Functional 'composite' pheochromocytomaeganglioneuroma presenting as a pancreatic mass

Pheochromocytomas rarely have 'composite' forms in which they demonstrate histologic features of a typical paraganglioma in combination with those of a neural component. Extra-adrenal 'composite' pheochromocytomas are distinctly uncommon.We describe herein a unique case of a 34-year-old female patient with type 1 neurofibromatosis who presented with abdominal pain and paroxysmal hypertension. Imaging revealed a pancreatic mass with biliary and pancreatic ductal dilatation and a hormonal assay led to the diagnosis of functional pheochromocytoma. She underwent surgical resection and histopathology revealed a composite paragangliomaeganglioneuroma. Clinical, biochemical and radiological aspects of this rare tumor and its association with neurofibromatosis and other hereditary cancer syndromes are discussed.