Kienitz Tina, Schwander Jörg, Bogner Ulrich, Schwabe Michael, Steinmüller Thomas, Quinkler Marcus
Endocrinology in Charlottenburg, Stuttgarter Platz 1, Berlin, Germany.
Pathology, DRK-Kliniken Westend, Berlin, Germany.
Endocrinol Diabetes Metab Case Rep. 2021 Mar 31;2021. doi: 10.1530/EDM-20-0204.
Apart from adrenal myelolipomas, adrenal lipomatous tumors are rare and only seldom described in the literature. We present the case of a 50-year-old man, with a classical form of congenital adrenal hyperplasia (CAH), which was well treated with prednisolone and fludrocortisone. The patient presented with pollakisuria and shortness of breath while bending over. On MRI, fat-equivalent masses were found in the abdomen (14 × 19 × 11 cm on the right side and 10 × 11 × 6 cm on the left side). The right adrenal mass was resected during open laparotomy and the pathohistological examination revealed the diagnosis of an adrenal lipoma. Symptoms were subdued totally postoperatively. This is the first report of a bilateral adrenal lipoma in a patient with CAH that we are aware of.
Macronodular hyperplasia is common in patients with congenital adrenal hyperplasia (CAH). Solitary adrenal tumors appear in approximately 10% of adult CAH patients and are often benign myelolipomas. The Endocrine Society Clinical Practice Guideline does not recommend routine adrenal imaging in adult CAH patients. Adrenal imaging should be performed in CAH patients with clinical signs for an adrenal or abdominal mass. Adrenal lipoma is rare and histopathological examinations should rule out a differentiated liposarcoma.
除肾上腺髓脂肪瘤外，肾上腺脂肪瘤性肿瘤较为罕见，文献中仅有很少的描述。我们报告一例50岁男性患者，患有典型的先天性肾上腺皮质增生症（CAH），使用泼尼松龙和氟氢可的松治疗效果良好。患者出现尿频及弯腰时呼吸急促症状。磁共振成像（MRI）检查发现腹部有脂肪等效肿块（右侧为14×19×11厘米，左侧为10×11×6厘米）。在开放剖腹手术中切除了右侧肾上腺肿块，病理组织学检查确诊为肾上腺脂肪瘤。术后症状完全缓解。据我们所知，这是首例关于CAH患者双侧肾上腺脂肪瘤的报告。
大结节性增生在先天性肾上腺皮质增生症（CAH）患者中很常见。约10%的成年CAH患者会出现孤立性肾上腺肿瘤，且多为良性髓脂肪瘤。美国内分泌学会临床实践指南不建议对成年CAH患者进行常规肾上腺成像检查。对于有肾上腺或腹部肿块临床体征的CAH患者，应进行肾上腺成像检查。肾上腺脂肪瘤很罕见，组织病理学检查应排除分化型脂肪肉瘤。
