatrasomy 8)

  12. Picropodophyllin inhibits the growth of Ewing's sarcoma cells through the insulin‑like growth factor‑1 receptor/Akt signaling pathway.

    Science.gov (United States)

    Wu, Yong-Tao; Wang, Bao-Jun; Miao, Sheng-Wu; Gao, Jian-Jun

    2015-11-01

    Ewing's sarcoma (ES) is the second most common type of pediatric bone tumor, and is associated with a poor prognosis. Picropodophyllin (PPP), a novel selective inhibitor of insulin‑like growth factor‑1 receptor (IGF‑1R), is able to strongly inhibit various types of cancers. However, the effect of IGF‑1R on ES remains unclear. Following treatment with various concentrations of PPP for various times, cell viability was determined using an MTT assay. In addition, cell proliferation and apoptosis was investigated separately by bromodeoxyuridine staining and flow cytometry, respectively. The PPP‑associated signaling pathway was also investigated. The results of the present study suggested that PPP inhibited cell proliferation and viability of A673 and SK‑ES‑1 human Ewing's sarcoma cells in a dose- and time‑dependent manner. In addition, cell apoptosis rates were increased following treatment with PPP. Further investigation of the underlying mechanism revealed that PPP inhibited Akt phosphorylation. Fumonisin B1, an Akt‑specific activator, reversed the inhibitory effects of PPP on cell growth. Furthermore, the results suggested that PPP decreased the expression levels of IGF‑1R, a common activator of Akt signaling. PPP inhibited the growth of human Ewing's sarcoma cells by targeting the IGF‑1R/Akt signaling pathway. Therefore, PPP may prove useful in the development of an effective strategy for the treatment of Ewing's sarcoma.

  13. Kaposi’s Sarcoma Herpesvirus Genome Persistence

    Directory of Open Access Journals (Sweden)

    Franceline Juillard

    2016-08-01

    Full Text Available Kaposi’s sarcoma-associated herpesvirus (KSHV has an etiologic role in Kaposi’s sarcoma, primary effusion lymphoma and multicentric Castleman’s disease. These diseases are most common in immunocompromised individuals, especially those with AIDS. Similar to all herpesviruses, KSHV infection is lifelong. KSHV infection in tumor cells is primarily latent, with only a small subset of cells undergoing lytic infection. During latency, the KSHV genome persists as a multiple copy, extrachromosomal episome in the nucleus. In order to persist in proliferating tumor cells, the viral genome replicates once per cell cycle and then segregates to daughter cell nuclei. KSHV only expresses several genes during latent infection. Prominent among these genes, is the latency-associated nuclear antigen (LANA. LANA is responsible for KSHV genome persistence and also exerts transcriptional regulatory effects. LANA mediates KSHV DNA replication and in addition, is responsible for segregation of replicated genomes to daughter nuclei. LANA serves as a molecular tether, bridging the viral genome to mitotic chromosomes to ensure that KSHV DNA reaches progeny nuclei. N-terminal LANA attaches to mitotic chromosomes by binding histones H2A/H2B at the surface of the nucleosome. C-terminal LANA binds specific KSHV DNA sequence and also has a role in chromosome attachment. In addition to the essential roles of N- and C-terminal LANA in genome persistence, internal LANA sequence is also critical for efficient episome maintenance. LANA’s role as an essential mediator of virus persistence makes it an attractive target for inhibition in order to prevent or treat KSHV infection and disease.

  14. Proliferation of Ewing sarcoma cell lines is suppressed by the receptor tyrosine kinase inhibitors gefitinib and vandetanib

    Directory of Open Access Journals (Sweden)

    Åman Pierre

    2008-01-01

    Full Text Available Abstract Background Tyrosine kinase inhibitors (TKIs have gained much attention in recent years as targeted agents for the treatment of a wide range of human cancers. We have investigated the effect of the TKIs gefitinib and vandetanib on tumor cell lines derived from Ewing sarcoma, a highly malignant tumor affecting bone and soft tissue in children and young adults. Gefitinib is an inhibitor of epidermal growth factor receptor tyrosine kinase activity (EGFR and vandetanib selectively targets vascular endothelial growth factor receptor-2 (VEGFR-2 with additional activity against VEGFR-3, EGFR and RET kinase receptors. Results Two Ewing sarcoma cell lines investigated showed high levels of nuclear EGFR expression as well as moderate expression in plasma membrane and cytoplasm. When treated with concentrations of 5 μM and more of either gefitinib or vandetanib, we observed a significant decrease in cell proliferation. However, there were no detectable changes in p44/42 MAPK and Akt-1 phosphorylation, or in the expression of cyclin D1 or c-Myc following gefitinib or vandetanib treatment. Conclusion We conclude that Ewing sarcoma tumor cell proliferation is not highly sensitive to inhibition of EGFR signaling alone or the simultaneous inhibition of VEGFR receptors, EGFR and RET kinase. Decreased tumor cell proliferation could be achieved with gefitinib and vandetanib, but only at higher doses where non-specific effects of the compounds may be overriding. As Ewing tumor cells do not seem to depend on EGFR and VEGFR pathways for survival, other key factors in the cellular signaling of Ewing sarcoma should be targeted in order to obtain a potent therapeutic response.

  15. 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.

  16. 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.

  17. Guidance for the management of breast cancer treatment-induced bone loss: a consensus position statement from a UK Expert Group.

    Science.gov (United States)

    Reid, David M; Doughty, Julie; Eastell, Richard; Heys, Steven D; Howell, Anthony; McCloskey, Eugene V; Powles, Trevor; Selby, Peter; Coleman, Robert E

    2008-01-01

    In postmenopausal women, the use of aromatase inhibitors increases bone turnover and induces bone loss at sites rich in trabecular bone at an average rate of 1-3% per year leading to an increase in fracture incidence compared to that seen during tamoxifen use. The bone loss is much more marked in young women with treatment-induced ovarian suppression followed by aromatase inhibitor therapy (average 7-8% per annum). Pre-treatment with tamoxifen for 2-5 years may reduce the clinical significance of the adverse bone effects associated with aromatase inhibitors, particularly if this leads to a shortening in the duration of exposure to an aromatase inhibitor. However, skeletal status should still be assessed at the commencement of aromatase inhibitor therapy. The rate of bone loss in women who experience a premature menopause before the age of 45 or are receiving ovarian suppression therapy is accelerated by the concomitant use of aromatase inhibitors. These patients are considered to be at high risk of clinically important bone loss and should have a baseline dual energy X-ray absorptiometry (DXA) assessment of bone mineral density (BMD). Randomised clinical trials in postmenopausal women indicate that bisphosphonates prevent the bone loss and accelerated bone turnover associated with aromatase inhibitor therapy and are a promising strategy for the prevention and treatment of osteoporosis in this setting. Treatment initiation recommendations are based on a combination of risk factors for osteoporotic fracture and BMD levels. Bisphosphonates, along with a healthy lifestyle and adequate intake of calcium and vitamin D are the treatments of choice to prevent bone loss. Due to the rate of bone loss associated with breast cancer treatments, and uncertainties about the interaction between aromatase inhibitor use and BMD for fracture risk, the threshold for intervention has been set at a higher level than that generally recommended for postmenopausal osteoporosis. Management

  18. Complications of bone tumors after multimodal therapy

    Energy Technology Data Exchange (ETDEWEB)

    Shapeero, L.G., E-mail: lshapeero@usuhs.edu [Department of Radiology, Uniformed Services University of the Health Sciences, 4301 Jones Bridge Road, Bethesda, MD 20814 (United States); Bone and Soft Tissue Program, United States Military Cancer Institute, 6900 Georgia Ave, NW, Washington, DC 20307 (United States); Poffyn, B. [Department of Orthopaedic Surgery, Ghent University Hospital, De Pintelaan 185, B-9000 Ghent (Belgium); De Visschere, P.J.L. [Department of Radiology and Magnetic Resonance/MR-1K12 IB, Ghent University Hospital, De Pintelaan 185, B-9000 Ghent (Belgium); Sys, G. [Department of Orthopaedic Surgery, Ghent University Hospital, De Pintelaan 185, B-9000 Ghent (Belgium); Uyttendaele, D. [Department of Radiology and Magnetic Resonance/MR-1K12 IB, Ghent University Hospital, De Pintelaan 185, B-9000 Ghent (Belgium); Vanel, D. [Department of Radiology, Rizzoli Institute, 40136 Bologna (Italy); Forsyth, R. [Department of Pathology, Ghent University Hospital, De Pintelaan 185, B-9000 Ghent (Belgium); Verstraete, K.L. [Department of Radiology and Magnetic Resonance/MR-1K12 IB, Ghent University Hospital, De Pintelaan 185, B-9000 Ghent (Belgium)

    2011-01-15

    Purpose: To define and compare the complications of bone tumors after resection, extracorporeal irradiation and re-implantation, with or without radiotherapy. Materials and methods: Eighty patients (40 males and 40 females, ages 4-77 years) with 61 malignant and 19 benign bone tumors were evaluated for local and distant complications after treatment. Two groups of patients were studied: (1) 53 patients had resection without (43 patients) or with external beam radiotherapy (RadRx) (10 patients) and (2) 27 patients underwent extracorporeal irradiation and re-implantation without (22 patients) or with RadRx (5 patients). Patient follow-up varied from 1 month to 13.63 years with mean follow-up of 4.7 years. Imaging studies included bone and chest radiography, spin echo T1- and T2-weighted (or STIR) magnetic resonance imaging (MRI), dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI), computed tomography (CT) for thoracic and abdominopelvic metastases and 3-phase technetium-99m-labeled-methylene-diphosphonate (Tc99m MDP) scintigraphy for bone metastases. Results: DCE-MRI differentiated the rapidly enhancing recurrences, residual tumors and metastases from the slowly enhancing inflammation, and the non-enhancing seromas and fibrosis. Recurrences, metastases (mainly to lung and bone), and seromas were greater than twice as frequent in patients after resection than after ECCRI. Although 11.3% of post-resection patients had residual tumor, no ECRRI-treated patient had residual tumor. In contrast, after ECRRI, infection was almost three times as frequent and aseptic loosening twice as frequent as compared with the post-resection patients. Bones treated with RadRx and/or ECRRI showed increased prevalence of fractures and osteoporosis. In addition, muscle inflammation was more common in the externally irradiated patient as compared with the patient who did not receive this therapy. However, another soft tissue complication, heterotopic ossification, was rare in the

  19. 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...

  20. Ewing sarcoma family of tumors: a model for the new era of integrated laboratory diagnostics.

    Science.gov (United States)

    Khoury, Joseph D

    2008-01-01

    The Ewing sarcoma family of tumors (ESFT) represents one of the best models illustrating the multifaceted approach to the diagnosis of cancer that has evolved over the past decade. ESFT encompasses tumors that arise in bone or soft tissues and may have disparate histologic features. As a result, it was not until the discovery that these tumors share a common underlying molecular pathogenesis (chromosomal translocations involving the EWS gene and one of several members of the ETS family of transcription factors) that significant advances in the diagnosis and therapy of ESFT became possible. As a result, ESFT has come to embody the amalgamation of classical diagnostic tools, such as histology and routine microscopy, with newer techniques, such as immunohistochemistry and molecular techniques; the latter include PCR-based methods and fluorescence in situ hybridization. This review will address the features of ESFT and how it has emerged as a model for the new era of integrated diagnostics.

  1. Primary alveolar soft part sarcoma of vertebra: a case report and literature review

    Energy Technology Data Exchange (ETDEWEB)

    Zhu, Fei-Peng [Nanjing University, Department of Medical Imaging, Jinling Hospital, Clinical School of Medical College, Nanjing, Jiangsu Province (China); Xuzhou Medical College, Xuzhou, Jiangsu Province (China); Lu, Guang-Ming; Zhang, Long-Jiang [Nanjing University, Department of Medical Imaging, Jinling Hospital, Clinical School of Medical College, Nanjing, Jiangsu Province (China); Wang, Jian-Dong [Nanjing University, Department of Pathology, Jinling Hospital, Clinical School of Medical College, Nanjing, Jiangsu Province (China); An, Xiao-Jing [Nanjing University, Department of Pathology, Jinling Hospital, Clinical School of Medical College, Nanjing, Jiangsu Province (China); Nanjing University, Medical College, Nanjing, Jiangsu Province (China); Dong, Ying-Chun [Nanjing Stomatology Hospital/The Affiliated Stomatology Hospital of Medical School of Nanjing University, Department of Anesthesiology, Nanjing, Jiangsu Province (China)

    2009-08-15

    Alveolar soft part sarcoma (ASPS) is a rare malignant soft tissue tumor, which rarely occurs in bone. We present a case of ASPS in a 23-year-old man with a 2-month history of back pain. Computed tomography scanning and magnetic resonance images demonstrated a destructive process in the 12th thoracic vertebra associated with a unilateral soft tissue mass. The tumor showed evidence of hypervascularity on MRI; it obviously was enhanced on T1-weighted images after injection of Gd-GDPA, and signal voids were shown on all pulse sequences which may help to differentiate ASPS from other tumors of the vertebra. We believe that this is the first case of ASPS arising in a vertebra. (orig.)

  2. Bone Markers

    Science.gov (United States)

    ... markers may be seen in conditions such as: Osteoporosis Paget disease Cancer that has spread to the bone (metastatic bone disease) Hyperparathyroidism Hyperthyroidism Osteomalacia in adults and rickets in children—lack of bone mineralization, ...

  3. Bone scan

    Science.gov (United States)

    ... legs, or spine fractures) Diagnose a bone infection (osteomyelitis) Diagnose or determine the cause of bone pain, ... 2015:chap 43. Read More Broken bone Metabolism Osteomyelitis Review Date 12/10/2015 Updated by: Jatin ...

  4. Bone Cancer

    Science.gov (United States)

    Cancer that starts in a bone is uncommon. Cancer that has spread to the bone from another ... more common. There are three types of bone cancer: Osteosarcoma - occurs most often between ages 10 and ...

  5. Bone Diseases

    Science.gov (United States)

    Your bones help you move, give you shape and support your body. They are living tissues that rebuild constantly ... childhood and your teens, your body adds new bone faster than it removes old bone. After about ...

  6. [Biochemical markers of bone remodeling: pre-analytical variations and guidelines for their use. SFBC (Société Française de Biologie Clinique) Work Group. Biochemical markers of bone remodeling].

    Science.gov (United States)

    Garnero, P; Bianchi, F; Carlier, M C; Genty, V; Jacob, N; Kamel, S; Kindermans, C; Plouvier, E; Pressac, M; Souberbielle, J C

    2000-01-01

    Biochemical markers of bone turnover have been developed over the past 20 years that are more specific for bone tissue than conventional ones such as total alkaline phosphatase and urinary hydroxyproline. They have been widely used in clinical research and in clinical trials of new therapies as secondary end points of treatment efficacy. Most of the interest has been devoted to their use in postmenopausal osteoporosis, a condition characterized by subtle modifications of bone metabolism that cannot be detected readily by conventional markers of bone turnover. Although several recent studies have suggested that biochemical markers may be used for the management of the individual patient in routine clinical practice, this has not been clearly defined and is a matter of debate. Because of the crucial importance to clarify this issue, the Société Francaise de Biologie Clinique prompted an expert committee to summarize the available data and to make recommendations. The following paper includes a review on the biochemical and analytical aspects of the markers of bone formation and resorption and on the sources of variability such as sex, age, menstrual cycle, pregnancy and lactation, physical activity, seasonal variation and effects of diseases and treatments. We will also describe the effects of pre-analytical factors on the measurements of the different markers. Finally based on that review, we will make practical recommendations for the use of these markers in order to minimize the variability of the measurements and improve the clinical interpretation of the data.

  7. Effects of TRAIL and taurolidine on apoptosis and proliferation in human rhabdomyosarcoma, leiomyosarcoma and epithelioid cell sarcoma.

    Science.gov (United States)

    Karlisch, C; Harati, K; Chromik, A M; Bulut, D; Klein-Hitpass, L; Goertz, O; Hirsch, T; Lehnhardt, M; Uhl, W; Daigeler, A

    2013-03-01

    Soft tissue sarcomas (STS) are a heterogeneous group of malignant tumours representing 1% of all malignancies in adults. Therapy for STS should be individualised and multimodal, but complete surgical resection with clear margins remains the mainstay of therapy. Disseminated soft tissue sarcoma still represents a therapeutic dilemma. Commonly used chemotherapeutic agents such as doxorubicin and ifosfamide have proven to be effective in fewer than 30% in these cases. Therefore, we tested the apoptotic and anti-proliferative in vitro effects of TNF-related apoptosis-inducing ligand (TRAIL) and taurolidine (TRD) on rhabdomyosarcoma (A-204), leiomyosarcoma (SK-LMS-1) and epithelioid cell sarcoma (VA-ES-BJ) cell lines. Viability, apoptosis and necrosis were quantified by FACS analysis (propidium iodide/Annexin V staining). Gene expression was analysed by DNA microarrays and the results validated for selected genes by rtPCR. Protein level changes were documented by western blot analysis. Cell proliferation was analysed by BrdU ELISA assay. The single substances TRAIL and TRD significantly induced apoptotic cell death and decreased proliferation in rhabdomyosarcoma and epithelioid cell sarcoma cells. The combined use of TRAIL and TRD resulted in a synergistic apoptotic effect in all three cell lines, especially in rhabdomyosarcoma cells leaving 18% viable cells after 48 h of incubation (p<0.05). Analysis of the differentially regulated genes revealed that TRD and TRAIL influence apoptotic pathways, including the TNF-receptor associated and the mitochondrial pathway. Microarray analysis revealed remarkable expression changes in a variety of genes, which are involved in different apoptotic pathways and cross talk to other pathways at multiple levels. This in vitro study demonstrates that TRAIL and TRD synergise in inducing apoptosis and inhibiting proliferation in different human STS cell lines. Effects on gene expression differ relevantly in the sarcoma entities. These

  8. Kaposi's sarcoma herpesvirus and HIV-1 seroprevalences in prostitutes in Djibouti.

    Science.gov (United States)

    Marcelin, Anne-Geneviève; Grandadam, Marc; Flandre, Philippe; Nicand, Elisabeth; Milliancourt, Catherine; Koeck, Jean-Louis; Philippon, Michel; Teyssou, Remy; Agut, Henri; Dupin, Nicolas; Calvez, Vincent

    2002-10-01

    Kaposi's sarcoma herpesvirus (KSHV) is linked causally to Kaposi's sarcoma. Epidemiological studies have shown that KSHV transmission can occur during sex among homosexual men, but heterosexual transmission seems to be very rare in KSHV low prevalence countries. A seroepidemiological study was conducted to determine whether KSHV is transmitted sexually between heterosexuals in an endemic country. Sera from 282 subjects of African origin living in Djibouti were tested for antibodies to KSHV and HIV-1. Among the 282 individuals, 43 were female prostitutes working in the streets (group 1), 123 were female prostitutes working in luxury bars (group 2), 41 were non-prostitute females (group 3), and 75 were non-prostitute males (group 4). KSHV seroprevalence was 26, 20, 17, and 36% in groups 1, 2, 3, and 4, respectively. The seroprevalence of KSHV is not different between street or bar prostitutes and non-prostitute females (OR = 1.67; P = 0.34 and OR = 1.18; P = 0.73). These results suggest that in this endemic country commercial sex work does not seem to be a risk factor for KSHV infection and provides evidence against heterosexual transmission of KSHV in the female population studied.

  9. Computed tomography in the analysis of calcification patterns in pediatric bone tumors of the hip: a new approach; Tomografia computadorizada na analise dos padroes de calcificacoes nos tumores osseos da bacia em pediatria: nova abordagem

    Energy Technology Data Exchange (ETDEWEB)

    Oliveira, Gabriel Antonio de; Werlang, Henrique Zambenedetti; Bergoli, Pedro Martins [Hospital Universitario Cassiano Antonio de Morais/Hospital Infantil Nossa Senhora da Gloria, Vitoria, ES (Brazil). Centro de Diagnostico por Imagem]. E-mail: hzwerlang@gmail.com; Frechiani, Madalena; Oliveira, Fernao [Hospital Infantil Nossa Senhora da Gloria, Vitoria, ES (Brazil)

    2006-11-15

    Objective: In the pediatric group, the radiological diagnosis of bone tumors of the hip is difficult and presents some peculiarities, but reviewed literature does not approach this specific problem. The objective of the present study was to investigate the existence of reliable radiological patterns for the differential diagnosis of these tumors. Materials And Methods: Radiological findings of bone tumors of the hip in ten patients in the age range between 8 and 19 years have been reviewed. Results: Bone reaction (sclerosis or lysis), periosteal reaction (lamellar with single or multiple layers, or radial), tumor extent in the bone and level of soft tissues invasion have presented low specificity. Soft tissue calcifications, when considered as a whole, were non-specific. However, when those calcifications with varied shapes and sizes, nearby the affected bone (pattern I) were separated from those, thin and amorphous, away from the bone (pattern II), we have observed that the pattern I was totally non-specific, and the pattern II was found in the three cases of osteosarcoma (100%) and in only one case of Ewing's sarcoma (16.6%). Conclusion: In the present study, pattern II calcifications have shown a 100% sensitivity and 90% specificity for osteosarcoma. However, their importance may be not limited to the radiological diagnosis. Pattern II calcifications indicate probably ideal sites for biopsy. (author)

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

    Directory of Open Access Journals (Sweden)

    Lucas K. Vitzthum

    2016-06-01

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

  11. A rare case of translocation (12;22 (p13;Q in Ewing′s sarcoma

    Directory of Open Access Journals (Sweden)

    S K Kousar Jahan

    2014-01-01

    Full Text Available Cytogenetic or immunohistochemical studies are often required to differentiate Ewing′s sarcoma (ES from other small round cell tumors. Herein we report a case of 13-year-old boy who presented with a large presacral lesion. Hemogram and biochemical parameters were normal except lactate dehydrogenase showing value of 96.40/IU/L, magnetic resonance imaging of the spine showed a large mass in presacral lesion (8 cm × 7 cm × 9 cm, with destruction of the sacrum (S2 S3 and S4 with interspinal extension. Bone scan showed multiple pelvic bone lesions, radiograph of chest, ultrasound of abdomen, pelvis and electrocardiogram were within normal limits. Bone marrow was not involved. Cells from the fine needle aspirate were cultured for short term using RPMI medium and karyotype obtained showed a t(12;22(p12;q12 instead of the classic t(11;22. Diagnosis of ES was also confirmed by studies using immunohistochemistry for MIC2 which was positive, synaptophysin was inconclusive and leukocyte common antigen, desmin negative. This case provides evidence of the importance of chromosome 22, in the etiology of the disease.

  12. Bone development

    DEFF Research Database (Denmark)

    Tatara, M.R.; Tygesen, Malin Plumhoff; Sawa-Wojtanowicz, B.

    2007-01-01

    The objective of this study was to determine the long-term effect of alpha-ketoglutarate (AKG) administration during early neonatal life on skeletal development and function, with emphasis on bone exposed to regular stress and used to serve for systemic changes monitoring, the rib. Shropshire ram...... lambs were randomly assigned to two weight-matched groups at birth. During the first 14 days of life AKG was administered orally to the experimental group (n=12) at the dosage of 0.1 g/kg body weight per day, while the control group (n=11) received an equal dose of the vehicle. Lambs were slaughtered...... has a long-term effect on skeletal development when given early in neonatal life, and that changes in rib properties serve to improve chest mechanics and functioning in young animals. Moreover, neonatal administration of AKG may be considered as an effective factor enhancing proper development...

  13. Akv murine leukemia virus enhances bone tumorigenesis in hMT-c-fos-LTR transgenic mice

    DEFF Research Database (Denmark)

    Schmidt, Jörg; Krump-Konvalinkova, Vera; Luz, Arne

    1995-01-01

    hMt-c-fos-LTR transgenic mice (U. Rüther, D. Komitowski, F. R. Schubert, and E. F. Wagner. Oncogene 4, 861–865, 1989) developed bone sarcomas in 20% (3/15) of females at 448 ± 25 days and in 8% (1/12) of males at 523 days. After infection of newborns with Akv, an infectious retrovirus derived fro...

  14. Genomic signatures predict poor outcome in undifferentiated pleomorphic sarcomas and leiomyosarcomas

    DEFF Research Database (Denmark)

    Silveira, Sara Martoreli; Villacis, Rolando Andre Rios; Marchi, Fabio Albuquerque;

    2013-01-01

    Undifferentiated high-grade pleomorphic sarcomas (UPSs) display aggressive clinical behavior and frequently develop local recurrence and distant metastasis. Because these sarcomas often share similar morphological patterns with other tumors, particularly leiomyosarcomas (LMSs), classification...

  15. [Efficacy and problems of radio- and chemo-combination therapy for malignant bone tumors].

    Science.gov (United States)

    Yamawaki, S; Isu, K; Ubayama, Y; Goto, T; Goto, M; Kagami, Y; Ishii, S; Usui, M; Sasaki, T; Yagi, T

    1988-04-01

    In this study we reviewed cases of osteosarcoma, malignant lymphoma of bone and Ewing's sarcoma. Historically, osteosarcoma was unresponsive to chemotherapy. Most patients were treated by radiation or amputation alone and 80% of them died from pulmonary metastasis within 2 years. Five-year survival rate was 13%. Introduction of ADM, HD-MTX and CDDP improved dramatically the prognosis of these cases. Five-year survival rate was 60%. On the other hand, 11 cases of malignant lymphoma of bone and 4 cases of Ewing's sarcoma were treated by radiation with no local recurrence. VEPA or CHOP chemotherapy was used for the former with a five-year survival rate of 45%. For the latter, T-11 protocol (Rosen) was applied, and all patients survive with no metastasis. Other organ injuries circulatory disturbance, bone necrosis and growth-disturbance of bone in radiotherapy, myocardiopathy caused by ADM and renal toxicity of CDDP are all problematic.

  16. Feline injection-site sarcoma / Sarcoma de aplicação felino

    Directory of Open Access Journals (Sweden)

    Julia Maria Matera

    2008-08-01

    Full Text Available The feline injection-site sarcoma (FIS is a challenge for the veterinarian and the affected cat’s owner. The injectable applications (vaccines, medications seems to be the reason for that neoplasia, more specifically, the inflammation caused by injury of given drugs or antigens to the health tissue. Generally the FIS presents a more aggressive behavior when compared to sarcoma not associated to application. The most effective treatment has not been established yet, but it is believed that a multimodality of therapies, surgery, radiotherapy, and chemotherapy would be the most indicated option. The knowledge of the illness in all of its aspects will supply to professionals colleges subsidies in relation to the best way to approach its diagnosis and treatment.O sarcoma de aplicação felino (SAF é atualmente um grande desafio para o médico veterinário e também para o proprietário do felino acometido. Aplicações injetáveis por via subcutânea ou intramuscular, como vacinas e medicações, aparecem como iniciadoras do processo de neogênese dessa neoplasia, mais precisamente a inflamação persistente, causada pela lesão ao tecido sadio decorrente do fármaco ou antígeno administrado. Geralmente o SAF apresenta comportamento mais agressivo quando comparado ao sarcoma não associado à aplicação. O tratamento mais eficaz ainda não está estabelecido, mas acredita-se que a multimodalidade de terapias, cirurgia, radioterapia e quimioterapia seja a opção mais indicada. O conhecimento da afecção em todos os seus aspectos irá fornecer aos colegas profissionais subsídios em relação a melhor maneira de abordá-la em termos de diagnóstico, tratamento e prevenção.

  17. 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.)

  18. Kinetics of Kaposi’s Sarcoma-Associated Herpesvirus Gene Expression

    OpenAIRE

    Sun, Ren; Lin, Su-Fang; Staskus, Katherine; Gradoville, Lyndle; Grogan, Elizabeth; Haase, Ashley; Miller, George

    1999-01-01

    Herpesvirus gene expression can be classified into four distinct kinetic stages: latent, immediate early, early, and late. Here we characterize the kinetic class of a group of 16 Kaposi’s sarcoma-associated herpesvirus (KSHV)/human herpesvirus 8 genes in a cultured primary effusion cell line and examine the expression of a subset of these genes in KS biopsies. Expression of two latent genes, LANA and vFLIP, was constitutive and was not induced by chemicals that induce the lytic cycle in prima...

  19. Massive myeloid sarcoma affecting the central nervous system, mediastinum, retroperitoneum, liver, and rectum associated with acute myeloblastic leukaemia: a case report

    Science.gov (United States)

    Best-Aguilera, C R; Vazquez-Del Mercado, M; Muñoz-Valle, J F; Herrera-Zarate, L; Navarro-Hernandez, R E; Martin-Marquez, B T; Oregon-Romero, E; Ruiz-Quezada, S; Bonilla, G M; Lomeli-Guerrero, A

    2005-01-01

    Myeloid sarcomas are extramedullary tumours with granulocytic precursors. When associated with acute myelogenous leukaemia (AML), these tumours usually affect no more than two different extramedullary regions. This report describes a myeloid sarcoma associated with AML with tumour formation at five anatomical sites. The patient was a 37 year old man admitted in September 1999 with a two month history of weight loss, symptoms of anaemia, rectal bleeding, and left facial nerve palsy. The anatomical sites affected were: the rectum, the right lobe of the liver, the mediastinum, the retroperitoneum, and the central nervous system. A bone marrow smear was compatible with AML M2. Flow cytometry showed that the peripheral blood was positive for CD4, CD11, CD13, CD14, CD33, CD45, and HLA-DR. A karyotypic study of the bone marrow revealed an 8;21 translocation. The presence of multiple solid tumours in AML is a rare event. Enhanced expression of cell adhesion molecules may be the reason why some patients develop myeloid sarcomas. PMID:15735171

  20. Synovial Sarcoma of the Larynx: Report of a Case and Review of Literature

    Science.gov (United States)

    Baby, Anto; Somanathan, Thara; Konoth, Sreedevi

    2017-01-01

    Sarcomas account for less than 1% of malignant neoplasms arising in the head and neck in adults. Laryngeal synovial sarcoma is an extremely rare form of laryngeal malignancy with less than 20 cases reported in the literature. We report the case of a 48-year-old man with synovial sarcoma of the larynx. He underwent excision of the tumor followed by radiation. He is alive in remission at 36 months. The literature on synovial sarcoma of the larynx is reviewed. PMID:28280643

  1. Inhibitory effects of the ferment liquids on sarcoma

    Institute of Scientific and Technical Information of China (English)

    Ming Lu; Yunqing Cai; Yan Zhao; Juanhua Xu; Xiaoli Wu

    2005-01-01

    Objective: To study the anti-tumor effects of the ferment liquid (FL) and to explore the anti-mutagenic effects induced by cyclophosphamide (CP). Methods: Anti-tumor effects and Anti-mutagenic effects induced by cyclophosphamide (CP) of the ferment liquid were observed in tumor-bearing mice in vivo. Spleen lymphocytes proliferation of mice was stimulated by ConA and NK cell activity was analyzed by the lactate dehydrogenase (LDH) release assay. Results: FL Ⅰ ,FL Ⅱ and FL Ⅲ of 16.7, 33.3 and 66.6ml/kg inhibited growth of Sarcoma 180 sighificanth by 11.6%, 24.2%, 28.0%, 4.8%, 15.2%, 2.2% and 2.1%, 8.5%, 5.2%respectively. Polychromatic erythrocytes (PCE) at all doses of FL Ⅰ and FL Ⅱ were greatly decreased compared with control group ( P <0.05), but FL Ⅲ has no inhibition effents( P > 0.05). Production of IL-2 showed no significant difference between all treatment groups.The NK cell killing activity in all treated mice increased by FL Ⅰ and FL Ⅱ was significantly potentiated compared to controls ( P <0.05). Lymphocytes proliferation of ICR mice were stimulated by ConA 2.5 ml/L and there were no differences between groups.Conclusion: The present data suggested that three kinds of ferment liquid used as health drink have anti-mutagenesis, immune enhancement effects and showed certain inhibitory effects on S180 tumor growth in vivo.

  2. Brain Metastasis in Bone and Soft Tissue Cancers: A Review of Incidence, Interventions, and Outcomes

    Directory of Open Access Journals (Sweden)

    Faris Shweikeh

    2014-01-01

    Full Text Available Bone and soft tissue malignancies account for a small portion of brain metastases. In this review, we characterize their incidence, treatments, and prognosis. Most of the data in the literature is based on case reports and small case series. Less than 5% of brain metastases are from bone and soft tissue sarcomas, occurring most commonly in Ewing’s sarcoma, malignant fibrous tumors, and osteosarcoma. Mean interval from initial cancer diagnosis to brain metastasis is in the range of 20–30 months, with most being detected before 24 months (osteosarcoma, Ewing sarcoma, chordoma, angiosarcoma, and rhabdomyosarcoma, some at 24–36 months (malignant fibrous tumors, malignant peripheral nerve sheath tumors, and alveolar soft part sarcoma, and a few after 36 months (chondrosarcoma and liposarcoma. Overall mean survival ranges between 7 and 16 months, with the majority surviving < 12 months (Ewing’s sarcoma, liposarcoma, malignant fibrous tumors, malignant peripheral nerve sheath tumors, angiosarcoma and chordomas. Management is heterogeneous involving surgery, radiosurgery, radiotherapy, and chemotherapy. While a survival advantage may exist for those given aggressive treatment involving surgical resection, such patients tended to have a favorable preoperative performance status and minimal systemic disease.

  3. Intronic deletions of tva receptor gene decrease the susceptibility to infection by subgroup A avian sarcoma and leukosis virus subgroup A

    Science.gov (United States)

    The group of avian sarcoma and leukosis virus (ASLV) in chickens contains six highly related subgroups, A to E and J. Four genetic loci, tva, tvb, tvc and tvj, encode for corresponding receptors that determine the susceptibility to the ASLV subgroups. The prevalence of ASLV in hosts may have imposed...

  4. 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

  5. Primary synovial sarcoma of the abdominal wall: A case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Alsaif H Saif

    2008-01-01

    Full Text Available Synovial sarcoma is a malignant mesenchymal neoplasm which commonly occurs in the extremities of adults, in close association with joint capsules, tendon sheaths, bursae and fascial structures. Only a few cases of synovial sarcoma occurring in the abdominal wall have been reported. A case of a primary synovial sarcoma arising from the anterior abdominal wall fascial aponeurosis is presented.

  6. Epithelioid sarcoma with muscle metastasis detected by positron emission tomography

    Directory of Open Access Journals (Sweden)

    Oya Masafumi

    2008-08-01

    Full Text Available Abstract Background Epithelioid sarcoma is an uncommon high-grade sarcoma, mostly involving the extremities. Case presentation A 33-year-old man was referred to our institute with a diagnosis of Volkmann's contracture with the symptom of flexion contracture of the fingers associated with swelling in his left forearm. Magnetic resonance imaging (MRI showed abnormal signal intensity, comprising iso-signal intensity on T1- and high-signal intensity on T2-weighted images surrounding the flexor tendons in the forearm. Diagnosis of epithelioid sarcoma was made by open biopsy, and amputation at the upper arm was then undertaken. [18F]-2-fluoro-2-deoxy-D-glucose-positron emission tomography (FDG-PET detected multiple lesions with an increased uptake in the right neck, the bilateral upper arms and the right thigh, as well as in the left axillary lymph nodes, with maximum standardized uptake value (SUVmax ranging from 2.0 to 5.5 g/ml. Magnetic resonance imaging confirmed that there was a lesion within the right thigh muscle which was suggestive of metastasis, even though the lesion was occult clinically. Conclusion Increased uptake on FDG-PET might be representative of epithelioid sarcoma, and for this reason FDG-PET may be useful for detecting metastasis. Muscle metastasis is not well documented in epithelioid sarcoma. Accordingly, the frequency of muscle metastasis, including occult metastasis, needs to be further analyzed.

  7. 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.

  8. 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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2011-03-15

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

  10. Evaluation of Clinical Results and Complications of Structural Allograft Reconstruction after Bone Tumor Surgery

    Directory of Open Access Journals (Sweden)

    Mohammad Gharedaghi

    2016-07-01

    Full Text Available Background: Massive bone allograft is an option in cases of limb preservation and reconstruction after massive benign and malignant bone tumor resection. The purpose of this study was to analyze the outcome of these procedures at Imam Reza Hospital, Mashhad University of Medical Sciences. Methods: In this study, 113 cases have been presented. Eleven cases were excluded (patients has a traumatic defect or they passed away before the completion of the study’s two-year follow up period. Each patient completed a questionnaire, went through a physical examination and, if indicated, X-ray information was collected. The patients were divided into three groups: chemotherapy, chemotherapy plus radiation therapy, and no-adjuvant-therapy. Results: Fifty-four cases were male and the mean age was 24.5±5.39. The number of cases and indications for surgery were: 33 cases of aggressive benign tumors or low grade malignant bone tumors (large bone defects including 16 germ cell tumors, eight aneurysmal bone cysts, five low grade osteosarcomas, and four chondrosarcomas. Another 69 cases were high-grade malignant bone tumors including 42 osteosarcomas, 21 Ewing’s sarcoma, and six other high grade osteosarcomas. Patients were divided into three groups: the first group received no adjuvant therapy, the second group received chemotherapy, and the third group received chemotherapy plus radiotherapy. The location of tumors were as follows: eight cases in the pelvic bone, 12 in the proximal femur, 18 in the femoral shaft, 36 in the distal femur, 12 in the proximal tibia, and 16 in the humeral bone. The 12 cases of proximal femoral defects were reconstructed by allograft composite prosthesis, 18 diaphyseal defects with intercalary allograft, and 36 distal femoral defects were reconstructed using osteoarticular allograft. The rate of deep infection was 7:8% (eight patients and in this regard, we found a significant difference among the three groups, such that most

  11. Spinal intradural extraosseous Ewing’s sarcoma

    Directory of Open Access Journals (Sweden)

    Daniel Lachance

    2011-03-01

    Full Text Available Extraosseous Ewing’s sarcoma (EES involving the central nervous system is rare, but can be diagnosed and distinguished from other primitive neuroectodermal tumors (PNET by identification of the chromosomal translocation (11;22(q24;q12. We report EES arising from the spinal intradural extramedullary space, based on imaging, histopathological, and molecular data in two men, ages 50 and 60 years old and a review of the literature using PubMed (1970-2009. Reverse transcriptase polymerase chain reaction (RT-PCR identified the fusion product FL1-EWS. Multimodal therapy, including radiation and alternating chemotherapy including vincristine, cyclophosphamide, doxorubicin and ifosfamide and etoposide led to local tumor control and an initial, favorable therapeutic response. No systemic involvement was seen from the time of diagnosis to the time of last follow-up (26 months or death (4 years. This report confirms that EES is not confined to the earliest decades of life, and like its rare occurrence as an extra-axial meningeal based mass intracranially, can occasionally present as an intradural mass in the spinal canal without evidence of systemic tumor. Gross total resection followed by multimodal therapy may provide for extended progression free and overall survival.

  12. Myeloid Sarcoma: The Clinician's Point of View

    Directory of Open Access Journals (Sweden)

    M. Malagola

    2011-01-01

    Full Text Available Myeloid Sarcoma may occur in patients with an acute or chronic myeloproliferative disorder as well as de novo, with no apparent sign or symptom of concomitant haematological disease. The patients are preferentially young male and the site of disease localization may vary from central nervous system to pleura and thorax, with a common involvement of the reticuloendothelial system. The disease often shows chromosomal rearrangements, involving chromosomes 7, 8 and 3 and sometimes a complex karyotype (more than 3 abnormalities is detected at diagnosis. The prognosis of this disease is dismal and only high-dose chemotherapy with autologous or allogeneic stem cells transplantation (auto or allo-SCT may be potentially curative. In the absence of definitive elements that can define the prognosis of extra-medullary localization of “standard risk” AML, Clinicians should pursue the collection of data from different Centres and design of homogeneous treatment strategies, that could integrate standard chemotherapy with specific approaches, such as radiotherapy, transplant procedures or, in selected cases (such as those displaying molecular abnormalities involving protein tyrosine-kinases, molecularly targeted therapies.

  13. Sarcoma de Kaposi en paciente con SIDA

    Directory of Open Access Journals (Sweden)

    Jesús Ramón León Polanco

    2015-01-01

    Full Text Available Se presenta el caso de un paciente masculino de 33 años de edad, con antecedentes de VIH-SIDA desde hace 10 años, que se mantiene en tratamiento con antirretrovirales. Durante todo este tiempo ha presentado varios episodios de infecciones respiratorias, incluyendo tuberculosis pulmonar 5 años atrás. Acude a consulta refiriendo edemas en miembros inferiores acompañado de lesiones en piel de color violáceo de un año de evolución, previamente interpretado como linfangitis rebelde al tratamiento y que se extendió a la cara interna de los muslos y a los miembros inferiores. Con pérdida de peso, no prurito en las lesiones, fiebre, lesiones en la mucosa oral. Se determinó hemoglobina 89 g/L, leucocitos 4,5 x 109 /L, se estudiaron las funciones hepática y renales resultando normales. Radiografías de tórax y ultrasonido abdominal normales. Se realizó estudio anatomopatológico de piel que informó Sarcoma de Kaposi. Se impuso tratamiento con quimioterapia

  14. [Retroperitoneal sarcoma: report of 6 cases].

    Science.gov (United States)

    Joual, A; Faik, H; Rabii, R; Hafiani, M; Bennani, S; el Mrini, M; Benjelloun, S

    2000-06-01

    Retroperitoneal soft tissue sarcomas (RPS) are uncommon tumors. The diagnosis is frequently made later in the evolution of the disease due to the absence of specific symptomatology. Surgery with total resection of the tumor is the treatment of choice, but is only possible in 38 to 75% of cases. Six cases of RPS have been retrospectively reviewed; the mean time to diagnosis was 5 months; diagnosis was established via CT scan, which determined the retroperitoneal tumor location and its relationship to the neighboring organs. The most common symptoms were the presence of an abdominal mass and accompanying abdominal pain; signs of urinary and vascular compression were not found until later. Surgery with total resection was performed in 4 cases, and with partial resection in 2 cases. The histological findings were as follows: 3 liposarcomas, 2 rhabdomyosarcomas, and 1 fibrosarcoma. Tumor recurrence developed in 2 cases (liposarcomas), necessitating further surgery and complete resection. Two patients were lost to follow-up. RPS are characterized by locoregional relapse and metastases. Disease outcome depends on the histological type, tumor grade, and on the possibility of carrying out complete resection.

  15. Biochemical markers can predict the response in bone mass during alendronate treatment in early postmenopausal women. Alendronate Osteoporosis Prevention Study Group

    DEFF Research Database (Denmark)

    Ravn, Pernille; Clemmesen, B; Christiansen, C

    1999-01-01

    Data from the Danish cohort (n = 67) of a multicenter trial of oral alendronate in the prevention of postmenopausal osteoporosis were used to evaluate the capacity of the biochemical markers to predict changes in bone mineral density (BMD). A panel of markers were measured: serum N-terminal midfr......Data from the Danish cohort (n = 67) of a multicenter trial of oral alendronate in the prevention of postmenopausal osteoporosis were used to evaluate the capacity of the biochemical markers to predict changes in bone mineral density (BMD). A panel of markers were measured: serum N...

  16. Study of vitamin D status of rheumatoid arthritis patients Rationale and design of a cross-sectional study by the osteoporosis and metabolic bone diseases study group of the Italian Society of Rheumatology (SIR

    Directory of Open Access Journals (Sweden)

    M. Antonelli

    2011-09-01

    Full Text Available The fundamental role of Vitamin D has been long known in regulating calcium homeostasis and bone metabolism. An increased contribution of Vitamin D was recently described in association with a lower incidence of Rheumatoid Arthritis (RA. This must not be surprising, as the immunomodulating effects of Vitamin D are clear, which have been attributed protective effects in autoimmune disorders such as some chronic inflammatory bowel diseases, multiple sclerosis and type I diabetes. An interaction was suggested between Vitamin D metabolism and inflammation indexes through mediation of TNF-a which is also especially involved in osteoclastic resorption and therefore in bone loss processes. Some preliminary data would indicate an association between seasonal changes of Vitamin D serum levels, latitude and disease activity (DAS28 in RA patients. Consequently, the Osteoporosis and Metabolic Bone Diseases Study Group of SIR believes that there are grounded reasons for assessing the Vitamin D status of RA patients in order to investigate whether this is to be related to physiopathological and clinical aspects of disease other than those of bone involvement. Primary end point of the study will be to assess the levels of 25 OH Vitamin D in RA patients. Secondary endpoints will include correlation with disease activity, densitometry values and bone turnover. The cross-sectional study will enrol patients of both sex genders, age ranging between 30 and 75 years according to the 1988 ACR criteria, onset of symptoms at least 2 years prior to study enrollment. Patients will be excluded suffering from osteometabolic diseases, liver and kidney insufficiency and those administered Vitamin D boli in the previous 12 months. Disease activity will be evaluated with the HAQ. Haematochemical tests and femoral and lumbar bone densitometry will be performed, unless recently undergone by patients. Blood levels of 25 OH C Vitamin D and PHT and of the two bone remodeling markers

  17. Extracellular high mobility group box 1 plays a role in the effect of bone marrow mononuclear cell transplantation for heart failure.

    Directory of Open Access Journals (Sweden)

    Masahiro Kaneko

    Full Text Available Transplantation of unfractionated bone marrow mononuclear cells (BMCs repairs and/or regenerates the damaged myocardium allegedly due to secretion from surviving BMCs (paracrine effect. However, donor cell survival after transplantation is known to be markedly poor. This discrepancy led us to hypothesize that dead donor BMCs might also contribute to the therapeutic benefits from BMC transplantation. High mobility group box 1 (HMGB1 is a nuclear protein that stabilizes nucleosomes, and also acts as a multi-functional cytokine when released from damaged cells. We thus studied the role of extracellular HMGB1 in the effect of BMC transplantation for heart failure. Four weeks after coronary artery ligation in female rats, syngeneic male BMCs (or PBS only as control were intramyocardially injected with/without anti-HMGB1 antibody or control IgG. One hour after injection, ELISA showed that circulating extracellular HMGB1 levels were elevated after BMC transplantation compared to the PBS injection. Quantitative donor cell survival assessed by PCR for male-specific sry gene at days 3 and 28 was similarly poor. Echocardiography and catheterization showed enhanced cardiac function after BMC transplantation compared to PBS injection at day 28, while this effect was abolished by antibody-neutralization of HMGB1. BMC transplantation reduced post-infarction fibrosis, improved neovascularization, and increased proliferation, while all these effects in repairing the failing myocardium were eliminated by HMGB1-inhibition. Furthermore, BMC transplantation drove the macrophage polarization towards alternatively-activated, anti-inflammatory M2 macrophages in the heart at day 3, while this was abolished by HMGB1-inhibition. Quantitative RT-PCR showed that BMC transplantation upregulated expression of an anti-inflammatory cytokine IL-10 in the heart at day 3 compared to PBS injection. In contrast, neutralizing HMGB1 by antibody-treatment suppressed this anti

  18. Bone Densitometry (Bone Density Scan)

    Science.gov (United States)

    ... of DXA Bone Densitometry? What is a Bone Density Scan (DXA)? Bone density scanning, also called dual-energy x-ray absorptiometry ( ... is today's established standard for measuring bone mineral density (BMD). An x-ray (radiograph) is a noninvasive ...

  19. Correlation of non-mass-like abnormal MR signal intensity with pathological findings surrounding pediatric osteosarcoma and Ewing's sarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Masrouha, Karim Z.; Haidar, Rachid; Saghieh, Said [American University of Beirut Medical Center, Department of Surgery, Beirut (Lebanon); Musallam, Khaled M. [American University of Beirut Medical Center, Internal Medicine Division of Hematology and Oncology, Beirut (Lebanon); Samra, Alexis Bou; Tawil, Ayman; Chakhachiro, Zaher [American University of Beirut Medical Center, Pathology, Beirut (Lebanon); Abdallah, Abeer; Khoury, Nabil J. [American University of Beirut Medical Center, Diagnostic Radiology, Beirut (Lebanon); Saab, Raya; Muwakkit, Samar; Abboud, Miguel R. [American University of Beirut Medical Center, Children' s Cancer Center of Lebanon, Beirut (Lebanon)

    2012-11-15

    The aim of this work was to determine the role of MRI in interpreting abnormal signals within bones and soft tissues adjacent to tumor bulk of osteosarcoma and Ewing's sarcoma in a pediatric population by correlating MR findings with histopathology. Thirty patients met the inclusion criteria, which included (1) osteosarcoma or Ewing's sarcoma, (2) MR studies no more than 2 months prior to surgery, (3) presence of abnormal MR signal surrounding the tumor bulk, (4) pathological material from resected tumor. The patients received standard neoadjuvant chemotherapy. Using grid maps on gross pathology specimens, the abnormal MR areas around the tumor were matched with the corresponding grid sections. Histopathology slides of these sections were then analyzed to determine the nature of the regions of interest. The MR/pathological correlation was evaluated using Mann-Whitney U test and Fisher's exact test. Twenty-seven patients had osteosarcoma and three patients had Ewing's sarcoma. Of the studied areas, 17.4% were positive for tumor (viable or necrotic). There was no statistically significant correlation between areas positive for tumor and age, gender, signal extent and intensity on MRI, or tissue type. There was, however, a statistically significant correlation between presence of tumor and the appearance of abnormal soft tissue signals. A feathery appearance correlated with tumor-negative areas whereas a bulky appearance correlated with tumor-positive regions. MR imaging is helpful in identifying the nature of abnormal signal areas surrounding bone sarcomas that are more likely to be tumor-free, particularly when the signal in the soft tissues surrounding the tumor is feathery and edema-like in appearance. (orig.)

  20. 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

  1. Primary pulmonary synovial sarcoma: a rare case report

    Directory of Open Access Journals (Sweden)

    Sumalatha Kasturi

    2014-08-01

    Full Text Available Primary lung sarcoma is an extremely rare tumor, accounting for less than 0.5% of all lung tumors. Histological subtypes are differentiated on the basis of immunohistochemical markers, such as vimentin, desmin, actin, CD99, and epithelial membrane antigen. A 50-year-old male presented with progressively increasing shortness of breath with cough for 2 months. On Contrast Enhanced Computed Tomography (CECT of thorax a large heterogeneous mass with multiple areas of necrosis, occupying almost whole of left hemithorax was seen. CT-guided Fine Needle Aspiration Cytology (FNAC revealed spindle cell neoplasm. Histopathological examination revealed a spindle cell sarcoma. On immunohistochemistry the tumor cells expressed both epithelial membrane antigen and vimentin. Hence, final impression from immunohistochemistry was primary monophasic synovial sarcoma of lung. [Int J Res Med Sci 2014; 2(4.000: 1729-1731

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

    DEFF Research Database (Denmark)

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

    1996-01-01

    We investigated the relationship between immunohistochemical estimates of proliferative activity and expression of bcl-2 protein and mutant p53 protein in 23 cases of soft tissue sarcoma. Furthermore, the reproducibility of estimates of proliferative activity was analysed and correlations between...... the variables and with mitotic score were investigated. Proliferative activity was assessed by use of monoclonal antibody MIB-1 and staining for iododeoxyuridine (IdUrd), and evaluated in multiple, random, systematically sampled fields of vision. MIB-1 indices were higher than those of IdUrd but for each case...... improve the accuracy of conventional malignancy grading of soft tissue sarcomas. Furthermore, the results indicate that neither mutant p53 protein nor bcl-2 oncogene alone are sufficient to induce increased proliferation in these sarcomas....

  3. 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.

  4. 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.

  5. Transarticular spread of Ewing sarcoma mimicking septic arthritis

    Energy Technology Data Exchange (ETDEWEB)

    Jordanov, Martin I.; Block, John J. [Vanderbilt University Medical Center, Department of Radiology and Radiological Sciences, Nashville, TN (United States); Gonzalez, Adriana L. [Vanderbilt University Medical Center, Department of Pathology, Nashville, TN (United States); Green, Neil E. [Vanderbilt Children' s Hospital, Department of Pediatric Orthopaedics, Nashville, TN (United States)

    2009-04-15

    Transarticular spread of tumor is rare; it has only been reported in the sacroiliac joint, intervertebral disk spaces, and facet joints. The anatomic and kinetic characteristics of the sacroiliac joint, as well as the changes the joint undergoes during a lifetime, make it particularly vulnerable to transarticular tumor invasion. Although extremely rare, Ewing sarcoma can extend through the sacroiliac joint and be virtually indistinguishable radiologically from septic arthritis. Furthermore, the clinical presentation of a child with Ewing sarcoma can be similar to that of a child with osteomyelitis. Laboratory values are quite nonspecific and are not always helpful in differentiating between the entities. Therefore, the possibility of sacroiliac joint transarticular Ewing sarcoma should be considered in a child presenting with hip pain, despite clinical, radiological and laboratory findings suggesting an infectious process. (orig.)

  6. Bone marrow aspiration

    Science.gov (United States)

    Iliac crest tap; Sternal tap; Leukemia - bone marrow aspiration; Aplastic anemia - bone marrow aspiration; Myelodysplastic syndrome - bone marrow aspiration; Thrombocytopenia - bone marrow aspiration; Myelofibrosis - bone marrow aspiration

  7. Development of a food group-based diet score and its assocation with bone mineral density in the elderly: the Rotterdam Study

    NARCIS (Netherlands)

    Jonge, de E.A.L.; Kiefte-de Jong, J.C.; Groot, de C.P.G.M.; Voortman, T.; Schoufour, J.D.; Zillikens, M.C.; Hofman, A.; Uitterlinden, A.G.; Franco, O.H.; Rivadeneira, F.

    2015-01-01

    No diet score exists that summarizes the features of a diet that is optimal for bone mineral density (BMD) in the elderly. Our aims were (a) to develop a BMD-Diet Score reflecting a diet that may be beneficial for BMD based on the existing literature, and (b) to examine the association of the BMD-Di

  8. Development of a food group-based diet score and its association with bone mineral density in the elderly: The Rotterdam study

    NARCIS (Netherlands)

    E.A.L. de Jonge (Ester); J.C. Kiefte-de Jong (Jessica); L.C.P.G.M. de Groot (Lisette); R.G. Voortman (Trudy); J.D. Schoufour (Josje); M.C. Zillikens (Carola); A. Hofman (Albert); A.G. Uitterlinden (André); O.H. Franco (Oscar); F. Rivadeneira Ramirez (Fernando)

    2015-01-01

    textabstractNo diet score exists that summarizes the features of a diet that is optimal for bone mineral density (BMD) in the elderly. Our aims were (a) to develop a BMD-Diet Score reflecting a diet that may be beneficial for BMD based on the existing literature, and (b) to examine the association o

  9. Value of routine bone marrow examination in pediatric acute myeloid leukemia (AML) : A study of the Dutch Childhood Oncology Group (DCOG)

    NARCIS (Netherlands)

    Hageman, Ilse M. G.; Peek, Annemarie M. L.; de Haas, Valerie; Damen-Korbijn, Carin M.; Kaspers, Gertjan J. L.

    2012-01-01

    Background The outcome of the treatment of pediatric acute myeloid leukemia (AML) is still disappointing, due to relatively high treatment-related mortality and relapse rates (3040%). Past treatment protocols have called for routine screening via bone marrow aspiration (BMA) after achievement of fir

  10. Body size accounts for most differences in bone density between Asian and Caucasian women. The EPIC (Early Postmenopausal Interventional Cohort) Study Group

    DEFF Research Database (Denmark)

    Ross, P D; He, Y; Yates, A J;

    1996-01-01

    We compared bone mineral density (BMD) of the whole body (and subregions: arm, leg, and pelvis), hip, spine, lateral spine, wrist, and forearm among Caucasian and Asian women at four geographic centers (Honolulu, HI; Nottingham, UK; Portland, OR; Copenhagen, Denmark). Data were derived from...

  11. 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

  12. Primary renal undifferentiated sarcoma as an infiltrative mass in a 12 year old boy

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Yong Hee; Kim, Myung Joon; Lee, Mi Jung [Dept. of Radiology and Research Institute of Radiological Science, Severance Children' s Hospital, Yonsei University College of Medicine, Seoul (Korea, Republic of); Kim, Se Hwa [Dept. of Pathology, Severance Hospital, Yonsei University College of Medicine, Seoul (Korea, Republic of)

    2015-09-15

    Undifferentiated sarcomas are rare tumors not classified into any sarcoma subtype. Due to their rarity, imaging findings of undifferentiated sarcomas are poorly characterized. The purpose of this report was to present imaging findings of a pathologically confirmed undifferentiated sarcoma originated from the left kidney of a 12-year-old boy. The mass was infiltrative involving the renal pelvis. It mimicked massive hilar lymphadenopathy with a preserved renal contour visible by both ultrasonography and CT. Renal vein thrombosis was also observed. Although undifferentiated sarcomas are rare, they should be considered in differential diagnosis of infiltrative renal masses with renal pelvis invasion in children.

  13. Follicular Dendritic Cell Sarcoma of the Abdomen: the Imaging Findings

    Energy Technology Data Exchange (ETDEWEB)

    Kang, Tae Wook; Lee, Soon Jin; Song, Hye Jong [Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of)

    2010-04-15

    Follicular dendritic cell sarcoma is a rare neoplasm that originates from follicular dendritic cells in lymphoid follicles. This disease usually involves the lymph nodes, and especially the head and neck area. Rarely, extranodal sites may be affected, including tonsil, the oral cavity, liver, spleen and the gastrointestinal tract. We report here on the imaging findings of follicular dendritic cell sarcoma of the abdomen that involved the retroperitoneal lymph nodes and colon. It shows as a well-defined, enhancing homogenous mass with internal necrosis and regional lymphadenopathy.

  14. 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.

  15. 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

  16. Combinatorial Drug Screening Identifies Ewing Sarcoma-specific Sensitivities

    DEFF Research Database (Denmark)

    Radic-Sarikas, Branka; Tsafou, Kalliopi P; Emdal, Kristina B.;

    2017-01-01

    including approved drugs. We were able to retrieve highly synergistic drug combinations specific for Ewing sarcoma and identified signaling processes important for Ewing sarcoma cell proliferation determined by EWS-FLI1 We generated a molecular target profile of PKC412, a multikinase inhibitor with strong...... and IGF1R inhibitors. The mechanism of the drug synergy between these inhibitors is different from the sum of the mechanisms of the single agents. The combination effectively inhibited pathway crosstalk and averted feedback loop repression, in EWS-FLI1-dependent manner. Mol Cancer Ther; 16(1); 88...

  17. 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.

  18. Primary Pulmonary Synovial Sarcoma: A Very Rare Presentation

    Directory of Open Access Journals (Sweden)

    Ekrem Cengiz Seyhan

    2014-01-01

    Full Text Available Synovial sarcoma (SS is a rare tumor originating from mesenchymal tissue and accounting for approximately 5–10% of all soft tissue sarcomas. A rare case of primary pulmonary SS in an asymptomatic 18-year-old man admitted to our hospital for investigation of a 6 × 6.5 cm, oval-shaped, well-delineated pleural based peripheral mass in the left lower lobe in his thorax CT is presented. Left lower lobectomy was done. Immunohistochemically, tumor cells were positive for cytokeratin, epithelial membrane antigen (EMA, and vimentin so that the histopathological diagnosis was compatible with biphasic spindle cell type SS in the lung.

  19. Imaging diagnosis--spinal cord histiocytic sarcoma in a dog.

    Science.gov (United States)

    Taylor, Amanda; Eichelberger, Bunita; Hodo, Carolyn; Cooper, Jocelyn; Porter, Brian

    2015-01-01

    A 12-year-old mixed breed dog was presented for evaluation of progressive paraparesis and ataxia. Magnetic resonance (MR) imaging was performed and identified multifocal intradural spinal cord mass lesions. The lesions were hyperintense in T2-weighted sequences, isointense to mildly hyperintense in T1-weighted sequences with strong contrast enhancement of the intradural lesions and spinal cord meninges. Spinal cord neoplasia was suspected. A diagnosis of intramedullary spinal cord histiocytic sarcoma, confined to the central nervous system, was confirmed histopathologically. Spinal cord histiocytic sarcoma is a rare neoplasm, but should be included in the differential diagnosis for dogs with clinical signs of myelopathy.

  20. 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

  1. Patología Molecular de los sarcomas

    OpenAIRE

    Álava, Enrique

    2005-01-01

    Los sarcomas de hueso y tejidos blandos son un grupo poco frecuente de tumores. Su prevalencia es inferior a 1 caso por 10000 habitantes, lo que los convierte en una enfermedad rara. Algunos de estos tumores, como el sarcoma sinovial, el tumor de Ewing o el osteosarcoma, son más habituales en los adolescentes o en los adultos jóvenes, mientras que existen neoplasias como el leiomiosarcoma o el liposarcoma, más frecuentes en pacientes de edad superior a los 55 años. Desde el punto de vista his...

  2. Pathogenesis and Associated Diseases of Kaposi's Sarcoma-associated Herpesvirus

    Institute of Scientific and Technical Information of China (English)

    Lin-ding WANG

    2007-01-01

    Kaposi's sarcoma-associated herpesvirus (KSHV) is the primary etiological agent of Kaposi's sarcoma, primary effusion lymphoma and muticentric Castleman's disease. In common with the other herpesviruses, KSHV exhibits both latent and lytic life cycles, both of which are characterized by distinct gene expression profiles and programs. KSHV encodes proteins which play essential roles in the inhibition of host adaptive and innate immunity, the inhibition of apoptosis, and the regulation of the cell cycle. KSHV also encodes several proteins which have transforming and intrcellular signalling activity.

  3. The genomic landscape of the Ewing Sarcoma family of tumors reveals recurrent STAG2 mutation.

    Directory of Open Access Journals (Sweden)

    Andrew S Brohl

    2014-07-01

    Full Text Available The Ewing sarcoma family of tumors (EFT is a group of highly malignant small round blue cell tumors occurring in children and young adults. We report here the largest genomic survey to date of 101 EFT (65 tumors and 36 cell lines. Using a combination of whole genome sequencing and targeted sequencing approaches, we discover that EFT has a very low mutational burden (0.15 mutations/Mb but frequent deleterious mutations in the cohesin complex subunit STAG2 (21.5% tumors, 44.4% cell lines, homozygous deletion of CDKN2A (13.8% and 50% and mutations of TP53 (6.2% and 71.9%. We additionally note an increased prevalence of the BRCA2 K3326X polymorphism in EFT patient samples (7.3% compared to population data (OR 7.1, p = 0.006. Using whole transcriptome sequencing, we find that 11% of tumors pathologically diagnosed as EFT lack a typical EWSR1 fusion oncogene and that these tumors do not have a characteristic Ewing sarcoma gene expression signature. We identify samples harboring novel fusion genes including FUS-NCATc2 and CIC-FOXO4 that may represent distinct small round blue cell tumor variants. In an independent EFT tissue microarray cohort, we show that STAG2 loss as detected by immunohistochemistry may be associated with more advanced disease (p = 0.15 and a modest decrease in overall survival (p = 0.10. These results significantly advance our understanding of the genomic and molecular underpinnings of Ewing sarcoma and provide a foundation towards further efforts to improve diagnosis, prognosis, and precision therapeutics testing.

  4. 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.

  5. 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.

  6. Sarcoma sinovial primário da orofaringe: relato de caso Primary synovial sarcoma of the oropharinx: a case report

    OpenAIRE

    Hugo Meister; Thaís C. Miranda; Manuel G. G. Nóbrega

    2003-01-01

    O sarcoma sinovial primário da cabeça e pescoço é um tumor raro. Em torno de 90 casos foram descritos na literatura. Relatamos um caso adicional ocorrido em um paciente jovem do sexo masculino comprometendo a orofaringe. O tumor apresentou típico crescimento bifásico, fibrossarcomatoso e epitelial, com estruturas pseudo-glandulares. O diagnóstico final foi obtido pelo exame da peça cirúrgica com preparações histológicas de rotina e análise imunohistoquímica.Primary synovial sarcoma of the hea...

  7. Kaposi sarcoma as initial presentation of HIV infection

    Directory of Open Access Journals (Sweden)

    Bhushan Malhari Warpe

    2014-01-01

    Full Text Available Context: Kaposi′s sarcoma (KS, a vascular tumor that manifests as nodular lesions on the skin and to a lesser extent, the visceral organs, is the most common neoplasm encountered in human immunodeficiency virus (HIV-infected patients. It consists of an angiosarcomatous change of not only the epithelial and mucous membrane-associated connective tissue in various sites, for example, skin, gastrointestinal system, lungs, and so on, but may also involve non-epithelial organs, such as lymph nodes. Surgical excision is the line of management for the tumor. Case Report: We present one case of a 65-year-old heterosexual Indian male, clinically unsuspected for acquired immunodeficiency syndrome (AIDS who presented with multiple non-blanching, bluish-red nodules on all extremities, chest, back and bilateral submandibular and cervical lymphadenopathy. Fine needle aspiration cytology (FNAC was performed from subcutaneous nodule and lymph node. Smears showed hypercellular plump spindle cell groups in a hemorrhagic background. Diagnosis was given as low-grade spindle cell neoplasm consistent with KS, which was later confirmed on histopathology. Conclusion: The first line diagnostic aid of FNAC has several advantages over the traditional biopsy in testing such vascular tumors. The latter is generally needed for confirmation of KS. However, FNAC of such vascular tumors has advantages of better patient compliance, ease of procedure, no recurrences, and safety in immuno-compromised patients. Ancillary studies can be done on aspirates along with polymerase chain reaction (PCR amplification techniques in confirming the detection of associated human herpes virus-8 (HHV-8 infection with KS.

  8. Histological Subgroups in Classic Kaposi Sarcoma: A Preliminary Study

    Directory of Open Access Journals (Sweden)

    Nilüfer Onak Kandemir

    2010-06-01

    Full Text Available Background and Design: Kaposi sarcomas (KS are vascular tumors with a low malignant potential which include overlapping infectious, immunologic, and neoplastic processes. Recently, many histological subtypes have been defined. Material and Method: In the present study, 151 cutaneous classic KS lesions in 56 patients were retrospectively evaluated with regard to histological subtypes. Determination of the subtypes was based on the predominant histopathological component in the lesion. We examined changes in epidermis and dermis along with intratumoral inflammatory response characteristics in the lesions. By defining histopathological variants of the cases, differences regarding subtypes were investigated. Results: Cases that bear the ordinary characteristics of KS and those that can not be classified otherwise, comprised 82..8% of the study group. Twenty-six cases showed consistency with the subtypes outlined in the literature in terms of their histopathological properties. The most common histological subtype was the lymphangiectatic variant in 7.3% of the cases. Bullous (2.6%, lymphangioma like (2.6%, intravascular (2%, and pyogenic granuloma like (2% variants were less common. The most uncommon histological subtype was micronodular (0.6% type. Lymphangiectatic, bullous, intravascular, and pyogenic granuloma like variants were frequently observed in the nodular stage of KSs. Lympangioma like changes were seen to be present in the early KS lesions. Lymphangiectatic type was oftenly associated with bullous component, whereas pyogenic granuloma like type demonstrated superficial ulceration and intense inflammatory response. Lymphangioma like and intravascular types exhibited a characteristic appearance, while other variants were accompanied by components belonging to different subtypes. Conclusion: In KS, histopathological subtypes can develop as a result of different pathological processes. The next stage of the current study, which is one of the

  9. Mucosal Kaposi sarcoma, a Rare Cancer Network study

    Directory of Open Access Journals (Sweden)

    Robert C. Miller

    2012-10-01

    Full Text Available Kaposi’s sarcoma (KS most often affect the skin but occasionally affect the mucosa of different anatomic sites. The management of mucosal KS is seldom described in the literature. Data from 15 eligible patients with mucosal KS treated between 1994 and 2008 in five institutions within three countries of the Rare Cancer Network group were collected. The inclusion criteria were as follows: age >16 years, confirmed pathological diagnosis, mucosal stages I and II, and a minimum of 6 months’ follow-up after treatment. Head and neck sites were the most common (66%. Eleven cases were HIV-positive. CD4 counts correlated with disease stage. Twelve patients had biopsy only while three patients underwent local resection. Radiotherapy (RT was delivered whatever their CD4 status was. Median total radiation dose was 16.2 Gy (0-45 delivered in median 17 days (0-40 with four patients receiving no RT. Six patients underwent chemotherapy and received from 1 to 11 cycles of various regimens namely vinblastin, caelyx, bleomycine, or interferon, whatever their CD4 counts was. Five-year disease free survival were 81.6% and 75.0% in patients undergoing RT or not, respectively. Median survival was 66.9 months. Radiation-induced toxicity was at worse grade 1-2 and was manageable whatever patients’ HIV status. This small series of mucosal KSs revealed that relatively low-dose RT is overall safe and efficient in HIV-positive and negative patients. Since there are distant relapses either in multicentric cutaneous or visceral forms in head and neck cases, the role of systemic treatments may be worth investigations in addition to RT of localized disease. Surgery may be used for symptomatic lesions, with caution given the risk of bleeding.

  10. RNA helicase DDX3: a novel therapeutic target in Ewing sarcoma.

    Science.gov (United States)

    Wilky, B A; Kim, C; McCarty, G; Montgomery, E A; Kammers, K; DeVine, L R; Cole, R N; Raman, V; Loeb, D M

    2016-05-19

    RNA helicase DDX3 has oncogenic activity in breast and lung cancers and is required for translation of complex mRNA transcripts, including those encoding key cell-cycle regulatory proteins. We sought to determine the expression and function of DDX3 in sarcoma cells, and to investigate the antitumor activity of a novel small molecule DDX3 inhibitor, RK-33. Utilizing various sarcoma cell lines, xenografts and human tissue microarrays, we measured DDX3 expression at the mRNA and protein levels, and evaluated cytotoxicity of RK-33 in sarcoma cell lines. To study the role of DDX3 in Ewing sarcoma, we generated stable DDX3-knockdown Ewing sarcoma cell lines using DDX3-specific small hairpin RNA (shRNA), and assessed oncogenic activity. DDX3-knockdown and RK-33-treated Ewing sarcoma cells were compared with wild-type cells using an isobaric mass-tag quantitative proteomics approach to identify target proteins impacted by DDX3 inhibition. Overall, we found high expression of DDX3 in numerous human sarcoma subtypes compared with non-malignant mesenchymal cells, and knockdown of DDX3 by RNA interference inhibited oncogenic activity in Ewing sarcoma cells. Treatment with RK-33 was preferentially cytotoxic to sarcoma cells, including chemotherapy-resistant Ewing sarcoma stem cells, while sparing non-malignant cells. Sensitivity to RK-33 correlated with DDX3 protein expression. Growth of human Ewing sarcoma xenografts expressing high DDX3 was inhibited by RK-33 treatment in mice, without overt toxicity. DDX3 inhibition altered the Ewing sarcoma cellular proteome, especially proteins involved in DNA replication, mRNA translation and proteasome function. These data support further investigation of the role of DDX3 in sarcomas, advancement of RK-33 to Ewing sarcoma clinical trials and development of RNA helicase inhibition as a novel anti-neoplastic strategy.

  11. ESF-EMBO Symposium Molecular Biology and Innovative Therapies in Sarcomas of Childhood and AdolescenceSept 29 – Oct 4, Polonia Castle Pultusk, Poland

    Directory of Open Access Journals (Sweden)

    Beat W Schäfer

    2013-06-01

    Full Text Available Rhabdomyosarcoma (RMS and Ewing sarcoma (ES are among the most common pediatric sarcomas (Arndt et al., 2012. Despite sarcomas representing a highly heterogeneous group of tumors, ES and alveolar RMS (ARMS typically share one common genetic characteristic, namely a specific chromosomal translocation (Helman and Meltzer, 2003; Lessnick and Ladanyi, 2012. These translocations generate fusion proteins, which are composed of two transcription factors (TF. Typically, one TF is a developmentally regulated factor that is essential for proper specification of a given lineage and provides the DNA-binding domain, while the partner TF contributes a transactivation domain that drives aberrant expression of target genes. Based on these common genetic characteristics, the first ESF-EMBO research conference entitled "Molecular Biology and Innovative Therapies in Sarcomas of Childhood and Adolescence" with special focus on RMS and ES was held at the Polonia Castle in Pultusk, Poland. The conference gathered 70 participants from more than 15 countries and several continents representing most research groups that are active in this field.

  12. ESF-EMBO Symposium “Molecular Biology and Innovative Therapies in Sarcomas of Childhood and Adolescence” Sept 29–Oct 4, Polonia Castle Pultusk, Poland

    Science.gov (United States)

    Schäfer, Beat W.; Koscielniak, Ewa; Kovar, Heinrich; Fulda, Simone

    2013-01-01

    Rhabdomyosarcoma (RMS) and Ewing sarcoma (ES) are among the most common pediatric sarcomas (Arndt et al., 2012). Despite sarcomas representing a highly heterogeneous group of tumors, ES and alveolar RMS (ARMS) typically share one common genetic characteristic, namely a specific chromosomal translocation (Helman and Meltzer, 2003; Lessnick and Ladanyi, 2012). These translocations generate fusion proteins, which are composed of two transcription factors (TF). Typically, one TF is a developmentally regulated factor that is essential for proper specification of a given lineage and provides the DNA-binding domain, while the partner TF contributes a transactivation domain that drives aberrant expression of target genes. Based on these common genetic characteristics, the first ESF-EMBO research conference entitled “Molecular Biology and Innovative Therapies in Sarcomas of Childhood and Adolescence” with special focus on RMS and ES was held at the Polonia Castle in Pultusk, Poland. The conference gathered 70 participants from more than 15 countries and several continents representing most research groups that are active in this field. PMID:23761860

  13. ESF-EMBO Symposium "Molecular Biology and Innovative Therapies in Sarcomas of Childhood and Adolescence" Sept 29-Oct 4, Polonia Castle Pultusk, Poland.

    Science.gov (United States)

    Schäfer, Beat W; Koscielniak, Ewa; Kovar, Heinrich; Fulda, Simone

    2013-01-01

    Rhabdomyosarcoma (RMS) and Ewing sarcoma (ES) are among the most common pediatric sarcomas (Arndt et al., 2012). Despite sarcomas representing a highly heterogeneous group of tumors, ES and alveolar RMS (ARMS) typically share one common genetic characteristic, namely a specific chromosomal translocation (Helman and Meltzer, 2003; Lessnick and Ladanyi, 2012). These translocations generate fusion proteins, which are composed of two transcription factors (TF). Typically, one TF is a developmentally regulated factor that is essential for proper specification of a given lineage and provides the DNA-binding domain, while the partner TF contributes a transactivation domain that drives aberrant expression of target genes. Based on these common genetic characteristics, the first ESF-EMBO research conference entitled "Molecular Biology and Innovative Therapies in Sarcomas of Childhood and Adolescence" with special focus on RMS and ES was held at the Polonia Castle in Pultusk, Poland. The conference gathered 70 participants from more than 15 countries and several continents representing most research groups that are active in this field.

  14. Epigenetic Landscape of Kaposi's Sarcoma-Associated Herpesvirus Genome in Classic Kaposi's Sarcoma Tissues

    Science.gov (United States)

    Wang, Xiaodong; Yang, Lei; Robertson, Erle S.; Lan, Ke

    2017-01-01

    Kaposi's sarcoma-associated herpesvirus (KSHV) is etiologically related to Kaposi's sarcoma (KS), primary effusion lymphoma (PEL) and multicentric Castleman's disease (MCD). It typically displays two different phases in its life cycle, the default latency and occasional lytic replication. The epigenetic modifications are thought to determine the fate of KSHV infection. Previous studies elegantly depicted epigenetic landscape of latent viral genome in in vitro cell culture systems. However, the physiologically relevant scenario in clinical KS tissue samples is unclear. In the present study, we established a protocol of ChIP-Seq for clinical KS tissue samples and mapped out the epigenetic landscape of KSHV genome in classic KS tissues. We examined AcH3 and H3K27me3 histone modifications on KSHV genome, as well as the genome-wide binding sites of latency associated nuclear antigen (LANA). Our results demonstrated that the enriched AcH3 was mainly restricted at latent locus while H3K27me3 was widespread on KSHV genome in classic KS tissues. The epigenetic landscape at the region of vIRF3 gene confirmed its silenced state in KS tissues. Meanwhile, the abundant enrichment of LANA at the terminal repeat (TR) region was also validated in the classic KS tissues, however, different LANA binding sites were observed on the host genome. Furthermore, we verified the histone modifications by ChIP-qPCR and found the dominant repressive H3K27me3 at the promoter region of replication and transcription activator (RTA) in classic KS tissues. Intriguingly, we found that the TR region in classic KS tissues was lacking in AcH3 histone modifications. These data now established the epigenetic landscape of KSHV genome in classic KS tissues, which provides new insights for understanding KSHV epigenetics and pathogenesis. PMID:28118409

  15. Kaposi's sarcoma-associated herpesvirus infection and Kaposi's sarcoma in Brazil

    Directory of Open Access Journals (Sweden)

    S. Ramos-da-Silva

    2006-05-01

    Full Text Available Kaposi's sarcoma (KS became a critical health issue with the emergence of acquired immunodeficiency syndrome (AIDS in the 1980s. Four clinical-epidemiological forms of KS have been described: classical KS, endemic KS, iatrogenic KS, and AIDS-associated KS. In 1994, Kaposi's sarcoma-associated herpesvirus (KSHV or human herpesvirus type 8 was identified by Chang and colleagues, and has been detected worldwide at frequencies ranging from 80 to 100%. The aim of the present study was to evaluate the frequency of KSHV infection in KS lesions from HIV-positive and HIV-negative patients in Brazil, as well as to review the current knowledge about KS transmission and detection. For these purposes, DNA from 51 cases of KS was assessed by PCR: 20 (39.2% cases of classical KS, 29 (56.9% of AIDS-associated KS and 2 (3.9% of iatrogenic KS. Most patients were males (7.5:1, M/F, and mean age was 47.9 years (SD = ± 18.7 years. As expected, HIV-positive KS patients were younger than patients with classical KS. On the other hand, patients with AIDS-associated KS have early lesions (patch and plaque compared to classical KS patients (predominantly nodular lesions. This is assumed to be the result of the early diagnose of KS in the HIV-positive setting. KSHV infection was detected by PCR in almost all cases (48/51; 94.1%, irrespectively of the clinical-epidemiological form of KS. These results show that KSHV is associated with all forms of KS in Brazilian patients, a fact that supports the role of this virus in KS pathogenesis.

  16. The prognosis factor of adjuvant radiation therapy after surgery in uterine sarcomas

    Directory of Open Access Journals (Sweden)

    Hou HL

    2015-08-01

    Full Text Available Hai-Ling Hou, Mao-Bin Meng, Xiu-Li Chen, Lu-Jun Zhao, Li Zhu, Bai-Lin Zhang, Ping Wang Department of Radiation Oncology, CyberKnife Center, Key Laboratory of Cancer Prevention and Therapy, Tianjin Medical University Cancer Institute and Hospital, National Clinical Research Center for Cancer, Tianjin, People’s Republic of China Objective: This retrospective study evaluated the role of adjuvant radiotherapy (AR after surgery in patients with uterine sarcoma and analyzed the prognostic factors of local-regional failure-free survival (LRFFS and overall survival (OS.Patients and methods: A study of a total of 182 patients with uterine sarcoma was conducted between June 1994 and October 2014. Adjuvant radiotherapy was defined as postoperative external beam radiation to the pelvis (30–50 Gray/10–25 fractions at five fractions/week. The primary end point was LRFFS, and the secondary end point was OS. Kaplan–Meier curves were compared using the log-rank test. Cox regression analyses were used to determine prognosticators for LRFFS and OS.Results: The median follow-up time of all patients was 75 months, with a 5-year LRFFS of 62.1%. The 2-year and 5-year LRFFS rates were longer for those who received AR than for those who did not receive AR (83.4% vs 70.3%; 78% vs 55.3%; P=0.013. The 5-year OS of all patients was 56.2%, and no significant differences were observed in the 2-year and 5-year OS rates between these two groups (82.7% vs 71.4%; 64.1% vs 51.7%; P=0.067. Importantly, in patients with leiomyosarcoma, the 2-year and 5-year LRFFS and OS rates were longer for those who received AR than for those who did not receive AR (P=0.04 and P=0.02 for the 2-year and 5-year LRFFS, respectively.Conclusion: Patients with uterine sarcoma who were treated with AR after surgery demonstrated an improved LRFFS compared with those who were treated with surgery alone, especially those patients with leiomyosarcoma. Therefore, the role of personalized adjuvant

  17. Microsatellites with Macro-Influence in Ewing Sarcoma

    Directory of Open Access Journals (Sweden)

    Stephen L. Lessnick

    2012-07-01

    Full Text Available Numerous molecular abnormalities contribute to the genetic derangements involved in tumorigenesis. Chromosomal translocations are a frequent source of these derangements, producing unique fusion proteins with novel oncogenic properties. EWS/ETS fusions in Ewing sarcoma are a prime example of this, resulting in potent chimeric oncoproteins with novel biological properties and a unique transcriptional signature essential for oncogenesis. Recent evidence demonstrates that EWS/FLI, the most common EWS/ETS fusion in Ewing sarcoma, upregulates gene expression using a GGAA microsatellite response element dispersed throughout the human genome. These GGAA microsatellites function as enhancer elements, are sites of epigenetic regulation and are necessary for EWS/FLI DNA binding and upregulation of principal oncogenic targets. An increasing number of GGAA motifs appear to substantially enhance EWS/FLI-mediated gene expression, which has compelling biological implications as these GGAA microsatellites are highly polymorphic within and between ethnically distinct populations. Historically regarded as junk DNA, this emerging evidence clearly demonstrates that microsatellite DNA plays an instrumental role in EWS/FLI-mediated transcriptional regulation and oncogenesis in Ewing sarcoma. This unprecedented role of GGAA microsatellite DNA in Ewing sarcoma provides a unique opportunity to expand our mechanistic understanding of how EWS/ETS fusions influence cancer susceptibility, prognosis and transcriptional regulation.

  18. Synovial sarcoma. An immunohistochemical study of the epithelial component

    DEFF Research Database (Denmark)

    Jørgensen, L J; Lyon, H; Myhre-Jensen, O;

    1994-01-01

    Twenty-five synovial sarcomas were studied with a battery of antibodies directed against keratin and epithelial membrane antigen (EMA). The keratin antibody MNF 116 showed reactivity in 24 tumors. In addition, 22 tumors showed reactivity with the antibody Keratin Wide Spectrum, 20 with the antibody...

  19. Presentation of Ewing’s sarcoma as unilateral sacroiliitis

    Directory of Open Access Journals (Sweden)

    Balebail Gopalakrishna Dharmanand

    2013-05-01

    Full Text Available Presence of sacroiliitis (SI is one of the hallmarks for diagnosing seronegative spondyloarthropathy, especially ankylosing spondylitis. In certain cases, the occurrence of erosions around sacroiliac joint due to other causes can mislead the diagnosis. We are discussing here a rare case of Ewing’s sarcoma, which clinically presented as SI.

  20. Cancer incidence after retinoblastoma - Radiation dose and sarcoma risk

    NARCIS (Netherlands)

    Wong, FL; Boice, JD; Abramson, DH; Tarone, RE; Kleinerman, RA; Stovall, M; Goldman, MB; Seddon, JM; Tarbell, N; Fraumeni, JF; Li, FP

    1997-01-01

    Context.-There is a substantial risk of a second cancer for persons with hereditary retinoblastoma, which is enhanced by radiotherapy. Objective.-To examine long-term risk of new primary cancers in survivors of childhood retinoblastoma and quantify the role of radiotherapy in sarcoma development. De