Synchronous duodenal neuroendocrine tumours in von Recklinghausen's disease—a case report of co‐existing gangliocytic paraganglioma and somatostatin‐rich glandular carcinoid

M. STEPHENS, G. T. WILLIAMS, B. JASANI, E. D. WILLIAMS

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35 Citations (Scopus)

Abstract

The co‐existence of a gangliocytic paraganglioma and a glandular psammomatous carcinoid in the duodenum of a patient with von Recklinghausen's disease and bilateral phaeochromocytomas is reported. The two lesions were considered to be distinctive by light microscopy, electron microscopy and immunocytochemistry. The cells of the glandular carcinoid showed strong cytoplasmic immunoreactivity for somatostatin and contained only scanty intracytoplasmic microfilaments on electron microscopy. In contrast, the endocrine cells of the gangliocytic paraganglioma were positive for pancreatic polypeptide and serotonin, were negative for somatostatin, and contained conspicuous intracytoplasmic aggregates of filaments. The histogenic relationship between the two tumours is discussed. This case strengthens the known association of glandular duodenal somatostatinoma with von Recklinghausen's disease and phaeochromocytoma and, in the light of a previous case report, suggests that von Recklinghausen's disease and gangliocytic paraganglioma may co‐exist more commonly than expected.

Original languageEnglish
Pages (from-to)1331-1340
Number of pages10
JournalHistopathology
Volume11
Issue number12
DOIs
Publication statusPublished - Dec 1987

Keywords

  • Keywords: von Recklinghausen's disease
  • duodenal carcinoid
  • gangliocytic paraganglioma
  • neurofibromatosis
  • somatostatinoma

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Histology

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