{"orpha":70573,"name":"Small cell lung cancer","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["SCLC"],"definition":"Small cell lung cancer (SCLC) is a highly aggressive malignant neoplasm, accounting for 10-15% of lung cancer cases, characterized by rapid growth, and early metastasis. SCLC usually manifests as a large hilar mass with bulky mediastinal lymphadenopathy presenting clinically with chest pain, persistent cough, dyspnea, wheezing, hoarseness, hemoptysis, loss of appetite, weight loss, and neurological and endocrine paraneoplastic syndromes. SCLC is primarily reported in elderly people with a history of long-term tobacco exposure.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-70573/","orphanet_url":"https://www.orpha.net/en/disease/detail/70573","names":{"fr":{"name":"Cancer du poumon à petites cellules","definition":"Tumeur maligne très agressive rare représentant entre 10 et 15 % des cas de cancer du poumon, caractérisée par une croissance rapide et des métastases précoces. Le cancer du poumon à petites cellules (CPPC) se manifeste généralement par une masse hilaire importante avec une lymphadénopathie médiastinale volumineuse se traduisant sur le plan clinique par des douleurs thoraciques, une toux persistante, une dyspnée, une respiration sifflante, un enrouement, une hémoptysie, une perte d'appétit, une perte de poids et des syndromes paranéoplasiques affectant le système nerveux et endocrinien. Le CPPC est principalement rapporté chez les personnes âgées ayant des antécédents d'exposition prolongée au tabac."},"de":{"name":"Bronchialkarzinom, kleinzelliges","synonyms":["Lungenkrebs, kleinzelliger","SCLC"]},"es":{"name":"Cáncer de pulmón de células pequeñas","synonyms":["CPCP","Cáncer de pulmón microcítico"],"definition":"Es una neoplasia maligna muy agresiva que representa el 10-15% de los casos de cáncer de pulmón caracterizada por un crecimiento rápido y metástasis temprana. Este tipo de cáncer se manifiesta generalmente como una gran masa hiliar con linfadenopatías mediastínicas voluminosas que se presenta clínicamente con dolor torácico, tos persistente, disnea, sibilancias, ronquera, hemoptisis, pérdida de apetito y de peso y síndromes paraneoplásicos neurológicos y endocrinos. Esta neoplasia se ha descrito principalmente en personas de edad avanzada con antecedentes de larga exposición al tabaco."},"it":{"name":"Cancro del polmone a piccole cellule","synonyms":["SCLC"],"definition":"Il cancro del polmone a piccole cellule (SCLC) è una neoplasia maligna molto aggressiva, che rappresenta il 10-15% dei casi di tumore polmonare; è caratterizzata da crescita rapida e metastasi precoci. Di solito esordisce con una massa ilare di ampie dimensioni associata a notevole linfoadenopatia del mediastino; i segni clinici comprendono il dolore toracico, la tosse persistente, la dispnea, l'affanno, la raucedine, l'emottisi, la perdita di appetito e di peso e le sindromi neurologiche ed endocrine paraneoplastiche. Il cancro polmonare a piccole cellule si manifesta soprattutto nei soggetti anziani con una storia di esposizione a lungo termine al tabacco."},"nl":{"name":"Kleincellige longkanker","synonyms":["SCLC"],"definition":"Kleincellige longkanker (SCLC) is een zeer agressief, maligne neoplasma dat 10-15% van de gevallen van longkanker vertegenwoordigt, en wordt gekarakteriseerd door snelle groei en vroege metastase. SCLC manifesteert zich meestal als een grote hilaire massa met omvangrijke mediastinale lymfadenopathie en uit zich klinisch met pijn in de borst, aanhoudende hoest, dyspneu, piepende ademhaling, heesheid, hemoptoë, verlies van eetlust, gewichtsverlies, en neurologische en endocriene paraneoplastische syndromen. SCLC wordt voornamelijk gerapporteerd bij oudere mensen met een voorgeschiedenis van langdurige blootstelling aan tabak."},"pt":{"name":"Cancro pulmonar de células pequenas","synonyms":["CPCP"]},"pl":{"name":"Drobnokomórkowy rak płuc","synonyms":["SCLC"]},"cs":{"name":"Malobuněčný karcinom plic","synonyms":["SCLC"]},"uk":{"name":"Дрібноклітинний рак легенів","synonyms":["SCLC"],"definition":"Small cell lung cancer (SCLC) is a highly aggressive malignant neoplasm, accounting for 10-15% of lung cancer cases, characterized by rapid growth, and early metastasis. SCLC usually manifests as a large hilar mass with bulky mediastinal lymphadenopathy presenting clinically with chest pain, persistent cough, dyspnea, wheezing, hoarseness, hemoptysis, loss of appetite, weight loss, and neurological and endocrine paraneoplastic syndromes. SCLC is primarily reported in elderly people with a history of long-term tobacco exposure."},"zh":{"name":"小细胞肺癌","synonyms":["SCLC"]},"tr":{"name":"Küçük hücreli akciğer kanseri","synonyms":["SCLC"]}},"xrefs":[{"source":"OMIM","code":"182280","qualifier":"E","icd_relation":null,"validated":true},{"source":"ICD-10","code":"C34.9","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2C25.1","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1800431439"},{"source":"MONDO","code":"0008433","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0149925","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"9344","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10041067","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":101945,"name":"Rare bronchopulmonary and pleural cavity tumors","classifications":[184,202]}],"children":[],"genes":[{"symbol":"RB1","name":"RB transcriptional corepressor 1","gene_type":"gene with protein product","association":"Disease-causing somatic mutation(s) in","status":"Assessed","source":"26168399[PMID]","hgnc":"9884","ensembl":"ENSG00000139687","omim":"614041","loci":["13q14.2"]},{"symbol":"TP53","name":"tumor protein p53","gene_type":"gene with protein product","association":"Disease-causing somatic mutation(s) in","status":"Assessed","source":"26168399[PMID]","hgnc":"11998","ensembl":"ENSG00000141510","omim":"191170","loci":["17p13.1"]},{"symbol":"TP73","name":"tumor protein p73","gene_type":"gene with protein product","association":"Disease-causing somatic mutation(s) in","status":"Assessed","source":"26168399[PMID]","hgnc":"12003","ensembl":"ENSG00000078900","omim":"601990","loci":["1p36.32"]}],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-5 / 10 000","value":12.0,"area":"Europe","status":"Validated","source":"European Medicines Agency 2018[OTHER]"}],"phenotypes":null}