Article Text

PDF

Androgen secreting adrenocortical carcinomas
  1. RICHARD GRUNDY, Clinical Senior Lecturer in Paediatric Oncology
  1. Institute of Child Health, University of Birmingham
  2. Whittal Street, Birmingham B4 6NH, UK
  3. email: r.g.grundy{at}bham.ac.uk
  4. Paediatric Oncology
  5. Division of Child Health, School of Human Development
  6. Queen's Medical Centre, University of Nottingham
  7. Nottingham NG7 2UH, UK
  8. email: David.Walker{at}nottingham.ac.uk
    1. DAVID A WALKER, Chairman UKCCSG Rare Tumour Committee, Honorary Consultant/Senior Lecturer in
    1. Institute of Child Health, University of Birmingham
    2. Whittal Street, Birmingham B4 6NH, UK
    3. email: r.g.grundy{at}bham.ac.uk
    4. Paediatric Oncology
    5. Division of Child Health, School of Human Development
    6. Queen's Medical Centre, University of Nottingham
    7. Nottingham NG7 2UH, UK
    8. email: David.Walker{at}nottingham.ac.uk

      Statistics from Altmetric.com

      Editor,—As oncologists we read Woltherset al's retrospective experience of androgen secreting adrenal cortical tumours with interest.1 This report is very important because it represents a substantial proportion of the UK's experience of this rare tumour of childhood. It also presents an opportunity to raise a number of important issues.

      The main scientific content of this paper focuses on an attempt to correlate malignant tumour behaviour (local invasion and metastasis) with an analysis of histological characteristics. Like others, Woltherset al were unable to identify any such correlation. There is, however, agreement that larger tumours (> 5 cm, 200 g, 200 cm3) are associated with a greater frequency of expression of malignant characteristics. To believe that small tumours are benign and big ones malignant is a misunderstanding of cancer, and to make progress we need to understand better intrinsic tumour biology. We therefore recommend that tumour samples from each of these rare cases are appropriately stored for future biological analysis (United Kingdom Children's Study Group wide protocol for collecting and banking tumour specimens for biological studies (98 BS 05); The UKCCSG, University of Leicester, 22–28 Princess Road West, Leicester LE1 6TP, UK).

      We do not know the optimal treatment for these tumours following surgery.2 As they are rare and have a substantial mortality, we propose a multicentre collaboration between endocrinologists and oncologists to address this issue. Such a collaboration would apply a multidisciplinary methodology to evaluate approaches to treatment through cancer registration, standardised clinical management, clinical trials of treatment and national/international audit of long term endocrinological and oncological outcome. This approach will prospectively recruit tumours nationally over a shorter time period and will have greater statistical power. It will involve specialists familiar with the needs of the child with cancer and with established links to those interested in researching factors that might predict tumour invasion and metastasis. We have initiated the first steps towards this goal through the officers of the UK Children's Cancer Study Group and the Royal College of Paediatrics and Child Health Endocrine Group.

      Finally, it is now recognised that adrenal cortical tumours are frequently associated with genetic causes of cancer predisposition, which may be the first manifestation of a familial cancer predisposition syndrome.3 Genetic investigation and counselling of the family are therefore an important part of patient management.

      References

      View Abstract

      Request permissions

      If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.