Endocrine Abstracts

Bilateral macronodular or micronodular adrenal hyperplasias are related to ACTH-independent or ACTH-dependant pathologies. ACTH-independent bilateral macronodular adrenal hyperplasia (AIMAH) and primary pigmented adrenocortical disease (PPNAD) are classical but rare examples of ACTH-independent pathologies, causing Cushing Syndrome. PPNAD is presented as bilateral micronodular adrenal hyperplasia.

On the other hand, inherited defects in the enzymatic steps of cortisol biosynthesis result in a decrease in cortisol biosynthesis and a consequent increase in the secretion of ACTH, thereby stimulating adrenal hyperplasia. The groups of syndromes related to enzyme deficiencies is termed congenital adrenal hyperplasia (CAH). CAH can be found as bilateral diffuse, macronodular or micronodular adrenal hyperplasia.

We present five cases with different etiologies in order to review the topic which is sophisticated.

Case 1: A 68-year-old woman with a 40-year history of hypertension was refferred for further evaluation of bilateral adrenal metastases. Endocrinologic data of the patient showed ACTH-independent hypercortisolemia, bilateral macronodular adrenal hyperplasia, type 2 DM and hypertension.

Case 2: A 43-year-old woman with a 10-year of obesity was refferred for further evaluation of adrenal adenoma. Endocrinologic data of the patient showed ACTH-independent hypercortisolemia, bilateral macronodular adrenal hyperplasia and type2 DM.

Case 3: A 20-year-old woman with a 3-year history of hypertension was refferred for further evaluation. Endocrinologic data of the patient showed ACTH-independent hypercortisolemia, bilateral micronodular adrenal hyperplasia (PPNAD).

Case 4: A 45 years-old man was referred because of ambiguous genitalia. Endocrinologic data of the patient showed 21OH deficiency, hyperandrogenism, bilateral macronodular adrenal hyperplasia. The karyotype was 46XX. He/she was diagnosed as simple virilizing form of CAH due to 21OH deficiency.

Case 5: A 35-year-old women with a 17-year history of hirsutism, and a 2-year history of hypertension was refferred for further evaluation. Endocrinologic data of the patient showed partial 11beta-hydroxylase deficiency, hyperandrogenism and bilateral micronodular adrenal hyperplasia.