Familial Adenomatous Polyposis (FAP) is a systemic disorder that encompasses multiple tumors and several syndromes.
Syndromes associated with FAP:
(1) Turcot syndrome (with any brain tumor, see below)
(2) Gardner syndrome (with epidermoid cysts, fibromas, desmoid tumors and osteomas)
(3) Zanca’s syndrome (with multiple cartilaginous exostoses)
Extracolonic gastrointestinal tumors:
(1) gastric fundic gland polyps
(2) gastric adenomas
(3) gastric adenocarcinoma
(4) duodenal adenomas
(5) periampullary adenocarcinomas
(6) adenomas in jejunum and ileum
(7) adenocarciinomas in jejunum and ileum
(8) carcinoid tumors
Additional malignancies:
(1) hepatic tumors: hepatoblastoma, hepatocellular carcinoma, cholangiocarcinoma
(2) breast cancer
(3) embryonal carcinoma of testis
(4) cancer of the urinary bladder
(5) thyroid adenomas
(6) papillary carcinoma of the thyroid
(7) pancreatic adenomas
(8) pancreatic adenocarcinomas
(9) adrenal adenomas
(10) adrenal carcinomas including pheochromocytoma
(11) rhabdomyosarcoma
(12) liposarcoma
(13) brain tumors (medulloblastoma, glioblastoma, astrocytoma, spongioblastoma, lymphoma, glioma, ependymoma, craniopharyngioma, cavernous hemangioma, arteriovenous malformation)
(14) endometrial carcinoma
(15) cartilaginous exostosis
(16) possibly malignant melanoma
Additional lesions:
(1) desmoid tumors
(2) multiple lymphoid polyps
(3) pituitary adenomas
(4) nasopharyngeal angiofibromas
(5) congenital hyperplastia of retinal pigment epithelium (CHRPE)
(6) dental abnormalities (supernumerary teet, impacted teeth)
(7) osteomas of mandible and/or skull
(8) epidermoid cysts
(9) fibromas
(10) lipomas
Specialty: Gastroenterology