Management of Giant Bilateral Adrenal Myelolipomas in Congenital Adrenal Hyperplasia

先天性肾上腺增生症伴巨大双侧肾上腺髓脂肪瘤的治疗

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Abstract

Adrenal myelolipomas are rare, benign lesions containing mature adipose tissue and hematopoietic elements, typically identified incidentally on radiographic imaging. Management of small masses less than 5 cm usually requires no intervention, whereas those exceeding 6 cm in size are classified as "giant" and can cause mass effect and potentially fatal hemorrhage. Surgery must be considered for masses deemed at risk to cause complications. A 44-year-old man with a history of congenital adrenal hyperplasia presented with giant bilateral adrenal myelolipomas, which on computed tomography (CT) imaging showed significant displacement of the kidneys and compression of the inferior vena cava. Open adrenalectomy was performed, resulting in the resection of a 30.0-cm left adrenal myelolipoma and a 27.5-cm right adrenal myelolipoma. The postoperative course was uneventful. Bilateral adrenal myelolipomas of this size have rarely been reported. Management of such giant adrenal masses requires an individualized treatment approach. Bilateral adrenalectomy proved to be a safe and effective option for this patient after performing shared decision-making regarding quality of life. Highlighted here is the importance of patient-centered care in management of similar benign lesions.

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