Objective To analyze the prognosis, clinicopathological characteristics, and genetic mutation features of patients with very low- and low-risk gastrointestinal stromal tumors (GISTs).

Methods  A retrospective data collection of 433 patients with very low- and low-risk GISTs, diagnosed by pathology after surgical or endoscopic resection at Tianjin Medical University Cancer Institute & Hospital, was conducted to analyze clinical pathology and prognosis.

Results  Compared with low-risk GIST, a higher proportion of patients with very low-risk GIST had gastric GISTs, a Ki-67 index≤3%, and 0–1 mitotic indexes/5 mm², whereas the proportion of patients with small intestinal GIST was low (all P<0.05). Genetic testing was performed in 118 patients, and there was no significant difference in the mutation types and KIT mutation types between low-risk and very low-risk GISTs (all P>0.05). Patient follow-up was conducted on 87 very low-risk and 210 low-risk GIST patients without other concurrent diseases, with a median follow-up of 86(27–158) months. None of the patients with very low-risk GISTs showed progression; three cases of low-risk GIST had recurrence or metastasis, with progression-free survival (PFS) ranging from 27 to 84 months. The mitotic indices of the primary tumors in these three patients were all 4/5 mm², which is close to the critical value between the low- and intermediate-risk GIST mitotic indices, and all had KIT exon11 mutations. After the first progression, these three patients were treated with imatinib, and all survived until the end of follow-up, with overall survival ranging from 116 to 155 months.

Conclusions  Among patients with low-risk GISTs, a very small number with high mitotic indices may have the potential for recurrence or metastasis, but after recurrence or metastasis, they can still maintain long-term, high-quality survival through targeted therapy, surgery, and other treatment methods. The possibility of postoperative progression in very low-risk patients with GIST is extremely low, and the follow-up interval can be appropriately extended to reduce medical costs and alleviate the psychological burden on the patients and their families.