Endocrine Abstracts

Introduction: Pheochromocytoma is a rare neuroendocrine tumor derived from the adrenal medulla characterized by a high potential of production of catecholamines. The majority of pheochromocytomas are sporadic. However, recent studies show an increasing incidence of hereditary forms.

Patients and methods: This is a retrospective descriptive study including 30 patients with pheochromocytoma followed-up in the Endocrinology-Diabetology and Nutrition Department of the University Hospital Center of Mohammed-VI-Oujda in Morocco.

Results: Among 30 patients with pheochromocytoma, 22 cases (13 females and 9 males) had a sporadic form, and 8 patients (6 females and 2 males) had an hereditary form, associated with multiple endocrine neoplasia type 2 in 7 patients and Von Hippel-Lindau disease in only one case. Hereditary forms were confirmed genetically in 5 patients. The mean age was lower in the hereditary group compared with the sporadic group (35.4±12.5 years vs 51.8±17.1 years respectively). Hypertension was noted in 15 cases with sporadic pheochromocytoma, 8 of which had cardiovascular complications ; however, in the hereditary group, only one case had hypertension without cardiovascular repercussions. Menard triad, was more frequent in the sporadic cases (63.6% vs 37.5%). Hereditary pheochromocytomas, compared to the sporadic forms, had lower 24-hours urinary metanephrines (2.51 vs 44 times upper limit of normal), represented mainly by elevated normetanephrines compared to metanephrines in both groups.

Conclusion: Hereditary pheochromocytomas are becoming more and more frequent. A genetic testing should be offered to patients with pheochromocytoma to allow a more rigorous follow-up with avoiding recurrence and for an early detection of the disease in relatives, in purpose to reduce morbidity and mortality given the risk of additional tumors.