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NCBI: db=pubmed; Term=adrenal tumor
Updated: 15 hours 29 min ago

[Clinicopathological analysis of patients with papillary renal cell carcinoma].

Tue, 10/23/2018 - 18:42
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[Clinicopathological analysis of patients with papillary renal cell carcinoma].

Beijing Da Xue Xue Bao Yi Xue Ban. 2018 Oct 18;50(5):805-810

Authors: Ding ZS, Qiu M, Xu ZC, Xiao RT, Ge LY, Ma LL

Abstract
OBJECTIVE: To investigate the clinicopathological features,treatment and prognosis of patients with papillary renal cell carcinoma (PRCC) and PRCC-complicated with tumor thrombus.
METHODS: Single center retrospective analysis of 75 patients with PRCC treated from January 2012 to October 2017 was performed. There were 55 males and 20 females at an age range of 24-82 years. Sixteen PRCC patients were complicated with tumor thrombus. All the patients were with a surgery and had clear pathological diagnosis and detailed follow-up data. The clinicopathological features, prognosis and influencing factors of the patients with PRCC and PRCC complicated with tumor thrombus were analyzed and summarized.
RESULTS: The average age of the 75 patients was(56.05±11.59)years,the average body mass index (BMI) was (26±3) kg/m², and the average tumor maximum diameter was (5.17±3.85) cm. There were significant differences between tumor maximum diameter larger than 7 cm and less than 7 cm (69.6% vs. 94.4%, P<0.001), lymph node metastasis and no lymph node metastasis (<38% vs. 98%, P<0.001), adrenal metastasis and no adrenal metastasis (0% vs. 95.3%, P<0.001), pulmonary metastasis and no pulmonary metastasis (0% vs.90.7%, P<0.001), complicated with and without tumor thrombus (<66.4% vs. 93.5%, P<0.001) on the effect of 3-year survival rate of the PRCC patients. In this study, there were 16 patients with type 2 PRCC complicated with tumor thrombus. There were significant differences in concomitant symptoms (62.5% vs. 22.0%, P=0.005), maximum tumor diameter (68.8% vs.13.3%, P<0.001), adrenal metastasis (18.8% vs. 0.02%, P=0.029), pulmonary metastasis (18.8% vs. 0%, P=0.008), nuclear grade (P<0.001) and pathological type (100% vs. 44.1%, P<0.001) between the PRCC patients with and without tumor thrombus.
CONCLUSION: There were significant differences in tumor diameter,lymph node metastasis,adrenal metastasis, pulmonary metastasis,pathological type, nuclear grade and tumor thrombus in the effect of the 3-year survival rate of PRCC patients. PRCC patients with tumor thrombus were more commonly suffered from type 2 PRCC, for whom the tumor diameter was larger,the nuclear grade was higher,and the distance metastasis happened more easily.

PMID: 30337739 [PubMed - in process]

Association of Papillary Thyroid Carcinoma with Primary Aldosteronism.

Tue, 10/23/2018 - 18:42
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Association of Papillary Thyroid Carcinoma with Primary Aldosteronism.

Intern Med. 2018 Oct 17;:

Authors: Nakamura S, Ishimori M, Yamakita N

Abstract
Objective The association of primary aldosteronism (PA) with thyroid disease has already been suggested. The aim of this study was to examine the presence of PA in patients with papillary thyroid carcinoma (PC) and to characterize such PC patients with PA Patients and Methods We examined the presence of PA in 81 consecutive patients with PC, whose random sitting blood pressure (BP) was ≥140/90 mmHg in the office (n= 68), who had an incidental adrenal tumor or adrenal enlargement (n=9), or who showed hypokalemia (n=4). Thirty-one of these 81 patients had been treated with anti-hypertensive drugs. The plasma aldosterone concentration (PAC) and plasma renin activity (PRA) were first measured before operation in 16 patients and after operation in 65 patients. PA was diagnosed according to the guidelines of the Japan Endocrine Society. Results Forty patients with PC with a random PAC/PRA ratio of over 200 were subjected to a further study (12 of these patients had been treated with anti-hypertensive drugs). Ultimately, 15 patients with PC were diagnosed with PA. Adrenal venous sampling was done in 9 out of 15 patients with PC associated with PA. No patients were diagnosed as having unilateral lesions. Among the 15 patients, white -coat hypertension was observed in 5 patients, and normotension was observed in 1 patient. Conclusion These findings suggest that the prevalence of PA may be high among patients with PC. An active examination is needed to detect PA, as its signs and symptoms may be mild in patients with PC associated with hypertension.

PMID: 30333389 [PubMed - as supplied by publisher]

An Interesting Case of Hepatic Adrenocortical Carcinoma.

Tue, 10/23/2018 - 18:42
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An Interesting Case of Hepatic Adrenocortical Carcinoma.

Acta Med Indones. 2018 Jul;50(3):257-259

Authors: Permana H, Darmawan G, Ritonga E, Kusumawati M, Miftahurachman M, Soetedjo NN

Abstract
Adrenocortical carcinoma (ACC) is a rare solid tumor with an incidence of 0.5 to 2 cases per million per year. It affects women more commonly than men with a ratio of 1.5:1. Ectopic ACC are considered to be extremely rare with no exact incidence data yet. We report an interesting case of hepatic ACC in a young woman with clinical signs of virilization.A-21-year old Sundanese woman visited our endocrine clinic with progressive hirsutism over the face, body, and extremities starting 14 years previously. She had irregular, heavy periods when she was 7 years old. She also experienced pubertal development of her breasts. However, both menstrual cycle and breast development ceased when she was 8 years old. She noticed voice deepening and alopecia. Physical examination showed male-type alopecia and intense hirsutism. Tanner stage was 3 for breast tissue and 5 for pubic hair. There was no galactorrhea. Body mass index was 21.4 kg/m2. Hormonal evaluation revealed increased level of free testosterone (>1500 ng/dl; NV: 8.4-48.1 ng/dl), dehydroepiandrosterone sulfate (>1000 ug/ml; NV: 65.1-369 ug/ml), and estradiol (533.60 pg/ml; NV: 14-124 pg/ml), low level of LH (<0.07 mIU/ml; NV: 1.7-11 mIU/ml) and FSH (<0.30 mIU/ml; NV: 1.34-9.40 mIU/ml), slight increased in morning serum cortisol (26.61 ug/ml; NV: 4.3-22.4 ug/ml), normal serum thyroid stimulating hormone (3.2 mIU/l; NV: 0.34-4.25 mIU/l) and prolactin (14.70 ng/ml; NV: 3.30-15.80 ng/ml). Gynecological ultrasound and brain MRI examination showed no structural abnormality. Abdominal CT scan demonstrated contrast enhanced solid inhomogenous mass sized 11.6 x 14.2 x 15.6 cm in right liver lobe. Neither suprarenal mass nor paraaortic lymphadenopathy was seen in the abdominal CT scan. Chromosomal examination revealed normal female karyotype (46, XX). Further liver biopsy showed morphology and immunohistochemistry (positive for CD 56, HEP 1, and NSE) consistent with adrenocortical carcinoma. Surgical therapy with referral to other institution was offered to the patient as first line treatment. Meanwhile, the patient got spironolactone 100 mg OD.Virilizing tumors are rare and few of them are androgen-producing adrenal tumors. Ectopic adrenal tumors are even rarer. Ectopic adrenal tissue can be found close to the adrenal glands, or along the path of descent or in association with gonad. Moreover, they have ever been reported in nervous system, stomach, gall bladder, and liver. There have been several case reports of adrenal rest tumor of liver; however, our literature review found no report of ectopic ACC of liver. Cortical tissue, embrologically derived from mesoderm, seems to be the sole component of the tumor which can undergo malignant transformation or become hormonally functional. Functioning tumors are more frequent in women. Our case demonstrated virilization as chief complaint. The ectopically located functioning tumors display the same clinical picture as tumors located in adrenal gland, with Cushing's syndrome and virilization are the most frequent symptoms in order of frequency. The virilization, as shown in our case, is due to excessive androgen production of dehydroepiandrosterone sulfate and testosterone. The distinction of ACC from benign adrenocortical tumor is important. Since there is no previous report of hepatic ACC and surgery is the keystone of curative treatment modality for ACC at adrenal gland, we planned the patient for surgical resection. Adjuvant treatment with chemotherapy (mitotane and combination of cytotoxic drugs), irradiation might be considered in ACC treatment. To control androgen effects, spironolactone was administered in our patient. However, there was no significant improvement in symptoms.In conclusion, we present the first reported case of hepatic ACC. A thorough history, physical examination, and appropriate laboratory, imaging examination are critical in evaluating virilized female patients. Elevated serum concentration of  dehydroepiandrosterone sulfate and testosterone might direct clinician to functioning adrenal cortical tissue as etiology, with further investigation of exact tumor site.

PMID: 30333277 [PubMed - in process]

Withholding Perioperative Steroids in Patients Undergoing Transsphenoidal Resection for Pituitary Disease: Randomized Prospective Clinical Trial to Assess Safety.

Tue, 10/23/2018 - 18:42
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Withholding Perioperative Steroids in Patients Undergoing Transsphenoidal Resection for Pituitary Disease: Randomized Prospective Clinical Trial to Assess Safety.

Neurosurgery. 2018 Oct 15;:

Authors: Sterl K, Thompson B, Goss CW, Dacey RG, Rich KM, Zipfel GJ, Chicoine MR, Kim AH, Silverstein JM

Abstract
BACKGROUND: Perioperative steroid protocols for patients undergoing transsphenoidal surgery (TSS) for pituitary pathology vary by institution.
OBJECTIVE: To assess the safety of withholding glucocorticoids in patients undergoing TSS.
METHODS: Patients with an intact hypothalamic-pituitary-adrenal (HPA) axis undergoing TSS for a pituitary tumor at the same academic institution between 2012 and 2015 were randomized to either receive 100 mg of intravenous hydrocortisone followed by 0.5 mg of intravenous dexamethasone every 6 h for 4 doses (STER, n = 23) or to undergo surgery without steroids (NOSTER, n = 20). Postoperative cortisol levels were then used to determine the need for glucocorticoids after surgery. Data regarding postoperative cortisol levels, hospital stay length, and complications were collected.
RESULTS: Mean postoperative 8 am cortisol levels were higher in the NOSTER group compared to the STER group (745 ± 359 nmol/L and 386 ± 193 nmol/L, respectively, P = .001) and more patients were discharged on glucocorticoids in the STER group (42% vs 12%, P = .07). There was no difference in the incidence of postoperative complications, including hyperglycemia, diabetes insipidus, or permanent adrenal insufficiency. Permanent adrenal insufficiency occurred in 8% of patients.
CONCLUSION: Perioperative steroids can be safely withheld in patients with an intact HPA axis undergoing TSS. Although administration of perioperative glucocorticoids does not appear to increase the risk of complications, it may interfere with assessment of the HPA axis after surgery.

PMID: 30325449 [PubMed - as supplied by publisher]

High-throughput gene expression analysis identifies p53-dependent and -independent pathways contributing to the adrenocortical dysplasia (acd) phenotype.

Tue, 10/23/2018 - 18:42
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High-throughput gene expression analysis identifies p53-dependent and -independent pathways contributing to the adrenocortical dysplasia (acd) phenotype.

Gene. 2018 Dec 30;679:219-231

Authors: Sucularli C, Thomas P, Kocak H, White JS, O'Connor BC, Keegan CE

Abstract
In mammalian cells TPP1, encoded by the Acd gene, is a key component of the shelterin complex, which is required for telomere length maintenance and telomere protection. In mice, a hypomorphic mutation in Acd causes the adrenocortical dysplasia (acd) phenotype, which includes limb and body axis anomalies, and perinatal lethality. p53 deficiency partially rescues limb and body axis anomalies in acd mutant embryos, but not perinatal lethality, implicating p53-independent mechanisms in the acd phenotype. Loss of function of most shelterin proteins results in early embryonic lethality. Thus, study of the hypomorphic acd allele provides a unique opportunity to understand telomere dysfunction at an organismal level. The aim of this study was to identify transcriptome alterations in acd mutant and acd, p53 double mutant embryos to understand the p53-dependent and -independent factors that contribute to the mutant phenotypes in the context of the whole organism. Genes involved in developmental processes, cell cycle, metabolic pathways, tight junctions, axon guidance and signaling pathways were regulated by p53-driven mechanisms in acd mutant embryos, while genes functioning in immune response, and RNA processing were altered independently of p53 in acd, p53 double mutant embryos. To our best of knowledge, this is the first study revealing detailed transcriptomic alterations, reflecting novel p53-dependent and -independent pathways contributing to the acd phenotype. Our data confirm the importance of cell cycle and DNA repair pathways, and suggest novel links between telomere dysfunction and immune system regulation and the splicing machinery. Given the broad applicability of telomere maintenance in growth, development, and genome stability, our data will also provide a rich resource for others studying telomere maintenance and DNA damage responses in mammalian model systems.

PMID: 30189268 [PubMed - indexed for MEDLINE]

Perioperative management during laparoscopic resection of large pheochromocytomas: A single-institution retrospective study.

Tue, 10/23/2018 - 18:42
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Perioperative management during laparoscopic resection of large pheochromocytomas: A single-institution retrospective study.

J Surg Oncol. 2018 Sep;118(4):709-715

Authors: Liu H, Li B, Yu X, Huang Y

Abstract
BACKGROUND AND OBJECTIVES: The safety of laparoscopic resection of very large pheochromocytomas is unclear, and preoperative preparation may benefit from refinement. This retrospective study was designed to determine the correlation between tumor size and perioperative characteristics by evaluating data from patients who underwent laparoscopic resection of pheochromocytomas.
METHODS: A total of 253 patients were grouped according to their tumor diameter: diameter ≥8 cm, Glarge (Glg ) (n = 30); diameter 6 to 8 cm, Gmedium (Gmd ) (n = 57); and diameter <6 cm, Gsmall (Gsm ) (n = 166). The perioperative data were compared among the three groups using the analysis of variance test, Wilcoxon's rank-sum test, and the chi-square test.
RESULTS: Patients in the Glg and Gmd groups had a higher preoperative phenoxybenzamine daily dose and 24-hour urine levels of noradrenaline and adrenaline than those in the Gsm group, as they needed longer vasopressor support. Transfusion of allogeneic blood products was found to be increased in patients of the Glg group compared with the other two groups and their intensive care unit stays were longer.
CONCLUSIONS: Compared with small-sized pheochromocytomas, laparoscopic resection of medium-sized pheochromocytomas requires vigilant monitoring and resolution of hemodynamic fluctuations. Patients with very large pheochromocytomas are at greater risk of intraoperative bleeding and may benefit from the ensured availability of blood products and intensive postoperative monitoring.

PMID: 30175399 [PubMed - indexed for MEDLINE]

Enzyme autoinduction by mitotane supported by population pharmacokinetic modelling in a large cohort of adrenocortical carcinoma patients.

Tue, 10/23/2018 - 18:42
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Enzyme autoinduction by mitotane supported by population pharmacokinetic modelling in a large cohort of adrenocortical carcinoma patients.

Eur J Endocrinol. 2018 10 16;179(5):287–297

Authors: Arshad U, Taubert M, Kurlbaum M, Frechen S, Herterich S, Megerle F, Hamacher S, Fassnacht M, Fuhr U, Kroiss M

Abstract
OBJECTIVE: Mitotane is used for the treatment of adrenocortical carcinoma. High oral daily doses of typically 1- 6 g are required to attain therapeutic concentrations. The drug has a narrow therapeutic index and patient management is difficult because of a high volume of distribution, very long elimination half-life, and drug interaction through induction of metabolizing enzymes. The present evaluation aimed at the development of a population pharmacokinetic model of mitotane to facilitate therapeutic drug monitoring.
METHODS: Appropriate dosing information, plasma concentrations (1137 data points) and covariates were available from therapeutic drug monitoring (TDM) of 76 adrenocortical carcinoma patients treated with mitotane. Using nonlinear mixed effects modeling, a simple structural model was first developed, with subsequent introduction of metabolic autoinduction. Covariate data were analyzed to improve overall model predictability. Simulations were performed to assess the attainment of therapeutic concentrations with clinical dosing schedules.
RESULTS: A one-compartment pharmacokinetic model with first order absorption was found suitable to describe the data, with an estimated central volume of distribution of 6086 L related to a high interindividual variability of 81.5%. Increase in clearance of mitotane during treatment could be modeled by a linear enzyme autoinduction process. Body mass index was found to have an influence upon disposition kinetics of mitotane. Model simulations favor a high dose regimen to rapidly attain therapeutic concentrations, with the first TDM suggested on day 16 of treatment to avoid systemic toxicity.
CONCLUSION: The proposed model describes mitotane pharmacokinetics and can be used to facilitate therapy by predicting plasma concentrations.

PMID: 30087117 [PubMed - indexed for MEDLINE]

Electrically conductive MEH-PPV:PCL electrospun nanofibres for electrical stimulation of rat PC12 pheochromocytoma cells.

Tue, 10/23/2018 - 18:42
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Electrically conductive MEH-PPV:PCL electrospun nanofibres for electrical stimulation of rat PC12 pheochromocytoma cells.

Biomater Sci. 2018 Aug 21;6(9):2342-2359

Authors: Borah R, Ingavle GC, Sandeman SR, Kumar A, Mikhalovsky S

Abstract
The purpose of this study was to prepare an electrically conducting poly[2-methoxy-5-(2-ethyl-hexyloxy)-1,4-phenylene vinylene] (MEH-PPV) based nanofibrous scaffold and to investigate the synergetic effect of nanofibre structure and electrical stimulation on neuronal growth for possible use in nerve repair. Nanofibres were produced by electrospinning of blended MEH-PPV with polycaprolactone (PCL) at a ratio of 20 : 80, 40 : 60, 50 : 50 and 60 : 40 (v/v). A better electrical conductivity was achieved by using core-sheath structured nanofibres of PCL (core) and MEH-PPV (sheath) produced using the coaxial electrospinning technique. The highest electrical conductivity was observed in the core-sheath nanofibres, while it increased with increasing concentration of MEH-PPV for the blended electrospun nanofibres. The biocompatibility of the electrospun nanofibres was confirmed by MTS and live-dead staining assays using 3T3 fibroblasts and a neuronal rat pheochromocytoma (PC12) cell line. Beta (III) tubulin immunochemistry showed that PC12 cells differentiated into sympathetic neurons on these porous and stiffer electrospun nanofibres coated with collagen I. Improved cell morphology and attachment on the collagen I coated electrospun meshes has been confirmed by SEM analysis. Significant enhancement in neurite formation and neurite outgrowth of PC12 cells on the conductive scaffolds under electrical potential of 500 mV cm-1 for 2 h day-1 suggests the potential use of these scaffolds for nerve repair.

PMID: 30019048 [PubMed - indexed for MEDLINE]

Multiples of Median-Transformed, Normalized Reference Ranges of Steroid Profiling Data Independent of Age, Sex, and Units.

Tue, 10/23/2018 - 18:42
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Multiples of Median-Transformed, Normalized Reference Ranges of Steroid Profiling Data Independent of Age, Sex, and Units.

Horm Res Paediatr. 2018;89(4):255-264

Authors: Zalas D, Reinehr T, Niedziela M, Borzikowsky C, Flader M, Simic-Schleicher G, Akkurt HI, Heger S, Hornig N, Holterhus PM, Kulle AE

Abstract
BACKGROUND/AIMS: The high complexity of pediatric reference ranges across age, sex, and units impairs clinical application and comparability of steroid hormone data, e.g., in congenital adrenal hyperplasia (CAH). We developed a multiples-of-median (MoM) normalization tool to overcome this major drawback in pediatric endocrinology.
METHODS: Liquid chromatography tandem mass spectrometry data comprising 10 steroid hormones representing 905 controls (555 males, 350 females, 0 to > 16 years) from 2 previous datasets were MoM transformed across age and sex. Twenty-three genetically proven CAH patients were included (21-hydroxylase deficiency [21OHD], n = 19; 11β-hydroxylase deficiency [11OHD], n = 4). MoM cutoffs for single steroids predicting 21OHD and 11OHD were computed and validated through new, independent patients (21OHD, n = 8; adrenal cortical carcinoma, n = 6; obesity, n = 40).
RESULTS: 21OHD and 11OHD patients showed disease-typical, easily recognizable MoM patterns independent of age, sex, and concentration units. Two single-steroid cutoffs indicated 21OHD: 3.87 MoM for 17-hydroxyprogesterone (100% sensitivity and 98.83% specificity) and 12.28 MoM for 21-deoxycortisol (94.74% sensitivity and 100% specificity). A cutoff of 13.18 MoM for 11-deoxycortisol indicated 11OHD (100% sensitivity and 100% specificity).
CONCLUSIONS: Age- and sex-independent MoMs are straightforward for a clinically relevant display of multi-steroid patterns. In addition, defined single-steroid MoMs can serve alone as predictors of 21OHD and 11OHD. Finally, MoM transformation offers substantial enhancement of routine and scientific steroid hormone data exchange due to improved comparability.

PMID: 29694956 [PubMed - indexed for MEDLINE]

A novel pipeline for adrenal tumour segmentation.

Tue, 10/23/2018 - 18:42
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A novel pipeline for adrenal tumour segmentation.

Comput Methods Programs Biomed. 2018 Jun;159:77-86

Authors: Koyuncu H, Ceylan R, Erdogan H, Sivri M

Abstract
BACKGROUND AND OBJECTIVE: Adrenal tumours occur on adrenal glands surrounded by organs and osteoid. These tumours can be categorized as either functional, non-functional, malign, or benign. Depending on their appearance in the abdomen, adrenal tumours can arise from one adrenal gland (unilateral) or from both adrenal glands (bilateral) and can connect with other organs, including the liver, spleen, pancreas, etc. This connection phenomenon constitutes the most important handicap against adrenal tumour segmentation. Size change, variety of shape, diverse location, and low contrast (similar grey values between the various tissues) are other disadvantages compounding segmentation difficulty. Few studies have considered adrenal tumour segmentation, and no significant improvement has been achieved for unilateral, bilateral, adherent, or noncohesive tumour segmentation. There is also no recognised segmentation pipeline or method for adrenal tumours including different shape, size, or location information.
METHODS: This study proposes an adrenal tumour segmentation (ATUS) pipeline designed to eliminate the above disadvantages for adrenal tumour segmentation. ATUS incorporates a number of image methods, including contrast limited adaptive histogram equalization, split and merge based on quadtree decomposition, mean shift segmentation, large grey level eliminator, and region growing.
RESULTS: Performance assessment of ATUS was realised on 32 arterial and portal phase computed tomography images using six metrics: dice, jaccard, sensitivity, specificity, accuracy, and structural similarity index. ATUS achieved remarkable segmentation performance, and was not affected by the discussed handicaps, on particularly adherence to other organs, with success rates of 83.06%, 71.44%, 86.44%, 99.66%, 99.43%, and 98.51% for the metrics, respectively, for images including sufficient contrast uptake.
CONCLUSIONS: The proposed ATUS system realises detailed adrenal tumour segmentation, and avoids known disadvantages preventing accurate segmentation.

PMID: 29650321 [PubMed - indexed for MEDLINE]

[Bronchial adenoid cystic carcinoma masquerading as bronchial asthma: a case report].

Tue, 10/23/2018 - 18:42
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[Bronchial adenoid cystic carcinoma masquerading as bronchial asthma: a case report].

Beijing Da Xue Xue Bao Yi Xue Ban. 2018 Apr 18;50(2):378-380

Authors: Lu M, Wang M, Zhu X, Chen YH, Yao WZ

Abstract
Here we reported a case of bronchial adenoid cystic carcinoma from Peking University Third Hospital. A 40-year-old female presented with dry cough for 1 year and nocturnal paroxysmal attacks of wheezing for 4 months. She was a non-smoker, and did not have past histories of asthma or allergy. On physical examination, no stridor, wheezing and cyanosis were present and the general appearance was good. The results of the laboratory analysis, including blood eosinophils count, immunoglobulin E level and chest X-ray were normal. Spirometry revealed reversible airflow obstruction, and post-bronchodilator forced expiratory volume in one second (FEV1) showed an increase of 12% and 230 mL from baseline. Bronchial asthma was diagnosed, however, she responded poorly despite the adequate anti-asthma therapy including high dose inhaled corticosteroid plus long-acting beta2-agonist, theophylline and montelukast. Then chest computed tomography (CT) was performed which showed a polypoid mass occupying the lumen of left main bronchus. Then the bronchoscopy revealed a polypoid endo-bronchial mass arising from the left main bronchus, causing subtotal obstruction of the lumen. Biopsy was carried out through the bronchoscopy, the pathological findings showed characteristic cribriform and tubular pattern which was formed by two-layered cells with ductal and myoepithelial phenotypes, which were consistent with adenoid cystic carcinoma. Re-examining the patient, the lung was clear without any wheeze when she was seated. However, inspiratory wheeze was heard in her left upper lung when she was supine, and disappeared after sitting up again. Subsequently the patient underwent a resection surgery. At the operational site, the tumor was seen on the anterolateral wall of the left main bronchus, without submucosally expanding histologically. Therefore, a sleeve resection surgery of the left main bronchus was performed. Following surgery, chest CT scan revealed complete resolution of the tumor. Her symptoms improved significantly, as did her pulmonary function tests, although all the medicines for asthma were stopped. Now, two years after the operation, the patient remained asymptomatic, and spirometry was performed again which showed normal completely. The presenting case report emphasizes the fact that not all wheezes and reversible airflow obstruction are asthma. It is critical to bear in mind that if a "difficult asthma" patient does not respond to appropriate anti-asthma therapy; localized obstructions should be differentiated.

PMID: 29643544 [PubMed - indexed for MEDLINE]

Serum Cortisol Levels via Radioimmunoassay vs Liquid Chromatography Mass Spectrophotometry in Healthy Control Subjects and Patients With Adrenal Incidentalomas.

Tue, 10/23/2018 - 18:42
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Serum Cortisol Levels via Radioimmunoassay vs Liquid Chromatography Mass Spectrophotometry in Healthy Control Subjects and Patients With Adrenal Incidentalomas.

Lab Med. 2018 Jul 05;49(3):259-267

Authors: Huayllas MKP, Netzel BC, Singh RJ, Kater CE

Abstract
Background: Adrenal incidentalomas (AIs) are present in 4% of adults. As many as 30% may secrete cortisol autonomously in the absence of specific signs of overt hypercortisolism, in a phenomenon called subclinical hypercortisolism (SH). Diagnosis of SH is established by serum cortisol resistance to dexamethasone suppression.
Methods: We compared serum cortisol concentrations, as determined by radioimmunoassay (RIA) and liquid chromatography/tandem mass spectronomy (LC/MS-MS), in 73 patients with AI group (52 with unilateral AI) and 34 control subjects in 3 scenarios: basal; after 1-mg dexamethasone suppression; and after 0.25-mg stimulation with cosyntropin, a synthetic derivative of adrenocorticotropic hormone (ACTH). To bolster evidence for the diagnosis of SH, we also measured salivary cortisol levels at 11 PM and after DST, as well as plasma ACTH and serum dehydroepiandrosterone sulfate (DHEA-S) levels.
Results: We observed significant positive correlation (r = 0.9345, P <.001) for all 318 pairs of serum cortisol values, as measured by both methods.
Conclusions: Serum cortisol concentrations in patients with AI and in control subjects were very similar, as measured by RIA and LC/MS-MS.

PMID: 29608696 [PubMed - indexed for MEDLINE]

A rare cause of chronic ataxia in childhood: ganglioneuroma.

Tue, 10/23/2018 - 18:42
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A rare cause of chronic ataxia in childhood: ganglioneuroma.

World J Pediatr. 2018 04;14(2):204-206

Authors: Arslan EA, Kamaşak T, Turgut BD, Saygın İ, Sarıhan H, Cansu A

PMID: 29532434 [PubMed - indexed for MEDLINE]

The penetrance of MEN2 pheochromocytoma is not only determined by RET mutations.

Tue, 10/23/2018 - 18:42
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The penetrance of MEN2 pheochromocytoma is not only determined by RET mutations.

Endocr Relat Cancer. 2017 08;24(8):L63-L67

Authors: Castinetti F, Maia AL, Peczkowska M, Barontini M, Hasse-Lazar K, Links TP, Toledo RA, Dvorakova S, Mian C, Bugalho MJ, Zovato S, Alevizaki M, Kvachenyuk A, Bausch B, Loli P, Bergmann SR, Patocs A, Pfeifer M, Costa JB, von Dobschuetz E, Letizia C, Valk G, Barczynski M, Czetwertynska M, Plukker JTM, Sartorato P, Zelinka T, Vlcek P, Yaremchuk S, Weryha G, Canu L, Wohllk N, Sebag F, Walz MK, Eng C, Neumann HPH

PMID: 28649091 [PubMed - indexed for MEDLINE]

SDHB mutation carriers with malignant pheochromocytoma respond better to CVD.

Tue, 10/23/2018 - 18:42
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SDHB mutation carriers with malignant pheochromocytoma respond better to CVD.

Endocr Relat Cancer. 2017 08;24(8):L51-L55

Authors: Fishbein L, Ben-Maimon S, Keefe S, Cengel K, Pryma DA, Loaiza-Bonilla A, Fraker DL, Nathanson KL, Cohen DL

PMID: 28566531 [PubMed - indexed for MEDLINE]

KIF1B and NF1 are the most frequently mutated genes in paraganglioma and pheochromocytoma tumors.

Tue, 10/23/2018 - 18:42
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KIF1B and NF1 are the most frequently mutated genes in paraganglioma and pheochromocytoma tumors.

Endocr Relat Cancer. 2017 08;24(8):L57-L61

Authors: Evenepoel L, Helaers R, Vroonen L, Aydin S, Hamoir M, Maiter D, Vikkula M, Persu A

PMID: 28515046 [PubMed - indexed for MEDLINE]

StatPearls

Tue, 10/23/2018 - 18:42
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StatPearls

Book. 2018 01

Authors:

Abstract
In 1969, a remarkable cancer predisposition syndrome was reported by Li and Fraumeni.[1] Li-Fraumeni syndrome (LFS) is an inherited autosomal dominant disorder that is usually associated with abnormalities in the tumor suppressor protein P53 gene (TP53) located on chromosome 17p13.[2] It is also known as the sarcoma, breast, leukemia, and adrenal gland (SBLA) cancer syndrome. LFS variants include LFS1, LFS2, LFSL. LFS1 is associated with mutations in TP53, tumor suppressor gene. LFS2 is associated with mutations in CHEK2 (checkpoint kinase two), a tumor suppressor gene. LFS-L  are individuals that do not have detectable mutations in P53.


PMID: 30335319

adrenal tumor; +23 new citations

Tue, 10/16/2018 - 18:02

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 2018/10/16

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; +32 new citations

Tue, 10/09/2018 - 17:52

32 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 2018/10/09

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.

Robotic Adrenalectomy: Are We Expanding the Indications of Minimally Invasive Surgery?

Tue, 10/02/2018 - 15:14
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Robotic Adrenalectomy: Are We Expanding the Indications of Minimally Invasive Surgery?

J Laparoendosc Adv Surg Tech A. 2018 Sep 28;:

Authors: Quadri P, Esposito S, Coleoglou A, Danielson KK, Masrur M, Giulianotti PC

Abstract
INTRODUCTION: Laparoscopic adrenalectomy (LA) is accepted as the gold standard treatment for most adrenal pathologies. Open surgery is still considered the standard of care for large tumors and malignancies. In the past decade, robotic adrenalectomy (RA) has become an alternative to the laparoscopic and open approaches. The aim of this study was to analyze perioperative and postoperative outcomes in a series of consecutive nonselected patients undergoing a RA, to determine whether factors that negatively affect outcomes in LA (body mass index [BMI], size, and side of the tumor) have the same impact in RA.
MATERIALS AND METHODS: This is a single-center single-surgeon retrospective study with 43 patients who underwent a RA. Patients were divided into different groups according to tumor size (cutoff values of 5 or 8 cm), tumor side (left/right), and BMI (cutoff value of kg/m2). Perioperative and postoperative outcomes included operative time, length of hospital stay, blood loss, readmissions, complications, and conversions to open.
RESULTS: There were no significant differences between the groups with tumors <5 cm versus ≥5 cm regarding gender, age, race, BMI, American Society of Anesthesiologists (ASA) score, history of previous abdominal surgery, tumor side, and histopathological diagnosis (all P values ≥.06). There were no significant differences in any of the outcomes analyzed with respect to the tumor size (all P values ≥.14) except for a higher occurrence of complications in patients with tumors ≥8 cm versus <8 cm (P = .03). There were no significant differences in any outcomes related to side (left versus right) of the tumor nor BMI (<30 versus ≥30 kg/m2). The overall readmission and conversion rates were both 2.3% and no mortalities were registered.
CONCLUSION: Patient's BMI, tumor side, and size did not demonstrate a negative impact on perioperative and postoperative outcomes of RA. This approach could potentially expand the indications of minimally invasive surgery.

PMID: 30265584 [PubMed - as supplied by publisher]

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