PEComa

(redirected from perivascular epithelioid cell tumor)

PEComa

A group of mesenchymal tumors composed of epithelioid and spindle cells displaying perivascular epithelioid (PEC) differentiation. The PEComa family of tumors includes angiomyolipoma, clear-cell "sugar" tumor of lung and gastrointestinal tract and other sites, lymphangioleimyomatosis, clear-cell myomelanocytic tumor of the falciform ligament.

PEComa

A rare, possibly mesenchymal tumour of unknown origin and of uncertain malignant potential, which can arise anywhere in the body and more commonly affects females.
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3) The term PEComa, as an acronym for perivascular epithelioid cell tumor, was first introduced in 1996 by Zamboni et al, (4) who used it to describe a pancreatic tumor composed of human melanoma black 45 (HMB-45)-positive clear epithelioid cells.
For metastatic CCRCC to the pancreas, the differential diagnoses include ductal adenocarcinoma with clear cell appearance (clear cell carcinoma), clear cell endocrine pancreatic tumor, solid serous adenoma of the pancreas, clear cell variant of solid pseudopapillary tumor of the pancreas, and perivascular epithelioid cell tumor (PEComa).
Epithelioid AML (EAML), a member of the perivascular epithelioid cell tumor (PEComa) family, is an uncommon renal tumor that can be mistaken for RCC and may have morphologic and immunophenotypic overlap with t-RCC.
Synovial sarcoma, malignant granular cell tumor, and perivascular epithelioid cell tumor can have epithelioid or spindle cell morphologies that overlap with CCSLGT, and again, immunohistochemistry is vital in distinguishing these entities.
Perivascular Epithelioid Cell Tumor Versus LMS and Endometrial Stromal Sarcoma.
The differential diagnostic considerations of a clear cell lesion in the skin are broad and include melanocytic lesions (balloon cell nevus or balloon cell melanoma), epithelial neoplasms (benign adnexal neoplasm and carcinoma with clear cells), xanthomas, and various mesenchymal neoplasms (eg, perivascular epithelioid cell tumor or clear cell sarcoma; Table 1).
Perivascular epithelioid cell tumor of the gallbladder is an unusual epithelioid neoplasm.
The microscopic differential diagnosis includes endometrial stromal nodule, angiomyofibroblastoma, and perivascular epithelioid cell tumor.
11,20-25) Other conditions that may enter into the differential diagnostic consideration less frequently are adenosarcoma, carcinosarcoma, perivascular epithelioid cell tumor, and adenomatoid tumor.
Here, we describe an unusual mesenchymal tumor of the appendix with morphologic and immunohistochemical features of transcription factor E3 (TFE3)-positive, benign perivascular epithelioid cell tumor (PEComa) presenting as acute appendicitis.
Perivascular epithelioid cell tumor (PEComa) is a mesenchymal tumor with perivascular epithelioid clear cell that coexpresses melanocytic and muscle markers.