Unusual cystic presentation of pheochromocytoma in a case of suspected von Hippel–Lindau disease

Von Hippel-Lindau (VHL) disease is a rare autosomal dominant disorder characterized by multisystem involvement. Pheochromocytoma is a known manifestation of VHL and typically presents as a solid hypervascular adrenal mass. Cystic pheochromocytoma is an uncommon variant and may pose a diagnostic challenge, especially in the absence of classical clinical features. We report the case of a 51-year-old male who presented with acute pancreatitis. Contrast-enhanced computed tomography revealed multiple cystic lesions involving the pancreas, kidneys, and liver along with a predominantly cystic lesion replacing the left adrenal gland showing a small enhancing solid component. In the background of suspected VHL disease, the adrenal lesion was considered likely to represent a cystic pheochromocytoma. This case highlights the importance of recognizing atypical imaging appearances of pheochromocytoma and considering an underlying syndromic association such as VHL in the presence of multisystem involvement. Early identification is crucial for appropriate management, surveillance, and screening of family members.