PT - JOURNAL ARTICLE AU - Laura C. Price AU - Michael J. Seckl AU - Peter Dorfmüller AU - S. John Wort TI - Tumoral pulmonary hypertension AID - 10.1183/16000617.0065-2018 DP - 2019 Mar 31 TA - European Respiratory Review PG - 180065 VI - 28 IP - 151 4099 - http://err.ersjournals.com/content/28/151/180065.short 4100 - http://err.ersjournals.com/content/28/151/180065.full SO - EUROPEAN RESPIRATORY REVIEW2019 Mar 31; 28 AB - Tumoral pulmonary hypertension (PH) comprises a variety of subtypes in patients with a current or previous malignancy. Tumoral PH principally includes the tumour-related pulmonary microvascular conditions pulmonary tumour microembolism and pulmonary tumour thrombotic microangiopathy. These inter-related conditions are frequently found in post mortem specimens but are notoriously difficult to diagnose ante mortem. The outlook for patients remains extremely poor although there is some emerging evidence that pulmonary vasodilators and anti-inflammatory approaches may improve survival. Tumoral PH also includes pulmonary macroembolism and tumours that involve the proximal pulmonary vasculature, such as angiosarcoma; both may mimic pulmonary embolism and chronic thromboembolic PH. Finally, tumoral PH may develop in response to treatments of an underlying malignancy. There is increasing interest in pulmonary arterial hypertension induced by tyrosine kinase inhibitors, such as dasatanib. In addition, radiotherapy and chemotherapeutic agents such as mitomycin-C can cause pulmonary veno-occlusive disease. Tumoral PH should be considered in any patient presenting with unexplained PH, especially if it is poorly responsive to standard approaches or there is a history of malignancy. This article will describe subtypes of tumoral PH, their pathophysiology, investigation and management options in turn.Tumoral PH includes pulmonary tumour micro-embolism and pulmonary tumour thrombotic microangiopathy. Diagnosis is difficult and often delayed, with high mortality. Improved survival is reported with some cancer therapies so early recognition is imperative. http://ow.ly/slAn30mLDW7