Department of Neurological Surgery, University of California, San Francisco, 505 Parnassus Ave., Room 779M, San Francisco, CA 94143-0112, USA.
J Clin Neurosci. 2013 Sep;20(9):1232-6. doi: 10.1016/j.jocn.2013.01.004. Epub 2013 Jun 10.
Intracranial chondrosarcomas are primary cartilaginous neoplasms that represent 6% of all skull base tumors. Intracranial extraskeletal chondrosarcomas are more rare, often arising from the meninges at the falx, tentorium, or cerebral convexity. They are generally characterized as classical or mesenchymal, with the latter associated with worse outcomes. We present our institutional series of falcine and parasagittal chondrosarcomas along with a review of the literature. Although skull base chondrosarcomas pose significant challenges due to their invasive biology and proximity to vital brainstem structures and cranial nerves, intracranial extraskeletal chondrosarcomas are generally associated with a good prognosis. Our review of the literature identified 29 patients with falcine and parasagittal chondrosarcomas. There were six recurrences, five among patients with the mesenchymal subtype and one in a patient with the classical subtype. All deaths occurred in patients with the mesenchymal subtype. Management of skull base chondrosarcomas is controversial but extraskeletal intracranial tumors can generally be managed by surgical resection alone. Treatment should be tailored to the biology of the tumor, with radiation therapy reserved for patients with the mesenchymal subtype.
颅内软骨肉瘤是原发性软骨性肿瘤,占所有颅底肿瘤的 6%。颅内颅外软骨肉瘤更为罕见,常起源于镰状窦、天幕或大脑凸面的脑膜。它们通常表现为经典型或间充质型,后者与更差的预后相关。我们介绍了我们机构的镰状窦和矢状窦软骨肉瘤系列,并对文献进行了回顾。尽管颅底软骨肉瘤由于其侵袭性生物学以及靠近重要的脑干结构和颅神经而带来了重大挑战,但颅内颅外软骨肉瘤通常与良好的预后相关。我们对文献的回顾确定了 29 例镰状窦和矢状窦软骨肉瘤患者。有 6 例复发,其中 5 例为间充质亚型,1 例为经典亚型。所有死亡均发生在间充质亚型患者中。颅底软骨肉瘤的治疗存在争议,但颅内颅外肿瘤通常可以单独通过手术切除来治疗。治疗应根据肿瘤的生物学特性进行调整,对间充质亚型患者保留放疗。