Skip to main content
SpringerLink
Log in

Round Cell Sarcomas

  • Chapter
  • First Online:
Small Volume Biopsy in Pediatric Tumors

Abstract

Round cell sarcomas are a part of a larger group of malignant small round cell tumors which, in addition to sarcomas, includes lymphoma and blastemal tumors like neuroblastoma and nephroblastoma. They are high-grade sarcomas and are much more common in children and young adults as compared to older populations [1]. Since they are morphologically similar and even share certain antigenic characteristics, it is often difficult to tell them apart, especially in small-volume biopsies. Molecular diagnostics and flow cytometry are useful in their differentiation [2]. This chapter contains only round cell sarcomas. Lymphomas and blastemal tumors are described in separate chapters. Osteogenic sarcoma and synovial sarcoma are not round cell tumor; however, both have a round cell variant. Round cell osteosarcoma is extremely rare and will be mentioned together with conventional osteosarcomas that are much more common in children. Synovial sarcoma, on the other hand, occurs rather frequently in young people. Therefore, its round cell variant is included in this chapter.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 169.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 219.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 219.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Singh HK, Kilpatrik SE, Silverman JF. Fine needle aspiration biopsy of soft tissue sarcomas. Utility and diagnostic challenges. Adv Anat Pathol. 2004;11:24–37.

    Article  PubMed  Google Scholar 

  2. Barroca H, Bom-Sucesso M. Fine needle biopsy with cytology in paediatrics: the importance of a multidisciplinary approach and the role of ancillary techniques. Cytopatology. 2013;25:6–20.

    Article  Google Scholar 

  3. Ognjanović S, Linabery AM, Charbonneau B, Ross JA. Trends in childhood rhabdomyosarcoma incidence and survival in the United States, 1975-2005. Cancer. 2009;115:4218–26.

    Article  PubMed  PubMed Central  Google Scholar 

  4. Data from cancer registry of Slovenia for period 1983–1997, Institute of Oncology, Ljubljana, Slovenia; 2000.

    Google Scholar 

  5. Pohar Marinšek Ž, Bračko M. Rhabdomyosarcoma. Cytomorphology, subtyping and differential diagnostic dilemas. Acta Cytol. 2000;44:524–32.

    Article  PubMed  Google Scholar 

  6. Akerman M, Domanski HA. The cytological features of soft tissue tumors in fine needle aspiration smears according to histiotype. In: Akerman M, Domanski HA, editors. The cytology of soft tissue tumors. Basel: Karger; 2003. p. 17–84.

    Chapter  Google Scholar 

  7. Layfield LJ. Neoplasms of striated muscle. In: Layfield LJ, editor. Cytopathology of bone and soft tissue tumors. New York: Oxford University Press; 2002. p. 119–30.

    Google Scholar 

  8. Atahan S̹, Aksu Ö, Ekinci C. Cytologic diagnosis and subtyping of rhabdomyosarcoma. Cytopathology. 1998;9:389–97.

    Article  CAS  PubMed  Google Scholar 

  9. Sebire NJ, Gibson S, Rampling D, Williams S, Malone M, Ramsay AD. Immunohistochemical findings in embryonal small round cell tumors with molecular diagnostic confirmation. Appl Immunohistochem Mol Morphol. 2005;13:1–5.

    Article  PubMed  Google Scholar 

  10. Pohar Marinšek Ž. Difficulties in diagnosing small round cell tumours of childhood from fine needle aspiration cytology samples. Cytopathology. 2008;19:67–79.

    Article  PubMed  Google Scholar 

  11. Bahrami A, Gown AM, Baird GS, Hicks MJ, Folpe AL. Aberrant expression of epithelial and neuroendocrine markers in alveolar rhabdomyosarcoma: a potentially serious diagnostic pitfall. Mod Pathol. 2008;21:795–806.

    Article  CAS  PubMed  Google Scholar 

  12. Carpentieri DF, Nichols K, Chou PM, Matthews M, Pawel B, Huff D. The expression of WT1 in the differentiation of rhabdomyosarcoma from other pediatric small round blue cell tumors. Mod Pathol. 2002;15:1080–6.

    Article  CAS  PubMed  Google Scholar 

  13. Folpe AL, Patterson K, Gown AM. Antineuroblastoma antibody NB-84 also identifies a significant subset of other small blue round cell tumors. Appl Immunohistochem. 1997;5:239–45.

    Article  Google Scholar 

  14. Davicioni E, Anderson MJ, Finckenstein FG, Lynch JC, Qualman SJ, Shimada H, et al. Molecular classification of rhabdomyosarcoma—genotypic and phenotipic determinants of diagnosis. A report from the Children’s Oncology group. Am J Pathol. 2009;174:550–64.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  15. Parham DM, Barr FG. Embryonal rhabdomyosarcoma. In: CDM F, Bridge J, PCW H, Fl M, editors. WHO classification of tumors of soft tissue and bone. Lyon: IARC Press; 2013. p. 127–9.

    Google Scholar 

  16. Klijanienko J, Caillaud JM, Orbach D, Brisse H, Lagacé R, Vielh P, et al. Cyto-histological correlations in primary, recurrent and metastatic rhabdomyosarcoma. The Institut Curie’s experience. Diagn Cytopathol. 2007;35:482–7.

    Article  PubMed  Google Scholar 

  17. Dehner LP. Ewing sarcoma-primitive neuroectodermal tumor (EWS/PNET). In: Stocker JT, Dehner LP, Husain AN, editors. Stocker and Dehner’s Pediatric Pathology. Philadelphia: Wolters Kluwer/Lippincott Williams and Wilkins; 2011. p. 1087–90.

    Google Scholar 

  18. Layfield LJ. Malignant peripheral primitive neuroectodermal tumor. In: Layfield LJ, editor. Cytopathology of bone and soft tissue tumors. New York: Oxford University Press; 2002. p. 113–6.

    Google Scholar 

  19. De Alava E, Lessnick SL, Sorensen PH. Ewing sarcoma. In: Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F, editors. WHO classification of tumors of soft tissue and bone. Lyon: IARC Press; 2013. p. 306–9.

    Google Scholar 

  20. Pohar Marinšek Ž, Kavalar R, Jereb B. Ewing sarcoma/PNET. 27 years of experience in Slovenia. Pediatr Hematol Oncol. 2006;23:355–67.

    Article  Google Scholar 

  21. Hameed M. Small round cell tumors of bone. Arch Pathol lab med. 2007;131:192–204.

    PubMed  Google Scholar 

  22. Suciu V, Fabre M, Klijanienko J, Pohar Marinšek Ž, Vielh P. Childhood tumors. In: Gray W, Kocjan G, editors. Diagnostic cytopathology. Edinburgh: Churchill Livingstone; 2010. p. 873–88.

    Google Scholar 

  23. Pohar Marinšek Ž. Cytopathology of Ewing/PNET. A research project (J3-5299-0302-03/3.04) sponsored by the Slovenian Research Agency, 2004. Report published in Slovene.

    Google Scholar 

  24. Akerman M, Domanski HA. The cytological features of soft tissue tumors in fine needle aspiration smears classified according to histiotype. In: Akerman M, Domanski HA, editors. The cytology of soft tissue tumors. Basel: Karger; 2003. p. 81.

    Chapter  Google Scholar 

  25. Gu M, Antonescu CR, Guiter G, Huvos AG, Ladanyi M, Zakowski MF. Cytokeratin immunoreactivity in Ewing’s sarcoma. Prevalence in 50 cases confirmed by molecular diagnostic studies. Am J Surg Pathol. 2000;24:410–6.

    Article  CAS  PubMed  Google Scholar 

  26. Barnoud R, Sabourin JC, Pasquier D, Ranchère D, Bailly C, Terrier-Lacombe MJ, et al. Immunohistochemical expression of WT1 by desmoplstic small round cell tumor: a comparative study with other small round cell tumors. Am J Surg Pathol. 2000;24(6):830.

    Article  CAS  PubMed  Google Scholar 

  27. Klijanienko J, Couturier J, Bourdeaut F, Fréneaux P, Ballet S, Brisse H, et al. Fine-needle aspiration as a diagnostic technique in 50 cases of primary Ewing sarcoma /peripheral neuroectodermal tumor. Institut Curie's experience. Diagn Cytopathol. 2012;40:19–25.

    Article  PubMed  Google Scholar 

  28. Åkerman M, Ryd W, Skytting B. Fine-needle aspiration of synovial sarcoma: criteria for diagnosis: retrospective reexamination of 37 cases, including ancillary diagnostics. A Scandinavian sarcoma group study. Diagn Cytopathol. 2003;28:232–8.

    Article  PubMed  Google Scholar 

  29. Klijanienko J, Caillaud JM, Lagacé R, Vielh P. Cytohistologic correlations in 56 synovial sarcomas in 36 patients. The Institute Curie Experience Diagn Cytopathol. 2002;27:96–102.

    Article  Google Scholar 

  30. Fröstad B, Tani E, Brosjö O, Skook L, Kogner P. Fine needle aspiration cytology in the diagnosis and management of children and adolescents with ewing sarcoma and peripheral primitive neuroektodermal tumor. Med Pediatr Oncol. 2002;38:33–40.

    Article  PubMed  Google Scholar 

  31. Dehner LP. Desmoplastic small round cell tumor. In: Stocker JT, Dehner LP, Husain AN, editors. Stocker and Dehner’s pediatric pathology. Philadelphia: Wolters Kluwer/Lippincott Williams and Wilkins; 2011. p. 1090–1.

    Google Scholar 

  32. Antonescu SR, Ladanyi M. Desmoplastic small round cell tumor. In: Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F, editors. WHO classification of tumors of soft tissue and bone. Lyon: IARC Press; 2013. p. 225–7.

    Google Scholar 

  33. PoharMarinšek Ž, Khalbuss WE. Cytopathology of soft tissue lesions with predominance of epithelioid, round/polygonal cells, glandular cells, inflammatory cells. In: Khalbuss WE, Parwani AV, editors. Cytopathology of soft tissue and bone lesions. New York: Springer; 2011. p. 147–50.

    Google Scholar 

  34. Klijanienko J, Colin P, Couturier J, Lagacé R, Fréneaux P, Pierron G, et al. Fine-needle aspiration in desmoplastic small round cell tumor. A report of 10 new tumors in 8 patients with clinicopathological and molecular correlations with review of the literature. Cancer Cytopathol. 2014;122:386–93.

    Article  PubMed  Google Scholar 

  35. Crapanzano JP, Cardillo M, Lin O, Zakowski MF. Cytology of desmoplastic small round cell tumor. A series including findings in ThinPrep. Cancer Cytopathol. 2002;25:21–31.

    Article  Google Scholar 

  36. Granja NM, Begnami MD, Bortolan J, Filho AL, Schmitt FC. Desmoplastic small round cell tumor: cytological and immunocytochemical features. Cytojournal. 2005;2(1):a6.

    Article  Google Scholar 

  37. Šebenik M, Pohar Marinšek Ž. Cytology of soft tissue lesions. In: Miettinen M, editor. Modern soft tissue pathology tumors and non-neoplastic conditions. Cambridge: Cambridge Univ Press; 2010. p. 1016–56.

    Chapter  Google Scholar 

  38. Dave B, Shet T, Chinoy R. Desmoplastic round cell tumor of childhood: can cytology with immunocytochemistry serve as an alternative for tissue diagnosis? Diagn Cytopathol. 2005;32:330–5.

    Article  PubMed  Google Scholar 

  39. Oda Y, Biegel JA. Extrarenal rhabdoid tumor. In: Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F, editors. WHO Classification of tumors of soft tissue and bone. Lyon: IARC Press; 2013. p. 228–9.

    Google Scholar 

  40. Dehner LP. Malignant rhabdoid tumor. In: Stocker JT, Dehner LP, Husain AN, editors. Stocker and Dehner’s pediatric pathology. Philadelphia: Wolters Kluwer/Lippincott Williams and Wilkins; 2011. p. 1096–7.

    Google Scholar 

  41. Drut R, Drut RM. Renal and extrarenal congenital rhabdoid tumor: diagnosis by fine-needle aspiration biopsy and FISH. Diagn Cytopathol. 2002;27:32–4.

    Article  PubMed  Google Scholar 

  42. Thomson TA, Klijanienko J, Couturier J, Brisse H, Pierron G, Freneaux P, et al. Fine-needle aspiration of renal and extrarenal rhabdoid tumors. The experience of the Institute Curie regarding 20 tumors in 13 patients. Cancer Cytopathol. 2011;119:49–57.

    Article  PubMed  Google Scholar 

  43. Pogačnik A, Zidar N. Malignant rhabdoid tumor of the liver diagnosed by fine needle aspiration cytology. A case report. Acta Cytol. 1997;41:539–43.

    Article  PubMed  Google Scholar 

  44. Salamanca J, Rodríguez-Peralto JL, Azorín D, Ballestín C, De Augustín P. Paratesticular congenital malignant rhabdoid tumor diagnosed by fine-needle aspiration cytology. A case report. Diagn Cytopathol. 2004;30:46–50.

    Article  PubMed  Google Scholar 

  45. Nakashima Y. de Pinieux, Ladanyi M. Mesenchymal chondrosarcoma. In: Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F, editors. WHO classification of tumors of soft tissue and bone. IARC Press: Lyon; 2013. p. 271–2.

    Google Scholar 

  46. Dantonello TM, Int-Veen C, Leuschner I, Schuck A, Furtwaengler R, Claviez A, et al. Mesenchymal chondrosarcoma of soft tissues and bone in children, adolescents and young adults: experiences of the CWS and COSS study groups. Cancer. 2008;112:2424–31.

    Article  PubMed  Google Scholar 

  47. Misra V, Singh PA. Cytodiagnosis of extraosseous mesenchymal chondrosarcoma of meninges: a case report. Acta Cytol. 2008;52:366–9.

    Article  PubMed  Google Scholar 

  48. Trembath DG, Dash R, Major NM, Dodd LG. Cytopathology of mesenchymal chondrosarcoma. A report and comparison of four patients. Cancer Cytopathol. 2003;99:211–6.

    Article  Google Scholar 

  49. Domanski HA, Quian X, Stanley DE, Skeletal FNAC. Mesenchymal chondrosarcoma. In: Domanski HA, editor. Atlas of fine needle aspiration cytology. London: Springer-Verlag; 2014. p. 500–5.

    Chapter  Google Scholar 

  50. Layfield LJ. Mesenchymal chondrosarcoma. In: Layfield LJ, editor. Cytopathology of bone and soft tissue tumors. New York: Oxford University Press; 2002. p. 183–5.

    Google Scholar 

  51. Doria MI Jr, Wang HH, Chinoy MJ. Retroperitoneal mesenchymal chondrosarcoma. Report of a case diagnosed by fine needle aspiration cytology. Acta Cytol. 1990;34:529–32.

    PubMed  Google Scholar 

  52. Handa V, Singhal N, Punia RS, Garg S, Mohan H. Cytologic features and differential diagnosis in a case of extraskeletal mesenchymal chondrosarcoma. Acta Cytol. 2009;53(6):704.

    Article  PubMed  Google Scholar 

  53. Šebenik M, Pohar Marinšek Ž. Cytology of soft tissue lesions. In: Miettinen M, editor. Modern soft tissue pathology tumors and non-neoplastic conditions. Cambridge: Cambridge Univ Press; 2010. p. 1044.

    Google Scholar 

  54. Suurmeijer AJH, deBruijn D, Geurts van Kessel A, Miettinen MM. Synovial sarcoma. In: Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F, editors. WHO classification of tumors of soft tissue and bone. Lyon: IARC Press; 2013. p. 213–5.

    Google Scholar 

  55. Miettinen M. Primary soft tissue tumors with epithelil differentiation. In: Miettinen M, editor. Modern soft tissue pathology: Tumors and non-neoplastic conditions. Cambridge: Cambridge University Press; 2010. p. 783.

    Chapter  Google Scholar 

  56. Åkerma M, Domanski HA. Synovial sarcoma. In: Akerman M, Domanski HA, editors. The cytology of soft tissue tumors. Basel: Karger; 2003. p. 98–9.

    Google Scholar 

  57. Machen SK, Fisher C, Gautam RS, Tubbs RR, Goldblum JR. Utility of cytokeratin subsets for diagnosing poorly differentiated synovial sarcoma from peripheral neuroectodermal tumor. Histopathol. 1998;33:501–7.

    Article  CAS  Google Scholar 

  58. Åkerman M, Domanski HA. The complex cytological features of synovial sarcoma in fine needle aspirates, an analysis of four illustrative cases. Cytopathology. 2007;18:234–40.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Cytopathology, Institute of Oncology, Zaloška cesta 2, 1000, Ljubljana, Slovenia

    Živa Pohar Marinšek M.D., Ph.D.

Authors
  1. Živa Pohar Marinšek M.D., Ph.D.
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Živa Pohar Marinšek M.D., Ph.D. .

Editor information

Editors and Affiliations

  1. Department of Pathology, Institut Curie, Paris Cedex, France

    Jerzy Klijanienko

  2. Department of Cytopathology, Institute of Oncology, Ljubljana, Slovenia

    Živa Pohar Marinšek

  3. Department of Pathology, Skåne University Hospital, Lund, Sweden

    Henryk A Domanski

Rights and permissions

Reprints and permissions

Copyright information

© 2018 Springer International Publishing AG

About this chapter

Cite this chapter

Pohar Marinšek, Ž. (2018). Round Cell Sarcomas. In: Klijanienko, J., Pohar Marinšek, Ž., Domanski, H. (eds) Small Volume Biopsy in Pediatric Tumors. Springer, Cham. https://doi.org/10.1007/978-3-319-61027-6_3

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-61027-6_3

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-61026-9

  • Online ISBN: 978-3-319-61027-6

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation