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NCBI: db=pubmed; Term=adrenal tumor
Updated: 5 days 5 hours ago

Intravascular B-large cell lymphoma: an unexpected diagnosis of an incidental adrenal mass.

Wed, 02/27/2019 - 16:28
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Intravascular B-large cell lymphoma: an unexpected diagnosis of an incidental adrenal mass.

J Surg Case Rep. 2019 Feb;2019(2):rjz048

Authors: Kiriakopoulos A, Linos D

Abstract
Adrenal incidentalomas originally defined as tumors discovered serendipitously in the course of diagnostic evaluation or follow-up of unrelated disorders, may occasionally pose serious diagnostic challenges. Intravascular large B-cell lymphoma (IVLBCL) may be a rare example of such a case. We present an IVLBCL confined to the adrenal gland in a 52-year-old man focusing on its diagnostic and therapeutic aspects. On endocrine work up, the tumor was hormonally inactive and exhibited inconclusive imaging characteristics without signs of locoregional spread. After a left laparoscopic adrenalectomy, histologic sections revealed the presence of tumor cells inside dilated, thin-walled vascular spaces. Immunohistochemical stains confirmed the diagnosis of IVLBCL. The patient was then referred to a Hematology Unit for further staging and treatment and received six cycles of R-CHOP. Despite the fact that IVLBCL carries a dismal prognosis our patient remains alive and in complete remission 6 years after the initial diagnosis.

PMID: 30800279 [PubMed]

Differences in Genomic Profiles and Outcomes between Thoracic and Adrenal Neuroblastoma.

Wed, 02/27/2019 - 16:28
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Differences in Genomic Profiles and Outcomes between Thoracic and Adrenal Neuroblastoma.

J Natl Cancer Inst. 2019 Feb 21;:

Authors: Oldridge DA, Truong B, Russ D, DuBois SG, Vaksman Z, Mosse YP, Diskin SJ, Maris JM, Matthay KK

Abstract
BACKGROUND: Neuroblastoma is a biologically and clinically heterogeneous disease. Based on recent studies demonstrating an association between the primary tumor site, prognosis, and commonly measured tumor biological features, we hypothesized that neuroblastomas arising in different sites would show distinct genomic features reflective of the developmental biology of the sympathicoadrenal nervous system.
METHODS: We first compared genomic and epigenomic data of primary diagnostic neuroblastomas originating in the adrenal gland (N = 646) compared to thoracic sympathetic ganglia (N = 118). We also evaluated association of common germline variation with these primary sites in 1,027 European-American neuroblastoma patients.
RESULTS: We observed higher rates of MYCN amplification, chromosome 1q gain, and chromosome 11q deletion among adrenal tumors, which were highly predictive of functional RNA signatures. Surprisingly, thoracic neuroblastomas were more likely to harbor ALK driver mutations than adrenal cases among all cases (OR = 1.89, 95% CI = 1.04-3.43), and among cases without MYCN amplification (OR = 2.86, 95% CI = 1.48-5.49). Common germline SNPs in BARD1 (previously associated with high-risk neuroblastoma) were found to be strongly associated with predisposition for origin at adrenal, rather than thoracic, sites.
CONCLUSIONS: Neuroblastomas arising in the adrenal gland are more likely to harbor structural DNA aberrations including MYCN amplification, whereas thoracic tumors show defects in mitotic checkpoints resulting in hyperdiploidy. Despite the general association of ALK mutations with high-risk disease, thoracic tumors are more likely to harbor gain-of-function ALK aberrations. Site of origin is likely reflective of stage of sympathetic nervous system development when malignant transformation occurs and is a surrogate for underlying tumor biology.

PMID: 30793172 [PubMed - as supplied by publisher]

Limited significance of repeated long-term radiological and hormonal examination in nonfunctioning adrenal incidentalomas.

Wed, 02/27/2019 - 16:28
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Limited significance of repeated long-term radiological and hormonal examination in nonfunctioning adrenal incidentalomas.

Int Braz J Urol. 2018 Dec 18;44:

Authors: Tasaki M, Kasahara T, Takizawa I, Saito K, Nishiyama T, Tomita Y

Abstract
PURPOSE: The purposes of the present study were to evaluate growth rate of nonfunctioning adrenal incidentalomas (AIs) and their development to hormonal hypersecretion on follow-up.
MATERIALS AND METHODS: A retrospective study was conducted from the electronic medical records. A total of 314 patients were diagnosed with adrenal tumors between 2000 and 2016. After excluding patients who had overt adrenal endocrine disorders or whose adrenal tumors were clinically diagnosed as metastatic malignancies, we investigated 108 patients with nonfunctioning AIs including characteristics, the treatment, the way of follow-up and pathology.
RESULTS: Fifteen patients received immediate adrenalectomy because of the initial tumor size or patient's preference. Pathological examination revealed malignancy in 2 patients. In the remaining 93 patients, radiological examinations were performed periodically. Tumor enlargement of ≥ 1.0cm was observed in 8.6% of the patients who were followed up as nonfunctioning AIs with a median follow-up period of 61.5 months (range: 4-192). Eleven patients underwent adrenalectomy. On the pathological examinations, all of the tumors, which showed a size increase, were diagnosed as benign tumors. Regarding the followed up patients without adrenalectomy, only 2.4% of the patients had tumor enlargement during the prolonged follow-up. Furthermore, none of the patients developed hormonal hypersecretion or clinical signs such as obesity, glucose intolerance or poorly controlled hypertension.
CONCLUSIONS: Tumor enlargement of AIs did not correlate with malignancy. The value of repeat radiological and hormonal examinations may be limited in the long-term follow-up of patients whose AIs are not enlarged.

PMID: 30785700 [PubMed - as supplied by publisher]

Late-onset isolated adrenocorticotropic hormone deficiency caused by nivolumab: a case report.

Wed, 02/27/2019 - 16:28
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Late-onset isolated adrenocorticotropic hormone deficiency caused by nivolumab: a case report.

BMC Endocr Disord. 2019 Feb 19;19(1):25

Authors: Takeno A, Yamamoto M, Morita M, Tanaka S, Kanazawa I, Yamauchi M, Kaneko S, Sugimoto T

Abstract
BACKGROUND: Immune checkpoint inhibitors including nivolumab, an anti-programmed cell death protein 1 antibody, are recently developed cancer immunotherapy agents. Immune checkpoint inhibitors are known to cause autoimmune-related side effects including endocrine dysfunctions. However, there are few reports on late-onset isolated adrenocorticotropic hormone (ACTH) deficiency caused by nivolumab.
CASE PRESENTATION: The patient was a 72-year-old female. When she was 64 years old, she was diagnosed with malignant melanoma of the left thigh accompanied by left inguinal lymph node metastases, and she received several courses of chemotherapy for malignant melanoma followed by the resection of these lesions. At 71 years of age, multiple metastases were found and treatment with nivolumab 2 mg/kg every 3 weeks was initiated. Six months later, replacement with levothyroxine was started because of hypothyroidism following mild transient thyrotoxicosis. Eleven months after the beginning of nivolumab, the treatment was discontinued because of tumor expansion. Four months after the discontinuation of nivolumab, general malaise and appetite loss worsened, and 2 months later, hyponatremia (Na; 120-127 mEq/L) and hypoglycemia (fasting plasma glucose; 62 mg/dL) appeared. Her ACTH and cortisol levels were extremely low (ACTH; 9.6 pg/mL, cortisol; undetectable). Challenge tests for anterior pituitary hormones showed that responses of ACTH and cortisol secretion to corticotropin-releasing hormone were disappeared, although responses of other anterior pituitary hormones were preserved. Thus, she was diagnosed with isolated ACTH deficiency. Her symptoms were improved after treatment with hydrocortisone.
CONCLUSIONS: The present report showed a case of late-onset isolated ACTH deficiency accompanied by hyponatremia, which was diagnosed 6 months after the discontinuation of nivolumab. The effects of nivolumab last for a long time and the side effects of nivolumab can also appear several months after discontinuation of the drug. Repeated monitoring of serum sodium levels may be a beneficial strategy to find the unexpected development of adrenal insufficiency even after discontinuation of nivolumab.

PMID: 30782163 [PubMed - in process]

Operative Management of Recurrent and Metastatic Adrenocortical Carcinoma: A Systematic Review.

Wed, 02/27/2019 - 16:28
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Operative Management of Recurrent and Metastatic Adrenocortical Carcinoma: A Systematic Review.

Am Surg. 2019 Jan 01;85(1):23-28

Authors: Lo WM, Kariya CM, Hernandez JM

Abstract
Metastatic adrenocortical carcinoma (ACC) is associated with a poor 5-year survival rate and high rate of recurrence. Outcomes after resection for patients with limited disease remain poorly described. We conducted a PubMed search for articles published between 1950 and 2017 using the terms "ACC," "recurrence," and "surgery." Patients with metastatic ACC at any anatomic site who had undergone surgical resection were included. Thirteen studies met the criteria. Patients were grouped according to the recurrence site. Pulmonary metastasectomy was reported in 50 patients with moderate complications and without perioperative mortality. Disease recurrence rates range from 25 to 42 per cent, with median overall survival of 40 to 50 months. Hepatic metastasectomy was reported in 108 patients with a single perioperative mortality. Disease recurrence rates range from 65 to 100 per cent, with median disease-free survival (DFS) and OS of five to nine months and 22 to 76 months. Peritoneal cytoreduction and heated intraperitoneal chemotherapy have been reported for 10 patients with minimal morbidity and without perioperative mortality. The disease recurrence rate was 70 per cent, with DFS of 19 months. For selected patients with recurrent ACC in the lungs, liver, or peritoneum, metastasectomy is safe and can be associated with prolonged survival. However, subsequent disease recurrence is common, and patients should be counseled accordingly.

PMID: 30760340 [PubMed - indexed for MEDLINE]

Comparison of transperitoneal laparoscopic versus open adrenalectomy for large pheochromocytoma: A retrospective propensity score-matched cohort study.

Wed, 02/27/2019 - 16:28
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Comparison of transperitoneal laparoscopic versus open adrenalectomy for large pheochromocytoma: A retrospective propensity score-matched cohort study.

Int J Surg. 2019 Jan;61:26-32

Authors: Bai S, Yao Z, Zhu X, Li Z, Jiang Y, Wang R, Wu B

Abstract
BACKGROUND: Surgical resection is the main treatment strategy for pheochromocytoma. Whether laparoscopic techniques are feasible for large pheochromocytoma treatments is controversial. The aim of this study was to evaluate the feasibility and safety of transperitoneal laparoscopic adrenalectomy (LA) compared with open adrenalectomy (OA).
METHODS: We retrospectively studied 182 patients with radiographic tumor sizes ≥ 6 cm who underwent adrenalectomy at our center between 1 January 2007 and 31 December 2017. After propensity score-matching to balance baseline variables, 82 patients treated with LA and 100 patients treated with OA were grouped into 64 pairs. Patient demographics, and extensive peri-operative and oncologic data were recorded and compared.
RESULTS: In the matched group, the incidence of intra-operative hemodynamic instability, transfusion rate, prolonged hypotension, and cardiovascular morbidity in the LA group were lower than the OA group (25.0% vs. 48.4%, 29.7% vs. 46.9%, 4.7% vs. 23.4%, and 14.1% vs. 37.5%, respectively). The estimated blood loss was less (100 ml vs. 300 ml) and bowel recovery was quicker (1.9 d vs. 2.3 d) in the LA group. The proportion of patients in whom blood pressure returned to normal (P = 0.184), had recurrences (P = 0.197), and survived (P = 0.763) were equivalent. The surgical approach (OA vs. LA) was an independent risk factor for IHD, prolonged hypotension, cardiovascular morbidity, and longer bowel recovery.
CONCLUSIONS: This propensity score-matched cohort study showed that LA was feasible, safe, and superior to OA for patients with large pheochromocytoma, meanwhile LA had comparable oncological outcomes compared with OA after a lengthy follow-up.

PMID: 30503601 [PubMed - indexed for MEDLINE]

Plasmodium falciparum Treated with Artemisinin-based Combined Therapy Exhibits Enhanced Mutation, Heightened Cortisol and TNF-α Induction.

Wed, 02/27/2019 - 16:28
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Plasmodium falciparum Treated with Artemisinin-based Combined Therapy Exhibits Enhanced Mutation, Heightened Cortisol and TNF-α Induction.

Int J Med Sci. 2018;15(13):1449-1457

Authors: Idowu AO, Bhattacharyya S, Gradus S, Oyibo W, George Z, Black C, Igietseme J, Azenabor AA

Abstract
The artemisinin-based combined therapy (ACT) post-treatment illness in Plasmodium falciparum-endemic areas is characterized by vague malaria-like symptoms. The roles of treatment modality, persistence of parasites and host proinflammatory response in disease course are unknown. We investigated the hypothesis that ACT post-treatment syndrome is driven by parasite genetic polymorphisms and proinflammatory response to persisting mutant parasites. Patients were categorized as treated, untreated and malaria-negative. Malaria positive samples were analyzed for Pfcrt, Pfmdr1, K13 kelch gene polymorphisms, while all samples were evaluated for cytokines (TNF-α, IL-12p70, IL-10, TGF-β, IFN-γ) and corticosteroids (cortisol and dexamethasone) levels. The treated patients exhibited higher levels of parasitemia, TNF-α, and cortisol, increased incidence of parasite genetic mutations, and greater number of mutant alleles per patient. In addition, corticosteroid levels declined with increasing number of mutant alleles. TGF-β levels were negatively correlated with parasitemia, while IL-10 and TGF-β were negatively correlated with increasing number of mutant alleles. However, IL-12 displayed slight positive correlation and TNF-α exhibited moderate positive correlation with increasing number of mutant alleles. Since post-treatment management ultimately results in patient recovery, the high parasite gene polymorphism may act in concert with induced cortisol and TNF-α to account for ACT post-treatment syndrome.

PMID: 30443164 [PubMed - indexed for MEDLINE]

Surgical Treatment of Malignant Pheochromocytomas in Spine.

Wed, 02/27/2019 - 16:28
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Surgical Treatment of Malignant Pheochromocytomas in Spine.

Chin Med J (Engl). 2018 Nov 05;131(21):2614-2615

Authors: Liu SZ, Zhou X, Song A, Huo Z, Wang YP, Liu Y

Abstract

PMID: 30381598 [PubMed - indexed for MEDLINE]

Two siblings with familial neuroblastoma with distinct clinical phenotypes harboring an ALK germline mutation.

Wed, 02/27/2019 - 16:28
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Two siblings with familial neuroblastoma with distinct clinical phenotypes harboring an ALK germline mutation.

Genes Chromosomes Cancer. 2018 12;57(12):665-669

Authors: Kudo K, Ueno H, Sato T, Kubo K, Kanezaki R, Kobayashi A, Kamio T, Sasaki S, Terui K, Kurose A, Yoshida K, Shiozawa Y, Toki T, Ogawa S, Ito E

Abstract
The authors report two siblings with familial neuroblastoma with a germline R1275Q mutation of the tyrosine kinase domain of ALK. Whole exome sequencing and copy number variation assay were performed to investigate genetic alterations in the two cases. No common somatic mutations or gene polymorphisms related to the tumorigenesis of neuroblastoma were detected. A distinct pattern involving both segmental chromosomal alteration and MYCN amplification was detected. The diversity of biological behavior of familial neuroblastoma harboring a germline ALK mutation may depend on conventional prognostic factors, such as segmental chromosomal alterations and MYCN amplification, rather than additional acquired mutations.

PMID: 30350464 [PubMed - indexed for MEDLINE]

[Polymetastatic highly aggressive Small Cell Undifferentiated Neuroendocrine Carcinoma of Ascending Colon].

Wed, 02/27/2019 - 16:28
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[Polymetastatic highly aggressive Small Cell Undifferentiated Neuroendocrine Carcinoma of Ascending Colon].

Cir Cir. 2018;86(5):446-449

Authors: Ioannidis O, Chatzidelios C, Mantzoros I, Tatsis D, Kitsikosta L, Chatzakis C, Makrantonakis A, Paraskevas G, Kotidis E, Pramateftakis MG, Tsalis K

Abstract
Neuroendocrine carcinoma (NEC) of the colon is a rare and very aggressive tumor with poor prognosis. The current case report presents a 53-year-old male with a 6 cm × 10 cm ascending colon carcinoma, causing large intestine obstruction, with simultaneous multiple hepatic metastases and peritoneal carcinomatosis. Surgical resection of the primary tumor was performed, because of the bowel obstruction, to ameliorate the symptoms before the onset of chemotherapy. Histopathology revealed that the tumor was a small-cell undifferentiated NEC. During the post0operative period, the patient presented pulmonary metastases, and on the 36th post-operative day, death occurred due to respiratory failure.

PMID: 30226488 [PubMed - indexed for MEDLINE]

Characterization of adrenal lesions using MDCT wash-out parameters: diagnostic accuracy of several combinations of intermediate and delayed phases.

Wed, 02/27/2019 - 16:28
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Characterization of adrenal lesions using MDCT wash-out parameters: diagnostic accuracy of several combinations of intermediate and delayed phases.

Radiol Med. 2018 Nov;123(11):833-840

Authors: Foti G, Malleo G, Faccioli N, Guerriero A, Furlani L, Carbognin G

Abstract
PURPOSE: To evaluate the diagnostic accuracy of wash-out parameters calculated using multiple intermediate and delayed phases.
MATERIALS AND METHODS: This prospective study had institutional review board approval and informed consent was obtained from all patients. Between January 2012 and October 2016, 108 consecutive oncologic patients (59 males, 49 females, mean age 52.6 years; 129 diagnosed lesions) underwent multiphasic CT protocol including unenhanced (UE), arterial (AE), portal (PE), 5-min (DE-5) and the 15-min (DE-15) delayed phases of adrenal glands. All images were randomly reviewed in consensus by two radiologists experienced in abdominal CT, unaware of clinical or pathologic data. Location, size and density were recorded. Absolute wash-out, percentage wash-out (PWO) and percentage enhancement wash-out ratio were calculated. The thresholds yielding the best accuracy in differentiating adenomas from nonadenomas were retrospectively determined on the basis of ROC curves. The corresponding diagnostic accuracy values were calculated. Paired sample t test was used to assess differences among imaging parameters within subgroups. Student t test was applied to compare lesions between independent subgroups. p values ≤ 0.05 were considered significant.
RESULTS: The final diagnosis included 82 adenomas (62 lipid-rich and 20 lipid-poor) and 47 nonadenomas (42 metastases, 3 pheochromocytomas, 2 carcinomas). All the 62 lipid-rich adenomas were correctly diagnosed as benign lesions on the basis of their UE attenuation < 10 HU. The PEAK attenuation was achieved during AE phase for 51/129 lesions (39.5%) and at the time of PE phase in 78/129 lesions (60.5%). The best overall accuracy in diagnosing adenomas (97.6%; 126/129 lesions correctly diagnosed) was obtained using 40% threshold for calculating PWO from PEAK to DE-15 scan.
CONCLUSIONS: If only an intermediate phase is available, the 15-min delayed scan should be acquired to avoid any drop in diagnostic accuracy. The availability of two intermediate phase may be used to easy CT schedule by obviating the need to acquire a longer delayed phase.

PMID: 29923085 [PubMed - indexed for MEDLINE]

Anesthetic Management of Staged Bilateral Adrenalectomy for Neuroendocrine Tumors in a Heart-Lung Transplant Patient: A Case Report.

Wed, 02/27/2019 - 16:28
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Anesthetic Management of Staged Bilateral Adrenalectomy for Neuroendocrine Tumors in a Heart-Lung Transplant Patient: A Case Report.

A A Pract. 2018 Nov 01;11(9):253-257

Authors: Liu AC, Andrews G, Ben-Menachem E

Abstract
Neuroendocrine tumors may rarely present after organ transplantation, including cardiac transplant. Treatment is surgical resection with careful perioperative management to optimize blood pressure and intravascular volume. We present the anesthetic management of a patient who was diagnosed with bilateral neuroendocrine tumors soon after heart-lung transplantation and underwent successful staged bilateral adrenalectomy.

PMID: 29851693 [PubMed - indexed for MEDLINE]

Nicotinamide Nucleotide Transhydrogenase as a Novel Treatment Target in Adrenocortical Carcinoma.

Wed, 02/27/2019 - 16:28
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Nicotinamide Nucleotide Transhydrogenase as a Novel Treatment Target in Adrenocortical Carcinoma.

Endocrinology. 2018 08 01;159(8):2836-2849

Authors: Chortis V, Taylor AE, Doig CL, Walsh MD, Meimaridou E, Jenkinson C, Rodriguez-Blanco G, Ronchi CL, Jafri A, Metherell LA, Hebenstreit D, Dunn WB, Arlt W, Foster PA

Abstract
Adrenocortical carcinoma (ACC) is an aggressive malignancy with poor response to chemotherapy. In this study, we evaluated a potential new treatment target for ACC, focusing on the mitochondrial reduced form of NAD phosphate (NADPH) generator nicotinamide nucleotide transhydrogenase (NNT). NNT has a central role within mitochondrial antioxidant pathways, protecting cells from oxidative stress. Inactivating human NNT mutations result in congenital adrenal insufficiency. We hypothesized that NNT silencing in ACC cells will induce toxic levels of oxidative stress. To explore this, we transiently knocked down NNT in NCI-H295R ACC cells. As predicted, this manipulation increased intracellular levels of oxidative stress; this resulted in a pronounced suppression of cell proliferation and higher apoptotic rates, as well as sensitization of cells to chemically induced oxidative stress. Steroidogenesis was paradoxically stimulated by NNT loss, as demonstrated by mass spectrometry-based steroid profiling. Next, we generated a stable NNT knockdown model in the same cell line to investigate the longer lasting effects of NNT silencing. After long-term culture, cells adapted metabolically to chronic NNT knockdown, restoring their redox balance and resilience to oxidative stress, although their proliferation remained suppressed. This was associated with higher rates of oxygen consumption. The molecular pathways underpinning these responses were explored in detail by RNA sequencing and nontargeted metabolome analysis, revealing major alterations in nucleotide synthesis, protein folding, and polyamine metabolism. This study provides preclinical evidence of the therapeutic merit of antioxidant targeting in ACC as well as illuminating the long-term adaptive response of cells to oxidative stress.

PMID: 29850793 [PubMed - indexed for MEDLINE]

AR Signaling in Prostate Cancer Regulates a Feed-Forward Mechanism of Androgen Synthesis by Way of HSD3B1 Upregulation.

Wed, 02/27/2019 - 16:28
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AR Signaling in Prostate Cancer Regulates a Feed-Forward Mechanism of Androgen Synthesis by Way of HSD3B1 Upregulation.

Endocrinology. 2018 08 01;159(8):2884-2890

Authors: Hettel D, Zhang A, Alyamani M, Berk M, Sharifi N

Abstract
3βHSD1 enzymatic activity is essential for synthesis of potent androgens from adrenal precursor steroids in prostate cancer. A germline variant in HSD3B1, the gene that encodes 3βHSD1, encodes for a stable enzyme, regulates adrenal androgen dependence, and is a predictive biomarker of poor clinical outcomes after gonadal testosterone deprivation therapy. However, little is known about HSD3B1 transcriptional regulation. Generally, it is thought that intratumoral androgen synthesis is upregulated after gonadal testosterone deprivation, enabling development of castration-resistant prostate cancer. Given its critical role in extragonadal androgen synthesis, we sought to directly interrogate the transcriptional regulation of HSD3B1 in multiple metastatic prostate cancer cell models. Surprisingly, we found that VCaP, CWR22Rv1, LNCaP, and LAPC4 models demonstrate induction of HSD3B1 upon androgen stimulation for approximately 72 hours, followed by attenuation around 120 hours. 3βHSD1 protein levels mirrored transcriptional changes in models harboring variant (LNCaP) and wild-type (LAPC4) HSD3B1, and in these models androgen induction of HSD3B1 is abrogated via enzalutamide treatment. Androgen treatment increased flux from [3H]-dehydroepiandrosterone to androstenedione and other downstream metabolites. HSD3B1 expression was reduced 72 hours after castration in the VCaP xenograft mouse model, suggesting androgen receptor (AR) regulation of HSD3B1 also occurs in vivo. Overall, these data suggest that HSD3B1 is unexpectedly positively regulated by androgens and ARs. These data may have implications for the development of treatment strategies tailored to HSD3B1 genotype status.

PMID: 29850791 [PubMed - indexed for MEDLINE]

Hormone-secreting adrenal tumours cause severe hypertension and high rates of poor pregnancy outcome; a UK Obstetric Surveillance System study with case control comparisons.

Wed, 02/27/2019 - 16:28
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Hormone-secreting adrenal tumours cause severe hypertension and high rates of poor pregnancy outcome; a UK Obstetric Surveillance System study with case control comparisons.

BJOG. 2018 May;125(6):719-727

Authors: Quartermaine G, Lambert K, Rees K, Seed PT, Dhanjal MK, Knight M, McCance DR, Williamson C

Abstract
OBJECTIVE: To examine the management and outcomes of adrenal tumours in pregnancy.
DESIGN: A national observational, cohort study over 4 years using the UK Obstetric Surveillance System (UKOSS).
SETTING: Consultant-led obstetric units.
PATIENTS: Women with phaeochromocytoma, primary aldosteronism or Cushing's syndrome diagnosed before or during pregnancy.
METHODS: Clinical features of UKOSS cases were compared with those of women with adrenal tumours reported from 1985-2015. Nested case-control comparisons involving the UKOSS cases as well as those identified in the literature were performed for pregnancy outcome data using UKOSS controls with uncomplicated singleton (n = 2250) pregnancy and data from the Office of National Statistics (ONS).
MAIN OUTCOME MEASURES: Incidence, management and frequency of adverse maternal and offspring outcomes of adrenal tumours in pregnancy.
RESULTS: Fifteen pregnant women met the inclusion criteria: ten phaeochromocytoma, three primary aldosteronism and two Cushing's syndrome. All of the tumours had an incidence rate <2 per 100 000 pregnancies. Clinical symptoms were similar to those in non-pregnant women due to the hormones released. All women had severe hypertension, and in those diagnosed in pregnancy prior to conception. There was a significantly increased risk of adverse pregnancy outcomes in affected women, with increased rates of stillbirth, preterm labour and operative delivery.
CONCLUSIONS: Adrenal tumours are associated with increased risks for pregnant women and their babies. Data on these tumours to inform practice are limited and international collaborative efforts are likely to be needed.
TWEETABLE ABSTRACT: Study of hormone-secreting adrenal tumours in pregnancy linked with high BP and high rates of fetal morbidity.

PMID: 28872770 [PubMed - indexed for MEDLINE]

Adrenal Collision Tumor Composed of Pheochromocytoma and Diffuse Large B-Cell Lymphoma: A Case Report.

Wed, 02/20/2019 - 15:54
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Adrenal Collision Tumor Composed of Pheochromocytoma and Diffuse Large B-Cell Lymphoma: A Case Report.

Int J Hematol Oncol Stem Cell Res. 2018 Oct 01;12(4):249-252

Authors: Khorsand A, Khatami F, Sefidbakht S, Saffar H, Sadeghipour A, Tavangar SM

Abstract
Adrenal involvement in the course of malignant lymphoma occurs in about 4% of patients, but primary adrenal lymphoma (PAL) is extremely rare. To the best of our knowledge, only one case study reported the combination of PAL with pheochromocytoma. In the current study, we present the second case who was a 63-year-old man admitted to our hospital with hematuria and abdominal discomfort. Ultrasound imaging indicated the presence of a lesion, 5×4×3 cm in size, in the upper pole of his right kidney. Histopathologic study confirmed a collision tumor composed of pheochromocytoma and diffuse large B-cell lymphoma (DLBCL).

PMID: 30774823 [PubMed]

The Value of Histological Algorithms to Predict the Malignancy Potential of Pheochromocytomas and Abdominal Paragangliomas-A Meta-Analysis and Systematic Review of the Literature.

Wed, 02/20/2019 - 15:54
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The Value of Histological Algorithms to Predict the Malignancy Potential of Pheochromocytomas and Abdominal Paragangliomas-A Meta-Analysis and Systematic Review of the Literature.

Cancers (Basel). 2019 Feb 15;11(2):

Authors: Stenman A, Zedenius J, Juhlin CC

Abstract
Pheochromocytomas (PCCs) and abdominal paragangliomas (PGLs), collectively abbreviated PPGLs, are neuroendocrine tumors of the adrenal medulla and paraganglia, respectively. These tumors exhibit malignant potential but seldom display evidence of metastatic spread, the latter being the only widely accepted evidence of malignancy. To counter this, pre-defined histological algorithms have been suggested to stratify the risk of malignancy: Pheochromocytoma of the Adrenal Gland Scaled Score (PASS) and the Grading system for Adrenal Pheochromocytoma and Paraganglioma (GAPP). The PASS algorithm was originally intended for PCCs whereas the GAPP model is proposed for stratification of both PCCs and PGLs. In parallel, advances in terms of coupling overtly malignant PPGLs to the underlying molecular genetics have been made, but there is yet no combined risk stratification model based on histology and the overall mutational profile of the tumor. In this review, we systematically meta-analyzed previously reported cohorts using the PASS and GAPP algorithms and acknowledge a "rule-out" way of approaching these stratification models rather than a classical "rule-in" strategy. Moreover, the current genetic panorama regarding possible molecular adjunct markers for PPGL malignancy is reviewed. A combined histological and genetic approach will be needed to fully elucidate the malignant potential of these tumors.

PMID: 30769931 [PubMed]

[A Case of Gastric Neuroendocrine Carcinoma with Bulky Lymph Node Metastases, Solitary Liver Metastasis, and Left Adrenal Metastasis Curatively Resected after Chemotherapy].

Wed, 02/20/2019 - 15:54
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[A Case of Gastric Neuroendocrine Carcinoma with Bulky Lymph Node Metastases, Solitary Liver Metastasis, and Left Adrenal Metastasis Curatively Resected after Chemotherapy].

Gan To Kagaku Ryoho. 2019 Jan;46(1):103-105

Authors: Tsukamoto T, Togano S, Edagawa E, Kaizaki R, Hori T, Takatsuka S

Abstract
A 68-year-old man presented with generalfatigue due to anemia. Gastrointestinalendoscopy showed a tumor approximately 60mm in diameter in the lesser curvature of the angle of the stomach. Large cell neuroendocrine carcinoma was diagnosed by biopsy of a tumor specimen. Computed tomography revealed bulky regional lymph node metastases, solitary liver metastasis, and left adrenal metastasis. After three courses of combined chemotherapy with S-1 and CDDP, the gastric lesion and lymph node metastases shrunk, but the liver metastasis and left adrenal metastasis enlarged. After three courses of combined chemotherapy with ramucirumab and paclitaxel, the liver metastasis and left adrenal metastasis also shrunk, and no other new metastatic lesions appeared. Accordingly, total gastrectomy with lymph nodes dissection, partial resection of the liver and left adrenalectomy were performed for a curative resection. The histological findings revealed neuroendocrine carcinoma and adenocarcinoma of the stomach. The lesions of the liver and lymph nodes were all burned out, and a tiny metastatic lesion of neuroendocrine carcinoma was left in the left adrenal gland. S-1 was administrated for 3 months after surgery. The patient has been alive for 16months without recurrence after surgery.

PMID: 30765655 [PubMed - in process]

Maintaining a methods database to optimize solid tumor tissue culture: Review of a 15-year database from a single institution.

Wed, 02/20/2019 - 15:54
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Maintaining a methods database to optimize solid tumor tissue culture: Review of a 15-year database from a single institution.

Cancer Genet. 2019 Feb 02;:

Authors: Smith SC, Warren LM, Cooley LD

Abstract
Chromosome analysis of solid tumors provides valuable information for diagnosis and patient management, yet successfully culturing solid tumors can be challenging. The Children's Mercy (CM) Cytogenetics laboratory has compiled a database of 1371 non-lymphoma solid tumors cultured since 2002. Analysis of the tumor culture data found a culture success rate of 91.6%. Abnormal karyotypes were identified in 47.0% of these tumors. A quality improvement project reviewed the database for methods, cell culture success, yield of clonally abnormal karyotypes, culture failure, tumor diagnostic category, and other. This review revealed processes that could be optimized with minor changes to methods in a subset of tumors. Three tumor/method pair examples are provided including adrenal cortical carcinomas (ACCs), choroid plexus tumors (CPTs), and neuroblastoma. The successful culture of tumors as defined by capture of clonally abnormal cells is dependent upon several factors including culture medium, monolayer versus suspension culture, length of time in culture, method of disaggregation and other. The database serves as a quality assurance tool that enables continuous improvement in culture success rate and abnormal yield. It is also an educational resource for laboratory technologists, residents and fellows. Using the database to track methods and results ensures consistency in routine tumor processing, facilitates oversight to optimize methods for quality, and improves results for patient care.

PMID: 30765326 [PubMed - as supplied by publisher]

The Pediatric Surgical Team: a Model for Increased Surgeon Index Case Exposure.

Wed, 02/20/2019 - 15:54
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The Pediatric Surgical Team: a Model for Increased Surgeon Index Case Exposure.

J Pediatr Surg. 2019 Jan 21;:

Authors: Johnson SM, Lee WG, Puapong DP, Woo RK

Abstract
BACKGROUND/PURPOSE: High surgical volume for both surgeons and hospital systems has been linked to improved outcomes for many surgical problems, yet case volumes per pediatric surgeon are diminishing nationally in complex pediatric surgery. We therefore sought to review our experience in a geographically isolated setting where a surgical team approach has been used to improve per-surgeon exposure to index pediatric surgical cases.
METHODS: As a surgical group, we incorporated a surgical team approach to complex pediatric surgical cases in 2010. We obtained institutional review board approval to review our pediatric surgeon index case volume experience. We then compared our surgeon experience to published surgical volumes for complex pediatric surgical cases.
RESULTS: A surgical team approach (2 or 3 board certified pediatric surgeons/urologists working as co-surgeons or assistant surgeon) was used in the majority of cases for tracheoesophageal fistula/esophageal atresia (77%), congenital pulmonary airway malformation (73.5%), cloaca (75%), anorectal malformation (43.6%) biliary atresia (77.8%), Hirschsprung's disease (51.9%), congenital diaphragmatic hernia (67.6%), robotic choledochal cyst (100%), and complex oncology (adrenal tumors, neuroblastoma, Wilms tumor and Hepatoblastoma surgery) (85-100%). Over the 5-year period, surgeon index case exposure for all index pediatric surgical cases was above the published national median for pediatric surgeons, except for in splenic operations when contrasted to published experience.
CONCLUSIONS: A surgical team approach to complex pediatric surgery may help maintain exposure to adequate index case volumes. This model may be useful for maintaining competence in geographically-isolated practice settings and low-volume pediatric hospitals that provide surgical care; the model has implications for systems development and workforce allocation within pediatric surgery.
LEVEL OF EVIDENCE: 4.

PMID: 30765153 [PubMed - as supplied by publisher]

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