Incidental Discovery of Synchronous Ileal Neuroendocrine Tumors at Fluorodopa PET/CT in a Patient With Bilateral Pheochromocytoma

Pheochromocytomas are tumors originating from neuroendocrine cells in the adrenal medulla, with an incidence of 0.05%. They are most often unilateral (92% of cases) and implicated in ~0.1% of hypertension cases. About 30% of pheochromocytomas are associated with hereditary syndromes such as MEN2, VHL, and NF1. When bilateral, a genetic disease is identified in 80% of patients. While coexistence of pheochromocytoma with pancreatic NETs has been described in Von Hippel-Lindau disease, coexistence of pheochromocytoma with duodenal, jejunal, or ileal NET is very rare: about 20 cases were reported in the literature, synchronous or metachronous, often associated with neurofibromatosis. We describe the case of a 58-year-old patient with a previously unremarkable history, referred to fluorodopa PET/CT for staging of a bilateral pheochromocytoma discovered in front of a typical clinical triad, who was incidentally diagnosed with 2 synchronous neuroendocrine tumors of the small bowel.