Gastrinoma of Cystic Duct: A Rare Association With Multiple Endocrine Neoplasia Type 1
Aline Barbosa Moraes, Natalia Treistman, Mariana Coutinho Studart, Vera Lucia Antunes Chagas, Eloa Pereira Brabo, Leonardo Vieira Neto
Abstract
Neuroendocrine tumors (NETs) of cystic duct are extremely rare, accounting for less than 2% of NET cases. The association of biliary tree NET and multiple endocrine neoplasm type 1 (MEN1) are even more rare. In this report, we described a case of a 65-year-old woman who was referred to our neuroendocrine outpatient clinic to investigate MEN1 after an incidental diagnosis of gastrinoma. Her medical history initiated 7 years earlier with severe peptic disease not responsive to proton pump inhibitor therapy. Endoscopic study revealed erosive antral gastritis, erosive duodenitis, bulbar ulcer and pyloric deformity. During follow-up she presented with abdominal pain, chronic diarrhea and weight loss; an ultrasonography was performed and showed only a cholelithiasis. She underwent a video laparoscopic cholecystectomy and all her symptoms were solved. Histopathological study found a 1.0 cm well differentiated NET (Ki-67 labeling index < 2%) located in cystic duct infiltrating the entire wall and subserosa. The MEN1 investigation revealed a primary hyperparathyroidism with a brown tumor in right iliac bone; the patient was referred to a total parathyroidectomy with autotransplantation. No evidence of pituitary tumor was found. The patient remains asymptomatic 24 months after surgery. To conclude, this case highlights an unusual presentation of a cystic duct primary NET gastrinoma in a MEN1 context.
J Clin Med Res. 2018;10(11):843-847
doi: https://doi.org/10.14740/jocmr3541w
Keywords
Neuroendocrine tumor; Cystic duct; Biliary tree; Multiple endocrine neoplasia type 1; Gastrinoma; Zollinger-Ellison syndrome
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