Ceskoslovenska patologie最新文献

{"title":"EBV-associated plasmacytic variant of Castleman disease: more than a decade-long diagnostic odyssey - a case report.","authors":"Ivanna Boichuk, Zdeněk Adam","doi":"","DOIUrl":"","url":null,"abstract":"<p><strong>Background: </strong>Castleman disease is a rare and heterogeneous lymphoproliferative disorder with variable clinical presentation. The plasma cell variant, particularly when associated with Epstein-Barr virus (EBV), is uncommon and diagnostically challenging. We present a complex and long-lasting case of EBV-associated plasma cell variant Castleman disease with fluctuating systemic symptoms, multiorgan involvement, and delayed definitive diagnosis.</p><p><strong>Case presentation: </strong>A 35year old man (born 1974) first presented in 2009 with high-grade fever, diarrhea, elevated inflammatory markers, hepatomegaly and biochemical signs of liver injury. Initial findings were attributed to rotavirus infection. Over the following years, he developed recurrent episodes of fever, night sweats, fatigue, arthralgias, hepatosplenomegaly, lymphadenopathy, and progressive laboratory abnormalities including persistent elevation of CRP, leukocytosis, hyperfibrinogenemia and polyclonal hypergammaglobulinemia. Extensive diagnostic workup repeatedly ruled out infectious, rheumatologic and malignant causes. Imaging eventually demonstrated retroperitoneal lymphadenopathy, hepatosplenomegaly, spinal lesions (Th5- Th8), narrowing and occlusion of the inferior vena cava, and multiorgan inflammatory changes. Multiple biopsies (lymph nodes, spleen, liver, pancreas, bone lesions) initially showed only nonspecific reactive changes. Repeated PET/CT scans revealed multifocal FDG-avid lesions of low to moderate metabolic activity. In 2023, after multidisciplinary reassessment, lymph node tissue demonstrated EBV positivity in the absence of peripheral viremia, leading to a diagnosis of EBV-associated plasma cell variant Castleman disease. The patient was initiated on targeted therapy. During the third-line treatment, a sustained clinical and laboratory remission was achieved.</p><p><strong>Conclusion: </strong>This case illustrates the diagnostic complexity of EBV-associated plasma cell variant Castleman disease, especially when presenting with longstanding systemic inflammation, nonspecific multiorgan involvement, and repeatedly inconclusive histopathology. Early consideration of Castleman disease in chronic inflammatory syndromes with lymphadenopathy may reduce diagnostic delay and improve outcome.</p>","PeriodicalId":9861,"journal":{"name":"Ceskoslovenska patologie","volume":"62 1","pages":"58-61"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147716188","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Update from the 5th Edition of the World Health Organization Classification of Head and Neck Tumors: Salivary Glands.","authors":"Alena Skálová, Martina Bradová, Jan Laco","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>The salivary gland section in the 5th edition of the World Health Organization classification of head and neck tumors features a description and inclusion of several new entities, including sclerosing polycystic adenoma, keratocystoma, intercalated duct adenoma, and striated duct adenoma among the benign neoplasms; and microsecretory adenocarcinoma and sclerosing microcystic adenocarcinoma as the new malignant entities. The new entry also includes mucinous adenocarcinoma subdivided into papillary, colloid, signet ring, and mixed subtypes with recurrent AKT1 E17K mutation across patterns suggesting that mucin-producing salivary adenocarcinomas represent a histologically diverse single entity that may be related to salivary intraductal papillary mucinous neoplasm (IPMN). Cribriform adenocarcinoma of salivary gland origin (CASG) now represents a distinctive subtype of polymorphous adenocarcinoma (PAC). PAC is defined as a clinically, histologically and molecularly heterogeneous disease group. Whether CASG is a different diagnostic category or a subtype of PAC is still controversial. New defining genomic alterations have been characterized in many salivary gland tumors. In particular, they include gene fusions, which have shown to be tightly tumor-type specific, and thus valuable for use in diagnostically challenging cases. The recurrent molecular alterations were included in the definition of mucoepidermoid carcinoma, adenoid cystic carcinoma, secretory carcinoma, polymorphous adenocarcinoma, hyalinizing clear cell carcinoma, mucinous adenocarcinoma, and microsecretory adenocarcinoma. Importantly, the number of entities in the salivary chapter has been reduced by omitting tumors or lesions if they do not occur exclusively or predominantly in salivary glands, including hemangioma, lipoma, nodular fasciitis and hematolymphoid tumors. They are now discussed in detail elsewhere in the book.</p>","PeriodicalId":9861,"journal":{"name":"Ceskoslovenska patologie","volume":"62 2","pages":"75-86"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148497365","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"News in the 5th edition of the WHO Classification of Tumours of the Head and Neck: Tumours of the Sinonasal Tract and Skull Base Tumors.","authors":"Martina Bradová, Jan Laco, Alena Skálová","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>The World Health Organization (WHO) recently published the 5th edition of head and neck tumors. This edition describes both existing entities and a group of emerging entities, along with updates regarding taxonomy and detailed characteristics of tumors and tumor-like lesions. Sinonasal tumors and skull base tumors represent a heterogeneous group of tumors with significant histological variability and overlap in imaging methods. An important change in the 5th edition of the WHO classification is the relocation of recurrent soft tissue, hematolymphoid, and neuroectodermal tumors into a separate chapter, meaning they are no longer repeated in other chapters as they were previously. Only those tumors that are unique to the sinonasal area remain classified in this chapter. In this review article, we will primarily provide a brief overview of all 24 diagnostic entities, allowing readers to gain a concise understanding. We will focus in detail on the new entities of SWItch/Sucrose Non-Fermentable complex-deficient sinonasal carcinomas and human papillomavirus-related multiphenotypic sinonasal carcinoma. In another review article in this issue, we detailed IDH-mutated sinonasal malignancies; therefore, we will exclude them from this overview and concentrate on DEK::AFF2 carcinomas, currently classified as sinonasal undifferentiated carcinomas or non-keratinizing squamous cell carcinomas, respectively.</p>","PeriodicalId":9861,"journal":{"name":"Ceskoslovenska patologie","volume":"62 2","pages":"87-95"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148497371","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Testing Claudin 18.2 Expression in Gastric and Gastroesophageal Junction Adenocarcinoma: Current Status and Near‑Future Outlook.","authors":"Pavel Dundr, Radoslav Matěj","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Claudin 18.2 (CLDN18.2) represents one of the newest biomarkers expected to enter routine testing in the near future and expanding the spectrum of available predictive markers. It is currently a clinically relevant predictor for adenocarcinomas of the stomach and the gastroesophageal junction, although its use will likely extend also to other diagnoses. The aim of this report is to provide an overview of selected aspects of CLDN18.2 expression testing, including the choice of appropriate tissue, the issue of tumor heterogeneity, antibodies suitable for testing and their evaluation, where such testing can be performed, and the prospects for the future.</p>","PeriodicalId":9861,"journal":{"name":"Ceskoslovenska patologie","volume":"62 1","pages":"13-16"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147716193","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Tumor microenvironment of head and neck squamous cell carcinomas.","authors":"Anna Fialová, Lucie Pavelková, Klára Plačková","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>The character of the tumor microenvironment is a relevant prognostic and predictive biomarker across a wide range of malignancies. The composition, density, and functional capacity of tumor-infiltrating immune cells are especially crucial for selecting suitable immunotherapy. Head and neck squamous cell carcinomas are considered immunologically hot tumors, with high numbers of tumor-infiltrating effector and regulatory T cells. Higher T cell counts, along with a better prognosis, were observed in patients with head and neck squamous cell carcinomas associated with human papillomavirus infection. The immune profile of smoking-associated tumors was more variable, with higher numbers of suppressive myeloid cells and a substantial variability in T cell numbers between the patients. Nevertheless, the high density of cytotoxic T cells was a stronger prognostic factor for head and neck squamous cell carcinoma patients than HPV status alone. Thus, prognostic markers based on knowledge of the tumor microenvironment and tumor-infiltrating immune cells could significantly improve patient stratification for immunotherapeutic and de-escalation treatment protocols.</p>","PeriodicalId":9861,"journal":{"name":"Ceskoslovenska patologie","volume":"62 2","pages":"114-119"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148497395","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Targeted gene expression profiling as a tool for diagnostic cell-of-origin determination and prognostic stratification in diffuse large B-cell lymphoma.","authors":"Veronika Navrkalová, Andrea Marečková, Lenka Radová, Klára Činátlová, Václav Kubeš, David Šálek, Michael Doubek, Šárka Pospíšilová, Leoš Křen, Jana Kotašková","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Classification of diffuse large B-cell lymphoma (DLBCL) according to cell-of-origin (COO) distinguishes two main biological subtypes: activated B-cell-like (ABC) and germinal center B-cell-like (GCB). Although this distinction reflects different pathogenetic mechanisms, its prognostic impact diminishes in the context of evolving therapeutic strategies. Molecular subtyping of DLBCL, which is based on the spectrum of affected genes and aims to personalize treatment approaches, is currently gaining importance. In the study, we applied targeted gene expression profiling (GEP) using a custom Lympho-qPCR panel, enabling rapid and practically applicable ABC/GCB classification together with risk stratification of patients. RNA isolated from a cohort of 89 DLBCL tissue samples was analyzed using three GEP-based classification models. Model A compared the expression profile with immunohistochemical (IHC) COO determination and showed the expected lower correlation (62 %). Model B employed the expression scores of selected genes to predict COO regardless of IHC classification. Model C was developed as a new IHC-independent prognostic tool allowing patient stratification based on expected survival. Patients identified as high-risk by Model C had significantly worse outcomes, regardless of existing clinical prognostic indicators. In patients with early progression, parallel DNA sequencing analysis (integrative LYNX panel) confirmed complex chromosomal aberrations and defects in BCL2, TP53 and CDKN2A/B. Our results demonstrate that targeted GEP testing represents a robust, rapid, and clinically applicable method for COO determination and risk stratification in DLBCL patients. In the near future, the predictive value of ABC/GCB classification is expected to increase in relation to novel targeted therapeutic regimens. Integration of transcriptomic and genetic data will be essential for independent and individualized risk assessment in the molecular diagnostics of DLBCL.</p>","PeriodicalId":9861,"journal":{"name":"Ceskoslovenska patologie","volume":"62 1","pages":"43-49"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147716146","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The advantages and limitations of the new FIGO 2023 staging system for endometrial carcinoma from the perspective of the clinician and pathologist.","authors":"Martin Hruda, Radoslav Matěj, Borek Sehnal, Jana Drozenová, Helena Robová, Tomáš Pichlík, Michael J Halaška, Lukáš Rob, Pavel Dundr","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>The Czech Republic is one of the countries with the highest incidence of endometrial cancer in the world. In June 2023, the Women's Cancer Committee of the International Federation of Gynaecology and Obstetrics (FIGO) introduced a new staging system for endometrial cancer, FIGO 2023, which replaced the 2009 version. The FIGO 2023 staging system differs significantly from the previous version by incorporating the result of molecular classification of the tumour and some histopathological parameters - histological type of tumour, tumour grade and presence of substantial lymphovascular invasion - into the definitions of stage I and stage II. For stage I and II tumours, specific separate stages are reserved when the molecular profile of POLEmut or TP53mut is detected. Stages III and IV have also been modified, but the result of the molecular classification of the tumour and other histopathological parameters do not influence the staging. However, the molecular classification result should be reported for all stages. These changes have further strengthened the role of the pathologist in staging. The changes, which are partly based on the recommendations of the three European professional societies ESGO/ESTRO/ESP for the diagnosis and treatment of endometrial cancer, better reflect the biological behaviour of the tumour and significantly refine the prognosis of the patient at a given stage. On the other hand, the FIGO 2023 staging system is quite complex and requires expensive tests, which may pose a problem for its routine use in a global context. The implementation of the FIGO 2023 endometrial cancer staging system in daily practice requires the full involvement of all stakeholders.</p>","PeriodicalId":9861,"journal":{"name":"Ceskoslovenska patologie","volume":"62 1","pages":"35-42"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147716176","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Hypothermia in the home setiing - case report.","authors":"Lucia Moravčíková, Ján Bajaj, Ubomír Straka","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>In the present case report, the authors describe the deaths of two individuals-an 85-year-old female and her 56-year-old son - both discovered within a shared household, exhibiting advanced postmortem changes. The fatalities occurred in a confined apartment environment. The decedents had been residing under conditions of extreme environmental neglect, characterized by prolonged accumulation of domestic waste and an almost complete absence of interaction with the external community. Postmortem examinations revealed morphological indicators consistent with hypothermia, including pale postmortem lividity, frostbite lesions, and Visnevsky's spots within the gastric mucosa. The terminal causes of death in both cases were determined to be combined cardiovascular and respiratory failure secondary to hypothermia. Relevant comorbidities were identified: in the female, predominantly chronic cardiac and hepatic pathology; in the male, marked malnutrition. In both individuals, ethanol was detected in postmortem blood specimens at concentrations consistent with endogenous production during the late stages of decomposition. In both cases, different degrees of development of hypothermia-related Visnevsky spots were observed, which the authors explain by different reserve capacities of the organism.</p>","PeriodicalId":9861,"journal":{"name":"Ceskoslovenska patologie","volume":"71 2","pages":"12-15"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148497377","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Castleman disease - one name, many faces.","authors":"Kateřina Kamarádová, Václav Stejskal, Dominika Écsiová","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Castleman disease (CD) is a  mesmerising group of disorders mainly affecting lymph nodes sharing some morphological features but with heterogeneous aetiology, clinical presentation and therapeutic approaches. Morphologically, hyaline-vascular (or hypervascular), plasmacytic, and mixed types of changes are distinguished. Confirmation of the diagnosis and subtype of Castleman disease involves meeting or excluding several clinical criteria and therefore requires close cooperation with a clinician. Unicentric Castleman disease involves usually a solitary enlarged lymph node with mild symptoms and excision surgery is often curative. Multicentric forms of Castleman disease affect multiple groups of lymph nodes and are associated with varying degrees of systemic clinical symptoms. Multicentric Castleman disease is either idiopathic or associated with human herpesvirus 8 infection or POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes). Idiopathic multicentric Castleman disease is further divided into a variant associated with TAFRO syndrome (thrombocytopenia, anasarca, fever, reticulin fibrosis / renal dysfunction, and organomegaly), idiopathic plasmacytic lymphadenopathy type, and not otherwise specified variant. The treatment of multicentric forms of Castleman disease is complex and depends on etiological factors, including biological therapy, chemotherapy, or interleukin-6 activity inhibition. The aim of this educational text is to present the current view of Castleman disease and provide a comprehensive description of the morphological changes and clinical characteristics of the individual subtypes of Castleman disease.</p>","PeriodicalId":9861,"journal":{"name":"Ceskoslovenska patologie","volume":"62 1","pages":"17-34"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147716182","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Castleman-Like Lymphadenopathy in a Patient with Mixed Connective Tissue Disease: A Case Report and Review of the Literature.","authors":"Evelina Rogges, Sabrina Pelliccia, Gianluca Lopez, Roberta Soscia, Arianna Di Napoli","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Differentiating reactive lymphadenopathies in the context of autoimmune disease from Idiopathic Multicentric Castleman Disease (iMCD) poses a significant diagnostic challenge. Castleman-like histological features have been described in various autoimmune disorders, necessitating a strict and comprehensive integration of clinical and laboratory findings to reach the correct diagnosis. Although the Castleman Disease Collaborative Network (CDCN) consensus guidelines list several autoimmune conditions as exclusion criteria for an iMCD diagnosis, mixed connective tissue disease (MCTD) is not currently among them. We report the case of a  77-year-old woman presenting with fatigue, Raynaud's  phenomenon, sclerodactyly, mild generalized lymphadenopathy, in whom the lymph node biopsy revealed a Castleman-like histology. The absence of systemic inflammatory symptoms and the presence of high-titer anti-U1- RNP antibodies were, however, inconsistent with iMCD, favouring the diagnosis of a reactive Castleman-like lymphadenitis secondary to MCTD. This report highlights that Castleman-like lymphadenopathy can occur in MCTD, closely mimicking iMCD. Therefore, in patients with autoimmune diseases not explicitly listed among the CDCN exclusion criteria, comprehensive clinicopathological integration is essential to avoid misdiagnosis and potentially inappropriate antiIL-6-based therapy.</p>","PeriodicalId":9861,"journal":{"name":"Ceskoslovenska patologie","volume":"62 1","pages":"50-57"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147716190","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}