Mucosal neuromas and plexiform neurofibromas: An immunocytochemical study

J. Cangiarella, J. Jagirdar, H. Adelman, G. Budzilovich, And M.A. Greco

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Abstract

Mucosal neuromas (MN), a component of multiple endocrine neoplasia (MEN) type IIb, may be confused histologically with plexiform neurofibromas (PN), a component of neurofibromatosis. The ability to distinguish between these two markers for different genetic diseases is crucial, as the risk of development of medullary thyroid carcinoma and pheochromocytoma in affected patients with MEN IIb is great. We studied two cases each of MN and PN by immunocytochemistry (IC). Epithelial membrane antigen (EMA) proved to be the most useful marker. MN consisted of bundles of disorganized and tortuous nerve fibers surrounded by a thickened perineurium that expressed the cellular phenotype EMA(+ S-100(- PN consisted of enlarged neroe fascicles with a loose myxoid stroma and was EMA negative. Thus, IC highlighted the differing pattern of growth and histogenesis of the proliferating cells in the two lesions and is likely to be especially useful in those lesions with atypical histology..

Original languageEnglish (US)
Pages (from-to)281-288
Number of pages8
JournalFetal and Pediatric Pathology
Volume13
Issue number3
DOIs
Publication statusPublished - Jan 1 1993

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Keywords

  • Epithelial membrane antigen
  • Mucosal neuroma
  • Multiple endocrine neoplasia type IIb
  • Neurofibromatosis
  • Plexiform neurofibroma

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Pathology and Forensic Medicine

Cite this

Cangiarella, J., Jagirdar, J., Adelman, H., Budzilovich, G., & Greco, A. M. A. (1993). Mucosal neuromas and plexiform neurofibromas: An immunocytochemical study. Fetal and Pediatric Pathology, 13(3), 281-288. https://doi.org/10.3109/15513819309048214