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

Ailing Zhao,1–3,* Daling Ding,4,* Xueqin Li,1–3 Jiangtao Wang1–3 1Department of Infant Ward, Children’s Hospital Affiliated of Zhengzhou University, Zhengzhou, Henan 450018, People’s Republic of China; 2Department of Infant Ward, Henan Children&r...

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Main Authors: Zhao A, Ding D, Li X, Wang J
Format: Article
Language:English
Published: Dove Medical Press 2019-12-01
Series:Cancer Management and Research
Subjects:
Online Access:https://www.dovepress.com/malignant-triton-tumor-in-a-child-case-report-and-literature-review-peer-reviewed-article-CMAR
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author Zhao A
Ding D
Li X
Wang J
author_facet Zhao A
Ding D
Li X
Wang J
author_sort Zhao A
collection DOAJ
description Ailing Zhao,1–3,* Daling Ding,4,* Xueqin Li,1–3 Jiangtao Wang1–3 1Department of Infant Ward, Children’s Hospital Affiliated of Zhengzhou University, Zhengzhou, Henan 450018, People’s Republic of China; 2Department of Infant Ward, Henan Children’s Hospital, Zhengzhou, Henan 450018, People’s Republic of China; 3Department of Infant Ward, Zhengzhou Children’s Hospital, Zhengzhou, Henan 450018, People’s Republic of China; 4Department of Neurosurgery, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan 450052, People’s Republic of China*These authors contributed equally to this workCorrespondence: Jiangtao WangDepartment of Infant Ward, Children’s Hospital Affiliated of Zhengzhou University, Zhengzhou, Henan 450018, People’s Republic of ChinaEmail syd2004@yeah.netObjective: 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.Keywords: malignant triton tumor, malignant peripheral nerve-sheath tumors, children, treatment strategies, prognosis
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spelling doaj.art-360859391eef4951bfc62932c60024d92022-12-22T01:59:57ZengDove Medical PressCancer Management and Research1179-13222019-12-01Volume 11107591076650681Malignant Triton Tumor in a Child: Case Report and Literature ReviewZhao ADing DLi XWang JAiling Zhao,1–3,* Daling Ding,4,* Xueqin Li,1–3 Jiangtao Wang1–3 1Department of Infant Ward, Children’s Hospital Affiliated of Zhengzhou University, Zhengzhou, Henan 450018, People’s Republic of China; 2Department of Infant Ward, Henan Children’s Hospital, Zhengzhou, Henan 450018, People’s Republic of China; 3Department of Infant Ward, Zhengzhou Children’s Hospital, Zhengzhou, Henan 450018, People’s Republic of China; 4Department of Neurosurgery, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan 450052, People’s Republic of China*These authors contributed equally to this workCorrespondence: Jiangtao WangDepartment of Infant Ward, Children’s Hospital Affiliated of Zhengzhou University, Zhengzhou, Henan 450018, People’s Republic of ChinaEmail syd2004@yeah.netObjective: 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.Keywords: malignant triton tumor, malignant peripheral nerve-sheath tumors, children, treatment strategies, prognosishttps://www.dovepress.com/malignant-triton-tumor-in-a-child-case-report-and-literature-review-peer-reviewed-article-CMARmalignant triton tumormalignant peripheral nerve-sheath tumorschildrentreatment strategiesprognosis
spellingShingle Zhao A
Ding D
Li X
Wang J
Malignant Triton Tumor in a Child: Case Report and Literature Review
Cancer Management and Research
malignant triton tumor
malignant peripheral nerve-sheath tumors
children
treatment strategies
prognosis
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 malignant triton tumor
malignant peripheral nerve-sheath tumors
children
treatment strategies
prognosis
url https://www.dovepress.com/malignant-triton-tumor-in-a-child-case-report-and-literature-review-peer-reviewed-article-CMAR
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AT lix malignanttritontumorinachildcasereportandliteraturereview
AT wangj malignanttritontumorinachildcasereportandliteraturereview