Malignant fibrous histiocytoma in childhood

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Abstract
Background. Malignant fibrous histiocytoma (MFH) is a neoplasm of late adult life and often is reported in the pediatric population. It is thought to behave more benignly in children than in adults. Method. Clinical and pathologic features, treatment, and outcome of nine pediatric patients seen at British Columbia's Children's Hospital between 1983 and 1990 were examined. The literature regarding pediatric malignant fibrous histiocytoma was reviewed. Results. The tumors included one primary renal tumor and two occurring in the orbit after radiation therapy for retinoblastoma during the neonatal period. Histologic examination showed that six tumors had a stori-form-pleomorphic pattern, one was myxoid, and two were angiomatoid in type. Six children are alive with a disease-free survival of 20 months to 8 years after surgical resection. Two of these received adjuvant chemotherapy, none had radiation therapy. Three patients have died of disease, two with pulmonary metastases that developed despite multiagent chemotherapy and radiation therapy. Poor outcome was associated with large tumors, deep and proximal location, and the storiform-pleomorphic histologic type with atypical mitoses. Conclusion. Malignant fibrous histiocytoma is similar in children and adults. Surgery is the mainstay of therapy, but the risk of local recurrence and pulmonary metastases indicates the need for adjuvant therapy in selected patients.