Heuer Gregory G, Kiefer Heather, Judkins Alexander R, Belasco Jean, Biegel Jaclyn A, Jackson Eric M, Cohen Marc, O'Malley Bert W, Storm Phillip B
Department of Neurosurgery, Children's Hospital of Philadelphia, University of Pennsylvania Medical Center, Philadelphia, Pennsylvania 19104-4399, USA.
J Neurosurg Pediatr. 2010 Jan;5(1):75-9. doi: 10.3171/2009.8.PEDS08421.
The authors present the case of en bloc resection of a clival-C2 atypical teratoid/rhabdoid tumor. These aggressive lesions of early childhood generally occur in the cerebellum or cerebral hemispheres. This 7-year-old boy presented with pain on turning his head and was found to have a clival-C2 mass. A metastatic workup was negative for disseminated disease. A transoral biopsy procedure revealed an atypical teratoid/rhabdoid tumor on histological examination. The tumor was resected via a transoral approach, and the patient's spine was stabilized with posterior instrumented fusion from the occiput to C-5. Postoperatively, the patient underwent 16 months of chemotherapy along with 6 weeks of overlapping radiation therapy. Twenty-seven months after the initial surgery he presented with leg pain and was found to have a solitary metastatic lesion at the conus medullaris. There was no local recurrence at the clivus. The conus tumor was resected and found to be consistent with the primary tumor. Several months later the patient presented with disseminated intrathecal disease and ultimately died 42 months after the initial resection.
作者报告了1例斜坡- C2非典型畸胎样/横纹肌样瘤整块切除的病例。这些儿童早期的侵袭性病变通常发生在小脑或大脑半球。这名7岁男孩转头时出现疼痛，检查发现斜坡- C2有肿块。转移检查未发现播散性疾病。经口活检组织学检查显示为非典型畸胎样/横纹肌样瘤。肿瘤通过经口入路切除，患者的脊柱通过从枕骨到C-5的后路器械融合固定。术后，患者接受了16个月的化疗以及6周的同步放疗。初次手术后27个月，他出现腿痛，检查发现脊髓圆锥有一个孤立的转移病灶。斜坡处无局部复发。脊髓圆锥肿瘤切除后发现与原发肿瘤一致。几个月后，患者出现鞘内播散性疾病，最终在初次切除后42个月死亡。
