Radiotherapy for pediatric adrenocortical carcinoma - Review of the literature

Verena Wiegering; Maria Riedmeier; Lester D R Thompson; Calogero Virgone; Antje Redlich; Michaela Kuhlen; Melis Gultekin; Bilgehan Yalcin; Boris Decarolis; Christoph Härtel; Paul-Gerhardt Schlegel; Martin Fassnacht; Beate Timmermann

doi:10.1016/j.ctro.2022.05.003

Radiotherapy for pediatric adrenocortical carcinoma - Review of the literature

Clin Transl Radiat Oncol. 2022 May 14:35:56-63. doi: 10.1016/j.ctro.2022.05.003. eCollection 2022 Jul.

Authors

Verena Wiegering^{1

2}, Maria Riedmeier¹, Lester D R Thompson³, Calogero Virgone⁴, Antje Redlich⁵, Michaela Kuhlen⁶, Melis Gultekin⁷, Bilgehan Yalcin⁸, Boris Decarolis⁹, Christoph Härtel^{1

2}, Paul-Gerhardt Schlegel^{1

2}, Martin Fassnacht^{2

10}, Beate Timmermann¹¹

Affiliations

¹ University Childreńs Hospital, Department of Pediatric Hematology, Oncology and Stem Cell Transplantation, University of Wuerzburg, Josef-Schneiderstr. 2, 97080 Wuerzburg, Germany.
² Comprehensive Cancer Centre Mainfranken, University of Wuerzburg Medical Centre, Josef-Schneiderstr. 2, 97080 Wuerzburg, Germany.
³ Head and Neck Pathology Consultations, Woodland Hills, CA, USA.
⁴ Pediatric Surgery, Department of Woman's and Child's Health, University Hospital of Padua, Padua, Italy.
⁵ Pediatric Oncology, Otto-von-Guericke-University, Magdeburg, Germany.
⁶ Swabian Children's Cancer Center, University Children's Hospital Augsburg, Augsburg, Germany.
⁷ Department of Radiation Oncology, Hacettepe University, Faculty of Medicine, 06100 Ankara, Turkey.
⁸ Department of Pediatric Oncology, Hacettepe University, Faculty of Medicine, 06100 Ankara, Turkey.
⁹ Department of Pediatric Oncology and Hematology, University Children's Hospital of Cologne, Medical Faculty, Cologne, Germany.
¹⁰ Department of Medicine, Division of Endocrinology and Diabetes, University Hospital, University of Wuerzburg, Oberduerrbacherstr. 6, 97080 Wuerzburg, Germany.
¹¹ Department of Particle Therapy, West German Proton Therapy Centre Essen (WPE), West German Cancer Center (WTZ), Germany, German Cancer Consortium (DKTK), University Hospital Essen, Essen, Germany.

Abstract

Background and purpose: Pediatric adrenocortical carcinoma (pACC) is a rare disease with poor prognosis. Publications on radiotherapy (RT) are scarce. This review summarizes the current data on RT for pACC and possibly provides first evidence to justify 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 screening 2961 references and 269 full articles. In addition, we added data of 4 unreported pACC patients treated by co-authors. All reports based on retrospective data. Median age at first diagnosis was 11.1 years (70% female); 78% of patients presented 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.

Keywords: Pediatric adrenocortical cancer; Pediatric adrenocortical carcinoma; Pediatric adrenocortical tumor; Radiotherapy; Therapy; Treatment.

Publication types

Review