Medulloblastoma is the most common malignant posterior fossa tumor in children and the second most common of all pediatric central nervous system cancers (about 20% of the total). It has a bimodal peak at age 3 to 4 years and at age 8 to 10 years but can occur throughout childhood. Medulloblastoma is a type of primitive neuroectodermal tumor (PNET).
Etiology of medulloblastoma is unknown in most patients, but medulloblastoma may occur with certain syndromes (eg, Gorlin syndrome, Turcot syndrome).
Symptoms and Signs
Diagnosis
MRI with gadolinium contrast is the test of choice for initial evaluation of possible medulloblastoma. Definitive diagnosis is made using tumor tissue obtained by biopsy or ideally by gross total resection of the tumor at initial presentation.
Once the initial diagnosis is established, staging and risk group determination are critical in medulloblastoma.
Staging tests include
Risk assessment is based on amount of residual tumor, and evidence of spread of disease:
Prognosis
Prognosis depends on the stage, histology, and biologic (eg, histologic, cytogenetic, molecular) parameters of the tumor and patient age, but generally
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Age > 3 years: Likelihood of 5-year disease-free survival is 50 to 60% if the tumor is high risk and 80% if the tumor is average risk.
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Age ≤ 3 years: Prognosis is more problematic, in part because up to 40% of children have disseminated disease at diagnosis. Children who survive are at risk of severe long-term neurocognitive deficits (eg, in memory, verbal learning, and executive function).
Treatment
Treatment of medulloblastoma includes surgery, radiation, and chemotherapy. Cure with chemotherapy alone has been shown in some children < 3 years of age. Autologous hematopoietic stem cell transplantation may also be used to treat certain patients. Combination therapy typically provides the best long-term survival.
