PEComas: Natural History and Prognostic Factors

University Hospital, Strasbourg, France

Status: Unknown

Conditions: PEComas

Study type: Observational

Funder types: Other

Identifiers

NCT03916575

7337

Details and patient eligibility

About

Perivascular epithelioid cell tumors are rare and characterized by the expression of myomelanocytic markers. They belong to a complex tumor family that includes angiomyolipomas, lymphangioleiomyomatosis and other soft-tissue tumors. Given their rarity, the natural history of pecomas is not yet understood, and a comprehensive classification that integrates clinical, pathological and molecular features has not been achieved as of today.

This study is a national multicenter retrospective study to better understand the natural history of PEComas, excluding lymphangioleiomyomatosis and classic triphasic angiomyolipomas.

The primary purpose is the identification of prognostic markers impacting the relapse. Secondaries purposes are to identify prognostic markers impacting the overall survival and to have a better understanding of natural history

Enrollment: 60 estimated patients

Sex: All

Ages: 1+ year old

Volunteers: No Healthy Volunteers

Inclusion criteria

• Major patient (≥18 years old)
• Minor patient (1 to 17 years old)
• PEComas diagnosed in France between 1990 and 2018 and read back by a pathologist referent of sarcomas
• Patient having agreed to the use of his medical data for the purpose of this research.
• Parental authorities having given their agreement for the reuse of their child's medical data for the purpose of this research.

Exclusion criteria

• Opposition of the patient (or his legal representative) to the reuse of his data for the purposes of this research
• Impossibility of giving the subject clear information
• Patient under safeguard of justice
• Patient under guardianship
• Lymphangioleiomyomatosis
• Classic triphasic angiomyolipoma