Mucosal neuromas and plexiform neurofibromas: An immunocytochemical study

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

doi:10.3109/15513819309048214

Mucosal neuromas and plexiform neurofibromas: An immunocytochemical study

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

Department of Pathology

Research output: Contribution to journal › Article › peer-review

18 Scopus citations

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 language	English (US)
Pages (from-to)	281-288
Number of pages	8
Journal	Fetal and Pediatric Pathology
Volume	13
Issue number	3
DOIs	https://doi.org/10.3109/15513819309048214
State	Published - Jan 1 1993

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

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title = "Mucosal neuromas and plexiform neurofibromas: An immunocytochemical study",

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..",

keywords = "Epithelial membrane antigen, Mucosal neuroma, Multiple endocrine neoplasia type IIb, Neurofibromatosis, Plexiform neurofibroma",

author = "J. Cangiarella and J. Jagirdar and H. Adelman and G. Budzilovich and Greco, {And M.A.}",

year = "1993",

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T2 - An immunocytochemical study

AU - Cangiarella, J.

AU - Jagirdar, J.

AU - Adelman, H.

AU - Budzilovich, G.

AU - Greco, And M.A.

PY - 1993/1/1

Y1 - 1993/1/1

N2 - 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..

AB - 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..

KW - Epithelial membrane antigen

KW - Mucosal neuroma

KW - Multiple endocrine neoplasia type IIb

KW - Neurofibromatosis

KW - Plexiform neurofibroma

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Mucosal neuromas and plexiform neurofibromas: An immunocytochemical study

Abstract

Keywords

ASJC Scopus subject areas

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