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

Abstract

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.

Primary Cardiac Angiosarcoma: A Case Report and Review of the Literature

“…Angiosarcoma is the most common histologic subtype in primary cardiac malignant tumor among adults 1,2 , which usually occurs in men aged under 65 years, with a male to female ratio of around 2-3:1 4,6 . The most vulnerable site is in the right atrium, for 74% of primary cardiac angiosarcoma occur in the right atrium and often interfere with adjacent structures 2 .…”

“…At present, advanced inspection technology have been applied in the detection of heart tumors, such as tumor markers, echocardiography, CT, MRI, and PET/CT. Above all, echocardiography valued as the most effective way for the test, but heart tissue biopsy is the golden standard [2][3][4][5] . In this case, it was the echocardiogram that prompted the abnormal position of the heart.…”

“…Highly malignant tumors with local infiltration and systemic metastasis lead to poor prognosis of cardiac angiosarcoma. The survival time of the patients is only 5 to 13 months 2,3 . The rarity of this diagnosis makes it hard to regulate standardized treatments.…”

“…Primary cardiac angiosarcoma is a rare tumor, but it is the most common histologic subtype in primary cardiac malignant tumor in adults 1,2 . Although advanced technologies have been applied to diagnose heart tumors, such as tumor markers, echocardiography, computer tomography (CT), magnetic resonance imaging (MRI), and positron emission tomography/computer tomography (PET/CT), cardiac tumor are still easily misdiagnosed as other diseases at the onset of disease.…”

“…Clinicians try everything possible such as surgical therapy and other comprehensive treatments to actively treat primary cardiac angiosarcoma. However, due to the highly aggressive of this disease, the patient's outcome is often poor 2,3 .…”