大量骨质溶解症的研究进展

Research progress on massive osteolysis

大量骨质溶解症是一种病因不明,主要侵犯骨骼的罕见疾病。典型表现为X线片上患骨溶解消失,病变可越过关节和关节盘侵犯邻近骨。病理表现为骨小梁间血管纤维和(或)结缔组织增生。通常认为该病属于良性血管肿瘤或脉管畸形,可导致畸形和残疾,在合并乳糜胸和侵犯颈椎时可危及生命。该病国外已有百余例报道,国内亦有部分文献报道,但大部分报道仅为诊断,缺乏有效的治疗。本文针对大量骨质溶解症的病理、临床表现、辅助检查、诊断标准和治疗方法作一综述。

Massive osteolysis is a rare disease of unknown etiology primarily affecting bones. It is characterized by lysis of affected bone on radiograph, which can get across joints and intervertebral disks to adjacent bone by progression. Histologically, it is characterized by nonmalignant vascular proliferation with fibrosis background of intertrabecular spaces or in later phases dense fibrotic tissue with minimal vascularity. It is generally classified as a benign vascular tumor or vascular malformation. The disease can cause deformity and disability, with chylothorax and cervical vertebral involvement it can be lethal. More than one hundred cases have been reported in the Eng- lish literature. Several cases have been reported in China, but most of them lack effective treatment. This review was focused mainly upon the pathology, clinical features, laboratory tests and radiographic appearance, diagnosis criteria and treatment modalities of the disease.