A case of pulmonary tumour thrombotic microangiopathy

Abstract

Pulmonary tumour thrombotic microangiopathy (PTTM) is a rapidly progressive pulmonary disease that is a fatal complication of malignancy. It manifests clinically as subacute respiratory failure with pulmonary hypertension, progressive right sided heart failure, and sudden death. We describe here a case of PTTM associated with occult metastatic signet ring cell carcinoma of the stomach. Although rare, PTTM needs to be considered in the differential diagnosis of dyspnoea of unknown origin, particularly in patients with respiratory failure and also pulmonary hypertension, and in patients were there is no improvement in respiratory symptoms with steroid therapy.

Case report: apatinib plus selexipag as a novel therapy for pulmonary tumor thrombotic microangiopathy accompanied by pulmonary hypertension associated with gastric carcinoma

“…Remodeling of pulmonary vasculature characterized by luminal stenosis or obstruction leads to an increase in vascular resistance and results in pulmonary hypertension. [3,4,11,12] Postmortem autopsy or biopsy antemortem results have revealed the morphologic findings of PTTM are similar to microscopic tumor embolization mainly characterized by mechanical occlusion of pulmonary vessels, whereas intimal proliferation is rare in the latter situations. [1,2,13] Several cytokines and molecules secreted by carcinoma cells, including vascular endothelial growth factor (VEGF), [6,9,10] platelet-derived growth factor PDGF [5,10] , tissue factor (TF) [14] and osteopontin, [15][16][17] play key roles in the complex mechanism of PTTM.…”

Acute cor pulmonale due to pulmonary tumour thrombotic microangiopathy from renal cell carcinoma

“…PTTM has a spectrum of severity, with some cases being discovered incidentally at autopsy and others presenting with rapid right heart failure. Generally the progression of pulmonary hypertension is rapid, resulting in a clinical syndrome of progressive dyspnoea, subacute respiratory failure and sudden death 2. Pulmonary tumour embolism is a separate clinical entity resulting from mechanical blockage of the right heart or pulmonary vessels by large pieces of solid tumour.…”

Pulmonary tumor thrombotic microangiopathy caused by prostate carcinoma

“…The tree-in-bud appearance usually suggests infectious bronchiolitis and is also seen in patients with PTTM caused by tumor embolism or fibrocellular intimal proliferation in the small arteries or arterioles (9). Interlobular septal thickening, centrilobular ground glass opacities, reticular nodular opacities, and cavitation are also seen in PTTM (10)(11)(12). Dilatation of the main pulmonary artery suggests secondary pulmonary hypertension.…”