Teinturier C, Pauchard M S, Brugières L, Landais P, Chaussain J L, Bougnères P F
Pediatric Endocrinology Service, Saint-Vincent de Paul Hospital, Paris, France.
Med Pediatr Oncol. 1999 Feb;32(2):106-11. doi: 10.1002/(sici)1096-911x(199902)32:2<106::aid-mpo7>3.0.co;2-j.
A retrospective study of 54 children was undertaken to define the clinical presentation and secretory patterns of adrenal tumors and to evaluate the outcome of surgical resection and medical therapy.
Different factors were studied in univariate and multivariate analysis by using the Cox proportional hazard model.
Median age at diagnosis was 4 years. Boys and girls were affected equally. The disease was revealed by virilization (61%) or by a palpable mass (39%) with a 0.1-5.5 year delay from initial symptoms. At initial examination, we found that 76% of children were virilized. Ninety-four percent of the tested tumors secreted androgens, which were associated with glucocorticoids in 36%. Adrenal tumors in children were smaller than in adults. Half of them measured less than 10 cm. There were recurrences in 40% of children. The survival rate at 5 years was 49%, 70% if resection was microscopically complete and 7% if not (P < 0.001).
In children, rare adrenal tumors have different diagnostic and prognostic characteristics than in adults; however, recurrences remain frequent. The efficacy of chemotherapy, mainly o,p'-DDD (Mitotane), remains to be evaluated in comparative trials.
对54例儿童进行了一项回顾性研究，以确定肾上腺肿瘤的临床表现和分泌模式，并评估手术切除和药物治疗的结果。
使用Cox比例风险模型对不同因素进行单变量和多变量分析。
诊断时的中位年龄为4岁。男孩和女孩受影响的程度相同。疾病通过男性化表现（61%）或可触及的肿块（39%）发现，从初始症状出现到确诊有0.1至5.5年的延迟。在初次检查时，我们发现76%的儿童有男性化表现。94%的检测肿瘤分泌雄激素，其中36%与糖皮质激素有关。儿童肾上腺肿瘤比成人的小。一半的肿瘤直径小于10厘米。40%的儿童出现复发。5年生存率为49%，显微镜下切除完整者为70%，不完整者为7%（P<0.001）。
在儿童中，罕见的肾上腺肿瘤具有与成人不同的诊断和预后特征；然而，复发仍然很常见。化疗的疗效，主要是邻对滴滴滴（米托坦），仍有待在比较试验中进行评估。
