Detailed Characteristic of Duodenal Gastrinoma Associated with Multiple Endocrine Neoplasia Type1 (MEN1) Detected by Esophagogastroduodenoscopy (EGD), Which was Buried under Ulcer

A 66-year-old Japanese male was shown to have severe ulcers with hypergastrinemia in the stomach
through proximal horizontal part of the duodenum, who complained of dysphagia and vomiting. He
suffered from gastric ulcer at 63, proven to have undergone parathyroidectomy for
hyperparathyroidism at his 5th decade. Though had no definitely enlarged pituitary detectable by
computed tomography (CT), he had slight defects in the visual field and hyperprolactinemia. A
diagnosis of MEN1 was entertained. Follow up EGD revealed 5 small sessile submucosal tumors
(SMTs) with a central depression or erosion in the duodenal bulb through descending part of the
duodenum, which had been obscured beneath ulcers. Demonstrated in the regenerative mucosa by
their biopsy were clusters of small tumor cells, which, though considered the tips of neuroendocrine
(NE) tumor (NET) lying in the deeper layer, were not large enough to be proven so by immunostaining
with the markers in the serial sections, and diffuse hyperplasia of synaptophysin-, chromogranin Aand

gastrin-positive NE cells in Brunner glands (BGs), the preneoplastic lesion characteristic of
MEN1-associated duodenal gastrinoma, supporting the diagnosis, which was firmly guaranteed by
positively elevated glucagon-provoked plasma gastrin. Subtotal stomach-preserving
pancreaticoduodenectomy (SSPPD) established the final diagnosis of duodenal gastrinomas graded
G1 associated with MEN1, which were shown to be tightly contained in the densely conglomerated
hyperplastic BGs. No metastases were detected. This is the first case in the world, in which all the
three characteristics of MEN1-associated duodenal gastrinoma were demonstrated by EGD. Difficulty
in endoscopically detecting the NET lies in the fact that, in addition to its smallness and deep
localization, it might be buried under peptic ulcer at a certain stage of the natural history and that an
attempt to biopsy it is hampered by the densely conglomerated barrier of the hyperplastic BGs in
some cases.

Author (s) Details

Kenji Sasaki
Department of Internal Medicine, Shiogama City Hospital, Japan.

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