Malignant Peripheral Nerve Sheath Tumor (MPNST) Arising from Orbital Plexiform Neurofibroma in a Small Child With Neurofibromatosis Type 1

Orbital PNF is a rare and characteristic manifestation of facial NF1. Typically, tumors in this localization are associated with severe functional disabilities and aesthetic disfigurement, resulting from invasive tumor growth and skeletal deformities. Histological classification of the tumors may be challenging due to varying histological differentiation in different tumor locations. Thus, early diagnosis with representative tumor sampling and complete histological work-up of the specimen together with multimodal therapy are essential prerequisites to overcome the poor prognosis of these tumors.