目的 通过学习1例少见的组织学特征与IgG4相关性淋巴结病类似的浆细胞型特发性多中心型Castleman病（PC-iMCD）,总结两种易误诊疾病的鉴别要点,提高病理诊断水平。方法 回顾性分析1例PC-iMCD患者临床资料,常规苏木素-伊红（HE）染色分析淋巴结组织结构及细胞形态,免疫组织化学染色及原位杂交分析免疫表型及EB病毒（EBV）感染状态,并结合文献分析讨论其与IgG4相关淋巴结病的鉴别诊断。结果 48岁女性患者,临床表现为口干、多饮、皮肤瘙痒伴全身多处淋巴结肿大。实验室检查血清IgG、IgA、IgM及IgE水平均升高,血清IgG4显著升高（14.7 g/L）,白介素- 6（IL-6）异常升高（150.84 pg/mL）。病理检查显示淋巴结生发中心萎缩,套区淋巴细胞呈“洋葱皮”样围绕生发中心排列,滤泡间区扩张,其内见大量成熟的浆细胞呈片状浸润,灶区见含铁血黄素沉积及血管增生;免疫组化染色显示IgG4阳性浆细胞数大于100/高倍视野,IgG4阳性细胞/IgG阳性细胞比值>40%,Kappa及Lambda轻链呈非限制性表达;EB病毒编码RNA原位杂交（EBER）阴性。结论 部分PC-iMCD与IgG4相关淋巴结病具有相似的组织病理学特征,单纯根据组织学及免疫表型难以将两者鉴别,正确诊断需结合IgG4相关病变诊断标准、排除性诊断标准、临床表现及实验室检查综合判断。

Objective By studying a rare case of the plasma cell type idiopathic multicentric Castleman disease（PC-iMCD）with histological characteristics similar to IgG4-related lymphadenopathy,the differential points of the two easily misdiagnosed diseases were summarized to improve the level of pathological diagnosis．Methods The clinical data of one patient with PC-iMCD were collected,the structure alteration and cell morphology were observed by hematoxylin-eosin（HE）stains．The immunophenotype of cells was marked by immunohistochemical staining and the infections status of EB virus was detected by in situ hybridization．Besides,the differential diagnosis between IgG4-RD and PC-iMCD were analyzed and discussed based on literature analysis．Results This article reported a 48-year-old female who was admitted to the hospital with dry mouth,polydipsia,skin itching and multiple lymphadenopathy．The levels of various classes of serum immunoglobulin were all increased,such as IgG,IgA,IgM and IgE．Specially,the serum IgG4 was also significantly increased（14.7 g/L）and interleukin 6（IL-6）was abnormally raised（150.84 pg/mL）．The pathological examination indicated that the lymph node germinal center was atrophied and mantle zones were expanded which were composed of concentric rings of lymphocytes in an “onion skinning” appearance．Besides,the interfollicular area was expanded in which mature plasma cells were infiltrated in sheet-like,hemosiderin was deposited as well as the vessels were proliferated．Immunohistochemistry showed that the number of IgG4-positive plasma cells was >100/HPF,IgG4/IgG-positive cells ratio was >40%,and Kappa and Lambda light chains were expressed unrestrictedly．In situ hybridization revealed that the expression of EBER was negative．Conclusions PC-iMCD and IgG4-related lymphadenopathy shares similar histopathological characteristics and it’s challenging to distinguish these two diseases by their histology and immunophenotype．In conclusion,the correct diagnosis should be combined with the diagnostic criteria,exclusion diagnostic criteria,clinical manifestations and related laboratory examinations．

目的 探讨原发于前列腺的产黏液尿路上皮型腺癌的临床病理特征、诊断及鉴别诊断。方法 对1例极其罕见的原发于前列腺的产黏液尿路上皮型腺癌病例的临床诊治经过、病理组织学及免疫组织化学特征进行观察和总结,并复习国内外相关文献。结果 患者77岁,因排尿困难入院, B超提示前列腺增大,前列腺异常回声性质待查;CT及肠镜检查均未发现膀胱及结直肠恶性肿瘤;血清PSA未见升高。在当地医院行前列腺穿刺检查,病理诊断为前列腺黏液腺癌。遂于我院行腹腔镜下前列腺根治手术,镜下表现为黏液腺癌伴多量黏液湖形成,并见尿路上皮的腺性化生及原位腺癌与黏液腺癌的移行过渡;免疫组化示CK7及34βE12弥漫表达,CDX-2及CEA局灶表达,其余CK20、β-catenin、GATA3、PSA、PSAP、AR及P504S均阴性。结论 原发于前列腺的产黏液尿路上皮型腺癌十分罕见,其预后差,对内分泌治疗不敏感,准确诊断将有利于指导临床医生选择正确的治疗方法及评估其预后。

Objective To investigate clinicopathological characteristics, diagnosis and differential diagnosis of primary mucin-producing urothelial-type adenocarcinoma of prostate. Methods We reported a rare case of mucin-producing urothelial-type adenocarcinoma of prostate and reviewed relevant literatures to discuss the clinicopathological features, diagnosis and differential diagnosis. Results In this case, the patient was a 77-year-old male with the history of dysuria. B-ultrasound indicated benign prostatic enlargement and abnormal echogenicity remained to be determined. CT scan and gastrointestinal endoscopy didn't show any evidence of bladder and colorectal tumor. No serum prostate-specific antigen (PSA) increased. The patient underwent laparoscopic radical resection of prostate cancer. Microscopically, the tumor presented as mucinous carcinoma, similar to colorectal mucinous carcinoma, but the migration from the normal prostatic urethra was observed and the urethral epithelium at the transitional site was characterized by adenoepithelial metaplasia and adenocarcinoma in situ. Immunohistochemical staining showed neoplastic cells were diffuse and strongly positive for CK7 and 34βE12, focally positive for CDX-2 and CEA and negative for CK20, β-catenin, GATA3, PSA, PSAP, AR and P504S. Conclusion Mucin-producing urothelial-type adenocarcinoma of prostate is an extremely rare tumor. It has a poor prognosis and it is not sensitive to endocrine therapy.

目的 分析肺肝样腺癌(HAL)的临床病理特征、诊断、免疫表型、基因检测及治疗预后等。方法 对1例HAL临床及影像学、组织学形态、免疫组化及基因检测结果等进行观察,并结合相关文献综合分析。结果 患者为48岁吸烟男性,镜下肿瘤具有肝细胞样和腺样分化特征,血清AFP升高。免疫组化: Hepatocyte,AFP, Arginase-1均阳性,ARMS-PCR法均未检测到EGFR,ALK/ROS1,KRAS及BRAF突变。结合相关文献分析: HAL常见于有吸烟史的男性,血清AFP值升高也是该肿瘤的一个特点。肿物多见于肺上叶,体积较大,易发生淋巴结和远处转移,预后相对较差。结论 HAL非常少见,易误诊,其诊断需结合形态学特点、临床病理特征及免疫组化结果等。

Objective To explore the clinicopathologic characteristics, diagnosis, immunophenotype, gene detection and prognosis of primary hepatoid adenocarcinoma of the lung (HAL). Methods A case of hepatoid adenocarcinoma of the lung was analyzed with clinical manifestations, histology, immunohistochemical staining and gene detection, and relevant literatures were reviewed. Results The patient was a 48-years-old man with smoking history. Microscopically, the tumor has the characteristics of hepatocellular carcinoma and adenoid differentiation, also serum AFP was elevated. The immunohistochemical results showed that Hepatocyte, AFP and Arginase-1were positive. No mutations were detected for EGFR, ALK/ROS1, KRAS and BRAF by ARMS-PCR. Combining with literature analysis, HAL is common in males and most patients with this tumor are smokers. Serum AFP in very high levels has been a distinguishing feature of this tumor. HAL usually presents as a large bulky solitary mass in the upper lobe. Lymph nodes and distant metastases are prone to occur. Therefore, the prognosis is very poor. Conclusion HAL is a rare malignant tumor and easy to be misdiagnosed. The diagnosis of primary hepatoid adenocarcinoma of the lung should be combined with morphological features, clinicopathological features and immunohistochemical findings.

目的 分析KRAS基因突变在左半结肠、右半结肠癌分布几率的差异性。方法 回顾性分析我院病理科分子实验室2015年到2017年间接收的537例结肠癌手术标本,用ARMS-PCR法进行了KRAS基因的检测,并对左右半结肠腺癌KRAS基因突变比例进行χ²检验,P＜0.05为差异有统计学意义。结果 537例中有187(34.82%)例为右半结肠腺癌,左半结肠腺癌有350(65.18%)例。KRAS基因突变检测阳性例数为240例,阳性率为44.69%;右半结肠腺癌KRAS基因突变阳性数为114例,阳性率为60.96%;左半结肠癌KRAS基因突变阳性数为126例,阳性率为36.00%。双侧结肠腺癌KRAS基因突变差异有统计学意义。结论 右半结肠癌的患病例数低于左半结肠癌,右半结肠癌KRAS基因突变阳性率较左半结肠癌高,本研究为区分左右半结肠癌的分子靶向治疗提供了数据支持。

Objective To analyze the differences distribution of KRAS gene mutations in the left hemi colon cancer and right hemi colon cancer. Methods Retrospectively to analyse the 537 colorectal cancer patients who were received by our molecular laboratory of pathology from 2015 to 2017, ARMS-PCR method was used to detect the KRAS gene. The ratio of KRAS gene mutation in left and right hemi colon cancer was tested by χ² test, P<0.05 was considered statistically significant. Results There were 187(34.82%) patients with right hemi colon cancer; and there were 350 (65.18%)patients with left half hemi colon cancer. The KRAS positive patients were 240 cases, which were 44.69% in total. The positive patients with right hemi colon cancer were 114 cases which had a higher rate of 60.96%, the left hemi colon cancer patients were 126 cases with a lower rate of 36.00%. Conclusion The number of right hemi colon cancer was lower than the number of left hemi colon cancer. And the KRAS gene mutation in right hemi colon cancer had more positive than left hemi colon cancer. This study provides a number aid for treating right and left colon cancer in the subsequent molecular targeted drug therapy.

目的 探讨结肠原发性腺鳞癌和鳞癌的临床病理特征、诊断、发病机制及预后。方法 分析2例结肠原发性腺鳞癌和鳞癌的临床特点、组织学、免疫组化及基因检测特点,并结合相关文献进行讨论。结果 2例患者均因右下腹隐痛入院,平均年龄69岁,肿瘤均位于右半结肠。腺鳞癌可见腺鳞两种成分随机混合在一起,鳞癌成分免疫组化结果示CK5/6,P63均阳性,基因检测结果(ARMS-PCR法):腺鳞癌KRAS 2号外显子突变,BRAF未突变,鳞癌KRAS和BRAF均未突变。腺鳞癌患者术后放弃治疗1个月后死亡,鳞癌患者5个月后死于肝转移。结论 结肠原发性腺鳞癌/鳞癌非常少见,其临床症状与腺癌相似,但临床过程却更具侵袭性,预后相对较差,所以在临床中需得到重视。

Objective To study the clinicopathologic features, diagnosis, pathogenesis and prognosis of primary colon adenosquamous carcinoma and squamous cell carcinoma. Methods We analyzed clinical features, histological morphology, immunohistochemical results and gene mutation. Besides, relevant literatures were also reviewed. Results The two patients with an average of 69 years were admitted to the hospital due to abnormal pain. The tumors were all located in the right hemicolon. Adenosquamous carcinoma contained both components that mixed haphazardly. Immunohistochemistry showed that both CK5/6 and P63 were positive in squamous cell carcinoma. KRAS exon 2 mutations and BRAF wide-type were found in adenosquamous carcinoma patient, while KRAS and BRAF wide-type were found in squamous cell carcinoma patient using ARMS-PCR method. Adenosquamous carcinoma patient abandoned treatment and died after one month and the other died of liver metastasis in five months after surgery. Conclusion Primary colon adenosquamous carcinoma and squamous cell carcinoma are very rare. They have the similar clinical symptoms, more invasive clinical processes and worse prognosis compared with adenocarcinoma, therefore should be valued in clinic.