The management of chondrosarcoma of bone.

The management of chondrosarcoma of bone.
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DOI:
10.1097/00003086-198011000-00007
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发表时间:
1980-11
影响因子:
4.2
通讯作者:
Eriksson Ai;A. Schiller;H. Mankin
Eriksson Ai;A. Schiller;H. Mankin
中科院分区:
医学2区
文献类型:
--
作者:
Eriksson Ai;A. Schiller;H. Mankin

文献摘要

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骨软骨肉瘤是骨科肿瘤学诊断和治疗的难点之一。除了“标准”透明样、粘液样或纤维样病变表现的变异性外,现在还认识到三种不寻常的肿瘤形式--间质细胞、透明细胞和去分化--它们显示出生物学行为的差异。软骨肉瘤,只有极少数例外,是放射抵抗性的,只有有限的反应,连续化疗。目前最佳的治疗方法是外科手术,但显然选择的外科手术类型应根据肿瘤的恶性程度和范围而变化,以避免治疗不足(导致局部复发和/或远处转移的高发生率)或过度和不必要的正常组织牺牲。怀疑患有软骨肉瘤的患者应通过临床检查、放射学检查和特殊研究进行全面评估,以尽可能全面地确定局部肿瘤的部位和范围、是否存在软组织肿块以及是否存在远处转移。活检应通过一个切口进行,该切口随后可在定义过程中切除。所获得的组织应进行组织学分级,并补充临床和放射学资料,以确定病变是良性还是低度或高度软骨肉瘤。使用Enneking和Spanier最近描述的一种特殊系统,应根据级别(G1或G2)、病变部位与解剖分区的关系(T1或T2)以及是否存在远处转移(M0或M1)来评估病变。根据本文提出的方案,应在四种手术方式中选择合适的一种。对于良性肿瘤,I级手术(病灶内刮除或切除)通常是足够的。对于IA期肿瘤,偶尔II级手术,边缘切除可能就足够了,但更常见的是III级手术(广泛的间室切除术)。IIA和IIB期病变应采用III级或IV级(根治性切除)外科手术治疗。不能手术或IV期肿瘤(具有转移的那些)的治疗是不令人满意的,并且包括姑息性切除、放射疗法和化学疗法。
Chondrosarcomas of bone are among the most difficult problems in diagnosis and management in the field of orthopaedic oncology. In addition to the variability of presentation of the "standard" hyaline, myxoid or fibrous types of lesion, three unusual forms of the tumor are now recognized--mesenchymal, clear cell and dedifferentiated--which show differences in their biologic behavior. Chondrosarcomas, with only rare exception, are radio-resistant and show only a limited response to adjunctive chemotherapy. The optimal therapy at present is surgical, but clearly the type of surgical procedure selected should vary with the malignity and extent of the tumor in order to avoid either undertreatment (resulting in a high rate of local recurrence and/or distant metastases) or excessive and unnecessary sacrifice of normal tissue. Patients suspected of having chondrosarcomas should be thoroughly evaluated by clinical examination, radiographic and special studies to define as fully as possible the site and extent of the local tumor, the presence or absence of a soft-tissue mass and the existence of distant metastases. A biopsy should be performed through an incision which can be subsequently excised during the definition procedure. The tissue obtained should be graded histologically and the clinical and radiologic data added to define whether the lesion is benign or a low-grade or high-grade chondrosarcoma. Using a special system recently described by Enneking and Spanier, the lesions should be evaluated on the basis of the grade (G1 or G2); the site of the lesion in relation to the anatomic compartments (T1 or T2) and the presence or absence of distant metastasis (M0 or M1). Based on a scheme presented in this article, the appropriate one of four grades of surgical procedures should be chosen. For benign tumors, Grade I procedures (intralesional curettage or excision) are generally adequate. For Stage IA tumors, occasionally a Grade II procedure, marginal excision may be sufficient, but more frequently Grade III procedures (wide intracompartmental resections) are indicated. Stage IIA and IIB lesions should be treated with Grade III or Grade IV (radical resection) surgical procedures. The treatment of inoperable or Stage IV tumors (those with metastasis) is unsatisfactory and consists of palliative resections, radiation therapy and chemotherapy.