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指骨软骨肉瘤:一种局部侵袭性病变,转移潜能极小:35例报告及文献复习

Chondrosarcoma of the phalanx: a locally aggressive lesion with minimal metastatic potential: a report of 35 cases and a review of the literature.

作者信息

Bovée J V, van der Heul R O, Taminiau A H, Hogendoorn P C

机构信息

Department of Pathology, Leiden University Medical Center, Leiden, The Netherlands.

出版信息

Cancer. 1999 Nov 1;86(9):1724-32. doi: 10.1002/(sici)1097-0142(19991101)86:9<1724::aid-cncr14>3.0.co;2-i.

Abstract

BACKGROUND

Enchondroma is the most common primary benign bone tumor of the hand, especially the phalanges, whereas chondrosarcoma is uncommon at this site. Although phalangeal chondrosarcoma may have ominous histologic features, its biologic behavior is relatively indolent.

METHODS

Thirty-five cases of phalangeal lesions previously diagnosed as chondrosarcoma were studied. Histologic and tumor-biologic parameters (Ki-67 and p53 immunohistochemistry) were investigated and correlated with clinical behavior.

RESULTS

All cases were characterized by unequivocal malignant histologic features (Grade 2 or higher) or Grade 1 malignant histologic features combined with the presence of cortical destruction and soft tissue extension. The median age of the patients at the time of diagnosis was 67 years (range 21-87 years), with a slight female predominance. Occurrence in the hand was more common than in the foot, with the proximal phalanx affected most often. Treatment varied from local therapy (curettage or local excision) in 16 patients to amputation or exarticulation in 19 cases. Follow-up ranged from 8-432 months for 28 patients. Ten of 15 tumors treated by local therapy recurred whereas none of 13 tumors treated by radical surgery recurred. The median survival was 20.8 years; none of 28 patients developed metastases nor died of disease. Both the type of treatment and localization in the proximal phalanx were associated independently with local recurrence.

CONCLUSIONS

Phalangeal chondrosarcoma behaves as a locally aggressive lesion and, in contrast to chondrosarcomas located elsewhere, rarely metastasizes. Treatment is indicated only because of its locally destructive growth. The authors believe that given the excellent survival data, curettage with adequate follow-up should be considered as the treatment of choice if technically feasible, especially in those cases in which amputation would lead to a significant loss of hand function.

摘要

背景

内生软骨瘤是手部最常见的原发性良性骨肿瘤,尤其是在指骨,而软骨肉瘤在该部位并不常见。尽管指骨软骨肉瘤可能具有不祥的组织学特征,但其生物学行为相对惰性。

方法

研究了35例先前诊断为软骨肉瘤的指骨病变。研究了组织学和肿瘤生物学参数(Ki-67和p53免疫组织化学)并与临床行为相关联。

结果

所有病例均具有明确的恶性组织学特征(2级或更高)或1级恶性组织学特征并伴有皮质破坏和软组织扩展。诊断时患者的中位年龄为67岁(范围21-87岁),女性略占优势。手部发病比足部更常见,近端指骨最常受累。治疗方法从16例患者的局部治疗(刮除术或局部切除术)到19例患者的截肢或关节离断术不等。28例患者的随访时间为8-432个月。15例接受局部治疗的肿瘤中有10例复发,而13例接受根治性手术的肿瘤均未复发。中位生存期为20.8年;28例患者均未发生转移,也没有死于该疾病。治疗类型和近端指骨的定位均与局部复发独立相关。

结论

指骨软骨肉瘤表现为局部侵袭性病变,与其他部位的软骨肉瘤不同,很少发生转移。仅因其局部破坏性生长才需要治疗。作者认为,鉴于良好的生存数据,如果技术上可行,刮除术并进行充分随访应被视为首选治疗方法,尤其是在截肢会导致手部功能严重丧失的情况下。

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