Carcinoid tumor of the minor papilla in complete pancreas divisum presenting as recurrent abdominal pain

Abstract

BackgroundTumors of the minor papilla of the duodenum are extremely rare, and they are mostly neuroendocrine tumors, such as somatostatinomas and carcinoid tumors. However, true incidence of carcinoid tumors in minor papilla might be much higher, because patients with minor papillary tumors usually remain asymptomatic. We report a very unusual case of carcinoid tumor in a patient with complete pancreas divisum with a review of the literature.Case presentationA 56-year-old female patient was referred for evaluation of pancreatic duct dilatation noted on abdominal ultrasonography and computerized tomography. She complained of intermittent epigastric pain for 6 months. A MRCP and ERCP revealed complete pancreas divisum with dilatation of the main pancreatic duct. On duodenoscopy, a small, yellows, subepithelial nodule was visualized at the minor papilla; biopsy of this lesion revealed a carcinoid tumor. She underwent a pylorus-preserving pancreaticoduodenectomy. The histologic evaluation showed a single nodule, 1 cm in diameter, in the submucosa with duodenal and vascular invasion and metastasis to the regional lymph nodes.ConclusionAlthough the size of the carcinoid tumor was small and the tumor was hormonally inactive, the concomitant pancreas divisum led to an early diagnosis, the tumor had aggressive behavior. Carcinoid tumors of the minor papilla should be included in the differential diagnosis of recurrent abdominal pain or pancreatitis of unknown cause.

Gastroenteropancreatic neuroendocrine tumor of the accessory papilla of the duodenum: a case report

“…Contrary to the increasing number of GEP-NETs, GEP-NETs of the accessory papilla of the duodenum are very rare. The characteristics in reported cases of GEP-NETs of the accessory papilla of the duodenum are demonstrated in Table 1 [3][4][5][6][7][8][9][10][11]. The average tumor size was approximately 11 mm.…”

“…Furthermore, GEP-NETs of the accessory papilla of the duodenum are extremely rare. To date, there have been only nine cases of GEP-NETs of the accessory papilla of the duodenum reported in the English literature [3][4][5][6][7][8][9][10][11]. We hereby present a case of GEP-NET of the accessory papilla of the duodenum successfully treated with robotic pancreatoduodenectomy.…”

Long-standing malignant pancreatic carcinoid treated with octreotide

“…Over 200 cases of pancreatic carcinoids have most likely been reported as of today; the following ensues from a literature review (Table I) (16): a) pancreatic carcinoid or serotoninoma secretes and releases sero-tonin (5-HIAA in urine), since it is made up of EC cells (argyrophilia) within the pancreas, with positive immunohistochemistry for said hormone; b) tumors are malignant in about 70% of cases, and present with either typical or atypical CS (fewer than 50%) as well as a triad of abdominal pain, diarrhea, and weight loss; c) survival at 5 years is low, around 30%, but may respond to somatostatin analogs and radical surgery; d) frequency is around 1% of all carcinoids, and 10-year survival is 10% (35) because of high malignant potential (36); e) they may be associated with MHS, MEN-1 (9), and gastroduodenal carcinoids (37); and f) in contrast to pancreatic carcinoids, carcinoids in the common bile duct and minor papilla are extremely rare (8 cases reported), and those of the papilla are rare (70 cases reported) (38).…”

Synchronous Periampullary Tumors in a Patient With Pancreas Divisum and Neurofibromatosis Type 1

“…The anomaly is caused by absent or incomplete fusion of the ventral (main or Wirsung) and dorsal (marginal or Santorini) ducts (Kanth et al, 2014) and results in coexistence of two ampullary systems: the ventral duct drains the pancreatic head through the major ampulla, while the dorsal duct drains the pancreatic body and tail through the minor ampulla (Ferri et al, 2019). Ampullary neoplasms are rare, comprising 7% of all periampullary malignancies (Adsay et al, 2012), and association of theses tumors with pancreas divisum is considered an episodic event (Singh et al, 2003;Outtas et al, 2004;Kim et al, 2010). Their occurrence has been reported in families with hereditary cancer syndromes, such as Familial Adenomatous Polyposis (Pérez-Cuadrado-Robles et al, 2019) and Neurofibromatosis type 1 (NF1) (Tewari et al, 2014).…”