Schwann cell-onion bulb tumor of the trigeminal nerve: hyperplasia, dysplasia or neoplasia?

Abstract:

:Onion bulbs are concentric lamellar structures formed by Schwann or perineurial cells, which may be seen in several generalized or localized diseases of the peripheral nerve. There is debate regarding the pathogenesis of localized tumefactions displaying these microscopic structures. We report the fifth case, to our knowledge, of a Schwann cell-onion bulb tumor, which arose in the trigeminal nerve of a child with an unclassifiable, probably distinct, neurocutaneous syndrome; we also provide evidence for a neoplastic or hamartomatous origin. Molecular studies failed to establish an abnormality in the NF1, NF2, PMP22, or Connexin 32 genes. Similar and previously reported cases are discussed, as well as other onion bulb-forming entities.

journal_name

Acta Neuropathol

journal_title

Acta neuropathologica

authors

LaPoint SF,Powers JM,Woodruff JM,MacCollin M,Jacoby LB,Vortmeyer AO,Zhuang Z,Fong CT,Ifthikharuddin SF,Teot L,Coniglio JU,Sullivan RP

doi

10.1007/pl00007408

subject

Has Abstract

pub_date

2000-01-01 00:00:00

pages

67-72

issue

1

eissn

0001-6322

issn

1432-0533

journal_volume

99

pub_type

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