Primary malignant peripheral nerve sheath tumor of the cauda equina with metastasis to the brain in a child: case report and literature review.

BACKGROUND CONTEXT: Primary intradural malignant peripheral nerve sheath tumors (MPNSTs) are extremely rare; only 23 cases have been reported in the English-language literature till now. No gold standard for treating primary intradural MPNSTs has yet been established.

PURPOSE: To report a rare case of primary intradural MPNSTs in a child and review the literature pertaining to this rare disease.

STUDY DESIGN/

SETTING

Case report and literature review.

METHODS

We report our experience with one new case. An 8-year-old boy diagnosed with primary intradural MPNSTs underwent three surgical excisions and two rounds of radiotherapy; however, metastasis to the brain was found, and the boy died 16 months after the first surgery. We also review the literature pertaining to both MPNSTs in general and primary intradural MPNSTs.

RESULTS

Surgery is currently the mainstay of MPNST treatment. Radiotherapy and chemotherapy are of limited value in these tumors. Based on the review of the 24 cases described in the literature, including the present case, primary intradural MPNST is a very aggressive tumor with a very high recurrence rate even after gross total resection and with significant potential for leptomeningeal and systemic metastasis. The overall prognosis is very poor and seems to be worse than that of MPNSTs in general.

CONCLUSIONS

Primary intradural MPNST is a very rare entity with a poor prognosis. Surgical tumor removal combined with postoperative high-dose radiation may be recommended. Chemotherapy is usually reserved for patients with disseminated metastases or tumors that are unresectable at the time of diagnosis.