- Author: George I Jallo, MD; Chief Editor: Amy Kao, MD more...
Further Outpatient Care
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- Imaging is the primary mode of monitoring residual disease, efficacy of continuing medical treatment, and recurrence or metastasis. Because medulloblastoma is aggressive, frequent monitoring is essential. MRI should be repeated every 3 months the first year; every 4 months the second year; every 6 months the following 3 years; and yearly thereafter.
- Radiation therapy is an outpatient procedure.
- Any signs of change in mental status are indications for outpatient visits, as they may herald hydrocephalus and possible recurrence.
- Lower cranial nerve or cerebellar signs also may signal recurrence.
- Taper steroids and monitor adverse effects.
Further Inpatient Care
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- Admit patients in whom further surgical intervention is being contemplated.
- Chemotherapy usually is administered on an inpatient basis.
Inpatient & Outpatient Medications
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- Taper steroid use. However, chemotherapy and radiation therapy may exacerbate edema and necessitate low-dose corticosteroids for a short period of time.
- Antiepileptic medication usually is not necessary when the tumor is located in the posterior fossa.
- Spread of disease to the supratentorial compartment may cause seizures and indicate antiseizure medication.
Transfer may be necessary for treatment at a center familiar with pediatric neurosurgery, pediatric oncology, or pediatric radiotherapy.
No known precautions currently exist to prevent the disease or its recurrence.
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- Hydrocephalus (the most common complication of medulloblastoma) can cause secondary visual problems. Cerebellar dysfunction (the second most common complication of the disease) may lead to problems with coordination and gait. Cranial nerve palsy from brainstem involvement can lead to difficulties with vision, speech, and swallowing. With subarachnoid spread to the spinal cord, the unfortunate complications are radiculopathy and weakness.
- Complications accompany the treatment of medulloblastoma. Fortunately, most of these complications are transient. The most common complication after surgery is a temporary worsening of ataxia accompanied by nystagmus. One of the most commonly cited complications is cerebellar mutism. The anatomic site of origin is thought to be the deep cerebellar nuclei. The constellation of symptoms includes apathy, minimal-to-absent speech, emotional lability, and refusal to initiate movement. Hemiparesis can accompany mutism. Lower cranial nerves are intact, but the syndrome is accompanied by a swallowing apraxia. It becomes apparent several hours after surgery and persists for several weeks, usually resolving completely. Other complications include a temporary Parinaud syndrome and pneumocephalus. A common complication of any surgery in the posterior fossa is aseptic meningitis. This can be alleviated with a short course of corticosteroids.
- Complications of radiation therapy have been discussed previously and include lowered IQ, small stature, endocrine dysfunction, behavioral abnormalities, secondary neoplasms, and radiation necrosis of the white matter.
- Chemotherapy also has numerous adverse effects on multiple organ systems including renal toxicity, ototoxicity, hepatotoxicity, pulmonary fibrosis, and gastrointestinal disturbance. Methotrexate, when used in combination with irradiation, can cause permanent necrotizing leukoencephalopathy.
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- Medulloblastoma is a very aggressive tumor. Even after a good response to surgery and radiation, recurrence is common; most recurrences occur within 2 years after treatment.
- The most common location of recurrence is at the primary tumor site in the posterior fossa.
- With the use of adjuvant chemotherapy, incidence of recurrence in the spinal canal and the supratentorial region seems to decrease.
- Systemic metastases, in the absence of a CSF shunting system, are also a recognized problem in 10-20% of patients. Bone is the most common site of systemic metastasis; regional lymph node sites follow.
- The Collin law was first hypothesized for Wilms tumor and has been expanded since to cover many pediatric tumors thought to be congenital in origin.
- The Collin law states that, if a tumor has not recurred in a period of time equal to age of patient plus 9 months, that patient can be considered to be cured.
- The Collin law generally holds for medulloblastoma; however, several late recurrences (longer than 10 years after diagnosis) have been reported. The 5-year progression-free survival rate in that group is 70-80% for patients at low risk and 60-70% for patients at high risk.
- Greater age at diagnosis has been associated with a better prognosis, most likely because adults more often harbor the less aggressive desmoplastic variant of medulloblastoma.
- Why females have a longer recurrence-free interval is not understood.
Teach patients and families about early signs of hydrocephalus, especially if the patient has a ventricular shunt in place.
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