10 January 2007 – Case of the Week #69

 

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We thank Dr. Sharon Bihlmeyer, University of Vermont, Burlington, Vermont (USA), for contributing this case.  We invite you to contribute a Case of the Week by sending an email to NPernick@PathologyOutlines.com with microscopic images (any size, we will shrink if necessary) in JPG or GIF format, a clinical history, your diagnosis and any other images (gross, immunostains, EM, etc.) that may be helpful or interesting.  We will write the discussion (unless you want to), list you as the contributor, and send you a check for $35 (US) for your time after we send out the case.  Please only send cases with a definitive diagnosis. 

 

Case of the Week #69

 

Clinical history

 

A 34 year old man had a scrotal abscess, and underwent an incision and drainage procedure.

 

The specimen showed large areas of necrosis (image #1), and infiltrative margins (image #2).  There were also granuloma-like areas (image #3) and rhabdoid-like cells (image #4).  The tumor cells were immunoreactive for EMA and CD34 (EMA, CD34) and negative for PLAP (PLAP).

 

What is your diagnosis? 

 

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Diagnosis:

 

Epithelioid sarcoma - reviewed by Dr. Chris Fletcher, who concurs

 

Discussion

 

Epithelioid sarcoma is a rare soft-tissue sarcoma, which typically presents as a subcutaneous or deep dermal mass in the distal extremities of individuals ages 10-35 years, with two thirds occurring in males.  They are often misdiagnosed due to their relatively bland initial appearance.

 

In 1997, Guillou described a more aggressive "proximal" type sarcoma that primarily involved the pelvis (Am J Surg Pathol 1997;21:130).  These tumors are composed primarily of large epithelioid cells with more atypia than classic epithelioid sarcoma, and often resemble a rhabdoid tumor due to intracytoplasmic hyaline inclusions.  Large areas of necrosis and a multinodular pattern are common, but a granuloma-like pattern is uncommon.  The tumor cells are immunoreactive for keratin, EMA and vimentin, with variable immunostaining for desmin, CD34 and smooth muscle actin.  They are negative for S100.  The scrotum is an unusual site for epithelioid sarcoma, with only two case reports identified (Eur Urol 2006;49:406, Diagn Cytopathol 2001;24:36)

 

Enzinger and Weiss believe this tumor may be a variant of extrarenal malignant rhabdoid tumor (Weiss: Enzinger and Weiss’s Soft Tissue Tumors; 2001 (4th edition), p 1534).  The differential diagnosis includes epithelioid MPNST (S100+, keratin negative, rarely EMA+) and melanoma (S100+, usually HMB45+), as well as classic epithelioid sarcoma.

 

These tumors behave aggressively, with frequent metastases leading to death. 

 

 

 

Nat Pernick, M.D., President
PathologyOutlines.com, Inc.

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