Ampullary large-cell neuroendocrine carcinomas (LCNECs) are extremely rare, and available data are limited on case reports. They present with jaundice, non-specific abdominal pain, or weight loss, imitating adenocarcinoma. Their incidence increases due to the improved diagnostic techniques. However, preoperative diagnosis remains challenging. We report the case of a 70-year-old man with a history of metabolic syndrome, cholecystectomy, and right hemicolectomy, presenting with jaundice. Laboratory results showed increased liver biochemistry indicators and elevated CA 19-9. Esophagogastroduodenoscopy revealed an ulcerative tumor on the ampulla of Vater, and the biopsy revealed neuroendocrine carcinoma. Although computed tomography (CT) detected enlarged regional lymph nodes, the positron emission tomography (PET) showed a hyperactive lesion only in this area. Pylorus-preserving pancreatoduodenectomy with R0 resection was performed. Pathologic evaluation of the 3.1 × 1.9 cm tumor revealed an LCNEC with immunohistochemical positivity at Synaptophysin, EMA, CD56, and cytokeratin CK8/18. The Ki-67 index was 45%. Two out of the nine dissected lymph nodes were occupied by the neoplasm. The patient was discharged home free of symptoms, and adjuvant chemotherapy with carboplatin + etoposide was initiated. A comprehensive review of the reported cases showed that the preoperative biopsy result was different from the final diagnosis in few cases, regarding the subtypes. Conventional radiology cannot identify small masses, and other methods, such as endoscopy, magnetic resonance cholangiopancreatography (MRCP), and FDG-PET scan, might aid the diagnosis. Diagnosis is based on histology and immunohistochemical markers of the surgical specimens. The treatment of choice is pancreatoduodenectomy, followed by adjuvant chemotherapy. However, recurrence is frequent, and the prognosis remains poor.

Introduction: Intraductal papillary mucinous neoplasms (IPMNs) are the most frequent cystic pancreatic neoplasm. They derive from the main pancreatic duct or branch ducts. Aim: This narrative review aims to present and compare the current guidelines on the management of IPMNs. Materials and methods: We reviewed the most important scientific literature on the management of IPMNs. Discussion: The clinical presentation of IPMNs is usually non-specific; common symptoms are abdominal pain, weight loss, and jaundice. There are no sex differences, and the incidence increases with age. It is considered a premalignant lesion associated with synchronous or metachronous carcinomas. Multifocal sites within the pancreas and the presence of solid components, like mural nodules, are predictive factors for developing malignancy. Magnetic resonance imaging (MRI) is the imaging technique of choice. However, computed tomography (CT) and endoscopic ultrasound (EUS) with fine-needle aspiration (FNA) can also contribute to the diagnosis. Resection is the optimal treatment for IPMNs that arise from the main duct, while several indications are suggested for the surgery on IPMNs of branch ducts. Conclusion: The decision on surgery is not always a simple task and should be based on high-risk features of the neoplasm. In any case, re-examination and follow-up are highly recommended.