Park S H, Chi J G, Cho B K, Wang K C
Department of Pathology, Seoul National University Children's Hospital, College of Medicine, Korea.
Pathol Res Pract. 1993 Mar;189(2):189-96. doi: 10.1016/S0344-0338(11)80091-9.
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.
本文描述了两例儿童脊髓节细胞胶质瘤病例。两例病例在临床表现、影像学和形态学上都表现出显著的相似性。第一例是一名6岁半的男孩,其节细胞胶质瘤累及脊髓全长。自1岁起,他就患有长期的脊柱侧弯和步态障碍。肿瘤内有多个空洞。第二例是一名3岁女孩,其节细胞胶质瘤累及从延髓下部到T3的大部分脊髓。该病例在多个层面也存在延髓空洞症和脊髓空洞症。她患有四肢进行性肌无力。由于肿瘤与正常脊髓界限清晰,两例病例均通过二期手术经显微手术轻松切除肿瘤。组织病理学和超微结构检查显示,肿瘤为良性节细胞胶质瘤。术后症状有明显改善。基于我们的两例病例并结合文献复习,我们证实脊髓节细胞胶质瘤是发生于儿童期或青年期的缓慢生长肿瘤。它们常累及脊髓的广泛节段或全长,如我们的病例所示,当肿瘤在婴儿期或幼儿期出现时,常伴有脊柱侧弯和步态障碍。它们还常表现为肿瘤的囊性改变,伴有大小不一的脊髓空洞症。由于与正常脊髓界限清晰,几乎可以完全切除肿瘤。因此,早期诊断和手术治疗对于避免不必要的手术延误和取得良好疗效至关重要。