MALIGNANT FIBROUS HISTIOCYTOMA IN CHILDREN

Because malignant fibrous histiocytoma (MFH) rarely occurs in children , the natural history of this tumor and prognostic factors predictive of outcome have not been well described. The charts of all pediatric p atients with MFH seen at M.D. Anderson Cancer Center were reviewed wit h respect presentation, treatment, and outcome, in an attempt to deter mine prognostic factors that are predictive of survival. Forty-four pe diatric patients were identified. Extremities were the most common tum or site (31 of 44 patients). Five patients presented with angiomatoid histology subtype; all subsequently survived. The estimated ii-year su rvival rate was 85% for clinical group I patients, 87% for clinical gr oup II, 53% for clinical group III, and 0% for clinical group IV. The estimated 5-year survival rate was 95% for patients with tumors of les s than 5 cm in diameter and 45% for those with larger tumors. Overall, the estimated 5-year survival rate was 71%. Significant prognostic fa ctors found to affect survival (by univariate analysis) were clinical group, tumor size, and recurrence. Gender and race were not significan t predictors. The use of chemotherapy and radiation was not found to i mprove the chance of survival, but this most likely reflected the more frequent use of adjuvant therapy in patients with unresectable or hig h grade tumors. Although adequate surgical resection continues to be t he most effective treatment, investigation of adjuvant chemotherapy an d radiation therapy on protocol is warranted. Copyright (C) 1996 by W. B. Saunders Company