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NCBI: db=pubmed; Term=adrenal tumor
Updated: 15 hours 17 min ago

The role of deep hypothermic circulatory arrest in surgery for renal or adrenal tumor with vena cava thrombus: a single-institution experience.

Mon, 07/09/2018 - 22:12
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The role of deep hypothermic circulatory arrest in surgery for renal or adrenal tumor with vena cava thrombus: a single-institution experience.

J Cardiothorac Surg. 2018 Jul 05;13(1):85

Authors: Zhu P, Du S, Chen S, Zheng S, Hu Y, Liu L, Zheng S

Abstract
BACKGROUND: The aim of this study was to review our experience in managing renal or adrenal tumors with level III or IV inferior vena cava thrombus by using deep hypothermic circulatory arrest (DHCA), and to evaluate survival outcomes.
METHODS: Between September 2004 and March 2016, we treated 33 patients with renal or adrenal malignancy tumor and thrombus extending into the inferior vena cava. Patients were identified according to radiographic records and operative findings. Clinicopathological and operative characteristics were recorded, and comparisons of clinical and operative characteristics through DHCA were performed. A Cox regression model was used to determine predictors of perioperative mortality.
RESULTS: Twenty-one out of 33 patients with level III (n = 15), level IV (n = 5), or level II (n = 1) renal or adrenal tumors were treated surgically through cardiopulmonary bypass (CPB) with DHCA, and 12 patients with level II or III tumors were treated surgically through normothermic CPB. Three complications were observed, and one death occurred perioperatively, owing to multiple organ failure. The overall perioperative mortality was 4.7%. There were significant differences in the clinicopathological characteristics, operative duration, estimated blood loss, transfusions and hospital stay depending on use of DHCA. Multivariate analysis indicated that the operative duration (OR, 3.78; P < 0.001), estimated blood loss (OR, 1.08; P = 0.02), and transfusion (OR, 2.13; P = 0.038) during/after surgery were positively associated with higher mortality and morbidity. DHCA failed to reach statistical significance (P = 0.378).
CONCLUSIONS: Use of CPB and DHCA to treat renal or adrenal tumors allows for complete tumor resection, especially at the T4 stage. Although it can cause physical damage, this technique does not increase operative risk and is a relatively safe approach.

PMID: 29976214 [PubMed - in process]

Surgical approach to adrenal ganglioneuroma: Case report and literature review.

Mon, 07/09/2018 - 22:12
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Surgical approach to adrenal ganglioneuroma: Case report and literature review.

Arch Ital Urol Androl. 2018 06 30;90(2):145-146

Authors: Abate D, Giusti G, Caria N, Lucci Chiarissi M, De Lisa A

Abstract
OBJECTIVE: Ganglioneuroma (GN) is a benign tumor with a slow growth that can originate from any paravertebral sympathetic plexus. It is usually asymptomatic or with nonspecific symptoms. TC and RM scan are helpful to study GN. It is usually represented by an ovalshaped retroperitoneal mass or, in case of adrenal impairment, by low radiologic contrast media attenuation. Surgical treatment is mandatory. Literature shows how the laparoscopic approach is the most used, especially in lesions that are 6 cm or smaller. Our purpose is to describe our experience on an incidental adrenal GN of about 5 cm treated by the laparoscopic transperitoneal approach.
MATERIALS AND METHODS: A 33-year-old male had ultrasound occasional finding of an about 4 cm adrenal mass. TC and RM scan identified a retroperitoneal mass (max diameter 48 mm). The lesion was removed with a transperitoneal laparoscopic approach.
RESULTS: No intraoperative or postoperative complications occurred. The patient was discharged 3 days after surgery.
CONCLUSIONS: Up to the present laparoscopic surgery is the best approach for GN treatment.

PMID: 29974732 [PubMed - in process]

Protective effect and mechanism of theanine on lipopolysaccharide-induced inflammation and acute liver injury in mice.

Mon, 07/09/2018 - 22:12
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Protective effect and mechanism of theanine on lipopolysaccharide-induced inflammation and acute liver injury in mice.

J Agric Food Chem. 2018 Jul 03;:

Authors: Wang D, Gao Q, Zhao G, Kan Z, Wang X, Wang H, Huang J, Wang T, Qian F, Ho CT, Wang Y

Abstract
Theanine, a unique bioactive constituent from tea (Camellia sinensis) leaves, is widely used as a functional ingredient and dietary supplement. To evaluate the anti-inflammatory and hepatoprotective effects of theanine and its molecular mechanism, the lipopolysaccharide (LPS)-induced inflammation mouse model was employed in this study. The survival rate of mice in the theanine-treated group increased significantly compared with that of LPS-only group mice. Furthermore, ICR male mice were randomly divided into three or four groups: control, LPS (LPS treatment only), LPS + theanine (20 mg/kg/day), and theanine (theanine treatment only). The results showed that compared with the LPS group, the liver damage and oxidative stress of the theanine-treated group decreased significantly, based on plasma alanine aminotransferase (ALT) and aspartate aminotransferase (AST) concentrations, hepatic total superoxide dismutase (T-SOD) and malondialdehyde (MDA) levels, and histological scores and apoptosis [terminal deoxynucleotide transferase-mediated deoxyuridine triphosphate nick end-labeling (TUNEL) staining and caspase-3 activity] in the liver tissues. Furthermore, compared with no treatment, pretreatment with theanine significantly decreased the release of interleukin (IL)-1β and tumor necrosis factor (TNF)-α, inhibited the expression of several inflammatory factors (including IL-1β, TNF-α, and IL-6), and increased the IL-10/interferon (IFN)-γ ratio in the hepatic tissues. In the LPS-induced inflammation model, theanine inhibited the expression of proinflammatory mediators involved in the nuclear factor-kappa B (NF-κB) pathway, such as inducible nitric oxide synthase (iNOS) and matrix metalloproteinase-3 (MMP-3), and attenuated the phosphorylation of NF-κB in the hepatic tissues. Moreover, theanine suppressed the acute-phase response (elevated nitric oxide and C-reactive protein levels). Furthermore, theanine suppressed the LPS-induced inflammatory state by normalizing hypothalamic-pituitary-adrenal (HPA) axis hyperactivity. Taken together, the results suggest that theanine potentially ameliorates LPS-induced inflammation and acute liver injury; molecular mechanism of action may involve normalization of HPA axis hyperactivity and inactivation of the NF-κB signaling pathway.

PMID: 29969892 [PubMed - as supplied by publisher]

Higher sympathetic activity as a risk factor for skeletal deterioration in pheochromocytoma.

Mon, 07/09/2018 - 22:12
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Higher sympathetic activity as a risk factor for skeletal deterioration in pheochromocytoma.

Bone. 2018 Jun 30;:

Authors: Kim BJ, Kwak MK, Kim JS, Lee SH, Koh JM

Abstract
Despite the potential biological importance of sympathetic activity in human bone metabolism, its effects on bone microarchitecture, a key determinant of bone quality, has not been thoroughly studied. In the present study, we investigated the lumbar spine trabecular bone score (TBS) as an indicator of skeletal deterioration in pheochromocytoma. Among 620 consecutive patients with newly diagnosed adrenal incidentaloma, 29 with histologically confirmed pheochromocytoma (a catecholamine-secreting neuroendocrine tumor) and 266 with nonfunctional adrenal incidentaloma were defined as cases and controls, respectively. After adjustment for confounders, subjects with pheochromocytoma had 2.9% lower lumbar spine TBS than those without pheochromocytoma (P = 0.038). Moreover, urinary normetanephrine level, but not urinary metanephrine level, was inversely correlated with lumbar spine TBS (P = 0.009). Subjects in the highest urinary normetanephrine quartile showed markedly lower lumbar spine TBS than those in the lowest quartile (P = 0.018), in a dose-response manner across increasing urinary normetanephrine quartile categories (P for trend = 0.021). Consistent with the results of previous studies, subjects with pheochromocytoma had significantly lower bone mass at the lumbar spine and higher serum level of C-terminal telopeptide of type I collagen than controls (P = 0.013 and 0.002, respectively). These findings provide clinical evidence that catecholamine excess and the resultant sympathetic overstimulation in pheochromocytoma may contribute to bone fragility, especially in the trabecular bone, through a weak microarchitecture in addition to a lower bone mass and increased bone resorption, and support the possibility of pheochromocytoma as a secondary cause of osteoporosis.

PMID: 29969750 [PubMed - as supplied by publisher]

What the radiologist needs to know: the role of preoperative computed tomography in selection of operative approach for adrenalectomy and review of operative techniques.

Mon, 07/09/2018 - 22:12
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What the radiologist needs to know: the role of preoperative computed tomography in selection of operative approach for adrenalectomy and review of operative techniques.

Abdom Radiol (NY). 2018 Jul 02;:

Authors: Rowe SP, Lugo-Fagundo C, Ahn H, Fishman EK, Prescott JD

Abstract
Adrenalectomy is the standard of care for management of many adrenal tumor types and, in the United States alone, approximately 6000 adrenal surgeries are performed annually. Two general approaches to adrenalectomy have been described; (1) the open approach, in which a diseased adrenal is removed through a large (10-20 cm) abdominal wall incision, and (2) the minimally invasive approach, in which laparoscopy is used to excise the gland through incisions generally no longer than 1-2 cm. Given these disparate technique options, clear preoperative characterization of those specific disease features that inform selection of adrenalectomy approach is critically important to the surgeon. Because most of these features are directly assessed via preoperative abdominal imaging, in particular computed tomography (CT) scanning, a clear mutual understanding among surgeons and radiologists of those adrenal tumor features impacting operative approach selection is vital for planning adrenal surgery. In this context, we review the preoperative CT imaging features that specifically inform adrenalectomy approach selection, provide illustrative examples from our institution's imaging and surgical archives, and provide a stepwise guide to both the open and laparoscopic adrenalectomy approaches.

PMID: 29967985 [PubMed - as supplied by publisher]

The role of Insulin-like Growth Factor (IGF) system in the adrenocortical tumors.

Mon, 07/09/2018 - 22:12
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The role of Insulin-like Growth Factor (IGF) system in the adrenocortical tumors.

Minerva Endocrinol. 2018 Jul 02;:

Authors: Altieri B, Colao A, Faggiano A

Abstract
INTRODUCTION: The different presentation of adrenocortical tumors in benign adenoma (ACA) or adrenocortical carcinoma (ACC) is related to the variability at the molecular level. The insulin-like growth factor (IGF) system is one of the most frequently altered pathways in ACC. In this review we will critically analyze the evidence regarding the pathogenic role of the IGF system in adrenal tumorigenesis, focusing on ACC. We will also examine the pre-clinical and clinical studies which investigated the targeting of the IGF system as a therapeutic approach in ACC.
EVIDENCE ACQUISITION: The IGF system plays a crucial role in the embryogenesis of adrenal glands. No significant alterations of the IGF system were observed in ACA. In ACC, the IGF2 overexpression is one of the most frequent molecular change presented in more than 85% of cases. However, IGF2 seems to be only a tumor progression factor which requires additional hits to trigger adrenal tumorigenesis. Also, the IGF1R appears to be higher expressed in ACC. Many IGF1R target-drugs have been developed to inhibit the activation of the IGF system. Preclinical studies using antibody or tyrosine kinase which target the IGF1R, or the dual-targeting of IGF1R and insulin receptor (IR) reduced ACC cells proliferation both in vitro and in vivo in mouse xenograft model. However, these promising results were not confirmed in clinical trials.
CONCLUSIONS: Nowadays, predictive markers for the response of target-IGF therapy are missing and further studies which investigate new molecular markers and evaluate the entire IGF receptors, including the IR, are urgently needed.

PMID: 29963827 [PubMed - as supplied by publisher]

A 15-Month-Old Girl Presenting With Clitoromegaly and a Chest Mass.

Mon, 07/09/2018 - 22:12
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A 15-Month-Old Girl Presenting With Clitoromegaly and a Chest Mass.

Semin Pediatr Neurol. 2018 Jul;26:128-131

Authors: Darcy C, Ullrich NJ

Abstract
A 15-month-old girl was initially referred for endocrine evaluation for clitoromegaly and subsequently found to have an adrenal mass that tracked along the paravertebral region and was associated with increased vascular markings along the skin. Neurologic examination was normal. Magnetic resonance imaging of the chest mass demonstrated a serpiginous lesion along the intercostal margins. Initial differential diagnosis included neuroblastoma, ganglioneuroblastoma, vascular lesion, or nerve sheath tumor. Biopsy was consistent with plexiform neurofibroma. Subsequent examination revealed features consistent with neurofibromatosis type 1. A review of clinical features of NF1 is provided in the case report.

PMID: 29961503 [PubMed - in process]

Evaluation of computed tomography vascular reconstruction for the localization diagnosis of perigastric mass.

Mon, 07/09/2018 - 22:12
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Evaluation of computed tomography vascular reconstruction for the localization diagnosis of perigastric mass.

Medicine (Baltimore). 2018 Jun;97(26):e11177

Authors: Wang P, Zhang CZ, Wang GB, Li YY, Jiang XY, Fang FJ, Li XX, Bian J, Cao XS, Zhong XF

Abstract
BACKGROUND: The aim of this study was to evaluate the utility of computed tomography (CT) vascular reconstruction in the localization diagnosis of perigastric mass.
METHODS: Fifty-eight patients with pathologically detected perigastric mass underwent abdominal dynamic contrast-enhanced CT. CT vascular reconstructions were produced from arterial phase data using volume rendering (VR), multiplanar reconstruction (MPR), and maximal intensity projection (MIP). Image analysis was focused on the relationship between the mass, perigastric arteries, and the gastric wall. Localization diagnosis values were compared between CT vascular reconstruction and dynamic-enhanced CT images.
RESULTS: Among the 58 cases of perigastric mass, 41 cases originated from the stomach, 7 cases from the left liver lobe, 6 from the pancreas, 2 from lessor omental bursa, 1 from transverse mesocolon, and 1 from left adrenal gland. The accuracy of CT vascular reconstruction images in the localization diagnosis of perigastric mass was higher than that of dynamic-enhanced CT images (98.3% and 86.2%, respectively, P = .04). On the reference level, 35 (35/41) patients with stomach-originated masses showed the mass adjacent perigastric arteries pushed away from the stomach (arterial displacement sign), and 15 (15/17) patients with nonstomach-originated masses showed perigastric arteries between the mass and the stomach (arterial entrapment sign). The sensitivity, specificity, positive predictive value, and negative predictive value of the localization diagnosis of perigastric mass with arterial displacement sign were 85.4%, 100%, 100%, and 73.9%, respectively, and with arterial entrapment sign, 88.2%, 100%, 100%, and 95.3%, respectively.
CONCLUSION: CT vascular reconstruction can clearly depict the relationship between perigastric mass and adjacent perigastric arteries, which may help us more accurately differentiate between stomach-originated and nonstomach-originated masses compared with original dynamic-enhanced CT images.

PMID: 29952968 [PubMed - indexed for MEDLINE]

Enhanced wild-type p53 expression by small activating RNA dsP53-285 induces cell cycle arrest and apoptosis in pheochromocytoma cell line PC12.

Mon, 07/09/2018 - 22:12
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Enhanced wild-type p53 expression by small activating RNA dsP53-285 induces cell cycle arrest and apoptosis in pheochromocytoma cell line PC12.

Oncol Rep. 2017 Nov;38(5):3160-3166

Authors: Lin D, Meng L, Xu F, Lian J, Xu Y, Xie X, Wang X, He H, Wang C, Zhu Y

Abstract
Malignant pheochromocytoma (PHEO) is diagnosed only when metastasis has occurred, making it less likely for patients to obtain the benefits of traditional chemotherapy. Anti-oncogene TP53 mutation has been detected in PHEO and is possibly related to disease progression. However, whether the upregulation of wild-type TP53 has antitumoral effects on PHEO remains completely unknown. In the present study, we used RNA activation (RNAa) technique to upregulate the expression of wild-type TP53 by transfecting synthetic dsP53‑285 into PHEO cell line PC12. We found that the upregulation of wild-type p53 blocked the transition of PC12 cells from the G0/G1 to the S phase, with induction of apoptosis. Additionally, the above-mentioned findings were attested in vivo. Most importantly, dsP53-285-induced antitumoral effects were reversible following co-transfection with siRNA that targeted p53 mRNA. Collectively, our results revealed that the upregulation of p53 and possibly other anti-oncogenes may provide a potential effective therapeutic strategy for PHEO.

PMID: 29048679 [PubMed - indexed for MEDLINE]

Bilateral pheochromocytoma with ganglioneuroma component associated with multiple neuroendocrine neoplasia type 2A: a case report.

Mon, 07/09/2018 - 22:12
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Bilateral pheochromocytoma with ganglioneuroma component associated with multiple neuroendocrine neoplasia type 2A: a case report.

J Med Case Rep. 2017 Aug 01;11(1):208

Authors: Efared B, Atsame-Ebang G, Tahirou S, Mazaz K, Hammas N, El Fatemi H, Chbani L

Abstract
BACKGROUND: Composite pheochromocytoma/paragangliomas are very rare tumors composed of ordinary pheochromocytoma paragangliomas associated with neurogenic tumors. Several hereditary susceptibility disorders are known to be associated with pheochromocytoma/paragangliomas such as multiple endocrine neoplasia type 2 (2A or B). To the best of our knowledge, only four cases of composite pheochromocytoma/paragangliomas associated with multiple endocrine neoplasia type 2 have been reported.
CASE PRESENTATION: A 40-year-old Arabic woman presented with headache, palpitations, paroxysmal hypertension, and weight loss, which she had had for the last 3 years. She had a familial history of diabetes and multiple endocrine neoplasia type 2. A radiological examination revealed thyroid lesions and bilateral adrenal medulla tumors. Our patient had undergone bilateral adrenalectomy, total thyroidectomy with cervical lymphadenectomy, and parathyroidectomy. A pathological examination confirmed the multiple endocrine neoplasia type 2A consisting of left medullary pheochromocytoma, right medullary composite pheochromocytoma-ganglioneuroma, medullary carcinoma of the thyroid with lymph node metastasis and parathyroid hyperplasia. A genetic analysis also revealed that our patient had a RET germline mutation.
CONCLUSION: Composite pheochromocytoma/paraganglioma associated with multiple endocrine neoplasia type 2 is a very rare occurrence, as the current literature provides only a few cases. Further reported cases are needed in order to understand the behavior and the pathogenesis of this uncommon entity.

PMID: 28760150 [PubMed - indexed for MEDLINE]

Inflammatory profile in X-linked adrenoleukodystrophy patients: Understanding disease progression.

Mon, 07/09/2018 - 22:12
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Inflammatory profile in X-linked adrenoleukodystrophy patients: Understanding disease progression.

J Cell Biochem. 2018 Jan;119(1):1223-1233

Authors: Marchetti DP, Donida B, Jacques CE, Deon M, Hauschild TC, Koehler-Santos P, de Moura Coelho D, Coitinho AS, Jardim LB, Vargas CR

Abstract
X-linked adrenoleukodystrophy (X-ALD) is an inherited disease characterized by progressive inflammatory demyelization in the brain, adrenal insufficiency, and an abnormal accumulation of very long chain fatty acids (VLCFA) in tissue and body fluids. Considering that inflammation might be involved in pathophysiology of X-ALD, we aimed to investigate pro- and anti-inflammatory cytokines in plasma from three different male phenotypes (CCER, AMN, and asymptomatic individuals). Our results showed that asymptomatic patients presented increased levels of pro-inflammatory cytokines IL-1β, IL-2, IL-8, and TNF-α and the last one was also higher in AMN phenotype. Besides, asymptomatic patients presented higher levels of anti-inflammatory cytokines IL-4 and IL-10. AMN patients presented higher levels of IL-2, IL-5, and IL-4. We might hypothesize that inflammation in X-ALD is related to plasmatic VLCFA concentration, since there were positive correlations between C26:0 plasmatic levels and pro-inflammatory cytokines in asymptomatic and AMN patients and negative correlation between anti-inflammatory cytokine and C24:0/C22:0 ratio in AMN patients. The present work yields experimental evidence that there is an inflammatory imbalance associated Th1, (IL-2, IL-6, and IFN-γ), Th2 (IL-4 and IL-10), and macrophages response (TNF-α and IL-1β) in the periphery of asymptomatic and AMN patients, and there is correlation between VLCFA plasmatic levels and inflammatory mediators in X-ALD. Furthermore, we might also speculate that the increase of plasmatic cytokines in asymptomatic patients could be considered an early biomarker of brain damage and maybe also a predictor of disease progression.

PMID: 28722826 [PubMed - indexed for MEDLINE]

An unusual adrenal cause of hypoglycaemia.

Mon, 07/09/2018 - 22:12
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An unusual adrenal cause of hypoglycaemia.

QJM. 2017 Oct 01;110(10):675-676

Authors: O'Toole SM, Turner BC, Plowman PN, Batterham RL, Drake WM

PMID: 28666340 [PubMed - indexed for MEDLINE]

Neutrophil to Lymphocyte Ratio in Castration-Resistant Prostate Cancer Patients Treated With Daily Oral Corticosteroids.

Mon, 07/09/2018 - 22:12
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Neutrophil to Lymphocyte Ratio in Castration-Resistant Prostate Cancer Patients Treated With Daily Oral Corticosteroids.

Clin Genitourin Cancer. 2017 Dec;15(6):678-684.e1

Authors: Mehra N, Sharp A, Lorente D, Dolling D, Sumanasuriya S, Johnson B, Dearnaley D, Parker C, de Bono J

Abstract
BACKGROUND: The neutrophil to lymphocyte ratio (NLR) has been shown to be highly prognostic across many tumor types, and predictive of treatment outcome in advanced prostate cancer, and has been postulated to be an indirect measure of tumor inflammation. We evaluated the effect of low-dose steroids on NLR in men suffering from castration-resistant prostate cancer (CRPC).
PATIENTS AND METHODS: The NLR was evaluated in a prospective randomized phase II trial that compared prednisolone 5 mg twice daily and dexamethasone 0.5 mg daily administered to 75 chemotherapy and abiraterone/enzalutamide-naive CRPC patients. NLR was examined at baseline (BL), after 6 and 12 weeks of corticosteroid treatment; associations with >50% prostate-specific antigen (PSA) response, duration of response (PSA progression-free interval), and overall survival (OS) were tested using logistic regression and Cox regression analysis.
RESULTS: The median NLR for all evaluable patients was 2.6 at BL; 2.9 at 6 weeks; and 4.0 at 12 weeks. After low-dose corticosteroid initiation, 46 patients had a decline in PSA with 24 confirmed responders. BL NLR (log10) associated with a PSA response (odds ratio, .029, 95% confidence interval [CI], .002-.493; P = .014), and with the extent of the PSA decline (P = .009). A favorable BL NLR (less than median) associated with a 5.5-fold higher odds of a PSA >50% response (95% CI, 1.3-23.9; P = .02). Higher BL NLR (log10) associated with a shorter time to PSA progression (hazard ratio [HR], 9.5; 95% CI, 2.3-39.9; P = .002). In multivariate analysis BL NLR as a discrete variable was independently associated with PSA progression (HR, 3.5; 95% CI, 1.5-8.1; P = .003). NLR at 6 weeks was also associated with duration of benefit; in the favorable NLR category time to PSA progression was 10.8 months, for those who converted to an unfavorable (greater than median) category 4.5 months, and for those remaining in a unfavorable category only 1.5 months (95% CI, 0.5-2.5; P = .003). OS was 33.1 months (95% CI, 24.2-42.0) and 21.9 months (95% CI, 19.3-24.4) for those with an favorable and unfavorable BL NLR, respectively.
CONCLUSION: Treatment-naive CRPC patients with a high BL or during-treatment NLR appear not to benefit from low-dose corticosteroids. The immunological implications of an unfavorable NLR, and whether corticosteroids might drive prostate cancer progression in patients harboring a high NLR, warrant further study.

PMID: 28606735 [PubMed - indexed for MEDLINE]

Reassessing the clinical spectrum associated with hereditary leiomyomatosis and renal cell carcinoma syndrome in French FH mutation carriers.

Mon, 07/09/2018 - 22:12
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Reassessing the clinical spectrum associated with hereditary leiomyomatosis and renal cell carcinoma syndrome in French FH mutation carriers.

Clin Genet. 2017 Dec;92(6):606-615

Authors: Muller M, Ferlicot S, Guillaud-Bataille M, Le Teuff G, Genestie C, Deveaux S, Slama A, Poulalhon N, Escudier B, Albiges L, Soufir N, Avril MF, Gardie B, Saldana C, Allory Y, Gimenez-Roqueplo AP, Bressac-de Paillerets B, Richard S, Benusiglio PR

Abstract
We addressed uncertainties regarding hereditary leiomyomatosis and renal cell carcinoma (HLRCC) by exploring all French cases, representing the largest series to date. Fumarate hydratase (FH) germline testing was performed with Sanger sequencing and qPCR/MLPA. Enzyme activity was measured when necessary. We carried out whenever possible a pathology review of RCC and S-(2-succino)-cysteine (2SC)/fumarate hydratase immunohistochemistry. We estimated survival using non-parametric Kaplan-Meier. There were 182 cases from 114 families. Thirty-seven RCC were diagnosed in 34 carriers (19%) at a median age of 40. Among the 23 RCC with pathology review, 13 were papillary type 2. There were 4 papillary RCC of unspecified type, 3 unclassified, 2 tubulocystic, and 1 collecting duct (CD) RCC, all 2SC+ and most (8/10) FH-. Of the remaining 14, papillary type 2, papillary unspecified, CD, and clear cell histologies were reported. The vast majority of RCC (82%) were metastatic at diagnosis or rapidly became metastatic. Median survival for metastatic disease was 18 months (95%CI: 11-29). 133 cases (73%) had a history of cutaneous leiomyomas, 3 developed skin leiomyosarcoma. Uterine leiomyomas were frequent in women (77%), but no sarcomas were observed. Only 2 cases had pheochromocytomas/paraganglioma.
CONCLUSION: Our findings have direct implications regarding the identification and management of HLRCC patients.

PMID: 28300276 [PubMed - indexed for MEDLINE]

adrenal tumor; +37 new citations

Mon, 07/02/2018 - 21:53

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Synchronous Epstein-Barr virus-associated skull base and adrenal smooth-muscle tumors in an 8-year-old girl with recent Epstein-Barr virus infection.

Mon, 06/18/2018 - 19:02
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Synchronous Epstein-Barr virus-associated skull base and adrenal smooth-muscle tumors in an 8-year-old girl with recent Epstein-Barr virus infection.

J Neurosurg Pediatr. 2018 Jun 15;:1-5

Authors: Brown DA, Deep NL, Driscoll CL, Link MJ, Jentoft ME, Daniels DJ

Abstract
Epstein-Barr virus-associated smooth-muscle tumors are rare tumors seen in immunocompromised patients. Most cases occur in the context of AIDS and organ transplantation, and very rarely in the setting of congenital immunodeficiency, with only 5 case reports of the latter published so far in the literature. The authors report the case of a previously healthy 8-year-old girl with headaches and precocious puberty who was found to have a large skull base lesion. There was a synchronous left adrenal lesion. She underwent resection of the skull base lesion and a left adrenalectomy. Thorough evaluation for immunodeficiency was negative for a known congenital immunodeficiency syndrome. She had a short course of intravenous immunoglobulin and has had no recurrence of disease or new lesions in the 17 months since presentation. Continued surveillance for the development of opportunistic infections and new or recurrent lesions is warranted in this case. Repeat surgery for surgically accessible tumors or chemoradiation would be recommended for any additional lesions.

PMID: 29905497 [PubMed - as supplied by publisher]

Plasma cell-free DNA quantification is highly correlated to tumor burden in children with neuroblastoma.

Mon, 06/18/2018 - 19:02
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Plasma cell-free DNA quantification is highly correlated to tumor burden in children with neuroblastoma.

Cancer Med. 2018 Jun 14;:

Authors: Wang X, Wang L, Su Y, Yue Z, Xing T, Zhao W, Zhao Q, Duan C, Huang C, Zhang D, Jin M, Cheng X, Chen S, Liu Y, Ma X

Abstract
To evaluate plasma cell-free DNA (cfDNA) as a promising biomarker for neuroblastoma (NB) tumor burden. Seventy-nine eligible patients with newly diagnosed NB were recruited from Beijing Children's Hospital between April 2016 and April 2017. Additionally, from September 2011 to June 2017, 79 patients with stable NB were evaluated with a median follow-up time of 21 months. Approximately 2 mL of peripheral blood was drawn upon enrollment, and plasma cfDNA levels were measured via quantitative polymerase chain reaction (qPCR). Total cfDNA analysis was performed using the long interspersed nuclear element 1 (LINE-1) 79 bp fragment, and DNA integrity was calculated by the ratio of the LINE-1 300 bp fragment to the LINE-1 79 bp fragment. A total of 79 NB patients with a median age of 36 months comprised the group of newly diagnosed NB patients. The main primary tumor site was the retroperitoneal and adrenal region (81%). Three or more metastatic sites were found in 17.7% of patients. Stable NB patients older than 18 months comprised 98.7% of the stable NB patients. Neuron-specific enolase (NSE), lactate dehydrogenase (LDH), and cfDNA levels were dramatically increased in the newly diagnosed NB patients and significantly different from those in the stable NB patients. Moreover, the concentration of cfDNA was much higher in patients with larger tumors. By analyzing the area under the receiver operator characteristic (ROC) curve (AUC), the areas of total cfDNA, NSE, and LDH levels were 0.953, 0.929, and 0.906, respectively. The sensitivity and specificity data clarified that the level of circulating cfDNA in plasma can be considered as a reliable biomarker for describing tumor load in NB. The plasma cfDNA concentration was as good as the levels of LDH and NSE to discriminate the tumor burden in children with NB.

PMID: 29905010 [PubMed - as supplied by publisher]

Long-term survival in recurrent adrenocortical cancer.

Mon, 06/18/2018 - 19:02
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Long-term survival in recurrent adrenocortical cancer.

BMJ Support Palliat Care. 2018 Jun 13;:

Authors: Shuayb M, Das A, Uddin MN

Abstract
Adrenocortical carcinoma (ACC) comprises approximately 0.02% of all malignant tumours, which are a very small fraction of a group of cancers that affect in 0.7 to 2 in 1 000 000 people per year. Recurrence is very common even after complete resection and prognosis is poor. We report a case of a sporadic form of ACC found in a 41-year-old Asian Bangladeshi man. His tumour was surgically excised completely with negative margins and he did not receive any adjuvant therapy. Four years later, adrenal adenoma was developed at his opposite side which was also excised. Then after a total duration of 7 years, he developed recurrence in both adrenal glands and extensive metastases to bilateral lungs, liver and abdominal wall. As per FIRM-ACT study, we started treatment with etoposide, doxorubicin, cisplatin plus mitotane (EDP-M), and the patient responded dramatically. He became symptom free and achieved radiological partial response just on completion of three cycles. To our knowledge, this is the first case of ACC in Bangladesh published in the literature. Managing rare cancers is always challenging due to the fact that clinicianslack practical experience in it. We believe that patient with a rare cancer with poor prognosis like ACC may also survive long, and extensive metastases can also be controlled.

PMID: 29903850 [PubMed - as supplied by publisher]

Anti-tumor activity of phenoxybenzamine and its inhibition of histone deacetylases.

Mon, 06/18/2018 - 19:02
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Anti-tumor activity of phenoxybenzamine and its inhibition of histone deacetylases.

PLoS One. 2018;13(6):e0198514

Authors: Inchiosa MA

Abstract
The principal finding from this study was the recognition that the α-adrenergic antagonist, phenoxybenzamine, possesses histone deacetylase inhibitory activity. Phenoxybenzamine is approved by the United States Food and Drug Administration for the treatment of hypertensive crises associated with tumors of the adrenal medulla, pheochromocytomas. It has several "off label" indications relative to its capacity to relax vascular smooth muscle and smooth muscle of the urogenital tract. The drug also has a long history of apparent efficacy in ameliorating, and perhaps reversing, the severe symptoms of neuropathic pain syndromes. Our interest in this feature of the drug relates to the fact that certain types of neuropathic pain, in particular complex regional pain syndrome, demonstrate a proliferative nature, with the capacity to spread from an injured limb, for example, to a non-injured limb and perhaps to essentially the entire body. Sensory neuronal sprouting in the spinal cord has been observed under conditions where there is a high sensory input from painful stimuli. Searches of gene expression signatures in the BroadBuild02 Molecular Signature Database using their connectivity map software suggested that phenoxybenzamine may have histone deacetylase inhibitory activity. Studies by others have reported inhibitory effects of phenoxybenzamine on growth, invasion and migration of human tumor cell cultures and, in one study, inhibition of tumor expansion in animal experiments. Inhibitory effects on human tumor cell cultures are also reported in the present study. Phenoxybenzamine was also found to have histone deacetylase inhibitory activity; histone deacetylase isoforms 5, 6, and 9 were the most sensitive to inhibition by phenoxybenzamine. The importance of elevated levels of these isoforms as biomarkers of poor prognosis in human malignant disease, and the recognized suppression of tumor growth that may accrue from their inhibition, opens consideration of possible translation of phenoxybenzamine to new clinical applications. This might be facilitated by the fact that phenoxybenzamine is already an approved drug entity. There appears to be no previous report of the activity of phenoxybenzamine as a histone deacetylase inhibitor.

PMID: 29897996 [PubMed - in process]

Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor in the Adrenal Gland of a Child.

Mon, 06/18/2018 - 19:02
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Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor in the Adrenal Gland of a Child.

Iran J Kidney Dis. 2018 05;12(3):190-192

Authors: Goudarzipour K, Farahmandi F, Mohammadi A, Taherian R

Abstract
Ewing sarcoma/peripheral primitive neuroectodermal tumor (ES/PNET) typically occurs in long or flat bones, soft tissues, or less often, solid organs. Ewing sarcoma/peripheral primitive neuroectodermal tumor arising from the adrenal gland is extremely rare, especially in children, and only limited cases are reported previously. Herein, we review a case of a 22-month-old girl who presented to our department with abdominal pain, bulging of the left flank, and a nonfunctioning adrenal lesion which was found to be an adrenal ES/PNET. The patient was successfully treated with surgery and adjuvant chemotherapy. Since delayed diagnosis may result in metastatic lesions, this case underscores the importance of considering ES/PNET in the differential diagnosis of large adrenal masses.

PMID: 29891750 [PubMed - in process]

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