National Clinical-biological Prospective Cohort of Incident Cases of Aggressive Fibromatosis (ALTITUDES)

Centre Oscar Lambret

Status: Active, not recruiting

Conditions: Aggressive Fibromatosis

Treatments: Procedure: Blood sampling (facultative); Other: Pain evaluation; Procedure: biopsy; Other: biobank constitution; Procedure: Tumor biobank realization; Procedure: Coloscopy

Study type: Interventional

Funder types: Other

Identifiers

NCT02867033

ALTITUDES-1508

Details and patient eligibility

About

The purpose of this study is to constitute the French largest Aggressive fibromatosis cohort.

Full description

Aggressive fibromatosis (AF) is a rare non-metastasizing connective tissue tumor (< 300 cases/year in France), associated with high risk of local relapse, functional impairment and pain. AF can occur at any age, but most commonly between 25 and 40 with a significant female predominance. AF is most frequently (about 85%) sporadic and then associated with a somatic mutation of the CTNNB1 gene. AF is associated with heredity condition, as complication of familial adenomatous polyposis (with germinal mutation of Adenomatous polyposis coli (APC) gene). Most of AF arises on lims or abdominal wall. Nevertheless, some particular locations are life-threatening (mesenteric or cervical locations). The natural course of AF is unpredictable. One third of tumors are spontaneously stable. One third of tumor spontaneously decreases. One third of tumor is progressive, with a non-linear tumor growth dynamic. As the consequence the decision making for starting curative intent treatment is difficult, since some treatment could be mutilating (large en bloc surgery) or associated with late and severe complications (radiotherapy) and since these treatments could fail to control this benign tumor. Therapeutic options are: wait-and-see policy, surgery (sometimes mutilating), radiotherapy or systemic treatment (non-steroidal anti-inflammatory drugs, hormonotherapy, imatinib, chemotherapy). Level of evidence associated these options is very low, based on retrospective studies and rare non-randomized phase II clinical trials.

Regarding these uncertainties, physicians can hardly answer to patient questions.

Prospective data provided by a large multi-center cohort is needed. The objective of the present study is to create a large cohort of incident cases of AF associated with tumor bank and collection of blood samples.

Enrollment: 628 patients

Sex: All

Volunteers: No Healthy Volunteers

Inclusion criteria

• Incident Case of aggressive fibromatosis in France, diagnosed after 01/01/2016
• Confirmed diagnosis by the French anatomopathological diagnosis network (including search for mutation of the β-Catenin Gene, CTNNB1)
• Affiliation to the National Health System
• Informed consent signed (both parents signature for non adult patients)

Exclusion criteria

• Administrative or legal measure of liberty privation
• Patient not able to give consent or unwilling to provide consent

Trial design

Primary purpose: Other

Allocation: N/A

Interventional model: Single Group Assignment

Masking: None (Open label)

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628 participants in 1 patient group

Study procedure

Other group

Description:

Tumor biobank realization (biopsy...) and biobank constitution. coloscopy associated with colonic chromoscopy. Blood sampling (facultative). Pain evaluation

Treatment:

Procedure: Coloscopy

Procedure: Tumor biobank realization

Other: biobank constitution

Other: Pain evaluation

Procedure: biopsy

Procedure: Blood sampling (facultative)