Endocrine Abstracts

Introduction: Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disorder consisting in the development of primary hyperparathyroidism (PHPT), pancreatic neuroendocrine tumors, and pituitary adenomas. Occurrence of glucagonoma in MEN1 patients is rare (3%) and metastatic disease is present in 50% to 80% of patients at the time of diagnosis. The association of adrenal nodules/tumors and MEN1 is also rare.

Case report: We present the case of a 37-year-old female patient with a personal history of type II diabetes mellitus and severe hypokalemia who presented to the “C.I Parhon” National Institute of Endocrinology for further investigations of hypopotasemia. She had normal blood pressure, weakness and fatigue. Laboratory showed high values of renin and aldosterone (renin >300 pg/ml, aldosterone 547 pg/ml) with persistence of severe hypopotasemia (K=2.30 mmol/l), hyperparathyroidism (PTH=324.10 pg/ml with normocalcemia), hyperprolactinemia (PRL=91.93 ng/ml), without hypercortizolism. Chromogranin A=1.700.00 ng/ml and glucagon were high, with normal levels of 5HIAA, NSE and serotonin. Abdominal MRI reveals a pancreatic corporeal-caudal tumor mass whose appearance is compatible with a neuroendocrine tumour with secondary lymph node and liver determinations. Parathyroid scintigraphy identified an aspect suggestive of lower left parathyroid gland. It was decided surgery, with enucleoresection of cephalopancreatic exopthytic tumour, corporal-caudal splenopancreatectomy en bloc with partial left adrenalectomy. Pathology certifies a well-differentiated neuroendocrine tumor G1pT3pN1pM1a and immunohistochemistry shows positivity for AE1-AE3, chromogranin and synaptophysin, negative staining for CD56, Ki67<1%. Microwave ablation of tumoral liver segment VII was performed considering secondary determinations in the liver. Medical management included somatostatin analogues.

Conclusions: The discovery of elevated levels of renin in the presence of glucagonoma in this patient suggests a tumoral co-secretion. This association is very rare and constitutes a life threatening situation, requiring multidisciplinary approach.