OBJECTIVE:Retroperitoneal fibrosis (RPF) complicated by mediastinal fibrosis (MF) is rare but fatal. We aimed to explore the features and indicators of poor prognosis for the population of those affected. METHODS:Patients with idiopathic RPF were recruited in Peking University International Hospital. Literature related to RPF with MF was searched from PubMed, Web of Science, and Embase until September 2024. Systematic review and case-control studies were conducted. RESULTS:One patient with RPF and MF from our center and nine cases identified through literature search formed the study group. The remaining 51 patients with RPF who did not have MF were enrolled as the control group. Patients with RPF and MF were more likely to present specific symptoms, including emaciation (30% vs 2%), fever (20% vs 0), pericardial effusion (30% vs 0), pleurisy (20% vs 0) and dyspnea (40% vs 0) (all P < 0.05). Hyperglobulinemia (elevated IgG levels) was also more predominant in the study group, whereas low back pain (20% vs 56.9%, P = 0.043) was less prevalent. Four of 10 patients in the study group died, but none died in the control group. A second case-control study was performed among patients with RPF and MF, with the three patients who died of fibrosis disease as the study group and the surviving six patients as the control group. It was found that pleural effusions (100% versus 16.7%, P = 0.048) and the absence of glucocorticoid treatment were risk factors for death in patients with RPF and MF. CONCLUSION:RPF with MF has specific clinical features and poor prognosis. Early detection and glucocorticoids-based treatment could improve the outcome.

患者,男性,31岁,查体发现"甲状腺占位".甲状腺彩超示左侧叶体积增大,实质内见多个囊实性结节及实性低回声结节,界清(图1). 病理检查 细胞学镜检:丰富的淋巴细胞,细胞成分杂,以小淋巴细胞为主,见中心细胞、中心母细胞等,未见甲状腺滤泡细胞、胶质及上皮细胞(图2).

Background This study evaluates the diagnostic accuracy of ultrasound-guided fine needle aspiration (US-FNA) and core needle biopsy (US-CNB) for detecting axillary lymph nodes in women with breast cancer. Methods Eligible studies and pertinent literature resources were identified in Cochrane, PubMed, Embase, CNKI, VIP, and Wanfang databases using subject-specific keywords. Study outcomes were tested for heterogeneity, and meta-analyses were performed to estimate sensitivity, specificity, and diagnostic odds ratios (DORs). The summary receiver operating characteristic (SROC) curve analysis was also performed. Results A total of 22 studies involving 3,548 patients were included to evaluate the diagnostic accuracy of US-FNA and 11 studies involving 758 patients were included to evaluate the diagnostic accuracy of US-CNB in identifying axillary lymph nodes in women with breast cancer. The accuracy of US-FNA in identifying suspicious axillary lymph nodes was as follows: overall sensitivity, 79% (95% CI: 73%–84%); global specificity, 96% (95% CI: 92%–98%); overall positive likelihood ratio, 18.55 (95% CI: 10.53–32.69); overall negative likelihood ratio, 0.22 (95% CI: 0.17–0.28); DOR, 71.68 (95% CI: 37.19–138.12); and the area under the SROC curve, 0.94 (95% CI: 0.92–0.96). The accuracy of US-CNB in identifying suspicious axillary lymph nodes was as follows: overall sensitivity, 85% (95% CI: 81%–89%); global specificity, 93% (95% CI: 87%–96%); overall positive likelihood ratio, 11.88 (95% CI: 6.56–21.50); overall negative likelihood ratio, 0.16 (95% CI: 0.12–0.21); overall DOR, 66.83 (95% CI: 33.28–134.21), and the area under SROC curve 0.96 (95% CI: 0.94–0.97). Conclusions The results indicate that both US-FNA and US-CNB have high accuracy for suspicious axillary lymph nodes.

目的:探讨腹膜后滑膜肉瘤的临床病理特征、免疫表型及分子遗传学改变.方法:收集北京大学国际医院病理科2014年12月至2021年07月期间手术切除腹膜后标本病理诊断为滑膜肉瘤6例,进行光镜观察、免疫表型及分子病理学分析,并复习相关文献.结果:男性2例,女性4例,年龄12～56岁(平均年龄29岁,中位年龄26岁);肿瘤大小8～20 cm,平均15.2 cm,界限较清,梭形细胞型5例,双相型1例;CKpan、EMA、Bcl-2、CD99、TLE1、Vimentin、CD34、Calponin阳性比例分别为5/6、6/6、6/6、3/6、6/6、5/6、0/6、4/6,INI1呈特殊模式表达6/6,Ki-67高表达;FISH结果显示6例均有SS18-SSX基因融合;3例为SS18-SSX2基因融合,并有伴随基因突变.结论:腹膜后滑膜肉瘤是少见的发病部位,明确诊断常需结合组织病理学形态、免疫组织化学表达及分子遗传学特征进行综合分析.

目的 探讨伴有惰性T淋巴母细胞增生(iT-LBP)性疾病的临床病理学特征、诊断及鉴别诊断.方法 分析2例伴有iT-LBP的Castleman病的临床特征、免疫表型及基因重排检测结果,并复习总结伴有iT-LBP的相关文献.结果 镜下观察可见典型的Castleman病改变,滤泡间区可见多量淋巴母细胞样细胞,免疫表型表达TdT及T细胞标记物,细胞增生活跃;T细胞受体重排检测未发现克隆性重排,诊断为伴有惰性T淋巴母细胞增生的Castleman病.总结文献报道及本组iT-LBP共25例,发现iT-LBP最常见于Castleman病和/或滤泡树突状细胞肉瘤,均伴有TdT及T细胞标记物阳性表达,且无T细胞克隆性重排.随访发现无一例死于iT-LBP.结论 iT-LBP常伴发于其他疾病,临床呈惰性过程,诊断时应进行仔细的形态学观察、免疫表型的检查、TCR检查以及密切的临床随访,然后作出准确的诊断,避免误诊引起不必要的治疗.

目的 探讨硬化性上皮样纤维肉瘤(sclerosing epithelioid fibrosarcoma,SEF)及其密切相关的低度恶性纤维黏液样肉瘤(low-grade fibromyxoid sarcoma,LGFMS)的临床病理学特征、长期预后、免疫表型和分子表型.方法 回顾性分析11例SEF和5例LGFMS的临床病理资料,行HE及免疫组化染色,分别行FISH检测或高通量测序检测其基因改变,并复习相关文献.结果 11例SEF肿瘤细胞呈圆形、卵圆形,细胞形态较为一致,间质可见大量深嗜伊红色的胶原纤维,部分区域细胞稀疏,肿瘤细胞呈条索状或单个夹杂在硬化性间质中,其中1例为杂合性SEF/LGFMS;5例LGFMS镜下由稀疏区和致密区构成,稀疏区间质呈黏液样,可见大量弓形血管,致密区细胞外胶原丰富,两种区域掺杂或交替分布;免疫表型:SEF和LGFMS中MUC4和vimentin均阳性;分子检测显示单纯SEF中90％(9/10)发生EWSR1 基因断裂分离,10％(1/10)发生FUS基因断裂分离;1例杂合性SEF/LGFMS伴有FUS-CREB3L2基因融合;LGFMS中均检测到FUS基因断裂分离(5/5).结论 SEF和LGFMS均为低-中度恶性的纤维肉瘤,具有局部侵袭性;MUC4是其特异性标志物;SEF和LGFMS相关的基因突变常为EWSR1或FUS基因,但在发生频率上差异较大;LGFMS、杂合性SEF/LGFMS和SEF可能为一个疾病谱系,而SEF位于谱系的恶性一端,更具侵袭性.

Background Complete resection (CR) serves as the standard of surgical treatment for retroperitoneal liposarcoma (RPLS). Unfortunately, even at referral centers, recurrence rates are high, and CR may not address multifocal diseases, which are a common phenomenon in RPLS. We sought to retrospectively compare the clinical outcomes of RPLS patients treated with total (ipsilateral) retroperitoneal lipectomy (TRL) and CR. Because TRL remove potentially multifocal tumors in the fat, patients may have a better prognosis than CR. Methods Patients with primary/first-recurrent RPLS who had been treated at 5 referral centers were recruited from December 2014 to June 2018. Multivariable Cox regression analyses were conducted to determine the effects of demographic, operative, and clinicopathological variables on the following primary endpoints: local recurrence (LR), local recurrence-free survival (LRFS), and overall survival (OS). Results A total of 134 patients were enrolled in this retrospective study, 53 of whom underwent TRL, and 81 of whom underwent CR. The 2 groups were comparable in terms of age, gender, presentation (primary vs. first-recurrent RPLS), number of tumors (unifocal vs. multifocal) at presentation, and Fédération Nationale des Centres de Lutte Contre le Cancer (FNCLCC) grade. The TRL group had higher levels of preoperative hemoglobin (Hb) (13 vs. 12.5 g/dL; P=0.008) and a lower amount of intraoperative blood loss (400 vs. 500 mL; P=0.034), but there were no significant differences in the length of hospital stay (23 vs. 22 d; P=0.47) or complications (32 vs. 30; P=0.82) between the 2 groups. In a subset of patients with multifocal tumors at initial presentation, OS was more prolonged in those treated with TRL than those treated with CR (P=0.0272). Based on the multivariable analysis, primary liposarcoma and a low FNCLCC grade were associated with decreased LR and improved OS. Conclusions TRL is a safe procedure that positively affects the OS of patients with multifocal RPLS. This novel strategy deserves further investigation in prospective studies.

Background: Cellular schwannoma (CS) is a relatively rare neural tumor with few reports. This study aimed to compare the clinicopathological characteristics of CS in the retroperitoneum and other sites by analyzing the hematoxylin-eosin (HE) staining and immunohistochemical (IHC) staining results, to provide some help for pathological diagnosis. Methods: A total of 79 CS cases from the Department of Pathology, Peking University International Hospital were collected, and the diagnosis was based on the 5th WHO classification of soft tissue tumors. The staining results of HE and IHC were judged and analyzed according to the instructions. The t-tests, Chi-square test and Fisher's exact probability test were used for statistical analysis. Results: Compared with other sites, the volume of retroperitoneal CS tumors were larger (t=4.265, P=0.001) and more likely to recur (chi(2)=4.223, P=0.04). Nerve sheath structures were rare around the tumors (chi(2)=60.096, P=0.000). Immunohistochemically, there was a difference in the expression of glial fibrillary acidic protein (GFAP), Cytokeratin (CK), and myelin basic protein (MBP) between the two groups (chi(2)=54.290, P=0.000; chi(2)=4.879, P=0.027; chi(2)=31.792, P=0.000). But there was no difference in expression between the two groups in the other indexes. Conclusions: It founded that Retroperitoneal CS was often positive for GFAP and CK, suggesting it originated from unmyelinated Schwann cells. CS in other sites, the expression of GFAP and CK was often negative, indicating they derived from myelinated Schwann cells. The expression of MBP in the peripheral nerve sheath structure of CS can be used to determine whether the tumor originates from myelinated or unmyelinated Schwann cells. These findings may provide a reference for revealing pathogenesis, diagnosis and evaluating prognosis of CS.

目的 探讨胃底腺肿瘤的临床病理学特征及分子遗传学特征、鉴别诊断、治疗及预后.方法 收集12例胃底腺肿瘤患者的临床病理资料,采用免疫组化EnVision法染色,应用二代测序法及原位杂交法对分子特征进行分析.结果 内镜下胃底或胃体中上部见孤立性病灶,直径0.3～1.2 cm,隆起或浅表凹陷状,伴血管扩张.镜下肿瘤以主细胞为主,呈轻～中度异型,1例局限黏膜内,10例侵至黏膜下,未见坏死、脉管侵犯,周围黏膜轻～中度慢性炎症,未见肠上皮化生、萎缩,未见活动性炎.免疫表型:PepsinogenⅠ、MUC6均弥漫阳性,β-catenin膜阳性,Ki-67增殖指数为1％～20％.EBV未检出.7例GNAS和2例KRAS错义突变.随访10～42个月,患者均无复发、转移.结论 胃底腺肿瘤以主细胞为主型多见,根据形态学特征、辅以免疫组化检测可确诊.胃底腺型腺癌GNAS突变较常见,异型轻微、无脉管侵犯患者预后良好.

Objective To compare the clinicopathological features, differential diagnosis and treatment between perirenal angiomyolipoma (AML) and liposarcoma. Methods Eleven cases of AML and thirty-nine cases of liposarcoma around the kidney from Peking University International Hospital from January 2015 to December 2020 were reviewed in terms of clinical manifestations, imaging examination, pathological morphology, immunophenotype and treatment follow-up. Results The tumors in both groups were close to the kidney, and the imaging signs of renal defect were not distinctive enough to diagnose correctly. Perirenal AML was mainly female (P=0.003), the age of onset was younger than liposarcoma (P 0.05). Conclusions Retroperitoneal perirenal AML and liposarcoma have similar imaging features, and gender, age, tumor number, primary/recurrent character, histomorphology, protein expression and gene amplification detection are helpful to differentiate them. Liposarcoma needs more extensive resection, active treatment and follow-up. DOI: 10.11855/j.issn.0577-7402.2021.08.08

目的:探讨生物潜能未定非典型神经纤维瘤(atypical neurofibromatous neoplasm of uncertain biologic potential,ANNUBP)的临床病理特征、免疫表型、分子遗传学改变.方法:分析2014年12月至2020年8月收集于北京大学国际医院病理诊断为非典型或富于细胞性神经纤维瘤14例患者的临床资料,其中7例具有ANNUBP的特征,光镜观察肿瘤细胞形态、免疫表型特征、并进行总结.结果:ANNUBP中男性5例,女性2例,年龄14～44岁(平均年龄27岁,中位年龄27岁);6例位于腹膜后,1例位于头颈部,最大径4.5～21.5 cm,平均11.0 cm,界限较清.镜下肿瘤细胞可见细胞异型性、丰富密集、失去神经纤维瘤结构和/或核分裂像增加(>1/50 HPF和<3/10 HPF);其中4例多次复发,2例进展为恶性外周神经鞘膜瘤(malignant peripheral nerve sheath tumor,MPNST),1例无瘤生存,复发率85.71％(6/7),恶变率28.57％(2/7);S-100、SOX-10、H3K27Me3在7例中均弥漫强表达,1例CD34染色显示网状结构消失,Ki-67增殖指数<2％～5％.结论:ANNUBP是具有较高复发率和恶变率的肿瘤,术后应结予相应的辅助治疗,并密切随访,同时也应避免过度治疗;结合免疫组织化学,有助于其诊断和鉴别诊断.

目的 分析甲状腺嗜酸细胞肿瘤的超声图像特征,提高对良、恶性甲状腺嗜酸细胞肿瘤的诊断和鉴别诊断水平.方法 回顾性分析13例甲状腺嗜酸细胞肿瘤患者的超声图像及临床病理特征.结果 13例甲状腺嗜酸细胞肿瘤(HCN)患者共计14个结节,其中嗜酸细胞腺瘤(HCA)10例,嗜酸细胞癌(HCC)3例.HCN结节大多具有良性病变的超声特征.HCC与甲状腺乳头状癌(PTC)比较,结节的大小、形态、边界、纵横比、周边晕征、有无钙化、血流等差异均具有统计学意义(P＜0.05);而成分、回声、被膜连续性差异无统计学意义(P＞0.05).结论 甲状腺Hürthle细胞肿瘤具有特定的超声特征,FNA可诊断嗜酸细胞肿瘤,但不能判断有无血管和包膜侵犯;手术后组织学病理是明确良、恶性病变的金标准.

目的 分析纵横比对于不同部位甲状腺乳头状癌结节的超声诊断价值.方法 回顾性分析病理确诊为甲状腺乳头状癌结节的超声图像,根据结节所在部位分为A组(峡部)、B组(双叶两极)和C组(双叶中部).统计结节各径线,计算纵切面及横切面的纵横比值.结果 共纳入274个结节,无论纵切面还是横切面,峡部结节的纵横比值均最小;在A、B、C三组,横切面纵横比＞1的诊断灵敏度均较纵切面高,差异有统计学意义.把横切面和/或纵切面纵横比＞1定义为"综合"纵横比＞1,其诊断灵敏度为57.7％;诊断A组结节的灵敏度最低,差异有统计学意义;诊断C组结节的灵敏度数值高于B组,但差异无统计学意义."综合"纵横比＞1诊断最大径d＜10 mm、10 mm≤d＜20 mm、d≥20 mm结节的灵敏度依次递减,差异有统计学意义.结论 "综合"纵横比＞1诊断甲状腺双叶中部乳头状癌结节的灵敏度最高,双叶两极次之,峡部最低,且与结节大小相关.

患者女性,28岁,孕2产0.患者孕17周无明显诱因出现持续性阴道排液(清亮)1个月.阴道超声检查见子宫颈后唇约3. 5cm直径的息肉状突起,有严重糜烂和接触性出血.子宫颈涂片检测为HPV 16型和其他高危型阳性,HPV 18型阴性.血清肿瘤标志物检查仅CA125和AFP分别比正常最高值高2和7倍.临床诊断为Ⅰb1期子宫颈癌(FIGO分期).引产后行3个疗程静脉化疗(泰素240mg+伯尔定600mg).然而肿瘤体积未减小,遂行子宫、双侧附件及盆腔和主动脉旁淋巴结清扫术.术后1个月CT复查发现新的淋巴结转移灶,暂行化疗.

目的 探讨鞍区混合性神经节细胞瘤-垂体腺瘤的临床病理特征及发病机制.方法 分析3例混合性神经节细胞瘤-垂体腺瘤的临床资料、病理组织学特点、免疫表型及预后,并进行该疾病相关文献复习.结果 1例为男性,2例为女性,年龄38～ 59岁.1例伴有生长激素水平增高及肢端肥大症,1例伴催乳素水平升高但无其他临床症状,另1例无激素水平改变但伴有反复低钠.3例病理组织学均为混合性生长激素细胞腺瘤与神经节细胞瘤,其中2例为稀疏颗粒型.垂体腺瘤垂体特异性转录因子(Pit-1)及GH(+),神经节细胞区域除了NeuN和Calretinin(CR)(+),部分还Pit-1及CK18(+).3例随访7～90个月,均无复发.结论 神经节细胞瘤常与稀疏颗粒型生长激素腺瘤并发,常有肢端肥大症或无临床症状.更多实验结果支持生长激素腺瘤发生神经元转化是其发生机制,有待进一步研究;其预后与单纯垂体腺瘤相同.

Objective To explore the application value of ultrasound-guided manual biopsy of superficial lymph node.Methods 105 cases underwent ultrasound-guided manual biopsy of superficial lymph nodes were analyzed retrospectively.Results All patients puncture success rate of 100％.Each patient was punctured 1 or 2 needles, the average length of each needle drawn (3.09±1.88) cm.Puncture pathology results showed that 61.90％ (65/105) of malignant lymph nodes, 36.20％ (38/105) of benign lymph nodes and 2 case failed to confirm the pathological diagnosis.Puncture pathologic findings were better consistent with postoperative pathology or clinical diagnosis (Kappa=0.979, P＜0.001).Conclusions Ultrasound-guided manual biopsy is simple, minimally invasive, accurate and safe, can be used as preferred qualitative diagnostic method for superficial lymph node disease and has important application value for early diagnosis and treatment of disease.

Hematoxylin and Eosin H&E stained breast tissue samples from biopsies are observed under microscopy for the gold standard diagnosis of breast cancer. However, a substantial workload increases and the complexity of the pathological images make this task time-consuming and may suffer from pathologist's subjectivity. Facing this problem, the development of automatic and precise diagnosis methods is challenging but also essential for the field. In this paper, we propose a new hybrid convolutional and recurrent deep neural network for breast cancer pathological image classification. Our method considers the short-term as well as the long-term spatial correlations between patches through RNN which is directly incorporated on top of a CNN feature extractor. Experimental results showed that our method obtained an average accuracy of 90.5% for 4-class classification task, which outperforms the state-of-the-art method. At the same time, we release a bigger dataset with 1568 breast cancer pathological images to the scientific community, which are now publicly available from http://ear.ict.ac.cn/?page id=1576. In particular, our dataset covers as many different subclasses spanning different age groups as possible, thus alleviating the problem of relatively low classification accuracy of benign.

浆膜腔积液细胞块免疫组织化学检测是近十余年来兴起并逐步得到推广应用的一种辅助性诊断技术,但由于免疫组织化学抗体种类繁多,在诊断中如何选择适宜的一线抗体成为难题. 我们选取5种常用的间皮抗体和5种上皮性抗体,对72例浆膜腔积液细胞蜡块进行回顾性研究,探讨抗体组合在浆膜腔积液细胞蜡块中的应用价值.

Purpose To investigate the clinicopathology and the expression of H3K27me3 in retroperitoeal malignant pe-ripheral nerve sheath tumors (MPNST). Methods The clini-copathology and prognosis of 13 cases MPNST were analyzed. Immunohistochemical analysis was used to detect H3K27me3 in MPNST, synovial sarcoma, dedifferentiated liposarcoma and leiomyosarcoma. Results 13 cases of MPNST were high-grade. The mean diameter of tumors was 20 cm. 2-year survival rate of MPNST was about 60% . 5-year survival rate of MPNST was a-bout 30% . Compared to NF-1 associated and sporadic MPNST (P＜0. 05), the RT-induced MPNST had a poor prognosis. Re-currence and distant metastasis patient had a poor prognosis( P＜0. 05). Age had no significant effect on patient survival. In addition, immunohistochemical staining showed that the expres-sion of H3k27me3 was absent in 11 of 13 cases of MPNST. And compared with the expression of H3K27me3 in synovial sarco-ma, dedifferentiated liposarcoma and leiomyosarcoma, it had statistical significance of that expression in MPNST (P＜0. 05). Conclusion Retroperitoeal MPNST is common at high-grade. Tumor volume is relatively large and prognosis is poor. RT-in-duced, recurrence and distant metastasis play an important role in survival rate of MPNST. H3K27me3 which is more common absence in high-grade could be an effective marker of MPNST.

软骨母细胞瘤是一种较少见的良性软骨性肿瘤，术前多误诊为其他病变，且由于软骨母细胞瘤术中刮除组织中多含有骨性成分，行快速冷冻检查时难以制片，导致术中快速病理检查应用价值有限。我们总结4例软骨母细胞瘤术中印片细胞学特点，并与其他含有巨细胞骨关节肿瘤性病变的细胞学特点作比较，以提高对此病变的认识。