Not provided
| ID | Type | Description | Link |
|---|---|---|---|
| 2015-A01655-44 | Other Identifier | ANSM |
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
| Name | Class |
|---|---|
| Ligue contre le cancer, France | OTHER |
| Institut Curie | OTHER |
| Hôpital de la Timone | OTHER |
| SOS Desmoid e.V. |
Not provided
Not provided
Not provided
Not provided
The purpose of this study is to constitute the French largest Aggressive fibromatosis cohort.
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.
Not provided
Not provided
Not provided
Not provided
| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Study procedure | Other | Tumor biobank realization (biopsy...) and biobank constitution. coloscopy associated with colonic chromoscopy. Blood sampling (facultative). Pain evaluation |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| biopsy | Procedure | pre-therapeutic or post-therapeutic biopsy or resected tissues |
| |
| Measure | Description | Time Frame |
|---|---|---|
| Incident cases of aggressive fibromatosis, diagnosed after 01/01/2016 in France | To constitute, at a national level, the largest cohort of incident cases of desmoid tumours | through study completion, an average of 5 years |
| Measure | Description | Time Frame |
|---|---|---|
| Number of Aggressive Fibromatosis associated with familial adenomatous polyposis | To describe and analyse the link between Aggressive Fibromatosis and familial adenomatous polyposis | through study completion, an average of 5 years |
| Percentage of CTNNB1 mutation in non-selected cases of Aggressive Fibromatosis |
| Measure | Description | Time Frame |
|---|---|---|
| Mutation rate of APC | To determine the mutation rate of APC at constitutional and somatic levels | through study completion, an average of 5 years |
| Mutation rate of CTNNB1 | To determine the mutation rate of CTNNB1 at constitutional and somatic levels |
Inclusion Criteria:
Exclusion Criteria:
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
| Name | Affiliation | Role |
|---|---|---|
| Nicolas PENEL, PhD | Centre Oscar Lambret | Principal Investigator |
| Sébastien SALAS, PhD | Hopital Timone adultes | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Institut de Cancérologie de l'Ouest - Paul Papin | Angers | 49055 | France | |||
| CHU Angers |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 35294527 | Derived | Penel N, Bonvalot S, Bimbai AM, Meurgey A, Le Loarer F, Salas S, Piperno-Neumann S, Chevreau C, Boudou-Rouquette P, Dubray-Longeras P, Kurtz JE, Guillemet C, Bompas E, Italiano A, Le Cesne A, Orbach D, Thery J, Le Deley MC, Blay JY, Mir O. Lack of Prognostic Value of CTNNB1 Mutation Profile in Desmoid-Type Fibromatosis. Clin Cancer Res. 2022 Sep 15;28(18):4105-4111. doi: 10.1158/1078-0432.CCR-21-4235. |
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
| UNKNOWN |
| APICIL funding | UNKNOWN |
| GIRCI NO | UNKNOWN |
| UNICANCER | OTHER |
| Groupement Interrégional de Recherche Clinique et d'Innovation | OTHER |
Not provided
Not provided
Not provided
Not provided
Not provided
| biobank constitution |
| Other |
Constitution of a biobank with pre-therapeutic or post-therapeutic biopsy or resected tissues |
|
| Coloscopy | Procedure | For adult patients, a coloscopy with chromoscopy of ascending and sigmoid colon will be performed |
|
| Blood sampling (facultative) | Procedure | Blood sample can be collected at diagnostic or after medically significant events (progressive disease, local or systemic treatment, pregnancy...) |
|
| Pain evaluation | Other | Pain evaluation (EVA scale), anxiety (HADS questionnaire), quality of life questionnaire (EORTC-QLQ-C30) |
|
| Tumor biobank realization | Procedure | Realization of a tumor biobank is part of classical procedure of participating centers |
|
To describe the proportion of AF cases characterized by CTNNB1 somatic mutation |
| through study completion, an average of 5 years |
| Management of AF | 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. | through study completion, an average of 5 years |
| Hospital Anxiety and Depression Scale (HADS) | 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. | at baseline, one year |
| Quality of Life Questionnaire (QLQC30) | 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. | at baseline, one year |
| Impact of pregnancy and hormonal exposure | To study the impact of pregnancy and hormonal exposure on the evolution of the disease according to recurrence/progression rates | Through study completion, an average of 5 years |
| Incidence of polyposis and colorectal cancer | Rate of polyposis and colorectal cancer in the AF population | Through study completion, an average of 5 years |
| Pain | Pain according to Numeric Pain Scale, assessed at baseline and then at each annual follow-up. | Through study completion, an average of 5 years |
| through study completion, an average of 5 years |
| Correlation between APC and CTNNB1 mutations rates | To demonstrate that APC and CTNNB1 mutations are two mutually exclusive molecular alterations | through study completion, an average of 5 years |
| APC mutation rate | To correlate mutational somatic and constitutional rate of APC gene | through study completion, an average of 5 years |
| Occurrence of other mutations | To search other molecular anomalies for patients without APC or CTNNB1 mutations (10 % of cases) | through study completion, an average of 5 years |
| Cell free (circulating) nucleic acid extraction technics | To determine sensibility and specificity of cell free (circulating) nucleic acid extraction techniques | through study completion, an average of 5 years |
| AF outcome | To describe patient outcomes and identify prognostic factors | through study completion, an average of 5 years |
| Treatment response | To search for factors involved in response treatment prediction | through study completion, an average of 5 years |
| Angers |
| 49933 |
| France |
| CHU de Besançon | Besançon | 25030 | France |
| Hôpital des Enfants | Bordeaux | 33076 | France |
| Institut Bergonié | Bordeaux | 33076 | France |
| CHU de Caen-Côte de Nacre | Caen | 14033 | France |
| Centre François Baclesse | Caen | 14076 | France |
| Centre Jean Perrin | Clermont-Ferrand | 63011 | France |
| Centre Georges François Leclerc | Dijon | 21079 | France |
| CHU de Grenoble- Hôpital Couple Enfant | Grenoble | 38043 | France |
| Centre Oscar Lambret | Lille | 59020 | France |
| Centre Léon Bérard | Lyon | 69373 | France |
| Hôpital la Timone Enfants Service Oncologie Pédiatrique | Marseille | 13005 | France |
| Hôpital la Timone Service Oncologie Médicale | Marseille | 13005 | France |
| Institut Paoli Calmettes | Marseille | 13273 | France |
| ICM Val d'Aurelle | Montpellier | 34298 | France |
| Hôpital Mère Enfant - CHU Nantes | Nantes | 44093 | France |
| Hôpital Archet 2 | Nice | 06202 | France |
| Institut Curie Département Oncologie Médicale | Paris | 75005 | France |
| Institut Curie Département Oncologie Pédiatrique | Paris | 75005 | France |
| Hôpital Saint Louis | Paris | 75010 | France |
| Hôpîtal d'Enfants Armand Trousseau | Paris | 75012 | France |
| Hôpital Cochin | Paris | 75014 | France |
| Hôpital Saint Antoine | Paris | France |
| CHU de Reims | Reims | 51100 | France |
| CHU de Rennes- Hôpital Sud | Rennes | 35023 | France |
| Centre Henri Becquerel | Rouen | 76038 | France |
| Institut Curie-Hôpital René Huguenin | Saint-Cloud | 92210 | France |
| CHU Saint-Étienne - Hôpital Nord | Saint-Etienne | 42055 | France |
| Hôpital Privé de la Loire | Saint-Etienne | 42100 | France |
| Institut de Cancérologie de l'Ouest - Site René Gauducheau | Saint-Herblain | 44805 | France |
| Institut de Cancérologie et d'Hématologie Universitaire de Saint Etienne | Saint-Priest-en-Jarez | France |
| Hôpitaux Universitaires de Strasbourg | Strasbourg | 67098 | France |
| CHU Toulouse - Hôpital des Enfants | Toulouse | 31059 | France |
| Institut Claudius Régaud | Toulouse | 31059 | France |
| CHU Tours - Clocheville | Tours | 37044 | France |
| Hôpital d'Enfants- CHU Nancy | Vandœuvre-lès-Nancy | 54511 | France |
| Institut de Cancérologie de Lorraine | Vandœuvre-lès-Nancy | 54519 | France |
| Institut Gustave Roussy | Villejuif | 94800 | France |
| ID | Term |
|---|---|
| D018222 | Desmoid Tumors |
| ID | Term |
|---|---|
| D005350 | Fibroma |
| D018218 | Neoplasms, Fibrous Tissue |
| D009372 | Neoplasms, Connective Tissue |
| D018204 | Neoplasms, Connective and Soft Tissue |
| D009370 | Neoplasms by Histologic Type |
| D009369 | Neoplasms |
Not provided
Not provided
| ID | Term |
|---|---|
| D001706 | Biopsy |
| D001800 | Blood Specimen Collection |
| ID | Term |
|---|---|
| D003581 | Cytodiagnosis |
| D003584 | Cytological Techniques |
| D019411 | Clinical Laboratory Techniques |
| D019937 | Diagnostic Techniques and Procedures |
| D003933 | Diagnosis |
| D013048 | Specimen Handling |
| D003949 | Diagnostic Techniques, Surgical |
| D013514 | Surgical Procedures, Operative |
| D008919 | Investigative Techniques |
| D011677 | Punctures |
Not provided
Not provided