Endocrine Abstracts

The association between primary hyperparathyroidism and pheochromocytoma is present in multiple endocrine neoplasia type 2A (MEN 2A) along with medullary carcinoma or it can be a simple simultaneousness. The presence of the genetic mutation is mandatory in order to have a positive diagnosis of MEN. We report the case of a female patient 63 years old admitted in our department for a large adrenal incidentaloma (10 cm) with no clinical signs of adrenal dysfunction. An adrenal biopsy was already performed in the urology department and the histological result was positive for pheochromocytoma. The medical history of our patient revealed the presence of primary hyperparathyroidism 12 years ago. At the time, the severe hypercalcemia (14 mg/dl total plasmatic calcium) was the trigger for the diagnosis. After we normalize the calcium and we identify the parathyroid adenoma (ultrasound and sestamibi scan) the right inferior parathyroidectomy was performed along with subtotal thyroidectomy for multiple thyroid nodules. After the surgery our patient did not follow any treatment or medical control. The family medical history was negative for medullary carcinoma, pheochromocytoma and hyperparathyroidism. In our department hormonal tests showed: upper limit for plasmatic metanephrines, over 1000 ng/ml chromogranin A, normal PTH and slightly increase calcitonine 12 pg/ml (NV:0–10 pg/ml). The patient is currently on alpha-blocker medication as pre-surgical protocol requires. After surgery a calcitonine stimulation test is planned in order to check for medullary carcinoma. If this test is positive the genetic testing for RET mutation is necessary. This case report emphasizes the importance of medical follow-up after severe hyperparathyroidism no matter patient’s age. The voluminous pheochromocytoma shows a long evolution. Lack of hypertensive crisis and other specific symptoms are explained by the intratumoral consumption of catecholamine. A close monitoring is necessary for the possible thyroid involvement (medullary carcinoma) and also for the parathyroid and adrenal function.