OBJECTIVES We describe the morphologic and immunohistochemical features of 17 endometrial stromal neoplasms, 16 sarcomas, and one stromal nodule. METHODS We reviewed 35 cases interpreted as endometrial stromal neoplasms, but 17 high-grade endometrial stromal sarcomas (ESS) and one case of mixed endometrial sarcoma and leiomyosarcoma were excluded from the study. Data from the Surveillance Epi...

Dear Editor, Perivascular epithelioid cell tumor, also known as PEComa, is a type of mesenchymal neoplasm. Here, we encountered an interesting case of primary PEComa in a cervical lymph node (LN) that was associated with leukoderma. A 63-year-old Japanese man was referred to us with a 3month history of a swollen LN in the left neck. The patient was a white collar worker belonging to an advertis...

Perivascular epithelioid cell tumor (PEComa) is a rare entity with distinctive morphology and of expressing myomelanocytic markers. Gastrointestinal tract (GI) is one of the most common anatomic sites of origin and counts for 20% to 25% of all reported cases of perivascular epithelioid cell tumors not otherwise specified (PEComas-NOS). However, the biologic behavior of perivascular epithelioid ...

We report a case of a 45-year-old woman who had a palpable mass in the left lower quadrant of the abdomen. Computed tomography (CT) showed a circumscribed mass in the left round ligament of the uterus. The mass had heterogeneous density and enhancement accompanied by mottled calcification, which was initially identified as a leiomyoma. A histopathological examination revealed a malignant periva...

Perivascular epithelioid cell tumors (PEComa) are mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular epithelial cells. Although the uterine corpus seems to be one of the most prevalent sites of involvement, PEComa of the uterine cervix are very rare. Only four cervical PEComa cases have been described, and were treated with hysterectomy and radiothe...

PEComas are uncommon mesenchymal tumors often involving the pelvic organs. They have an unpredictable behavior. Accurate diagnosis and long-term follow-up is therefore essential in these patients. We report this case of PEComa of uncertain malignant potential in an unusual location with excellent prognosis.

PEComas are a collection of generally rare tumors, defined by the World Health Organization as 'mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular epitheloid cells'. We describe the case of retroperitoneal PEComa with a liposarcoma-like appearance on cross-sectional imaging, but distinctive immunohistochemistry revealing the correct diagnosis.

Perivascular epithelioid cell-containing tumors (PEComas) represent a rare family of neoplasms. Their dichotomous phenotypic features, including both myogenic and mylanocytic features, can make a definitive diagnosis difficult. Such tumors have been associated with the overexpression of transcription factor E3 (TFE3). An Xp11 translocation could account for the aberrant activity of TFE3 but has...

A 54-year-old woman had a mass located in the right suprarenal area. On imaging, this mass appeared to be infiltrating the inferior vena cava (IVC). Exploratory laparotomy was undertaken and excision of the tumour was done with the sleeve of the involved IVC. The mass turned out to be a perivascular epithelioid cell tumour (PEComa) on histopathological examination. This report describes previou...