Cardiac Angiosarcoma Research Articles

Primary Cardiac Angiosarcoma with Spontaneous Ruptures of the Right Atrium and Right Coronary Artery

Primary tumors of the heart are rarely seen. Cardiac angiosarcomas are malignant tumors that almost always have a poor prognosis. Atrium rupture and coronary artery fistula are very rare complications of primary cardiac angiosarcoma. We describe a 57-year-old man suffering from primary cardiac angiosarcoma with spontaneous ruptures of the right atrium and right coronary artery (RCA). Theoretically, either of these ruptures invariably results in pericardial effusion and tamponade that is rare but potentially life threatening. In this instance, however, the patient might have developed fibrous adhesions resulted from previous bloody pericardial effusion. A massive pericardial effusion was localized, which consequently prevented cardiac tamponade and hemodynamic collapse. Echocardiography revealed the tumor progression leading to detectable infiltration of solid mass into the right atrial (RA) wall, which is close to RCA. And color Doppler displayed the flow into the pericardial cavity through a disrupted RA wall and perforated RCA. Echocardiography remains the primary method of choice for evaluation of cardiac masses.

回転原体照射による術後放射線療法が奏功した心臓血管肉腫の1例

心臓血管肉腫は稀な疾患であり，生命予後はきわめて不良とされている．われわれは，術後放射線治療が奏功し長期生存が得られている症例を経験したので報告する．症例は71歳，女性．主訴は全身倦怠感，下腿浮腫．経胸壁心エコーおよびCTで多量の心嚢液貯留と右房に腫瘤を認め，手術を行った．右房と下大静脈接合部に大きな血腫を伴う腫瘍を認め，右房壁，下大静脈，横隔膜，右側心膜に浸潤していた．完全切除を断念し，体外循環下に右房壁を一部切除し右房壁の欠損部をウシ心膜パッチで修復した．術後病理検査で，心臓血管肉腫の診断であり，切除断端陽性であった．術後11日目から術後放射線療法（回転原体照射10MV-X線，54 Gy/18回/4.5週間の分割照射（通常分割64 Gy相当））を行った．照射終了後1カ月のCTで腫瘍の縮小が見られ，照射18カ月後の心エコーでは腫瘍は痕跡程度の残存のみでほぼ消失していた．放射線治療の発達により，術後放射線療法が原発巣の局所制御に有効である可能性があり，完全切除が不可能であっても，術後放射線療法を行うことでquality of lifeの維持および生命予後の改善が期待できると考えられた．

Unusual treatment of postoperative bleeding after cardiac surgery

A young patient was presented to the emergency department with chest pain and palpitations. A transthoracic echocardiogram showed a right atrial mass. Coronary angiography showed a right coronary artery with collateral circulation to a large mass. The tumor could only be partially resected and the patient experienced persistent postoperative bleeding. We performed a new right coronary artery angiography which showed an important free extravasation of contrast into the pericardium through the collateral circulation. Using covered stents, the bleeding was controlled. The pathological examination performed later revealed a primary cardiac angiosarcoma. After asymptom-free survival of 14 months the patient presented bone metastases.

Cardiac angiosarcoma in a pregnant woman: a case report and review of the literature

Cardiac angiosarcomas, although very rare, are the most common primary malignant tumours. They are characterized by no specifi c clinical fi ndings, rapid growth with frequent metastasis at the time of diagnosis and a reduced response to treatment. We describe the case of a 34-year-old pregnant woman who began to complain of dyspnoea and palpitations at 32 weeks of gestation. The echocardiogram showed a mass in the right atrial cavity and the CT scan showed extensive local invasion and hepatic metastasis. After 4 weeks, because of progressive heart failure due to obstruction of the tricuspid valve by the mass, the patient was submitted to an urgent caesarean section followed, two days later, by palliative cardiac surgery in order to alleviate the symptoms. Adjuvant chemotherapy was performed.

Ruptured cardiac angiosarcoma with pulmonary metastases: A rare disease with a common (mis)diagnosis!

In Indian settings pulmonary tuberculosis remains the most common diagnosis in a patient presenting with constitutional symptoms, hemoptysis and lung opacities. We describe a case report of a fifty-year-old woman who was receiving empirical anti-tubercular drugs for a metastatic illness to lungs arising from a primary angiosarcoma in the right atrium. This rare entity was misdiagnosed and typical echocardiographic findings suggested this diagnosis.

Metabolic activity assessment of cardiac angiosarcoma by 18FDG PET-CT

Angiosarcoma is a neoplasm of an endothelial origin, whichcould arise from blood or lymphatic vessels, respectively hemangiosarcomaor lymphosarcoma.

Internal cardiac defibrillator implant-associated angiosarcoma presenting as suspected implant pouch infection

Medical implants have been rarely associated with the development of sarcomas. We report, to our knowledge, the first case of a high-grade epithelioid angiosarcoma developing in the capsule of an internal cardiac defibrillator implanted subcutaneously over the abdomen over 20 years ago. Although the internal cardiac defibrillator generator had been exchanged in the intervening years, recently, the patient showed a year-long history of systemic symptoms suspicious for implant infection, necessitating several internal cardiac defibrillator pocket hematoma evacuations, always with negative microbiologic cultures. Final reexploration identified suspicious tissue with excessive bleeding which was biopsied and proven to be angiosarcoma on histologic examination. Subsequent imaging demonstrated precipitous progression to liver and lung metastases. Though rare, foreign-body-related sarcoma should be considered in the differential diagnosis for presentations suggestive of hemorrhagic or infectious sequelae of medical implants; this case broadens the range of medical devices associated with angiosarcoma to include now very commonly used implantable medical devices.

Metabolic activity assessment of cardiac angiosarcoma by 18FDG PET-CT

Metabolic activity assessment of cardiac angiosarcoma by 18FDG PET-CT

Cardiac Angiosarcoma Management and Outcomes: 20-Year Single-institution Experience

To identify the clinicopathologic characteristics, treatments, and outcomes of a series of patients with primary cardiac angiosarcoma (AS). This retrospective case series was set in a tertiary referral center with a multidisciplinary clinic. Consecutive patients with institutionally confirmed pathologic diagnosis of cardiac AS from January 1990 to May 2011 were reviewed. Main outcome measures included patient demographics, tumor characteristics, management strategies, disease response, and survival. Data from 18 patients (78 % male) were reviewed. Sixteen patients (89 %) had AS originating in the right atrium. At diagnosis, eight patients (44 %) had localized/locally advanced disease and ten patients (56 %) had metastatic disease. Initial treatment strategies included resection (44 %), chemotherapy (39 %), and radiotherapy (11 %). Of the eight patients with localized/locally advanced AS, two underwent macroscopically complete resection with negative microscopic margins, one underwent macroscopically complete resection with positive microscopic margins, one underwent macroscopically incomplete resection, two received chemotherapy followed by surgery and intraoperative radiotherapy, one received chemotherapy alone, and one died before planned radiotherapy. Median follow-up was 12 months. Median overall survival (OS) was 13 months for the entire cohort; median OS was 19.5 months for those presenting with localized/locally advanced AS and 6 months for those with metastatic disease at presentation (p = 0.08). Patients who underwent primary tumor resection had improved median OS compared with patients whose tumors remained in situ (17 vs. 5 months, p = 0.01). Cardiac AS is associated with poor prognosis. Resection of primary tumor should be attempted when feasible, as OS may be improved. Nevertheless, most patients die of disease progression.

Primary cardiac angiosarcoma and diffuse pulmonary hemorrhage: a case report

Primer kardiyak anjiyosarkom, cok kotu seyreden, oldukca nadir gorulen kotu huylu bir tumordur. Hastalik semptomlari nonspesifiktir. Ender klinik gorunumlerinden biri de, diffuz pulmoner hemorajidir. Altmis bir yasinda erkek olgu, 10 gundur devam eden sag yan agrisi, oksuruk ve kan tukurme yakinmalari ile klinigimize basvurdu. Akciger grafisinde iki tarafli noduler lezyonlar ve kardiyotorasik indekste artis izlendi. Toraks bilgisayarli tomografide, kardiyomegali, sag atrium anterolateral komsulugunda 7x5 cm kitle lezyonu, her iki akcigerde orta ve alt zonlarda buzlu cam gorunumleri izlendi. Ekokardiyografide sol ventrikul dilate ve hipertrofik idi. Olgu miksoma on tanisi ile ameliyat edildi. Ameliyat sirasinda kardiyak anjiyosarkom saptandi. Hasta, ameliyattan kisa bir sure sonra, solunum yetmezligi nedeniyle kaybedildi. Bu makalede, birtakim tanisal zorluklar ile birlikte, pulmoner semptomlar goz onunde bulundurularak yurutulen tanisal surecin ayrintili olarak irdelenmesi amaciyla bir olgu calismasi sunuldu. Anah tar soz cuk ler: Anjiyosarkom; diffuz pulmoner hemoraji; kalp tumoru. Primary cardiac angiosarcoma is a very rare malignant tumor with very poor prognosis. Symptoms of the disease are nonspecific. One of the rare clinical presentations is diffuse pulmonary hemorrhage. A 61-year-old male was admitted to our clinic with the complaints of right side pain lasting for 10 days, cough and hemoptysis. The chest X-ray showed bilateral nodular lessions and increased cardiothoracic index. Thoracic computed tomography revealed cardiomegaly, showing a mass in 7x5 cm in the anterolateral right atrium with ground-glass appearance, predominantly in the mid and lower zones of bilateral lung fields. Echocardiography showed dilatation and hypertrophy of the left ventricle. The case underwent surgery with the initial diagnosis of myxoma. During surgery, cardiac angiosarcoma was diagnosed. The patient died due to respiratory failure soon after surgery. In this article, we present a case report, discussing the details of the diagnostic process based on pulmonary symptoms along with certain challenges.

Cardiac Angiosarcoma

Cardiac angiosarcoma is the most common of the cardiac sarcomas. It can infiltrate into adjacent structures in addition to seeding distant metastases. Coronary artery encasement is a rare but potentially life-threatening complication of cardiac angiosarcoma. We report a case of cardiac angiosarcoma encasing the right coronary artery with subsequent pseudoaneurysm formation with mural thrombus. Magnetic resonance first-pass, contrast-enhanced images are very useful for defining the communication between such lesions and underlying cardiac chambers. In the presence of distant metastases, there are limited treatment options. No intervention was attempted for the pseudoaneurysm, and thus far (12 mo) it has been stable.

Cardiac Angiosarcoma Presenting with Tamponade

Primary cardiac angiosarcoma is extremely rare, but it is the most common primary malignant cardiac tumor. We herein present the case of a 51-year-old man who presented with symptoms of acute right heart failure, secondary to pericardial tamponade. Pericardiocentesis showed bloody fluid with negative pathology. Repeat 2-D echocardiography and a trans-esophageal echocardiogram showed a right atrial mass. The patient underwent surgery and adjuvant chemotherapy, but died seven months after the diagnosis. Despite being rare, cardiac angiosarcoma should be included in the differential diagnosis of bloody pericardial effusions, even with negative early investigations. The prognosis of the disease is usually poor. Treatment is mainly surgical resection if the cancer is localized, and can include neadjuvant and adjuvant chemotherapy, radiation treatment, and immunotherapy.

Angiosarcomas of the Bilateral Breast and Heart: Which One is the Primary Site?

A 29-year-old pregnant woman with recurrent pericardial effusion and a cardiac tumor, diagnosed as an angiosarcoma, was treated with surgical resection of the tumor followed by radiotherapy. Immediately after completion of radiotherapy, she developed bilateral breast masses, which were also confirmed as angiosarcomas. We thought this might be the first case of bilateral angiosarcoma of the breast metastasizing to heart mimicking a primary cardiac angiosarcoma, although we could not conclude with certainty that angiosarcoma of the heart was not the primary site.

Cardiac angiosarcoma presenting as progressive dyspnea on exertion

Chenier, Michael; Johnson, Douglas; Ohman, Magnus; Pavlisko, Elizabeth Author Information

Clinicopathologic and immunohistochemical characteristics of adult primary cardiac angiosarcomas: analysis of 10 cases

Primary cardiac angiosarcoma is a rare but the most common malignant neoplasm of the heart in adults. The objective of this study is to analyze the clinicopathologic characteristics of primary cardiac angiosarcoma. Ten cases of primary cardiac angiosarcoma treated in a single institution were analyzed for their clinical, pathologic, and immunohistochemical features. There were 6 men and 4 women, with a mean age of 40 years (range, 20-61 years). The patients commonly presented with dyspnea and distant metastasis. All tumors were located in the right atrium, with a mean tumor size of 6.8 cm. Tumors were hemorrhagic, with variegated tan-brown solid areas. Histologically, they exhibited high-grade morphology with mixed solid growth and anatomizing channels. Frequent mitoses and tumor necrosis were common. The tumors were strongly positive for CD31, CD34, FLI-1, and WT-1 but negative for AE1/3, D2-40, human herpesvirus 8, and epidermal growth factor receptor. The tumor cells were focally reactive to p53, with a high rate of Ki-67 expression. A complete tumor resection was not possible in any of the patients because of the size or extensive local invasion of the tumor. Overall survival ranged from 1 to 81 months (mean, 26.6 months) after initial histologic diagnosis. Primary cardiac angiosarcomas are rare tumors that commonly arise in the right atrium. The mean age is much younger than that of soft tissue angiosarcoma. Regional tumor extension and distant metastasis are extremely common at the time of diagnosis. Surgical resection with adjuvant chemotherapy is currently the preferred treatment, and survival time appears to be inversely correlated with the tumor size and degree of regional tumor extension at the time of surgery.

Angiosarcoma Arising from Right Atrium: Remarkable Response to Concurrent Chemoradiotherapy with Carboplatin and Paclitaxel

We read with interest the case report by Fehr et al.1Fehr M Kuhn M Mayer K et al.Metastatic angiosarcoma arising from the right atrium: unusual presentation and excellent response to treatment in a young patient.J Thorac Oncol. 2010; 5: 1301-1302Abstract Full Text Full Text PDF PubMed Scopus (3) Google Scholar and the concise review by Orlandi et al.2Orlandi A Ferlosio A Roselli M et al.Cardiac sarcomas: an update.J Thorac Oncol. 2010; 5: 1483-1489Abstract Full Text Full Text PDF PubMed Scopus (76) Google Scholar regarding cardiac sarcomas. Concurrent chemoradiotherapy (CRT) to cardiac angiosarcomas has been scarcely documented, and only one case was reported with the benefit from concurrent CRT with docetaxel.3Nakamura-Horigome M Koyama J Eizawa T et al.Successful treatment of primary cardiac angiosarcoma with docetaxel and radiotherapy.Angiology. 2008; 59: 368-371Crossref PubMed Scopus (28) Google Scholar Although efficacy of weekly paclitaxel (PTX) for unresectable angiosarcoma has been demonstrated,4Penel N Bui BN Bay JO et al.Phase II trial of weekly paclitaxel for unresectable angiosarcoma: the ANGIOTAX Study.J Clin Oncol. 2008; 26: 5269-5274Crossref PubMed Scopus (387) Google Scholar CRT with weekly PTX was not reported before the report by Fehr et al. We present herein a case of metastatic angiosarcoma arising from the right atrium, which remarkably responded to concurrent CRT with weekly carboplatin and PTX. A 40-year-old man was referred to our institute, presenting a tumor in the right atrium and multiple bilateral lung nodules with the computed tomography (Figures 1A, B). Histology obtained by wedge resection of a lung nodule revealed a high-grade epithelial angiosarcoma, thus the patient was diagnosed with angiosarcoma from right atrium with multiple lung metastases. We judged it to be unresectable and decided to perform concurrent CRT (30 fractions of 2Gy) with weekly carboplatin (area under the curve = 2) and PTX (60 mg/m2) for 6 consecutive weeks. The radiation field was limited to the primary cardiac tumor. After the therapy, almost all bilateral pulmonary nodules disappeared, and a large tumor in the right atrium was markedly reduced (Figure 2A, B). Although grade 2 esophagitis and neutropenia were observed, the treatment was generally well tolerated. No evidence of progression has been confirmed after more than 5 months of follow-up.FIGURE 2A, Almost all bilateral pulmonary nodules disappeared 3 months after the initiation of chemoradiation therapy. B, The cardiac tumor in the right atrium was remarkably reduced 3 months after the initiation of chemoradiation therapy.View Large Image Figure ViewerDownload (PPT) Multimodality treatment combined with surgery and adjuvant chemotherapy and/or radiotherapy is indicated as the standard care for patients with cardiac angiosarcomas. Nevertheless, the majority of patients with cardiac sarcoma are already unresectable and metastatic disease at their initial diagnosis and have a poor prognosis. CRT is desirable in some cases with cardiac sarcoma to control both the primary cardiac tumor and metastatic lesions. In our case, a combined regimen including PTX was chosen to obtain better response of metastatic lesions, and good response was clearly observed. Moreover, the efficacy and tolerability of concurrent CRT with carboplatin + PTX have been recently demonstrated in locally advanced non-small cell lung cancer.5Yamamoto N Nakagawa K Nishimura Y et al.Phase III study comparing second- and third-generation regimens with concurrent thoracic radiotherapy in patients with unresectable stage III non-small-cell lung cancer: West Japan Thoracic Oncology Group WJTOG0105.J Clin Oncol. 2010; 28: 3739-3745Crossref PubMed Scopus (206) Google Scholar To our knowledge, this is the first report demonstrating the efficacy of concurrent CRT with carboplatin + PTX for cardiac angiosarcoma. Nevertheless, there is no other evidence of CRT with doublet regimens. Future studies to explore usefulness of concurrent CRT for cardiac angiosarcomas are warranted.

Primary cardiac angiosarcoma treated by complete tumor resection with cardiac reconstruction

Primary cardiac sarcoma is a rare malignant neoplasm, with an incidence of .0001% in collected autopsy series. Angiosarcoma is the most common cardiac sarcoma and is present in up to 33% of the cases that are associated with a poor prognosis. Because angiosarcoma is essentially not responsive to current regimens of chemotherapy and irradiation, early complete resection is recommended as the treatment choice. However, complete resection is difficult because of the limited amount of myocardium and expansion of the tumor at the time of diagnosis. We report a case of right atrial angiosarcoma treated by complete tumor resection with cardiac reconstruction with a bovine pericardium patch.

Successful surgical excision of primary right atrial angiosarcoma

Primary cardiac angiosarcoma is a rare and aggressive tumor with a high incidence of metastatic spread (up to 89%) at the time of diagnosis, which restricts the indication for surgical resection to a small number of patients. We report the case of a 50-year old Caucasian woman with non-metastatic primary right atrial angiosarcoma, who underwent successful surgical excision of the tumor (with curative intent) and reconstruction of the right atrium with a porcine pericardial patch. However, after a symptom-free survival of five months the patient presented with bone and liver metastases without evidence of local tumor recurrence.

Echocardiography in differential diagnosis of chest pain and elevated cardiac biomarkers: a case of cardiac angiosarcoma

Angiosarcoma is the most common primary cardiac malignancy with a poor prognosis. In this report, we describe a 28-year-old male patient presenting with symptoms and findings of myocardial ischaemia who has been finally diagnosed with cardiac angiosarcoma.

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.