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  • Title: Congenital intestinal fibrosarcoma with rapid recurrence requiring adjuvant chemotherapy.
    Author: See WSQ, Cheuk DKL, To KF, Ip PP, Chiang AKS, Ha SY, Chan GCF.
    Journal: Pediatr Int; 2017 Jun; 59(6):733-736. PubMed ID: 28436622.
    Abstract:
    A total of 16 cases of congenital fibrosarcoma have been reported from 1975 to March 2015. Five of the 16 had abnormal fusion between erythroblast transformation specific translocation variant 6 and neurotrophin recptor gene neurotrophic tyrosine kinase, receptor, type 3 (ETV6-NTRK3); in another five out of 16 this was absent, and six were not tested. All were managed by surgical resection but none involved metastasis. Herein we report the case of a newborn baby girl with congenital fibrosarcoma negative for ETV6-NTRK3 gene fusion, who presented with ileal perforation and positive resection margin. She had rapid recurrence with lymph node metastasis treated with postoperative chemotherapy. There was no further recurrence at >3 years of follow up.
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