Primary cardiac angiosarcoma: a prolonged response to surgical resection followed by concurrent chemoradiotherapy with docetaxel

Jang, Youngwoo; Kim, Joonhwan; Shim, Jung Ho; Kim, Eun Young; Moon, Jungkeun; An, Jungsuk; Jeon, Yang Bin; Lee, Kyu Chan; Ahn, Hee Kyung

doi:10.1186/s40064-016-2248-8

Cited by 13 publications

(13 citation statements)

References 15 publications

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“…Primary cardiac angiosarcomas have a poor prognosis with median survival rates ranging from 6 to 11 months ( 11 ). Survival after surgical intervention has a median of 14 months ( 11 ), while with systemic metastasis has only 3.8 ± 2.5 months' difference without surgical resection ( 12 ). Surgery is a commonly chosen therapy, particularly in the setting of localized disease, in that it seems to provide the best option for palliative care and potential cure ( 13 ).…”

Section: Discussionmentioning

confidence: 99%

Case Report: Thromboembolism and Hemorrhagic Pericardial Effusion—The Janus Face of Primary Pericardial Angiosarcoma

Chen

Jiang

Dong

et al. 2021

Front. Cardiovasc. Med.

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Background: Primary cardiac angiosarcomas, especially those originating in the pericardium, are extremely rare and aggressive tumors with poor prognosis. These types of malignant tumors have diverse clinical presentations and are often masked by other comorbidities.Case Summary: Our hospital reported a 59-year-old woman who initially presented with pulmonary thromboembolism (PTE) and was subsequently treated with low-molecular-weight heparin. However, she experienced acute pericardial tamponade after anticoagulation therapy, where no obvious mass was primarily identified upon imaging, both in the pericardium or within the heart. Emergency pericardiocentesis and drainage were performed, where a total of 210 mL of bloody effusion was drained. Four months later, she was hospitalized with progressive hemoptysis and dyspnea. A large mixed mass occupying the right pericardium was later identified by coronary computed tomography angiography (CCTA). The mass was consistent with the right atrium, with heterogeneous thickened pericardium and localized moderate pericardial effusion. CCTA and positron emission tomography scans later showed metastases in both lungs and bilateral pleura. Nodules in hilar and mediastinal lymph nodes were also significant. Ultrasound-guided biopsy was performed, and the patient was ultimately diagnosed with an angiosarcoma based on final positive results for both CD31 and CD34 markers. The patient refused chemotherapy and passed away while waiting for her pathology results. The patient survived for 6 months since the first reported episode of PTE.Conclusions: Our case indicates that patients presenting with both embolism and hemorrhage should urgently be channeled to a clinical specialist to confirm any malignant etiology. This would be beneficial to confirm an early diagnosis and lengthen the duration of patient survival. However, the diagnosis of primary cardiac angiosarcoma is still challenging and requires multiple imaging modalities and biopsies in order to assist the accurate diagnosis of disease and achieve effective patient management.

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Section: Discussionmentioning

confidence: 99%

Case Report: Thromboembolism and Hemorrhagic Pericardial Effusion—The Janus Face of Primary Pericardial Angiosarcoma

Chen

Jiang

Dong

et al. 2021

Front. Cardiovasc. Med.

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“…High doses of radiation could improve control of local tumor, but increases severe adverse events. [ 26 ]…”

Section: Discussionmentioning

confidence: 99%

“…[ 28 , 29 ] Recent studies showed that docetaxel has similar efficacy for metastatic angiosarcomas. [ 26 , 30 , 31 ] Gemcitabine was also effective for treating advanced angiosarcoma. [ 29 , 32 ] Moreover, the successful administration of molecular targeted therapy in some tumors indicated that it may have possible therapeutic benefit in angiosarcoma as well.…”

Section: Discussionmentioning

confidence: 99%

Right atrial epithelioid angiosarcoma with multiple pulmonary metastasis confirmed by multimodality imaging-guided pulmonary biopsy

et al. 2018

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Rationale:Primary cardiac tumors are very rare, and angiosarcoma accounts for about 33% of all primary malignant cardiac tumors. Primary cardiac epithelioid angiosarcoma is a highly aggressive and difficult to diagnose tumor, with early systemic metastasis and poor prognosis.Patient concerns:A 35-year-old Han male experienced sudden severe palpitation and moderate dyspnea. The patient received a whole body F-18 fluoro-deoxyglucose positron emission tomography (18F-FDG PET)/computed tomography (CT) scan, the scan showed a large mass in the right atrium (RA) and numerous pulmonary nodules in both lungs.Diagnoses:The patient was diagnosed as right atrial epithelioid angiosarcoma with multiple pulmonary metastasis by pulmonary biopsy through CT-guided percutaneous transthoracic fine needle aspiration.Interventions:The patient received a cycle of chemotherapy with docetaxel and gemcitabine, followed by another cycle with epirubicin and ifosfamide.Outcomes:The chemotherapy was ineffective. After the two cycles, the bilateral pleural effusion steadily increased, the patient had severe dyspnea and palpitation, and died three weeks later, with an overall survival of 2.5 months.Lessons:Primary angiosarcoma of heart is a very rare and aggressive disease, and its diagnosis and treatment are difficult. Most patients may have systemic metastasis at diagnosis, and have a very short survival without surgical resection. Hence, early diagnosis and surgical resection is extremely important to treat this disease.

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“…[ 20 ] Although, this case responds poorly to chemotherapy and targeted therapy, some of limited cases, after radical resection, a multidisciplinary approach with adjuvant chemotherapy, radiotherapy, and targeted therapy may improve overall survival even reaching 33 months. [ 21 – 23 ]…”

Section: Discussionmentioning

confidence: 99%

Primary cardiac angiosarcoma

Zhang¹,

Lu²,

Li³

et al. 2017

Medicine

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Background:Primary cardiac angiosarcoma is rare, but it is the most aggressive type of cardiac malignant tumor. When disease is confirmed it is often late in its stage resulting in a poor prognosis.Case presentation:We reported a 52-year-old woman who complained of dyspnea for 2 months and dry cough for 10 days. Computed tomography (CT), 18F-fluorodeoxyglucose (18F-FDG), positron emission tomography-computed tomography (PET-CT), and transthoracic echocardiography showed a space-occupying lesion 7 cm × 5 cm × 5 cm in the right atrium. Malignant cells can be seen in pericardial fluid by pericardiocentesis in local hospital, but not be diagnosed definitely. We conducted CT-guided biopsy of the tumor and observed the necrotic tissues. An exploratory thoracotomy was performed and histological examination confirmed angiosarcoma. Unable to be surgical excision due to infiltrating widely, the patients received chemotherapy with docetaxel for 2 cycles and the disease was progressive. Antiangiogenesis-targeted therapy was combined with chemotherapy for 3 months. Unfortunately, the tumor was still in progress. She died of respiratory failure, survived for 10 months.Conclusion:It is difficult to diagnose the primary cardiac angiosarcoma in early stage. Chemotherapy and target therapy are very limited in this case. Surgery remains the preferred treatment for patients with primary cardiac angiosarcoma. More efforts should be carried out on the effective diagnosis and multimodality treatment for this malignant disease.

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Primary cardiac angiosarcoma: a prolonged response to surgical resection followed by concurrent chemoradiotherapy with docetaxel

Cited by 13 publications

References 15 publications

Case Report: Thromboembolism and Hemorrhagic Pericardial Effusion—The Janus Face of Primary Pericardial Angiosarcoma

Case Report: Thromboembolism and Hemorrhagic Pericardial Effusion—The Janus Face of Primary Pericardial Angiosarcoma

Right atrial epithelioid angiosarcoma with multiple pulmonary metastasis confirmed by multimodality imaging-guided pulmonary biopsy

Primary cardiac angiosarcoma

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