Background: Ovarian adrenal rest tumours (OARTs) are rare in contrast to testicular adrenal rest tumours (TARTs).
Objective and hypotheses: To summarise the characterization of OART in children and adolescent females with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency (21-OHD).
Method: We have diagnosed four cases of CAH 21-OHD with OART in the recent 5 years and summarised the characterisations.
Results: The four cases of CAH 21-OHD included three salt wasters and one simple virilizers. OART were diagnosed at the age of 8.9 years, 15.8 years, 21.4 years and 9.3 years respectively. There were histories of CAH poor control before the diagnosis of OART. The diagnosis could be confirmed before the operation in only one case. The diagnosis could not be made until the exploratory surgery in the other three cases. The follow-up periods of OART were 4.8 years, 4.7 years, 3.8 years and 2.7 years respectively. Removal of OART resulted in symptoms relieved at least partly.
Conclusion: The diagnosis of OART is much more difficult than TART. Doctors should think about OART in CAH 21-OHD patients with poor control. In difficult cases of CAH with negative imaging finding may need further exploratory surgery. Removal of the OART resulted in symptoms relieved at least partly.
01 - 03 Oct 2015
European Society for Paediatric Endocrinology