Malignant peripheral nerve sheath tumor of the orbit in a 15-month-old child. Nine-year survival after local excision.

Abstract

Malignant peripheral nerve sheath tumors are extremely rare orbital tumors that carry a poor prognosis despite wide excision with disfiguring surgery, chemotherapy, or radiotherapy. The authors present the youngest reported case, a 15-month-old boy who underwent an orbitotomy to excise a bilobed tumor from the right orbit. Histologic examination revealed a malignant peripheral nerve sheath tumor; standard treatment options, including orbital exenteration, chemotherapy, and radiotherapy, were denied and instead the patient was followed with serial computed tomography scans and magnetic resonance imaging. Nine years later, the patient remains without evidence of recurrent tumor and visual acuity is 20/20. This patient's course suggests that orbital malignant peripheral nerve sheath tumors, if believed to be completely excised at the time of surgery, might be followed with careful neuroimaging studies for signs of recurrence.