Improved outcome with multimodality therapy in primary cardiac angiosarcoma

Pigott, Courtney; Welker, Michael; Khosla, Pam; Higgins, Robert S.D.

doi:10.1038/ncponc1023

Cited by 37 publications

(22 citation statements)

References 7 publications

(4 reference statements)

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“…9) Systemic symptoms only, such as weight loss, fever, night sweats, and chills occur in approximately 10% of these patients in the absence of cardiac symptoms. 10) These nonspecific symptoms, the rarity of the disease, and its rapid progression, with tumor metastases to multiple organs, are responsible for the late diagnosis and poor prognosis. Therefore, it is important to make a diagnosis at early stages of the disease.…”

Section: Discussionmentioning

confidence: 99%

Cardiac Angiosarcoma Diagnosed by Transvenous Endomyocardial Biopsy With the Aid of Transesophageal Echocardiography and Intra-Procedural Consultation

et al. 2010

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SummaryWe report a case who had confirmed tumor cells in the biopsy specimens by transvenous endomyocardial biopsy with intra-procedural consultation and fast smear cytology. A 57-year-old female was admitted to our hospital because of shortness of breath and left back pain. Transthoracic echocardiography (TTE) and contrast-enhanced computed tomography (CT) scans demonstrated a large mass in the right atrium and multiple liver tumors thought to be due to spread of the disease. Coronary angiography showed the right coronary artery was involved in the mass. In order to confirm the histological diagnosis, we attempted transvenous endomyocardial tumor biopsy under fluoroscopic guidance. However, we failed to obtain adequate tissue material. Due to several risks associated with a surgical procedure such as an open surgical biopsy, transvenous endomyocardial tumor biopsy was again attempted with the aid of transesophageal echocardiography (TEE). Intra-procedural consultation and fast smear cytology enabled us to finish the procedure. Hematoxylineosin stained sections demonstrated spindle-shaped cells. Immunohistochemical stains of these cells were positive for anti-factor VIII antigen, CD31, and CD34. These findings indicated a definite diagnosis of angiosarcoma. Since there was no surgical indication for this tumor, the patient underwent chemotherapy with docetaxel and radiotherapy. Three months later, CT scans showed a reduction in the size of the cardiac tumor. (Int Heart J 2010; 51: 367-369) Key words: Primary cardiac angiosarcoma, Transvenous endomyocardial biopsy, Transesophageal echocardiography, Intra-procedural consultation, Smear cytology P rimary cardiac tumors are extremely rare and difficult to diagnose. A final diagnosis depends on histopathological confirmation. At the time of discovery, up to 75% have systemic metastases 1) and the effect of surgical resection is only temporary, 2) and hence the prognosis is relatively poor. A pathological diagnosis with cardiac biopsy is established antemorten in only 50% of patients.3) The specimen material is usually obtained by open biopsy or surgical resection. There have been reports of transvenous biopsy for cardiac tumors under transesophageal echocardiography (TEE) guidance, 4-7) although sometimes specimen material is not successfully obtained. 8) Case ReportA 57-year-old woman was admitted to our hospital because of shortness of breath and left back pain. She had been healthy previously without recent chest pain or pyrexia, and she denied any exposure to vinyl chloride. On physical examination, her heart rate was 94/minute, blood pressure was 90/54 mmHg, and SpO 2 was 98% with a 10 L/minute O 2 . Jugular vein dilatation and hepatomegaly were detected. Neither heart murmur nor lung rales were detected. The electrocardiogram showed sinus tachycardia (94 beats/minute), low voltage in limb leads, and a QS pattern in V 1 and V 2 leads. Chest X-rays revealed cardiomegaly and bilateral pleural effusion. Transthoracic echocardiography (TTE) showed reasonable left ve...

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Section: Discussionmentioning

confidence: 99%

Cardiac Angiosarcoma Diagnosed by Transvenous Endomyocardial Biopsy With the Aid of Transesophageal Echocardiography and Intra-Procedural Consultation

et al. 2010

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“…Cardiac transplantation has been performed, but the use of immunosuppressant drugs is a limiting factor because it predisposes to tumor recurrence and metastases (11). The appropriate evidence-based treatment guidelines are still not established because of the rarity of the tumor, although multimodality palliative therapy with surgery and chemotherapy may be useful (12). In the present case, metastasis had already exsited, complete surgical excision was not achieved and no other therapy modality was used, so survival time was limited.…”

Section: Discussionmentioning

confidence: 93%

A Right Heart Angiosarcoma with Rapidly Progressing Hemorrhagic Pericardial Effusion

2011

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Primary tumors of the heart are rare. Among them angiosarcoma is the most common malignant tumor of the heart and is characterized by rapid growth, local invasion, and distant metastasis. Its clinical presentation is variable. We describe a 66-year-old man who suffered from cough and hemoptysis and then experienced rapidly progressing hemorrhagic pericardial effusion. After a set of examinations he was diagnosed as right heart angiosarcoma. His outcome was poor.

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“…If surgical resection is not possible, median survival is approximately 6 months (7,17). In one case report of a metastatic patient, multimodality palliative treatment with neoadjuvant and adjuvant chemotherapy and surgery improved survival (18). The patient presented herein had minimal symptomatic disease for about 8 months with multimodality treatment including incomplete resection and chemotherapy.…”

Section: Discussionmentioning

confidence: 99%

Primary Cardiac Angiosarcoma: A Case Report

Durnalı¹,

Yüksel²,

Altundag³

et al. 2015

Acta Oncol Tur.

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ÖZETPrimer kardiyak anjiosarkoma, kötü prognozlu nadir bir hastalıktır. Açık kardiyak cerrahi operasyon olan ve sağ atriumda kardiyak anjiosarkoma tanısı alan 34 yaşında erkek hasta tıbbi onkoloji bölümüne başvurdu. Tümörün tam küratif rezeksiyonu mümkün olmamıştı ve hastaya postoperatif dönemde 6 siklus kombinasyon kemoterapi rejimi (doksorubisin ve mesna korumasıyla ifosfamid) verildi. Hastada 8 ay boyunca stabil hastalık mevcuttu ve progresyon sonrasında ikinci sıra kemoterapi (paklitaksel) başlandı, ancak hasta tanıdan 15 ay sonra kaybedildi. Kardiyak anjiosarkoma olgularının çoğunda küratif rezeksiyonlar mümkün olmadığından daha ileri tedavi seçeneklerine son derece ihtiyaç vardır. Anahtar Kelimeler: Kardiyak anjiosarkoma, Kemoterapi ABSTRACT Primary cardiac angiosarcoma is a rare disease with poor prognosis. A 34-year-old male patient who underwent an open cardiac surgery and diagnosed as cardiac angiosarcoma on the right atrium was admitted to the medical oncology department. Complete curative resection of the tumor was not possible and postoperatively 6 cycles of combination chemotherapy regimen (ifosphamide with mesna protection and doxorubicin) was given. Patient had stable disease for 8 months, and after progression a second-line chemotherapy (paclitaxel) was started but he died after 15 months of diagnosis. Since curative resections are not possible in most of the cardiac angiosarcoma cases, further treatment options are desperately needed.

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Improved outcome with multimodality therapy in primary cardiac angiosarcoma

Abstract: Neoadjuvant and adjuvant chemotherapy with doxorubicin and complete surgical resection of the tumor with reconstruction of the right atrium using bovine pericardium.

Cited by 37 publications

References 7 publications

Cardiac Angiosarcoma Diagnosed by Transvenous Endomyocardial Biopsy With the Aid of Transesophageal Echocardiography and Intra-Procedural Consultation

Cardiac Angiosarcoma Diagnosed by Transvenous Endomyocardial Biopsy With the Aid of Transesophageal Echocardiography and Intra-Procedural Consultation

A Right Heart Angiosarcoma with Rapidly Progressing Hemorrhagic Pericardial Effusion

Primary Cardiac Angiosarcoma: A Case Report

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