Peritoneal Perivascular Epithelioid Cell Tumor with Pulmonary Metastasis

Abstract

32 Perivascular epithelioid cell tumor (PEComa) is part of a rare family of mesenchymal tumors. PEComas occur at multiple sites and can undergo metastasis, recurrence, and aggressive clinical courses. The lung is a common metastatic site of PEComas. The role of estrogen in PEComas is unclear. In humans, estrogen might stimulate PEComa tumour cells because of a mutation in the TSC2 tumour suppressor gene, through MEK pathway.1 Blocking estrogen receptors might therefore inactivate the MEK pathway and provide a rationale for therapeutic efficacy.1 There are few reports about uterine malignant PEComa tumours with pulmonary metastases and fewer about the use of hormonal therapy, including tamoxifen.1-5 We report a case of a malignant peritoneal PEComa with pulmonary metastasis which has been treated for 2 years with tamoxifen and to the date the patient is alive and well, with a controlled disease.