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Les adénocarcinomes cort ico-surréna liens (ACC) sont des tumeurs rares classiquement d'évolution très péjorative. Il semble toutefois que leur pronostic s'est amélioré au cours des dernières années, probablement grâce à un diagnostic plus précoce et aux progrès réalisés dans les traitements chirurgicaux et chimiothérapeutiques de ce cancer. Objectifs : Le but principal de cette étude rétrospective a été d'étudier les caractéristiques cliniques et biologiques de I'ACC au diagnostic ainsi que le traitement et d'identifier les facteurs pronostiques. Patients et méthodes : Cette étude est une analyse rétrospective d'une cohorte de 45 patients atteints d'un ACC diagnostiqué entre 1990 et 2012 en provenance d'un seul centre. Résultats : Le sex ratio (femme/ homme) était de 1,6 et l'âge moyen au diagnostic était de 51± 15 ans (intervalle : 18-81 ans). Vingt-et-un patients (48%) présentaient une tumeur sécrétante (cortisol et androgènes (12%), cortisol seul (18%), androgènes seuls (18%)) et 10 patients (24%) présentaient des métastases au diagnostic. L'ACC a été découvert fortuitement chez 18% des patients. Le diamètre maximal tumoral moyen objectivé au CT-scanner était de 10,2 cm. Le taux de survie globa le à 5 ans était de 50%. Quarante-quatre pourcent des patients ne présenta ient toujours pas de récidive à 5 ans et 41% n'ont pas présenté de progression endéans ce suivi. En analyse multi-variée,une moins bonne survie globale était principalement associée au diamètre tumora l (HR=l,161 par cm; P=0,001) et à la présence d'une sécrétion hormona le par I'ACC (HR=3,212 ;P=0,011), alors que l'utilisation de mitotane améliorait significativement le pronostic vital (HR=0,216 ; P=0,002). La survenue d'une récidive après rémission initiale n'était significativement influencée que par la nature sécrétante ou non de la tumeur (HR= 4,920 ; P=0,006) alors que l'hypersécrétion hormona le au diagnostic (HR=4,548 ; P=0,005) et la présence de métastases au diagnostic (HR=3,503 ;P=0,038) prédisaient indépendamment la progression tumorale.Conclusions : L'ACC reste une tµmeur rare, prédominante chez les femmes d'âge moyen et dont le pronostic reste mauvais malgré l'utilisation des traitements actuels disponibles. Le facteur péjoratif principal associé à un risque accru de mortalité, de récidive ou de progression est la nature sécrétante de la tumeur. De plus, le diamètre tumoral et l'utilisation de mitotane influencent la survie alors que la présence de métastases au diagnostic est associée à un risque de progression tumorale. Adrenocortical carcinomas (ACC) are rare tumors with a poor prognosis.However, their prognosis is improving, probably due to an earlier diagnosis and progress realized in the therapeutic management of this cancer.Objectives: The principal aims of this retrospective study were to evaluate the clinical and biological characteristics of ACC at diagnosis, its treatment and to identify prognostic factors.Design and methods: This study was a retrospective study of a cohort of 45 patients with ACC diagnosed between 1990 and 2012,and from a single academic center.Results: The sex ratio (female vs. male) was 1,6. Mean age at diagnosis was 51± 15 yr (range: 18-81 yr). We found that 22 patients (48%) had hypersecreting tumors (cortisol and androgens (12%), cortisol alone (18%) or androgens alone (18%)) and 10 patients (24%) had metastases at diagnosis. ACC was discovered incidentally in 18% of the cases. The mean tumor diameter at imaging was 10,2 cm. At 5 years, the overall survival rate was 50%, progression free survival was 44% and recurrence free survival was 41%. ln multivariate analysis, a poorer survival was associated with a great tumor diameter (HR=l,161 per cm; P=0,001) and with the presence of hormonal hypersecretion(HR=3,212;P=0,011),whereas the use of mitotane was increasing the vital prognosis (HR=0,216; P=0,002).Recurrence after initial remission was only influenced by the secretion character of the tumor (HR=4,920;P= 0,006) while hormonal hypersecretion (HR=4,548;P=0,005) and the presence of metastases at diagnosis (HR=3,503;P=0,038) predicted tumor progression.Conclusions: ACC is a rare tumor with female predominance and poor prognosis despite the use of currently available treatments. The most important factor determining overall, progression-free and recurrence-free survivals is the presence of hormonal hypersecretion at diagnosis. Moreover, tumor diameter and the use of mitotane influence overall survival, while the presence of metastases at time of diagnosis also predicts progression.

Adenocarcinoma --- Adrenocortical Carcinoma

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This first edition of the textbook “Adrenocortical Carcinoma: Basic Sciences and Clinical Concepts” provides a concise compilation of relevant data advancing our current understanding of this rare cancer. Chapters are dedicated to various aspects of both the basic biology and the clinical science relating to adrenocortical carcinoma (ACC). Expert clinicians introduce the reader to current state-of-the-art clinical care of patients with ACC. As a considerable body of knowledge on ACC has accumulated over the last decades, expert scientists have authored additional sections that serve as a reference source for the experienced researcher as well as an introduction for the trainee in the laboratory or clinic. Clinical topics span the epidemiology, diagnosis and therapy of ACC. Scientific topics areas span the pathology and pathophysiology of ACC with a special focus on experimental models of adrenocortical tumors and recent advances in the genetic aspects and signaling pathways in ACC. The content of this book was compiled and edited with the intent of facilitating the translation of knowledge into future diagnostic and therapeutic tools that will ultimately improve patient care.

Endocrine Gland Neoplasms. --- Endocrine glands -- Cancer. --- Adrenal Cortex Neoplasms --- Adenocarcinoma --- Adrenal Cortex Diseases --- Carcinoma --- Adrenal Gland Neoplasms --- Adrenocortical Carcinoma --- Adrenal Gland Diseases --- Neoplasms, Glandular and Epithelial --- Endocrine Gland Neoplasms --- Neoplasms by Site --- Endocrine System Diseases --- Neoplasms by Histologic Type --- Diseases --- Neoplasms --- Medicine --- Health & Biological Sciences --- Oncology --- Adrenal glands --- Diseases. --- Tumors. --- Medicine. --- Cancer research. --- Pharmacology. --- Biomedicine. --- Cancer Research. --- Pharmacology/Toxicology. --- Oncology. --- Toxicology. --- Chemicals --- Pharmacology --- Poisoning --- Poisons --- Tumors --- Toxicology --- Drug effects --- Medical pharmacology --- Medical sciences --- Chemotherapy --- Drugs --- Pharmacy --- Cancer research --- Physiological effect

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MicroRNAs are the best representatives of the non-coding part of the genome and their functions are mostly linked to their target genes. During the process of carcinogenesis, both dysregulation of microRNAs and their target genes can explain the development of the disease. However, most of the target genes of microRNAs have not yet been elucidated. In this book, we add new information related to the functions of microRNAs in various tumors and their associated targetome.

Medicine --- Oncology --- miR526b --- miR655 --- breast cancer --- angiogenesis --- lymphangiogenesis --- EP4 --- PI3K/Akt --- microRNA-361 --- EMT --- tumor microenvironment --- cancer diagnosis --- cancer treatment --- Bladder cancer --- microRNA --- genetic marker --- progression --- ccRCC --- prognostic biomarker --- miRNA --- transcription factor --- interplay --- microRNAs --- exosomes --- liquid biopsy --- metastasis --- cancer --- liquid biopsies --- tumor --- SNAIL (SNAI1) transcription factor --- epithelial to mesenchymal transition (EMT) --- long non-coding RNAs (lncRNAs) --- circular RNAs --- viral miRNAs --- EBV --- HHV-8 --- HPV --- HCV --- HBV --- MCPyV --- glioblastoma --- MGMT --- survival --- radiotherapy --- chemotherapy --- temozolomide --- translational medicine --- oncomiRNA --- post-transcriptional regulation --- immune regulation --- adrenocortical carcinoma --- micro RNA --- non-coding RNA --- thyroid carcinoma --- radioactive iodine --- drug resistance --- prognosis --- Burkitt lymphoma --- miR-378a-3p --- cell growth --- pancreatic cancer --- radioresistance --- personalized medicine --- biomarker --- target --- miR526b --- miR655 --- breast cancer --- angiogenesis --- lymphangiogenesis --- EP4 --- PI3K/Akt --- microRNA-361 --- EMT --- tumor microenvironment --- cancer diagnosis --- cancer treatment --- Bladder cancer --- microRNA --- genetic marker --- progression --- ccRCC --- prognostic biomarker --- miRNA --- transcription factor --- interplay --- microRNAs --- exosomes --- liquid biopsy --- metastasis --- cancer --- liquid biopsies --- tumor --- SNAIL (SNAI1) transcription factor --- epithelial to mesenchymal transition (EMT) --- long non-coding RNAs (lncRNAs) --- circular RNAs --- viral miRNAs --- EBV --- HHV-8 --- HPV --- HCV --- HBV --- MCPyV --- glioblastoma --- MGMT --- survival --- radiotherapy --- chemotherapy --- temozolomide --- translational medicine --- oncomiRNA --- post-transcriptional regulation --- immune regulation --- adrenocortical carcinoma --- micro RNA --- non-coding RNA --- thyroid carcinoma --- radioactive iodine --- drug resistance --- prognosis --- Burkitt lymphoma --- miR-378a-3p --- cell growth --- pancreatic cancer --- radioresistance --- personalized medicine --- biomarker --- target

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This book includes 17 papers published in the Special Issue/Article Collection “New Insights in the Genetics and Genomics of adrenocortical tumors and pheochromocytomas” including an editorial, 10 research papers and six review articles. Adrenal tumors represent a hot topic in contemporary endocrine oncology. Significant advancements in the genetics of genomics of these tumors have been made in recent years, and these articles give a useful and comprehensive overview of these issues. Questions regarding molecular pathogenesis, diagnosis (biomarkers) and even treatment are discussed in the papers written by international leaders of the field. Manuscripts are focused on three main topics: i. primary aldosteronism (the most common cause of secondary endocrine hypertension), ii. adrenocortical cancer and iii. pheochromocytoma/paraganglioma, which are the tumors with the highest heritability in humans. The book is edited by Prof. Peter Igaz (Department of Endocrinology, Faculty of Medicine, Semmelweis University).

Medicine --- pheochromocytoma --- paraganglioma --- genetics --- non-coding RNA --- malignancy --- biomarker --- treatment --- adaptive metabolism --- adrenal gland --- conn adenoma --- fatty acid metabolism --- ferroptosis --- hyperaldosteronism --- metabolic reprogramming --- β-oxidation --- PPARα --- tumor microenvironment --- Next Generation Sequencing --- hereditary cancer --- endocrine tumor syndrome --- KCNJ5 somatic mutation --- pulse wave velocity --- aldosterone-producing adenoma --- adrenalectomy --- propensity score matching --- arterial stiffness --- pheochromocytomas --- paragangliomas --- mutations --- susceptibility genes --- driver mutations --- hereditary --- germline --- somatic --- environment --- variants --- tumor suppressor genes --- metastatic --- RNAseq --- next generation sequencing --- adrenocortical carcinoma --- in silico analysis --- machine learning --- bioinformatic clustering --- biomarker prediction --- aldosterone producing adenoma --- ATP2B3 --- K416-F418delinsN mutation --- primary aldosteronism --- PPGL --- telomeres --- TERT --- ATRX --- NOP10 --- prognostic biomarker --- ALT --- phaeochromocytoma --- cancer --- mitochondrial complex II --- zebrafish --- therapy --- drug discovery --- redox balance pathway --- Vitamin C --- mitotane --- H295 strains --- microRNA --- aldosterone --- circulating --- adrenocortical --- transcriptomics --- epigenetics --- metabolomics --- epidemiology --- genetic analysis --- mortality --- surveillance --- TP53 R337H --- genetic testing --- adrenocortical tumor --- neonatal screening --- screening --- adenoma --- adrenal --- tissue --- artificial intelligence --- neural network --- pheochromocytoma --- paraganglioma --- genetics --- non-coding RNA --- malignancy --- biomarker --- treatment --- adaptive metabolism --- adrenal gland --- conn adenoma --- fatty acid metabolism --- ferroptosis --- hyperaldosteronism --- metabolic reprogramming --- β-oxidation --- PPARα --- tumor microenvironment --- Next Generation Sequencing --- hereditary cancer --- endocrine tumor syndrome --- KCNJ5 somatic mutation --- pulse wave velocity --- aldosterone-producing adenoma --- adrenalectomy --- propensity score matching --- arterial stiffness --- pheochromocytomas --- paragangliomas --- mutations --- susceptibility genes --- driver mutations --- hereditary --- germline --- somatic --- environment --- variants --- tumor suppressor genes --- metastatic --- RNAseq --- next generation sequencing --- adrenocortical carcinoma --- in silico analysis --- machine learning --- bioinformatic clustering --- biomarker prediction --- aldosterone producing adenoma --- ATP2B3 --- K416-F418delinsN mutation --- primary aldosteronism --- PPGL --- telomeres --- TERT --- ATRX --- NOP10 --- prognostic biomarker --- ALT --- phaeochromocytoma --- cancer --- mitochondrial complex II --- zebrafish --- therapy --- drug discovery --- redox balance pathway --- Vitamin C --- mitotane --- H295 strains --- microRNA --- aldosterone --- circulating --- adrenocortical --- transcriptomics --- epigenetics --- metabolomics --- epidemiology --- genetic analysis --- mortality --- surveillance --- TP53 R337H --- genetic testing --- adrenocortical tumor --- neonatal screening --- screening --- adenoma --- adrenal --- tissue --- artificial intelligence --- neural network