Advances in the adjuvant treatment of infantile fibrosarcoma

Infantile fibrosarcoma is a rare soft tissue tumor, predominately affecting young infants. It grows rapidly and is locally infiltrative but rarely metastases. Complete surgical removal is usually curative but is impossible in some patients and would result in significant functional or cosmetic consequences in many others. Neoadjuvant chemotherapy will cause many tumors to shrink significantly, allowing less mutilating surgical resections to be performed--this is the current recommendation where immediate surgical removal cannot be accomplished without unacceptable morbidity. In contrast, there is no defined role for adjuvant chemotherapy or radiation following complete surgical resection. Although there is a significant risk of local recurrence, most of these can be successfully treated with further surgery and the overall survival rate exceeds 90%.