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[Pheochromocytoma as a cause of hypertension in a patient with neurofibromatosis type 1].

Sat, 11/11/2017 - 02:38
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[Pheochromocytoma as a cause of hypertension in a patient with neurofibromatosis type 1].

Med Clin (Barc). 2016 Nov 18;147(10):474

Authors: Roa-Chamorro R, González-Bustos P, Mediavilla García JD, Jaén Águila F

PMID: 27084557 [PubMed - indexed for MEDLINE]

adrenal tumor; +22 new citations

Sat, 11/04/2017 - 00:58

22 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 2017/11/04

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.

Time Until Partial Response in Metastatic Adrenocortical Carcinoma Long-Term Survivors.

Fri, 10/27/2017 - 23:11
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Time Until Partial Response in Metastatic Adrenocortical Carcinoma Long-Term Survivors.

Horm Cancer. 2017 Oct 25;:

Authors: Vezzosi D, Do Cao C, Hescot S, Bertherat J, Haissaguerre M, Bongard V, Drui D, De La Fouchardière C, Illouz F, Borson-Chazot F, Djobo B, Berdelou A, Tabarin A, Schlumberger M, Briet C, Caron P, Leboulleux S, Libe R, Baudin E, For Comete-Cancer Network

Abstract
A partial response (PR) has been proposed as a surrogate for overall survival in advanced adrenocortical carcinoma (ACC). The primary endpoint of the study was to characterize the time until a PR in patients with metastatic ACC treated with a standard therapy is achieved. Long-term survivors were selected to allow evaluation of delayed tumor response to mitotane. Records from patients with metastatic ACC that survived for > 24 months were retrieved. Tumor response was analyzed according to the Response Evaluation Criteria in Solid Tumors (RECIST) 1.1 criteria. Time until a tumor response, after treatment initiation or therapeutic plasma mitotane level, was analyzed. Sixty-eight patients were analyzed. The first-line systemic therapy was mitotane as a monotherapy (M) (n = 57) or cytotoxic polychemotherapy plus/minus mitotane (PC ± M) (n = 11). The second-line therapy was M (n = 2) or PC ± M (n = 41). Thirty-two PRs occurred in 30/68 patients (44.1%): this was obtained for 13 (40.6%) during M and during PC ± M for 19/32 responders (59.4%). PRs were observed within 6 months of starting M or PC ± M in 76.9 and 94.7% of responses, respectively, within 6 months of therapeutic plasma mitotane being first observed in 88.9% of responses with M and in 53.3% of responses with PC ± M. All PRs (but one) occurred within 1 year after initiating treatment. To conclude, Most patients with metastatic ACC and long survival times had PRs within the first 6 months of standard systemic therapy, and almost all within the first year. The absence of response after that period could be considered as a treatment failure. Maintenance of mitotane therapy in non-responders after 1 year should be questioned in future randomized trials.

PMID: 29071575 [PubMed - as supplied by publisher]

Primary adrenal teratoma: An unusual tumor - Challenges in diagnosis and surgical management.

Fri, 10/27/2017 - 23:11
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Primary adrenal teratoma: An unusual tumor - Challenges in diagnosis and surgical management.

J Postgrad Med. 2017 Oct 23;:

Authors: Ramakant P, Rana C, Singh KR, Mishra A

Abstract
We report a rare case of a 25-year-old lady who presented with right hypochondriac pain associated with weakness for 3 months. Her hormonal evaluation was normal. Computed tomography scan revealed an adrenal mass for which a right open transperitoneal adrenalectomy was performed, and the lesion was diagnosed as mature teratoma on histopathological examination. Teratoma is an unusual neoplasm which arises from one or all the three germ layers. Extragonadal teratomas are rare in adults as compared to children and are mostly retroperitoneal in location, constituting only 4% of all primary teratomas. Primary adrenal teratomas are even rarer and to the best of our knowledge, only eight cases have been reported in adults in the past 10 years. Adrenal teratoma can pose a diagnostic challenge because radiologically it mimics myelolipoma, angiomyolipoma, liposarcoma, or pheochromocytoma. Mature teratomas are usually benign but may possess malignant potential, the chances of which are greater in adults as compared to children, making it an important entity requiring a proper diagnosis and management.

PMID: 29067922 [PubMed - as supplied by publisher]

Surgical Management of Primary Retroperitoneal Sarcomas: Rationale for Selective Organ Resection.

Fri, 10/27/2017 - 23:11
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Surgical Management of Primary Retroperitoneal Sarcomas: Rationale for Selective Organ Resection.

Ann Surg Oncol. 2017 Oct 24;:

Authors: Fairweather M, Wang J, Jo VY, Baldini EH, Bertagnolli MM, Raut CP

Abstract
BACKGROUND: Recently, some have argued for routine resection of adjacent but uninvolved organs in patients with retroperitoneal sarcoma (RPS) without stipulating the rationale for such organ resection (beyond the need to achieve a macroscopically complete resection) or examining histopathologic organ invasion (HOI). This study reviewed the authors' experience with primary RPS to investigate the rate and rationale for individual organ resection and the rate of HOI.
METHODS: Operative and pathology reports for patients with primary RPS who underwent resection at our institution were retrospectively reviewed. Histopathologic organ invasion was confirmed by a dedicated sarcoma pathologist.
RESULTS: From 2002 through 2011, 118 patients underwent resection of a primary RPS, and 99 of these patients (84%) had at least one organ resected. Kidney (n = 57), colon (n = 51), and adrenal (n = 41) were the most commonly resected organs. For the 302 organs removed, the perioperative clinical rationale for the resection was suspected invasion or tumor origin (n = 52, 17%), involved end-organ vasculature (n = 39, 13%), organ encasement (n = 42, 14%), tumor adherence (n = 127, 42%), resection required for R0/R1 resection (n = 25, 8%), or other (n = 17, 6%). The presence of HOI was found in 77 (25%) of the 302 organs resected. In the reviewed studies, HOI was identified in 34 (65%) of 52 organs suspected of invasion or tumor origin, in 19% of organs resected due to tumor encasement, and in 26% of organs with adherent tumor, even when not suspected intraoperatively, but was never identified in organs resected purely as part of a liberal en bloc resection of adjacent organs. When invasion was suspected intraoperatively, HOI was confirmed in 50, 78, and 100% of resected organs respectively for well-dedifferentiated liposarcoma, dedifferentiated liposarcoma (DDLPS), and leiomyosarcoma (LMS).
CONCLUSIONS: Histologic organ invasion was observed more commonly in organs resected with suspicion of invasion than in organs resected simply to achieve a negative margin, although this reflects a degree of subjectivity and selection bias. In more than one-fourth of adherent organs, HOI was present even when not suspected intraoperatively. Histologic subtype may predict HOI because DDLPS and LMS are associated with high rates of HOI when invasion is suspected intraoperatively. Development of a data-driven, histology-specific rationale for adjacent organ resection is critical.

PMID: 29067605 [PubMed - as supplied by publisher]

[Inflammatory mechanism of depression and its new strategy for diagnosis and treatment].

Fri, 10/27/2017 - 23:11
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[Inflammatory mechanism of depression and its new strategy for diagnosis and treatment].

Sheng Li Xue Bao. 2017 Oct 25;69(5):715-722

Authors: Su WJ, Cao ZY, Jiang CL

Abstract
Depression is a major class of mental illness; owing to its high prevalence, high disability rate and heavy disease burden, it has become a stern and formidable global health problem. It is generally believed that the etiology of depression is multifactorial, which is related to gender differences, chronic stress, dietary behavior and drug abuse. At present, the exact pathophysiological mechanism of depression still remains unclear, but researchers across the globe put forward various hypotheses to interpret the possible access to this disease, including monoamine neurotransmitter disturbance, hypothalamic-pituitary-adrenal (HPA) axis dysfunction, lack of neurotrophic factors and excessive pro-inflammatory cytokines. Based on the latest research evidence and the objective fact that traditional antidepressants may be ineffective in some particular patients, the "cytokine theory" tends to attract more and more attention recently. To date, researches on the role of cytokines in the pathogenesis of depression mainly focus on pro-inflammatory cytokines, especially categories including tumor necrosis factor-α (TNF-α), interleukin-1β (IL-1β) and interleukin-6 (IL-6). With the proceeding of researches from all over the world, a variety of novel molecules and mechanisms were postulated. This paper summarized a large amount of in vitro and in vivo research evidence, in order to review the current progress of the researches on pathophysiology of depression from the perspective of pro-inflammatory cytokines. Since the response rate of antidepressant therapy during present medical practice is unsatisfying, we suggest a new feasible diagnosis and treatment strategy, that is to distinguish the inflammatory status of patients with depression and take anti-inflammatory treatment into consideration. Totally, this novel strategy aims at modulating the conventional clinical protocol for treatment-resistant depressive patients and overcoming the limitation of insufficient antidepressant response possibly resulted from inflammation.

PMID: 29063119 [PubMed - in process]

Immune Thrombocytopenia in a Child with Neuroblastoma.

Fri, 10/27/2017 - 23:11
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Immune Thrombocytopenia in a Child with Neuroblastoma.

Case Rep Pediatr. 2017;2017:1329489

Authors: Ikizoglu HT, Ayan I, Tokat F, Tecimer T, Topuzlu Tekant G

Abstract
Thrombocytopenia is a frequent finding in patients with solid tumors. It is usually caused by bone marrow infiltration or by myelosuppression due to anticancer therapy; however immune thrombocytopenia (ITP) associated with solid tumors is rare. Neuroblastoma is the most common extracranial solid tumor in children. Here we report the case of a two-year-nine-month-old patient with adrenal neuroblastoma who presented with ITP. Paraneoplastic ITP was considered in the differential diagnosis. Bone marrow infiltration and other causes of thrombocytopenia were excluded and the patient was treated with intravenous immunoglobulin and tumor resection. Platelet count increased rapidly after surgery and complete remission of ITP was achieved.

PMID: 29062578 [PubMed]

WNT signaling, the development of the sympathoadrenal-paraganglionic system and neuroblastoma.

Fri, 10/27/2017 - 23:11
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WNT signaling, the development of the sympathoadrenal-paraganglionic system and neuroblastoma.

Cell Mol Life Sci. 2017 Oct 22;:

Authors: Becker J, Wilting J

Abstract
Neuroblastoma (NB) is a tumor of the sympathoadrenal system arising in children under 15 years of age. In Germany, NB accounts for 7% of childhood cancer cases, but 11% of cancer deaths. It originates from highly migratory progenitor cells that leave the dorsal neural tube and contribute neurons and glial cells to sympathetic ganglia, and chromaffin and supportive cells to the adrenal medulla and paraganglia. Clinically, histologically and molecularly, NBs present as extremely heterogeneous, ranging from very good to very poor prognosis. The etiology of NB still remains unclear and needs to be elucidated, however, aberrant auto- and paracrine embryonic cell communications seem to be likely candidates to initiate or facilitate the emergence, progression and regression of NB. The wingless-type MMTV integration site (WNT) family of proteins represents an evolutionary highly conserved signaling system that orchestrates embryogenesis. At least 19 ligands in the human, numerous receptors and co-receptors are known, which control not only proliferation, but also cell polarity, migration and differentiation. Here we seek to interconnect aspects of WNT signaling with sympathoadrenal and paraganglionic development to define new WNT signaling cues in the etiology and progression of NB.

PMID: 29058015 [PubMed - as supplied by publisher]

Development and validation of a liquid chromatography tandem mass spectrometry method for the measurement of urinary catecholamines in diagnosis of pheochromocytoma.

Fri, 10/27/2017 - 23:11
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Development and validation of a liquid chromatography tandem mass spectrometry method for the measurement of urinary catecholamines in diagnosis of pheochromocytoma.

Biomed Chromatogr. 2017 Nov;31(11):

Authors: Shen Y, Cheng L, Guan Q, Li H, Lu J, Wang X

Abstract
The measurement of catecholamines in human body fluids is requested frequently for the differential diagnosis and monitoring of pheochromocytoma. The methods in most clinical laboratories focus on high-performance liquid chromatography coupled with electrochemical detection, which suffers from high background noise, low sensitivity, and poor separation. We reported and developed a robust high-throughput liquid chromatography tandem mass spectrometry method in routine clinical laboratories for the measurement of urinary catecholamines for diagnosis of pheochromocytoma. The method was validated for consistent linearity, good recovery (88-112%), excellent stability and low carryover. Intra- and inter-assay precision values for catecholamines were all below 3.35 and 4.83% respectively. Dilution linearity was investigated with satisfactory linearly dependent coefficients (r > 0.9988). The reference intervals were obtained from 310 results derived from patients in which the diagnosis of pheochromocytoma was excluded. This method was successfully used in our laboratory. The clinical characteristics of patients have been explored with satisfactory sensitivity and specificity. Therefore, we have developed a reliable assay for the liquid chromatography tandem mass spectrometry measurement of catecholamines in a routine clinical laboratory. The assay requires a small volume of urine, and all analytes are measured simultaneously. The assay is rapid and reliable to be executed, offering the potential for routine clinical laboratories.

PMID: 28481409 [PubMed - indexed for MEDLINE]

The aurora kinase inhibitor AMG 900 increases apoptosis and induces chemosensitivity to anticancer drugs in the NCI-H295 adrenocortical carcinoma cell line.

Fri, 10/27/2017 - 23:11
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The aurora kinase inhibitor AMG 900 increases apoptosis and induces chemosensitivity to anticancer drugs in the NCI-H295 adrenocortical carcinoma cell line.

Anticancer Drugs. 2017 Jul;28(6):634-644

Authors: Borges KS, Andrade AF, Silveira VS, Marco Antonio DS, Vasconcelos EJR, Antonini SRR, Tone LG, Scrideli CA

Abstract
Adrenocortical tumor (ACT) is a malignancy with a low incidence rate and the current therapy for advanced disease has a limited impact on overall patient survival. A previous study from our group suggested that elevated expression of aurora-A and aurora-B is associated with poor outcome in childhood ACT. Similar results were also reported for adult ACTs. The present in-vitro study shows that AMG 900 inhibits aurora kinases in adrenocortical carcinoma cells. AMG 900 inhibited cell proliferation in NCI-H295 cells as well as in the ACT primary cultures and caused apoptosis in the cell line NCI-H295. Furthermore, it potentialized the mitotane, doxorubicin, and etoposide effects on apoptosis induction and acted synergistically with mitotane and doxorubicin in the inhibition of proliferation. In addition, we found that AMG 900 activated Notch signaling and rendered the cells sensitive to the combination of AMG 900 and Notch signaling inhibition. Altogether, these data show that aurora kinases inhibition using AMG 900 may be an adjuvant therapy to treat patients with invasive or recurrent adrenocortical carcinomas.

PMID: 28410270 [PubMed - indexed for MEDLINE]

Failure of MIBG scan to detect metastases in SDHB-mutated pediatric metastatic pheochromocytoma.

Fri, 10/27/2017 - 23:11
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Failure of MIBG scan to detect metastases in SDHB-mutated pediatric metastatic pheochromocytoma.

Pediatr Blood Cancer. 2017 Nov;64(11):

Authors: Sait S, Pandit-Taskar N, Modak S

Abstract
(123) I-meta-iodo benzyl guanidine (MIBG) scans are considered the gold standard imaging in neuroblastoma; however, flouro deoxy glucose positron emission tomography (FDG-PET) scans have increased sensitivity in adults with pheochromocytoma/paraganglioma. We describe a pediatric patient initially considered to have localized neuroblastoma based on anatomical imaging and (123) I-MIBG scan, but subsequent investigations revealed germline succinate dehydrogenase complex iron sulfur subunit B (SDHB) mutation-associated pheochromocytoma with multiple FDG-avid skeletal metastases. We then compared (123) I-MIBG and FDG-PET scans in children with metastatic pheochromocytoma/paraganglioma. FDG-PET was superior to (123) I-MIBG scan for the detection of skeletal metastases (median number of skeletal lesions detected 10 [range 1-30] vs. 2 [range 1-26], respectively; P = 0.005 by t-test). FDG-PET should be considered the functional scan of choice in children with pheochromocytoma/paraganglioma.

PMID: 28409892 [PubMed - indexed for MEDLINE]

Inflammatory Bowel Disease (IBD) pharmacotherapy and the risk of serious infection: a systematic review and network meta-analysis.

Fri, 10/27/2017 - 23:11
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Inflammatory Bowel Disease (IBD) pharmacotherapy and the risk of serious infection: a systematic review and network meta-analysis.

BMC Gastroenterol. 2017 Apr 14;17(1):52

Authors: Wheat CL, Ko CW, Clark-Snustad K, Grembowski D, Thornton TA, Devine B

Abstract
BACKGROUND: The magnitude of risk of serious infections due to available medical therapies of inflammatory bowel disease (IBD) remains controversial. We conducted a systematic review and network meta-analysis of the existing IBD literature to estimate the risk of serious infection in adult IBD patients associated with available medical therapies.
METHODS: Studies were identified by a literature search of PubMed, Cochrane Library, Medline, Web of Science, Scopus, EMBASE, and ProQuest Dissertations and Theses. Randomized controlled trials comparing IBD medical therapies with no restrictions on language, country of origin, or publication date were included. A network meta-analysis was used to pool direct between treatment comparisons with indirect trial evidence while preserving randomization.
RESULTS: Thirty-nine articles fulfilled the inclusion criteria; one study was excluded from the analysis due to disconnectedness. We found no evidence of increased odds of serious infection in comparisons of the different treatment strategies against each other, including combination therapy with a biologic and immunomodulator compared to biologic monotherapy. Similar results were seen in the comparisons between the newer biologics (e.g. vedolizumab) and the anti-tumor necrosis factor agents.
CONCLUSIONS: No treatment strategy was found to confer a higher risk of serious infection than another, although wide confidence intervals indicate that a clinically significant difference cannot be excluded. These findings provide a better understanding of the risk of serious infection from IBD pharmacotherapy in the adult population.
PROSPERO REGISTRATION: The protocol for this systematic review was registered on PROSPERO ( CRD42014013497 ).

PMID: 28407755 [PubMed - indexed for MEDLINE]

Controlling Tumor Progression with Cyclophosphamide, Vincristine, and Dacarbazine Treatment Improves Survival in Patients with Metastatic and Unresectable Malignant Pheochromocytomas/Paragangliomas.

Fri, 10/27/2017 - 23:11
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Controlling Tumor Progression with Cyclophosphamide, Vincristine, and Dacarbazine Treatment Improves Survival in Patients with Metastatic and Unresectable Malignant Pheochromocytomas/Paragangliomas.

Horm Cancer. 2017 Apr;8(2):108-118

Authors: Asai S, Katabami T, Tsuiki M, Tanaka Y, Naruse M

Abstract
Evidence has not been established to support that combination chemotherapy with cyclophosphamide, vincristine, and dacarbazine (CVD) improves survival in patients with malignant pheochromocytoma and paraganglioma (M-PPGL). To investigate the efficacy of CVD for this disease, we retrospectively analyzed data of 23 patients with metastatic and unresectable M-PPGL (mean age, 41.7 ± 15.4 years) who received at least 2 cycles of this regimen. The follow-up period after initiation of CVD ranged from 0.3 to 13.7 years, with a median of 3.3 years. CVD therapy achieved a complete tumor response (CR) in 1 patient (4%), a partial response (PR) in 5 (22%), stable disease (SD) in 5 (22%), and progressive disease (PD) in 13 (52%), respectively. All of the responders (CR and PR) but 6% of the non-responders (SD and PD) showed substantial biochemical improvement. The progression-free survival period in the responders was significantly longer than in the non-responders (p < 0.01). Although the overall survival and survival after the diagnosis of M-PPGL were longer in the responders than the non-responders, the difference was not statistically significant (p = 0.08). The progression-free and overall survival period were significantly longer in the non-progression group (CR, PR, and SD) than in the progression group (PD) (1.7 ± 3.3 vs. 0.3 ± 0.3 years, p < 0.01, and 4.6 ± 3.6 vs. 2.0 ± 3.7 years, p = 0.01, respectively). It is therefore suggested that CVD chemotherapy could be useful in controlling tumor progression and improving survival in patients with metastatic and progressive M-PPGL.

PMID: 28108930 [PubMed - indexed for MEDLINE]

Regulation of hypothalamic-pituitary-adrenal axis activity and immunologic function contributed to the anti-inflammatory effect of acupuncture in the OVA-induced murine asthma model.

Fri, 10/27/2017 - 23:11
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Regulation of hypothalamic-pituitary-adrenal axis activity and immunologic function contributed to the anti-inflammatory effect of acupuncture in the OVA-induced murine asthma model.

Neurosci Lett. 2017 Jan 01;636:177-183

Authors: Wei Y, Dong M, Zhong L, Liu J, Luo Q, Lv Y, Mo S, Sun J, Liu F, Xu F, Yan C, Dong J

Abstract
Asthma is a complex inflammatory disease of the airways and acupuncture is one of the effective therapies widely used to treat asthma in China. The aim of the study was to evaluate the regulatory role of acupuncture in airway inflammation and the hypothalamic-pituitary-adrenal (HPA) axis activity in OVA-induced murine asthma model. Our results demonstrated that acupuncture was effective in suppression of AHR, inhibition of total leukocyte, neutrophil, lymphocyte and eosinophil counts in BALF, attenuation of airway inflammation and TNF-α, IL-1β, IL-5 and eotaxin secretion. Furthermore, the HPA axis activity was also regulated by acupuncture, which included promotion of adrenocorticotropic hormone and cortisol secretion in the plasma. Our findings revealed that acupuncture could attenuate airway inflammation and regulate HPA axis and immunologic function in the OVA-induced murine asthma model, which may provide support to better understand the contribution of acupuncture to the regulation of airway inflammation and HPA axis activity in asthma.

PMID: 27816549 [PubMed - indexed for MEDLINE]

Drug Synergism of Proteasome Inhibitors and Mitotane by Complementary Activation of ER Stress in Adrenocortical Carcinoma Cells.

Fri, 10/27/2017 - 23:11
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Drug Synergism of Proteasome Inhibitors and Mitotane by Complementary Activation of ER Stress in Adrenocortical Carcinoma Cells.

Horm Cancer. 2016 Dec;7(5-6):345-355

Authors: Kroiss M, Sbiera S, Kendl S, Kurlbaum M, Fassnacht M

Abstract
Mitotane is the only drug approved for treatment of the orphan disease adrenocortical carcinoma (ACC) and was recently shown to be the first clinically used drug acting through endoplasmic reticulum (ER)-stress induced by toxic lipids. Since mitotane has limited clinical activity as monotherapy, we here study the potential of activating ER-stress through alternative pathways. The single reliable NCI-H295 cell culture model for ACC was used to study the impact MG132, bortezomib (BTZ) and carfilzomib (CFZ) on mRNA and protein expression of ER-stress markers, cell viability and steroid hormone secretion. We found all proteasome inhibitors alone to trigger expression of mRNA (spliced X-box protein 1, XBP1) and protein markers indicative of the inositol-requiring enzyme 1 (IRE1) dependent pathway of ER-stress but not phosphorylation of eukaryotic initiation factor 2α (eIF2α), a marker of the PRKR-like endoplasmic reticulum kinase (PERK)-dependent pathway. Whereas mitotane alone activated both pathways, combination of BTZ and CFZ with low-dose mitotane blocked mitotane-induced eIF2α phosphorylation but increased XBP1-mRNA splicing indicating that proteasome inhibitors can commit signalling towards a single ER-stress pathway in ACC cells. By applying the median effect model of drug combinations using cell viability as a read out, we determined significant drug synergism between mitotane and both BTZ and CFZ. In conclusion, combination of mitotane with activators of ER-stress through the unfolded protein response is synergistic in an ACC cell culture model. Since proteasome inhibitors are readily available clinically, they are attractive candidates to study for ACC treatment in clinical trials in combination with mitotane.

PMID: 27631436 [PubMed - indexed for MEDLINE]

Synchronous vs. Metachronous Metastases in Adrenocortical Carcinoma: an Analysis of the Dutch Adrenal Network.

Fri, 10/27/2017 - 23:11
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Synchronous vs. Metachronous Metastases in Adrenocortical Carcinoma: an Analysis of the Dutch Adrenal Network.

Horm Cancer. 2016 Dec;7(5-6):336-344

Authors: Ettaieb MH, Duker JC, Feelders RA, Corssmit EP, Menke-van der Houven van Oordt CW, Timmers HJ, Kerstens MN, Wilmink JW, Zelissen PM, Havekes B, Haak HR

Abstract
Adrenal Cortical Carcinoma (ACC) is a rare malignancy with an incidence of 1.0 per million per year in the Netherlands. Median survival varies according to the European Network for the Study of Adrenal Tumours (ENS@T) tumour stage. It is unknown whether time until development of metastases is of influence on prognosis. To asses this, data were retrospectively obtained from centres of the Dutch Adrenal Network. Patients who presented with ACC between January 1, 2004 and October 31, 2013 were included. Date of detection of metastases, number of metastases and affected organs were registered. One hundred sixty patients were included in the analysis. Synchronous metastases were defined as diagnosis of metastasis ≤6 months after the initial diagnosis of ACC. Overall survival rate was calculated from the date of diagnosis of metastasis until death from any cause. At first presentation, 50 patients (31 %) had ACC with metastases (ENS@T stage IV). Another 67 (42 %) developed metastases during follow-up. Amongst the 117 patients with metastases, 84 (72 %) patients had synchronous metastases and 33 (28 %) developed metachronous metastases. Diagnosis of synchronous metastases (p = 0.046), more than one affected organ (p < 0.001) and four or more metastases (p < 0.001) were found to be associated with reduced overall survival. Limitations included retrospective design and limited details regarding pathological data. We conclude that synchronous metastases of ACC are associated with a poorer prognosis compared to metachronous metastases of ACC. The clinical characteristics associated with prognosis in this study support the view to refine the prognostic classification for patients with stage IV ACC.

PMID: 27422613 [PubMed - indexed for MEDLINE]

Different Types of Urinary Steroid Profiling Obtained by High-Performance Liquid Chromatography and Gas Chromatography-Mass Spectrometry in Patients with Adrenocortical Carcinoma.

Fri, 10/27/2017 - 23:11
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Different Types of Urinary Steroid Profiling Obtained by High-Performance Liquid Chromatography and Gas Chromatography-Mass Spectrometry in Patients with Adrenocortical Carcinoma.

Horm Cancer. 2016 Dec;7(5-6):327-335

Authors: Velikanova LI, Shafigullina ZR, Lisitsin AA, Vorokhobina NV, Grigoryan K, Kukhianidze EA, Strelnikova EG, Krivokhizhina NS, Krasnov LM, Fedorov EA, Sablin IV, Moskvin AL, Bessonova EA

Abstract
Urinary steroid profiling (USP) was studied using high-performance liquid chromatography (HPLC) and gas chromatography-mass spectrometry (GC-MS) methods in 108 patients with adrenocortical adenoma (ACA) and in 31 patients with adrenocortical carcinoma (ACC). Thirteen ACC and Cushing's syndrome (ACC-CS) patients had two types of USP as well as 18 ACC patients without hypercortisolism. These four types differed by androgen and glucocorticoid secretion of the adrenal cortex. Fifteen main ACC features were observed by GC-MS. Urinary excretion of dehydroepiandrosterone (DHEA) was increased in 67.7 % of ACC patients and tetrahydro-11-deoxycortisol (THS) in 74.2 %. By combination of the following parameters: THS >900 μg/24 h and/or DHEA >1500 μg/24 h with ratios of 3α,16,20-pregnentriol/3β,16,20-pregnentriol (3α,16,20dP3/3β,16,20dP3) less than 6.0 and 3α,17,20dP3/3β,17,20dP3 less than 9.0 and the detection of "non-classical" 5-en-pregnens, not found in ACA and healthy persons, 100 % sensitivity and specificity of ACC and ACA differential diagnosis were achieved. Features of 21-hydroxylase and 11β-hydroxylase deficiency were observed by GC-MS in 32.2 and 61.3 % of the ACC patients, respectively. Additional features for ACC-CS diagnostic were increased urinary excretion of 6β-hydroxycortisol, 18-hydroxycorticosterone, the sum (UFF + UFE) obtained by HPLC, tetrahydrocorticosterone, and the sum (THF + THE + allo-THF) obtained by GC-MS.

PMID: 27370636 [PubMed - indexed for MEDLINE]

The landscape of bilateral adrenal incidentalomas associated with subclinical hypercortisolism.

Fri, 10/27/2017 - 23:11
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The landscape of bilateral adrenal incidentalomas associated with subclinical hypercortisolism.

Endocrine. 2016 09;53(3):621-3

Authors: Di Dalmazi G

PMID: 27369188 [PubMed - indexed for MEDLINE]

Mutated KCNJ5 activates the acute and chronic regulatory steps in aldosterone production.

Fri, 10/27/2017 - 23:11
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Mutated KCNJ5 activates the acute and chronic regulatory steps in aldosterone production.

J Mol Endocrinol. 2016 Jul;57(1):1-11

Authors: Hattangady NG, Karashima S, Yuan L, Ponce-Balbuena D, Jalife J, Gomez-Sanchez CE, Auchus RJ, Rainey WE, Else T

Abstract
Somatic and germline mutations in the inward-rectifying K(+) channel (KCNJ5) are a common cause of primary aldosteronism (PA) in aldosterone-producing adenoma and familial hyperaldosteronism type III, respectively. Dysregulation of adrenal cell calcium signaling represents one mechanism for mutated KCNJ5 stimulation of aldosterone synthase (CYP11B2) expression and aldosterone production. However, the mechanisms stimulating acute and chronic production of aldosterone by mutant KCNJ5 have not been fully characterized. Herein, we defined the effects of the T158A KCNJ5 mutation (KCNJ5(T158A)) on acute and chronic regulation of aldosterone production using an adrenal cell line with a doxycycline-inducible KCNJ5(T158A) gene (HAC15-TRE-KCNJ5(T158A)). Doxycycline incubation caused a time-dependent increase in KCNJ5(T158A) and CYP11B2 mRNA and protein levels. Electrophysiological analyses confirm the loss of inward rectification and increased Na(+) permeability in KCNJ5(T158A)-expressing cells. KCNJ5(T158A) expression also led to the activation of CYP11B2 transcriptional regulators, NURR1 and ATF2. Acutely, KCNJ5(T158A) stimulated the expression of total and phosphorylated steroidogenic acute regulatory protein (StAR). KCNJ5(T158A) expression increased the synthesis of aldosterone and the hybrid steroids 18-hydroxycortisol and 18-oxocortisol, measured with liquid chromatography-tandem mass spectrometry (LC-MS/MS). All of these stimulatory effects of KCNJ5(T158A) were inhibited by the L-type Ca(2+) channel blocker, verapamil. Overall, KCNJ5(T158A)increases CYP11B2 expression and production of aldosterone, corticosterone and hybrid steroids by upregulating both acute and chronic regulatory events in aldosterone production, and verapamil blocks KCNJ5(T158A)-mediated pathways leading to aldosterone production.

PMID: 27099398 [PubMed - indexed for MEDLINE]

Paroxysmal hypertension due to paraganglioma.

Fri, 10/27/2017 - 23:11
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Paroxysmal hypertension due to paraganglioma.

Am J Surg. 1947 Jan;73(1):111-5

Authors: PHILLIPS JR

PMID: 20279390 [PubMed - indexed for MEDLINE]

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