What are the guideline sources, guidelines and references for Gastrointestinal stromal tumors ?
Guideline sources The following summarized guidelines for the evaluation and management of gastrointestinal stromal tumors (GIST) are prepared by our editorial team based on guidelines from the Spanish Group for Research on Sarcomas (GEIS/SEOM 2023), the European Reference Network on Genetic Tumour Risk Syndromes (GENTURIS/EURACAN/ESMO 2022), the British Sarcoma Group (BSG 2017), and the American Society for Gastrointestinal Endoscopy (ASGE 2017; 2015). 1 2 3 4 5 Guidelines 1. Screening and diagnosis Diagnosis: Make the diagnosis of gastrointestinal stromal tumor by a pathologist experienced in the disease. E Confirm the diagnosis before initiating neoadjuvant therapy with imatinib, since there is a wide differential. E 2. Diagnostic investigations https://web.pathway.md/diseases/reccYKyQAenMjDTV9 1/5 6/24/23, 3:16 AM Gastrointestinal stromal tumors Pathway Imaging for staging: obtain contrast-enhanced abdominopelvic CT with image acquisitions of the arterial and portal phases for evaluating tumor extension. B Circulating tumor DNA: insufficient evidence to recommend assessing circulating tumor DNA for clinical purposes. I 3. Diagnostic procedures Endoscopic ultrasound: as per BSG 2017 guidelines, obtain EUS in patients with lesions > 2 cm in diameter. E Core-needle biopsy: Perform a core needle biopsy for the initial diagnosis of GIST. B Do not perform a biopsy in patients with imatinib-resistant GIST with the sole purpose of determining the KIT/PDGFRA genotype. D Excisional biopsy: As per GENTURIS 2022 guidelines, perform resection if diagnosis of a gastrointestinal stromal tumor is made on biopsy unless major morbidity is expected. B As per BSG 2017 guidelines: Perform needle biopsy in patients with lesions > 2 cm in diameter, particularly if the lesion is in the stomach. E Consider performing percutaneous core needle biopsy if the tumor is inaccessible to EUS- guided biopsy. E Mutational analysis: As per SEOM 2023 guidelines, obtain mutational analysis in all patients with GIST requiring medical treatment. B As per BSG 2017 guidelines: Obtain mutational analysis and immunohistochemistry by an accredited laboratory for the diagnosis of GIST. E Obtain mutational analysis before neoadjuvant therapy since some GIST are insensitive to imatinib, such as patients with D842V mutation in exon 18 of PDGFRA. E 4. Medical management General principles: manage patients with gastrointestinal stromal tumor by an experienced multidisciplinary team. E Management of local/locoregional disease, neoadjuvant therapy: As per GEIS/SEOM 2023 guidelines, consider offering neoadjuvant imatinib in certain patients with high volume, requiring function-sparing surgery, or at risk of bleeding. C https://web.pathway.md/diseases/reccYKyQAenMjDTV9 2/5 6/24/23, 3:16 AM Gastrointestinal stromal tumors Pathway As per GENTURIS/EURACAN/ESMO 2022 guidelines, consider offering neoadjuvant avapritinib in patients with PDGFRA-D842V mutation. B As per BSG 2017 guidelines, consider offering preoperative imatinib in patients with large gastric or rectal primaries where immediate resection is likely to be morbid, such as total gastrectomy or abdominoperineal resection. E Management of local/locoregional disease, surgical excision: As per GENTURIS/EURACAN/ESMO 2022 guidelines, perform complete surgical excision of the lesion with no dissection of clinically negative lymph nodes in patients with localized gastrointestinal stromal tumor. B Show 2 more As per ASGE 2017 guidelines, perform surgery in patients with gastric and colorectal GIST > 2 cm in size and in patients with high-risk features. B Management of local/locoregional disease, adjuvant therapy: As per GEIS/SEOM 2023 guidelines, use the NIH-modified risk criteria to determine the risk of relapse for the indication of adjuvant imatinib. A Show 4 more As per GENTURIS/EURACAN/ESMO 2022 guidelines: Offer adjuvant therapy with imatinib 400 mg/day for 3 years in patients with a significant risk of relapse. A Consider offering adjuvant imatinib at a higher dose of 800 mg/day for 3 years in patients with KIT exon 9 mutation. C As per BSG 2017 guidelines, offer adjuvant imatinib for 3 years in patients at high risk of recurrence or distant relapse, provided the tumor is not likely to be resistant to therapy (PDGFRA exon 18 mutation D842V). E Management of advanced/metastatic disease, systemic therapy, first line: As per GEIS/SEOM 2023 guidelines, offer imatinib 400 mg/day as first-line therapy in patients with metastatic GIST. A Show 3 more As per GENTURIS/EURACAN/ESMO 2022 guidelines, offer imatinib 400 mg/day as first-line therapy in patients with locally advanced, inoperable, and metastatic disease, except for GIST without KIT/PDGFRA mutations or with a PDGFRA exon 18 D842V mutation. A Show 4 more As per BSG 2017 guidelines, offer imatinib 400 mg/day in patients with unresectable or metastatic disease. Consider offering a larger dose of imatinib in patients with exon 9 mutation in KIT. E Management of advanced/metastatic disease, systemic therapy, second line: As per GEIS/SEOM 2023 guidelines, offer sunitinib 50 mg/day 4 weeks on, 2 weeks off as second-line therapy in patients with GIST. A Consider offering a continuous dose of sunitinib 37.5 mg once daily for better tolerance. B https://web.pathway.md/diseases/reccYKyQAenMjDTV9 3/5 6/24/23, 3:16 AM Gastrointestinal stromal tumors Pathway As per GENTURIS/EURACAN/ESMO 2022 guidelines, offer sunitinib 50 mg/day 4 weeks on/2 weeks off or, as an alternative schedule, 37.5 mg once daily as second-line therapy in case of confirmed progression or rare intolerance on imatinib. A As per BSG 2017 guidelines, offer sunitinib 50 mg/day for 4 weeks every 6 weeks, or 37.5 mg/day continuously as second-line therapy in patients with advanced/metastatic disease. E Management of advanced/metastatic disease, systemic therapy, third and fourth lines: As per GEIS/SEOM 2023 guidelines, offer regorafenib 160 mg/day 3 weeks on, 1 week off as third-line therapy, and ripretinib 150 mg/day as fourth-line therapy in patients with GIST. A As per GENTURIS/EURACAN/ESMO 2022 guidelines: Offer regorafenib 160 mg/day for 3 out of every 4 weeks as third-line therapy in patients progressing on or failing to respond to imatinib and sunitinib. A Offer ripretinib 150 mg/day as fourth-line therapy in patients progressing on or intolerant to imatinib, sunitinib, and regorafenib. A As per BSG 2017 guidelines, offer regorafenib as third-line therapy in patients with advanced/metastatic disease. E Management of advanced/metastatic disease, surgical excision: As per GEIS/SEOM 2023 guidelines, consider performing surgery in metastatic disease on an individual basis within a multidisciplinary tumor board. C As per BSG 2017 guidelines, consider performing surgery or other local measures, such as radiofrequency ablation, in patients with isolated progression. E Management of advanced/metastatic disease (radiotherapy): consider offering radiotherapy as a palliative resource in selected patients with gastrointestinal stromal tumor. C 5. Follow-up and surveillance Surveillance imaging: As per ESMO 2022 guidelines, obtain active surveillance in patients with gastrointestinal stromal tumor, if a biopsy is not feasible. B As per ASGE 2017 guidelines, consider obtaining surveillance EUS in patients with GIST < 2 cm in size. C As per BSG 2017 guidelines, obtain cross-sectional follow-up imaging in patients with a high- risk disease on adjuvant therapy at intervals of every 3-6 months during 3 years of treatment, 3 monthly for 2 years following cessation of treatment, and thereafter every 6 months for 3 years and annually for 5 years. E Show 2 more As per ASGE 2015 guidelines, consider obtaining annual EUS in patients with GIST < 2 cm to determine the progression of size or change in echo features, if surgical resection is not performed. C https://web.pathway.md/diseases/reccYKyQAenMjDTV9 4/5 6/24/23, 3:16 AM Gastrointestinal stromal tumors Pathway References 1. P G Casali, J Y Blay, N Abecassis et al. Gastrointestinal stromal tumours: ESMO EURACAN GENTURIS Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2022 Jan;33 1 20 33. Open 2. C sar Serrano, Rosa lvarez, Juan Antonio Carrasco et al. SEOM GEIS clinical guideline for gastrointestinal stromal tumors 2022 . Clin Transl Oncol. 2023 May 2. Online ahead of print. Open 3. Ian Judson, Ramesh Bulusu, Beatrice Seddon et al. UK clinical practice guidelines for the management of gastrointestinal stromal tumours GIST . Clin Sarcoma Res. 2017 Apr 21;7 6. Open 4. ASGE Standards of Practice Committee, John A Evans, Vinay Chandrasekhara et al. The role of endoscopy in the management of premalignant and malignant conditions of the stomach. Gastrointest Endosc. 2015 Jul;82 1 1 8. Open 5. Standards of Practice Committee, Ashley L Faulx, Shivangi Kothari et al. The role of endoscopy in subepithelial lesions of the GI tract. Gastrointest Endosc. 2017 Jun;85 6 1117 1132. Open 6. Casali PG, Abecassis N, Aro HT et al. Gastrointestinal stromal tumours: ESMO EURACAN clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2018 Oct 1;29 Suppl 4):iv68-iv78. 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