Preeyasombat C, Sirikulchayanonta V, Mahachokelertwattana P, Sriphrapradang A, Boonpucknavig S
Department of Pediatrics, Ramathibodi Hospital, Bangkok, Thailand.
Am J Dis Child. 1992 Sep;146(9):1103-5. doi: 10.1001/archpedi.1992.02160210105034.
Procedures were carried out in a 12-year-old girl to relate Ewing's sarcoma of the left tibia with Cushing's syndrome. Computed tomography revealed a normal pituitary and hypothalamus but bilateral adrenal hyperplasia without focal enlargement, thus readily excluding hypothalamic-pituitary-adrenal tumor. Negative results from a high-dose dexamethasone suppression test do not support pituitary-dependent Cushing's disease. Ewing's sarcoma was diagnosed on tibial biopsy. The regression of the physical and biochemical findings of Cushing's syndrome subsequent to amputation of the left lower leg strongly suggests ectopic Cushing's syndrome caused by Ewing's sarcoma. Immunohistochemical studies of the resected bone were negative for corticotropin but positive for corticotropin releasing factor-like peptide. We conclude that this is the first reported case of ectopic Cushing's syndrome in a child that is caused by Ewing's sarcoma secreting corticotropin releasing factor-like peptide.
对一名12岁女孩进行了一系列检查，以探究左胫骨尤因肉瘤与库欣综合征之间的关系。计算机断层扫描显示垂体和下丘脑正常，但双侧肾上腺增生且无局灶性增大，因此很容易排除下丘脑 - 垂体 - 肾上腺肿瘤。高剂量地塞米松抑制试验结果为阴性，不支持垂体依赖性库欣病。经胫骨活检诊断为尤因肉瘤。左小腿截肢后，库欣综合征的体格检查和生化检查结果消退，强烈提示为尤因肉瘤所致的异位库欣综合征。对切除的骨组织进行免疫组织化学研究，促肾上腺皮质激素检测为阴性，但促肾上腺皮质激素释放因子样肽检测为阳性。我们得出结论，这是首例报道的儿童因尤因肉瘤分泌促肾上腺皮质激素释放因子样肽而导致的异位库欣综合征病例。
