A 59 year old patient with metastases of gastrinoma in the context of MEN-1 treated with Sandostatin – surgical revision versus conservative therapy

Abstract

Background: Multiple endocrine neoplasia type 1 (MEN-1) is an autosomal dominant predisposition for tumors of the parathyroid glands, anterior pituitary and pancreatic island cells. We report of a 59 year old patient with MEN-1 who underwent partial resection of abdominal metastases of gastrinoma 5 years ago and is treated with Octreotid LAR since then. The patient now presented for his annual staging examination. Past medical history: MEN-1 had been diagnosed with the patient in 1999, following workup of primary hyperparathyroidism. The patient had undergone resection of four hyperplastic epithelial bodies as well as resection of a mediastinal mass, emerging to be a thymus carcinoid. Furthermore, a macroprolactinoma was found, being treated with cabergolin since then. High gastrin levels as well as a history of gastrointestinal ulceration raised suspicion of gastrinoma in the context of MEN-1. Somatostatin receptor scintigraphy with indium-octreotide confirmed three sites of enhanced storage intraabdominally. Patient underwent explorative laparotomy. Two sites of metastatic infiltration of a then confirmed gastrinoma could be resected totally, a third site was only partially resected. Diffuse metastatic peritoneal infiltration was found. Pancreas itself presented free of tumor manifestation. Being treated with Sandostatin LAR, the patient showed no symptoms of Zollinger-Ellison-syndrome the following years and needed to take PPI only occassionally. History of present workup: The patient now presented for an extended staging to clarify the indication for re-operation. Parathyroid hormone, calcium and phosphate as well as prolactin, testosterone and LH/FSH were found normal; gastrin secretion was low under therapy with Octreotide. Somatostatin receptor scintigraphy with indium-octreotid found the well-known abdominal storage site being constant in size. Also in the pancreatic region storage enhancement was found, a finding which had not been described in prior szintigraphies. However, retrospective analysis of former examinations showed discrete storage enhancement in this region, representing the primary tumor site. Abdominal CT scanning showed two intrapancraetic contrast agent storing lesions in the early arterial phase. Endoscopic ultrasound confirmed two lesions in the corpus/cauda region.