PDF(Pubmed)
Abstract：
Pulmonary tumor thrombotic microangiopathy (PTTM ) is a rare condition of uncertain incidence given its likely underdiagnosis. PTTM has been described most frequently in association with gastric adenocarcinoma, but other primary malignancies have been identified. The prognosis of PTTM is very poor, and patients often die within days or weeks of diagnosis. There are, however, several medications currently being used with unknown therapeutic benefits. The case presented below describes a patient with PTTM and esophageal adenocarcinoma, which may be the first report of its kind. One other case of esophageal cancer associated with PTTM was found in the literature review, but it is of squamous cell carcinoma histology. Herein, we report a case of a male with rapidly progressive pulmonary hypertension and right heart failure who, in the course of treatment/evaluation, was found to have esophageal adenocarcinoma. While early diagnosis may not alter the course of the disease, antemortem diagnosis may identify better therapeutic options and better inform patients of their prognosis, allowing them to maintain autonomy in their medical decisions.
摘要：
肺肿瘤血栓性微血管病（PTTM）是一种罕见的不确定发病率的疾病，因为它可能被诊断不足。PTTM被描述为最常见的与胃腺癌有关，但其他原发性恶性肿瘤已被确认。PTTM的预后很差,患者通常在确诊后几天或几周内死亡。有,然而,目前正在使用的几种药物具有未知的治疗益处。以下病例描述了一名患有PTTM和食管腺癌的患者，这可能是同类报告中的第一份。在文献复习中发现另一例与PTTM相关的食管癌，但它是鳞状细胞癌的组织学。在这里，我们报道一例男性快速进行性肺动脉高压和右心衰竭,在治疗/评估过程中，被发现患有食管腺癌。虽然早期诊断可能不会改变疾病的进程，产前诊断可以确定更好的治疗方案，并更好地告知患者其预后，允许他们在医疗决策中保持自主权。
