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Clinicopathological features with outcomes of a series of conventional and proximal-type epithelioid sarcomas, diagnosed over a period of 10 years at a tertiary cancer hospital in India

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Abstract

Epithelioid sarcoma (ES) is an uncommon sarcoma. Lately, its variants, including proximal-type ES, have been recognized. The present study highlights clinicopathological features of 26 (65%) conventional and 14 (35%) cases of proximal-type ES. Thirty-eight percent of cases were seen in 21–30-year age group, including 77.5% cases in men. Extremities were the commonest sites in both the subtypes. Histologically, conventional-type ES displayed nodular tumor aggregates with necrosis, while proximal-type showed solid arrangement of large, “rhabdoid-like” cells. More cases (64.2%) of the proximal type were of grade 3. A range of differential diagnoses was considered. Most important immunohistochemical markers were vimentin, epithelial membrane antigen, cytokeratin, CD34, and desmin. Maximum (72.5%) cases were treated surgically. Recurrences and metastasis were observed more in the proximal type. The 7-year disease-free survival was 19.4% in the conventional and nil in the proximal subtype (p = 0.06). The overall survival rate was also lower in the proximal (31.3%) than conventional type (90.2%; p < 0.001). Other unfavorable parameters were deeper location, larger size, and higher tumor stage. This unusual sarcoma, with characteristic growth patterns, merits a proper histological evaluation, as it has many mimics. Proximal-type ES is rather a morphological subtype, associated with an aggressive course.

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References

  1. Billings SD, Folpe AL, Weiss SW (2003) Epithelioid sarcoma-like hemangioendothelioma. Am J Surg Pathol 27:48–57

    Article  PubMed  Google Scholar 

  2. Bos GD, Pritchard DJ, Reiman HM, Dobyns JH, Ilstrup DM, Landon GC (1988) Epithelioid sarcoma. An analysis of fifty-one cases. J Bone Jt Surg Am 70:862–870

    CAS  Google Scholar 

  3. Casanova M, Ferrari A, Collini P, Bisogno G, Alaggio R, Cecchetto G, Gronchi A, Meazza C, Garaventa A, Di Cataldo A, Carli M, Italian Soft Tissue Sarcoma Committee (2006) Epithelioid sarcoma in children and adolescents: a report from the Italian Soft Tissue Sarcoma Committee. Cancer 106:708–717

    Article  PubMed  Google Scholar 

  4. Chase DR, Enzinger FM (1985) Epithelioid sarcoma. Diagnosis, prognostic indicators, and treatment. Am J Surg Pathol 9:241–263

    Article  PubMed  CAS  Google Scholar 

  5. Deyrup AT, Weiss SW (2006) Grading of soft tissue sarcomas: the challenge of providing precise information in an imprecise world. Histopathology 48:42–50

    Article  PubMed  CAS  Google Scholar 

  6. Enneking WF, Spanier SS, Goodman MD (1990) A system for the surgical staging of musculoskeletal sarcoma. Clin Orthop Relat Res 153:106–120

    Google Scholar 

  7. Enzinger FM (1970) Epithelioid sarcoma: a sarcoma simulating a granuloma or a carcinoma. Cancer 26:1029–1041

    Article  PubMed  CAS  Google Scholar 

  8. Evans HL, Baer SC (1993) Epithelioid sarcoma: a clinicopathologic and prognostic study of 26 cases. Semin Diagn Pathol 10:286–291

    PubMed  CAS  Google Scholar 

  9. Fletcher CDM, Unni KK, Mertens F (2002) In: Fletcher CDM, Unni KK, Mertens F (eds) World Health Organization (WHO) classification of tumors of soft tissues and bones. Pathology and genetics. IARC, Lyon, France

    Google Scholar 

  10. Guillou L, Wadden C, Coindre JM, Krausz T, Fletcher CD (1997) “Proximal-type” epithelioid sarcoma, a distinctive aggressive neoplasm showing rhabdoid features. Clinicopathologic, immunohistochemical, and ultrastructural study of a series. Am J Surg Pathol 21:130–146

    Article  PubMed  CAS  Google Scholar 

  11. Gururangan S, Bowman LC, Parham DM, Wilimas JA, Rao B, Pratt CB, Douglass EC (1993) Primary extracranial rhabdoid tumors. Clinicopathologic features and response to ifosfamide. Cancer 71:2653–2659

    Article  PubMed  CAS  Google Scholar 

  12. Hall DJ Grimes MM, Goplerud DR (1980) Epithelioid sarcoma of vulva. Gynecol Oncol 9:237–246

    Article  Google Scholar 

  13. Hasegawa T, Matsuno Y, Shimoda T, Umeda T, Yokoyama R, Hirohashi S (2001) Proximal-type epithelioid sarcoma: a clinicopathologic study of 20 cases. Mod Pathol 14:655–663

    Article  PubMed  CAS  Google Scholar 

  14. Iossifides I, Ayala AG, Johnson DE (1979) Epithelioid sarcoma of penis. Urology 14:190–191

    Article  PubMed  CAS  Google Scholar 

  15. Kodet R, Newton WA Jr, Sachs N, Hamoudi AB, Raney RB, Asmar L, Gehan EA (1991) Rhabdoid tumors of soft tissues: a clinicopathologic study of 26 cases enrolled on the Intergroup Rhabdomyosarcoma Study. Human Pathol 22:674–684

    Article  CAS  Google Scholar 

  16. Laskin WB, Miettinen M (2003) Epithelioid sarcoma: new insights based on an extended immunohistochemical analysis. Arch Pathol Lab Med 127:1161–1168

    PubMed  Google Scholar 

  17. Miettinen M, Fanburg-Smith JC, Virolainen M, Shmookler BM, Fetsch JF (1999) Epithelioid sarcoma: an immunohistochemical analysis of 11 classical and variant cases and a discussion of the differential diagnosis. Human Pathol 30:934–942

    Article  CAS  Google Scholar 

  18. Mirra JM, Kessler S, Bhuta S, Eckardt J (1992) The fibroma-like variant of epithelioid sarcoma. A fibrohistiocytic/myoid cell lesion often confused with benign and malignant spindle cell tumors. Cancer 69:1382–1395

    Article  PubMed  CAS  Google Scholar 

  19. Modena P, Lualdi E, Facchinetti F, Galli L, Teixeira MR, Pilotti S, Sozzi G (2005) SMARCB1/INI1 tumor suppressor gene is frequently inactivated in epithelioid sarcomas. Cancer Res 15:4012–4019

    Article  Google Scholar 

  20. Molenaar WM, DeJong B, Dam-Meiring A, Postma A, DeVries J, Hoekstra HJ (1989) Epithelioid sarcoma or malignant rhabdoid tumor of soft tissue? Epithelioid immunophenotype and rhabdoid karyotype. Human Pathol 4:347–351

    Article  Google Scholar 

  21. Prat J, Woodruff JM, Marcove RC (1978) Epithelioid sarcoma: an analysis of 22 cases indicating the prognostic significance of vascular invasion and regional lymph node metastasis. Cancer 41:1472–1487

    Article  PubMed  CAS  Google Scholar 

  22. Ramanathan RC, A’Hern R, Fisher C, Thomas JM (1999) Modified staging system for extremity soft tissue sarcomas. Ann Surg Oncol 6:57–69

    Article  PubMed  CAS  Google Scholar 

  23. Smith MEF, Awasthi R, O’Shaugnessy S et al (2005) Evaluation of perineurial differentiation in epithelioid sarcoma. Histopathology 47:575–581

    PubMed  CAS  Google Scholar 

  24. Spillane AJ, Thomas JM, Fisher C (2000) Epithelioid sarcoma: the clinicopathological complexities of this rare soft tissue sarcoma. Ann Surg Oncol 7:218–225

    Article  PubMed  CAS  Google Scholar 

  25. van de Rijn M, Rouse RV (1994) CD34: A review. Appl Immunohistochem 2:71–80

    Google Scholar 

  26. von Hochstetter AR, Grant JW, Meyer VE, Honegger HP, Schreiber A (1990) Angiomatoid variant of epithelioid sarcoma. The value of immunohistochemistry in the differential diagnosis. Chir Organi Mov 75:158–162

    Google Scholar 

  27. Zevallos-Giampietri EA, Barrionuevo C (2005) Proximal-type epithelioid sarcoma: report of two cases in the perineum: differential diagnosis and review of soft tissue tumors with epithelioid and/or rhabdoid features. Appl Immunohistochem Mol Morphol 13:221–230

    Article  PubMed  Google Scholar 

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Acknowledgments

We are highly grateful to Dr. Rajesh Dikshit, Ph.D., Epidemiologist, and Mr. Sanjay Talole, Scientific Officer, Biostatistics and Epidemiology division, for the statistical analysis. We would like to thank Dr. Siddharth Laskar, M.D., Associate Professor, Radiation Oncology, for his valuable inputs in the treatment details. We thank Mr. Nilesh Ganthade, Officer In-charge, Medical Graphics Department for his inputs in formatting the images. We have no financial relationship with any organization in terms of this study.

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We declare that we have no conflict of interest.

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  1. Deparment of Pathology, Tata Memorial Hospital, Dr E.B. Road, Parel, Mumbai, India, 400012

    Bharat Rekhi, Biru D. Gorad & R. F. Chinoy

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  1. Bharat Rekhi
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  2. Biru D. Gorad
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  3. R. F. Chinoy
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Correspondence to Bharat Rekhi.

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Rekhi, B., Gorad, B.D. & Chinoy, R.F. Clinicopathological features with outcomes of a series of conventional and proximal-type epithelioid sarcomas, diagnosed over a period of 10 years at a tertiary cancer hospital in India. Virchows Arch 453, 141–153 (2008). https://doi.org/10.1007/s00428-008-0639-0

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  • DOI: https://doi.org/10.1007/s00428-008-0639-0

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