Prevalence of Adrenal Abnormalities in Patients with Cushing's Disease: Correlation with Clinical Characteristics at Diagnosis and Impact on Postoperative Outcome

Presentation Number: SAT 404
Date of Presentation: April 1st, 2017

Ramon Marcelino do Nascimento*1, Pedro Junqueira de Godoy Pereira2, Bruna Sabrina de Sanabria Irigoitia3, Aline Guimarães de Faria2, Valter Angelo Sperling Cescato4, Nina de Castro Musolino5, Maria Candida Barisson Villares Fragoso6, Marcello D Bronstein7, Publio Cesar Cavalcanti Viana2 and Marcio Carlos Machado8
1Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil, 2Hospital das Clínicas da Faculdade de Medicina da Universidade de Sao Paulo, Sao Paulo, Brazil, 3Faculdade de Medicina da Universidade de São Paulo, Sao Paulo, Brazil, 4Hosp das Clin Faculd Med USP, Sao Paulo, Brazil, 5Hospital das Clínicas, FMUSP, São Paulo, Brazil, 6Hospital das Clinicas, University of Sao Paulo, School of Medicine, Sao Paulo, BRAZIL, 7Disciplina de Endocrinologia e Metabologia, Unidade de Neuroendocrinologia do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, Brazil., 8University of Sao Paulo Medical School, Sao Paulo, Brazil


Background: During the etiological investigation of ACTH-dependent Cushing's syndrome is not uncommon to observe hyperplasia of the adrenal glands. In addition, adrenal nodules also are eventually found making important the differential diagnosis of ACTH-producing source.

Objective: To study the prevalence of adrenal changes in patients with CD and to correlate the findings with clinical characteristics at diagnosis and impact on postoperative outcome.

Methods: From 2007 to 2016, were evaluated 163 patients with ACTH-dependent Cushing's syndrome. Of these, we selected 89 cases with confirmed diagnosis of CD, who had CT (93%) or MRI of the abdomen/adrenal with imaging available for reassessment by a single radiologist with experience in adrenal diseases. Cases were excluded when imaging was not available for the re-evaluation or in cases with exams done out of our Service.

Results: There was a prevalence of 30% (27/89) of adrenal abnormal findings: 16 cases (18%) had hyperplasia of the adrenal glands (Hyperplasia Group: HPG) and 11 cases (12%) had unilateral or bilateral nodules (Nodules Group: NDG). In NDG, seven cases were unilateral with an average diameter of 16.6 ± 5.1 mm and 12.7 ± 13.5 Hounsfield unit (HU) and four cases were bilateral with a diameter of 17.6 mm ± 4.9 and 1.3 ± 6.9 HU. All nodules had sharp edges and 85% had a homogeneous texture. In relation to the hormonal profile, higher cortisol levels were found in HPG patients. Late-night salivary cortisol (LNSC): HPG: 2270.3 ± 4818.6 ng/dL (Reference [R]: <120 ng/dL); normal group (NG): 644.6 ± 660.4 ng/dL; NDG: 574.5 ± 453.0 ng/dL. 24h urinary cortisol (UC): HPG: 2994.7 ± 8041.3 µg/24h (R: 50-310 µg/24h); NG: 876.4 ± 864.4 µg/24h; NDG: 808.6 ± 856.9 µg/24h. Overall, remission rate after transsphenoidal surgery was 68% (59/87), similar in all subgroups of adrenal imaging. Recurrence was detected in 24% of cases in a median follow-up of 36 months: 12 cases in NG, two cases in HPG and one case in NG. Adrenal imaging (CT) was reassessed in six patients of NG (55%, 3 uni- and 3 bilateral): unilateral: one nodule reduced (21 to 9 mm), one described as hyperplasia and one case did not show nodule; bilateral (six nodes in three cases): four reduced (18.0 ± 4.5 to 14.3 ± 5.9 mm) and two (one patient without remission) increased in size (15.0 to 27.5 mm). Hyperaldosteronism and pheochromocytoma were excluded in the researched cases.

Conclusion: There was a higher prevalence of adrenal abnormalities in patients with CD, greater than observed in the normal population. Concentrations of LNSC and UC denote greater severity of Cushing's syndrome in the HPG. There was no impact on remission and recurrence rates compared to adrenal findings. The partial re-evaluation of these nodules during follow-up suggests that chronic stimulation of ACTH is responsible for the appearance of these changes.