A Rare Cause of Pulmonary Nodules Diagnosed as Angiosarcoma Was Misdiagnosed as Vasculitis and Wegener’s Granuloma in an Elderly Man: A Case Report

Abstract

BackgroundAngiosarcoma is a rare, highly malignant tumor prone to recurrence and metastasis. Angiosarcoma is insidious in the initial stage, and its clinical manifestation lacks specificity. The diagnosis is based on histopathology and immunohistochemistry findings.Case presentationA 73-year-old man was hospitalized following complaints of persistent cough 6 months and hemoptysis for 2 months. Anti-infective treatment was ineffective. A CT-guided percutaneous core needle biopsy of pulmonary lesions revealed organized pneumonia, and the removed skin of purpuric rash area on the left calf revealed vasculitis. Chest CT was used during the patient follow-up. Hormonal therapy combined with immunoglobulins did not lead to improvement, and there was rapid progression of the lung lesions. Subsequently, the patient underwent a surgery, the diseased tissue was separated and removed completely beside the left submandibular gland under local anaesthesia. The immunohistochemical staining indicated CD31 (+) and CD34 (+) confirming a diagnosis of metastatic angiosarcoma. The expression of PD-L1 was 70%, therefore, anlotinib and pembrolizumab treatments were initiated. The patient eventually died.ConclusionAngiosarcoma is a malignant tumor in the clinic that lacks typical and specific signs and symptoms. The diagnosis depends on immunohistochemistry, which requires repeated biopsies of multiple sites in highly suspected cases.

Primary pulmonary epithelioid angiosarcoma with thyroid tumor history: A case report and literature review

“…Angiosarcoma most commonly occurs in the skin of the head and neck, particularly on the scalp, in elderly individuals, accounting for approximately half of all of these tumors. Other common sites include the breast, thyroid, heart, liver, kidney, spleen, pulmonary vessels and limbs ( 6 , 7 ). Pulmonary angiosarcomas are invariably (>90%) metastatic tumors from primary malignancies of the skin, bone, liver, breast or heart ( 8 ).…”

“…The pathogenesis of the PPA has remained to be fully elucidated. Angiosarcoma originates from blood vessels or lymphatic vessels; thus, abnormal activation of vascular endothelial growth factor (VEGF) and its receptor VEGFR may result in angiosarcoma ( 6 ). According to previous reports, the risk factors for PPA are radiotherapy, thorium dioxide, poly-vinyl chloride, radon, thorotrast, copper mining dust, mastectomy, chronic empyema and tuberculosis ( 3 , 8 , 10-12 ).…”

“…PPA is associated with symptoms such as cough, hemoptysis, chest pain, shortness of breath, pneumothorax, spontaneous hemothorax, cyanosis, syncope or rarely massive pulmonary hemorrhage and weight loss ( 6-8 , 10 , 11 ). In addition, most patients present with cough and hemoptysis as the initial symptoms ( 6 ). However, up to 20% of PPA cases are asymptomatic and diagnosis mainly occurs incidentally during autopsy ( 3 , 6 , 11 ).…”

“…In addition, most patients present with cough and hemoptysis as the initial symptoms ( 6 ). However, up to 20% of PPA cases are asymptomatic and diagnosis mainly occurs incidentally during autopsy ( 3 , 6 , 11 ). PPA does not have any specific clinical manifestations and thus, it is easily misdiagnosed as similar diseases, such as lung cancer, metastatic malignancies, malignant pleural mesothelioma, malignant endobronchial lesions, pulmonary embolism, interstitial pneumonia associated with autoimmunological disease or infectious pneumonia (e.g.…”

“…PPA does not have any specific clinical manifestations and thus, it is easily misdiagnosed as similar diseases, such as lung cancer, metastatic malignancies, malignant pleural mesothelioma, malignant endobronchial lesions, pulmonary embolism, interstitial pneumonia associated with autoimmunological disease or infectious pneumonia (e.g. invasive pulmonary mycoses, tuberculosis) ( 6-9 ). The patient of the current study was admitted to the hospital after presenting with cough and hemoptysis as the initial symptoms.…”