Searchable abstracts of presentations at key conferences in endocrinology
Endocrine Abstracts (2013) 32 P41 | DOI: 10.1530/endoabs.32.P41

ECE2013 Poster Presentations Adrenal cortex (64 abstracts)

Non-classic adrenal hyperplasia (NCAH) in patients with bilateral adrenal incidentally discovered tumors

Elwira Przybylik-Mazurek , Marta Tracz-Bujnowicz , Sylwia Kuzniarz-Rymarz & Alicja Hubalewska-Dydejczyk


Department of Endocrinology, Jagiellonian University Medical College, Krakow, Poland.


The prevalence of adrenal incidentalomas in computed tomography (CT) studies ranges from 0.6–1.9%. Adrenal masses are detected bilaterally in 10–15% of the cases.

Non-classic adrenal hyperplasia (NCAH), also termed as late onset of CAH, is a very mild form of 21-hydroxylase deficiency. The incidence of disease is estimated at 0.1% of population. Reported prevalence in women with androgen excess range from 0.6 to 9%.

Some patients have an overresponsive glucocorticoid response to ACTH stimulation, possibly reflective of subtle adrenal hyperplasia.

The aim of the study was to evaluate the prevalence of NCAH among patients with incidentally discovered bilateral adrenal tumors.

Material and methods: Seventy eight patients, 18 men and 60 women aged from 42 to 74 years with incidentally discovered in computed tomography (CT) bilateral adrenal tumors were examined. The diameter of tumors ranged from 17 to 52 millimeter and features of CT scanning suggested their benign character. Excess of the glucocorticoids or mineralocorticoids was excluded. Patients were asymptomatic or presented hardly mild symptoms (in women hirsutism and/or menstrual dysfunction). In 41 persons, 10 men and 31 women basal and ACTH-stimulated 17-hydroxyprogesterone (17-HP) concentrations were measured. A diagnosis of 21-OH-deficient NCAH was considered in patients with the basal 17-HP elevated or with the stimulated 17-HP level more than or equal to 10 ng/ml.

Results: In 19 patients (46.3%) – 4 men and 15 women NCAH was diagnosed. Fourteen patients had both elevated basal and stimulated 17-hydroxyprogesterone, while in five cases only elevation of stimulated level was found.

Conclusions: i). Non-classic congenital adrenal hyperplasia is a common autosomal recessive disorder and in case of incidentally discovered bilateral adrenal hypertrophy NCAH should be taken into consideration. ii). A basal 17-HP level is a useful screening tool for NCAH. iii). ACTH stimulation tests are essential to make proper diagnosis in some cases.

Keywords: incidentaloma, NCAH, 17-hydroxyprogesterone, ACTH.

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