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Published on: March 15, 2024
Pediatric Patients With SHH Medulloblastoma Fail Differently as Compared With Adults: Possible Implications for
Archya Dasgupta1, Tejpal Gupta1, Epari Sridhar2
1Departments of Radiation Oncology.
Purpose:
The purpose of this work was to study the diversity of sonic hedgehog (SHH) medulloblastoma across different age groups with an emphasis on patterns of relapse.
Methods:
All data for the study were obtained through review of medical records, imaging, radiation charts, treatment planning, and chemotherapy details.
Results:
Sixty-three patients with SHH medulloblastoma were identified from a prospectively maintained database and classified into 3 groups-infantile: ≤3 years (i-SHH, n=11); pediatric: >3 to <18 years (p-SHH, n=21); and adult: ≥18 years (a-SHH; n=31). Lateralized tumors were common with increasing age (81% a-SHH, 67% p-SHH, 27% i-SHH; P=0.01). Large cell anaplastic histology was relatively common for p-SHH (33%), while the nodular/desmoplastic variant was more frequent in i-SHH (64%) and adults (51%). Median follow-up was 38 months (range, 5 to 91 mo). Five-year event-free survival was 80%, 31%, and 52% for i-SHH, p-SHH, and a-SHH, respectively (P=0.001). Median time to failure for p-SHH and a-SHH were 12 and 36 months, respectively. For p-SHH, 83% were metastatic relapses compared with localized failure in 75% for a-SHH. Five-year overall survival for i-SHH, p-SHH, and a-SHH were 91%, 31%, and 70%, respectively (P=0.001). On univariate analysis, event-free survival was significantly worse for superiorly located tumors (P=0.01), nondesmoplastic histology (P=0.02), and histology alone for overall survival (P=0.04) (none on multivariate analysis).
Conclusions:
SHH medulloblastoma demonstrates varied outcomes depending on age, with p-SHH associated with early and metastatic relapses, while for a-SHH it tends to be delayed and localized.
Insights
Outcomes for sonic hedgehog (SHH) medulloblastoma vary by age. Pediatric patients experience early, metastatic relapses, while adult patients have delayed, localized recurrences, impacting survival rates.
Area of Science:
- Pediatric Oncology
- Neuro-oncology
- Cancer Genomics
Background:
- Medulloblastoma is the most common malignant brain tumor in children.
- Sonic hedgehog (SHH) medulloblastoma is a distinct molecular subtype with varying clinical behavior.
- Understanding age-related differences in SHH medulloblastoma is crucial for optimizing treatment strategies.
Purpose of the Study:
- To investigate the diversity of sonic hedgehog (SHH) medulloblastoma across infantile, pediatric, and adult age groups.
- To analyze age-specific patterns of tumor characteristics and relapse in SHH medulloblastoma.
- To evaluate the impact of age on event-free and overall survival in SHH medulloblastoma patients.
Main Methods:
- Retrospective review of medical records, imaging, and treatment details for 63 SHH medulloblastoma patients.
- Classification of patients into infantile (≤3 years), pediatric (>3 to <18 years), and adult (≥18 years) groups.
- Statistical analysis of tumor characteristics, relapse patterns, and survival outcomes across age groups.
Main Results:
- Lateralized tumors and large cell anaplastic histology were more common in older patients.
- Five-year event-free survival was significantly lower in pediatric patients (31%) compared to infantile (80%) and adult (52%) groups.
- Pediatric SHH medulloblastoma showed early, metastatic relapses, whereas adult SHH medulloblastoma exhibited delayed, localized failures.
Conclusions:
- Age is a significant factor influencing the clinical presentation and outcomes of SHH medulloblastoma.
- Pediatric SHH medulloblastoma is associated with poorer prognosis and distinct relapse patterns.
- Tailored therapeutic approaches considering age-specific characteristics are warranted for SHH medulloblastoma.
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