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NCBI: db=pubmed; Term=adrenal tumor
Updated: 2 hours 12 min ago

adrenal tumor; +26 new citations

2 hours 12 min ago

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adrenal tumor

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Comparison between the clinical characteristics of patients with adrenal incidentalomas and those with hypertension-associated adrenal tumors in a single center in Japan.

Sun, 03/29/2020 - 01:32
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Comparison between the clinical characteristics of patients with adrenal incidentalomas and those with hypertension-associated adrenal tumors in a single center in Japan.

Endocr J. 2020 Mar 25;:

Authors: Aoe M, Okada A, Usui T, Manaka K, Nangaku M, Makita N

Abstract
In the largest retrospective study of adrenal incidentalomas (AIs) in Japan between 1999 and 2004, adrenal tumors detected during secondary hypertension (HT) screening were included. The characteristics of patients with adrenal tumors detected during HT screening may differ from those of patients with AIs. This study aimed to compare the characteristics of patients with AIs with those of patients with adrenal tumors detected during HT screening. We retrospectively analyzed patients referred to our division for detailed examination of adrenal tumors between April 2009 and April 2017. When the purposes of imaging tests included HT screening, we defined adrenal tumors as HT associated, otherwise as strictly defined AIs. We reviewed data on age, sex, purpose and modality of imaging, location of tumor, tumor diameter, and hormonal evaluation. We identified 104 patients with HT-associated adrenal tumors and 413 with AIs. Patients with HT-associated adrenal tumors were younger (54.2 years vs. 61.7 years, p < 0.001) and had smaller tumor diameters (1.3 cm vs. 1.9 cm, p ‍< ‍0.001), lower prevalence of nonfunctioning tumors (24.0% vs. 67.6%, p < 0.001), and higher prevalence of primary aldosteronism (58.7% vs. 4.8%, p < 0.001) than those with AIs. There were no differences in terms of tumor location and prevalence of subclinical Cushing's syndrome, Cushing's syndrome, and pheochromocytoma (18.3% vs. 16.0%, 7.7% vs. 8.0%, and 2.9% vs. 4.6%, respectively). In conclusion, patients with HT-associated tumors were younger and had a smaller tumor with higher prevalence of primary aldosteronism than those with AIs.

PMID: 32213723 [PubMed - as supplied by publisher]

Is Follow-up of Adrenal Incidentalomas Always Mandatory?

Sun, 03/29/2020 - 01:32
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Is Follow-up of Adrenal Incidentalomas Always Mandatory?

Endocrinol Metab (Seoul). 2020 Mar;35(1):26-35

Authors: Reimondo G, Muller A, Ingargiola E, Puglisi S, Terzolo M

Abstract
Adrenal masses are mainly detected unexpectedly by an imaging study performed for reasons unrelated to any suspect of adrenal diseases. Such masses are commonly defined as "adrenal incidentalomas" and represent a public health challenge because they are increasingly recognized in current medical practice. Management of adrenal incidentalomas is currently matter of debate. Although there is consensus on the need of a multidisciplinary expert team evaluation and surgical approach in patients with significant hormonal excess and/or radiological findings suspicious of malignancy demonstrated at the diagnosis or during follow-up, the inconsistency between official guidelines and the consequent diffuse uncertainty on management of small adrenal incidentalomas still represents a considerable problem in terms of clinical choices in real practice. The aim of the present work is to review the proposed strategies on how to manage patients with adrenal incidentalomas that are not candidates to immediate surgery. The recent European Society of Endocrinology/European Network for the Study of Adrenal Tumors guidelines have supported the view to avoid surveillance in patients with clear benign adrenal lesions <4 cm and/or without any hormonal secretion; however, newer prospective studies are needed to confirm safety of this strategy, in particular in younger patients.

PMID: 32207261 [PubMed - in process]

Adrenal Tumors Found During Staging and Surveillance for Colorectal Cancer: Benign Incidentalomas or Metastatic Disease?

Sun, 03/29/2020 - 01:32
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Adrenal Tumors Found During Staging and Surveillance for Colorectal Cancer: Benign Incidentalomas or Metastatic Disease?

World J Surg. 2020 Mar 23;:

Authors: Yanagisawa M, Malik DG, Loehfelm TW, Fananapazir G, Corwin MT, Campbell MJ

Abstract
OBJECTIVE: To evaluate the incidence of adrenal metastases in patient with colorectal cancer (CRC) and determine the clinical and radiographic features associated metastatic CRC to the adrenal glands.
MATERIALS AND METHODS: The review of consecutive adults with newly diagnosed CRC found to have adrenal tumors > 1 cm in size on staging or surveillance CT scans with at least two scans to evaluate progression or stability of disease.
RESULTS: Fifty-eight of 856 (6.8%) CRC patients had an adrenal tumor. Forty-three patients (74%) with 46 adrenal tumors had benign adrenal tumors, and 15 (26%) patients with 17 adrenal tumors had metastatic disease. On univariate analysis, patients with metastatic CRC had larger adrenal tumors (26.7 mm vs 12.4 mm, p < 0.01), a higher mean CEA (239 ng/mL vs 14.2 ng/mL, p = 0.03), and were more likely to have other sites of metastatic disease seen on imaging 8/43 (19%) vs 14/15 (93%), p < 0.01. On multivariable analysis, adrenal tumor size > 1.8 cm (OR 49.6 CI 8-306), CEA > 2.5 ng/mL (OR 15.8 CI 1.7-144) and other metastatic disease seen on imaging (OR 68.1 CI 7-661) were independently associated with adrenal metastases.
CONCLUSION: CRC patients with small adrenal tumors, normal CEA levels and no evidence of other metastatic disease are unlikely to have spread to the adrenal glands. Adrenal tumors found during staging and surveillance of CRC patients should be evaluated with appropriate imaging and biochemical analysis.

PMID: 32206857 [PubMed - as supplied by publisher]

Primary aldosteronism diagnostics: KCNJ5 mutations and hybrid steroid synthesis in aldosterone-producing adenomas.

Sun, 03/29/2020 - 01:32
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Primary aldosteronism diagnostics: KCNJ5 mutations and hybrid steroid synthesis in aldosterone-producing adenomas.

Gland Surg. 2020 Feb;9(1):3-13

Authors: Rege J, Turcu AF, Rainey WE

Abstract
Primary aldosteronism (PA) is characterized by autonomous aldosterone production by renin-independent mechanisms and is most commonly sporadic. While 60-70% of sporadic PA can be attributed to bilateral hyperaldosteronism, the remaining 30-40% is caused by a unilateral aldosterone-producing adenoma (APA). Somatic mutations in or near the selectivity filter the KCNJ5 gene (encoding the potassium channel GIRK4) have been implicated in the pathogenesis of both sporadic and familial PA. Several studies using tumor tissue, peripheral and adrenal vein samples from PA patients have demonstrated that along with aldosterone, the hybrid steroids 18-hydroxycortisol (18OHF) and 18-oxocortisol (18oxoF) are a hallmark of APA harboring KCNJ5 mutations. Herein, we review the recent advances with respect to the molecular mechanisms underlying the pathogenesis of PA and the steroidogenic fingerprints of KCNJ5 mutations. In addition, we present an outlook toward the future of PA subtyping and diagnostic work-up utilizing steroid profiling.

PMID: 32206594 [PubMed]

[Virilizing ovarian tumor: the challenges of differential diagnosis].

Sun, 03/29/2020 - 01:32
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[Virilizing ovarian tumor: the challenges of differential diagnosis].

Probl Endokrinol (Mosk). 2019 Dec 25;65(4):273-277

Authors: Kalashnikova MF, Likhodey NV, Tiulpakov AN, Fedorova EV, Bryunin DV, Bakhvalova AA, Glushakova MA, Smirnova SA, Fadeyev VV

Abstract
SertoliLeydig cell tumor is a rather rare type of ovarian neoplasms belonging to the group of sex cordstromal tumors. This malignancy is characterized by androgen overproduction, which results in the so-called virilization and can be accompanied by various metabolic disorders such as abdominal obesity, disturbances of carbohydrate and protein metabolism, and high blood pressure. During differential diagnosis, it is important to identify the source of androgen overproduction. An androgen-secreting ovarian tumor needs to be differentiated from androgen-secreting adrenal tumor, ovarian stromal thecomatosis (hyperthecosis), and endogenous hypercorticism (the Cushings syndrome). In most cases, the SertoliLeydig cell tumor is associated with DICER1 mutation carriership. If a patient is found to carry the DICER1 mutation, patients relatives need to undergo genetic testing as the individuals with mutations in this gene have an elevated risk of developing a broad range of benign and malignant tumors (most of these tumors are relatively rare in the overall population). The awareness of this rare ovarian neoplasm among medical specialists (obstetriciansgynecologists, endocrinologists, and oncologists) is supposed to ensure timely diagnosis and adequate treatment of this disease.

PMID: 32202730 [PubMed - in process]

[The glucagon test in diagnosis of secondary adrenal insufficiency after craniospinal irradiation: the feasibility of application, the features of performing the test, and its diagnostic informativity].

Sun, 03/29/2020 - 01:32
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[The glucagon test in diagnosis of secondary adrenal insufficiency after craniospinal irradiation: the feasibility of application, the features of performing the test, and its diagnostic informativity].

Probl Endokrinol (Mosk). 2019 Dec 25;65(4):227-235

Authors: Yudina AE, Pavlova MG, Sotnikov VM, Tselovalnikova TY, Mazerkina NA, Zheludkova OG, Gerasimov AN, Teryaeva NB, Martynova E, Kim EI

Abstract
BACKGROUND: The glucagon test (GT) is a promising alternative to the insulin hypoglycemia test (IHT) in diagnosis of secondary adrenal insufficiency (SAI).
AIM: To study the feasibility of using the GT in patients after craniospinal irradiation and to determine the cut-off value to rule out SAI.
METHODS: A total of 28 patients (14 males and 14 females) with the median age of 19 years (17; 23) who had undergone combination treatment (surgery, craniospinal irradiation (35 Gy) with boost to the tumor bed, and polychemotherapy) of extrapituitary brain tumors no later than 2 years before study initiation and 10 healthy volunteers of matching sex and age were examined. All the subjects underwent the GT and IHT with an interval of at least 57 days. The cortisol, ACTH, and glucose levels were measured.
RESULTS: Twelve out of 28 patients were diagnosed with SAI according to the IHT results. ROC analysis revealed that cortisol release during the GT 499 nmol/L ruled out SAI [100% sensitivity (Se); 62% specificity (Sp)], while the absence of a rise 340 nmol/l verified SAI (Sp 100%; 55% Se). For GT, the area under a curve (AUC) was 93.6%, which corresponds to a very good diagnostic informativity. In 19 patients, the IHT and GT results were concordant (in ten patients, the release of cortisol occurred above the cut-off value in both tests; no release was detected in nine patients). In nine cases, the results were discordant: the maximum cortisol level detected in the GT was 500 nmol/l, but the IHT results ruled out SAI (the GT yielded a false positive outcome). Contrariwise, in three (10.7%) patients the release of cortisol detected in the GT was adequate, while being insufficient in the IHT test. Adverse events (nausea) were reported during the GT test in 9 (25%) subjects; one patient had hypoglycemia (1.8 mmol/l).
CONCLUSION: GT is highly informative and can be used as a first-level stimulation test for ruling out SAI in patients exposed to craniospinal irradiation performed to manage brain tumors. The cortisol level of 500 nmol/L is the best cut-off value for ruling out SAI according to the GT results. The insulin hypoglycemia test is used as the second-level supporting test in patients with positive GT results.

PMID: 32202724 [PubMed - in process]

Minimally Invasive Adrenalectomy: Technical Aspects of the Laparoscopic and the Robotic Approach.

Sun, 03/29/2020 - 01:32
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Minimally Invasive Adrenalectomy: Technical Aspects of the Laparoscopic and the Robotic Approach.

Chirurgia (Bucur). 2020 Jan-Feb;115(1):80-88

Authors: Makkai-Popa ST, Pascotto B, Arru L, Blasi V, Goergen M, Azagra JS

Abstract
Adrenalectomy is nowadays a procedure routinely performed by minimally invasive surgery. In this article we aim to describe in depth our technique for laparoscopic and robotic left and right adrenalectomies, by using four cases and discussing the advantages and disadvantages of each technique.

PMID: 32155402 [PubMed - indexed for MEDLINE]

Ginsenoside Rd attenuates ACTH-induced corticosterone secretion by blocking the MC2R-cAMP/PKA/CREB pathway in Y1 mouse adrenocortical cells.

Sun, 03/29/2020 - 01:32
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Ginsenoside Rd attenuates ACTH-induced corticosterone secretion by blocking the MC2R-cAMP/PKA/CREB pathway in Y1 mouse adrenocortical cells.

Life Sci. 2020 Mar 15;245:117337

Authors: Jin W, Ma R, Zhai L, Xu X, Lou T, Huang Q, Wang J, Zhao D, Li X, Sun L

Abstract
BACKGROUND: Higher levels of glucocorticoids (GCs), and impaired regulation of the hypothalamic-pituitary-adrenal (HPA) axis may cause or exacerbate the occurrence of metabolic and psychiatric disorders. It has been reported that ginseng saponin extract (GSE) has an inhibitory effect on the hyperactivity of the HPA axis induced by stresses and increased corticosterone level induced by intraperitoneal injection of adrenocorticotrophic hormone (ACTH) in mice. However, the molecular mechanisms by which GSE and its active ginsenosides inhibit corticosterone secretion remain elusive.
MAIN METHODS: Y1 mouse adrenocortical cells were treated with ACTH for up to 60 min to establish a cell model of corticosterone secretion. After treatment with different concentrations of GSE or ginsenoside monomers for 24 h prior to the addition of ACTH, analyses of cAMP content, PKA activity, and the levels of steroidogenesis regulators, melanocortin-2 receptor (MC2R), and melanocortin-2 receptor accessory protein (MRAP) in ACTH-induced Y1 cells were performed.
RESULTS: We demonstrated that GSE inhibits ACTH-stimulated corticosterone production in Y1 cells by inhibiting factors critical for steroid synthesis. Ginsenoside Rd, an active ingredient of GSE, inhibits corticosterone secretion in the cells and impedes ACTH-induced corticosterone biosynthesis through down-regulation of proteins in the cAMP/PKA/CREB signaling pathway. In addition, Western blot and qPCR analyses showed that ginsenoside Rd attenuated the induction of MC2R and MRAP by ACTH.
CONCLUSION: Our findings indicate that ginsenoside Rd inhibits ACTH-induced corticosterone production through blockading the MC2R-cAMP/PKA/CREB pathway in adrenocortical cells. Overall, this mechanism may represent an important therapeutic option for the treatment of stress-related disorders, further supporting the pharmacological benefits of ginseng.

PMID: 31972205 [PubMed - indexed for MEDLINE]

Craniopharyngiomas presenting as incidentalomas: results of KRANIOPHARYNGEOM 2007.

Sun, 03/29/2020 - 01:32
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Craniopharyngiomas presenting as incidentalomas: results of KRANIOPHARYNGEOM 2007.

Pituitary. 2019 Oct;22(5):532-541

Authors: Boekhoff S, Bison B, Eveslage M, Sowithayasakul P, Müller HL

Abstract
PURPOSE: Childhood-onset craniopharyngiomas (CP) are diagnosed due to clinical symptoms (symCP) or incidentally (incCP). We investigated clinical manifestations and outcome in incCPs and symCPs.
METHODS: IncCP were discovered in 4 (3 m/1 f) and symCP in 214 (101 m/113 f) CP recruited 2007-2014 in KRANIOPHARYNGEOM 2007. Age, sex, height, body mass index (BMI), tumor volume, degree of resection, pre- and postsurgical hypothalamic involvement/lesions, pituitary function and outcome were compared between both subgroups.
RESULTS: Reasons for imaging in incCP were cerebral palsy, head trauma, nasal obstruction, and tethered-cord syndrome, whereas headache (44%), visual impairment (25%), and growth retardation (17%) lead to imaging in symCP. Tumor volume at diagnosis was smaller in incCP (median 2.39 cm3; range 0.14-4.10 cm3) when compared with symCP (15.86 cm3; 0.002-286.34 cm3). Age, gender, BMI, height, hydrocephalus, tumor location, and hypothalamic involvement at diagnosis of incCP were within the range of these parameters in symCP. Complete resections were achieved more frequently (3/4 patients) in incCP when compared with symCP (20%). Surgical hypothalamic lesions were distributed similar in incCP and symCP. Irradiation was performed only in symCP (33%). No noticeable differences were observed concerning survival rates, endocrine deficiencies, BMI, height, functional capacity and quality of life of the 4 incCP cases when compared with the symCP cohort.
CONCLUSIONS: IncCP are rare (1.8%) and characterized by lack of endocrine deficiencies, resulting in normal height and BMI, no hydrocephalus, and smaller tumor volume at diagnosis when compared with symCPs. Outcome of the observed incCP is similar with symCP.
CLINICAL TRIAL REGISTRATION NUMBER: NCT01272622.

PMID: 31440945 [PubMed - indexed for MEDLINE]

Surgical approaches and results of treatment for hereditary paragangliomas.

Sun, 03/29/2020 - 01:32
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Surgical approaches and results of treatment for hereditary paragangliomas.

Best Pract Res Clin Endocrinol Metab. 2019 10;33(5):101298

Authors: Iacobone M, Belluzzi A, Torresan F

Abstract
Paragangliomas (PGL) are rare neuroendocrine tumours; parasympathetic PGL are predominantly non-secreting and located at the skull base and neck, while sympathetic PGL are typically catecholamine-secreting and located at abdomino-pelvic level. Approximately 40% of PGL may be caused by germline mutations; hereditary variants should be suspected especially in case of positive family history, early onset, multifocal, or recurrent PGL. Significant genotype-phenotype correlation has been recognized, including syndromic presentation, location, multifocality and risk of malignancy. Surgical resection remains the only curative strategy, but the outcomes may be unsatisfactory because of surgical morbidity and recurrence rate. However, due to the rarity of the disease, most data derive from case-report or limited series. This paper was aimed to review the available literature on the epidemiology, diagnosis, clinical features, treatment of PGL in order to discuss the surgical approach and the results of treatment in hereditary PGL.

PMID: 31401056 [PubMed - indexed for MEDLINE]

The relevance of cortisol co-secretion from aldosterone-producing adenomas.

Sun, 03/29/2020 - 01:32
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The relevance of cortisol co-secretion from aldosterone-producing adenomas.

Hormones (Athens). 2019 Sep;18(3):307-313

Authors: Bhatt PS, Sam AH, Meeran KM, Salem V

Abstract
AIMS AND OBJECTIVES: Adrenal adenomas are usually non-functioning, but can secrete aldosterone or cortisol. It has recently been suggested that many more adenomas than previously thought secrete more than one hormone. This has important implications for their clinical management. Our aim was to determine the frequency of cortisol co-secretion in primary hyperaldosteronism at our institution and investigate the difference in metabolic profiles and clinical outcomes between co-secreting and non-co-secreting patients.
DESIGN AND PATIENTS: A retrospective study of 25 patients with primary hyperaldosteronism who also underwent formal dexamethasone suppression tests to determine cortisol co-secretion.
MEASUREMENTS: Post-dexamethasone suppression test cortisol, serum ALT, total cholesterol, HDL-cholesterol, LDL-cholesterol, HbA1C (were recorded) and mean arterial pressure are reported in this cohort of patients with primary hyperaldosteronism.
RESULTS: Four out of 25 patients with primary hyperaldosteronism failed dexamethasone suppression tests. This suggests a frequency of co-secretion ranging between 4 and 16%. No significant difference was found in serum ALT, total cholesterol, serum HDL-cholesterol, LDL-cholesterol and mean arterial blood pressure at presentation between co-secretors and non-co-secretors.
CONCLUSION: A frequency range of 4-16% suggests that a significant proportion of patients with primary hyperaldosteronism co-secrete cortisol. Co-secretors did not have a worse metabolic profile than non-secretors. The impact of co-secretion on metabolic profile and surgical management remains unclear and warrants further study.

PMID: 31399957 [PubMed - indexed for MEDLINE]

Imprint cytology of adrenal cortical carcinoma. A valuable surrogate for frozen sections.

Sun, 03/29/2020 - 01:32
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Imprint cytology of adrenal cortical carcinoma. A valuable surrogate for frozen sections.

Diagn Cytopathol. 2019 Dec;47(12):1310-1313

Authors: Sakuma T, Kokubo C, Koike S, Shinohara S

PMID: 31381263 [PubMed - indexed for MEDLINE]

EglN3 hydroxylase stabilizes BIM-EL linking VHL type 2C mutations to pheochromocytoma pathogenesis and chemotherapy resistance.

Sun, 03/29/2020 - 01:32
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EglN3 hydroxylase stabilizes BIM-EL linking VHL type 2C mutations to pheochromocytoma pathogenesis and chemotherapy resistance.

Proc Natl Acad Sci U S A. 2019 08 20;116(34):16997-17006

Authors: Li S, Rodriguez J, Li W, Bullova P, Fell SM, Surova O, Westerlund I, Topcic D, Bergsland M, Stenman A, Muhr J, Nistér M, Holmberg J, Juhlin CC, Larsson C, von Kriegsheim A, Kaelin WG, Schlisio S

Abstract
Despite the discovery of the oxygen-sensitive regulation of HIFα by the von Hippel-Lindau (VHL) protein, the mechanisms underlying the complex genotype/phenotype correlations in VHL disease remain unknown. Some germline VHL mutations cause familial pheochromocytoma and encode proteins that preserve their ability to down-regulate HIFα. While type 1, 2A, and 2B VHL mutants are defective in regulating HIFα, type 2C mutants encode proteins that preserve their ability to down-regulate HIFα. Here, we identified an oxygen-sensitive function of VHL that is abolished by VHL type 2C mutations. We found that BIM-EL, a proapoptotic BH3-only protein, is hydroxylated by EglN3 and subsequently bound by VHL. VHL mutants fail to bind hydroxylated BIM-EL, regardless of whether they have the ability to bind hydroxylated HIFα or not. VHL binding inhibits BIM-EL phosphorylation by extracellular signal-related kinase (ERK) on serine 69. This causes BIM-EL to escape from proteasomal degradation, allowing it to enhance EglN3-induced apoptosis. BIM-EL was rapidly degraded in cells lacking wild-type VHL or in which EglN3 was inactivated genetically or by lack of oxygen, leading to enhanced cell survival and chemotherapy resistance. Combination therapy using ERK inhibitors, however, resensitizes VHL- and EglN3-deficient cells that are otherwise cisplatin-resistant.

PMID: 31375625 [PubMed - indexed for MEDLINE]

Pheochromocytoma and Pregnancy.

Sun, 03/29/2020 - 01:32
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Pheochromocytoma and Pregnancy.

Endocrinol Metab Clin North Am. 2019 09;48(3):605-617

Authors: Lenders JWM, Langton K, Langenhuijsen JF, Eisenhofer G

Abstract
Pheochromocytoma during pregnancy, although rare, is a perilous condition. The wellbeing of mother and fetus are at stake if not diagnosed and treated antenatally and timely. The diagnosis is frequently overlooked because of the aspecific nature of signs and symptoms and confusion with pregnancy-related hypertension. Measurements of plasma or urinary free metanephrines have the highest diagnostic accuracy. MRI is preferred over ultrasonography. The optimal time for surgical removal is before 24 weeks of gestation or at/after delivery. Laparoscopic adrenalectomy should be preceded by medical pretreatment. Cesarean delivery is preferred in these patients; vaginal delivery might be considered in selected pretreated patients.

PMID: 31345526 [PubMed - indexed for MEDLINE]

Volume-outcome relationship in adrenal surgery: A review of existing literature.

Sun, 03/29/2020 - 01:32
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Volume-outcome relationship in adrenal surgery: A review of existing literature.

Best Pract Res Clin Endocrinol Metab. 2019 10;33(5):101296

Authors: Kazaure HS, Sosa JA

Abstract
The relationship between operative volume and perioperative outcomes after several oncologic operations is well documented. Recent studies on adrenalectomy reveal a robust association between higher surgeon volume and improved patient outcomes. Statistical analyses have demonstrated that outcomes are improved when surgeons perform at least six adrenalectomies annually; based on this threshold definition of a 'high-volume' surgeon, more than 80% of adrenalectomies in the United States are performed by 'low-volume' surgeons. When compared to low-volume surgeons, high-volume surgeons on average achieve lower rates of postoperative complications and mortality, as well as a shorter length of hospital stay, and lower cost of hospitalization. There does not appear to be a similar association between hospital adrenalectomy volume and improved patient outcomes; however, there is evidence of benefit for the subset of patients with adrenocortical carcinoma. Despite limitations of existing literature, evidence is sufficient to recommend the referral of patients with adrenal tumors to high-volume surgeons.

PMID: 31331729 [PubMed - indexed for MEDLINE]

Abnormal glucose tolerance in a patient with pheochromocytoma and ACTH-independent subclinical Cushing's syndrome involving the same adrenal gland.

Sun, 03/29/2020 - 01:32
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Abnormal glucose tolerance in a patient with pheochromocytoma and ACTH-independent subclinical Cushing's syndrome involving the same adrenal gland.

J Int Med Res. 2019 Jul;47(7):3360-3370

Authors: Morita N, Hosaka T, Yamazaki Y, Takahashi K, Sasano H, Ishida H

PMID: 31256733 [PubMed - indexed for MEDLINE]

Nivolumab-induced hypophysitis causing secondary adrenal insufficiency after transient ACTH elevation.

Sun, 03/29/2020 - 01:32
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Nivolumab-induced hypophysitis causing secondary adrenal insufficiency after transient ACTH elevation.

Endocr J. 2019 Oct 28;66(10):937-941

Authors: Sekizaki T, Kameda H, Oba C, Yong Cho K, Nakamura A, Miyoshi H, Osawa T, Shinohara N, Atsumi T

Abstract
A 62-year-old man was referred to our department for elevation of plasma ACTH and cortisol levels during nivolumab administration for renal cell carcinoma. Although his ACTH and cortisol levels had been maintained within their reference ranges, they were elevated to 232.7 pg/mL and 21.9 μg/dL, respectively, after eight courses of nivolumab without any subjective symptoms or Cushing's sign. He was hospitalized for endocrinological investigation. ACTH and cortisol returned to their normal ranges (29.18 pg/mL and 11.4 μg/dL, respectively) in the early morning on day 1, but fell down sharply to 3.7 pg/mL and 1.6 μg/dL, respectively, in the early morning on day 2 without subjective symptoms or vital sign changes. Brain magnetic resonance imaging showed no abnormality in his pituitary gland. ACTH response to CRH was apparently normal, but cortisol did not respond to increased ACTH. A rapid ACTH stimulation test showed slightly reduced response of cortisol to exogenous ACTH (1-24). These findings and his clinical course suggested secondary adrenal insufficiency arising from nivolumab-induced hypophysitis. In previous reports, most cases of immune checkpoint inhibitor (ICI)-induced hypophysitis were diagnosed based on adrenal insufficiency symptoms or hyponatremia with low ACTH and cortisol. The ACTH elevation observed in the present case may reflect destruction of the pituitary gland, suggesting that this finding may be important for early detection of ICI-induced hypophysitis. Our case underlines the necessity of close monitoring for subsequent onset of adrenal insufficiency when ACTH elevation is observed during ICI administration.

PMID: 31217401 [PubMed - indexed for MEDLINE]

Intraaortic Counterpulsation Therapy Allowing the Diagnosis of a Pheochromocytoma.

Sun, 03/29/2020 - 01:32
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Intraaortic Counterpulsation Therapy Allowing the Diagnosis of a Pheochromocytoma.

Am J Respir Crit Care Med. 2019 10 15;200(8):e89-e90

Authors: Abrard S, Parfait E, Fouquet O, Rineau E

PMID: 31150270 [PubMed - indexed for MEDLINE]

Adrenal tumors: when to search for a germline abnormality?

Sun, 03/29/2020 - 01:32
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Adrenal tumors: when to search for a germline abnormality?

Curr Opin Oncol. 2019 05;31(3):230-235

Authors: Gimenez-Roqueplo AP

Abstract
PURPOSE OF REVIEW: Over the last 20 years, the genetic landscape of adrenal tumours has been transformed by the identification of multiple susceptibility genes for the various tumour types. We review these recent developments here, and describe current recommendations for genetic testing in patients with tumours of the adrenal medulla and extra-adrenal paraganglia or the adrenal cortex.
RECENT FINDINGS: Phaeochromocytomas (adrenal medulla tumours) and paragangliomas, aldosterone-producing adenomas, primary macronodular adrenal hyperplasia, primary pigmented nodular adrenocortical disease and adrenocortical carcinoma (adrenal cortex tumours) may all be caused by a germline mutation in a specific gene, regardless of the presence/absence of a family history or syndromic disease at initial diagnosis. Dedicated genetic testing is now indicated in all these conditions, and in patients with clinical features suggestive of a specific inherited disease.
SUMMARY: Genetic testing should be considered in all patients with adrenal tumours, and is crucial for correct management. The identification of a germline mutation in a susceptibility gene guides treatment in patients with adrenal cancer and will facilitate risk-adapted screening/surveillance protocols in mutation carriers.

PMID: 30985498 [PubMed - indexed for MEDLINE]

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