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1.
J Cardiothorac Surg ; 19(1): 388, 2024 Jun 26.
Article in English | MEDLINE | ID: mdl-38926775

ABSTRACT

BACKGROUND: Cardiac myxomas are the most common type of primary cardiac tumors in adults, but they can have variable features that make them difficult to diagnose. We report two cases of atrial myxoma with calcification or ossification, which are rare pathological subgroups of myxoma. CASE PRESENTATION: A 47-year-old woman and a 35-year-old man presented to our hospital with different symptoms. Both patients had a history of chronic diseases. Transthoracic and transesophageal echocardiography revealed a mass in the left or right atrium, respectively, with strong echogenicity and echogenic shadows. The masses were suspected to be malignant tumors with calcification or ossification. Contrast transthoracic echocardiography(cTEE) showed low blood supply within the lesions. The patients underwent surgical resection of the atrial mass, and the pathology confirmed myxoma with partial ossification or massive calcification. CONCLUSION: We report two rare cases of atrial myxoma with calcification or ossification and analyze their ultrasonographic features. Transthoracic echocardiography and cTEE can provide valuable information for the diagnosis and management of such mass. However, distinguishing calcification and ossification in myxoma from calcification in malignant tumors is challenging. More studies are needed to understand the pathogenesis and imaging characteristics of these myxoma variants.


Subject(s)
Calcinosis , Heart Atria , Heart Neoplasms , Myxoma , Ossification, Heterotopic , Humans , Myxoma/diagnosis , Myxoma/surgery , Myxoma/complications , Heart Neoplasms/diagnosis , Heart Neoplasms/surgery , Heart Neoplasms/complications , Heart Neoplasms/pathology , Heart Neoplasms/diagnostic imaging , Male , Middle Aged , Calcinosis/diagnostic imaging , Calcinosis/diagnosis , Calcinosis/surgery , Heart Atria/pathology , Heart Atria/diagnostic imaging , Female , Adult , Ossification, Heterotopic/diagnosis , Ossification, Heterotopic/complications , Ossification, Heterotopic/surgery , Echocardiography , Echocardiography, Transesophageal
2.
Methodist Debakey Cardiovasc J ; 20(1): 40-44, 2024.
Article in English | MEDLINE | ID: mdl-38855040

ABSTRACT

Transcatheter extraction of an intracardiac mass is a newer approach that may lead to nonsurgical treatment of complex cardiac masses. We present a case in which thrombectomy devices were combined to extract a right atrial mass, which highlights new frontiers in the treatment of complex transcatheter mass extraction. The combined use of two transcatheter thrombectomy devices (Kong and Godzilla) may provide a powerful addition to the existing armamentarium.


Subject(s)
Cardiac Catheterization , Heart Neoplasms , Thrombectomy , Humans , Thrombectomy/instrumentation , Treatment Outcome , Heart Neoplasms/surgery , Heart Neoplasms/diagnostic imaging , Cardiac Catheterization/instrumentation , Equipment Design , Male , Female , Heart Atria/surgery , Heart Atria/diagnostic imaging , Cardiac Catheters , Echocardiography, Transesophageal
4.
BMC Cardiovasc Disord ; 24(1): 307, 2024 Jun 17.
Article in English | MEDLINE | ID: mdl-38886700

ABSTRACT

BACKGROUND: Carney syndrome is an uncommon autosomal disorder closely linked to mutations in the PRKAR1A gene. Skin lesions are the most pronounced feature of Carney syndrome, affecting over 80% of individuals with this condition. This syndrome is characterized by a triad of myxomas, skin pigmentation, and endocrine hyperfunction, featuring multiple endocrine neoplasms with skin and cardiac involvement. Dilated cardiomyopathy, a primary cardiomyopathy, is defined as the dilation and impaired systolic function of the left or both ventricles. Its clinical presentation varies from being asymptomatic to heart failure or sudden cardiac death, making it a leading global cause of heart failure. Currently, Dilated cardiomyopathy has an estimated prevalence of 1/2500-1/250 individuals, predominantly affecting those aged 30-40 years, with a male-to-female ratio of 3:1. This case report describes a heart failure patient with cardiac myxoma caused by Carney syndrome combined with dilated cardiomyopathy. The patient was successfully treated for heart failure by heart transplantation. CASE PRESENTATION: Herein, we report a case of heart failure due to Carney syndrome that resulted in cardiac myxoma combined with dilated cardiomyopathy. A 35-year-old male was admitted to the hospital three years ago because of sudden chest tightness and shortness of breath. Echocardiography indicated myxoma, and a combination of genetic screening and physical examination confirmed Carney syndrome with cardiac myxoma. Following symptomatic management, he was discharged. Surgical interventions were not considered at the time. However, the patient's chest tightness and shortness of breath symptoms worsened, and he returned to the hospital. A New York Heart Association grade IV heart function was confirmed, and echocardiography indicated the presence of dilated cardiomyopathy accompanied by cardiac myxoma. Ultimately, the patient's heart failure was successfully treated with heart transplantation. CONCLUSIONS: Cardiac myxoma caused by Carney syndrome combined with heart failure caused by dilated cardiomyopathy can be resolved by heart transplantation.


Subject(s)
Cardiomyopathy, Dilated , Carney Complex , Heart Failure , Heart Neoplasms , Heart Transplantation , Myxoma , Humans , Cardiomyopathy, Dilated/surgery , Cardiomyopathy, Dilated/etiology , Cardiomyopathy, Dilated/diagnosis , Cardiomyopathy, Dilated/diagnostic imaging , Male , Carney Complex/genetics , Carney Complex/diagnosis , Carney Complex/surgery , Carney Complex/complications , Adult , Myxoma/complications , Myxoma/surgery , Myxoma/diagnostic imaging , Myxoma/diagnosis , Myxoma/genetics , Heart Failure/etiology , Heart Failure/diagnosis , Heart Failure/surgery , Heart Neoplasms/surgery , Heart Neoplasms/complications , Heart Neoplasms/diagnostic imaging , Heart Neoplasms/diagnosis , Heart Neoplasms/genetics , Treatment Outcome , Cyclic AMP-Dependent Protein Kinase RIalpha Subunit/genetics
5.
Am J Case Rep ; 25: e943568, 2024 Jun 23.
Article in English | MEDLINE | ID: mdl-38909277

ABSTRACT

BACKGROUND Papillary fibroelastoma is the most common type of benign primary cardiac tumor and is usually asymptomatic. However, tumor fragments or surface thrombus can embolize and cause transient ischemic attacks, strokes, or myocardial infarction. This report describes a 76-year-old woman who presented with dysarthria and right-sided weakness due to a stroke associated with a left atrial papillary fibroelastoma. CASE REPORT A 76-year-old woman visited the Emergency Department because she had right-sided weakness and dysarthria from 12 h ago. Brain magnetic resonance image was done at the Emergency Department, showing multiple small embolic, acute infarction in left basal ganglia and fronto-temporo-parietal lobes. Transthoracic and transesophageal echocardiogram showed a hypermobile echogenic mass (0.8×1.5 cm) with villous surface on the orifice of left atrial appendage. Twenty-four-hour Holter monitoring was performed to evaluate the cause of cerebral infarction, and there was no paroxysmal atrial fibrillation. Thoracic computed tomography angiography also showed a sea anemone-shaped mass around the left atrial appendage. Cardiac tumor excision was done via a lower partial sternotomy. Histopathologic analysis showed multiple delicate fronds, and the avascular fibroelastic cores were lined by a single layer of CD31-positive endothelial cells. Histopathologic findings were consistent with papillary fibroelastoma. The patient was discharged without any other complications on day 30 of hospitalization. CONCLUSIONS This case highlights the importance of cardiac imaging in patients with acute stroke, including transthoracic and transesophageal echocardiography, which can show the typical imaging features of papillary fibroelastoma and other intracardiac sources of embolus.


Subject(s)
Cardiac Papillary Fibroelastoma , Stroke , Humans , Female , Aged , Stroke/etiology , Heart Neoplasms/complications , Heart Neoplasms/diagnostic imaging , Heart Neoplasms/surgery , Heart Atria , Echocardiography, Transesophageal
6.
Microvasc Res ; 154: 104697, 2024 Jul.
Article in English | MEDLINE | ID: mdl-38801942

ABSTRACT

Cardiac myxoma is the most common primary cardiac tumor in adults. The histogenesis and cellular composition of myxoma are still unclear. This study aims to reveal the role of myxoma cell components and their gene expression in tumor development. We obtained single living cells by enzymatic digestion of tissues from 4 cases of surgically resected cardiac myxoma. Of course, there was 1 case of glandular myxoma and 3 cases of nonglandular myxoma. Then, 10× single-cell sequencing was performed. We identified 12 types and 11 types of cell populations in glandular myxoma and nonglandular myxoma, respectively. Heterogeneous epithelial cells are the main components of glandular myxoma. The similarities and differences in T cells in both glandular and nonglandular myxoma were analyzed by KEGG and GO. The most important finding was that there was active communication between T cells and epithelial cells. These results clarify the possible tissue occurrence and heterogeneity of cardiac myxoma and provide a theoretical basis and guidance for clinical diagnosis and treatment.


Subject(s)
Heart Neoplasms , Myxoma , Single-Cell Analysis , Humans , Heart Neoplasms/pathology , Heart Neoplasms/genetics , Heart Neoplasms/surgery , Heart Neoplasms/metabolism , Myxoma/pathology , Myxoma/genetics , Myxoma/surgery , Myxoma/metabolism , Female , Male , Middle Aged , Epithelial Cells/pathology , Epithelial Cells/metabolism , Biomarkers, Tumor/genetics , Biomarkers, Tumor/metabolism , T-Lymphocytes/pathology , T-Lymphocytes/metabolism , Aged , Adult , Cell Communication , Gene Expression Regulation, Neoplastic , Transcriptome , Phenotype
7.
Prenat Diagn ; 44(6-7): 876-878, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38752660

ABSTRACT

Fetal pericardial teratomas are rare. They present with pericardial effusion and hydrops. The definitive management is postnatal resection of the tumor. The exact antenatal management is not known due to its rarity. We present a case of fetal pericardial teratoma with pericardial tamponade. Pericardiocentesis performed at 31 weeks significantly relieved the venous compression, leading to resolution of hydrops and prolonging the gestational age for the definitive management.


Subject(s)
Heart Neoplasms , Pericardiocentesis , Teratoma , Humans , Teratoma/surgery , Teratoma/complications , Teratoma/diagnosis , Teratoma/diagnostic imaging , Pericardiocentesis/methods , Female , Heart Neoplasms/complications , Heart Neoplasms/surgery , Heart Neoplasms/diagnostic imaging , Heart Neoplasms/diagnosis , Pregnancy , Adult , Ultrasonography, Prenatal , Pericardial Effusion/surgery , Pericardial Effusion/diagnostic imaging , Pericardial Effusion/etiology , Cardiac Tamponade/etiology , Cardiac Tamponade/surgery , Cardiac Tamponade/diagnosis , Hydrops Fetalis/etiology , Hydrops Fetalis/diagnosis , Hydrops Fetalis/surgery , Fetal Diseases/surgery
8.
J Cardiothorac Surg ; 19(1): 305, 2024 May 30.
Article in English | MEDLINE | ID: mdl-38816838

ABSTRACT

BACKGROUND: Cardiac hemangiomas are very uncommon benign primary tumors. They are usually located preferentially in the right atrium and their location in the interatrial septum is extremely rare. CASE PRESENTATION: We report the case of a 41-year-old patient who was admitted due to a stroke. The transthoracic echocardiogram revealed a large mass in the right atrium adhered to the interatrial septum. Suspecting an atrial myxoma, surgical intervention was performed confirming that the mass extended within the thickness of the interatrial septum, protruding into the right atrial cavity. The histologic report confirmed a hemangioma. CONCLUSIONS: Cardiac hemangiomas are rare primary tumors and are usually misdiagnosed as other cardiac tumors. Histopathological examination is essential for a definitive diagnosis.


Subject(s)
Atrial Septum , Echocardiography , Heart Neoplasms , Hemangioma , Humans , Heart Neoplasms/surgery , Heart Neoplasms/diagnosis , Heart Neoplasms/diagnostic imaging , Heart Neoplasms/pathology , Adult , Hemangioma/diagnosis , Hemangioma/surgery , Hemangioma/diagnostic imaging , Atrial Septum/diagnostic imaging , Atrial Septum/surgery , Atrial Septum/pathology , Male , Diagnosis, Differential , Heart Atria/diagnostic imaging , Heart Atria/pathology , Female
9.
Kyobu Geka ; 77(5): 369-372, 2024 May.
Article in Japanese | MEDLINE | ID: mdl-38720607

ABSTRACT

An 81-year-old man underwent total arch replacement for thoracic aortic aneurysm 8 years ago and catheter ablation for paroxysmal atrial fibrillation 1 year ago. Transthoracic echocardiography revealed a mass in the right atrium, and the patient was admitted for close examination and treatment. Transesophageal echocardiography revealed a 23×17 mm large well-defined mass above the cavotricuspid isthmus. Two venous drainage cannulas were inserted directly to the superior vena cava and to the inferior vena cava via the right femoral vein, in order to avoid the direct contact with the right atrium prior to institution of cardiopulmonary bypass. The right atrial tumor was found attached to the cavotricuspid isthmus, and was resected together with the right atrial wall. Pathological examination showed myxomatous tissue. Postoperative course was uneventful. He was discharged 23 days after the operation.


Subject(s)
Catheter Ablation , Heart Atria , Heart Neoplasms , Myxoma , Humans , Myxoma/surgery , Myxoma/diagnostic imaging , Male , Aged, 80 and over , Heart Neoplasms/surgery , Heart Neoplasms/diagnostic imaging , Heart Atria/surgery
10.
Zhonghua Yi Xue Za Zhi ; 104(18): 1628-1630, 2024 May 14.
Article in Chinese | MEDLINE | ID: mdl-38742351

ABSTRACT

A total of 25 patients with right cardiac system tumors in the Department of Cardiac Surgery, Beijing Anzhen Hospital from January 2012 to October 2022 were retrospectively included in the study. The preoperative data, and information of surgical treatment and perioperative management on these patients were analyzed and summarized. One patient developed pulmonary embolism and died before surgery, and the other 24 patients (16 males and 8 females) received surgical treatment, with an average age of (44.7±10.2) years (24-74 years). Nine patients were diagnosed with malignant tumors. Among the 24 patients who received surgical treatment, two patients died during the perioperative period, in-situ tumor recurrence was seen in three patients within about 1 year after surgery (two patients died without surgery, and one patient died 3 months after surgery), two patients had distant metastasis, and 17 patients had a good prognosis. Right cardiac system tumors are rare, with a high malignant rate, and the clinical manifestations vary greatly. Active surgical intervention is found to be effective, and the prognosis is closely related to the pathological type and extent of tumor invasion.


Subject(s)
Heart Neoplasms , Humans , Middle Aged , Male , Adult , Female , Heart Neoplasms/surgery , Retrospective Studies , Aged , Prognosis , Neoplasm Recurrence, Local , Young Adult
11.
BMC Anesthesiol ; 24(1): 159, 2024 Apr 25.
Article in English | MEDLINE | ID: mdl-38664657

ABSTRACT

Serratus posterior intercostal plane block (SPSIPB) is a novel periparavertebral block. It provides anterolateral posterior chest wall analgesia. It is an interfascial plane block, performed under ultrasound guidance, and the visualization of landmarks is easy. It is performed deep into the serratus posterior superior muscle at the level of the third rib. Until now, there have been case reports about the usage of single-shot SPSIPB, but there are no reports about the usage of the block catheterization technique of SPSIPB. Continuous infusion from a catheter of interfascial plane blocks is important for postoperative analgesia management after painful surgeries such as thoracic and cardiac surgeries. Thus, we performed SPSIPB catheterization in a patient who underwent right atrial mass excision with minimally invasive thoracotomy surgery. Here, we present our successful analgesic experience with continuous SPSIPB in this case report.


Subject(s)
Heart Atria , Nerve Block , Pain, Postoperative , Female , Humans , Middle Aged , Heart Atria/surgery , Heart Neoplasms/surgery , Intercostal Nerves , Nerve Block/methods , Pain, Postoperative/drug therapy , Pain, Postoperative/prevention & control , Thoracotomy/methods , Ultrasonography, Interventional/methods
12.
Am J Case Rep ; 25: e942864, 2024 Apr 23.
Article in English | MEDLINE | ID: mdl-38650318

ABSTRACT

BACKGROUND We present a case of metachronous cardiac and intramuscular metastases in a patient with a known history of radical nephroureterectomy for upper-tract urothelial carcinoma (UTUC). CASE REPORT A 58-year-old man had a history of metachronous renal pelvis urothelial carcinoma with prior left radical nephroureterectomy. He was also diagnosed with malignancy-associated deep vein thrombosis (DVT) and was on rivaroxaban. He presented at an oncology follow-up consult with shortness of breath and right scapular lump. CT scan revealed a soft-tissue mass at the surgical bed suspicious for local recurrence, as well as intracardiac hypodensities and intramuscular nodules in the right latissimus dorsi and right adductor muscles. The intracardiac hypodensities were located in the left atrial appendage and inter-atrial septum. Given that the patient had a history of DVT and in a pro-thrombotic state, differentials for the intracardiac densities included intracardiac thrombi or metastases. The intramuscular hypodensities were rim-enhancing. Given that the patient was on rivaroxaban, differentials included hematomas or metastases. As there was no overlying bruising and the lesions remained unchanged in size clinically, they were treated as metastases. The patient was treated with clexane but re-presented with worsening of shortness of breath and palpitations. CT scan showed increased size of intracardiac lesions, suggesting no response to anticoagulation, and therefore were likely metastatic in nature. He completed a 2-year course of IV pembrolizumab and was in complete remission. CONCLUSIONS Our case highlights the importance of this clinically challenging scenario when patients with known malignancy and on anticoagulation present with cardiac or musculoskeletal symptoms. Though these patients are at risk of thrombus and haematoma, cardiac and intramuscular metastasis should be considered, as the prognosis is guarded.


Subject(s)
Carcinoma, Transitional Cell , Heart Neoplasms , Kidney Neoplasms , Muscle Neoplasms , Nephroureterectomy , Humans , Male , Middle Aged , Heart Neoplasms/secondary , Heart Neoplasms/surgery , Muscle Neoplasms/secondary , Carcinoma, Transitional Cell/secondary , Carcinoma, Transitional Cell/surgery , Kidney Neoplasms/pathology , Neoplasms, Second Primary , Venous Thrombosis/etiology , Tomography, X-Ray Computed
13.
Kyobu Geka ; 77(4): 311-314, 2024 Apr.
Article in Japanese | MEDLINE | ID: mdl-38644180

ABSTRACT

The patient is a 76-year-old man. His chief complaint of chest pain led to a diagnosis of pericardial effusion of unknown cause, and pericardial drainage was performed. On the 30th day, chest pain appeared again. Echocardiography revealed a pericardial fluid reaccumulation and a substantial mass in the pericardial space. Surgical drainage was performed to find the cause. A hematoma/mass was present on the epicardium. The pericardial sac was filled with hematoma. The hematoma was removed, but part of the mass infiltrated close to the anterior descending branch of the left coronary artery, and removal of that part was abandoned. The intrapericardial hematoma and epicardium were submitted to pathology leading to the diagnosis of synovial sarcoma. The patient was discharged home 14 days after surgery.


Subject(s)
Heart Neoplasms , Pericardial Effusion , Sarcoma, Synovial , Humans , Male , Sarcoma, Synovial/complications , Sarcoma, Synovial/surgery , Sarcoma, Synovial/diagnostic imaging , Pericardial Effusion/etiology , Pericardial Effusion/diagnostic imaging , Pericardial Effusion/surgery , Aged , Heart Neoplasms/complications , Heart Neoplasms/surgery , Heart Neoplasms/diagnostic imaging , Recurrence
15.
J Cardiothorac Surg ; 19(1): 229, 2024 Apr 16.
Article in English | MEDLINE | ID: mdl-38627746

ABSTRACT

An 80-year-old female was referred to our institution due to transient right upper limb weakness. Transthoracic and transesophageal echocardiography revealed a tumor in the left atrium. The tumor was attached to the posterior wall of the left atrium near the atrioventricular node. Intraoperative pathological examination revealed that the tumor was a myxoma, and complete resection was successfully performed. However, she experienced persistent complete atrioventricular block postoperatively and required pacemaker implantation.


Subject(s)
Atrioventricular Block , Heart Neoplasms , Myxoma , Female , Humans , Aged, 80 and over , Echocardiography , Atrioventricular Block/etiology , Atrioventricular Block/surgery , Heart Neoplasms/complications , Heart Neoplasms/diagnostic imaging , Heart Neoplasms/surgery , Heart Atria/diagnostic imaging , Myxoma/complications , Myxoma/diagnostic imaging , Myxoma/surgery
16.
J Cardiothorac Surg ; 19(1): 243, 2024 Apr 18.
Article in English | MEDLINE | ID: mdl-38632629

ABSTRACT

Synovial sarcoma of the heart is a rare tumor. Herein we would like to report a case of giant intrapericardial cardiac synovial sarcoma that originated from the right ventricle and grew outward near the diaphragm. After making adequate preoperative preparation, we performed the surgery as quickly as possible and resected the tumor completely. Based on the identification of the translocation on chromosome 18 rearrangement, the tumor can be diagnosed as a primary cardiac synovial sarcoma. Through this study, we aim to afford more information about cardiac synovial sarcomas as well as a reference for similar cases.


Subject(s)
Heart Neoplasms , Mediastinal Neoplasms , Sarcoma, Synovial , Thymus Neoplasms , Humans , Sarcoma, Synovial/diagnosis , Heart Neoplasms/surgery , Heart Ventricles/pathology
17.
Cancer Rep (Hoboken) ; 7(4): e2033, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38600050

ABSTRACT

BACKGROUND: Primary cardiac myxofibrosarcoma is a rare and aggressive malignancy, with the majority of approaching strategies relying on case reports. This article provides insights into its diagnosis and treatment. CASE PRESENTATION: This paper presents the case of a 40-year-old man with sudden onset hemoptysis, leading to the diagnosis of primary cardiac myxofibrosarcoma. Treatment involved open-heart surgery to excise the left atrium tumor, followed by 6 cycles of adjuvant chemotherapy. Unfortunately, brain metastasis developed, leading to the patient's death 1 year after initial diagnosis. CONCLUSION: Primary cardiac myxofibrosarcoma remains a clinical challenge with an unfavorable prognosis. Early diagnosis through advanced imaging is crucial, and research is needed to explore innovative treatments. This case underscores the complexities of managing this rare cardiac malignancy and highlights the necessity for ongoing investigations to enhance patient outcomes.


Subject(s)
Fibrosarcoma , Heart Neoplasms , Mediastinal Neoplasms , Male , Adult , Humans , Heart Neoplasms/diagnosis , Heart Neoplasms/surgery , Heart Neoplasms/pathology , Heart Atria/diagnostic imaging , Heart Atria/surgery , Heart Atria/pathology , Prognosis , Fibrosarcoma/diagnosis , Fibrosarcoma/surgery , Mediastinal Neoplasms/pathology
18.
J Cardiothorac Surg ; 19(1): 197, 2024 Apr 10.
Article in English | MEDLINE | ID: mdl-38600499

ABSTRACT

BACKGROUND: Left atrial myxoma during pregnancy is rare. We present three cases in order to aid in the management. CASE PRESENTATION: Three cases of left atrial myxoma during pregnancy were presented in this article. Three patients all received multidisciplinary team work and acquired good outcomes. The case 1 had no symptoms and delivered before traditional cardiac surgery. The case 2 and case 3 undergone totally endoscopic minimally invasive cardiac surgery during pregnancy. The case 3 maintained pregnancy to term and gave birth to a healthy baby via vaginal delivery. No relapse of the tumor was observed. CONCLUSIONS: The management of left atrial myxoma during pregnancy ought to be individualized and combined with the gestational age. If the diagnosis was made in the first two trimesters of pregnancy, totally endoscopic minimally invasive cardiac surgery during pregnancy would be an optimal choice. The patients can benefit from the multidisciplinary team work.


Subject(s)
Heart Neoplasms , Myxoma , Humans , Pregnancy , Female , Pregnant Women , Heart Atria/surgery , Neoplasm Recurrence, Local , Heart Neoplasms/diagnosis , Heart Neoplasms/surgery , Myxoma/diagnosis , Myxoma/surgery
19.
J Cardiothorac Surg ; 19(1): 168, 2024 Apr 02.
Article in English | MEDLINE | ID: mdl-38566159

ABSTRACT

Cardiac leiomyosarcomas are a rare subset of the already infrequent, primary malignant cardiac neoplasia spectrum. The most common site for a primary leiomyosarcoma of the ventricle is on the right with fewer than five globally reported cases in the left ventricle. Most present with non-specific symptoms but attention is usually sought after the appearance of compressive symptoms or arrhythmias. We present a case of a left ventricular leiomyosarcoma in a 50-year old female patient that had a delayed diagnosis and its subsequent surgical resection and oncological management with docetaxel and gemcitabine. This case highlights the need for a high index of suspicion for cardiac masses especially if there are competing chronic diseases with similar symptomatology. Given the rare presentation of left ventricular leiomyosarcomas, case reports may provide valuable information that is otherwise unavailable.


Subject(s)
Heart Neoplasms , Leiomyosarcoma , Female , Humans , Middle Aged , Heart Ventricles/pathology , Leiomyosarcoma/diagnosis , Leiomyosarcoma/surgery , Leiomyosarcoma/pathology , Heart Neoplasms/diagnosis , Heart Neoplasms/surgery , Heart Neoplasms/pathology
20.
Zhonghua Bing Li Xue Za Zhi ; 53(4): 358-363, 2024 Apr 08.
Article in Chinese | MEDLINE | ID: mdl-38556819

ABSTRACT

Objective: To assess the clinicopathological features, immunophenotype, molecular characteristics and differential diagnosis of primary cardiac synovial sarcoma (PCSS). Methods: Five cases of PCSS were collected at Guangdong Provincial People's Hospital from 2008 to 2023, and their clinicopathological features were summarized. Immunohistochemical staining, fluorescence in-situ hybridization (FISH) and next-generation sequencing (NGS) were performed, and relevant literatures were reviewed. Results: The cases were found in four males and one female, ranging in ages from 16 to 51 years (median 30 years). Two cases were located in the pericardium, two in the right ventricle, and one in the left ventricle. Follow-up data were available in four cases. All the four patients died of disease at 3, 7, 13 and 26 months, respectively, after diagnosis. The tumor maximum diameter ranged from 6.0 to 14.0 cm in (mean 10.0 cm). Microscopically, three cases were monophasic and two cases were biphasic. Immunohistochemically, all cases were immunoreactive for EMA, vimentin, bcl-2 and CD56. The tumor cells were variably positive for pan-cytokeratin, SS18-SSX, SOX2, TLE1, CD99, synaptophysin, calretinin and calponin. FISH showed the presence of SS18 rearrangement in all the cases. NGS detected SS18-SSX gene fusion in three cases (SS18-SSX1 in one and SS18-SSX2 in two). Conclusions: PCSS is an exceedingly rare neoplasm, and should be distinguished from other various malignant epithelial and mesenchymal tumors. The clinical history, histopathological and immunohistochemical features, and molecular findings are all essential to the definitive diagnosis of PCSS.


Subject(s)
Heart Neoplasms , Mediastinal Neoplasms , Sarcoma, Synovial , Male , Humans , Female , Biomarkers, Tumor/genetics , Biomarkers, Tumor/analysis , Sarcoma, Synovial/genetics , Sarcoma, Synovial/diagnosis , Sarcoma, Synovial/pathology , Proto-Oncogene Proteins/genetics , Repressor Proteins/genetics , Oncogene Proteins, Fusion/genetics , Heart Neoplasms/genetics , Heart Neoplasms/surgery
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