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Endocrine Abstracts (2023) 90 EP32 | DOI: 10.1530/endoabs.90.EP32

ECE2023 Eposter Presentations Adrenal and Cardiovascular Endocrinology (124 abstracts)

Hereditary and sporadic pheochromocytoma: Comparison of clinical and biological features

Rania El Amel 1 , Hanae Rachedi 1 , Rami Imane 1 , Siham Rouf 2 & Hanane Latrech 2


1Endocrinology-Diabetology and Nutrition Department Hospital University Centre of Mohammed-VI, Oujda, Morocco; 2Endocrinology-Diabetology and Nutrition Department Hospital University Centre of Mohammed-VI, Laboratory of Epidemiology, Clinical Research and Public Health, Faculty of Medicine and Pharmacy Mohammed First University , Oujda, Morocco


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.

Volume 90

25th European Congress of Endocrinology

Istanbul, Turkey
13 May 2023 - 16 May 2023

European Society of Endocrinology 

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