Metastatic Cardiac Angiosarcoma Research Articles

A durable response to gemcitabine monotherapy in metastatic cardiac angiosarcoma

Introduction: Primary cardiac angiosarcoma is a rare neoplasm with high rates of local recurrence and distant metastasis for which optimum treatment is poorly defined. Case Report: We present the case of a 49-year-old man with cardiac angiosarcoma with distant metastases who exhibits durable response to gemcitabine monotherapy. At initial diagnosis, he underwent complete resection with adjuvant Adriamycin and Ifosfamide for four cycles as well as external beam radiation for possible residual disease. He had recurrence with liver and bone metastases four years later. He received Gemcitabine and Docetaxel for six cycles followed by Gemcitabine monotherapy with no evidence of recurrence for the next three years. Conclusion: This patient’s consistent response to gemcitabine maintenance therapy in metastatic cardiac angiosarcoma adds to relative paucity of data regarding the management of this rare malignancy, offering insight into best practice and hope for patients afflicted with this disease.

Abstract 14933: A Rare Case of Cardiac Angiosarcoma Presenting as Hemorrhagic Pericardial Effusion

Cardiac sarcomas are exceptionally rare, with an incidence of only 0.02% and comprising less than 1% of all primary cardiac tumors. Consequently, diagnosis of the disease can be challenging due to non-specific presenting symptoms and diverse clinical manifestations. We present a unique case of cardiac sarcoma that highlights these challenges. A 70-year-old man with a history of hypertension, hyperlipidemia, and latent tuberculosis presented with palpitations, chest pain, and dyspnea, was found to have hemorrhagic pericardial effusion with tamponade. Pericardial fluid studies were negative for malignancy, and echo not suggestive of cardiac mass or pericardial abnormalities. He presented several months later with hemoptysis and was found to have innumerable pulmonary nodules on CT chest and a mediastinal mass adjacent to the right atrium. Bronchoscopy was negative for infection or malignancy. Ultimately the patient underwent CT-guided lung biopsy, which showed a malignant spindle cell neoplasm with a positive stain for vimentin and vascular markers suggestive of angiosarcoma of unknown primary. Given unclear relation of his mediastinal mass, cardiac MRI was done which showed a rapidly enhancing bulky neoplasm with abnormal contrast enhancement arising from the lateral right atrium (Figure 1) consistent with metastatic cardiac angiosarcoma. Patient was started on systemic anthracycline-based chemotherapy; however the overall prognosis remains poor. Given the rarity of cardiac sarcomas, prognosis is generally poor due to the advanced stage at the time of diagnosis. These tumors exhibit aggressive behavior and have a high propensity for metastasis. Due to their infrequency, knowledge and research is limited regarding effective treatments. This case highlights the significance of considering cardiac angiosarcoma in cases of hemorrhagic pericardial effusion even in the absence of obvious cardiac mass or pericardial nodularity on initial presentation.

Clinical and imaging characteristics of patients with primary cardiac angiosarcoma

Objective: To investigate the clinical, cardiac imaging characteristics and prognosis of patients with primary cardiac angiosarcoma. Methods: The clinical data of 14 patients hospitalized with primary cardiac angiosarcoma from January 2001 to December 2017 in Peking Union Medical College Hospital were collected and analyzed. Metastatic cardiac angiosarcoma was not included in this study. Patients were followed up post discharge per telephone call or clinical visit. Results: Of the 14 patients, 8 were males and 6 were females, average age was 48 years. The main clinical symptoms were shortness of breath (8/14), hemoptysis (6/14), fever (5/14), chest pain (4/14) and cough (3/14). Imaging examinations showed that the tumors of 8 patients were located in the right heart and 6 in the pericardial cavity. Tumors in the right heart often infiltrate the atrial wall and cause pericardial effusion (7/8). Tumors in the pericardium were characterized by recurrent bloody pericardial effusion (6/6), prone to progressive constrictive pericarditis (3/6), pericardial fluid cytology was often negative (6/6). MRI showed heterogeneous high signal intensity (cauliflower aspect) on T2-weighted image and heterogeneous enhancement with a"sunray" aspect at the perfusion study. At the time of diagnosis, 8 patients developed lung or adrenal metastasis (8/14). The median survival was only 305 days. Conclusions: Primary cardiac angiosarcoma is a rare disease with non-specific clinical manifestation and poor prognosis. Imaging examinations may help diagnosis. The high invasiveness and the easy-to-metastasis feature of the tumor contribute to the poor prognosis of cardiac angiosarcoma.

ANGIOSARCOMAS: A TALE OF THE RIGHT ATRIAL RARITY

SESSION TITLE: Medical Student/Resident Cardiovascular Disease Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Primary cardiac tumors are quite rare with reported incidence rates of 0.001-0.3%. Angiosarcomas most commonly occur in the head and neck, but also account for the majority of malignant primary cardiac tumors. Presenting symptoms are nonspecific and disease is often advanced at time of diagnosis, making early identification crucial. CASE PRESENTATION: A 65 year-old male with a history of CAD and tobacco use was transferred from an outside hospital with fevers, chest pain, hemoptysis, and respiratory distress. He had been admitted twice in the past two months for dyspnea, chest pain, and fatigue. Prior evaluation included a cardiac echo, which showed a large pericardial effusion. Pericardiocentesis was completed twice, but was non-diagnostic with negative fluid studies. Lung nodules were noted on CT, but thought to be benign and pulmonology had recommended outpatient follow-up. He was thus treated with ibuprofen and colchicine and discharged. On this admission, CXR and CT angiography again showed nodular lung infiltrates and a moderate pericardial effusion, but a new heterogeneous 5.1 x 6.0 x 5.0 cm right atrial mass was also noted. A normocytic anemia (Hgb=8.8) and thrombocytosis (platelets=490) were present with a WBC count of 6.69. Repeat echo showed a large hypoechoic lesion next to the right atrium without effusion. Cardiac MRI demonstrated a 9 cm lobulated lesion along the anterior border of the right atrium, suspicious for a mycotic pseudoaneurysm. The lesion was inseparable from the right coronary artery and two globular thrombi were visualized within the lesion. Pericardial thickening suspicious for loculation/abscess and innumerable pulmonary opacities suspicious for septic emboli were noted. Cardiothoracic surgery was consulted, but preoperative left heart catheterization showed neovascularization of the right atrial mass concerning for cardiac angiosarcoma. Given the high risk of endomyocardial biopsy, biopsy of a lung lesion was completed and confirmed metastatic cardiac angiosarcoma. Treatment and prognosis were reviewed, and the patient elected hospice care. DISCUSSION: Primary cardiac tumors present with non-specific symptoms, including chest pain, shortness of breath, and malaise. TEE is the test of choice with a sensitivity of 97% although CTA or MRI may be necessary. 90% of tumors arise in the right atrium with the most common site of metastasis being the lungs. Treatment is not standardized given the low incidence and lack of randomized trials. Wide resection is the main treatment modality, as primary tumors are generally resistant to chemotherapy and radiation, but surgery with adjuvant therapy is still considered. Despite therapy, outcomes are poor with a median survival of 6 months. CONCLUSIONS: Recognizing a primary cardiac tumor may be difficult as presenting symptoms are non-specific. Echo is the diagnostic modality of choice with the primary treatment being resection. Reference #1: Metastatic Cardiac Angiosarcoma to the Lung, Spine, and Brain: A Case Report and Review of the Literature. Lin CT, Ducis K, Tucker S, Tranmer B. World Neurosurg. 2017 Nov;107:1049.e9-1049.e12. doi: 10.1016/j.wneu.2017.08.023. Epub 2017 Aug 9. Review. PMID: 28803168 Reference #2: Echocardiographic Features of Cardiac Angiosarcomas: The Mayo Clinic Experience (1976-2013). Kupsky DF, Newman DB, Kumar G, Maleszewski JJ, Edwards WD, Klarich KW. Echocardiography. 2016 Feb;33(2):186-92. doi: 10.1111/echo.13060. Epub 2015 Oct 13. PMID: 26460068 Reference #3: Primary malignant tumors of the heart: Outcomes of the surgical treatment. Pacini D, Careddu L, Pantaleo A, Parolari A, Leone O, Daprati A, Gargiulo GD, Di Bartolomeo R. Asian Cardiovasc Thorac Ann. 2015 Jul;23(6):645-51. doi: 10.1177/0218492315573674. Epub 2015 Feb 18. PMID: 25698795 DISCLOSURES: No relevant relationships by Courtney Certain, source=Web Response No relevant relationships by Kim Jordan, source=Web Response No relevant relationships by Craig Sewell, source=Web Response No relevant relationships by Shenali Wickramanayake, source=Web Response

THE TUMOR PLOP WHO CRIED WOLF: A CASE OF METASTATIC CARDIAC ANGIOSARCOMA

SESSION TITLE: Monday Abstract Posters SESSION TYPE: Original Investigation Posters PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: Cardiac sarcomas are extremely rare, primary malignant tumors arising from the heart or great vessels. Due to their complex nature and infrequency, there is often a delay in diagnosis and thus, a dismal prognosis. Here, we review the case of a patient who presented with a constellation of symptoms that, over many weeks, led to the final diagnosis of metastatic cardiac angiosarcoma. CASE PRESENTATION: Our patient is a 63 year-old female with history of type 2 diabetes. She was undergoing an outpatient workup for multiple myeloma and was found to have worsening anemia, renal function and hip pain, secondary to a pathologic ischial fracture. On initial presentation, her chief complaint was progressive dyspnea. Physical exam was notable for tachypnea and tachycardia, and she was found to have an elevated D-dimer. CT angiogram of her chest was negative for pulmonary embolism, however, revealed a large left atrial mass with possible extension into the ventricle and bilateral pleural effusions. The multi-lobulated mass measured approximately 3 x 4 cm on echocardiogram. Secondary metastasis to the heart or primary benign myxoma was initially suspected. Further study with cardiac MRI determined that the mass was centered over the ostium of the left inferior pulmonary vein and covered in thrombus, thus favoring the diagnosis of sarcoma. Biopsy of the pathologic hip fracture was also consistent with metastatic sarcoma. Upon further staging, she was noted to have several abdominal soft tissue nodules, scattered lytic bony lesions, and a filling defect within the proximal jejunum. Initial treatment consisted of diuresis, steroids, bisphosphonates, as well as Lovenox for the associated thrombus. Later, she developed sharp abdominal discomfort and was found to have jejunal intussusception. EGD revealed an adjacent duodenal ulcer and biopsy with immunohistochemical studies was consistent with metastatic cardiac angiosarcoma. Given the location and size of the atrial tumor, surgical resection was not an option. The patient was started on a trial of olaratumab, typically used in soft-tissue sarcomas, and doxorubicin with possible future palliative radiation. Over the next six months, after multiple admissions with various complications and worsening pain, she elected to pursue hospice care. She passed away peacefully at home, approximately one year after her initial presentation. DISCUSSION: This case illustrates a complex case of metastatic cardiac angiosarcoma. Given the late presentation with distant metastasis to various sites within the abdomen and bone, it serves as an example of the devastating effects this rare form of cancer can have. CONCLUSIONS: The complex location of the mass and its paroxysmal extension through the mitral valve into the left ventricle likely caused transient mitral regurgitation and pulmonary hypertension, which likely caused her initial dyspnea on presentation. Reference #1: Patel SD, Peterson A, Bartczak A, et al. Primary cardiac angiosarcoma - a review. Med Sci Monit. 2014;20:103-9. Published 2014 Jan 23. https://doi.org/10.12659/MSM.889875 DISCLOSURES: No relevant relationships by Logan Rhea, source=Web Response

Primary Cardiac Angiosarcoma with Extensive Visceral Metastases: Utility of 18F-Fluorodeoxyglucose Positron Emission Tomography-Computed Tomography in Response Assessment to Sorafenib.

Primary cardiac angiosarcomas although rare neoplasms remain the most common primary malignant neoplasms affecting the heart. We discuss the sequential positron-emission tomography–computed tomography findings of a 50-year-old man who was diagnosed with the metastatic cardiac angiosarcoma at the outset and developed progressive disease despite sorafenib therapy.

Comprehensive treatment of unresectable cardiac angiosarcoma: A case report and review of literature.

Cardiac angiosarcoma is a rare but lethal tumor that is difficult to diagnose and treat, due to its rapid local relapse and high incidence of systemic metastasis. The prognosis of cardiac angiosarcoma is dismal, with a mean life expectancy of only a few months. We herein report a case of unresectable angiosarcoma arising from the right atrium. The patient received first-line chemotherapy with weekly paclitaxel, and second-line therapy with vinorelbine and bevacizumab upon disease progression. The progression-free survival was 6 months and the overall survival was 7 months. The patient eventually succumbed to respiratory failure. A study of the present case and a review of the relevant literature suggest that treatment decisions for unresectable locally advanced or metastatic cardiac angiosarcoma are difficult, as the published literature on this disease mainly consists of case reports without sufficient data. Therefore, further clinical trials specific to the treatment of unresectable cardiac angiosarcoma are warranted.

Clinical, histological, and immunohistochemical features of a mandibular metastasis from a primary cardiac angiosarcoma

Primary cardiac angiosarcoma is an extremely rare malignant tumor. Distant metastases are common at the time of diagnosis but have never been reported in the jaw. A 45-year-old female patient with primary cardiac angiosarcoma was referred for dental care due to pain in the mandibular alveolar ridge. Oral examination revealed a red-violet lesion that was soft on palpation and had been present for 3 months. Histological analysis confirmed the diagnosis of metastatic cardiac angiosarcoma. The patient died of multiple metastases.

Cardiac Angiosarcoma-Associated Membranoproliferative Glomerulonephropathy

Primary cardiac angiosarcoma is a rare cardiac tumor. The initial clinical course is often asymptomatic, and metastatic disease is present in a majority of affected patients at diagnosis. We present a patient who presented with a hemorrhagic pericardial effusion. No malignant cells were evident on cytological examination. He subsequently developed membranoproliferative glomerulonephritis requiring hemodialysis. Metastatic cardiac angiosarcoma was diagnosed 5 months later. To our knowledge, this is the first reported case of paraneoplastic membranoproliferative glomerulopathy associated with cardiac angiosarcoma.

Cardiac Angiosarcoma Presenting With Right Coronary Artery Pseudoaneurysm

ANGIOSARCOMA OF THE HEART, although rare, is the most common primary malignant cardiac tumor. 1 Yu K. Liu Y. Wang H. et al. Epidemiological and pathological characteristics of cardiac tumors: A clinical study of 242 cases. Interact Cardiovasc Thorac Surg. 2007; 6: 636-639 Crossref PubMed Scopus (121) Google Scholar Prognosis is poor regardless of treatment, although survival is longest when surgical resection is possible. 2 Putnam Jr, J.B. Sweeney M.S. Colon R. et al. Primary cardiac sarcomas. Ann Thorac Surg. 1991; 51: 906-910 Abstract Full Text PDF PubMed Scopus (262) Google Scholar , 3 Simpson L. Kumar S.K. Okuno S.H. et al. Malignant primary cardiac tumors: Review of a single institution experience. Cancer. 2008; 112: 2440-2446 Crossref PubMed Scopus (204) Google Scholar Diagnosis is usually delayed because this tumor is typically associated with a variety of nonspecific symptoms. 3 Simpson L. Kumar S.K. Okuno S.H. et al. Malignant primary cardiac tumors: Review of a single institution experience. Cancer. 2008; 112: 2440-2446 Crossref PubMed Scopus (204) Google Scholar , 4 Donsbeck A.V. Ranchere D. Coindre J.M. et al. Primary cardiac sarcomas: An immunohistochemical and grading study with long-term follow-up of 24 cases. Histopathology. 1999; 34: 295-304 Crossref PubMed Scopus (163) Google Scholar The authors describe a patient with metastatic cardiac angiosarcoma whose presentation before diagnosis included a pericardial hematoma as a result of a coronary artery pseudoaneurysm.

Cardiac angiosarcoma: a case report and review of the literature

We present the case of a 36-year-old woman with widespread metastatic cardiac angiosarcoma to the lungs and the liver. She was treated with preoperative chemotherapy and underwent extensive surgical resection of the cardiac mass and is currently doing well. Analysis of the effectiveness of various treatment modalities from cases which have been reported has also been reviewed and discussed.

Lobular distributed ground-glass opacification due to metastatic cardiac angiosarcoma: High resolution CT, MIP, and pathologic findings

We report the case of a 48-year-old man with a recent history of cardiac tamponade, who presented 6 months later recurrent hemoptysis of small volume. The CT scan revealed a bilateral pulmonary infiltration associating areas of ground-glass opacification of lobular distribution and tiny pulmonary nodules. Maximum intensity projection reconstructions were useful to better characterize the pulmonary infiltrates highlighting the CT halo sign. Contrast enhanced CT depicted a mass involving the right atrium and pericardium, confirmed by cardiac MRI. The surgical biopsy allowed radiological–pathological correlation revealing epithelioid angiosarcoma of the right atrium with pulmonary metastases.