Uterine PEcoma: A Case Report with Diagnostic Conundrums

Excerpt

Perivascular epithelioid cell tumor (PEComa) is a rare type of mesenchymal neoplasm exhibiting melanocytic and myogenic differentiation and expressing lineage specific markers of the same [1]. The term PEComa was first used in 1943 by Apitz et al. to describe an "abnormal myoblast" in renal angiomyolipoma and includes myriad tumors including the more common angiomyolipoma (AML), clear cell "sugar" tumor of the lung (CCST), lymphangioleiomyomatosis (LAM), and other unusual clear cell tumors. The most common sites of affliction for PEComa, NOS are the gastrointestinal and the genitourinary tracts (around 20%, with the uterus being the most common site). Diagnosing uterine PEComas can be challenging due to its rarity and non-specific clinical and radiologic features. A definite diagnosis requires awareness of this entity along with accurate microscopy and immunohistochemical work up. This case report describes a patient with uterine PEComa along with the diagnostic conundrums. …