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原发性骨恶性纤维组织细胞瘤:6例报告及超微结构研究与文献分析

Primary malignant fibrous histiocytoma of bone: report of six cases with ultrastructural study and analysis of the literature.

作者信息

Ghandur-Mnaymneh L, Zych G, Mnaymneh W

出版信息

Cancer. 1982 Feb 15;49(4):698-707. doi: 10.1002/1097-0142(19820215)49:4<698::aid-cncr2820490418>3.0.co;2-7.

DOI:10.1002/1097-0142(19820215)49:4<698::aid-cncr2820490418>3.0.co;2-7
PMID:6948602
Abstract

Six cases of malignant fibrous histiocytoma (MFH) primary in bone were studied clinicopathologically with ultrastructural study of three. The histologic differentiation of MFH from osteosarcoma and from fibrosarcoma is discussed. Two of the six cases had highly malignant tumors histologically designated as Grade III and IV; one died with diffuse metastatic disease eight months after diagnosis and the second developed pulmonary metastases within ten months. Review of the literature revealed that 45 of the total 119 reported cases had been followed for less than five years; these cases were considered unevaluable for assessing biologic behavior. The remaining 74 cases, followed for more than five years or until death, showed a 36.5% five-year survival rate. All but one of the cases which developed metastasis within five years did so within the first two years. It is therefore concluded that MFH of bone is a rapidly metastasizing tumor with a relatively low five-year survival rate. Paradoxically, 13 of these 74 cases (17.6%) survived between five and 38 years with no evidence of disease. This is comparable to the long-term survival rate in osteosarcoma. The five-year survival rate is also comparable to that of fibrosarcoma of bone.

摘要

对6例原发性骨恶性纤维组织细胞瘤(MFH)进行了临床病理研究,并对其中3例进行了超微结构研究。讨论了MFH与骨肉瘤及纤维肉瘤的组织学鉴别。6例中有2例组织学上为高度恶性肿瘤,分级为III级和IV级;1例在诊断后8个月死于弥漫性转移性疾病,另1例在10个月内发生肺转移。文献复习显示,119例报告病例中,45例随访时间不足5年;这些病例被认为无法评估生物学行为。其余74例随访时间超过5年或直至死亡,5年生存率为36.5%。5年内发生转移的病例,除1例外在头两年内均发生转移。因此得出结论,骨MFH是一种转移迅速的肿瘤,5年生存率相对较低。矛盾的是,这74例中有13例(17.6%)存活了5至38年,无疾病证据。这与骨肉瘤的长期生存率相当。5年生存率也与骨纤维肉瘤相当。

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