Primary bilateral macronodular adrenal hyperplasia: A series of 32 cases and literature review.

Primary bilateral macronodular adrenal hyperplasia (PBMAH) accounts for <2% of cases of Cushing's syndrome. The majority of patients present with no obvious steroid excess it means with autonomous cortisol secretion (ACS). The classic treatment for patients with overt Cushing's syndrome is bilateral adrenalectomy, but unilateral resection of the larger adrenal gland can result in clinical and/or biochemical remission in >90% of cases, especially in cases of ACS. In this article, a series of 32 cases with PBMAH is described. Most of the cases of PBMAH had ACS, except for one case with overt Cushing's syndrome. A study of aberrant receptors was performed in six patients, being negative in three cases, positive in the metoclopramide test in two cases and positive in the metoclopramide test and in the mixed meal test in another patient. The patient with overt Cushing's syndrome was treated with adrenostatic therapy achieving biochemical control, while two patients with ACS underwent unilateral adrenalectomy with resection of the largest adrenal gland, demonstrating hypercortisolism remission and improvement of cardiovascular risk factors after surgery. This article describes a series of 32 cases of PBMAH and offers a comprehensive review of PBMAH.

Prevalencia y fenotipo de la hiperplasia suprarrenal macronodular bilateral primaria con secreción autónoma de cortisol: un estudio de 98 pacientes / Prevalence and phenotype of primary bilateral macronodular adrenal hyperplasia with autonomous cortisol secretion: a study of 98 patients

Objetivo Se evaluó la prevalencia de hiperplasia suprarrenal macronodular bilateral primaria (PBMAH). También se analizó el fenotipo diferencial de los pacientes con PBMAH en comparación con otras lesiones suprarrenales bilaterales que no cumplían con la definición de PBMAH. Métodos Revisamos las historias clínicas de 732 pacientes diagnosticados de incidentaloma suprarrenal en nuestro centro. Se incluyeron 98 pacientes con hipercortisolismo subclínico para el análisis. Se definió PBMAH como la presencia de cortisol plasmático > 1,8 μg/dL después de una prueba de 1 mg de dexametasona durante la noche, hiperplasia suprarrenal bilateral y nódulos suprarrenales bilaterales > 1 cm. Resultados Un total de 31 pacientes tenían PBMAH. Los pacientes con PBMAH mostraron una mayor prevalencia de secreción autónoma de cortisol (cortisol plasmático > 5,0 μg/dL después de la prueba de 1 mg de dexametasona durante la noche) que los pacientes sin PBMAH (OR 4,1, IC del 95%: 1,38-12,09, p = 0,010). El tamaño del tumor y la masa adenomatosa total fueron significativamente mayores en pacientes con PBMAH en comparación con los pacientes sin PBMAH (30,2 ± 12,16 vs. 24,3 ± 8,47 mm, p = 0,010 y 53,9 ± 20,8 vs. 43,3 ± 14,62 mm, p = 0,023), respectivamente. Una mayor proporción de pacientes con PBMAH tenían diabetes en comparación con los pacientes sin PBMAH (45,2% vs. 25,4%, p = 0,05). Conclusión PBMAH está presente en un tercio de los pacientes con incidentaloma suprarrenal e hipercortisolismo subclínico. Los pacientes con PBMAH mostraron una mayor secreción autónoma de cortisol, mayor tamaño del tumor y diabetes que aquellos sin PBMAH (AU)

Aim This study evaluated prevalence of primary bilateral macronodular adrenal hyperplasia (PBMAH). It also analyzed the differential phenotype of patients with PBMAH compared to other bilateral adrenal lesions that do not meet the definition of PBMAH. Methods We reviewed the medical records of 732 patients diagnosed with an adrenal incidentaloma at our center. Ninety-eight patients with subclinical hypercortisolism were included in the analysis. We defined PBMAH as the presence of plasma cortisol > 1.8 μg/dL after an over-night 1-mg dexamethasone test, bilateral adrenal hyperplasia, and bilateral adrenal nodules > 1 cm. Results A total of 31 patients had PBMAH. Patients with PBMAH showed greater prevalence of autonomous cortisol secretion (plasma cortisol > 5.0 μg/dL after an overnight 1-mg dexamethasone test) than patients without PBMAH (OR 4.1, 95%CI 1.38-12.09, p = 0.010). Tumor size and total adenomatous mass were significantly greater in patients with PBMAH compared to patients without PBMAH (30.2 ± 12.16 vs. 24.3 ± 8.47 mm, p = 0.010 and 53.9 ± 20.8 vs. 43.3 ± 14.62 mm, p = 0.023), respectively. A greater proportion of patients with PBMAH had diabetes compared to patients without PBMAH (45.2% vs. 25.4%, p = 0.05). Conclusion PBMAH is present in one-third of patients with adrenal incidentaloma and subclinical hypercortisolism. Patients with PBMAH showed greater autonomous cortisol secretion, bigger tumor size, and higher rates of diabetes than those without PBMAH (AU)

Prevalence and phenotype of primary bilateral macronodular adrenal hyperplasia with autonomous cortisol secretion: a study of 98 patients.

AIM: This study evaluated prevalence of primary bilateral macronodular adrenal hyperplasia (PBMAH). It also analyzed the differential phenotype of patients with PBMAH compared to other bilateral adrenal lesions that do not meet the definition of PBMAH. METHODS: We reviewed the medical records of 732 patients diagnosed with an adrenal incidentaloma at our center. Ninety-eight patients with subclinical hypercortisolism were included in the analysis. We defined PBMAH as the presence of plasma cortisol > 1.8 µg/dL after an over-night 1-mg dexamethasone test, bilateral adrenal hyperplasia, and bilateral adrenal nodules > 1 cm. RESULTS: A total of 31 patients had PBMAH. Patients with PBMAH showed greater prevalence of autonomous cortisol secretion (plasma cortisol > 5.0 µg/dL after an overnight 1-mg dexamethasone test) than patients without PBMAH (OR 4.1, 95%CI 1.38-12.09, p = 0.010). Tumor size and total adenomatous mass were significantly greater in patients with PBMAH compared to patients without PBMAH (30.2 ±â€¯12.16 vs. 24.3 ±â€¯8.47 mm, p = 0.010 and 53.9 ±â€¯20.8 vs. 43.3 ±â€¯14.62 mm, p = 0.023), respectively. A greater proportion of patients with PBMAH had diabetes compared to patients without PBMAH (45.2% vs. 25.4%, p = 0.05). CONCLUSION: PBMAH is present in one-third of patients with adrenal incidentaloma and subclinical hypercortisolism. Patients with PBMAH showed greater autonomous cortisol secretion, bigger tumor size, and higher rates of diabetes than those without PBMAH.

Riesgo cardiometabólico en pacientes con hiperaldosteronismo primario y secreción autónoma de cortisol. Estudio de casos y controles / Cardiometabolic risk in patients with primary aldosteronism and autonomous cortisol secretion. Case-control study

Objetivo: Analizar las diferencias en el perfil cardiometabólico de los pacientes con hiperaldosteronismo primario (HAP) y secreción autónoma de cortisol (SAC), emparejados por edad y sexo.MétodosEstudio de casos y controles; casos de HAP sin SAC asociada y como controles, pacientes con SAC (test de supresión de dexametasona ≥ 1.8 μg/dL en ausencia de datos específicos de hipercortisolismo), emparejados por edad y sexo. Se analizaron las comorbilidades HTA, diabetes, obesidad, dislipemia, insuficiencia renal crónica y eventos cardiovasculares y cerebrovasculares, así como su grado de control.ResultadosSe incluyeron 57 pacientes con HAP y 57 con SAC. Al diagnóstico, aparte de una mayor prevalencia de HTA en los pacientes con HAP (100 vs. 52,7%, p < 0,0001) y niveles más altos de TAS (143,2 [2,5] vs. 135,3 [2,6] mmHg, p = 0,032) que en SAC, no se detectaron diferencias en la prevalencia de otras comorbilidades. No obstante, los pacientes con SAC presentaban cifras más elevadas de HbA1c (p = 0,028).Tras una mediana de seguimiento de 2,25 años, los pacientes con HAP presentaron un mayor deterioro de la función renal (descenso medio del filtrado glomerular [MDRD-4] -17,4 [3] vs. -2,3 [4,4] mL/min/1,73 m2, p = 0,005) y del perfil lipídico (Δtriglicéridos de 34,5 [15,8] vs. -6,7 [11,3] mg/dL, p = 0,038) que los SAC.ConclusionesA pesar la mayor prevalencia de HTA en los pacientes con HAP que con SAC, emparejados por edad y sexo, no se detectaron diferencias en la prevalencia de otras comorbilidades cardiometabólicas. No obstante, los HAP presentaron un mayor deterioro de la función renal y del perfil lipídico a lo largo del seguimiento que el grupo de SAC. (AU)

Objective: To analyse the differences in the cardio-metabolic profile of patients with primary aldosteronism (PA) and autonomous cortisol secretion (ACS) matched by age and sex.MethodsCase-control study; cases of PA without associated ACS and as controls patients with ACS (dexamethasone suppression test ≥ 1.8 μg/dL in the absence of specific hypercortisolism clinical data), matched by age and sex. Comorbidities of hypertension, diabetes, obesity, dyslipidaemia, chronic kidney failure, and cardiovascular and cerebrovascular events were analysed, as well as their degree of control.Results57 patients with PA and 57 with ACS were included. On diagnosis, in addition to a higher prevalence of hypertension in the PA patients (100 vs. 52.7%, p < .0001) and higher systolic blood pressure levels (143.2 (2.5) vs. 135.3 (2.6) mmHg, p = .032) than in the ACS patients, no other differences were detected in the prevalence of other cardio-metabolic comorbidities. Nevertheless, the patients with ACS had higher HbA1c levels (p = .028) than the PA patients.After a median follow-up of 2.25 years, the patients with PA presented a greater deterioration in kidney function (Average decrease in glomerular filtration rate (MDRD-4) -17.4 (3.0) vs. -2.3 (4.4) mL/min/1.73 m2, p = .005) and lipid profile (Δtriglycerides of 34.5 (15.8) vs. -6.7 (11.3) mg/dL, p = .038) than the ACS patients.ConclusionsDespite the higher prevalence of hypertension in the patients with PA than in the patients with ACS matched by age and sex, no differences were detected in the prevalence of other cardio-metabolic comorbidities. However, the PA patients showed a greater deterioration in kidney function and lipid profile throughout the follow-up than the ACS patients. (AU)

[Cardiometabolic risk in patients with primary aldosteronism and autonomous cortisol secretion. Case-control study]. / Riesgo cardiometabólico en pacientes con hiperaldosteronismo primario y secreción autónoma de cortisol. Estudio de casos y controles.

OBJECTIVE: To analyse the differences in the cardio-metabolic profile of patients with primary aldosteronism (PA) and autonomous cortisol secretion (ACS) matched by age and sex. METHODS: Case-control study; cases of PA without associated ACS and as controls patients with ACS (dexamethasone suppression test ≥ 1.8 µg/dL in the absence of specific hypercortisolism clinical data), matched by age and sex. Comorbidities of hypertension, diabetes, obesity, dyslipidaemia, chronic kidney failure, and cardiovascular and cerebrovascular events were analysed, as well as their degree of control. RESULTS: 57 patients with PA and 57 with ACS were included. On diagnosis, in addition to a higher prevalence of hypertension in the PA patients (100 vs. 52.7%, p < .0001) and higher systolic blood pressure levels (143.2 (2.5) vs. 135.3 (2.6) mmHg, p = .032) than in the ACS patients, no other differences were detected in the prevalence of other cardio-metabolic comorbidities. Nevertheless, the patients with ACS had higher HbA1c levels (p = .028) than the PA patients. After a median follow-up of 2.25 years, the patients with PA presented a greater deterioration in kidney function (Average decrease in glomerular filtration rate (MDRD-4) -17.4 (3.0) vs. -2.3 (4.4) mL/min/1.73 m2, p = .005) and lipid profile (Δtriglycerides of 34.5 (15.8) vs. -6.7 (11.3) mg/dL, p = .038) than the ACS patients. CONCLUSIONS: Despite the higher prevalence of hypertension in the patients with PA than in the patients with ACS matched by age and sex, no differences were detected in the prevalence of other cardio-metabolic comorbidities. However, the PA patients showed a greater deterioration in kidney function and lipid profile throughout the follow-up than the ACS patients.

Practical guide on the initial evaluation, follow-up, and treatment of adrenal incidentalomas Adrenal Diseases Group of the Spanish Society of Endocrinology and Nutrition. / Guía práctica sobre la evaluación inicial, seguimiento y tratamiento de los incidentalomas adrenales. Grupo de patología adrenal de la Sociedad Española de Endocrinología y Nutrición.

Initial evaluation of adrenal incidentalomas should be aimed at ruling out malignancy and functionality. For this, a detailed clinical history should be taken, and an adequate radiographic assessment and a complete blood chemistry and hormone study should be performed. The most controversial condition, because of the lack of consensus in its definition, is autonomous cortisol secretion. Our recommendation is that, except when cortisol levels <1.8µg/dL in the dexamethasone suppression test rule out diagnosis and levels ≥5µg/dL establish the presence of autonomous cortisol secretion, diagnosis should be based on a combined definition of dexamethasone suppression test ≥3µg/dL and at least one of the following: elevated urinary free cortisol, ACTH level <10 pg/mL, or elevated nocturnal cortisol (in serum and/or saliva). During follow-up, dexamethasone suppression test should be repeated, usually every year, on an individual basis depending on the results of prior tests and the presence of comorbidities potentially related to hypercortisolism. The initial radiographic test of choice for characterization of adrenal incidentalomas is a computed tomography scan without contrast, but there is no unanimous agreement on subsequent monitoring. Our general recommendation is a repeat imaging test 6-12 months after diagnosis (based on the radiographic characteristics of the lesion). If the lesion remains stable and there are no indeterminate characteristics, no additional radiographic studies would be needed. We think that patients with autonomous cortisol secretion with comorbidities potentially related to hypercortisolism, particularly if they are young and there is a poor control, may benefit from unilateral adrenalectomy. The indication for unilateral adrenalectomy is clear in patients with overt hormonal syndromes or suspected malignancy. In conclusion, adrenal incidentalomas require a comprehensive evaluation that takes into account the possible clinical signs and comorbidities related to hormonal syndromes or malignancy; a complete hormone profile (taking into account the conditions that may lead to falsely positive and negative results); and an adequate radiographic study. Monitoring and/or treatment will be decided based on the results of the initial evaluation.