Neurological Disorders: Medulloblastoma

Introduction

Medulloblastoma is an aggressive brain cancer that occurs predominantly in children. The younger the child the higher risk of developng this tumor.

It belongs to a group of tumors called as PNET (primitive neuroectodermal tumors), and as the name implies the tumors arise from primitive cells (cells before get fully differentiated) belonging to the ectodermal line.

This tumor arises in the infratentorial area especially in the 4th ventricle and from the cerebellum. It is an aggressive cancer and has a tendency to spread to other areas of the CNS (central nervous system) through the CSF (cerebrospinal fluid).

How does patient present?

The manifestations are secondary to the tumor mass itself, due to the hydrocephalus that usually develops, and due to any spread of the tumor through the CSF. The following are the common manifestations;

• Vomiting
• Headache
• Gait difficulty
• In coordination
• Child becoming irritable
• Later becomes lethargic, obtunded due to hydrocephalus

Diagnosis

A CT or more preferably an MRI of the brain will show the mass in the cerebellar/brain stem area without difficulty. The precise diagnosis can be confirmed by a biopsy.

Treatment

The following modes of treatment are approached;

• Surgical resection of the growth as much as possible
• Chemotherapy
• Radiotherapy

A ventriculoperitoneal shunt maybe necessary to treat the hydrocephalus.

A pediatric and/or adult neurologist and a neurosurgeon are frequently involved in the care of patients with PNET tumors.



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