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高级别颅内软骨肉瘤伴出血。

High-grade intracranial chondrosarcoma presenting with haemorrhage.

机构信息

University of Toronto, Faculty of Medicine, Toronto, Ontario, Canada.

出版信息

J Clin Neurosci. 2013 Oct;20(10):1457-60. doi: 10.1016/j.jocn.2012.10.036. Epub 2013 Jun 5.

Abstract

Chondrosarcomas are rare sarcomas that produce malignant cartilage, infrequently arising as a primary intracranial tumour. We present a patient with intracranial chondrosarcoma with intratumoural haemorrhage arising in an unusual location and with unusual imaging findings. A 46-year-old man presented with headache, nausea, and vomiting over the previous 24 hours. Physical and neurological examinations were normal. Cranial CT scans and MRI revealed a large right pre-frontal (subdural) and interhemispheric heterogeneous density associated with a frontal, partially calcified mass and midline shift. An awake craniotomy was performed. With the intra-operative quick section favouring subdural hematoma, the lesion was subtotally resected. Follow-up imaging confirmed residual mass. Pathology examination revealed a high-grade malignant neoplasm with chondroid differentiation, diagnosed as conventional Grade III chondrosarcoma. The patient was referred to oncology for follow-up and radiation therapy. Intracranial chondrosarcoma was first reported in 1899, and since then continues to be an extremely rare malignancy of the brain. These tumours commonly present as extra-axial masses, originating from the skull base, and produce symptoms due to progressive enlargement and compression of local structures. Unusual presentations of these tumours, such as vascularity, intratumoural haemorrhage, and intra-axial location, may complicate pre-surgical decision making by altering the provisional diagnosis prior to intervention. This patient emphasises the importance of careful analysis and incorporation of imaging findings into surgical decision making. Specific imaging characteristics that, in such unusual situations, are suggestive of chondrosarcoma should motivate an aggressive surgical approach to optimise adjuvant interventions.

摘要

软骨肉瘤是一种罕见的肉瘤,会产生恶性软骨,很少作为原发性颅内肿瘤发生。我们报告了一例颅内软骨肉瘤患者,其肿瘤内出血发生在一个不寻常的部位,具有不寻常的影像学表现。一名 46 岁男性因头痛、恶心和呕吐在过去 24 小时内出现。体格检查和神经系统检查均正常。头颅 CT 扫描和 MRI 显示右侧额前部(硬膜下)和大脑半球间存在不均匀密度,伴有额部、部分钙化肿块和中线移位。进行了清醒开颅术。由于术中快速切片支持硬膜下血肿,因此对病变进行了大部分切除。随访影像学检查证实有残留肿块。病理检查显示为高级别恶性肿瘤,具有软骨分化,诊断为常规 3 级软骨肉瘤。患者被转介至肿瘤科进行随访和放疗。颅内软骨肉瘤于 1899 年首次报道,此后一直是一种极其罕见的脑恶性肿瘤。这些肿瘤通常表现为来自颅底的轴外肿块,并因局部结构的进行性增大和压迫而产生症状。这些肿瘤的不寻常表现,如血管生成、肿瘤内出血和轴内位置,可能会改变术前诊断,从而使术前决策复杂化。该患者强调了仔细分析和将影像学结果纳入手术决策的重要性。在这种不寻常的情况下,提示软骨肉瘤的特定影像学特征应促使采取积极的手术方法,以优化辅助干预。

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