PEComes abdomino-pelviens : à propos d'une série de cinq cas

We report five cases of abdomino-pelvic PEComas diagnosed in the last 10 years in the Rouen University Hospital. Four are hepatic and one is in a pelvic location which is unusual due to its strongly pigmented aspect. The tumors derived from “perivascular epithelioid cells” are rare. They are characterized by spindle or epithelioid cells in an immediate perivascular location. The immunochemistry is positive for HMB45, MelanA and smooth muscle Actin. The criteria for malignancy are infiltrative growth pattern, necrosis, high cellularity, high nuclear grade and mitotic activity. There are 8% of recurrence and 20% of metastasis (lung, bones, liver). This study presents the clinical, pathologic, immunohistochemical and molecular aspects of these PEComas and discusses the main differential diagnosis of the pigmented one.