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目的探讨指状突树突细胞肉瘤(IDCS)的临床病理特征、免疫表型及其鉴别诊断。方法报道2例少见的指状突树突细胞肉瘤并行文献复习。结果 2例患者临床症状均为局部无痛性淋巴结肿大,结外皮肤受累。镜下见肿瘤组织边界不清楚呈巢片状,部分为束状、旋涡状排列;瘤细胞弥漫分布,呈梭形、圆形、椭圆形,胞质丰富透亮或轻度嗜酸性,核卵圆形、空泡状,核仁清楚,部分有核沟,有异型性,可见核分裂象。肿瘤通常无坏死,周边常有较多淋巴细胞、浆细胞浸润。免疫组化示瘤细胞vimentin和S-100(+),CD68(+)/(-),Ki-67部分(+);CD21、CD35,HMB45、melanA、CD4和CgA(-)。结论指状突树突细胞肉瘤是一种罕见的组织细胞和树突细胞的恶性肿瘤,确诊依靠病理检查及免疫组化,必要时可电镜辅助诊断。临床应与其他类型的组织细胞肿瘤、恶性黑色素瘤和软组织肿瘤等鉴别。肿瘤单发以手术完整切除为主,可能治愈;而多发或多器官累及者可手术结合放、化疗,但目前尚无公认有效的标准治疗方案。
Objective To investigate the clinicopathological characteristics, immunophenotype and differential diagnosis of finger-like dendritic cell sarcoma (IDCS). Methods Two cases of rare finger dendritic cell sarcoma were reviewed. Results The clinical symptoms of 2 patients were all local painless lymphadenopathy, extracranial skin involvement. Microscopically, the tumor tissue boundary was unclear and lamellar, some of them were bundles and arranged in a swirling pattern. The tumor cells were diffusely distributed with fusiform, round, oval and abundant cytoplasm translucent or slightly eosinophilic nuclei Shaped, vacuolar, clear nucleolus, part of a nuclear ditch, with atypia, mitotic figures can be seen. Tumors are usually no necrosis, often more peripheral lymphocytes, plasma cell infiltration. Immunohistochemistry showed tumor cells vimentin and S-100 (+), CD68 (+) / (-), Ki-67 part (+); CD21, CD35, HMB45, melanA, CD4 and CgA (-). Conclusion Finger-like dendritic cell sarcoma is a rare malignant tumor of tissue cells and dendritic cells. The diagnosis depends on pathological examination and immunohistochemistry, and may be assisted by electron microscopy if necessary. Clinical and other types of tissue cell tumors, malignant melanoma and soft tissue tumor identification. Tumor single surgery with complete removal of the main may be cured; and multiple or multiple organ involvement surgery and radiotherapy and chemotherapy, but there is no recognized effective standard treatment.