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Gastrointestinal stromal tumors

Key sources
The following summarized guidelines for the evaluation and management of gastrointestinal stromal tumors 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).
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Guidelines

1.Screening and diagnosis

Diagnosis
Make the diagnosis of GIST by a pathologist experienced in the disease.
E
Confirm the diagnosis before initiating neoadjuvant therapy with imatinib, since there is a wide differential.
E
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2.Diagnostic investigations

Imaging for staging: obtain contrast-enhanced abdominopelvic CT with image acquisitions of the arterial and portal phases for evaluating tumor extension.
B

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  • Circulating tumor DNA

3.Diagnostic procedures

Endoscopic ultrasound: as per BSG 2017 guidelines, obtain EUS in patients with lesions > 2 cm in diameter.
E

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  • Core-needle biopsy

  • Excisional biopsy

  • Mutational analysis

4.Medical management

General principles: manage patients with GIST by an experienced multidisciplinary team.
E

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  • Management of local/locoregional disease (neoadjuvant therapy)

  • Management of local/locoregional disease (surgical excision)

  • Management of local/locoregional disease (adjuvant therapy)

  • Management of advanced/metastatic disease (systemic therapy, first-line)

  • Management of advanced/metastatic disease (systemic therapy, second-line)

  • Management of advanced/metastatic disease (systemic therapy, third- and fourth-lines)

  • Management of advanced/metastatic disease (surgical excision)

  • Management of advanced/metastatic disease (radiotherapy)

5.Follow-up and surveillance

Surveillance imaging: as per ESMO 2022 guidelines, obtain active surveillance in patients with GIST, if a biopsy is not feasible.
B