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The Lancet. Diabetes & Endocrinology Nov 2021Adrenal myelolipomas are benign, lipomatous tumours with elements of myeloid cells, most of which present as adrenal incidentalomas and comprise 3·3-6·5% of all... (Review)
Review
Adrenal myelolipomas are benign, lipomatous tumours with elements of myeloid cells, most of which present as adrenal incidentalomas and comprise 3·3-6·5% of all adrenal masses. Adrenal myelolipomas are usually unilateral (in 95% of cases), variable in size, most often found during midlife, and affect both sexes almost equally. On imaging, adrenal myelolipomas show pathognomonic imaging features consistent with the presence of macroscopic fat. Large adrenal myelolipomas can cause symptoms of mass effect, and can occasionally be complicated by haemorrhage. In the event of a concomitant adrenal cortical adenoma or hyperplasia, adrenal hormone excess might be detected in patients with adrenal myelolipoma. Patients with congenital adrenal hyperplasia exhibit a higher prevalence of adrenal myelolipomas than other patient groups, and are at risk of developing large and bilateral lesions. This Review discusses the pathogenesis, clinical presentation, and management of adrenal myelolipomas.
Topics: Adrenal Gland Neoplasms; Adrenal Hyperplasia, Congenital; Adrenocorticotropic Hormone; Aged; Female; Humans; Male; Middle Aged; Mutation; Myelolipoma; Prognosis; Proto-Oncogene Proteins; Tomography, X-Ray Computed
PubMed: 34450092
DOI: 10.1016/S2213-8587(21)00178-9 -
Endocrine Pathology Mar 2022The new WHO classification of adrenal cortical proliferations reflects translational advances in the fields of endocrine pathology, oncology and molecular biology. By... (Review)
Review
The new WHO classification of adrenal cortical proliferations reflects translational advances in the fields of endocrine pathology, oncology and molecular biology. By adopting a question-answer framework, this review highlights advances in knowledge of histological features, ancillary studies, and associated genetic findings that increase the understanding of the adrenal cortex pathologies that are now reflected in the 2022 WHO classification. The pathological correlates of adrenal cortical proliferations include diffuse adrenal cortical hyperplasia, adrenal cortical nodular disease, adrenal cortical adenomas and adrenal cortical carcinomas. Understanding germline susceptibility and the clonal-neoplastic nature of individual adrenal cortical nodules in primary bilateral macronodular adrenal cortical disease, and recognition of the clonal-neoplastic nature of incidentally discovered non-functional subcentimeter benign adrenal cortical nodules has led to redefining the spectrum of adrenal cortical nodular disease. As a consequence, the most significant nomenclature change in the field of adrenal cortical pathology involves the refined classification of adrenal cortical nodular disease which now includes (a) sporadic nodular adrenocortical disease, (b) bilateral micronodular adrenal cortical disease, and (c) bilateral macronodular adrenal cortical disease (formerly known primary bilateral macronodular adrenal cortical hyperplasia). This group of clinicopathological entities are reflected in functional adrenal cortical pathologies. Aldosterone producing cortical lesions can be unifocal or multifocal, and may be bilateral with no imaging-detected nodule(s). Furthermore, not all grossly or radiologically identified adrenal cortical lesions may be the source of aldosterone excess. For this reason, the new WHO classification endorses the nomenclature of the HISTALDO classification which uses CYP11B2 immunohistochemistry to identify functional sites of aldosterone production to help predict the risk of bilateral disease in primary aldosteronism. Adrenal cortical carcinomas are subtyped based on their morphological features to include conventional, oncocytic, myxoid, and sarcomatoid subtypes. Although the classic histopathologic criteria for diagnosing adrenal cortical carcinomas have not changed, the 2022 WHO classification underscores the diagnostic and prognostic impact of angioinvasion (vascular invasion) in these tumors. Microscopic angioinvasion is defined as tumor cells invading through a vessel wall and forming a thrombus/fibrin-tumor complex or intravascular tumor cells admixed with platelet thrombus/fibrin. In addition to well-established Weiss and modified Weiss scoring systems, the new WHO classification also expands on the use of other multiparameter diagnostic algorithms (reticulin algorithm, Lin-Weiss-Bisceglia system, and Helsinki scoring system) to assist the workup of adrenal cortical neoplasms in adults. Accordingly, conventional carcinomas can be assessed using all multiparameter diagnostic schemes, whereas oncocytic neoplasms can be assessed using the Lin-Weiss-Bisceglia system, reticulin algorithm and Helsinki scoring system. Pediatric adrenal cortical neoplasms are assessed using the Wieneke system. Most adult adrenal cortical carcinomas show > 5 mitoses per 10 mm and > 5% Ki67. The 2022 WHO classification places an emphasis on an accurate assessment of tumor proliferation rate using both the mitotic count (mitoses per 10 mm) and Ki67 labeling index which play an essential role in the dynamic risk stratification of affected patients. Low grade carcinomas have mitotic rate of ≤ 20 mitoses per 10 mm, whereas high-grade carcinomas show > 20 mitoses per 10 mm. Ki67-based tumor grading has not been endorsed in the new WHO classification, since the proliferation indices are continuous variables rather than being static thresholds in tumor biology. This new WHO classification emphasizes the role of diagnostic and predictive biomarkers in the workup of adrenal cortical neoplasms. Confirmation of the adrenal cortical origin of a tumor remains a critical requirement when dealing with non-functional lesions in the adrenal gland which may be mistaken for a primary adrenal cortical neoplasm. While SF1 is the most reliable biomarker in the confirmation of adrenal cortical origin, paranuclear IGF2 expression is a useful biomarker in the distinction of malignancy in adrenal cortical neoplasms. In addition to adrenal myelolipoma, the new classification of adrenal cortical tumors has introduced new sections including adrenal ectopia, based on the potential role of such ectopic tissue as a possible source of neoplastic proliferations as well as a potential mimicker of metastatic disease. Adrenal cysts are also discussed in the new classification as they may simulate primary cystic adrenal neoplasms or even adrenal cortical carcinomas in the setting of an adrenal pseudocyst.
Topics: Adrenal Cortex Neoplasms; Adrenal Gland Neoplasms; Adrenocortical Adenoma; Adrenocortical Carcinoma; Adult; Child; Humans; World Health Organization
PubMed: 35288842
DOI: 10.1007/s12022-022-09710-8 -
European Journal of Endocrinology Apr 2023While most benign lesions of the adrenal glands represent either an adrenocortical adenoma or a myelolipoma, the advent and frequent use of high-resolution radiological... (Review)
Review
While most benign lesions of the adrenal glands represent either an adrenocortical adenoma or a myelolipoma, the advent and frequent use of high-resolution radiological investigations have led to relatively increased incidental discovery of rare adrenal lesions, specifically benign adrenal cysts, adrenal ganglioneuromas, adrenal schwannomas, adrenal hemorrhage, and adrenal calcifications. Radiological characteristics of the different rare benign adrenal lesions could vary from distinct to indeterminate. Though typically nonfunctional, these rare lesions require evaluation for adrenal hormone excess, as they may phenotypically appear similar to pheochromocytoma or adrenocortical carcinoma and could sometimes be associated with or conceal an underlying functional adrenal tumor. In this review, we discuss the various rare benign adrenal lesions, emphasizing a practical perspective.
Topics: Humans; Tomography, X-Ray Computed; Adrenal Gland Neoplasms; Adrenocortical Adenoma; Adrenal Glands; Adrenal Cortex Neoplasms
PubMed: 36943310
DOI: 10.1093/ejendo/lvad036 -
Endocrinology and Metabolism Clinics of... Sep 2017Cross-sectional imaging can make a specific diagnosis in lesions, such as myelolipomas, cysts, and hemorrhage, and is often sufficient to distinguish benign from... (Review)
Review
Cross-sectional imaging can make a specific diagnosis in lesions, such as myelolipomas, cysts, and hemorrhage, and is often sufficient to distinguish benign from malignant adrenal processes. CT and MRI are useful studies to identify pheochromocytomas and cortisol-secreting or androgen-secreting tumors. In patients with primary aldosteronism, adrenal venous sampling remains the most accurate localizing study and should be performed in all patients older than 35. Radiolabeled isotope studies serve as second-line diagnostic tests for malignant adrenal tumors, primary or metastatic, as well as for pheochromocytoma. Nuclear imaging studies should follow a robust hormonal diagnosis and be correlated with findings on cross-sectional imaging.
Topics: Adrenal Gland Neoplasms; Adrenal Glands; Humans; Hyperaldosteronism; Magnetic Resonance Imaging; Myelolipoma; Pheochromocytoma
PubMed: 28760236
DOI: 10.1016/j.ecl.2017.04.009 -
The British Journal of Radiology Feb 2023Incidental adrenal masses are among the most common incidental lesions detected on cross-sectional imaging. The majority are benign lesions, adenomas and myelolipomas... (Review)
Review
Incidental adrenal masses are among the most common incidental lesions detected on cross-sectional imaging. The majority are benign lesions, adenomas and myelolipomas being the most common. Simple cross-sectional imaging techniques using CT and MRI permit the characterization of over 80%, thereby requiring no further imaging. The remaining lesions are considered indeterminate. These lesions consist of benign and malignant lesions sharing imaging features. Further imaging and management of these indeterminate lesions should be guided by close collaboration between different specialists in an MDT setting. Advanced imaging options include dedicated adrenal scintigraphy, positron emission tomography CT, biopsy and surveillance. Biochemical and hormonal evaluation is also important to identify hyperfunctioning adrenal lesions. This review focuses on imaging features of benign and malignant adrenal masses used for characterization and suggests an imaging pathway for indeterminate adrenal masses.
Topics: Humans; Adrenal Gland Neoplasms; Tomography, X-Ray Computed; Adenoma; Magnetic Resonance Imaging; Biopsy
PubMed: 35543634
DOI: 10.1259/bjr.20220281 -
Histopathology Jan 2024Mediastinal tumours represent a heterogeneous group of entities derived from the manifold structures located in or adjacent to the mediastinum. Due to the occurrence of... (Review)
Review
Mediastinal tumours represent a heterogeneous group of entities derived from the manifold structures located in or adjacent to the mediastinum. Due to the occurrence of some of these tumours in characteristic mediastinal compartments, an anatomical subdivision of the mediastinum in the prevascular (anterior), visceral (middle), and paravertebral (posterior) is helpful for the differential diagnosis. Benign anterior mediastinal tumours linked to an enlargement of the thymic gland mainly consist of thymic cysts and several types of thymic hyperplasia: true thymic hyperplasia, rebound hyperplasia, lymphofollicular hyperplasia, and so-called thymic hyperplasia with lymphoepithelial sialadenitis (LESA)-like features. Mature teratomas, ectopic (para)thyroid tissue, and benign thymic tumours such as thymolipoma or thymofibrolipoma represent further typical tumours of the anterior mediastinum. Pericardial, bronchogenic, or oesophageal duplication cysts predominate in the middle mediastinum, whereas neurogenic tumours and myelolipomas are characteristic findings in the posterior compartment. Vascular tumours, lipomas, adenomatoid tumours, Castleman disease, or mediastinitis are further examples of less frequent tumours or tumorous lesions affecting the mediastinum. This review focuses on benign mediastinal lesions with an emphasis on benign tumours of the thymus. Besides histology, characteristic epidemiological and clinical aspects prerequisite for the correct diagnosis and patient management are discussed.
Topics: Humans; Mediastinum; Mediastinal Neoplasms; Thymus Hyperplasia; Hyperplasia; Thymus Neoplasms
PubMed: 37988262
DOI: 10.1111/his.15088 -
Current Opinion in Endocrinology,... Jun 2019Circulating microRNAs represent promising minimally invasive markers of several diseases including tumors. As the preoperative diagnosis of different adrenal tumors is... (Review)
Review
PURPOSE OF REVIEW
Circulating microRNAs represent promising minimally invasive markers of several diseases including tumors. As the preoperative diagnosis of different adrenal tumors is difficult, for example, diagnosis of adrenocortical or adrenomedullary malignancy, circulating microRNAs might be helpful in their clinical management.
RECENT FINDINGS
Observations regarding the applicability of circulating microRNAs isolated both from unfractionated plasma or serum and from extracellular vesicle preparations for the diagnosis of adrenocortical malignancy have been published. Data show that circulating microRNA might be exploited for monitoring adrenocortical cancer progression. Circulating microRNA profiles of adrenal myelolipoma have also been published that might be useful for differentiating adrenocortical cancer and adrenal myelolipoma in dubious cases.
SUMMARY
In this review, recent advances in the field of circulating microRNAs in adrenal tumors are discussed.
Topics: Adrenal Cortex Neoplasms; Adrenal Gland Neoplasms; Adrenocortical Carcinoma; Biomarkers, Tumor; Circulating MicroRNA; Disease Progression; Humans; MicroRNAs
PubMed: 30801265
DOI: 10.1097/MED.0000000000000472 -
Mayo Clinic Proceedings Dec 2021
Topics: Adrenal Gland Neoplasms; Adrenal Glands; Adrenalectomy; Aged; Humans; Incidental Findings; Male; Myelolipoma
PubMed: 34863406
DOI: 10.1016/j.mayocp.2021.10.007