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

Study Status: Recruiting
Recruit Status: Recruiting
Study Type: Interventional

Official Title: National Clinical-biological Prospective Cohort of Incident Cases of Aggressive Fibromatosis

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

Detailed Summary:

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.


Sponsor: Centre Oscar Lambret

Current Primary Outcome: Incident cases of aggressive fibromatosis [ Time Frame: through study completion, an average of 5 years ]

To constitute, at a national level, the largest cohort of incident cases of desmoid tumours


Original Primary Outcome: Same as current

Current Secondary Outcome:

  • Number of Aggressive Fibromatosis associated with familial adenomatous polyposis [ Time Frame: 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 [ Time Frame: through study completion, an average of 5 years ]
    To describe the proportion of AF cases characterized by CTNNB1 somatic mutation
  • Treatment used for management of AF [ Time Frame: through study completion, an average of 5 years ]
    To describe the impact of different therapeutic strategies on AF recurrence and progression free survival, in homogenous risk level sub-groups of patients
  • Hospital Anxiety and Depression Scale (HADS) [ Time Frame: 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.
  • Quality of Life Questionnaire (QLQC30) [ Time Frame: 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.


Original Secondary Outcome:

  • Number of Aggressive Fibromatosis associated with familial adenomatous polyposis [ Time Frame: 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 [ Time Frame: through study completion, an average of 5 years ]
    To describe the proportion of AF cases characterized by CTNNB1 somatic mutation
  • Treatment used for management of AF [ Time Frame: through study completion, an average of 5 years ]
    To describe the impact of different therapeutic strategies on AF recurrence and progression free survival, in homogenous risk level sub-groups of patients
  • Quality of life questionnaires [ Time Frame: at baseline, one year ]
    To describe the impact of AF management/efficacy on psychological, emotional and social parameters, and quality of life. And to compare changes between the time of diagnosis and one year after the treatments used.


Information By: Centre Oscar Lambret

Dates:
Date Received: July 15, 2016
Date Started: March 2016
Date Completion: December 2021
Last Updated: August 16, 2016
Last Verified: August 2016