Functional 'composite' pheochromocytoma-ganglioneuroma 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 paraganglioma-ganglioneuroma. Clinical, biochemical and radiological aspects of this rare tumor and its association with neurofibromatosis and other hereditary cancer syndromes are discussed.
Department of Internal Medicine, University of Connecticut, 263 Farmington Avenue, Farmington, CT 06030, United States. smajumder@resident.uchc.edu
Shounak
S
Majumder
Joanna
J
Grabska
Guru
G
Trikudanathan
Pamela
P
Kowalczyk
Elena
E
Stoica-Mustafa
Constantin A
CA
Dasanu
D000310:Adrenal Gland Neoplasms / Q000150:complications* / Q000473:pathology; D000328:Adult; D005260:Female; D005729:Ganglioneuroma / Q000150:complications* / Q000175:diagnosis / Q000473:pathology; D006801:Humans; D009456:Neurofibromatosis 1 / Q000150:complications; D010190:Pancreatic Neoplasms / Q000175:diagnosis / Q000473:pathology*; D010673:Pheochromocytoma / Q000150:complications* / Q000175:diagnosis / Q000473:pathology