Adrenocortical tumours in children: a review of surgical management at a tertiary care centre.
- Resource Type
- Academic Journal
- Authors
- Ardicli B; Department of Medical and Surgical Research, Hacettepe University, Institute of Health Sciences, Ankara, Turkey.; Department of Paediatric Surgery, Hacettepe University, Faculty of Medicine, Ankara, Turkey.; User IR; Department of Medical and Surgical Research, Hacettepe University, Institute of Health Sciences, Ankara, Turkey.; Department of Paediatric Surgery, Hacettepe University, Faculty of Medicine, Ankara, Turkey.; Ciftci AO; Department of Paediatric Surgery, Hacettepe University, Faculty of Medicine, Ankara, Turkey.; Akyuz C; Department of Paediatric, Paediatric Oncology Unit, Hacettepe University, Faculty of Medicine, Ankara, Turkey.; Kutluk MT; Department of Paediatric, Paediatric Oncology Unit, Hacettepe University, Faculty of Medicine, Ankara, Turkey.; Yalcin B; Department of Paediatric, Paediatric Oncology Unit, Hacettepe University, Faculty of Medicine, Ankara, Turkey.; Gonc N; Department of Paediatric, Paediatric Endocrinology Unit, Hacettepe University, Faculty of Medicine, Ankara, Turkey.; Ozon ZA; Department of Paediatric, Paediatric Endocrinology Unit, Hacettepe University, Faculty of Medicine, Ankara, Turkey.; Alikasifoglu A; Department of Paediatric, Paediatric Endocrinology Unit, Hacettepe University, Faculty of Medicine, Ankara, Turkey.; Oguz B; Department of Radiology, Paediatric Radiology Unit, Hacettepe University, Faculty of Medicine, Ankara, Turkey.; Haliloglu M; Department of Radiology, Paediatric Radiology Unit, Hacettepe University, Faculty of Medicine, Ankara, Turkey.; Orhan D; Department of Paediatrics, Paediatric Pathology Unit, Hacettepe University, Faculty of Medicine, Ankara, Turkey.; Tanyel FC; Department of Paediatric Surgery, Hacettepe University, Faculty of Medicine, Ankara, Turkey.; Karnak I; Department of Paediatric Surgery, Hacettepe University, Faculty of Medicine, Ankara, Turkey.; Ekinci S; Department of Medical and Surgical Research, Hacettepe University, Institute of Health Sciences, Ankara, Turkey.; Department of Paediatric Surgery, Hacettepe University, Faculty of Medicine, Ankara, Turkey.
- Source
- Publisher: Wiley-Blackwell Publishing Asia Country of Publication: Australia NLM ID: 101086634 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1445-2197 (Electronic) Linking ISSN: 14451433 NLM ISO Abbreviation: ANZ J Surg Subsets: MEDLINE
- Subject
- Language
- English
Background: Adrenocortical tumours (ACT) are rare tumours of childhood usually presenting with endocrine dysfunction. This retrospective study is designed to review our institutional experience in surgical management.
Methods: Records of children treated for ACT between 1999 and 2019 were reviewed retrospectively.
Results: The median age of 24 children was 78 months. Fourteen patients had adrenocortical carcinoma, nine had adrenocortical adenoma and one had neuroendocrine differentiation of ACT. Endocrine dysfunction was noted in 79% of the patients. Five patients had preoperative chemotherapy but none had a decrease in tumour size. Transabdominal approach was used in all but two patients who had thoracoabdominal incision for excision of giant tumours and ipsilateral lung metastases. Two patients had visceral excision to achieve R0 resection. Five patients, four of whom had spillage and one with partial resection died of widespread disease. Two patients with stage 4 adrenocortical carcinoma are still on chemotherapy. All patients with stage I-III disease who had total excision without spillage (n = 17) are disease-free for 2-170 months.
Conclusions: Our results show the importance of excision in ACT without spillage for survival. However, multicentre prospective studies should enhance the knowledge of children about ACT and develop alternative therapies for stage III and IV cases.
(© 2021 Royal Australasian College of Surgeons.)