Retroperitoneal sclerosing PEComa with melanin pigmentation and granulomatous inflammation-A rare association within an uncommon tumor
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...
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Wolters Kluwer Medknow Publications,
2012-01-01T00:00:00Z.
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| LEADER | 00000 am a22000003u 4500 | ||
|---|---|---|---|
| 001 | doaj_6c9c9dd09cfc42f9ab36f07a2a3e87b4 | ||
| 042 | |a dc | ||
| 100 | 1 | 0 | |a Bharat Rekhi |e author |
| 700 | 1 | 0 | |a Mukund Sable |e author |
| 700 | 1 | 0 | |a Sangeeta B Desai |e author |
| 245 | 0 | 0 | |a Retroperitoneal sclerosing PEComa with melanin pigmentation and granulomatous inflammation-A rare association within an uncommon tumor |
| 260 | |b Wolters Kluwer Medknow Publications, |c 2012-01-01T00:00:00Z. | ||
| 500 | |a 0377-4929 | ||
| 500 | |a 10.4103/0377-4929.101757 | ||
| 520 | |a 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. | ||
| 546 | |a EN | ||
| 690 | |a HMB45 | ||
| 690 | |a melanoma | ||
| 690 | |a myomelanocytic tumor | ||
| 690 | |a PEComa | ||
| 690 | |a retroperitoneal sarcomas | ||
| 690 | |a sclerosing PEComa | ||
| 690 | |a uncommon soft tissue tumors | ||
| 690 | |a Pathology | ||
| 690 | |a RB1-214 | ||
| 690 | |a Microbiology | ||
| 690 | |a QR1-502 | ||
| 655 | 7 | |a article |2 local | |
| 786 | 0 | |n Indian Journal of Pathology and Microbiology, Vol 55, Iss 3, Pp 395-398 (2012) | |
| 787 | 0 | |n http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2012;volume=55;issue=3;spage=395;epage=398;aulast=Rekhi | |
| 787 | 0 | |n https://doaj.org/toc/0377-4929 | |
| 856 | 4 | 1 | |u https://doaj.org/article/6c9c9dd09cfc42f9ab36f07a2a3e87b4 |z Connect to this object online. |