We present the case of a 39-year-old woman who was diagnosed with SLE and antiphospholipid antibodies 8 years ago. The chief manifestations of her disease included low-grade fever and polyarthritis. Eight months before presentation, she experienced symptoms attributed to a flare of SLE, leading to an increase in immunomodulatory treatment with no improvement. She presented to the emergency room with acute onset of dyspnea. Clubbing of her fingers and toes was noted. When questioned, she reported the onset of clubbing 5 months earlier. A CTA was performed to rule out pulmonary embolism, which was excluded, although it revealed a severely damaged mitral valve with severe insufficiency and a large mass on the valve, protruding into the left atrium. Antibiotics were started, with a working diagnosis of infectious endocarditis; however, the severe mitral valve dysfunction lead to emergency mitral valve replacement, revealing an organized thrombus. She was treated with anticoagulation, with a working diagnosis of Libman-Sacks endocarditis, with no improvement. Additional immunosuppression failed to improve her symptoms. Enlargement of the thrombotic mass and an increased gradient across the prosthetic mitral valve led to repeat surgery, culminating in a diagnosis of high-grade sarcoma within the left atrial mass. We further discuss cardiac sarcoma and describe the occurrence of clubbing in patients with sarcoma. This case highlights the importance of interdisciplinary collaboration and the need for vigilant monitoring in refractory cases, particularly when atypical presentations arise.

Recurrent pericardial effusion poses a diagnostic challenge, especially in young patients. We present a case of a 22-year-old female who experienced recurrent pericardial effusion and cardiac tamponade. Despite initial treatment with anti-tubercular drugs and prednisolone, the patient had a relapse of symptoms, necessitating further investigation. Imaging studies revealed massive pericardial effusion with septations, suggestive of constrictive pericarditis with impending cardiac tamponade. To establish a definitive diagnosis, the patient underwent an FDG PET-CT scan after adhering to a specific dietary regimen. The scan revealed an ill-defined mediastinal mass with high metabolic activity, along with a gross pericardial effusion showing metabolic activity in the periphery and septations. Subsequent biopsy of the mediastinal mass confirmed a diagnosis of high-grade sarcoma.Primary malignant cardiac tumors are rare, and their prognosis is generally poor due to limited treatment options. While echocardiography and MRI are commonly used imaging techniques, FDG PET-CT is not routinely employed for evaluating cardiac tumors. However, in this case, FDG PET-CT played a crucial role in identifying the mediastinal mass and confirming the diagnosis.Early detection and accurate diagnosis of cardiac tumors are vital for initiating appropriate treatment strategies. Further studies are needed to explore the utility of FDG PET-CT in the evaluation of cardiac tumors, especially in cases of recurrent pericardial effusion.

UNASSIGNED: Primary cardiac tumors remain exceptionally rare, characterized by a poor prognosis. Among them, sarcomas originating in the pulmonary arteries constitute the most infrequent subgroup within primary cardiac sarcomas.
UNASSIGNED: This report presents the case of a 76-year-old female experiencing a recurrence of an undifferentiated pleomorphic intracardiac pulmonary artery sarcoma located in the right ventricular outflow tract, manifesting 8 years after initial remission. Successful outcomes were attained through a combination of surgical resection, state-of-the-art radiotherapy, and chemotherapy. This comprehensive approach proved essential for optimizing both survival and quality of life.
UNASSIGNED: The unexpectedly prolonged recurrence-free survival observed in this case underscores the effectiveness of the comprehensive multimodal treatment approach outlined in the existing literature. This highlights the pivotal role of a multidisciplinary strategy in addressing primary cardiac sarcomas, particularly those arising in the pulmonary arteries.

We present a case of a patient with a four-month history of gradual-onset dyspnea and generalized body weakness. During the clinical evaluation, a mass was found in the left atrium. Coronary angiography was performed and showed normal coronary arteries. We proceeded with a complete surgical excision of the tumoral mass, and histopathology confirmed it as undifferentiated cardiac sarcoma. Six months after the surgical intervention and adjuvant chemotherapy, the patient is in complete remission, with no evidence of a recurrence of the malignant pathology. Cardiac sarcoma is a rare clinical finding and a diagnostic and therapeutic challenge due to its numerous non-specific clinical presentations.

Primary cardiac tumours are relatively uncommon (75% are benign). Across the other 25%, representing malignant neoplasia, sarcomas account for 75-95%, and primary cardiac intimal sarcoma (PCIS) is one of the rarest findings. We aimed to present a comprehensive review and practical considerations from a multidisciplinary perspective with regard to the most recent published data in the specific domain of PCIS. We covered the issues of awareness amid daily practice clinical presentation to ultra-qualified management in order to achieve an adequate diagnosis and prompt intervention, also emphasizing the core role of MDM2 immunostaining and MDM2 genetic analysis. An additional base for practical points was provided by a novel on-point clinical vignette with MDM2-positive status. According to our methods (PubMed database search of full-length, English publications from January 2021 to March 2023), we identified three studies and 23 single case reports represented by 22 adults (male-to-female ratio of 1.2; male population with an average age of 53.75 years, range: 35-81; woman mean age of 55.5 years, range: 34-70) and a 4-year-old child. The tumour-related clinical picture was recognized in a matter of one day to ten months on first admission. These non-specific data (with a very low index of suspicion) included heart failure at least NYHA class II, mitral regurgitation and pulmonary hypertension, acute myocardial infarction, ischemic stroke, obstructive shock, and paroxysmal atrial fibrillation. Awareness might come from other complaints such as (most common) dyspnoea, palpitation, chest pressure, cough, asthenia, sudden fatigue, weakness, malaise, anorexia, weight loss, headache, hyperhidrosis, night sweats, and epigastric pain. Two individuals were initially misdiagnosed as having endocarditis. A history of prior treated non-cardiac malignancy was registered in 3/23 subjects. Distant metastasis as the first step of detection (n = 2/23; specifically, brain and intestinal) or during follow-up (n = 6/23; namely, intestinal, brain and bone, in two cases for each, and adrenal) required additional imagery tools (26% of the patients had distant metastasis). Transoesophageal echocardiography, computed tomography (CT), magnetic resonance imagery, and even 18F-FDG positronic emission tomography-CT (which shows hypermetabolic lesions in PCIS) represent the basis of multimodal tools of investigation. Tumour size varied from 3 cm to ≥9 cm (average largest diameter of 5.5 cm). The most frequent sites were the left atrium followed by the right ventricle and the right atrium. Post-operatory histological confirmation was provided in 20/23 cases and, upon tumour biopsy, in 3/23 of them. The post-surgery maximum free-disease interval was 8 years, the fatal outcome was at the earliest two weeks since initial admission. MDM2 analysis was provided in 7/23 subjects in terms of MDM2-positive status (two out of three subjects) at immunohistochemistry and MDM2 amplification (four out of five subjects) at genetic analysis. Additionally, another three studies addressed PCISs, and two of them offered specific MDM2/MDM2 assays (n = 35 patients with PCISs); among the provided data, we mention that one cohort (n = 20) identified a rate of 55% with regard to MDM2 amplification in intimal sarcomas, and this correlated with a myxoid pattern; another cohort (n = 15) showed that MDM2-positive had a better prognostic than MDM2-negative immunostaining. To summarize, MDM2 amplification and co-amplification, for example, with MDM4, CDK4, HMGA3, CCND3, PDGFRA, TERT, KIT, CCND3, and HDAC9, might improve the diagnosis of PCIS in addition to MDM2 immunostaining since 10-20% of these tumours are MDM2-negative. Further studies are necessary to highlight MDM2 applicability as a prognostic factor and as an element to be taken into account amid multi-layered management in an otherwise very aggressive malignancy.

UNASSIGNED: A quarter of all cardiac tumors are malignant, and most (~ 95%) are sarcomas. It is the most aggressive malignant cardiac tumor carrying the worst prognosis. Tumor involvement with the vital intracardiac structures makes it difficult for complete surgical resection. We aimed to study the role of complete surgical resection and its importance in long-term outcomes.
UNASSIGNED: We analyzed published literature from 2002 to 2022 using PubMed. Cases reported adult, intracardiac sarcomas, and received surgical resections were included. We reviewed 132 published case reports, including and analyzed the following variables: demographics, clinical presentations, diagnostic imaging modality, the extent of surgical resection, pathological diagnosis, tumor location, postoperative chemo-radiation therapy, and follow-up (including re-operation, local and distant recurrence).
UNASSIGNED: A total of 135 patients are included from 132 articles. The mean age was 46.69 (18-86) and 76 patients were females. The main complaints were dyspnea (70%) and chest pain (32%). Performed investigations were transthoracic echocardiography (TTE) in 114/135 (84%), computer tomography (CT) scan 89/135 (66%), trans-esophageal echocardiography (TOE) 22%, and cardiac magnetic resonance imaging (MRI) 29%. The most common location was the atrium (left 46%, right 30%). Complete surgical resection was performed in 91 cases (67%), and frozen section was performed in 62 patients (43 positives). Incomplete resections were in 42 cases. Patients who underwent complete surgical resection had mean survival of 14.58 months and median of 10.5 months, compared to incomplete resection patients with 9.12 months and 6.5 months respectively.
UNASSIGNED: Our review shows complete surgical resection results in better short- and long-term outcomes in intracardiac sarcoma patients. Furthermore, combining chemo-radiotherapy has additional benefits towards long-term survival.

The case highlights the good survival after radical surgery and chemotherapy of a cardiac sarcoma, and the need for close follow-up due to possible early postsurgical complications.

We herein report a 37-year-old man who experienced recurrence of metastatic cardiac rhabdomyosarcoma along with intractable ventricular tachycardia (VT) 7 years after resection of rhabdomyosarcoma in his right elbow. At 36 years old, he developed VT unresponsive to radiofrequency catheter ablation (RFCA). Initially, the cardiac tumor was not detected, but it gradually grew in size at the RFCA site. A surgical biopsy confirmed the diagnosis of metastatic cardiac rhabdomyosarcoma. Despite radiation therapy, cardiac tumor progression and VT instability could not be prevented. Ultimately, the patient died 27 months after the initial documentation of VT.

暂无摘要。

Malignant cardiac tumors of the heart are extremely rare and may present tremendous diagnostic and therapeutic challenges. These tumors are able to infiltrate the heart and metastasize systemically. Early detection is often elusive as the clinical presentation is highly variable, posing significant diagnostic and therapeutic difficulties. Despite a multidisciplinary approach, the prognosis for patients with malignant cardiac tumors remains guarded. Early diagnosis and a multidisciplinary approach involving cardiac surgeons, oncologists and critical care specialists are crucial in the management of this disease. Further research is needed to better understand the pathomechanisms of tumor-related complications and to develop effective treatment strategies to improve patient outcomes. The rare case of a 78-year-old woman with left atrial tumor requiring emergency surgery for acutely developing mitral valve obstruction is presented. Pathology confirmed an undifferentiated pleomorphic sarcoma. This patient tragically did not survive, highlighting the difficulties of managing such a rare and deceptive heart disease.