Journal of Clinical Medicine Research, ISSN 1918-3003 print, 1918-3011 online, Open Access
Article copyright, the authors; Journal compilation copyright, J Clin Med Res and Elmer Press Inc
Journal website http://www.jocmr.org

Case Report

Volume 10, Number 11, November 2018, pages 843-847


Gastrinoma of Cystic Duct: A Rare Association With Multiple Endocrine Neoplasia Type 1

Figures

Figure 1.
Figure 1. Esophago-gastro-duodenoscopy images. (A) Reflux esophagitis (Los Angeles B). (B) Erosive antral gastritis. (C) Bulbar ulcer. (D) Erosive duodenitis.
Figure 2.
Figure 2. Immunohistopathological analysis of neuroendocrine tumor of cystic duct. (A) Neuroendocrine tumor displaying cells with eosinophilic cytoplasm and rounded and regular nuclei (H&E, × 400). (B) Neoplastic cells positive for chromogranin (× 400). (C) Neoplastic cells positive for synaptophysin (× 400). (D) Neuroendocrine tumor (*) occupying the lumen of the cystic duct (←) and infiltrating the wall (▲) (H&E, × 100).
Figure 3.
Figure 3. Normal esophago-gastro-duodenoscopy examination 10 months after surgery (A, B, C and D).

Tables

Table 1. Post-Operative Laboratory Evaluation
 
ValuesNormal range
Calcium (mg/dL)11.88.5 - 10.5
Phosphorus (mg/dL)3.02.5 - 4.5
Parathyroid hormone (pg/mL)15412 - 65
24 h urine calcium (mg/24h)170112 - 353
Prolactin (ng/mL)6.49< 20.3
Insulin growth factor 1 (ng/mL)14175 - 212
Gastrin (pg/mL)1013 - 115
Chromogranin A (U/mL)101.6Up to 100
5-hydroxyindoleacetic acid (mg/24h)2.72 - 9

 

Table 2. Literature Series of Patients With Multiple Endocrine Neoplasia Type 1 Presenting Ectopic Gastrinomas
 
AuthorAgeSexSymptomsEGDSSize of gastrinoma in biliary tree (cm)KI-67Biliary tree gastrinoma localizationOther endocrine neoplasmsTherapeutical approach for MEN 1MetastasisTime of follow-up free of cancer (months)
aZollinger-Ellison syndrome. bTwo out of 31 lymph nodes examined had neuroendocrine tumor tissue positive for gastrin after surgery including the removal of the regional lymph nodes. NA: not available.
Price et al [7]55FNAaNAa0.6NACommon bile duct, cystic ductPituitary adenoma, hyperparathyroidism, non-functioning NET in the pancreasDistal splenopancreatectomy, radio-frequency ablation hepatic excision bile ductLiver (solitary)24
Price et al [7]43FSymptomatic gastroesophageal reflux diseaseNA1.5 × 1.2 × 0.1NACommon bile ductPituitary adenoma, hyperparathyroidism, non-functioning NET in the pancreas, duodenal gastrinomaPylorus preserving duodenopancreatectomy, enucleation, gastric excisionLymph nodes (common bile duct)24
Lee et al [8]39FAbdominal pain, nausea, and vomiting; history of spontaneous perforation of the duodenumNA1.6 × 1.5 × 0.9NALiver (II-III segment)Pituitary macroadenoma, hyperparathyroidism, insulinomaWhipple’s duodenopancreatectomy, II-III liver segmentectomyNo12
Tonelli et al [3]46FRecurrent renal colic episodes; epigastralgiaUlceration of the first and second portions of the duodenum1.2< 1%Bile ductHyperparathyroidism, non-functioning NET in the pancreas, duodenal, gastrinomaTotal parathyroidectomy, total pancreatectomy with gastroduodenal resection and splenectomy, bile duct resectionNo1
Tonelli et al [3]55FKidney stones with recurrent renal colic; episodes of epigastric painDuodenal ulcer0.2< 1%Gallbladder fundusHyperparathyroidism, non-functioning NET in the pancreas, duodenal gastrinomaTotal parathyroidectomy, with a parathyroid autograft in the non-dominant forearm, Whipple’s duodenopancreatectomyNoNA
Tonelli et al [3]37FHeartburn, abdominal pain and diarrheaEsophagitis and gastroduodenitis with peptic ulcers1.5< 1%Bile ductNon-functioning NET of the pancreas, duodenal gastrinomaPylorus-preserving duodenopancreatectomy extended to the pancreatic body, with removal of the regional lymph nodes and the gallbladder, hepatic resection (V-VIII segment) and enucleation of a pancreatic head noduleLymph nodesbNA
Our case65FMesogastric pain; chronic diarrhea, steatorrhea, weight lossReflux esophagitis, erosive antral gastritis, duodenitis bulbar and pyloric deformity1.0< 2%Cystic ductHyperparathyroidismCholecystectomyNo24