College of Medicine, Alfaisal University, P.O. Box 50927, Riyadh, 11533, Saudi Arabia.
Endocr Pathol. 2015 Mar;26(1):27-32. doi: 10.1007/s12022-014-9350-7.
Gastrointestinal stromal tumors (GISTs) are rare neoplasms of the gastrointestinal (GI) tract. The occurrence of these neoplasms ectopically outside the GI tract is extremely uncommon. Only one case of primary adrenal GIST has been reported in the literature. In this account, we report a second case of primary adrenal GIST in a 34-year-old male who presented with a 5-week history of gradually progressive left flank pain and early satiety. Whole-body positron emission tomography (PET)/computed tomography (CT) scan showed a 14 × 11 cm hypermetabolic (18)fluorodeoxyglucose (FDG)-avid mass lesion involving the left adrenal gland and dorsal part of the left hemi-diaphragm. Biopsy of the lesion revealed tumor cells that are immunoreactive to CD-117 and CD-34 and negative to CD-31, S-100, cytokeratin, desmin, and vimentin, features characteristic of GIST. The patient was given imatinib, which drastically decreased his complaints with almost complete resolution of the tumor on his last follow-up radiographic images. Primary left adrenal GIST is an extremely rare neoplasm and can be confused with GISTs arising from the greater curvature of the stomach. Imatinib therapy is optimal in the management of these tumors.
胃肠道间质瘤(GISTs)是胃肠道(GI)的罕见肿瘤。这些肿瘤在胃肠道外异位发生的情况极为罕见。文献中仅报道过一例原发性肾上腺 GIST。在本报告中,我们报道了第二例发生于 34 岁男性的原发性肾上腺 GIST,该患者表现为 5 周逐渐进展的左侧腰痛和早饱。全身正电子发射断层扫描(PET)/计算机断层扫描(CT)显示一个 14×11cm 的高代谢(18)氟脱氧葡萄糖(FDG)活性肿块病变,累及左肾上腺和左膈肌背部。病变活检显示肿瘤细胞对 CD-117 和 CD-34 免疫反应阳性,对 CD-31、S-100、细胞角蛋白、结蛋白和波形蛋白阴性,具有 GIST 的特征。患者接受了伊马替尼治疗,几乎完全缓解了他的症状,肿瘤也明显缩小,在最后一次影像学随访时完全消失。原发性左肾上腺 GIST 是一种非常罕见的肿瘤,可能与起源于胃大弯的 GIST 混淆。伊马替尼治疗是这些肿瘤的最佳治疗选择。