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Orbital hybrid peripheral nerve sheath tumors

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

Correspondence Address:
Kai Ching Peter Leung,
Department Of Ophthalmology, Tung Wah Eastern Hospital, Causewaybay
Hong Kong
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/tjo.tjo_28_20

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.

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