Giant myelolipomas and inadvertent bilateral adrenalectomy in classic congenital adrenal hyperplasia

[摘要] Myelolipomas have been reported in patients with congenital adrenal hyperplasia (CAH). ACTH excess, as seen withnon-adherence to glucocorticoid therapy, may be responsible for tumor development. We report a case of a 51-year-old manwith classic salt-wasting CAH managed on prednisone 7.5 mg daily and fludrocortisone who presented with chronic backpain and was found to have giant bilateral retroperitoneal masses. On computed tomography (CT) imaging, the masses wereheterogeneous, but contained predominantly low-density fat attenuation. The tumors were resected due to concern formalignancy and mass symptoms. Pathologic examination identified both retroperitoneal masses as myelolipomas. The lefttumor was 34!20!13 cm and weighed 4.7 kg and the right tumor was 20 cm in the largest dimension. Adrenal tissue waspresent in the specimen. The patient reported long-term compliance with glucocorticoid treatment. However, nobiochemical monitoring of ACTH levels had occurred. Therefore, it is unclear if ACTH excess contributed to the developmentof these large tumors in this patient. It was presumed that both adrenal glands were inadvertently removed during surgeryand the patient was treated with physiologic replacement doses of hydrocortisone and fludrocortisone postoperatively.In this case, the bilateral adrenalectomy was inadvertent. However, adrenalectomy can be considered as a treatment optionin patients with classical CAH under certain circumstances to avoid complications of glucocorticoid excess.

[学科分类] 血液学

[关键词] Adult;Male;White;United States;Adrenal;Adrenal;Cortisol;Congenital adrenal hyperplasia;Myelolipoma;Back pain;Paraesthesia;CT scan;MRI;PET scan;Histopathology;Adrenalectomy;Resection of tumour;Hydrocortisone;Fludrocortisone;Mineralocorticoids;Glucocorticoids;Radiology/Rheumatology;Surgery;Urology;Unique/unexpected symptoms or presentations of a disease;October;2015