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Published on: August 25, 2021
Malignant Peripheral Nerve Sheath Tumors in Children and Adolescents: A Population-Based Study
Hongjun Wu1, Zhenqi Liao2, Yinuo Wu3
1Department of Pediatric Surgery, Hangzhou Linping District Maternal & Child Health Care Hospital, Hangzhou, Zhejiang, China.
Insights
Prognosis for pediatric malignant peripheral nerve sheath tumors (MPNSTs) is challenging. Early diagnosis and surgical intervention are key to improving survival rates in these rare pediatric cancers.
Area of Science:
- Pediatric Oncology
- Cancer Epidemiology
- Tumor Prognosis
Background:
- Malignant peripheral nerve sheath tumors (MPNSTs) are rare and aggressive pediatric cancers.
- Understanding prognostic factors is crucial for improving outcomes in pediatric MPNSTs.
Purpose of the Study:
- To analyze the prognosis of pediatric MPNSTs using the SEER database.
- To identify significant prognostic factors influencing survival in pediatric MPNSTs patients.
Main Methods:
- Retrospective cohort analysis of pediatric MPNSTs cases (2000-2019) from the SEER database.
- Kaplan-Meier and Cox proportional hazards models were used to assess overall survival (OS).
- A nomogram was developed for OS prediction in pediatric MPNSTs.
Main Results:
- The study analyzed 208 pediatric MPNSTs cases; 5-year OS was 59.9%.
- Surgical treatment was associated with better survival outcomes compared to no surgery.
- SEER stage at diagnosis was identified as the sole independent predictor of OS.
Conclusions:
- Pediatric MPNSTs have a difficult prognosis, heavily influenced by SEER stage.
- Surgery is a critical first-line treatment, particularly for localized tumors.
- Early diagnosis and surgical management are vital for improving pediatric MPNSTs survival.
Objective:
This study aims to analyze the prognosis of malignant peripheral nerve sheath tumors (MPNSTs) in children using the Surveillance, Epidemiology, and End Results (SEER) database to identify significant prognostic factors affecting survival.
Methods:
A retrospective cohort analysis was conducted using the SEER database, identifying pediatric patients diagnosed with MPNSTs from 2000 to 2019. Demographic data, tumor characteristics, treatment modalities, and survival outcomes were extracted and analyzed. The main outcome measure was overall survival (OS), analyzed using Kaplan-Meier methods and Cox proportional hazards models to assess the impact of clinical and demographic factors on survival. Furthermore, we constructed a nomogram to predict OS in pediatric MPNSTs patients.
Results:
The study included 208 pediatric patients with MPNSTs, with a near-equal distribution across gender and a majority being white. The most common sites for MPNSTs were the trunk and core areas (38.9%) and the limbs (34.1%). A majority of the patients (87.0%) underwent surgical treatment, and radiation therapy was administered to 44.7% of the patients. The overall 5-year survival rate for the entire cohort was 59.9%. When compared to no surgery, surgery had better survival outcomes. According to the results of Cox proportional hazard regression, only the SEER stage was an important independent predictor of OS in this model.
Conclusions:
Pediatric MPNSTs presents with a challenging prognosis, significantly influenced by the SEER stage at diagnosis. Surgery is crucial as first-line treatment for MPNSTs, especially if the tumor is localized at diagnosis.
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