Merad M, Alibay A, Ammari S, Antoun S, Bouguerba A, Ayed S, Vincent F
Service d'urgence en oncologie médicale, Gustave-Roussy Cancer Campus Grand Paris, Villejuif, 94805 Villejuif cedex, France.
Service de radiologie, Gustave-Roussy Cancer Campus Grand Paris, Villejuif, 94805 Villejuif cedex, France.
Rev Mal Respir. 2017 Dec;34(10):1045-1057. doi: 10.1016/j.rmr.2017.02.008. Epub 2017 Nov 17.
Pulmonary tumor thrombotic microangiopathy syndrome is a rare clinicopathological entity in which tumor cell micro-emboli in the pulmonary microcirculation induced thrombotic microangiopathy. This can cause respiratory failure, and acute or sub-acute right heart failure. Histological features include micro tumor emboli in the small arteries and arterioles of the lung associated with thrombus formation and fibro-cellular and fibro-muscular intimal proliferation. The diagnosis is however extremely difficult to make before death. Thus, most of the observations reported are based on autopsy data. Very rare diagnostic observations made before death suggest the potential effectiveness of chemotherapy. Many details remain to be elucidated, interdisciplinary research is a priority with close collaboration between pathologists and clinicians to better understand this, often fatal, syndrome. It may be that the use of targeted therapies will improve the very poor prognosis allowing survival of several weeks or months after diagnosis.
肺肿瘤血栓性微血管病综合征是一种罕见的临床病理实体，其中肺微循环中的肿瘤细胞微栓子可诱发血栓性微血管病。这可导致呼吸衰竭以及急性或亚急性右心衰竭。组织学特征包括肺小动脉和小动脉中的微肿瘤栓子，伴有血栓形成以及纤维细胞和纤维肌性内膜增生。然而，在死亡前极难做出诊断。因此，大多数报告的观察结果基于尸检数据。死亡前做出的非常罕见的诊断观察提示化疗可能有效。许多细节仍有待阐明，跨学科研究是当务之急，病理学家和临床医生需密切合作以更好地了解这种往往致命的综合征。也许使用靶向治疗将改善极差的预后，使患者在诊断后能存活数周或数月。
