Long-term complete remission of a patient with high grade neuroendocrine carcinoma of ampulla of Vater

Abstract

We describe a case report of a 53-year-old man with a 5-months history of progressive jaundice and upper abdominal pain. The patient was further evaluated and finally diagnosed with a high-grade ampullary neuroendocrine tumour (based on endoscopic-guided biopsy). Thereafter, he underwent pancreatoduodenectomy and adjuvant platinum-based chemotherapy. This extremely rare case presents his long-lasting disease-free survival compared with similar cases; this case report exemplifies a new, potentially efficient method for treating high-grade papillary neuroendocrine tumour and may pave the way for further clinical trials utilising this blueprint in the treatment of related conditions.

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

“…Conventional radiology can detect a highly vascularized mass and dilated main pancreatic duct (MPD) and common bile duct (CBD) [ 42 ]. Since abdominal computed tomography (CT) scan cannot usually identify small ampullary tumors, additional methods, such as endoscopic resonance cholangiopancreatography (ERCP) and magnetic resonance cholangiopancreatography (MRCP), might also aid the diagnosis [ 43 ]. In addition, the use of endoscopic ultrasound (EUS) might define the locoregional staging of ampullary NENs [ 44 , 45 ].…”

“…The use of somatostatin receptors is not proven; however, positive imaging could indicate a well-differentiated tumor [ 49 , 50 ]. Since NETs of the Vater’s ampulla and their metastases often express somatostatin receptors, somatostatin receptor scan (SRS) can detect them with an 86% sensitivity [ 8 , 43 ]. However, although somatostatin receptors are often expressed in NET G3, SRS cannot detect the usually somatostatin-receptor-negative NECs [ 46 ].…”

Intra-ampullary papillary-tubular neoplasm combined with ampullary neuroendocrine carcinoma: A case report

“…Given the shorter survival time and high risk of recurrence after upfront surgery, the authors proposed alternative treatment approaches for ampullary NECs, provided that biopsy availability is ascertained before the final therapeutic decision[ 7 ]. Adjuvant chemotherapy after R0 resection seems to offer improved survival[ 25 , 26 ]; however, the clinical relevance of this finding cannot be determined solely on the basis of individual reported cases.…”