title: Endocrine carcinoma of the major papilla: report of two cases and review of the literature.
abstract: To date, about 100 cases of ampullary NET are reported in International literature. These tumors can cause symptoms mainly secondary to their periampullary location. Up to 25% of patients have von Recklinghausen's disease. Carcinoid syndrome is uncommon, unless hepatic metastasis is present. Determination of histopathology is of utmost importance and involves specific immunohistochemical staining. The published data indicate that these tumors, metastasize in approximately half of cases irrespective of primary tumor size. Therefore, radical excision in the form of pancreaticoduodenectomy is recommended regardless of tumor size. Local excision should be confined to patients unable to tolerate more extensive surgery. We here report two case of ampullary neuroendocrine tumors presenting as melena and painless jaundice respectively in a 51-year old man and in a 54-year old man and review the relevant literature, giving special attention to the morphologic features, clinical characteristics, and treatment modalities associated with this disease process.
authors: Department of Surgery and Advanced Technologies, University of Naples Federico II, Via Pansini, Naples, Italy. giovanni.depalma@unina.it
authors: Giovanni D
authors: GD
authors: De Palma
authors: Stefania
authors: S
authors: Masone
authors: Saverio
authors: S
authors: Siciliano
authors: Francesco
authors: F
authors: Maione
authors: Jessica
authors: J
authors: Falleti
authors: Gelsomina
authors: G
authors: Mansueto
authors: Gaetano
authors: G
authors: De Rosa
authors: Giovanni
authors: G
authors: Persico
mesh_terms: D014670:Ampulla of Vater / Q000473:pathology*; D018278:Carcinoma, Neuroendocrine / Q000473:pathology*; D003138:Common Bile Duct Neoplasms / Q000473:pathology*; D018450:Disease Progression; D006801:Humans; D008297:Male; D008875:Middle Aged