Open AccessOrbital hybrid peripheral nerve sheath tumors
Author(s) -
Kai Ching Peter Leung,
Tak Chuen Simon Ko
Publication year - 2020
Publication title -
taiwan journal of ophthalmology
Language(s) - Uncategorized
Resource type - Journals
SCImago Journal Rank - 0.519
H-Index - 9
eISSN - 2211-5072
pISSN - 2211-5056
DOI - 10.4103/tjo.tjo_28_20
Subject(s) - medicine , neurofibromatosis , malignant peripheral nerve sheath tumor , pathology , soft tissue , peripheral
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.