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骨恶性巨细胞瘤的组织学和临床特征。

Histological and clinical characteristics of malignant giant cell tumor of bone.

机构信息

Department of Pathology, Beijing Jishuitan Hospital, Fourth Medical College of Peking University, Teaching Hospital of Tsinghua University, 31 Xinjiekou East Street, Xicheng District, Beijing 100035, China.

出版信息

Virchows Arch. 2012 Mar;460(3):327-34. doi: 10.1007/s00428-012-1198-y. Epub 2012 Feb 17.

Abstract

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.

摘要

骨恶性巨细胞瘤(MGCTB)较为罕见,由于多种含有巨细胞的恶性肿瘤的发生,其诊断较为困难。为了更好地了解其临床病理特征,我们回顾了我们治疗的 17 例 MGCTB 经验。其中 5 例为原发性骨恶性巨细胞瘤(PMGCTB),12 例为最初诊断为良性但在复发病灶中为恶性的骨巨细胞瘤(继发性 MGCTB,SMGCTB)。患者包括 6 名女性和 11 名男性(年龄 17-52 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)。镜下,PMGCTB 既有常规巨细胞瘤又有恶性肉瘤特征。SMGCTB 最初诊断为常规骨巨细胞瘤,复发病灶具有恶性特征。组织学上,恶性成分包括骨肉瘤(11 例)、未分化高级多形性肉瘤(2 例)和纤维肉瘤(4 例)。SMGCTB 病例中 p53 表达较强。随访信息显示,4 例患者死于肺转移,2 例患者带肺转移生存,11 例患者无肿瘤生存。MGCTB 应被视为高级别肉瘤。它必须与 GCTB 和其他含有巨细胞的恶性肿瘤相区别。p53 可能在 GCTB 的恶性转化中发挥作用。

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