American Journal of Respiratory and Critical Care Medicine
With an estimated five-year survival of less than 40 percent, angiosarcomas are rare and often misdiagnosed fataltumors originating from blood or lymphatic vessels with a predilection for the head and neck. They account for 1percent of all soft tissue sarcomas and are often mistaken for benign mimickers leading to the late-stage diagnosiswhen the body is already riddled with metastatic disease with the lung being the most common site of metastases.This case illustrates the myriad of pulmonary complications associated with this uncommon but fatal vasoformativemalignancy. The patient is a 73-year-old male diagnosed with angiosarcoma of the right scalp status post excision,reconstruction, and adjuvant proton radiotherapy. He presented to the hospital 8 weeks after treatment completionwith new onset acute hypoxic respiratory failure secondary to bilateral pleural effusions as well as bilateralhydropneumothoraces. He underwent bilateral thoracenteses as well as right and left chest tubes with a Heimlichvalve. On CT chest, he was found to have resolution of the effusions and pneumothoraces but an increase in thenumber of pulmonary cysts, nodules and ground glass opacities. Cytology of the effusions was positive formetastatic angiosarcoma. When angiosarcoma is diagnosed, it is imperative that clinicians familiarize themselveswith the pulmonary manifestations of metastasis as they indicate rapid progression of the disease as well as worseprognosis. Patients can present with hemoptysis, cough, pleuritic chest pain or dyspnea resulting from severalapathologies ranging from diffuse alveolar hemorrhage to pneumothorax to pleural effusion. Imaging is often non-specific and can show a pneumothorax (often recurrent due to cyst rupture), effusions, irregular pulmonary cysts ornodules as well as reticulonodular opacities. Due to the multitude of symptoms associated with metastasis, patientsoften get misdiagnosed with vasculitis, COPD flares or infections such as non-mycobacterium TB, furtherpostponing appropriate chemotherapies. Angiosarcoma in it of itself is an often missed or delayed diagnosis.When the disease process is confirmed, one must be aware of the high rate of metastasis with the lung being themost likely site. Due to the high mortality rate, it is imperative that pulmonary symptoms not be confounded with theaforementioned less lethal etiologies. Early detection and treatment with excision, radiation therapy and chemo willallow for higher rates of disease-free survival.
Salimnia T, Abplanalp LA, Chawla R, Hughes K, Ghandchi R, Weatherhead M, et al. [Dogra S]. From head to hilum: angiosarcoma with pulmonary metastasis. Am J Respir Crit Care Med. 2022 May 1;205(Suppl):A5507. doi:10.1164/ajrccm-conference.2022.205.1_MeetingAbstracts.A5507.
American Thoracic Society 2022 International Conference, May 13-18, 2022, San Francisco, CA.