National Clinical-biological Prospective Cohort of Incident Cases of Aggressive Fibromatosis (ALTITUDES)
- Conditions
- Aggressive Fibromatosis
- Interventions
- Procedure: biopsyOther: biobank constitutionProcedure: ColoscopyProcedure: Blood sampling (facultative)Other: Pain evaluationProcedure: Tumor biobank realization
- Registration Number
- NCT02867033
- Lead Sponsor
- Centre Oscar Lambret
- Brief Summary
The purpose of this study is to constitute the French largest Aggressive fibromatosis cohort.
- Detailed 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.
Recruitment & Eligibility
- Status
- ACTIVE_NOT_RECRUITING
- Sex
- All
- Target Recruitment
- 628
- 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)
- Administrative or legal measure of liberty privation
- Patient not able to give consent or unwilling to provide consent
Study & Design
- Study Type
- INTERVENTIONAL
- Study Design
- SINGLE_GROUP
- Arm && Interventions
Group Intervention Description Study procedure Blood sampling (facultative) Tumor biobank realization (biopsy...) and biobank constitution. coloscopy associated with colonic chromoscopy. Blood sampling (facultative). Pain evaluation Study procedure biopsy Tumor biobank realization (biopsy...) and biobank constitution. coloscopy associated with colonic chromoscopy. Blood sampling (facultative). Pain evaluation Study procedure Tumor biobank realization Tumor biobank realization (biopsy...) and biobank constitution. coloscopy associated with colonic chromoscopy. Blood sampling (facultative). Pain evaluation Study procedure Pain evaluation Tumor biobank realization (biopsy...) and biobank constitution. coloscopy associated with colonic chromoscopy. Blood sampling (facultative). Pain evaluation Study procedure biobank constitution Tumor biobank realization (biopsy...) and biobank constitution. coloscopy associated with colonic chromoscopy. Blood sampling (facultative). Pain evaluation Study procedure Coloscopy Tumor biobank realization (biopsy...) and biobank constitution. coloscopy associated with colonic chromoscopy. Blood sampling (facultative). Pain evaluation
- Primary Outcome Measures
Name Time Method Incident cases of aggressive fibromatosis, diagnosed after 01/01/2016 in France through study completion, an average of 5 years To constitute, at a national level, the largest cohort of incident cases of desmoid tumours
- Secondary Outcome Measures
Name Time Method Number of Aggressive Fibromatosis associated with familial adenomatous polyposis through study completion, an average of 5 years To describe and analyse the link between Aggressive Fibromatosis and familial adenomatous polyposis
Percentage of CTNNB1 mutation in non-selected cases of Aggressive Fibromatosis through study completion, an average of 5 years To describe the proportion of AF cases characterized by CTNNB1 somatic mutation
Hospital Anxiety and Depression Scale (HADS) at baseline, one year To describe the psychological impact of the disease at diagnosis and a year after diagnosis. And to compare changes between the time of diagnosis and one year after the treatments used.
Impact of pregnancy and hormonal exposure Through study completion, an average of 5 years To study the impact of pregnancy and hormonal exposure on the evolution of the disease according to recurrence/progression rates
Management of AF through study completion, an average of 5 years Description of the management of AF. Study of prognosis factor for progressive disease and death. Study of tumor response to treatments (Best response and progression-free survival) according to RECIST 1.1.
Quality of Life Questionnaire (QLQC30) at baseline, one year To describe the consequences of the disease on the quality of life at diagnosis and a year after diagnosis. And to compare changes between the time of diagnosis and one year after the treatments used.
Incidence of polyposis and colorectal cancer Through study completion, an average of 5 years Rate of polyposis and colorectal cancer in the AF population
Trial Locations
- Locations (38)
Institut de Cancérologie de l'Ouest - Paul Papin
🇫🇷Angers, France
CHU Angers
🇫🇷Angers, France
CHU de Besançon
🇫🇷Besancon, France
Hôpital des Enfants
🇫🇷Bordeaux, France
Institut Bergonié
🇫🇷Bordeaux, France
CHU de Caen-Côte de Nacre
🇫🇷Caen, France
Centre Jean Perrin
🇫🇷Clermont Ferrand, France
Centre Georges François Leclerc
🇫🇷Dijon, France
CHU de Grenoble- Hôpital Couple Enfant
🇫🇷Grenoble, France
Centre Léon Bérard
🇫🇷Lyon, France
Institut Paoli Calmettes
🇫🇷Marseille, France
Hôpital la Timone Enfants Service Oncologie Pédiatrique
🇫🇷Marseille, France
Hôpital la Timone Service Oncologie Médicale
🇫🇷Marseille, France
Hôpital Mère Enfant - CHU Nantes
🇫🇷Nantes, France
Hôpital Archet 2
🇫🇷Nice, France
Institut Curie Département Oncologie Médicale
🇫🇷Paris, France
Institut Curie Département Oncologie Pédiatrique
🇫🇷Paris, France
Hôpital Saint Antoine
🇫🇷Paris, France
Hôpîtal d'Enfants Armand Trousseau
🇫🇷Paris, France
Hôpital Cochin
🇫🇷Paris, France
CHU de Reims
🇫🇷Reims, France
Centre Henri Becquerel
🇫🇷Rouen, France
CHU de Rennes- Hôpital Sud
🇫🇷Rennes, France
Institut de Cancérologie de l'Ouest - Site René Gauducheau
🇫🇷Saint Herblain, France
Institut Curie-Hôpital René Huguenin
🇫🇷Saint Cloud, France
Institut de Cancérologie Lucien Neuwirth
🇫🇷Saint Priest En Jarez, France
Institut Claudius Régaud
🇫🇷Toulouse, France
CHU Saint-Étienne - Hôpital Nord
🇫🇷Saint-Étienne, France
Hôpitaux Universitaires de Strasbourg
🇫🇷Strasbourg, France
CHU Tours - Clocheville
🇫🇷Tours, France
Institut de Cancérologie de Lorraine
🇫🇷Vandoeuvre Les Nancy, France
Institut Gustave Roussy
🇫🇷Villejuif, France
Hôpital d'Enfants- CHU Nancy
🇫🇷Vandoeuvre Les Nancy, France
Hôpital Saint Louis
🇫🇷Paris, France
Centre François Baclesse
🇫🇷Caen, France
Centre Oscar Lambret
🇫🇷Lille, France
ICM Val d'Aurelle
🇫🇷Montpellier, France
CHU Toulouse - Hôpital des Enfants
🇫🇷Toulouse, France