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NCBI: db=pubmed; Term=adrenal tumor
Updated: 6 days 16 hours ago

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]

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]

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