L. Mwizerwa , V.L. Cousin , T. Sologashvili , J.P. Vallée , F. Gumy-Pause , J. Wacker , A.L. Rougemont
{"title":"Intracardiac myoepithelial carcinoma with EWSR1::KLF15 fusion: A rare case of pediatric primary cardiac malignancy","authors":"L. Mwizerwa , V.L. Cousin , T. Sologashvili , J.P. Vallée , F. Gumy-Pause , J. Wacker , A.L. Rougemont","doi":"10.1016/j.carpath.2025.107728","DOIUrl":"10.1016/j.carpath.2025.107728","url":null,"abstract":"<div><div>Primary pediatric heart tumors are rare, and the vast majority are benign. Primary malignant cardiac tumors are exceedingly uncommon in this age group. Due to their rarity and overlapping imaging features with the more common benign tumors, the diagnosis of primary malignant cardiac tumors is particularly challenging. We report a case of a 12-year-old male with a 7-year history of a left ventricular mass that progressively increased in size, eventually requiring surgical resection. The histological diagnosis was a myoepithelial carcinoma with an <em>EWSR1::KLF15</em> fusion. Consistent with previously reported tumors harboring this fusion, a poorly differentiated small cell component was observed. Adjuvant chemotherapy comprising four cycles of ICpE and one cycle of IVE. At 11 months after completion of chemotherapy, there is no evidence of recurrent or metastatic disease.</div></div>","PeriodicalId":9451,"journal":{"name":"Cardiovascular Pathology","volume":"77 ","pages":"Article 107728"},"PeriodicalIF":2.3,"publicationDate":"2025-02-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143531287","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
L. Maximilian Buja , Michelle M. McDonald , Bihong Zhao , Navneet Narula , Jagat Narula , Rolf F. Barth
{"title":"Insights from autopsy-initiated pathological studies of the pathogenesis and clinical manifestations of atherosclerosis and ischemic heart disease: Part I. Atherosclerosis","authors":"L. Maximilian Buja , Michelle M. McDonald , Bihong Zhao , Navneet Narula , Jagat Narula , Rolf F. Barth","doi":"10.1016/j.carpath.2025.107726","DOIUrl":"10.1016/j.carpath.2025.107726","url":null,"abstract":"<div><h3>Context</h3><div>Ischemic heart disease (IHD) due to coronary atherosclerosis constitutes the leading cause of morbidity and mortality worldwide. This review was undertaken to document the historical basis for our contemporary understanding of atherosclerosis-based disease and to provide a rationale for continued support for autopsy-based research to make further progress in reducing the morbidity and mortality from atherosclerosis-related disease.</div></div><div><h3>Objectives</h3><div>To analyze the contributions of the autopsy-initiated pathological studies to complement and validate other lines of investigation in determining the pathology and pathogenesis of the leading worldwide cause of morbidity and mortality, namely, atherosclerosis and its major complications of coronary atherosclerosis, ischemic heart disease, coronary thrombosis, acute myocardial infarction, and sudden cardiac death.</div></div><div><h3>Data sources</h3><div>Systematic search on PubMed to gather relevant studies concerning autopsy studies and reviews of the pathology and pathogenesis of atherosclerosis, ischemic heart disease, coronary atherosclerosis, coronary thrombosis, myocardial infarction, and sudden cardiac death</div></div><div><h3>Conclusions</h3><div>Extensive published reports have confirmed the continuing importance of the autopsy as a powerful tool to understand the pathogenesis, clinical features, and therapeutic options for major diseases. This specifically has been shown by the analysis of atherosclerosis and its major manifestation of ischemic heart disease, as presented in this (Part I) and its companion (Part II) review. Autopsy-initiated pathological studies have documented the prevalence and natural history of atherosclerosis in different human populations in relationship to the prevalence of risk factors and established that the clinically silent phase of the disease begins in the first decades of life. Insights from these studies have been essential in developing and evaluating strategies for continued progress in preventing and controlling the disability and death associated with atherosclerotic heart disease.</div></div>","PeriodicalId":9451,"journal":{"name":"Cardiovascular Pathology","volume":"76 ","pages":"Article 107726"},"PeriodicalIF":2.3,"publicationDate":"2025-02-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143456995","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
L. Maximilian Buja , Michelle M. McDonald , Bihong Zhao , Navneet Narula , Jagat Narula , Rolf F. Barth
{"title":"Insights from autopsy-initiated pathological studies of the pathogenesis and clinical manifestations of atherosclerosis and ischemic heart disease: Part II. Ischemic heart disease","authors":"L. Maximilian Buja , Michelle M. McDonald , Bihong Zhao , Navneet Narula , Jagat Narula , Rolf F. Barth","doi":"10.1016/j.carpath.2025.107727","DOIUrl":"10.1016/j.carpath.2025.107727","url":null,"abstract":"<div><h3>Context</h3><div>Ischemic heart disease (IHD) due to coronary atherosclerosis constitutes the leading cause of morbidity and mortality worldwide. This review was undertaken to retrospectively analyze the lines of research that generated the evidence for our contemporary understanding of atherosclerosis-based coronary artery disease and to provide a rationale for continued support for autopsy-based research in order to make further progress in reduction of the morbidity and mortaility from IHD.</div></div><div><h3>Objectives</h3><div>To analyze the contributions of the autopsy to complement and validate other lines of investigation in determining the complex interactions between coronary artery alterations linked to the major manifestations of coronary atherosclerosis, namely, coronary thrombosis, acute myocardial infarction, and sudden cardiac death.</div></div><div><h3>Data Sources</h3><div>Systematic search on PubMed to gather relevant studies concerning autopsy studies and reviews of the pathology and pathogenesis of atherosclerosis, ischemic heart disease, coronary atherosclerosis, coronary thrombosis, myocardial infarction and sudden cardiac death.</div></div><div><h3>Conclusions</h3><div>An extensive search of the published literature has confirmed the continuing importance of the autopsy as a powerful tool to understand the pathogenesis, clinical features, and therapeutic options for the treatment of atherosclerosis and its major manifestation, ischemic heart disease. This has been described in the Part I companion of the present review. Autopsy-initiated studies have documented the prevalence and clinicopathological significance of atherosclerosis in different human populations and its relationship to risk factors. It has been shown that the clinically silent phase of ischemic heart disease (IHD) begins in the first decades of life. Pathological studies have clarified the complex relationship between coronary atherosclerosis, coronary thrombosis, and myocardial ischemic events. These studies also have elucidated the pathological basis of sudden cardiac death. Insights from these studies also have been important in developing and evaluating strategies for continued progress in reducing the morbidity and mortality attributed to atherosclerosis and IHD.</div></div>","PeriodicalId":9451,"journal":{"name":"Cardiovascular Pathology","volume":"76 ","pages":"Article 107727"},"PeriodicalIF":2.3,"publicationDate":"2025-02-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143432313","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Mitral Isthmus anatomy: Detailed examination and classification proposal","authors":"Buse Naz Çandır Gürses , Kader Yılar , Çağla Ergin , Özcan Gayretli","doi":"10.1016/j.carpath.2025.107723","DOIUrl":"10.1016/j.carpath.2025.107723","url":null,"abstract":"<div><h3>Aim</h3><div>The aim of this study was to investigate the anatomical features of the mitral isthmus (MI) line, the vessels located there and their localisation in the MI line.</div></div><div><h3>Methods</h3><div>MI length and wall thickness were measured in a total of 65 autopsied fresh hearts. The distances of the vessels at the level of MI to the left inferior pulmonary vein (LIPV), mitral annulus (MA), endocardium surface (ES) and lateral adipose tissue (LAT) were recorded.</div></div><div><h3>Results</h3><div>The mean linear length of MI and left atrial wall thickness were 49.6 ± 9.9 mm and 3.9 ± 1.2 mm, respectively. GCV and LCx are approximately 1 cm from MA, 4 cm from LIPV, 5-6 mm from ES and 7 mm from LAT. Great cardiac vein (GCV) was found to be located in the MI line in 100 %, left circumflex artery (LCx) in 60 %, and vein of Marshall (VOM) in 63.1 % of cases. Presence of only GCV was recorded as Type-1 (18.5 %), GCV and LCx as Type-2 (18.5 %), GCV and VOM as Type-3 (21.5 %) and presence of all three as Type-4 (41.5 %). LCx located below the GCV was recorded as Type-A (59 %), above the GCV as Type-B (25.6 %), and at the same level but with LCx on the endocardial surface as Type-C1 (12.8 %) and on the epicardial surface as Type-C2 (2.6 %).</div></div><div><h3>Conclusion</h3><div>This study proves that the anatomy of MI is far from standardised and helps to raise awareness of the vascular pattern that may be encountered prior to ablation intervention.</div></div>","PeriodicalId":9451,"journal":{"name":"Cardiovascular Pathology","volume":"76 ","pages":"Article 107723"},"PeriodicalIF":2.3,"publicationDate":"2025-01-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143073978","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Severi M Mattila , Hanna Latola , Lasse Pakanen , Jenni J Hekkanen , M Anette E Eskuri , Juha Vähätalo , Olavi H Ukkola , M Juhani Junttila , Heikki V Huikuri , Juha S Perkiömäki
{"title":"Seasonal variation of unexpected sudden cardiac death in northern Finland","authors":"Severi M Mattila , Hanna Latola , Lasse Pakanen , Jenni J Hekkanen , M Anette E Eskuri , Juha Vähätalo , Olavi H Ukkola , M Juhani Junttila , Heikki V Huikuri , Juha S Perkiömäki","doi":"10.1016/j.carpath.2025.107725","DOIUrl":"10.1016/j.carpath.2025.107725","url":null,"abstract":"<div><h3>Background</h3><div>Data on the occurrence of unexpected sudden cardiac death (SCD) in different seasons are limited.</div></div><div><h3>Methods</h3><div>All unexpected sudden death victims have to undergo medico-legal autopsy obligated by the Finnish law. Consecutive series of all unexpected autopsied SCD victims (n = 5,869) were prospectively collected from the geographically defined area in the Northern Finland during a twenty years period from 1998 to 2017. We evaluated the seasonal variation of SCD and its possible causes.</div></div><div><h3>Results</h3><div>Unexpected SCD occurred more frequently during the first quarter of a year (from January to March) than during the other quarters. The occurrence of SCD during the twenty years follow-up was 4.5 ± 0.24 during the first quarter of the year per month and 3.9 ± 0.12 during the other quarters of the year per month on average (SCDs during the quarter of a year per month on average per 100,000 inhabitants per year, p < 0.001). The weather was colder during the first quarter of the year (average temperature -9.1 degrees centigrade) than during the other quarters (average temperature +5.7 degrees centigrade). The subjects who experienced SCD during the first quarter of the year had more commonly severe (75 %-90 %) coronary artery stenosis, (p < 0.001).</div></div><div><h3>Conclusion</h3><div>Unexpected SCD occurred more commonly during the first quarter of a year (from January to March) than during the other quarters (from April to December). Cold weather with its physiological consequences and more severe coronary artery disease predisposing to ischemia may have contributed to the increased occurrence of SCD during the first quarter of the year.</div></div>","PeriodicalId":9451,"journal":{"name":"Cardiovascular Pathology","volume":"76 ","pages":"Article 107725"},"PeriodicalIF":2.3,"publicationDate":"2025-01-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143063838","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Patent foramen ovale: A variant of normal or a true congenital heart disease?","authors":"Stefania Rizzo, Monica De Gaspari, Cristina Basso, Chiara Fraccaro, Gaetano Thiene","doi":"10.1016/j.carpath.2025.107722","DOIUrl":"10.1016/j.carpath.2025.107722","url":null,"abstract":"<div><div>Foramen ovale plays a key role in foetal circulation, however it may remain patent after birth throughout the life. Its patency is so frequent in healthy people (27-35 %), such as to be considered a variant of normal. It is at risk of complications, like paradoxical embolism by right to left shunt with stroke, migraine, temporary blindness, as well as aneurysm, thrombosis and endocarditis of the fossa ovalis. There is no doubt that it should be considered a congenital heart disease at all effects. Invasive cardiology closure with umbrella and even with stiches is nowadays feasible and indicated in specific clinical scenarios. Further research is needed to determine whether cardiac echo-doppler screening for identifing affected patients as a primary prevention measure is advisable.</div></div>","PeriodicalId":9451,"journal":{"name":"Cardiovascular Pathology","volume":"76 ","pages":"Article 107722"},"PeriodicalIF":2.3,"publicationDate":"2025-01-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143037126","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Intimal sarcoma of the lower pulmonary vein diagnosed by Endobronchial Ultrasound-Guided Fine-Needle Aspiration (EBUS-FNA): A case report and comprehensive literature review","authors":"H. Elmas , I. Kholová , S. Meierling , L. Welker","doi":"10.1016/j.carpath.2025.107717","DOIUrl":"10.1016/j.carpath.2025.107717","url":null,"abstract":"<div><div>Intimal sarcoma of blood vessels is a rare, aggressive tumor originating from vascular endothelial cells. This report presents a 22-year-old male diagnosed with an intimal sarcoma of the lower pulmonary vein, detailing diagnosis, treatment, and prognosis information. Additionally, this report explores the application application of Endobronchial Ultrasound-Guided Fine-Needle Aspiration (EBUS-FNA) alongside with Rapid Remote Online Evaluation (ROLE) for identifying a mass-like lesion in the pulmonary vein. We emphasize the value of this technique for supporting ancillary studies like immunohistochemistry. The importance of accurate diagnostic methods and personalized treatment management to improve prognosis in rare cancers is highlighted by this thorough analysis.</div></div>","PeriodicalId":9451,"journal":{"name":"Cardiovascular Pathology","volume":"76 ","pages":"Article 107717"},"PeriodicalIF":2.3,"publicationDate":"2025-01-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143000885","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}