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Relapse patterns in pediatric embryonal central nervous system tumors.

Sébastien Perreault¹, Robert M Lober, Anne-Sophie Carret

¹Division of Child Neurology, Department of Neurology, Lucile Packard Children's Hospital at Stanford, Stanford University, 750 Welch Road, Suite 317, Palo Alto, CA, 94304, USA, sebperreault@hotmail.com.

Journal of Neuro-Oncology

|August 8, 2013

Summary

Central nervous system (CNS) embryonal tumors show distinct relapse patterns. Medulloblastoma varies, supratentorial PNETs recur locally, and pineoblastomas spread diffusely, often involving the spine.

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Area of Science:

Pediatric Neuro-oncology
Central Nervous System (CNS) Tumors
Cancer Recurrence Patterns

Background:

Embryonal CNS tumors were historically grouped as primitive neuroectodermal tumors (PNET) due to shared histology.
This grouping led to similar treatment approaches, potentially overlooking tumor-specific behaviors.
Understanding distinct relapse patterns is crucial for refining treatment strategies.

Purpose of the Study:

To investigate and compare the relapse patterns of specific embryonal CNS tumors.
To identify differences in recurrence sites and frequencies among medulloblastoma, supratentorial PNET (sPNET), pineoblastoma (PB), and atypical teratoid/rhabdoid tumor (ATRT).

Main Methods:

Historical cohort study of pediatric patients (≤21 years) diagnosed with embryonal CNS tumors between 2000-2011.
Inclusion criteria: diagnosis of medulloblastoma, sPNET, PB, or ATRT with at least one surveillance MRI.
Analysis of relapse patterns, including local recurrence, distant recurrence, and leptomeningeal disease.

Main Results:

Of 133 patients, 49 (37%) relapsed. sPNET relapses were predominantly local (79%).
Pineoblastoma relapses (100%) were characterized by diffuse leptomeningeal disease.
Medulloblastoma showed diverse relapse patterns: 27% local, 35% distant, 38% leptomeningeal. Spinal involvement frequency varied significantly (p < 0.001) by tumor type.

Conclusions:

Embryonal CNS tumors exhibit divergent recurrence patterns.
Relapse characteristics differ significantly between medulloblastoma, sPNET, PB, and ATRT.
Findings suggest potential for upfront treatment stratification based on distinct relapse patterns.