Malignant orbital schwannoma with massive intracranial recurrence.

摘要

BACKGROUND: A 62 year old male presented with progressive diplopia, left orbital pain and impairment of visual acuity. METHOD AND FINDINGS: Neuroradiological investigation disclosed an orbital tumour. The lesion was totally excised. Histopathology examination revealed a malignant peripheral nerve sheath tumour (MPNST). The tumour recurred with intracranial extension. The patient died 13 months after the initial diagnosis. CONCLUSIONS: To our knowledge, this is the first reported example of a massive intracranial recurrence of an orbital MPNST. The epidemiological features, clinical course and treatment of these lesions are discussed.