AIM: Adrenocortical tumors are very rare in children.
The distinction between adenoma and carcinoma is complex because of their clinical/histological characteristics. The analysis of the cases registered in two consecutive Italian Studies is described, in order to provide additional insight into their nature and possibly identify benign and malignant lesions.
MATERIALS AND METHODS: The analysis includes patients registered from?? 1.1982 to 6.2011 into two consecutive Italian protocols.
RESULTS: Fifty-eight children (age 2-210months) were evaluated. Endocrine manifestations were the most frequent symptoms. Stage distribution at diagnosis was: ST I 35, ST II 17, ST III 1, ST IV 5. Treatment consisted in mitotane for ST II, mitotane+chemotherapy for ST III/IV. Forty-four patients are alive without evidence of disease, 1 is alive with disease, 12 died of disease and 1 because of cardiomyopathy. The Wienecke score system was applied in 24 patients with good significance. A p53 mutation was found in 7 cases, and it was diagnostic for Li-Fraumeni syndrome in 2 benign tumors.
CONCLUSIONS: The results highlight the importance of a complete excision to obtain the cure of patients. The efficacy of chemotherapy is controversial, however it was able to control the disease in 4 patients in ST II. The value of the Wienecke score system in predicting patients' outcome was confirmed. p53 mutation was more frequent in malignant tumors and represented the sentinel of the Li-Fraumeni syndrome.
Written by:
Dall'Igna P, Virgone C, De Salvo GL, Bertorelle R, Indolfi P, De Paoli A, Buffa P, Conte M, Esposito G, Inserra A, Candiotto C, D'Onofrio V, Boldrini R, Ferrari A, Bisogno G, Alaggio R, Cecchetto G. Are you the author?
Pediatric Surgery Department, University-Hospital of Padua, Padua, Italy; Clinical Trials and Biostatistics Unit, Istituto Oncologico Veneto IRCCS, Padua, Italy; Immunologia e Diagnostica Molecolare Oncologica, Istituto Oncologico Veneto IRCCS, Padua, Italy; Pediatric Department, Pediatric Oncology Service, Second University of Naples, Naples, Italy; Pediatric Surgery Department, Istituto Giannina Gaslini, Genova, Italy; Pediatric Oncology Department, Istituto Giannina Gaslini, Genova, Italy; Pediatric Surgery Department, Bambino Gesù Hospital, Rome, Italy; Servizio di Anatomia e Istologia Patologica, Azienda Ospedaliera di Rilievo Nazionale, Santobono Pausillipon, Naples, Italy; Pathology Department, Bambino Gesù Hospital, Rome, Italy; Pediatric Oncology Unit, Istituto Nazionale Tumori, Milan, Italy; Pediatric Oncology Department, University-Hospital of Padua, Padua, Italy; Pathology Department, University-Hospital of Padua, Padua, Italy.
Reference: J Pediatr Surg. 2014 Sep;49(9):1367-71.
doi: 10.1016/j.jpedsurg.2014.03.006
PubMed Abstract
PMID: 25148739
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