PEComas: Natural History and Prognostic Factors

• Conditions: PEComas

• Registration Number: NCT03916575
• Lead Sponsor: University Hospital, Strasbourg, France

Brief Summary

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

Detailed Description

Not available

Study Timeline

• Status Verified: 2019-04
• Study First Submitted: 2019-04-03
• Study Start: 2019-04-09
• Study First Submitted QC: 2019-04-15
• Study First Posted: 2019-04-16
• Last Update Submitted: 2019-04-29
• Last Update Posted: 2019-05-01
• Primary Completion: 2020-04
• Study Completion: 2020-04

Recruitment & Eligibility

• Status: UNKNOWN
• Sex: All
• Target Recruitment: 60

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

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
Study of the prognostic factors associated with the risk of relapse	The period from January 1st, 1990 to December 31, 2018 will be examined

Trial Locations

Locations (1)

Service D'Hematologie Et D'Oncologie; 🇫🇷 Strasbourg, France