Adrenal Diseases: An Update

A special issue of Biomedicines (ISSN 2227-9059). This special issue belongs to the section "Molecular and Translational Medicine".

Deadline for manuscript submissions: closed (30 April 2024) | Viewed by 4295

Special Issue Editor


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Guest Editor
Neuroendocrinology Unit, Endocrinology and Nutrition Department, Hospital Universitario Ramón y Cajal, 28034 Madrid, Spain
Interests: acromegaly; primary aldosteronism; endocrine; adrenocortical carcinoma; adrenal tumours; pheochromocytoma; Cushing´s syndrome; pituitary tumours

Special Issue Information

Dear Colleagues,

Adrenal disease is among the most common reasons for a patient to seek a consultation in endocrinology. When an adrenal tumor is identified, the key clinical questions are whether it is functioning and whether it is malignant. As such, it is vital for scholars to be familiar with the imaging characteristics of adrenal tumors in order to enable appropriate diagnosis and management. In addition, hormonal study is another important point that should be evaluated in all patients with adrenal tumors as more than 40% of the cases may be associated with overt or subtle hormonal hypersecretion. The most frequent adrenal hormonal syndromes include hypercortisolism, primary hyperaldosteronism, and phaeochromocytoma. The most clinically significant pathologies of the adrenal glands are adenomas and adrenal hyperplasia, adrenocortical carcinomas, phaeochromocytomas, and metastases. Other less common diseases that affect the adrenal glands include primary adrenal insufficiency and congenital adrenal hyperplasia, among others. Several advances have occurred in the last decades, including in the development, molecular characterization, diagnosis and treatment of the different adrenal diseases.

This Research Topic will emphasize the molecular mechanisms and therapeutic approaches associated with the diagnosis and treatment of adrenal diseases (pheochromocytoma, primary aldosteronism, adrenocortical carcinoma, adrenal tumors with associated autonomous cortisol secretion, non-functioning adrenal tumors, etc.). This Special Issue welcomes original research and review articles.

Dr. Marta Araujo-Castro
Guest Editor

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Keywords

  • adrenal tumors
  • pheochromocytoma
  • primary aldosteronism
  • adrenocortical carcinoma
  • cushing syndrome
  • autonomous cortisol secretion
  • adrenal incidentalomas.

Published Papers (3 papers)

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Research

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18 pages, 1620 KiB  
Article
Dexamethasone Suppression Testing in a Contemporary Cohort with Adrenal Incidentalomas in Two U.S. Integrated Healthcare Systems
by Mackenzie Crawford, Bennett McDonald, Wansu Chen, Hina Chowdhry, Richard Contreras, Iris Anne C. Reyes, Eleena Dhakal, Tish Villanueva, Joshua I. Barzilay, Candace F. Vaughn, Frank S. Czerwiec, David A. Katz, Annette L. Adams and Jennifer C. Gander
Biomedicines 2023, 11(12), 3167; https://doi.org/10.3390/biomedicines11123167 - 28 Nov 2023
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Abstract
Autonomous cortisol secretion (ACS) from an adrenal adenoma can increase the risk for comorbidities and mortality. The dexamethasone suppression test (DST) is the standard method to diagnose ACS. A multi-site, retrospective cohort of adults with diagnosed adrenal tumors was used to understand patient [...] Read more.
Autonomous cortisol secretion (ACS) from an adrenal adenoma can increase the risk for comorbidities and mortality. The dexamethasone suppression test (DST) is the standard method to diagnose ACS. A multi-site, retrospective cohort of adults with diagnosed adrenal tumors was used to understand patient characteristics associated with DST completion and ACS. Time to DST completion was defined using the lab value and result date; follow-up time was from the adrenal adenoma diagnosis to the time of completion or censoring. ACS was defined by a DST > 1.8 µg/dL (50 nmol/L). The Cox proportional hazards regression model assessed associations between DST completion and patient characteristics. In patients completing a DST, a logistic regression model evaluated relationships between elevated ACS and covariates. We included 24,259 adults, with a mean age of 63.1 years, 48.1% obese, and 28.7% with a Charlson comorbidity index ≥ 4. Approximately 7% (n = 1768) completed a DST with a completion rate of 2.36 (95% CI 2.35, 2.37) per 100 person-years. Fully adjusted models reported that male sex and an increased Charlson comorbidity index were associated with a lower likelihood of DST completion. Current or former smoking status and an increased Charlson comorbidity index had higher odds of a DST > 1.8 μg/dL. In conclusion, clinical policies are needed to improve DST completion and the management of adrenal adenomas. Full article
(This article belongs to the Special Issue Adrenal Diseases: An Update)
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10 pages, 865 KiB  
Communication
Diagnostic Accuracy of Adrenal Iodine-131 6-Beta-Iodomethyl-19-Norcholesterol Scintigraphy for the Subtyping of Primary Aldosteronism
by Marta Araujo-Castro, Miguel Paja Fano, Marga González Boillos, Eider Pascual-Corrales, Ana María García Cano, Paola Parra Ramírez, Patricia Martín Rojas-Marcos, Almudena Vicente Delgado, Anna Casteràs, Albert Puig, Iñigo García Sanz, Patricia Díaz Guardiola, Cristina Robles Lázaro, Miguel Antonio Sampedro Núñez, Raquel Guerrero-Vázquez, María del Castillo Tous, Theodora Michalopoulou Alevras, Susana Tenes Rodrigo and Felicia A. Hanzu
Biomedicines 2023, 11(7), 1934; https://doi.org/10.3390/biomedicines11071934 - 07 Jul 2023
Cited by 1 | Viewed by 1116
Abstract
Purpose: To evaluate the diagnostic accuracy of the 131I-6β-iodomethyl-19-norcholesterol (NP-59) adrenal scintigraphy for the subtyping diagnosis of primary aldosteronism (PA), considering as gold standard for the diagnosis of unilateral PA (UPA), either the results of the adrenal venous sampling (AVS) or the outcome [...] Read more.
Purpose: To evaluate the diagnostic accuracy of the 131I-6β-iodomethyl-19-norcholesterol (NP-59) adrenal scintigraphy for the subtyping diagnosis of primary aldosteronism (PA), considering as gold standard for the diagnosis of unilateral PA (UPA), either the results of the adrenal venous sampling (AVS) or the outcome after adrenalectomy. Methods: A retrospective multicenter study was performed on PA patients from 14 Spanish tertiary hospitals who underwent NP-59 scintigraphy with an available subtyping diagnosis. Patients were classified as UPA if biochemical cure was achieved after adrenalectomy or/and if an AVS lateralization index > 4 with ACTH stimulation or >2 without ACTH stimulation was observed. Patients were classified as having bilateral PA (BPA) if the AVS lateralization index was ≤4 with ACTH or ≤2 without ACTH stimulation or if there was evidence of bilateral adrenal nodules >1 cm in each adrenal gland detected by CT/MRI. Results: A total of 86 patients with PA were included (70.9% (n = 61) with UPA and 29.1% (n = 25) with BPA). Based on the NP-59 scintigraphy results, 16 patients showed normal suppressed adrenal gland uptake, and in the other 70 cases, PA was considered unilateral in 49 patients (70%) and bilateral in 21 (30%). Based on 59-scintigraphy results, 10.4% of the patients with unilateral uptake had BPA, and 27.3% of the cases with bilateral uptake had UPA. The AUC of the ROC curve of the NP-59 scintigraphy for PA subtyping was 0.812 [0.707–0.916]. Based on the results of the CT/MRI and NP-59 scintigraphy, only 6.7% of the patients with unilateral uptake had BPA, and 24% of the cases with bilateral uptake had UPA. The AUC of the ROC curve of the model combining CT/MRI and 59-scintigraphy results for subtyping PA was 0.869 [0.782–0.957]. Conclusion: The results of NP-59 scintigraphy in association with the information provided by the CT/MRI may be useful for PA subtyping. However, their diagnostic accuracy is only moderate. Therefore, it should be considered a second-line diagnostic tool when AVS is not an option. Full article
(This article belongs to the Special Issue Adrenal Diseases: An Update)
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Review

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29 pages, 1096 KiB  
Review
Landscape of Adrenal Tumours in Patients with Congenital Adrenal Hyperplasia
by Mara Carsote, Ana-Maria Gheorghe, Claudiu Nistor, Alexandra-Ioana Trandafir, Oana-Claudia Sima, Anca-Pati Cucu, Adrian Ciuche, Eugenia Petrova and Adina Ghemigian
Biomedicines 2023, 11(11), 3081; https://doi.org/10.3390/biomedicines11113081 - 16 Nov 2023
Cited by 1 | Viewed by 1154
Abstract
Our aim is to update the topic of adrenal tumours (ATs) in congenital adrenal hyperplasia (CAH) based on a multidisciplinary, clinical perspective via an endocrine approach. This narrative review is based on a PubMed search of full-length, English articles between January 2014 and [...] Read more.
Our aim is to update the topic of adrenal tumours (ATs) in congenital adrenal hyperplasia (CAH) based on a multidisciplinary, clinical perspective via an endocrine approach. This narrative review is based on a PubMed search of full-length, English articles between January 2014 and July 2023. We included 52 original papers: 9 studies, 8 case series, and 35 single case reports. Firstly, we introduce a case-based analysis of 59 CAH-ATs cases with four types of enzymatic defects (CYP21A2, CYP17A1, CYP17B1, and HSD3B2). Secondarily, we analysed prevalence studies; their sample size varied from 53 to 26,000 individuals. AT prevalence among CAH was of 13.3–20%. CAH prevalence among individuals with previous imaging diagnosis of AT was of 0.3–3.6%. Overall, this 10-year, sample-based analysis represents one of the most complex studies in the area of CAH-ATs so far. These masses should be taken into consideration. They may reach impressive sizes of up to 30–40 cm, with compressive effects. Adrenalectomy was chosen based on an individual multidisciplinary decision. Many tumours are detected in subjects with a poor disease control, or they represent the first step toward CAH identification. We noted a left lateralization with a less clear pathogenic explanation. The most frequent tumour remains myelolipoma. The risk of adrenocortical carcinoma should not be overlooked. Noting the increasing prevalence of adrenal incidentalomas, CAH testing might be indicated to identify non-classical forms of CAH. Full article
(This article belongs to the Special Issue Adrenal Diseases: An Update)
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