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NCBI: db=pubmed; Term=adrenal tumor
Updated: 14 hours 55 min ago

Erratum: Adrenal collision tumor (parachordoma and ganglioneuroma): A case report.

Tue, 08/21/2018 - 06:22
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Erratum: Adrenal collision tumor (parachordoma and ganglioneuroma): A case report.

Mol Clin Oncol. 2018 Aug;9(2):238

Authors: Lai Y, Zhou L, Hu J, Li W, Cui L, Lai Y, Ni L

Abstract
[This corrects the article DOI: 10.3892/mco.2018.1610.].

PMID: 30101030 [PubMed - in process]

[A case of pheochromocytoma discovered during a twin pregnancy: a diagnosis not to be misunderstood and literature review].

Tue, 08/21/2018 - 06:22
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[A case of pheochromocytoma discovered during a twin pregnancy: a diagnosis not to be misunderstood and literature review].

Pan Afr Med J. 2018;29:168

Authors: Habra B, Mghari GE, Ansari NE

Abstract
The pheochromocytoma is a rare, but potentially serious, which can be revealed by pregnancy. Less than 250 cases described in the literature .The rarity of this association and similarity with pregnancy-induced hypertension explains the frequency of unknown diagnoses during pregnancy. A privileged time of discovery, the prognosis associated with early diagnosis, a multidisciplinary management. Hence the need to explore all arterial hypertension poorly defined or family nature during pregnancy. The certainty diagnosis is conducted by biological tests provided are thought of, a review of localization by ultrasound or magnetic resonance imaging (MRI), a medical preparation can choose according to the term to remove the tumor before or after delivery. We related a case of pheochromocytoma diagnosed during a twin pregnancy of 26 weeks of amenorrhea (SA), bringing us the elements of the clinical and laboratory diagnostic imaging, treatment and maternal and fetal prognosis.

PMID: 30050632 [PubMed - indexed for MEDLINE]

Treatment of sarcoidosis: grading the evidence.

Tue, 08/21/2018 - 06:22
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Treatment of sarcoidosis: grading the evidence.

Expert Rev Clin Pharmacol. 2018 Jul;11(7):677-687

Authors: James WE, Baughman R

Abstract
INTRODUCTION: Treatment of sarcoidosis recommendations are often based on clinical experience and expert opinion. However, there are an increasing number of studies which are providing evidence to support decisions regarding treatment. Areas covered: Several studies have identified factors associated with increased risk for organ failure or death ('danger'). There have been several studies focused on the role of treatment to improve quality of life for the patient. Sarcoidosis treatment often follows a progression, based on response. Corticosteroids remain the initial treatment of choice for most patients. Second-line therapy includes cytotoxic agents. Immunosuppressives such as methotrexate, azathioprine, leflunomide, and mycophenolate have all been reported as effective in sarcoidosis. Biologics and other agents are third-line therapy. The monoclonal antibodies directed against tumor necrosis factor have been shown to be particularly effective for advanced disease. Infliximab has been the most studied drug in this class. Newer treatments, including repository corticotropin injection and rituximab have been reported as effective in some cases. Expert commentary: In this review, we use the GRADE system to evaluate the currently available evidence and make recommendations regarding treatment.

PMID: 29883224 [PubMed - indexed for MEDLINE]

Integrating in vitro and in silico approaches to evaluate the "dual functionality" of palmatine chloride in inhibiting and disassembling Tau-derived VQIVYK peptide fibrils.

Tue, 08/21/2018 - 06:22
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Integrating in vitro and in silico approaches to evaluate the "dual functionality" of palmatine chloride in inhibiting and disassembling Tau-derived VQIVYK peptide fibrils.

Biochim Biophys Acta. 2018 07;1862(7):1565-1575

Authors: Haj E, Losev Y, Guru KrishnaKumar V, Pichinuk E, Engel H, Raveh A, Gazit E, Segal D

Abstract
BACKGROUND: Alzheimer's disease (AD) is the most common neurodegenerative disorder which is characterized by the deposits of intra-cellular tau protein and extra-cellular amyloid-β (Aβ) peptides in the human brain. Understanding the mechanism of protein aggregation and finding compounds that are capable of inhibiting its aggregation is considered to be highly important for disease therapy.
METHODS: We used an in vitro High-Throughput Screening for the identification of potent inhibitors of tau aggregation using a proxy model; a highly aggregation-prone hexapeptide fragment 306VQIVYK311 derived from tau. Using ThS fluorescence assay we screened a library of 2401 FDA approved, bio-active and natural compounds in attempt to find molecules which can efficiently modulate tau aggregation.
RESULTS: Among the screened compounds, palmatine chloride (PC) alkaloid was able to dramatically reduce the aggregation propensity of PHF6 at sub-molar concentrations. PC was also able to disassemble preformed aggregates of PHF6 and reduce the amyloid content in a dose-dependent manner. Insights obtained from MD simulation showed that PC interacted with the key residues of PHF6 responsible for β-sheet formation, which could likely be the mechanism of inhibition and disassembly. Furthermore, PC could effectively inhibit the aggregation of full-length tau and disassemble preformed aggregates.
CONCLUSIONS: We found that PC possesses "dual functionality" towards PHF6 and full-length tau, i.e. inhibit their aggregation and disassemble pre-formed fibrils.
GENERAL SIGNIFICANCE: The "dual functionality" of PC is valuable as a disease modifying strategy for AD, and other tauopathies, by inhibiting their progress and reducing the effect of fibrils already present in the brain.

PMID: 29634991 [PubMed - indexed for MEDLINE]

Minimally invasive adrenal surgery: virtue or vice?

Tue, 08/21/2018 - 06:22
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Minimally invasive adrenal surgery: virtue or vice?

Future Oncol. 2018 Feb;14(3):267-276

Authors: Winoker JS, Ahlborn DT, Omidele OO, Fernandez-Ranvier G, Derweesh IH, Mehrazin R

Abstract
Adrenocortical carcinoma (ACC) is a rare malignancy associated with poor prognosis despite available treatments. In patients with localized or locally advanced disease, complete resection with negative margins offers the only potential for cure. Unfortunately, most patients develop local and distant recurrence following initial resection highlighting the importance of meticulous surgical technique in the hands of an experienced surgeon. While minimally invasive surgery (MIS) has supplanted open surgery for small to medium-sized benign adrenal tumors, controversy surrounds the use of MIS for resection of ACC. We sought to provide an overview of the key oncological principles in the surgical management of ACC and to critically review the literature comparing outcomes between the open and MIS approaches.

PMID: 29345155 [PubMed - indexed for MEDLINE]

Quercetin restores corticosteroid sensitivity in cells from patients with chronic obstructive pulmonary disease.

Tue, 08/21/2018 - 06:22
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Quercetin restores corticosteroid sensitivity in cells from patients with chronic obstructive pulmonary disease.

Exp Lung Res. 2017 Nov - Dec;43(9-10):417-425

Authors: Mitani A, Azam A, Vuppusetty C, Ito K, Mercado N, Barnes PJ

Abstract
Corticosteroid resistance is a major barrier to the effective treatment of chronic obstructive pulmonary disease (COPD). Oxidative stress from cigarette smoke and chronic inflammation is likely to induce this corticosteroid insensitivity. Quercetin is a polyphenol that has been reported to be an active oxygen scavenger as well as a functional adenosine monophosphate-activated protein kinase (AMPK) activator. The aim of this study was to investigate the effect of quercetin on corticosteroid responsiveness in COPD cells. Corticosteroid sensitivity was examined in human monocytic U937 cells exposed to cigarette smoke extract (CSE) and peripheral blood mononuclear cells (PBMC) collected from patients with COPD. Corticosteroid sensitivity was determined as the dexamethasone concentration causing 40% inhibition of tumor necrosis factor alpha-induced CXCL8 production (Dex-IC40) in the presence or absence of quercetin. In U937 cells, treatment with quercetin activated AMPK and induced expression of nuclear factor erythroid 2-related factor 2, and consequently reversed CSE-induced corticosteroid insensitivity. PBMC from patients with COPD showed corticosteroid insensitivity compared with those from healthy volunteers, and treatment with quercetin restored corticosteroid sensitivity. In conclusion, quercetin restores corticosteroid sensitivity, and has the potential to be a novel treatment in combination with corticosteroids in COPD.

PMID: 29227717 [PubMed - indexed for MEDLINE]

Clinical Case Seminar: Postmenopausal androgen excess-challenges in diagnostic work-up and management of ovarian thecosis.

Tue, 08/21/2018 - 06:22
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Clinical Case Seminar: Postmenopausal androgen excess-challenges in diagnostic work-up and management of ovarian thecosis.

Clin Endocrinol (Oxf). 2018 Jan;88(1):13-20

Authors: Mamoojee Y, Ganguri M, Taylor N, Quinton R

Abstract
Postmenopausal hyperandrogenism can be tumour- or non-tumour-related, with pathology residing either in the ovary or adrenal gland(s). The tempo of investigation is determined by the clinical severity of hyperandrogenism (presence/absence of actual virilisation) and degree of serum testosterone elevation. When clinical or biochemical hyperandrogenism is severe, rapidly developing, or associated with hypercortisolism, screening for adrenocortical or ovarian carcinoma with cross-sectional imaging should be prioritised over detailed biochemical evaluation. Adrenal hyperandrogenism is readily characterised, both biochemically and radiologically. By contrast, even a combination of high-resolution imaging with laboratory evaluation, including dynamic endocrine testing, often cannot distinguish between ovarian hyperthecosis (OH) and virilising ovarian tumour (VOT); a definitive diagnosis usually emerging only after histological examination of excised ovaries. VOTs are typically below the resolution-limit of current imaging modalities and exhibit suppression of gonadotropin-dependent androgen secretion with GnRH-analogue therapy. Thus, for well-characterised ovarian hyperandrogenism, laparoscopic bilateral salpingo-oophorectomy may serve both as a diagnostic and therapeutic procedure. Nevertheless, women unable or unwilling to undergo ovarian surgery can be reassured that malignant VOTs are exceedingly rare and that long-term medical therapy with oral antiandrogens or GnRH-analogues is safe and well-tolerated. OH is strongly associated with insulin-resistance, with hyperinsulinaemia acting synergistically with raised gonadotropin levels to stimulate thecal cell hyperplasia and androgen secretion by the postmenopausal ovary, which lacks granulosa cell aromatase activity and thus cannot convert testosterone to 17 beta estradiol. Thus, features of metabolic syndrome may indicate OH, and significant reductions in androgens can thereby potentially be achieved with lifestyle measures and/or insulin-sensitising drugs.

PMID: 28980338 [PubMed - indexed for MEDLINE]

TERT structural rearrangements in metastatic pheochromocytomas.

Tue, 08/21/2018 - 06:22
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TERT structural rearrangements in metastatic pheochromocytomas.

Endocr Relat Cancer. 2018 Jan;25(1):1-9

Authors: Dwight T, Flynn A, Amarasinghe K, Benn DE, Lupat R, Li J, Cameron DL, Hogg A, Balachander S, Candiloro ILM, Wong SQ, Robinson BG, Papenfuss AT, Gill AJ, Dobrovic A, Hicks RJ, Clifton-Bligh RJ, Tothill RW

Abstract
Pheochromocytomas (PC) and paragangliomas (PGL) are endocrine tumors for which the genetic and clinicopathological features of metastatic progression remain incompletely understood. As a result, the risk of metastasis from a primary tumor cannot be predicted. Early diagnosis of individuals at high risk of developing metastases is clinically important and the identification of new biomarkers that are predictive of metastatic potential is of high value. Activation of TERT has been associated with a number of malignant tumors, including PC/PGL. However, the mechanism of TERT activation in the majority of PC/PGL remains unclear. As TERT promoter mutations occur rarely in PC/PGL, we hypothesized that other mechanisms - such as structural variations - may underlie TERT activation in these tumors. From 35 PC and four PGL, we identified three primary PCs that developed metastases with elevated TERT expression, each of which lacked TERT promoter mutations and promoter DNA methylation. Using whole genome sequencing, we identified somatic structural alterations proximal to the TERT locus in two of these tumors. In both tumors, the genomic rearrangements led to the positioning of super-enhancers proximal to the TERT promoter, that are likely responsible for the activation of the normally tightly repressed TERT expression in chromaffin cells.

PMID: 28974544 [PubMed - indexed for MEDLINE]

Diagnostic performance of unenhanced computed tomography and 18 F-fluorodeoxyglucose positron emission tomography in indeterminate adrenal tumours.

Tue, 08/21/2018 - 06:22
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Diagnostic performance of unenhanced computed tomography and 18 F-fluorodeoxyglucose positron emission tomography in indeterminate adrenal tumours.

Clin Endocrinol (Oxf). 2018 Jan;88(1):30-36

Authors: Delivanis DA, Bancos I, Atwell TD, Schmit GD, Eiken PW, Natt N, Erickson D, Maraka S, Young WF, Nathan MA

Abstract
OBJECTIVE: Evidence on the diagnostic performance of adrenal imaging is limited. We aimed to assess the diagnostic performance of unenhanced computed tomography (CT) and 18 F-fluorodeoxyglucose (18 FDG) positron emission tomography (PET)/CT imaging in a high-risk population for adrenal malignancy using an optimal reference standard.
DESIGN: Retrospective cohort study.
METHODS: Imaging studies of patients with adrenal nodules who underwent adrenal biopsy and/or adrenalectomy between 1994 and 2014 were reviewed and compared to the reference standard of histology. Eighty % of patients presented with known or suspected extra-adrenal malignancy.
RESULTS: Unenhanced abdominal CT was performed in 353 patients with adrenal lesions; median size was 3 (0.7-15) cm and median radiodensity was 33 (-21-78) Hounsfield units (HU). Radiodensity of >10 HU diagnosed malignancy with a sensitivity of 100%, specificity of 33%, positive predictive value (PPV) of 72% and negative predictive value (NPV) of 100%. 18 FDG-PET/CT was performed in 89 patients; median tumour size was 2.1 (0.7-9.2) cm. Maximum standardized uptake (SUV max) was higher in malignant lesions when compared to benign lesions (median=10 [2.3-29.4] vs 3.7 [1.4-24.5], respectively, P<.0001). Similarly, median SUV max lesion to SUV max liver ratio (ALR) in malignant lesions was higher than in benign lesions (median=3 [0.74-13.4] vs 1.2 [0.5-6.6], respectively, P<.0001). 18 FDG-PET/CT ALR >1.8 diagnosed malignancy with a sensitivity of 87%, specificity of 84%, PPV of 85% and NPV of 86%.
CONCLUSION: Noncontract CT radiodensity of ≤10 HU excludes malignancy even in a high-risk population. For indeterminate adrenal lesions, given a superior specificity, 18 FDG-PET/CT could be considered as a second stage imaging study.

PMID: 28815667 [PubMed - indexed for MEDLINE]

Pheochromocytoma Masked by Mutation in the TH Gene.

Tue, 08/21/2018 - 06:22
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Pheochromocytoma Masked by Mutation in the TH Gene.

Clin Chem. 2016 07;62(7):924-8

Authors: Abid K, Afshar K, Fontana E, Ducry J, Rotman S, Stauffer E, Fellmann F, Tschopp O, Bhuiyan ZA, Grouzmann E

PMID: 27354566 [PubMed - indexed for MEDLINE]

GeneReviews®

Tue, 08/21/2018 - 06:22
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GeneReviews®

Book. 1993

Authors: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Stephens K, Amemiya A

Abstract
CLINICAL CHARACTERISTICS: Carney complex (CNC) is characterized by skin pigmentary abnormalities, myxomas, endocrine tumors or overactivity, and schwannomas. Pale brown to black lentigines are the most common presenting feature of CNC and typically increase in number at puberty. Cardiac myxomas occur at a young age, may occur in any or all cardiac chambers, and can manifest as intracardiac obstruction of blood flow, embolic phenomenon, and/or heart failure. Other sites for myxomas include the skin, breast, oropharynx, and female genital tract. Primary pigmented nodular adrenocortical disease (PPNAD), which causes Cushing syndrome, is the most frequently observed endocrine tumor in CNC, occurring in approximately 25% of affected individuals. Large-cell calcifying Sertoli cell tumors (LCCSCTs) are observed in one third of affected males within the first decade and in most adult males. Up to 75% of individuals with CNC have multiple thyroid nodules, most of which are nonfunctioning thyroid follicular adenomas. Clinically evident acromegaly from a growth hormone (GH)-producing adenoma is evident in approximately 10% of adults. Psammomatous melanotic schwannoma (PMS), a rare tumor of the nerve sheath, occurs in an estimated 10% of affected individuals. The median age of diagnosis is 20 years.
DIAGNOSIS/TESTING: The diagnosis of CNC is established in a proband with two or more major diagnostic criteria and/or by identification of a heterozygous germline pathogenic variant in PRKAR1A on molecular genetic testing.
MANAGEMENT: Treatment of manifestations: Open-heart surgery for cardiac myxomas; surgical excision of cutaneous and mammary myxomas; bilateral adrenalectomy for Cushing syndrome; transsphenoidal surgery for pituitary adenoma; surgery for cancerous thyroid adenomas; orchiectomy for boys with aggressive LCCSCT and gynecomastia to avoid premature epiphyseal fusion and induction of central precocious puberty (mild gynecomastia may be treated medically); surgery to remove primary and/or metastatic PMS. Prevention of primary manifestations: Surgical removal of a cardiac myxoma prior to the development of heart dysfunction, stroke, or other embolism. Prevention of secondary complications: Medical or surgical treatment of endocrine manifestations may prevent the metabolic abnormalities from Cushing syndrome or arthropathy and complications from acromegaly. Surveillance: For prepubertal children: echocardiography annually or biannually for those with a history of excised myxoma; testicular ultrasound with close monitoring of linear growth rate and annual pubertal staging. For postpubertal children and adults: echocardiogram annually or biannually for those with a history of excised myxoma; annual testicular ultrasound; baseline thyroid ultrasound with repeat as necessary; baseline transabdominal ultrasound of the ovaries with repeat as necessary; annual urinary free cortisol levels; annual serum IGF-1 levels. Further evaluation in all age groups may include: diurnal cortisol levels, dexamethasome stimulation test, and adrenal computed tomography for primary pigmented nodular adrenocortical disease; pituitary MRI, 3-hour oral glucose tolerance test, and 90-minute thyroid releasing hormone testing for gigantism/acromegaly; MRI (brain, spine, chest, abdomen, retroperitoneum, pelvis) for psammamotous melanotic schwannoma. Evaluation of relatives at risk: When the family-specific pathogenic variant is known, molecular genetic testing to clarify the genetic status of at-risk family members so that appropriate evaluation and surveillance can enable early diagnosis of treatable manifestations.
GENETIC COUNSELING: CNC is inherited in an autosomal dominant manner. Approximately 70% of individuals diagnosed with CNC have an affected parent; approximately 30% have a de novo pathogenic variant. Each child of an individual with CNC has a 50% chance of inheriting the pathogenic variant. Prenatal testing for pregnancies at increased risk is possible if the pathogenic variant in the family is known.


PMID: 20301463

adrenal tumor; +41 new citations

Tue, 08/14/2018 - 03:28

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

adrenal tumor

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PubMed comprises more than millions of citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.

adrenal tumor; +34 new citations

Tue, 07/31/2018 - 00:07

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

adrenal tumor

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PubMed comprises more than millions of citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.

adrenal tumor; +25 new citations

Mon, 07/23/2018 - 23:46

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

adrenal tumor

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PubMed comprises more than millions of citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.

adrenal tumor; +26 new citations

Mon, 07/16/2018 - 23:24

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

adrenal tumor

These pubmed results were generated on 2018/07/16

PubMed comprises more than millions of citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.

65 YEARS OF THE DOUBLE HELIX: Classification of endocrine tumors in the age of integrated genomics.

Mon, 07/09/2018 - 22:12
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65 YEARS OF THE DOUBLE HELIX: Classification of endocrine tumors in the age of integrated genomics.

Endocr Relat Cancer. 2018 Aug;25(8):T171-T187

Authors: Giordano TJ

Abstract
The classification of human cancers represents one of the cornerstones of modern pathology. Over the last century, surgical pathologists established the current taxonomy of neoplasia using traditional histopathological parameters, which include tumor architecture, cytological features and cellular proliferation. This morphological classification is efficient and robust with high reproducibility and has served patients and health care providers well. The most recent decade has witnessed an explosion of genome-wide molecular genetic and epigenetic data for most cancers, including tumors of endocrine organs. The availability of this expansive multi-dimensional genomic data, collectively termed the cancer genome, has catalyzed a re-examination of the classification of endocrine tumors. Here, recent cancer genome studies of various endocrine tumors, including those of the thyroid, pituitary and adrenal glands, pancreas, small bowel, lung and skin, are presented with special emphasis on how genomic insights are impacting endocrine tumor classification.

PMID: 29980645 [PubMed - in process]

Caval replacement strategy in pediatric retroperitoneal tumors encasing the vena cava: a single-center experience and review of literature.

Mon, 07/09/2018 - 22:12
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Caval replacement strategy in pediatric retroperitoneal tumors encasing the vena cava: a single-center experience and review of literature.

J Pediatr Surg. 2018 Jun 10;:

Authors: Grimaldi C, Bertocchini A, Crocoli A, de Ville de Goyet J, Castellano A, Serra A, Spada M, Inserra A

Abstract
BACKGROUND: Complete encasement of the inferior vena cava by retroperitoneal tumors is rare. Although replacement of the vena cava has been considered for various conditions in adults, it is rarely used in children except for challenging resections and as a last chance approach - often aiming more at debulking than cure.
MATERIALS AND METHODS: From January 2009 to February 2017, 4 patients (2 adrenal neuroblastomas, 1 renal cell carcinoma, 1 infantile fibrosarcoma) underwent elective en-bloc resection of tumor and of the infrahepatic portion of the inferior vena cava (IVC), with planned IVC prosthetic replacement. In three cases a portion of the left renal vein had to be resected as well, with the vein reanastomosed onto the prosthesis, and a concomitant auto-transplantation of the right kidney was associated in one neuroblastoma patient.
RESULTS: All patients had an uncomplicated postoperative course. In one patient, the prosthetic conduit is patent at long-term (43 months), while the middle portion of the prosthesis did eventually thrombose at mid-term after surgery in the three others - with no related symptoms. Interestingly, all renal venous reconstructions remain patent. Three patients (2 neuroblastomas and 1 infantile fibrosarcoma) are alive and disease-free at 43, 74 and 108 months after surgery, respectively. One patient with renal cell carcinoma died of recurrence of the disease 21 months after surgery.
CONCLUSION: Resection and reconstruction of the vena cava, including the renal vein portion, can be considered and planned electively in case of tumoral encasement. This strategy is associated with good tolerance of the operation, low morbidity and satisfactory long-term function, even in cases with progressive and/or secondary partial thrombosis.
LEVEL OF EVIDENCE: IV.

PMID: 29980348 [PubMed - as supplied by publisher]

Pediatric paraganglioma of the posterior mediastinum: A case report and review of literature.

Mon, 07/09/2018 - 22:12
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Pediatric paraganglioma of the posterior mediastinum: A case report and review of literature.

Medicine (Baltimore). 2018 Jul;97(27):e11212

Authors: Yuan M, Xu C, Yang G, Wang W

Abstract
RATIONALE: Paraganglioma is rare in children and most pheochromocytomas originate in the adrenal gland.
PATIENT CONCERNS: The clinical characteristics, diagnosis, and managements in a 9-year-old boy who presented with vomiting and occasional headache with a blood pressure of 210/170 mm Hg was retrospectively reviewed. CT scan of the chest revealed a 7 × 5-cm-sized soft tissue mass in the left paraspinal area. Biochemical reports revealed elevated levels of serum norepinephrine, urine norepinephrine, urine dopamine, and serum neuron specific enolase.
DIAGNOSES: The immunohistochemical studies suggested that the tumor was a paraganglioma of the posterior mediastinum.
INTERVENTIONS: The patient underwent mass resection with thoracotomy. Before operation, the patient was prepared by orally administering captopril, propranolol hydrochloride, and phenoxybenzamine. Body fluid volume was also prepared by vein and mouth in 3 days.
OUTCOMES: The patient was followed up postoperatively for 1 year without recurrence.
LESSONS: We should be highly vigilant the pediatric tumor of the posterior mediastinum with vomiting and headache as the first clinical manifestation.

PMID: 29979384 [PubMed - in process]

Pheochromocytoma and Paraganglioma in Neurofibromatosis type 1: frequent surgeries and cardiovascular crises indicate the need for screening.

Mon, 07/09/2018 - 22:12
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Pheochromocytoma and Paraganglioma in Neurofibromatosis type 1: frequent surgeries and cardiovascular crises indicate the need for screening.

Clin Diabetes Endocrinol. 2018;4:15

Authors: Petr EJ, Else T

Abstract
Background: Pheochromocytomas and Paragangliomas (PCC/PGL) are rare endocrine tumors that are mostly benign, but often hormone producing, causing significant morbidity and mortality due to excess catecholamine secretion and cardiovascular crises. It is estimated that 30% of PCC/PGL are due to germline mutations, including Neurofibromatosis type 1 (NF1). There is little published data describing the phenotype of NF1-associated PCC/PGL and there are no established recommendations for PCC/PGL screening in NF1.
Methods: We conducted a retrospective chart review of 17 patients with NF1-associated PCC/PGL who received care at a large academic referral center between the years of 1992-2016.
Results: Average age of diagnosis was 42 years old. Both genders were equally affected. Average tumor size was 3.9 cm. Nine patients were hypertensive; one had orthostatic hypotension; three had tachycardia; the remaining two patients had normal BP and HR. Most tumors were benign, unilateral adrenal tumors that were hormonally active. Two had metastatic disease. Six patients experienced cardiovascular crises; three of which occurred during elective surgeries for neurofibroma removal, and a fourth occurred during labor and delivery.
Conclusion: These data highlight the importance of screening for PCC/PGL in NF1, especially prior to surgical procedures and pregnancy, labor and delivery as these events can trigger a cardiovascular crisis. Screening is easily accomplished with plasma or urine free fractionated metanephrine levels.

PMID: 29977594 [PubMed]

The Characteristics and Trends in Adrenocortical Carcinoma: A United States Population Based Study.

Mon, 07/09/2018 - 22:12
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The Characteristics and Trends in Adrenocortical Carcinoma: A United States Population Based Study.

J Clin Med Res. 2018 Aug;10(8):636-640

Authors: Sharma E, Dahal S, Sharma P, Bhandari A, Gupta V, Amgai B, Dahal S

Abstract
Background: Adrenocortical carcinoma (ACC) is a rare malignancy with poor prognosis. Data on the incidence of ACC, however, are scarce and not recent. The purpose of this study was to characterize the tumor and the patients developing ACC over the last four decades using a large population based database.
Methods: We identified all cases of ACC diagnosed between 1973 - 2014 from the Surveillance, Epidemiology, and End Results-18 registry. Descriptive analyses were used for all extracted demographic, clinical, pathological, therapeutic and survival data, and were compared between the four time periods of 1973 to 1984, 1985 to 1994, 1995 to 2004 and 2005 to 2014 using Chi-square tests for categorical variables and one-way analysis of variance for continuous variables.
Results: There were a total of 2,014 cases of ACC between 1973 and 2014 with an age-adjusted incidence of 1.02 per million populations. The median age at diagnosis was 55 years with the majority of them being females and whites. The proportion of cases by different genders, races and age at diagnosis had not changed significantly over time. These malignancies were mostly the only primary malignancy, unilateral and of high grades at diagnosis. Surgical resection of the tumor remained the mainstay of treatment. However, there was a significant increase in the use of adjuvant radiotherapy, adjuvant chemotherapy and chemotherapy alone in recent times. The median survival time was 17 months, but continues to decrease in recent time periods.
Conclusions: ACC continues to be a rare malignancy in the United States. However, most cases continue to be diagnosed only in advanced stages and are associated with poor survival. These findings underline the need for specific diagnostics tools with new and more effective treatment options.

PMID: 29977421 [PubMed]

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