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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 -
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 -
Diagnostics (Basel, Switzerland) Sep 2022Adrenal lesions are frequently incidentally diagnosed during investigations for other clinical conditions. Despite being usually benign, nonfunctioning, and silent, they... (Review)
Review
Adrenal lesions are frequently incidentally diagnosed during investigations for other clinical conditions. Despite being usually benign, nonfunctioning, and silent, they can occasionally cause discomfort or be responsible for various clinical conditions due to hormonal dysregulation; therefore, their characterization is of paramount importance for establishing the best therapeutic strategy. Imaging techniques such as ultrasound, computed tomography, magnetic resonance, and PET-TC, providing anatomical and functional information, play a central role in the diagnostic workup, allowing clinicians and surgeons to choose the optimal lesion management. This review aims at providing an overview of the most encountered adrenal lesions, both benign and malignant, including describing their imaging characteristics.
PubMed: 36140572
DOI: 10.3390/diagnostics12092171 -
Medicina 2023Myelolipoma is a benign non-functional tumor. Most of them are asymptomatic and discovered incidentally, either through imaging studies or at autopsy. While it most...
Myelolipoma is a benign non-functional tumor. Most of them are asymptomatic and discovered incidentally, either through imaging studies or at autopsy. While it most commonly occurs in the adrenal gland, it has also been reported at extra-adrenal sites. We present the case of a 65-year-old woman with a primary mediastinal myelolipoma. Computer tomographic scan of the thorax showed an ovoid tumor with well-defined borders of 6.5 × 4.2 cm, located in the posterior mediastinum. A transthoracic biopsy of the lesion was made, and the microscopic observation revealed hematopoietic cells and mature adipose tissue. Although computed tomography and magnetic resonance imaging are effective in diagnosing mediastinal myelolipoma, histopathological examination is essential for the definitive diagnosis.
Topics: Female; Humans; Aged; Mediastinum; Myelolipoma; Thoracic Surgery, Video-Assisted; Radionuclide Imaging; Adrenal Gland Neoplasms
PubMed: 37379542
DOI: No ID Found -
Endocrine Practice : Official Journal... Jun 2018
Topics: Adrenal Gland Neoplasms; Adrenal Hyperplasia, Congenital; Adult; Humans; Hypogonadism; Male; Myelolipoma
PubMed: 29498907
DOI: 10.4158/EP-2017-0241 -
Internal Medicine (Tokyo, Japan) Apr 2019
PubMed: 30568133
DOI: 10.2169/internalmedicine.1692-18 -
The Kaohsiung Journal of Medical... Jul 2012Adrenal myelolipoma is a rare, nonfunctioning, and benign tumor. We report our experience of surgically treated patients from a single institute and review the... (Review)
Review
Adrenal myelolipoma is a rare, nonfunctioning, and benign tumor. We report our experience of surgically treated patients from a single institute and review the literature. Six patients (three men and three women) were diagnosed and received surgical intervention. A retrospective analysis was done by reviewing medical records. In our series, three patients were diagnosed incidentally and the others were discovered due to symptoms. All received surgery, including laparoscopic adrenalectomy. There was no recurrence. In the literature review, right adrenal gland was dominant and the prevalent age was from the fourth to sixth decades. The most common symptoms were abdominal and flank pain. Adrenal myelolipoma is uncommon and easily confused with malignancy when of large size (≥ 6 cm). Surgery may be reserved for symptomatic cases and those lesions that cannot reliably be diagnosed. Large tumors (≥ 6 cm) can be excised surgically or laparoscopically.
Topics: Adrenal Gland Neoplasms; Adult; Female; Humans; Male; Middle Aged; Myelolipoma; Tomography, X-Ray Computed
PubMed: 22726900
DOI: 10.1016/j.kjms.2012.02.005