骨原发性恶性纤维组织细胞瘤的病理研究

A Pathological Study of Malignant Fibrous Histiocytoma

本文报道97例骨原发性恶性纤维组织细胞瘤,发病年龄以中、老年人(31～60岁)为最多,发病部位几遍及全身各骨,惟好发于四肢长骨(69例,71.13%),尤以股骨下端(28例)及胫骨上端(15例)为最多,两者合计为43例(44.33%),在随访之62例中,仅2例分化高者,仍存活,其余60例,大都在9个月至2年内死亡(41例,占62例的66.13%),其余19例(30.65%)均在3～5年内死亡。此瘤来源于具有多能性分化的间充质细胞,后者分化为组织细胞及纤维母细胞等,构成瘤细胞的主要成分,同时伴有炎细胞及良、恶性多核巨细胞,由于这些细胞的分布不匀,易致误诊。在本组之97例中,有28例初诊对仅凭一张切片做出诊断,误诊为其它恶性肿瘤,经进一步多处取材活检,始予确诊为骨MFH。

Ninety-seven cases of primary malignant fibrous histiocytoma (MFH) of bone were presented, among which, 62 cases (63.92%) were followed-up. The highest incidence occurred at age of 31～60 years. In this series, 69 cases (71.13%) of the tumor located in the long bone of extremities and 43 cases (44.33%) were around the knee.The data of 62 cases followed-up revealed that most of them died within 9 months to 2 years(41 cases. 66.13%), 19 cases died within 3-5 years; and only 2 cases with highly differentiated tumor survive 8 and 10 years.The cellular components of MFH were composed of an admixture of various types of tumor cells, which were histiocytic, fibro-blastic, intermediate (fibrohistiocyte) and tumor giant cells, accompanied with some benign and malignant multinucleated giant cells and inflammatory cells. Our study supports the concept that MFH arises from primitive mesenchymal cells which are capable to differentiate toward histiocytic and fibroblastictumor cells. The uneven distribution of the various tumor cells in the same or different specimens, often caused misdiagnosis. Differential diagnosis of MFH and osteosarcoma, fibrosarcoma, malignant giant cell tumor was discussed also.