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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.


Clinical Trial Description

n/a


Study Design


Related Conditions & MeSH terms


NCT number NCT06177327
Study type Observational
Source Hospices Civils de Lyon
Contact Roland Chapurlat, MD, PhD
Phone + 33 (0)4 72 11 74 82
Email roland.chapurlat@chu-lyon.fr
Status Recruiting
Phase
Start date January 19, 2024
Completion date December 12, 2025

See also
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Completed NCT05422833 - Effectiveness of Medical Management of Fibrous Dysplasia of Bone.
Recruiting NCT03838991 - Epigenetic Regulation in Fibrous Dysplasia of Bone: mirDYS Study. N/A
Active, not recruiting NCT05509595 - Burosumab for Fibroblast Growth Factor-23 Mediated Hypophosphatemia in Fibrous Dysplasia Phase 2