Congenital intestinal fibrosarcoma with rapid recurrence requiring adjuvant chemotherapy

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 fiv...

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Bibliographic Details
Published in:Pediatrics international 2017-06, Vol.59 (6), p.733-736
Main 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
Format: Article
Language:English
Subjects:
Antineoplastic Agents - therapeutic use
Antineoplastic Combined Chemotherapy Protocols - therapeutic use
Chemotherapy
Chemotherapy, Adjuvant
Colectomy
congenital fibrosarcoma
Female
Fibrosarcoma
Fibrosarcoma - congenital
Fibrosarcoma - drug therapy
Fibrosarcoma - pathology
Fibrosarcoma - surgery
Gene fusion
Genetic transformation
Humans
Ileal Neoplasms - congenital
Ileal Neoplasms - drug therapy
Ileal Neoplasms - pathology
Ileal Neoplasms - surgery
Infant, Newborn
intestinal
Intestine
Intestine, Small - surgery
Jejunal Neoplasms - congenital
Jejunal Neoplasms - drug therapy
Jejunal Neoplasms - pathology
Jejunal Neoplasms - surgery
Lymph
Lymph nodes
Lymphatic Metastasis
Metastases
Metastasis
Neoplasm Recurrence, Local - drug therapy
Neoplasm Recurrence, Local - pathology
Neoplasm Recurrence, Local - surgery
NTRK3 gene
Pediatrics
Protein-tyrosine kinase
rare tumor
Translocation
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Summary: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.
ISSN:1328-8067
1442-200X
DOI:10.1111/ped.13252