Case Report: Successful surgical management of a challenging primary cardiac angiosarcoma
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PROVIDER: S-EPMC10655095 | biostudies-literature | 2023 Jan
REPOSITORIES: biostudies-literature
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Project description:Epithelioid angiosarcoma is an extremely rare subtype of cardiac angiosarcoma that is highly aggressive and associated with poor prognosis. Due to its rare nature, the epidemiology and pathogenesis of this disease are not well-known. Thus, effective diagnostic and treatment modalities are limited. Here, we report a case of a primary epithelioid angiosarcoma in a patient who was treated successfully with surgical resection. A 45-year-old woman who initially presented with chronic systemic symptoms and severe anemia and subsequently developed new-onset atrial fibrillation with rapid ventricular rate was found to have a right-sided cardiac mass with a large pericardial effusion. Several years prior to presentation, she was treated for localized papillary thyroid cancer with Gc protein-derived macrophage-activating factor (Gc-MAF) therapy after declining thyroidectomy. After initial workup of her cardiac mass, including a transthoracic echocardiogram and cardiac MRI, she was transferred to an outside hospital where her mass was surgically resected. She was found to have stage IIIA high-grade epithelioid angiosarcoma involving the inferior vena cava, right atrium, and pericardium. She subsequently had complete resolution of her pericardial effusion and anemia and continues to have good performance status 16 months after her excellent surgical outcome without evidence of recurrence. This unique case contributes to our knowledge of epithelioid cardiac angiosarcoma, of which limited number of cases has been reported. It highlights a favorable outcome following surgical resection of a rare, life-threatening primary cardiac tumor.
| S-EPMC10601732 | biostudies-literature
Project description:A 63-year-old woman was admitted to our department for the investigation of superior vena cava (SVC) syndrome. Computed tomography revealed an azygos tumor extending into the SVC. Video-assisted thoracic surgery (VATS) was performed to remove the distal end of the azygos vein in the left lateral position, followed by complete resection of the entire tumor under median sternotomy in the supine position. The histological diagnosis was a primary angiosarcoma of the azygos vein. The patient was discharged without any complications and is now alive and tumor-free 24 months after surgery. In addition, contrast-enhanced computed tomography revealed no graft occlusion in the two reconstructed brachiocephalic veins. Thoracoscopic surgery in the lateral position is useful for safe and reliable complete resection of a tumor arising from the azygos vein.
| S-EPMC10912536 | biostudies-literature
Project description:Primary pericardial angiosarcoma is a rare malignancy of the pericardium with variable clinical features and imaging characteristics. Herein, we report a case of histopathologically confirmed pericardial angiosarcoma in a 66-year-old man. The patient developed cardiac tamponade in a short time period. The transthoracic echocardiography showed the presence of multiple irregular echodensities, heterogeneous in echogenicity, encasing the apex of both ventricles in the pericardial space, initially misinterpreted as pericardial effusion. The patient died of cardiogenic shock despite undergoing a surgical pericardiectomy. Pericardial angiosarcoma can manifest as a mass obliterating the pericardial sac, rather than the typical pericardial effusion observed on echocardiography. Multimodality imaging studies aid in diagnosing primary pericardial angiosarcoma, but the final diagnosis relies on tissue histopathology.
| S-EPMC10897014 | biostudies-literature
Project description:Cardiac angiosarcoma is a rare disease with a high mortality rate despite its low incidence. Surgery is currently the mainstay treatment strategy for patients with this condition. Herein, we describe a case of primary cardiac angiosarcoma, including symptoms, examination findings, treatment strategy and prognosis. In 2020, the patient was admitted to our hospital, and Next-Generation Sequencing (NGS) revealed a mutation in the DNMT3A gene. Generally, DNMT3A mutations are most commonly seen in atherosclerosis and myeloid leukemia. To our knowledge, this is the first reported case of primary cardiac angiosarcoma with DNMT3A gene mutation.
| S-EPMC9647023 | biostudies-literature
Project description:BackgroundMalignant primary cardiac neoplasms are rare and primary cardiac angiosarcoma is the most common and aggressive subtype. It most commonly presents in middle-aged males and due to its non-specific clinical presentation, the diagnosis is often delayed until advanced disease is already present. Clinical presentation is determined by manifestations of local infiltration or metastatic disease and making an early diagnosis is extremely challenging.Case summaryA 15-year-old previously healthy boy was admitted to the emergency department with a history of pathological weight loss and functional decline. The patient was found to have a left-sided pneumothorax as well as bilateral diffusely spread pulmonary nodules on plain chest radiograph. Computed tomography chest confirmed widespread pulmonary metastases and a right atrial filling defect. Echocardiography revealed a right atrial tumour and transvenous endomyocardial biopsy of the tumour was done under fluoroscopic and echocardiographic guidance. A diagnosis of primary cardiac angiosarcoma was made. The patient demised shortly after presentation.DiscussionPrimary cardiac angiosarcoma is rare and even more so in patients as young as the case described. The diagnostic process poses several challenges to the clinician, of which the obtaining of a histological sample is one. This case report demonstrates aspects both unique and typical of this rare disease. It also describes an effective option for obtaining tissue for a histological diagnosis in patients whose clinical condition may not allow biopsy under general anaesthesia.
| S-EPMC7898572 | biostudies-literature
Project description:BackgroundPrimary cardiac angiosarcomas are rare, but they are the most aggressive type of primary cardiac neoplasms. When patients do present, it is with advanced pulmonary and/or cardiac symptoms. Therefore, many times the correct diagnosis is not made at the time of initial presentation. These patients have metastatic disease and the vast majority of these patients die within a few months after diagnosis. Currently the treatment choices are limited and there are no targeted therapies available.Case presentationA 56-year-old male presented with shortness of breath, night sweats, and productive cough for a month. Workup revealed pericardial effusion and multiple bilateral pulmonary nodules suspicious for metastatic disease. Transthoracic echocardiogram showed a large pericardial effusion and a large mass in the base of the right atrium. Results of biopsy of bilateral lung nodules established a diagnosis of primary cardiac angiosarcoma. Aggressive pulmonary disease caused rapid deterioration; the patient went on hospice and subsequently died. Whole exome sequencing of the patient's postmortem tumor revealed a novel KDR (G681R) mutation, and focal high-level amplification at chromosome 1q encompassing MDM4, a negative regulator of TP53.ConclusionMutations in KDR have been reported previously in angiosarcomas. Previous studies also demonstrated that KDR mutants with constitutive KDR activation could be inhibited with specific KDR inhibitors in vitro. Thus, patients harboring activating KDR mutations could be candidates for treatment with KDR-specific inhibitors.
| S-EPMC5217318 | biostudies-literature
Project description:RationaleAngiosarcoma is a rare malignant tumors. The objective of this study is to report a patient who suffered from a progressive low back pain and left lower extremities radiation pain for about 8 months, After diagnoses, this was identified as an extremely rare case of primary multiple angiosarcoma of vertebra.Patient concernsA 54-year-old man with a history of 2-year hypertension and 8-year diabetes, both of which were well controlled by drug management. Lately, he suffered from a progressive low back pain and left lower extremities radiation pain for about 8 months.DiagnosesMagnetic resonance imaging of lumbar showed a clear pathological fracture and primary multiple angiosarcoma of all vertebra. Postoperative pathology and High-throughput sequencing confirmed the diagnosis of primary multiple angiosarcoma of vertebra.InterventionsThe patient underwent minimally invasive pedicle screw fixation combined with bone cement augmentation for the purpose of stabilizing the damaged vertebrae. Following operation, he received both radiotherapy and chemotherapy for a period of time.OutcomesThe operation has achieved positive results in relieving pain and stabilizing the spine. No wound problem or operative complications occurred after operation. The patient reported an obvious remission of low back pain and was only capable to perform restricted physiological activities. A long-term palliative radiotherapy and chemotherapy were performed after operation. Unfortunately, the patient died 18 months later.ConclusionThis article emphasizes primary multiple angiosarcoma of vertebra. Despite being rare, it should be part of the differential when the patient manifested back pain and radiculopathy. We recommended the minimally invasive pedicle screw fixation for angiosarcoma of vertebra. Osteoplasty by bone cement augmentation was also an ideal choice for surgical treatment. It also advocates the use of specific targeted radiotherapy drugs based on gene analysis of tumors.
| S-EPMC7738032 | biostudies-literature
Project description:We present the case of a young woman without a medical history who presented with a giant right atrial, transtricuspid, and right ventricular mass and in a severe clinical state. Multimodal imaging raised the suspicion of primary cardiac angiosarcoma. Due to rapid hemodynamic and respiratory deterioration, we were forced to perform surgical removal of the mass with a concomitant reconstruction of the involved right heart structures, only 48 h after presentation. The postoperative course was uneventful, and the patient was discharged from the intensive care unit 2 days later. Radical surgical resection with reconstruction of the resected heart structures was the only possible salvage option for giant angiosarcoma, which led to hemodynamic instability. Followed by chemotherapy, this radical approach may prolong survival.
| S-EPMC10014811 | biostudies-literature
Project description:BackgroundBreast Angiosarcoma can be divided into primary breast angiosarcoma (PBA) and second breast angiosarcoma (SBA). PBA is rare malignant breast cancer with poor outcomes. PBA usually occurs in women between 30-40 years old. PBA does not have a specific clinical manifestation. Clinically, PBA presents with a rapidly enlarging breast mass and skin involvement with skin color changes. Ultrasonography of PBA can be hypoechoic or hyperechoic, or mixed disordered areas. Microscopically, PBA can be classified into three grades according to the degree of differentiation, and the grade is related to the prognosis. And PBA can also express vascular endothelial markers. The main treatment for PBA is surgery, especially mastectomy. There are other treatments, such as chemotherapy and radiotherapy, but their effectiveness needs further confirmation. Targeted drugs may be helpful.Case descriptionA 32-year-old woman with main clinical presentation of a rapidly growing mass located in the upper inner quadrant of the right breast with skin involvement. PBA was diagnosed with first extended local resection, and then the patient underwent a second right mastectomy. The patient is now undergoing chemotherapy.ConclusionsSince this is a rare form of breast cancer, we report this case to raise the attention of breast surgeons to avoid misdiagnoses.
| S-EPMC10175007 | biostudies-literature
Project description:BackgroundPrimary hepatic angiosarcoma (PHA) is a rare and aggressive solid tumor, with high rates of local recurrence and distant metastasis, and poor prognosis. There are no established treatment guidelines for PHA.Case presentationA 78-year-old asymptomatic man with PHA that was successfully treated with pazopanib plus PD-1 inhibitor and RetroNectin-activated killer cells (RAK cells). After one month of treatment, there was a clear reduction in the size and number of the liver metastases; and after nearly 15 months, most of the lesions were stable, no new lesions had developed, and the side effect of treatment was minor.ConclusionPazopanib, PD-1 inhibitor and RAK cells could serve as a potential option for the treatment of advanced PHA.
| S-EPMC5822655 | biostudies-literature