Endocrine Abstracts

Background: MEN1 is an autosomal dominant inherited syndrome. Primary hyperparathyroidism, tumors of the endocrine pancreas, and of the pituitary, are the characteristic features of the syndrome.

Objective: To present a case of MEN1 in a patient with extreme obesity, causing serious difficulties in diagnostic procedures leading to localization of pancreatic tumors.

Case presentation: 22-year-old male with extreme obesity (BMI 59), hypogonadism, gynaecomastia, galactorrhoea and duodenal ulceration, came to our Department before a planned surgery for obesity. Hormonal tests showed elevated levels of PRL (2000 ug/l), low levels of LH/FSH (<0.1 U/l) and of testosterone (0.7 ng/ml) with heighten estradiol (55.6 pg/ml) and elevated levels of S-DHEA (12312 ng/ml). MR examination showed a pituitary macroadenoma, size 45×32×25 mm. Treatment with bromocriptin lowered PRL to 45 ug/l. Further diagnostic confirmed hyperparathyroidism (PTH-115 pg/ml; Ca-10.41 mg/dl) and a left adrenal gland tumor. High levels of gastrin (1340 μU/ml; N<115) and of chromogranin A (833 U/l; N<18) led to suspicion of gastrinoma. Endosonography showed 4 hypoechogenic foci in the head of pancreas. Octreoscan confirmed a high expression of somatostatin receptors. It was impossible to perform computed tomography because of the extreme obesity. In spite of that subtotal splenopancreatectomy, left side adrenalectomy and subtotal gastrectomy were performed. Histopathological examination confirmed multifocal well differentiated neuroendocrine carcinoma with a single metastasis to lymphatic node, and a benign adrenal tumor. Postoperative scintigraphy did not show abnormal uptake of radioisotope. The level of gastrin decreased to 113 μU/ml, and of CgA to 81 U/l. Patient is currently treated with IPP and bromocriptin. In case of relapse or liver metastasis radiotherapy will be considered, using radiolabeled somatostatin’s analogs.

Summary: Localizing diagnostic and treatment procedures in cases of tumors of the endocrine pancreas as a part of MEN1 remain a significant challenge. In case of the above mentioned patient the decision of surgery was made on the basis of the result of octreoscan and endosonography because the extreme obesity made computed tomography impossible.