Le mutazioni somatiche nelle surrenali di pazienti con ipoaldosteronismo primitivo non guarito dalla surrenectomia suggeriscono un meccanismo condiviso fra la malattia unilaterale e bilaterale

n/

A multidisciplinary approach to the management of adrenal incidentaloma

Introduction: An adrenal incidentaloma (AI) is an adrenal neoplasm incidentally-discovered during an imaging unrelated to suspected adrenal disease. The aim of the present review is to offer practical guidance on the multidisciplinary approach of AIs. Areas covered: The prevalence of AI is high in the aging population (up to 5-8%); however, hormonally-active or malignant conditions are rare. After the discovery of an AI, it is suggested to assess in parallel if the mass is potentially malignant and functionally active. The answer for the former question is mainly based on medical history (extra-adrenal malignancies, new-onset of signs or symptoms) and imaging (conventional radiology and/or nuclear medicine). The answer for the latter question is a complete endocrine evaluation for both cortical (glucocorticoids, mineralocorticoids) and medullary (catecholamines) secretion. Expert opinion: A multidisciplinary discussion is suggested for patients with adrenal disease, after the exclusion of non-functioning benign cortical adenoma, in order to plan a close and tailored follow-up for the suspected malignant or functioning forms. Surgery is advised for patients with malignant disease (adrenocortical cancer) or with clinically relevant secreting neoplasm (primary aldosteronism, Cushing\u2019s Syndrome and pheochromocytoma)

Role of Estrogen and Estrogen Receptor in GH-Secreting Adenomas

Acromegaly is a rare disease with several systemic complications that may lead to increased overall morbidity and mortality. Despite several available treatments, ranging from transsphenoidal resection of GH-producing adenomas to different medical therapies, complete hormonal control is not achieved in some cases. Some decades ago, estrogens were first used to treat acromegaly, resulting in a significant decrease in IGF1 levels. However, due to the consequent side effects of the high dose utilized, this treatment was later abandoned. The evidence that estrogens are able to blunt GH activity also derives from the evidence that women with GH deficiency taking oral estro-progestins pills need higher doses of GH replacement therapy. In recent years, the role of estrogens and Selective Estrogens Receptor Modulators (SERMs) in acromegaly treatment has been re-evaluated, especially considering poor control of the disease under first- and second-line medical treatment. In this review, we analyze the state of the art concerning the impact of estrogen and SERMs on the GH/IGF1 axis, focusing on molecular pathways and the possible implications for acromegaly treatment

Vitamin D: An Overview of Gene Regulation, Ranging from Metabolism to Genomic Effects

Vitamin D is a pro-hormone characterized by an intricate metabolism and regulation. It is well known for its role in calcium and phosphate metabolism, and in bone health. However, several studies have assessed a huge number of extra-skeletal functions, ranging from cell proliferation in some oncogenic pathways to antioxidant and immunomodulatory functions. Vitamin D exerts its role by binding to VDRs (vitamin D receptors), which are located in many different tissues. Moreover, VDRs are able to bind hundreds of genomic loci, modulating the expression of various primary target genes. Interestingly, plenty of gene polymorphisms regarding VDRs are described, each one carrying a potential influence against gene expression, with relapses in several chronic diseases and metabolic complications. In this review, we provide an overview of the genetic aspects of vitamin D and VDR, emphasizing the gene regulation of vitamin D, and the genetic modulation of VDR target genes. In addition, we briefly summarize the rare genetic disease linked to vitamin D metabolism

Pasireotide-Induced Shrinkage in GH and ACTH Secreting Pituitary Adenoma: A Systematic Review and Meta-Analysis

Introduction: Pasireotide (PAS) is a novel somatostatin receptor ligands (SRL), used in controlling hormonal hypersecretion in both acromegaly and Cushing’s Disease (CD). In previous studies and meta-analysis, first-generation SRLs were reported to be able to induce significant tumor shrinkage only in somatotroph adenomas. This systematic review and meta-analysis aim to summarize the effect of PAS on the shrinkage of the pituitary adenomas in patients with acromegaly or CD. Materials and methods: We searched the Medline database for original studies in patients with acromegaly or CD receiving PAS as monotherapy, that assessed the proportion of significant tumor shrinkage in their series. After data extraction and analysis, a random-effect model was used to estimate pooled effects. Quality assessment was performed with a modified Joanna Briggs’s Institute tool and the risk of publication bias was addressed through Egger’s regression and the three-parameter selection model. Results: The electronic search identified 179 and 122 articles respectively for acromegaly and CD. After study selection, six studies considering patients with acromegaly and three with CD fulfilled the eligibility criteria. Overall, 37.7% (95%CI: [18.7%; 61.5%]) of acromegalic patients and 41.2% (95%CI: [22.9%; 62.3%]) of CD patients achieved significant tumor shrinkage. We identified high heterogeneity, especially in acromegaly (I2 of 90% for acromegaly and 47% for CD), according to the low number of studies included. Discussion: PAS treatment is effective in reducing tumor size, especially in acromegalic patients. This result strengthens the role of PAS treatment in pituitary adenomas, particularly in those with an invasive behavior, with progressive growth and/or extrasellar extension, with a low likelihood of surgical gross-total removal, or with large postoperative residual tissue. Systematic Review Registration: https://www.crd.york.ac.uk/prospero/display_record.php?ID=CRD42022328152, identifier CRD4202232815

[18F]FDG PET/CT and PET/MR in Patients with Adrenal Lymphoma: A Systematic Review of Literature and a Collection of Cases

Aim. The present study aimed to assess the existing data about Primary Adrenal Lymphoma (PAL) evaluated with FDG PET and to describe a small monocentric series of cases. A systematic analysis (from 2010 to 2022) was made by using PubMed and Web of Science databases reporting data about the role of FDG PET/CT in patients with suspicious or known adrenal lymphoma. The quality of the papers was assessed by using QUADAS-2 criteria. Moreover, from a single institutional collection between 2010 and 2021, data from patients affected by adrenal lymphoma and undergoing contrast-enhanced compute tomography (ceCT)/magnetic resonance (MR) and FDG PET/CT or PET/MR were retrieved and singularly described. Seventy-eight papers were available from PubMed and 25 from Web of Science. Forty-seven (Nr. 47) Patients were studied, most of them in the initial staging of disease (n = 42; 90%). Only in one paper, the scan was made before and after therapy. The selected clinical cases were relative to the initial staging of disease, the restaging, and the evaluation of response to therapy. PET/CT and PET/MR always showed a high FDG uptake in the primary adrenal lesions and in metastatic sites. Moreover, PET metrics, such as maximum standardized uptake value (SUVmax) and metabolic tumor volume (MTV), were elevated in all primary adrenal lesions. In conclusions, FDG PET either coupled with CT or MRI can be useful in staging, restaging, and for the evaluation of treatment response in patients affected by PA