| 摘要 |
Although the most common soft tissue sarcoma of childhood is rhab-domyosarcoma, 47% of all soft tissue sarcomas in children have a histology other than rhabdomyosarcoma. These tumors make up 3% of all tumors in children; thus, a better understanding of the biology and treatment of this heterogeneous group of individually uncommon but cumulatively impor-tant tumors is needed. Because soft tissue sarcomas more commonly occur in adults, much of the information about their treatment comes from adult studies. In some specific circumstances, however, the prognosis of an in-dividual soft tissue sarcoma in children is much better than its counterpart in adults, resulting in markedly different treatment recommendations. The difference in prognosis is most pronounced for infants and younger children whose tumors often have a benign behavior and execllent prognosis with surgery alone. Although malignant fibrous histiocytoma (MFH) was
the most common histologic diagnosis in adult patients with extremity
sarcomas, it is much less common in children. Fewer than 5% of all MFHs
occur in children, and previous reports have not reviewed their patients
according to age. Thus, although occasional case reports of MFH in children
have appeared, no series has delineated its natural history. During a 6-year
period, we have treated five patients less than 18 years of age. Wide local
excision were performed in all patient without radiotherapy and/or chemo-
therapy. All patients remain free of local recurrence or distant metastasis
with a median follow-up in excess of 3 years. The purpose of this report is
to review our results of treatment of children with malignant fibrous histiocytomas. |
