Other Neoplasia

Several other malignancies are known to have an increased lifetime risk in FAP patients compared to the general population. These include pancreatic cancer, cholangiocarcinoma, hepatoblastoma, and possibly adrenal [145,146]. Both adenomatous changes and cancer have been reported in the gallbladder, bile duct and pancreas. The relative risk of pancreatic adenocarcinoma in the John's Hopkins FAP registry was 4.4 [139], whereas the absolute risk of pancreatic cancer is 2% or less.

Hepatoblastoma affects 0.75-1.6% of children with FAP, exhibits male predominance, and occurs mainly in the first 5 years of life and, therefore, may precede the development of FAP by many years [147, 148]. The risk in children with FAP is 800-fold higher than in the general population.

Adrenal adenomas have been shown to occur more frequently in FAP. Several cases of functioning adenomas and adrenal carcinomas have also been reported in FAP patients, but the association is uncertain [149, 150]. Most adrenal lesions are found incidentally on imaging studies done for other reasons and should be managed similarly to those in the general population.

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