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Successful management of a third-trimester pregnancy complicated by pheochromocytoma: case report.

Mon, 06/18/2018 - 19:02
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Successful management of a third-trimester pregnancy complicated by pheochromocytoma: case report.

Gynecol Endocrinol. 2018 Jun 12;:1-3

Authors: Giampaolino P, Della Corte L, Formisano C, Cuomo L, Maurea S, Romeo V, Bifulco G

Abstract
Pheochromocytoma (PH) is a tumor that arises from chromaffin cells of the adrenal medulla. Though being this benign neoplasm very rare in pregnancies, lack of treatment nevertheless causes high mortality rates for both the mother and the fetus. Classic symptoms related to PH are hypertension, abdominal pain, diaphoresis, and headache; but it can be easily misdiagnosed as gestational hypertension or preeclampsia. Its appearance is sporadic, but there are some genetic disorders that favor its onset (e.g. MEN 2A and 2B). Individual management is needed, because no single protocol is suitable in such a complex and rare condition. In this paper we describe our experience in the clinical and surgical management of a young pregnant patient affected by PH, and in particular the specific and unique pharmacological treatment with doxazosin, the use of corticosteroids and a close monitoring of fetal well-being, which proved being an effective approach.

PMID: 29890868 [PubMed - as supplied by publisher]

Utilization of Ultrasound Guided Tissue-directed Cellular Implantation for the Establishment of Biologically Relevant Metastatic Tumor Xenografts.

Mon, 06/18/2018 - 19:02
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Utilization of Ultrasound Guided Tissue-directed Cellular Implantation for the Establishment of Biologically Relevant Metastatic Tumor Xenografts.

J Vis Exp. 2018 05 25;(135):

Authors: Thomas TT, Chukkapalli S, Van Noord RA, Krook M, Hoenerhoff MJ, Dillman JR, Lawlor ER, Opipari VP, Newman EA

Abstract
Preclinical testing of anticancer therapies relies on relevant xenograft models that mimic the innate tendencies of cancer. Advantages of standard subcutaneous flank models include procedural ease and the ability to monitor tumor progression and response without invasive imaging. Such models are often inconsistent in translational clinical trials and have limited biologically relevant characteristics with low proclivity to produce metastasis, as there is a lack of a native microenvironment. In comparison, orthotopic xenograft models at native tumor sites have been shown to mimic the tumor microenvironment and replicate important disease characteristics such as distant metastatic spread. These models often require tedious surgical procedures with prolonged anesthetic time and recovery periods. To address this, cancer researchers have recently utilized ultrasound-guided injection techniques to establish cancer xenograft models for preclinical experiments, which allows for rapid and reliable establishment of tissue-directed murine models. Ultrasound visualization also provides a noninvasive method for longitudinal assessment of tumor engraftment and growth. Here, we describe the method for ultrasound-guided injection of cancer cells, utilizing the adrenal gland for NB and renal sub capsule for ES. This minimally invasive approach overcomes tedious open surgery implantation of cancer cells in tissue-specific locations for growth and metastasis, and abates morbid recovery periods. We describe the utilization of both established cell lines and patient derived cell lines for orthotopic injection. Pre-made commercial kits are available for tumor dissociation and luciferase tagging of cells. Injection of cell suspension using image-guidance provides a minimally invasive and reproducible platform for the creation of preclinical models. This method is utilized to create reliable preclinical models for other cancers such as bladder, liver and pancreas exemplifying its untapped potential for numerous cancer models.

PMID: 29889191 [PubMed - in process]

Characteristics of Adrenal Incidentalomas in a Large, Prospective Computed Tomography-Based Multicenter Study: The COAR Study in Korea.

Mon, 06/18/2018 - 19:02
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Characteristics of Adrenal Incidentalomas in a Large, Prospective Computed Tomography-Based Multicenter Study: The COAR Study in Korea.

Yonsei Med J. 2018 Jun;59(4):501-510

Authors: Ahn SH, Kim JH, Baek SH, Kim H, Cho YY, Suh S, Kim BJ, Hong S, Koh JM, Lee SH, Song KH

Abstract
PURPOSE: Previous studies on adrenal incidentalomas (AIs) are limited by their retrospective design, small numbers of patients, Western populations, or use of an outdated imaging technique. We investigated the characteristics of AIs in Korean patients and compared them with those reported in the largest retrospective study in Italy to discover the effects of improved imaging techniques and ethnicity differences.
MATERIALS AND METHODS: This was a prospective, multicenter, cross-sectional observational study including 1005 Korean patients. Levels of plasma adrenocorticotrophic hormone, 24-h urinary free cortisol (UFC), serum cortisol after a 1 mg-dexamethasone suppression test, 24-h urinary fractionated metanephrine, and plasma aldosterone and plasma renin activity were measured. All AIs were characterized using computed tomography (CT).
RESULTS: Compared with the results of the Italian study, AIs in Korean patients were observed more frequently in men and predominantly on the left side. Korean patients with AIs were slightly younger, and fewer patients underwent surgery. Most AIs were nonfunctional in both studies, while fewer subclinical hypercortisolism and more primary aldosteronism (PA) cases were detected in Korean patients. In our study, high UFC levels showed very low sensitivity, compared to those in the Italian study. In pheochromocytoma or PA cases, there were no hormonal differences between the studies. AIs in Korean patients were smaller, such that a lower cutoff size for detecting adrenocortical carcinoma (ACC) could be warranted.
CONCLUSION: Recent advances in CT technology were leveraged to provide accurate characteristics of AIs and to detect smaller ACCs.

PMID: 29749133 [PubMed - indexed for MEDLINE]

Behçet's disease: New insights into pathophysiology, clinical features and treatment options.

Mon, 06/18/2018 - 19:02
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Behçet's disease: New insights into pathophysiology, clinical features and treatment options.

Autoimmun Rev. 2018 Jun;17(6):567-575

Authors: Greco A, De Virgilio A, Ralli M, Ciofalo A, Mancini P, Attanasio G, de Vincentiis M, Lambiase A

Abstract
Behçet's disease (BD) is a rare systemic vasculitis characterized by oral aphthous ulcers, genital ulcers, ocular lesions and other systemic manifestations. BD occurs most frequently in Eurasian populations along the ancient trading route known as the "Silk Road" which extends from eastern Asia to the Mediterranean basin. The causes of BD are unknown: it is believed to be due to an autoimmune process triggered by an infectious or environmental agent in genetically predisposed individuals. HLA-B51 allele located in the MHC locus, on chromosome 6p, has been the most strongly associated risk factor for BD in areas along the Old Silk Route. Herpes simplex virus-1 and Streptococcus have been postulated as possible environmental triggers of BD. T cell homeostasis perturbation, especially Th1 and Th17 expansion and decreased regulation by Tregs are now supposed to be the cornerstone of BD pathogenesis. The histology shows vasculitis that involves both arteries and veins, and vessels of any size. BD is a systemic vasculitis with significant neutrophil infiltration, endothelial cell swelling, and fibrinoid necrosis. The diagnosis of BD is only supported by clinical criteria and requires the exclusion of other diagnoses based on clinical presentation. There are no pathognomonic laboratorial findings of BD. This rare disease often leads to blindness and fatal systemic involvement. Main causes of death include major vessel disease and central nervous system involvement. Corticosteroids are commonly used to treat clinical manifestations of BD in combination with immunosuppressant drugs. Tumor necrosis factor (TNF)-blocking agents such as Infliximab, Etanercept, and Adalimumab have been reported to have success in patients with BD.

PMID: 29631062 [PubMed - indexed for MEDLINE]

A Case of Hypertensive Crisis without a Surge in Adrenal Hormones after Radiofrequency Ablation as a Treatment for Primary Hepatocellular Carcinoma.

Mon, 06/18/2018 - 19:02
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A Case of Hypertensive Crisis without a Surge in Adrenal Hormones after Radiofrequency Ablation as a Treatment for Primary Hepatocellular Carcinoma.

Korean J Gastroenterol. 2017 Oct 25;70(4):198-201

Authors: Lee KJ, Ryu SH

Abstract
Radiofrequency ablation (RFA) is a minimally invasive procedure that has been considered as a relatively safe treatment for patients with small hepatocellular carcinoma (HCC). However, RFA has been shown to be associated with complications including mechanical and thermal damage. A 74-year-old man with hepatitis C virus-associated HCC was admitted to our hospital. Abdominal computed tomography revealed two lobulated-HCC in segments 4 and 5. He had no medical history of hypertension and cardiac disease. During RFA, blood pressure was elevated to 200/140 mmHg. There was no evidence of pulmonary embolism, aortic dissection, or ischemic heart disease. Laboratory findings for catecholamine surge were all within normal limits. After continuous intravenous nitroglycerin and oral beta-blocker treatment, patient's blood pressure gradually decreased and back within the normal range. Hypertensive crisis after RFA treatment for HCC is rare. Most reported cases of hypertensive crisis during RFA were related to adrenal gland injury with a release of catecholamine. In our case, the site of HCC was not close to the adrenal gland, and there was no evidence of catecholamine surge. Herein, we report a very rare case of hypertensive crisis without a surge in adrenal hormones after RFA treatment for HCC.

PMID: 29060958 [PubMed - indexed for MEDLINE]

Perioperative management of paraganglioma and catecholamine-induced cardiomyopathy in child- a case report and review of the literature.

Mon, 06/18/2018 - 19:02
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Perioperative management of paraganglioma and catecholamine-induced cardiomyopathy in child- a case report and review of the literature.

BMC Anesthesiol. 2017 Oct 17;17(1):142

Authors: Jia X, Guo X, Zheng Q

Abstract
BACKGROUND: Paragangliomas are catecholamine-secreting tumors of the paraganglia. Perioperative mortality of children with paraganglioma is high, but preoperative therapy and anesthetic management of paraganglioma resection are controversial in children. The literatures on catecholamine-induced cardiomyopathy are limited to several case reports,with few reports of studies on children.
CASE PRESENTATION: Here we report the anesthetic management of a child with paraganglioma and catecholamine-induced cardiomyopathy, and the possible perioperative anesthesia problems of the paraganglioma resection are discussed.
CONCLUSION: Preoperative and intraoperative anesthetic management of Pheochromocytomas children should follow the same principles as for adults, The most important aspects are the control of blood pressure liability and maintenance of adequate blood volume. Pheochromocytomas patient may have cardiomoyopathy due to myocardial toxicity of excessive circulating catecholamines level. The perioperative management of catecholamine-induced cardiomyopathy should include lowering sympathetic activation by means of α-and β-adrenergic receptor blocker and diuretics administration in case of volume overload.

PMID: 29041921 [PubMed - indexed for MEDLINE]

Composite tumor with pheochromocytoma and immature neuroblastoma: report of two cases with cytogenetic analysis and discussion of current terminology.

Mon, 06/18/2018 - 19:02
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Composite tumor with pheochromocytoma and immature neuroblastoma: report of two cases with cytogenetic analysis and discussion of current terminology.

Virchows Arch. 2017 Oct;471(4):553-557

Authors: Tran L, Fitzpatrick C, Cohn SL, Pytel P

PMID: 28864906 [PubMed - indexed for MEDLINE]

[Pheochromocytoma presenting with secondary enuresis in a 13-year-old girl].

Mon, 06/18/2018 - 19:02
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[Pheochromocytoma presenting with secondary enuresis in a 13-year-old girl].

Arch Argent Pediatr. 2017 Aug 01;115(4):e255-e259

Authors: Zoido Garrote E, Fernández Fernández M, Álvarez Cañas MC, García Aparicio C, Revilla Orias MD, Martínez Badás JP

Abstract
Pheochromocytoma is a rare tumor which is infrequent in children. Although the clinical presentation in children can be atypical, the classic symptoms are headache, sweating and tachycardia. Hypertension is often a constant sign in most patients. There are few cases in literature reporting pheochromocytoma presented with polyuria. We present a 13-year-old girl who came to the Pediatric Nephrologist due to a year of evolution of secondary enuresis. When her blood pressure was taken, she was above the 99th percentile that corresponds to her age and her height that is why she was admitted for treatment and diagnostic study.

PMID: 28737880 [PubMed - indexed for MEDLINE]

Genetic status determines 18 F-FDG uptake in pheochromocytoma/paraganglioma.

Mon, 06/18/2018 - 19:02
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Genetic status determines 18 F-FDG uptake in pheochromocytoma/paraganglioma.

J Med Imaging Radiat Oncol. 2017 Dec;61(6):745-752

Authors: Tiwari A, Shah N, Sarathi V, Malhotra G, Bakshi G, Prakash G, Khadilkar K, Pandit R, Lila A, Bandgar T

Abstract
INTRODUCTION: Although few studies have demonstrated utility of 18 F- fluoro-2-deoxy-d-glucose positron emission tomography/computerised tomography (18 F-FDG PET/CT) in benign pheochromocytoma/paragangliomas (PCC/PGLs), there limited data on factors predicting the FDG uptake in PCC/PGL.
METHODS: The study was conducted at a tertiary health care centre. In addition to the routine investigations, all patients (n = 96) with PCC/PGL were evaluated with 18 F-FDGPET/CT and majority (n = 78) underwent 131 I-metaiodobenzyl guanidine (131 I-MIBG) scintigraphy. Forty-three patients also underwent testing for germline mutations in five PCC/PGL susceptibility genes (VHL, RET, SDHB, SDHC and SDHD) and all patients were evaluated clinically for neurofibromatosis-1.
RESULTS: The study included 96 patients with PCC/PGL(82 benign and 14 malignant). FDGSUVmax was significantly higher for malignant than benign PCC/PGL(P = 0.009) and for extra-adrenal PGL than adrenal PCC (P = 0.017). In subgroup analysis, metanephrine-secreting PCC and non-secretory PCC had significantly lower FDG SUVmax than normetanephrine-secreting PCC (P = 0.017, P = 0.038 respectively), normetanephrine-secreting-sympathetic PGL (P = 0.008, P = 0.019 respectively) and non-secretory sympathetic PGL (P = 0.003, P = 0.009 respectively). Patients with mutations in cluster 1 genes (n = 14) had significantly higher FDG SUVmax than those with mutations in cluster 2 genes (n = 4) (P = 0.04). Sensitivities of 131 I-MIBG and 18 F-FDG PET/CTwere 77.78% and 100% for cluster 1 genes-related PCC/PGL whereas they were 100% and 50% for cluster 2 genes-related PCC/PGL, respectively. Multivariate regression analysis of mutation positive patients identified genetic status as the only independent predictor of FDG SUVmax.
CONCLUSION: The study suggests that the underlying genetic status determines FDG uptake in PCC/PGL and not location, secretory status or malignancy.

PMID: 28585398 [PubMed - indexed for MEDLINE]

Examination of PHOX2B in adult neuroendocrine neoplasms reveals relatively frequent expression in phaeochromocytomas and paragangliomas.

Mon, 06/18/2018 - 19:02
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Examination of PHOX2B in adult neuroendocrine neoplasms reveals relatively frequent expression in phaeochromocytomas and paragangliomas.

Histopathology. 2017 Oct;71(4):503-510

Authors: Lee JP, Hung YP, O'Dorisio TM, Howe JR, Hornick JL, Bellizzi AM

Abstract
AIMS: Paired-like homeobox 2b (PHOX2B) is a transcription factor with expression outside of the central nervous system restricted to neurons and chromaffin cells of the autonomic nervous system. Germline mutations cause congenital central hypoventilation syndrome and predispose to neuroblastoma and Hirschsprung disease. Among paediatric small round cell tumours, PHOX2B is neuroblastoma-specific. Two studies of adult autonomic nervous system tumours (n = 62) produced conflicting results (all tumours stained in one; expression restricted to 40% of paragangliomas in the other). We examined PHOX2B expression in a large cohort of phaeochromocytomas and paragangliomas, as well as well-differentiated neuroendocrine tumours (WDNETs) and poorly differentiated neuroendocrine carcinomas (PDNECs).
METHODS AND RESULTS: Tissue microarrays (TMAs) were constructed from 609 tumours: 111 phaeochromocytomas, 146 paragangliomas, 250 WDNETs, and 102 PDNECs. PHOX2B immunohistochemistry was scored for extent (%) and intensity (0-3+), and an H-score (extent × intensity) was calculated. PHOX2B expression was seen in 32% of phaeochromocytomas and in 47% of paragangliomas. Mean/median H-scores for these tumours were in the 30-55 range (i.e. weak to moderate staining). No WDNETs and only 7% of PDNECs stained, the latter often strongly. In a representative cohort of corresponding whole sections (n = 55), the results in WDNETs and PDNECs were unchanged, whereas half of the phaeochromocytomas/paragangliomas that were negative on TMAs became focally, weakly positive.
CONCLUSIONS: We found frequent, weak to moderate PHOX2B expression in phaeochromocytomas/paragangliomas and no expression in WDNETs, which could be diagnostically useful in the distinction of these tumours. Expression in a minority of PDNECs probably reflects the transcription factor lineage infidelity that is characteristic of this tumour class.

PMID: 28464318 [PubMed - indexed for MEDLINE]

adrenal tumor; +84 new citations

Mon, 06/11/2018 - 16:37

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

adrenal tumor

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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]

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