Title

Pigmented Perivascular Epithelioid Cell Tumor of the Skin: First Case Report

Publication Date

2015

Journal Title

Am J Dermatopathol

Abstract

The authors present a first case of pigmented perivascular epithelioid cell tumor (PEComa) of the skin, which was misdiagnosed histopathologically as metastatic melanoma. PEComa are exceedingly rare neoplasms, and only a few cases have been reported in kidney, liver, and pelvis. Herein, we describe this very rare entity in the subcutaneous tissue of the flank with very similar if not identical morphology of renal clear cell carcinoma. Because of the presence of focal melanin pigmentation and strong positivity for HMB-45, the lesion was mistaken for metastatic melanoma. To the best of authors' knowledge, this is the first case of cutaneous PEComa with melanin pigmentation. Awareness about occurrence of the lesion in the subcutaneous tissue and its unique immunoprofile would be helpful to reach correct diagnosis and to distinguish this lesion from melanoma or metastatic renal clear cell carcinoma.

Volume Number

37

Issue Number

11

Pages

866-9

Document Type

Article

EPub Date

2015/09/12

Status

Faculty

Facility

School of Medicine

Primary Department

Pathology and Laboratory Medicine

Additional Departments

Dermatology

PMID

26355762

DOI

10.1097/dad.0000000000000320