Gastrointestinal stromal tumors - a review

被引:46
作者
Joensuu, H [1 ]
Kindblom, LG
机构
[1] Univ Helsinki, Cent Hosp, Dept Radiotherapy & Oncol, Helsinki, Finland
[2] Sahlgrenska Univ Hosp, Dept Pathol, Gothenburg, Sweden
来源
ACTA ORTHOPAEDICA SCANDINAVICA | 2004年 / 75卷
关键词
D O I
10.1080/00016470410001708340
中图分类号
R826.8 [整形外科学]; R782.2 [口腔颌面部整形外科学]; R726.2 [小儿整形外科学]; R62 [整形外科学(修复外科学)];
学科分类号
摘要
Gastrointestinal stromal tumors (GISTs) may be defined as intraabdominal nonepithelial (mesenchymal) tumors that express the KIT protein or have an activating mutation in a class III receptor tyrosine kinase gene (KIT or PDGFRA). GISTs are diagnosed at a frequency of about 15 new cases annually per million, though small indolent GISTs are likely to occur more frequently in the general population. The clinical behavior is variable, and assessment of the malignancy potential is usually based mainly on the size and the proliferation characteristics of the tumor. The overwhelming majority of GISTs express the KIT protein, the transmembrame receptor tyrosine kinase for the stem cell factor. The majority of GISTs harbor a mutation in the KIT proto-oncogene that translates into constitutively activated KIT protein kinase, and a minority have mutated PDGFRA gene resulting in activated platelet-derived growth factor alpha receptor tyrosine kinase. Most GISTs respond to imatinib mesylate, which selectively inhibits both KIT and PDGFRA, and is now considered as the standard systemic therapy for advanced GIST. In contrast, responses to conventional chemotherapy are infrequent (generally less than 10%), but combination therapies with imatinib have not been explored. Research on adjuvant imatinib and novel targeted therapies is ongoing.
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页码:62 / 71
页数:10
相关论文
共 57 条
  • [11] KIT mutations are common in incidental gastrointestinal stromal tumors one centimeter or less in size
    Corless, CL
    McGreevey, L
    Haley, A
    Town, A
    Heinrich, MC
    [J]. AMERICAN JOURNAL OF PATHOLOGY, 2002, 160 (05) : 1567 - 1572
  • [12] Malignant gastrointestinal stromal tumors of the small intestine: A review of 50 cases from a prospective database
    Crosby, JA
    Catton, CN
    Davis, A
    Couture, J
    O'Sullivan, B
    Kandel, R
    Swallow, CJ
    [J]. ANNALS OF SURGICAL ONCOLOGY, 2001, 8 (01) : 50 - 59
  • [13] Two hundred gastrointestinal stromal tumors - Recurrence patterns and prognostic factors for survival
    DeMatteo, RP
    Lewis, JJ
    Leung, D
    Mudan, SS
    Woodruff, JM
    Brennan, MF
    [J]. ANNALS OF SURGERY, 2000, 231 (01) : 51 - 58
  • [14] Efficacy and safety of imatinib mesylate in advanced gastrointestinal stromal tumors
    Demetri, GD
    von Mehren, M
    Blanke, CD
    Van den Abbeele, AD
    Eisenberg, B
    Roberts, PJ
    Heinrich, MC
    Tuveson, DA
    Singer, S
    Janicek, M
    Fletcher, JA
    Silverman, SG
    Silberman, SL
    Capdeville, R
    Kiese, B
    Peng, B
    Dimitrijevic, S
    Druker, BJ
    Corless, C
    Fletcher, CDM
    Joensuu, H
    [J]. NEW ENGLAND JOURNAL OF MEDICINE, 2002, 347 (07) : 472 - 480
  • [15] DEMETRI GD, 2003, P AN M AM SOC CLIN, V22, P814
  • [16] Effects of a selective inhibitor of the Abl tyrosine kinase on the growth of Bcr-Abl positive cells
    Druker, BJ
    Tamura, S
    Buchdunger, E
    Ohno, S
    Segal, GM
    Fanning, S
    Zimmermann, J
    Lydon, NB
    [J]. NATURE MEDICINE, 1996, 2 (05) : 561 - 566
  • [17] Efficacy and safety of a specific inhibitor of the BCR-ABL tyrosine kinase in chronic myeloid leukemia.
    Druker, BJ
    Talpaz, M
    Resta, DJ
    Peng, B
    Buchdunger, E
    Ford, JM
    Lydon, NB
    Kantarjian, H
    Capdeville, R
    Ohno-Jones, S
    Sawyers, CL
    [J]. NEW ENGLAND JOURNAL OF MEDICINE, 2001, 344 (14) : 1031 - 1037
  • [18] ElRifai W, 1996, CANCER RES, V56, P3230
  • [19] Prognosis of gastrointestinal smooth-muscle (Stromal) tumors - Dependence on anatomic site
    Emory, TS
    Sobin, LH
    Lukes, L
    Lee, DH
    O'Leary, TJ
    [J]. AMERICAN JOURNAL OF SURGICAL PATHOLOGY, 1999, 23 (01) : 82 - 87
  • [20] Diagnosis of gastrointestinal stromal tumors: A consensus approach
    Fletcher, CDM
    Berman, JJ
    Corless, C
    Gorstein, F
    Lasota, J
    Longley, BJ
    Miettinen, M
    O'Leary, TJ
    Remotti, H
    Rubin, BP
    Shmookler, B
    Sobin, LH
    Weiss, SW
    [J]. HUMAN PATHOLOGY, 2002, 33 (05) : 459 - 465