Von Hippel–Lindau Disease Revealed by Bilateral Pheochromocytomas: A Series of 4 Cases and Literature Review

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.