Inoue Akihiro, Ohnishi Takanori, Kohno Shohei, Nishikawa Masahiro, Nishida Naoya, Ohue Shiro
Department of Neurosurgery, Ehime University School of Medicine, Ehime, Japan.
Department of Neurosurgery, Washoukai Sadamoto Hospital, Ehime, Japan.
World Neurosurg. 2016 Jul;91:674.e1-6. doi: 10.1016/j.wneu.2016.04.054. Epub 2016 Apr 23.
Giant cell tumors (GCTs) are histologically benign bone neoplasms with a locally aggressive nature that primarily occur in the epiphyses of the long bones. A small proportion of these tumors, however, occur in the pelvis, spine, or skull bones. Among these, GCTs of the skull base cannot be completely resected and require adjuvant therapy. We report a juvenile case of clival GCT that was successfully treated by endoscopic endonasal transsphenoidal surgery and subsequent adjuvant therapy with denosumab, a monoclonal antibody to receptor activator of nuclear factor-κB ligand.
A 16-year-old boy was admitted to our hospital with progressively intolerable headache and right oculomotor nerve palsy. Computed tomography and magnetic resonance imaging showed a large tumor mass in the sphenoid sinus with extensive erosion of the clivus and compression of the right cavernous sinus. The tumor was resected by endonasal transsphenoidal surgery and histologically diagnosed as GCT. The giant cells showed positive immunostaining for CD68 and Mib-1 labeling index was less than 1.0%. Postoperative course was uneventful and the oculomotor disturbance was markedly improved. However, magnetic resonance imaging 2 weeks after surgery revealed marked enlargement of the tumor. Adjuvant therapy with denosumab was therefore initiated, resulting in marked reductions in tumor size.
This is the first report to describe beneficial effects of denosumab in the treatment of GCT of the skull base.
骨巨细胞瘤（GCTs）是组织学上为良性的骨肿瘤，具有局部侵袭性，主要发生于长骨的骨骺。然而，这些肿瘤中有一小部分发生在骨盆、脊柱或颅骨。其中，颅底骨巨细胞瘤无法完全切除，需要辅助治疗。我们报告一例青少年斜坡骨巨细胞瘤病例，该病例通过鼻内镜经蝶窦手术成功治疗，并随后接受了地诺单抗（一种针对核因子κB受体激活剂配体的单克隆抗体）辅助治疗。
一名16岁男孩因进行性难以忍受的头痛和右侧动眼神经麻痹入院。计算机断层扫描和磁共振成像显示蝶窦内有一个大肿瘤块，斜坡广泛侵蚀，右侧海绵窦受压。通过鼻内镜经蝶窦手术切除肿瘤，组织学诊断为骨巨细胞瘤。巨细胞CD68免疫染色呈阳性，Mib-1标记指数小于1.0%。术后过程顺利，动眼神经功能障碍明显改善。然而，术后2周的磁共振成像显示肿瘤明显增大。因此开始用地诺单抗进行辅助治疗，肿瘤大小显著缩小。
这是第一份描述地诺单抗治疗颅底骨巨细胞瘤有益效果的报告。
