LETTERS TO THE EDITOR
Androgen secreting adrenocortical carcinomas
EDITOR,-As oncologists we read Wolthers et 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 metas- tasis) with an analysis of histological charac- teristics. Like others, Wolthers et 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 malig- nant 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 intrin- sic tumour biology. We therefore recommend that tumour samples from each of these rare cases are appropriately stored for future bio- logical analysis (United Kingdom Children’s Study Group wide protocol for collecting and banking tumour specimens for biological studies (98 BS 05); The UKCCSG, Univer- sity 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 pro- pose 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 manage- ment, clinical trials of treatment and national/ international audit of long term endocrino- logical 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 metasta- sis. We have initiated the first steps towards this goal through the officers of the UK Chil- dren’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.
RICHARD GRUNDY
Clinical Senior Lecturer in Paediatric Oncology, Institute of Child Health, University of Birmingham, Whittal Street, Birmingham B4 6NH, UK email: r.g.grundy@bham.ac.uk
DAVID A WALKER
Chairman UKCCSG Rare Tumour Committee, Honorary Consultant/Senior Lecturer in Paediatric Oncology, Division of Child Health, School of Human Development, Queen’s Medical Centre, University of Nottingham, Nottingham NG7 2UH, UK
email: David. Walker@nottingham.ac.uk
1 Wolthers OD, Cameron FJ, Schimberg I, et al. Androgen secreting adrenocortical carcinomas. Arch Dis Child 1999;80:46-50.
2 Bonacci R, Gigliotti A, Baudin E, et al. Cytotoxic therapy with etoposide and cisplatin in advanced adrenocortical carcinoma. Br J Cancer 1998;78:546-9.
3 Wagner J, Portwine C, Rabin K, Leclerc J-M, Narod SA, Malkin D. High frequency of germ- line mutations in childhood adrenocortical cancer. J Natl Cancer Inst 1994;86:1707-10.
Fatal chickenpox: negative electron microscopy of vesicular samples may be misleading
EDITOR,-A 12 year old boy was referred with a three day history of severe abdominal and chest pain radiating to the back. He had a vesiculo-haemorrhagic rash affecting his trunk and face for two days. He had been taking prednisolone (2 mg/kg/day) and diu- retics since being diagnosed with rheumatic carditis four weeks previously. Two days before admission chickenpox was considered as a diagnosis in the referring hospital, but a vesicle scrape taken for electron microscopy was negative.
On admission ischaemic bowel was sus- pected, and a laparotomy and oesophagogas- troduodenoscopy were performed. Apart from a haemorrhagic oesophagitis and a moderately engorged liver, no abnormalities were found. The next day his condition dete- riorated with shock, hepatitis, and coagulopa- thy. A repeat vesicle scrape was again negative on electron microscopy, but immunofluores- cence demonstrated varicella zoster virus (VZV); serum VZV IgG was not detected. Despite treatment with intravenous aciclovir and foscarnet along with full intensive care unit support, he developed multiorgan failure and died three days later.
Chickenpox specific, and possibly lifesaving,1 antiviral treatment was delayed by two days in this case as a result of negative electron microscopy of a vesicle scrape. This
is not a sensitive technique, only detecting virus at a concentration of 106 to 107/ml.2 In a review of paired samples sent to our labora- tory over the past two years, nine of 33 sam- ples positive for VZV by immunofluorescence gave false negative results by electron micros- copy. Severe abdominal or back pain, fre- quently preceding the vesicular rash, appears to be a common feature of complicated vari- cella in immunocompromised patients,3 and aciclovir may be beneficial even at the time of visceral dissemination.1 4 Treatment of these children should thus start without delay, and the results of examination of vesicular material, particularly where electron micros- copy alone is available, must be interpreted with caution.
JL KLEIN Lecturer in Microbiology, Guy’s, King’s and St Thomas’ School of Medicine, London SE1 7EH, UK
D C GARVIE Consultant Paediatrician, Children’s Hospital, Lewisham, London SE13 6LH, UK
R TULLOH Consultant Paediatric Cardiologist, Guy’s and St Thomas’ NHS Trust
M MARSH Consultant in Paediatric Intensive Care, Guy’s and St Thomas’ NHS Trust
E MACMAHON Consultant Virologist, Guy’s and St Thomas’ NHS Trust
1 Balfour HH Jr. Intravenous acyclovir therapy for varicella in immunocompromised children. } Paediatr 1984;104:134-6.
2 Doane FW, Anderson N. Methods for preparing specimens for electron microscopy. In: Electron microscopy in diagnostic virology. Cambridge: Cambridge University Press 1987:14-31.
3 Morgan ER, Smalley LA. Varicella in immuno- compromised children. Incidence of abdomi- nal pain and organ involvement. Am J Dis Child 1983;137:883-5.
4 Shulman ST. Acyclovir treatment of dissemi- nated varicella in childhood malignant neo- plasms. Am J Dis Child 1985;139:137-40.
CORRECTION
Kearns Sayre syndrome initially pre- senting as hypomelanosis of Ito. Arch Dis Child 1999;81:280.
The authors for this letter should have been: Talia Kakourou, Anastasia Garoufi, Nikolaidou Polyxeni, Evmorfia Dafni, Migdaleni Tsamouri, A Papadimitriou, T Karpathios and not as published.
The error is regretted.
Protected by copyright, including for uses related to text and data mining, Al training, and similar technologies. Arch Dis Child: first published as 10.1136/adc.82.2.183 on 1 February 2000. Downloaded from http://adc.bmj.com/ on April 4, 2026 at National Library of Medicine.