Angiosarcoma in the chest: radiologic–pathologic correlation

Angiosarcomas are rare, malignant vascular tumors. They represents about 2% of all soft tissue sarcoma, which can often metastasize through the hematogenous route. The radiological features have been analyzed in 4 patients with metastatic angiosarcoma in the chest. The main radiologic findings included nodules, cysts, nodules with halo sign, and vascular tree-in-bud. Morphologic features, as observed in the histologic specimen, have been correlated with radiologic appearance. Metastatic angiosarcomas to the lung are characterized by a wide variety of radiologic appearances that can be very characteristic. Computed tomographic findings observed include bilateral solid nodules, cystic, and bullous lesions sometimes associated with spontaneous hemopneumothoraces.

Related papers:

title: Extracutaneous Angiosarcomas Metastatic to the Lungs: Clinical and Pathologic Features of Twenty-One Cases
abstract: Angiosarcomas are rare malignant vascular tumors with a high rate of metastasis involving lungs (most commonly), liver, regional lymph nodes, bone, and other sites. In this study, we have reviewed the clinical presentation and histopathology of 21 cases of extracutaneous angiosarcoma metastatic to the lungs. Tumors with exclusively pleural involvement were excluded. Patients presented with dyspnea, chest pain, and/or hemoptysis lasting a few weeks to months. Radiologically, the most common finding comprised multiple peripheral lung nodules (57%), often accompanied by infiltrates. For 11 cases (52%), the primary tumor was not identified at the time of presentation. Vasoformative areas were identified in 15 cases (71%). Nine cases comprised spindle cells (43%), two contained epithelioid cells (9.5%), and 10 consisted of both spindle and epithelioid cells (48%). Nuclear pleomorphism was at least moderate in all cases. However, five tumors contained regions of minimal nuclear atypia. Hemorrhage, siderophages, and fibrosis were commonly present. Immunohistochemical staining (IHS) was performed on 14 cases. Thirteen tumors showed reactivity for vascular markers. Tumor cells reacted for Von Willebrand factor in 13 of 14 cases, and CD31 and CD34 were each positive in 2/2 cases. Two cases (of nine examined) also expressed cytokeratins. Because the tumor often first presented in the lungs before the primary sarcoma was identified, the clinical impression included both benign and malignant entities. For patients with primary cardiac tumors, symptoms referable to the primary tumor complicated the clinical presentation, and radiologic evaluation supported a clinical diagnostic impression of non-neoplastic pericarditis. Thus, angiosarcoma in the lung may elude diagnosis until histopathologic evaluation of the lung biopsy.

title: Angiosarcoma in the lung.
abstract: Angiosarcoma involving the lung is a rare disorder and its clinical features are not well known. We conducted a retrospective analysis of 15 patients seen at our institution from 1950 to 1990 in an attempt to better characterize the spectrum of clinical and radiographic findings of angiosarcoma in the lung. No documented case of primary angiosarcoma of the lung was seen. The diagnosis of metastatic angiosarcoma to the lung was made antemortem in 12 of 15 cases, either by lung biopsy specimen (5 patients), biopsy evidence of metastatic disease elsewhere with abnormal chest radiograph (4 patients), or a compatible clinical picture in a patient with previously documented angiosarcoma arising in an extrapulmonary site (3 patients). The median age at the time of diagnosis was 45 years with the most common presenting symptom being hemoptysis (7 of 15 patients). Other presenting complaints included weight loss (6 of 15), cough (4 of 15), and chest pain (4 of 15) occurring 6 weeks to 1 year prior to diagnosis. Chest radiographs frequently disclosed multiple pulmonary nodules (11 of 15). Primary origins of the angiosarcoma most commonly included the heart and breast. Metastatic sites other than the lung included the pericardium, liver, spleen, kidney, adrenal gland, bone, and brain. The prognosis is generally poor, with our study population surviving an average of 9 months after diagnosis.

title: AJR teaching file: Right atrial mass in a woman with dyspnea on exertion.
abstract: Received December 27, 2007; accepted after revision April 7, 2008. 1Department of Radiology, University Hospital Birmingham NHS Foundation Trust, Metchley La., Birmingham, West Midlands, B67 5HR, United Kingdom. Address correspondence to B. J. Holloway (Ben.Holloway@uhb.nhs.uk). 2Department of Radiology, Division of Cardiothoracic Radiology, University of Michigan, Ann Arbor, MI. AJR 2009;192:S49–S52 0361–803X/09/1923–S49  This is followed by cardiac MRI to further evaluate abnormal cardiac findings identified on CT pulmonary angiography. CT of the abdomen that was performed 7 months previously to investigate upper abdominal pain is also reviewed and found to include the area of abnormality.

title: Primary angiosarcoma of the aorta, great vessels, and the heart.
abstract: Primary angiosarcomas originating from the heart, aorta, or great vessels are extremely rare and hence poorly understood. We reviewed our experience to identify a preferred diagnostic and treatment strategy and evaluate the role of adjunctive therapy.



We reviewed the clinical data of all patients diagnosed with primary angiosarcoma of the heart, aorta, and great vessels from 1985 to 2011, including presentation, diagnosis, management, and outcomes.



Thirteen patients (five males and eight females; mean age, 54 ± 4 years) had primary angiosarcoma arising from the aorta (n = 7), heart (n = 3), pericardium (n = 2), and pulmonary artery (n = 1). Patients with aortic tumors most commonly presented with lower extremity claudication (n = 2), renovascular hypertension (n = 3), abdominal pain (n = 5), and weight loss (n = 4). Patients with cardiac and pericardial tumors presented with dyspnea (n = 5) due to pleural effusion or cardiac tamponade. All 13 patients underwent computed tomographic scan, which demonstrated irregular, lobulated mass/thrombus with peripheral enhancement, and eight patients underwent diagnostic echocardiography. Metastatic disease was present in 10 patients. The most common site was the lungs (n = 6). All except one patient exhibited high-grade morphology histopathologically. Nine patients were treated surgically: resection with aortic reconstruction (n = 5), thromboendarterectomy (n = 2), pericardiectomy/atrial septal resection with patch reconstruction (n = 2), and just biopsy (n = 1). Adjunctive treatment included chemotherapy (n = 6) and radiation (n = 4). Median survival was 8 months (range, 1-75 months). Patients treated with all three treatment modalities had longer survival than did patients treated with a single modality (P = .013). Patients treated with chemotherapy had a more favorable survival than did those without chemotherapy (P = .048).



Primary angiosarcoma of the heart and great vessels is rare but is a harbinger of poor prognosis. Pathologic examination is necessary to confirm the diagnosis. Combined therapy with surgical resection and chemoradiotherapy offers patients the best survival.

title: Pulmonary Artery Angiosarcoma Confused with Acute Pulmonary Thromboembolism: Focusing on Clinical and Echocardiographic Features in the Differentiation of Two Categories
abstract: Although pulmonary artery angiosarcoma is rare, it can be misdiagnosed as pulmonary embolism because of its similar clinical and diagnostic features. The diagnosis is often delayed and the misdiagnosis brings unnecessary treatment. Because we made a wrong diagnosis of pulmonary artery angiosarcoma as an acute pulmonary embolism, we did thrombolytic therapy which could be dangerous to the patient. In this case report, we focused on the clinical and echocardiographic features of pulmonary artery angiosarcoma which can be used in differentiating the diagnosis from pulmonary embolism.

title: METASTATIC ANGIOSARCOMA PRESENTING AS DIFFUSE ALVEOLAR HEMORRHAGE
abstract: Angiosarcoma is a rare malignant neoplasm of the vascular or lymphatic endothelium. Diffuse alveolar hemorrhage is a rare presenting manifestation of angiosarcoma. We describe a case of pulmonary metastasis of angiosarcoma who presented with diffuse alveolar hemorrhage as initial manifestation.

title: Metastatic Angiosarcoma Masquerading as Diffuse Pulmonary Hemorrhage
abstract: Context.—Diffuse pulmonary hemorrhage is an uncommon presenting manifestation of angiosarcoma. Objective.—To review the clinical, radiologic, and pathologic findings of patients with metastatic angiosarcoma who presented with diffuse pulmonary hemorrhage. Design.—Patients fulfilling inclusion criteria were identified from the consultation files. Clinical and radiologic data were obtained from referring pathologists. Histologic slides were reviewed in all patients. Results.—Our patients included 6 men and 1 woman, aged 31 to 73 years; 4 patients were younger than 40 years. Six patients presented with hemoptysis, and all had diffuse abnormalities on radiographic studies. Clinical considerations prior to biopsy included pulmonary hemorrhage syndrome (n = 2), acute respiratory failure (n = 1), and infection (n = 1). Metastatic disease was included in the differential diagnosis in only 1 patient. None had a diagnosis of malignancy prior to lung biopsy. All biopsies showed hemorrhage associated with at...

title: Bilateral lung lesions: when the eyes deceive the brain!
abstract: We report a clinic case of patient in whom angiosarcoma of the heart presents as bilateral pulmonary nodular infiltrates. The cardiac tumor was clinically silent, the electrocardiogram was normal and the cough was the only symptom. Chest CT scan  showed bilateral diffuse nodular infiltrates ranging. Clinical clues, the results of laboratory tests and all of the cultures obtained excluded an infectious etiologies; the findings of CT-guided needle biopsy was inconclusive for a definitive diagnosis. Thus, the patient was scheduled for a thoracoscopic biopsy. Surprisingly, the pre-operatory echocardiogram showed a soft tissue mass fixed to the posterior wall of the right atrium. On retrospective reviewing of chest CT scan, a tumor was evident in the right atrium, but it was missed initially. In theory, the lung lesions attract the attention of the observer who had not taken into account anything else as to say: "the brain knows what the eyes want". The diagnosis ofpulmonary metastases was obtained by means ofpleural biopsy during right thoracoscopy. Immunoistochemical staining revealed a CK-, CK7-, EMA-, ESA-, CEA-, TTF1-, Vimentina+, CD31+, CD117+ lesion. Because at the time of diagnosis our patient already had lung metastases, he underwent chemotherapy.

title: Angiosarcoma of the lung: 2 cases report and literature reviewed.
abstract: Angiosarcoma is a kind of malignant tumors derived from vascular endothelial cell. Its incidence rate is very low. Pulmonary angiosarcomas are usually secondary tumors, and primary cases are less than 20 so far. Here we presented two cases of angiosarcoma involved the lung, and we only confirmed case 2 as a primary pulmonary angiosarcoma. The clinical characteristics, diagnosis, treatment options and prognosis of pulmonary angiosarcoma were reviewed in this article too.

title: Metastatic angiosarcoma of the lung: spectrum of CT findings.
abstract: The purpose of our study was to summarize the CT features of pulmonary metastases in angiosarcoma in 24 patients.



A variety of CT features were associated with metastatic angiosarcoma of the lung. The common CT manifestations of metastatic angiosarcoma were multiple solid nodular lesions and multiple thin-walled cysts that were often accompanied by hemorrhagic change.

title: Pulmonary nodules and the CT halo sign.
abstract: To determine whether the "halo sign" at computed tomography (CT) could be used to differentiate hemorrhagic from nonhemorrhagic pulmonary nodules.



CT scans of 12 patients with hemorrhagic pulmonary nodules and of a control group of 10 patients with nonhemorrhagic pulmonary nodules were randomized and reviewed by two chest radiologists. Nodules were considered hemorrhagic if a surrounding halo of ground-glass attenuation was present.



Nodules were correctly identified as either hemorrhagic or nonhemorrhagic by both observers in 19 of the 22 cases (86%). Eight of the 12 patients with hemorrhagic nodules had an infectious process including invasive aspergillosis, candidiasis, cytomegalovirus, herpes simplex virus, or coccidioidomycosis. Four of 12 patients with hemorrhagic nodules had a noninfectious cause including Wegener granulomatosis, metastatic angiosarcoma, and Kaposi sarcoma.



Most hemorrhagic pulmonary nodules have a characteristic CT appearance consisting of a central area of soft-tissue attenuation with a surrounding halo of ground-glass attenuation that allows distinction from nonhemorrhagic nodules.

title: Pulmonary metastases from angiosarcoma: a spectrum of CT findings
abstract: Though a few reports have summarized the computed tomography (CT) findings of pulmonary metastases from angiosarcoma, the detailed CT findings of cysts are not well known, except for their characteristic thin walls.  To retrospectively summarize the CT findings of pulmonary metastases from angiosarcoma, focusing mainly on the CT findings of cysts.  Thirty-three patients with pulmonary metastases from angiosarcoma were selected retrospectively. Two radiologists reviewed and assessed patients’ chest CT images on a consensus basis for nodules, cysts, the CT halo sign, pneumothorax, pleural effusion, and enlarged lymph nodes. Cysts were also evaluated by wall thickness and smoothness, air-fluid levels, and vessels or bronchi penetrating the cysts. The relationship between cysts and pneumothorax was assessed using the Chi-square test.  Nodules were found in 28 (85%) patients. Cysts were found in 19 (58%) patients; 17 had thin and smooth walls, 10 had thin and irregular walls, and four had thick and irregular walls. In addition, 12 patients showed vessels or bronchi penetrating the cysts, and six showed air-fluid levels. The CT halo sign, pneumothorax, pleural effusion, and mediastinal lymphadenopathy were seen in 19 (58%), 16 (48%), 26 (78.8%), and five (15.2%) patients, respectively. Pneumothorax occurred significantly more frequently in patients with cysts (P = 0.002).  Cysts showed variability in their walls, and air-fluid levels and vessels or bronchi penetrating the cysts appeared to be characteristic findings, which may be useful for detection and accurate diagnosis in patients with pulmonary metastases from angiosarcoma.

title: Angiosarcoma with pulmonary metastasis presenting with spontaneous bilateral pneumothorax in an elderly man.
abstract: Cutaneous angiosarcoma is a rare and invasive endothelial-derived sarcoma that occurs most frequently in the scalp and facial skin of elderly men. It is frequently accompanied by thin-walled cavitary pulmonary metastasis, and is often obscure on chest radiograph. We report a case of angiosarcoma of the scalp with cystic metastasis to the lung in a 63-year-old man, presenting as recurrent bilateral spontaneous pneumothorax. Lung metastasis was missed at the first episode of pneumothorax because the lung-expanded chest radiograph showed no significant abnormality. Two months later, bilateral pneumothorax recurred, and high-resolution computed tomography revealed multiple cystic, cavitary and nodular lesions. Pulmonary metastasis was confirmed by video-assisted thoracoscopic surgery, and pleurodesis was performed. After the operation, the patient received chemotherapy; no recurrence of pneumothorax was found during 6 months of follow-up. Pneumothorax in the elderly should be differentiated from malignant metastatic lung tumors.

title: Pneumothorax: a classical presentation of metastatic scalp angiosarcoma.
abstract: Metastatic pulmonary angiosarcomas frequently present with pneumothorax, especially those arising from the scalp. The metastases on chest radiographs are initially subtle and easily missed. Computed tomographic scans can show a spectrum of diagnostic findings that may be misinterpreted because of a lack of awareness related to the rarity of the disease. We report a patient who presented with pneumothorax and lung cysts before his primary scalp angiosarcoma was diagnosed. This case highlights the sometimes ominous nature of innocuous-looking, thin-walled cystic pulmonary lesions.

title: A rare cause of hemoptysis
abstract: Angiosarcomas are rare, malignant, endothelial-cell tumors of vascular origin that can arise at any body site. They frequently metastasize to the lung, heralded by dyspnea, hemoptysis, chest pain, pneumothoraces, and diffuse pulmonary hemorrhage. However, in most cases lung metastases are discovered after the diagnosis of a primary angiosarcoma has already been established. Very rarely will an undiagnosed metastatic angiosarcoma present as diffuse pulmonary hemorrhage. We describe the case of a 59-year-old male who presented to hospital with dyspnea and hemoptysis. CT chest revealed rapidly progressing nodular changes and broncho-alveolar lavage returns were progressively bloody. Open lung wedge biopsy ultimately revealed metastatic angiosarcoma and extensive pulmonary hemorrhage. Our case highlights the key clinical, radiological, and pathological features of this rare malignancy that frequently metastasizes to the lung and reminds clinicians to consider it as a cause of hemoptysis and pulmonary hemorrhage.

title: Pulmonary tumor thrombotic microangiopathy from metastatic epithelioid angiosarcoma.
abstract: The lung is most common site for metastatic disease via hematogenous route. Tumor emboli of the vessels of the lung induces fibrocellular and fibromuscular intimal proliferation. These histopathological changes may cause pulmonary tumor trombotic microangiopaty. Few cases are diagnosed antemortem. We report a 60 year old woman with by metastatic epithelioid angiosarcoma involving the lung. Tumor cells were positive for VEGF and topoisomerase II. VEGF may be involved in the pathogenesis pulmonary tumor trombotic microangioapy and topoisomerase II positivity showed sensitivity against catalytic topoisomerase II inhibitors.