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

Autor: Ailing Zhao, Daling Ding, Xueqin Li, Jiangtao Wang
Rok vydání: 2019
Předmět:
Zdroj: Cancer Management and Research. 11:10759-10766
ISSN: 1179-1322
DOI: 10.2147/cmar.s221110
Popis: 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
Databáze: OpenAIRE