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Non Hodgkin Lymphoma Among Children: Pathological Aspects and Diagnostic Challenges 儿童非霍奇金淋巴瘤:病理方面和诊断挑战
IF 1.3 Q3 PATHOLOGY Pub Date : 2022-01-01 DOI: 10.1177/2632010X221090156
S. Derqaoui, I. Boujida, Oussama Marbouh, L. Rouas, L. Hessissen, N. Lamalmi
Non-Hodgkin lymphoma (NHL) are common malignancies in children. Available data on clinico-pathological aspects of pediatric NHL in developping countries are limited and diagnostic approach appears more delicate with absence of molecular studies. The objectives of our study are: analyzing the pathological spectrum of NHL among children and highlighting challenges in the diagnosis including: limited biopsic material; unususal subtyptes, age group, or localization. We retrospectively analyzed clinico pathological characteristics of 101 NHL’s cases among children diagnosed in the Pediatric’s pathology unit over a period of 4 years There were 78 (77.2%) male and 23 (22.8%) female. The median age was 7.2 years. The most common histologic subtypes of NHL were Burkitt lymphoma in 65 patients (64.4% ); followed by lymphoblastic lymphoma in 22 patients, large B-cell lymphoma in 9 patients ( 8.9%); anaplastic T cell lymphoma in 3 patients; NOS mature T cell lymphoma and pediatric type follicular lympoma in 1 patient each. In conclusion, this study Morocco illustrates the pattern of distribution of NHL and emphasizes challenges in the diagnosis of these neoplasms.
非霍奇金淋巴瘤是儿童常见的恶性肿瘤。在发展中国家,关于儿童NHL的临床病理方面的现有数据有限,由于缺乏分子研究,诊断方法显得更加微妙。我们研究的目的是:分析儿童NHL的病理谱,并强调诊断中的挑战,包括:有限的生物材料;不常见的亚密码、年龄组或本地化。我们回顾性分析了在4年的时间里,在儿科病理科诊断的101例儿童NHL的临床病理特征 男78例(77.2%),女23例(22.8%)。中位年龄为7.2岁 年。NHL最常见的组织学亚型为伯基特淋巴瘤65例(64.4%);其次是淋巴母细胞淋巴瘤22例、大B细胞淋巴瘤9例(8.9%);间变性T细胞淋巴瘤3例;NOS成熟T细胞淋巴瘤和儿童型滤泡性淋巴瘤各1例。总之,摩洛哥的这项研究阐明了NHL的分布模式,并强调了诊断这些肿瘤的挑战。
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引用次数: 2
Hyperargininemia Due to Arginase 1 Deficiency: Variability in Clinical and Biochemical Presentations in Malaysian children 精氨酸酶1缺乏引起的高精氨酸血症:马来西亚儿童临床和生化表现的变异性
IF 1.3 Q3 PATHOLOGY Pub Date : 2022-01-01 DOI: 10.1177/2632010x221093274
A. Habib, Norashareena Mohamed Shakrin
Objective: Hyperargininemia due to Arginase 1 deficiency is a rare inborn error of the urea cycle with an incidence estimated at 1:950 000. It has typical severe and progressive abnormal neurological features with biochemical findings of hyperargininemia and hyperexcretion of orotic acid. The aim of our study is to review the clinical and biochemical presentations of 4 children diagnosed with Arginase 1 deficiency in Malaysia and compare with the literature review. Design and Methods: We retrospectively reviewed the medical records of 4 patients with molecularly confirmed Arginase 1 deficiency. Patients were identified from a selective high-risk screening of 51 682 symptomatic patients from January 2006 to December 2020. Results: Our patients exhibited heterogeneous clinical presentations with acute and progressive neurological abnormalities and varying degrees of plasma arginine and urine orotic acid excretions. Interestingly, an unusual hyperexcretion of homocitrulline was found in 3 patients. Conclusions: Hyperargininemia due to Arginase 1 deficiency can present acutely and hyperexcretion of homocitrulline can be an additional biochemical feature of Arginase 1 deficiency.
目的:精氨酸酶1缺乏引起的高精氨酸血症是一种罕见的先天性尿素循环错误,发生率约为1:95 000。它具有典型的严重和进行性神经系统异常特征,生化表现为高精氨酸血症和山茱萸酸高排泄。我们研究的目的是回顾马来西亚诊断为精氨酸酶1缺乏症的4名儿童的临床和生化表现,并与文献综述进行比较。设计与方法:我们回顾性分析了4例分子证实精氨酸酶1缺乏症患者的病历。从2006年1月至2020年12月对51 682例有症状的患者进行选择性高风险筛查。结果:我们的患者表现出不同的临床表现,急性和进行性神经异常,不同程度的血浆精氨酸和尿乳酸酸排泄。有趣的是,在3例患者中发现了异常的高瓜氨酸排泄。结论:精氨酸酶1缺乏症引起的高精氨酸血症可急性出现,高精氨酸排泄可能是精氨酸酶1缺乏症的另一个生化特征。
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引用次数: 0
Is the Presence of Helicobacter Pylori in the Colonic Mucosa, Provocative of Activity in Ulcerative Colitis? 结肠粘膜中幽门螺杆菌的存在是否会引发溃疡性结肠炎的活动?
IF 1.3 Q3 PATHOLOGY Pub Date : 2022-01-01 DOI: 10.1177/2632010X221096660
Javad Ranjbar, B. Geramizadeh, K. Bagheri Lankarani, Z. Jowkar, M. Mirzai, E. Moazamian
Background: Epidemiologic studies have shown world-wide increasing incidence of ulcerative colitis (UC) as an autoimmune disease of intestine. In the meantime, gastrointestinal H. Pylori infection is being decreased. Objectives: There are very few studies about comparing the presence of H. Pylori in the colon and the disease activity of UC. There is no study form Iran. In this study, we tried to investigate the presence of H. Pylori in the mucosa of colon by molecular and microbiological as well as pathological methods to find any association between the presence of this organism in the colon and the presence and activity of UC. Patients and Methods: In 100 patients who referred to colonoscopy clinic, colonoscopy was performed. Fifty-seven patients with the new diagnosis of UC were considered as cases and 43 patients with normal screening colonoscopy for polyps were considered as controls. Colon biopsies were evaluated according to histopathology, clinical findings, and laboratory results to confirm the diagnosis and the degree of activity in the cases of UC. Molecular studies were also performed to evaluate the presence of H. Pylori genome in the colon biopsies. A sample of colon was also cultured for H. Pylori. ELISA test was performed in a sample of blood to evaluate the level of IL-10 and IL-17 as regulatory cytokines of inflammation. Results: Cases with the diagnosis of UC showed significantly higher number of positive colonic H. Pylori comparing to normal colonic mucosa. Also, the presence of H. Pylori genome in the colon was associated with higher activity in the cases with UC and higher levels of inflammatory mediators especially IL17 and lower levels of inhibitory mediators such as IL-10. Conclusion: Colonic colonization of H. Pylori was higher in the patients with UC and higher activity of this disease comparing with normal control colonic mucosa.
背景:流行病学研究表明,溃疡性结肠炎(UC)是一种肠道自身免疫性疾病,其发病率在全球范围内呈上升趋势。与此同时,胃肠道幽门螺杆菌感染正在减少。目的:很少有研究比较幽门螺杆菌在结肠中的存在与UC的疾病活性。没有来自伊朗的研究。在这项研究中,我们试图通过分子、微生物学和病理学方法研究幽门螺杆菌在结肠粘膜中的存在,以发现结肠中这种生物的存在与UC的存在和活性之间的任何联系。患者和方法:在100名转诊到结肠镜检查诊所的患者中,进行结肠镜检查。57名新诊断为UC的患者被视为病例,43名息肉筛查正常的患者被认为是对照。根据组织病理学、临床表现和实验室结果评估结肠活检,以确认UC病例的诊断和活动程度。还进行了分子研究,以评估结肠活检中幽门螺杆菌基因组的存在。结肠样品也被培养用于H.Pylori。在血液样本中进行ELISA测试,以评估作为炎症调节细胞因子的IL-10和IL-17的水平。结果:诊断为UC的患者结肠幽门螺杆菌阳性率明显高于正常结肠粘膜。此外,结肠中幽门螺杆菌基因组的存在与UC患者的较高活性和较高水平的炎症介质(尤其是IL17)以及较低水平的抑制介质(如IL-10)有关。结论:与正常对照组相比,UC患者的幽门螺杆菌结肠定植率较高,该病的活性较高。
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引用次数: 0
Retraction Notice: Heterogeneous Longitudinal Antibody Responses to Covid-19 mRNA Vaccination 撤回通知:新冠肺炎mRNA疫苗接种的异质性纵向抗体反应
IF 1.3 Q3 PATHOLOGY Pub Date : 2022-01-01 DOI: 10.1177/2632010X221098313
S. Monte, Christine E. Long, Nicole Szczepanski, Christopher Griffin, A. Fitzgerald, K. Chapin
BACKGROUND: Public health measures to stem the coronavirus disease 2019 (COVID-19) pandemic are challenged by social, economic, health status, and cultural disparities that facilitate disease transmission and amplify its severity. Prior pre-clinical biomedical technologic advances in nucleic acid-based vaccination enabled unprecedented speed of conceptualization, development, production, and widespread distribution of mRNA vaccines that target SARS-CoV-2’s Spike (S) protein. DESIGN: Twenty-five female and male volunteer fulltime employees at the Providence VA Medical Center participated in this study to examine longitudinal antibody responses to the Moderna mRNA-1273 vaccine. IgM-S and IgG-S were measured in serum using the Abbott IgMS-Qualitative and IgG2-S-Quantitative chemiluminescent assays. RESULTS: Peak IgM responses after Vaccine Dose #1 were delayed in 6 (24%) and absent in 7 (28%) participants. IgG2-S peak responses primarily occurred 40 to 44 days after Vaccine Dose #1, which was also 11 to 14 days after Vaccine Dose #2. However, subgroups exhibited Strong (n = 6; 24%), Normal (n = 13; 52%), or Weak (n = 6; 24%) peak level responses that differed significantly from each other (P < .005 or better). The post-peak IgG2-S levels declined progressively, and within 6 months reached the mean level measured 1 month after Vaccine Dose #1. Weak responders exhibited persistently low levels of IgG2-S. Variability in vaccine responsiveness was unrelated to age or
背景:遏制2019冠状病毒病(新冠肺炎)大流行的公共卫生措施受到社会、经济、健康状况和文化差异的挑战,这些差异促进了疾病传播并加剧了其严重性。先前基于核酸的疫苗接种的临床前生物医学技术进步使靶向严重急性呼吸系统综合征冠状病毒2型刺突蛋白的信使核糖核酸疫苗的概念化、开发、生产和广泛分发速度达到了前所未有的速度。设计:弗吉尼亚州普罗维登斯医疗中心的25名全职女性和男性志愿者参与了这项研究,以检测对莫德纳mRNA-1273疫苗的纵向抗体反应。使用Abbott IgMS定性和IgG2-S-定量化学发光测定法测定血清中的IgM-S和IgG-S。结果:接种第1剂疫苗后,6名(24%)参与者的IgM峰值反应延迟,7名(28%)参与者的反应缺失。IgG2-S峰值反应主要发生在第1剂疫苗接种后40至44天,也是第2剂疫苗接种之后11至14天。然而,亚组表现出强(n=6;24%)、正常(n=13;52%)或弱(n=6,24%)的峰值水平反应,这些反应彼此之间存在显著差异(P<.005或更好)。峰值后IgG2-S水平逐渐下降,并在6个月内达到疫苗剂量#1后1个月测得的平均水平。弱应答者表现出持续低水平的IgG2-S。疫苗反应性的变异性与年龄或
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引用次数: 0
Infrared Spectroscopic Analysis in the Differentiation of Epithelial Misplacement From Adenocarcinoma in Sigmoid Colonic Adenomatous Polyps 红外光谱分析鉴别乙状结肠腺瘤性息肉上皮错位与腺癌
IF 1.3 Q3 PATHOLOGY Pub Date : 2022-01-01 DOI: 10.1177/2632010X221088960
J. Nallala, R. Griggs, G. Lloyd, N. Stone, N. Shepherd
Purpose: The differential diagnosis of epithelial misplacement from invasive cancer in the colon is a challenging endeavour, augmented by the introduction of bowel cancer population screening. The main aim of the work is to test, as a proof-of concept study, the ability of the infrared spectroscopic imaging approach to differentiate epithelial misplacement from adenocarcinoma in sigmoid colonic adenomatous polyps. Methods: Ten samples from each of the four diagnostic groups, normal colonic mucosa, adenomatous polyps with low grade dysplasia, epithelial misplacement in adenomatous polyps and adenocarcinoma, were analysed using IR spectroscopic imaging and data processing methods. IR spectral images were subjected to data pre-processing and cluster analysis based segmentation to identify epithelial, connective tissue and stromal regions. Statistical analysis was carried out using principal component analysis and linear discriminant analysis based cross validation, to classify spectral features according to the pathology, and the diagnostic attributes were compared. Results: The combined 4-group classification model on an average showed a sensitivity of 64%, a specificity of 88% and an accuracy of 76% for prediction based on a ‘single spectrum’, whilst a ‘majority-vote’ prediction on an average showed a sensitivity of 73%, a specificity of 90% and an accuracy of 82%. The 2-group model, for the differential diagnosis of epithelial misplacement versus adenocarcinoma, showed an average sensitivity and specificity of 82.5% for prediction based on a ‘single spectrum’ whilst a ‘majority-vote’ classification showed an average sensitivity and specificity of 90%. A 92% area under the curve (AUC) value was obtained when evaluating the classifier using the Receiver Operating Characteristics (ROC) curves. Conclusions: IR spectroscopy shows promise in its ability to differentiate epithelial misplacement from adenocarcinoma in tissue sections, considered as one of the most challenging endeavours in population-wide diagnostic histopathology. Further studies with larger series, including cases with challenging diagnostic features are required to ascertain the capability of this modern digital pathology approach. In the long-term, IR spectroscopy based pathology which is relatively low-cost and rapid, could be a promising endeavour to consider for integration into the existing histopathology pathway, in particular for population based screening programmes where large number of samples are scrutinised.
目的:结肠侵袭性癌症上皮错位的鉴别诊断是一项具有挑战性的工作,由于引入了癌症人群筛查,这项工作得到了加强。这项工作的主要目的是测试红外光谱成像方法在乙状结肠腺瘤性息肉中区分上皮错位和腺癌的能力,作为概念验证研究。方法:使用红外光谱成像和数据处理方法分析来自四个诊断组(正常结肠粘膜、腺瘤性息肉伴低度发育不良、腺瘤性腺瘤性息肉和腺癌的上皮错位)的10个样本。对红外光谱图像进行数据预处理和基于聚类分析的分割,以识别上皮、结缔组织和基质区域。采用主成分分析和基于交叉验证的线性判别分析进行统计分析,根据病理对光谱特征进行分类,并对诊断属性进行比较。结果:对于基于“单一谱”的预测,组合的4组分类模型平均显示出64%的灵敏度、88%的特异性和76%的准确度,而“多数票”预测平均显示出73%的灵敏度、90%的特异性以及82%的准确度。用于鉴别诊断上皮错位与腺癌的2组模型显示,基于“单一谱”的预测平均灵敏度和特异性为82.5%,而“多数票”分类显示平均灵敏度和特异度为90%。当使用受试者操作特征(ROC)曲线评估分类器时,获得92%的曲线下面积(AUC)值。结论:红外光谱在组织切片中区分上皮错位和腺癌的能力方面显示出了希望,这被认为是全人群诊断组织病理学中最具挑战性的工作之一。需要进行更大系列的进一步研究,包括具有挑战性诊断特征的病例,以确定这种现代数字病理学方法的能力。从长远来看,基于红外光谱的病理学相对低成本且快速,可能是一项很有前途的尝试,可以考虑整合到现有的病理学途径中,特别是对于需要仔细检查大量样本的基于人群的筛查计划。
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引用次数: 0
Disseminated Cryptococcal Infection in HIV-Infected Patients: A Retrospective Clinicopathological Review of 4 Autopsy Cases hiv感染者的播散性隐球菌感染:4例尸检病例的回顾性临床病理回顾
IF 1.3 Q3 PATHOLOGY Pub Date : 2022-01-01 DOI: 10.1177/2632010X221078234
M. C. Khaba, Mamokoma Becky Kgole, Lesedi M Nevondo, W. van Aswegen, T. Mabelane, Ndivhuho A Makhado
Cryptococcosis is an opportunistic infection with high mortality if not diagnosed and treated in time. The objective of this study was to review the clinicopathological information of decendents with final autopsy diagnosis of disseminated cryptococcal infection. This study collected data from 4 decendents who presented to an academic hospital/laboratory between 1 January 2015 to 31 December 2018. Their clinical, radiological and pathological findings including treatment were reviewed. Two decendents presented with respiratory symptoms whilst the other 2 presented with meningeal symptoms. Three were confirmed HIV positive. One decendent was on ART, one had defaulted treatment and one was ART naïve. Two decendents were diagnosed with cryptococcal meningitis, one with bacterial pneumonia and one with pulmonary tuberculosis. Three decendents died in emergency unit and one in the ward whilst on antifungal therapy. The autopsy findings confirmed disseminated cryptococcal infection in all cases. A high index of suspicion should be maintained in the right clinical context. Multi-organ involvement should be suspected in all patients and be actively sought out.
隐球菌病是一种机会性感染,如果不及时诊断和治疗,死亡率很高。本研究的目的是回顾最终尸检诊断为弥散性隐球菌感染的患者的临床病理信息。本研究收集了2015年1月1日至2018年12月31日期间在学术医院/实验室就诊的4名患者的数据。本文对其临床、影像学及病理表现及治疗进行综述。两名后代出现呼吸道症状,另外两名出现脑膜症状。其中三人被确认为艾滋病毒阳性。一个家属正在接受抗逆转录病毒治疗,一个没有接受治疗,一个正在接受抗逆转录病毒治疗naïve。两名后代被诊断为隐球菌性脑膜炎,一名患有细菌性肺炎,一名患有肺结核。3名患者在急诊室死亡,1名患者在病房接受抗真菌治疗时死亡。尸检结果证实所有病例均为播散性隐球菌感染。在正确的临床环境中,应保持高度的怀疑指数。所有患者都应怀疑有多脏器受累,并积极寻找。
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引用次数: 0
Hepatoid Adenocarcinoma of the Lung: A Rare Form of Lung Cancer 肺肝样腺癌:一种罕见的肺癌
IF 1.3 Q3 PATHOLOGY Pub Date : 2022-01-01 DOI: 10.1177/2632010X221088961
Yasmine Laraqui Houssein, S. Derqaoui, Raissouni Khouloud, A. Jahid, F. Zouaidia, Z. Bernoussi, K. Znati
Hepatoid adenocarcinoma is a rare type of extrahepatic adenocarcinoma with glandular and hepatocyte differenciation. The tumor can occur in many organs, generally in the stomach, the location of the lung being extremely rare. Despite poor prognosis and few effective treatment options, a timely and accurate histopathological diagnosis is key to optimal clinical management for long-term survival. Given the few reports published to characterized hepatoid adenocarcinoma, the emergence of any new case will contribute to improve understanding of the disease. Very few reports have been published to characterize hepatoid adenocarcinoma cytologically or even histologically. The aim of this work is to focus on the anatomoclinical characteristics of this rare entity. We present a retrospective study of 2 cases of pulmonary hepatoid adenocarcinoma confirmed by histological and immunohistochemical study.
肝样腺癌是一种罕见的肝外腺癌,有腺细胞和肝细胞分化。肿瘤可发生在许多器官,一般在胃,肺的位置极为罕见。尽管预后差,有效的治疗方案很少,但及时准确的组织病理学诊断是长期生存的最佳临床管理的关键。鉴于少有关于特征性肝样腺癌的报道,任何新病例的出现都将有助于提高对该疾病的了解。关于肝样腺癌的细胞学甚至组织学特征的报道很少。这项工作的目的是集中在这种罕见的实体解剖临床特征。我们对2例经组织学和免疫组织化学检查证实的肺肝样腺癌进行回顾性研究。
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引用次数: 2
Extreme Thrombocytosis in Refractory ITP Post-Splenectomy With Associated Fatal Thromboembolism 脾切除术后难治性ITP伴致死性血栓栓塞的极端血小板增多
IF 1.3 Q3 PATHOLOGY Pub Date : 2022-01-01 DOI: 10.1177/2632010X221083218
R. Nedumannil, E. Leitinger, S. Juneja
Thrombopoietin (TPO)-receptor agonists have heralded a paradigm shift in the treatment of refractory immune thrombocytopenia (ITP). Reactive thrombocytosis has been described as a secondary effect of such therapies. However, the phenomenon of extreme thrombocytosis with morphology mimicking a myeloproliferative neoplasm (MPN) followed by fatal thromboembolism is unusual in this setting. Caution is required in the diagnosis of refractory ITP as well as TPO-receptor agonist dosing in such cases.
血小板生成素(TPO)受体激动剂预示着治疗难治性免疫性血小板减少症(ITP)的范式转变。反应性血小板增多症已被描述为此类疗法的次要作用。然而,在这种情况下,出现形态类似骨髓增生性肿瘤(MPN)的极端血小板增多现象,然后出现致命的血栓栓塞是不常见的。在诊断难治性ITP以及在这种情况下给药TPO受体激动剂时需要谨慎。
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引用次数: 0
An Unusual Case of EBV-Negative Primary CNS Lymphoma of Natural Killer/T-Cell Lineage. 自然杀伤/ t细胞系ebv阴性原发性中枢神经系统淋巴瘤1例。
IF 1.3 Q3 PATHOLOGY Pub Date : 2021-12-12 eCollection Date: 2021-01-01 DOI: 10.1177/2632010X211065692
Naim I Kajtazi, Mohammed Bafaquh, Juman Al Ghamdi, Zahra AlEissa, Arwa Al Shmeikh, Ali Alsaeed, Tarek Sulaiman, M Adelita Vizcaino, Majed Al Hameed, Aditya Raghunathan

Extranodal NK/T-cell lymphoma (ENKTL) is a well-defined cytotoxic lymphoma strongly associated with Epstein-Barr virus (EBV) infection, commonly affecting the nasopharynx and upper aerodigestive tract. Primary central nervous system (CNS) involvement is rare, and only 17 cases were previously reported in the literature. Here, we report the case of a 44-year-old male admitted with a 3-month history of personality changes and progressive right leg weakness. Brain magnetic resonance imaging studies (MRIs) revealed multiple rim-enhancing brain lesions bilaterally. An extensive clinical and laboratory workup was unrevealing, and 2 brain biopsies were initially considered inconclusive. Pertinently, no systemic lymphoproliferative disorder was identified. The patient initially experienced remarkable clinical improvement with dexamethasone, pulse methylprednisolone, and rituximab therapy. However, he eventually had rapid clinical deterioration, was found to have increased brain lesions, and died nearly 6 months after the initial presentation. During this time, the second brain biopsy was found to show involvement by T-cell lymphoma of NK-cell lineage, which was EBV negative. No post-mortem examination was done to identify any systemic lymphoma. This case serves to expand the spectrum of lymphomas involving the CNS.

结外NK/ t细胞淋巴瘤(ENKTL)是一种明确的细胞毒性淋巴瘤,与eb病毒(EBV)感染密切相关,通常影响鼻咽部和上呼吸道消化道。原发性中枢神经系统(CNS)受累是罕见的,只有17例以前的文献报道。在此,我们报告一例44岁男性,因3个月的人格改变和进行性右腿无力而入院。脑磁共振成像研究(mri)显示双侧多发边缘增强脑病变。广泛的临床和实验室检查没有发现,两次脑活检最初被认为是不确定的。相应的,未发现系统性淋巴增生性疾病。患者最初通过地塞米松、脉冲甲基强的松龙和利妥昔单抗治疗获得了显著的临床改善。然而,他最终出现了快速的临床恶化,被发现有更多的脑部病变,并在最初的表现后近6个月死亡。在此期间,第二次脑活检发现nk细胞谱系的t细胞淋巴瘤,EBV阴性。没有进行尸检以确定任何系统性淋巴瘤。本病例扩大了累及中枢神经系统的淋巴瘤的范围。
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引用次数: 1
Clinicopathological and Molecular Characteristics of Macroscopically Yellowish-Colored Chromophobe Renal Cell Carcinoma Compared to Non-Yellowish-Colored Chromophobe Renal Cell Carcinoma. 宏观上黄色憎色性肾细胞癌与非黄色憎色性肾细胞癌的临床病理及分子特征比较。
IF 1.3 Q3 PATHOLOGY Pub Date : 2021-12-12 eCollection Date: 2021-01-01 DOI: 10.1177/2632010X211064821
Fumiyoshi Kojima, Ibu Matsuzaki, Naoto Kuroda, Yurina Mikasa, Fidele Y Musangile, Ryuta Iwamoto, Yuichi Takahashi, Akiko Matsubara, Yasuo Kohjimoto, Isao Hara, Shin-Ichi Murata

Each histological variant of renal cell tumors has a unique color. The yellowish color of clear cell renal cell carcinoma (CCRCC) is explained by the presence of intracytoplasmic lipid and glycogen accumulation. Color changes in CCRCC are correlated with clinicopathological and metabolic changes, as well as biological behavior. We analyzed and compared the clinical, histopathological, and immunohistochemical features and gene expression profiles, in lipid metabolism of yellowish-colored ChRCC (ChRCC-Y), non-yellowish-colored ChRCC (ChRCC-N), and CCRCC. Of 14 ChRCCs, we retrieved 6 ChRCC-Ys. Patients with ChRCC-Y are younger than those with ChRCC-N, and the tumor is not predominant in males. ChRCC-Ys are smaller than ChRRC-Ns. Three ChRCC-Ys exhibited individual discrete tubule formation. No ChRCC-Ns exhibited individual discrete tubule formation. Two of 6 ChRCC-Ys showed relatively diffuse adipophilin positivity. No ChRCC-Ns demonstrated diffuse positivity for adipophilin. The expression of SCD, FDFT1, and E2F1 showed a tendency to be lower in ChRCC-Y than in ChRCC-N. The expression of PDGFB showed a tendency to be higher in ChRCC-Y than in ChRCC-N. This study demonstrated ChRCC-Y did not indicate an increase in lipid and cholesterol metabolism and that ChRCC-Y did not have the common molecular alteration of CCRCC. The absence of such metabolic acceleration in ChRCC-Y might support the biological indolent behavior. Furthermore, we revealed that macroscopic color changes might be correlated with various clinicopathological features and immunohistochemical and molecular changes from different perspectives. We believe further characterization of RCC, including tumor heterogeneity, is needed to improve the management of patients with RCC.

肾细胞瘤的每一种组织学变异都有独特的颜色。透明细胞肾细胞癌(CCRCC)的淡黄色是由于存在胞浆内脂质和糖原积累。CCRCC的颜色变化与临床病理和代谢变化以及生物学行为有关。我们分析并比较了黄色ChRCC (ChRCC- y)、非黄色ChRCC (ChRCC- n)和CCRCC的脂质代谢的临床、组织病理学、免疫组织化学特征和基因表达谱。在14个chrcc中,我们检索到6个ChRCC-Ys。与ChRCC-N患者相比,ChRCC-Y患者更年轻,且该肿瘤在男性中并不占优势。ChRCC-Ys小于ChRRC-Ns。三个ChRCC-Ys表现出独立的离散小管形成。没有ChRCC-Ns表现出单独的离散小管形成。6例ChRCC-Ys中2例呈相对弥漫性亲脂素阳性。无ChRCC-Ns显示嗜脂素弥漫性阳性。SCD、FDFT1、E2F1在ChRCC-Y中的表达低于在ChRCC-N中的表达。PDGFB在ChRCC-Y中的表达有高于ChRCC-N的趋势。本研究表明,ChRCC-Y不显示脂质和胆固醇代谢的增加,并且ChRCC-Y不具有CCRCC常见的分子改变。在ChRCC-Y中缺乏这种代谢加速可能支持生物惰性行为。此外,我们从不同角度揭示了宏观颜色变化可能与各种临床病理特征以及免疫组织化学和分子变化有关。我们认为需要进一步表征RCC,包括肿瘤异质性,以改善RCC患者的管理。
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Clinical Pathology
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