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Solitary fibrous tumor of thyroid: Case report. 甲状腺单发纤维瘤:病例报告
Q4 Medicine Pub Date : 2024-01-01
Jiří Dušek

Solitary fibrous tumour is a relatively rare soft tissue fibroblastic tumour, accounting for approximately 2% of soft tissue tumours. It has been described primarily as a tumour of the pleural cavity; however, up to 70% of cases occur elsewhere, in any anatomical location, which can make diagnosis difficult. If this is the diagnosis being considered, the STAT6 antibody is currently available with high sensitivity and specificity. In this paper we describe the case of a 72-year-old female patient, followed up and treated by an outpatient endocrinologist for a multinodular euthyroid goitre for several years. Due to complete nodular remodelling of the left lobe of the thyroid gland and sonographic findings of several small nodules in the right lobe of the thyroid gland, total thyroidectomy was recommended to the patient. The operation was performed at the ENT department in Jindřichův Hradec Hospital. Material from the operation was subsequently sent for histopathological examination. Several hyperplastic colloid nodules and a small oncocytic adenoma were detected microscopically in the right lobe of the thyroid gland. In the left lobe, an imprecisely delineated, greyish-white lesion measuring 2 x 1.8 x 1.5 cm was observed on the section. Microscopically, the tumour consisted of spindle-shaped cells in a focally hyalinised stroma. In the immunohistochemical examination, tumour cells reacted positively with the CD34 antibody, and negatively with antibodies against thyroglobulin, cytokeratins (CK AE1/AE3) and S100 protein. Further immunohistochemical examinations (Bcl2, CD99, STAT6) with positive results were supplemented upon consultation at a higher facility. Based on morphology and the results of the immunohistochemical examinations, the tumour was diagnosed as a solitary fibrous tumour of the thyroid gland. This is a relatively unusual finding in this location; according to literature, only a few dozen cases have been described.

孤立性纤维瘤是一种相对罕见的软组织纤维肿瘤,约占软组织肿瘤的 2%。它主要被描述为胸膜腔肿瘤;然而,多达 70% 的病例发生在其他部位的任何解剖位置,这可能会给诊断带来困难。如果考虑进行诊断,STAT6 抗体目前具有高灵敏度和特异性。本文描述了一名 72 岁女性患者的病例,该患者因多结节甲状腺肿接受内分泌门诊医生的随访和治疗已有数年。由于甲状腺左叶完全结节性重塑,且声像图发现甲状腺右叶有几个小结节,医生建议患者进行全甲状腺切除术。手术在金日赫拉夫赫拉德茨医院耳鼻喉科进行。手术材料随后被送去进行组织病理学检查。在甲状腺右叶的显微镜下发现了几个增生性胶状结节和一个小的肿瘤细胞腺瘤。在左叶的切片上,观察到一个界限不清的灰白色病变,大小为 2 x 1.8 x 1.5 厘米。显微镜下,肿瘤由纺锤形细胞组成,基质局部透明。在免疫组化检查中,肿瘤细胞与 CD34 抗体呈阳性反应,与甲状腺球蛋白、细胞角蛋白(CK AE1/AE3)和 S100 蛋白抗体呈阴性反应。进一步的免疫组化检查(Bcl2、CD99、STAT6)结果呈阳性,经上级医疗机构会诊后予以补充。根据形态学和免疫组化检查结果,该肿瘤被诊断为甲状腺单发纤维性肿瘤。在这个部位,这种情况比较少见;根据文献记载,仅有几十例这样的病例。
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引用次数: 0
Histopathology of skin melanocytic lesions. 皮肤黑色素细胞病变的组织病理学。
Q4 Medicine Pub Date : 2024-01-01
Lumír Pock, Alena Skálová

Melanocytic lesions are instable tumors, the genome of which and its changes determinate their morphology and biological properties. Intermediate lesions share histomorphological features of both, nevi and melanoma. Melanocytomas represent a group of them separated on the basis of recent molecular-biological studies. The article summarizes benign, intermediate, malignant and combined melanocytic skin lesions and offers practical recommendations for diagnosis.

黑色素细胞病变是一种不稳定的肿瘤,其基因组及其变化决定了其形态和生物学特性。中间病变具有痣和黑色素瘤的组织形态学特征。黑色素细胞瘤是根据最新的分子生物学研究分离出来的一类肿瘤。文章总结了良性、中间型、恶性和混合型黑色素细胞皮肤病变,并提供了实用的诊断建议。
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引用次数: 0
Clinical, Morphological and Molecular Features of Spitz tumors. 斯皮茨肿瘤的临床、形态和分子特征。
Q4 Medicine Pub Date : 2024-01-01
Michele Donati, Boulos Mansour, Michael Hagstrom, Pedram Gerami, Dmitry V Kazakov

Spitz tumors represent a heterogeneous group of challenging melanocytic neoplasms, displaying a range of biological behaviors, spanning from benign lesions, Spitz nevi (SN) to Spitz melanomas (SM), with intermediate lesions in between known as atypical Spitz tumors (AST). They are histologically characterized by large epithelioid and/or spindled melanocytes arranged in fascicles or nests, often associated with characteristic epidermal hyperplasia and fibrovascular stromal changes. In the last decade, the detection of mutually exclusive structural rearrangements involving receptor tyrosine kinases ROS1, ALK, NTRK1, NTRK2, NTRK3, RET, MET, serine threonine kinases BRAF and MAP3K8, or HRAS mutation, led to a clinical, morphological and molecular based classification of Spitz tumors. The recognition of some reproducible histological features can help dermatopathologist in assessing these lesions and can provide clues to predict the underlying molecular driver. In this review, we will focus on clinical and morphological findings in molecular Spitz tumor subgroups.

斯皮茨瘤是一组具有挑战性的黑素细胞瘤,表现出一系列生物学行为,包括从良性病变斯皮茨痣(SN)到斯皮茨黑素瘤(SM),以及介于两者之间的被称为非典型斯皮茨瘤(AST)的中间病变。它们的组织学特征是大的上皮样和/或纺锤形黑素细胞呈束状或巢状排列,通常伴有特征性的表皮增生和纤维血管基质改变。在过去的十年中,由于发现了涉及受体酪氨酸激酶 ROS1、ALK、NTRK1、NTRK2、NTRK3、RET、MET、丝氨酸苏氨酸激酶 BRAF 和 MAP3K8 或 HRAS 突变的相互排斥的结构重排,从而对 Spitz 肿瘤进行了基于临床、形态学和分子学的分类。对一些可重复的组织学特征的认识有助于皮肤病理学家评估这些病变,并为预测潜在的分子驱动因素提供线索。在本综述中,我们将重点讨论分子Spitz肿瘤亚组的临床和形态学发现。
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引用次数: 0
Pitfalls of cytological diagnosis of tumours of the pancreaticobiliary tract. 胰胆管肿瘤细胞学诊断的陷阱。
Q4 Medicine Pub Date : 2024-01-01
Dušan Žiak, Vladimír Židlík, Eva Kundrátová, Jaroslav Horáček, Robert Ondruššek, Valeria Skopelidou, Pavel Hurník

The recent introduction of the WHO cytology classification of pancreatobiliary tumours aimed to improve the diagnosis and management of these tumours. The present paper briefly describes the methods of diagnosis. Emphasis is then put on a detailed comparison of the previous Papanicolaou classification and the new WHO classification and description of the changes brought about by the introduction of the WHO classification. In the last part of the paper, we present interesting cases from our practice illustrating possible diagnostic pitfalls of cytological evaluation.

最近推出的世界卫生组织胰胆管肿瘤细胞学分类法旨在改善这些肿瘤的诊断和管理。本文简要介绍了诊断方法。然后重点详细比较了以前的帕氏分类法和新的世卫组织分类法,并描述了世卫组织分类法的引入所带来的变化。在文章的最后部分,我们介绍了一些有趣的病例,说明细胞学评估可能存在的诊断误区。
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引用次数: 0
Parathyroid tumors in the 5th edition of the WHO Classification of Tumors of the Endocrine Organs. 第五版《世界卫生组织内分泌器官肿瘤分类》中的甲状旁腺肿瘤。
Q4 Medicine Pub Date : 2024-01-01
Dušková J

The diagnosis of pathological conditions of the parathyroid glands is the answer to clinically more frequently detected hypercalcemic conditions, including MEN syndromes. In routine biopsy practice, enlarged bodies are also a differential diagnosis for the diagnosis of thyroid nodules. In the chapter of parathyroid tumors, the 5th edition of the WHO classification brings changes influenced similarly to other endocrine organs by the increase in genetic information. At the terminological level, the concept of hyperplasia has been narrowed down to secondary hyperplasia, most of the previously primary hyperplasias are referred to as multiglandular parathyroid disease due to evidence of multiglandular clonal proliferations. The term atypical parathyroid tumor replacing atypical adenoma is newly introduced - the uncertain biological behaviour is emphasized. The basic examination includes parafibromin immunohis- tochemistry, the deficiency of parafibromin being an indicator of an inactivating CDC73 mutation and an increased risk of familial forms, or MEN. Methodologically, refinements are introduced in the quantification of mitotic activity per 10 mm2. Oncocytic subtypes have an arbitrarily declared threshold of more than 75% oncocytes. The definition of lipoadenoma (multiplication of both components, more than 50% of adipose tissue in the tumor) is similarly specified. The diagnosis of cancer remains histopathological with unequivocal evidence of invasion, or microscopically verified metastasis.

甲状旁腺的病理诊断是临床上较常发现的高血钙症(包括MEN综合征)的答案。在常规活检实践中,肿大体也是甲状腺结节诊断的鉴别诊断之一。在甲状旁腺肿瘤一章中,第五版世界卫生组织分类法与其他内分泌器官一样,也因遗传信息的增加而发生了变化。在术语层面上,增生的概念被缩小为继发性增生,以前的原发性增生大多被称为多腺体甲状旁腺疾病,因为有证据表明存在多腺体克隆性增生。非典型甲状旁腺瘤取代了非典型腺瘤,这是新引入的术语,强调了其不确定的生物学行为。基本检查包括副纤维蛋白免疫组化,副纤维蛋白缺乏是CDC73失活突变和家族性或MEN风险增加的指标。在方法上,对每 10 平方毫米有丝分裂活动的定量进行了改进。肿瘤细胞亚型的阈值为肿瘤细胞超过 75%。脂肪腺瘤的定义(两种成分均有增殖,肿瘤中脂肪组织超过 50%)也有类似规定。癌症的诊断仍然是组织病理学诊断,有明确的侵袭证据或经显微镜证实的转移。
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引用次数: 0
What a pathologist needs to know about disorders of the complement system. 病理学家需要了解的补体系统紊乱知识。
Q4 Medicine Pub Date : 2024-01-01
Eva Honsová

The complement system is an important component of innate immunity, which is part of the first line of defense against infections. In addition, complement plays an important role in the removal of apoptotic and damaged endogenous cells and, according to recent discoveries, contributes significantly to the homeostasis of the organism. The complement system includes several dozen soluble and membrane-bound proteins, which, after activation, function as a cascade, at the end of which is the elimination of the infectious agent. Complement activation occurs through one of 3 pathways (classical, lectin, and alternative) and all 3 pathways lead to the central C3 component. The cleavage of C3 starts the activation of the so-called effector terminal cascade, which participates in the elimination of pathogens through pro-inflammatory mechanisms, opsonization and, at the end, the creation of a channel in the basement membrane. The systematic control of complement activation plays an important role, because that represents prevention against damage to one's own tissues. Especially, the alternative pathway, which provides more than 80% of the activity of the terminal complement cascade, requires tight control. Dysregulation of complement and especially its alternative pathways is behind many acute and chronic diseases.

补体系统是先天免疫的重要组成部分,是抵御感染的第一道防线的一部分。此外,补体还在清除凋亡和受损的内源性细胞方面发挥着重要作用,而且根据最新发现,补体还对机体的平衡做出了重大贡献。补体系统包括几十种可溶性蛋白和膜结合蛋白,它们在激活后发挥级联作用,最终消除感染病原体。补体激活通过 3 种途径(经典途径、凝集素途径和替代途径)之一进行,所有 3 种途径都以 C3 为中心成分。C3 的裂解启动了所谓的效应终端级联的激活,它通过促炎机制、蛋白溶解以及最后在基底膜上形成通道参与消灭病原体。补体激活的系统控制起着重要作用,因为这代表着防止自身组织受损。尤其是提供末端补体级联 80% 以上活性的替代途径需要严格控制。补体,尤其是其替代途径的失调是许多急性和慢性疾病的根源。
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引用次数: 0
Calcific Aortic Stenosis - Inflammatory Disease. 钙化性主动脉瓣狭窄--炎症性疾病。
Q4 Medicine Pub Date : 2024-01-01
Ivo Šteiner

In developed countries, calcific aortic stenosis (CAS) has become the most common acquired valvular disease and cause for valve replacement. The prevalence of the disease increases with age, reaching over 5 % in adults over 75 years of age. The cases of CAS are classified as either of a previously normal (tricuspid) aortic valve (senile, syn. age - related, "sclerotic" type), or based on a congenitally malformed, usually bicuspid aortic valve. This paper is a brief summary of our 5 previous publications from the years 2007 - 2021, devoted to histopathology of CAS, namely to vascularization, inflammatory infiltrate and metaplastic ossification of the valve, and also to topography of these lesions in individual valve cusps. We conclude that calcification of the aortic valve is not a passive degenerative lesion, but an active multifactorial inflammatory process driven by cells native to the aortic valve. Pathogenesis of CAS is similar to that of atherosclerosis.

在发达国家,钙化性主动脉瓣狭窄(CAS)已成为最常见的后天性瓣膜疾病和瓣膜置换的原因。该病的发病率随着年龄的增长而增加,在 75 岁以上的成年人中发病率超过 5%。CAS 病例可分为以前正常(三尖瓣)的主动脉瓣(衰老型,与年龄相关的 "硬化 "型)或先天性畸形(通常为双尖瓣)的主动脉瓣。本文简要总结了我们在 2007 - 2021 年间发表的 5 篇关于 CAS 组织病理学的论文,即瓣膜的血管化、炎症浸润和移行性骨化,以及这些病变在单个瓣尖的形貌。我们的结论是,主动脉瓣钙化不是一种被动的退行性病变,而是由主动脉瓣原生细胞驱动的一种主动的多因素炎症过程。CAS 的发病机制与动脉粥样硬化相似。
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引用次数: 0
Current diagnostic and treatment options for pancreatic cancer in 2024. 2024 年胰腺癌的现有诊断和治疗方案。
Q4 Medicine Pub Date : 2024-01-01
Radim Němeček, Petr Karásek

Pancreatic ductal adenocarcinoma is a cancer disease with a very poor prognosis, which poses the third-leading cause of cancer-related deaths and whose incidence and mortality have been predicted to increase significantly in the upcoming years. Almost 80% of patients are diagnosed with advanced unresectable disease and therefore rely on palliative anticancer treatment with limited efficacy. However, even in case of 10-20 % of patients who have successfully undergone radical surgical resection of the localized disease and subsequent adjuvant chemotherapy, the vast majority will relapse within 2-3 years of surgery. The reasons can be found in late diagnosis due to the prolonged clinically asymptomatic course of the disease, complicated anatomical localization, significant tumor heterogeneity, which makes it difficult to test new drugs and, last but not least, in the presence of dense tumor stroma, that complicates the access of cytostatics and targeted drugs into the tumor tissue. Here we present a summary of current treatment options of localized and advanced pancreatic cancer, including molecular diagnostics and targeted treatment of small patients subgroups.

胰腺导管腺癌是一种预后极差的癌症疾病,是导致癌症相关死亡的第三大原因,预计其发病率和死亡率在未来几年将大幅上升。近 80% 的患者被诊断为无法切除的晚期疾病,因此只能依靠疗效有限的姑息性抗癌治疗。然而,即使有 10-20% 的患者成功接受了局部疾病的根治性手术切除和随后的辅助化疗,绝大多数患者也会在术后 2-3 年内复发。究其原因,主要是由于临床无症状病程较长导致诊断较晚、解剖定位复杂、肿瘤异质性大,导致新药试验困难,最后但并非最不重要的一点是,肿瘤基质致密,使细胞抑制剂和靶向药物进入肿瘤组织变得复杂。在此,我们总结了目前对局部和晚期胰腺癌的治疗方案,包括分子诊断和针对小患者亚群的靶向治疗。
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引用次数: 0
Intraductal and cystic pancreatic neoplasia. 胰腺导管内肿瘤和囊性肿瘤。
Q4 Medicine Pub Date : 2024-01-01
Markéta Hermanová

The current WHO classification of digestive system tumours (2019) has presented the concept of diagnostics of intraductal and cystic neoplasms of the pancreas mostly based on integrated molecular data and evaluations of their malignant potential. Intraductal pancreatic neoplasms with ductal phenotype include microscopic precursor lesions of pancreatic ductal adenocarcinoma - the pancreatic intraepithelial neoplasia and macroscopic precursor lesions of pancreatic cancer, where intraductal papillary mucinous neoplasm represents the most common neoplasm of the pancreas with cystic appearance. Both intraductal oncocytic papillary neoplasm and intraductal tubulopapillary neoplasm are now classified as separate entities associated with less aggressive subtypes of pancreatic carcinoma and better prognosis. Clinical significance of microscopic pancreatic intraepithelial neoplasias is limited, in contrast to other intraductal neoplasms, which are presented as cystic and/or solid tumours by imaging methods with important consequences for further treatment and indication of surgical therapy (resection versus "watch and wait" strategies). Neoplasms of nonductal origin, such as acinar cell carcinomas and neuroendocrine neoplasms, can uncommonly display an intraductal growth and their correct classification has a great clinical importance. Moreover, differential diagnostics of cystic pancreatic lesions include not only cystic and pseudocystically transformed neoplasms, but also a large spectrum of reactive, inflammatory and dysontogenetic cystic lesions.

目前的世界卫生组织消化系统肿瘤分类(2019 年)提出了胰腺导管内肿瘤和囊性瘤的诊断概念,主要基于综合分子数据和对其恶性潜能的评估。具有导管表型的胰腺导管内肿瘤包括胰腺导管腺癌的微观前驱病变--胰腺上皮内瘤变和胰腺癌的宏观前驱病变,其中导管内乳头状黏液瘤是最常见的囊性胰腺肿瘤。导管内肿瘤性乳头状瘤和导管内管状乳头状瘤现在都被归类为与侵袭性较低的胰腺癌亚型和较好的预后相关的独立实体。与其他导管内肿瘤相比,显微镜下胰腺上皮内肿瘤的临床意义有限,这些肿瘤通过影像学方法表现为囊性和/或实性肿瘤,对进一步治疗和手术治疗指征(切除与 "观察和等待 "策略)有重要影响。非导管源性肿瘤,如尖头细胞癌和神经内分泌肿瘤,也会罕见地出现导管内生长,因此对它们进行正确分类具有重要的临床意义。此外,胰腺囊性病变的鉴别诊断不仅包括囊性和假性囊变性肿瘤,还包括大量反应性、炎症性和发育不良性囊性病变。
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引用次数: 0
Will we change our view of the causes of the arterial hypertension development? 我们会改变对动脉高血压发病原因的看法吗?
Q4 Medicine Pub Date : 2024-01-01
Eva Honsová, Olga Snížková, Karolína Krátká

Arterial hypertension is one of the most common chronic diseases in the world. It is reported that it affects a third of the adult population and that in 2025 it will become the most common chronic disease. Hypertension does not have attacks and remissions; and if it occurs, it usually does not disappear and requires long-term lifelong treatment. Despite extensive and numerous studies of risk factors, we do not know the cause of hypertension. There are thousands of studies focused on various risk factors for the development of arterial hypertension. None of them apply in general and do not clarify the reasons for the development and progression of the disease. Recent experimental data strongly support a role for complement in all stages of arterial hypertension. Evidence that in a significant proportion of patients with so-called malignant hypertension, thrombotic microangiopathy is a manifestation of atypical hemolytic-uremic syndrome; conclusively shows that the disease is part of complement dysregulation. These facts shift our view of the role of complement, which is much more important in many diseases, including hypertension, than we previously thought.

动脉高血压是世界上最常见的慢性疾病之一。据报道,三分之一的成年人患有高血压,到 2025 年,高血压将成为最常见的慢性疾病。高血压不会发作和缓解,即使发作,通常也不会消失,需要长期终身治疗。尽管对风险因素进行了大量研究,但我们仍不知道高血压的病因。有数以千计的研究集中于动脉高血压发病的各种危险因素。这些研究都不具有普遍性,也没有阐明疾病发生和发展的原因。最近的实验数据有力地证明了补体在动脉高血压各个阶段的作用。有证据表明,在相当一部分所谓的恶性高血压患者中,血栓性微血管病变是非典型溶血性尿毒症综合征的一种表现形式;这一证据确凿地表明,该病是补体失调的一部分。这些事实改变了我们对补体作用的看法,补体在包括高血压在内的许多疾病中的作用比我们以前想象的要重要得多。
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引用次数: 0
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Ceskoslovenska patologie
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