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From the archives of the AFIP: medulloblastoma: a comprehensive review with radiologic-pathologic correlation
Kelly K Koeller1, Elisabeth J Rushing
1Department of Radiologic Pathology, Armed Forces Institute of Pathology, 14th St at Alaska Ave, Bldg 54, Washington, DC 20306-6000, USA. koeller@afip.osd.mil
Insights
Medulloblastoma, a common pediatric brain tumor, is highly malignant but treatable. Advances in surgery, radiation, and chemotherapy improve survival rates, though metastasis and recurrence remain challenges.
Area of Science:
- Pediatric Oncology
- Neuro-oncology
- Medical Imaging
Background:
- Medulloblastoma is the most common pediatric central nervous system malignancy.
- It predominantly affects males under 10, arising in the cerebellum.
- Adult cases occur but are less common.
Purpose of the Study:
- To provide a comprehensive overview of medulloblastoma.
- To discuss diagnostic imaging characteristics.
- To highlight current treatment outcomes and challenges.
Main Methods:
- Review of clinical presentation and epidemiology.
- Description of computed tomographic (CT) and magnetic resonance (MR) imaging findings.
- Summary of current treatment modalities including surgery, radiation, and chemotherapy.
Main Results:
- 5-year survival rates exceed 50% due to multimodal therapy.
- Leptomeningeal metastasis is present in 33% of cases at diagnosis.
- Imaging shows characteristic hyperattenuation and heterogeneity.
Conclusions:
- Medulloblastoma treatment has improved significantly, but cure remains challenging due to metastasis and recurrence.
- Advanced MR imaging is crucial for evaluating disease spread.
- Ongoing research is vital for improving outcomes and achieving a cure.
Abstract:
Medulloblastoma is the most common pediatric central nervous system malignancy and the most common primary tumor of the posterior fossa in children. This highly malignant neoplasm occurs more frequently in males and usually before 10 years of age. Clinical symptoms and signs are generally brief, typically less than 3 months in duration, and reflect the strong predilection of this tumor to arise within the cerebellum, most often in the vermis. Although much less common, the disease may also occur in adults, usually in the 3rd and 4th decades of life. Surgical resection, radiation therapy, and chemotherapy have substantially lowered the mortality associated with this tumor, with 5-year survival rates now commonly well above 50%. Still, both dissemination at the time of diagnosis and recurrence remain obstacles in achieving a cure. The tumor has characteristic hyperattenuation on unenhanced computed tomographic scans that reflects the high nuclear-cytoplasmic ratio seen at histologic analysis. The tumor typically appears heterogeneous on images, findings that are related to cyst formation, hemorrhage, and calcification and that are even more pronounced with magnetic resonance (MR) imaging. Evidence of leptomeningeal metastatic spread is present in 33% of all cases at the time of diagnosis and is well evaluated with contrast-enhanced MR imaging of the brain and the spine. Although controversial, postoperative surveillance with MR imaging is performed at most institutions in the hope of facilitating a better outcome. With continued research, treatment of these common neoplasms should improve, perhaps even achieving a cure in the future.
