Radiotherapy for pediatric adrenocortical carcinoma - Review of the literature

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Wiegering V., Riedmeier M., Thompson L. D. R., Virgone C., Redlich A., Kuhlen M., ...More

CLINICAL AND TRANSLATIONAL RADIATION ONCOLOGY, vol.35, pp.56-63, 2022 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Review
  • Volume: 35
  • Publication Date: 2022
  • Doi Number: 10.1016/j.ctro.2022.05.003
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.56-63
  • Keywords: Pediatric adrenocortical cancer, Pediatric adrenocortical carcinoma, Pediatric adrenocortica l tumor, Radiotherapy, Therapy, Treatment, ADRENAL-CORTICAL TUMORS, LI-FRAUMENI SYNDROME, PROGNOSTIC-FACTORS, HOSPITAL VOLUME, CHILDREN, MANAGEMENT, CHILDHOOD, NEOPLASMS, MUTATION, IMPACT
  • Hacettepe University Affiliated: Yes


Background and purpose: Pediatric adrenocortical carcinoma (pACC) is a rare disease with poor prognosis. Pub-lications on radiotherapy (RT) ar e scarce. This review summarizes the current data on RT for pACC and possibly provides first evidence to justi f y its use in this setting. Materials and methods: We searched the PubMed and Embase database for manuscripts regarding RT for pACC. Results: We included 17 manuscripts reporting on 76 patients treated with RT, after screenin g 2961 references and 269 f u l l articles. In addition, we added data of 4 unreported pACC patients treated by co-authors. A l l reports based on retrospective data. Median age at first diagnosis was 11.1 years (70% female); 78% of patients pre-sented with hormonal activity. RT was mostly performed for curative intent (78%). 88% of RT were administered during primary therapy. The site of RT was predominantly the local tumor bed (76%). Doses of RT ranged from 15 to 62 Gy (median 50 Gy). Information on target volumes or fractionation were lacking. Median follow-up was 6,9 years and 64% of the patients died of disease, with 33% alive without disease. In 16 of 48 patients with available follow-up data after adjuvant RT (33%) no recurrence was reported and in 3 of 9 patients palliative RT seemed to induce some benefit for the patient. Conclusions: Our first systematic review on RT for pACC provides too few data for any general recommendation, but adjuvant RT in patients with high risk might be considered. International collaborative studies are urgently needed to establish better evidence on the role of RT in this rare malignancy.