RECKGIST scores may predict recurrence among some patients with gastrointestinal stromal tumors (GISTs), study findings have shown.
Among patients with neurofibromatosis type 1 GISTs less than 30 millimeters (mm), prognosis without relapse is excellent, and RECKGIST score accurately predicted recurrence. RECKGIST score needs to be validated in an external cohort, but it may help treatment decision making, according to study findings published in ESMO Open.
After a median follow-up of six years, for GISTs less than 30 mm (35 patients), none relapsed. For GISTs greater than 30 mm (84 patients), 18 developed metastases (21%). There was no difference in relapse according to tumor location or tumor rupture, whereas KIT/PDGFRA-mutated GISTs were at higher risk of relapse (recurrence-free survival at 10 years of 30% versus 82.5% for wild type).
Furthermore, Miettinen and Joensuu classification did not predict relapse accurately. For the RECKGIST score A (size up to 30 mm, 34 patients) group, 10-year recurrence-free survival was 100%; it was 78.5% in the RECKGIST B group (size greater than 30 mm and mitotic index between 0 and 5, 60 patients) and 45.5% in the RECKGIST C group (size greater than 30 mm and mitotic index greater than 5, 20 patients). After matching, 10-year recurrence-free survival was similar between adjuvant and surveillance groups.
Glossary:
Neurofibromatosis type 1: a genetic disorder causing tumors on nerve tissue, skin changes and increased cancer risk.
KIT/PDGFRA mutations: genetic changes in GISTs that affect tumor behavior and treatment.
Mitotic index: a measure of tumor cell division, with a higher index indicating more aggressive tumors.
RECKGIST score: a tool to predict GIST recurrence based on size and mitotic index.
Recurrence-free survival: the time after treatment without cancer returning.
Sporadic tumors: tumors that arise randomly without known cause.
Wild type: the normal, non-mutated version of a gene or protein.
The Miettinen and Joensuu classification is a risk stratification system for GISTs that estimates the likelihood of recurrence based on tumor size, mitotic rate and location. This classification was described in a study published in Seminars in Diagnostic Pathology.
“To our knowledge, this is one of the largest multicentric cohorts of [neurofibromatosis type 1]-GISTs for which clinical and histological characteristics, as well as risk factors for relapse and survival, have been evaluated,” lead study author Dr. Charlotte Cuvelier and co-authors wrote in the study.
Cuvelier works for the Department of Gastroenterology and Digestive Oncology at Amiens University Hospital in Amiens, France.
Eighteen patients died, 44.4% from GIST, 11.2% from another cancer linked to neurofibromatosis type 1, mostly nervous system tumors, and 33.3% from causes unrelated to neurofibromatosis type 1 or GIST. The median age at death was 67 years.
A total of 119 patients were included from 2008 to 2023, 61% of whom were women. The median age was 53 years. The primary tumor location was the small bowel in 86% and the stomach in 11%. The median tumor size was 45 mm, and the median mitotic count was 2 mit per 5 mm². Most cases were KIT/PDGFRA wild type, with KIT and PDGFRA mutations occurring in 2% and 3% of patients, respectively.
Gastrointestinal stromal tumors, or GISTs, are rare tumors of the digestive tract that most often develop in the stomach or small intestine, according to study authors. While usually sporadic, they may be linked to genetic conditions such as neurofibromatosis type 1. In some cases, neurofibromatosis type 1-related mutations can lead to tumor development by affecting cell growth pathways.
Reference:
“Clinical description and development of a prognostic score for neurofibromatosis type 1 (NF1)-associated GISTs: a retrospective study from the NETSARCD,” by Dr. C. Cuvelier, et al., ESMO Open.
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