Carcinoid of the ampulla of Vater

Abstract

Endocrine neoplasms only rarely occur at the ampulla of Vater, comprising mostly carcinoids and malignant carcinoids, as well as few cases of poorly differentiated endocrine carcinomas (small cell carcinomas). Only 105 cases are reported in the literature, most as single case reports. For many years, the neoplasms of the disseminated neuroendocrine cell system of the gastrointestinal tract have been subsumed as 'carcinoids'. Instead, in the latest World Health Organization (WHO) classification published in 2000, it is recommended to distinguish between (i) well-differentiated endocrine tumors (carcinoids); (ii) well-differentiated endocrine carcinomas (malignant carcinoids); and (iii) poorly differentiated endocrine carcinomas (small cell carcinomas). Patients with carcinoid tumors of the ampulla of Vater are very often free of clinical and laboratory findings that belong to the carcinoid syndrome. Approximately 26% of all patients with carcinoid tumor reported in the literature had neurofibromatosis. Besides endoscopic retrograde cholangiopancreatography, endosonography, computed tomography or magnetic resonance imaging may complete the staging approach of this tumor. The Kausch-Whipple procedure or pylorus-preserving pancreaticoduodenectomy is considered the treatment of choice for ampullary, well-differentiated carcinoids >2.0 cm and for ampullary neuroendocrine carcinomas. However, it should be considered that long-term survival of patients with ampullary carcinoids is also reported after local tumor excision (5-year survival rate of 90%). The dilemma is that the differentiation of neuroendocrine tumors cannot be assessed intraoperatively in most cases. Therefore, considering that the 5-year survival rate in patients with neuroendocrine carcinomas of the ampulla of Vater is very low without radical resection, neuroendocrine tumors of the ampulla of Vater without definite histological differentiation should undergo extended surgery.

Neoplasms Associated with Germline and Somatic NF1 Gene Mutations

“…NF1-associated carcinoids are more common in females (1:1.5) [96] and African Americans (87.5%) [87,101,102]. Periampullary carcinoids generally present with jaundice (65%) and nonspecific abdominal pain (31%) [103]. Less common clinical presentations include melena, iron deficiency anemia, gastrointestinal obstruction, cholangitis, and pancreatitis [86,104,105].…”

Neuroendocrine tumors of the small bowels are on the rise: Early aspects and management

“…Unfortunately, there are no controlled studies on the optimal management of early ampullary NETs. Both endoscopic papillectomy and surgical tumor resection can be curative approaches in early disease whereas in patients with ampullary carcinoids > 2 cm a Whipple resection or pylorus preserving pancreatico-duodenectomy is considered the treatment of choice [66] . Endoscopic therapy for early, small ampullary NETs is characterized by low morbidity and minimal hospital lethality.…”

Endoscopic resection of an ampullary carcinoid presenting with upper gastrointestinal bleeding: A case report and review of the literature

“…Up to 26% of patients may have associated Von Recklinghausen disease [5] . There is a higher incidence (46%) of finding metastatic disease at diagnosis in carcinoids of the ampullary area.…”