XXXX in the Gastrointestinal Tract.

@entity1 @entity1127 @entity548 @entity5 @entity2838 @entity1050 @entity272 @entity5277 @entity1412
BACKGROUND: Digestive @entity1127 are a rare entity. They may be sporadic (solitary @entity1127 ) or reveal genetic predisposition like @entity2838 , @entity1412 or @entity1050 . SUMMARY: Diagnosis is based on personal and family history and on clinical data including physical signs (in particular dermatological), endoscopic findings (the number of @entity1127 ) and histological features of the @entity1127 . The risk of complications and of digestive and extra-digestive cancers may be high, especially in case of genetic predisposition syndromes, and requires multidisciplinary management of the @entity1 (oncogenetic counseling, gastroenterologist, pathologist, dermatologist, gynecologist and endocrinologist). Endoscopic evaluation is very helpful to establish the current situation, to perform polypectomy and to allow for good histological examination of the @entity1127 , whose degeneration has been exceptionally described. The recent development of new molecular techniques (next-generation DNA sequencing) allows for rapid multiple gene sequencing and facilitates diagnosis. KEY MESSAGE: Discovery of a @entity1127 requires a rigorous work-up which should be performed by a multidisciplinary team, including a genetic oncologist, experienced in this pathology. PRACTICAL IMPLICATIONS: The diagnostic procedure in @entity1127 should be based on the number of @entity1127 identified during endoscopy (solitary versus multiple) and on their histological characteristics. The clinical examination must search for mucosal and @entity272 . If a @entity1412 is identified, oncogenetic consultation is necessary in order to define screening modalities for family members, aiming at preventing @entity5 development. Endoscopic resection (polypectomy) of the lesions may prevent complications like @entity548 and degeneration and also diminish the risk of surgery and subsequent @entity5277 .
