Pulmonary tumor thrombotic microangiopathy presenting as recurrent syncope

Abstract

Pulmonary tumor thrombotic microangiopathy is a rare condition in which embolization of tumor cells to the pulmonary arterioles causes fibrocellular intimal thickening and activation of the coagulation cascade resulting in pulmonary hypertension and right heart failure. Herein, we highlight a young 35-year-old male with no known past medical history who presented with recurrent syncope and dyspnea, and was found to have severe right heart failure and pulmonary hypertension. He developed sudden clinical deterioration and died after a cardiac arrest. Autopsy revealed poorly differentiated gastric adenocarcinoma and pulmonary tumor thrombotic microangiopathy. New onset severe pulmonary hypertension and right heart failure without any other obvious etiology should encourage the reader to evaluate for pulmonary tumor thrombotic microangiopathy and undergo a diligent search for underlying malignancy. This case highlights recurrent syncope as a rare presentation of this rapidly fatal disease.

Keywords: Pulmonary hypertension; cancer; pulmonary tumor thrombotic microangiopathy.

Figure 1.

Chest Tomography showing subtle diffuse ground-glass (blue arrow) and multiple tree-in-bud opacities (red arrow) and enlarged right ventricle and right atrium.

Figure 2.

H and E stain showing highly atypical cells causing fibroblastic intimal hypertrophy in the pulmonary vessels, consistent with pulmonary tumor thrombotic microangiopathy.

Figure 3.

H and E stain showing highly atypical cells invading muscularis externa in the gastric wall.