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Clinical and biological features of neuroblastic tumors: A comparison of neuroblastoma and ganglioneuroblastoma.

Thu, 05/11/2017 - 01:24
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Clinical and biological features of neuroblastic tumors: A comparison of neuroblastoma and ganglioneuroblastoma.

Oncotarget. 2017 Apr 17;:

Authors: He WG, Yan Y, Tang W, Cai R, Ren G

Abstract
Neuroblastoma (NB), ganglioneuroblastoma intermixed (GNBi) and ganglioneuroblastoma nodular (GNBn) are neuroblastic tumors that present with a wide range of symptoms and variable prognoses. We retrospectively reviewed the pretreatment clinical (age, sex and tumor stage) and biological (MYCN amplification; and levels of lactate dehydrogenase, ferritin and neuron-specific enolase) characteristics of 279 patients who were diagnosed with pathologically confirmed NB and GNB from January 2005 to December 2015. The median age at diagnosis increased with grade of differentiation (NB: 28.9 months; GNBn: 38.4 months; GNBi: 47.5 months; p < 0.01). NB patients were more frequently diagnosed with adrenal tumors and had a higher prevalence of abnormal serum ferritin at the time of diagnosis (60.0% vs. 40.0% vs. 12.0%, P<0.001), NSE (96.0% vs. 93.0% vs. 81.0%, P=0.013) when compared with GNBn and GNBi patients. The prevalence rates of disseminated tumors and MYCN amplified tumors were lower in the GNBi group than in the GNBn and NB groups (13.0% vs. 25.0% vs. 44.0%, P=0.002; 0 vs. 14.0% vs. 26.0%, P=0.032, respectively). The overall survival (OS) of patients with GNB was significantly better than that of patients with NB (GNBi: 100%, GNBn: 74.5±11.4%, NB: 50.8±4.5%, respectively; P<0.01). Our study revealed that both NB and GNB have a wide range of presentations, and clinicians should be aware of both typical and atypical symptoms and signs. Children with GNB (especially GNBi) were more likely to present favorable prognostic factors than their NB counterparts, which consequently lead to better outcomes and longer survival for these patients.

PMID: 28465480 [PubMed - as supplied by publisher]

Impaired Dual-Specificity Protein Phosphatase DUSP4 Reduces Corticosteroid Sensitivity.

Thu, 05/11/2017 - 01:24
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Impaired Dual-Specificity Protein Phosphatase DUSP4 Reduces Corticosteroid Sensitivity.

Mol Pharmacol. 2017 May;91(5):475-481

Authors: Kobayashi Y, Ito K, Kanda A, Tomoda K, Mercado N, Barnes PJ

Abstract
We have reported that phosphorylation of the glucocorticoid receptor (GR) at Ser(226) reduces GR nuclear translocation, resulting in corticosteroid insensitivity in patients with severe asthmas. A serine/threonine protein phosphatase 2A, which regulates c-Jun N-terminal kinase (JNK) 1 and GR-Ser(226) signaling, is involved in this mechanism. Here, we further explored protein kinase dual-specificity phosphatases (DUSPs) with the ability to dephosphorylate JNK, and identified DUSP4 as a phosphatase involved in the regulation of corticosteroid sensitivity. The effects of knocking down DUSPs (DUSP1, 4, 8, 16, and 22) by small interfering RNA (siRNA) were evaluated in a monocytic cell line (U937). Corticosteroid sensitivity was determined by dexamethasone enhancement of FK506-binding protein 51 or inhibition of tumor necrosis factor α (TNFα)-induced interferon γ and interleukin 8 expression and GR translocation from cell cytoplasm to nucleus. The nuclear/cytoplasmic GR, phosphorylation levels of GR-Ser(226) and JNK1, coimmunoprecipitated GR-JNK1-DUSP4, and DUSP4 expression were analyzed by western blotting and/or imaging flow cytometry. Phosphatase activity of immunoprecipitated (IP)-DUSP4 was measured by fluorescence-based assay. Knockdown of DUSP4 enhanced phosphorylation of GR-Ser(226) and JNK1 and reduced GR nuclear translocation and corticosteroid sensitivity. Coimmunoprecipitation experiments showed that DUSP4 is associated with GR and JNK1. In peripheral blood mononuclear cells from severe asthmatics, DUSP4 expression was reduced versus healthy subjects and negatively correlated with phosphorylation levels of GR-Ser(226) and JNK1. Formoterol enhanced DUSP4 activity and restored corticosteroid sensitivity reduced by DUSP4 siRNA. In conclusion, DUSP4 regulates corticosteroid sensitivity via dephosphorylation of JNK1 and GR-Ser(226) DUSP4 activation by formoterol restores impaired corticosteroid sensitivity, indicating that DUSP4 is crucial in regulating corticosteroid sensitivity, and therefore might be a novel therapeutic target in severe asthma.

PMID: 28283554 [PubMed - indexed for MEDLINE]

Thyroid lesions in patients with acromegaly - case-control study and update to the meta-analysis.

Thu, 05/11/2017 - 01:24
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Thyroid lesions in patients with acromegaly - case-control study and update to the meta-analysis.

Endokrynol Pol. 2017;68(1):2-6

Authors: Woliński K, Stangierski A, Gurgul E, Bromińska B, Czarnywojtek A, Lodyga M, Ruchała M

Abstract
INTRODUCTION: Acromegaly results from oversecretion of growth hormone and subsequently insulin growth factor-1. According to some authors, the disease can cause increased prevalence of nodular goitre and thyroid cancer (TC). However, the number of studies comparing acromegalic patients with control groups is low. We aimed to assess the prevalence of thyroid lesions in patients with acromegaly in comparison to an age- and sex-matched control group and to update the meta-analysis previously performed in our department by the same authors.
MATERIAL AND METHODS: We searched medical documentation of patients with acromegaly treated in our department between 2003 and 2013. The prevalence of thyroid abnormalities was compared with the group of patients with hormonally inactive adrenal incidentalomas. To perform the meta-analytic part of the paper we also searched ten databases to find relevant papers.
RESULTS: Two hundred and five patients with acromegaly and 184 patients with incidentalomas were included. Any thyroid lesions were present in 77.6% of patients with acromegaly vs. 63.0% with incidentalomas (p = 0.002), multinodular goitre - 66.8% vs. 47.8% (p = 0.0002), and TC- 5.4% vs. 2.7% (p = 0.21) respectively. For thyroid lesions the pooled odds ratio (OR) was 3.1 (95% confidence interval [CI] 1.8-5.5), and for TCs the OR was 4.5 (95% CI 1.9-10.3).
CONCLUSIONS: According to our results thyroid lesions were significantly more common in patients with acromegaly; in case of TC the difference was not significant. The updated meta-analysis showed significantly increased prevalence of both disorders. In conclusion, systematic thyroid examination should be an important part of follow-up in case of acromegalic patients. (Endokrynol Pol 2017; 68 (1): 2-6).

PMID: 28255974 [PubMed - indexed for MEDLINE]

Mutational signature analysis identifies MUTYH deficiency in colorectal cancers and adrenocortical carcinomas.

Thu, 05/11/2017 - 01:24
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Mutational signature analysis identifies MUTYH deficiency in colorectal cancers and adrenocortical carcinomas.

J Pathol. 2017 May;242(1):10-15

Authors: Pilati C, Shinde J, Alexandrov LB, Assié G, André T, Hélias-Rodzewicz Z, Ducoudray R, Le Corre D, Zucman-Rossi J, Emile JF, Bertherat J, Letouzé E, Laurent-Puig P

Abstract
Germline alterations in DNA repair genes are implicated in cancer predisposition and can result in characteristic mutational signatures. However, specific mutational signatures associated with base excision repair (BER) defects remain to be characterized. Here, by analysing a series of colorectal cancers (CRCs) using exome sequencing, we identified a particular spectrum of somatic mutations characterized by an enrichment of C > A transversions in NpCpA or NpCpT contexts in three tumours from a MUTYH-associated polyposis (MAP) patient and in two cases harbouring pathogenic germline MUTYH mutations. In two series of adrenocortical carcinomas (ACCs), we identified four tumours with a similar signature also presenting germline MUTYH mutations. Taken together, these findings demonstrate that MUTYH inactivation results in a particular mutational signature, which may serve as a useful marker of BER-related genomic instability in new cancer types. Copyright © 2017 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.

PMID: 28127763 [PubMed - indexed for MEDLINE]

Adrenal lymphoma: presentation, management and prognosis.

Thu, 05/11/2017 - 01:24
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Adrenal lymphoma: presentation, management and prognosis.

QJM. 2017 Feb 01;110(2):103-109

Authors: Laurent C, Casasnovas O, Martin L, Chauchet A, Ghesquieres H, Aussedat G, Fornecker LM, Bologna S, Borot S, Laurent K, Bouillet B, Verges B, Petit JM

Abstract
Aim: This study aimed to identify the clinical, radiological and prognostic features of primary adrenal lymphoma (PAL) in order to diagnose the disease more accurately.
Materials and methods: A retrospective multi-centre study was conducted on the clinical, biological and radiological features as well as the treatment and overall survival outcomes in PAL.
Results: Between 1994 and 2014, 28 patients from five regions of eastern France were diagnosed with primary adrenal lymphoma. The revealing symptoms were a worsening general state (77%), weight loss (77%) and abdominal pain (42%). Biological features of PAL were almost omnipresent: increased LDH, β2 microglobulin, CRP or ferritinaemia levels. The PAL was bilateral in 20 cases (71%), adrenal insufficiency was searched for in 11 patients and found in eight (73%). CT scans showed masses of various sizes measuring up to 180 mm. On MRI, the lesions were hypointense in T1 and hyperintense in T2. When done, positron emission tomography with fluorodeoxyglucose (FDG-PET) showed locations not seen on the CT and revealed extra-adrenal locations in 70% of examinations. Adrenalectomy brought no benefit. The overall survival rate was poor (61.9% at 2 years) despite polychemotherapy.
Conclusion: The clinical presentation of PAL comprised major general symptoms. Adrenal insufficiency was very common in patients with bilateral involvement but was not systematically tested. PET was an efficient examination to visualize extra-adrenal locations. The preliminary results of MRI to distinguish between PAL and adrenocortical carcinoma should be confirmed. Further studies are needed to establish an optimal strategy for the management of these primary adrenal lymphomas.

PMID: 27795295 [PubMed - indexed for MEDLINE]

Three siblings with familial non-medullary thyroid carcinoma: a case series.

Thu, 05/11/2017 - 01:24
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Three siblings with familial non-medullary thyroid carcinoma: a case series.

J Med Case Rep. 2016 Aug 02;10:213

Authors: Rashid MO, Haq N, Farooq S, Kiran Z, Siddique S, Pervez S, Islam N

Abstract
BACKGROUND: In 2015, thyroid carcinoma affected approximately 63,000 people in the USA, yet it remains one of the most treatable cancers. It is mainly classified into medullary and non-medullary types. Conventionally, medullary carcinoma was associated with heritability but increasing reports have now begun to associate non-medullary thyroid carcinoma with a genetic predisposition as well. It is important to identify a possible familial association in patients diagnosed with non-medullary thyroid carcinoma because these cancers behave more destructively than would otherwise be expected. Therefore, it is important to aggressively manage such patients and screening of close relatives might be justified. Our case series presents a diagnosis of familial, non-syndromic, non-medullary carcinoma of the thyroid gland in three brothers diagnosed over a span of 6 years.
CASE PRESENTATIONS: We report the history, signs and symptoms, laboratory results, imaging, and histopathology of the thyroid gland of three Pakistani brothers of 58 years, 55 years, and 52 years from Sindh with non-medullary thyroid carcinoma. Only Patients 1 and 3 had active complaints of swelling and pruritus, respectively, whereas Patient 2 was asymptomatic. Patients 2 and 3 had advanced disease at presentation with lymph node metastasis. All patients underwent a total thyroidectomy with Patients 2 and 3 requiring a neck dissection as well. No previous exposure to radiation was present in any of the patients. Their mother had died from adrenal carcinoma but also had a swelling in the front of her neck which was never investigated. All patients remained stable at follow-up.
CONCLUSIONS: Non-medullary thyroid carcinoma is classically considered a sporadic condition. Our case report emphasizes a high index of suspicion, a detailed family history, and screening of first degree relatives when evaluating patients with non-medullary thyroid carcinoma to rule out familial cases which might behave more aggressively.

PMID: 27484350 [PubMed - indexed for MEDLINE]

Oral Low-dose Theophylline on Top of Inhaled Fluticasone-Salmeterol Does Not Reduce Exacerbations in Patients With Severe COPD: A Pilot Clinical Trial.

Thu, 05/11/2017 - 01:24
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Oral Low-dose Theophylline on Top of Inhaled Fluticasone-Salmeterol Does Not Reduce Exacerbations in Patients With Severe COPD: A Pilot Clinical Trial.

Chest. 2016 Jul;150(1):123-30

Authors: Cosío BG, Shafiek H, Iglesias A, Yanez A, Córdova R, Palou A, Rodriguez-Roisin R, Peces-Barba G, Pascual S, Gea J, Sibila O, Barnes PJ, Agusti A

Abstract
BACKGROUND: COPD is characterized by chronic inflammation. In vitro and ex vivo observations suggest that this inflammatory response is partially resistant to the effect of corticosteroids and that low-dose theophylline can restore this response via enhancement of histone deacetylase (HDAC) activity. Whether this occurs in vivo and what its potential clinical consequences are is unclear.
OBJECTIVES: The objective of this trial was to determine whether low-dose theophylline on top of inhaled long-acting β2-agonists and inhaled corticosteroids (ICS) in patients with COPD (1) enhances HDAC activity and the antiinflammatory effects of ICS in vivo, (2) reduces the concentration of inflammatory markers, and (3) reduces exacerbation frequency.
METHODS: In this prospective, double-blind, placebo-controlled clinical trial, we randomized patients with COPD (FEV1 < 50% predicted plus at least one hospitalization due to exacerbation in the previous year) to ICS plus theophylline 100 mg bid or matched placebo. We determined the following at baseline and at the end of 52 weeks of follow-up: (1) HDAC activity in blood monocytes and sputum macrophages, (2) the concentration of several inflammatory markers (IL-8, IL-6, IL-1β, and tumor necrosis factor -α) in serum and sputum supernatant, and (3) the rates of exacerbations and adverse effects.
RESULTS: Seventy patients were randomized-36 to theophylline and 34 to placebo. HDAC activity and inflammatory marker levels were not different in the two arms either at baseline or after 52 weeks. Likewise, the rate of exacerbations during follow-up was similar in both groups.
CONCLUSIONS: The combination of low-dose oral theophylline and ICS did not enhance the antiinflammatory properties of ICS in vivo or influence exacerbation rate.
TRIAL REGISTRY: ClinicalTrials.gov; No.: NCT01599871; URL: www.clinicaltrials.gov.

PMID: 27107490 [PubMed - indexed for MEDLINE]

Clinical Features and Outcome of Pheochromocytoma-Induced Takotsubo Syndrome: Analysis of 80 Published Cases.

Thu, 05/11/2017 - 01:24
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Clinical Features and Outcome of Pheochromocytoma-Induced Takotsubo Syndrome: Analysis of 80 Published Cases.

Am J Cardiol. 2016 Jun 01;117(11):1836-44

Authors: Y-Hassan S

Abstract
Myriads of physical stress factors including pheochromocytoma have been reported triggering takotsubo syndrome (TS). The aim of this study was to report on the clinical features and outcome of pheochromocytoma-induced TS (Pheo-TS) in a large cohort of patients. Eighty published cases of Pheo-TS were retrieved from the literature and compared with 1,750 cases of all patients with TS (All-TS) published recently by Templin et al. Patients with Pheo-TS were on average 19.87 years younger than those with All-TS (p <0.0001). The women were still predominating in Pheo-TS but significantly in a lower percentage (70% in Pheo-TS vs 89.8% in All-TS, p <0.00001). Almost 1/3 (30%) of Pheo-TS cases had basal TS pattern compared with 2.2% of cases in All-TS (p <0.00001) and 1/5 (20%) had global TS compared with no cases in All-TS. Two thirds of Pheo-TS cases (67.9%) developed complications, which was significantly higher than complication rates in All-TS (21.8%), but there was no difference in the inhospital mortality between the 2 studies. The most important risk factors for the development of complications in Pheo-TS were age <50 years and global and basal TS localization patterns. The recurrence rate of 17.7% in Pheo-TS was significantly higher than the 3.26% in All-TS (p <0.00001). In conclusion, Pheo-TS is characterized by a dramatic clinical presentation with high complication rates and relatively high recurrence rate. Patients with Pheo-TS are significantly younger than All-TS. The TS localization pattern in Pheo-TS differed significantly from All-TS with basal pattern in almost 1/3 of cases and global pattern in 1/5 of the cases.

PMID: 27103159 [PubMed - indexed for MEDLINE]

Corticosteroids compromise survival in glioblastoma.

Thu, 05/11/2017 - 01:24
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Corticosteroids compromise survival in glioblastoma.

Brain. 2016 May;139(Pt 5):1458-71

Authors: Pitter KL, Tamagno I, Alikhanyan K, Hosni-Ahmed A, Pattwell SS, Donnola S, Dai C, Ozawa T, Chang M, Chan TA, Beal K, Bishop AJ, Barker CA, Jones TS, Hentschel B, Gorlia T, Schlegel U, Stupp R, Weller M, Holland EC, Hambardzumyan D

Abstract
Glioblastoma is the most common and most aggressive primary brain tumour. Standard of care consists of surgical resection followed by radiotherapy and concomitant and maintenance temozolomide (temozolomide/radiotherapy→temozolomide). Corticosteroids are commonly used perioperatively to control cerebral oedema and are frequently continued throughout subsequent treatment, notably radiotherapy, for amelioration of side effects. The effects of corticosteroids such as dexamethasone on cell growth in glioma models and on patient survival have remained controversial. We performed a retrospective analysis of glioblastoma patient cohorts to determine the prognostic role of steroid administration. A disease-relevant mouse model of glioblastoma was used to characterize the effects of dexamethasone on tumour cell proliferation and death, and to identify gene signatures associated with these effects. A murine anti-VEGFA antibody was used in parallel as an alternative for oedema control. We applied the dexamethasone-induced gene signature to The Cancer Genome Atlas glioblastoma dataset to explore the association of dexamethasone exposure with outcome. Mouse experiments were used to validate the effects of dexamethasone on survival in vivo Retrospective clinical analyses identified corticosteroid use during radiotherapy as an independent indicator of shorter survival in three independent patient cohorts. A dexamethasone-associated gene expression signature correlated with shorter survival in The Cancer Genome Atlas patient dataset. In glioma-bearing mice, dexamethasone pretreatment decreased tumour cell proliferation without affecting tumour cell viability, but reduced survival when combined with radiotherapy. Conversely, anti-VEGFA antibody decreased proliferation and increased tumour cell death, but did not affect survival when combined with radiotherapy. Clinical and mouse experimental data suggest that corticosteroids may decrease the effectiveness of treatment and shorten survival in glioblastoma. Dexamethasone-induced anti-proliferative effects may confer protection from radiotherapy- and chemotherapy-induced genotoxic stress. This study highlights the importance of identifying alternative agents such as vascular endothelial growth factor antagonists for managing oedema in glioblastoma patients. Beyond the established adverse effect profile of protracted corticosteroid use, this analysis substantiates the request for prudent and restricted use of corticosteroids in glioblastoma.

PMID: 27020328 [PubMed - indexed for MEDLINE]

ERBB-2 overexpression as a risk factor for malignant phaeochromocytomas and paraganglinomas.

Thu, 05/11/2017 - 01:24
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ERBB-2 overexpression as a risk factor for malignant phaeochromocytomas and paraganglinomas.

Clin Endocrinol (Oxf). 2016 Jun;84(6):822-9

Authors: Wang W, Zhong X, Ye L, Qi Y, Su T, Wei Q, Xie J, Jiang L, Jiang Y, Zhou W, Cui B, Ning G

Abstract
OBJECTIVE: There are currently no good histological or molecular markers to differentiate benign from malignant phaeochromocytomas and paraganglinomas (PPGLs). Our previous cross-sectional study observed that ERBB-2 overexpression was associated with malignant PPGLs. This study aimed to evaluate the predictive value of ERBB-2 overexpression for metastasis in PPGLs in a large population.
METHODS: A total of 262 patients diagnosed as PPGLs in our institution between 2002 and 2012 were included. We analysed ERBB-2 protein expression in the primary PPGL tumours by immunohistochemistry (IHC) and ERBB-2 amplification by fluorescence in situ hybridization (FISH). Direct Sanger sequencing was performed to examine ERBB-2 exon 20 mutations. The occurrence of malignant PPGLs was documented in the follow-up period. Kaplan-Meier analysis and Cox proportional hazard models were used to evaluate the association between ERBB-2 overexpression and metastasis of PPGLs.
RESULTS: Twenty-six (9·9%) patients had ERBB-2 overexpression in their primary PPGL tumours, which was significantly associated with ERBB-2 amplification (17/25, 68%). No ERBB-2 mutation was found. At a median follow-up of 4·5 years, a total of 23 malignant PPGLs were documented, including eight (30·8%) patients in the ERBB-2 overexpression group and 15 (6·4%) patients in the ERBB-2-negative group. The incidence rate of metastasis was 5·3 per 100 person-years vs 1·4 per 100 person-years in the ERBB-2 overexpression and ERBB-2-negative groups (P < 0·001), respectively. Kaplan-Meier analysis showed that ERBB-2 overexpression was associated with decreased metastasis-free survival (P = 0·001, log-rank test). After adjusting for primary tumour size and location, Cox regression analysis revealed that ERBB-2 overexpression was independently associated with risk of malignant PPGLs (HR = 2·78; 95% CI, 1·12-6·90; P = 0·028).
CONCLUSION: Patients harbouring tumours with ERBB-2 overexpression have a significantly higher risk of developing malignant PPGLs.

PMID: 26749044 [PubMed - indexed for MEDLINE]

Experience in optimizing fertility outcomes in men with congenital adrenal hyperplasia due to 21 hydroxylase deficiency.

Thu, 05/11/2017 - 01:24
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Experience in optimizing fertility outcomes in men with congenital adrenal hyperplasia due to 21 hydroxylase deficiency.

Clin Endocrinol (Oxf). 2016 Jun;84(6):830-6

Authors: King TF, Lee MC, Williamson EE, Conway GS

Abstract
OBJECTIVE: Men with congenital adrenal hyperplasia (CAH) have impaired fertility. We aimed to assess fertility outcomes and the importance of hypogonadotropic hypogonadism, testicular failure and the presence of testicular adrenal rest tumours (TART).
DESIGN: Retrospective analysis of men attending an adult CAH clinic in a tertiary centre.
PATIENTS: Fifty men with CAH due to 21 hydroxylase deficiency were identified of whom 35 were salt wasting and 15 were non-salt-wasting.
MEASUREMENTS: Review of fertility history and parameters including luteinizing hormone (LH), follicle-stimulating hormone (FSH), androstenedione, 17-hydroxyprogesterone (17-OHP), semen analysis and the presence of testicular adrenal rest tissue (TART) on ultrasound.
RESULTS: TART were detected by ultrasound in 21 (47%), and their presence was associated with an elevated FSH (P = 0·01). Severe oligospermia was present in 11 of 23 (48%), and this was associated with an elevated FSH (P = 0·02), suppressed LH (P < 0·01) and TART (P = 0·03) when compared to those with a sperm count >5 × 10(6) per ml. Of those that desired fertility, 10 of 17 (59%) required treatment intensification and four underwent in vitro fertilization. Intensification resulted in a rise in median LH (0·6-4·3 IU/l; P = 0·01). Live birth rate was 15 of 17 (88%) with a median (range) time to conception of 8 (0-38) months.
CONCLUSIONS: Suppressed LH is a marker for subfertility and is often reversible. Testicular failure is closely associated with TART formation. If TART are detected, sperm cryopreservation should be offered given the risk of progression to irreversible testicular failure. Male fertility in CAH can be improved by intensified treatment and assisted reproductive technology.

PMID: 26666213 [PubMed - indexed for MEDLINE]

An unusual cause of back pain: a case of large nonfunctioning retroperitoneal paraganglioma presented as a large cystic lesion. A case report and review of literature.

Wed, 05/03/2017 - 22:26
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An unusual cause of back pain: a case of large nonfunctioning retroperitoneal paraganglioma presented as a large cystic lesion. A case report and review of literature.

J Surg Case Rep. 2017 Apr;2017(4):rjx059

Authors: Kok SYA, Leung CY, Chow KY

Abstract
Pheochromocytoma arising from outside the adrenal glands is also called paraganglioma. When it occurs below the diaphragm, in the organ of Zuckerkandl or retroperitoneum, it is also called extra-adrenal pheochromocytoma. Paragangliomas are rare tumors which arise from neuroendocrine cells and extra-adrenal paragangliomas (EAPs) account for only 10-15% if all paragangliomas and may present incidentally as a symptomless mass. Typical triad of sweating, headache and fluctuating hypertension if not present makes preoperative diagnosis difficult. Surgical excision is the treatment of choice. Definitive diagnosis is usually made with histological findings. We report a case of large retroperitoneal tumor with size >10 cm which was a high risk depicting malignancy.

PMID: 28458869 [PubMed - in process]

Acute intestinal obstruction due to metastatic lung cancer-case report.

Wed, 05/03/2017 - 22:26
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Acute intestinal obstruction due to metastatic lung cancer-case report.

J Surg Case Rep. 2017 Feb;2017(2):rjx031

Authors: Janež J

Abstract
We present a case of male patient, who was referred to our department because of acute intestinal obstruction, which was the initial clinical symptom of primary lung cancer. The abdominal computed tomography (CT) prior to the emergency operation showed small intestinal obstruction and metastases to both adrenal glands. The patient underwent an emergency abdominal exploratory laparotomy, that confirmed small bowel obstruction and diffuse metastatic lesions along the entire small bowel length. During the operation we took a sample of one metastasis for pathological examination and we created an intestinal bypass to relieve small bowel obstruction. The pathologist suspected to primary lung cancer according to the immunohistochemical staining. The chest CT after the emergency operation showed a large primary tumor in the left upper pulmonary lobe.

PMID: 28458837 [PubMed - in process]

Surgical approaches in hereditary endocrine tumors.

Wed, 05/03/2017 - 22:26
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Surgical approaches in hereditary endocrine tumors.

Updates Surg. 2017 Apr 28;:

Authors: Iacobone M, Citton M, Viel G, Schiavone D, Torresan F

Abstract
Endocrine tumors of thyroid, adrenal and parathyroid glands may be due to germline and inheritable mutations in 5-30% of patients. Medullary Thyroid Carcinoma, Pheochromocytoma, Paraganglioma, and Familial Primary Hyperparathyroidism are the most frequent entity. Hereditary endocrine tumors usually have a suggestive familial history; they occur earlier than sporadic variants, are multifocal, and have increased recurrence rates. They may be present as isolated variant or associated to other neoplasms in a syndromic setting. Genetic diagnosis should be preferably available before surgery because specific and targeted operative management are needed to achieve the best chance of cure. This review was aimed to discuss the surgical approaches for some of the most frequent hereditary endocrine tumors of thyroid, adrenal and parathyroid glands, focusing on medullary thyroid carcinoma, Pheochromocytoma, Paraganglioma and hereditary primary hyperparathyroidism (pHPT). Hereditary Medullary Thyroid Carcinoma is caused by RET mutations, and may be associated to Pheochromocytomas in MEN 2 setting. Total thyroidectomy and at least central neck nodal dissection is required. The availability of genetic screening allows prophylactic or early surgery in asymptomatic patients, with subsequent definitive cure. Hereditary Pheochromocytomas may be present in several syndromes (MEN 2, VHL, NF1, Paraganglioma/Pheochromocytoma syndrome); it may involve both adrenals; in these cases, a cortical sparing adrenalectomy should be performed to avoid permanent hypocorticosurrenalism. Hereditary Primary Hyperparathyroidism may frequently occur associated to MEN 1, MEN 2A, MEN 4, Hyperparathyroidism-Jaw Tumor Syndrome; it may involve all the parathyroid glands, requiring subtotal parathyroidectomy or total parathyroidectomy plus autotransplantation. In some cases, a selective parathyroidectomy might be performed.

PMID: 28455835 [PubMed - as supplied by publisher]

Robotic Radiosurgery for Adrenal Gland Metastases.

Wed, 05/03/2017 - 22:26
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Robotic Radiosurgery for Adrenal Gland Metastases.

Cureus. 2017 Mar 26;9(3):e1120

Authors: Haidenberger A, Heidorn SC, Kremer N, Muacevic A, Fürweger C

Abstract
INTRODUCTION: The purpose of this study was to investigate the safety and efficacy of CyberKnife (CK) robotic radiosurgery for treatment of adrenal metastases.
METHODS: We performed a retrospective analysis of 23 patients with adrenal metastases who had been treated with CK between October 2006 and December 2015. Fifteen patients received chemotherapy prior to radiosurgery, all patients underwent computer tomography (CT) fluoroscopically guided percutaneous placement of one to three gold fiducials into the adrenal gland. Nineteen patients were selected for single-fraction radiosurgery with a median dose of 22 Gy, four patients were treated in three fractions with a median dose of 13.5 Gy.
RESULTS: Median follow-up time was 23.6 months. Four patients (17%) experienced local relapse during the evaluation period with a mean time of 19 months to tumor progression. The actuarial local tumor control rate was 95% after one year and 81% after two years. Three of the four patients with local recurrence were retreated with CK radiosurgery. Dynamic tumor tracking enabled accurate treatment with correlation errors less than 2 mm, despite extensive respiration-induced target motion up to 22 mm. Apart from nausea directly after treatment in five patients, we observed no early or late treatment-related side effects.
CONCLUSIONS: Single fraction robotic radiosurgery for adrenal gland metastases is a safe and effective treatment option for patients who are not eligible for surgical resection.

PMID: 28451479 [PubMed - in process]

Unique and novel urinary metabolomic features in malignant versus benign adrenal neoplasms.

Wed, 05/03/2017 - 22:26
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Unique and novel urinary metabolomic features in malignant versus benign adrenal neoplasms.

Clin Cancer Res. 2017 Apr 27;:

Authors: Patel D, Thompson MD, Manna SK, Krausz KW, Zhang L, Nilbuol N, Gonzalez FJ, Kebebew E

Abstract
Purpose: <p>Adrenal incidentalomas must be differentiated from adrenocortical cancer (ACC). Currently, size, growth, and imaging characteristics determine the potential for malignancy but are imperfect. The aim was to evaluate whether urinary small molecules (<800 Da) are associated with ACC.</p> <p>Experimental Design:</p> <p>Preoperative fasting urine specimens from patients with ACC (n=19) and benign adrenal tumors (n=46) were analyzed by unbiased ultra performance liquid chromatography/mass spectrometry. Creatinine-normalized features were analyzed by Progenesis, SIMCA, and unpaired t-test adjusted by false discovery rate. Features with an AUC >0.8 were identified through fragmentation patterns and database searches. All lead features were assessed in an independent set from patients with ACC (n=11) and benign adrenal tumors (n=46) and in a subset of tissue samples from patients with ACC (n=15) and benign adrenal tumors (n=15) in the training set.</p> <p>Results:</p> <p>Sixty-nine features were discovered and four known metabolites identified. Urinary creatine riboside was elevated 2.1-fold(p=0.0001) in patients with ACC. L-tryptophan, Nε,Nε,Nε-trimethyl-L-lysine and 3-methylhistidine were lower 0.33-fold(p<0.0001), 0.56-fold(p<0.0001), and 0.33-fold(p=0.0003) in patients with ACC, respectively. Combined multivariate analysis of the four biomarkers showed an AUC of 0.89 (sensitivity 94.7%(CI 73.9-99.1%), specificity 82.6%(CI 68.6-92.2%), PPV 69.2%(CI 48.2-85.6%), and NPV 97.4%(CI 86.5-99.6%)) for distinguishing ACC from benign tumors. Of the four, creatine riboside and four unknown features were validated. Creatine riboside, Nε,Nε,Nε-trimethyl-L-lysine, and two unknown features were elevated in ACC tumors.</p> <p>Conclusions:</p> <p>There are unique urinary metabolic features in patients with ACC with some metabolites present in patient tumor samples. Urinary creatine riboside can differentiate benign adrenal neoplasms from ACC.

PMID: 28450405 [PubMed - as supplied by publisher]

Analysis of treatment of large abdominal malignancies in children complicated with abdominal compartment syndrome: Report of six cases.

Wed, 05/03/2017 - 22:26
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Analysis of treatment of large abdominal malignancies in children complicated with abdominal compartment syndrome: Report of six cases.

Medicine (Baltimore). 2017 Apr;96(17):e6705

Authors: Le-Xiang Z, Yao-Hao W, Na L, Rong-Lin Q, Jia-Jia Z, Wen-Li J, Jie Z, Xiao-Geng D

Abstract
To explore effective treatment of large abdominal malignancies in children complicated with abdominal compartment syndrome (ACS).Six children with large abdominal malignancies complicated with ACS were admitted to our department from January 2013 to January 2016, and the changes in their breathing, heart rate, oxygen saturation, abdominal circumference, bladder pressure, and urine output, as well as the treatment measures and outcomes, were retrospectively analyzed.The 6 children included 1 child with bilateral nephroblastoma, 1 child with abdominal alveolar rhabdomyosarcoma, 1 child with right ovarian malignant teratoma complicated with abdominal glioma, 1 child with abdominal malignant teratoma, 1 child with right nephroblastoma, and 1 child with left adrenal gland neuroblastoma. All patients were treated in a timely manner. The first 4 children underwent abdominal cavity decompression through surgical resection of the tumor, and the ACS was successfully cured allowing for follow-up care, whereas the last 2 patients failed to receive emergency surgery and eventually died due to the gradual aggravation of ACS.Decompression through surgical resection of the tumor is the only effective measure for treating large abdominal malignancies in children complicated with ACS.

PMID: 28445278 [PubMed - in process]

A Case Report of Giant Adrenal Ganglioneuroma.

Wed, 05/03/2017 - 22:26
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A Case Report of Giant Adrenal Ganglioneuroma.

Urol Case Rep. 2017 Jul;13:37-38

Authors: Chen P, Lin C, Jin L, Ding Y, Peng J, Chen Z, Yang S, Mao X, Lai Y

Abstract
Adrenal ganglioneuroma (AGN) is an extremely rare and benign entity comprising Schwann cells and ganglion cells. If the lesion approaches to 6 cm, laparoscopic resection is considered as relative contraindication. Now we present a giant adrenal AGN in a 25-year-old male patient. After abdominal computed tomography (CT) scanning, the patient underwent an exploratory laparotomy with right adrenalectomy and histopathological examination further confirmed the lesions as giant AGN, which measured 7 cm × 5 cm × 3.5 cm. Because giant AGN with lesion size greater than 6 cm should be treated by open surgery, it is significant to report the laparoscopic adrenalectomy in patients with large adrenal lesions.

PMID: 28443238 [PubMed - in process]

Long-Term Outcomes of Adjuvant Mitotane Therapy in Patients With Radically Resected Adrenocortical Carcinoma.

Wed, 05/03/2017 - 22:26
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Long-Term Outcomes of Adjuvant Mitotane Therapy in Patients With Radically Resected Adrenocortical Carcinoma.

J Clin Endocrinol Metab. 2017 Apr 01;102(4):1358-1365

Authors: Berruti A, Grisanti S, Pulzer A, Claps M, Daffara F, Loli P, Mannelli M, Boscaro M, Arvat E, Tiberio G, Hahner S, Zaggia B, Porpiglia F, Volante M, Fassnacht M, Terzolo M

Abstract
Context: In 2007, a retrospective case-control study provided evidence that adjuvant mitotane prolongs recurrence-free survival (RFS) in patients with radically resected adrenocortical carcinoma (ACC).
Objective and Design: We aimed to confirm the prognostic role of adjuvant mitotane in the same series after 9 additional years of follow-up.
Setting, Patients, and Interventions: One hundred sixty-two ACC patients who did not recur or die after a landmark period of 3 months were considered. Forty-seven patients were enrolled in four Italian centers where adjuvant mitotane was routinely recommended (mitotane group), 45 patients in four Italian centers where no adjuvant strategy was undertaken (control group 1), and 70 German patients left untreated after surgery (control group 2).
Main Outcome Measures: The primary aim was RFS, the secondary was overall survival.
Results: An increased risk of recurrence was found in both control cohorts [group 1: hazard ratio (HR) = 2.98; 95% confidence interval (CI), 1.75 to 5.09; P < 0.0001; group 2: HR = 2.61; 95% CI, 1.56 to 4.36; P < 0.0001] compared with the mitotane group. The risk of death was higher in control group 1 (HR = 2.03; 95% CI, 1.17 to 3.51; P = 0.011) but not in control group 2 (HR = 1.60; 95% CI, 0.94 to 2.74; P = 0.083), which had better prognostic factors and more aggressive treatment of recurrences than control group 1. The benefit of adjuvant mitotane on RFS was observed regardless of the hormone secretory status.
Conclusions: Adjuvant mitotane is associated with prolonged RFS, without any apparent influence by the tumor secretory status. The retrospective nature of the study is a major limitation.

PMID: 28324035 [PubMed - indexed for MEDLINE]

Antiangiogenic or Corticosteroid Treatment in Patients With Radiation Maculopathy After Proton Beam Therapy for Uveal Melanoma.

Wed, 05/03/2017 - 22:26
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Antiangiogenic or Corticosteroid Treatment in Patients With Radiation Maculopathy After Proton Beam Therapy for Uveal Melanoma.

Am J Ophthalmol. 2016 Aug;168:31-9

Authors: Seibel I, Hager A, Riechardt AI, Davids AM, Böker A, Joussen AM

Abstract
PURPOSE: To reveal differences or advantages in regard to different treatment options after proton beam therapy for uveal melanoma.
DESIGN: Retrospective, comparative, interventional case series.
METHODS: All patients receiving intravitreal treatment between January 2011 and July 2014 for radiation maculopathy after proton beam therapy were included. Excluded were all patients who required re-irradiation, vitrectomies, or tumor resections; and those whose treatment was performed for potentially other reasons, such as radiation-induced optic neuropathy, or where visual outcome was influenced by tumor growth under the macula or macular ischemia. Minimum follow-up was 12 months after last injection.
RESULTS: Of 78 patients, 38 (48.7%) received bevacizumab injections, 35 (44.9%) triamcinolone acetonide injections, and 5 (6.4%) a dexamethasone implant. In the bevacizumab group visual acuity improved in 11 patients (28.9%) by 0.25 logMAR (0.1-0.4 logMAR) and remained stable in 24 patients (63.2%) 4 weeks after injection. In the triamcinolone group visual acuity showed improved outcomes in 10 patients (28.6%) by 0.25 logMAR (0.1-0.4 logMAR) and stability in function in 20 patients (57.1%). Four weeks after dexamethasone implantation visual acuity remained stable in 4 patients (80%). No differences among the groups were detected regarding functional outcome or reduction in central foveal thickness.
CONCLUSIONS: This study showed that antiangiogenic or corticosteroid intravitreal treatment led to reduced central foveal thickness and visual improvement in some patients without showing differences or advantages. Therefore a patient-specific treatment choice can be recommended.

PMID: 27163234 [PubMed - indexed for MEDLINE]

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