Analysis of 3144 case reports in the gastrointestinal stromal tumor literature: Identification of underappreciated demographics, clinical presentations, and biology

Objective

Gastrointestinal stromal tumors (GIST) were first described in 1998 and are now known to be the most common soft tissue sarcoma. Numerous case reports are still frequently published reporting various aspects of GIST. This prompted us to review these reports to identify common elements, changes over time, and underappreciated aspects of GIST, as well as to ask whether further exploration is truly warranted.

Methods

A search of the NCBI PubMed database from 5/1/1998–8/5/2023 identified all case reports on GIST using the terms “GIST” OR “gastrointestinal stromal tumor” AND “case report.” Publications were classified into 19 categories, subcategorized, and analyzed using descriptive statistics.

Results

A total of 4350 articles were found, with 3144 case reports representing 3348 patients 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 %). Notable findings included rare but clinically important features such as tumor-associated paraneoplastic syndromes (0.6 %) and brain metastases (0.6 %). Additionally, 38 cases (1.2 %) were reported in neonates, infants, and children ≤ 12 years old.

Conclusions

We describe GIST case reports published over a 25-year period and classified them 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.