31 months old young girl. Adrenal mass. Virilization.
Left adrenal mass (3.5 X 3.4 X 3.4 cm) with high FDG avidity (SUV max 10.2). There is no hypometabolic region (necrosis). On CT, there is a calcification in the inferolateral part of the mass.
No regional or distant metastasis.
Moderate inflammatory activity of adenoids and tonsils associated with mild splenic uptake. Left elbow was the injection site. Focal cutaneous contamination on left side.
Adrenocortical carcinoma (ACC) is rare in pediatric and adult population. There is a bimodal age distribution with peaks in the first decade as well as in the 30-50 years range. In children, ACC and adrenocortical adenoma (ACA) are difficult to distinguish each other, even through pathological findings. They are usually regrouped as adrenocortical tumors (ACT) and most of them labeled as malignant in the literature.
The incidence is higher among girls, mostly before the age of four. In teenagers, ratio female/male is 1:1. Li-Fraumeni and Beckwith-Wiedemann syndromes are associated with childhood ACT.
Clinical features depend on the hormonal secretion of the tumor. Unlike tumors in adult, non-functional tumors comprise only 10% of the pediatric cases. Virilization is the most common presentation and occurs in young age. Cushing’s syndrome alone is relatively rare and is mostly associated with virilizing signs (mixed syndrome). Adolescents and young adult tend to have Cushing’s syndrome or non-functional tumors like older adults. Report from Michalkiewicz et al. suggests that in young child, ACT arises from fetal zone of adrenal cortex which is oriented toward dehydroepiandrosterone (DHEA) production. On the other hand, ACT in teenagers would originate from definitive adrenal cortex.
Good prognostic factors include stage 1 disease (defined as tumor completely excised with negative margins, tumor weight ≤ 200 g, and absence of metastasis), age < 4 years old and syndrome of virilizing alone.
Roles of PET/CT FDG
Few studies investigated the role of PET/CT FDG in ACT. Mackie et al. studied 12 patients with ACC to assess local or metastatic recurrence. They included 8 adults (24-71 years old) and 4 children (5-8 years old). They concluded that PET FDG was useful for the detection of recurrence. Most lesions were clearly FDG-avid. Occasional or very small tumor may not have been detected.
More recently, Takeuchi et al. published an analysis of 106 adults concerning the impact of PET/CT on the management of ACC. PET/CT changed the management in a small proportion of patients. Therefore, PET may be preferred to assess therapeutic response before anatomical changes.
Satoh et al. analyzed some PET parameters (whole body metabolic tumor volume, total lesion glycolysis and SUVmax) in stage IV ACC in adult population and found that patients with high metabolic burden had a worse prognosis.
Michalkiewicz et al : Clinical and outcome characteristics of children with adrenocortical tumors: a report from the International Pediatric Adrenocortical Tumor Registry. J Clin Oncol. 2004 Mar 1;22(5):838-45
Mackie at al : Use of [18F]fluorodeoxyglucose positron emission tomography in evaluating locally recurrent and metastatic adrenocortical carcinoma. J Clin Endocrinol Metab. 2006 Jul;91(7):2665-71
McHugh K. : Renal and adrenal tumours in children. Cancer Imaging. 2007 Mar 5;7:41-51
McAteer et al : Predictors of survival in pediatric adrenocortical carcinoma: a Surveillance, Epidemiology, and End Results (SEER) program study. J Pediatr Surg. 2013 May;48(5):1025-31
Takeuchi et al : Impact of ¹⁸F-FDG PET/CT on the management of adrenocortical carcinoma: analysis of 106 patients. Eur J Nucl Med Mol Imaging. 2014 Nov;41(11):2066-73
Satoh et al : Whole Body Metabolic Tumor Volume and Total Lesion Glycolysis Predict Survival in Patients with Adrenocortical Carcinoma. Ann Surg Oncol. 2015 Aug 18
Wilson J. : Adrenal tumors. Pediatric urology book.