Malignant peripheral nerve sheath tumor of the occipital region: case report.

OBJECTIVE

A rare case of a malignant peripheral nerve sheath tumor of the occipital region is presented. The role of postoperative radiotherapy in such cases is reviewed.

CLINICAL PRESENTATION: A 36-year-old man presented with a small spongy swelling in the posterior aspect of the cranium since childhood. The swelling gradually increased for 2 years and then rapidly over the next 7 months. There were no associated symptoms of headache, vomiting, vertigo, focal weakness, or visual impairment.

INTERVENTION: A small occipital craniectomy for total excision of the tumor was attempted. However, as a result of intracranial extension to the transverse sinus, the tumor could not be completely excised. Local radiotherapy to a dose of 59.4 Gy in 33 fractions over 6.5 weeks was delivered to the residual microscopic disease in the posterior part of the cranium. The patient has been followed up regularly for the past 28 months and continues to remain asymptomatic.

CONCLUSION

An aggressive malignant peripheral nerve sheath tumor of an atypical site such as the scalp, in which complete surgery may not always be possible, could benefit from postoperative radiotherapy. A dose of approximately 60 Gy could be effectively delivered with a satisfactory outcome.