Platelet-derived growth factor receptor-tyrosine kinase inhibitor, imatinib, is effective for treating pulmonary hypertension induced by pulmonary tumor thrombotic microangiopathy

Int Heart J. 2015;56(2):245-8. doi: 10.1536/ihj.14-220. Epub 2015 Feb 23.


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.

Publication types

  • Case Reports

MeSH terms

  • Adenocarcinoma / secondary*
  • Adenocarcinoma / therapy
  • Antineoplastic Agents / therapeutic use*
  • Benzamides / therapeutic use*
  • Female
  • Humans
  • Hypertension, Pulmonary / drug therapy*
  • Hypertension, Pulmonary / etiology
  • Imatinib Mesylate
  • Lung Neoplasms / secondary*
  • Lung Neoplasms / therapy
  • Middle Aged
  • Piperazines / therapeutic use*
  • Pyrimidines / therapeutic use*
  • Receptors, Platelet-Derived Growth Factor / antagonists & inhibitors*
  • Stomach Neoplasms / pathology
  • Stomach Neoplasms / therapy
  • Thrombotic Microangiopathies / complications
  • Thrombotic Microangiopathies / drug therapy*


  • Antineoplastic Agents
  • Benzamides
  • Piperazines
  • Pyrimidines
  • Imatinib Mesylate
  • PDGF receptor tyrosine kinase
  • Receptors, Platelet-Derived Growth Factor