Case and management of incidentalomas in patients with Von Hippel-Lindau disease

Adrenal incidentalomas, those adrenal masses discovered on imaging studies undertaken for other indications, represent an evaluation and management conundrum. Evaluating every incidentaloma for functional status and/or resecting all incidentalomas would not be cost-effective because the vast majority of incidentalomas are benign, non-functioning adenomas. Current management strategies focus on size, functionality and imaging characteristics. These strategies do not take into account individual patient characteristics, for example, comorbid hereditary syndromes. In this article we report a case of a pheochromocytoma presenting as a small incidentaloma in a patient with Von Hippel-Lindau disease. We review the current literature describing the appropriate evaluation and management of adrenal incidentalomas and investigate the nuances of evaluation of these masses in patients with Von Hippel-Lindau disease.