ARTICLE TYPE : CASE SERIES
Published on : 22 Jan 2026, Volume - 2
Journal Title : WebLog Journal of Endocrinology and Diabetes | WebLog J Endocrinol Diabetes
Source URL: 
https://weblogoa.com/articles/wjed.2026.a2203
Permanent Identifier (DOI) : 
https://doi.org/10.5281/zenodo.18447867
Von Hippel–Lindau Disease Revealed by Bilateral Pheochromocytomas: A Series of 4 Cases and Literature Review
2Department of Dermatology, Mohammed VI University Hospital, Agadir, Morocco
Abstract
Introduction: Von Hippel–Lindau (VHL) disease is an autosomal dominant genetic disorder predisposing to the development of benign and malignant tumors, notably pheochromocytomas, which are often bilateral and of early onset.
Objective: To describe the clinical, biological, radiological, and evolutionary characteristics of patients with bilateral pheochromocytomas associated with VHL disease and to compare our results with the literature data.
Methods: A retrospective descriptive study of four patients managed for bilateral pheochromocytoma with genetic confirmation of a VHL gene mutation.
Results: Age at diagnosis ranged from 12 to 44 years. All patients had bilateral pheochromocytomas with predominant noradrenergic secretion. Total bilateral adrenalectomywas performed in all patients. One patient experienced a local recurrence after the first surgery. Clinical and biological outcomes were overall favorable under substitution therapy and regular follow-up.
Conclusion: Bilateral pheochromocytomas are a common mode of presentation of VHL disease, especially in young subjects. Genetic screening, appropriate surgical management, and prolonged surveillance are essential to improve prognosis.
Keywords: Von Hippel–Lindau; Pheochromocytoma; Bilateral; Adrenalectomy; Genetics
Citation
K. Gorgi, M. Chaouche, K. Rifai, H. Iraqi, M.H. Gharbi. Von Hippel Lindau Disease Revealed by Bilateral Pheochromocytomas: A Series of 4 Cases and Literature Review. WebLog J Endocrinol Diabetes. wjed.2026. a2203. https://doi.org/10.5281/zenodo.18447867