Surgical Outcomes of Aldosterone-Producing Adenoma on the Basis of the Histopathological Findings

Wang, Huiping; Wang, Fen; Zhang, Yushi; Wen, Ji; Dong, Dexin; Chang, Xiaoyan; Sun, Hao; Ma, Xiaosen; Cui, Yunying; Shi, Chen; Lin, Li; Ren, Weidong; Tong, Anli; Li, Yuxiu

doi:10.3389/fendo.2021.663096

Cited by 15 publications

(21 citation statements)

References 23 publications

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“…It is also not uncommon to have microscopic clonal nodular proliferations that cannot be identified on imaging studies [16,107]. For this reason, the new WHO classification endorses the use CYP11B2 immunohistochemistry in the workup of all adrenalectomy specimens from patients with primary aldosteronism to identify functional sites of aldosterone secretion for the appropriate distinction of bilateral from unilateral aldosterone secreting lesions and predicting possible biochemical recurrence [114]. It is also important to recognize that APAs and APNs have distinct cytomorphological features that are reflected in their genotypephenotype correlations [1,4,5].…”

Section: Question 6: What Are the Pathological Correlates Of Adrenal ...mentioning

confidence: 99%

Overview of the 2022 WHO Classification of Adrenal Cortical Tumors

et al. 2022

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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 scheme...

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Section: Question 6: What Are the Pathological Correlates Of Adrenal ...mentioning

confidence: 99%

Overview of the 2022 WHO Classification of Adrenal Cortical Tumors

et al. 2022

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“…Recently, non-classical histopathological lesions associated with aldosterone excess were found in 25% of the cases in a German cohort of unilateral PA (111). On the other side, APMs were found in only 5% (7 out of 137) of the cases in a Chinese cohort of unilateral PA (113). Therefore, additional studies from patients with different genetic backgrounds are essential to define the prevalence of classical and non-classical unilateral PA among different cohorts.…”

Section: Impact On Clinical Outcomementioning

confidence: 99%

Pathogenesis of Primary Aldosteronism: Impact on Clinical Outcome

Santana

Guimarães²,

Almeida³

2022

Front. Endocrinol.

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Primary aldosteronism (PA) is the most common form of secondary arterial hypertension, with a prevalence of approximately 20% in patients with resistant hypertension. In the last decade, somatic pathogenic variants in KCNJ5, CACNA1D, ATP1A1 and ATP2B3 genes, which are involved in maintaining intracellular ionic homeostasis and cell membrane potential, were described in aldosterone-producing adenomas (aldosteronomas). All variants in these genes lead to the activation of calcium signaling, the major trigger for aldosterone production. Genetic causes of familial hyperaldosteronism have been expanded through the report of germline pathogenic variants in KCNJ5, CACNA1H and CLCN2 genes. Moreover, PDE2A and PDE3B variants were associated with bilateral PA and increased the spectrum of genetic etiologies of PA. Of great importance, the genetic investigation of adrenal lesions guided by the CYP11B2 staining strongly changed the landscape of somatic genetic findings of PA. Furthermore, CYP11B2 staining allowed the better characterization of the aldosterone-producing adrenal lesions in unilateral PA. Aldosterone production may occur from multiple sources, such as solitary aldosteronoma or aldosterone-producing nodule (classical histopathology) or clusters of autonomous aldosterone-producing cells without apparent neoplasia denominated aldosterone-producing micronodules (non-classical histopathology). Interestingly, KCNJ5 mutational status and classical histopathology of unilateral PA (aldosteronoma) have emerged as relevant predictors of clinical and biochemical outcome, respectively. In this review, we summarize the most recent advances in the pathogenesis of PA and discuss their impact on clinical outcome.

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“…Adrenal tumours display a highly heterogeneous profile with respect to molecular and pathological/immunohistochemistry profile, as well as genetic background, and this field has recently seen spectacular progress [ 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 127 , 128 , 129 , 130 , 131 , 132 , 133 , 134 ].…”

Section: Approach Of Connshing Syndromementioning

confidence: 99%

“…Traditionally, PA is associated with an adrenal tumour, bilateral adrenal hyperplasia, adrenal cortex carcinoma and ectopic aldosterone production, but benign adrenal sources are described as aldosteronona (solitary tumour), aldosterone-producing nodule or multiple micronodules (non-classical type), respective bilateral adrenal disease; for instance, unilateral PA may underline a unilateral single or multiple aldosterone-producing adenoma or an aldosterone-producing cell cluster [ 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 127 , 128 , 129 , 130 , 131 , 135 , 136 ].…”

Section: Approach Of Connshing Syndromementioning

confidence: 99%

The Entity of Connshing Syndrome: Primary Aldosteronism with Autonomous Cortisol Secretion

Cârșote¹

2022

Diagnostics

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Connshing syndrome (CoSh) (adrenal-related synchronous aldosterone (A) and cortisol (C) excess) represents a distinct entity among PA (primary hyperaldosteronisms) named by W. Arlt et al. in 2017, but the condition has been studied for more than 4 decades. Within the last few years, this is one of the most dynamic topics in hormonally active adrenal lesions due to massive advances in steroids metabolomics, molecular genetics from CYP11B1/B2 immunostaining to genes constellations, as well as newly designated pathological categories according to the 2022 WHO classification. In gross, PA causes 4–10% of all high blood pressure (HBP) cases, and 20% of resistant HBP; subclinical Cushing syndrome (SCS) is identified in one-third of adrenal incidentalomas (AI), while CoSh accounts for 20–30% to 77% of PA subjects, depending on the tests used to confirm autonomous C secretion (ACS). The clinical picture overlaps with PA, hypercortisolemia being mild. ACS is suspected in PA if a more severe glucose and cardiovascular profile is identified, or there are larger tumours, ACS being an independent factor risk for kidney damage, and probably also for depression/anxiety and osteoporotic fractures. It seems that one-third of the PA-ACS group harbours mutations of C-related lines like PRKACA and GNAS. A novel approach means we should perform CYP11B2/CYP11B1 immunostaining; sometimes negative aldosteronoma for CYP11B1 is surrounded by micronodules or cell clusters with positive CYP11B1 to sustain the C excess. Pitfalls of hormonal assessments in CoSh include the index of suspicion (check for ACS in PA patients) and the interpretation of A/C ratio during adrenal venous sample. Laparoscopic adrenalectomy is the treatment of choice. Post-operative clinical remission rate is lower in CoSh than PA. The risk of clinically manifested adrenal insufficiency is low, but a synthetic ACTH stimulating testing might help to avoid unnecessary exposure to glucocorticoids therapy. Finally, postponing the choice of surgery may impair the outcome, having noted that long-term therapy with mineralocorticoids receptors antagonists might not act against excessive amounts of C. Awareness of CoSh improves management and overall prognosis.

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Surgical Outcomes of Aldosterone-Producing Adenoma on the Basis of the Histopathological Findings

Cited by 15 publications

References 23 publications

Overview of the 2022 WHO Classification of Adrenal Cortical Tumors

Overview of the 2022 WHO Classification of Adrenal Cortical Tumors

Pathogenesis of Primary Aldosteronism: Impact on Clinical Outcome

The Entity of Connshing Syndrome: Primary Aldosteronism with Autonomous Cortisol Secretion

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