Perivascular epithelioid cell tumour (PEComa) of the uterus associated with malignant neoplasm of the female genital tract

A Case of Malignant PEComa of the Uterus Associated with Intramural Leiomyoma and Endometrial Carcinoma

“…There was a report of a concomitant PEComa and an endometrioid carcinoma with synchronous/metastatic bilateral ovary carcinomas and uterine leiomyoma [12]. The case had a finding common to ours in that there was a well differentiated endometrial carcinoma involving a depth of more than half of the myometrium with a synchronous separate PEComa.…”

Perivascular epithelioid cell neoplasms: pathology and pathogenesis

“…Equally important was the expansion of the morphologic spectrum of renal and hepatic AML to include "carcinoma-like" purely epithelioid variants [25,26], respectively, and the recognition of purely spindled PEComas, initially termed clear cell myomelanocytic tumors [3]. Most recently, PEComas showing a spectrum of histologic appearances and clinical behaviors have been described in a wide variety of intra-abdominal, visceral, gynecologic, soft tissue, bone, and cutaneous sites, including the falciform ligament/ligamentum teres, uterus, mesentery, heart, thigh and skull base, among others [3,5,7,[27][28][29][30][31][32][33][34][35][36][37][38][39][40][41][42][43][44][45][46].…”

Perivascular epithelioid cell tumor (PEComa) of the uterine cervix associated with intraabdominal "PEComatosis": A clinicopathological study with comparative genomic hybridization analysis

“…Members of the PEComa family that were recognized include AML, CCST, lymphangioleiomyomatosis (LAM), clear cell myomelanocytic tumor (CCMMT) of the falciform ligament/ ligamentum teres and a heterogeneous group of other "unusual clear cell tumors" at various anatomic sites [13]. The latter group includes tumors that have been reported under varying designations, such as abdominopelvic sarcoma of perivascular epithelioid cells [6], primary extrapulmonary sugar tumor (PEST) [38], clear cell myomelanocytic tumors of the skin [7] and thigh [15], and simply PEComa of various anatomic sites [1,9,12,19,24,27,28,31,40,41,45,46]; these, in addition to CCMMT of the falciform ligament [14] will henceforth be referred to as PEComa n ot o therwise s pecified (PEComa NOS). This descriptive designation, as used in this report, excludes the well-established entities LAM, CCST of the lungs and all variants of AML.…”