Medulloblastoma with Homer-Wright Rosettes
The histopathology will most likely show small, round, blue cells with Homer-Wright rosettes, consistent with medulloblastoma—the most common malignant pediatric brain tumor presenting in the posterior fossa with these exact clinical features. 1
Clinical Presentation Analysis
This 4-year-old boy presents with the classic triad of posterior fossa tumor symptoms:
- Morning headaches with vomiting indicate elevated intracranial pressure from cerebrospinal fluid obstruction 1
- Ataxia (stumbling) reflects cerebellar dysfunction from a posterior fossa mass 1
- Diplopia with lateral rectus palsy represents a false localizing sign from elevated intracranial pressure causing sixth cranial nerve compression 1, 2
- Papilledema confirms increased intracranial pressure 1, 2
The 4-month progressive course with symptoms of raised intracranial pressure (headache, vomiting), followed by cerebellar signs (ataxia), and cranial nerve involvement is pathognomonic for medulloblastoma in this age group 1.
Histopathological Features of Medulloblastoma
Medulloblastoma demonstrates the following characteristic microscopic findings:
- Small, round, blue cells with hyperchromatic nuclei and scant cytoplasm arranged in sheets 3, 4
- Homer-Wright rosettes (neuroblastic rosettes with cells arranged around a central neuropil core without a true lumen) 5
- High mitotic activity reflecting the WHO grade IV designation 4, 6
- Undifferentiated neural cells with evidence of neuronal differentiation 4
- Areas of necrosis may be present in high-grade tumors 4
Why Other Options Are Incorrect
CD20-positive neoplastic B cell lymphocytes would indicate CNS lymphoma, which is exceedingly rare in immunocompetent children and typically presents in older adults or immunocompromised patients 1
Psammoma bodies are calcified concretions characteristic of meningioma, which accounts for only a small fraction of pediatric brain tumors, typically presents in adolescents rather than young children, and is usually supratentorial rather than in the posterior fossa 1
Pyogenic abscess with central necrosis and rim of gliosis would present with fever, systemic signs of infection, and a different imaging appearance with ring enhancement—not consistent with this 4-month progressive course 1
Spindle cells in a collagen-rich matrix describe meningioma histology, which again is uncommon in this age group and location 1
Molecular and Prognostic Context
Medulloblastoma represents 60% of childhood intracranial embryonal tumors and is the most common malignant pediatric brain tumor 3. Modern classification recognizes four molecular subgroups (WNT, SHH, Group 3, and Group 4), each with distinct prognosis and treatment implications 7, 6. The 5-year survival rate is approximately 75% overall, though high-risk patients (age <3 years, subtotal resection, metastatic disease) have worse outcomes 3.
Standard treatment includes maximal safe surgical resection followed by craniospinal radiation (for children ≥3 years) plus chemotherapy 1. For this 4-year-old, clinical trial enrollment is preferred, or standard brain radiation with concurrent and adjuvant temozolomide 1.
Critical Management Points
Early genetic testing should be considered, as certain cancer predisposition syndromes (Li-Fraumeni, Turcot syndrome/CMMRD) are associated with medulloblastoma 1. CSF evaluation for disseminated disease is essential, as medulloblastomas frequently spread through cerebrospinal fluid pathways 1.