{"orpha":97285,"name":"Thyroid lymphoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare primary organ-specific lymphoma characterized by primary origin in the thyroid gland, sometimes involving cervical lymph nodes, and infrequently more distant sites. Diffuse large B-cell lymphoma is most common, followed by MALT lymphoma, and follicular lymphoma. More rare types include T-cell lymphomas, Burkitt lymphoma, or classic Hodgkin lymphoma. The condition is usually associated with Hashimoto thyroiditis. Patients typically present with a mass in the thyroid, with or without cervical lymphadenopathy. Hoarseness and dyspnea may occur, while constitutional symptoms are rare. Prognosis is favorable for patients with localized tumors.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-97285/","orphanet_url":"https://www.orpha.net/en/disease/detail/97285","names":{"fr":{"name":"Lymphome thyroïdien","definition":"Lymphome primitif prenant spécifiquement naissance dans la glande thyroïde, affectant parfois les ganglions lymphatiques cervicaux et, rarement, un site plus distal. Le lymphome diffus à grandes cellules B est le plus fréquent, suivi du lymphome MALT et du lymphome folliculaire. Les types plus rares sont les lymphomes à cellules T, le lymphome de Burkitt ou le lymphome de Hodgkin classique. La maladie est généralement associée à la thyroïdite de Hashimoto. Les patients présentent généralement une masse dans la thyroïde, avec ou sans lymphadénopathie cervicale. On peut observer un enrouement et une dyspnée, tandis que les symptômes constitutionnels sont rares. Le pronostic est favorable pour les patients dont les tumeurs sont localisées."},"de":{"name":"Schilddrüsenlymphom","definition":"Ein primäres organspezifisches Lymphom, das seinen Ursprung in der Schilddrüse hat, manchmal mit Beteiligung der Halslymphknoten, seltener auch an weiter entfernten Stellen. Am häufigsten ist das diffuse großzellige B-Zell-Lymphom, gefolgt vom MALT-Lymphom und dem follikulären Lymphom. Zu den selteneren Formen gehören T-Zell-Lymphome, das Burkitt-Lymphom oder das klassische Hodgkin-Lymphom. Die Erkrankung ist in der Regel mit einer Hashimoto-Thyreoiditis verbunden. Die Patienten stellen sich typischerweise mit einer Gewebeverdichtung in der Schilddrüse vor, mit oder ohne zervikale Lymphadenopathie. Heiserkeit und Dyspnoe können auftreten, während konstitutionelle Symptome selten sind. Die Prognose ist für Patienten mit lokalisierten Tumoren günstig."},"es":{"name":"Linfoma tiroideo","definition":"Es un linfoma órgano específico primario poco frecuente caracterizado por un origen primario en la glándula tiroides, que afecta, en ocasiones, a los ganglios linfáticos cervicales y, con poca frecuencia, sitios más distantes. El linfoma difuso de células B grandes es el más común, seguido del linfoma MALT y el linfoma folicular. Los tipos menos frecuentes incluyen los linfomas de células T, linfoma de Burkitt o linfoma de Hodgkin clásico. Por lo general, el trastorno está asociado a tiroiditis de Hashimoto. Los pacientes suelen presentarse con una masa tiroidea, con o sin linfadenopatía cervical. Puede asociar ronquera y disnea, mientras que los síntomas constitucionales son poco frecuentes. El pronóstico es favorable para pacientes con tumores localizados."},"it":{"name":"Linfoma tiroideo","definition":"Si tratta di un linfoma raro, organo-specifico primitivo, che origina dalla tiroide, occasionalmente interessando i linfonodi cervicali e, raramente, le aree più distanti. Il linfoma diffuso a grandi cellule B è il più comune, seguito dal linfoma MALT e dal linfoma follicolare. Sono più rari i linfomi a cellule T, il linfoma di Burkitt e il linfoma di Hodgkin classico. In genere la malattia si associa alla tiroidite di Hashimoto. Di solito i pazienti presentano una massa localizzata nella tiroide, con o senza linfoadenopatia cervicale. Può associarsi a raucedine e dispnea, mentre i sintomi costituzionali sono rari. La prognosi è favorevole per i pazienti con tumori localizzati."},"nl":{"name":"Schildklierlymfoom","definition":"Een zeldzaam primair orgaanspecifiek lymfoom, gekarakteriseerd door een primaire oorsprong in schildklier, soms met betrokkenheid van cervicale lymfeklieren, en zelden verder verwijderde plaatsen. Diffuus grootcellig B-cellymfoom komt het meest voor, gevolgd door MALT-lymfoom en folliculair lymfoom. Zeldzamere types zijn onder meer T-cellymfomen, Burkitt-lymfoom, of klassiek Hodgkinlymfoom. De aandoening is meestal geassocieerd met Hashimoto-thyreoïditis. Patiënten presenteren zich doorgaans met een massa in de schildklier, met of zonder cervicale lymfadenopathie. Heesheid en dyspneu kunnen voorkomen, terwijl constitutionele symptomen zeldzaam zijn. De prognose is gunstig voor patiënten met gelokaliseerde tumoren."},"pt":{"name":"Linfoma da tiroide"},"pl":{"name":"Chłoniak tarczycy"},"cs":{"name":"Lymfom štítné žlázy"},"uk":{"name":"Лімфома щитовидної залози","definition":"A rare primary organ-specific lymphoma characterized by primary origin in the thyroid gland, sometimes involving cervical lymph nodes, and infrequently more distant sites. Diffuse large B-cell lymphoma is most common, followed by MALT lymphoma, and follicular lymphoma. More rare types include T-cell lymphomas, Burkitt lymphoma, or classic Hodgkin lymphoma. The condition is usually associated with Hashimoto thyroiditis. Patients typically present with a mass in the thyroid, with or without cervical lymphadenopathy. Hoarseness and dyspnea may occur, while constitutional symptoms are rare. Prognosis is favorable for patients with localized tumors."},"zh":{"name":"甲状腺淋巴瘤"},"tr":{"name":"Tiroid lenfoması"}},"xrefs":[{"source":"ICD-10","code":"C85.7","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2B33.5","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"571063415"},{"source":"MONDO","code":"0019962","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1336753","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":279911,"name":"Primary organ-specific lymphoma","classifications":[194,202,233]},{"orpha":100087,"name":"Rare thyroid tumor","classifications":[193,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["No data available"],"prevalence":[{"type":"Annual incidence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"23714679[PMID]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-06-08","source":"23714679[PMID]_24077086[PMID]_20497878[PMID]_12417514[PMID]","terms":[{"hpo":"HP:0000853","label":"Goiter","frequency":"Obligate (100%)","criteria":null},{"hpo":"HP:0002665","label":"Lymphoma","frequency":"Obligate (100%)","criteria":null},{"hpo":"HP:0000475","label":"Broad neck","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002015","label":"Dysphagia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002094","label":"Dyspnea","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000872","label":"Hashimoto thyroiditis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001609","label":"Hoarse voice","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000821","label":"Hypothyroidism","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002716","label":"Lymphadenopathy","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0010307","label":"Stridor","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002781","label":"Upper airway obstruction","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000836","label":"Hyperthyroidism","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012531","label":"Pain","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002098","label":"Respiratory distress","frequency":"Very rare (<4-1%)","criteria":null}]}}