An unusual case of hemoptysis.

Sir,

We read with great interest the article entitled “Metastatic angiosarcoma presenting as diffuse alveolar hemorrhage” by Rai et al., published in Lung India in 2008. Recently, we too came across a young 20-year-old female presenting with sudden onset hemoptysis and mild breathlessness. Her general physical examination showed no significant finding. Auscultation of chest revealed bilateral basal creptations. Heart sounds were diminished. Chest X-ray showed multiple ill-defined, small, nodular shadows in both lungs. Enlargement of cardiac silhouette was seen. Laboratory reports were within normal limits. Electrocardiogram (ECG) showed normal sinus rhythm with no significant abnormality. Fiberoptic bronchoscopy was done, which revealed blood in airways with no obvious endobronchial lesion or any active bleeder. Transthoracic echocardiography showed presence of pericardial effusion with ill-defined hypoechoeic lesion along wall of right atrium. Thickening of adjoining pericardium and septations in pericardial cavity were more clearly seen on transthoracic ultrasound [Figure 1]. Plain and contrast-enhanced computed tomography (CT) scan of chest showed multiple nodules (1.5-6 mm) diffusely scattered in both lungs [Figure 2]. Most of the nodules showed surrounding ground glass opacity. Ground glass opacity was more prominent in basal segments of both lower lobes. In addition there was an ill-defined, heterogeneously enhancing lesion in the wall of right atrium [Figure 3]. There was thickening of adjoining pericardium with associated pericardial effusion on anterior aspect. Based on imaging findings, possibility of primary cardiac tumor likely angiosarcoma with pericardial involvement and multiple pulmonary metastases was suggested. Pericardiocentesis revealed bloody aspirate with no cells. Open lung biopsy for pulmonary nodules was performed which confirmed the diagnosis of metastatic angiosarcoma. Histologic examination showed presence of highly pleomorphic malignant spindle and epitheloid cells arranged in thin-walled anastamosis like vascular channels. Immunohistochemical staining for CD34, CD31, and vimentin was positive. Disease prognosis was explained to the patient. Patient refused surgical resection of tumor and was referred for chemotherapy. Thereafter, patient did not come for follow-up.

Figure 1

(a) Transthoracic echocardiography and (b and c) transthoracic ultrasound images showing presence of hypoechoeic mass lesion along wall of right atrium (white arrows) with associated pericardial effusion (white asterisk). Thickening of adjacent pericardium is also seen (black arrow)

Figure 2

Axial computed tomography (CT) section of chest showing multiple, well-defined nodules in both lungs with most of the nodules showing ground glass opacity around them (black arrow)

Figure 3

Axial section contrast-enhanced CT chest showing presence of heterogeneously enhancing right atrial mass (black arrow) with thickening of adjacent pericardium (white arrow). Associated pericardial effusion is also noted (white asterisk)

Primary cardiac tumors are rare with benign cardiac tumors being more common.[1] Among the primary benign tumors, cardiac myxoma is the commonest; while cardiac sarcomas are the most common malignant cardiac tumors.[2] Cardiac angiosarcoma is the commonest subtype of cardiac sarcoma in adults. It carries a poor prognosis due to its rapid growth, high metastatic potential and disappointing treatment results. Cardiac angiosarcoma commonly involves right atrium. It may spread to involve other cardiac chambers, cardiac valves, superior or inferior vena cava, and pericardium.

It is more common in males. Commonly involved age group is 30–50 years.[2] In few patients, it is detected incidentally during the autopsy with an incidence of ~0.001–0.03%.[2] However in symptomatic patients, clinical presentation varies. Metastasis is commonly present at the time of initial presentation in many cases. Symptoms and signs of cardiac angiosarcoma include dyspnea, hemoptysis, chest pain, or other nonspecific complaints like fever or weight loss. It may present as hypotension or shock due to pericardial involvement and cardiac temponade or with features of right heart failure (RHF). At times, symptoms due to hemorrhagic metastasis to other organs like lung, brain, liver, bones, or skin may lead to initial clinical presentation.[3]

ECG findings are nonspecific and include right axis deviation, ST and T wave changes, low voltage QRS, arrhythmias, or heart block. Chest radiograph often shows cardiomegaly with pericardial effusion. Signs of right heart failure (RHF), pulmonary nodules, or pleural effusion may also be seen. Echocardiography is the initial investigation of choice in patients suspected to have a cardiac mass. It often shows presence of tumor involving right atrium with associated pericardial effusion. Involvement of tricuspid valve can be detected. Pericardiocentesis often reveals only bloody fluid with no malignant cells even when pericardium has been involved by tumor.

Cross sectional imaging like contrast-enhanced magnetic resonance imaging (MRI) and CT scan can provide detailed information about tumor size, its location, and extent and involvement of adjacent structures. Cardiac angiosarcoma commonly presents as heterogeneously enhancing right atrial mass with areas of hemorrhage and necrosis. Involvement of adjoining chambers, superior and inferior vena cava, and pericardium can be seen more clearly on MRI. Interspersed areas of iso- or hypointensity on T1- and T2-weighted images due to blood products give cauliflower appearance to the tumor. Sunray appearance has also been described on MRI produced by linear enhancement of vascular channels.[4]

Lungs are one of the most common sites for metastasis. Pulmonary metastases are seen as well-defined nodules with surrounding ground glass opacity due to alveolar hemorrhage or interlobular septal thickening.

Microscopically, tumor is composed of endothelial and mesenchymal cells. It shows multiple vascular channels lined by highly pleomorphic malignant cells. Irregular vascular spaces with papillary intraluminal tufting are characteristic. On immunohistochemistry, tumor cells typically show positivity for CD 31, CD 34, vimentin, and factor VIII-related antigen. Classical finding of cardiac angiosarcoma on electron microscopy is Weibel-Palade bodies; however, they are not seen in all cases.[5]

Treatment of cardiac angiosarcoma has been very challenging with poor results. Median survival after clinical presentation is about 4–6 months without surgical resection, which may improve to 10 months after surgery. Heart transplant has also been attempted with unsatisfactory outcome.[5] Chemotherapy and radiotherapy have also been used (especially in with metastatic disease) with however unfavorable results.

With this case we wish to highlight that initial clinical presentation of primary cardiac angiosarcoma can be with hemoptysis and those patient with pulmonary nodules and associated unexplained pericardial effusion should be further evaluated with echocardiography and cross-sectional imaging to rule out cardiac mass.