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Inflammatory Myofibroblastic Tumors in Children: A Clinical Retrospective Study on 19 Cases
Background: Inflammatory myofibroblastic tumor (IMFT) is a rare neoplasm mainly affecting children and young adults. We conducted a retrospective study to evaluate the clinical features and treatment alternatives of childhood inflammatory myofibroblastic tumors. Methods: A total of 19 patients who w...
Autores principales: | , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Frontiers Media S.A.
2021
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8295553/ https://www.ncbi.nlm.nih.gov/pubmed/34307241 http://dx.doi.org/10.3389/fped.2021.543078 |
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author | Da, Min Qian, Bo Mo, Xuming Xu, Cheng Wu, Haiyan Jiang, Bin Peng, Wei Qi, Jirong Sun, Jian Wu, Kaihong |
author_facet | Da, Min Qian, Bo Mo, Xuming Xu, Cheng Wu, Haiyan Jiang, Bin Peng, Wei Qi, Jirong Sun, Jian Wu, Kaihong |
author_sort | Da, Min |
collection | PubMed |
description | Background: Inflammatory myofibroblastic tumor (IMFT) is a rare neoplasm mainly affecting children and young adults. We conducted a retrospective study to evaluate the clinical features and treatment alternatives of childhood inflammatory myofibroblastic tumors. Methods: A total of 19 patients who were pathologically diagnosed with IMT between December 2008 and October 2018 were included. Collected data were demographic information, main complaints, tumor characteristics, treatment, pathological results, immunohistochemical analysis, and prognosis. Results: The male/female ratio was 13:6. The mean age at disease onset was 44.9 ± 33.9 months (range 4 to 111 months). The mean tumor size was 6.5 ± 4.0 cm (range 1.2 to 17.0 cm). The most common site was the abdomen (13/19). The most commonly used detection tool was CT. Eleven patients (57.9%) had aggressive tumor growth, including eight receiving extensive resection and three receiving palliative resection due to high local invasiveness and postoperative chemotherapy. Eight cases whose tumors were completely enveloped received complete resection. Immunohistochemistry was performed for 17 patients and ALK positivity was found in 11 patients. Despite three children lost to follow-up, sixteen patients were followed up for 6 to 132 months (average 63.9 months, median 66 months). Of which, twelve children survived with no evidence of IMT, and four cases (21%) showed local recurrences (two of them died). No distant metastasis was detected. Conclusions: IMT is rare in children with various locations, mostly appearing in the abdomen. Whether the tumor could be completely removed, the location and the invasiveness of surrounding tissues might be highly prognosis-related. |
format | Online Article Text |
id | pubmed-8295553 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
spelling | pubmed-82955532021-07-23 Inflammatory Myofibroblastic Tumors in Children: A Clinical Retrospective Study on 19 Cases Da, Min Qian, Bo Mo, Xuming Xu, Cheng Wu, Haiyan Jiang, Bin Peng, Wei Qi, Jirong Sun, Jian Wu, Kaihong Front Pediatr Pediatrics Background: Inflammatory myofibroblastic tumor (IMFT) is a rare neoplasm mainly affecting children and young adults. We conducted a retrospective study to evaluate the clinical features and treatment alternatives of childhood inflammatory myofibroblastic tumors. Methods: A total of 19 patients who were pathologically diagnosed with IMT between December 2008 and October 2018 were included. Collected data were demographic information, main complaints, tumor characteristics, treatment, pathological results, immunohistochemical analysis, and prognosis. Results: The male/female ratio was 13:6. The mean age at disease onset was 44.9 ± 33.9 months (range 4 to 111 months). The mean tumor size was 6.5 ± 4.0 cm (range 1.2 to 17.0 cm). The most common site was the abdomen (13/19). The most commonly used detection tool was CT. Eleven patients (57.9%) had aggressive tumor growth, including eight receiving extensive resection and three receiving palliative resection due to high local invasiveness and postoperative chemotherapy. Eight cases whose tumors were completely enveloped received complete resection. Immunohistochemistry was performed for 17 patients and ALK positivity was found in 11 patients. Despite three children lost to follow-up, sixteen patients were followed up for 6 to 132 months (average 63.9 months, median 66 months). Of which, twelve children survived with no evidence of IMT, and four cases (21%) showed local recurrences (two of them died). No distant metastasis was detected. Conclusions: IMT is rare in children with various locations, mostly appearing in the abdomen. Whether the tumor could be completely removed, the location and the invasiveness of surrounding tissues might be highly prognosis-related. Frontiers Media S.A. 2021-07-08 /pmc/articles/PMC8295553/ /pubmed/34307241 http://dx.doi.org/10.3389/fped.2021.543078 Text en Copyright © 2021 Da, Qian, Mo, Xu, Wu, Jiang, Peng, Qi, Sun and Wu. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. |
spellingShingle | Pediatrics Da, Min Qian, Bo Mo, Xuming Xu, Cheng Wu, Haiyan Jiang, Bin Peng, Wei Qi, Jirong Sun, Jian Wu, Kaihong Inflammatory Myofibroblastic Tumors in Children: A Clinical Retrospective Study on 19 Cases |
title | Inflammatory Myofibroblastic Tumors in Children: A Clinical Retrospective Study on 19 Cases |
title_full | Inflammatory Myofibroblastic Tumors in Children: A Clinical Retrospective Study on 19 Cases |
title_fullStr | Inflammatory Myofibroblastic Tumors in Children: A Clinical Retrospective Study on 19 Cases |
title_full_unstemmed | Inflammatory Myofibroblastic Tumors in Children: A Clinical Retrospective Study on 19 Cases |
title_short | Inflammatory Myofibroblastic Tumors in Children: A Clinical Retrospective Study on 19 Cases |
title_sort | inflammatory myofibroblastic tumors in children: a clinical retrospective study on 19 cases |
topic | Pediatrics |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8295553/ https://www.ncbi.nlm.nih.gov/pubmed/34307241 http://dx.doi.org/10.3389/fped.2021.543078 |
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