Functional 'composite' pheochromocytomaeganglioneuroma presenting as a pancreatic mass

Shounak Majumder, Joanna Grabska, Guru Trikudanathan, Pamela Kowalczyk, Elena Stoica-Mustafa, Constantin A. Dasanu

Research output: Contribution to journalArticlepeer-review

8 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)211-214
Number of pages4
Issue number3
StatePublished - 2012

Bibliographical note

Copyright 2017 Elsevier B.V., All rights reserved.


  • Ganglioneuroma
  • Neuroendocrine tumor
  • Pancreas
  • Paraganglioma


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