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An ovarian Leydig cell tumor of ultrasound negative in a postmenopausal woman with hirsutism and hyperandrogenism: A case report.

Sun, 03/11/2018 - 16:52
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An ovarian Leydig cell tumor of ultrasound negative in a postmenopausal woman with hirsutism and hyperandrogenism: A case report.

Medicine (Baltimore). 2018 Mar;97(10):e0093

Authors: Chen M, Zhou W, Zhang Z, Zou Y, Li C

Abstract
RATIONALE: The incidence of severe hyperandrogenism associated with masculinity in women is very low. While rare and difficult to diagnose, androgen secreting tumors should be suspected in women with hyperandrogenism and hirsutism, especially in the postmenopausal population. Herein we present one case of ovarian Leydig cell tumor (LCT) with markedly elevated serum testosterone levels and frank hirsutism.
PATIENT CONCERNS: A 60-year-old woman, presented with increased hair growth and androgenic alopecia and the hormonal laboratory examination showed that she had elevated serum testosterone level and normal dehydroepiandrosterone sulfate (DHEAS), androstenedione, 17- hydroxyprogesterone, cortisol and thyroid stimulating hormone (TSH).
DIAGNOSES: The diagnosis of possible testosterone secreting tumor was performed when pelvic computed tomography (CT) and magnetic resonance image (MRI) showed a right adnexal mass of 15mm×16mm indicative of sex cord- stromal tumors.
INTERVENTIONS: The patient received laparoscopic total abdominal hysterectomy and bilateral salpingo-oophorectomy.
OUTCOMES: After operation, testosterone got back to the normal level and clinical symptoms subsided.
LESSONS: It is common that postmenopausal androgen excess is a state of relative or absolute androgen excess originating from the adrenal gland and/or ovaries. In either case, doctors need to assess such patients and exclude relatively rare potential causes of tumors. Any woman who has hirsutism or frank evidence of markedly increased testosterone should exclude this kind of possibility of androgen producing tumors. It is possible to determine the origin of androgen hypersecretion with the severity of symptoms, the extent of androgen excess, and the relevant imaging studies. Since LCT are rare ovarian sex-cord stromal tumors, it can be beneficial for diagnosis with careful research of patient history of the defeminization followed by virilization, and a CT and MRI image.

PMID: 29517680 [PubMed - in process]

A rare mediastinal occurrence of neuroblastoma in an adult: case report.

Sun, 03/11/2018 - 16:52
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A rare mediastinal occurrence of neuroblastoma in an adult: case report.

Sao Paulo Med J. 2018 Mar 05;:0

Authors: Yanik F, Karamustafaoglu YA, Yoruk Y

Abstract
CONTEXT: Neuroblastoma is the most common extracranial malignant solid tumor that occurs during childhood. It arises from primitive cells and is seen in the adrenal medulla and sympathetic ganglia of the sympathetic nervous system.
CASE REPORT: We present a rare case of a 40-year-old man who was diagnosed with the onset of neuroblastoma arising in the mediastinum. He was treated by means of surgical resection in the superior mediastinum after neoadjuvant chemotherapy. The patient's surgical outcome was satisfactory.
CONCLUSION: There are still no standard treatment guidelines for adult neuroblastoma patients. Although they have a poor prognosis, the main treatment option should be complete surgery at an early stage. This situation may become clarified through biological and genetic studies in the future.

PMID: 29513786 [PubMed - as supplied by publisher]

Skeletal metastasis in renal cell carcinoma: A review.

Sun, 03/11/2018 - 16:52
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Skeletal metastasis in renal cell carcinoma: A review.

Ann Med Surg (Lond). 2018 Mar;27:9-16

Authors: Umer M, Mohib Y, Atif M, Nazim M

Abstract
Background: Renal cell carcinoma account for 3% of all cancers, with peak incidence between 60 and 70 years of age predominantly affecting male population. Renal carcinoma is the most common malignancy of kidney constitutes for 80-90% of renal neoplasm with an overall 45% five years survival rate. Majority are diagnosed incidentally during investigation for other disease process of abdomen. Classical triad of gross hematuria, pain and palpable mass in abdomen is rare accounting to only 6-10%. Treatment of early stages of disease i.e. localized disease is partial or radical nephrectomy. Most common metastasis in RCC occurs to lung, followed by bone involvement in 20-35%, lymph nodes, liver, adrenal gland and brain. In metastatic disease median survival rate of patient is about eight months with 50% mortality rate within first year of life, five years survival rate is 10%. Skeletal metastasis are very destructive in patients with renal cell carcinoma compromising bone integrity leading to skeletal related events including pains, impending fractures, nerve compressions, hypercalcemia and even pathological fractures which may require surgical interventions and other therapy. In addition to skeletal complications, presence of bone metastases in RCC has negative impact on progression free survival and overall survival of patients treated with systemic therapies.
Objective: In this review we discuss pathophysiology of tumor metastasis, diagnosis, management and Case examples of metastatic renal cell carcinoma.
Conclusion: Incidence of metastatic renal carcinoma is increasing. Overall prognosis of patient with advanced RCC is poor, emphasizing the importance of early detection and prompt treatment of primary lesion in its early stage. Advancement in targeted therapy in recent decades had made some improvement in treatment of SREs and has helped in improving patent's quality of life but still we are in need of further improvement in treatment modalities to cure disease thereby decreasing morbidity and mortality.

PMID: 29511536 [PubMed]

Single-plane retroperitoneoscopic adrenalectomy: a new operative procedure for benign adrenal disease.

Sun, 03/11/2018 - 16:52
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Single-plane retroperitoneoscopic adrenalectomy: a new operative procedure for benign adrenal disease.

Sci Rep. 2018 Mar 05;8(1):4027

Authors: Li S, Wang J, Zhang E, Gao W, Yang J, Jia Z

Abstract
To evaluate the therapeutic effect of single-plane retroperitoneoscopic adrenalectomy. From February 2014 to March 2017, 251 patients underwent single-plane retroperitoneoscopic adrenalectomy, and their operative outcomes were compared with those of 98 patients who underwent anatomical three-plane retroperitoneoscopic adrenalectomy. Among 35 patients with a body mass index (BMI) of ≥30 kg/m2, their operative outcomes were compared between two operative procedures. The demographic data and perioperative outcomes of the patients were statistically analysed. The single-plane and three-plane groups were comparable in terms of estimated blood loss, time to oral intake, hospital stay, and incidence of complications among patients with similar baseline demographics. The single-plane group had a significantly shorter operation time (46.9 ± 5.8 vs 54.8 ± 7.0 mins, P < 0.0001) and lower analgesia requirement (56/251 vs 33/98, p = 0.03). For obese patients with a BMI of ≥30 kg/m2, single-plane adrenalectomy was also associated with a significantly shorter operation time(48.1 ± 6.2 vs 64.1 ± 5.1 mins, p < 0.0001). Single-plane retroperitoneoscopic adrenalectomy is feasible, safe, and effective in the treatment of adrenal masses <5 cm in size and provides a shorter operation time and better pain control than anatomical retroperitoneal adrenalectomy, especially in obese patients.

PMID: 29507374 [PubMed - in process]

[A comparison of clinical characteristics between 2 pedigrees of multiple endocrine neoplasia type 2A with different RET mutations].

Sun, 03/11/2018 - 16:52
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[A comparison of clinical characteristics between 2 pedigrees of multiple endocrine neoplasia type 2A with different RET mutations].

Zhonghua Nei Ke Za Zhi. 2018 Feb 01;57(2):134-137

Authors: Weng Y, Xue SN, Zhang SL, Cheng H, Yan L

Abstract
Multiple endocrine neoplasia type 2A (MEN2A) is a hereditary syndrome. Here, two different RET proto-oncogen mutation were identified from family members of two MEN2A pedigrees by genetic screening. One RET mutations were found at codons 1893 and 1895 in exon 11 (1893-1895delCGA) from pedigree 1, which is a novel mutation, the other occurs at codon 634 (Cys634Arg) in exon 11 from pedigree 2. However, the clinical characteristics were similar in the patients of the two pedigrees. All the patients were in middle-age at onset. Most of them were firstly diagnosed with bilateral adrenal pheochromocytoma with different degrees of thyroid abnormalities (elevated serum calcitonin with or without thyroid mass, or had been diagnosed with medullary thyroid carcinoma). Some family members were with elevated serum parathyroid hormone but with no other evidences for hyperparathyroidism.

PMID: 29397600 [PubMed - indexed for MEDLINE]

Management of incidental adrenal tumours.

Sun, 03/11/2018 - 16:52
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Management of incidental adrenal tumours.

BMJ. 2018 01 18;360:j5674

Authors: Hanna FWF, Issa BG, Sim J, Keevil B, Fryer AA

PMID: 29348269 [PubMed - indexed for MEDLINE]

A Four-Tiered Prognostic Score for Patients Receiving Palliative Thoracic Radiotherapy for Lung Cancer.

Sun, 03/11/2018 - 16:52
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A Four-Tiered Prognostic Score for Patients Receiving Palliative Thoracic Radiotherapy for Lung Cancer.

Cancer Invest. 2018 Jan 02;36(1):59-65

Authors: Nieder C, Tollåli T, Haukland E, Reigstad A, Randi Flatøy L, Dalhaug A

Abstract
Palliative radiotherapy improves lung cancer related symptoms. Prognosis should be taken into account when deciding about fractionation. In this study, prognostic factors derived from multivariate analysis were used to assign a point sum reflecting 6-month survival. Four prognostic groups were compared. Performance status, lactate dehydrogenase, C-reactive protein, liver/adrenal gland metastases, and extrathoracic disease status significantly predicted survival and formed the basis of the score. The four groups had a median survival of 0.8, 1.6, 3.3, and 10.5 months (6-month survival 0, 10, 30, 70%; 12-month survival 0, 0, 12, 40%; p = 0.0001), respectively. In the unfavorable group best supportive care might be preferable.

PMID: 29319371 [PubMed - indexed for MEDLINE]

Primary mediastinal paraganglioma associated with a familial variant in the succinate dehydrogenase B subunit gene.

Sun, 03/11/2018 - 16:52
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Primary mediastinal paraganglioma associated with a familial variant in the succinate dehydrogenase B subunit gene.

J Surg Oncol. 2018 Feb;117(2):160-162

Authors: Samuel N, Ejaz R, Silver J, Ezzat S, Cusimano RJ, Kim RH

Abstract
Surgical management is the mainstay of therapy for primary cardiac tumors, yet due to the rarity of these malignancies, their management and workup remains a challenge. Here, we report a unique case of a patient with a primary left ventricular cardiac paraganglioma (PGL) and describe the role of a medical genetics assessment leading to the identification of a rare variant in the SDHB gene to be the causative etiology of this cardiac tumor. Due to decreasing costs and accessibility of molecular genetic analysis, genetic testing may become an emerging diagnostic adjunct in cases of cardiac tumors.

PMID: 28891197 [PubMed - indexed for MEDLINE]

Immune suppression of IgG response against dairy proteins in major depression.

Sun, 03/11/2018 - 16:52
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Immune suppression of IgG response against dairy proteins in major depression.

BMC Psychiatry. 2017 Jul 24;17(1):268

Authors: Rudzki L, Pawlak D, Pawlak K, Waszkiewicz N, Małus A, Konarzewska B, Gałęcka M, Bartnicka A, Ostrowska L, Szulc A

Abstract
BACKGROUND: Interactions between the digestive system, brain functions and immunoglobulin G (IgG) mediated immunity against food antigens became recently a topic of growing interest in psychiatry research. Psychological stress can activate hypothalamic-pituitary-adrenal axis (HPA) with subsequent hypercortisolemia. It can also influence intestinal permeability and dynamics of IgG response. Major depression can by accompanied either by activation of inflammatory response or by immune suppression (e.g. decreased antibody production) where hypercortisolemia is a significant immune modulator. The aim of our study was to assess IgG immune response against 44 food products in depressed patients and controls along with markers of psychological stress, inflammation, psychometric and dietary parameters.
METHODS: Serum IgG concentrations against 44 food antigens, plasma cortisol, TNF-α, IL-6, IL-1b concentrations were measured and psychometric parameters were evaluated using Hamilton Depression Rating (HAM-D 17), Perceived Stress (PSS-10), and Symptom Checklist (SCL-90) scales in 34 depressed patients and 29 controls. Dietary parameters such as frequency of exposure to food antigens, appetite and weight change were assessed.
RESULTS: There was a significantly lower IgG concentration against dairy in depressed patients compared to controls (post hoc p < 0.05) when there was a high exposure (consumption) to dairy. Our research revealed a significant interaction of IgG concentration against dairy proteins and exposure to dairy between groups (F (2.63) = 3.92, p = 0.025, η2 = 0.12). There was no significant difference in mean IgG concentration against food antigens between patients and controls. We found increased concentration of cortisol in depressed patients (t (1.61) = 2.37, p = 0.02) compared to controls. Patients with melancholic depression had significantly higher (M rank  = 21.27) concentration of cortisol (U = 41, p = 0.006), when compared with the non-melancholic group of patients (M rank  = 12.16). Cortisol concentration significantly positively correlated with HAM-D 17 (r = 0.442, p = 0.009) and with phobias in SCL-90 scale in patients' group (r = 0.531, p = 0.001). There was decreased concentration of TNF-α (t = 4.256, p < 0.001) in depressed patients compared to controls. IgG concentration of 38.63% food products positively correlated with TNF-α concentration in depressed patients compared to 9.09% of those in healthy controls.
CONCLUSIONS: We observed an immune suppression of IgG response to dairy proteins in depressed patients. Hypercortisolemia with involvement of decreased concentration of TNF-α might play a significant role in suppression of IgG response in depressed patients.

PMID: 28738849 [PubMed - indexed for MEDLINE]

Extra-adrenal myelolipoma and extramedullary hematopoiesis: Imaging features of two similar benign fat-containing presacral masses that may mimic liposarcoma.

Sun, 03/11/2018 - 16:52
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Extra-adrenal myelolipoma and extramedullary hematopoiesis: Imaging features of two similar benign fat-containing presacral masses that may mimic liposarcoma.

Eur J Radiol. 2017 Aug;93:185-194

Authors: Littrell LA, Carter JM, Broski SM, Wenger DE

Abstract
OBJECTIVE: To describe the imaging characteristics of extra-adrenal myelolipoma and mass-forming extramedullary hematopoiesis (EMH) occurring in the presacral region.
MATERIALS AND METHODS: Retrospective review between 1980 and 2015 revealed 11 patients with biopsy-proven presacral extra-adrenal myelolipoma and 11 patients with presacral EMH and radiologic imaging studies.
RESULTS: All cases of both myelolipoma and EMH directly abutted the anterior sacrum and were centered on the midline. Myelolipomas were round or bilobed (8/11), or were multilobulated in appearance (3/11). On CT, margins could be smooth or ill defined. On MRI, all masses were well-marginated with evidence of a "capsule". All myelolipomas showed evidence of macroscopic fat on both CT and MRI with scattered hazy and nodular soft tissue components. On MRI, all cases showed areas of high T1 signal intermixed with areas of intermediate T1 signal. On T2, all cases showed areas of low and high T2 signal. Enhancement was usually minimal or mild. Most EMH cases had a well-defined round, bilobed or macro-lobulated presacral mass (9/11); 2/11 cases were ill-defined. On CT, when a dominant fatty component was present, the soft tissue components had hazy and nodular features, similar to myelolipoma. On MRI, the masses demonstrated intermediate T1 signal, isointense to skeletal muscle with varying amounts of hyperintense T1 signal. On T2-weighted images, 3 of 4 cases showed areas of mildly hyperintense T2 signal with varying amounts of dark signal. All patients with clinically or biopsy-proven EMH had a hematologic or myeloid disorder.
CONCLUSION: Presacral myelolipoma and presacral EMH are two benign fat-containing masses that can present as discrete, often encapsulated, lipomatous masses with varying amounts of non-lipomatous soft tissue components and should be considered in the differential diagnosis of a well-defined fat-containing presacral mass.

PMID: 28668414 [PubMed - indexed for MEDLINE]

Comparison of neurons derived from mouse P19, rat PC12 and human SH-SY5Y cells in the assessment of chemical- and toxin-induced neurotoxicity.

Sun, 03/11/2018 - 16:52
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Comparison of neurons derived from mouse P19, rat PC12 and human SH-SY5Y cells in the assessment of chemical- and toxin-induced neurotoxicity.

BMC Pharmacol Toxicol. 2017 Jun 05;18(1):42

Authors: Popova D, Karlsson J, Jacobsson SOP

Abstract
BACKGROUND: Exposure to chemicals might be toxic to the developing brain. There is a need for simple and robust in vitro cellular models for evaluation of chemical-induced neurotoxicity as a complement to traditional studies on animals. In this study, neuronally differentiated mouse embryonal carcinoma P19 cells (P19 neurons) were compared with human neuroblastoma SH-SY5Y cells and rat adrenal pheochromocytoma PC12 cells for their ability to detect toxicity of methylmercury (MeHg), okadaic acid and acrylamide.
METHODS: Retinoic acid-treated P19 and SH-SY5Y cells and nerve growth factor-stimulated PC12 cells, allowed to differentiate for 6 days, were exposed to MeHg, okadaic acid and acrylamide for 48 h. Cell survival and neurite outgrowth were assessed with the calcein-AM assay and fluorescence detection of antibodies against the cytoskeletal neuron-specific protein βIII-tubulin, respectively. The effects of glutathione (GSH) and the potent inhibitor of GSH synthesis buthionine sulfoximine (BSO) on the MeHg induced-toxicity were assessed using the PrestoBlue™ cell viability assay and the TMRE mitochondrial membrane potential assay.
RESULTS: Differentiated P19 cells developed the most extensive neuronal network among the three cell models and were the most sensitive neuronal model to detect neurotoxic effects of the test compounds. MeHg produced a concentration-dependent toxicity in differentiated P19 cells and SH-SY5Y cells, with statistically significant effects at concentrations from 0.1 μM in the P19 neurons and 1 μM in the SH-SY5Y cells. MeHg induced a decrease in the cellular metabolic activity and mitochondrial membrane potential (ΔΨm) in the differentiated P19 cells and SH-SY5Y cells, that were attenuated by GSH. Okadaic acid and acrylamide also showed statistically significant toxicity in the P19 neurons, but not in the SH-SY5Y cells or the P12 cells.
CONCLUSIONS: P19 neurons are more sensitive to detect cytotoxicity of MeHg, okadaic acid and acrylamide than retinoic acid-differentiated SH-SY5Y cells and nerve growth factor-treated PC12 cells. P19 neurons are at least as sensitive as differentiated SH-SY5Y cells to detect the loss of mitochondrial membrane potential produced by MeHg and the protective effects of extracellular GSH on MeHg toxicity. P19 neurons may be a useful model to study neurotoxic effects of chemicals.

PMID: 28583171 [PubMed - indexed for MEDLINE]

Combined pituitary hormone deficiency in a girl with 48, XXXX and Rathke's cleft cyst.

Sun, 03/11/2018 - 16:52
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Combined pituitary hormone deficiency in a girl with 48, XXXX and Rathke's cleft cyst.

Hormones (Athens). 2017 Jan;16(1):92-98

Authors: Uppal S, Jee YH, Lightbourne M, Han JC, Stratakis CA

Abstract
BACKGROUND: Tetrasomy X is a rare chromosomal aneuploidy seen in girls, associated with facial dysmorphism, premature ovarian insufficiency and intellectual disability. A Rathke's cleft cyst (RCC) is a remnant of Rathke's pouch which may cause multiple pituitary hormone deficiencies by exerting pressure on the pituitary gland in the sella.
METHODS/RESULTS: The patient was diagnosed with tetrasomy X by karyotyping during infancy. Brain MRI and multiple endocrine stimulation tests revealed RCC and combined pituitary hormone deficiency (growth hormone deficiency, secondary adrenal insufficiency and central hypothyroidism) likely due to RCC.
CONCLUSION: We report the first case in the literature of a girl with 48, XXXX and combined pituitary hormone deficiency due to Rathke's cyst.

PMID: 28500832 [PubMed - indexed for MEDLINE]

Arterial stiffness and blood pressure improvement in aldosterone-producing adenoma harboring KCNJ5 mutations after adrenalectomy.

Sun, 03/11/2018 - 16:52
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Arterial stiffness and blood pressure improvement in aldosterone-producing adenoma harboring KCNJ5 mutations after adrenalectomy.

Oncotarget. 2017 May 02;8(18):29984-29995

Authors: Chang CH, Hu YH, Tsai YC, Wu CH, Wang SM, Lin LY, Lin YH, Satoh F, Wu KD, Wu VC

Abstract
The aim of this study was to show the effect of KCNJ5 mutational status on arterial stiffness in aldosterone-producing adenomas after adrenalectomy. Between February 2008 and January 2010, we prospectively enrolled 108 aldosterone-producing adenoma patients undergoing adrenalectomy. We conducted repeated measurements of pulse wave velocity at baseline, 6 months, and 12 months after adrenalectomy, grouped by KCNJ5 mutational status. Prognostic factors of arterial stiffness and risk for hypertension at 12 months after adrenalectomy were analyzed after propensity score matching in a 1:1 ratio. After matching for age, sex and body mass index, 88 patients were divided equally into KCNJ5-mutant and non-mutant groups. KCNJ5 mutational status was not an independent variable in either the generalized estimating equation model (p = 0.147) or the percentage change of brachial-ankle pulse wave velocity (p = 0.106). The generalized additive model smoothing plot showed that aldosterone-producing adenoma patients who carried the KCNJ5 mutation and were aged between 37 and 60 may have a hypertension recovery advantage. According to our observations during a 12-month follow-up after adrenalectomy, KCNJ5 mutational status was not associated with improvement in arterial stiffness.

PMID: 28415786 [PubMed - indexed for MEDLINE]

Temporal Trends in Initiation of Therapy With Tumor Necrosis Factor Antagonists for Patients With Inflammatory Bowel Disease: A Population-based Analysis.

Sun, 03/11/2018 - 16:52
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Temporal Trends in Initiation of Therapy With Tumor Necrosis Factor Antagonists for Patients With Inflammatory Bowel Disease: A Population-based Analysis.

Clin Gastroenterol Hepatol. 2017 Jul;15(7):1061-1070.e1

Authors: Targownik LE, Tennakoon A, Leung S, Lix LM, Singh H, Bernstein CN

Abstract
BACKGROUND & AIMS: Anti-tumor necrosis factor (anti-TNF) agents are effective treatments for Crohn's disease (CD) and ulcerative colitis (UC). We aimed to determine their patterns of use and changes in these patterns over time, as well as use of immunomodulators and corticosteroids before anti-TNF therapy for persons with inflammatory bowel diseases.
METHODS: We used the University of Manitoba IBD Epidemiology Database to identify all anti-TNF users with CD and UC from 2001 through 2014. We assessed changes in the prevalence and incidence of anti-TNF use during different time periods (April 2001-March 2005, April 2005-March 2009, or April 2009-March 2013). We also characterized patterns of corticosteroid use, corticosteroid dependence, and immunomodulator use before anti-TNF administration and determined how these changed over time. The primary end point was change in time to first receipt of anti-TNF among the different time periods.
RESULTS: We identified 950 persons (761 with CD and 189 with UC) who received anti-TNF agents. The cumulative prevalence of persons with current or prior anti-TNF exposure in 2014 was 20.4% for CD and 6.0% for UC. In 2014 the cumulative incidence values of anti-TNF exposure within 5 years of diagnosis were 23.4% for patients with CD and 7.8% for patients with UC. Most users of anti-TNF agents had evidence of corticosteroid dependence (more than 2 g prednisone within any 12-month period) before initiation of anti-TNF therapy. Cumulative corticosteroid exposure before anti-TNF use decreased over time for patients with UC, but not significantly for patients with CD. There was no increase over time in the use of concomitant immunomodulators with anti-TNF therapy.
CONCLUSIONS: Use of anti-TNF agents increased from 2001 through 2014, with a concomitant significant decrease in cumulative use of corticosteroids before anti-TNF therapy for patients with UC. However, there has been no reduction in cumulative use of corticosteroids before anti-TNF therapy for patients with CD and no change in use of immunomodulators by patients with CD. These findings indicate a continuing need for optimization of anti-TNF therapy for patients with inflammatory bowel disease.

PMID: 28238957 [PubMed - indexed for MEDLINE]

Case of parotid mucoepidermoid carcinoma: Expanding the spectrum of von Hippel-Lindau-related neoplasms.

Sun, 03/11/2018 - 16:52
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Case of parotid mucoepidermoid carcinoma: Expanding the spectrum of von Hippel-Lindau-related neoplasms.

Head Neck. 2017 Mar;39(3):E51-E54

Authors: Berger MH, Kerr DA, Rangel Filho AE, Sargi ZB

Abstract
BACKGROUND: von Hippel-Lindau (VHL)-related tumors occurring outside the spectrum of VHL-defining tumors are rare, and mucoepidermoid carcinoma (MEC) in the setting of VHL disease has not been described.
METHODS AND RESULTS: We describe a patient with confirmed VHL mutation who presented with a parotid mass and a history of 2 central nervous system (CNS) hemangioblastomas and 1 pheochromocytoma. Fine-needle aspiration (FNA) of the mass suggested a benign Warthin tumor. The mass was resected and final pathology revealed a low-grade MEC. Fluorescence in situ hybridization for the MECT1/MAML2 fusion gene frequently associated with MEC was performed and was negative. Molecular testing of tumor cells displayed a likely "second hit" VHL gene mutation.
CONCLUSION: There is a possible broader role of VHL mutations in tumorigenesis beyond the development of classically described VHL-defining neoplasms. Our case also demonstrates the importance of always considering the possibility of a parotid malignancy in patients with VHL despite a benign FNA. © 2016 Wiley Periodicals, Inc. Head Neck 39: E51-E54, 2017.

PMID: 28006088 [PubMed - indexed for MEDLINE]

Synchronous Microscopic Epstein-Barr Virus-Positive Diffuse Large B-Cell Lymphoma of the Adrenal and Lymphoplasmacytic Lymphoma: De Novo Disease or Transformation?

Sun, 03/11/2018 - 16:52
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Synchronous Microscopic Epstein-Barr Virus-Positive Diffuse Large B-Cell Lymphoma of the Adrenal and Lymphoplasmacytic Lymphoma: De Novo Disease or Transformation?

Int J Surg Pathol. 2017 Jun;25(4):326-332

Authors: Moonim MT, Nasir A, Hubbard J, Ketley N, Fields P

Abstract
Lymphomas arising in the adrenal are rare, and to our knowledge, 2 cases of Epstein-Barr virus (EBV)-positive diffuse large B-cell lymphomas (DLBCL) in an adrenal pseudocyst have been reported. We report an incidental EBV-positive DLBCL arising in an adrenal pseudocyst in a 58-year-old man with a 7-year history of lymphoplasmacytic lymphoma (LPL). The DLBCL was present in the fibrinous exudate, while the LPL resided in the cyst wall. The patient underwent de-roofing of the same cyst 3 years previously; review of histology revealed foci of LPL in the cyst wall, but not of DLBCL. There have been reports of similar microscopic EBV-positive DLBCLs within enclosed cystic spaces. However, all these cases were incidental extranodal primary DLBCLs. Since residual LPL was present alongside DLBCL, with similar light chain restriction, we propose that this may represent transformation, rather than a de novo primary EBV-driven lymphoma.

PMID: 27913781 [PubMed - indexed for MEDLINE]

Preoperative Levels of Catecholamines and Metanephrines and Intraoperative Hemodynamics of Patients Undergoing Pheochromocytoma and Paraganglioma Resection.

Sun, 03/11/2018 - 16:52
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Preoperative Levels of Catecholamines and Metanephrines and Intraoperative Hemodynamics of Patients Undergoing Pheochromocytoma and Paraganglioma Resection.

Urology. 2017 Feb;100:131-138

Authors: Weingarten TN, Welch TL, Moore TL, Walters GF, Whipple JL, Cavalcante A, Bancos I, Young WF, Gruber LM, Shah MZ, McKenzie TJ, Schroeder DR, Sprung J

Abstract
OBJECTIVE: To determine whether, despite pharmacologic adrenergic receptor blockade, higher preoperative levels of catecholamines and metanephrines (adrenergic activity) are associated with increased intraoperative complications.
MATERIALS AND METHODS: Records of patients undergoing paraganglioma and pheochromocytoma (PGL-PCC) resection from January 1, 2000, to June 30, 2015, were reviewed for preoperative levels of adrenergic activity, intraoperative variability in blood pressure and heart rate (range), and postoperative outcomes (hypotension requiring treatment). Patients were categorized by maximum preoperative adrenergic activity by greater degree of abnormality, categorized as normal (≤100%) or 101%-200%, 201%-500%, 501%-1000%, and ≥1001% of upper limit of normal.
RESULTS: In total, 258 patients underwent intrathoracic or intra-abdominal PGL-PCC resection, of whom 240 received pretreatment with nonselective α1,2-blockers and 7 received pretreatment with selective α1-blockers. Intraoperative hemodynamic variability was greater with higher preoperative levels of adrenergic activity (P <.001). However, substantial variability was observed even with adrenergic activity levels within the normal range: systolic blood pressure (median [interquartile range], 75 [63-83] mm Hg) and heart rate (34 [26-43] beats per minute). Among patients with preoperative levels of adrenergic activity ≤500% vs ≥501% of the upper limit of normal, higher levels were associated with greater likelihood of postoperative diagnosis of volume overload (8% vs 2%, P = .04) and greater requirement for vasopressor infusions for hypotension (5% vs 1%, P = .01).
CONCLUSION: Substantial intraoperative hemodynamic instability was encountered in patients with PGL-PCC resection, regardless of preoperative hormonal activity level; therefore, universal preoperative adrenergic receptor blockade should be recommended. Postoperative hypotension was rare and more prevalent in those with higher preoperative hormonal activity.

PMID: 27769919 [PubMed - indexed for MEDLINE]

Doxazosin treatment of phaeochromocytoma during pregnancy: placental transfer and disposition in breast milk.

Sun, 03/11/2018 - 16:52
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Doxazosin treatment of phaeochromocytoma during pregnancy: placental transfer and disposition in breast milk.

Br J Clin Pharmacol. 2016 08;82(2):568-9

Authors: Versmissen J, Koch BC, Roofthooft DW, Ten Bosch-Dijksman W, van den Meiracker AH, Hanff LM, Visser W

PMID: 27194370 [PubMed - indexed for MEDLINE]

Two-stage resection of a bilateral pheochromocytoma and pancreatic neuroendocrine tumor in a patient with von Hippel-Lindau disease: A case report.

Sun, 03/04/2018 - 13:34

Two-stage resection of a bilateral pheochromocytoma and pancreatic neuroendocrine tumor in a patient with von Hippel-Lindau disease: A case report.

Int J Surg Case Rep. 2018 Feb 16;44:139-142

Authors: Endo Y, Kitago M, Miyajima A, Kurihara I, Kameyama K, Shinoda M, Yagi H, Abe Y, Hibi T, Takagi C, Nakano Y, Koizumi W, Itano O, Kitagawa Y

Abstract
INTRODUCTION: von Hippel-Lindau disease (vHL disease) is a hereditary disease in which tumors and cysts develop in many organs, in association with central nervous system hemangioblastomas, pheochromocytomas, and pancreatic tumors. We herein report a case of vHL disease (type 2A) associated with bilateral pheochromocytomas, pancreatic neuroendocrine tumors (PNET), and cerebellar hemangioblastomas treated via pancreatectomy after adrenalectomy.
CASE PRESENTATION: A 51-year-old woman presented with a cerebellar tumor, bilateral hypernephroma, and pancreatic tumor detected during a medical checkup. 18F-fluorodeoxyglucose positron emission tomography-computed tomography revealed a bilateral adrenal gland tumor and a tumor in the head of the pancreas, while an abdominal computed tomography examination revealed a 30-mm tumor with strong enhancement in the head of the pancreas. Cranial magnetic resonance imaging showed a hemangioblastoma in the cerebellum. Therefore, a diagnosis of vHL disease (type 2A) was made. Her family medical history included renal cell carcinoma in her father and bilateral adrenal pheochromocytoma and spinal hemangioblastoma in her brother. A detailed examination of endocrine function showed that the adrenal mass was capable of producing catecholamine. Treatment of the pheochromocytoma was prioritized, and therefore, laparoscopic left adrenalectomy and subtotal resection of the right adrenal gland were performed. Once the postoperative steroid levels were replenished, subtotal stomach-preserving pancreatoduodenectomy was performed for the PNET. After a good postoperative course, the patient was discharged in remission on the 11th day following surgery. Histopathological examination findings indicated NET G2 (MIB-1 index 10-15%) pT3N0M0 Stage II A and microcystic serous cystadenoma throughout the resected specimen. The patient is scheduled to undergo treatment for the cerebellar hemangioblastoma.
CONCLUSION: A two-staged resection is a safe and effective treatment option for bilateral pheochromocytoma and PNET associated with vHL disease.

PMID: 29501019 [PubMed - as supplied by publisher]

A black adrenal adenoma with high FDG uptake on PET/CT scan in a patient with esophageal carcinoma: A case report.

Sun, 03/04/2018 - 13:34

A black adrenal adenoma with high FDG uptake on PET/CT scan in a patient with esophageal carcinoma: A case report.

Int J Surg Case Rep. 2017 Dec 07;44:118-121

Authors: Matsumoto S, Hosoya Y, Lefor AK, Haruta H, Ui T, Kurashina K, Saito S, Ashizawa K, Sasaki T, Kitayama J, Sata N

Abstract
INTRODUCTION: Black adrenal adenoma (BAA) is a rare, benign adrenal lesion with a black or brown appearance. This is the first report of this lesion in a patient with a synchronous esophageal cancer and highlights the importance of considering a false positive finding on a Positron Emission Tomography (PET) scan, which might otherwise preclude resection.
PRESENTATION OF CASE: A 73-year-old male was diagnosed with mid-esophagus carcinoma. Computed tomography scan revealed an enlarged left adrenal gland. Plasma adrenocorticotropic hormones levels were normal. To characterize the adrenal lesion, a PET scan was obtained which showed high uptake of 18F-fluoro-2-deoxy-d-glucose (FDG), consistent with a metastasis, suggesting T3N2M1, clinical stage IV esophageal cancer. After two courses of neo-adjuvant therapy, sub-total esophagectomy and left adrenalectomy were performed. The adrenal tumor was soft, and black in color, diagnosed as a BAA on histology. The pathologic stage of the esophageal cancer was T3N0M0, Stage II. Six months after surgery, he is alive without recurrence.
DISCUSSION: High FDG uptake by an adrenal lesion on PET scan, as in this patient, usually suggests a metastatic lesion. Although rare, patients with esophageal cancer and adrenal metastases have been reported to have long-term survival, so it is important to characterize an adrenal lesion when found.
CONCLUSION: Most adrenal lesions with high FDG uptake are malignant, but BAA is also positive on PET scan. Although rare, BAA should be considered in patients with solitary adrenal lesions with high uptake on PET scan, even in the presence of a malignancy.

PMID: 29499515 [PubMed - as supplied by publisher]

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