Sugunaraj Jaya Prakash, Pedroso Claire, Kethireddy Shravan, Mogri Mohammed
Geisinger Medical Center, USA.
Respir Med Case Rep. 2015 Jun 4;16:38-40. doi: 10.1016/j.rmcr.2015.05.016. eCollection 2015.
Pulmonary epitheloid hemangioendothelioma (PEHE) is a rare, often incidentally diagnosed, endothelial tumor of the lung. We present a case of a young adult who presented with acute hypoxemic respiratory failure and severe pulmonary hypertension with subsequent imaging and tissue biopsy confirming a diagnosis of PEHE. We briefly highlight the unique clinical, radiographic and histopathologic aspects of this rare disease. We propose that PEHE should be considered in the differential diagnosis for acute hypoxemic respiratory failure associated with bilateral pulmonary cavitary nodules and bronchoscopy should be considered as an initial diagnostic test.
肺上皮样血管内皮瘤（PEHE）是一种罕见的、常为偶然诊断出的肺部内皮肿瘤。我们报告一例年轻成人病例，该患者表现为急性低氧性呼吸衰竭和严重肺动脉高压，随后经影像学检查和组织活检确诊为PEHE。我们简要强调了这种罕见疾病独特的临床、影像学和组织病理学特征。我们建议，对于伴有双侧肺空洞性结节的急性低氧性呼吸衰竭，鉴别诊断时应考虑PEHE，且应将支气管镜检查作为初始诊断检查。
