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NCBI: db=pubmed; Term=adrenal tumor
Updated: 19 hours 17 min ago

adrenal tumor; +24 new citations

Wed, 03/20/2019 - 19:10

24 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 2019/03/20

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.

Concomitant occurrence of primary renal non-Hodgkin lymphoma and a colon cancer: A rare case report.

Wed, 03/13/2019 - 18:47
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Concomitant occurrence of primary renal non-Hodgkin lymphoma and a colon cancer: A rare case report.

Medicine (Baltimore). 2019 Mar;98(10):e14802

Authors: Li J, Zou Y, Wang B, Meng X, Sun X

Abstract
RATIONALE: Primary renal lymphoma (PRL) is a rare malignancy due to the absence of lymphatic tissues in the kidney, and patients with PRL have been reported to have a poor prognosis due to its rapid invasiveness and limited treatment strategies. Colon cancer is the third most common cancer, and has a high mortality rate. Both malignant diseases predominantly affected elderly men; however, a case with concomitant occurrence of the 2 cancers is extremely rare.
PATIENT CONCERNS: A 78-year-old male patient with abdominal pain came to our hospital. Computed tomography (CT) indicated malignant masses in the left kidney, left adrenal gland, and the lower part of the descending colon.
DIAGNOSES: PRL and colon cancer were diagnosed based on pathological examinations.
INTERVENTIONS: The patient was treated with laparoscopic radical nephrectomy and laparoscopic radical resection of colon cancer.
OUTCOMES: The patient was then transferred to the intensive care unit (ICU) because of poor condition after surgery. He died 3 months after discharge without receiving any other treatment.
LESSONS: It is worth thinking about whether surgery was reasonable for elderly patients with double malignancies, or palliative treatment to improve the quality of life was more meaningful. This case also contributes to the understanding of the 2 malignancies and highlights the need to pay more attention to patients with multiple primary malignant neoplasms (MPMNs), explore genetic features, and investigate treatments with more survival benefits.

PMID: 30855498 [PubMed - in process]

Inhibition of Autophagy Improves the Efficacy of Abiraterone for the Treatment of Prostate Cancer.

Wed, 03/13/2019 - 18:47
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Inhibition of Autophagy Improves the Efficacy of Abiraterone for the Treatment of Prostate Cancer.

Cancer Biother Radiopharm. 2019 Mar 11;:

Authors: Ma X, Zou L, Li X, Chen Z, Lin Z, Wu X

Abstract
OBJECTIVES: Although androgen deprivation therapy remains the standard treatment for the initial therapy of advanced prostate cancer (PC), castration does not eliminate persistent intratumoral androgens within the prostate tumor microenvironment, which is capable of activating androgen receptor. Abiraterone effectively target adrenal and tumor androgen production in castration-resistant PC (CRPC). However, abiraterone-resistant CRPC is now common challenge in clinic via multiple mechanisms.
METHODS: In this study, human CRPC cell line PC3 and androgen-sensitive cells LNCaP were used. The authors investigated the role of autophagy during the therapy of abiraterone in CRPC by analysis of transmission electron microscopy (TEM), Western blot and immunofluorescence assay. Cell cycle and apoptosis using flow cytometry analysis.
RESULTS: The analysis of TEM showed more autophagic vesicles (AVs) in PC3 cell line than that in LNCaP cell line and indicated the high basic cellular autophagy in CRPC cell line PC3, which was confirmed by the upregulation of autophagy-related protein LC3, Atg5, and Beclin1. Interestingly, the treatment of abiraterone reduced the level of autophagic vesicles in two cell lines and inhibited the expressions of autophagic markers LC3, Atg5 and Beclin1 in parallel with decreased cell vitality and induced G2/M arrest in PC3 cell line and LNCaP cell line. Moreover, the addition of the autophagy inhibitor 3-methyladenine to the treatment of abiraterone inhibited the formation of AVs with downregulated autophagic markers, and inhibition of autophagy promoted the efficiency of cytotoxicity of abiraterone with further impaired cell vitality and G2/M arrest.
CONCLUSION: These data suggested that inhibition of autophagy by its inhibitor benefits the treatment of abiraterone for CRPC patients.

PMID: 30855185 [PubMed - as supplied by publisher]

Clinical, Diagnostic, and Treatment Characteristics of SDHA-Related Metastatic Pheochromocytoma and Paraganglioma.

Wed, 03/13/2019 - 18:47
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Clinical, Diagnostic, and Treatment Characteristics of SDHA-Related Metastatic Pheochromocytoma and Paraganglioma.

Front Oncol. 2019;9:53

Authors: Jha A, de Luna K, Balili CA, Millo C, Paraiso CA, Ling A, Gonzales MK, Viana B, Alrezk R, Adams KT, Tena I, Chen A, Neuzil J, Raygada M, Kebebew E, Taieb D, O'Dorisio MS, O'Dorisio T, Civelek AC, Stratakis CA, Mercado-Asis L, Pacak K

Abstract
Background: Pheochromocytoma and paraganglioma (PHEO/PGL) are rare neuroendocrine tumors which may cause potentially life-threatening complications, with about a third of cases found to harbor specific gene mutations. Thus, early diagnosis, treatment, and meticulous monitoring are of utmost importance. Because of low incidence of succinate dehydrogenase complex subunit A (SDHA)-related metastatic PHEO/PGL, currently there exists insufficient clinical information, especially with regards to its diagnostic and treatment characteristics. Methods: Ten patients with SDHA-related metastatic PHEO/PGL were followed-up prospectively and/or retrospectively between January 2010-July 2018. They underwent biochemical tests (n = 10), 123I-MIBG (n = 9) scintigraphy, and multiple whole-body positron emission tomography/computed tomography (PET/CT) scans with 68Ga-DOTATATE (n = 10), 18F-FDG (n = 10), and 18F-FDOPA (n = 6). Results: Our findings suggest that these tumors can occur early and at extra-adrenal locations, behave aggressively, and have a tendency to develop metastatic disease within a short period of time. None of our patients had a family history of PHEO/PGL, making them appear sporadic. Nine out of 10 patients showed abnormal PHEO/PGL-specific biochemical markers with predominantly noradrenergic and/or dopaminergic phenotype, suggesting their utility in diagnosing and monitoring the disease. Per patient detection rates of 68Ga-DOTATATE (n = 10/10), 18F-FDG (n = 10/10), 18F-FDOPA (n = 5/6) PET/CT, and 123I-MIBG (n = 7/9) scintigraphy were 100, 100, 83.33, and 77.77%, respectively. Five out of 7 123I-MIBG positive patients had minimal 123I-MIBG avidity or detected very few lesions compared to widespread metastatic disease on 18F-FDG PET/CT, implying that diagnosis and treatment with 123/131I-MIBG is not a good option. 68Ga-DOTATATE PET/CT was found to be superior or equal to 18F-FDG PET/CT in 7 out of 10 patients and hence, is recommended for evaluation and follow-up of these patients. All 7 out of 7 patients who received conventional therapies (chemotherapy, somatostatin analog therapy, radiation therapy, 131I-MIBG, peptide receptor radionuclide therapy) in addition to surgery showed disease progression. Conclusion: In our cohort of patients, SDHA-related metastatic PHEO/PGL followed a disease-course similar to that of SDHB-related metastatic PHEO/PGL, showing highly aggressive behavior, similar imaging and biochemical phenotypes, and suboptimal response to conventional therapies. Therefore, we recommend careful surveillance of the affected patients and a search for effective therapies.

PMID: 30854332 [PubMed]

Renal infarction associated with extra-adrenal pheochromocytoma.

Wed, 03/13/2019 - 18:47
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Renal infarction associated with extra-adrenal pheochromocytoma.

Urology. 2019 Mar 06;:

Authors: Yang C, Liu K, Huang X, Chen X

Abstract
Pheochromocytomas have been described to be associated with renal infarction in a few sporadic cases. The underlying pathophysiologic mechanisms are catecholoamine-induced vasospasm and direct compression or invasion of the tumor on renal artery or its branches. However, renal infarction caused by extra-adrenal pheochromocytoma is rarely reported. We describe an unusual case of benign extra-adrenal pheochromocytoma complicated with left renal infarction by severe vasospasm.

PMID: 30851287 [PubMed - as supplied by publisher]

In-vitro pre-treatment of cancer cells with TGF-β1: A novel approach of Tail vein lung cancer metastasis mouse model for anti-metastatic studies.

Wed, 03/13/2019 - 18:47
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In-vitro pre-treatment of cancer cells with TGF-β1: A novel approach of Tail vein lung cancer metastasis mouse model for anti-metastatic studies.

Curr Mol Pharmacol. 2019 Mar 06;:

Authors: Khan GJ, Sun L, Abbas M, Naveed M, Jamshaid T, Baig MMFA, Yuan S

Abstract
BACKGROUND: Aggressive behavior of tumor metastasis comes from certain mutations, changes in cellular metabolic and signaling pathways that are majorly altered by tumor microenvironment (TME), its other components and growth factors like transforming growth factor-β1 (TGF-β1) which is chiefly known for its epithelial to mesenchymal transformation (EMT). EMT is a critical step of metastasis cascade in actual human lung cancer scenario.
OBJECTIVE: Our present study is focused to unveil the in-vivo metastatic behavior of TGF-β1 treated lung cancer cells that undergone EMT.
METHODS: The lung cancer epithelial A549 cells were treated in-vitro with TGF-β1 (3-5ng/ml for 72 h) for EMT. After confirming the transformation of cells by phenotype modifications, wound healing and cell migration assay and qRT-PCR analyses of EMT biomarkers including E. Cadherin, Vimentin, Snail, Slug, MMP2 and MMP9; those TGF-β1 modified cells were probed with fluorescent trackers and were injected into tail vein of BALB/c nude mice for metastatic dissemination studies.
RESULTS: Our findings indicate that distribution of TGF-β1 treated A549 cells as compared to W.T A549 towards lungs is less in terms of total relative fluorescent cluster count, however the difference is insignificant (52±4, 60±5 respectively). Additionally we show that TGF-β1 treated cells tend to metastasize almost 2, 3, 1.5, 2 and 1.7 times more than W.T towards liver, brain, ovaries, bones and adrenal gland respectively which is very much like human lung cancer metastasis.
CONCLUSION: Conclusively it is ever first study reporting that a pre-treatment of cells with TGF-β1 for experimental lung cancer metastasis mouse model may portray more precise approach for the development of potential therapeutic treatments. Additional pre-treatment studies with the application of other TME conditions like hypoxia and factors like NFκB, VEGF etc. may be a future prospect to develop a better understanding.

PMID: 30848226 [PubMed - as supplied by publisher]

Screening in adrenal tumors.

Wed, 03/13/2019 - 18:47
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Screening in adrenal tumors.

Curr Opin Oncol. 2019 Mar 05;:

Authors: Corssmit EPM, Dekkers OM

Abstract
PURPOSE OF REVIEW: Adrenal tumors are mostly encountered as incidentalomas in patients undergoing imaging not performed for suspected adrenal disease; although the majority are benign and nonfunctioning, malignant tumors and functioning tumors need to be excluded. The purpose of this review is to highlight recent advances in the evaluation of adrenal tumors.
RECENT FINDINGS: As a consequence of increased use of technologically improved imaging techniques, the detection of adrenal incidentalomas has continued to increase. The vast majority of adrenal tumors are adrenocortical adenomas. To discriminate malignant from benign tumors and to identify clinically relevant functioning tumors, necessitating therapeutic intervention, adrenal tumors are best evaluated with unenhanced computed tomography (CT) attenuation and 1 mg dexamethasone overnight suppression test. An unenhanced CT attenuation value of 10 Hounsfield units or less excludes adrenocortical carcinoma and pheochromocytoma. Testing for hyperaldosteronism should be performed in hypertensive and/or hypokalemic patients, sex hormones, and steroid precursors in patients with clinical features suggestive of adrenocortical carcinoma. In patients with active extraadrenal malignancy and a single adrenal lesion without suspicion for metastasis elsewhere, CT-guided biopsy can be considered to rule out metastatic disease.
SUMMARY: All patients with an adrenal tumor and without a prior history of cancer should be initially evaluated by unenhanced CT attenuation and 1 mg overnight dexamethasone suppression test, and additional hormone testing when indicated.

PMID: 30844886 [PubMed - as supplied by publisher]

Minichromosome Maintenance Proteins MCM-3, MCM-5, MCM-7, and Ki-67 as Proliferative Markers in Adrenocortical Tumors.

Wed, 03/13/2019 - 18:47
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Minichromosome Maintenance Proteins MCM-3, MCM-5, MCM-7, and Ki-67 as Proliferative Markers in Adrenocortical Tumors.

Anticancer Res. 2019 Mar;39(3):1151-1159

Authors: Aporowicz M, Czopnik P, Kubicka E, Piotrowska A, Dziegiel P, Bolanowski M, Domoslawski P

Abstract
BACKGROUND/AIM: Morphological features, combined with Ki-67 proliferative index, remain the standard for discriminating benign and malignant adrenocortical tumors. The aim of this study was to evaluate the role of minichromosome maintenance proteins MCM-3, MCM-5, MCM-7, and Ki-67 as proliferative markers in adrenocortical tumors.
MATERIALS AND METHODS: Specimens of 81 adrenocortical adenomas and 3 adrenocortical carcinomas were stained with antibodies against MCM-3, 5, 7 and Ki-67.
RESULTS: Malignant tumors were characterized by a greater size (p=0.017), volume (p=0.017), and higher levels of Ki-67 (p=0.005), MCM-3 (p=0.005), MCM-7 (p=0.008), but not MCM-5 (p=0.069). The markers' levels were independent from the tumors' size and volume, the patient's age and hormonal status. ROC curves showed Ki-67 (AUC 0.984), MCM-3 (AUC 0.984), and MCM-7 (AUC 0.950), but not MCM-5 (AUC 0.820) to be reliable markers.
CONCLUSION: Ki-67, MCM-3, and MCM-7, but not MCM-5 are reliable proliferative and diagnostic markers in discerning benign and malignant adrenocortical tumors.

PMID: 30842144 [PubMed - indexed for MEDLINE]

Insulin Resistance as a Shared Pathogenic Mechanism Between Depression and Type 2 Diabetes.

Wed, 03/13/2019 - 18:47
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Insulin Resistance as a Shared Pathogenic Mechanism Between Depression and Type 2 Diabetes.

Front Psychiatry. 2019;10:57

Authors: Lyra E Silva NM, Lam MP, Soares CN, Munoz DP, Milev R, De Felice FG

Abstract
Neuropsychiatric disorders and type 2 diabetes (T2D) are major public health concerns proposed to be intimately connected. T2D is associated with increased risk of dementia, neuropsychiatric and mood disorders. Evidences of the involvement of insulin signaling on brain mechanisms related to depression indicate that insulin resistance, a hallmark of type 2 diabetes, could develop in the brains of depressive patients. In this article, we briefly review possible molecular mechanisms associating defective brain insulin signaling with reward system, neurogenesis, synaptic plasticity and hypothalamic-pituitary-adrenal (HPA) stress axis in depression. We further discuss the involvement of tumor necrosis factor α (TNFα) promoting defective insulin signaling and depressive-like behavior in rodent models. Finally, due to the high resistant rate of anti-depressants, novel insights into the link between insulin resistance and depression may advance the development of alternative treatments for this disease.

PMID: 30837902 [PubMed]

Relationship between clinical course of nivolumab-related myositis and immune status in a patient with Hodgkin's lymphoma after allogeneic hematopoietic stem cell transplantation.

Wed, 03/13/2019 - 18:47
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Relationship between clinical course of nivolumab-related myositis and immune status in a patient with Hodgkin's lymphoma after allogeneic hematopoietic stem cell transplantation.

Int J Hematol. 2019 Mar;109(3):356-360

Authors: Kobayashi T, Guo YM, Yamashita T, Nara M, Yoshioka T, Kameoka Y, Fukuda T, Takahashi N

Abstract
Although programmed cell death (PD)-1 blockade induces immune-related adverse events (irAEs), little is known about the safety of PD-1 blockade after allogeneic hematopoietic stem cell transplantation (HSCT). Here, we describe immune system changes during nivolumab-related myositis in a patient with Hodgkin's lymphoma after allogeneic HSCT; to our knowledge, this is the first such report in the literature. At the onset of myositis, the patient lost lower limb mobility against gravity, and had an activated immune profile with increased cytotoxic CD107a and granzyme B expression, as well as pro-inflammatory cytokines, interferon-γ, tumor necrosis factor-α, interleukin-2 in T and NK cells compared to healthy donor. Pulse steroid therapy decreased creatine kinase levels and induced PD-1 expression and regulatory T cells, but did not improve myositis; previously activated markers remained high. Four-week corticosteroid therapy decreased previously activated markers and the myositis improved. These findings provide new insights into nivolumab-induced irAE pathogenesis and suggest possible optimal treatments for irAEs.

PMID: 30604316 [PubMed - indexed for MEDLINE]

[Cushing's syndrome during pregnancy : diagnostic and therapeutic difficulties].

Wed, 03/13/2019 - 18:47
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[Cushing's syndrome during pregnancy : diagnostic and therapeutic difficulties].

Rev Med Liege. 2018 Dec;73(12):603-609

Authors: Gellner K, Emonts P, Hamoir E, Beckers A, Valdes-Socin H

Abstract
Cushing's syndrome (CS), which is often associated with infertility, exceptionally occurs in pregnancy, and markedly increases maternal and fetal morbidity and mortality. Gestational CS may be challenging. Indeed, symptoms of hypercorticism may overlap with physiological hyperactivity of the hypothalamus-pituitary-adrenal axis in normal pregnancy. This case report describes a pregnant patient that underwent a fertility treatment and developed a gestational CS due to an adrenocortical adenoma. Diagnosis of gestational CS was suspected at 13 weeks by a new onset of hypokalemia and arterial hypertension. A multidisciplinary approach was necessary during follow up. At 24 weeks, laparoscopic surgery retrieved a 4 cm adrenocortical adenoma. Cesarean surgery was successfully practiced at 31 weeks, because of preeclampsia. We discuss the differential diagnosis of hypokalemia and arterial hypertension during pregnancy and the diagnosis and management of gestational CS.

PMID: 30570230 [PubMed - indexed for MEDLINE]

A hypothesis-driven approach identifies CDK4 and CDK6 inhibitors as candidate drugs for treatments of adrenocortical carcinomas.

Wed, 03/13/2019 - 18:47
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A hypothesis-driven approach identifies CDK4 and CDK6 inhibitors as candidate drugs for treatments of adrenocortical carcinomas.

Aging (Albany NY). 2017 12 26;9(12):2695-2716

Authors: Hadjadj D, Kim SJ, Denecker T, Ben Driss L, Cadoret JC, Maric C, Baldacci G, Fauchereau F

Abstract
High proliferation rate and high mutation density are both indicators of poor prognosis in adrenocortical carcinomas. We performed a hypothesis-driven association study between clinical features in adrenocortical carcinomas and the expression levels of 136 genes involved in DNA metabolism and G1/S phase transition. In 79 samples downloaded from The Cancer Genome Atlas portal, high Cyclin Dependent Kinase 6 (CDK6) mRNA levels gave the most significant association with shorter time to relapse and poorer survival of patients. A hierarchical clustering approach assembled most tumors with high levels of CDK6 mRNA into one group. These tumors tend to cumulate mutations activating the Wnt/β-catenin pathway and show reduced MIR506 expression. Actually, the level of MIR506 RNA is inversely correlated with the levels of both CDK6 and CTNNB1 (encoding β-catenin). Together these results indicate that high CDK6 expression is found in aggressive tumors with activated Wnt/β-catenin pathway. Thus we tested the impact of Food and Drug Administration-approved CDK4 and CDK6 inhibitors, namely palbociclib and ribociclib, on SW-13 and NCI-H295R cells. While both drugs reduced viability and induced senescence in SW-13 cells, only palbociclib was effective on the retinoblastoma protein (pRB)-negative NCI-H295R cells, by inducing apoptosis. In NCI-H295R cells, palbociclib induced an increase of the active form of Glycogen Synthase Kinase 3β (GSK3β) responsible for the reduced amount of active β-catenin, and altered the amount of AXIN2 mRNA. Taken together, these data underline the impact of CDK4 and CDK6 inhibitors in treating adrenocortical carcinomas.

PMID: 29283884 [PubMed - indexed for MEDLINE]

Genotype phenotype correlation in Asian Indian von Hippel-Lindau (VHL) syndrome patients with pheochromocytoma/paraganglioma.

Wed, 03/13/2019 - 18:47
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Genotype phenotype correlation in Asian Indian von Hippel-Lindau (VHL) syndrome patients with pheochromocytoma/paraganglioma.

Fam Cancer. 2018 07;17(3):441-449

Authors: Lomte N, Kumar S, Sarathi V, Pandit R, Goroshi M, Jadhav S, Lila AR, Bandgar T, Shah NS

Abstract
The data in genotype-phenotype correlation in Indian von Hippel-Lindau (VHL) patients is limited. We have retrospectively studied 31 genetically proven VHL patients with pheochromocytoma/paraganglioma (PCC/PGL) from families and have reviewed the World literature on PCC/PGL in patients with large VHL deletions. Three patients had large deletions and 28 patients had other mutations [missense mutations in 25, 3 bp deletion in 2 and single bp duplication in one]. Unilateral PCC were significantly more common in patients with large VHL deletions whereas multiple PCC (bilateral PCC or PCC + sympathetic PGL) were significantly more common in those with other mutations. World literature review confirmed the rarity of PCC/PGL in patients with large deletions and we report the first definitive case of PCC associated with complete VHL deletion. Pancreatic neuroendocrine tumours were more common, often metastatic and the most common cause of death in our cohort. Our study had eight parent off-spring pairs from five families. The off-springs were significantly younger at presentation and had significantly higher number of PCC/PGL. In conclusion, PCC/PGL are rare in patients with large VHL deletions and if occur are most likely to be solitary. Patients with bilateral PCC or multifocal PCC/PGL are least likely to have large VHL deletions. Our study also provides additional evidence for existence of the phenomenon of anticipation in VHL syndrome.

PMID: 29124493 [PubMed - indexed for MEDLINE]

Pheochromocytoma presenting as anxiety disorder: A case report.

Wed, 03/13/2019 - 18:47
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Pheochromocytoma presenting as anxiety disorder: A case report.

Asian J Psychiatr. 2017 Oct;29:83-84

Authors: Sriram P, Raghavan V

PMID: 29061435 [PubMed - indexed for MEDLINE]

Ectopic, retroperitoneal adrenocortical carcinoma in the setting of Lynch syndrome.

Wed, 03/13/2019 - 18:47
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Ectopic, retroperitoneal adrenocortical carcinoma in the setting of Lynch syndrome.

Fam Cancer. 2018 07;17(3):381-385

Authors: Wright JP, Montgomery KW, Tierney J, Gilbert J, Solórzano CC, Idrees K

Abstract
Adrenocortical carcinoma (ACC) is rare within the adult population. Ectopic ACC proves even rarer. This variant is formed by cortical fragments arrested during embryologic migration. ACC is also known to be associated with several genetic syndromes and has recently been linked to Lynch syndrome in 3% of cases. We present the case of a 68-year-old male with a confirmed diagnosis of Lynch syndrome secondary to a germline MSH2 mismatch-repair gene-mutation who presented with 2 months history of non-specific abdominal pain. After imaging work-up, the patient was found to have a right upper quadrant, retroperitoneal mass. Biochemical tests were without any evidence of a hormonally active process. Fine needle aspiration of the mass revealed a poorly differentiated carcinoma of unknown etiology. The lesion was resected and found to be consistent with ectopic ACC with an associated MSH2 mutation.

PMID: 28940135 [PubMed - indexed for MEDLINE]

Pediatric Posterior Reversible Encephalopathy Syndrome (PRES) With Spinal Cord Involvement Due to Pheochromocytoma.

Wed, 03/13/2019 - 18:47
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Pediatric Posterior Reversible Encephalopathy Syndrome (PRES) With Spinal Cord Involvement Due to Pheochromocytoma.

Pediatr Neurol. 2017 12;77:92-93

Authors: Freedman D, Koram A, Gillson N, Aylward SC

PMID: 28939016 [PubMed - indexed for MEDLINE]

Epigenetic dysregulation in adrenocortical carcinoma, a systematic review of the literature.

Wed, 03/13/2019 - 18:47
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Epigenetic dysregulation in adrenocortical carcinoma, a systematic review of the literature.

Mol Cell Endocrinol. 2018 07 05;469:77-84

Authors: Jonker PKC, Meyer VM, Kruijff S

Abstract
Adrenocortical carcinoma (ACC) is a rare and aggressive endocrine malignancy with a poor prognosis. Diagnosis and treatment of this tumor remains challenging. The Weiss score, the current gold standard for the histopathological diagnosis of ACC, lacks diagnostic accuracy of borderline tumors (Weiss score 2 or 3) and is subject to inter observer variability. Furthermore, adjuvant and palliative systemic therapy have limited effect and no proven overall survival benefit. A better insight in the molecular background of ACC might identify markers that improve diagnostic accuracy, predict treatment response or even provide novel therapeutic targets. This systematic review of the literature aims to provide an overview of alterations in DNA methylation, histone modifications and their potential clinical relevance in ACC.

PMID: 28830787 [PubMed - indexed for MEDLINE]

Sleep-Related Laryngeal Stridor in Opsoclonus Myoclonus Syndrome.

Wed, 03/13/2019 - 18:47
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Sleep-Related Laryngeal Stridor in Opsoclonus Myoclonus Syndrome.

Pediatr Neurol. 2017 12;77:91

Authors: Mizuno T, Kumada S, Naito R

PMID: 28823798 [PubMed - indexed for MEDLINE]

Succinate dehydrogenase (SDH) deficiency, Carney triad and the epigenome.

Wed, 03/13/2019 - 18:47
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Succinate dehydrogenase (SDH) deficiency, Carney triad and the epigenome.

Mol Cell Endocrinol. 2018 07 05;469:107-111

Authors: Settas N, Faucz FR, Stratakis CA

Abstract
In this report, we review the relationship between succinate dehydrogenase (SDH) deficiency and the epigenome, especially with regards to two clinical conditions. Carney triad (CT) is a very rare disease with synchronous or metachronous occurrence of at least three different tumor entities; gastric gastrointestinal stromal tumor (GIST), paraganglioma (PGL), and pulmonary chondroma. This condition affects mostly females and it is never inherited. Another disease that shares two of the tumor components of CT, namely GIST and PGL is the Carney-Stratakis syndrome (CSS) or dyad. CSS affects both genders during childhood and adolescence. We review herein the main clinical features and molecular mechanisms behind those two syndromes that share quite a bit of similarities, but one is non-hereditary (CT) whereas the other shows an autosomal-dominant, with incomplete penetrance, inheritance pattern (CSS). Both CT and CSS are caused by the deficiency of the succinate dehydrogenase (SDH) enzyme. The key difference between the two syndromes is the molecular mechanism that causes the SDH deficiency. Most cases of CT show down-regulation of SDH through site-specific hyper-methylation of the SDHC gene, whereas CSS cases carry inactivating germline mutations within one of the genes coding for the SDH subunits A, B, C, or D (SDHA, SDHB, SDHC, and SDHD). There is only partial overlap between the two conditions (there are a few patients with CT that have SDH subunit mutations) but both lead to increased methylation of the entire genome in the tumors associated with them. Other tumors (outside CT and CSS) that have SDH deficiency are associated with increased methylation of the entire genome, but only in CT there is site-specific methylation of the SDHC gene. These findings have implications for diagnostics and the treatment of patients with these, often metastatic tumors.

PMID: 28739378 [PubMed - indexed for MEDLINE]

Clinical utility of routine postoperative morning cortisol monitoring in detecting new hypothalamic-pituitary-adrenal axis insufficiency following endoscopic transsphenoidal surgery for sellar lesions.

Wed, 03/06/2019 - 16:46
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Clinical utility of routine postoperative morning cortisol monitoring in detecting new hypothalamic-pituitary-adrenal axis insufficiency following endoscopic transsphenoidal surgery for sellar lesions.

J Neurosurg. 2019 Mar 01;:1-5

Authors: Jackanich A, Tavakol S, Strickland BA, Rutkowski M, Kamel D, Carmichael JD, Weiss M, Zada G

Abstract
OBJECTIVEHypothalamic-pituitary-adrenal (HPA) axis dysfunction is a well-documented complication of transsphenoidal craniotomy (TSC) for sellar lesions. The authors aimed to assess their multidisciplinary approach to the diagnosis and treatment of postoperative hypocortisolemia utilizing conservative screening methods.METHODSThe authors performed a retrospective review of 257 patients who underwent TSC for pituitary adenoma (PA) or Rathke cleft cyst (RCC) at the University of Southern California between 2012 and 2017. Patients with preoperative adrenal insufficiency, Cushing's disease, or < 3 months of postoperative follow-up were excluded. Patient demographics, pathology, tumor characteristics, and complications were recorded. Postoperative day 1 (POD1) morning serum cortisol was assessed in all patients. Hypocortisolemia on POD1 (serum cortisol < 5 μg/dl) prompted a 7 am cortisol level measurement on POD 2 (POD2). Clinical signs and symptoms of hypocortisolemia were consistently monitored. After two serum cortisol levels < 5 μg/dl, or one serum level < 5 μg/dl plus a high clinical suspicion for HPA dysfunction, high-risk patients received glucocorticoid supplementation.RESULTSData on 165 patients were included in the analysis; there were 101 women (61.2%) and 64 men (38.7%). Preoperative diagnoses included nonfunctional adenoma (n = 97, 58.7%), growth hormone-secreting adenoma (n = 37, 22.4%), RCC (n = 18, 10.9%), prolactinoma (n = 8, 4.8%), and other (n = 5, 3.0%). One hundred thirty-eight patients (63.0%) had either suprasellar extension or cavernous sinus invasion. POD1 hypocortisolemia was diagnosed in 8 patients (4.8%). Of these patients, 2 (1.2%) were clinically asymptomatic and had normalized POD2 cortisol levels. Six patients (3.6%) had clinical symptoms and POD2 cortisol levels confirming HPA axis deficiency. Of these 6 patients treated with early glucocorticoid replacement, 2 patients recovered HPA axis function during follow-up, making the incidence of new, permanent HPA axis deficiency 2.5%.CONCLUSIONSIn the authors' institutional review, all patients warranting postoperative glucocorticoid replacement had both complicated surgical courses and associated clinical symptoms of hypocortisolemia. The authors' algorithm of withholding steroids until patients demonstrate clear evidence of postoperative hypocortisolemia is safe and clinically efficacious. Their data further suggest that routine postoperative cortisol screening may not be necessary following an uncomplicated operative resection, with gland preservation and the absence of clinical symptoms indicative of HPA dysfunction.

PMID: 30835697 [PubMed - as supplied by publisher]

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