Clinicopathological features of medulloblastoma: an overview with an emphasis on molecular biology.

Medulloblastoma is a malignant, invasive embryonal tumor of the cerebellum with preferential manifestation in children, predominantly neuronal differentiation and an inherent tendency to metastasize via CSF pathways. In this review we present an overview of the clinicopathological aspects of medulloblastoma and the recent developments in the biology and genetics of these embryonal brain tumors. Increased knowledge of the signaling pathways that play a role in normal cerebellum development and tumor formation may lead to new therapeutic approaches resulting in a higher cure rate and a better quality of life of medulloblastoma patients.

[Children with stumbling gait due to acute spinal cord compression]. / Kinderen met een wankel looppatroon door acute ruggenmergcompressie.

Three previously healthy children, two girls aged 2 and almost 5 years and a boy aged 20 months, developed a progressively stumbling gait within days. In two this occurred after a period of weeks during which they complained of, or seemed to have back pain. In all three cases acute spinal cord compression by a malignant tumour was diagnosed. Histological examination revealed Ewing sarcoma, granulocytic sarcoma and T-cell lymphoma. Surgical decompression led to complete neurological recovery. Although rare, acute spinal cord compression during childhood is a medical emergency because of the risk of neurological morbidity. Back pain, weakness and a stumbling gait usually are the first symptoms. Sensory symptoms and sphincter dysfunction may develop later. Early recognition is essential, as prognosis depends on neurological findings and duration of symptoms when treatment is started.