Medulloblastoma!

As we have the discussion over medullobastome to day, I thought to revise it briefly.

The commonest malignancyin children, accounting for 10-20% of primary Neuro neoplasm and approximately 40% of all posterior fossa tumors.

From the diagram, we can see the malignancy from cerebellum(Cerebellar vermis). If the same growth occurs in other part of CNS, it is termed as primitive neuroectodermal tumor. Though 50% of medulloblastoma patients can be treated with surgery and chemotherapy, endocrine and neural functions are compromised. 

ncogenes ERBB2 andMYCC are associated with worse outcome. Of all pediatric neoplasms, medulloblastoma has highest propensity to spread extra neural sites, specially bone and bone marrow. But it is not more than 4 percent.

Hydrocephalus: reason is obstruction of CSF through 4th ventricle.

Epidemiology:

No racial predisposition is noted. Data shows patients aged 0-14 years in the United States have an incidence rate per million population of 5.7 in whites and 5 in blacks.

US incidence per 1 million population for patients aged 0-14 years is 6.1 for boys and 4.5 for girls.

Peak age of incidence is 3-5 years. Approximately 80% of patients are diagnosed in the first 15 years of life. WHile I was doing my internship project with Einstein, our sample didn’t have pediatric age group

Symptoms:

1)Increased intracranial pressure: Headache, Vomitting and lethargy

2)Cerebellar functions compromised: ATaxia worsens

3)Brain Stem Deficit: Most of the functions of cranial nerves are affected: for example 7th and 8th nerve: facial weakness, tinnitus, hearing loss, head tilt, and stiff neck.

Hereditary Association:  isochromosome 17q [i(17q)].

Differential Diagnosis: Epindymoma, Astrocytome, Aseeptic Meningtis, Bacterial Meningits.

In a report by the International Society of Pediatric Oncology, children with average-risk medulloblastoma randomly received either the standard 36 Gy or a reduced dose of 24 Gy to the neuraxis (Tait et al). However, the role of radiation therapy in the patients younger than 3 years remain controversial. What is survival rate?

 

(Source:emedicine)