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- Title
Congenital intestinal fibrosarcoma with rapid recurrence requiring adjuvant chemotherapy.
- Authors
See, Wing Shan Queenie; Cheuk, Daniel Ka Leung; To, Ka Fai; Ip, Philip Pun Ching; Chiang, Alan Kwok Shing; Ha, Shau Yin; Chan, Godfrey Chi Fung
- 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.
- Subjects
CANCER relapse; COMBINED modality therapy; CONNECTIVE tissue tumors; INTESTINAL tumors; GENETICS
- Publication
Pediatrics International, 2017, Vol 59, Issue 6, p733
- ISSN
1328-8067
- Publication type
Article
- DOI
10.1111/ped.13252