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Medullomyoblastoma. A rare cerebellar tumour in children
A K Mahapatra1, A K Sinha, M C Sharma
1Department of Neurosurgery, ALL India Institute of Medical Sciences, New Delhi.
Insights
Medullomyoblastoma, a rare posterior fossa tumor, presents with symptoms of increased intracranial pressure (ICP). Despite aggressive treatment including surgery, radiation, and chemotherapy, survival beyond three years is not achieved in this patient cohort.
Area of Science:
- Pediatric Oncology
- Neuropathology
- Neurosurgery
Background:
- Medullomyoblastoma is an uncommon posterior fossa tumor.
- Patients typically present with symptoms of increased intracranial pressure (ICP).
Purpose of the Study:
- To report the largest collection of medullomyoblastoma cases.
- To detail the clinical presentation, treatment, and outcomes of these rare tumors.
Main Methods:
- Histological diagnosis of medullomyoblastoma in seven patients (ages 3-24).
- CT scans revealed posterior fossa tumors with hydrocephalus.
- Treatment included ventriculoperitoneal shunting, radical tumor excision, radiation, and chemotherapy.
Main Results:
- Six of seven patients survived surgery; one died from shunt complications.
- Three patients died within 6 months, and three between 2.5 and 3 years post-surgery.
- No patients survived beyond 3 years; one developed paraplegia.
Conclusions:
- Medullomyoblastoma is an aggressive tumor with a poor long-term prognosis.
- Aggressive multimodal treatment offers limited survival benefit.
- Further research is needed to improve outcomes for this rare pediatric cancer.
Abstract:
Seven patients between the ages of 3 and 24 years were admitted to our hospital in the last 28 years who had a histological diagnosis of medullomyoblastoma. These patients presented with classic symptoms of a posterior fossa midline mass associated with evidence of raised ICP. A CT scan in each patient revealed a uniformly high-attenuating tumour in the posterior fossa with gross hydrocephalus. In all seven patients a ventriculoperitoneal shunt was placed prior to definitive surgery. Radical tumour excision was carried out in all cases 3-5 days after CSF diversion. The histological diagnosis was made on H&E-stained slides. In two cases each, the tumour tissue was subjected to electron microscopy and immunohistochemical studies. Six of the seven patients survived the operation. One patient died 21 days after surgery as a result of shunt block and shunt infection. All surviving patients received cranial and spinal radiation 2-4 weeks after surgery, and also chemotherapy. The cranial radiation dose ranged from 4500 to 5000 rad, while the spinal radiation dose was limited to 1500 rad. Patients were followed up carefully. Three patients died within 6 months, and the remaining three between 2.5 and 3 years after surgery. None of the patients in our study survived longer than 3 years. One patient had developed paraplegia. This study highlights the details of an uncommon entity and reports the largest collection of such cases in the literature.