Roger B Rathna, Dhali Arkadeep, Ramesh Rakesh S, Dsouza Christopher
Department of Surgical Oncology, St. John's Medical College, Bangalore, Karnataka, India.
Indian J Endocrinol Metab. 2020 Nov-Dec;24(6):551-553. doi: 10.4103/ijem.IJEM_737_20. Epub 2021 Jan 12.
Adrenal myelolipomas are nonfunctional tumors that are usually asymptomatic; however, they have been known to coexist with other endocrine disorders, such as Cushing's syndrome, congenital adrenal hyperplasia (CAH), Conn's syndrome, and pheochromocytoma. We report a case of a 49-year-old man with hypertension and diabetes mellitus who complained of chronic abdominal pain, vomiting, and early satiety. Preoperative contrast-enhanced computerized tomography (CECT) was performed, and adrenal myelolipoma was considered, lab investigations revealed a nonfunctional tumor. CECT also revealed bilateral renal cortical cyst, right renal calculi, and hepatic cyst. A left open cortical sparing adrenalectomy was performed, pathological examination confirmed the diagnosis, and a radiological surveillance was planned for the right tumor. Four years following this, the patient came back with a similar presentation. Right adrenalectomy was performed after preoperative workup, and subsequently steroid replacement therapy was initiated. We suggest adequate follow-up of a patient presenting with adrenal myelolipoma and to explore the possibility of establishing a syndromic diagnosis such as autosomal dominant polycystic kidney disease (ADPKD).
肾上腺髓质脂肪瘤是无功能肿瘤，通常无症状；然而，已知它们可与其他内分泌疾病共存，如库欣综合征、先天性肾上腺增生（CAH）、原发性醛固酮增多症和嗜铬细胞瘤。我们报告一例49岁男性患者，患有高血压和糖尿病，主诉慢性腹痛、呕吐和早饱。术前行增强计算机断层扫描（CECT），考虑为肾上腺髓质脂肪瘤，实验室检查显示为无功能肿瘤。CECT还显示双侧肾皮质囊肿、右肾结石和肝囊肿。实施了左侧开放式保留肾上腺皮质切除术，病理检查确诊，计划对右侧肿瘤进行影像学监测。四年后，患者再次出现类似症状。术前检查后实施了右侧肾上腺切除术，随后开始进行类固醇替代治疗。我们建议对肾上腺髓质脂肪瘤患者进行充分随访，并探讨建立综合征诊断的可能性，如常染色体显性多囊肾病（ADPKD）。
