Extragastrointestinal stromal tumor involving the adrenal region: A rare case report

This is the authors' abstract. We don't add key points for this paper.

Adrenal masses are increasingly detected due to advanced imaging modalities, yet their differential diagnoses remain broad and challenging. Extragastrointestinal stromal tumors (EGISTs) are rare mesenchymal tumors sharing histologic and immunophenotypic features with GISTs but occurring outside the gastrointestinal (GI) tract. EGISTs involving the adrenal region are exceptionally rare, with only five cases previously reported. In this case report, we present the sixth documented case, involving a male patient in his early 60s who presented with acute abdominal pain and a nonfunctioning retroperitoneal mass abutting the adrenal gland. Surgical resection and histopathological analysis confirmed a GIST with cluster of differentiation 117 (CD117) and Discovered on GIST-1 (DOG1) positivity. The patient received adjuvant imatinib and remained recurrence-free at two-year follow-up. This case highlights the diagnostic complexity of EGISTs involving the adrenal region and emphasizes the role of immunohistochemistry and surgical management. While early outcomes appear favorable, the rarity and limited follow-up data call for cautious interpretation and continued reporting to better define the prognosis and optimal treatment strategies.