[Corticosuprarenaloma in children].
Thirty-four cases of corticosurrenaloma with clinical onset before 15 years of age have been studied. Higher frequency in girls (65 percent of the cases) and in young age (80 percent before 5 years of age, 43 percent before 2 years), association with personal or familial other tumors and malformations, are noticeable features. Virilism is the major manifestation in children (83 percent of the cases), either isolated or associated with hypercortisolism and/or feminization. Hormonal assays are of little value, and the dynamic adrenal tests are of some help only in small tumors or isolated hypercortisolism. Radiological diagnosis by urography with cavography, arteriography, is easy in most cases. Evaluation of prognosis is very difficult. Clinical and biological data are not significant, histological data are often of little help. Only a very large mass, and occurrence of metastases (liver, lungs) evidence malignancy. This series does not allow to assess definitely the best therapeutic regimen. Surgery has to be performed as soon as possible. The effects of radiations have not yet been ascertained. High doses of op'DDD have led to a sustained remission of the tumor or metastases in 2 patients, and perhaps to a longer survival in 4 others.
D015065:17-Hydroxycorticosteroids / Q000652:urine; D015068:17-Ketosteroids / Q000652:urine; D000236:Adenoma / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000235:genetics / Q000473:pathology*; D000293:Adolescent; D000306:Adrenal Cortex Neoplasms / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000235:genetics / Q000473:pathology*; D000310:Adrenal Gland Neoplasms / Q000473:pathology*; D000308:Adrenocortical Hyperfunction / Q000175:diagnosis; D000792:Angiography; D002648:Child; D002675:Child, Preschool; D003480:Cushing Syndrome / Q000175:diagnosis; D003687:Dehydroepiandrosterone / Q000652:urine; D005260:Female; D005262:Feminization / Q000175:diagnosis; D006801:Humans; D008297:Male; D008939:Mitotane / Q000627:therapeutic use; D014567:Urography; D014770:Virilism / Q000175:diagnosis
D015065:17-Hydroxycorticosteroids; D015068:17-Ketosteroids; D003687:Dehydroepiandrosterone; D008939:Mitotane
Les tumeurs cortico-surrenales de l'enfant. Etude retrospective de 34 cas observes de 1954 a 1973