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目的 探讨骨原发性恶性纤维组织细胞瘤（ＭＦＨ）的形态特征和组织发生。方法 应用光镜、电镜观察骨ＭＦＨ的组织学结构和细胞形态；应用组织化学和免疫组织化学检测２１例骨ＭＦＨ中非特异性酯酶（ＡＮＡＥ），酸性磷酸酶（ＡＣＰ）的酶活性和α１－抗胰蛋白酶（α１－ＡＴ）、α１－抗胰糜蛋白酶（α１－ＡＣＴ）、ＣＤ６８、波形蛋白（Ｖｉｍｅｎｔｉｎ）、神经特异性烯醇化酶（ＮＳＥ）、嗜铬素Ａ（ＣｇＡ）和突触素（ＳＹ）的表达水平。结果 骨原发性ＭＦＨ由多种细胞成分组成，组织化学和免疫组织化学染色证实瘤细胞具有组织细胞或成纤维细胞特征。其中３例电镜观察到部分瘤细胞内有神经内分泌颗粒，这些细胞对ＮＳＥ、ＣｇＡ、ＳＹ阳性表达率分别为２０％、１８％、１５％。结论 我们研究结果支持骨原发性ＭＦＨ起源于原始间叶细胞，并指出骨ＭＦＨ少数瘤细胞可能伴有神经内分泌分化，同时对诊断和鉴别诊断进行了讨论。
Objective To investigate the morphological characteristics and histogenesis of primary malignant fibrous histiocytoma (MFH) of bone. Methods The histological structure and cell morphology of bone MFH were observed by light and electron microscopy. The enzyme activities of non-specific esterase (ANAE) and acid phosphatase (ACP) in bone MFH were detected by histochemistry and immunohistochemistry. Antitrypsin (α1-AT), α1-antitrypsin (α1-ACT), CD68, Vimentin, Neurospecific Enolase (NSE), Chromosomal A (CgA) and Synaptophysin (SY) expression level. Results The primary MFH of bone consisted of various cellular components. Histochemical and immunohistochemical staining confirmed that the tumor cells had the characteristics of histiocytes or fibroblasts. Among 3 cases of electron microscopy, neuroendocrine granules were observed in some of the tumor cells. The positive expression rates of these cells to NSE, CgA, SY were 20%, 18%, and 15%, respectively. Conclusion Our findings support the observation that bone primary MFH originated from the primitive mesenchymal cells, and pointed out that bone MFH oligodendrocytic cells may be accompanied by neuroendocrine differentiation. Diagnostic and differential diagnosis are also discussed.