Screening and Epidemiology of Hepato-pancreato-biliary Abnormalities in Fibrous Dysplasia of Bone /McCune Albright Syndrome: the TIM-DYS Study

Status: Recruiting
Location: See all (16) locations...
Intervention Type: Other
Study Type: Observational
SUMMARY

Fibrous dysplasia of bone /McCune Albright syndrome (FD/MAS) is a rare bone disease caused by somatic mutations in GNAS gene. This GNAS mutation predisposes to cancers, including breast cancer, thyroid cancer, chondrosarcoma and osteosarcoma, as well as biliary tract anomalies, liver-tumors or pancreatic tumors - IPMNs. Intraductal papillary and mucinous neoplasms of the pancreas (IPMN) are cystic intraepithelial ductal lesions developed at the expense of pancreatic ducts. They are pre-cancerous lesions, requiring monitoring and, in case of progression or malignant degeneration, surgical resection. Pancreatic MRI screening of patients with polyostotic FD and MAS is recommended. The aim of this study is to investigate the epidemiology and characteristics of these hepato-pancreato-biliary abnormalities (prevalence, age of onset, degeneration), based on magnetic resonance imaging (MRI) realized during the follow-up of patients with FD/MAS treated in a French FD expert center. A better understanding of these IPMNs and other digestive abnormalities will enable clinicians to improve the management and monitoring in this high-risk population.

Eligibility
Participation Requirements
Sex: All
Minimum Age: 18
Maximum Age: 100
Healthy Volunteers: f
View:

• Adults ≥18 years with polyostotic FD/MAS

• Followed up in the national FD reference center (Lyon) or in a FD French expert center: Angers, Bordeaux, Brest, Caen, Clermont-Ferrand, Lille, Marseille, Montpellier, Nice, Paris, Poitiers, Rennes, Saint-Etienne, Strasbourg.

• who had an hepato-bilio-pancreatic MRI during their follow-up, since January 2002

• No objection to data collection

Locations
Other Locations
France
CHU Angers
NOT_YET_RECRUITING
Angers
Groupe Hospitalier Pellegrin - Chu
NOT_YET_RECRUITING
Bordeaux
CHU de Brest - Hôpital de la Cavale Blanche
NOT_YET_RECRUITING
Brest
Chu de Caen Normandie
NOT_YET_RECRUITING
Caen
Chu de Clermont-Ferrand
NOT_YET_RECRUITING
Clermont-ferrand
Chu de Lille
NOT_YET_RECRUITING
Lille
Service Rhumatologie, pavillon F Hopital E. Herriot
NOT_YET_RECRUITING
Lyon
Assistance Publique - Hopitaux de Marseille (Ap-Hm
NOT_YET_RECRUITING
Marseille
Chu de Montpellier
NOT_YET_RECRUITING
Montpellier
CHU de Nice
NOT_YET_RECRUITING
Nice
Assistance Publique - Hopitaux de Paris (Ap-Hp)
NOT_YET_RECRUITING
Paris
CHU de Poitiers
NOT_YET_RECRUITING
Poitiers
Chu de Rennes
NOT_YET_RECRUITING
Rennes
Chu de Saint-Etienne
RECRUITING
Saint-étienne
Hopitaux Universitaires de Strasbourg
NOT_YET_RECRUITING
Strasbourg
Chu de Toulouse
NOT_YET_RECRUITING
Toulouse
Contact Information
Primary
Roland Chapurlat, MD, PhD
roland.chapurlat@chu-lyon.fr
+ 33 (0)4 72 11 74 82
Backup
Mélanie Legrand, MD
melanie.legrand@chu-lyon.fr
+ 33 (0)4 72 11 74 79
Time Frame
Start Date: 2024-01-19
Estimated Completion Date: 2025-12-12
Participants
Target number of participants: 545
Treatments
Adults with polyostotic fibrous dysplasia McCune Albright syndrome
A retrospective data collection and statistical analyses will be performed. Adults with polyostotic fibrous dysplasia McCune Albright syndrome, who underwent an hepato-bilio-pancreatic MRI since January 2002, during their follow-up
Sponsors
Leads: Hospices Civils de Lyon

This content was sourced from clinicaltrials.gov