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脂肪硬化性黏液纤维瘤:纤维发育不良的一种创伤后变体?4例报告并文献复习

Liposclerosing myxofibrous tumour: a traumatized variant of fibrous dysplasia? Report of four cases and review of the literature.

作者信息

Heim-Hall J M, Williams R P

机构信息

Department of Pathology, The University of Texas Health Science Center at San Antonio, San Antonio, TX 78229-3900, USA.

出版信息

Histopathology. 2004 Oct;45(4):369-76. doi: 10.1111/j.1365-2559.2004.01951.x.

Abstract

AIMS

To describe the pathological and radiological features of four cases of liposclerosing myxofibrous tumour (LSMFT). LSMFT is a benign fibro-osseous lesion of bone with a marked predilection for the intertrochanteric region of the proximal femur. It is characterized by a complex mixture of histological elements including fibrous dysplasia-like bony trabeculae, myxofibrous tissue, lipomatous areas, xanthoma cells and pseudo-Paget's bone. This lesion is not a universally accepted pathological entity and often appears in the literature under variants of fibrous dysplasia or other benign lytic bone lesions.

METHODS AND RESULTS

All lesions exhibited histological and/or radiological overlap with fibrous dysplasia. A relationship to trauma was noted in three of the cases. The hypothesis that these lesions represent a traumatized variant of fibrous dysplasia was explored. After reviewing the biomechanics of the proximal femur, a possible relationship between predilection of LSFMT for this anatomical region and increased susceptibility to fracture was noted.

CONCLUSIONS

We hypothesize that when fibrous dysplasia involves the proximal femur, it makes the bone more susceptible to fatigue fracture, thereby altering its histological appearance. The wide variety of histological patterns in LSMFT could represent the end result of repeated reaction to fatigue stresses.

摘要

目的

描述4例脂肪硬化性黏液纤维瘤(LSMFT)的病理和放射学特征。LSMFT是一种骨的良性纤维骨病变,明显好发于股骨近端的转子间区域。其特征是组织学成分复杂,包括纤维结构不良样骨小梁、黏液纤维组织、脂肪区域、黄色瘤细胞和假性佩吉特骨。这种病变并非普遍认可的病理实体,在文献中常以纤维结构不良的变体或其他良性溶骨性骨病变的形式出现。

方法与结果

所有病变在组织学和/或放射学上均与纤维结构不良有重叠。3例病例中发现与创伤有关。探讨了这些病变代表纤维结构不良的创伤性变体这一假说。在回顾股骨近端的生物力学后,注意到LSMFT对该解剖区域的偏好与骨折易感性增加之间可能存在的关系。

结论

我们推测,当纤维结构不良累及股骨近端时,会使骨骼更容易发生疲劳骨折,从而改变其组织学外观。LSMFT中多种多样的组织学模式可能代表了对疲劳应力反复反应的最终结果。

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