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    MANAGEMENT OF ENDOCRINE DISEASE: Adrenocortical carcinoma: differentiating the good from the poor prognosis tumors.
    Eur J Endocrinol 2018 Feb 23. Epub 2018 Feb 23.
    J Bertherat, Reference Center for rare adrenal diseases, Department of Endocrinology. Hôpitaux universitaires Paris-Centre, Assistance Publique - Hopitaux de Paris, Paris, France
    Adrenocortical carcinoma (ACC) is a rare malignancy with a poor prognosis, the five-years overall survival being below 40%. However there is great variability of outcomes and we have now a better view of the heterogeneity of tumor aggressiveness. The extent of the disease at the time of diagnosis, best assayed by the European Network for the Study of Adrenal Tumors (ENSAT) staging score, is a major determinant of survival. Read More

    The GRP78/BiP inhibitor HA15 synergizes with mitotane action against adrenocortical carcinoma cells through convergent activation of ER stress pathways.
    Mol Cell Endocrinol 2018 Feb 20. Epub 2018 Feb 20.
    Université Côte d'Azur, Valbonne, 06560, France; CNRS UMR 7275, Sophia Antipolis, Valbonne, 06560, France; NEOGENEX CNRS International Associated Laboratory, Valbonne, 06560, France; Institut de Pharmacologie Moléculaire et Cellulaire, Valbonne, 06560, France. Electronic address:
    Many types of cancer cells present constitutively activated ER stress pathways because of their significant burden of misfolded proteins coded by mutated and rearranged genes. Further increase of ER stress by pharmacological intervention may shift the balance towards cell death and can be exploited therapeutically. Recent studies have shown that an important component in the mechanism of action of mitotane, the only approved drug for the medical treatment of adrenocortical carcinoma (ACC), is represented by activation of ER stress through inhibition of the SOAT1 enzyme and accumulation of toxic lipids. Read More

    Yttrium-90 microsphere selective internal radiation therapy for liver metastases following systemic chemotherapy and surgical resection for metastatic adrenocortical carcinoma.
    World J Clin Oncol 2018 Feb;9(1):20-25
    Division of Vascular and Interventional Radiology, Department of Radiology, the Ohio State University Comprehensive Cancer Center, Columbus, OH 43210, United States.
    Adrenocortical carcinoma (ACC) is a rare malignancy with generally poor outcomes and limited treatment options. While surgical resection can be curative for early local disease, most patients present with advanced ACC owing to nonspecific symptoms. For those patients, treatment options include systemic chemotherapy and locoregional therapies including radiofrequency ablation and transarterial chemoembolization. Read More

    Percutaneous microwave ablation for local control of metastatic renal cell carcinoma.
    Abdom Radiol (NY) 2018 Feb 20. Epub 2018 Feb 20.
    Department of Radiology, University of Wisconsin School of Medicine and Public Health, 600 Highland Avenue, E3/376 Clinical Science Center, Madison, WI, 53792, USA.
    Purpose: The purpose of the article is to evaluate the safety and oncologic efficacy of microwave ablation for metastatic renal cell carcinoma (mRCC).

    Materials And Methods: From September 2011 to December 2016, 33 mRCC were ablated in 18 patients using percutaneous microwave ablation. Sites of mRCC include retroperitoneum (n = 12), contralateral kidney (n = 6), liver (n = 6), lung (n = 5), adrenal gland (n = 5). Read More

    Georgian Med News 2018 Jan(274):19-24
    Medical Center "MediClubGeorgia" Tbilisi; Pathology Research Center, Tbilisi, Georgia.
    Adrenocortical carcinoma is a very rare and aggressive endocrinological malignancy arising from the adrenal cortex. The estimated incidence is 1 per million people, with an estimated 5-year survival rate of 16-47%. It can be bilateral in roughly 2-10% of cases, but the data is scarce and there is no conclusive evidence whether the contralateral mass is an independent tumor or a metastasis from the other adrenal gland cancer. Read More

    Multiple endocrine neoplasia 2A (MEN 2A) syndrome.
    Bratisl Lek Listy 2018 ;119(2):120-125
    Introduction: In the MEN 2A syndrome, which is the most common of the three types of MEN, three endocrine systems are affected simultaneously or subsequently by the development of tumours manifested by medullary thyroid gland carcinoma, pheochromocytoma (often bilateral) and hyperparathyroidism.

    Material And Methods: 27 patients from 3 families affected by MEN 2A syndrome were examined clinically (by detecting the effects of catecholamine overproduction), biochemically (screening for metanephrine and normetanephrine in the serum), visualization (CT, MRI, MIBG, PET CT) and some of them also genetically (DNA fragment analysis obtained by PCR amplification).

    Results: Familial incidence of pheochromocytoma was confirmed in 10 patients (4 males, 5 females and one girl) aged 6 to 54 years (average 22. Read More

    Mitotane monotherapy in patients with advanced adrenocortical carcinoma.
    J Clin Endocrinol Metab 2018 Feb 14. Epub 2018 Feb 14.
    Dept. of Internal Medicine I, Division of Endocrinology and Diabetes, University Hospital Würzburg, University of Würzburg, Germany.
    Context: While mitotane is the only approved drug for the treatment of adrenocortical carcinoma (ACC), data on monotherapy in advanced disease is still scarce.

    Objective: To assess the efficacy of mitotane in advanced ACC in a contemporary setting and to identify predictive factors.

    Design/setting: Multicenter cohort study of three German referral centers. Read More

    Analysis of 10 Adrenocortical Carcinoma Patients in the Cohort of the Precision Medicine Platform MONDTI.
    Oncology 2018 Feb 14. Epub 2018 Feb 14.
    Division of Oncology, Department of Medicine I, Comprehensive Cancer Center, Medical University Vienna, Vienna, Austria.
    Objective: Adrenocortical carcinoma (ACC) is a rare disease with a dismal prognosis. We aimed to evaluate if a personalized medicine approach may be useful for matching patients with ACC to targeted therapies.

    Methods: This is an analysis of 10 molecularly profiled ACCs that were progressing under standard of care treatment. Read More

    Common module analysis reveals prospective targets and mechanisms of pediatric adrenocortical adenoma and carcinoma.
    Oncol Lett 2018 Mar 19;15(3):3267-3272. Epub 2017 Dec 19.
    Department of Applied Sciences, Indian Institute of Information Technology, Allahabad, Uttar Pradesh 211012, India.
    Pediatric adrenocortical carcinoma and adrenocortical adenoma are two rare diseases affecting children. Molecular analyses were performed to identify commonalities in gene expression between the diseases. Differentially expressed genes were identified for the pediatric adrenocortical adenoma and carcinoma tissues, as compared with normal tissues, using the expression dataset. Read More

    BCL9 Upregulation in Adrenocortical Carcinoma: A Novel Wnt/β-Catenin Activating Event Driving Adrenocortical Malignancy.
    J Am Coll Surg 2018 Feb 8. Epub 2018 Feb 8.
    Department of Surgery, Yale Endocrine Neoplasia Laboratory, Yale University School of Medicine, New Haven, CT. Electronic address:
    Background: B-Cell CLL/Lymphoma 9 (BCL9) is a recently described oncogene that promotes tumorigenesis via activation of the Wnt/β-Catenin signaling cascade. Though constitutively active Wnt/β-Catenin signaling is a molecular hallmark of adrenocortical carcinoma (ACC), a potential role for BCL9 to promote Wnt/β-Catenin pathway dysregulation in adrenocortical tumorigenesis remains to be elucidated.

    Study Design: This study involved a retrospective analysis at a tertiary academic referral center of 27 patients with adrenocortical tumors, including in vitro investigation of BCL9. Read More

    Targeting the multidrug transporter Patched potentiates chemotherapy efficiency on adrenocortical carcinoma in vitro and in vivo.
    Int J Cancer 2018 Feb 7. Epub 2018 Feb 7.
    Université Côte d'Azur, Sophia Antipolis, Valbonne, France.
    One of the crucial challenges in the clinical management of cancer is the resistance to chemotherapeutics. We recently demonstrated that the Hedgehog receptor Patched, which is overexpressed in many recurrent and metastatic cancers, is a multidrug transporter for chemotherapeutic agents such as doxorubicin. The present work provides evidences that Patched is expressed in adrenocortical carcinoma (ACC) patients, and is a major player of the doxorubicin efflux and the doxorubicin resistance in the human ACC cell line H295R. Read More

    Development of biocompatible and VEGF-targeted paclitaxel nanodrugs on albumin and graphene oxide dual-carrier for photothermal-triggered drug delivery in vitro and in vivo.
    Int J Nanomedicine 2018 17;13:439-453. Epub 2018 Jan 17.
    Department of Urinary Surgery, Dongying People's Hospital, Dongying.
    In this study, we performed the characterization and synthesis of biocompatible and targeted albumin and graphene oxide (GO) dual-carrier paclitaxel (PTX) nanoparticles for photothermal-triggered tumor therapy. PTX absorbed on GO nanosheets as cores were coated with human serum albumin (HSA), following surface conjugation with monoclonal antibodies (mAb) against vascular endothelial growth factor (VEGF; denoted as mAbVEGF) via polyethylene glycol linker to form targeted nanoparticles (PTX-GHP-VEGF). The spherical nanoparticles were 191±5 nm in size with good stability and biocompatibility. Read More

    Adrenocortical carcinoma characterized by gynecomastia: A case report.
    Clin Pediatr Endocrinol 2018 30;27(1):9-18. Epub 2018 Jan 30.
    Department of Pediatrics, Sapporo Medical University School of Medicine, Sapporo, Japan.
    We present a 4-yr-old boy with adrenocortical carcinoma (ACC), diagnosed due to the appearance of gynecomastia as the presenting symptom. Six months prior to admission, an acute growth spurt along with the development of bilateral breast swelling was observed. He did not present any features of virilization, including enlargement of the testes, increase in testis volume, and penis size. Read More

    An image-based small-molecule screen identifies vimentin as a pharmacologically relevant target of simvastatin in cancer cells.
    FASEB J 2018 Jan 18:fj201700663R. Epub 2018 Jan 18.
    Department of Cell Biology and Physiology, University of North Carolina-Chapel Hill, Chapel Hill, North Carolina, USA.
    Vimentin is a cytoskeletal intermediate filament protein that is expressed in mesenchymal cells and cancer cells during the epithelial-mesenchymal transition. The goal of this study was to identify vimentin-targeting small molecules by using the Tocriscreen library of 1120 biochemically active compounds. We monitored vimentin filament reorganization and bundling in adrenal carcinoma SW13 vimentin-positive (SW13-vim) cells via indirect immunofluorescence. Read More

    Tumor thrombus: incidence, imaging, prognosis and treatment.
    Cardiovasc Diagn Ther 2017 Dec;7(Suppl 3):S165-S177
    Division of Interventional Radiology, Department of Radiology, Mayo Clinic-Arizona, Phoenix, AZ, USA.
    Intravascular tumor extension, also known as tumor thrombus, can occur in many different types of cancer. Those with the highest proclivity include Wilm's tumor, renal cell carcinoma (RCC), adrenal cortical carcinoma (ACC) and hepatocellular carcinoma (HCC). The presence of tumor thrombus markedly worsens prognosis and impacts treatment approach. Read More

    Alveolar Soft Part Sarcoma Presenting as Hypervascular Adrenal Metastasis.
    World J Nucl Med 2018 Jan-Mar;17(1):62-64
    Department of Endocrinology, Seth G S Medical College and KEM Hospital, Mumbai, Maharashtra, India.
    Hypervascular adrenal masses include pheochromocytoma, metastases caused by clear renal cell carcinoma/hepatocellular carcinoma. Alveolar soft part sarcoma (ASPS) causing hypervascular metastases is not described in the literature. Here, we describe the first case of ASPS presenting as hypervascular metastasis. Read More

    [A comparison of clinical characteristics between 2 pedigrees of multiple endocrine neoplasia type 2A with different RET mutations].
    Zhonghua Nei Ke Za Zhi 2018 Feb;57(2):134-137
    Department of Endocrinology, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou 510120, China.
    Multiple endocrine neoplasia type 2A (MEN2A) is a hereditary syndrome. Here, two different RET proto-oncogen mutation were identified from family members of two MEN2A pedigrees by genetic screening. One RET mutations were found at codons 1893 and 1895 in exon 11 (1893-1895delCGA) from pedigree 1, which is a novel mutation, the other occurs at codon 634 (Cys634Arg) in exon 11 from pedigree 2. Read More

    [Four Cases of Gastrointestinal Perforation Associated with Bevacizumab].
    Gan To Kagaku Ryoho 2017 Nov;44(12):1604-1606
    Dept. of Surgery, Hokkaido Medical Center.
    Four cases of gastrointestinal perforation associated with bevacizumab(BEV)were examined. Case 1: A 82-year-old male received FOLFIRI plus BEV for recurrent liver metastasis after rectal cancer resection. A lower esophageal perforation occurred 22 days after BEV administration and drainage was performed. Read More

    [Esophagogastric Junction Cancer Resection with Bilateral Adrenal Metastasis - A Case Report].
    Gan To Kagaku Ryoho 2017 Nov;44(12):1443-1445
    Dept. of Surgery, Bellland General Hospital.
    The patient was a 64-year-old man with esophagogastric junction cancer. We performed right thoracotomy-laparotomy for lower esophageal and cardiac gastric resection, D2 lymphadenectomy, and reconstruction of a gastric tube in October 2011. Histopathology confirmed T4aN1M1(LYM), Stage IV cancer(Japanese Classification of Gastric Carcinoma, 14th edition) with R0 resection. Read More

    [An Enhanced Multimodality Treatment Strategy in Pancreatic Ductal Carcinoma Requiring Heminephrectomy for Radical Resection - A Case Report].
    Gan To Kagaku Ryoho 2017 Nov;44(12):1367-1369
    Dept. of Gastroenterological Surgery, Graduate School of Medicine, Osaka University.
    A 76-year-old male visited a clinic and the images incidentally revealed a tumor in the tail of pancreas. Pancreatic ductal adenocarcinoma was diagnosed by the following examination, indicating that the tumor invasion to spleen, left adrenal gland and left kidney(cT3N0M0, cStage II A), requiring heminephrectomy for radical resection. Enhanced preoperative treatments were performed while taking into account a possibility of any restrictions for the age and the standard adjuvant chemotherapies after heminephrectomy. Read More

    Chronic primary adrenal insufficiency after unilateral adrenonephrectomy: A case report.
    Medicine (Baltimore) 2017 Dec;96(51):e9091
    Department of Diabetes, Endocrinology and Nutrition, Kyoto University Graduate School of Medicine, Kyoto.
    Rationale: Unilateral adrenalectomy as part of surgical resection of renal cell carcinoma (RCC) is not thought to increase the risk of chronic adrenal insufficiency, as the contralateral adrenal gland is assumed to be capable of compensating for the lost function of the resected gland. However, recent studies have indicated that adrenalectomy might cause irreversible impairment of the adrenocortical reserve. We describe a case of chronic primary adrenal insufficiency in a 68-year-old man who previously underwent unilateral adrenonephrectomy, which was complicated by severe postoperative adrenal stress that involved cardiopulmonary disturbance and systemic infection. Read More

    Overexpression of HSD17B4 exerts tumor suppressive function in adrenocortical carcinoma and is not associated with hormone excess.
    Oncotarget 2017 Dec 1;8(70):114736-114745. Epub 2017 Dec 1.
    Department of Urology, Huashan Hospital, Fudan University, Shanghai 200040, PR China.
    Aim: Adrenocortical carcinoma (ACC) is characterized with excessive hormone production. We therefore investigated expression of hormone-related genes in ACC.

    Results: We queried status of 14 key genes directly involved in adrenal hormone production and found HSD17B4 expression was upregulated in 39% of ACC cases on top of all queried genes. Read More

    Characteristics of Adrenal Masses in Familial Adenomatous Polyposis.
    Dis Colon Rectum 2018 Jan 24. Epub 2018 Jan 24.
    Department of Surgery, University of Toronto, Toronto, Canada.
    Background: Adrenal masses are a known extraintestinal manifestation of familial adenomatous polyposis. However, the literature on this association is largely confined to case reports.

    Objective: This study aimed to determine the characteristics of adrenal masses in familial adenomatous polyposis and their clinical significance, as well as to estimate their prevalence. Read More

    Functional plurihormonal adrenal oncocytoma: case report and literature review.
    Clin Case Rep 2018 Jan 22;6(1):37-44. Epub 2017 Nov 22.
    Servicio de Endocrinología, Metabolismo y Medicina NuclearHospital Italiano de Buenos AiresBuenos AiresArgentina.
    We present a 27-year-old woman with an adrenal oncocytoma. This is a very rare entity. We provide a review of the clinical, biochemical and pathological features of cases reported in the literature. Read More

    Anti-hMC2RL1 Functionalized Gold Nanoparticles for Adrenocortical Tumor Cells Targeting and Imaging.
    J Biomed Nanotechnol 2017 Jan;13(1):68-76
    The low rate of cure of adrenocortical carcinomas (ACC) in children and adults is related to germ line TP53 mutation, late diagnosis, incomplete surgical resection, and lack of an efficient adjunctive therapy. To provide a new approach for the improvement of ACC diagnosis and therapy, the present study aimed to explicitly target ACC cells using gold nanoparticle (AuNP) probes bound to specific antibodies. Immunohistochemistry of ACC and positive and negative control tissue micro-sections under light microscopy was used to test a purified polyclonal antibody raised against the 80–93, outer loop 1 position of the human melanocortin receptor 2 (hMC2R). Read More

    Development of new preclinical models to advance adrenocortical carcinoma research.
    Endocr Relat Cancer 2018 Jan 25. Epub 2018 Jan 25.
    M Wierman, Medicine, University of Colorado Anchutz Medical Campus, Aurora, 80045, United States.
    Adrenocortical cancer (ACC) is an orphan malignancy that results in heterogeneous clinical phenotypes and molecular genotypes. There are no curative treatments for this deadly cancer with 35% survival at five years. Our understanding of the underlying pathobiology and our ability to test novel therapeutic targets has been limited due to the lack of preclinical models. Read More

    Delayed presentation of a virilising, pure testosterone-secreting adrenocortical carcinoma with coexistent composite myelolipoma and a venous thrombus extending to the heart.
    BMJ Case Rep 2018 Jan 23;2018. Epub 2018 Jan 23.
    Department of Pathology, Apollo Hospitals, Hyderabad, Andhra Pradesh, India.
    A 40-year-old normotensive woman presented with abnormal facial hair for 4 years and amenorrhoea for 13 years. Hormonal, biochemical and haematological evaluation showed isolated elevation of serum testosterone and free testosterone. Her follicle-stimulating hormone and luteinising hormone were in the premenopausal range. Read More

    [Three Cases of Advanced or Recurrent Adrenocortical Carcinoma Patients Treated with Etoposide, Doxorubicin and Cisplatin plus Mitotane].
    Gan To Kagaku Ryoho 2018 Jan;45(1):67-70
    You Home Clinic Heiwadai.
    Adrenocortical carcinoma is a rare cancer with poor clinical outcomes due to its propensity to transform or relapse. Reported here are 3 cases of adrenocortical carcinoma that were treated between January 2007a nd December 2013. Etoposide, doxorubicin, and cisplatin plus mitotane(EDP plus mitotane)were administered as adjuvant therapy to 1 patient, while the other patients received the therapy upon relapse of their cancers following surgery. Read More

    Anaplastic Thyroid Cancer With Extensive Skeletal Muscle Metastases on 18F-FDG PET/CT.
    Clin Nucl Med 2018 Jan 19. Epub 2018 Jan 19.
    From the Division of Nuclear Medicine and Molecular Imaging, Department of Radiology, Stanford University, Stanford, CA.
    A 61-year-old woman with newly diagnosed anaplastic thyroid cancer and known metastases to the brain, lungs, and adrenal glands complained of groin muscle pain. F-FDG PET/CT was performed to assess for extent of disease and showed extensive hypermetabolic lesions throughout the skeletal musculature concerning for metastatic disease. As this would be a very rare presentation for anaplastic thyroid carcinoma, a biopsy of the left gluteal muscle was conducted. Read More

    Microsatellite stability and mismatch repair proficiency in nasopharyngeal carcinoma may not predict programmed death-1 blockade resistance.
    Oncotarget 2017 Dec 5;8(68):113287-113293. Epub 2017 Dec 5.
    Department of Radiation Oncology, Xiamen Cancer Hospital, The First Affiliated Hospital of Xiamen University, Teaching Hospital of Fujian Medical University, Xiamen, China.
    The US FDA granted accelerated approval to pembrolizumab for microsatellite instability-high and mismatch repair deficient cancers. The response of programmed death-1 blockade in mismatch repair proficiency (pMMR) colorectal cancer is very poor, however, whether such treatment is effective in pMMR nasopharyngeal carcinoma (NPC) remains unknown. We report a case of a 51-year-old man with NPC. Read More

    Minimally invasive adrenal surgery: virtue or vice?
    Future Oncol 2018 Feb 18;14(3):267-276. Epub 2018 Jan 18.
    Department of Urology and Oncological Science, Icahn School of Medicine at Mount Sinai, 1 Gustave L Levy Place, New York, NY 10029-6574, USA.
    Adrenocortical carcinoma (ACC) is a rare malignancy associated with poor prognosis despite available treatments. In patients with localized or locally advanced disease, complete resection with negative margins offers the only potential for cure. Unfortunately, most patients develop local and distant recurrence following initial resection highlighting the importance of meticulous surgical technique in the hands of an experienced surgeon. Read More

    Causes, patterns and severity of androgen excess in 1205 consecutively recruited women.
    J Clin Endocrinol Metab 2018 Jan 12. Epub 2018 Jan 12.
    Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, UK.
    Context: Androgen excess in women is predominantly due to underlying polycystic ovary syndrome (PCOS). However there is a lack of clarity regarding patterns and severity of androgen excess that should be considered predictive of non-PCOS pathology.

    Objective: We examined the diagnostic utility of simultaneous measurement of serum dehydroepiandrosterone sulfate (DHEAS), androstenedione (A4) and testosterone (T) to delineate biochemical signatures and cut-offs predictive of non-PCOS disorders in women with androgen excess. Read More

    Immunohistochemical Biomarkers of Mesenchymal Neoplasms in Endocrine Organs: Diagnostic Pitfalls and Recent Discoveries.
    Endocr Pathol 2018 Jan 16. Epub 2018 Jan 16.
    Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, 75 Francis Street, Boston, MA, 02115, USA.
    Mesenchymal neoplasms rarely present in or adjacent to endocrine organs. In this context, the recognition of these rare tumor types can be challenging, with significant potential for misdiagnosis as sarcomatoid carcinomas (i.e. Read More

    Mitotane treatment in patients with metastatic testicular Leydig cell tumor associated with severe androgen excess.
    Eur J Endocrinol 2018 Mar 12;178(3):K21-K27. Epub 2018 Jan 12.
    Institute of Metabolism and Systems ResearchUniversity of Birmingham, Birmingham, UK
    Mitotane (o,p'DDD) is established in the adjuvant and advanced-stage treatment of adrenocortical carcinoma and counteracts both tumor growth and tumor-related steroid production. Both the adrenal glands and the gonads are steroidogenically active organs and share a common embryogenic origin. Here, we describe the effects of mitotane in two patients with metastatic Leydig cell tumor (LCT) of the testes and associated severe androgen excess (serum testosterone 93 and 88 nmol/L, respectively; male reference range 7-27 nmol/L). Read More

    The impact of patient-, disease-, and treatment-related factors on survival in patients with adrenocortical carcinoma.
    Can Urol Assoc J 2017 Dec 22. Epub 2017 Dec 22.
    Division of Urology, Department of Surgery; Western University, London, ON, Canada.
    Introduction: Adrenal cortical carcinoma (ACC) is a rare and aggressive endocrine tumour. Most present with advanced disease and have poor prognosis. Optimal treatment includes complete surgical resection. Read More

    Laparoscopic Versus Open Adrenalectomy for Localized/Locally Advanced Primary Adrenocortical Carcinoma (ENSAT I-III) in Adults: Is Margin-Free (R0) Resection the Key Surgical Factor that Dictates Outcome?-A Review of the Literature.
    J Laparoendosc Adv Surg Tech A 2018 Jan 10. Epub 2018 Jan 10.
    3 Second Department of Propaedeutic Surgery, Laikon General Hospital, University of Athens Medical School , Athens, Greece .
    Background: The aim of this study was to review the current literature on the role of laparoscopic adrenalectomy (LA) in the treatment of primary adrenocortical carcinoma (ACC; European Network for the Study of Adrenal Tumors [ENSAT] I-III) in adults.

    Materials And Methods: Nonrandomized controlled trials published between January 1999 and February 2017 were identified by searching the Pubmed, EMBASE, Cochrane Library, and Google Scholar databases. Primary and secondary endpoints included surgical and pathological parameters (patients age, tumor size, ENSAT stage, type of surgical approach, and period of follow-up), surgical outcomes (operative time, estimated blood loss, length of hospital stay, conversion rate to laparotomy, R0 resection, and surgical margin's status), and oncological outcomes (rate of recurrence, disease-free survival [DFS], and overall survival [OS] rates). Read More

    [Surgical strategies for non-metastatic adrenocortical carcinoma].
    Chirurg 2018 Jan 8. Epub 2018 Jan 8.
    Klinik für Radiologie, Campus Virchow-Klinikum, Charité - Universitätsmedizin Berlin, Berlin, Deutschland.
    Adrenocortical carcinomas (ACC) are rare but highly aggressive tumors. It is very difficult to differentiate small locally limited ACCs from benign adenomas. A spontaneous density >10 Hounsfield units in non-enhanced CT scan and a slow washout after contrast injection are suspicious of malignancy but with a low specificity. Read More

    Radiological features and metastatic patterns of thymic neuroendocrine tumours.
    Clin Radiol 2018 Jan 5. Epub 2018 Jan 5.
    Department of Radiology, Brigham and Women's Hospital and Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA. Electronic address:
    Aim: To investigate the clinical and image features of thymic neuroendocrine tumours (NETs), and characterise the radiological patterns of recurrence and metastasis on serial imaging studies.

    Materials And Methods: The study included 14 patients (11 males) with a histopathological diagnosis of thymic NETs (one typical carcinoid, eight atypical carcinoid, and five large cell neuroendocrine carcinoma). Preoperative images were assessed for features of primary tumours. Read More

    Pancreatic panniculitis: the "bright" side of the moon in solid cancer patients.
    BMC Gastroenterol 2018 Jan 4;18(1). Epub 2018 Jan 4.
    Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti, Università degli Studi di Milano, Unità Operativa di Dermatologia, I.R.C.C.S. Fondazione Cà Granda, Ospedale Maggiore Policlinico, via Pace 9, 20122, Milan, Italy.
    Background: Pancreatic panniculitis is a rare complication of pancreas disorders occurring in 0.3-3% of patients, most often accompanied by the pancreatic acinar carcinoma. It presents multiple, painful, deep, ill-defined, red-brown, migratory nodules and plaques of hard elastic consistency; often ulcerated and typically located on the lower proximal and distal extremities. Read More

    Analysis of circulating extracellular vesicle-associated microRNAs in cortisol-producing adrenocortical tumors.
    Endocrine 2018 Feb 3;59(2):280-287. Epub 2018 Jan 3.
    2nd Department of Medicine, Semmelweis University, 1088, Budapest, Szentkiralyi str. 46., Hungary.
    Purpose: Circulating microRNAs (miRNA) have been described in patients with adrenocortical tumors, but the expression of miRNAs in non-functioning and cortisol-producing tumors has not been yet compared. Therefore, the objective of this study was to evaluate the expression of plasma extracellular vesicle (EV)-associated microRNAs in patients with non-functioning adrenocortical adenoma (NFA), cortisol-producing adrenocortical adenoma (CPA) and cortisol-producing adrenocortical carcinoma (CP-ACC).

    Methods: Preoperative plasma EV samples of 13 NFAs, 13 CPAs and 9 CP-ACCs were subjected to extracellular vesicle isolation. Read More

    Identifying mitotane-induced mitochondria-associated membranes dysfunctions: metabolomic and lipidomic approaches.
    Oncotarget 2017 Dec 4;8(66):109924-109940. Epub 2017 Jul 4.
    INSERM UMR-S 1185, Le Kremlin-Bicêtre, France.
    Mitotane (o,p'DDD), the most effective drug in adrenocortical carcinoma, concentrates into the mitochondria and impacts mitochondrial functions. To address the molecular mechanisms of mitotane action and to identify its potential target, metabolomic and lipidomic approaches as well as imaging analyses were employed in human adrenocortical H295R cells allowing identification of Mitochondria-Associated Membranes dysfunction as a critical impact of mitotane. Study of intracellular energetic metabolites by NMR spectroscopy showed that mitotane significantly decreased aspartate while concomitantly increased glutamate content in a time- and concentration-dependent manner. Read More

    Paraneoplastic Neuromyelitis Optica Spectrum Disorder: A single center cohort description with two cases of histological validation.
    Mult Scler Relat Disord 2017 Dec 23;20:37-42. Epub 2017 Dec 23.
    Department of Medicine, Division of Neurology, University of British Columbia, Vancouver, Canada.
    Background: Paraneoplastic syndromes are remote effects of cancer caused by an autoimmune response triggered by tumor cells. Paraneoplastic Neuromyelitis Optica Spectrum Disorders (NMOSD) has been previously described, but the underlying mechanism for these rare cases is not well characterized. This paper presents a newly described case series of paraneoplastic NMOSD, including 2 new histological types of cancer and histological validation. Read More

    Oncocytic Adreno Cortical Tumors: Pathological Features of 16 Cases and Review of the Literature.
    J Environ Pathol Toxicol Oncol 2017 ;36(3):237-244
    Department of General Surgery, Ege University, Izmir, Turkey.
    Oncocytic neoplasms of the adrenal gland are extremely rare tumors. These tumors differ from their nononcocytic counterparts in some respects. The aim of this study was to review and discuss the clinical, histological, and immunohistochemical features of as well as the prognosis for these rare tumors. Read More

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