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Adam MP, Feldman J, Mirzaa GM, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2024.

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Figure 17.

Figure 17.

Recommended management for pancreatic involvement in individuals with fibrous dysplasia / McCune-Albright syndrome

EUS = endoscopic ultrasound; MRCP = magnetic resonance cholangiopancreatography; MRI = magnetic resonance imaging

1. Based on the 2012 International Consensus Guidelines for the management of intraductal papillary mucinous neoplasm (IPMNs) [Tanaka et al 2012]. Worrisome features include: pancreatitis; cyst ≥3 cm; thickened enhanced cystic walls; main pancreatic duct dilatation of 5-9 mm; non-enhanced mural nodules; and abrupt change in caliber of the main pancreatic duct with distal pancreatic atrophy, lymphadenopathy, an elevated serum level of carbohydrate antigen (CA) 19-9, and a rapid rate of cyst growth >5 mm within two years. High-risk stigmata on CT, MRI, or EUS include: obstructive jaundice in affected individuals with a cystic lesion in the pancreatic head; enhanced solid component (mural nodule) within the cyst (≥5 mm); and main pancreatic duct dilatation ≥10 mm.

2. The interval for repeat MRI/MRCP is not established [Gaujoux et al 2014, Wood et al 2017].

From: Fibrous Dysplasia / McCune-Albright Syndrome

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Szymczuk V, Florenzano P, de Castro LF, et al. Fibrous Dysplasia / McCune-Albright Syndrome. 2015 Feb 26 [Updated 2024 Feb 8]. In: Adam MP, Feldman J, Mirzaa GM, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2024. Figure 17. [Recommended management for pancreatic involvement...].
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