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Management and Outcomes of Children with Malignant Germ Cell Tumor
Kaushal Kulkarni1, Sandeep Agarwala2, Vishesh Jain1
1Department of Pediatric Surgery, AIIMS, New Delhi, 110029, India.
Insights
Extracranial malignant germ cell tumors (MGCTs) in children show favorable outcomes, with a 5-year overall survival of 84.7%. Advanced stages (III/IV) and extra-gonadal sites predict poorer event-free survival (EFS).
Area of Science:
- Pediatric Oncology
- Oncology
- Clinical Research
Background:
- Extracranial malignant germ cell tumors (MGCTs) are rare in children.
- Understanding clinico-pathological features and outcomes is crucial for effective management, especially in resource-challenged settings.
Purpose of the Study:
- To evaluate the clinico-pathological characteristics, treatment strategies, and outcomes of pediatric extracranial MGCTs.
- To identify factors influencing survival in children treated at a tertiary care center in a resource-limited environment.
Main Methods:
- A prospective analysis of 177 children under 14 years treated for extracranial MGCT from 1994 to 2023.
- Data collected on patient demographics, tumor characteristics, management (including neoadjuvant chemotherapy and surgery), event-free survival (EFS), and overall survival (OS).
- Multivariate logistic regression used to determine predictors of unfavorable outcomes.
Main Results:
- The cohort included 177 children, with endodermal sinus tumor being the most common subtype (73%).
- Metastatic disease at presentation occurred in 27% of cases, with lungs being the most frequent site.
- Five-year OS was 84.7% and 5-year EFS was 69.9%. Stage III/IV disease and extra-gonadal sites were significantly associated with poorer EFS.
Conclusions:
- Pediatric extracranial MGCTs demonstrate a favorable prognosis with high survival rates.
- Advanced disease stages (III and IV) and extra-gonadal tumor locations are independent predictors of unfavorable outcomes, necessitating tailored treatment approaches.
Objectives:
To assess the clinico-pathological features, management and outcomes, amongst extracranial malignant germ cell tumors (MGCTs) in children treated primarily at a tertiary care center in a resource-challenged nation.
Methods:
The prospectively maintained data for children below 14 y of age treated for extracranial MGCT from May 1994 to January 2023 was analyzed for patient characteristics, management, event-free survival (EFS) and overall survival (OS) and factors effecting survival. Events was defined as death, recurrence and progression. Multivariate logistic regression analysis was performed to identify the factors independently predicting unfavorable outcomes.
Results:
One hundred and seventy-seven children (37% males) with a median (IQR) age at presentation of 30 mo (range 2-168 mo) were included. The cohort consisted of 87 (49%) extra-gonadal and 90 (51%) gonadal cases. Disease was metastatic at presentation in 48 (27%) with lungs being the most common site. Neoadjuvant chemotherapy (NACT) was given to 119 (67%) and finally 162/177 (92%) had undergone resection of the primary tumor. Endodermal sinus tumor (EST) was the commonest histological subtype in 141 children (73%). Twenty-two (12%) patients had died giving a 5-y OS of 84.7% (95% CI 78.3- 91.1). Recurrence occurred in 25 patients, and an additional 5 patients had progression giving a 5-y EFS of 69.9% (95% CI 62.5- 77.3). Stage III (p = 0.05), Stage IV (p = 0.006) and extra-gonadal site (p = 0.05) were significantly associated with poorer EFS.
Conclusions:
Children with MGCT have a favorable outcome with 5-y OS of 84.7% and EFS of 69.9%. Stage III and IV disease and extra-gonadal sites were independent predictors of a poor outcome.
