摘要
目的探讨Langerhans细胞肉瘤(Langerhans cell sarcoma,LCS)的临床病理学特征、诊断及鉴别诊断。方法收集2014-12—2020-08攀枝花市中心医院1例LCS和宜宾市第二人民医院1例LCS,回顾性分析其临床特征、组织形态学、免疫表型、BRAF V600E基因突变情况及预后,并复习相关文献。结果例1男性,年龄58岁;例2女性,年龄43岁。前者就诊时表现为全身多发淋巴结肿大及肝脾大,后者以多发性皮损为首发症状。病理组织学显示低倍镜下淋巴结和皮肤真皮层、皮下脂肪组织间炎症背景中见散在或成片肿瘤细胞,高倍镜下瘤细胞形态多样,异型性明显,核分裂象及核沟易见。免疫组织化学染色显示肿瘤细胞弥漫性表达S-100,不同程度表达Langerin、CD1a、CD68。Ki-67增殖指数分别为70%、80%。分子检测显示例2存在BRAF V600E基因突变。例1术后随访8个月死亡,例2术后行多药物联合化疗,随访12个月病情稳定。结论LCS是一种罕见的高度侵袭性肿瘤,预后较差。它具有独特的形态学、免疫表型,需要与多种恶性肿瘤进行鉴别,仔细的形态学观察并适当地应用免疫组织化学染色可准确地鉴别。
Objective To explore the clinicopathological characteristics,diagnosis and differential diagnosis of Langerhans cell sarcoma(LCS).Methods Two cases of LCS were collected from December 2014 to August 2020.The clinical feathers,histomorphology,immunohistochemistry,BRAF gene mutation and prognosis were retrospectively analyzed and the relevant literature was reviewed.Results Two cases included 1 men and 1 women,aged 58 and 43 years respectively.The former presented with systemic multiple lymph node enlargement and hepatosplenomegaly;the later presented with multiple skin lessons.Histopathology showed scattered or diffuse tumor cells were seen in the inflammatory background of lymph node,dermis and subcutis.At the high power field,the heteromorphism of the tumor cells was great and the morphologic features were various.Mitotic activity and nuclear groove were noted.Immunohistochemical study showed that the tumor cells were diffusely positive for S-100 protein,expressed langerin,CD1 a,CD68 in varying degrees.Ki-67 proliferative index was 70%and 80%respectively.Molecular detection showed that case 2 had BRAF V600 E mutation.At follow-up of 8 months,case 1 was died,while case 2 showed stable disease was achieved within 12 months when treated with combinations of multidrug chemotherapy.Conclusion LCS is a rare highly aggressive neoplasm with poor prognosis.It has distinctive morphologic,immunohistochemical characteristics and needs to be distinguished from other malignant tumors.Careful attentions to its characteristic histomorphology with the judicious use of immunohistochemistry can help to distinguish this tumor from its many mimickers.
作者
施登超
杨阳
王代文
景兰兰
SHI Deng-chao;YANG Yang;WANG Dai-wen;JING Lan-lan(Department of Pathology,Central Hospital of Panzhihua,Panzhihua 617000,China;Department of Pathology,the Second People's Hospital of Yibin,Yibin 644000,China)
出处
《诊断病理学杂志》
2022年第9期826-829,833,共5页
Chinese Journal of Diagnostic Pathology
