双侧巨大肾上腺髓脂瘤

肾上腺髓脂瘤是较罕见的良性肿瘤，由成熟脂肪细胞和造血细胞组成。通常在尸检或影像学检查时被偶尔发现。多数病灶孤立，体积较小，虽然无激素活性，但与功能性肾上腺疾病如库欣综合征及先天性肾上腺增生症罕有关联。该类患体内高水平的促肾上腺皮质激素被认为是肿瘤的促发因素。该报道了1例有先天性肾上腺增生症的双侧巨大肾上腺髓脂瘤患的影像学资料。据了解，这是所报道的与先天性肾上腺增生症相关的最大双侧肾上腺髓脂瘤。该首次报道了经一段时间皮质激素治疗后，该患影像学表现的显变化。

Bilateral giant adrenal myelolipomas

Adrenal myelolipomas are rare benign tumors that comprise mature adipose cells and hematopoietic elements. They are usually found incidentally at autopsy and on imaging. Most cases arc isolated and small. Although they are not hormonally active, there is very rarely an association with functional adrenal disorders such as Cushing syndrome and congenital adrenal hyperplasia. It is believed that in these patients high corticotropin levels contribute to the pathogenesis of these neoplasms. We report the imaging appearances of bilateral giant adrenal myelolipomas in a patient known to have congenital adrenal hyperplasia. To our knowledge, these are the largest bilateral adrenal myelolipomas reported in association with congential adrenal hyperplasia. A distinct change in their appearances after a period of steroid treatment is described for the first time.