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颌骨间叶性软骨肉瘤:19例临床病理研究

Mesenchymal chondrosarcoma of the jaw bones: clinicopathologic study of 19 cases.

作者信息

Vencio E F, Reeve C M, Unni K K, Nascimento A G

机构信息

Department of Laboratory Medicine and Pathology, Mayo Clinic and Mayo Foundation, Rochester, Minnesota 55905, USA.

出版信息

Cancer. 1998 Jun 15;82(12):2350-5. doi: 10.1002/(sici)1097-0142(19980615)82:12<2350::aid-cncr8>3.0.co;2-w.

Abstract

BACKGROUND

Patients with mesenchymal chondrosarcomas in general have a 5-year survival rate ranging from 42-54.6% and a 10-year survival rate of 28%. Nineteen cases of mesenchymal chondrosarcoma of the jaw bones were reviewed to study their clinicopathologic features and to compare their clinical behavior with that of mesenchymal chondrosarcomas of other locations.

METHODS

The patients were 10 males and 9 females (age range, 2-51 years). Sixteen patients were age < 30 years, and the average age at presentation was 19 years. Eleven tumors involved the mandible and eight involved the maxilla.

RESULTS

Histologically, the classic picture of a bimorphic tumor, composed of islands of well differentiated hyaline cartilage juxtaposed to a small cell undifferentiated malignancy, was present in every case. Resection, including hemimandibulectomy and hemimaxillectomy, was the main treatment in 16 patients. Seven patients had local recurrence, and five patients developed distant metastases. Six patients died of disease, and the 5-year and 10-year survival rates were 82% and 56%, respectively.

CONCLUSIONS

Mesenchymal chondrosarcoma of the jaw bones appears to have a more indolent course than mesenchymal chondrosarcoma of other anatomic

摘要

背景

间叶性软骨肉瘤患者的总体5年生存率为42%-54.6%,10年生存率为28%。回顾19例颌骨间叶性软骨肉瘤病例,以研究其临床病理特征,并将其临床行为与其他部位的间叶性软骨肉瘤进行比较。

方法

患者中男性10例,女性9例(年龄范围2-51岁)。16例患者年龄<30岁,就诊时的平均年龄为19岁。11例肿瘤累及下颌骨,8例累及上颌骨。

结果

组织学上,每例均呈现典型的双相肿瘤表现,由分化良好的透明软骨岛与小细胞未分化恶性肿瘤并列组成。16例患者的主要治疗方法为切除,包括半侧下颌骨切除术和半侧上颌骨切除术。7例患者出现局部复发,5例患者发生远处转移。6例患者死于疾病,5年和10年生存率分别为82%和56%。

结论

颌骨间叶性软骨肉瘤的病程似乎比其他解剖部位的间叶性软骨肉瘤更为惰性。

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