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Histomorphological Subtypes of Hepatocellular Carcinoma and Intrahepatic Cholangiocarcinoma: Review and Update 肝细胞癌和肝内胆管癌的组织形态学亚型:综述和最新进展
Pub Date : 2022-11-01 DOI: 10.1097/PCR.0000000000000531
Yoon Jung Hwang, Haeryoung Kim
Abstract Primary liver cancers comprise a heterogeneous group of neoplasms, with the 2 main entities being hepatocellular carcinoma (HCC) and intrahepatic cholangiocarcinoma (iCCA). Over the past decades, HCC and iCCA have been recognized to be heterogeneous in morphology, molecular features, and biological behavior, and the most recent World Health Organization classification of digestive system tumors describes various histomorphological subtypes of HCC and iCCA, some of which also have characteristic molecular features, clinical correlates, and prognostic implications. In this review, we discuss 4 recently described subtypes of HCC and iCCA—macrotrabecular HCC, steatohepatitic HCC, scirrhous HCC, and small duct iCCA—based on a series of cases.
原发性肝癌是一类异质性肿瘤,主要有肝细胞癌(HCC)和肝内胆管癌(iCCA)两种。在过去的几十年里,HCC和iCCA在形态、分子特征和生物学行为上被认为是异质的,最近世界卫生组织对消化系统肿瘤的分类描述了HCC和iCCA的各种组织形态学亚型,其中一些也具有特征性的分子特征、临床相关性和预后意义。在这篇综述中,我们基于一系列病例,讨论了最近被描述的4种HCC和icca亚型:大梁型HCC、脂肪性HCC、硬化性HCC和小导管icca。
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引用次数: 0
A Case Review on Combined Hepatocellular Cholangiocarcinoma 合并肝细胞胆管癌1例回顾
Pub Date : 2022-11-01 DOI: 10.1097/PCR.0000000000000532
Reiichiro Kondo, J. Akiba, H. Yano
Abstract Combined hepatocellular-cholangiocarcinoma (cHCC-CCA) is a rare primary liver tumor with neoplastic components of both hepatocytic and cholangiocytic differentiation within the same tumor, such as the case reported herein. There is a need to standardize the pathological diagnosis of cHCC-CCA and to improve the concordance of diagnosis among pathologists based on consensus paper because of its histological and phenotypic diversity that leads to confusion in terminology and classification. This case review addresses the terminology, classification, and clinical and pathological characteristics of cHCC-CCA and its differential diagnosis.
摘要:合并肝细胞胆管癌(Combined hepatocellular- cholangicarcinoma, cHCC-CCA)是一种罕见的原发性肝脏肿瘤,在同一肿瘤内同时具有肝细胞和胆管细胞分化的肿瘤成分,如本文报道的病例。由于cHCC-CCA的组织学和表型多样性导致术语和分类的混淆,需要规范其病理诊断,提高病理学家基于共识论文的诊断一致性。本病例综述讨论了cHCC-CCA的术语、分类、临床和病理特征及其鉴别诊断。
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引用次数: 0
Hemophagocytic Lymphohistiocytosis: A Practical Review for Liver Pathologists 噬血细胞性淋巴组织细胞病:肝脏病理学家的实践回顾
Pub Date : 2022-11-01 DOI: 10.1097/PCR.0000000000000535
Yuqing Xiong, A. Del Portillo, Ladan Fazlollahi, H. Remotti, Michael J. Lee, S. Lagana
Abstract Hemophagocytic lymphohistiocytosis (HLH) is a rare disease with a high mortality. Liver involvement is common, with most patients demonstrating acute hepatitis. Therefore, liver biopsies are frequently obtained in the setting of suspected HLH. Several studies have described the clinicopathologic manifestations of liver involvement by HLH. The histology is highly variable, as are rates of demonstrated hemophagocytosis. This review summarizes the clinical characteristics of HLH, with particular emphasis on hepatic manifestations thereof. We then describe the histologic patterns that have been commonly and uncommonly reported. Finally, we address difficulties faced by liver pathologists when considering this entity and offer our opinions regarding the worst “pain points.”
噬血细胞淋巴组织细胞病(HLH)是一种死亡率高的罕见疾病。肝脏受累是常见的,大多数患者表现为急性肝炎。因此,肝活检往往是在设定怀疑HLH。一些研究描述了HLH累及肝脏的临床病理表现。组织学是高度可变的,正如所证明的噬血细胞症的发生率。本文综述了HLH的临床特点,特别强调其肝脏表现。然后我们描述了常见和不常见的组织学模式。最后,我们讨论了肝脏病理学家在考虑这一实体时所面临的困难,并就最严重的“痛点”提供了我们的意见。
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引用次数: 0
Hepatocellular Adenoma: A Case Report, Current Updates on Subtyping, and Diagnostic Pitfalls 肝细胞腺瘤:一个病例报告,当前亚型更新和诊断缺陷
Pub Date : 2022-11-01 DOI: 10.1097/PCR.0000000000000527
Geunyoung Jung, Yongjun Liu
Abstract Hepatocellular adenoma (HCA) is a benign liver neoplasm that typically arises in the noncirrhotic liver. Based on molecular findings, HCA can be subclassified into the following subtypes: hepatocyte nuclear factor 1α–inactivated HCA, inflammatory HCA, β-catenin–activated HCA, sonic hedgehog-activated HCA, and unclassified HCA. Mixed β-catenin–activated inflammatory HCA may rarely occur. We report a case of HCA that was initially diagnosed as inflammatory HCA on a biopsy but finally was confirmed to be β-catenin–activated inflammatory HCA on the resection specimen. Most recent updates on molecular classification, key histologic features of each subtype, and common diagnostic pitfalls are discussed.
肝细胞腺瘤(HCA)是一种良性肝脏肿瘤,通常发生在非肝硬化肝脏。根据分子特征,HCA可分为以下亚型:肝细胞核因子1α -失活HCA、炎性HCA、β-连环蛋白活化HCA、音刺猬活化HCA和未分类HCA。混合β-连环蛋白激活的炎性HCA可能很少发生。我们报告一例HCA,最初在活检中被诊断为炎症性HCA,但最终在切除标本上被证实为β-连环蛋白激活的炎症性HCA。最新的分子分类,每个亚型的关键组织学特征和常见的诊断陷阱进行了讨论。
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引用次数: 0
Congestive Hepatopathy: A Case of Fontan-Associated Liver Disease and Review of Literature 充血性肝病:方丹相关肝病1例并文献复习
Pub Date : 2022-11-01 DOI: 10.1097/PCR.0000000000000534
Matthew Gosse, D. Bosch
Abstract We describe a case of congestive hepatopathy due to altered circulation with a Fontan procedure. Congestive hepatopathy is characterized microscopically by sinusoidal dilation and congestion, interstitial edema, and hepatocyte atrophy. Congestive hepatic fibrosis typically exhibits pericentral pattern fibrosis at an early stage, progressing to bridging fibrosis and cirrhosis. Fibrosis scoring systems have recently been described for Fontan-associated liver disease and congestive heart disease–associated hepatopathy. The clinical differential diagnosis for congestive hepatopathy is broad and includes right-sided heart failure (“cardiac hepatopathy”), hepatic vein obstruction (such as Budd-Chiari syndrome), and intrahepatic vessel or sinusoidal obstruction (such as sinusoidal obstructive syndrome). Treatment of congestive hepatopathy is primarily directed toward the etiology of congestion.
摘要我们描述一例充血性肝病由于改变循环与丰坦程序。充血性肝病的显微特征是窦状窦扩张和充血,间质水肿和肝细胞萎缩。充血性肝纤维化早期典型表现为中心周围型纤维化,发展为桥接性纤维化和肝硬化。纤维化评分系统最近被描述为丰坦相关肝病和充血性心脏病相关肝病。充血性肝病的临床鉴别诊断很广泛,包括右侧心力衰竭(“心源性肝病”)、肝静脉梗阻(如Budd-Chiari综合征)、肝内血管或肝窦梗阻(如肝窦梗阻综合征)。充血性肝病的治疗主要针对充血的病因。
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引用次数: 0
Undifferentiated Endometrial Carcinoma—Diagnostic and Therapeutic Challenges 未分化子宫内膜癌的诊断和治疗挑战
Pub Date : 2022-09-01 DOI: 10.1097/PCR.0000000000000525
Yow-Shan Lee, Cheng-Han Lee
Abstract Undifferentiated/dedifferentiated endometrial carcinoma is clinically highly aggressive, particularly when it is defined by inactivation of core SWI/SNF proteins (co-loss of ARID1B, loss of SMARCA4, or loss SMARCB1 expression). We report here the case of a 37-year-old woman who presented with menorrhagia and the subsequent pathologic and clinical workup showed an advanced-stage ARID1A/ARID1B-deficient mismatch repair–proficient undifferentiated endometrial carcinoma. Despite pelvic external beam radiation and platinum/taxane-based chemotherapy (4 of 6 planned cycles), the patient experienced disease progression with osseous metastasis to her left elbow and died of her disease 7 months after clinical presentation. This case highlights the diagnostic and treatment challenges associated with such undifferentiated/dedifferentiated cancer types of the endometrium.
未分化/去分化子宫内膜癌在临床上具有高度侵袭性,特别是当其定义为核心SWI/SNF蛋白失活时(ARID1B、SMARCA4或SMARCB1表达缺失的共同缺失)。我们在此报告一位37岁女性的病例,她表现为月经过多,随后的病理和临床检查显示为晚期ARID1A/ arid1b缺陷错配修复熟练的未分化子宫内膜癌。尽管盆腔外束放疗和铂/紫杉烷为基础的化疗(6个计划周期中的4个),患者经历了疾病进展,骨性转移到左肘,并在临床表现后7个月死于疾病。本病例强调了与子宫内膜未分化/去分化癌相关的诊断和治疗挑战。
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引用次数: 0
Counterpoint: Integration of Molecular Subtype and Histotype/Grade Into One Classification System for Endometrial Carcinoma 对照:将子宫内膜癌的分子亚型和组织型/分级整合为一个分类系统
Pub Date : 2022-09-01 DOI: 10.1097/PCR.0000000000000529
M. Köbel
Abstract The establishment of 4 endometrial carcinoma molecular subtypes by The Cancer Genome Atlas project and their surrogates (POLE mutated, mismatch repair–deficient, p53 abnormal, and no specific molecular profile) invigorated a debate on how to integrate these within the traditional histotype/grade classification. Specific issues of contradicting histotype/grade and molecular subtype diagnoses will be discussed with illustrated examples. The hierarchy of histotype/grade and molecular subtype is interdependent. Pathologists should aim to integrate histotypes/grades and molecular subtypes to provide one consistent and clinically relevant diagnosis.
Cancer Genome Atlas项目建立了4种子宫内膜癌分子亚型及其替代品(POLE突变、错配修复缺陷、p53异常和无特异性分子谱),引发了如何将这些亚型整合到传统的组织型/分级分类中的争论。与组织型/分级和分子亚型诊断相矛盾的具体问题将通过举例来讨论。组织型/等级和分子亚型的层次是相互依赖的。病理学家应致力于整合组织型/分级和分子亚型,以提供一个一致的和临床相关的诊断。
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引用次数: 0
Carcinomas With Concurrent Involvement of the Endometrium and Uterine Adnexa—Implications for Pathological Diagnosis and Clinical Management in Current Practice 同时累及子宫内膜和子宫附件的癌——当前实践中病理诊断和临床处理的意义
Pub Date : 2022-09-01 DOI: 10.1097/PCR.0000000000000524
N. Singh, A. Tinker, C. Gilks
Abstract A proportion of endometrial and adnexal carcinomas have concurrent involvement of the other site. In the case of high-grade serous carcinomas involving the tubal epithelium as well as endometrium, distinction of tubo-ovarian high-grade serous from endometrial serous carcinoma can have implications for surgical as well as nonsurgical treatment approaches, including targeted therapies and referral to clinical genetics services. The other situation is involvement of the endometrium and ovary by low-grade endometrioid carcinoma; here separation of high-stage endometrial carcinoma from 2 low-stage, low-grade tumors determines adjuvant treatment decisions. These challenging scenarios are illustrated with case presentations and criteria for pathological reporting while acknowledging uncertainty where this is warranted. It is accepted that these are areas in transition, and any criteria offered are likely to change in the light of new information.
一部分子宫内膜癌和附件癌同时累及其他部位。在涉及输卵管上皮和子宫内膜的高级别浆液性癌的情况下,区分输卵管卵巢高级别浆液性癌和子宫内膜浆液性癌可能对手术和非手术治疗方法有影响,包括靶向治疗和转诊到临床遗传学服务。另一种情况是低级别子宫内膜样癌累及子宫内膜和卵巢;高分期子宫内膜癌与2个低分期、低分级肿瘤的分离决定了辅助治疗的决定。这些具有挑战性的情况说明了病例介绍和病理报告的标准,同时承认不确定性,这是必要的。大家都认为,这些是处于过渡时期的领域,所提出的任何标准都可能根据新的资料而改变。
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引用次数: 1
Chronic Endometritis: Diagnostic Considerations in Patients With Infertility 慢性子宫内膜炎:不孕患者的诊断考虑
Pub Date : 2022-09-01 DOI: 10.1097/PCR.0000000000000523
Sandra Lee
Abstract Chronic endometritis (CE) is a controversial clinical and pathological entity. Although the presence of plasma cells (PCs) is the most frequently used diagnostic criterion for CE, the minimal diagnostic criteria remain controversial and undefined. The clinical setting of CE (asymptomatic, pelvic inflammatory disease, infertility) is an important consideration regarding the clinical significance of endometrial PCs. In the setting of infertility, specifically recurrent implantation failure (RIF) and recurrent pregnancy loss (RPL), CE may have a negative impact on endometrial receptivity, resulting in higher rates of implantation failure. The proposed pathophysiology is that a subclinical intrauterine infection alters the local inflammatory milieu resulting in a shift of the normal inflammatory cell profiles toward responses associated with implantation failure and poorer reproductive outcomes. Cure rates following antibiotic treatment are reported to be high (up to 90%). The results of meta-analyses describing the effect of CE on reproductive outcomes are mixed. There is evidence supporting CE as a treatable factor with improved reproductive outcomes following treatment and there are also results showing no differences in reproductive outcomes. Variable diagnostic criteria for CE, inclusion criteria, and treatment regimens between studies are the main limitations, hampering the ability to compare results across studies. Results regarding the optimal diagnostic criteria in the setting of RIF and RPL are also variable, with some authors recommending a cutoff of 1 or more CD138+ PCs per high-power field and some recommending a cutoff of 5 or more CD138+ PCs per high-power field. There are some studies indicating CE as a negative prognostic factor in patients with RIF and RPL, which may be reversible with antibiotic treatment. The optimal diagnostic criteria for CE in this clinical setting are undefined. For the investigation of infertility patients whom clinicians intend to treat, a descriptive diagnosis (indicating the presence/number of PCs and method of identification) is a reasonable approach. Routine use of CD138 immunohistochemistry is of limited value for the diagnosis of CE.
慢性子宫内膜炎(CE)是一个有争议的临床和病理实体。虽然浆细胞(PCs)的存在是CE最常用的诊断标准,但最低诊断标准仍然存在争议和不明确。CE的临床背景(无症状、盆腔炎、不孕症)是子宫内膜pc临床意义的重要考虑因素。在不孕症的情况下,特别是复发性着床失败(RIF)和复发性妊娠丢失(RPL), CE可能对子宫内膜容受性产生负面影响,导致更高的着床失败率。提出的病理生理学是亚临床宫内感染改变了局部炎症环境,导致正常炎症细胞向与植入失败和较差生殖结果相关的反应转变。据报道,抗生素治疗后的治愈率很高(高达90%)。描述CE对生殖结果影响的荟萃分析结果是混合的。有证据支持CE是一种可治疗的因素,治疗后可改善生殖结果,也有结果显示生殖结果没有差异。不同的CE诊断标准、纳入标准和不同研究之间的治疗方案是主要的局限性,阻碍了跨研究比较结果的能力。关于RIF和RPL设置的最佳诊断标准的结果也各不相同,一些作者建议每个大功率视场切断1个或更多CD138+ PCs,而另一些作者建议每个大功率视场切断5个或更多CD138+ PCs。有一些研究表明CE是RIF和RPL患者的负面预后因素,这可能通过抗生素治疗是可逆的。在这种临床环境中,CE的最佳诊断标准尚不明确。对于临床医生打算治疗的不孕症患者的调查,描述性诊断(表明pc的存在/数量和识别方法)是一种合理的方法。常规使用CD138免疫组化对CE的诊断价值有限。
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引用次数: 0
Mesonephric-Like Adenocarcinoma of the Endometrium: Review of the Literature and Practical Diagnostic Recommendations 子宫内膜中肾样腺癌:文献综述和实用诊断建议
Pub Date : 2022-09-01 DOI: 10.1097/PCR.0000000000000526
David B. Chapel, Kay J. Park
Abstract Mesonephric-like adenocarcinomas are endometrial and ovarian neoplasms of müllerian origin with morphologic, immunophenotypic, and molecular evidence of mesonephric-type transdifferentiation, as well as considerable homology with endometrioid tumors. First described in 2016, mesonephric-like adenocarcinomas are morphologically indistinguishable from “true” mesonephric adenocarcinomas of the uterine cervix, but the latter are distinguished by (1) primary localization to the cervical wall, (2) frequent association with mesonephric remnants, and (3) in some cases, lack of mucosal involvement. Despite an overall low-grade morphology, mesonephric-like adenocarcinoma follows an aggressive clinical course, characterized by frequent and early recurrences, most often in the lung. Accordingly, accurate distinction of mesonephric-like adenocarcinoma from morphologic mimics—especially low-grade endometrioid adenocarcinoma—is critical. However, available evidence indicates that endometrial mesonephric-like adenocarcinomas are significantly underdiagnosed, likely due to their relative novelty, rarity, and considerable overlap with endometrioid neoplasia. Prospective recognition of characteristic morphologic features, a low threshold for application of diagnostic immunohistochemistry, and judicious use of molecular studies will permit accurate diagnosis in almost all cases.
中肾样腺癌是起源于勒氏杆菌的子宫内膜和卵巢肿瘤,具有中肾型转分化的形态学、免疫表型和分子证据,并且与子宫内膜样肿瘤具有相当的同源性。在2016年首次报道中,间肾样腺癌在形态学上与“真正的”子宫颈间肾腺癌难以区分,但后者的区别在于(1)主要定位于宫颈壁,(2)经常与间肾残余相关,(3)在某些情况下,不累及粘膜。尽管整体形态学较低,但间肾样腺癌具有侵袭性的临床病程,其特点是频繁和早期复发,最常见于肺部。因此,准确区分中肾样腺癌和形态模拟腺癌,尤其是低级别子宫内膜样腺癌是至关重要的。然而,现有证据表明,子宫内膜中肾样腺癌的诊断明显不足,这可能是由于其相对新颖、罕见,且与子宫内膜样瘤有相当大的重叠。对特征性形态学特征的前瞻性识别,诊断性免疫组织化学应用的低门槛,以及明智地使用分子研究,将允许几乎所有病例的准确诊断。
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