Abstract Medullablastoma Rahmi Alfiah Nur Alam,Herlina Uinarni Medulloblastoma is the most common malignant brain tumor in children.

Medulloblastomas account for 12-25 % of all paediatric CNS tumours, and 30-40% of paediatric posterior fossa tumours. There is a moderate male predilection with a M:F ratio of 2-4:1. They usually present in childhood with 77% of cases before the age of 19. The median age of diagnosis is 9 years. A girl, 9 year, since 6 months ago often complained of headaches. Head MSCT examination with and without contrast was performed. Radiological findings: hyperdense and prominent contrast enchanced mass in the vermis cerebellum protrude into the fourth ventricle. Surgical operation was already done and the results of anatomic pathology test were consistent with medulloblastoma. Medulloblastoma is a highly malignant primary brain tumor that originates in the cerebellum or posterior fossa. Tumors that originate in the cerebellum are referred to as infratentorial because they occur below the tentorium, a thick membrane that separates the cerebral hemispheres of the brain from the cerebellum. Another term for medulloblastoma is infratentorial primitive neuroectodermal tumor (PNET). Medulloblastoma is the most common PNET originating in the brain.[1] Incidence Medulloblastomas affect just under 2 people per million per year, and affect children 10 times more than adults.[2] Medulloblastoma is the most common malignant brain tumor in children, comprising 14.5% of newly diagnosed cases.[3] In adults, medulloblastoma is rare, comprising fewer than 2% of CNS malignancies.[4] Pathogenesis Medulloblastomas usually form in the vicinity of the fourth ventricle, between the brainstem and the cerebellum. Tumors with similar appearance and characteristics originate in other parts of the brain, but they are not identical to medulloblastoma.[7] Clinical manifestation Symptoms are mainly due to secondary increased intracranial pressure due to blockage of the fourth ventricle and are usually present for 1 to 5 months before diagnosis is made. The child typically becomes listless, with repeated episodes of vomiting, and a morning headache, which may lead to a misdiagnosis of gastrointestinal disease or migraine. Soon after, the child will develop a stumbling gait, frequent falls, diplopia, papilledema, and sixth cranial nerve palsy. Positional dizziness and nystagmus are also

frequent and facial sensory loss or motor weakness may be present. Decerebrate attacks appear late in the disease. Diagnosis The tumor is distinctive on T1 and T2-weighted MRI with heterogeneous enhancement and typical location adjacent to and extension into the fourth ventricle. Histologically, the tumor is solid, pink-gray in color, and is well circumscribed. The tumor is very cellular, many mitoses, little cytoplasm, and has the tendency to form clusters and rosettes. Treatment and prognosis Treatment begins with maximal resection of the tumor. The addition of radiation to the entire neuraxis and chemotherapy may increase the disease-free survival. This combination may permit a 5 year survival in more than 80% of cases. The presence of desmoplastic features such as connective tissue formation offers a better prognosis. Prognosis is worse if the child is less than 3 years old, there is an inadequate degree of resection, or if there is any CSF, spinal, supratentorial or systemic spread. Dementia post radiotherapy and chemotherapy is a common outcome appearing two to four years following treatment. Chemotherapy Chemotherapy is now an important part of treatment for all patients with medulloblastoma. It can significantly reduce risk of recurrence (which is typically fatal). There are a couple of different chemotheraputic regimens for medulloblastoma, but most involve a combination of lomustine, cisplatin, carboplatin, vincristine or cyclophosphamide. In younger patients (less than 34 years of age), chemotherapy can delay, or in some cases possibly even eliminate, the need for radiotherapy.[14] key word :medullablastoma,msct head