组织细胞肉瘤的临床病理观察

Histiocytic sarcoma： a clinicopathologic study of 6 cases

目的探讨组织细胞肉瘤（histiocytic sarcoma，HS）的病理形态学、免疫表型、鉴别诊断和预后。方法分析6例HS患者临床病理资料，对标本行光镜观察、免疫组织化学EliVision法染色，并对患者进行6～36个月的随访。结果6例HS中男女各3例，年龄12～81岁（平均54．6岁）。发生部位为淋巴结（2例）、皮肤及软组织（4例）。组织形态改变：瘤细胞弥漫性浸润，呈中至大的多角形上皮样细胞，胞质丰富，嗜酸性，核圆形或不规则，空泡状，有1至多个明显核仁，核分裂象多见。细胞境界较清。6例均见双核瘤细胞，2例多形性明显，可见多核瘤巨细胞，3例可见灶状泡沫状胞质的瘤细胞，1例可见灶状肉瘤样梭性细胞区，2例可见噬血细胞现象。6例均见到多少不一的炎细胞背景。均弥漫表达白细胞共同抗原、CD4、CD68、CD163，溶菌酶染色的5例中4例阳性。4例得到随访资料，3例确诊后6—11个月内死亡，1例局限于皮肤及皮下软组织者已存活3年。结论明确HS诊断需结合形态改变与免疫表型。该病就诊时多处于进展期，对化疗反应差，但少数病变局限的病例不表现为侵袭性过程，预后较好。

Objective To study the morphologic features, immanophenotypes, differential diagnoses and prognosis of histiocytic sarcoma （HS）. Methods The clinical and pathologic findings of 6 cases of HS were reviewed. Immunohistochemical assay （Elivision staining） was also performed. Followup information was available in 4 patients. Results There were altogether 3 males and 3 females. The age of patients ranged from 12 to 81 years old （median = 54. 6 years）. The sites of involvement included lymph node （ number = 2 cases） and skin or soft tissue （ number = 4 cases）. The tumor was composed of sheets of large epithelioid cells with abundant eosinophilic cytoplasm, oval to irregular nuclei, vesicular chromatin and large nucleoli. Binucleated form was not uncommon. Two of the cases showed increased pleomorphism with multinucleated tumor giant cell formation. Focal cytoplasmic with foamy appearance was identified in 3 cases. One case demonstrated foci of spindly sarcomatoid appearance. Hemophagocytosis was identified in 2 cases. Mitotic figures were readily identified. The tumor cells were often accompanied by various numbers of inflammatory cells. Immunohistochemical study showed that all cases were diffusely positive for leukocyte common antigen, CD4, CD68 and CD163. Four of the 5 cases studied also expressed lysozyme. Amongst the 4 patients with follow-up information available, 3 died of the disease at 6 to 11 months interval after diagnosis. One patient, whose lesion was localized at the skin and soft tissue, survived for 3 years, with no evidence of tumor recurrence. Conclusions Accurate diagnosis of the HS is based on the combination of morphologic examination and immunohistochemical assay. HS often presents with clinically advanced disease and pursues an aggressive clinical course, with a poor response to therapy. However, a subset of cases presenting with clinically localized lesion may carry a relatively favorable long-term outcome.