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Clinical characteristics and prognoses of six patients with multicentric giant cell tumor of the bone

机译:骨多中心巨细胞瘤6例临床特点及预后

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摘要

Multicentric giant cell tumor of the bone (MGCT) is a rare entity whose radiographic, pathological and biological features remain confusing. We retrospectively reviewed six patients (1 male, 5 female; average age, 22.33 years) treated for confirmed MGCT between 2001 and 2015. The patients' clinical information, images from radiographs (n = 14), CT (n = 13), MRI (n = 8), bone scintigraphy (n = 1) and PET-CT (n = 2), as well as histologic features, treatment and prognosis were analyzed. A total of 17 lesions were detected: 4 around the knee joint, 3 in the greater trochanter and head of the femur, 5 in the small bones of the feet, and 2 in flat bones. All these lesions occurred in an ipsilateral extremity. One patient had Paget's disease. On radiographs and CT, 12 lesions exhibited sclerotic margins or patchy sclerosis, 8 showed cortical discontinuity, and 5 showed soft tissue masses. On histopathology, 8 lesions showed signs of sarcomatous transformation and one had transformed into osteosarcoma. Ten lesions in 4 patients were initially treated with surgery, and 3 showed local recurrence. Seven lesions in 3 patients were treated with denosumab. All the patients are currently stable without metastasis. These results suggest MGCT tends to occur in uncommon sites with sclerosis. Because these lesions can be aggressive, patients should be carefully monitored for the recurrence or formation of other lesions, especially in an ipsilateral extremity.
机译:骨多中心巨细胞瘤(MGCT)是一种罕见的实体,其放射学,病理学和生物学特征仍然令人困惑。我们回顾性分析了2001年至2015年间接受确诊MGCT的6例患者(男性1例,女性5例;平均年龄22.33岁)。患者的临床信息,X射线照片(n = 14),CT(n = 13),MRI分析(n = 8),骨闪烁显像(n = 1)和PET-CT(n = 2)以及组织学特征,治疗和预后。总共检测到17个病变:膝关节周围4个,大转子和股骨头3个,脚小骨5个,扁平骨2个。所有这些病变都发生在同侧肢体中。一名患者患有佩吉特氏病。在X线照片和CT上,有12个病变表现出硬化边缘或斑状硬化,8个表现出皮质间断,5表现出软组织肿块。在组织病理学上,有8个病变显示出肉瘤转化的迹象,其中1个已转化为骨肉瘤。最初对4例患者的10个病变进行了手术治疗,其中3例表现出局部复发。 3例患者中有7个病变用地诺单抗治疗。目前所有患者均稳定无转移。这些结果表明,MGCT倾向于发生在罕见的硬化部位。由于这些病变可能是侵袭性的,因此应仔细监测患者其他病变的复发或形成,尤其是在同侧肢体中。

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