Malignant orbital schwannoma with massive intracranial recurrence.

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.