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Gorham-Stout综合征(Gorham-Stout syndrome，GSS)是一种罕见的病因不明的疾病，可能发生在任何年龄，但在儿童和青年人中最常见，其特征是大块骨溶解为特征的类肿瘤样损害。患者常表现为多发骨溶解、肿胀、疼痛、活动受限，严重的引起病理性骨折。因该病发病率低，报道以病例报告为主，目前尚无明确的诊疗标准，对临床医生的参考价值有限。现针对Gorham-Stout综合征的病因及发病机制、临床表现、影像学表现、病理学表现、诊断、鉴别诊断、并发症、治疗及预后作一综述，以提高临床医生对这一疾病的认识。“,”As an extremely rare disorder of unknown etiology, Gorham-Stout syndrome (GSS) occurs at any age. However, it is frequently found in children and youth. It is characterized by large bone dissolving as a characteristic tumor-like lesion. Most patients often present with multiple osteolysis, swelling, pain, limited mobility and severe pathologic fractures. Due to a low incidence, most reports have focused upon individual cases. Without a definite diagnostic and therapeutic standard, it has limited reference values for clinicians. With a purpose of enhancing its clinical awareness, this review summarized the etiology, pathogenesis, clinical manifestations, imaging findings, pathological features, diagnosis, differential diagnosis, complications, treatments and prognosis of GSS.