Malignant perivascular epithelioid cell tumor (malignant PEComa) is a rare disease for which the diagnostic criteria and treatment options have not been established. Since PEComa is associated with upregulation of mammalian target of rapamycin (mTOR) pathway which controls Glut-1 (glucose transporter) function, increased (18)F-fluorodeoxyglucose ((18)F-FDG) uptake may indicate the over activati...

Angiomyolipoma (AML) is a type of tumor in the perivascular epithelioid cell neoplasm (PEComa) family. Epithelioid angiomyolipoma (EAML), having malignant potential, is considered a rare variant of angiomyolipoma. The most common site of EAMLs is kidney, and extra-renal EAMLs are very uncommon, with liver being the most common site.1 Other locations including retroperitoneum, uterus, liver, lun...

We present a case of perivascular epithelioid cell tumors (PEComas) in the abdominal cavity at the falciform ligament. A 30-yr-old Korean man visited to hospital for the evaluation of a growing, palpable abdominal mass. He had felt the mass growing over 6 months. There was no family or personal history of tuberous sclerosis. The resected specimen showed a mass of 8.0x7.0x5.5 cm in size. Histolo...

Perivascular epithelioid cell tumors (PEComas) are rare soft-tissue tumors with an extremely heterogeneous clinical behavior. They may arise in different organs and may behave indolently or sometimes metastasize with different grades of biological aggressiveness. We report the case of a young woman with a primary inoperable PEComa of the liver with malignant histological features. Since the mTO...

We advance the hypothesis that the telocyte might be the cell of origin of both PEComas (perivascular epithelioid cell tumours) and GISTs (gastro-intestinal and extra-gastrointestinal stromal tumours). The hypothesis is supported by data from the literature reporting that both PEComas and GISTs, as well as telocytes, share the expression of several markers. These data were supplemented by origi...

BACKGROUND Perivascular epithelioid-cell tumor (PEComa) is a group of rare mesenchymal neoplasms that express myomelanocytic-cell markers and exhibit a wide variety of histopathological features. Although heterotopic pancreas has been reported to occur in the gastrointestinal tract, intrahepatic heterotopic pancreas has been reported only rarely. CASE PRESENTATION We present a case of intrahe...

Perivascular epithelioid cell tumors or PEComas can arise in any location in the body. However, a limited number of cases of gastric PEComa have been reported. We present two cases of gastric PEComas. The first case involved a 62-year-old woman who presented with a 4.2 cm gastric subepithelial mass in the prepyloric antrum, and the second case involved a 67-year-old man with a 5.0 cm mass sligh...