Malignant peripheral nerve sheath tumors in children: A single-institutiontwenty-year experience

A retrospective series of pediatric patients with localized malignant perip heral nerve sheath tumors (MPNST) treated during a 20-year period at one in stitution is reported. Between 1976 and 1996, 24 consecutive children were treated by a multimodality approach. Conservative surgery was the treatment of choice: primary radical surgery was performed in 10. Postoperative radi otherapy was administered in 12 and adjuvant chemotherapy in 19. Eight pati ents were alive without evidence of disease, six in first complete remissio n and two in second complete remission, after a median follow-up of 230 mon ths. The 10-year event-free survival (EFS) and survival were 29% and 41%, r espectively. Survival was 80% for the patients who underwent radical surger y, and 14% for the others; 71% for patients with tumors smaller than 5 cm, and 29% for those with tumors 5 cm or larger. Local recurrence was the majo r cause for treatment failure (13 of 17; 76%); the rate of local relapse wa s 33% v 75% in patients who either received or did not receive radiotherapy . Complete surgical excision re mains the most effective treatment for MPNS T and represents the main prognostic factor along with tumor size. Radiothe rapy seems to play a role in achieving local control, whereas the role of c hemotherapy is uncertain.