Histological and clinical characteristics of malignant giant cell tumor of bone
Histological and clinical characteristics of malignant giant cell tumor of bone
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DOI：
10.1007/s00428-012-1198-y
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发表时间：
2012-03-01
期刊：
影响因子：
3.5
通讯作者：
Huang, XiaoYuan
中科院分区：
文献类型：
--
作者：
Gong, Lihua;Liu, Weifeng;Huang, XiaoYuan
恶性骨巨细胞瘤（MGCTB）很少见，由于多种含有巨细胞的恶性肿瘤的发生，诊断可能很困难。为了更好地了解其临床病理特征，我们回顾了 17 例 MGCTB 的经验。 5例为原发性恶性骨巨细胞瘤（PMGCTB），12例为最初诊断为良性但复发性病变（继发性MGCTB、SMGCTB）的恶性骨巨细胞瘤。患者包括 6 名女性和 11 名男性（年龄从 17 岁到 52 岁；平均 30.5 岁）。肿瘤发生于股骨（6例）、胫骨（7例）、肱骨（3例）和腓骨（1例）。显微镜下，PMGCTB同时表现出常规巨细胞瘤和恶性肉瘤的特征。 SMGCTB最初被诊断为常规骨巨细胞瘤，复发性病变呈现恶性特征。组织学上，恶性成分包括骨肉瘤（11例）、未分化高级别多形性肉瘤（2例）、纤维肉瘤（4例）。 SMGCTB 病例显示 p53 强表达。随访信息显示，4名患者因肺转移死亡，2名肺转移患者存活，11名无肿瘤患者存活。 MGCTB 应被视为高级肉瘤。必须与GCTB及其他含有巨细胞的恶性肿瘤相鉴别。 p53可能在GCTB的恶性转化中发挥作用。
Malignant giant cell tumors of bone (MGCTB) are rare, and the diagnosis can be difficult due to the occurrence of a variety of malignant tumors containing giant cells. To better understand its clinicopathological features, we have reviewed our experience with 17 cases of MGCTB. Five cases were primary malignant giant cell tumor of bone (PMGCTB), and 12 cases were giant cell tumors of bone initially diagnosed as benign but malignant in a recurrent lesion (secondary MGCTB, SMGCTB). The patients included six women and 11 men (age ranged from 17 to 52 years; mean, 30.5 years). The tumor arose in the femur (six cases), the tibia (seven cases), the humerus (three cases), and the fibula (one case). Microscopically, PMGCTB showed both conventional giant cell tumor and malignant sarcoma features. SMGCTB were initially diagnosed as conventional giant cell tumor of bone, the recurrent lesion showing malignant features. Histologically, the malignant components included osteosarcoma (11 cases), undifferentiated high-grade pleomorphic sarcoma (two cases), and fibrosarcoma (four cases). SMGCTB cases showed strong expression of p53. Follow-up information revealed that four patients died of lung metastasis, two patients are alive with lung metastases, and 11 patients are alive without tumor. MGCTB should be considered as a high-grade sarcoma. It must be distinguished from GCTB and other malignant tumors containing giant cells. p53 might play a role in the malignant transformation of GCTB.