Criteria

World Health Organization (WHO) classification of tumors of the central nervous system (2021)[4]

Diagnosis is based on the pathologist's review of the gross specimen, the microscopic findings, and immunohistochemical staining.[4] In rare cases in which histopathology is not clearly diagnostic, tumor DNA/RNA sequencing and DNA methylation analysis can be used to confirm diagnosis and further classify the tumor molecular subgroup and clinical risk.[36]​​

Modified Chang classification[37][38]

No longer used alone but, particularly the M stage, can help inform treatment decisions and prognosis when considered alongside other factors including the age of patient, completeness of resection, histologic subtype, and genetic markers.​[39]

Primary tumor 

  • TX - Primary tumor cannot be assessed

  • T0 - No evidence of primary tumor

  • T1 - <3 cm in diameter

  • T2 - ≥3 cm with no evidence of extension

  • T3a - >3 cm with extension into aqueduct of Sylvius or foramen of Luschka

  • T3b - >3 cm with spread into brain stem

  • T4 - >3 cm with extension past aqueduct of Sylvius or foramen magnum.

Distant metastases

  • M0 - No dissemination

  • M1 - Microscopic tumor cells present in the cerebrospinal fluid (CSF)

  • M2 - Gross nodular seeding intracranially beyond the primary tumor site (cerebellum, cerebral subarachnoid space, or in the third or fourth ventricles)

  • M3 - Gross nodular seeding in the spinal subarachnoid space

  • M4 - Metastasis outside the cerebrospinal axis.

Clinical classification of medulloblastoma

Average-risk tumor

  • ages ≥3 years without evidence of metastatic spread and having ≤1.5 cm² (maximum cross-sectional area) of residual disease after surgery and no histologic or molecular high-risk factors (below).[3][40] 

High-risk tumor

  • Ages ≥3 years with evidence of CSF spread (M1-M3) and/or those with less complete resection (≥1.5 cm²) or ages <3 years at diagnosis.[40] 

  • Large cell/diffuse anaplastic histology (unclear whether this alone is a high-risk feature).

  • Tumor harbors MYC or MYCN amplification or TP53 mutation.[3]

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