title: Adrenocortical tumors in Italian children: analysis of clinical characteristics and P53 status. Data from the national registries.
abstract: OBJECTIVE
  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.
  
  
  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.
authors.affiliation: Pediatric Surgery Department, University-Hospital of Padua, Padua, Italy.
authors.forename: Patrizia
authors.initials: P
authors.lastname: Dall'Igna
authors.affiliation: Pediatric Surgery Department, University-Hospital of Padua, Padua, Italy. Electronic address: calogero.virgone@unipd.it.
authors.forename: Calogero
authors.initials: C
authors.lastname: Virgone
authors.affiliation: Clinical Trials and Biostatistics Unit, Istituto Oncologico Veneto IRCCS, Padua, Italy.
authors.forename: Gian Luca
authors.initials: GL
authors.lastname: De Salvo
authors.affiliation: Immunologia e Diagnostica Molecolare Oncologica, Istituto Oncologico Veneto IRCCS, Padua, Italy.
authors.forename: Roberta
authors.initials: R
authors.lastname: Bertorelle
authors.affiliation: Pediatric Department, Pediatric Oncology Service, Second University of Naples, Naples, Italy.
authors.forename: Paolo
authors.initials: P
authors.lastname: Indolfi
authors.affiliation: Clinical Trials and Biostatistics Unit, Istituto Oncologico Veneto IRCCS, Padua, Italy.
authors.forename: Angela
authors.initials: A
authors.lastname: De Paoli
authors.affiliation: Pediatric Surgery Department, Istituto Giannina Gaslini, Genova, Italy.
authors.forename: Piero
authors.initials: P
authors.lastname: Buffa
authors.affiliation: Pediatric Oncology Department, Istituto Giannina Gaslini, Genova, Italy.
authors.forename: Massimo
authors.initials: M
authors.lastname: Conte
authors.affiliation: Immunologia e Diagnostica Molecolare Oncologica, Istituto Oncologico Veneto IRCCS, Padua, Italy.
authors.forename: Giovanni
authors.initials: G
authors.lastname: Esposito
authors.affiliation: Pediatric Surgery Department, Bambino Gesù Hospital, Rome, Italy.
authors.forename: Alessandro
authors.initials: A
authors.lastname: Inserra
authors.affiliation: Immunologia e Diagnostica Molecolare Oncologica, Istituto Oncologico Veneto IRCCS, Padua, Italy.
authors.forename: Cinzia
authors.initials: C
authors.lastname: Candiotto
authors.affiliation: Servizio di Anatomia e Istologia Patologica, Azienda Ospedaliera di Rilievo Nazionale, Santobono Pausillipon, Naples, Italy.
authors.forename: Vittoria
authors.initials: V
authors.lastname: D'Onofrio
authors.affiliation: Pathology Department, Bambino Gesù Hospital, Rome, Italy.
authors.forename: Renata
authors.initials: R
authors.lastname: Boldrini
authors.affiliation: Pediatric Oncology Unit, Istituto Nazionale Tumori, Milan, Italy.
authors.forename: Andrea
authors.initials: A
authors.lastname: Ferrari
authors.affiliation: Pediatric Oncology Department, University-Hospital of Padua, Padua, Italy.
authors.forename: Gianni
authors.initials: G
authors.lastname: Bisogno
authors.affiliation: Pathology Department, University-Hospital of Padua, Padua, Italy.
authors.forename: Rita
authors.initials: R
authors.lastname: Alaggio
authors.affiliation: Pediatric Surgery Department, University-Hospital of Padua, Padua, Italy.
authors.forename: Giovanni
authors.initials: G
authors.lastname: Cecchetto
mesh_terms: D000306:Adrenal Cortex Neoplasms / Q000235:genetics* / Q000401:mortality / Q000473:pathology* / Q000601:surgery; D000970:Antineoplastic Agents / Q000627:therapeutic use; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D005260:Female; D016158:Genes, p53 / Q000235:genetics*; D006801:Humans; D007150:Immunohistochemistry; D007558:Italy; D008297:Male; D009154:Mutation; D009361:Neoplasm Invasiveness; D012042:Registries; D012189:Retrospective Studies; D017422:Sequence Analysis, DNA; D016019:Survival Analysis
chemical_list: D000970:Antineoplastic Agents
keywords: Pediatric adrenocortical tumors; TREP project; Wienecke score system; p53 mutation