Perivascular epithelioid cell tumours (PEComas) other than angiomyolipoma, clear cell "sugar" tumour of the lung, and lymphangioleiomyomatosis are very rare mesenchymal tumours. The uterus seems to be the most prevalent site of involvement, but only 13 cases of uterine PEComa have been described. Three of these cases exhibited local aggressive behaviour and only one showed metastasis. Because o...

Malignant perivascular epithelioid cell tumor (PEComa) is a rare tumor composed of hybrid tumor cells characterized by immunoreactivity for both melanocytic and smooth muscle markers. This paper describes the uncommon esophageal location of an 8 cm PEComa in a 75-year-old Caucasian man who was presented with ingravescent dysphagia. Although PEComas arising within the gastrointestinal tract are ...

RATIONAL Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal neoplasm which expresses both myogenic and melanocytic markers. PEComas are found in a variety locations in the body, but up to now only approximately 30 cases about hepatic perivascular epithelioid cell tumor are reported in English language worldwide. PATIENT CONCERNS A 32-year-old woman was admitted in our hospital...

Perivascular epithelioid cell tumors (PEComas) are a rare collection of tumors arising in a wide array of anatomic locations and characterized by a myomelanocytic phenotype. PEComas which occur in non-classic anatomic distributions are known as perivascular epithelioid cell tumor-not otherwise specified (PEComa-NOS), and one of the most common primary sites for PEComa-NOS is the uterus. The ris...

Perivascular epithelioid cell tumor (PEComa) is an extremely rare neoplasm that appears to arise most commonly at visceral (especially gastrointestinal and uterine), retroperitoneal, and abdominopelvic sites. Malignant PEComas exist but are very rare. These tumors represent a family of mesenchymal neoplasms, mechanistically linked through activation of the mTOR signaling pathway. Metastatic PEC...

Perivascular Epithelioid Cell Tumors (PEComas) are a family of rare neoplasms defined as mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular epithelioid cells [1]. Pea et al. first noted this unusual cell type in both renal angiomyolipoma (AML) and clear-cell “sugar” tumor (CCST) of the lung [2]. This generated the concept of a family of neoplasms co...

BACKGROUND We describe a case of malignant perivascular epithelial cell tumor (PEComa) arising primarily in the distal left femur of a 47-year-old male. CASE PRESENTATION The patient presented with pain accompanied by progressive swelling of his left thigh. Computed tomography (CT) scan and magnetic resonance imaging (MRI) revealed an osteolytic lesion. Curettage of the lesion was reported as...

We report a case of hepatic perivascular epithelioid cell tumor (PEComa) in a woman who was not a carrier of viral hepatitis and had a normal alpha-fetoprotein (AFP) level. CT scan showed a well-enhanced mass in the arterial-phase followed by early washout in the portal venous-phase in the lateral segment of the liver. Angiography revealed a hypervascular tumor in the liver with rapid washout o...

Perivascular epithelioid cell tumors (PEComas) constitute a rare subset of mesenchymal neoplasms classified by the World Health Organization in 2002. We present two cases of PEComas; the first is a cervical PEComa in a 35-year-old woman with no known past medical history who presented with a palpable pelvic mass; the second is an adnexal PEComa in a 39-year-old woman with a history of colitis w...