Aggressive clinical course of large cell neuroendocrine carcinoma of ampulla of Vater

Abstract

A 78-year-old male patient with history of righ hemicolectomy due to adenocarcinoma was admitted by the complaint of epigastric discomfort. Laboratory data showed increase in liver biochemistries (aspartate aminotransferase (AST): 159 IU/L, alanine aminotransferase (ALT): 235 IU/L, alkaline phosphatase (ALP): 350 IU/L, gamma glutamyl transferase (GGT): 911 IU/L, total bilirubin: 1.55 mg/dl and direct bilirubin: 0.82 mg/dl). Endoscopic retrograde cholangiopancreatiography (ERCP) done after the gastrointestinal (GI) upper endoscopy was compatible with tumoral lesion, and biopsy confirmed 'neuroendocrine carcinoma'. Pylorus-preserving pancreaticoduodenectomy (PPPD) had been performed with R0 resection. Pathologic evaluation revealed 1.5 cm tumor of large cell neuroendocrine carcinoma (LCNEC). Five months later, biyopsy of suspicious lesions in liver had been documented as 'high grade neuroendocrine carcinoma metastasis'. He was referred to oncology for chemotherapy but unfortunatelly he had expired three months later. Large cell neuroendocrine carcinoma (LCNECs) of ampulla of Vater may have aggressive clinical course despite radical resections involving lymph node dissections. Small tumor size and lymph node negativity are not reliable factors for this tumor type.

Ampullary Large-Cell Neuroendocrine Carcinoma, a Diagnostic Challenge of a Rare Aggressive Neoplasm: A Case Report and Literature Review

“…Recent literature emphasizes more in the histopathological subtypes than the TNM staging system to predict the tumor’s aggressiveness and decide on a treatment plan [ 24 ].…”

“…In addition, the use of endoscopic ultrasound (EUS) might define the locoregional staging of ampullary NENs [ 44 , 45 ]. However, despite the advancements in imaging technology, preoperative diagnosis of NECs is still challenging [ 24 ].…”

“…Ampullary LCNECs in the literature are limited to 24 case reports before ours, as identified by our review to date ( Table 2 ) [ 11 , 18 , 24 , 26 , 56 , 57 , 58 , 59 , 60 , 61 , 62 , 63 , 64 , 65 , 66 , 67 , 68 ]. Only 14 cases were pure LCNECs [ 24 , 26 , 56 , 57 , 59 , 60 , 61 , 62 , 63 , 66 ], while the other 11 had other components [ 11 , 18 , 26 , 59 , 60 , 65 , 66 , 68 ]. Many of these patients had early recurrence with metastases, especially in the liver [ 24 , 56 , 58 , 60 , 61 , 62 , 63 , 64 , 65 , 66 , 68 ].…”

“…Only 14 cases were pure LCNECs [ 24 , 26 , 56 , 57 , 59 , 60 , 61 , 62 , 63 , 66 ], while the other 11 had other components [ 11 , 18 , 26 , 59 , 60 , 65 , 66 , 68 ]. Many of these patients had early recurrence with metastases, especially in the liver [ 24 , 56 , 58 , 60 , 61 , 62 , 63 , 64 , 65 , 66 , 68 ].…”