Li Yuan, Xu Xiao-li, Wang Jian
Department of Pathology, Shanghai Miedical College, Fudan University, Shanghai 200032, China.
Zhonghua Bing Li Xue Za Zhi. 2011 Jun;40(6):363-7.
To study the clinicopathologic features of leiomyosarcoma with prominent osteoclast-like giant cells.
The clinical and pathologic features of 7 cases of leiomyosarcoma with prominent osteoclast-like giant cells were analyzed. Immunohistochemical and ultrastructural studies were performed. The literature was reviewed.
All cases occurred in adults, with a mean age of 63 years. There was no significant sex predilection (male-to-female ratio = 4:3). The tumor involved subcutaneous soft tissue of thigh (number = 2), left back (number = 1), retroperitoneum (number = 1), small intestine (number = 1), breast (number = 1) and uterus (number = 1). Histologic examination showed that the tumor was composed of relatively uniform spindly cells arranged in interlacing fascicles. The hallmark was the presence of prominent osteoclast-like giant cells, either intimately admixed with the spindly cells (number = 6) or forming giant cell tumor-like nodules (number = 1). Immunohistochemically, the spindly cells expressed smooth muscle actin, muscle-specific actin, desmin and h-caldesmon in various degrees, whereas the osteoclast-like giant cells expressed CD68. Ultrastructural study showed smooth muscle differentiation in the spindly cells and histiocytic differentiation in the osteoclast-like giant cells. Follow-up data were available in 6 cases. There were local recurrences and/or metastases in all the 6 patients. Three patients were alive with unresectable or recurrent/metastatic disease and two patients died of the disease.
Leiomyosarcoma with prominent osteoclast-like giant cells is a rare variant of leiomyosarcoma which should be distinguished from the so-called giant cell variant of malignant fibrous histiocytoma. The osteoclast-like giant cells are of histiocytic differentiation. Surgical resection remains the mainstay of management of this high-grade sarcoma.
研究具有显著破骨细胞样巨细胞的平滑肌肉瘤的临床病理特征。
分析7例具有显著破骨细胞样巨细胞的平滑肌肉瘤的临床和病理特征。进行免疫组织化学和超微结构研究。并复习相关文献。
所有病例均发生于成年人，平均年龄63岁。无明显性别倾向（男∶女 = 4∶3）。肿瘤累及大腿皮下软组织（2例）、左背部（1例）、腹膜后（1例）、小肠（1例）、乳腺（1例）和子宫（1例）。组织学检查显示，肿瘤由排列成交错束状的相对一致的梭形细胞组成。其特征是存在显著的破骨细胞样巨细胞，要么与梭形细胞紧密混合（6例），要么形成巨细胞瘤样结节（1例）。免疫组织化学显示，梭形细胞不同程度地表达平滑肌肌动蛋白、肌肉特异性肌动蛋白、结蛋白和h - 钙调蛋白，而破骨细胞样巨细胞表达CD68。超微结构研究显示梭形细胞有平滑肌分化，破骨细胞样巨细胞有组织细胞分化。6例有随访资料。所有6例患者均有局部复发和/或转移。3例患者存活，伴有不可切除或复发/转移性疾病，2例患者死于该病。
具有显著破骨细胞样巨细胞的平滑肌肉瘤是平滑肌肉瘤的一种罕见变异型，应与所谓恶性纤维组织细胞瘤的巨细胞变异型相鉴别。破骨细胞样巨细胞具有组织细胞分化。手术切除仍然是这种高级别肉瘤治疗的主要方法。
