Pathology · CNS Pathology (Tumors, Degenerative, Infections)

A 5-year-old boy presents with morning headache and truncal ataxia. MRI reveals a posterior fossa mass compressing the fourth ventricle. Biopsy shows small round blue cells with Homer Wright rosets. Which molecular subgroup of this tumor portends the most favorable prognosis?

  • A WNT activation
  • B SHH activation
  • C MYC amplification
  • D Group 3
Correct answer: A. WNT activation

Explanation

The biopsy shows a medulloblastoma. WNT-activated medulloblastomas have the best prognosis, often presenting with classic histology and arising near the brainstem or cerebellar peduncle. Group 3 tumors and those with MYC amplification have the worst prognosis. SHH-activated tumors have an intermediate prognosis.

Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

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