Primary Cardiac Epithelioid Angiosarcoma with Sustained Remission following Surgical Resection: A Case Report
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ABSTRACT:
SUBMITTER: Kyaw M
PROVIDER: S-EPMC10601732 | biostudies-literature | 2023 Jan
REPOSITORIES: biostudies-literature
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Project description:Primary cardiac tumors are exceptionally rare, with malignant tumor occurrences ranging from 0.0017% to 0.28%. Among these, primary cardiac angiosarcoma (PCA) stands as the most prevalent malignancy, primarily impacting the right cardiac system. In this case report, we present the instance of a 44-year-old woman who recently exhibited acute chest discomfort and was subsequently diagnosed with a microangiosarcoma within the right atrium and superior vena cava. Diagnostic modalities including chest x-rays, CT, MRI, and PET-CT were instrumental in pinpointing the tumor's location and nature. Surgical excision followed by pathological and immunological examinations confirmed the diagnosis. The patient's recovery post-surgery has been encouraging, with successful follow-up chemoradiotherapy administered. Despite advancements, devising optimal strategies for enhancing patient survival and quality of life in angiosarcoma cases remains a pressing research challenge.
| S-EPMC10655095 | biostudies-literature
Project description:IntroductionPrimary pleural epithelial angiosarcoma (EAS) is an extremely rare tumor with no specific clinical symptoms. Clinical data on primary pleural EAS are limited, and misdiagnosis often occurs.Case presentationThe present study reports the case of a 31-year-old patient diagnosed with primary pleural EAS with lung and bone metastases. The patient presented with persistent right chest pain for 5 months and dyspnea for 2 months. Chest computed tomography (CT) scan revealed right hydropneumothorax, diffuse thickening of the right pleura, passive atelectasis, and scattered nodules in the left lung. A medical thoracoscopic pleural biopsy revealed a vasogenic tumor. To further confirm the diagnosis, positron emission tomography/CT (PET/CT) examination was recommended to determine the biopsy site after multidisciplinary discussion. Increased 18F-FDG uptake in the right pleura and hypermetabolic nodules in the right chest wall, first lumbar vertebrae, second sacral vertebrae, and bilateral iliac crest was detected via PET/CT. CT-guided chest wall and lung biopsies were performed. Immunohistochemistry of specific markers was performed according to remote consultation with a pathologist, and tumor cells with strong positive expression of CD31, CD34, and ETS-related genes led to the final diagnosis of primary pleural EAS.ConclusionPrimary pleural EAS should be considered for hydropneumothorax of an unknown cause. PET/CT can accurately locate the lesion. The pathological examination is the basis for primary pleural EAS diagnosis. Moreover, multidisciplinary discussion and remote expert consultation can improve the diagnosis rate of primary pleural EAS.
| S-EPMC10791133 | 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:Primary renal epithelioid angiosarcoma (EAS) is extremely rare and carries a poor prognosis. Herein, we present a case of renal EAS in an 81-year-old male patient who complained of hematuria for 1 year. A computerized tomography (CT) scan revealed an occupying lesion at the upper pole of the left kidney, with scattered calcifications, along with retroperitoneal lymph node metastasis and possible lung metastasis. A laparoscopic palliative nephrectomy was performed, and postoperative pathology confirmed a malignant tumor with necrosis in the left kidney. Immunohistochemistry (IHC) revealed positive expression for CD31, CD10, and vimentin, consistent with the diagnosis of EAS. Although EAS is a rare, aggressive, and often misdiagnosed condition, IHC can help confirm its diagnosis, and in our case, the scattered calcifications observed on CT imaging might be helpful in its differential diagnosis.
| S-EPMC11646802 | biostudies-literature
Project description:•Vulvar angiosarcoma in a patient with pelvic radiation and chronic lymphedema•Epithelioid histologic staining positive for vimentin, ERG, CD31•Gross appearance of violaceous hyperpigmentation of the mons pubis.
| S-EPMC5537087 | biostudies-other
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:We report a novel observation of a cardiac hemangioma possible transformation into cardiac angiosarcoma. This report should give a new insight on the pathogenesis of the tumor, always thought to have a de-novo genesis. We emphasize in our report the value of combining imaging modalities to examine the anatomic and metabolic properties of cardiac tumors providing a valuable tool for comprehensive assessment. <Learning objective: Assessment of cardiac tumors should combine different imaging modalities to examine the anatomic and metabolic properties of the lesions. We report a novel observation on cardiac angiosarcoma possible transformation from a cardiac hemangioma. This report should give a new insight into the pathogenesis of the tumor, always thought to have a de-novo genesis.>.
| S-EPMC6149278 | biostudies-literature