Hepato-pancreato-biliary Abnormalities in Fibrous Dysplasia of Bone/McCune Albright Syndrome
- Conditions
- Fibrous Dysplasia of Bone
- Interventions
- Other: Screening for hepato-bilio-pancreatic abnormalities in Fibrous dysplasia of bone /McCune Albright syndrome , description and characterization of the lesions.
- Registration Number
- NCT06177327
- Lead Sponsor
- Hospices Civils de Lyon
- Brief 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.
- Detailed Description
Not available
Recruitment & Eligibility
- Status
- RECRUITING
- Sex
- All
- Target Recruitment
- 545
- 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
-Opposition to data collection
Study & Design
- Study Type
- OBSERVATIONAL
- Study Design
- Not specified
- Arm && Interventions
Group Intervention Description Adults with polyostotic fibrous dysplasia McCune Albright syndrome Screening for hepato-bilio-pancreatic abnormalities in Fibrous dysplasia of bone /McCune Albright syndrome , description and characterization of the lesions. 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
- Primary Outcome Measures
Name Time Method Describe hepato-bilio-pancreatic abnormalities in FD/MAS and estimate the prevalence of IPMN. hepato-bilio-pancreatic MRI performed from January 2002 until December 2024. Hepato-bilio-pancreatic abnormalities (size, number, complications) will be described based on hepato-bilio-pancreatic MRI.
- Secondary Outcome Measures
Name Time Method
Trial Locations
- Locations (16)
CHU Angers
🇫🇷Angers, France
Groupe Hospitalier Pellegrin - Chu
🇫🇷Bordeaux, France
CHU de Brest - Hôpital de la Cavale Blanche
🇫🇷Brest, France
Chu de Caen Normandie
🇫🇷Caen, France
Chu de Clermont-Ferrand
🇫🇷Clermont-Ferrand, France
Chu de Lille
🇫🇷Lille, France
Assistance Publique - Hopitaux de Marseille (Ap-Hm
🇫🇷Marseille, France
Service Rhumatologie, pavillon F Hopital E. Herriot
🇫🇷Lyon, France
Chu de Montpellier
🇫🇷Montpellier, France
CHU de Nice
🇫🇷Nice, France
CHU de Poitiers
🇫🇷Poitiers, France
Assistance Publique - Hopitaux de Paris (Ap-Hp)
🇫🇷Paris, France
Chu de Toulouse
🇫🇷Toulouse, France
Chu de Rennes
🇫🇷Rennes, France
Chu de Saint-Etienne
🇫🇷Saint-Étienne, France
Hopitaux Universitaires de Strasbourg
🇫🇷Strasbourg, France