Coexistence of unilateral adrenal macronodule and Cushing’s disease. Report of two cases

Abstract

The coexistence of an unilateral adrenal macronodule and Cushing's disease is well documented in only few reports. We hereby describe two patients with ACTH-dependent Cushing's syndrome and asymmetric adrenal involvement. In both patients the left-sided adrenal contained a nodule sized 1.5 and 1.4 cm, respectively, while the contralateral gland appeared as normal at abdominal computerized tomography. Adrenal scintiscan revealed a bilateral uptake of the tracer. Dynamic testing of hypothalamic-pituitary-adrenal axis was suggestive of pituitary dependency but neuroradiologic imaging was inconclusive. Inferior petrosal sinus sampling demonstrated a clear center to periphery ACTH gradient and an ACTH-producing pituitary microadenoma was found and removed in both cases. Hypopituitarism ensued postoperatively and Cushing's syndrome resolved but the adrenal nodules regressed only many month after operation. The present cases confirm that the presence of an adrenal mass in a patient with Cushing's syndrome is insufficient to confidently conclude for the adrenal dependency of the syndrome.

Adrenocortical Hyperplasia Associated with ACTH-dependent Cushing's Syndrome: Comparison of the Size of Adrenal Glands with Clinical and Endocrinological Data

“…The tumor mass in this female patient remained stable and had decreased only slightly 6 years after surgery when it appeared she was in clinical and endocrinological remission. The presence of unilateral adrenal macronodules associated with Cushing's disease is coincidental, as they represent approximately 1% of the non-functional adrenal mass found on routine CT [16]. Therefore, the coexistence of adrenocortical incidentaloma may be most considerable diagnosis at present.…”

Preclinical Cushing's Disease Characterized by Massive Adrenal Hyperplasia and Hormonal Changes after Three Years of Metyrapone Therapy

“…Chronic excess ACTH can cause adrenal hyperplasia [7,9,[12][13][14], but an adrenal hyperplasia as large as that of our patient in preclinical Cushing's disease has never been reported before. It is interesting that preclinical Cushing's disease was found incidentally with adrenal hyperplasia.…”

Concomitant coexistence of ACTH‐dependent and independent Cushing syndrome

“…However, the coexistence of cortisol‐secreting adrenal adenomas with a CD has been described in only a few case reports. 3 We report a case of a 46‐year‐old woman who presented with both CD and CS.…”

“…Although this is usually the case, macronodular hyperplasia is present in 10–15% of patients with Cushing disease (CD). 3 Nodules are generally bilateral, but when macronodular hyperplasia involves a single gland, it could be confused with an adrenal adenoma. However, the coexistence of cortisol‐secreting adrenal adenomas with a CD has been described in only a few case reports.…”

“…In ACTH‐dependent CS, the adrenal glands would be expected to develop diffuse hyperplasia and to appear uniformly enlarged in imaging studies. Although this is usually the case, macronodular hyperplasia is present in 10–15% of patients with Cushing disease (CD) 3 …”