International Heart Journal
Online ISSN : 1349-3299
Print ISSN : 1349-2365
ISSN-L : 1349-2365
Case Reports
Platelet-Derived Growth Factor Receptor-Tyrosine Kinase Inhibitor, Imatinib, Is Effective for Treating Pulmonary Hypertension Induced by Pulmonary Tumor Thrombotic Microangiopathy
Shun MinatsukiIchiro MiuraAtsushi YaoHiroyuki AbeHironori MuraokaMariko TanakaTeruhiko ImamuraToshiro InabaHisataka MakiMasaru HatanoKoichiro KinugawaTakashi YaoMasashi FukayamaRyozo NagaiIssei Komuro
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Supplementary material

2015 Volume 56 Issue 2 Pages 245-248

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Abstract

Pulmonary hypertension (PH) induced by pulmonary tumor thrombotic microangiopathy (PTTM) can be fatal because its rapid progression confounds diagnosis, and it is difficult to control with therapy. Here we describe a woman with symptomatic PTTM-PH accompanying gastric cancer that was suspected from perfusion scintigraphy. PTTM-PH was diagnosed by gastroesophageal endoscopy and lung biopsy after partial control of PH using the platelet-derived growth factor (PDGF) receptor (PDGFR) tyrosine kinase inhibitor, imatinib. Treatment with sildenafil and ambrisentan further decreased PH, and she underwent total gastrectomy followed by adjuvant TS-1 chemotherapy. PH did not recur before her death from metastasis. Postmortem histopathology showed recanalized pulmonary arteries where the embolized cancer masses disappeared. PDGF-A, -B, and PDGFR-α, β expression was detected in cancer cells and proliferating pulmonary vascular endothelial cells. Thus, PTTM-PH was successfully controlled using a combination of imatinib, drugs to treat pulmonary arterial hypertension, and cancer management.

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© 2015 by the International Heart Journal Association
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