目的 学习母细胞性浆细胞样树突细胞肿瘤(BPDCN)的临床病理及免疫表型特征,总结该少见肿瘤的病理诊断经验。方法 回顾分析2例BPDCN患者临床资料,通过苏木素-伊红(HE)染色分析肿瘤组织及细胞形态,通过免疫组织化学染色分析肿瘤免疫表型,通过流式细胞学检测骨髓有无肿瘤侵犯,并结合文献分析。结果 本报道中1例为97岁女性,临床以皮肤瘀斑结节为首发症状,肿瘤细胞真皮内弥漫浸润,不侵犯表皮,细胞中等大小,核形不规则,核仁不明显。另1例为69岁男性,临床以淋巴结肿大为首发症状,淋巴结结构完全破坏,肿瘤细胞弥漫浸润,细胞呈中等大小的母细胞样,核仁明显。2例免疫表型均表达CD123、CD4、CD56、TDT,不表达B系、T系淋巴细胞及髓系标志物,肿瘤均累及骨髓。结论 BPDCN是一种罕见的淋巴造血肿瘤,临床常以皮肤病变或淋巴结肿大为首发症状,临床过程具高度侵袭性,通常伴有骨髓侵犯。该肿瘤需与具有母细胞形态的淋巴系肿瘤和白血病相鉴别,诊断需结合临床信息、HE形态及免疫组化结果综合判断。

Objective To summarize the diagnostic experiences of blastic plasmacytoid dendritic cell neoplasm (BPDCN) based on the study of its clinicopathological features and immunophenotypes. Methods The clinical data of 2 patients with BPDCN were collected, the structure alteration and cell morphology were observed by HE staining, the immunophenotype of tumor cells were studied by immunohistochemistry staining and flow cytometry was adopted to confirm the bone marrow involvement. Results Two patients, one of whom was a 97 year-old female, presented with cutaneous ecchymosis nodules as the first symptom. The epidermis, but not the dermal, was diffusedly infiltrated by tumor cells, which were medium-sized with irregular nuclei without prominent nucleoli. The other case was a 69 year-old male with lymph node enlargement as the first symptom. The skin was normal, but the lymph nodes were invasively destroyed by tumor cells, which were medium-sized blast-like with prominent nucleoli. The immunophenotypes of the two patients were both positive for CD123, CD4, CD56 and TDT, but negative for B, T lymphocyte derived and myeloid origin markers, both of which involved bone marrow. Conclusions BPDCN is a rare form of hematological neoplasm, skin symptoms or lymph node enlargement may be presented as the initial symptom, the clinical course were highly aggressive with high frequency of bone marrow involvement. The blastic-like lymphoma and leukemia entities should be considered into account for differential diagnose. The precise diagnosis of BPDCN should be established by integrating histomorphology, immunophenotype and clinical presentation information comprehensively.

目的 介绍1例罕见反转型滤泡性淋巴瘤的诊断、鉴别诊断及文献复习。方法 对1例发生于儿童颈部淋巴结内淋巴瘤进行HE组织形态学、免疫组织化学、荧光原位杂交评估并复习相关文献,进行综合分析。结果 淋巴结结构消失,可见结节状靶环样结构,局灶弥漫。结节中央为非肿瘤性的生发中心（表达CD10、BCL-6,不表达BCL-2及IgD,Ki-67高表达）,紧接着为非肿瘤性套区结构（表达BCL-2及IgD,Ki-67低表达）,最外层为淡染区域,由肿瘤性中心细胞及中心母细胞组成（表达CD10、BCL-6及BCL-2）;局灶弥漫区细胞形态学及其免疫表型同结节状靶环样结构套区外淡染区域。荧光原位杂交未检测到BCL-2基因断裂。结论 本例与文献中报道的反转型滤泡性淋巴瘤（RVFL）有部分相似的特征,但又具有自身的一些特点。

目的 探讨CD117在肺基底样细胞鳞状细胞癌(BSCC)与小细胞肺癌(SCLC)中的鉴别诊断作用。方法 免疫组织化学检测CD117在肺BSCC与SCLC中的表达情况,并进行统计学分析,同时文献复习CD117在这两种肿瘤中的表达情况。结果 CD117在SCLC中特异性高表达(阳性率为78%),并且具有高的敏感度、特异度、阳性预测值及阴性预测值(分别为100%、78.2%、68.7%、100%),与文献报道一致(阳性率37%～100%);而在BSCC中未见有CD117的表达,文献未见有CD117在BSCC中表达情况报道。结论 CD117有可能成为鉴别诊断BSCC与SCLC的重要指标之一。但由于本报道例数有限,还需要更大型的研究进一步证明CD117在鉴别诊断二者中的敏感度及特异度。

Objective To investigate the role of CD117 in differentiating basaloid squamous cell carcinoma (BSCC) from small cell carcinoma (SCLC) in lung. Methods Immunohistochemistry staining of CD117 and statistic data were analyzed in BSCC and SCLC, and relevant literature were reviewed. Results CD117 was specifically expressed in high level ( positive rate 78%) in SCLC with high sensitivity, specificity, positive predictive value and negative predictive value (respectively 100%、78.2%、68.7%、100%), which was highly consistent with literature reports (positive rate 37%-100%). CD117 was not detected in BSCC in our report and it was the same as in literatures. Conclusion Although our results showed that it was possible that the expression of CD117 would play an important role in differentiating BSCC from SCLC, due to limited cases in number, more studies are needed to elucidated the sensitivity and specificity.

目的 分析总结12例原发性肺淋巴上皮瘤样癌(LELC)的临床病理特点。方法 回顾性分析12例肺LELC患者的临床病理资料。结果 12例肺LELC患者的组织学标本中,肿瘤细胞均明显表达HCK,原位杂交EBER阳性率为91.7%。全组中位生存期61.3个月,2年和5年生存率分别为84.6%和57.7%。结论 原发性肺LELC临床罕见,发病可能与EB病毒感染有关,经及时治疗有较好的预后。

Objective To investigate the pathological and clinical significance of 12 cases with primary Lymphoepithelioma-like carcinoma of the lung (LELC). Methods Retrospectively detectded and analyzed the clincalpathological feature of 12 cases of LELC of the lung. Results Histological study showed that tumor cells were significantly expressed HCK by immunohistochemistry staining and showed out 91.7% positive rate of EBER by situ hybridization. In this series, the median survival time was 61.3 months. The overall 2 and 5 year survival rates of the 12 cases were 84.6% and 57.7%. Conclusion Pulmonary LELC is very rare. It may be associated with Epstein-Barr virus infection, and has a batter prognosis after therapy.