English version of Japanese Clinical Practice Guidelines 2022 for gastrointestinal stromal tumor (GIST) issued by the Japan Society of Clinical Oncology

Abstract

The Japan Society of Clinical Oncology Clinical Practice Guidelines 2022 for gastrointestinal stromal tumor (GIST) have been published in accordance with the Minds Manual for Guideline Development 2014 and 2017. A specialized team independent of the working group for the revision performed a systematic review. Since GIST is a rare type of tumor, clinical evidence is not sufficient to answer several clinical and background questions. Thus, in these guidelines, we considered that consensus among the experts who manage GIST, the balance between benefits and harms, patients' wishes, medical economic perspective, etc. are important considerations in addition to the evidence. Although guidelines for the treatment of GIST have also been published by the National Comprehensive Cancer Network (NCCN) and the European Society for Medical Oncology (ESMO), there are some differences between the treatments proposed in those guidelines and the treatments in the present guidelines because of the differences in health insurance systems among countries.

Keywords: Clinical practice guidelines; Expert consensus; Gastrointestinal stromal tumor (GIST); Minds manual for guideline development.

Fig. 1

Algorithm 1, Outline of diagnosis and therapy for gastrointestinal submucosal tumors. a Methods to obtain the tissue are not restricted. They include percutaneous needle biopsy and biopsy at exploratory laparotomy

Fig. 2

Algorithm 2, Differential diagnosis of spindle cell type GIST. a Most of the spindle cell type GISTs are diffusely positive for KIT, and KIT-negative and partially KIT-positive spindle cell type GISTs are very rare. Tumors with partial KIT-positivity should be considered non GISTs with nonspecific KIT staining. b Histological findings of tumors with HE staining have to be consistent with those of GIST. c Confirmation of the presence of NAB2-STAT6 fusion gene is recommended. d Mutational analysis of CTNNB1 gene encoding beta-catenin is recommended. e Analysis of ALK fusion gene by PCR or FISH is recommended. P1, P2, P3 and P7 mean “see Pathology BQ1, BQ2, BQ3 and BQ7”, respectively

Fig. 3

Algorithm 3, Differential diagnosis of epithelioid cell type GIST. a Partially KIT-positive tumors should be considered non GISTs showing nonspecific KIT staining. b Because of rarity of DOG1-negative GISTs, gene analysis should be performed for those tumors especially PDGFRA gene. c Histological findings of the tumor with HE staining have to be consistent with those of GIST. P1, P2, P3 and P7 mean “see Pathology BQ1, BQ2, BQ3 and BQ7”, respectively

Fig. 4

Algorithm 4, Treatment strategy for resectable and localized gastrointestinal submucosal tumors. a Epithelial tumors have to be excluded by biopsy under endoscopy. Biopsy from the serosal side is prohibited. b Findings of ulcer formation, irregular margin, and enlargement are included. c Enhanced CT (oral or transvenous) with continuous slice 5-mm thick or less is recommended. d EUS-FNA is recommended but not necessary. e Findings of necrosis, hemorrhage, irregular margin, and heterogeneity by enhanced CT and those of heterogeneity, irregular margin, and lymph node enlargement by EUS are included. f Follow-up by endoscopy including EUS is recommended. g Intraoperative pathological examination is recommended when a preoperative pathological diagnosis is not made. R1, R2, R3, P6, S1, S2, S3, S4 and S5 mean “see Radiology BQ1, BQ2, BQ3, Pathology BQ6, Surgery CQ1, CQ2, CQ3, BQ4 and CQ5”, respectively

Fig. 5

Algorithm 5, Surgical treatment for localized GIST. S3, S4, S5 and M12 mean “see Surgery CQ3, BQ4, CQ5 and Medicine CQ12”, respectively

Fig. 6

Algorithm 6, Post-operative therapy for localized GIST. a Efficacy of adjuvant imatinib therapy is not established in GISTs with low and intermediate risk of recurrence. b Follow-up by enhanced CT is usually carried out every 6 months (evidence unknown). c Follow-up by enhanced CT is usually carried out every 4–6 months in the case of GISTs with high risk of recurrence and/or tumor rupture and every 6–12 months in the case of GISTs with very low, low, and intermediate risk of recurrence (evidence unknown). R2, R3, P4, P5, P9, S6, S7, S8, M1, M3, M4, M5-1, M5-2. M6, M9 and M12 mean “see Radiology BQ2, BQ3, Pathology BQ4, BQ5, BQ9, Surgery CQ6, BQ7, CQ8, Medicine CQ1, CQ3, CQ4, BQ5-1, CQ5-2, BQ6, CQ9 and CQ12”, respectively

Fig. 7

Algorithm 7, First-line drug therapy for GIST. a Follow-up by enhanced CT is usually carried out every 4–6 months (evidence unknown). b Efficacy of FDG-PET/CT has been reported, but it is not covered by insurance. R2, R3, R4, P8, P9, S8, S9, M1, M2, M3 and M12 mean “see Radiology BQ2, BQ3, CQ4, Pathology BQ8, BQ9, Surgery CQ8, CQ9, Medicine CQ1, BQ2, CQ3, and CQ12”, respectively

Fig. 8

Algorithm 8, Therapy for imatinib-resistant GIST. S10, M6, M7, M8, M9, M10, M11 and M12 mean “see Surgery CQ10, Medicine BQ6, BQ7, CQ8, CQ9, CQ10, CQ11 and CQ12”, respectively

Fig. 9

Comparison of duodenal local resection and pancreaticoduodenectomy for duodenal GIST—a meta-analysis of postoperative complications