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 journalArticle

5 Scopus citations

Abstract

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
JournalPancreatology
Volume12
Issue number3
DOIs
StatePublished - Jan 1 2012

Keywords

  • Ganglioneuroma
  • Neuroendocrine tumor
  • Pancreas
  • Paraganglioma

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism
  • Hepatology
  • Gastroenterology

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    Majumder, S., Grabska, J., Trikudanathan, G., Kowalczyk, P., Stoica-Mustafa, E., & Dasanu, C. A. (2012). Functional 'composite' pheochromocytomaeganglioneuroma presenting as a pancreatic mass. Pancreatology, 12(3), 211-214. https://doi.org/10.1016/j.pan.2012.02.001