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Sympathetic hearing loss: A review of current understanding and report of 2 cases.

Wed, 05/03/2017 - 22:26
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Sympathetic hearing loss: A review of current understanding and report of 2 cases.

Ear Nose Throat J. 2016 Apr-May;95(4-5):166-92

Authors: Morgan PF, Volsky PG, Strasnick B

Abstract
Sympathetic hearing loss (SHL) is a rare complication that appears to occur when the immune system is primed against sequestered inner ear antigens. SHL has been implicated in delayed hearing loss after acoustic tumor removal, revision stapedectomy, and temporal bone fractures. We present 2 cases of suspected SHL after excision of skull base tumors during which the surgical approach had violated the otic capsule. Both patients experienced delayed contralateral sensorineural hearing loss. In comparing our cases with those previously reported, we hope to inspire others to do likewise and to develop an understanding of this clinical entity.

PMID: 27140016 [PubMed - indexed for MEDLINE]

Corticosteroid-Induced MKP-1 Represses Pro-Inflammatory Cytokine Secretion by Enhancing Activity of Tristetraprolin (TTP) in ASM Cells.

Wed, 05/03/2017 - 22:26
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Corticosteroid-Induced MKP-1 Represses Pro-Inflammatory Cytokine Secretion by Enhancing Activity of Tristetraprolin (TTP) in ASM Cells.

J Cell Physiol. 2016 Oct;231(10):2153-8

Authors: Prabhala P, Bunge K, Ge Q, Ammit AJ

Abstract
Exaggerated cytokine secretion drives pathogenesis of a number of chronic inflammatory diseases, including asthma. Anti-inflammatory pharmacotherapies, including corticosteroids, are front-line therapies and although they have proven clinical utility, the molecular mechanisms responsible for their actions are not fully understood. The corticosteroid-inducible gene, mitogen-activated protein kinase (MAPK) phosphatase 1 (MKP-1, DUSP1) has emerged as a key molecule responsible for the repressive effects of steroids. MKP-1 is known to deactivate p38 MAPK phosphorylation and can control the expression and activity of the mRNA destabilizing protein-tristetraprolin (TTP). But whether corticosteroid-induced MKP-1 acts via p38 MAPK-mediated modulation of TTP function in a pivotal airway cell type, airway smooth muscle (ASM), was unknown. While pretreatment of ASM cells with the corticosteroid dexamethasone (preventative protocol) is known to reduce ASM synthetic function in vitro, the impact of adding dexamethasone after stimulation (therapeutic protocol) had not been explored. Whether dexamethasone modulates TTP in a p38 MAPK-dependent manner in this cell type was also unknown. We address this herein and utilize an in vitro model of asthmatic inflammation where ASM cells were stimulated with the pro-asthmatic cytokine tumor necrosis factor (TNF) and the impact of adding dexamethasone 1 h after stimulation assessed. IL-6 mRNA expression and protein secretion was significantly repressed by dexamethasone acting in a temporally distinct manner to increase MKP-1, deactivate p38 MAPK, and modulate TTP phosphorylation status. In this way, dexamethasone-induced MKP-1 acts via p38 MAPK to switch on the mRNA destabilizing function of TTP to repress pro-inflammatory cytokine secretion from ASM cells. J. Cell. Physiol. 231: 2153-2158, 2016. © 2016 Wiley Periodicals, Inc.

PMID: 26825339 [PubMed - indexed for MEDLINE]

Leydig cell tumour and giant adrenal myelolipoma associated with adrenogenital syndrome: a case report with a review of the literature.

Wed, 05/03/2017 - 22:26
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Leydig cell tumour and giant adrenal myelolipoma associated with adrenogenital syndrome: a case report with a review of the literature.

Urologia. 2016 Jan-Mar;83(1):43-8

Authors: Altieri VM, Altieri B, Castellucci R, Alba S, Bottone F, Fragalà E, Mirone V, Greco F

Abstract
CONTEXT: Male patients with congenital adrenal hyperplasia (CAH) may develop bilateral testicular adrenal rest tumours (TARTs). These tumours, in most cases, regress with glucocorticoid therapy and their histological differentiation from Leydig-cell tumors is quite difficult.
OBJECTIVE: The aim of this study was to differentiate the histological and clinical features of the TARTs from those of the Leydig-cell tumours.
METHODS: The authors report a case of bilateral Leydig-cell tumour associated with giant bilateral adrenal myelolipoma in a male with adrenogenital syndrome who was submitted to a bilateral orchiectomy.
RESULTS: Testicular neoplasia continue to grow despite adequate hormonal treatment and a bilateral orchiectomy was performed. The histopathological examination of the specimen ultimately confirmed the diagnosis of bilateral Leydig-cell tumour.
CONCLUSIONS: This case shows the importance of all the relevant examinations, blood chemistry analysis, and instrumental tests in the differential diagnosis of TARTs and other testicular tumours.

PMID: 26660266 [PubMed - indexed for MEDLINE]

Cushing's syndrome associated with an adrenocortical oncocytoma.

Wed, 05/03/2017 - 22:26
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Cushing's syndrome associated with an adrenocortical oncocytoma.

Endocrinol Nutr. 2015 Oct;62(8):416-8

Authors: Calderón Vicente DM, Ronquillo Rubio A, Yunta Abarca P, Quílez Toboso R, Aranda Regules J

PMID: 26066092 [PubMed - indexed for MEDLINE]

[Biological agents in ulcerative colitis. When to prescribe].

Wed, 05/03/2017 - 22:26
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[Biological agents in ulcerative colitis. When to prescribe].

Gastroenterol Hepatol. 2011 Dec;34 Suppl 3:36-42

Authors: Barreiro-de Acosta M

Abstract
Biological agents are effective both in inducing and in maintaining remission in patients with ulcerative colitis. These drugs began to be used later in ulcerative colitis than in Crohn's disease because, in addition to the possibility of surgery, other therapeutic options such as cyclosporine were available for the latter disease. Most studies and data concern infliximab, the only currently approved biological agent; however, the less than entirely satisfactory results of the first studies could also have delayed the use of this drug in Crohn's disease. Due to the results of a Scandinavian study, infliximab was initially indicated in severe, corticosteroid-resistant ulcerative colitis. Subsequently, numerous open series and observational studies have confirmed the efficacy of this drug in this subgroup of patients. However, after several years of using infliximab in colitis, clinicians are increasingly aware that its efficacy is greater in patients with corticosteroid-dependent, moderate-severe disease not requiring hospitalization than in hospitalized patients with severe disease. In the last few years, data have emerged to support the efficacy of infliximab in certain areas of ulcerative colitis refractory to other therapies, such as pediatric patients and reservoiritis. Recently, new data on another biological agent (adalimumab) in this disease have been published. The evidence indicates that this drug will soon be indicated in the treatment of ulcerative colitis.

PMID: 25443225 [PubMed - indexed for MEDLINE]

[No best treatment for severe outbreaks: Maintenance, the key in colitis].

Wed, 05/03/2017 - 22:26
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[No best treatment for severe outbreaks: Maintenance, the key in colitis].

Gastroenterol Hepatol. 2011 Dec;34 Suppl 3:18-24

Authors: Sicilia B

Abstract
Several drugs are currently available to maintain remission in patients who have responded after one or other type of induction therapy, depending on the initial severity of the outbreak. Salicylates are the drugs of choice to maintain remission after a mild-to-moderate outbreak controlled by salicylates or oral corticosteroids. To maintain remission after a severe outbreak or in patients with corticosteroid dependence or resistance, thiopurines are the drugs of choice. In patients who have failed to respond to thiopurines and in those with thiopurine intolerance, biological agents, mainly infliximab, can be used to maintain remission in patients after induction therapy with infliximab for a severe outbreak. However, these scenarios may not reflect reality of gastroenterologists' daily clinical practice. Treatment will therefore be based on the patient's individual characteristics (age, clinical course, previous treatment, adverse effects and personal preferences) as well as the physician's medical art.

PMID: 25443222 [PubMed - indexed for MEDLINE]

Primary Adrenal Angiosarcoma: A Rare and Potentially Misdiagnosed Tumor.

Wed, 04/26/2017 - 19:25
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Primary Adrenal Angiosarcoma: A Rare and Potentially Misdiagnosed Tumor.

Cancer Control. 2017 Apr;24(2):198-201

Authors: Grajales-Cruz A, Baco-Viera F, Rive-Mora E, Ramirez-Tanchez C, Tasso D, Arroyo-Portela N, Calderon E, Padua-Octaviani IJ, Caceres-Perkins W

Abstract
A man aged 69 years presented with acute right flank pain secondary to a hemorrhagic large adrenal tumor. En bloc resection was performed to repair the inferior vena cava. Immunoperoxidase levels in the tumor were positive for factor VIII and CD31 and negative for S100, protein Melan-A, CD34, synaptophysin, chromogranin, desmin, muscle specific actin, ETFA (EMA), KRT20 (CK20), CDX2, TTF1, LNPEP (PLAP), inhibin, ?-fetoprotein, CD30, hepatocyte paraffin, and aberrant expression of cytokeratin 7 and pankeratin. The pathological diagnosis was consistent with adrenal angiosarcoma. Obtaining appropriate immunoperoxidase stains and multidisciplinary evaluation helped make the diagnosis of this rare adrenal tumor and determine its management. The patient had an uneventful postoperative course and completed 4 cycles of adjuvant chemotherapy with doxorubicin/ifosfamide and adequately tolerated the treatment. However, positive surgical margins were found, so he was referred to radiation oncology specialists for possible adjuvant radiotherapy to the surgical bed. Weeks after the first initiation of therapy, the patient presented to the emergency department complaining of shortness of breath, fatigue, and generalized weakness for 3 days. He was admitted and found to have new-onset anemia and a new-onset, large, right pleural effusion. Thoracentesis performed showed sanguinolent fluid that, after microscopic evaluation, was suggestive of recurrent malignancy. Thoracic aortography performed with subselective catheterization to several arteries (right bronchial, right phrenic, and right renal arteries) did not show any active bleeding. However, the right inferior intercostal and adrenal arteries were presumed to be the reason for the bleeding event, so they were embolized until stasis. The patient remained hemodynamically unstable but eventually experienced multiorgan failure. In spite of aggressive measures, he died 10 days after admission to the hospital.

PMID: 28441375 [PubMed - in process]

Surgical Management of Non-small Cell Lung Cancer with Solitary Hematogenous Metastases.

Wed, 04/26/2017 - 19:25
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Surgical Management of Non-small Cell Lung Cancer with Solitary Hematogenous Metastases.

In Vivo. 2017 May-Jun;31(3):451-454

Authors: Karagkiouzis G, Spartalis E, Moris D, Patsouras D, Athanasiou A, Karathanasis I, Verveniotis A, Konstantinou F, Kouerinis IA, Potaris K, Dimitroulis D, Tomos P

Abstract
BACKGROUND/AIM: The treatment of patients with solitary hematogenous metastases from non-small cell lung cancer (NSCLC) remains controversial, although numerous retrospective studies have reported favorable results for patients offered combined surgical therapy. Our aim was to determine the role of surgical resection in the management of NSCLC with solitary extrapulmonary metastases and to investigate for possible prognostic factors.
PATIENTS AND METHODS: Between January 2004 and December 2012, 12 patients with NSCLC, from two Institutions, underwent metastasectomy for their solitary metastatic lesion. Sites of metastases included brain (n=3), adrenal gland (n=6), thoracic wall (n=2) and diaphragm (n=1). All patients had undergone pulmonary resections for their primary NSCLC.
RESULTS: Median survival for the entire cohort was 24.1 months, whereas 1- and 5-year survival rates were 73% and 39%, respectively. Patients with stage III intrathoracic disease had significantly worse survival than those with lower tumor stage. A tendency for adenocarcinomatous histology to positively affect survival was recognized, although it was proven not to be statistically significant.
CONCLUSION: Despite the retrospective nature of our study and the small cohort size, it is emerging that combined surgical resection might offer patients with NSCLC with solitary hematogenous metastases a survival benefit. Limited intrathoracic disease and adenocarcinomatous histology might be associated with better outcomes.

PMID: 28438878 [PubMed - in process]

Performance of 18F-FDG PET/CT in the characterization of adrenal masses in non-cancer patients: A prospective study.

Wed, 04/26/2017 - 19:25
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Performance of 18F-FDG PET/CT in the characterization of adrenal masses in non-cancer patients: A prospective study.

J Clin Endocrinol Metab. 2017 Apr 20;:

Authors: Guerin C, Pattou F, Brunaud L, Lifante JC, Mirallié E, Haissaguerre M, Huglo D, Olivier P, Houzard C, Ansquer C, Hindié E, Loundou A, Archange C, Tabarin A, Sebag F, Baumstarck K, Taïeb D

Abstract
Context: Few prospective studies have evaluated the role of 18F-Fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) in the characterization of adrenal masses.
Objective: To assess the performance of 18F-FDG PET/CT in the malignancy diagnosis of adrenal masses in non-cancer patients.
Design: Prospective multicenter study.
Material and methods: The study population consisted of 87 patients (87 adrenal masses) referred to endocrine surgeons: 56 with mass diameter ≥40 mm and 31 with a diameter <40 mm and of indeterminate nature based on unenhanced and washout CT attenuation densities. Fourteen patients had hypercortisolism. Adrenal masses were characterized by 18F-FDG PET/CT. Histology was the gold standard for the diagnosis of malignancy. In the absence of pathological proof (n=23), the nature of the lesion was based on the 12-month imaging follow-up.
Results: Fifteen adrenal masses were classified as malignant (including 11 adrenocortical carcinomas-ACC) and 72 as benign. Compared to benign lesions, malignant lesions were larger in size (p=0.003), had higher unenhanced densities (p=0.002), lower relative washout values (p=0.007), and higher 18F-FDG uptake parameters (p<10-3). The optimal threshold value of (Tumor SUVmax:Liver SUVmax) the ratio for malignancy was >1.5 with sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of 86.7%, 86.1%, 56.5, 96.9, and 86.2%, respectively.
Conclusions: Our results show that 18F-FDG-PET/CT complements adrenal washout CT in the evaluation of adrenal masses and should be recommended in the evaluation of large and/or indeterminate adrenal masses.

PMID: 28431167 [PubMed - as supplied by publisher]

Primary Ewing Sarcoma / Primitive Neuroectodermal Tumor of the Kidney: A Clinicopathologic Study of 23 Cases.

Wed, 04/26/2017 - 19:25
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Primary Ewing Sarcoma / Primitive Neuroectodermal Tumor of the Kidney: A Clinicopathologic Study of 23 Cases.

Pathol Oncol Res. 2017 Apr 20;:

Authors: Murugan P, Rao P, Tamboli P, Czerniak B, Guo CC

Abstract
Primary Ewing sarcoma / primitive neuroectodermal tumor (ES) of the kidney is a rare neoplasm with limited clinicopathologic data. We report 23 such cases with no history of ES elsewhere in the body. The patients included 13 male and 10 female, aged 8-70 years (mean, 31 years). The average tumor size was 11.7 cm (range, 5-20 cm). Microscopic analysis showed predominantly lobular growth (n = 14), with focal papillary (n = 3), alveolar (n = 1), and hemangiopericytoma-like (n = 1) patterns. Several tumors (n = 11) exhibited robust mitotic activity (>10 mitoses/10 high-power fields). Necrosis (n = 13) and lymphovascular invasion (n = 14) were common. Homer Wright rosettes (n = 6) and perivascular pseudorosettes (n = 1) were also identified. The tumors invaded the renal sinus or perinephric fat (n = 11), renal vein (n = 13), and adrenal gland (n = 2). Molecular and fluorescence in situ hybridization analysis showed rearrangement of EWSR1 gene (10/10), associated with EWSR1-FLI1 gene fusion (7/10). All patients with follow-up information (n = 18) had metastasis, commonly in the lungs (n = 12) and bone (n = 6). Twelve patients died of disease in a mean of 21 months; 6 patients were alive at a mean of 49 months after diagnosis. Primary kidney ES usually present at an advanced stage with extrarenal spread and metastasis. Although renal ES share histologic, immunohistochemical, and molecular features with their bone and soft tissue counterparts, they appear to be more aggressive tumors with poorer clinical outcome.

PMID: 28429277 [PubMed - as supplied by publisher]

Incidental posterior mediastinal paraganglioma: The safe approach to management, case report.

Wed, 04/26/2017 - 19:25
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Incidental posterior mediastinal paraganglioma: The safe approach to management, case report.

Int J Surg Case Rep. 2017 Mar 31;35:25-28

Authors: Muñoz-Largacha JA, Glocker RJ, Moalem J, Singh MJ, Litle VR

Abstract
INTRODUCTION: Paragangliomas are neuroendocrine tumors arising from chromaffin cells located in sympathetic paraganglia. Mediastinal paragangliomas are extremely rare and can be classified as functional or non-functional according to their ability for secreting catecholamines. Patients can be asymptomatic and the diagnosis is usually incidental. Complete surgical resection remains the standard of care for paragangliomas.
PRESENTATION OF CASE: We present a 44-year-old woman with a functional mediastinal paraganglioma incidentally found during the perioperative imaging workup for a diagnosed breast carcinoma. Chest radiograph and computed tomography (CT) showed a well-defined lesion in the posterior mediastinum suspicious for an esophageal malignancy. Endoscopic and CT-guided biopsies were performed confirming the diagnosis of a neuroendocrine tumor. Laboratory studies showed elevated catecholamines and chromogranin A levels, consistent with a paraganglioma. Appropriate pre-operative management was done and successful surgical resection without catecholamine related complications was achieved.
DISCUSSION: The workup and treatment of incidentally discovered adrenal and extra-adrenal lesions are controversial. Because of the absence of symptoms and the wider differential diagnosis of extra-adrenal lesions, an attempt for biopsying and surgically remove these lesions prior to biochemical testing is not an uncommon scenario, although this could be potentially harmful. Surgeons should have an index of suspicion for catecholamine-secreting tumors and hormonal levels should be assessed prior to biopsy or surgical resection.
CONCLUSION: Surgeons should consider paragangliomas as a differential diagnosis for extra-adrenal lesions. Biochemical testing with catecholamines and chromogranin A levels should be performed prior to biopsy or surgical removal in order to avoid catastrophic complications.

PMID: 28427002 [PubMed - as supplied by publisher]

Anthracyclines suppress pheochromocytoma cell characteristics, including metastasis, through inhibition of the hypoxia signaling pathway.

Wed, 04/26/2017 - 19:25
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Anthracyclines suppress pheochromocytoma cell characteristics, including metastasis, through inhibition of the hypoxia signaling pathway.

Oncotarget. 2017 Apr 04;8(14):22313-22324

Authors: Pang Y, Yang C, Schovanek J, Wang H, Bullova P, Caisova V, Gupta G, Wolf KI, Semenza GL, Zhuang Z, Pacak K

Abstract
Pheochromocytomas (PHEOs) and paragangliomas (PGLs) are rare, neuroendocrine tumors derived from adrenal or extra-adrenal chromaffin cells, respectively. Metastases are discovered in 3-36% of patients at the time of diagnosis. Currently, only suboptimal treatment options exist. Therefore, new therapeutic compounds targeting metastatic PHEOs/PGLs are urgently needed. Here, we investigated if anthracyclines were able to suppress the progression of metastatic PHEO. We explored their effects on experimental mouse PHEO tumor cells using in vitro and in vivo models, and demonstrated that anthracyclines, particularly idarubicin (IDA), suppressed hypoxia signaling by preventing the binding of hypoxia-inducible factor 1 and 2 (HIF-1 and HIF-2) to the hypoxia response element (HRE) sites on DNA. This resulted in reduced transcriptional activation of HIF target genes, including erythropoietin (EPO), phosphoglycerate kinase 1 (PGK1), endothelin 1 (EDN1), glucose transporter 1 (GLUT1), lactate dehydrogenase A (LDHA), and vascular endothelial growth factor (VEGFA), which consequently inhibited the growth of metastatic PHEO. Additionally, IDA downregulated hypoxia signaling by interfering with the transcriptional activation of HIF1A and HIF2A. Furthermore, our animal model demonstrated the dose-dependent suppressive effect of IDA on metastatic PHEO growth in vivo. Our results indicate that anthracyclines are prospective candidates for inclusion in metastatic PHEO/PGL therapy, especially in patients with gene mutations involved in the hypoxia signaling pathway.

PMID: 28423608 [PubMed - in process]

Synthesis of Neutral Ether Lipid Monoalkyl-diacylglycerol (MADAG) by Lipid Acyltransferases.

Wed, 04/26/2017 - 19:25
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Synthesis of Neutral Ether Lipid Monoalkyl-diacylglycerol (MADAG) by Lipid Acyltransferases.

J Lipid Res. 2017 Apr 18;:

Authors: Ma Z, Onorato JM, Chen L, Nelson DW, Yen CE, Cheng D

Abstract
In mammals, ether lipids exert a wide spectrum of signaling and structural functions such as stimulation of immune responses, anti-tumor activities and enhancement of sperm functions. Abnormal accumulation of monoalkyl-diacylglycerol (MADAG) was found in Wolman disease, a human genetic disorder defined by a deficiency in lysosomal acid lipase. In the current study, we found that among the 9 recombinant human lipid acyltransferases examined, DGAT1, DGAT2, MGAT2, MGAT3, AWAT2/MFAT and DC3 were able to use 1-monoalkylglycerol (1-MAkG) as an acyl acceptor for the synthesis of monoalkyl-monoacylglycerol (MAMAG). These enzymes demonstrated different enzymatic turnover rates and relative efficiencies for the first and second acylation steps leading to the synthesis of MAMAG and MADAG, respectively. They also exhibited different degrees of substrate preference when presented with 1-monooleoylglycerol versus 1-MAkG. In CHO-K1 cells, treatment with DGAT1 selective inhibitor XP-620 completely blocked the synthesis of MADAG, indicating that DGAT1 is the predominant enzyme responsible for the intracellular synthesis of MADAG in this model system. The levels of MADAG in the adrenal gland of DGAT1 knockout (KO) mice were reduced as compared to those of the wild type mice, suggesting that DGAT1 is a major enzyme for the synthesis of MADAG in this tissue. Our findings indicate that several of these lipid acyltransferases may be able to synthesize neutral ether lipids in mammals.

PMID: 28420705 [PubMed - as supplied by publisher]

Case report: ACTH-secreting pituitary carcinoma metastatic to the liver in a patient with a history of atypical pituitary adenoma and Cushing's disease.

Wed, 04/26/2017 - 19:25
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Case report: ACTH-secreting pituitary carcinoma metastatic to the liver in a patient with a history of atypical pituitary adenoma and Cushing's disease.

Diagn Pathol. 2017 Apr 18;12(1):34

Authors: Joehlin-Price AS, Hardesty DA, Arnold CA, Kirschner LS, Prevedello DM, Lehman NL

Abstract
BACKGROUND: Pituitary carcinoma is a rare entity requiring the presence of metastasis to confirm its malignant potential. We report a case of pituitary carcinoma and discuss the diagnosis and management of this lesion in relation to the existing literature.
CASE PRESENTATION: The patient is a 51-year-old woman with Cushing's disease and intact adrenal glands who was diagnosed with metastatic pituitary carcinoma to the liver, 29 months after initial resection of an ACTH-secreting primary atypical pituitary adenoma (APA). Prior to detection of this metastasis the patient underwent repeat resection and radiotherapy for residual cavernous sinus disease. The metastatic lesion was detected by interval surveillance of serum ACTH and 24-hour urine cortisol, which despite stable pituitary MRI, were significantly elevated. These abnormalities prompted a PET scan that demonstrated hypermetabolic liver parenchyma, which was suspicious for metastasis on abdominal MRI. An ultrasound-guided liver biopsy demonstrated nests of moderately-differentiated cells with intermediate-sized, monotonous nuclei, distinct nucleoli, and abundant basophilic cytoplasm, confirmed by immunohistochemistry to represent metastatic pituitary carcinoma. The liver lesion was subsequently successfully removed by wedge resection. One year later, the patient's residual cavernous sinus disease grew markedly, and she was placed on dual-agent chemotherapy consisting of oral temozolomide and capecitabine, with stabilization of her intracranial disease to present, although liver metastases recurred.
CONCLUSIONS: Pituitary carcinoma is a rare entity impossible to recognize as a primary tumor because its diagnosis by definition requires the presence of metastasis. Maintaining awareness of the entity and its precursor lesion APA is essential for its accurate pathologic diagnosis and appropriate management.

PMID: 28420444 [PubMed - in process]

Simultaneous assay of cortisol and dexamethasone improved diagnostic accuracy of the dexamethasone suppression test.

Wed, 04/26/2017 - 19:25
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Simultaneous assay of cortisol and dexamethasone improved diagnostic accuracy of the dexamethasone suppression test.

Eur J Endocrinol. 2017 Jun;176(6):705-713

Authors: Ueland GÅ, Methlie P, Kellmann R, Bjørgaas M, Åsvold BO, Thorstensen K, Kelp O, Thordarson HB, Mellgren G, Løvås K, Husebye ES

Abstract
OBJECTIVES: The overnight dexamethasone (DXM) suppression test (DST) has high sensitivity, but moderate specificity, for diagnosing hypercortisolism. We have evaluated if simultaneous measurement of S-DXM may correct for variable DXM bioavailability and increase the diagnostic performance of DST, and if saliva (sa) is a feasible adjunct or alternative to serum.
DESIGN AND METHODS: Prospective study of DST was carried out in patients with suspected Cushing's syndrome (CS) (n = 49), incidentaloma (n = 152) and healthy controls (n = 101). Cortisol, cortisone and DXM were assayed by liquid chromatography-tandem mass spectrometry (LC-MS/MS).
RESULTS: Three hundred and two subjects underwent DST; S-cortisol was ≥50 nmol/L in 83 patients, of whom 11 had CS and 27 had autonomous cortisol secretion. The lower 2.5 percentile of S-DXM in subjects with negative DST (n = 208) was 3.3 nmol/L, which was selected as the DXM cut-off level. Nine patients had the combination of low S-DXM and positive DST. Of these, three had been misdiagnosed as having autonomous cortisol secretion. DST results were highly reproducible and confirmed in a replication cohort (n = 58). Patients with overt CS had significantly elevated post-DST sa-cortisol and sa-cortisone levels compared with controls; 23 of 25 with autonomous cortisol secretion had elevated sa-cortisone and 14 had elevated sa-cortisol.
CONCLUSIONS: Simultaneous measurement of serum DXM and cortisol reduced false-positive DSTs by 20% and improved the specificity. S-DXM >3.3 nmol/L is sufficient for the suppression of cortisol <50 nmol/L. Measurement of glucocorticoids in saliva is a non-invasive and easy procedure and post-DST sa-cortisone was found particularly useful in the diagnosis of CS.

PMID: 28298353 [PubMed - indexed for MEDLINE]

Validation of the prognostic role of the "Helsinki Score" in 225 cases of adrenocortical carcinoma.

Wed, 04/26/2017 - 19:25
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Validation of the prognostic role of the "Helsinki Score" in 225 cases of adrenocortical carcinoma.

Hum Pathol. 2017 Apr;62:1-7

Authors: Duregon E, Cappellesso R, Maffeis V, Zaggia B, Ventura L, Berruti A, Terzolo M, Fassina A, Volante M, Papotti M

Abstract
Adrenocortical carcinoma patient prognosis is extremely variable and poorly predictable. The newly introduced Helsinki Score is the first so far proposed diagnostic and prognostic system based on the combined evaluation of morphological (mitoses and necrosis) and immunohistochemical (Ki-67) parameters. The aim of the study was to validate the prognostic role of the Helsinki Score for adrenocortical carcinoma characterization. Thus, 225 adrenocortical carcinomas were reclassified using the Weiss Score and the Helsinki Score (3× mitotic count + 5 × necrosis + Ki-67 index). At univariate analysis, statistically significant prognostic values were observed at the log-rank test for mitotic count (cutoff values: <6 and ≥55; P<.0001), Ki-67 (cutoff values: <20 and ≥50; P<.0001), Weiss Score (cutoff values: <5 and ≥8; P<.0001), Helsinki Score (cutoff values: <13 and ≥19; P<.0001), histological variant (conventional versus oncocytic; P=.009), necrosis (P=.001), and stage (P=.005). Cox multivariate analysis using a backward stepwise selection method retained only Helsinki Score and Weiss Score as predictors of poor prognosis (P<.0001 and P=.0005, respectively). Helsinki Score (with a threshold of 28.5 points; area under the curve [AUC]=0.729, 95% confidence interval=0.66-0.79) and Ki-67 (with a threshold of 20.5%; AUC=0.727, 95% confidence interval=0.66-0.79) showed the best and equivalent AUCs predicting disease-related deaths determined using receiver operating characteristic statistics. In conclusion, the Helsinki Score is a valuable system to predict prognosis in adrenocortical carcinoma, outperforming the currently established prognostic parameters.

PMID: 27916625 [PubMed - indexed for MEDLINE]

Dermatologic Manifestations of Monogenic Autoinflammatory Diseases.

Wed, 04/26/2017 - 19:25
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Dermatologic Manifestations of Monogenic Autoinflammatory Diseases.

Dermatol Clin. 2017 Jan;35(1):21-38

Authors: Shwin KW, Lee CR, Goldbach-Mansky R

Abstract
Autoinflammatory disorders are sterile inflammatory conditions characterized by episodes of early-onset fever, rash, and disease-specific patterns of organ inflammation. Gain-of-function mutations in innate danger-sensing pathways, including the inflammasomes and the nucleic acid sensing pathways, play critical roles in the pathogenesis of IL-1 and Type-I IFN-mediated disorders and point to an important role of excessive proinflammatory cytokine signaling, including interleukin (IL)-1b , Type-I interferons, IL-18, TNF and others in causing the organ specific immune dysregulation. The article discusses the concept of targeting proinflammatory cytokines and their signaling pathways with cytokine blocking treatments that have been life changing for some patients.

PMID: 27890235 [PubMed - indexed for MEDLINE]

Adrenal incidentalomas: A guide to assessment, treatment and follow-up.

Wed, 04/26/2017 - 19:25
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Adrenal incidentalomas: A guide to assessment, treatment and follow-up.

Maturitas. 2016 10;92:79-85

Authors: Paschou SA, Vryonidou A, Goulis DG

Abstract
Adrenal incidentalomas are clinically unsuspected lesions that are detected in adrenal glands during imaging procedures for other causes. With widespread use of imaging - both computed tomography (CT) and magnetic resonance imaging (MRI) - adrenal incidentalomas are now a common clinical problem. The two main clinical issues to be determined in this setting are the risk of malignancy and the hormonal activity of these lesions. The answers to these two questions, along with the clinical characteristics of each individual patient and co-morbidities, will guide the treatment strategy, which can vary from simple follow-up to surgical resection. The objective of this article is to present updated information on the definition, prevalence, imaging and functional features of adrenal incidentalomas and to provide a guide to their optimal assessment, treatment and follow-up. This review collected, analyzed and qualitatively re-synthesized information regarding: (1) the various clinical entities known as "adrenal incidentalomas", (2) the initial assessment of risk of malignancy, (3) the initial assessment of whether the lesion is hormonally active or non-functioning, (4) the absolute and relative indications for surgical treatment, (5) the follow-up of patients who are not deemed to need surgical treatment after initial assessment, and (6) the post-operative follow-up of patients who undergo surgical treatment. The evidence calls for clinicians to bear in mind the Hippocratian advice "ωϕελέειν ή μη βλάπτειν" ("first do no harm").

PMID: 27621243 [PubMed - indexed for MEDLINE]

Epithelial Cell Adhesion Molecule Expression in Canine Tumours.

Wed, 04/26/2017 - 19:25
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Epithelial Cell Adhesion Molecule Expression in Canine Tumours.

J Comp Pathol. 2016 Nov;155(4):299-304

Authors: Thamm DH, Hayes DF, Meuten T, Laver T, Thomas DG

Abstract
Epithelial cell adhesion molecule (EpCAM) is expressed in most human normal and neoplastic tissues of epithelial derivation and may have an association with tumour cell aggressiveness, a stem cell-like phenotype and clinical outcome. Antibody-based strategies for the targeting and capture of EpCAM-expressing tumour cells are showing promise, both as diagnostic tools and potential therapies. The aim of this study was to assess EpCAM expression in canine tumours. EpCAM expression was assessed in tumour cell lines via gene expression profiling and in formalin-fixed and paraffin wax-embedded tissues from canine carcinomas representing various anatomical sites by immunohistochemistry. EpCAM mRNA expression was higher in cell lines from carcinomas than those derived from sarcomas or haemopoietic tumours. EpCAM was expressed by >2/3 of tumour cells in 71% of canine carcinomas evaluated, irrespective of histotype, with the exception of carcinomas of the adrenal gland. Canine sarcomas and haemopoietic tumours were uniformly negative. Most canine carcinomas express EpCAM and so could be suitable for the study of EpCAM-directed diagnostics and therapeutics.

PMID: 27567927 [PubMed - indexed for MEDLINE]

Adrenocortical Oncocytic Carcinoma: A Case Report and Review of the Histopathologic Diagnostic Criteria.

Wed, 04/26/2017 - 19:25
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Adrenocortical Oncocytic Carcinoma: A Case Report and Review of the Histopathologic Diagnostic Criteria.

Turk Patoloji Derg. 2016;32(3):211-5

Authors: Arik D, Canaz F, Dündar E

Abstract
Oncocytic tumors are rare in the adrenal gland. The histopathological diagnosis of adrenocortical carcinoma is difficult due to the lack of precise diagnostic criteria for malignancy. A 44-year-old man was admitted to our hospital with left flank pain. Radiologically an adrenal mass was detected. After the excision and histopathologic evaluation of the mass, a diagnosis of adrenocortical oncocytic carcinoma was made. At least one of the features of more than 5 mitoses in 50 high power fields, atypical mitotic figures or venous invasion is required for the diagnosis of malignancy in adrenocortical tumors. It has been suggested that tumors that have more than one of the minor criteria of large size ( > 10 cm or > 200 gr), necrosis, capsular or sinusoidal invasion, should be evaluated as having uncertain malignant potential.

PMID: 27562395 [PubMed - indexed for MEDLINE]

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