Abstract
IntroductionPrimary pigmented nodular adrenocortical disease is a rare cause of adrenocorticotropic hormone-independent Cushing's syndrome. We report an uncommon primary pigmented nodular adrenocortical disease case presenting with a unilateral adrenocortical nodule and provide a brief overview of the existing literature.Case presentationA 27-year-old Caucasian woman was admitted to our Department with adrenocorticotropic hormone-independent Cushing's syndrome. Its cause was initially considered a left adrenocortical adenoma based on computer tomography imaging. The patient underwent left laparoscopic adrenalectomy and histological examination revealed pigmented micronodular adrenal hyperplasia. Evaluation for the presence of Carney complex was negative. Six months later recurrence of hypercortisolism was documented and a right laparoscopic adrenalectomy was performed further establishing the diagnosis of primary pigmented nodular adrenocortical disease. After a nine-year follow-up there is no evidence of residual disease.ConclusionsEven though primary pigmented nodular adrenocortical disease is a rare cause of Cushing's syndrome, it should be included in the differential diagnosis of adrenocorticotropic hormone-independent Cushing's syndrome, especially because adrenal imaging can be misleading mimicking other adrenocortical diseases. Bilateral laparoscopic adrenalectomy is the preferred treatment in these subjects.
Highlights
Primary pigmented nodular adrenocortical disease is a rare cause of adrenocorticotropic hormoneindependent Cushing’s syndrome
Even though primary pigmented nodular adrenocortical disease is a rare cause of Cushing’s syndrome, it should be included in the differential diagnosis of adrenocorticotropic hormone-independent Cushing’s syndrome, especially because adrenal imaging can be misleading mimicking other adrenocortical diseases
Bilateral laparoscopic adrenalectomy is the preferred treatment in these subjects
Summary
Primary pigmented nodular adrenocortical disease is a rare cause of adrenocorticotropic hormoneindependent Cushing’s syndrome. We report an uncommon primary pigmented nodular adrenocortical disease case presenting with a unilateral adrenocortical nodule and provide a brief overview of the existing literature. Case presentation: A 27-year-old Caucasian woman was admitted to our Department with adrenocorticotropic hormone-independent Cushing’s syndrome. Six months later recurrence of hypercortisolism was documented and a right laparoscopic adrenalectomy was performed further establishing the diagnosis of primary pigmented nodular adrenocortical disease. Primary pigmented nodular adrenocortical disease (PPNAD) and adrenocorticotropic hormone (ACTH)independent macronodular adrenal hyperplasia (AIMAH) account for approximately 10% of ACTHindependent Cushing’s syndrome (CS) [1,2,3]. Half of PPNAD patients appear to be sporadic cases and the other half are familial, mostly associated with Carney complex (CNC) [4,7,8]. The treatment of choice in CS due to PPNAD is bilateral adrenalectomy
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