Pulmonary Artery Angiosarcoma Research Articles

Pulmonary artery angiosarcoma mimicking chronic thromboembolic pulmonary hypertension

Pulmonary artery angiosarcoma (PAA) is an extremely rare malignancy that arises from endothelial cells. The pulmonary trunk is the most common anatomic site of these tumors. However, so far, there have only been a few examples of PAA reported. PAAs are aggressive and have a bad prognosis, thus early diagnosis is critical. In this study, we present the case of a 27-year-old male who was initially diagnosed with acute pulmonary thromboembolism (PTE) before being diagnosed with chronic thromboembolic pulmonary hypertension (CTEPH). Pulmonary endarterectomy was performed, and PAA was diagnosed histopathologically. The patient died three months after diagnosis.

Hemoptysis in a patient with pulmonary angiosarcoma

Pulmonary Artery Angiosarcoma (PAS) is a rare malignant vascular tumor with an aggressive clinical course and poor prognosis. The first line therapy is the surgical resection, but an early diagnosis is difficult due to the nonspecific clinical and radiological manifestations. The purpose of this case report is to analyze the difficulties in PAS diagnosis. A 32 years-old man, with a history of hemoptysis in the last two months, undergoes total body CT, which revealed multiple nodules in his left lung, surrounded by areas of ground glass opacity, without any extra-lung lesion. Fiberoptic bronchoscopy and bronchial washing were negative for neoplastic cells and open lung biopsy was necessary for diagnosis of PAS. A surgical resection was not allowed because the left lung was hepatized, so the surgeon couldn’t reach the hilum; furthermore, the subclavian vessels were infiltrated by cancer. After a month of supportive therapy, the patient died as a consequence of multiple organ failure. Our case report underlines that PAS should be considered as a possible diagnosis for patients with hemoptysis and lung nodules surrounded by ground glass opacity.

Don't stop at first glance: pulmonary artery angiosarcoma mimicking chronic thromboembolic pulmonary hypertension.

We report the case of an incomplete diagnosis of chronic thromboembolic pulmonary hypertension, with relevant prognostic implications, missing the presence of a primary pulmonary artery angiosarcoma. After the late neoplasm diagnosis, the patient, treated for months with riociguat, was considered inoperable and died soon after.This case highlights the need to manage patients with suspected pulmonary arterial hypertension by expert referral centers with specific and multi-professional expertise (heart and thoracic imaging) in order to avoid incomplete or delayed diagnoses.

Six-year survival of a patient with pulmonary artery angiosarcoma

Angiosarcomas of the pulmonary artery are rare and carry a very poor prognosis. We describe the case of a patient with pulmonary artery angiosarcoma who underwent radical resection followed by chemotherapy and was alive after 6 years of follow-up. Our case demonstrates that a multidisciplinary approach including cardiac diagnostics, radical surgery, and effective adjuvant chemoradiotherapy can improve outcome.

Pulmonary artery sarcoma with angiosarcoma phenotype mimicking pleomorphic malignant fibrous histiocytoma: a case report

Primary sarcomas of the major blood vessels can be classified based on location in relationship to the wall or by histologic type. Angiosarcomas are malignant neoplasms that arise from the endothelial lining of the blood vessels; those arising in the intimal compartment of pulmonary artery are rare. We report a case of pulmonary artery angiosarcoma in a 36-year old female with pulmonary masses. The patient had no other primary malignant neoplasm, thus excluding a metastatic lesion. Gross examination revealed a thickened right pulmonary artery and a necrotic and hemorrhagic tumor, filling and occluding the vascular lumen. The mass extended distally, within the pulmonary vasculature of the right lung. Microscopically, an intravascular undifferentiated tumor was identified. The tumor cells showed expression for vascular markers VEGFR, VEGFR3, PDGFRa, FGF, Ulex europaeus, FVIII, FLI-1, CD31 and CD34; p53 was overexpressed and Ki67 proliferative rate was increased. Intravascular angiosarcomas are aggressive neoplasms, often associated with poor outcome.Virtual slideThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/2315906377648045.

Diagnostic enigma: primary pulmonary artery sarcoma

Primary angiosarcoma of pulmonary artery is a very rare lesion. We present a case of primary angiosarcoma that was initially misdiagnosed as a subacute massive pulmonary thromboembolism in a 30-year-old man. This rare disease is usually indistinguishable from acute or chronic thromboembolic disease of the pulmonary arteries. The clinical and radiological findings of pulmonary artery angiosarcoma are similar to those of pulmonary thromboembolism. Although the incidence of pulmonary artery angiosarcoma is very low, our case demonstrates that this disease entity should be included in the differential diagnosis of pulmonary thromboembolism. Patients with early identification can have curative potential with aggressive surgical intervention.

5-Year Survival After Multiple Repeat Metastasectomy for Pulmonary Artery Angiosarcoma

Primary pulmonary artery angiosarcomas are rare malignant tumors with few reported survivors. We report a case of a 39-year-old woman without evidence of disease 5 years after initial diagnosis and after multiple metastasectomies and pulmonary artery tumor endarterectomy.

Primary Angiosarcoma of the Pulmonary Trunk Mimicking Pulmonary Thromboembolism

We present a case of primary angiosarcoma of the pulmonary trunk that was initially misdiagnosed as a subacute massive pulmonary thromboembolism in a 26-year-old woman. This is an extremely rare disease that is usually indistinguishable from acute or chronic thromboembolic disease of the pulmonary arteries because the clinical and radiologic findings of pulmonary artery angiosarcoma are similar to those of pulmonary thromboembolism. Although the incidence of pulmonary artery angiosarcoma is very low, our case demonstrates that this disease entity should be included in the differential diagnosis of pulmonary thromboembolism, especially in patients who do not respond to anticoagulant therapy or present with no identifiable source of thromboembolic events.

Pulmonary artery angiosarcoma: a clinicopathologic and radiological correlation

A 69-year-old man presented with cough, shortness of breath, and fatigue. He was initially treated for allergies and then for pulmonary embolism. Radiologically, a tumor mass was found to occlude the right pulmonary artery and involve the pulmonary trunk. A right pneumonectomy was performed. Histologically, a cellular malignant spindle and epithelioid tumor with areas of necrosis and brisk mitotic activity was seen. In some areas, the tumor appeared to form vascular channels. Focal osteosarcomatous differentiation was present. Immunohistochemical studies were performed including vimentin, smooth muscle actin, desmin, CD31, CD34, S100, and pan-cytokeratin. The tumor cells were positive for CD31 and vimentin and negative for pan-cytokeratin, CD34, and S100. Two months after surgery, the patient was alive and well.

Angiosarcoma of the Pulmonary Trunk Mimicking Pulmonary Thromboembolic Disease. A case report

We report on a 59-year-old male patient who suffered from dyspnea, cough and hemoptysis. Initial chest X-ray revealed a prominent right pulmonary artery (RPA) and MR imaging showed a "filling defect" within the main pulmonary trunk and RPA indicating pulmonary thromboembolic disease. Despite systemic anticoagulation, symptoms progressed. Follow-up CT showed an enlarging "filling defect" with additional extension into the left pulmonary artery as well as multiple intrapulmonary nodules. Lesion biopsy revealed a pulmonary artery angiosarcoma. Imaging findings are presented. Our case illustrates that pulmonary artery angiosarcoma should be included in the differential diagnosis of pulmonary thromboembolic disease in cases where a) symptoms do not respond to anticoagulation, b) no source of thrombi/emboli can be detected and c) pulmonary nodules/metastases develop on follow-up.

Epithelioid angiosarcoma of the pulmonary artery

Pulmonary artery angiosarcoma is a rare entity. We report a case of an epithelioid angiosarcoma developing from the right pulmonary artery with pulmonary parenchymal invasion. The patient was a 69-year-old man who presented with massive hemoptysis and shortness of breath. Right middle and lower lobectomies were performed because of uncontrollable bleeding. An angiosarcoma was observed developing from the right pulmonary artery with contiguous spread down smaller artery branches with invasion of the pulmonary parenchyma. Although typical angiosarcomatous areas were observed, the neoplasm was dominated by cells with an epithelioid morphology. Immunohistochemically, the majority of cells stained for endothelial markers factor VIII-related antigen and CD34, but in addition, the cells with an epithelioid morphology stained intensely for cytokeratin. Knowledge of cytokeratin positivity in epithelioid vascular neoplasms is critical to avoid a misdiagnosis of carcinoma, particularly at sites where carcinoma is a much more likely diagnosis.