Jones G S, Shah K J, Mann J R
Clin Radiol. 1985 May;36(3):257-62. doi: 10.1016/s0009-9260(85)80055-6.
Adreno-cortical carcinoma is a relatively rare neoplasm in infancy and childhood. This review, covering a period of 18 years (1965-83), revealed 10 cases, the study being prompted by three patients in whom the final diagnosis was considerably delayed. There were eight females and two males and their ages ranged from 6 months to 14 years; 50% were below the age of 18 months at the time of the presentation. Seven patients had features of either virilism or precocious puberty and three of these also had stigmas of Cushing's syndrome. In patients where the endocrine disturbance was evident, the clinical diagnosis was made rapidly. However, delay in diagnosis occurred when endocrinopathy was absent. Radiology, including newer methods of imaging, contributed by revealing the tumour and its spread but did not suggest a conclusive diagnosis. At times, clinical features, biochemical findings, radiological investigations and even histological studies mislead, thus delaying the final diagnosis. These cases are illustrated and discussed.
肾上腺皮质癌在婴幼儿和儿童时期是一种相对罕见的肿瘤。本综述涵盖了18年(1965 - 1983年)的时间,共发现10例病例,该研究是由3例最终诊断被大幅延迟的患者引发的。其中有8名女性和2名男性,年龄从6个月至14岁不等;50%的患者在就诊时年龄低于18个月。7例患者有男性化或性早熟的特征,其中3例还伴有库欣综合征的体征。在内分泌紊乱明显的患者中,临床诊断迅速做出。然而,当不存在内分泌病变时,诊断会出现延迟。放射学检查,包括更新的成像方法,有助于发现肿瘤及其扩散情况,但无法得出确定性诊断。有时,临床特征、生化检查结果、放射学检查甚至组织学研究都会产生误导,从而延迟最终诊断。本文对这些病例进行了举例说明和讨论。