摘要
目的探讨骨未分化高级别多形性肉瘤 （ undifferentiated high-grade pleomorphic sarcoma, UPS）的临床病理学特征、诊断及鉴别诊断。方法结合临床资料及影像学所见，分析8例骨UPS的组织病理学特征，并复习相关文献。结果8例UPS中男性4例，女性4例，患者年龄21-69岁，平均47．5岁，均以疼痛性骨性包块入院。病变累及肱骨近端3例，股骨下段2例，上颌窦、尺骨远端及腓骨上段各1例。X线检查示侵袭性溶骨性病变，表现为渗透性破坏伴明显的软组织肿块，但缺乏骨膜反应。大体特征与一般恶性（肌）纤维源性骨肿瘤无异，缺乏矿化。镜下见肿瘤与相应同类软组织肿瘤一样，异质性显著。多核巨细胞、泡沫细胞及慢性炎细胞主要由纤维母细胞及组织细胞样细胞组成，常见席纹状——多形性结构，几乎无钙化及肿瘤性骨组织形成，常出现细胞非典型、核分裂及坏死。免疫组化仅恒定表达vimentin，治疗与普通型骨肉瘤相似。结论骨UPS是高度恶性肿瘤，以细胞弥漫多形性及缺乏具体分化为特点，熟悉其形态学特征有助于正确诊断。
Purpose To investigate the clinicopathologic features, diagnosis and differential diagnosis of undifferentiated high-grade pleomorphic sarcoma （UPS） of the bone. Methods Eight cases of UPS were analyzed histopathologically in combination with clinical data and image findings, and the related literature was reviewed. Results The patients including 4 males and 4 females were admitted to the hospital for painful and palpable masses found in the bones. And their ages ranged from 21 to 69 years （mean 47.5 years）. Tumors involved in proximal humera in 3 cases, distal femur in 2 cases, maxillary sinus, the distal alna and proximal fibula each in 1 case. The X-ray revealed an aggressive osteolytic lesion with permeative distruction and extending into the soft tissue at the involved bone. Yet periosteal reaction was rare. Grossly, lack of mineralization, the tumor was displayed as an appearance of （myo） fibrogenic malignancies of the bone. Microscopically, like its soft tissue counterpart, the patterns of UPS of bone were quite heterogeneous. It consisted mainly of a mixed population of fibroblasts and histiocytoid cells in a background of varying amounts of muhinucleated giant cells, foamy cells and chronic inflammatory cells. The storiform pattern was commonly seen. However, calcifications and tumor bone production was hardly observed. Meanwhile, cytological atypia, mitoses and necroses were often presented. Immunohistochemically, the tumor cells were consistently positive for vimentin. Its treatment was similar to that of conventional osteosarcoma. Conclusions UPS of the bone is characterized by tumor cells with diffuse pleomorphism in the absence of a specific line of differentiation. Familiarity with its morphologic profiles is helpful to making correct diagnosis.
出处
《临床与实验病理学杂志》
CAS
CSCD
北大核心
2014年第2期171-175,共5页
Chinese Journal of Clinical and Experimental Pathology
关键词
骨肿瘤
未分化高级别多形性肉瘤
临床病理
鉴别诊断
bone neoplasms
undifferentiated high-grade pleomorphic sarcoma
clinicopathology
differential diagnosis
