Adreno-cortical carcinoma in infancy and childhood: a radiological report of ten cases

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.