Metastatic Primary Pulmonary Angiosarcoma

Abstract

Angiosarcoma is an extremely aggressive tumor with a high rate of mortality. It can arise in any tissue of the body and is most commonly found in the skin and soft tissue[1]. Pulmonary angiosarcoma is usually secondary to a primary location of the body and presents as both solitary and multiple nodules. Primary pulmonary angiosarcoma is a rare disease with less than 20 cases reported in the English literature. In our report we present a case of metastatic primary pulmonary angiosarcoma involving the most ( > 100) pulmonary nodules known to date. Novel treatment strategy using an anti-angiogenic inhibitor was used to treat this disease for the first time to our knowledge; however, it was unsuccessful in controlling disease progression. This report reviews the literature of this rare and devastating disease

Angiosarcoma of the lung

“…On CT scans, primary angiosarcoma can be multifocal or can manifest as solitary lesions. ( 4 , 6 , 7 ) Imaging with PET can be useful in the diagnosis, staging, ( 8 ) and follow-up of patients with angiosarcoma. ( 9 ) In the case presented here, the FDG PET-CT scan confirmed its usefulness in identifying the regions and systems involved.…”

“…Patients with pulmonary angiosarcoma have been treated with radiotherapy, ( 9 , 11 ) surgical resection, ( 4 , 9 ) immunotherapy, (4,6,11) chemotherapy, ( 4 , 6 , 9 ) or a combination of those. ( 4 , 6 , 9 , 11 ) Although none of those treatments have been shown to be effective, there have been reports of effective treatments for the condition, ( 4 , 9 , 11 ) especially in cases of localized disease. It has been proposed that the most effective treatment is surgery, which should be considered as early as possible for resectable tumors.…”

“…Na TC, o angiossarcoma primário pode ser multifocal ou se manifestar como lesões solitárias. ( 4 , 6 , 7 ) A PET pode ser útil no diagnóstico, estadiamento ( 8 ) e seguimento de pacientes com angiossarcoma. ( 9 ) No caso aqui descrito, a FDG PET-CT confirmou sua utilidade na identificação do envolvimento sistêmico.…”

“…Tendo em consideração a raridade do angiossarcoma, não há um esquema terapêutico padrão estabelecido, especialmente para o angiossarcoma pulmonar primário. De acordo com os relatos de caso descritos, os doentes com angiossarcoma pulmonar têm sido tratados com radioterapia, ( 9 , 11 ) ressecção cirúrgica, ( 4 , 9 ) imunoterapia, ( 4 , 6 , 11 ) quimioterapia, ( 4 , 6 , 9 ) ou uma combinação dessas modalidades. ( 4 , 6 , 9 , 11 )…”

Primary pulmonary angiosarcoma: Three case reports and literature review

“…Primary pulmonary angiosarcoma is a rare type of malignant vascular tumor characterized by the proliferation of tumor cells with endothelial features. To the best of our knowledge, only 28 cases, including our patients, have been reported in English literature (Table ) . The mean age of the reported cases was 54.7 years (range 23–85).…”