少见子宫间叶源性肿瘤病理诊断的新进展

<正>随着分子病理检测技术的不断发展,研究人员对各种子宫间叶源性肿瘤的认识进一步深化。WHO(2020)女性生殖系统肿瘤（简称第五版WHO）对子宫间叶源性肿瘤的分类、分级进行更新和补充,本文根据第五版WHO分类并结合最新研究进展,对原发于子宫和近年新发现的几种少见间叶源性肿瘤的特点进行归纳和总结,旨在为临床、病理医师的诊断和鉴别诊断提供新思路。1平滑肌源性肿瘤平滑肌源性肿瘤的亚型繁多,本文重点介绍新近引起重视的几种特殊类型的肿瘤。     

关注神经元和混合性神经元-胶质肿瘤的临床病理研究

神经元和混合性神经元-胶质肿瘤（neuronal and mixed neuronal-glial tumours）是一组有不同程度神经元分化和胶质分化的肿瘤,近些年来该类肿瘤得到了越来越多的重视。在最新出皈的WHO中枢神经系统肿瘤分类（2007版,简称WHO2007年分类）中,变化最大的就属神经元和混合性神经元胶质肿瘤这一章节。在WHO2007年分类中共新增了八个新的肿瘤类型,而有关神经元和混合性神经元胶质肿瘤这一章节中就增加了三种新肿瘤,使其包括了12种肿瘤类型;小脑发育不良性神经节细胞瘤（Lhermitte-Duelos）、婴儿促纤维增生性星形细胞瘤／节细胞胶质瘤（desmoplastic infantile astrocytoma／ganglioglioma）、胚胎发育不良性神经上皮肿瘤（dysembryoplastic neuroepithelial tumour,DNT）,     

低度恶性潜能之乳头状尿路上皮肿瘤

世界卫生组织／国际泌尿病理学2004年分类[WHO（2004）／ISUP]对尿路上皮肿瘤分级标准化进行了有益尝试。在乳头状膀胱肿瘤分类中,新加入了“具低度恶性潜能的乳头状尿路上皮肿瘤（PUNLMP）”条目。WHO（2004）／ISUP尿路上皮肿瘤分类系统与被广泛接受的WHO1973年分类相比,具有数项潜在优势：（1）详细定义了不同瘤前病变和不同分级肿瘤的形态标准,包括更加标准化地定义了非浸润性乳头状尿路上皮性肿瘤（NIPUT）的组织学分级标准,有利于提高病理医师间诊断的可重复性;（2）建立了统一的术语和一般性定义;（3）力图将肿瘤分类系统的术语设计得与尿细胞学术语更为吻合,使得细胞-组织学的对应关系更易于操作,为指导改善患者的治疗提供了可能性;（4）去除了模糊的分类级别,如TCC分级Ⅰ～Ⅱ,TCC分级Ⅱ～Ⅲ;（5）单独列出了具有高危险进展为浸润性癌的肿瘤。     

组蛋白H3 K27M和BRAF V600E共突变的儿童中线Ⅰ级节细胞胶质瘤

<正>节细胞胶质瘤是一种Ⅰ级肿瘤,其特点为MAPK通路突变,包括BRAF V600E突变。最近,伴H3 K27M突变的弥漫中线胶质瘤作为一种Ⅳ级的新类型被WHO(2016)新分类收录。同时出现H3 K27M和BRAF V600E突变已在中线胶质瘤和间变性节细胞胶质瘤中报道,作者检测54例儿童中线     

cIMPACT-NOW对胶质瘤分子分型的补充更新及意义

中枢神经系统肿瘤分类分子信息及实践方法联盟-非WHO官方组织(cIMPACT-NOW)就2016 WHO中枢神经系统肿瘤分类第4版修订版发布以来的神经系统肿瘤部分临床实践中的分类分级、争议问题与研究进展进行了深入的分析研究,发布了4次更新,包括(1)NEC、NOS术语的使用范围;(2)H3K27M突变型弥漫性中线胶质瘤的定义修订;(3)IDH野生型具有胶质母细胞瘤分子特征的弥漫性星形细胞胶质瘤,WHOⅣ级的分子遗传特征;(4)以MYB、MYBL1或FGFR1改变或BRAF V600E突变为特征的IDH野生型/H3野生型弥漫性胶质瘤。上述更新将成为未来第5版WHO中枢神经系统肿瘤分类改版建议,在该文中逐一进行分析解读。     

2017版WHO甲状腺肿瘤分类解读北大核心CSCD

2017版WHO内分泌肿瘤分类根据肿瘤的病理、临床及基因学特点,对内分泌肿瘤的分类进行了全面的更新。新版WHO中甲状腺肿瘤分类总体可分为上皮性肿瘤、非上皮性肿瘤和继发肿瘤三大类（表1）,其中最重要的进展在于甲状腺滤泡上皮细胞起源的高分化肿瘤。乳头和滤泡、良性和恶性分类的更新,并新增加了一组甲状腺交界性肿瘤。嗜酸细胞肿瘤从滤泡性肿瘤中剔除,成为一组独立的病变。低分化癌诊断标准进一步明确。     

解读2014年ITMIG胸腺上皮性肿瘤分类共识

WHO(2004)胸腺上皮性肿瘤分类依据形态学变化将胸腺上皮肿瘤分为A、AB、B1、B2、B3型胸腺瘤、胸腺癌及其它少数类型胸腺肿瘤[1]。尽管现阶段仍有少数学者采用其它分类方法,但WHO(2004)胸腺上皮性肿瘤分类因具有临床和病理的可比性及生物学行为和预后的相关性在国际范围内被广泛应用。该分类在近10年的应用中存在一些问题,如有些类型的诊断重复性差、定义模糊导致分类困难、少数     

间变性淋巴瘤激酶基因蛋白在不同级别胶质瘤中表达

我们应用免疫组化法检测间变性淋巴瘤激酶(ALK)基因蛋白在胶质瘤中的表达情况，分析其与胶质瘤分级、分期以及病人生存期的关系，为胶质瘤分级和预后判断找到新的指标。     

2004年WHO胸腺上皮肿瘤分类简介与浅评

WHO肿瘤分类《肺、胸膜、胸腺和心脏肿瘤病理学和遗传学》（2004年）（以下简称新版）由Travis等主编,全世界一百多位专家参加了编写。与以往的各分册相比,体现了发展的思想,有很多新颖和科学的观点,增加了一些新的肿瘤类型以及最新的诊断标准,对病理和临床医生的实际工作均有指导意义。胸腺肿瘤是该书的第3章,由Miiller-Hermelink主编,依然遵循了《胸腺肿瘤组织病理学分类》1999年版（以下简称99版）分类的原则。本文简评其中的胸腺上皮肿瘤（thymic epithelial tumours,TET）分类修改、增补和未完善之处。     

WHO中枢神经系统肿瘤分类(2007)评介

《WHO中枢神经系统肿瘤分类》第4版（以下简称第4版分类）于2007年6月由国际癌症研究机构（International Agency for Researchon Cancer,IARC）正式出版,WHO出版社发行,反映了自第3版WHO神经系统肿瘤分类（2000）出版㈨以来神经肿瘤病理学领域的重要进展。     

应用2008版WHO造血和淋巴组织肿瘤分类对4例母细胞性NK细胞淋巴瘤重新评估

目的 探讨2008版WHO淋巴造血系统分类对母细胞性NK细胞淋巴瘤的重新分类和命名,观察此类淋巴瘤的组织形态、免疫表型分型及临床特点,并探讨其起源.方法 对符合2001版WHO造血和淋巴系统肿瘤分类(WHO分类)中母细胞性NK细胞淋巴瘤标准的4例,结合临床特点,观察其组织学形态、行免疫组织化学EliVision法染色,按2008版WHO分类进行重新分类.结果 4例的组织形态均呈母细胞性NK细胞淋巴瘤改变,CD56阳性,不表达T、B细胞和髓系标记,EBER原位杂交4例均阴性,均符合2001版WHO分类中NK母细胞性淋巴瘤的诊断标准.按2008版WHO分类,3例原发于皮肤,表达CD56、CD4和CD123,应诊断为母细胞性浆细胞样树突细胞肿瘤;1例发生于淋巴结,CD56和CD4阳性,CD123阴性,也无皮肤病变,只能暂时纳入来源不确定的白血病-NK细胞淋巴母细胞白血病/淋巴瘤.结论 2001版WHO分类中所谓的母细胞性NK细胞淋巴瘤实际上是一组异源性肿瘤,具有不同的免疫表型和临床特点,对此类肿瘤的细化分类和准确命名是2008版WHO淋巴瘤分类的一大进步.     

Gastric cancer: problems in histological diagnosis

The reproducibility of the most recent histological classifications for gastric cancer was studied. A comparison of the results obtained independently by both authors using the criteria of Lauren, Ming, Mulligan and WHO, showed that highest reproducibility was achieved with the WHO classification. However, an acceptable accuracy in diagnosis for the other three classifications was obtained. This study confirms the validity of the WHO criterion for initial diagnosis, while the other classifications should be kept for further specific investigations, such as epidemiological or prognostic studies.     

Transitional cell tumours of the bladder: classification and diagnostic pitfalls

Two classifications of transitional cell tumours have been published since 1998. Both have sought to address the nomenclature of non-invasive papillary tumours, as the practice stemming from the 1973 WHO classification of labelling virtually all of these tumours as carcinomas was judged to be unsatisfactory. Both classifications introduced the term of ‘urothelial neoplasm of low malignant potential’. The 1998 WHO/ISUP considered that this replaced the 1973 WHO grade 1 carcinoma, and that grades 2 and 3 would then be subdivided into low and high grade, whereas the 1999 WHO retained the three grades of carcinoma. The classifications are therefore not equivalent and in the absence of prospective data on clinical outcome and reproducibility studies, it is difficult to recommend their adoption. The importance of clinical factors in assessing the risk of tumour recurrence and progression must be emphasized, and closer integration of pathological and other clinical data should lead to improvements in individual patient outcome. This should also be the case for patients with muscle invasive disease, since urothelial carcinomas have a propensity to differentiate along different pathways, most commonly squamous, but also glandular or neuroendocrine, and the type of differentiation may affect responses to therapy.     

Rarely metastasizing soft tissue tumours

Soft tissue tumours that rarely metastasize have been afforded their own subcategory in recent WHO classifications. This review discusses the nature of these tumours and the difficulty in constructing usefully simple classifications for heterogeneous and complex groups of tumours. We also highlight the specific rarely metastasizing soft tissue tumours that have been recently added to the WHO classification (phosphaturic mesenchymal tumour, pseudomyogenic haemangioendothelioma) and those entities where there have been recent important defining genetic discoveries (myxoinflammatory fibroblastic sarcoma, solitary fibrous tumour, myoepitheliomas).     

The role of histological investigation in prognostic evaluation of advanced gastric cancer. Analysis of histological structure and molecular changes compared with invasive pattern and stage

The relative contribution of tumour histology or molecular changes, compared with invasion pattern or stage, to prognostic assessment of gastric cancer was investigated in a series of 185 advanced (T2 to T4, stage IB to IV) cancers that had undergone intentionally curative surgery at Varese General Hospital. Survival analysis of the histological types considered in commonly used classifications, such as Lauren, Kubo, the World Health Organization (WHO) and related classifications, allowed separation of a small high-grade (Hg, 12 cases) group of adenosquamous, anaplastic and small cell endocrine carcinomas from a large cohesive group (C, 86 glandular or solid cancers) and from another large (87 cases) group of tumours with dissociated cells [29 diffuse (D) and 58 mixed (M) tumours]. Univariate and multivariate analysis showed the independent prognostic value of this C/M+D/Hg classification approach, which proved superior to other classifications and to cell dissociation at the growing front or angio, lympho and neuro-invasion. Expression of sialyl Lewis(c), the DUPAN-2 antigen, proved to be an independent predictor of worse survival among tumours beyond stage I, showing an exclusively or predominantly cohesive structure. Microsatellite instability (MSI) predicted favourable survival in purely cohesive tumours of intermediate (II) stage, especially of solid/medullary and lymphoid stroma/lympho-epithelioma-like structure, among which two distinct tumour subsets were characterised, one MSI-positive and the other Epstein-Barr virus positive. T2NOM0 (stage IB) tumours showed mostly favourable survival independently from histological type, invasive pattern, DUPAN-2 or MSI status. It is concluded that an appropriate histological evaluation, coupled with sialylated glycoproteins histochemistry and, for stage-II tumours, MSI tests may contribute significantly to prognostic assessment of tumours beyond stage I. However, the stage itself, with special reference to lymph-node metastases and invasion level beyond subserosa, remains the most important prognostic clue for gastric cancer.     

Prognostic analysis of pulmonary adenocarcinoma subclassification with special consideration of papillary and bronchioloalveolar types

AIMS: The third edition of the World Health Organization (WHO) classification of lung tumours has been published and is expected to become the standard nomenclature. The aim of this study was to assess the usability and prognostic significance of the WHO classification in comparison with other recent classifications. METHODS AND RESULTS: One hundred and forty-seven resected pulmonary adenocarcinoma cases were reviewed and histologically classified according to the WHO classification (1999) and the classification by Noguchi (1995). Papillary carcinomas as described by Silver and Askin (1997) were also identified. Since the papillary type in the WHO classification is not strictly defined, we compared the following two kinds of WHO classification: (i) WHO-N; WHO classification adopting Noguchi Type F as the definition of the papillary type, namely, pure papillary adenocarcinoma without a bronchioloalveolar component; (ii) WHO-SA; WHO classification adopting papillary carcinoma by Silver and Askin as the definition of the papillary type, namely, tumour with papillary structure constituting at least 75% of the lesion. The bronchioloalveolar carcinoma of the WHO classification showed a better prognosis than other subtypes in both overall and Stage I disease limited survival analysis. In analysis limited to Stage III disease, only the papillary type of WHO-SA showed a significantly worse prognosis. CONCLUSIONS: WHO-SA is recommended for prognostic correlation.     

Updates and review of neoplastic paediatric neuropathology

Tumours of the central nervous system are the second most common type of malignancy in the paediatric population, after haematopoietic malignancies. With the 2016 edition of the WHO Classification of the Tumours of the Central Nervous System (CNS), a diagnostic approach to paediatric CNS malignancies has been adopted, which increasingly incorporates molecular parameters, as well as histologic features. This classification system represents a major restructuring of many paediatric central nervous system tumours. This review aims to highlight the areas in the WHO 2016 classification system that have undergone the greatest changes in paediatric tumours of the central nervous system, as well as to review the key histologic and clinical components of these entities. The greatest changes in classification were adopted in embryonal tumours and paediatric diffuse midline gliomas with histone H3 mutations, while low grade astrocytic and glioneuronal tumours also underwent important grading changes.     

Tumours in Iceland. 16. Malignant tumours of the stomach. Histological classification and description of epidemiological changes in a high-risk population during 30 years.

Iceland is one of the high-risk countries for stomach cancer. During the period 1955-84 the incidence declined from 76 to 28 per 10(5) p.a. for males and from 30 to 12 for females. Tissue material from the primary site in 978 males and 448 females was available for histological typing. By the WHO classification tubular carcinoma was most frequent in both sexes, 66% in males and 63% in females, and signet-ring carcinoma second, 13% in males and 16% in females. By the Laurén classification in males 78.1% were intestinal and 16.5% diffuse carcinomas, and in females 73.1% were intestinal and 20.7% diffuse carcinomas. The decline in stomach cancer in Icelanders has mostly affected the intestinal type of tumour (Laurén) and the tubular type of tumour (WHO). Diffuse type tumours (Laurén) have declined slightly. This supports the theory that intestinal carcinomas are more influenced by environmental and especially dietary factors, and that diffuse carcinomas are more influenced by other as yet unknown factors. For epidemiological studies both histological classifications have their value, the WHO especially in that it is based on standard histopathological criteria and the Laurén especially in that it only includes two tumour types. The WHO classification can roughly be transcribed to the Laurén classification as tubular, mucinous and papillary carcinomas fall into the group of intestinal tumours, and signet-ring and more than half of undifferentiated carcinomas into the group of diffuse tumours.     

Diagnostic difficulties in cases of papillary urothelial neoplasm of low malignant potential,urothelial proliferation of uncertain malignant potential,urothelial dysplasia and urothelial papilloma: A review of current literature

Tumours of the urinary tract are the fifth most frequent type of cancer. The most common types are urothelial tumours, among which, non-invasive urothelial neoplasms represent 45% of all cases. The 2016 WHO classification of urinary tract tumours introduced new classifications of non-invasive lesions. Besides urothelial papilloma (UP) and papillary urothelial neoplasm of low malignant potential (PUNLMP), as described in the former classification, the new classification also includes new entities such as urothelial proliferation of uncertain malignant potential (UPUMP) and urothelial dysplasia (UD). Of the aforementioned, UPUMP is the lesion that most commonly progresses, but solely to non-invasive carcinomas. UD is associated with a high risk of progression to invasive carcinoma. Understanding the biological character, and establishing the correct differential diagnosis in cases of non-invasive, non-cancerous lesions of the urinary bladder, will be of importance in establishing outcome predictions for future patients. A systematic review of the current literature allows us to systematize genetic, morphologic and prognostic factors of such lesions. Moreover, the collected data provide the basis for a proposed diagnostic algorithm which facilitates quick and effective differential diagnoses in cases of non-invasive non-cancerous urinary bladder lesions.