Adrenocortical tumors in Italian children: Analysis of clinical characteristics and P53 status. Data from the national registries

作者：Dall' Igna Patrizia; Virgone Calogero^*; De Salvo Gian Luca; Bertorelle Roberta; Indolfi Paolo; De Paoli Angela; Buffa Piero; Conte Massimo; Esposito Giovanni; Inserra Alessandro; Candiotto Cinzia; D' Onofrio Vittoria; Boldrini Renata; Ferrari Andrea; Bisogno Gianni; Alaggio Rita; Cecchetto Giovanni

来源：Journal of Pediatric Surgery, 2014, 49(9): 1367-1371.

DOI：10.1016/j.jpedsurg.2014.03.006

• Pediatric adrenocortical tumors
• TREP project
• Wienecke score system
• p53 mutation

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. %26lt;br%26gt;Materials and Methods: The analysis includes patients registered from?? 1.1982 to 6.2011 into two consecutive Italian protocols. %26lt;br%26gt;Results: Fifty-eight children (age 2-210 months) 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. %26lt;br%26gt;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%26apos; outcome was confirmed. p53 mutation was more frequent in malignant tumors and represented the sentinel of the Li-Fraumeni syndrome.

• 出版日期2014-9