The standard diagnosis, treatment, and follow-up of gastrointestinal stromal tumors based on guidelines.
Although gastrointestinal stromal tumors (GISTs) are a rare type of cancer, they are the commonest sarcoma in the gastrointestinal tract. Molecularly targeted therapy, such as imatinib therapy, has revolutionized the treatment of advanced GIST and facilitates scientific research on GIST. Nevertheless, surgery remains a mainstay of treatment to obtain a permanent cure for GIST even in the era of targeted therapy. Many GIST guidelines have been published to guide the diagnosis and treatment of the disease. We review current versions of GIST guidelines published by the National Comprehensive Cancer Network, by the European Society for Medical Oncology, and in Japan. All clinical practice guidelines for GIST include recommendations based on evidence as well as on expert consensus. Most of the content is very similar, as represented by the following examples: GIST is a heterogeneous disease that may have mutations in KIT, PDGFRA, HRAS, NRAS, BRAF, NF1, or the succinate dehydrogenase complex, and these subsets of tumors have several distinctive features. Although there are some minor differences among the guidelines--for example, in the dose of imatinib recommended for exon 9-mutated GIST or the efficacy of antigen retrieval via immunohistochemistry--their common objectives regarding diagnosis and treatment are not only to improve the diagnosis of GIST and the prognosis of patients but also to control medical costs. This review describes the current standard diagnosis, treatment, and follow-up of GISTs based on the recommendations of several guidelines and expert consensus.
Department of Surgery, National Cancer Center Hospital East, 6-5-1 Kashiwanoha, Kashiwa, Chiba, 277-8577, Japan. tnishida@east.ncc.go.jp.
Toshirou
T
Nishida
Department of Medical Oncology, Centre Leon-Bernard, University Claude Bernard Lyon I, Lyon, France.
Jean-Yves
JY
Blay
Department of Surgical Pathology, Hyogo College of Medicine, Nishinomiya, Japan.
Seiichi
S
Hirota
Department of Surgery, Keio University School of Medicine, Tokyo, Japan.
Yuko
Y
Kitagawa
Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, South Korea.
Yoon-Koo
YK
Kang
D000970:Antineoplastic Agents / Q000627:therapeutic use; D019008:Drug Resistance, Neoplasm; D005500:Follow-Up Studies; D005770:Gastrointestinal Neoplasms / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy*; D046152:Gastrointestinal Stromal Tumors / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy*; D006801:Humans; D000068877:Imatinib Mesylate / Q000627:therapeutic use; D009154:Mutation; D009364:Neoplasm Recurrence, Local; D010671:Phenylurea Compounds / Q000627:therapeutic use; D017410:Practice Guidelines as Topic; D019009:Proto-Oncogene Proteins c-kit / Q000235:genetics; D011725:Pyridines / Q000627:therapeutic use
D000970:Antineoplastic Agents; D010671:Phenylurea Compounds; D011725:Pyridines; C559147:regorafenib; D000068877:Imatinib Mesylate; D019009:Proto-Oncogene Proteins c-kit
Consensus based; Evidence-based; Gastrointestinal stromal tumor; Guidelines