[Intramedullary holocord tumor. Report of an autopsy case and review of literature].
This report describes a case of cystic astrocytoma that extended from C4 to the conus, and reviews intramedullary holocord tumors. A two-year-old body was admitted because of gait disturbance. He had suffered from rigidity of his left upper and lower limbs and thoracic scoliosis for six months prior to admission. On admission, neurologic examination revealed spastic paraparesis with muscle atrophy of the left lower extremity, sphincter disturbances and neck rigidity. All deep tendon reflexes were bilaterally hyperactive. Spine roentgenography demonstrated widening of the interpedicular distance from the midthoracic to the upper lumbar segments. A lumbar myelography disclosed a block at the L2 level, and a suboccipital myelography revealed a block with tapering of contrast medium at the level of the C2 vertebra. A laminectomy was performed from C1 to L3, and a myelotomy was performed from C4 to L2. Grossly total removal of solid tumor which extended from Th3 to Th8 was carried out. There were large intramedullary cysts extending from C4 to Th3, and from Th9 to L2. Eight hours after surgery, high fever, tachycardia and anuria developed, and he died next day. Cross section of the spinal cord revealed the tumor cells infiltrated diffusely in the midthoracic region, and histologically the tumor was diagnosed to be astrocytoma. The cystic portion was separate from the central canal, and the lining of the cyst was a band of fibroglial tissue without infiltration of tumor cells. A tumor of this kind extending throughout the almost whole spinal cord is extremely rate. For such a holocord tumor complete removal is the ideal treatment, but the possibilities for removal must be evaluated in terms of the tumor's relationship to the spinal cord, the histological nature and the general condition of the patient.
D001254:Astrocytoma / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D002675:Child, Preschool; D006801:Humans; D008297:Male; D009192:Myelography; D013120:Spinal Cord Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery