PEComas: Natural History and Prognostic Factors

National Multicenter Retrospective Study: PEComas: Natural History and Prognostic

Status

UNKNOWN

Phases

Unknown

Study type

Observational

Source

ClinicalTrials.gov

Registry ID

NCT03916575

Acronym

PEComas

Enrollment

Registered

2019-04-16

Start date

2019-04-09

Completion date

2020-04-30

Last updated

2019-05-01

For informational purposes only — not medical advice. Sourced from public registries and may not reflect the latest updates. Terms

Conditions

PEComas

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

Interventions

None listed

Study design

Observational model

CASE_ONLY

Time perspective

RETROSPECTIVE

Eligibility

Sex/Gender

ALL

Age

1 Years to No maximum

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

Design outcomes

Primary

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

Countries

France

Contacts

Primary ContactGabriel MALOUF, MD, PhD

gabriel.malouf@chru-strasbourg.fr03 88 11 62 22

Outcome results