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Pathology Research and Practice 1993-Mar

Spinal cord ganglioglioma in childhood.

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S H Park
J G Chi
B K Cho
K C Wang

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抽象

Two childhood cases of spinal cord ganglioglioma are described. Both cases showed a remarkable similarity in presentation, radiologically and morphologically. The first case was a 6 1/2-year-old boy who had ganglioglioma involving the entire length of the spinal cord. He has been suffering from a long span of scoliosis and gait disturbance since he was 1 year. The tumor contained multiple syrinx. The second case was a 3-year-old girl with ganglioglioma involving a large portion of the spinal cord from lower medulla to T3. This case also contained syringobulbia and syringomyelia in the multiple levels. She suffered from progressive motor weakness of all extremities. Owing to a good demarcation from normal spinal cord, the tumors could easily be removed by the microsurgical dissection through second stage operation in both cases. Histopathologically and ultrastructurally, the tumors were benign gangliogliomas. The symptoms improved a lot after surgery. Based on our two cases and literature review we confirmed that the spinal cord gangliogliomas are slowly growing tumors occurring in childhood or young adulthood. They often involve usually extensive level or entire length of the spinal cord and may be accompanied by scoliosis and gait disturbance when the tumor presents since infancy or early childhood as in our cases. They also often exhibit a cystic change of the tumor associated with large or small syringomyelia. Nearly total removal is possible due to a good demarcation from the normal spinal cord. Therefore, early diagnosis and surgical management are fundamental for the avoidance of unnecessary delay of surgery and a good result.

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