Error message

Deprecated function: The each() function is deprecated. This message will be suppressed on further calls in menu_set_active_trail() (line 2405 of /mnt/stor5-wc1-dfw1/644438/www.adrenaltumors.org/web/content/includes/menu.inc).

News & Updates

Subscribe to News & Updates feed News & Updates
NCBI: db=pubmed; Term=adrenal tumor
Updated: 4 days 21 hours ago

adrenal tumor; +40 new citations

Thu, 05/16/2019 - 06:19

40 new pubmed citations were retrieved for your search. Click on the search hyperlink below to display the complete search results:

adrenal tumor

These pubmed results were generated on 2019/05/16

PubMed comprises more than millions of citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.

adrenal tumor; +21 new citations

Thu, 05/09/2019 - 03:45

21 new pubmed citations were retrieved for your search. Click on the search hyperlink below to display the complete search results:

adrenal tumor

These pubmed results were generated on 2019/05/09

PubMed comprises more than millions of citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.

adrenal tumor; +24 new citations

Thu, 05/02/2019 - 03:30

24 new pubmed citations were retrieved for your search. Click on the search hyperlink below to display the complete search results:

adrenal tumor

These pubmed results were generated on 2019/05/02

PubMed comprises more than millions of citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.

adrenal tumor; +27 new citations

Thu, 04/25/2019 - 02:09

27 new pubmed citations were retrieved for your search. Click on the search hyperlink below to display the complete search results:

adrenal tumor

These pubmed results were generated on 2019/04/25

PubMed comprises more than millions of citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.

adrenal tumor; +21 new citations

Thu, 04/18/2019 - 01:34

21 new pubmed citations were retrieved for your search. Click on the search hyperlink below to display the complete search results:

adrenal tumor

These pubmed results were generated on 2019/04/18

PubMed comprises more than millions of citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.

adrenal tumor; +23 new citations

Wed, 04/10/2019 - 23:00

23 new pubmed citations were retrieved for your search. Click on the search hyperlink below to display the complete search results:

adrenal tumor

These pubmed results were generated on 2019/04/10

PubMed comprises more than millions of citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.

[Surgical treatment of neurofibromatosis type I followed by retroperitoneal tumor (in Russian only)].

Wed, 04/03/2019 - 20:08
Related Articles

[Surgical treatment of neurofibromatosis type I followed by retroperitoneal tumor (in Russian only)].

Khirurgiia (Mosk). 2019;(3):5-14

Authors: Berelavichus SV, Struchkov VY, Son AI, Kriger AG

Abstract
AIM: To determine the optimal surgical approach in patients with abdominal and retroperitoneal manifestations of Recklinghausen's disease.
MATERIAL AND METHODS: There were 4 patients (3 women and 1 man) with neurofibromatosis type I who were treated at Vishnevsky National Medical Research Center of Surgery.
RESULTS: There were 3 robot-assisted procedures: excision of retroperitoneal tumors (plexiform neurofibroma and schwannoma) in 2 cases and right adrenal pheochromocytoma in 1 patient. We also included 1 clinical case of conventional surgery for neurofibromatosis type I followed by multiple gastrointestinal stromal duodenal and intestinal tumors. In one case, postoperative period was complicated by fluid accumulation in the bed of previously removed tumor that required US-assisted drainage. Postoperative period was uneventful in other cases.
CONCLUSION: Robot-assisted surgery is safe and effective in patients with Recklinghausen's disease followed by single abdominal and retroperitoneal tumors. It is more expedient to choose conventional technique for multiple tumors located in different parts of retroperitoneal space or abdominal cavity.

PMID: 30938352 [PubMed - in process]

Transumbilical laparoendoscopic single-site adrenalectomy: A feasible and safe alternative to standard laparoscopy.

Wed, 04/03/2019 - 20:08
Related Articles

Transumbilical laparoendoscopic single-site adrenalectomy: A feasible and safe alternative to standard laparoscopy.

Arch Ital Urol Androl. 2019 Mar 29;91(1):1-4

Authors: Carvalho JA, Nunes PT, Antunes H, Parada B, Retroz E, Tavares-da-Silva E, Paiva I, Figueiredo AJ

Abstract
OBJECTIVES: Standard multi-port laparoscopic adrenalectomy (LA) is considered the gold standard for benign adrenal tumors. Single-site LA has been proposed as a feasible and safe alternative because of lower invasiveness, improved cosmetics, less pain and shorter hospital stay. The objective was to evaluate and compare results of single-site transumbilical laparoendoscopic adrenalectomy with standard LA for adrenal tumors.
MATERIALS AND METHODS: One hundred consecutive adrenalectomies from 93 patients, performed between March 2009 and June 2017, were laparoscopically excised: 59 by standard multi-port LA (group 1) and 41 by transumbilical laparoendoscopic single-site adrenalectomy (group 2). Data gathered included demographics, comorbidities, preoperative imaging, tumor characteristics, perioperative data, surgical complications, pathology and follow-up. IBM SPSS Statistics 23 software was used and p value < 0.05 was considered significant.
RESULTS: Patients of group 2 were younger (48.7 ± 13.9 versus 59.7 ± 15.1 years; p < 0.001) and had fewer comorbidities (p < 0.05). Mean tumor diameter in group 2 was lower than those of group 1 (27.52 ± 14.3 versus 47.9 ± 30.6 mm; p < 0.001). Tumor laterality did not influence the choice of technique nor the surgical morbidity. All procedures were successfully completed, although one standard LA needed conversion to open surgery. Mean operative time, hemorrhagic losses, postoperative opioid analgesic requirement and hospital stay were not statistically different between groups. Most patients in group 2 (31 patients, 85.4%) did not require drainage, compared to 14 (25.4%) patients of group 1 (p < 0.001). Patients who underwent single-site LA resumed normal diet earlier (1.0 ± 0.2 versus 1.6 ± 0.7 days; p < 0.001). There were no reoperations and no perioperative mortality. Overall mean follow-up time was 94.9 ± 3.1 months, not statiscally different between groups (p = 0.7).
CONCLUSIONS: Our results revealed that transumbilical approach for laparoendoscopic single-site adrenalectomy for adrenal tumors is a feasible and safe alternative to standard laparoscopic adrenalectomy.

PMID: 30932420 [PubMed - in process]

Leveraging Prior Knowledge of Endocrine Immune Regulation in the Therapeutically Relevant Phenotyping of Women With Chronic Fatigue Syndrome.

Wed, 04/03/2019 - 20:08
Related Articles

Leveraging Prior Knowledge of Endocrine Immune Regulation in the Therapeutically Relevant Phenotyping of Women With Chronic Fatigue Syndrome.

Clin Ther. 2019 Mar 28;:

Authors: Morris MC, Cooney KE, Sedghamiz H, Abreu M, Collado F, Balbin EG, Craddock TJA, Klimas NG, Broderick G, Fletcher MA

Abstract
PURPOSE: The complex and varied presentation of myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS) has made it difficult to diagnose, study, and treat. Its symptoms and likely etiology involve multiple components of endocrine and immune regulation, including the hypothalamic-pituitary-adrenal axis, the hypothalamic-pituitary-gonadal axis, and their interactive oversight of immune function. We propose that the persistence of ME/CFS may involve changes in the regulatory interactions across these physiological axes. We also propose that the robustness of this new pathogenic equilibrium may at least in part explain the limited success of conventional single-target therapies.
METHODS: A comprehensive model was constructed of female endocrine-immune signaling consisting of 28 markers linked by 214 documented regulatory interactions. This detailed model was then constrained to adhere to experimental measurements in a subset of 17 candidate immune markers measured in peripheral blood of patients with ME/CFS and healthy control subjects before, during, and after a maximal exercise challenge. A set of 26 competing numerical models satisfied these data to within 5% error.
FINDINGS: Mechanistically informed predictions of endocrine and immune markers that were either unmeasured or exhibited high subject-to-subject variability pointed to possible context-specific overexpression in ME/CFS at rest of corticotropin-releasing hormone, chemokine (C-X-C motif) ligand 8, estrogen, follicle-stimulating hormone (FSH), gonadotropin-releasing hormone 1, interleukin (IL)-23, and luteinizing hormone, and underexpression of adrenocorticotropic hormone, cortisol, interferon-γ, IL-10, IL-17, and IL-1α. Simulations of rintatolimod and rituximab treatment predicted a shift in the repertoire of available endocrine-immune regulatory regimens. Rintatolimod was predicted to make available substantial remission in a significant subset of subjects, in particular those with low levels of IL-1α, IL-17, and cortisol; intermediate levels of progesterone and FSH; and high estrogen levels. Rituximab treatment was predicted to support partial remission in a smaller subset of patients with ME/CFS, specifically those with low norepinephrine, IL-1α, chemokine (C-X-C motif) ligand 8, and cortisol levels; intermediate FSH and gonadotropin-releasing hormone 1 levels; and elevated expression of tumor necrosis factor-α, luteinizing hormone, IL-12, and B-cell activation.
IMPLICATIONS: Applying a rigorous filter of known signaling mechanisms to experimentally measured immune marker expression in ME/CFS has highlighted potential new context-specific markers of illness. These novel endocrine and immune markers may offer useful candidates in delineating new subtypes of ME/CFS and may inform on refinements to the inclusion criteria and instrumentation of new and ongoing trials involving rintatolimod and rituximab treatment protocols.

PMID: 30929860 [PubMed - as supplied by publisher]

Toxicities with Immune Checkpoint Inhibitors: Emerging Priorities From Disproportionality Analysis of the FDA Adverse Event Reporting System.

Wed, 04/03/2019 - 20:08
Related Articles

Toxicities with Immune Checkpoint Inhibitors: Emerging Priorities From Disproportionality Analysis of the FDA Adverse Event Reporting System.

Target Oncol. 2019 Mar 29;:

Authors: Raschi E, Mazzarella A, Antonazzo IC, Bendinelli N, Forcesi E, Tuccori M, Moretti U, Poluzzi E, De Ponti F

Abstract
BACKGROUND: Immune checkpoint inhibitors (ICIs), including antibodies targeting cytotoxic T-lymphocyte associated protein 4 (CTLA4) and programmed cell death 1 or its ligand (PD1/PDL1), elicit different immune-related adverse events (irAEs), but their global safety is incompletely characterized.
OBJECTIVE: The aim of this study was to characterize the spectrum, frequency, and clinical features of ICI-related adverse events (AEs) reported to the FDA Adverse Event Reporting System (FAERS).
PATIENTS AND METHODS: AEs from FAERS (up to June 2018) recording ICIs (ipilimumab, nivolumab, pembrolizumab, atezolizumab, avelumab, durvalumab) as suspect were extracted. Comprehensive disproportionality analyses were performed through the reporting odds ratio (ROR) with 95% confidence interval (95% CI), using other oncological drugs as comparison. An overview of systematic reviews (OoSRs) was also undertaken to identify irAEs with consistent positive associations.
RESULTS: ICIs were recorded in 47,266 reports, submitted mainly by consumers receiving monotherapy with anti-PD1/PDL1 drugs. Three areas of toxicity emerged from both disproportionality analysis and the OoSRs (32 studies): endocrine (N = 2863; ROR = 6.91; 95% CI 6.60-7.23), hepatobiliary (2632; 1.33; 1.28-1.39), and respiratory disorders (7240; 1.04; 1.01-1.06). Different reporting patterns emerged for anti-CTLA4 drugs (e.g., hypophysitis, adrenal insufficiency, hypopituitarism, and prescribed overdose) and anti-PD1/PDL1 agents (e.g., pneumonitis, cholangitis, vanishing bile duct syndrome, tumor pseudoprogression, and inappropriate schedule of drug administration). No increased reporting emerged when comparing combination with monotherapy regimens, but multiple hepatobiliary/endocrine/respiratory irAEs were recorded.
CONCLUSIONS: This parallel approach through contemporary post-marketing analysis and OoSRs confirmed that ICIs are associated with a multitude of irAEs, with different reporting patterns between anti-CTLA4 and anti-PD1/PDL1 medications. Close clinical monitoring is warranted to early diagnose and timely manage irAEs, especially respiratory, endocrine, and hepatic toxicities, which warrant further characterization; patient- and drug-related risk factors should be assessed through analytical pharmaco-epidemiological studies and prospective multicenter registries.

PMID: 30927173 [PubMed - as supplied by publisher]

Pheochromocytomas and Paragangliomas: From Genetic Diversity to Targeted Therapies.

Wed, 04/03/2019 - 20:08
Related Articles

Pheochromocytomas and Paragangliomas: From Genetic Diversity to Targeted Therapies.

Cancers (Basel). 2019 Mar 28;11(4):

Authors: Pang Y, Liu Y, Pacak K, Yang C

Abstract
Pheochromocytoma and paraganglioma (PCPGs) are rare neuroendocrine tumors that arise from the chromaffin tissue of adrenal medulla and sympathetic ganglia. Although metastatic PCPGs account for only 10% of clinical cases, morbidity and mortality are high because of the uncontrollable mass effect and catecholamine level generated by these tumors. Despite our expanding knowledge of PCPG genetics, the clinical options to effectively suppress PCPG progression remain limited. Several recent translational studies revealed that PCPGs with different molecular subtypes exhibit distinctive oncogenic pathways and spectrum of therapy resistance. This suggests that therapeutics can be adjusted based on the signature molecular and metabolic pathways of PCPGs. In this review, we summarized the latest findings on PCPG genetics, novel therapeutic targets, and perspectives for future personalized medicine.

PMID: 30925729 [PubMed]

[Corticosurrenaloma: an exceptional cause of primary hyperaldosteronism].

Wed, 04/03/2019 - 20:08
Related Articles

[Corticosurrenaloma: an exceptional cause of primary hyperaldosteronism].

Pan Afr Med J. 2018;31:60

Authors: Zantour B, Charrada I, Elati Z, Ammari FL, Boubaker F, Arfa S, Berriche O, Alaya W, Sfar MH

Abstract
Corticosurrenaloma is a rare malignant tumor of the adrenal gland that often secretes corticosteroids, sex steroids and precursors. Aldosterone-producing corticosurrenaloma is very rare, accounting for 1 case/10million inhabitants. We report the case of a 38-year old man presenting with severe arterial hypertension associated with deep hypokalaemia (2.2 mmol/L). Exploration showed primary hyperaldosteronism (aldosterone = 2645 pmol/l, aldosterone/renin ratio = 327 pmol/MUI), with hypersecretion of glucocorticoids. Abdominopelvic CT scan revealed left poorly differentiated and heterogeneous adrenal mass measuring 9cm, infiltrating into the surrounding adipose tissue and the diaphragm, extending into the left renal vein, with regional adenopathy and hepatic nodule measuring 4cm. The patient underwent radical nephrectomy followed by right hepatectomy two months after resulting in remission. A year after the patient developed lung metastases. This study highlights that corticosurrenaloma should be suspected in patients with primary aldosteronism despite its rarity.

PMID: 30923605 [PubMed - in process]

Adrenocortical hypofunction with simultaneous primary aldosteronism: A case report.

Wed, 04/03/2019 - 20:08
Related Articles

Adrenocortical hypofunction with simultaneous primary aldosteronism: A case report.

Medicine (Baltimore). 2019 Mar;98(13):e15033

Authors: Liang K, Ou X, Huang X, Lan Q

Abstract
RATIONALE: Cases of adrenocortical hyperfunction combined with primary aldosteronism have been reported in the literature, and the underlying mechanism involves the secretion of aldosterone and glucocorticoids by a tumor or an adenoma. However, adrenocortical hypofunction and coexisting primary aldosteronism have not been reported until now. Herein, we report a case of adrenocortical hypofunction combined with primary aldosteronism.
PATIENT CONCERNS: A 66-year-old Chinese woman with rheumatoid arthritis who had been diagnosed with secondary adrenal insufficiency and was taking prednisone acetate tablets for replacement treatment presented to our department. She also had type 2 diabetes mellitus, osteoporosis, bilateral knee osteoarthritis, and lumbar vertebral compression fracture. She had previously developed tuberculosis, which had been cured.
DIAGNOSIS: The cortisol and adrenocorticotropic hormone rhythm indicated cortisol dysfunction in the patient. A 64-slice computed tomography and magnetic resonance imaging both showed bilateral adrenal hyperplasia. A postural stimulation test indicated a high level of aldosteronism and a high aldosterone-to-renin ratio (ARR, supine position: aldosterone 1788.73 pg/mL, ARR 146.62; upright position: aldosterone 2916.21 pg/mL, ARR 92.29). The captopril test showed the aldosterone level decreased by 364.70 pg/mL 1 hour after administration of captopril (from 2153.28 to 1788.58 pg/mL). The decline in aldosterone level was approximately 16.90% (i.e., <30%), and the ARR was still >40. Based on the above-mentioned findings, we diagnosed the patient with adrenocortical hypofunction with primary aldosteronism.
INTERVENTIONS: We administered spironolactone 20 mg twice daily and continued the glucocorticoid replacement therapy.
OUTCOMES: One week after diagnosis, the patient had an aldosterone level of 2201.16 pg/mL, plasma renin activity of 3.88 ng/mL/h, and an ARR of 56.7 (upright position). Her blood pressure was maintained within the normal range.
LESSONS: Although adrenocortical hypofunction with primary aldosteronism is rare, cases of primary aldosteronism complicated with hypercortisolism are occasionally encountered. Hence, whenever possible, we recommend testing both aldosterone and cortisol levels in all patients with adrenal dysfunction.

PMID: 30921227 [PubMed - in process]

microRNA-431 as a Chemosensitizer and Potentiator of Drug Activity in Adrenocortical Carcinoma.

Wed, 04/03/2019 - 20:08
Related Articles

microRNA-431 as a Chemosensitizer and Potentiator of Drug Activity in Adrenocortical Carcinoma.

Oncologist. 2019 Mar 27;:

Authors: Kwok GTY, Zhao JT, Glover AR, Gill AJ, Clifton-Bligh R, Robinson BG, Ip JCY, Sidhu SB

Abstract
BACKGROUND: Adrenocortical carcinoma (ACC) is a rare endocrine cancer with treatments limited in efficacy for metastatic disease. New molecular targeted therapies have yet to improve patient outcomes. In contrast, established treatment regimens of adrenolytics and chemotherapy have demonstrated treatment benefit, although admittedly in a minority of patients. Identification of microRNAs (miRNAs) in patients responsive to adjuvant therapy may offer a means to sensitize patients with progressive disease to existing adjuvant regimens.
MATERIALS AND METHODS: Samples from primary ACC tumors of 10 Stage IV patients were examined for differentially expressed miRNAs between a "sensitive" and "resistant" cohort. Candidate microRNAs were restored via transfection in two functional ACC cell lines. Gain of function and effects on apoptosis and cell cycle were assessed.
RESULTS: microRNA-431 (miR-431) was underexpressed in patients with ACC with progressive disease undergoing adjuvant therapy. Restoration of miR-431 in vitro decreased the half maximal inhibitory concentrations of doxorubicin and mitotane, with markedly increased apoptosis. We found that a reversal of epithelial-mesenchymal transition underlies the action of miR-431 with doxorubicin treatment, with Zinc Finger E-Box Binding Homeobox 1 implicated as the molecular target of miR-431 in ACC.
CONCLUSION: This is the first report of the potential of miRNA therapy to sensitize ACC to current established adjuvant therapy regimens, which may mitigate the resistance underlying treatment failure in patients with advanced ACC. Effective and well-studied methods of targeted miRNA delivery in existence hints at the imminent translatability of these findings.
IMPLICATIONS FOR PRACTICE: Adrenocortical carcinoma (ACC) is a rare endocrine cancer with outcomes not improving despite extensive research and new targeted therapies. Mitotane and etoposide/doxorubicin/cisplatin chemotherapy is trial validated for improved recurrence-free survival. However, a minority of patients experience sustained benefit. Significant side effects exist for this regimen, with patients often unable to attain target drug doses shown to give survival benefit. This preclinical study examines the role of microRNAs in sensitizing ACC to doxorubicin or mitotane. This study offers an important bridge between new and existing cancer treatments, offering an imminently translatable approach to the treatment of adrenocortical carcinoma.

PMID: 30918109 [PubMed - as supplied by publisher]

Transperitoneal versus retroperitoneal laparoscopic adrenalectomy for adrenal tumours in adults.

Wed, 04/03/2019 - 20:08
Related Articles

Transperitoneal versus retroperitoneal laparoscopic adrenalectomy for adrenal tumours in adults.

Cochrane Database Syst Rev. 2018 12 30;12:CD011668

Authors: Arezzo A, Bullano A, Cochetti G, Cirocchi R, Randolph J, Mearini E, Evangelista A, Ciccone G, Bonjer HJ, Morino M

Abstract
BACKGROUND: Laparoscopic adrenalectomy is an accepted treatment worldwide for adrenal gland disease in adults. The transperitoneal approach is more common. The retroperitoneal approach may be preferred, to avoid entering the peritoneum, but no clear advantage has been demonstrated so far.
OBJECTIVES: To assess the effects of laparoscopic transperitoneal adrenalectomy (LTPA) versus laparoscopic retroperitoneal adrenalectomy (LRPA) for adrenal tumours in adults.
SEARCH METHODS: We searched CENTRAL, MEDLINE, Embase, ICTRP Search Portal, and ClinicalTrials.gov to 3 April 2018. We applied no language restrictions.
SELECTION CRITERIA: Two review authors independently scanned the abstract, title, or both sections of every record retrieved to identify randomised controlled trials (RCTs) on laparoscopic adrenalectomy for preoperatively assessed adrenal tumours. Participants were affected by corticoid and medullary, benign and malignant, functional and silent tumours or masses of the adrenal gland, which were assessed by both laboratory and imaging studies.
DATA COLLECTION AND ANALYSIS: Two review authors independently extracted data, assessed trials for risk of bias, and evaluated overall study quality using GRADE criteria. We calculated the risk ratio (RR) for dichotomous outcomes, or the mean difference (MD) for continuous variables, and corresponding 95% confidence interval (CI). We primarily used a random-effects model for pooling data.
MAIN RESULTS: We examined 1069 publications, scrutinized 42 full-text publications or records, and included five RCTs. Altogether, 244 participants entered the five trials; 127 participants were randomised to retroperitoneal adrenalectomy and 117 participants to transperitoneal adrenalectomy. Two trials had a follow-up of nine months, and three trials a follow-up of 31 to 70 months. Most participants were women, and the average age was around 40 years. Three trials reported all-cause mortality; in two trials, there were no deaths, and in one trial with six years of follow-up, four participants died in the LRPA group and one participant in the LTPA group (164 participants; low-certainty evidence). The trials did not report all-cause morbidity. Therefore, we analysed early and late morbidity, and included specific adverse events under these outcome measures. The results were inconclusive between LRPA and LTPA for early morbidity (usually reported within 30 to 60 days after surgery; RR 0.56, 95% CI 0.27 to 1.16; P = 0.12; 5 trials, 244 participants; very low-certainty evidence). Nine out of 127 participants (7.1%) in the LRPA group, compared with 16 out of 117 participants (13.7%) in the LTPA group experienced an adverse event. Participants in the LRPA group may have a lower risk of developing late morbidity (reported as latest available follow-up; RR 0.12, 95% CI 0.01 to 0.92; P = 0.04; 3 trials, 146 participants; very low-quality evidence). None of the 78 participants in the LRPA group, compared with 7 of the 68 participants (10.3%) in the LTPA group experienced an adverse event.None of the trials reported health-related quality of life. The results were inconclusive for socioeconomic effects, assessed as time to return to normal activities and length of hospital stay, between the intervention and comparator groups (very low-certainty evidence). Participants who had LRPA may have had an earlier start on oral fluid or food intake (MD -8.6 hr, 95% CI -13.5 to -3.7; P = 0.0006; 2 trials, 89 participants), and ambulation (MD -5.4 hr, 95% CI -6.8 to -4.0 hr; P < 0.0001; 2 trials, 89 participants) than those in the LTPA groups. Postoperative and operative parameters (duration of surgery, operative blood loss, conversion to open surgery) showed inconclusive results between the intervention and comparator groups.
AUTHORS' CONCLUSIONS: The body of evidence on laparoscopic retroperitoneal adrenalectomy compared with laparoscopic transperitoneal adrenalectomy is limited. Late morbidity might be reduced following laparoscopic retroperitoneal adrenalectomy, but we are uncertain about this effect because of very low-quality evidence. The effects on other key outcomes, such as all-cause mortality, early morbidity, socioeconomic effects, and operative and postoperative parameters are uncertain. LRPA might show a shorter time to oral fluid or food intake and time to ambulation, but we are uncertain whether this finding can be replicated. New long-term RCTs investigating additional data, such as health-related quality of life, surgeons' level of experience, treatment volume of surgical centres, and details on techniques used are needed.

PMID: 30595004 [PubMed - indexed for MEDLINE]

Glomulin mutation and glomuvenous malformations: two case reports with the same mutation but different phenotypes.

Wed, 04/03/2019 - 20:08
Related Articles

Glomulin mutation and glomuvenous malformations: two case reports with the same mutation but different phenotypes.

Eur J Dermatol. 2018 Oct 01;28(5):716-718

Authors: Collantes-Rodríguez C, De La Varga-Martínez R, Ossorio-García L, Villegas-Romero I, Jiménez-Gallo D, Mora-López F, Linares-Barrios M

Abstract

PMID: 30325312 [PubMed - indexed for MEDLINE]

Autonomous hypercortisolism: definition and clinical implications.

Wed, 04/03/2019 - 20:08
Related Articles

Autonomous hypercortisolism: definition and clinical implications.

Minerva Endocrinol. 2019 Mar;44(1):33-42

Authors: Reimondo G, Puglisi S, Pia A, Terzolo M

Abstract
In current practice, an adrenal adenoma usually comes as an unexpected byproduct of an imaging study performed for unrelated reasons, without any prior suspect of adrenal disease. Therefore, these tumors currently represent a public health challenge because they are increasingly recognized due to the widespread use of high-resolution cross-sectional imaging for diagnostic purposes. In radiology series, the prevalence of adrenal adenomas increases steeply with age, from around 3% below the age of 50 years up to 10% in the ageing population. These tumors may have clinical relevance because they are able to secrete cortisol autonomously, independently from the pituitary control, in up to 20-30% of patients. In most of the cases the resulting cortisol excess is insufficient to produce a typical Cushing phenotype but may have clinical consequences, such as hypertension, diabetes, obesity, dyslipidemia and osteoporosis. Despite some controversy on the most effective diagnostic algorithm to define this subtle hypercortisolism, there is mounting evidence that a simple approach by using the 1-mg overnight dexamethasone suppression test (DST) may stratify patients for their cardiovascular risk. Cross-sectional, retrospective studies showed that patients with increasingly higher cortisol following DST have an adverse cardiovascular risk profile and are at increased risk of death. Therefore, also a subtle autonomous cortisol excess is associated with increased morbidity and mortality, mainly of cardiovascular origin.

PMID: 29963828 [PubMed - indexed for MEDLINE]

Pathogenesis of benign unilateral adrenocortical tumors: focus on cAMP/PKA pathway.

Wed, 04/03/2019 - 20:08
Related Articles

Pathogenesis of benign unilateral adrenocortical tumors: focus on cAMP/PKA pathway.

Minerva Endocrinol. 2019 Mar;44(1):25-32

Authors: Weigand I

Abstract
Somatic mutations affecting genes in the cAMP/PKA (protein kinase A) signaling pathway have been described as causative for the pathogenesis of benign unilateral adrenocortical adenomas associated with cortisol over secretion. These include predominantly somatic mutations in the PRKACA gene which encodes the catalytic subunit α of PKA. In addition, mutations in the GNAS gene, coding for the stimulatory G protein α, have been observed in approximately 10% of cortisol producing adenomas (CPA). The mutations render PKA signaling constitutively active and are therefore involved in cortisol over secretion of these tumors. Despite the prominent role of the cAMP/PKA pathway in the pathogenesis of unilateral CPA, also mutations in the CTNNB1 gene, encoding β-catenin, were identified in CPA. However, mutations in β-catenin are not limited to CPA and are not associated with cortisol secretion since they were predominantly found in endocrine-inactive adenomas (EIA) and might hence contribute to tumorigenesis in adrenocortical tissues. In this review, recent findings in the pathogenesis of benign adrenocortical tumors with a particular focus on the cAMP/PKA signaling pathway are summarized.

PMID: 29963826 [PubMed - indexed for MEDLINE]

The role of steroid metabolome analysis for the diagnosis and follow-up of adrenocortical tumors.

Wed, 04/03/2019 - 20:08
Related Articles

The role of steroid metabolome analysis for the diagnosis and follow-up of adrenocortical tumors.

Minerva Endocrinol. 2019 Mar;44(1):19-24

Authors: Chortis V

Abstract
The diagnostic work-up of adrenal tumors, often incidentally discovered, has emerged as an ever-increasing diagnostic problem for clinical endocrinologists. No imaging modality has sufficiently high sensitivity and specificity at differentiating benign from malignant adrenal lesions. It has long been observed that adrenocortical carcinomas (ACCs) present an immature pattern of steroidogenesis, dominated by steroid hormone precursors. Modern mass spectrometry-based assays can generate multi-steroid metabolite profiles in urine collections, which can detect differences between ACCs and benign adrenocortical adenomas (ACAs). This review summarizes the promising results of studies which have applied steroid metabolite profiling in biological fluids as a novel diagnostic tool for patients with adrenal tumors, as well as the challenges and limitations of this approach. It also discusses the potential role of steroid profiling as a biochemical surveillance tool to detect recurrence in patients who have undergone resection of an ACC.

PMID: 29905434 [PubMed - indexed for MEDLINE]

Adrenal incidentaloma: differential diagnosis and management strategies.

Wed, 04/03/2019 - 20:08
Related Articles

Adrenal incidentaloma: differential diagnosis and management strategies.

Minerva Endocrinol. 2019 Mar;44(1):4-18

Authors: Morelli V, Palmieri S

Abstract
Adrenal incidentaloma is a frequent clinical finding. Once an adrenal mass is detected, is mandatory to determine whether the lesion is malignant or benign and whether it is hormonally active or non-functioning, to estabilish an adequate treatement or follow-up. The European Society of Endocrinology and ENSAT Guideline recently provided the best recommendation based on the available literature. However, due to the retrospective design of the majority of the studies, the small number of patients included and the inadequate follow-up, some issues are still unresolved. In particular, there is a general consensus about the need of adrenalectomy in the presence of unilateral adrenal mass and clinically relevant hormone excess or radiological findings suspected for malignancy. On the other side, how to manage adrenal masses with indeterminate characteristics or subtle cortisol secretion, and how long the radiological and functional follow-up of benign adrenal mass should last in non-operated patients, are still open questions. Therefore, high-quality research for establish the adequate management of these patients and randomized clinical trials are needed to avoid unnecessary investigations and invasive procedures and ensure a clinically effective work-up.

PMID: 29808642 [PubMed - indexed for MEDLINE]

Pages