Hepato-pancreato-biliary Abnormalities in Fibrous Dysplasia of Bone/McCune Albright Syndrome

Fibrous Dysplasia of Bone Clinical Trial

— TIM-DYS  

Official title:

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

Clinical Trial Details — Status: Recruiting

Administrative data

• NCT number: NCT06177327
• Other study ID #: 23-5288
• Status: Recruiting
• Start date: January 19, 2024
• Est. completion date: December 12, 2025

Study information

• Verified date: February 2024
• Source: Hospices Civils de Lyon
• Contact: Roland Chapurlat, MD, PhD
• Phone: + 33 (0)4 72 11 74 82
• Email: roland.chapurlat[@]chu-lyon.fr
• Is FDA regulated: No
• Study type: Observational

Clinical Trial 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.

Recruitment information / eligibility

• Status: Recruiting
• Enrollment: 545
• Est. completion date: December 12, 2025
• Est. primary completion date: November 30, 2024
• Accepts healthy volunteers: No
• Gender: All
• Age group: 18 Years to 100 Years

Eligibility

Inclusion Criteria:

• 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

Exclusion Criteria:

-Opposition to data collection

Related Conditions & MeSH terms

• Fibrous Dysplasia of Bone
• Hyperplasia

Intervention

Other:
• Screening for hepato-bilio-pancreatic abnormalities in Fibrous dysplasia of bone /McCune Albright syndrome , description and characterization of the lesions.
Data collection from hepato-bilio-pancreatic Magnetic Resonance Imaging realized during the follow up of patients with Fibrous dysplasia of bone /McCune Albright syndrome

Locations

Country	Name	City	State
France	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	Service Rhumatologie, pavillon F Hopital E. Herriot	Lyon
France	Assistance Publique - Hopitaux de Marseille (Ap-Hm	Marseille
France	Chu de Montpellier	Montpellier
France	CHU de Nice	Nice
France	Assistance Publique - Hopitaux de Paris (Ap-Hp)	Paris
France	CHU de Poitiers	Poitiers
France	Chu de Rennes	Rennes
France	Chu de Saint-Etienne	Saint-Étienne
France	Hopitaux Universitaires de Strasbourg	Strasbourg
France	Chu de Toulouse	Toulouse

Sponsors (1)

Lead Sponsor	Collaborator
Hospices Civils de Lyon

Country where clinical trial is conducted

France, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	Describe hepato-bilio-pancreatic abnormalities in FD/MAS and estimate the prevalence of IPMN.	Hepato-bilio-pancreatic abnormalities (size, number, complications) will be described based on hepato-bilio-pancreatic MRI.	hepato-bilio-pancreatic MRI performed from January 2002 until December 2024.