Journal of Pathology and Translational Medicine

Original Articles

Indirect pathological indicators for cardiac sarcoidosis on endomyocardial biopsy: Myung-Jin Cha, Jeong-Wook Seo, Seil Oh, Eun-Ah Park, Sang-Han Lee, Moon Young Kim, Jae-Young Park; J Pathol Transl Med. 2020;54(5):396-410. Published online July 29, 2020; DOI: https://doi.org/10.4132/jptm.2020.06.10

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Background
The definitive pathologic diagnosis of cardiac sarcoidosis requires observation of a granuloma in the myocardial tissue. It is common, however, to receive a “negative” report for a clinically probable case. We would like to advise pathologists and clinicians on how to interpret “negative” biopsies.
Methods
Our study samples were 27 endomyocardial biopsies from 25 patients, three cardiac transplantation and an autopsied heart with suspected cardiac sarcoidosis. Pathologic, radiologic, and clinical features were compared.
Results
The presence of micro-granulomas or increased histiocytic infiltration was always (6/6 or 100%) associated with fatty infiltration and confluent fibrosis, and they showed radiological features of sarcoidosis. Three of five cases (60%) with fatty change and confluent fibrosis were probable for cardiac sarcoidosis on radiology. When either confluent fibrosis or fatty change was present, one-third (3/9) were radiologically probable for cardiac sarcoidosis. We interpreted cases with micro-granuloma as positive for cardiac sarcoidosis (five of 25, 20%). Cases with both confluent fibrosis and fatty change were interpreted as probable for cardiac sarcoidosis (seven of 25, 28%). Another 13 cases, including eight cases with either confluent fibrosis or fatty change, were interpreted as low probability based on endomyocardial biopsy.
Conclusions
The presence of micro-granuloma could be an evidence for positive diagnosis of cardiac sarcoidosis. Presence of both confluent fibrosis and fatty change is necessary for probable cardiac sarcoidosis in the absence of granuloma. Either of confluent fibrosis or fatty change may be an indirect pathological evidence but they are interpreted as nonspecific findings.

Citations

Citations to this article as recorded by

Advances in cellular and tissue-based imaging techniques for sarcoid granulomas
Junwoo Kim, Girish Dwivedi, Berin A. Boughton, Ankur Sharma, Silvia Lee
American Journal of Physiology-Cell Physiology.2024; 326(1): C10. CrossRef
Lipomatous Metaplasia Is Associated With Ventricular Tachycardia Recurrence Following Ablation in Patients With Nonischemic Cardiomyopathy
Lingyu Xu, Mirmilad Khoshknab, Juwann Moss, Ronald D. Berger, Jonathan Chrispin, David Callans, Francis E. Marchlinski, Stefan L. Zimmerman, Yuchi Han, Natalia Trayanova, Benoit Desjardins, Saman Nazarian
JACC: Clinical Electrophysiology.2024; 10(6): 1135. CrossRef
Cardiac sarcoidosis: phenotypes, diagnosis, treatment, and prognosis
Jukka Lehtonen, Valtteri Uusitalo, Pauli Pöyhönen, Mikko I Mäyränpää, Markku Kupari
European Heart Journal.2023; 44(17): 1495. CrossRef
Cardiac sarcoidosis: a comprehensive review of risk factors, pathogenesis, diagnosis, clinical manifestations, and treatment strategies
Hussain Haider Shah, Syeda Alishah Zehra, Aliza Shahrukh, Radeyah Waseem, Tooba Hussain, Muhammad Sheheryar Hussain, Fareeha Batool, Muhammad Jaffer
Frontiers in Cardiovascular Medicine.2023;[Epub] CrossRef
Histology of Cardiac Sarcoidosis with Novel Considerations Arranged upon a Pathologic Basis
Shu Kato, Yasuhiro Sakai, Asako Okabe, Yoshiaki Kawashima, Kazuhiko Kuwahara, Kazuya Shiogama, Masato Abe, Hiroyasu Ito, Shin’ichiro Morimoto
Journal of Clinical Medicine.2022; 11(1): 251. CrossRef
Cardiac sarcoidosis: A multimodal approach to reach the diagnosis
Nicolas Piriou, Patrick Bruneval
International Journal of Cardiology.2021; 323: 264. CrossRef
Value of 3D mapping‐guided endomyocardial biopsy in cardiac sarcoidosis
Danielle M. Haanschoten, Ahmet Adiyaman, Nils A. ‘t Hart, Piet L. Jager, Arif Elvan
European Journal of Clinical Investigation.2021;[Epub] CrossRef
Cardiac Sarcoidosis: A Clinical Overview
Ana Carolina Alba, Shyla Gupta, Lakshmi Kugathasan, Andrew Ha, Alejandro Ochoa, Meyer Balter, Alvaro Sosa Liprandi, Maria Inés Sosa Liprandi
Current Problems in Cardiology.2021; 46(10): 100936. CrossRef

Quilty Lesions in the Endomyocardial Biopsies after Heart Transplantation: Haeyon Cho, Jin-Oh Choi, Eun-Seok Jeon, Jung-Sun Kim; J Pathol Transl Med. 2019;53(1):50-56. Published online December 26, 2018; DOI: https://doi.org/10.4132/jptm.2018.11.30

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Abstract PDF Supplementary Material

Background
The aim of this study was to investigate the clinical significance of Quilty lesions in endomyocardial biopsies (EMBs) of cardiac transplantation patients.
Methods
A total of 1190EMBs from 117 cardiac transplantation patients were evaluated histologically for Quilty lesions,acute cellular rejection, and antibody-mediated rejection. Cardiac allograft vasculopathy wasdiagnosed by computed tomography coronary angiography. Clinical information, including thepatients’ survival was retrieved by a review of medical records.
Results
Eighty-eight patients(75.2%) were diagnosed with Quilty lesions, which were significantly associated with acute cellularrejection, but not with acute cellular rejection ≥ 2R or antibody-mediated rejection. In patientsdiagnosed with both Quilty lesions and acute cellular rejection, the time-to-onset of Quilty lesionsfrom transplantation was longer than that of acute cellular rejections. We found a significant associationbetween Quilty lesions and cardiac allograft vasculopathy. No significant relationship wasfound between Quilty lesions and the patients’ survival.
Conclusions
Quilty lesion may be an indicator of previous acute cellular rejection rather than a predictor for future acute cellular rejection.

Citations

Citations to this article as recorded by

The human myocardium harbors a population of naive B-cells with a distinctive gene expression signature conserved across species
Kevin C. Bermea, Nicolas Kostelecky, Sylvie T. Rousseau, Chieh-Yu Lin, Luigi Adamo
Frontiers in Immunology.2022;[Epub] CrossRef
Examination of tracheal allografts after long-term survival in dogs
Tao Lu, Yiwei Huang, Yulei Qiao, Yongxing Zhang, Yu Liu
European Journal of Cardio-Thoracic Surgery.2021; 59(1): 155. CrossRef
Essentials in the diagnosis of postoperative myocardial lesions similar to or unrelated to rejection in heart transplant
Costel Dumitru, Ancuta Zazgyva, Adriana Habor, Ovidiu Cotoi, Horațiu Suciu, Carmen Cotrutz, Bogdan Grecu, Ileana Anca Sin
Revista Romana de Medicina de Laborator.2021; 29(3): 307. CrossRef
Clinical outcome of donor heart with prolonged cold ischemic time: A single‐center study
Fazal Shafiq, Yixuan Wang, Geng Li, Zongtao Liu, Fei Li, Ying Zhou, Li Xu, Xingjian Hu, Nianguo Dong
Journal of Cardiac Surgery.2020; 35(2): 397. CrossRef
The XVth Banff Conference on Allograft Pathology the Banff Workshop Heart Report: Improving the diagnostic yield from endomyocardial biopsies and Quilty effect revisited
Jean-Paul Duong Van Huyen, Marny Fedrigo, Gregory A. Fishbein, Ornella Leone, Desley Neil, Charles Marboe, Eliot Peyster, Jan von der Thüsen, Alexandre Loupy, Michael Mengel, Monica P. Revelo, Benjamin Adam, Patrick Bruneval, Annalisa Angelini, Dylan V. M
American Journal of Transplantation.2020; 20(12): 3308. CrossRef

The Significance of Ventricular Volume in the Evaluation of Secondary Cardiomyopathy at Autopsy.: Joo Young Na, Byung Woo Min, Yeong Hui Kim, Seung Hyun Chung, Young Jik Lee, Hyung Seok Kim, Jong Tae Park; Korean J Pathol. 2011;45(4):336-347.; DOI: https://doi.org/10.4132/KoreanJPathol.2011.45.4.336

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Abstract PDF: BACKGROUND
The weight, shape and consistency of the heart, and the thickness of the ventricular wall are used as parameters for evaluating postmortem heart and diagnosing cardiomyopathy at autopsy.
METHODS
The weight and volume of the ventricles and the thickness of the left ventricular wall of 58 hearts were measured and analyzed.
RESULTS
In the group of dilated hearts, the ventricular weight, ventricular volume, ventricular volume/ventricular weight, and left ventricular volume/right ventricular volume increased, whereas ventricular wall thickness decreased. In the group of hypertrophied hearts, the ventricular weight, ventricular volume, and thickness of the ventricular wall increased but ventricular volume/ventricular weight and left ventricular volume/right ventricular volume did not change significantly. In the group of undetermined hearts, it was later found that four of the cases should have been included in the dilated heart group and another two cases in the hypertrophied heart group.
CONCLUSIONS
In addition to conventional methods, the measuring ventricular volume is useful for evaluating a postmortem heart and may suggest postmortem differential diagnoses of dilated or hypertrophied forms of secondary cardiomyopathies.

Pathological Analysis of Post-Transplantation Endomyocardial Biopsies.: Jaegul Chung, Soonae Oak, Gheeyoung Choe, Gyungyub Gong, Jooryung Huh, Eunsil Yu, Inchul Lee, Meong Gun Song, Kwang Hyun Sohn, Jae Joong Kim, Jong Goo Lee; Korean J Pathol. 1995;29(4):431-441.

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Abstract PDF: Heart transplantation was first performed in 1967. It is now regarded as a well-established treatment modality for end-stage cardiac diseases. Once the transplantation is performed, endomyocardial biopsy(EMB) is the examination of choice in monitoring the transplanted heart. We analyzed the pathological findings of follow-up EMB of 6 heart transplant patients. All patients have been suffered from severe heart failure. Four patients were adult male and two were adult females. All the hearts, except for one, displayed characteristic features of dilated cardiomyopathy. The remaining heart was diagnosed as having giant cell myocarditis. Post-transplantion EMBs were performed according to the protocol and standard cardiac biopsy grading of ISHT (1990). The standards were applied for grading of cellular rejection. In five patients, there were one or two episodes of biopsy proven acute rejection, grade II or IIIA without any clinical symptoms of rejection. Immediate "pulse therapy" was performed and follow-up biopsies were done. All episodes of rejection were cleared in subsequent biopsies. All patients are doing well without evidence of cardiac problem. The postoperative monitoring of acute rejection is critical since clinical signs of rejection are usually absent. At present, EMB is regarded as the most reliable method for diagnosis and grading of acute rejection and is an efficient guide to the monitoring of the cardiac recipients. Our experience of post-transplantation EMB corresponds with previously published reports.

Immunohistochemical Study of IL-4, IL-6, and TNF Expression in Cardiac Myxoma: Emphasis on Constitutional Symptoms of the Myxoma Patients.: Min Sun Cho, Soo Yeon Cho, Mi Jung Kim, Sung Sook Kim, Jeong Wook Seo, Woon Sup Han; Korean J Pathol. 1995;29(5):563-571.

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Abstract: It is well documented that cardiac myxomas are associated with immunologic features that can simulate systemic autoimmune diseases. Recently, it was reported that cardiac myxomas produce IL-6 constitutively, which could partly explain the immunologic features observed in these patients. However, only a few investigators have studied cytokines in regards to symptoms they may cause in patients with cardiac myxoma. Also there is very little information in the literature on the immunohistochmical localization of IL-6. We performed immunobistochemical stains for IL-4, TNF, and IL-6 on paraffm embbeded tissue of cardiac myxoma tissue. A bioassay of IL-6 activity in patient's serum and in cultured cells from fresh myxoma tissue was performed to ascertain the role of these cytokines in myxomas. In this study, we demonstrated inununohistochemically that there was a local overproduction of IL-4, TNF, and IL-6 in cytoplasm of the tumor cells in about half cases. Bioassays of the serum and cultured tumor cells revealed elevated IL-6 activities. Also these findings correlate to production of patient's constitutional symptoms with statistical significance (P<0.05). In conclusion, these results are of considerable importance in understanding the role of IL-4, TNF, and IL-6 in cardiac myxoma patient with constitutional symptoms, and have an impact on strategies for diagnosis and therapy of cardiac myxoma.

Case Report

Cardiac Fibroma: A surgically excised case.: Ho Jung Lee, Gyung Ub Gong, Jay Won Lee, Jae Gon Go, In Chul Lee; Korean J Pathol. 1996;30(6):544-547.

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Abstract PDF: Primary cardiac tumors in infancy and childhood are rare, with fibromas being the second most common tumor after rhabdomyomas. Although cardiac fibromas are characteristically benign intramural tumors, they may exhibit exhibit expansile growth resulting in obstruction, valvular dysfunction, as well as other problems so early diagnosis and successful surgical excision are important. We report a case of cardiac fibroma in a 2 month-old male infant. He presented with generalized cyanosis from birth. Echocardiography showed oval round large mass filing the right atrium and ventricle which infiltrated into the lateral wall of the ventricle. Partial excision of the tumor was done after another echocardiogram showed a pericardial effusion and restriction of blood flow to the right ventricle due to the tumor. The resected tumor was ovoid, gray-tan, slightly firm and measuring 5x3x2.5 cm. Histologically, the tumor was composed of spindle-shaped fibroblasts and hyalinized fibrous tissue interdigitating with the surrounding myocardium.

Original Articles

Pathologic Analysis of Endomyocardial Biopsies in Heart Transplantation.: Mee Hye Oh, Jeong Wook Seo, Kook Yang Park, Young Tak Lee, Yoon Seop Jeong, Suk Keun Hong, Joon Ryang Rho, Byung Hee Oh, Sung Sook Kim; Korean J Pathol. 1998;32(2):104-114.

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Abstract PDF: Endomyocardial biopsy (EMB) is a valuable diagnostic procedure for the surveillance of cardiac allograft rejection. Interpretation of individual cases is still problematic due to variations of findings for grading of rejection and other associated lesions. We reevaluated an experience on endomyocardial biopsies to develop better diagnostic criteria for rejection and other complications. Immunohistochemical studies against cytokines were performed to assess the usefulness of the method for the diagnosis or researches. A total of 249 EMBs taken from 33 cardiac allograft recipients were reviewed. There were 25 males and 8 females. Dilated cardiomyopathy was present (24 cases) and valvular heart disease (4 cases), restrictive cardiomyopathy (3 cases) were also common conditions. We applied the grading system of the International Society for Heart Transplantation (ISHT) for the assessment of acute cellular rejection. Grades of 0, 1A, 1B, 2, 3A and 3B were 39.0%, 28.1%, 11.2%, 11.5%, 12.4% and 1.6% respectively, but 3.2% were inadequate. Thirty five episodes of grade 3A or 3B were present in 17 patients. The response to therapy was assessed using a next follow up biopsy, which revealed resolving or resolved rejection in 85% of patients. The intensity of immunohistochemical stains for IL-6 and TNF-alpha was increased in proportion to the histologic grade but Quilty lesion and cardiomyopathy also showed a positive reaction. The other pathologic findings were ischemic change, previous biopsy site, interstitial edema and fibrosis, and Quilty lesion. These findings showed usefulness of endomyocardial biopsy not only for the evaluation of cardiac allograft rejection but also for the diagnosis of associated cardiac lesions. Immunohistochemical study of the cytokines was related to the degree of inflammation rather than degree of rejection.

Image Standardization and Determination of Gray Level Threshold in the Assessment of the Myocardial Fibrosis by the Computerized Image Analysis.: Nam Young Lee, Young Sik Park, Jin Haeng Chung, Jeong Wook Seo; Korean J Pathol. 1998;32(7):494-503.

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Abstract: The computerized image analysis is a useful tool for the quantitative assessment of histopathologic findings. In contrast to the usual microscopic examination by pathologists, the computerization should be accompanied with the standardization process of the image. We developed an algorithm to standardize images and to determine the optimal gray level threshold, using a myocardial fibrosis model. Sirius red staining was more convenient for the image analysis than Masson's trichrome staining because of a better contrast with the surrounding structures. To get an optimal measurement, light intensity was standardized at each of the fibrosis, myocardium and background. In this study, the most promising method to determine the degree of fibrosis was that of revising the background without tissue to a gray level of 200, obtaining a green component of the color image, revising the myocardial fiber to 163, and defining a partial ratio as fibrosis index when the gray level threshold was 120. These threshold levels and parameters were determined after drawing the binarization index curves according to the change of the gray level threshold and by the morphological examination of the actual binarization figures overlaid to the original color image. Through these processes we could get a consistent result on the myocardial fibrosis and we expect a similar principle applies when we analyze color images in the histopathologic quantitation by computerized image analysis.

Case Reports

Mesothelial/Monocytic Incidental Cardiac Excrescences, So-called "Cardiac MICE": A case report .: Nahye Myong, Min Chul Lee, Myung Yong Lee; Korean J Pathol. 1999;33(12):1199-1202.

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Abstract PDF: A rare case of mesothelial/monocytic incidental cardiac excrescences (cardiac MICE) is described in the aspect of pathological interest. This cardiac lesion is pathologically characterized by exuberant proliferation of mixed mesothelia and monocytes and might be misdiagnosed as metastatic carcinoma, rhabdomyosarcoma, and histiocytoid hemangioma, if the disease is not in the minds of pathologists. The reactive nodular hyperplasia due to irritation to mesothelia by various causes is a most prevailing pathogenetic mechanism. About 20 cases have been reported in the worldwide literature. A 67-year-old female patient presented with cough and dyspnea for 2 months, without any history of previous cardiac operation. 2D echocardiography of the heart revealed moderate amount of pericardial effusion with posterior wall thickening. Under the impression of metastatic malignancy, pericardiostomy was performed. Grossly, the tissue was dark hemorrhagic and friable and the histologic sections revealed the solid tumor-like proliferation of round to polygonal histiocytic cells admixed with small cuboidal mesothelial cells which formed strips and tubular arrays. They were found within the fibrinous network and there were scattered empty vacuolar spaces. Immunohistochemical staining confirmed their biphasic nature with the CD68 positivity of the histiocytes and the cytokeratin positivity of the cuboidal cells. Factor VIII positivity was not detected in any cell components. The lesion was considered the monocytic and mesothelial proliferation of reactive nature, so-called cardiac MICE in the pericardial cavity. We report a typical case of so-called MICE first in the Korean literature.

Cardiac Sarcoidosis Treated by Cardiac Transplantation: A Case Report.: Jaejung Jang, Kwangseon Min, Gyeong cheon Jung, Jaejung Kim, Inchul Lee; Korean J Pathol. 2001;35(1):71-75.

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Abstract PDF: Sarcoidosis, in general, has a low mortality rate. But cardiac sarcoidosis (CS) is a serious condition which may lead to death. Here, we report a rare case of CS that was treated by heart transplantation. A 47-year-old male had occasional syncopes and atypical chest pain. Ventricular tachycardia with right bundle branch block was noted by electrocardiogram. Multiple fixed myocardial perfusion defects in the interventricular septum and both the inferior-posterior ventricular walls were observed by thallium scan. Coronary angiography was unremarkable. Neither perihilar nor mediastinal lymphadenopathy was noted. The patient also suffered three times from tonic-clonic generalized seizures in 3 years, but no neurologic abnormalities were detected. The explanted heart displayed multiple white patches on the endomyocardial surface, measuring up to 8x7 cm. On microscopic examination, the lesion consisted of multiple well-formed and confluent granulomas with numerous scattered multinucleated giant cells, CD68-positive epithelioid histiocytes, and T-lymphocytes. Neither microorganisms nor foreign material was identified on special stain and culture study. It has been six months since the heart transplant, and the patient has been doing well.

Original Article

Relationships between Types of Proximal Gastric Mucosa and Clinicopathological Features.: Jong Sil Lee, Hwal Woong Kim, Jeong Hee Lee, Hee Shang Youn, Woon Tae Jung, Gyung Hyuck Ko; Korean J Pathol. 2003;37(1):15-18.

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Abstract PDF: BACKGROUND
It has been believed that there is a pure mucus-secreting cardiac mucosa (CM), about 2 cm in length, below the gastroesophageal junction. However, recent reports suggest that CM might not be located at the most proximal portion of the stomach. The purpose of this study is to investigate the relationships between the types of proximal gastric mucosa and patients' age, sex, their condition regarding the Helicobacter pylori infection, and severity of chronic gastritis.
METHODS
Two pieces of mucosal tissue from the most proximal portion of the stomach and the antrum of 44 pediatric and 85 adult patients were examined using a light microscope. A rapid urease test was performed on the other antral specimen from each patient.
RESULTS
In 46 (90.2%) out of 51 patients with aged 30 or under, only the pure acid-secreting oxyntic mucosa (OM) was present at the most proximal portion of the stomach. The cardiac or mixed oxyntocardiac mucosa (OCM) increased in prevalence with age. The CM or OCM was found more frequently in patients with H. pylori infection or severe gastritis than in those without H. pylori infection or those with mild gastritis. However, there were no statistically significant differences within the same age groups.
CONCLUSIONS
Although the OCM is sometimes present at the most proximal portion of the stomach, the CM is absent or rare in individuals under the age of 30. The OCM or CM increases in prevalence with age. There are no direct relationships between the type of proximal gastric mucosa and a patient's gender, his/her condition regarding the H. pylori infection, and severity of chronic gastritis.

Case Reports

Pulmonary Nocardiosis Diagnosed by Fine Needle Aspiration: A Case Report.: Hyunee Yim, Kwang Hwa Park; Korean J Cytopathol. 1995;6(2):169-173.

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Abstract PDF: Nocardia, aerobic members of the order of Actinomycetaceae, produces infections in human lung. Nocardial infection is associated with underlying diseases of immuno-suppression or treatment with corticosteroid. It is difficult to detect Nocardia by sputum examination or histologic sections and it has rarely been diagnosed by fine needle aspiration of the lung. We describe a case of pulmonary nocardiosis in a 72 year-old man. diagnosed by fine needle aspiration, which was confirmed by culture of aspirates. The aspirates showed neutrophil-predominant inflammatory cells with microoganisms demonstrated by Gomori methenamine silver and Gram stain. The organisms had characteristic long branching filamentous strucutres. The lesions on chest x-ray were in resolution with antimicrobial therapy.

Sudden Death from Cardiac Sarcoidosis: A Case Report.: Soo Kyoung Lee, Sun Zoo Kim, Yoon Seup Kum, Tae In Park, Sang Han Lee, Jong Min Chae, Jung Sik Kwak; Korean J Pathol. 2003;37(5):358-361.

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Abstract PDF: Sarcoidosis is a systemic granulomatous inflammation with an unknown cause. The commonly involved sites are the lymph nodes, lungs, skin, eyes, and heart. Although cardiac involvement in sarcoidosis is rarely detected clinically, it is reported in 20-50% of autopsied sarcoidosis patients. Cardiac involvement is one of the most severe conditions of sarcoidosis and may cause sudden death. We report a case of a sudden death due to a massive cardiac sarcoidosis in a 43-year-old man. The microscopic examination revealed an extensive noncaseating granulomatous inflammation in the mediastinal lymph nodes and the heart with no evidence of myocyte necrosis. A special stain and molecular study excluded the possibility of other causes such as fungi or mycobacterium. The authors concluded that the cause of death was attributed to arrhythmia due to a cardiac sarcoidosis with massive involvement of the conduction system.

Right Atrial Myxoma Showing Granulomatous Lesion with Pulmonary Infart: A case report.: Kun Chang Song, Soon Hee Jung, Dong Hwan Shin; Korean J Pathol. 1993;27(5):501-503.

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Abstract PDF: Cardiac myxoma is the most common primary tumor of the heart. The tumor contains a variety of cell types that are thought to arise from a focus of primitive pluripotential mesenchymal cells in the area of the fossa ovalis. Throughout the myxoid stroma, there are variable amounts of reticular fivers, collagen, elastic fibers and smooth muscle cells. A 38-year-old female had right atrial myxoma with multiple pulmonary infarcts. In this case, we experienced an unusual degenerative change in the tumor of granulomatous lesion consisting of hemosiderin pigments, foreign body giant cells and peculiar, spheroid, semilunar or bamboo-shaped degenerated elastic fibers. Microscopically it resembles Gamna-Gandy nodule seen in the spleen of chronic passive congestion.

Cardiac Fibroma of the Ventricular Septum: A case report.: Byung Tae Park, Se Jin Jang, Moon Hyang Park, Jung Dal Lee, Hyo Jin Lee; Korean J Pathol. 1991;25(1):37-41.

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Abstract PDF: This is an autopsy case of a 6 month old girl who suddenly died of respiratory distress during sleep. She had suffered from mild but frequent episodes of common cold and was treated for eczema for several days. At autopsy, the heart was enlarged and weighed 100 gm. A firm and gray-white tumor, measuring 4.5 x 3.8 x 2.8 cm, was located in the interventricular septum and encroached upon the wall of left ventricle. The mass was well demarcated but was not encapsulated. Neither necrosis nor calcification was present. Microscopically the tumor was composed of haphazardly arranged bundles of collagen fibers and fibroblasts. Myocardial cells are intermingled with the fibroblasts at the margin of the tumor. Massive edema of the lung and congestion of the liver and spleen were pronounced.

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