Federici S, Galli G, Ceccarelli P L, Ferrari M, Cicognani A, Cacciari E, Dòmini R
Department of Pediatric Surgery, University of Bologna, Italy.
Eur J Pediatr Surg. 1994 Feb;4(1):21-5. doi: 10.1055/s-2008-1066060.
Adrenocortical tumors in children are extremely rare, accounting only for 0.3-0.4% of all neoplasms in this age. Most frequently they secrete hormones, resulting in virilization, Cushing's syndrome or feminization, while the non-functioning ones are unusual. The authors describe 12 cases observed in 13 years (1976-1989), with a mean age of 5 years. 9 cases showed virilization, 4 presented with Cushing's syndrome and in 5 patients an abdominal mass was palpable. One case was affected by Beckwith-Wiedemann's syndrome. I.V. urography was performed in 8 patients, arteriography in 4 and since 1982 all patients were submitted to abdominal sonography and CT scan or MR imaging. Urinary 17-ketosteroids, 17-hydroxycorticoids and serum testosterone and cortisol were tested in all children. Dexamethasone suppression test was performed in 7. All patients were treated with surgery which seems to be the most suitable treatment, while the real effectiveness of treatment by drug therapy with suppressors of steroidogenesis is not confirmed in children. Histopathological examination showed typical features of adenoma in 5 cases, of adenocarcinoma in 4, while three cases revealed border line forms classified as "atypical adenomas". At the moment 10 patients are alive with a follow-up ranging from 18 months to 14 years, while 2 children with adenocarcinoma are dead.
儿童肾上腺皮质肿瘤极为罕见，仅占该年龄段所有肿瘤的0.3 - 0.4%。它们最常分泌激素，导致男性化、库欣综合征或女性化，而非功能性肿瘤则较为少见。作者描述了13年（1976 - 1989年）间观察到的12例病例，平均年龄为5岁。9例表现为男性化，4例患有库欣综合征，5例可触及腹部肿块。1例患有贝克威思 - 维德曼综合征。8例患者进行了静脉肾盂造影，4例进行了动脉造影，自1982年起所有患者均接受了腹部超声检查以及CT扫描或磁共振成像检查。对所有儿童均检测了尿17 - 酮类固醇、17 - 羟皮质类固醇以及血清睾酮和皮质醇。7例进行了地塞米松抑制试验。所有患者均接受了手术治疗，手术似乎是最合适的治疗方法，而儿童使用类固醇生成抑制剂进行药物治疗的实际效果尚未得到证实。组织病理学检查显示5例为典型腺瘤特征，4例为腺癌，3例显示为归类为“非典型腺瘤”的交界性形态。目前10例患者存活，随访时间为18个月至14年，2例腺癌患儿死亡。
