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Well-differentiated neuroendocrine tumor of the ileal pouch in a patient with ulcerative colitis and primary sclerosing cholangitis: report of a case and review of the literature. 溃疡性结肠炎和原发性硬化性胆管炎患者回肠袋分化良好的神经内分泌肿瘤:病例报告和文献综述。
Q4 Medicine Pub Date : 2024-01-01
Ondřej Fabián, Andrea Heribanová, Pavel Drastich, Luděk Voska

Gastrointestinal tract is the most common locality for well-differentiated neuroendocrine tumors (NET). While their occurrence in patients with ulcerative colitis (UC) is uncommon, it has been well documented. However, the causal relationship between development of NET and chronic intestinal inflammation or dysplasia remains controversial. The presence of NET in the ileal pouch in UC patients has been described only in a few reports to date. In this article, we present a case of such a tumor arising in the pouch in a patient with primary sclerosing cholangitis-associated UC, who underwent a restorative proctocolectomy with ileal pouch anal anastomosis and liver transplantation. The case is supported by a review of a relevant literature.   Correspondence address: Ondrej Fabian Clinical and Transplant Pathology Centre Institute for Clinical and Experimental Medicine Videnska 1958/9 Prague, 14021 Czech Republic ondrej.fabian@ikem.cz; ondrejfabian5@gmail.com.

胃肠道是分化良好的神经内分泌肿瘤(NET)最常见的部位。虽然它们在溃疡性结肠炎(UC)患者中的发生率并不常见,但已有大量文献记载。然而,NET的发生与慢性肠道炎症或发育不良之间的因果关系仍存在争议。迄今为止,关于 UC 患者回肠袋中出现 NET 的报道寥寥无几。在本文中,我们介绍了一例原发性硬化性胆管炎相关性 UC 患者的回肠袋肿瘤,该患者接受了回肠袋肛门吻合术和肝移植手术。本病例得到了相关文献的支持。 通讯地址Ondrej Fabian 临床与移植病理学中心 临床与实验医学研究所 Videnska 1958/9 Prague, 14021 Czech Republic ondrej.fabian@ikem.cz; ondrejfabian5@gmail.com.
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引用次数: 0
Changes in the diagnosis of thyroid tumours in the 5th edition of the WHO classification of endocrine neoplasms. 第五版世界卫生组织内分泌肿瘤分类中甲状腺肿瘤诊断的变化。
Q4 Medicine Pub Date : 2024-01-01
Dušková J

The WHO classification of thyroid tumours enters its second half-century of development with the 5th edition. Compared to the previous 4th edition of the clas- sification, the permanent increase in information is mainly at the molecular biological level. This has changed the view of very traditional entities - the preferred name for polynodous goiter is (given the monoclonal nature of some nodules) follicular nodular thyroid disease. Some terminological relics have also been re- moved - Hürthle cells are definitively referred to as oncocytes. Follicular adenoma has a new subtype with papillary arrangement (and missing nuclear features of papillary carcinoma). In the already used NIFTP unit, subtypes smaller than 10 mm and oncocytic are newly defined. All oncocytic tumours have an arbitrarily set minimum proportion of oncocytes at 75 %. A multidisciplinary approach to the treatment of thyropathies and the stratification of therapeutic procedures according to risk brought about the introduction of grading into several nosological units of papillary, follicular, and medullary carcinomas. Grading using the number of mitoses determines their quantification at 2 mm² instead of the previously used non-uniform HPFs (high power fields of view). Clarification was made on the basis of genetic findings in a number of other, less frequent diagnoses (e.g. classification of squamous cell carcinoma among anaplastic). Among rare tumors a new category of salivary gland - type carcinomas is formulated with two representatives: mucoepidermoid and secretory carcinoma. Cribriform morular carcinoma previously classified as a variant of papillary carcinoma is newly separated on the basis of the immunological and genetic profile into the newly created category of tumors of uncertain histogenesis. This category also includes sclerosing mucoepidermoid carcinoma with eosinophilia. Microcarcino- ma as a separate entity is not included in the 5th edition. A tumor smaller than 10 mm must be characterized by the appropriate features of the corresponding category. Thyroblastoma replaces terminologically malignant teratoma from the previous classification. Part of the newly established diagnostic criteria is also applicable in FNAB diagnosis. The newly introduced grading in some nosological units can exceptionally change the diagnosis (NIFTP/EFVPTC/non-invasive HG FVPTC), but above all it will affect the choice of therapeutic procedures.

世卫组织甲状腺肿瘤分类法第五版的问世,标志着该分类法的发展进入了第二个半世纪。与之前的第4版分类相比,信息的永久性增长主要体现在分子生物学层面。这改变了人们对传统实体的看法--多结节性甲状腺肿的首选名称是(鉴于某些结节的单克隆性质)滤泡结节性甲状腺疾病。一些术语遗存也被重新使用--Hürthle细胞被明确称为癌细胞。滤泡腺瘤有了新的亚型,具有乳头状排列(缺少乳头状癌的核特征)。在已使用的 NIFTP 单元中,新定义了小于 10 毫米的亚型和肿瘤细胞。所有肿瘤细胞的最低比例被任意设定为 75%。多学科方法治疗甲状腺疾病以及根据风险对治疗程序进行分层,使得乳头状癌、滤泡状癌和髓样癌的多个命名单元都引入了分级。分级使用有丝分裂的数量来决定其在 2 平方毫米范围内的定量,而不是以前使用的非均匀 HPF(高功率视野)。根据遗传学研究结果,对其他一些不太常见的诊断进行了澄清(如将鳞状细胞癌归入无性细胞癌)。在罕见肿瘤中,唾液腺型癌是一个新的类别,有两个代表:粘液表皮样癌和分泌性癌。以前被归类为乳头状癌变种的楔形蜕膜癌,根据免疫学和遗传学特征被新划分为组织发生不确定的肿瘤类别。这类肿瘤还包括嗜酸性硬化性粘液表皮样癌。微小癌作为一个独立的实体未被纳入第 5 版。小于 10 毫米的肿瘤必须具有相应类别的特征。甲状腺母细胞瘤在术语上取代了之前分类中的恶性畸胎瘤。新制定的部分诊断标准也适用于 FNAB 诊断。在某些命名单元中新引入的分级可例外地改变诊断(NIFTP/EFVPTC/非侵袭性 HG FVPTC),但最重要的是,它将影响治疗程序的选择。
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引用次数: 0
How to improve pre-operative diagnostics of pancreatobiliary lesions? From immunohistochemistry to Next Generation Sequencing. 如何改进胰胆管病变的术前诊断?从免疫组化到新一代测序。
Q4 Medicine Pub Date : 2024-01-01
Marián Švajdler, Ondřej Daum, Magdaléna Daumová, Jiřina Pintová, Robert Procházka

Preoperative cytopathology of pancreatobiliary neoplastic lesions is a sensitive and specific method and is irreplaceable in the diagnosis and clinical management of these diseases. Pathologists should make every attempt to provide diagnosis as precise as possible and minimize the rate of "atypical" results, which create management dilemmas. The diagnostic accuracy of cytopathology can be significantly improved by judicious use of ancillary studies, including immunohistochemistry and molecular genetics. Next generation sequencing (NGS) is the latest addition to pancreatobiliary cytopathology diagnostic arsenal. NGS is not only a very robust diagnostic tool, but also carries significant prognostic and therapeutic information.

胰胆管肿瘤病变的术前细胞病理学是一种敏感而特异的方法,在这些疾病的诊断和临床治疗中具有不可替代的作用。病理学家应尽一切努力提供尽可能精确的诊断,并尽量降低 "非典型 "结果的发生率,因为 "非典型 "结果会造成管理上的困境。通过合理使用辅助研究(包括免疫组化和分子遗传学),细胞病理学诊断的准确性可以大大提高。下一代测序(NGS)是胰胆细胞病理学诊断武器库中的最新成员。NGS 不仅是一种非常强大的诊断工具,还能提供重要的预后和治疗信息。
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引用次数: 0
Mesenchymal skin tumors - Novel entities in the 5th edition of WHO classification of skin tumors. 间质皮肤肿瘤--第五版世界卫生组织皮肤肿瘤分类中的新实体。
Q4 Medicine Pub Date : 2024-01-01
Michael Michal

The section on mesenchymal tumors in 5th edition of WHO classification of skin tumors has undergone several changes, the most important of which, as usual, is the inclusion of newly identified tumor entities, which will be the main focus of this review article. These specifically include three novel cutaneous mesenchymal tumors with melanocytic differentiation, and rearrangements of the CRTC1::TRIM11, ACTIN::MITF, and MITF::CREM genes. In addition, EWSR1::SMAD3-rearranged fibroblastic tumors, superficial CD34-positive fibroblastic tumors, and NTRK-rearranged spindle cell neoplasms were newly included. Of the other changes, only the most important ones will be briefly mentioned.

第五版世界卫生组织皮肤肿瘤分类中有关间质肿瘤的部分发生了一些变化,其中最重要的变化是纳入了新发现的肿瘤实体,这也是本综述文章的重点。这些肿瘤具体包括三种具有黑色素细胞分化的新型皮肤间质肿瘤,以及 CRTC1::TRIM11、ACTIN::MITF 和 MITF:CREM 基因重排。此外,EWSR1::SMAD3 基因重排的成纤维细胞瘤、浅表 CD34 阳性成纤维细胞瘤和 NTRK 基因重排的纺锤形细胞瘤也被新纳入其中。在其他变化中,仅简要提及最重要的变化。
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引用次数: 0
Changes in thyroid cytology reporting in the 3rd edition of the Bethesda system. 贝塞斯达系统第三版中甲状腺细胞学报告的变化。
Q4 Medicine Pub Date : 2024-01-01
Dušková J

Reporting fine-needle aspiration of thyroid nodules in the Bethesda classification is a practice widely used internationally and by us. The revised third edition of the Bethesda System of Reporting Thyroid Cytopathology brings changes in terminology, content, and new chapters. In terms of terminology, an obvious change is the removal of the two-word names of three categories while maintaining the six diagnostic categories of the previous versions - new: BI - non-diag- nostic, BIII - atypia of undetermined significance, BIV - follicular neoplasia. In the detailed description of the findings within the individual categories, the ter- minological changes adopted by the fifth edition of the WHO classification of thyroid neoplasia are respected - in particular, the recommended name follicular thyroid nodular disease for the most frequently represented category BII - benign. In the evaluation itself, the diagnostic specifications accepted by the current WHO classification of histopathological findings are reflected in the individual categories - if they are applicable at the cytological level. Targeted attention will need to be paid to high grade features. The revised version brings new chapters dedicated to molecular testing and evaluation of the paediatric population.

按照贝塞斯达分类法报告甲状腺结节的细针穿刺是国际上和我国广泛采用的一种做法。修订后的《贝塞斯达甲状腺细胞病理学报告系统》第三版在术语、内容和新章节方面都有所变化。在术语方面,一个明显的变化是取消了三个类别的两个字名称,同时保留了以前版本的六个诊断类别--新的:BI--非诊断性、BIII--意义未定的不典型、BIV--滤泡性肿瘤。在详细描述各个类别的检查结果时,第五版《世界卫生组织甲状腺肿瘤分类》所采用的技术变化得到了尊重--尤其是最常见的类别BII--良性,建议命名为滤泡性甲状腺结节病。在评估本身中,如果组织病理学结果适用于细胞学层面,则在各个类别中反映出当前世卫组织分类所接受的诊断规范。需要有针对性地关注高级别特征。修订版新增了关于分子检测和儿科评估的章节。
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引用次数: 0
ANCA-associated vasculitis from a clinical perspective. 从临床角度看 ANCA 相关性血管炎。
Q4 Medicine Pub Date : 2024-01-01
Zdenka Hrušková, Vladimír Tesař

ANCA-associated vasculitides (AAV) are small-vessel necrotizing vasculitides, with no or few immune deposits. They are usually associated with the presence of ANCA antibodies (AntiNeutrophil Cytoplasmic Antibody), targeted either against proteinase 3 (PR3-ANCA) or myeloperoxidase (MPO-ANCA). ANCA-associated vasculitides include granulomatosis with polyangiitis (formerly Wegener's), microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome). The most commonly afflicted organs involve the lungs and the respiratory tract, ENT area, and the kidneys. Renal involvement typically manifests as pauci-immune necrotizing crescentic rapidly progressive glomerulonpehritis. Pulmo-renal syndrome with lung haemorrhage and deteriorating kidney function may be acutely life-threatening. Diagnostic methods include ANCA measurement, imaging methods and biopsy. Early recognition of the diagnosis and an early start of adequate treatment are necessary for a good outcome. The current treatment typically consists of corticosteroids and either cyclophoshapmide or rituximab (a monoclonal antibody directed against CD20 antigen). The addition of plasma exchange may be considered in severe cases. Rituximab is preferred for the treatment of all relapsing forms of this vasculitis.

ANCA相关性血管炎(AAV)是一种小血管坏死性血管炎,没有或仅有少量免疫沉积。它们通常与 ANCA 抗体(抗中性粒细胞胞浆抗体)的存在有关,该抗体针对蛋白酶 3(PR3-ANCA)或髓过氧化物酶(MPO-ANCA)。ANCA 相关血管病包括肉芽肿伴多血管炎(原韦格纳氏病)、显微镜下多血管炎和嗜酸性肉芽肿伴多血管炎(原楚格-斯特劳斯综合征)。最常受累的器官包括肺和呼吸道、耳鼻喉科和肾脏。肾脏受累通常表现为贫免疫性坏死性新月体快速进展性肾小球肾炎。伴有肺出血和肾功能恶化的肺肾综合征可能会危及患者的生命。诊断方法包括 ANCA 测量、影像学方法和活组织检查。要想获得良好的治疗效果,就必须尽早确诊并开始适当的治疗。目前的治疗通常包括皮质类固醇和环磷酰胺或利妥昔单抗(一种针对 CD20 抗原的单克隆抗体)。严重病例可考虑进行血浆置换。利妥昔单抗是治疗所有复发性血管炎的首选药物。
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引用次数: 0
Endolymphatic sac tumour - a rare complication associated with von Hippel-Lindau disease. 内淋巴囊瘤-一种与希佩尔-林道病相关的罕见并发症。
Q4 Medicine Pub Date : 2023-01-01
Mária Wozniaková, Vladimír Židlík, Jozef Škarda

We report the case of a 42-year - old female with familiar form von Hippel-Lindau disease (VHL) and recurrent endolymphatic sac tumour (ELST), which was presented like non-homogenous, solid and cystic expansion of the left petrous temporal bone. Histologically, there was found lamellae of bone with adjacent ligament and with papillary projections with fibrovascular core. The papillae were lined by a single layer of cuboidal epithelium with hyperchromatic and lightly pleomorphic nuclei. Sporadically, small cystic formations with eosinophilic, PAS positive secretion were noted. Imunohistochemically, the cuboidal cells showed diffuse positivity for vimentin, epithelial membrane antigen (EMA), cytokeratin AE1/AE3 and S100 protein (weakly). Other markers examined, including TTF1, PAX8 and CD10, were negative. Endolymphatic sac tumour is rare low-grade malignant epithelial tumour arising from the endolymphatic sac in the temporal bone, which occurs in 1 out of 30 000 births, with just fewer than 300 cases reported in the literature. About one third of cases are associated with von Hippel- Lindau disease, an autosomal dominant familial cancer syndrome.

我们报告一位42岁女性,患有常见的von Hippel-Lindau病(VHL)和复发性内淋巴囊肿瘤(ELST),表现为左侧颞骨非均匀性、实性和囊性扩张。组织学上可见骨板及邻近韧带,并有纤维血管核心的乳头状突起。乳突内衬单层立方上皮,细胞核深染,浅多形性。偶见小囊性形成伴嗜酸性粒细胞,PAS阳性分泌。免疫组化结果显示,长方体细胞vimentin、上皮膜抗原(EMA)、细胞角蛋白AE1/AE3和S100蛋白弥漫性阳性(弱阳性)。其他标志物,包括TTF1, PAX8和CD10,均为阴性。内淋巴囊肿瘤是一种罕见的低级别恶性上皮性肿瘤,起源于颞骨内淋巴囊,发病率为1 / 30000,文献报道病例不足300例。大约三分之一的病例与von Hippel- Lindau病有关,这是一种常染色体显性家族性癌症综合征。
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引用次数: 0
Current possibilities of histopathologic separation of idiopathic pulmonary fibrosis from fibrotic hypersensitivity pneumonitis. How to do it? 目前特发性肺纤维化与纤维化性过敏性肺炎的组织病理学分离的可能性。怎么做呢?
Q4 Medicine Pub Date : 2023-01-01
Radoslav Matěj

Histopathological pattern of progressive pulmonary fibrosis could be seen in many different fibrotic lung interstitial diseases. Exact diagnosis is crucial for precise therapy, moreover, different diseases have different prognosis. The most important disorders in this group are idiopatic pulmonary fibrosis and fibrotic hypersensitivity pneumonitis, and their separation is crucial because of totally different treatment of the patients. The aim of this review is to sum up the most important characteristics of usual interstitial pneumonia, histopathological pattern of idiopatic pulmonary fibrosis, and fibrotic hypersensitivity pneumonitis and provide a practical work-up for precise diagnostics of these diseases in the frame of effectively cooperating multidisciplinary team.

进行性肺纤维化的组织病理学模式可以在许多不同的纤维化性肺间质性疾病中看到。准确的诊断是精确治疗的关键,而且不同的疾病有不同的预后。该组中最重要的疾病是特发性肺纤维化和纤维化过敏性肺炎,由于患者的治疗方法完全不同,因此将它们分开是至关重要的。本文的目的是总结常见间质性肺炎、特发性肺纤维化和纤维化性超敏性肺炎的最重要特征,并在多学科团队有效合作的框架下为这些疾病的精确诊断提供实用的工作。
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引用次数: 0
Clinical and histopathological aspects of the most common inflammatory non-infectious skin diseases. 最常见的炎症性非传染性皮肤病的临床和组织病理学方面。
Q4 Medicine Pub Date : 2023-01-01
Miroslav Důra, Eva Sticová, Andrea Felšöová, Jiří Štork

The authors present a didactic overview of the most common inflammatory non-infectious skin diseases. This overview is not exhaustive, but illustrative, especially when regarding the aspect of a systematic approach to the evaluation of skin biopsy with an initial evaluation of the morphological pattern of the inflammatory process. This will subsequently facilitate the diagnosis. Photodocumentation of typical primary skin manifestations is attached to the photomicrograph images. This enables the pathologist to make a basic clinical-pathological correlation, which is of fundamental importance in dermatopathology.

作者对最常见的炎症性非传染性皮肤病进行了总结。这一概述并非详尽无遗,而是说明性的,尤其是当涉及到评估皮肤活检的系统方法与炎症过程的形态学模式的初步评估时。这将有助于随后的诊断。典型原发性皮肤表现的照片记录附在显微照片图像上。这使病理学家能够进行基本的临床病理相关性,这在皮肤病理学中具有根本的重要性。
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引用次数: 0
Bleeding after childbirth / miscarriage - practical notes on the examination of biopsy material. 分娩/流产后出血-活检材料检查的实用注意事项。
Q4 Medicine Pub Date : 2023-01-01
Šárka Hadravská, Vladimír Korečko, Magdaléna Daumová

Postpartum haemorrhage is a significant cause of maternal morbidity and mortality worldwide. The pathologist encounters only a limited spectrum of causes leading to postpartum haemorrhage. The most common causes are retained placenta and placental site subinvolution. Both of these lesions can be diagnosed from material obtained by uterine curettage. Morbidly adherent placenta (placenta accreta spectrum) is a less frequent subject of investigation, the diagnosis of which can be reliably established only on the basis of histological examination of uterine specimens after hysterectomy.

产后出血是全世界孕产妇发病和死亡的一个重要原因。病理学家只遇到导致产后出血的有限原因。最常见的原因是胎盘保留和胎盘部位亚复旧。这两种病变都可以从子宫刮除获得的材料中诊断出来。病态附着性胎盘(胎盘增生谱)是一种不常见的研究对象,其诊断只能在子宫切除术后子宫标本组织学检查的基础上可靠地建立。
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引用次数: 0
期刊
Ceskoslovenska patologie
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