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NCBI: db=pubmed; Term=adrenal tumor
Updated: 21 hours 57 min ago

An exceedingly rare adrenal collision tumor: adrenal adenoma-metastatic breast cancer-myelolipoma.

Fri, 10/20/2017 - 20:52
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An exceedingly rare adrenal collision tumor: adrenal adenoma-metastatic breast cancer-myelolipoma.

J Community Hosp Intern Med Perspect. 2017 Oct;7(4):241-244

Authors: Liu D, Kumar SA

Abstract
Adrenal collision tumors (ACTs), in which distinct tumors coexist without histological intermingling in the same adrenal gland, are rare and their actual prevalence is unknown. ACTs commonly consist of adrenal cortical adenoma, myelolipoma, or metastatic malignant tumor. We report a 58-year-old woman with a past history of breast cancer, who presented with a 1 month history of fevers, chills, and abdominal fullness. The physical examination and the laboratory data including endocrine studies were unremarkable. Computed tomography of the abdomen showed a right adrenal gland mass, and a laparoscopic right adrenalectomy was performed. Histological and immunohistochemical examinations revealed three distinct tumors: an adrenal cortical adenoma, a myelolipoma, and metastatic breast tumors. Breast cancer metastases are rare in the adrenal gland and exist as ACTs only in exceptionally rare cases. To our knowledge, this is the first reported case of coexisting metastatic breast tumors, adrenal adenoma, and myelolipoma in the same adrenal gland.

PMID: 29046752 [PubMed]

Intraparenchymal Hemorrhage due to Brain Metastasis of Hepatocellular Carcinoma.

Fri, 10/20/2017 - 20:52
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Intraparenchymal Hemorrhage due to Brain Metastasis of Hepatocellular Carcinoma.

Case Rep Gastroenterol. 2017 Sep-Dec;11(3):516-525

Authors: Sartori Balbinot R, Facco Muscope AL, Dal Castel M, Sartori Balbinot S, Angelo Balbinot R, Soldera J

Abstract
Although extrahepatic metastases from hepatocellular carcinoma (HCC) are present in only 5-15% of cases, they are certainly factors associated with poor prognosis. The main sites include lung, lymph nodes, bones, and adrenal glands, in descending order. Metastasis in the central nervous system is extremely rare, and the incidences vary from 0.6 to 1.7%. We report a case of a 54-year-old man previously diagnosed with alcohol-induced cirrhosis of the liver and HCC. The patient was admitted presenting progressive left hemiparesis and headache which started 2 days earlier, with no history of cranioencephalic trauma. After admission, cranial computed tomography revealed an intraparenchymal hemorrhage area with surrounding edema in the right frontal lobe. An angioresonance requested showed a large extra-axial mass lesion located in the right frontal region with well-defined contours and predominantly hypointense signal on T2 sequence. At first, the radiological findings suggested meningioma as the first diagnostic hypothesis. However, the patient underwent surgery. The tumor was completely removed, and the morphological and immunohistochemical findings were consistent with metastatic hepatocarcinoma associated with meningioma. In postoperative care, the patient did not recover from the left hemiparesis and manifested Broca's aphasia. He had a survival time of 24 weeks, presenting acute liver failure as his cause of death. There is a lack of evidence supporting a specific management of patients with brain metastasis from HCC. Furthermore, there are no studies that evaluate different modalities of therapeutics in brain metastasis of HCC due to the rarity of this condition. Therefore, management must be individualized depending on probable prognostic factors in these patients.

PMID: 29033772 [PubMed]

Retinal Degeneration Protein 3 (RD3) in normal human tissues: Novel insights.

Fri, 10/20/2017 - 20:52
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Retinal Degeneration Protein 3 (RD3) in normal human tissues: Novel insights.

Sci Rep. 2017 Oct 13;7(1):13154

Authors: Aravindan S, Somasundaram DB, Kam KL, Subramanian K, Yu Z, Herman TS, Fung KM, Aravindan N

Abstract
The 195-amino-acid-long human Retinal Degeneration Protein 3 (RD3) is critical in the regulation of guanylate cyclase (GC) signaling and photoreceptor cell survival. Recently, we identified significant loss of RD3 in high-risk neuroblastoma and the influential role of RD3 in tumor progression. However, the functional characterization of RD3 in tumor systems has been hampered by the dearth of information on its localization in normal tissue and by the lack of antibodies suitable for staining FFPE tissue, primarily due to the inaccessibility of the epitopes. In this study, we validated a custom-synthesized RD3 antibody and investigated the expression/localization of RD3 in assorted human tissues. We observed stratified expression of RD3 in different cell types and subcellular location of retina. We demonstrated extensive positive RD3 immunoreactivity in various normal tissues and particularly strong dot-like perinuclear staining in the lining epithelial cells, suggesting that RD3 may play an important role in the normal functioning of epithelial cells. RD3 expression is limited in the CNS. While neuroblastoma is often RD3-positive, the adrenal medulla, where many neuroblastomas originate, is RD3-negative. Meta-analysis of RD3 transcriptional expression across normal tissues confirmed tissue-specific RD3 mRNA levels. Our results revealed the tissue-specific expression/localization profile of RD3 for the first time.

PMID: 29030614 [PubMed - in process]

New insights in the clinical and translational relevance of miR483-5p in adrenocortical cancer.

Fri, 10/20/2017 - 20:52
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New insights in the clinical and translational relevance of miR483-5p in adrenocortical cancer.

Oncotarget. 2017 Sep 12;8(39):65525-65533

Authors: Salvianti F, Canu L, Poli G, Armignacco R, Scatena C, Cantini G, Di Franco A, Gelmini S, Ercolino T, Pazzagli M, Nesi G, Mannelli M, Pinzani P, Luconi M

Abstract
Adrenocortical cancer (ACC) is a rare aggressive malignancy. Recent ACC integrated genomics analysis contributed to redefine the risk groups on molecular basis, including tumor microRNAs (miRs), detectable also in the bloodstream. We developed a quantitative real-time (RT) assay for the measurement of miR483 and miR483-5p absolute levels in plasma samples. miR483/miR483-5p levels were evaluated in plasma samples of 27 patients with ACC before surgery and at follow-up. Statistically significant differences in miR483-5p and miR483 levels were found between stage 1/2 and stage 3/4 ACCs in pre-surgery and post-surgery samples. ROC curve analysis of miR483-5p levels gave a prediction of the clinical stage (accuracy 0.917±0.084), with the best cut-off value of 0.221 ng/ml, prognosticating overall and recurrence-free survival. In a multivariate Cox analysis (HR 16.2, 95%CI[1.39-188.6, P<0.026]), miR483-5p was the only variable that significantly predicted recurrence, but not overall survival. In addition, miR483 and miR483-5p levels correlated with the number of circulating tumor cells (CTCs) detected in the same blood samples, independently of the timing of sampling. In conclusion, we demonstrated that miR483-5p absolute plasma levels in ACC patients are powerful molecular markers that may help in the follow-up of patients after surgery and chemotherapy, and contribute to more accurately classify and predict tumor progression.

PMID: 29029450 [PubMed]

Advances in understanding the molecular underpinnings of adrenocortical tumors.

Fri, 10/20/2017 - 20:52
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Advances in understanding the molecular underpinnings of adrenocortical tumors.

Curr Opin Oncol. 2017 Oct 11;:

Authors: Nicolson NG, Man J, Carling T

Abstract
PURPOSE OF REVIEW: Adrenocortical tumors are divided into benign adenomas and malignant carcinomas. The former is relatively common and carries a favorable prognosis, whereas the latter is rare and frequently presents at an advanced stage, with poor outcomes. Advances in next-generation sequencing, genome analysis, and bioinformatics have allowed for high-throughput molecular characterization of adrenal tumorigenesis.
RECENT FINDINGS: Although recent genomic, epigenomic, and transcriptomic studies in large tumor cohorts have confirmed the central roles of aberrant Wnt/ß-catenin signaling, constitutive protein kinase A pathway activation, cell cycle dysregulation, and ion channelopathies in adrenal tumorigenesis, these studies also revealed novel signature events underlying malignant differentiation of adrenocortical carcinomas.
SUMMARY: Recent advances in understanding of the molecular mechanisms underlying adrenocortical tumorigenesis provide new molecular diagnostic and prognostic tools and opportunities for novel therapeutic approaches. These findings are particularly important in adrenocortical carcinoma, for which current treatment options are limited.

PMID: 29028646 [PubMed - as supplied by publisher]

Zebrafish as a model to study neuroblastoma development.

Fri, 10/20/2017 - 20:52
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Zebrafish as a model to study neuroblastoma development.

Cell Tissue Res. 2017 Oct 13;:

Authors: Casey MJ, Stewart RA

Abstract
Neuroblastoma is a pediatric solid tumor arising from embryonic neural crest progenitor cells that normally generate the peripheral sympathetic nervous system. As such, the location of neuroblastoma tumors is correlated with the distribution of major post-ganglionic clusters throughout the sympathetic chain, with the highest incidence in the adrenal medulla or lumbar sympathetic ganglia (~65%). Neuroblastoma is an enigmatic tumor that can spontaneously regress with minimal treatment or become highly metastatic and develop resistance to aggressive treatments, including radiation and high-dose chemotherapy. Age of diagnosis, stage of disease and cellular and genetic features often predict whether the tumor will regress or advance to metastatic disease. Recent efforts using molecular and genomic technologies have allowed more accurate stratification of patients into low-, intermediate- and high-risk categories, thereby allowing for minimal intervention in low-risk patients and providing potential new therapeutic targets, such as the ALK receptor tyrosine kinase, for high-risk or relapsed patients. Despite these advances, the overall survival of high-risk neuroblastoma patients is still less than 50%. Furthermore, next-generation sequencing has revealed that almost two-thirds of neuroblastoma tumors do not contain obvious pathogenic mutations, suggesting that epigenetic mechanisms and/or a perturbed cellular microenvironment may heavily influence neuroblastoma development. Understanding the mechanisms that drive neuroblastoma, therefore, will likely require a combination of genomic, developmental and cancer biology approaches in whole animal systems. In this review, we discuss the contributions of zebrafish research to our understanding of neuroblastoma pathogenesis as well as the potential for this model system to accelerate the identification of more effective therapies for high-risk neuroblastoma patients in the future.

PMID: 29027617 [PubMed - as supplied by publisher]

Chromogranin A as a Biochemical Marker for Neuroendocrine Tumors: A Single Center Experience at Royal Hospital, Oman.

Fri, 10/20/2017 - 20:52
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Chromogranin A as a Biochemical Marker for Neuroendocrine Tumors: A Single Center Experience at Royal Hospital, Oman.

Oman Med J. 2017 Sep;32(5):365-370

Authors: Al-Risi ES, Al-Essry FS, Mula-Abed WS

Abstract
OBJECTIVES: To evaluate the significance of serum chromogranin A (CgA) status in patients with and without different neuroendocrine tumors (NETs) by conducting a retrospective assessment of the diagnostic utility and limitations of CgA as a biomarker for NETs in a tertiary care hospital in Oman.
METHODS: We conducted a retrospective analysis of CgA requests referred to the Clinical Biochemistry Laboratory, Royal Hospital, Oman over a 24-month period (April 2012 to March 2014). During this time, 302 CgA tests for 270 patients (119 males and 151 females; age range 11-86 years and mean±standard deviation (SD) 44.0±18.0 years), were requested. Of these CgA tests, 245 tests were performed for 245 patients investigated for the diagnosis of NETs, and 57 CgA tests were performed for 25 patients with diagnosed NETs who were undergoing follow-up. Serum CgA levels were analyzed using the enzyme-linked immunosorbent assay based on a cut-off value of 22 IU/L.
RESULTS: Of the 302 CgA tests reviewed, 197 (65.2%) were within the quoted normal range; however, 105 (34.8%) had CgA > 22 IU/L. Of the 245 patients with first-line CgA, 38 patients (15.5%) had NET that included carcinoid, pheochromocytoma, pancreatic NET, adrenal adenoma, prostatic adenocarcinoma, gastrointestinal NET, medullary thyroid carcinoma, Schwannoma, lung small cell carcinoma, parathyroid adenoma, and pituitary macroadenoma. The mean±SD of CgA in these patients with NETs was 205.0±172.0 IU/L. Meanwhile, there were 45 (18.3%) patients with CgA > 22 IU/L (83.0±116.0 IU/L) who did not have NETs. The conditions/diseases included: essential hypertension, chronic kidney disease, heart failure, peptic ulcer, chronic diarrhea, use of proton pump inhibitors, and other chronic diseases (hypothyroidism, asthma, diabetes mellitus). Of the 25 patients with known NET who were followed-up, there were 57 CgA results (29 with CgA ≤ 22 IU/L and 28 with CgA > 22 IU/L). The overall clinical sensitivity of CgA in the diagnosis of NETs was 84.2%, overall specificity was 78.2%, positive predictive value was 41.5%, negative predictive value was 96.4%, and overall efficiency was 79.2%. In patients with individual NET, a good reflection in CgA was noticed in the follow-up period following surgery or therapy.
CONCLUSIONS: Serum CgA is a sensitive and effective noninvasive laboratory test for the clinical detection and management of NETs. Awareness of the pitfalls of the tests in patients with non-NET conditions, particularly chronic diseases and use of certain drugs, is important to be considered during the interpretation of the CgA levels.

PMID: 29026467 [PubMed]

Lateral retroperitoneoscopic adrenalectomy for complicated adrenal tumor larger than 5 centimeters.

Fri, 10/20/2017 - 20:52
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Lateral retroperitoneoscopic adrenalectomy for complicated adrenal tumor larger than 5 centimeters.

Afr Health Sci. 2017 Mar;17(1):293-300

Authors: Chen W, Lin W, Han DJ, Liang Y

Abstract
BACKGROUND: The role of lateral retroperitoneoscopic adrenalectomy (LRA) for complicated tumor with large diameter remains controversial, this study aimed to evaluate the effectiveness of this procedure on the management of tumor larger than 5cm in diameter.
METHODS: A retrospective comparison was conducted of 67 patients with large complicated adrenal tumor (>5cm). 41 patients received LRA, and 26 received open adrenalectomy (OA) in our hospital between January 2011 and June 2015. Basic characteristics regarding mean age, gender, body mass index (BMI), tumor size, tumor side, previous abdominal surgery, resection method, pathology were preferentially analyzed. Operative indicators regarding operation time, estimated blood loss (EBL), conversion to ICU, complications, post-operative hospitalization, duration of drain, time to first oral intake and ambulation were compared between groups.
RESULTS: There were no significant differences between the two groups in the basic characteristics. The mean operation time for LRA was shorter than OA (98.7±32.3 min vs 152.7±72.3 min, P = 0.001). EBL was 31.9±20.0 ml for LRA and 590.0±1181.1 ml for OA (P = 0.03). There was no complication in LRA group and one patient in OA group had complications, but this difference was not significant (P = NS). The post-operative hospitalization in LRA was 7.4±2.8 days, and shorter than 9.8±2.7 days in OA group (P = 0.00). The time to first oral intake and ambulation for LRA was shorter than OA (first oral intake, 1.9±0.8 days vs 3.1±1.3 days, P = 0.00; time to ambulation, 2.6±1.4 days vs 4.2±1.6 days, P = 0.00). While the difference between groups were not significant in terms of ICU conversion (3/41 vs 4/26, P = NS) and duration of drain (3.9±2.2 days vs 4.7±1.9 days, P = NS).
CONCLUSION: Our study shows that LRA can be performed safely and effectively for complicated adrenal tumors larger than 5 cm in diameter, but it remains technically demanding.

PMID: 29026405 [PubMed - in process]

Endocrine side effects of cancer immunotherapy.

Fri, 10/20/2017 - 20:52
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Endocrine side effects of cancer immunotherapy.

Endocr Relat Cancer. 2017 Oct 12;:

Authors: Cukier P, Santini FC, Scaranti M, Hoff AO

Abstract
Immune checkpoint inhibitors have recently become a cornerstone for the treatment of different advanced cancers. These drugs, represented mainly by monoclonal antibodies anti-cytotoxic T-lymphocyte antigen 4 (CTLA-4), anti-programmed cell death protein-1 (PD-1) and anti-PD-1 ligand molecules (PD-L1 and L2), have the ability to reactivate the immune system against tumor cells, but can also trigger a myriad of autoimmune side effects, termed immune-related adverse events (irAEs). In particular, there are a number of endocrine related irAEs. Current data from clinical trials show increased incidence of hypophysitis with CTLA4 inhibition and thyroid dysfunction with PD-(L)1 blockade. In addition, a few cases of type 1 Diabetes Mellitus and primary adrenal insufficiency have been reported. We discuss the incidence, clinical manifestations, diagnosis and management of immune-related endocrinopathies in this highly complex context of oncological patients in need of immunotherapies.

PMID: 29025857 [PubMed - as supplied by publisher]

Bortezomib Alone and in Combination With Salinosporamid A Induces Apoptosis and Promotes Pheochromocytoma Cell Death In Vitro and in Female Nude Mice.

Fri, 10/20/2017 - 20:52
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Bortezomib Alone and in Combination With Salinosporamid A Induces Apoptosis and Promotes Pheochromocytoma Cell Death In Vitro and in Female Nude Mice.

Endocrinology. 2017 Oct 01;158(10):3097-3108

Authors: Bullova P, Cougnoux A, Marzouca G, Kopacek J, Pacak K

Abstract
Proteasome inhibitors have been frequently used in treating hematologic and solid tumors. They are administered individually or in combination with other regimens, to prevent severe side effects and resistance development. Because they have been shown to be efficient and are pharmaceutically available, we tested the first Food and Drug Administration-approved proteasome inhibitor bortezomib alone and in combination with another proteasome inhibitor, salinosporamid A, in pheochromocytoma cells. Pheochromocytomas/Paragangliomas (PHEOs/PGLs) are neuroendocrine tumors for which no definite cure is yet available. Therefore, drugs with a wide spectrum of mechanisms of action are being tested to identify suitable candidates for PHEO/PGL treatment. In the current study, we show that bortezomib induces PHEO cell death via the apoptotic pathway in vitro and in vivo. The combination of bortezomib with salinosporamid A exhibits additive effect on these cells and inhibits proliferation, cell migration and invasion, and angiogenesis more potently than bortezomib alone. Altogether, we suggest these proteasome inhibitors, especially bortezomib, could be potentially tested in PHEO/PGL patients who might benefit from treatment with either the inhibitors alone or in combination with other treatment options.

PMID: 28938421 [PubMed - indexed for MEDLINE]

Secondary Adrenal Insufficiency Following Nivolumab Therapy in a Patient with Metastatic Renal Cell Carcinoma.

Fri, 10/20/2017 - 20:52
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Secondary Adrenal Insufficiency Following Nivolumab Therapy in a Patient with Metastatic Renal Cell Carcinoma.

Tokai J Exp Clin Med. 2017 Sep 20;42(3):115-120

Authors: Seki T, Yasuda A, Oki M, Kitajima N, Takagi A, Nakajima N, Miyajima A, Fukagawa M

Abstract
Currently, nivolumab (an anti-programmed cell death-1 receptor monoclonal antibody) is available for many types of advanced cancers in Japan. However, there have been few detailed case reports about endocrine-related adverse events of this therapy. Here, we report a patient with metastatic renal cell carcinoma who presented with secondary adrenal insufficiency following nivolumab therapy. Endocrinological assessment by rapid adrenocorticotropic hormone (ACTH) and corticotropin-releasing hormone (CRH) tests revealed that the patient's disorder was a secondary adrenal insufficiency due to pituitary dysfunction. Moreover, the results of the thyrotropin-releasing hormone (TRH), luteinizing hormone-releasing hormone (LH-RH) and growth hormone-releasing peptide-2 (GHRP-2) tests showed that only the ACTH function was destroyed (isolated ACTH deficiency). The magnetic resonance imaging (MRI) findings of hypophysitis, which is the major cause of isolated ACTH deficiency, usually demonstrate enlargement of the pituitary gland. However, the MRI findings of our case showed no abnormalities of the pituitary gland and stalk. Therefore, not only oncologists, but also other specialists, including doctors in emergency units, should have knowledge of this specific feature. Our clinical observation could be useful to avoid a delay in diagnosis and to treat life-threatening adverse effects of nivolumab therapy, such as secondary adrenal insufficiency.

PMID: 28871578 [PubMed - indexed for MEDLINE]

Rottlerin as a novel chemotherapy agent for adrenocortical carcinoma.

Fri, 10/20/2017 - 20:52
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Rottlerin as a novel chemotherapy agent for adrenocortical carcinoma.

Oncotarget. 2017 Apr 04;8(14):22825-22834

Authors: Zhu Y, Wang M, Zhao X, Zhang L, Wu Y, Wang B, Hu W

Abstract
Adrenocortical carcinoma (ACC) is a rare, but aggressive endocrine malignancy with a generally poor clinical outcome. There is no effective therapy for advanced and metastatic ACC. In our study, we found that an existing drug (rottlerin) exerted its tumour-suppressive function in ACC. Specifically, rottlerin inhibited cellular proliferation of ACC cell lines (NCI-H295R and SW-13) in a dose- and time-dependent manner. We also found that rottlerin induced cell apoptosis and promoted G0/G1 cell cycle arrest in ACC cell lines. The cellular migration and invasion of ACC cell lines were decreased after treatment with rottlerin. Further, the molecular expression of lipoprotein receptor related protein 6 (LRP6) and β-catenin were down-regulated in rottlerin-treated ACC cells, which indicated that Wnt/β-catenin signaling was involved in the tumour-suppressive function of rottlerin. To further confirm the anti-tumour function of rottlerin, a nude mouse ACC xenograft model was used. The xenograft growth curves and TUNEL assays demonstrated that rottlerin inhibited proliferation and induced apoptosis in the ACC xenograft model. Furthmore, we verified that rottlerin down-regulated the expression of LRP6 and β-catenin in vivo. The ACC cell line and xenograft mouse model data indicated that rottlerin significantly inhibited proliferation and induced apoptosis of ACC cells, likely via suppression of the Wnt/β-catenin signaling pathway. Our study indicated the potential therapeutic utility of rottlerin as a novel and potential chemotherapeutic agent for ACC.

PMID: 28423559 [PubMed - indexed for MEDLINE]

Tumor necrosis factor antagonist-induced psoriasis in a 3-year-old boy with Kawasaki disease.

Fri, 10/20/2017 - 20:52
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Tumor necrosis factor antagonist-induced psoriasis in a 3-year-old boy with Kawasaki disease.

Dermatol Online J. 2017 Feb 15;23(2):

Authors: Geller L, Kellen R

Abstract
Tumor necrosis factor (TNF) antagonists have beenknown to trigger new-onset psoriasis in adult andpediatric patients. Here we report a case of TNFantagonist-induced psoriasis in a 3-year-old boytreated with infliximab for Kawasaki disease. Ourpatient is a 3-year-old boy with Kawasaki diseaseunresponsive to intravenous immunoglobulinwho was then treated with one dose of infliximab.A few days later he developed psoriatic plaqueson the face and extremities. The psoriatic plaqueswere treated with topical calcineurin inhibitors andtopical corticosteroids, with marked improvement.Prior reports of TNF antagonist-induced psoriasis inthe pediatric population have been in children withinflammatory bowel disease or juvenile idiopathicarthritis. To the best of our knowledge, this is thefirst case of TNF antagonist-induced psoriasis ina pediatric patient with Kawasaki disease, andthe youngest patient to date. Although we donot fully understand the mechanism behind thisphenomenon, in vitro studies have implicated theimportance of interferon-α, a pro-inflammatorycytokine, and plasmacytoid dendritic cells. Furtherresearch is necessary to understand who is at riskfor this condition and the molecular basis for thisparadoxical reaction.

PMID: 28329508 [PubMed - indexed for MEDLINE]

Primary aldosteronism: from case detection to histopathology with up to 6 years of follow-up.

Fri, 10/20/2017 - 20:52
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Primary aldosteronism: from case detection to histopathology with up to 6 years of follow-up.

J Clin Hypertens (Greenwich). 2017 Apr;19(4):424-430

Authors: Jonsdottir G, Gudmundsson J, Birgisson G, Sigurjonsdottir HA

Abstract
The authors aimed to investigate the clinical characteristics, accuracy of diagnostic tests, and long-term outcomes after interventions in patients diagnosed with primary aldosteronism (PA) in Iceland throughout 5 years. A retrospective chart review was performed for all patients diagnosed with PA during the years 2007-2011 at Landspitali Hospital in Iceland, a referral center for the whole country. Workup after detection included salt loading test, positional test, computed tomography, and adrenal vein sampling. Patients with unilateral disease were offered treatment with adrenalectomy. A total of 33 patients were diagnosed with PA during the study period: 17 patients with bilateral disease and 16 with unilateral disease. Results from salt loading test were positive in 90% of patients. In patients with adenoma, 36% were responsive on their positional test and computed tomography scan showed a nodule in 73%. All patients with unilateral disease had a lateralization index ≥3. After surgery, patients had lower systolic blood pressure (P<.001) and number of hypertensive medications (P<.01).

PMID: 27878955 [PubMed - indexed for MEDLINE]

S2k guideline for treatment of cutaneous lupus erythematosus - guided by the European Dermatology Forum (EDF) in cooperation with the European Academy of Dermatology and Venereology (EADV).

Fri, 10/20/2017 - 20:52
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S2k guideline for treatment of cutaneous lupus erythematosus - guided by the European Dermatology Forum (EDF) in cooperation with the European Academy of Dermatology and Venereology (EADV).

J Eur Acad Dermatol Venereol. 2017 Mar;31(3):389-404

Authors: Kuhn A, Aberer E, Bata-Csörgő Z, Caproni M, Dreher A, Frances C, Gläser R, Klötgen HW, Landmann A, Marinovic B, Nyberg F, Olteanu R, Ranki A, Szepietowski JC, Volc-Platzer B

Abstract
Cutaneous lupus erythematosus (CLE) is a rare inflammatory autoimmune disease with heterogeneous clinical manifestations. To date, no therapeutic agents have been licensed specifically for patients with this disease entity, and topical and systemic drugs are mostly used 'off-label'. The aim of the present guideline was to achieve a broad consensus on treatment strategies for patients with CLE by a European subcommittee, guided by the European Dermatology Forum (EDF) and supported by the European Academy of Dermatology and Venereology (EADV). In total, 16 European participants were included in this project and agreed on all recommendations. Topical corticosteroids remain the mainstay of treatment for localized CLE, and further topical agents, such as calcineurin inhibitors, are listed as alternative first-line or second-line topical therapeutic option. Antimalarials are recommended as first-line and long-term systemic treatment in all CLE patients with severe and/or widespread skin lesions, particularly in patients with a high risk of scarring and/or the development of systemic disease. In addition to antimalarials, systemic corticosteroids are recommended as first-line treatment in highly active and/or severe CLE. Second- and third-line systemic treatments include methotrexate, retinoids, dapsone and mycophenolate mofetil or mycophenolate acid, respectively. Thalidomide should only be used in selected therapy-refractory CLE patients, preferably in addition to antimalarials. Several new therapeutic options, such as B-cell- or interferon α-targeted agents, need to be further evaluated in clinical trials to assess their efficacy and safety in the treatment of patients with CLE.

PMID: 27859683 [PubMed - indexed for MEDLINE]

Isolated local recurrence or solitary solid organ metastasis after esophagectomy for cancer is not the end of the road.

Fri, 10/20/2017 - 20:52
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Isolated local recurrence or solitary solid organ metastasis after esophagectomy for cancer is not the end of the road.

Dis Esophagus. 2017 Jan 01;30(1):1-8

Authors: Depypere L, Lerut T, Moons J, Coosemans W, Decker G, Van Veer H, De Leyn P, Nafteux P

Abstract
Recurrent disease after esophagectomy bears an infaust prognosis, especially when multiple recurrences are present. But little is known about survival in patients with limited recurrence (solitary locoregional recurrence or solid organ metastasis). Herein, we report our experience with these subgroups. We analyzed 1754 consecutive patients surgically treated with curative resection for esophageal cancer and cancer of the gastroesophageal junction between 1990 and 2012. Seven subgroups were defined according to the recurrence type (locoregional vs. organ metastasis), the site of recurrence (abdominal, thoracic, cervical for lymph nodes and lung, liver, adrenals and others for organ metastasis) and also the number of lesions (one vs. multiple lymph node stations or organ metastasis) Of these groups; clinical isolated locoregional recurrence (ciLR) was defined as solitary lymph-node recurrence confined to one compartment (cervical, thoracic or abdominal, within or outside surgical dissection-field) at clinical staging. Clinical solitary solid organ metastasis (csSOM) was defined as metastasis in a resectable solid organ, i.e. liver, lung, brain or adrenal. Salvage therapies were grouped in five categories. Kaplan-Meier curves were used to calculate survival. Recurrent disease was observed in 766 patients (43.7%) with overall 5-year survival of 4.5% after diagnosis of recurrence. Fifty-seven patients (7.4%) showed ciLR and 110 (14.4%) csSOM. Median time-to-recurrence was 16.8 months in ciLR and 9.9 months in csSOM (P = 0.0074). Survival is significantly improved compared to supportive therapy when local therapy is possible (P < 0.0001). In 25 (15%) of ciLR or csSOM patients, surgical therapy with or without systemic therapy, yielded a 5-year survival of 49.9% (median 54.8 months) after diagnosis of recurrence. When surgery was impossible or contraindicated, the combination of chemoradiotherapy appeared to be superior to chemotherapy alone (respectively 27.0% vs. 4.6% 5-year survival) or radiotherapy alone (no 5-year survival). Recurrent disease after esophagectomy is a common problem with poor overall survival. However prolonged survival could be obtained in selected patients if the recurrent disease is limited to ciLR or csSOM, if surgery (+/- systemic therapy) can be performed. If not a combination of chemoradiotherapy seems to offer the second best option. Patients presenting with a ciLR or csSOM should be discussed in a dedicated multidisciplinary team meeting as to evaluate and define the place of salvage treatment which in well selected cases could offer a perspective of prolonged survival.

PMID: 27704661 [PubMed - indexed for MEDLINE]

Eczema as an adverse effect of anti-TNFα therapy in psoriasis and other Th1-mediated diseases: a review.

Fri, 10/20/2017 - 20:52
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Eczema as an adverse effect of anti-TNFα therapy in psoriasis and other Th1-mediated diseases: a review.

J Dermatolog Treat. 2017 May;28(3):237-241

Authors: Nakamura M, Lee K, Singh R, Zhu TH, Farahnik B, Abrouk M, Koo J, Bhutani T

Abstract
INTRODUCTION: There have been rare reports of eczema occurring as an adverse effect of anti-tumor necrosis factor-alpha (TNFα) therapy.
METHODS: A literature search was conducted on PubMed for articles describing new onset or worsening of preexisting eczema during anti-TNFα therapy for the treatment of various inflammatory diseases.
RESULTS: Eczema as an adverse effect of anti-TNFα therapy may occur in approximately 5-20% of patients with various Th1-mediated inflammatory diseases such as psoriasis, inflammatory arthritis and inflammatory bowel disease. Personal history of atopy appears to increase this risk. Out of the anti-TNFα agents indicated for the treatment of moderate-to-severe psoriasis, infliximab may be more strongly associated with development or exacerbation of preexisting eczema.
DISCUSSION: Inhibitors of key mediators in the Th1 pathway such as TNFα are successful therapeutic targets for the treatment of various inflammatory conditions such as psoriasis, psoriatic arthritis, rheumatoid arthritis and inflammatory bowel disease. Blocking the Th1 pathway may create an imbalance favoring increased activity of the opposing Th2 pathway implicated in inflammatory conditions such as eczema. Further research is needed to better understand the role of the Th1/Th2 balance in various inflammatory diseases and how the immunologic environment is affected by immunotherapies.

PMID: 27571340 [PubMed - indexed for MEDLINE]

Minimally Invasive Adrenalectomy for Adrenocortical Carcinoma: Five-Year Trends and Predictors of Conversion.

Fri, 10/13/2017 - 18:03
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Minimally Invasive Adrenalectomy for Adrenocortical Carcinoma: Five-Year Trends and Predictors of Conversion.

World J Surg. 2017 Oct 11;:

Authors: Calcatera NA, Hsiung-Wang C, Suss NR, Winchester DJ, Moo-Young TA, Prinz RA

Abstract
BACKGROUND: Adrenocortical carcinoma (ACC) is rare but often fatal. Surgery offers the only chance of cure. As minimally invasive (MI) procedures for cancer become common, their role for ACC is still debated. We reviewed usage of MI approaches for ACC over time and risk factors for conversion using a large national database.
METHODS: ACC patients with localized disease were identified in the National Cancer Data Base from 2010 to 2014. A retrospective review examined trends in the surgical approach over time. Patient demographics, surgical approach, and tumor characteristics between MI, open, and converted procedures were compared.
RESULTS: 588 patients underwent adrenalectomy for ACC, of which 200 were minimally invasive. From 2010 to 2014, MI operations increased from 26 to 44% with robotic procedures increasing from 5 to 16%. The use of MI operations compared to open was not different based on facility type (p = 0.40) or location (p = 0.63). MI tumors were more likely to be confined to the adrenal (p < 0.001) but final margin status was not different (p = 0.56). Conversion was performed in 38/200 (19%). Average tumor size was 10.2 cm in the converted group compared to 8.6 cm in the MI group (p = 0.09). There was no difference in extent of disease (p = 0.33), margin status (p = 0.12), or lymphovascular invasion (p = 0.59) between MI and converted procedures. Tumor size > 5 cm was the only significant predictor of conversion (p = 0.04). No patients with pathologic stage I disease required conversion (0/19).
CONCLUSIONS: The frequency of MI approaches for ACC is increasing. In the final year of the study, 44% of adrenalectomies were MI. Size > 5 cm was the only significant predictor of conversion.

PMID: 29022106 [PubMed - as supplied by publisher]

Imaging of Nonmalignant Adrenal Lesions in Children.

Fri, 10/13/2017 - 18:03
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Imaging of Nonmalignant Adrenal Lesions in Children.

Radiographics. 2017 Oct;37(6):1648-1664

Authors: Sargar KM, Khanna G, Hulett Bowling R

Abstract
The adrenal glands in children can be affected by a variety of benign lesions. The diagnosis of adrenal lesions can be challenging, but assessment of morphologic changes in correlation with the clinical presentation can lead to an accurate diagnosis. These lesions can be classified by their cause: congenital (eg, discoid adrenal gland, horseshoe adrenal gland, and epithelial cysts), vascular and/or traumatic (eg, adrenal hemorrhage), infectious (eg, granulomatous diseases), enzyme deficiency disorders (eg, congenital adrenal hyperplasia [CAH] and Wolman disease), benign neoplasms (eg, pheochromocytomas, ganglioneuromas, adrenal adenomas, and myelolipomas), and adrenal mass mimics (eg, extralobar sequestration and extramedullary hematopoiesis). Multimodality cross-sectional imaging helps to define the origin, extent, and relationship of these lesions to adjacent structures, as well as to guide treatment management. The anatomic and functional imaging modalities used to evaluate pediatric adrenal lesions include ultrasonography, computed tomography (CT), magnetic resonance imaging, and iodine 123 metaiodobenzylguanidine scintigraphy. Identifying the imaging features of nonmalignant adrenal lesions is helpful to distinguish these lesions from malignant adrenal neoplasms. Identifying characteristic imaging findings (eg, enlarged adrenal glands, with cerebriform surface, and stippled echogenicity in CAH; a T2-hyperintense mass with avid contrast enhancement in pheochromocytoma; low CT attenuation [<10 HU] and signal intensity drop on opposed-phase chemical shift images in adenoma; and enhancing suprarenal mass supplied by a systemic feeding artery in extralobar sequestration) can aid in making the correct diagnosis. In addition, clinical features (eg, ambiguous genitalia in CAH and hypertension in pheochromocytoma) can also guide the radiologist toward the correct diagnosis. (©)RSNA, 2017.

PMID: 29019745 [PubMed - in process]

Microenvironment-Driven Shift of Cohesion/Detachment Balance within Tumors Induces a Switch toward Metastasis in Neuroblastoma.

Fri, 10/13/2017 - 18:03
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Microenvironment-Driven Shift of Cohesion/Detachment Balance within Tumors Induces a Switch toward Metastasis in Neuroblastoma.

Cancer Cell. 2017 Oct 09;32(4):427-443.e8

Authors: Delloye-Bourgeois C, Bertin L, Thoinet K, Jarrosson L, Kindbeiter K, Buffet T, Tauszig-Delamasure S, Bozon M, Marabelle A, Combaret V, Bergeron C, Derrington E, Castellani V

Abstract
Neuroblastoma (NB) is a childhood cancer arising from sympatho-adrenal neural crest cells. Disseminated forms have high frequency of multiple tumoral foci whose etiology remains unknown; NB embryonic origin limits investigations in patients and current models. We developed an avian embryonic model driving human NB tumorigenesis in tissues homologous to patients. We found that aggressive NBs display a metastatic mode, secondary dissemination via peripheral nerves and aorta. Through tumor transcriptional profiling, we found that NB dissemination is induced by the shutdown of a pro-cohesion autocrine signal, SEMA3C, which constrains the tumoral mass. Lowering SEMA3C levels shifts the balance toward detachment, triggering NB cells to collectively evade the tumor. Together with patient cohort analysis, this identifies a microenvironment-driven pro-metastatic switch for NB.

PMID: 29017055 [PubMed - in process]

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