Oruckaptan H H, Berker M, Soylemezoglu F, Ozcan O E
Department of Neurosurgery, Hacettepe University, School of Medicine, Ankara, Turkey.
Surg Neurol. 2001 Mar;55(3):174-9. doi: 10.1016/s0090-3019(01)00329-9.
Intracranial chondroid tumors are infrequently seen in neurosurgical practice. These tumors usually arise from cartilaginous synchondroses at the base of the skull, but occasionally from the pluripotential mesenchymal cells of the meninges. We present here a case of classic low-grade giant chondrosarcoma of the falx cerebri. This is only the second case of this variant reported in this location, and we summarize the diagnostic criteria with a brief review of literature.
A 56-year-old female patient was admitted to the hospital with a history of progressive right-sided weakness occurring in the last 8 months and a recent grand mal seizure. Radiological evaluation demonstrated a large extra-axial mass in the left parafalcine area, suggesting a possible meningioma. An anterior interhemispheric approach enabled gross total removal of the tumor and a histologic diagnosis of a low-grade classic chondrosarcoma was made. The patient is currently stable and has shown no evidence of recurrence in more than 3 years without any adjuvant treatment.
Intracranial cartilaginous tumors include classical, mesenchymal and myxoid chondrosarcomas in addition to benign chondromas. Parafalcine localization should be considered for all these variants as well as for meningiomas, hemangiopericytomas, solitary fibrous tumors, and meningeal metastatic carcinomas. Detailed radiological evaluation, light microscopic and ultrastructural analyses, and immunocytochemistry are essential for correct diagnosis. In contrast to mesenchymal and myxoid types, the prognosis of classic variants is usually good and does not require adjuvant treatment modalities if a radical resection of the tumor can be obtained. Increased documentation of clinical, radiological, and histologic findings as well as response to treatment modalities will provide a better understanding of the pathophysiology of these rare tumors, and highlight the optimum treatment strategies
颅内软骨样肿瘤在神经外科实践中较为少见。这些肿瘤通常起源于颅底的软骨结合处，但偶尔也起源于脑膜的多能间充质细胞。我们在此报告一例大脑镰的经典低级别巨大软骨肉瘤病例。这是该部位报道的此变异型的第二例病例，我们通过简要回顾文献总结了诊断标准。
一名56岁女性患者因过去8个月出现进行性右侧肢体无力及近期一次癫痫大发作入院。影像学评估显示左侧大脑镰旁区域有一个巨大的轴外肿块，提示可能为脑膜瘤。采用经纵裂前部入路实现了肿瘤的全切，并作出了低级别经典软骨肉瘤的组织学诊断。患者目前情况稳定，在未接受任何辅助治疗的情况下3年多未出现复发迹象。
颅内软骨肿瘤除良性软骨瘤外，还包括经典型、间充质型和黏液样软骨肉瘤。所有这些变异型以及脑膜瘤、血管外皮细胞瘤、孤立性纤维瘤和脑膜转移癌都应考虑大脑镰旁定位。详细的影像学评估、光镜和超微结构分析以及免疫细胞化学对于正确诊断至关重要。与间充质型和黏液样型不同，经典型变异型的预后通常良好，如果能实现肿瘤的根治性切除则不需要辅助治疗方式。增加临床、影像学和组织学发现以及对治疗方式反应的记录，将有助于更好地理解这些罕见肿瘤的病理生理学，并突出最佳治疗策略。
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