Characterization of sarcoma-like cells derived from endarterectomized             tissues from patients with CTEPH and establishment of a mouse model of pulmonary             artery intimal sarcoma.
In general, intravascular thrombus formation in the pulmonary arteries is             considered to be the most common cause of chronic thromboembolic pulmonary hypertension             (CTEPH). The current mainstay of therapy for patients with CTEPH is pulmonary             endarterectomy (PEA). Recently, the existence of myofibroblast-like cells in endarterectomized             tissues has been demonstrated. At the 2nd passage of these myofibroblast-like             cells, a pleomorphic cell type was isolated. Pulmonary intimal sarcoma is a very             uncommon neoplastic tumor thought to originate from subendothelial-mesenchymal             cells of the pulmonary vascular wall. Because these pleomorphic cells were isolated             from the pulmonary vascular beds, it is believed that the analysis of these cells             may contribute to the understanding of pulmonary intimal sarcoma. We isolated             cells from the endarterectomized tissue from patients with CTEPH and identified             one type as sarcoma-like cells (SCLs). The SCLs were characterized as hyperproliferative,             anchorage-independent, invasive and serum-independent. Moreover, C.B-17/lcr-scid/scidJcl             mice injected subcutaneously with SCLs developed solid, undifferentiated tumors             at the site of injection, and those injected intravenously with SCLs via the tail             vein developed tumors which grew along the intimal surface of the pulmonary vessels,             thus, demonstrating the high tumorigenic potential of these cells. The behavior             of SCLs indicated that these cells may have a vascular cell-like potential which             can affiliate them with the intimal surface of the pulmonary artery, and which             may be shared with pulmonary intimal sarcoma. A further investigation of this             mouse model with SCLs may elucidate the mechanism(s) underlying the development             of pulmonary intimal sarcoma.
Department of Respirology, Graduate School of Medicine, Chiba University, Chuo-ku, Chiba, Japan.
Takayuki
T
Jujo
Seiichiro
S
Sakao
Masashi
M
Kantake
Miki
M
Maruoka
Nobuhiro
N
Tanabe
Yasunori
Y
Kasahara
Katsushi
K
Kurosu
Masahisa
M
Masuda
Kenichi
K
Harigaya
Koichiro
K
Tatsumi
D000818:Animals; D002465:Cell Movement; D049109:Cell Proliferation; D002471:Cell Transformation, Neoplastic; D002908:Chronic Disease; D003893:Desmin / Q000378:metabolism; D004195:Disease Models, Animal; D004691:Endarterectomy; D016326:Extracellular Matrix Proteins / Q000235:genetics / Q000378:metabolism; D020869:Gene Expression Profiling; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology / Q000473:pathology / Q000601:surgery; D008297:Male; D051379:Mice; D016513:Mice, SCID; D009361:Neoplasm Invasiveness; D009368:Neoplasm Transplantation; D011651:Pulmonary Artery / Q000473:pathology*; D011655:Pulmonary Embolism / Q000150:complications / Q000473:pathology / Q000601:surgery; D012509:Sarcoma / Q000378:metabolism / Q000473:pathology*; D014407:Tumor Cells, Cultured; D017539:Tunica Intima / Q000473:pathology*; D019043:Vascular Neoplasms / Q000378:metabolism / Q000473:pathology*; D014746:Vimentin / Q000378:metabolism
D003893:Desmin; D016326:Extracellular Matrix Proteins; D014746:Vimentin