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Falcine and parasagittal chondrosarcomas

机译:恶性和矢状软骨肉瘤

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摘要

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.
机译:颅内软骨肉瘤是原发性软骨肿瘤,占所有颅底肿瘤的6%。颅内骨骼外软骨肉瘤较为罕见,通常是由镰状,肌腱或脑凸处的脑膜引起。它们通常被表征为经典或间质,后者与较差的结果相关。我们介绍了我们的机构性系列的falcine和矢状软骨肉瘤以及文献复习。尽管颅底软骨肉瘤由于其侵袭性生物学以及邻近重要的脑干结构和颅神经而构成了重大挑战,但颅内骨骼外软骨肉瘤通常具有良好的预后。我们对文献的审查确定了29例患有镰状和旁矢状软骨肉瘤的患者。有6例复发,间充质亚型患者5例,经典亚型患者1例。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尚存争议,但一般仅通过手术切除即可解决颅外颅内肿瘤。治疗应针对肿瘤的生物学情况而定,放射治疗应留给间充质亚型患者。

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