Abstract
Congenital infantile fibrosarcomas (CIFS) is a rare tumor of childhood that can be diagnosed from birth to 15 years. It has a ratio of 3.74/100 000 children and is well defined nosological entity with a well-defined pathogenetic patterns: translocation (12, 15) (p13, q25) with fusion of the gene ETV6-NTRK3.
The differential diagnosis of upper CIFS in infants must be made with lymphatic malformations, and when associated with the Kasabach-Merritt phenomenon’s (disseminated intravascular coagulopathy), haemangiomas, emangioendotelioma kaposiforme. In 26% of cases is congenital, while in 63% is diagnosed in the first 5 years. Unlike fibrosarcomas of the adult is characterized by a low rate of metastasis and a high survival rate (90% at 5 years). 74% of cases is observed in the limbs (upper> lower, distal> proximal).
The treatment of choice should to be, where possible, limb salvage and the recurrences are variable between 17% and 43%. The purpose of this paper is to present a case of CIFS, the clinical features, the oncological treatment, the reconstructive solutions and functional results obtained after reconstruction. Case report.
The child (Z.A. female), was diagnosed with a neoplasia of soft tissues of the right forearm before birth.
At birth the child underwent a needle biopsy with a diagnosis of CIFS.
The patient received four cycles of chemotherapy with reduction tumor mass of more than 50% of volume.
At month four she underwent an exeresi with wide margings and sacrifice of the radial nerve.
The reconstruction required a free flap of re-innervated latissimus dorsi muscle. After 30 months from the surgical procedure the child is disease free and has recovered extesion of the muscles of the hand and fingers.
Correspondence should be addressed to Professor Stefan Bielack, Olgahospital, Klinikum Stuttgart, Bismarkstrasse 8, D-70176 Stuttgart, Germany. Email: s.bielack@klinikum_stuttgart.de
