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Malignant Triton Tumor in a Child: Case Report and Literature Review

OBJECTIVE: Malignant triton tumor (MTT) is a rare and devastating malignant peripheral nerve-sheath tumor, which shows rapid growth and poor clinical outcomes. Here, we reported a 2-year-old girl who was diagnosed as MTT, an overview of the literature was conducted to discuss the clinical features a...

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Autores principales: Zhao, Ailing, Ding, Daling, Li, Xueqin, Wang, Jiangtao
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Dove 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6935315/
https://www.ncbi.nlm.nih.gov/pubmed/31920385
http://dx.doi.org/10.2147/CMAR.S221110
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author Zhao, Ailing
Ding, Daling
Li, Xueqin
Wang, Jiangtao
author_facet Zhao, Ailing
Ding, Daling
Li, Xueqin
Wang, Jiangtao
author_sort Zhao, Ailing
collection PubMed
description OBJECTIVE: Malignant triton tumor (MTT) is a rare and devastating malignant peripheral nerve-sheath tumor, which shows rapid growth and poor clinical outcomes. Here, we reported a 2-year-old girl who was diagnosed as MTT, an overview of the literature was conducted to discuss the clinical features and optimal treatment strategies of MTT. METHODS: We conducted an analysis of 42 patients from the PubMed, Medline, Embase and Web of Science databases for relevant articles published between 1938 and 2018. RESULTS: A 2-year-old girl died of tumor recurrence. Forty-two eligible cases of MTT in children (birth to 18 years; mean age, 8.3 years), the highest frequency of occurrence was in 12–16 years; and the male-to-female ratio was 1.7:1. Only 33 provided complete treatment details: 11 patients received treatment by surgery alone; 2 received both surgery and chemotherapy; 4 received both surgery and radiation therapy; 14 received surgery, chemotherapy, and radiation therapy; 1 case received chemotherapy and radiation therapy; and only 1 received supportive care. In the 33 cases, the average OS and 5-year OS probability were 23.9 months (range, 0.3–156 months) and 27.5 ± 4.3%. There were significant differences between radiation therapy and patient survival (p<0.05), postoperative chemotherapy/radiation therapy and patient prognosis (p<0.05). CONCLUSION: The clinical and histopathological features and therapeutic options for MTT are discussed in the light of published data. Further studies are needed to improve survival in children with this rare malignant tumor.
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spelling pubmed-69353152020-01-09 Malignant Triton Tumor in a Child: Case Report and Literature Review Zhao, Ailing Ding, Daling Li, Xueqin Wang, Jiangtao Cancer Manag Res Case Report OBJECTIVE: Malignant triton tumor (MTT) is a rare and devastating malignant peripheral nerve-sheath tumor, which shows rapid growth and poor clinical outcomes. Here, we reported a 2-year-old girl who was diagnosed as MTT, an overview of the literature was conducted to discuss the clinical features and optimal treatment strategies of MTT. METHODS: We conducted an analysis of 42 patients from the PubMed, Medline, Embase and Web of Science databases for relevant articles published between 1938 and 2018. RESULTS: A 2-year-old girl died of tumor recurrence. Forty-two eligible cases of MTT in children (birth to 18 years; mean age, 8.3 years), the highest frequency of occurrence was in 12–16 years; and the male-to-female ratio was 1.7:1. Only 33 provided complete treatment details: 11 patients received treatment by surgery alone; 2 received both surgery and chemotherapy; 4 received both surgery and radiation therapy; 14 received surgery, chemotherapy, and radiation therapy; 1 case received chemotherapy and radiation therapy; and only 1 received supportive care. In the 33 cases, the average OS and 5-year OS probability were 23.9 months (range, 0.3–156 months) and 27.5 ± 4.3%. There were significant differences between radiation therapy and patient survival (p<0.05), postoperative chemotherapy/radiation therapy and patient prognosis (p<0.05). CONCLUSION: The clinical and histopathological features and therapeutic options for MTT are discussed in the light of published data. Further studies are needed to improve survival in children with this rare malignant tumor. Dove 2019-12-24 /pmc/articles/PMC6935315/ /pubmed/31920385 http://dx.doi.org/10.2147/CMAR.S221110 Text en © 2019 Zhao et al. http://creativecommons.org/licenses/by-nc/3.0/ This work is published and licensed by Dove Medical Press Limited. The full terms of this license are available at https://www.dovepress.com/terms.php and incorporate the Creative Commons Attribution – Non Commercial (unported, v3.0) License (http://creativecommons.org/licenses/by-nc/3.0/). By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. For permission for commercial use of this work, please see paragraphs 4.2 and 5 of our Terms (https://www.dovepress.com/terms.php).
spellingShingle Case Report
Zhao, Ailing
Ding, Daling
Li, Xueqin
Wang, Jiangtao
Malignant Triton Tumor in a Child: Case Report and Literature Review
title Malignant Triton Tumor in a Child: Case Report and Literature Review
title_full Malignant Triton Tumor in a Child: Case Report and Literature Review
title_fullStr Malignant Triton Tumor in a Child: Case Report and Literature Review
title_full_unstemmed Malignant Triton Tumor in a Child: Case Report and Literature Review
title_short Malignant Triton Tumor in a Child: Case Report and Literature Review
title_sort malignant triton tumor in a child: case report and literature review
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6935315/
https://www.ncbi.nlm.nih.gov/pubmed/31920385
http://dx.doi.org/10.2147/CMAR.S221110
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