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Congenital Infantile Fibrosarcoma A Multimodality Therapeutic


									  Congenital/ Infantile Fibrosarcoma of the Hand: A Multimodality Therapeutic
                        Approach Aimed At Limb Salvage.

       Sabino Zani MD, Christine Finck MD, Steven Nelson, Alan Babigian MD.

                         Connecticut Children’s Medical Center
                                     Hartford, CT
                              University of Connecticut
                                   Farmington, CT

        Characterized by an abundance of uniform spindle shape cells arranged in bundles
and fascicles, congenital or infantile fibrosarcoma morphologically resembles that of
adult fibrosarcoma. This is a very rare tumor that occurs most commonly in the
extremity and has a local recurrence rate of up to 32%, with metastatic rates of 8%.
Treatment options range from wide local excision with or without adjuvant chemotherapy
to limb amputation. Tumor size and extension into surrounding tissues can make wide
local excision complicated. The addition of neoadjuvant chemotherapy offers the
potential to reduce tumor size allowing for a greater success at limb salvage.
        We present a case of a 3 month old boy born with a large mass on the dorsum of
his right hand. Initial biopsy was consistent with infantile fibrosarcoma. Neoadjuvant
chemotherapy consisted of Vincristine, Cytoxan, and Dactinomycin. Radical excision of
the infantile fibrosarcoma was achieved with primary closure using a dorsal forearm
rotation flap for coverage. The patient has since healed well and maintains good function
in the affected hand. At one year postoperative, there is no evidence of recurrence.

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