Pediatric neurofibrosarcoma.
OBJECTIVE
Neurofibrosarcoma is rare in children, and the natural history and prognostic factors are not well described. The authors present a 57-year review of their experience.


METHODS
The charts of children with neurofibrosarcoma were reviewed retrospectively. Statistical analysis was performed using the Chi2 and unpaired t tests.


RESULTS
From 1944 to 2001, 38 patients under the age of 21 were diagnosed with neurofibrosarcoma. Twenty-two were boys. The average age at diagnosis was 13.8 years (range, 3 to 19.9 years). Nineteen patients (50%) had neurofibromatosis. The tumor site was as follows: extremity, 19 patients; trunk, 9 patients; head and neck, 7 patients; and retroperitoneum, 3 patients. The average tumor size was 10 cm. The margins after resection were as follows: grossly positive, 9; microscopically positive, 5; negative, 21; and unknown, 3. Patients with positive margins had a 22% survival rate, whereas those with negative or unknown margins had a 34% survival rate. Thirty-two patients achieved a complete response, 2 a partial response, and 4 progressed while on therapy. Twenty-six patients relapsed after a complete response (11 local, 10 distant, 5 both). Of the 15 patients with a distant relapse, 73% (11) relapsed in the lung. Twelve (32%) patients survived with an average follow-up of 14 years (range, 0.3 to 28 years). Nine patients were treated with chemotherapy, 9 with radiation, and 9 with both chemotherapy and radiation. Outcome was not significantly affected by gender, presence of neurofibromatosis, site, margin, or use of adjuvant therapy.


CONCLUSIONS
Neurofibrosarcoma remains a rare disease in children with insufficient contemporary numbers to assess efficacy of therapy. Prognosis remains poor with a high incidence of relapse, particularly in the lungs, suggesting that more aggressive therapies to control both local and distant relapses are needed.
Neville
Holly
H
M.D. Anderson Cancer Center and the University of Texas Medical School, Houston, Texas, USA.
Corpron
Cyndy
C
Blakely
Martin L
ML
Andrassy
Richard
R
D000293:Adolescent; D000328:Adult; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D008175:Lung Neoplasms / Q000453:epidemiology / Q000556:secondary; D008297:Male; D016609:Neoplasms, Second Primary / Q000453:epidemiology; D017253:Neurofibromatoses / Q000453:epidemiology; D018319:Neurofibrosarcoma / Q000453:epidemiology* / Q000556:secondary / Q000628:therapy; D011379:Prognosis; D018714:Radiotherapy, Adjuvant; D012189:Retrospective Studies; D012983:Soft Tissue Neoplasms / Q000453:epidemiology* / Q000628:therapy; D015996:Survival Rate; D013781:Texas / Q000453:epidemiology; D016896:Treatment Outcome