Platelet-derived growth factor receptor-tyrosine kinase inhibitor, imatinib, is effective for treating pulmonary hypertension induced by pulmonary tumor thrombotic microangiopathy.
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.
Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo.
Shun
S
Minatsuki
Ichiro
I
Miura
Atsushi
A
Yao
Hiroyuki
H
Abe
Hironori
H
Muraoka
Mariko
M
Tanaka
Teruhiko
T
Imamura
Toshiro
T
Inaba
Hisataka
H
Maki
Masaru
M
Hatano
Koichiro
K
Kinugawa
Takashi
T
Yao
Masashi
M
Fukayama
Ryozo
R
Nagai
Issei
I
Komuro
D000230:Adenocarcinoma / Q000556:secondary* / Q000628:therapy; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D001549:Benzamides / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology; D000068877:Imatinib Mesylate; D008175:Lung Neoplasms / Q000556:secondary* / Q000628:therapy; D008875:Middle Aged; D010879:Piperazines / Q000627:therapeutic use*; D011743:Pyrimidines / Q000627:therapeutic use*; D017479:Receptors, Platelet-Derived Growth Factor / Q000037:antagonists & inhibitors*; D013274:Stomach Neoplasms / Q000473:pathology / Q000628:therapy; D057049:Thrombotic Microangiopathies / Q000150:complications / Q000188:drug therapy*
D000970:Antineoplastic Agents; D001549:Benzamides; D010879:Piperazines; D011743:Pyrimidines; D000068877:Imatinib Mesylate; C099852:PDGF receptor tyrosine kinase; D017479:Receptors, Platelet-Derived Growth Factor