Lamas C, López L M, Lozano E, Atienzar M, Ruiz-Mondéjar R, Alfaro J J, Botella F
Department of Endocrinology, Complejo Hospitalario Universitario de Albacete, Universidad de Castilla-La Mancha, Albacete, Spain.
Exp Clin Endocrinol Diabetes. 2009 Sep;117(8):440-5. doi: 10.1055/s-0029-1202274. Epub 2009 Apr 16.
Adrenal myelolipomas are uncommon benign tumors, composed of mature adipose tissue and haematopoietic elements in varying proportions. They are usually asymptomatic, non-functioning adrenal incidentalomas, but there have been a few reports of myelolipomatous masses associated with adrenocortical hypersecretion. We report two cases of large mixed adrenal tumors, with heterogeneous appearance and areas of fat density in imaging techniques, and with autonomous cortisol production leading to Cushing's syndrome. Both underwent adrenalectomy and the histological study showed an adrenocortical adenoma with widespread myelolipomatous metaplasia. Hypercortisolism resolved in the one patient that could be evaluated after surgery. We review all the previous reported cases of hypercortisolism associated with adrenal myelolipomas. We also discuss the recommended diagnostic approach and therapeutic management of adrenal masses of lipomatous appearance.
肾上腺髓质脂肪瘤是一种罕见的良性肿瘤,由比例各异的成熟脂肪组织和造血成分组成。它们通常无症状,是无功能的肾上腺偶发瘤,但也有少数关于髓质脂肪瘤块与肾上腺皮质分泌过多相关的报道。我们报告两例大型混合性肾上腺肿瘤,在影像学检查中表现为外观不均一且有脂肪密度区域,伴有自主性皮质醇分泌导致库欣综合征。两例患者均接受了肾上腺切除术,组织学研究显示为肾上腺皮质腺瘤伴广泛的髓质脂肪瘤样化生。术后可评估的一名患者的高皮质醇血症得到缓解。我们回顾了既往所有报道的与肾上腺髓质脂肪瘤相关的高皮质醇血症病例。我们还讨论了对于脂肪瘤样外观肾上腺肿块的推荐诊断方法和治疗管理。