INTRODUCTION: Angiosarcoma is the most common primary malignant heart tumor with frequent metastasis to lungs at the time of diagnosis. However it is a rare cause of hemoptysis.
CASE PRESENTATION: A 50 year old previously healthy man with a 20-pack year smoking history was transferred for management of cough of one month duration and hemoptysis over the previous few days. The cough, which was initially productive of only thick white sputum, now demonstrated blood streaking. He also complained of low grade, subjective fevers and mild exertional dyspnea. He did not have chest pain, diaphoresis, lower extremity edema or swelling, tachycardia, or nasal symptoms. His physical exam was unremarkable. A CT scan with contrast demonstrated multiple bilateral pulmonary emboli and diffuse lung nodules with surrounding pulmonary infiltrates (Fig1.a.b.c). Routine laboratory data were unremarkable. Given concern for intraparenchymal bleeing due to the nodular process, the decision was made to place an inferior vena cava filter and forgo anticoagulation. A Bronchoscopy revealed normal appearing bronchial mucosa throughout both lungs. Bronchoalveolar lavage revealed sanguinous return from the right middle lobe bronchial segments consistent with alveolar hemorrhage. Bronchoalveolar lavage, fine needle aspiration of subcarinal lymph nodes and transbronchial biopsies were performed and were non-diagnostic. The work up for connective tissue diseases including Wegner's granulomatosis was negative as well. Over the subsequent few days, the patient appeared to be improving with cessation of his hemoptysis. An S3 gallop was auscultated and elevated jugular venous pressure was noted. An echocardiogram demonstrated a pericardial effusion and suggestive tamponade physiology. A pericardiocentesis revealed hemorrhagic fluid, and a subsequent pericardial window with simultaneous biopsy of lung nodules were performed. The lung biopsy revealed (Fig2) extensive involvement of the pulmonary vasculature with epithelioid and spindle-shaped cells, the majority of which had a high nuclear pleomorphism and mitotic rates, forming rudimentary vascular channels. The pulmonary vessels were filled with tumor emboli. Immunohistochemistry demonstrated the neoplastic cells to be positive for the endothelial cell markers CD31 and CD34 and negative for the epithelial cell marker cytokeratin supporting the diagnosis of angiosarcoma. A follow up echocardiogram was performed to assess the effectiveness of the pericardial window and a right atrial mass was suspected. Cardiac CT angiography revealed the mass to be located around right atrium and ventricle and encasing the right coronary artery consistent with a diagnosis of primary cardiac angiosarcoma with lung metastasis. The patient was enrolled in a clinical trial using sorafenib as the primary chemotherapeutic modality.
DISCUSSIONS: Angiosarcoma is a rare tumor of mesenchymal origin more commonly seen in males between the third and fifth decades of life. Cardiac angiosarcomas are most commonly found in the right atrium in contrast to benign tumors which are found in the left atrium. Clinical diagnosis of this rare entity is often difficult due to vague symptomatology at presentation. Although most of the cases are accompanied by hemoptysis, the non-specificity of this symptom leads to angiosarcoma rarely being considered in the differential diagnosis. In most of the cases, metastases are present at the time of diagnosis and are predominantly found in the lungs, liver, bone, lymph nodes, and central nervous system. Angiosarcoma carries a poor prognosis with a mean survival of less than a year following diagnosis. Effective treatment strategies have yet to be established.
CONCLUSION: Cardiac angiosarcoma, though a rare condition, should appear in the differential diagnosis of otherwise healthy patients presenting with hemoptysis and multiple pulmonary nodules. Optimal treatment strategies have not been developed.
DISCLOSURE: Sobia Farooq, No Financial Disclosure Information; No Product/Research Disclosure Information