AI Article Synopsis
- Primary pigmented nodular adrenocortical disease (PPNAD) is a rare form of Cushing's syndrome that can be challenging to diagnose preoperatively due to normal CT scans or misleading unilateral adrenal lesions.
- This study focused on 17 patients with ACTH-independent PPNAD, examining the effectiveness of adrenal CT scans and NP-59 scintigraphy in identifying the disease and correlating these findings with clinical, pathological, and genetic data.
- Results showed that while CT scans often revealed small nodules, NP-59 scintigraphy consistently indicated bilateral adrenal uptake, with asymmetrical uptake linked to the presence of larger nodules in some cases.
Article Abstract
Context: Primary pigmented nodular adrenocortical disease (PPNAD) is a rare cause of ACTH-independent Cushing's syndrome that may occur in an isolated form or as part of Carney complex. The diagnosis of this disease can be difficult preoperatively because computed tomography (CT) scan can be normal or suggest unilateral adrenal lesion, which can impede the correct diagnosis of bilateral adrenal disease.
Objective: The aim of our study was to describe the results of preoperative imaging (adrenal [6β-(131)I]iodomethyl-19-norcholesterol] [NP-59] scintigraphy and standard adrenal CT scan) and their correlations with clinical, pathological, and genetics investigations in patients with PPNAD.
Patients And Methods: Seventeen patients with ACTH-independent syndrome due to PPNAD were investigated with a standard adrenal CT scan and NP-59 scintigraphy. Hormonal, pathological, and genetics data were analyzed.
Results: Four males and 13 females (median age, 27 y) were included. PPNAD was isolated in 11 patients (with PRKAR1A mutation, n = 7; and without PRKAR1A mutation, n = 4) and was associated with extra-adrenal manifestations of Carney complex in six patients (with PRKAR1A mutation, n = 4; and without PRKAR1A mutation, n = 2). Standard adrenal CT scan revealed micronodules in 11 patients, macronodules in three patients, and was normal in three patients. All patients demonstrated bilateral adrenal radiocholesterol uptake. Adrenal uptake was asymmetrical in 10 of 17 patients (59%). Asymmetrical uptake correlated with the presence of macronodules at pathological analysis (P = .03).
Conclusion: Standard adrenal CT scan most often reveals micronodules but there is no specific CT imaging. NP-59 scintigraphy always shows a bilateral adrenal uptake confirming the bilateral nature of the disease, but asymmetrical scintigraphic uptake can be observed in patients with macronodules.
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| http://dx.doi.org/10.1210/jc.2015-2174 | DOI Listing |
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