Hybrid neurofibroma/schwannoma of the orbit.

Autor: Salazar-Huayna L; Pathology Department, Vall d'Hebron University Hospital, Barcelona, Spain., Naranjo L; Pathology Department, Vall d'Hebron University Hospital, Barcelona, Spain., Romagosa C; Pathology Department, Vall d'Hebron University Hospital, Barcelona, Spain., Arcediano MÁ; Ophthalmology Department, Vall d'Hebron University Hospital, Barcelona, Spain., Siurana S; Radiology Department, Vall d'Hebron University Hospital, Barcelona, Spain., Ramón Y Cajal S; Pathology Department, Vall d'Hebron University Hospital, Barcelona, Spain., Dinarès C; Pathology Department, Vall d'Hebron University Hospital, Barcelona, Spain. Electronic address: mcdinares@vhebron.net.
Jazyk: angličtina
Zdroj: Revista espanola de patologia : publicacion oficial de la Sociedad Espanola de Anatomia Patologica y de la Sociedad Espanola de Citologia [Rev Esp Patol] 2023 Apr-Jun; Vol. 56 (2), pp. 140-143. Date of Electronic Publication: 2022 Jun 15.
DOI: 10.1016/j.patol.2022.03.002
Abstrakt: Hybrid neurofibroma/schwannoma is a rare variant of hybrid peripheral nerve sheath tumours (HPNST). A Medline search up to December 2021 identified only six cases of this tumour in the orbit. We report the case of a 78-year-old man who presented with left exophthalmos. Computed tomography showed a left intraconal orbital mass. The clinico-radiological diagnosis was consistent with an intraconal cavernous angioma. Orbitotomy was performed, obtaining an 18×16×11mm mass. Two different morphologies were seen microscopically, diagnostic of hybrid neurofibroma/schwannoma. HPNSTs of the orbit are uncommon and most reported cases showed a hybrid neurofibroma/schwannoma morphology. Hybrid neurofibroma/schwannomas have been associated with neurofibromatosis and schwannomatosis. Local recurrences have been reported. The correct identification of these tumours is important due to their potential use as a syndromic marker.
(Copyright © 2022 Sociedad Española de Anatomía Patológica. Publicado por Elsevier España, S.L.U. All rights reserved.)
Databáze: MEDLINE