Orbital hybrid peripheral nerve sheath tumors

Kai Ching Peter Leung; Tak Chuen Simon Ko

doi:10.4103/tjo.tjo_28_20

Orbital hybrid peripheral nerve sheath tumors

Taiwan J Ophthalmol. 2020 Jul 27;10(3):181-183. doi: 10.4103/tjo.tjo_28_20. eCollection 2020 Jul-Sep.

Authors

Kai Ching Peter Leung¹, Tak Chuen Simon Ko¹

Affiliation

¹ Department of Ophthalmology, Tung Wah Eastern Hosptial, Causewaybay, Hong Kong.

Abstract

Hybrid peripheral nerve sheath tumors (HPNST) are recently classified tumors from the World Health Organization Classification of soft tissue tumors that display combined features of more than one peripheral nerve sheath tumor. Acknowledgment is important because of its association with the development of neurofibromatosis type 1, type 2, and schwannomatosis. Orbital involvement is rare and only six cases of HPNST have been documented on literature. This article serves to review the pathophysiology, clinical manifestation, diagnosis, treatment, and prognosis of this infrequent but important orbital tumor.

Keywords: Hybrid peripheral nerve sheath tumor; neurofibroma-perineurioma; neurofibroma-schwannoma; orbital; orbital tumor.

Publication types

Review