TY - JOUR T1 - Occult malignancy underlying a case of “hyperacute” onset of severe pulmonary hypertension JF - ERJ Open Research JO - erjor DO - 10.1183/23120541.00157-2019 VL - 5 IS - 4 SP - 00157-2019 AU - Maria Rosaria Gioia AU - Uberto Maccari AU - Luca Marchetti AU - Enivarco Maglioni AU - Giuseppina Ciarleglio AU - Valentina Granese AU - Laura Salerno AU - Luigi Spagnoli AU - Ilenia Zanellato AU - Maria Raffaella Ambrosio AU - Andrea Bianco AU - Raffaele Scala Y1 - 2019/10/01 UR - http://openres.ersjournals.com/content/5/4/00157-2019.abstract N2 - “Tumoral pulmonary hypertension (PH)” includes several subtypes of conditions leading to abnormal levels of pulmonary artery or venous pressure occurring in patients with a current or previous malignancy. Pulmonary tumour “microvascular disease” includes both pulmonary tumour microembolism (PTE) and pulmonary tumour thrombotic microangiopathy (PTTM) that are likely to be part of the same spectrum disease [1]. PTE was described in early studies, and was defined as the occlusion of the pulmonary microvasculature by tumour cells and associated thrombi. Tumour thrombi are frequently formed by malignant cells, platelets and fibrin, and are highly resistant to recanalisation and lead to an irreversible obstruction. In many cases, they are associated with vascular tissue reaction characterised by extensive fibrocellular intimal hyperplasia of small pulmonary arteries initiated by tumour microemboli known as PTTM [1, 2]. Most reported cases of PTE occur in association with adenocarcinomas, including liver [3, 4], kidney, breast [5, 6], stomach [4, 7], bladder and choriocarcinoma [8]. In a handful of cases of PTE, estimated at 5% [9], the primary cancer is unknown. Ante mortem diagnosis of PTE is often challenging and the majorities of cases are identified as results of post mortem studies. This is especially true for a subset of patients with occult malignancy and acute onset of severe PH.Investigations in a patient with new-onset pulmonary hypertension should include screening for undiagnosed malignancy http://bit.ly/2mrLmGM ER -