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

Authors Zhao A, Ding D, Li X, Wang J

Received 27 June 2019

Accepted for publication 14 November 2019

Published 24 December 2019 Volume 2019:11 Pages 10759—10766

DOI https://doi.org/10.2147/CMAR.S221110

Checked for plagiarism Yes

Review by Single-blind

Peer reviewer comments 2

Editor who approved publication: Professor Nakshatri


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 work

Correspondence: Jiangtao Wang
Department of Infant Ward, Children’s Hospital Affiliated of Zhengzhou University, Zhengzhou, Henan 450018, People’s Republic of China
Email syd2004@yeah.net

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.

Keywords: malignant triton tumor, malignant peripheral nerve-sheath tumors, children, treatment strategies, prognosis


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