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佩吉特骨肉瘤和放疗后骨肉瘤:新西兰米德尔莫尔医院的继发性骨肉瘤

Paget's osteosarcoma and post-radiation osteosarcoma: secondary osteosarcoma at Middlemore Hospital, New Zealand.

作者信息

Dray Michael S, Miller Mary V

机构信息

Department of Anatomical Pathology, Laboratory Services, Middlemore Hospital, Otahuhu, Auckland, New Zealand.

出版信息

Pathology. 2008 Oct;40(6):604-10. doi: 10.1080/00313020802320663.

Abstract

AIMS

To review the clinical and histological features of our cases of Paget's osteosarcoma and post-radiation osteosarcoma.

METHODS

A search through the files of the New Zealand Bone and Soft Tissue Tumour Registry was performed, patients were identified and the relevant details were collated.

RESULTS

Thirty-one cases of Paget's osteosarcoma and eight cases of post-radiation osteosarcoma were identified. Patients with Paget's osteosarcoma were aged between 48 and 67 years, predominantly female, and axial and appendicular skeleton were equally affected. The outcome was known in 29 cases, with a median survival of 7.25 months and 5 year overall survival of 10%. Patients with post-radiation osteosarcoma were aged between 17 and 68 years, equally male or female, and axial and appendicular skeleton were equally affected. Index lesions included benign or malignant osseous and non-osseous conditions. The average age at diagnosis of the index lesion was 30.1 years and the average latent period was 13.5 years. The outcome was known in all eight cases, with a median survival of 33 months and 5 year overall survival of 38%.

CONCLUSIONS

Paget's osteosarcoma and post-radiation osteosarcoma are examples of secondary osteosarcoma. The former affects elderly patients, and has a poor prognosis and response to treatment. The later affects a wide age group, and has a prognosis and response to treatment comparable with primary osteosarcoma.

摘要

目的

回顾我们所诊治的佩吉特骨肉瘤和放疗后骨肉瘤病例的临床及组织学特征。

方法

检索新西兰骨与软组织肿瘤登记处的档案,确定患者并整理相关细节。

结果

共确定31例佩吉特骨肉瘤和8例放疗后骨肉瘤。佩吉特骨肉瘤患者年龄在48至67岁之间,以女性为主,轴向和附属骨骼受累情况相同。29例患者的预后情况已知,中位生存期为7.25个月,5年总生存率为10%。放疗后骨肉瘤患者年龄在17至68岁之间,男女比例相同,轴向和附属骨骼受累情况相同。索引病变包括良性或恶性骨及非骨病变。索引病变的平均诊断年龄为30.1岁,平均潜伏期为13.5年。8例患者的预后情况均已知,中位生存期为33个月,5年总生存率为38%。

结论

佩吉特骨肉瘤和放疗后骨肉瘤是继发性骨肉瘤的例子。前者影响老年患者且预后差,对治疗反应不佳。后者影响广泛的年龄组,其预后和对治疗的反应与原发性骨肉瘤相当。

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