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Pancreatology. 2012 May-Jun;12(3):211-4. doi: 10.1016/j.pan.2012.02.001. Epub 2012 Feb 8.

Functional 'composite' pheochromocytoma-ganglioneuroma presenting as a pancreatic mass.

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  • 1Department of Internal Medicine, University of Connecticut, 263 Farmington Avenue, Farmington, CT 06030, United States.


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 paraganglioma-ganglioneuroma. Clinical, biochemical and radiological aspects of this rare tumor and its association with neurofibromatosis and other hereditary cancer syndromes are discussed.

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