Indian Journal of Pathology and Microbiology

CASE REPORT
Year
: 2012  |  Volume : 55  |  Issue : 3  |  Page : 395--398

Retroperitoneal sclerosing PEComa with melanin pigmentation and granulomatous inflammation-A rare association within an uncommon tumor


Bharat Rekhi, Mukund Sable, Sangeeta B Desai 
 Department of Pathology, Tata Memorial Hospital, Parel, Mumbai, India

Correspondence Address:
Bharat Rekhi
Department of Pathology, Tata Memorial Hospital, Dr. E.B. Road, Parel, Mumbai-400 012
India

PEComa, defined as a perivascular epithelioid cell tumor, displays a wide clinicopathological spectrum. Lately, a sclerosing PEComa has been identified as its distinct variant, but with limited documentation, in view of its rarity. Herein, we describe an uncommon case of a 53-year-old lady, who was referred to us with pain abdomen. Radiological imaging disclosed a well-defined, hypodense retroperitoneal mass. The excised tumor was a round, encapsulated soft tissue mass measuring 7 cm with a tan-brown cut surface. Microscopy showed uniform, epithelioid cells with clear cytoplasm, focal melanin pigmentation and mild nuclear atypia, arranged in sheets and nests around capillary-sized vessels in a dense sclerotic stroma. Additionally, co-existing epithelioid granulomas were noted. On immunohistochemistry (IHC), tumor cells were diffusely positive for HMB45; focally for desmin and smooth muscle actin (SMA), while negative for EMA, CD10, S100-P, Melan A, CD34, AMACR and CK MNF116. This case reinforces sclerosing PEComa as an uncommon, but a distinct clinicopathological entity and exemplifies diagnostic challenge associated with it; necessitating application of IHC markers for its correct identification. Presence of melanin pigment and granulomatous inflammation in the present tumor constitute as novel histopathological findings in a sclerosing PEComa.


How to cite this article:
Rekhi B, Sable M, Desai SB. Retroperitoneal sclerosing PEComa with melanin pigmentation and granulomatous inflammation-A rare association within an uncommon tumor .Indian J Pathol Microbiol 2012;55:395-398


How to cite this URL:
Rekhi B, Sable M, Desai SB. Retroperitoneal sclerosing PEComa with melanin pigmentation and granulomatous inflammation-A rare association within an uncommon tumor . Indian J Pathol Microbiol [serial online] 2012 [cited 2019 Oct 14 ];55:395-398
Available from: http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2012;volume=55;issue=3;spage=395;epage=398;aulast=Rekhi;type=0