Background: Ovarian adrenal rest tumors (OART) is a rare complication of congenital adrenal hyperplasia (CAH) and is not very well characterized yet.
Objective and hypotheses: We report four cases of OART in Chinese girls with CAH due to 21-hydroxylase deficiency (21 OHD).
Method: We describe the clinical, imaging, and surgical findings of the patients.
Results: The four cases of CAH with OART included three salt wasters and one simple virilizer. The median age of OART diagnosis was 12.5 years. The median follow-up period of OART was 3.6 years. There were histories of CAH poor control, the median persisted period was 6.5 years, before the diagnosis of OART in all of the four patients. Their clinical presentations included severe acne (4), deepened voice (1), accelerated growth (1), primary (1), or secondary (1) amenorrhea. Rectal ultrasonography and MRI scan can find the mass in only one case. All the four girls underwent surgical removal and confirmed the diagnosis of OART. The OARTs could be a big mass or several small nidus with diameter ranging from 0.3 to 0.6 cm. OART located in broad ligament of uterus, between the fimbriae of uterine tube and ovary, and rectal bladder pit. The patients showed clinical relief after the operations.
Conclusion: OART is rare and much more difficult to diagnose compared to testicular ART. The diagnosis of OART can remain unconfirmed before the operation. Surgical removal is effective.
20 - 22 Sep 2014
European Society for Paediatric Endocrinology