(P 162) ANALYSIS OF 3,144 CASE REPORTS IN THE GASTROINTESTINAL STROMAL TUMOR LITERATURE: IDENTIFICATION OF UNDERAPPRECIATED DEMOGRAPHICS, CLINICAL PRESENTATIONS, AND BIOLOGY

Objective: Gastrointestinal stromal tumors (GIST) were first described as a unique pathologic entity in 1998 and are now the most common soft tissue sarcoma. Despite the growing literature of over 11,000 studies on GIST, there continues to be a high volume of case reports published on practically a weekly basis. This prompted us to review these to try to understand common elements reported, how these may have changed over time, and to identify underappreciated aspects of GIST that stimulated writing the case report.

Methods: We searched the NCBI PubMed database to discover all case reports on GIST from 5/1/1998 to 8/5/2023. Search terms included “GIST” OR “gastrointestinal stromal tumor” AND “Case report.” Publications were manually classified into 19 categories, which were then subcategorized. Descriptive statistical analyses were performed.

Results: During the 25-year study period there were a total of 4,350 articles discovered using the specified search criteria. After manual curation, 3,144 case reports representing 3,348 patients were included for analysis. The five most represented categories were tumor location (30.3%), presentation (27.5%), operative approach (12.1%), concomitant malignancy (9.2%), and metastatic site (8.4%) (Panel A). While many reports described now well-known aspects of the disease, we identified rare but clinically important features of GIST including tumor-associated paraneoplastic syndromes (0.6%, Panel B) and reports of metastases to the brain (0.6%, Panel C). Moreover, 38 GIST occurrences (1.2%) were reported in neonates, infants, and children ≤ 12 years old (Panel D). This included only 18 (47.4%) gastric tumors in addition to 12 (31.6%) in the small bowel, 7 (18.4%) in the colon, and 1 (2.6%) involving the appendix. Fourteen (37%) of these were reported in males (Panel E).

Conclusion: We describe GIST case reports published over a 25-year period and classified these based upon abstract content. We were surprised to uncover an array of underappreciated aspects of GIST, speaking to the narrow but important role of publishing case reports on the novel and unusual aspects of rare diseases that would otherwise not be appreciated in larger case series or in population-based studies. Thus, we recommend judicious publication of GIST case reports, particularly those that emphasize previously unknown or unappreciated aspects of disease biology or novel approaches to management.