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adrenal tumor; +22 new citations

Mon, 05/14/2018 - 10:28

22 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 2018/05/14

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.

Root cause analysis of diagnostic and surgical failures in the treatment of suspected Cushing's disease.

Mon, 05/07/2018 - 10:19
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Root cause analysis of diagnostic and surgical failures in the treatment of suspected Cushing's disease.

J Clin Neurosci. 2018 Apr 28;:

Authors: Zaidi HA, Penn DL, Cote DJ, Laws ER

Abstract
Cushing's disease (CD) is a condition characterized by excess glucocorticoid from an ACTH pituitary adenoma. Identifying surgical candidates represents a major diagnostic challenge. We performed a root cause analysis (RCA) of treatment failures for patients with suspected CD. The present study aims to categorize failures in treatment. Medical records were reviewed from 2008 to 2017 for all patients treated surgically for suspected CD. Demographics, past medical history, endocrine outcomes, imaging findings, laboratory studies and clinical features were collected. Eighty-five patients were identified with pre-operative suspicion for CD. Thirty-four (40.0%) had undergone prior surgery confirming ACTH adenoma, leaving 51 (60.0%) for analysis. The average length of follow-up was 18.3 ± 24.1 months, 42 (82.4%) patients had postoperative biochemical remission of hypercortisolism. Forty-three (84.3%) had histologically confirmed CD, two (3.9%) were diagnosed with extracranial ACTH-secreting tumors, four (7.8%) had no obvious tumor upon intraoperative exploration, one (1.9%) had suspected pituitary ACTH hyperplasia, and one (1.9%) had no identifiable pathologic tissue despite apparent gross tumor observed during surgery. Thirty-four (66.7%) patients had remission following surgery alone, four (7.8%) after reoperation, and four (7.81%) after radiosurgery. One patient (1.9%) was found to have an ectopic source of ACTH, and one (1.9%) had immunohistochemically confirmed adrenal tumors. On RCA, we identified six categories of treatment failures. CD is a diagnostic challenge that can be difficult to distinguish from other forms of hypercortisolism. Surgical efficacy can be improved with more accurate patient selection, and perhaps with improved imaging methods.

PMID: 29716804 [PubMed - as supplied by publisher]

The difficulties of pseudo-Cushing's syndrome (or "non-neoplastic hypercortisolism").

Mon, 05/07/2018 - 10:19
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The difficulties of pseudo-Cushing's syndrome (or "non-neoplastic hypercortisolism").

Ann Endocrinol (Paris). 2018 Apr 28;:

Authors: Chabre O

Abstract
Pseudo-Cushing's syndrome covers different pathological conditions responsible for mild-to-moderate ACTH-dependent hypercortisolism, related not to an ACTH-secreting tumor but rather to CRH and/or AVP hypothalamic secretion through activation of various neural pathways, in patients generally displaying excess central adiposity. It is better termed "non-neoplastic hypercortisolism" (NNH). The main conditions implicated in NNH comprise: neuropsychiatric disorder, alcohol abuse, insulin-resistant obesity, polycystic ovary syndrome, and end-stage kidney disease. Glucocorticoid resistance is one differential diagnosis, as are some cases of primary adrenal disease with incompletely suppressed ACTH. Differentiating between NNH and mild-to-moderate Cushing's disease can be a real challenge. Clinical analysis, based on thorough history taking and screening for catabolic signs is essential; useful explorations include midnight serum or salivary cortisol and Dex/CRH and ddAVP stimulation response. Pituitary MRI suffers from limitations regarding both sensitivity and specificity, while bilateral inferior petrosal sinus sampling cannot distinguish between pituitary ACTH secretion by a tumor or by normal cells stimulated by endogenous CRH. Definitive diagnosis of functional etiology requires demonstrating that treatment of the underlying condition restores normal secretion of ACTH and cortisol, but this is not always possible. Lingering diagnostic uncertainty has to be accepted in certain patients, who will have to be followed up for some time before diagnosis can be considered more or less definitive.

PMID: 29716734 [PubMed - as supplied by publisher]

Clinical Investigation of Adrenal Incidentalomas in Japanese Patients of the Fukuoka Region with Updated Diagnostic Criteria for Sub-clinical Cushing's Syndrome.

Mon, 05/07/2018 - 10:19
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Clinical Investigation of Adrenal Incidentalomas in Japanese Patients of the Fukuoka Region with Updated Diagnostic Criteria for Sub-clinical Cushing's Syndrome.

Intern Med. 2018 Apr 27;:

Authors: Abe I, Sugimoto K, Miyajima T, Ide T, Minezaki M, Takeshita K, Takahara S, Nakagawa M, Fujimura Y, Kudo T, Miyajima S, Taira H, Ohe K, Ishii T, Yanase T, Kobayashi K

Abstract
Objectives We retrospectively investigated the clinical and endocrinological characteristics of adrenal incidentalomas. Methods We studied 61 patients who had been diagnosed with adrenal incidentalomas and had undergone detailed clinical and endocrinological evaluations while hospitalized. We used common criteria to diagnose the functional tumors, but for sub-clinical Cushing's syndrome, we used an updated set of diagnosis criteria: serum cortisol ≥1.8 μg/dL after a positive response to a 1-mg dexamethasone suppression test if the patient has a low morning ACTH level (<10 pg/mL) and a loss of the diurnal serum cortisol rhythm. Results Of the 61 patients, none (0%) had malignant tumors, 8 (13.1%) had pheochromocytoma, and 15 (24.6%) had primary aldosteronism; when diagnosed by our revised criteria, 13 (21.3%) had cortisol-secreting adenomas (Cushing's syndrome and sub-clinical Cushing's syndrome), and 25 (41.0%) had non-functional tumors. Compared with the non-functional tumor group, the primary aldosteronism group and the cortisol-secreting adenoma group were significantly younger and had significantly lower rates of hypokalemia, whereas the pheochromocytoma group had significantly larger tumors and a significantly lower body mass index. Conclusion Our study found a larger percentage of functional tumors among adrenal incidentalomas than past reports, partly because we used a lower serum cortisol level after a dexamethasone suppression test to diagnose sub-clinical Cushing's syndrome and because all of the patients were hospitalized and could therefore receive more detailed examinations. Young patients with hypokalemia or lean patients with large adrenal tumors warrant particularly careful investigation.

PMID: 29709936 [PubMed - as supplied by publisher]

Primary cervical ganglioneuroblastoma: A case report.

Mon, 05/07/2018 - 10:19
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Primary cervical ganglioneuroblastoma: A case report.

Medicine (Baltimore). 2018 Mar;97(12):e0090

Authors: Lu D, Liu J, Chen Y, Chen F, Yang H

Abstract
RATIONALE: Ganglioneuroblastoma is usually located in the adrenal gland, retroperitoneal ganglia, or posterior mediastinum, but rarely occurs in the parapharyngeal space.
PATIENT CONCERNS: A 4-year-old girl presented with complaint of progressive inspiratory dyspnea and dysphagia, accompanying left-side Horner's syndrome.
DIAGNOSE: Computed tomography (CT) scan revealed a giant mass with irregular low density in left oropharyngeal and posterior pharyngeal wall. The left carotid artery sheath was pushed to the right. After enhancement, the central part of the mass was strengthened, and the surrounding bones structures appeard normal. Magnetic resonance imaging (MRI) showed a solid mass in the left parapharyngeal space displacing the left carotid sheath posteriorly and laterally. A ganglioneuroblastoma was diagnosed.
INTERVATIONS: The girl was treated by surgery.
OUTCOMES: The postoperative course was uneventful. There was no recurrence was observed during the 1-year follow-up.
LESSONS: The primary cervical ganglioneuroblastoma is rare, we recommended the ganglioneuroblastoma should be considered in the differential diagnosis of a child presenting with a parapharyngeal space mass.

PMID: 29561407 [PubMed - indexed for MEDLINE]

Cardiovascular features of possible autonomous cortisol secretion in patients with adrenal incidentalomas.

Mon, 05/07/2018 - 10:19
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Cardiovascular features of possible autonomous cortisol secretion in patients with adrenal incidentalomas.

Eur J Endocrinol. 2018 May;178(5):501-511

Authors: Sbardella E, Minnetti M, D'Aluisio D, Rizza L, Di Giorgio MR, Vinci F, Pofi R, Giannetta E, Venneri MA, Vestri A, Morelli S, Lenzi A, Isidori AM

Abstract
BACKGROUND: Low-grade incomplete post-dexamethasone cortisol suppression in patients with adrenal incidentalomas - recently defined as possible autonomous cortisol secretion (pACS) - has been associated with increased cardiovascular events and mortality. However, prospective studies documenting cardiac abnormalities in these patients are lacking.
SUBJECTS AND METHODS: Between July 2016 and September 2017, 71 consecutive patients with adrenal lesions were prospectively screened for hypercortisolism by dexamethasone suppression test (NCT 02611258). Complete anthropometric, metabolic and hormonal parameters were recorded along with full cardiac ultrasound assessment and noninvasive measurement of arterial stiffness. All patients underwent chemical-shift magnetic resonance imaging to characterize the lesions. Cardiovascular outcomes were recorded in blind.
RESULTS: According to post-dexamethasone suppression cortisol values (post-DST), 34 patients had pACS and 37 non-functioning adenomas (NFA). The two groups were similar in sex, BMI, age distribution, cardiovascular risk factors and comorbidities. Left ventricular mass index (LVMIBSA) was increased in pACS compared to NFA (P = 0.006) and mildly correlated to the post-DST cortisol level (rho = 0.347; P = 0.004). The post-DST cortisol levels explained up to 13.7% of LVMIBSA variance (P = 0.002). Compared to NFA, patients with pACS had a higher prevalence of diastolic dysfunction (35.1% vs 82.6%; P = 0.001) and worse arterial stiffness assessed by pulse wave velocity (P = 0.033).
CONCLUSIONS: In apparently asymptomatic patients, mild autonomous cortisol secretion can sustain early cardiac and vascular remodeling, independently of other risk factors. The morphological and functional cardiovascular changes observed in pACS underline the need for further studies to correctly define the long-term management of this relatively common condition.

PMID: 29510982 [PubMed - indexed for MEDLINE]

Diagnostic accuracy of computed tomography to identify adenomas among adrenal incidentalomas in an endocrinological population.

Mon, 05/07/2018 - 10:19
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Diagnostic accuracy of computed tomography to identify adenomas among adrenal incidentalomas in an endocrinological population.

Eur J Endocrinol. 2018 May;178(5):439-446

Authors: Marty M, Gaye D, Perez P, Auder C, Nunes ML, Ferriere A, Haissaguerre M, Tabarin A

Abstract
CONTEXT: The recent recommendations of the European Endocrine Society states that the performance of computed tomography (CT) to characterize 'true' adrenal incidentalomas (AIs) remains debatable.
OBJECTIVE: To determine relevant thresholds for usual CT parameters for the diagnosis of benign tumors using robust reference standard among a large series of 'true' AIs recruited in an endocrinological setting.
DESIGN: Retrospective study of 253 AIs in 233 consecutive patients explored in a single university hospital: 183 adenomas, 33 pheochromocytomas, 23 adrenocortical carcinomas, 5 other malignant tumors and 9 other benign tumors. Reference standard was histopathology in 118 AIs, biological diagnosis of pheochromocytoma in 2 AIs and size stability after at least 1 year of follow-up in 133 AIs.
METHODS: Sensitivity, specificity and positive and negative predictive values were estimated for various thresholds of size, unenhanced attenuation (UA), relative and absolute wash-out (RPW, APW) of contrast media. 197 scans were reviewed independently in a blinded fashion by two expert radiologists to assess inter-observer reproducibility of measurements.
RESULTS: Criteria associated with a 100% positive predictive value for the diagnosis of benign AI were: a combination of size and UA: 30 mm and 20 HU or 40 mm and 15 HU, respectively; RPW >53%; and APW >78%. Non-adenomatous AIs with rapid contrast wash-out were exclusively benign pseudocysts and pheochromocytomas, suggesting that classical thresholds of 60% and 40% for APW and RPW, respectively, can be safely used for patients with normal metanephrine values. Inter-observer reproducibility of all parameters was excellent (intra-class correlation coefficients: 0.96-0.99).
CONCLUSIONS: Our study, the largest conducted in AIs recruited in an endocrinological setting, suggests safe thresholds for quantitative CT parameters to avoid false diagnoses of benignity.

PMID: 29467231 [PubMed - indexed for MEDLINE]

Calling Chromosome Alterations, DNA Methylation Statuses, and Mutations in Tumors by Simple Targeted Next-Generation Sequencing: A Solution for Transferring Integrated Pangenomic Studies into Routine Practice?

Mon, 05/07/2018 - 10:19
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Calling Chromosome Alterations, DNA Methylation Statuses, and Mutations in Tumors by Simple Targeted Next-Generation Sequencing: A Solution for Transferring Integrated Pangenomic Studies into Routine Practice?

J Mol Diagn. 2017 Sep;19(5):776-787

Authors: Garinet S, Néou M, de La Villéon B, Faillot S, Sakat J, Da Fonseca JP, Jouinot A, Le Tourneau C, Kamal M, Luscap-Rondof W, Boeva V, Gaujoux S, Vidaud M, Pasmant E, Letourneur F, Bertherat J, Assié G

Abstract
Pangenomic studies identified distinct molecular classes for many cancers, with major clinical applications. However, routine use requires cost-effective assays. We assessed whether targeted next-generation sequencing (NGS) could call chromosomal alterations and DNA methylation status. A training set of 77 tumors and a validation set of 449 (43 tumor types) were analyzed by targeted NGS and single-nucleotide polymorphism (SNP) arrays. Thirty-two tumors were analyzed by NGS after bisulfite conversion, and compared to methylation array or methylation-specific multiplex ligation-dependent probe amplification. Considering allelic ratios, correlation was strong between targeted NGS and SNP arrays (r = 0.88). In contrast, considering DNA copy number, for variations of one DNA copy, correlation was weaker between read counts and SNP array (r = 0.49). Thus, we generated TARGOMICs, optimized for detecting chromosome alterations by combining allelic ratios and read counts generated by targeted NGS. Sensitivity for calling normal, lost, and gained chromosomes was 89%, 72%, and 31%, respectively. Specificity was 81%, 93%, and 98%, respectively. These results were confirmed in the validation set. Finally, TARGOMICs could efficiently align and compute proportions of methylated cytosines from bisulfite-converted DNA from targeted NGS. In conclusion, beyond calling mutations, targeted NGS efficiently calls chromosome alterations and methylation status in tumors. A single run and minor design/protocol adaptations are sufficient. Optimizing targeted NGS should expand translation of genomics to clinical routine.

PMID: 28826610 [PubMed - indexed for MEDLINE]

Treatment of Unicameral Bone Cysts of the Proximal Femur With Internal Fixation Lessens the Risk of Additional Surgery.

Mon, 05/07/2018 - 10:19
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Treatment of Unicameral Bone Cysts of the Proximal Femur With Internal Fixation Lessens the Risk of Additional Surgery.

Orthopedics. 2017 Sep 01;40(5):e862-e867

Authors: Wilke B, Houdek M, Rao RR, Caird MS, Larson AN, Milbrandt T

Abstract
Little data exist to guide the treatment of unicameral bone cysts in the proximal femur. Methods of treatment include corticosteroid injections, curettage and bone grafting, and internal fixation. The authors completed a multi-institutional, retrospective review to evaluate their experience with proximal femoral unicameral bone cysts. They posed the following questions: (1) Does internal fixation reduce the risk of further procedures for the treatment of a unicameral bone cyst? (2) Is radiographic healing faster with internal fixation? Following institutional review board approval, the authors conducted a retrospective review of 36 patients treated for a unicameral bone cyst of the proximal femur at their institutions between 1974 and 2014. Medical records and radiographs were reviewed to identify patient demographics and treatment outcomes. Tumor locations included femoral neck (n=13), intertrochanteric (n=16), and subtrochanteric (n=7). Initial treatment included steroid injection (n=2), curettage and bone grafting (n=9), and internal fixation with curettage and bone grafting (n=25). Mean time was 9 months to radiographic healing and 15 months to return to full activity. The number of patients requiring additional surgeries was increased among those who did not undergo internal fixation. There was no difference in time to radiographic healing. However, time to return to normal activities was reduced if patients had received internal fixation. A significant reduction in additional procedures was observed when patients had been treated with internal fixation. Although this did not influence time to radiographic healing, patients did return to normal activities sooner. Internal fixation should be considered in the treatment of proximal femoral unicameral bone cysts. [Orthopedics. 2017; 40(5):e862-e867.].

PMID: 28817159 [PubMed - indexed for MEDLINE]

Adrenal myelolipoma(s) as presenting manifestation of subclinical Cushing's disease (eutopic ACTH-dependent Cushing's syndrome).

Mon, 05/07/2018 - 10:19
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Adrenal myelolipoma(s) as presenting manifestation of subclinical Cushing's disease (eutopic ACTH-dependent Cushing's syndrome).

BMJ Case Rep. 2017 Aug 16;2017:

Authors: Chakraborty PP, Patra S, Biswas SN, Bhattacharjee R

Abstract
Primary adrenal myelolipomas, relatively rare benign tumours of the adrenal cortex are typically unilateral, hormonally inactive and asymptomatic, hence often diagnosed as 'adrenal incidentaloma'. Bilateral adrenal myelolipomas, in particular, may be associated with underlying endocrinopathies associated with elevated circulating adrenocorticotropic hormone (ACTH) concentration. Subclinical cortisol hypersecretion, irrespective of its ACTH dependency, does not manifest typical clinical phenotype of hypercortisolemia, and thus termed subclinical Cushing's syndrome. In this article, hormonal evaluation in a middle-aged woman with diabetes, hypertension and incidentally discovered unilateral adrenal myelolipoma revealed underlying subclinical Cushing's disease. Abdominal CT revealed another tiny focus in the contralateral adrenal gland, probably representing incipient myelolipoma.

PMID: 28814582 [PubMed - indexed for MEDLINE]

Central hypoadrenocorticism associated with Rathke's cleft cyst.

Mon, 05/07/2018 - 10:19
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Central hypoadrenocorticism associated with Rathke's cleft cyst.

Neuro Endocrinol Lett. 2017 Jul;38(3):141-144

Authors: Kawano F, Itonaga T, Inoue M, Maeda M, Miyahara H, Ihara K

Abstract
Rathke's cleft cysts (RCCs) are non-neoplastic, sellar or suprasellar epithelium-lined cysts originating from Rathke's pouch in the pituitary gland. Patients with RCCs are usually asymptomatic, but some have only been identified when symptoms manifested in middle age. The characteristics of these patients during childhood or adolescence remains unknown. We describe an 18-year-old girl who had occasionally suffered from malicious fatigue in the morning since her early teens. Brain magnetic resonance imaging (MRI) revealed T1 hyperintense/T2 hypointense signals between the anterior and posterior pituitary glands, indicating the presence of RCC. Based on an authentic endocrinological evaluation, her adrenal function seemed normal; nevertheless, her serum cortisol level strangely dropped around noon. Furthermore, daily supplementation of oral hydrocortisone bizarrely suppressed ACTH secretion to below the detection range in the morning. These data appeared compatible with the presence of central adrenal dysfunction. We also review the literature for previously reported cases. In conclusion, the symptoms and endocrinological data for dysfunction of the hypothalamic pituitary system might be non-specific and vary among patients, especially in teenagers. Brain MRI and daily cortisol profiling in blood are key to obtaining a diagnosis of an impaired hypothalamic adrenal function due to RCC.

PMID: 28759180 [PubMed - indexed for MEDLINE]

[Adrenal surgery : Multidisciplinarity is mandatory].

Mon, 05/07/2018 - 10:19
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[Adrenal surgery : Multidisciplinarity is mandatory].

Rev Med Suisse. 2017 Jun 14;13(567):1253-1257

Authors: Matthey Gié ML, Nicod Lalonde M, Gonzalez Rodriguez E, Demartines N, Matter M

Abstract
Adrenal tumours are rare and their management is challenging. Every patient presenting with adrenal mass or symptoms of hormones hypersecretion should be investigated. The two important questions to be answered are : 1. Is the tumour secreting ? Is the tumour malignant or not ? A complete endocrine work-up and a nativ CT-scan may usually answer these two questions but have to be interpreted by specialists in a multidisciplinary team setting. The decisions about managements of adrenal pathologies do follow international guidelines which are regularly updated. It is not recommended to perform diagnostic or surgical procedure before excluding pheochromocytoma or adrenocortical carcinoma which could have fatal outcome for the patient. Every adrenal mass has to be investigated and should not be made commonplace.

PMID: 28643981 [PubMed - indexed for MEDLINE]

Incidental adrenal masses - A primary care approach.

Mon, 05/07/2018 - 10:19
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Incidental adrenal masses - A primary care approach.

Aust Fam Physician. 2017 Jun;46(6):385-390

Authors: Gendy R, Rashid P

Abstract
BACKGROUND: The common use of cross-sectional imaging for the investigation of abdominal and thoracic illness has resulted in the rise of the incidentally identified adrenal mass, or incidentaloma, which presents a diagnostic and management dilemma for the primary care physician.
OBJECTIVE: This article provides a framework for the investigation and management of incidental adrenal masses.
DISCUSSION: Adrenal incidentalomas are found in approximately 3-4% of abdominal computed tomography (CT) scans. It is important to evaluate these incidental adrenal lesions to determine what treatment, if any, is needed and when specialist referral may be necessary. In particular, incidentalomas must be evaluated in regard to their functional status and malignant potential, as lesions can range from being indolent, benign and non-functioning tumours that can simply be observed, to aggressive and hormonally active malignant lesions that require urgent surgical intervention.

PMID: 28609594 [PubMed - indexed for MEDLINE]

[Subclinical Pheochromocytoma: The Surgeons Point fo View - A Single-Institution Experience].

Mon, 05/07/2018 - 10:19
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[Subclinical Pheochromocytoma: The Surgeons Point fo View - A Single-Institution Experience].

Khirurgiia (Sofiia). 2016;82(1):24-30

Authors: Vidinov K, Ganchev G, Stoinov U

Abstract
Background: Pheochromocytoma is a rare neuroendocrine tumor. Most of the autopsy studies indicate that large numbers of these tumors remain undiagnosed during life. The most obvious explanation for this fact is its rather non-specific clinical presentation or the absence of any symptoms - the presence of subclinical pheochromocytoma. We report our institution experience with silent pehochromocytomas - their preoperative characteristics, intraoperative management and postoperative complications.
Material and Methods: A total of 104 patients underwent operation for pheochromocytoma between 19780 and 2010 in our department. Eleven of them had no clinical symptoms. We describe and analyze retrospectively: indication for diagnosis and surgery, length of hospital stay, intra and postoperative complications.
Results: In total, 11 of the 104 patent operated in our department had no clinical symptoms. From the classical triad (headache, palpitation and diaphoresis) only five of those patients had occasional palpitation when they felt severe anger, which is rather unspecific. When we analyzed the data of these patients we found a longer operative time (p = 0.04), more intraoperative complication (p = 0.02) - higher estimated blood loss and larger transfusion requirement. Postoperative complications however showed no statistical significance.
Conclusion: Pheochromocytoma can have variable presentation. Patients with small incidentalomas schedules for surgery should be closely evaluated and medically treated preoperatively to ensure an excellent outcome.

PMID: 29383900 [PubMed - indexed for MEDLINE]

adrenal tumor; +31 new citations

Mon, 04/30/2018 - 07:33

31 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 2018/04/30

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.

[Role of PET/CT in primitive non-small cell bronchopulmonary cancer].

Mon, 04/23/2018 - 07:24
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[Role of PET/CT in primitive non-small cell bronchopulmonary cancer].

Pan Afr Med J. 2017;28:289

Authors: Soumia F, Leila A, Mohamed R, Laila H, Mustapha E

Abstract
Bronchopulmonary cancer is a real public health problem. Morphological imaging plays a central role in its diagnosis, staging as well as post-therapeutic assessment but it has some limitations. Metabolic imaging is a more recent technique which allows to significantly improve the overall imagery performance. We conducted a retrospective, descriptive and analytical study at the Ibn Sina Hospital and at the Military Hospital of instruction Mohammed V in Rabat over a period of 18 months, between September 2014 and February 2016, in order to evaluate the role of Fluorodeoxyglucose-PET/CT in the staging and restaging of non-small cell bronchopulmonary cancer. Initial staging showed a vast majority of locally advanced and metastatic stages: stage IV (40%), Stage IIIB (36%), Stage IIIA (16%), Stage II (8%). PET-CT allowed to detect new sites which were not initially seen on CT scan in 24 cases: 15 new ganglion sites, 8 new adrenal sites and 6 sites of bone lesions. PET/CT allowed to modify initial tumor stage in 60% of cases: upstaging in 23 patients (46%) and downstaging in 7 patients(14%). The initial stage remained unchanged in 40% of patients. Our study confirms the data from the literature concerning the superiority of PET-CT in comparison with CT scan, but only in the optimization of the non-small cell bronchopulmonary cancer management, in particular in locoregional and distant staging.

PMID: 29675123 [PubMed - in process]

Interobserver agreement in distinguishing large adrenal adenomas and adrenocortical carcinomas on computed tomography.

Mon, 04/23/2018 - 07:24
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Interobserver agreement in distinguishing large adrenal adenomas and adrenocortical carcinomas on computed tomography.

Abdom Radiol (NY). 2018 Apr 19;:

Authors: Thomas AJ, Habra MA, Bhosale PR, Qayyum AA, Ahmed K, Vicens R, Elsayes KM

Abstract
PURPOSE: Large adrenal masses pose a diagnostic dilemma. The purpose of this study was twofold: first, to assess the degree of interobserver agreement in evaluating the morphology of pathologically proven adrenal adenomas and adrenocortical carcinomas larger than 4 cm in diameter; and second, to identify morphologic characteristics that correlated with the pathologic diagnosis.
MATERIALS AND METHODS: For this blinded, retrospective study, we collected cases of 25 adrenal adenomas and 33 adrenocortical carcinomas measuring larger than 4 cm. Two radiologists evaluated morphologic characteristics of the lesions on CT. Interobserver agreement was evaluated using kappa statistics, and the correlation of imaging characteristics with the diagnosis was evaluated using a logistic regression model.
RESULTS: We found the highest interobserver agreement in the assessment of precontrast attenuation (Κ = 0.81) as well as substantial agreement in determining the shape and the presence of calcifications (Κ = 0.69 and 0.74, respectively). Readers agreed less often regarding the presence of fat (Κ = 0.48), as well as regarding the presence of necrosis, heterogeneity, and the overall impression (Κ = 0.15, 0.24, and 0.26, respectively). CT characteristics correlated with benignity included round shape (p = 0.02), an overall radiologic impression of a benign lesion (p < 0.0001), the presence of fat (p = 0.01), and a precontrast attenuation of less than 10 Hounsfield units (p < 0.0001). The latter two of these characteristics were highly specific for benign pathology (93% and 100%, respectively).
CONCLUSION: Our study suggests that CT has the ability to consistently identify characteristics significantly correlated with benign vs. malignant adrenal tumors.

PMID: 29671009 [PubMed - as supplied by publisher]

Activating PRKACB somatic mutation in cortisol-producing adenomas.

Mon, 04/23/2018 - 07:24
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Activating PRKACB somatic mutation in cortisol-producing adenomas.

JCI Insight. 2018 Apr 19;3(8):

Authors: Espiard S, Knape MJ, Bathon K, Assié G, Rizk-Rabin M, Faillot S, Luscap-Rondof W, Abid D, Guignat L, Calebiro D, Herberg FW, Stratakis CA, Bertherat J

Abstract
Mutations in the gene encoding the protein kinase A (PKA) catalytic subunit α have been found to be responsible for cortisol-producing adenomas (CPAs). In this study, we identified by whole-exome sequencing the somatic mutation p.S54L in the PRKACB gene, encoding the catalytic subunit β (Cβ) of PKA, in a CPA from a patient with severe Cushing syndrome. Bioluminescence resonance energy transfer and surface plasmon resonance assays revealed that the mutation hampers formation of type I holoenzymes and that these holoenzymes were highly sensitive to cAMP. PKA activity, measured both in cell lysates and with recombinant proteins, based on phosphorylation of a synthetic substrate, was higher under basal conditions for the mutant enzyme compared with the WT, while maximal activity was lower. These data suggest that at baseline the PRKACB p.S54L mutant drove the adenoma cells to higher cAMP signaling activity, probably contributing to their autonomous growth. Although the role of PRKACB in tumorigenesis has been suggested, we demonstrated for the first time to our knowledge that a PRKACB mutation can lead to an adrenal tumor. Moreover, this observation describes another mechanism of PKA pathway activation in CPAs and highlights the particular role of residue Ser54 for the function of PKA.

PMID: 29669941 [PubMed - as supplied by publisher]

A pediatric case of pheochromocytoma without apparent hypertension associated with von Hippel-Lindau disease.

Mon, 04/23/2018 - 07:24
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A pediatric case of pheochromocytoma without apparent hypertension associated with von Hippel-Lindau disease.

Clin Pediatr Endocrinol. 2018;27(2):87-93

Authors: Igaki J, Nishi A, Sato T, Hasegawa T

Abstract
Pheochromocytomas are catecholamine-secreting tumors. These tumors are rare in children, and they may be associated with hereditary syndromes such as von Hippel-Lindau (VHL) disease. Most pediatric patients with pheochromocytoma present with sustained hypertension, while 10% to 69% of adult patients are asymptomatic. Herein, we present the case of a 12-yr-old Japanese girl with pheochromocytoma due to a germline mutation in the VHL (Arg161Gln). The only complaint was loss of weight. Pyrexia, anemia, and increases in C-reactive protein (CRP) and ferritin were observed. Abdominal ultrasonography revealed a right adrenal gland tumor. Fractionated catecholamines and metanephrines in plasma and 24-h collected urine revealed elevated levels of norepinephrine and normetanephrine. Although hypertension and tachycardia were inapparent by an ordinary physical examination, paroxysmal mild hypertension and tachycardia were identified by a thorough examination after walking and abdominal compression. Paroxysmal hypertension and tachycardia were profound during operation. In conclusion, pheochromocytoma can be a consideration in the differential diagnosis of weight loss. Hypertension and tachycardia can be inapparent and paroxysmal in pediatric patients as well as in adults; thus, thorough assessment should be repeated.

PMID: 29662268 [PubMed]

Partial Cystectomy of Paraganglioma of the Urinary Bladder Before Living Kidney Transplantation: Case Report.

Mon, 04/23/2018 - 07:24
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Partial Cystectomy of Paraganglioma of the Urinary Bladder Before Living Kidney Transplantation: Case Report.

Transplant Proc. 2018 Apr;50(3):898-901

Authors: Kido K, Hatakeyama S, Hamano I, Yamamoto H, Imai A, Yoneyama T, Hashimoto Y, Koie T, Fujita T, Murakami R, Tomita H, Suzuki T, Narumi S, Ohyama C

Abstract
BACKGROUND: Paraganglioma (extra-adrenal pheochromocytoma) of the bladder is a very rare disease, accounting for 0.06% of all bladder tumors. Optimal management of bladder paraganglioma before kidney transplantation is unknown. We report a case of partial cystectomy for urinary bladder paraganglioma before living kidney transplantation.
CASE PRESENTATION: A 59-year-old man with a 27-year history of hemodialysis was referred to our department for further examination of a bladder tumor detected during pre-transplantation testing. Cystoscopy revealed a submucosal tumor on the right side of the bladder. The patient experienced a hypertensive crisis during transurethral resection of the bladder tumor. Endocrinologic and pathologic examinations confirmed the diagnosis of paraganglioma in the urinary bladder. A partial cystectomy was performed before kidney transplantation. Nine months after partial cystectomy, the patient underwent AB0-incompatible living kidney transplantation from his spouse. No disease recurrence or graft rejection was observed 12 months after the transplantation.
CONCLUSIONS: To our knowledge, this is the 1st report on the management of paraganglioma in the urinary bladder before living kidney transplantation. Kidney transplantation after partial cystectomy is an option that may be considered in patients with paraganglioma of the urinary bladder, with careful observations of bladder function and vesicoureteral reflux to the grafts.

PMID: 29661460 [PubMed - in process]

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