Molecular Characterization of Perivascular Epithelioid Cell Tumors

Perivascular epithelioid cell tumors (PEComas) are rare and are characterized by the expression of myomelanocytic markers. They are a complex family that includes angiomyolipomas, lymphangioleiomyomatoses and other soft tissue and visceral tumors. Due to the low prevalence of these tumors, the natural history is unclear; furthermore, a molecular classification integrating clinical, pathological and molecular parameters has not been described to date.

Inclusion criteria:Major patient (≥18 years)Minor patient (1 to 17 years old)PEComas diagnosed in France between 01/01/1990 and 15/09/2019 and reviewed by a sarcoma referral pathologistExclusion criteria :Opposition of the patient (or the holders of parental authority) to participate in the studyBiological tissue from a patient who does not meet all the inclusion criteriaClassic triphasic angiomyolipoma or lymphangioleiomyomatosisInability to provide informed information to the subjectPatient under court protectionPatient under guardianship or curatorship