small cell sarcoma

Summary

Summary: A sarcoma characterized by the presence of small cells, cells measuring 9-14 micrometers with a faint or indistinct rim of cytoplasm and an oval-to-elongated nucleus with relatively dense chromatin. (From Segen, Dictionary of Modern Medicine, 1992)

Top Publications

  1. Veronesi G, Spaggiari L, De Pas T, Solli P, de Braud F, Catalano G, et al. Preoperative chemotherapy is essential for conservative surgery of Askin tumors. J Thorac Cardiovasc Surg. 2003;125:428-9 pubmed
  2. Michal M, Kazakov D, Sima R, Vanecek T. Primitive small cell tumor with epithelial, gangliocytic, neuroendocrine, and mesenchymal differentiation: report of 2 cases. Int J Surg Pathol. 2007;15:429-36 pubmed
    ..Granules were found in the cytoplasm. We suggest the name "primitive small cell tumor with epithelial, gangliocytic, neuroendocrine, and mesenchymal differentiation" for this neoplasm...
  3. Sawin R, Conrad E, Park J, Waldhausen J. Preresection chemotherapy improves survival for children with Askin tumors. Arch Surg. 1996;131:877-80 pubmed
    ..To test whether patients with Askin tumor treated with aggressive neoadjuvant chemotherapy have a better clinical outcome...
  4. Rekhi B, Basak R, Desai S, Jambhekar N. A t (11; 22) (p13; q12) EWS-WT 1 positive desmoplastic small round cell tumor of the maxilla: an unusual case indicating the role of molecular diagnosis in round cell sarcomas. J Postgrad Med. 2010;56:201-5 pubmed publisher
    ..An exact diagnosis of a round cell sarcoma has a therapeutic relevance...
  5. Rahman R, Pollono D, Drut R, Spizzirri F. Malignancy-associated membranoproliferative glomerulonephritis. Pediatr Nephrol. 1997;11:208-9 pubmed
    ..Renal biopsy revealed type I membranoproliferative glomerulonephritis (MPGN). The association of a malignant tumor and MPGN is extremely unusual in children, and the pathogenesis of the renal lesion under these circumstances is unknown...
  6. Parkins C, Holder A, Dennis M, Stratford M, Chaplin D. Involvement of oxygen free radicals in ischaemia-reperfusion injury to murine tumours: role of nitric oxide. Free Radic Res. 1998;28:271-81 pubmed
    ..Hydroxyl radical generation following I/R injury correlated with vascular damage and response of tumours in vivo, but not skin, which indicates a potential therapeutic benefit from this approach...
  7. Mertens F, Wiebe T, Adlercreutz C, Mandahl N, French C. Successful treatment of a child with t(15;19)-positive tumor. Pediatr Blood Cancer. 2007;49:1015-7 pubmed
    ..More importantly, our results also demonstrate that at least some patients with t(15;19)-positive tumors may be successfully treated...
  8. Zhang P, Harris K, Alobeid B, Brooks J. Immunoexpression of villin in neuroendocrine tumors and its diagnostic implications. Arch Pathol Lab Med. 1999;123:812-6 pubmed
    ..Villin immunoexpression was recently observed in a small number of carcinoids of the intestinal tract and lung, but its significance in a broad category of neuroendocrine tumors has not been evaluated...
  9. Yin W, Guo S, Yang H, Chan J. Desmoplastic small round cell tumor of the submandibular gland--a rare but distinctive primary salivary gland neoplasm. Hum Pathol. 2010;41:438-42 pubmed publisher
    ..Although rare, desmoplastic small round cell tumor has to be considered in the differential diagnosis of poorly differentiated neoplasms of the salivary gland, especially in young patients...

More Information

Publications62

  1. Lawlor E, Lim J, Tao W, Poremba C, Chow C, Kalousek I, et al. The Ewing tumor family of peripheral primitive neuroectodermal tumors expresses human gastrin-releasing peptide. Cancer Res. 1998;58:2469-76 pubmed
    ..Its expression in pPNETs provides further evidence for a neuroectodermal histogenesis of these tumors and suggests that autocrine growth of this family of tumors may be at least partially regulated by GRP...
  2. Motoyama T, Maejima T, Aizawa K, Fukuda T, Watanabe H. Biphasic intra-abdominal desmoplastic small cell tumor in a patient with proximal spinal muscular atrophy. Pathol Int. 1996;46:54-9 pubmed
    ..Although IDSCT are known to express multi-phenotypes immunohistochemically, attention should be paid to the broad spectrum of cell morphology in these tumors...
  3. Jamema S, Sharma P, Laskar S, Deshpande D, Shrivastava S. Treatment planning comparison of electron arc therapy and photon intensity modulated radiotherapy for Askin's tumor of chest wall. Radiother Oncol. 2007;84:257-65 pubmed
    ..A dosimetric study to quantitatively compare radiotherapy treatment plans for Askin's tumor using Electron Arc (EA) vs. photon Intensity Modulated Radiotherapy (IMRT)...
  4. Baz W, El Soueidi R, Nakhl F, Aoun N, Chin N, Dhar M. Desmoplastic small round-cell tumor: an adult with previous exposure to agent orange. Jpn J Clin Oncol. 2010;40:593-5 pubmed publisher
    ..He responded poorly to chemotherapy with high-dose cyclophosphamide, doxorubicin and vincristine and died 5 months after presentation secondary to neutropenic sepsis despite G-CSF support and antibiotics...
  5. Peydró Olaya A, Llombart Bosch A, Carda Batalla C, Lopez Guerrero J. Electron microscopy and other ancillary techniques in the diagnosis of small round cell tumors. Semin Diagn Pathol. 2003;20:25-45 pubmed
    ..Thus, short references on molecular genetics, complementing the structural findings, are given...
  6. Yang J, Kim J. Small cell extraskeletal osteosarcoma. Orthopedics. 2009;32:217 pubmed
    ..We performed chest computed tomography, magnetic resonance imaging, and positron emission tomography-computed tomography. There were no regional recurrence and distant metastasis. Presently the patient is disease free...
  7. Takanami I, Imamura T, Naruke M, Kodaira S. Long-term survival after repeated resections of Askin tumor recurrences. Eur J Cardiothorac Surg. 1998;13:313-5 pubmed
    ..It thus may be that postoperative chemo- and radiotherapy after repeated excisions of these local Askin tumor recurrences plays a role in prolonging survival...
  8. Pagani A, Fischer Colbrie R, Sanfilippo B, Winkler H, Cerrato M, Bussolati G. Secretogranin II expression in Ewing's sarcomas and primitive neuroectodermal tumors. Diagn Mol Pathol. 1992;1:165-72 pubmed
    ..This result supports the hypothesis that some Ewing's sarcomas represent a most primitive form of neuroectodermal tumor; in addition, it indicates a diagnostic role of SgII in cases of Ewing's sarcomas and PNETs...
  9. Werner H, Idelman G, Rubinstein M, Pattee P, Nagalla S, Roberts C. A novel EWS-WT1 gene fusion product in desmoplastic small round cell tumor is a potent transactivator of the insulin-like growth factor-I receptor (IGF-IR) gene. Cancer Lett. 2007;247:84-90 pubmed
    ..These findings raise the possibility that the oncogenic potential of EWS-WT1 fusions is not necessarily a consequence of the fusion protein product per se...
  10. Kushner B, Laquaglia M, Gerald W, Kramer K, Modak S, Cheung N. Solitary relapse of desmoplastic small round cell tumor detected by positron emission tomography/computed tomography. J Clin Oncol. 2008;26:4995-6 pubmed publisher
  11. Carmona Bernal C, Jiménez Mejías M, Borderas F, Iglesias L, Fernández Reyes J, Garcia Escudero A, et al. [Infrequent clinical presentation of Askin's tumor]. Rev Clin Esp. 1994;194:341-4 pubmed
    ..Given the patient's lack of response to antibiotics, a thoracotomy was performed. The anatomopathological diagnosis was Atkin's tumor. After beginning treatment, the patient died after ten days...
  12. Argatoff L, O Connell J, Mathers J, Gilks C, Sorensen P. Detection of the EWS/WT1 gene fusion by reverse transcriptase-polymerase chain reaction in the diagnosis of intra-abdominal desmoplastic small round cell tumor. Am J Surg Pathol. 1996;20:406-12 pubmed
    ..This report demonstrates the utility of molecular studies as an adjunct in the diagnosis of this rare and aggressive tumor...
  13. Kamishima T, Fukuda T, Kakihara T, Ohnishi Y, Naito M, Ueki H, et al. Small round and spindle cell sarcoma with neuronal differentiation and oncocyte-like features of the thoracic wall: a case report with histological, immunohistochemical and ultrastructural examinations. Acta Pathol Jpn. 1993;43:615-23 pubmed
    ..On the basis of these findings, the tumor of the present case corresponds to malignant peripheral nerve sheath tumor with neuronal differentiation and oncocytic features...
  14. Suryanto A, Herlambang K, Rachmatullah P. Comparison of tumor density by CT scan based on histologic type in lung cancer patients. Acta Med Indones. 2005;37:195-8 pubmed
    ..To investigate the differences of tumor density of lung cancer by CT scan based on histopathologic type of cancer cell...
  15. Walton J, Bass J, Sambey E, Rubin S. Use of human dura in pediatric chest wall reconstruction after tumor resection. J Pediatr Surg. 1994;29:1189-91 pubmed
    ..For moderate-size chest wall defects, it appears to be an excellent alternative to synthetic prosthesis...
  16. Leenman E, Kochurova N, Belogurova M, Pozharisskii K. [Immunohistochemical study of small cell sarcomas and factors(nm23, CD44) predicting metastatic spreading in children]. Arkh Patol. 2003;65:21-7 pubmed
    ..Increase in CD44 expression was more frequently observed in neuroblastomas with favorable prognostic signs and embryonic rhabdosarcomas that are not accompanied by metastases. This needs further study...
  17. Gautam U, Srinivasan R, Rajwanshi A, Bansal D, Marwaha R, Vasishtha R. Reverse transcriptase-polymerase chain reaction as an ancillary molecular technique in the diagnosis of small blue round cell tumors by fine-needle aspiration cytology. Am J Clin Pathol. 2010;133:633-45 pubmed publisher
    ..Overall, RT-PCR resolved 38 (86%) of 44 vs 35 (69%) of 51 cases by immunocytochemical analysis. RT-PCR is easily applied to fine-needle aspirates of SBRCT and greatly facilitates accurate tumor typing...
  18. Fukunaga M, Endo Y, Takaki K, Ishikawa E, Ushigome S. Postmenopausal intra-abdominal desmoplastic small cell tumor. Pathol Int. 1996;46:281-5 pubmed
    ..DSCT should be included in differential diagnoses of post-menopausal pelvic tumors which show light-microscopically and immunohistochemically divergent phenotypes...
  19. Budarin M. [Prognostic factors in childhood sarcomas of neuroectodermal histogenesis]. Vopr Onkol. 2002;48:335-9 pubmed
  20. Qureshi S, Ramadwar M, Viswanathan S, Bakshi A, Arora B, Gupta T, et al. Desmoplastic small round cell tumor of Meckels diverticulum. J Clin Oncol. 2007;25:3372-4 pubmed
  21. Ota S, Ushijima K, Fujiyoshi N, Fujimoto T, Hayashi R, Murakami F, et al. Desmoplastic small round cell tumor in the ovary: Report of two cases and literature review. J Obstet Gynaecol Res. 2010;36:430-4 pubmed publisher
    ..After primary therapy, the tumors recurred and both women died of the disease. The clinical presentation and differential diagnosis, as well as the treatment, including surgical debulking and combined chemotherapy are discussed...
  22. Lee Y, Hsiao C. Desmoplastic small round cell tumor: a clinicopathologic, immunohistochemical and molecular study of four patients. J Formos Med Assoc. 2007;106:854-60 pubmed
    ..Only a few studies using RT-PCR with formalin-fixed and paraffin-embedded (FFPE) tissue sections to detect the specific transcript have been reported...
  23. Yoshida A, Edgar M, Garcia J, Meyers P, Morris C, Panicek D. Primary desmoplastic small round cell tumor of the femur. Skeletal Radiol. 2008;37:857-62 pubmed publisher
    ..This is the second reported case of primary DSRCT of bone with genetic confirmation...
  24. Bismar T, Basturk O, Gerald W, Schwarz K, Adsay N. Desmoplastic small cell tumor in the pancreas. Am J Surg Pathol. 2004;28:808-12 pubmed
    ..This case is also another demonstrative example of how DSCT may form a dominant mass in intraabdominal organs...
  25. Tanas M, Goldblum J. Fluorescence in situ hybridization in the diagnosis of soft tissue neoplasms: a review. Adv Anat Pathol. 2009;16:383-91 pubmed publisher
    ..Finally, we discuss the advantages and disadvantages of FISH as a technique when appraising soft tissue neoplasms...
  26. Irwin G, Maclennan A, Carachi R. Interventional radiology cases from the royal hospital for sick children, glasgow: percutaneous metal biliary stent placement for palliation of intra-abdominal desmoplastic small round-cell tumour in a child. Eur J Pediatr Surg. 2004;14:130-2 pubmed
    ..We describe a case where the technique has been used successfully to treat obstructive jaundice in a ten-year-old boy suffering from intra-abdominal desmoplastic small round-cell tumour...
  27. Lal D, Su W, Wolden S, Loh K, Modak S, La Quaglia M. Results of multimodal treatment for desmoplastic small round cell tumors. J Pediatr Surg. 2005;40:251-5 pubmed
    ..We examined the effects of multimodal therapy including induction chemotherapy, aggressive surgical debulking, and external beam radiotherapy on patients with DSRCT...
  28. Kumar P. Fine needle aspiration cytologic findings in malignant small cell tumor of the thoracopulmonary region (Askin tumor). Acta Cytol. 1994;38:702-6 pubmed
    ..They were positively stained by periodic acid-Schiff stain. The histologic sections confirmed the cytologic diagnosis of Askin tumor. Four of the patients died within 7-11 months after treatment began...
  29. Odaka M, Yamazaki S, Akiba T, Mizuno R, Harada T, Yamazaki Y. [A resected case of malignant peripheral nerve sheath tumor (MPNST) necessary to distinguish from Askin tumor]. Jpn J Thorac Cardiovasc Surg. 1998;46:1215-20 pubmed
    ..The diagnosis should be based on the overall findings including the location and clinical data...
  30. D Amore E, Ninfo V. Soft tissue small round cell tumors: morphological parameters. Semin Diagn Pathol. 1996;13:184-203 pubmed
    ..We discuss the morphological clues that in our opinion are most useful for their diagnosis, the criteria for distinguishing between peripheral neuroepithelioma and Ewing's sarcoma, and the main diagnostic pitfalls...
  31. Wang L, Bhargava R, Zheng T, Wexler L, Collins M, Roulston D, et al. Undifferentiated small round cell sarcomas with rare EWS gene fusions: identification of a novel EWS-SP3 fusion and of additional cases with the EWS-ETV1 and EWS-FEV fusions. J Mol Diagn. 2007;9:498-509 pubmed
    ..As these data further support, these types of EWS fusions are associated with primitive extraskeletal small round cell sarcomas of uncertain lineage arising mainly in the pediatric population...
  32. Letson G, Muro Cacho C. Genetic and molecular abnormalities in tumors of the bone and soft tissues. Cancer Control. 2001;8:239-51 pubmed
    ..This article provides a comprehensive review of the cytogenetic abnormalities observed in bone and soft-tissue tumors, emphasizing known downstream molecular changes that may play a role in oncogenesis...
  33. ElHajj M, Mazurka J, Daya D. Desmoplastic small round cell tumor presenting in the ovaries: report of a case and review of the literature. Int J Gynecol Cancer. 2002;12:760-3 pubmed
    ..The clinical presentation and differential diagnoses as well as the treatment including aggressive surgical debulking and multiagent chemotherapy are discussed...
  34. Palmer H, Mukunyadzi P, Culbreth W, Thomas J. Subgrouping and grading of soft-tissue sarcomas by fine-needle aspiration cytology: a histopathologic correlation study. Diagn Cytopathol. 2001;24:307-16 pubmed
    ..Therefore, attention should be given to specimen adequacy, and caution should be exercised when attempting to grade myxoid and spindle-cell sarcomas by FNAB...
  35. Yeste L, Sierra A, Cañón R, Aristu J, Torre W. Successful use of intraoperative radiotherapy for local control of an Askin's tumor recurrence. J Cardiovasc Surg (Torino). 2001;42:143-5 pubmed
    ..After 2-year follow-up, the patient is alive and free of disease. The role of surgery is still the key to obtaining good survival, but in this case intraoperative radiotherapy proved to be a good adjuvant treatment...
  36. de Alava E, Lozano M, Sola I, Panizo A, Idoate M, Martínez Isla C, et al. Molecular features in a biphenotypic small cell sarcoma with neuroectodermal and muscle differentiation. Hum Pathol. 1998;29:181-4 pubmed
    ..Cytological specimens are a good source of RNA for molecular studies...
  37. Kushner B, LaQuaglia M, Cheung N, Kramer K, Hamelin A, Gerald W, et al. Clinically critical impact of molecular genetic studies in pediatric solid tumors. Med Pediatr Oncol. 1999;33:530-5 pubmed
    ..We present cases depicting the importance of including molecular diagnostic studies in the routine evaluation of pediatric solid tumors...
  38. Pisharodi L, Bedrossian C. Diagnosis and differential diagnosis of small-cell lesions of the liver. Diagn Cytopathol. 1998;19:29-32 pubmed
    ..Relevant clinical history and immunocytochemistry were helpful in making the final diagnosis in some cases. The diagnosis and differential diagnosis of small cell lesions of the liver are discussed in detail in this report...
  39. Abe S, Imamura T, Park P, Nakano H, Okita H, Hata J, et al. Small round-cell type of malignant peripheral nerve sheath tumor. Mod Pathol. 1998;11:747-53 pubmed
    ..The tumors were not consistent with peripheral primitive neuroectodermal tumor. We propose them as a small round-cell type of MPNST that might differentiate toward the immature neural cells...
  40. Insabato L, Di Vizio D, Lambertini M, Bucci L, Pettinato G. Fine needle aspiration cytology of desmoplastic small round cell tumor. A case report. Acta Cytol. 1999;43:641-6 pubmed
    ..Detailed information on the clinical and fine needle aspiration (FNA) biopsy and the immunocytochemical and ultrastructural findings in a patient with DSRCT is presented...
  41. Devaney K, Vinh T, Sweet D. Small cell osteosarcoma of bone: an immunohistochemical study with differential diagnostic considerations. Hum Pathol. 1993;24:1211-25 pubmed
  42. Pellin A, Boix J, Blesa J, Noguera R, Carda C, Llombart Bosch A. EWS/FLI-1 rearrangement in small round cell sarcomas of bone and soft tissue detected by reverse transcriptase polymerase chain reaction amplification. Eur J Cancer. 1994;30A:827-31 pubmed
    ..These results reinforce the value of molecular biology techniques for the correct assessment of histology difficult evaluable neoplasms, such as the group of small round cell tumours within the Es family...
  43. Benjamin L, Fredericks W, Barr F, Rauscher F. Fusion of the EWS1 and WT1 genes as a result of the t(11;22)(p13;q12) translocation in desmoplastic small round cell tumors. Med Pediatr Oncol. 1996;27:434-9 pubmed
    ..Thus, the WT1 locus may be the target for both gain- and loss-of-function mutations resulting in different disease outcomes. A summary of our ongoing analysis of the EWS-WT1 fusion gene is presented...
  44. Cole P, Ladanyi M, Gerald W, Cheung N, Kramer K, LaQuaglia M, et al. Synovial sarcoma mimicking desmoplastic small round-cell tumor: critical role for molecular diagnosis. Med Pediatr Oncol. 1999;32:97-101 pubmed
  45. Müller S, Soder S, Oliveira A, Inwards C, Aigner T. Type II collagen as specific marker for mesenchymal chondrosarcomas compared to other small cell sarcomas of the skeleton. Mod Pathol. 2005;18:1088-94 pubmed
    ..Thus, the presence of type II collagen in the extracellular tumor matrix significantly facilitates the diagnosis of mesenchymal chondrosarcomas in the absence of histologically visible chondroid matrix formation...
  46. Liptay M, Fry W. Malignant bone tumors of the chest wall. Semin Thorac Cardiovasc Surg. 1999;11:278-84 pubmed
    ..This article reviews these remaining five malignant bony chest wall tumors, along with their symptoms, presentations, and current approaches to therapy...
  47. Friedrichs N, Kriegl L, Poremba C, Schaefer K, Gabbert H, Shimomura A, et al. Pitfalls in the detection of t(11;22) translocation by fluorescence in situ hybridization and RT-PCR: a single-blinded study. Diagn Mol Pathol. 2006;15:83-9 pubmed
    ..We recommend FISH as a sensitive screening tool in the detection of t(11;22) followed by subsequent PCR amplification of the specific chimeric transcript...
  48. McPherson F, Chamyan G, Gilbert Barness E. Pathology quiz: small cell osteosarcoma. Pediatr Pathol Mol Med. 2001;20:345-8 pubmed
    ..The patient underwent a biopsy followed by excision of the mass (Figure 2) and decompressive laminectomy with reconstruction...
  49. Fujita J, Bandoh S, Ohtsuki Y, Dobashi N, Hiroi M, Takeuchi T, et al. The role of anti-epithelial cell antibodies in the pathogenesis of bilateral radiation pneumonitis caused by unilateral thoracic irradiation. Respir Med. 2000;94:875-80 pubmed
    ..The possible association between autoantibodies to cytokeratins and diffuse alveolar damage observed in patients with bilateral radiation pneumonitis are discussed...
  50. Mierau G, Berry P, Malott R, Weeks D. Appraisal of the comparative utility of immunohistochemistry and electron microscopy in the diagnosis of childhood round cell tumors. Ultrastruct Pathol. 1996;20:507-17 pubmed
    ..Application of these ancillary techniques resulted in a revision of the provisional diagnosis in 11 of 50 cases, and left only two cases without a firm specific diagnosis...
  51. Howman Giles R, Uren R, Kellie S. Gallium and thallium scintigraphy in pediatric peripheral primitive neuroectodermal tumor (Askin tumor) of the chest wall. J Nucl Med. 1995;36:814-6 pubmed
    ..Histology showed a peripheral neuroectodermal tumor of the chest wall typical of the malignancy described as the Askin tumor. The 201Tl studies were a more accurate method of following tumor response to therapy than 67Ga scintigraphy...
  52. Roy S, Saroha V, Jain D. Highly cellular leiomyoma mimics a malignant small round-cell tumor: a diagnostic dilemma on frozen sections. Taiwan J Obstet Gynecol. 2010;49:203-5 pubmed publisher
    ..Cellular leiomyoma is characterized by significantly increased cellularity and may mimic malignant tumors. Our aim was to differentiate these from other malignant small round-cell tumors...
  53. Ferrari A, Grosso F, Stacchiotti S, Meazza C, Zaffignani E, Marchiano A, et al. Response to vinorelbine and low-dose cyclophosphamide chemotherapy in two patients with desmoplastic small round cell tumor. Pediatr Blood Cancer. 2007;49:864-6 pubmed