去分化软骨肉瘤：我们对25例病例的临床病理经验及困境
Dedifferentiated chondrosarcoma: our clinico-pathological experience and dilemmas in 25 cases.
作者信息
Sopta Jelena, Dordević Aleksandar, Tulić Goran, Mijucić Vesna
机构信息
Institute of Pathology, School of Medicine, Belgrade, Serbia.
出版信息
J Cancer Res Clin Oncol. 2008 Feb;134(2):147-52. doi: 10.1007/s00432-007-0262-5. Epub 2007 Jul 25.
BACKGROUND
Dedifferentiated chondrosarcoma represents a special form of the chondrosarcoma characterized by the presence of well-differentiated cartilaginous component in juxtaposition with the malignant mesenchymal tumor of high-malignancy grade.
METHODS
The study was performed on 25 tissue samples obtained by biopsy of the primary malignant bone tumors.
RESULTS
Out of the total of 25 patients, 13 were males and 12 were females. Their average age was 59.9 years (39-75). Radiological examination verified the presence of the pathological fractures in 14 patients. The skeletal distribution clearly indicates that the femur is the most frequent site of affection (in 15 patients--60%). Morphological analysis verified the presence of "precursor cartilaginous lesion" or low-grade cartilaginous component in all cases. Classification of the high-grade components was based on the immunohistochemical analyses, which evidenced MFH in 18 cases, osteosarcoma in 4, while fibrosarcoma was evidenced in 2 and rhabdomyosarcoma in 1 case. In 20 patients, out of whom 14 had existing fractures, amputation was indicated. In five patients (fracture-free), resection limb-salvage surgical intervention was performed.
CONCLUSION
The lethal outcome ensues within less than 2 years in all patients.
背景
去分化软骨肉瘤是软骨肉瘤的一种特殊形式，其特征是存在高分化软骨成分与高恶性级别的恶性间充质肿瘤并存。
方法
本研究对25例原发性恶性骨肿瘤活检获取的组织样本进行。
结果
25例患者中，男性13例，女性12例。平均年龄59.9岁（39 - 75岁）。影像学检查证实14例患者存在病理性骨折。骨骼分布情况清楚表明，股骨是最常受累部位（15例患者，占60%）。形态学分析证实所有病例均存在“软骨前体病变”或低级别软骨成分。高级别成分的分类基于免疫组化分析，结果显示18例为恶性纤维组织细胞瘤，4例为骨肉瘤，2例为纤维肉瘤，1例为横纹肌肉瘤。20例患者（其中14例已有骨折）需行截肢术。5例（无骨折）患者接受了保肢手术切除干预。
结论
所有患者均在不到2年内出现致命结局。
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