Virchows Archiv

, Volume 453, Issue 2, pp 141–153 | Cite as

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

  • Bharat RekhiEmail author
  • Biru D. Gorad
  • R. F. Chinoy
Original Article


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.


Epithelioid sarcoma Proximal-type epithelioid sarcoma Uncommon sarcomas 



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.

Conflict of interest statement

We declare that we have no conflict of interest.


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Copyright information

© Springer-Verlag 2008

Authors and Affiliations

  1. 1.Deparment of PathologyTata Memorial HospitalMumbaiIndia

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