{"Name":"Plasmacytoma","DiseaseID__c":"GARD:0013446","id":13446,"encodedName":"plasmacytoma","IsDeleted":false,"Disease_Name_Full__c":"Plasmacytoma","Xref_IDs__c":"1187107005; 415112005; C0032131; C9349; D010954; DOID:3721; HP:0011857; MEDGEN:18506; MONDO:0005615; ORPHA:86855","USA_Estimate__c":"5,000","No_of_Specialist_Tagsa__c":3,"No_of_ClinGen_records__c":0,"No_of_GeneReviews__c":0,"No_of_HHS_records__c":0,"World_Estimate__c":"8,000 to 80,000","No_of_HRSA_records__c":0,"Evidence_Based_Score__c":0,"No_of_Disease_Descriptions__c":3,"Disease_Characteristics_Score__c":5,"No_of_Age_at_Onset__c":1,"Description_Source__c":"MONDO:0005615","Disease_Description__c":"Plasmacytoma is a localized mass of neoplastic monoclonal plasma cells that represents approximately 5% of all plasma cell neoplasms. There are two separate entities: primary plasmacytoma of the bone and extramedullary plasmacytoma of the soft tissues. Of the extramedullary plasmacytomas, 80% occur in the head and neck, usually in the upper respiratory tract. The median age at diagnosis is 50 years and the male to female ratio is 3:1. Long-term survival is possible following local radiotherapy, particularly for soft tissue presentations.","GARD_Name__c":"Plasmacytoma","GARD_Synonym__c":"plasmacytoma - disorder; solitary myeloma; solitary plasmacytoma","Curated_Disease_Description_Source__c":"PlainLanguagePilotV1-Sep23","Curated_Disease_Description__c":"Plasmacytoma is a type of cancer that begins in plasma cells (white blood cells that produce antibodies). It presents as a localized mass of abnormal plasma cells and makes up about 5% of all plasma cell cancers. There are two types of plasmacytoma: primary plasmacytoma of the bone and extramedullary plasmacytoma of the soft tissues.\nExtramedullary plasmacytomas usually occur in the head and neck, especially in the upper respiratory tract. They are more common in men than women, and are usually diagnosed around age 50.","Curated_USA_Estimate_Source__c":null,"Curated_USA_Estimate__c":"5,000","Age_at_Onset_Snippet_Text__c":"as an Adult","SourceID__c":"ORPHA:86855","Deprecated__c":"No","Disease_Concept_Type__c":"Rare Disease Grouping","MONDO_ID__c":"MONDO:0005615","ORPHANET_ID__c":"ORPHA:86855","Replaced_By_ID__c":null,"Display_Spanish_Disease_Name__c":"Plasmacitoma","Spanish_Description_Source__c":"ORPHA:86855","Spanish_Description__c":"Es un trastorno caracterizado por una masa localizada de células plasmáticas monoclonales neoplásicas que representa aproximadamente el 5% de todas las neoplasias de células plasmáticas. Comprende dos entidades diferenciadas: el plasmocitoma óseo primario y el plasmocitoma extramedular de tejidos blandos. Aproximadamente el 80% de los plasmocitomas extramedulares ocurre en la cabeza y el cuello, generalmente en el tracto respiratorio superior. La mediana de edad al momento del diagnóstico es de 50 años y el ratio hombre:mujer es de 3:1. La supervivencia a largo plazo es posible después de la radioterapia local, en particular para presentaciones en tejidos blandos.","Spanish_Disease_Name__c":"plasmacitoma","Spanish_GARD_Synonym__c":"plasmacitoma solitario","Category_Linearization__c":"ORPHA:250908","icd10_id__c":null,"mesh_id__c":null,"omim_id__c":null,"snomed_id__c":null,"umls_id__c":null,"GARD_Disease__c":[{"Curated_Disease_Description__c":"Plasmacytoma is a type of cancer that begins in plasma cells (white blood cells that produce antibodies). It presents as a localized mass of abnormal plasma cells and makes up about 5% of all plasma cell cancers. There are two types of plasmacytoma: primary plasmacytoma of the bone and extramedullary plasmacytoma of the soft tissues.\nExtramedullary plasmacytomas usually occur in the head and neck, especially in the upper respiratory tract. They are more common in men than women, and are usually diagnosed around age 50.","Curated_Disease_Description_Source__c":"PlainLanguagePilotV1-Sep23","GARD_Synonym__c":"plasmacytoma - disorder; solitary myeloma; solitary plasmacytoma","Name":"Plasmacytoma","Curated_USA_Estimate__c":"5,000","estimateUsa":"5,000"}],"Organization_Supported_Diseases__c":[{"Account_Name__c":"HealthTree Foundation","Website__c":"https://healthtree.org/"},{"Account_Name__c":"Blood Cancer United","Website__c":"https://bloodcancerunited.org/"},{"Account_Name__c":"American Cancer Society","Website__c":"https://www.cancer.org"}],"GARD_Disease_Tag__c":[{"Tag_Name__c":"Cancer - Oncologist","Tag_Category__c":"Specialist"},{"Tag_Name__c":"Cancer","Tag_Category__c":"Disease Category","category_description":"Cancer is a disease in which some of the body's cells grow uncontrollably and can spread to other parts of the body.","curated_tag_name":"Cancer"},{"Tag_Name__c":"Neurology","Tag_Category__c":"Disease Category;Specialist","category_description":"Neurological diseases affect the brain, spinal cord, cranial nerves, autonomic nerves, or other peripheral nerves.","curated_tag_name":"Neurological diseases"},{"Tag_Name__c":"Hematology","Tag_Category__c":"Disease Category;Specialist","category_description":"Blood diseases affect the blood or blood-forming organs, including red blood cells, white blood cells, platelets, plasma, and bone marrow.","curated_tag_name":"Blood diseases"},{"Tag_Name__c":"Peripheral Neuropathy","Tag_Category__c":"Account","curated_tag_name":"Peripheral neuropathy"}],"Age_At_Onset__c":[{"Age_At_Onset__c":"Adult","Provided_By__c":"ORPHA:86855"}],"External_Identifier_Disease__c":[{"URL__c":"https://www.orpha.net/en/disease/detail/86855","Source__c":"C0032131; MONDO:0005615; ORPHA:86855","Xref__c":"ORPHA:86855"},{"URL__c":"https://www.ebi.ac.uk/ols4/ontologies/doid/classes?obo_id=DOID%3A3721","Source__c":"MONDO:0005615","Xref__c":"DOID:3721"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=415112005","Source__c":"C0032131; MONDO:0005615","Xref__c":"415112005"},{"URL__c":"https://www.ncbi.nlm.nih.gov/medgen/?term=18506","Source__c":"C0032131","Xref__c":"MEDGEN:18506"},{"URL__c":"https://evsexplore.semantics.cancer.gov/evsexplore/concept/ncit/C9349","Source__c":"C0032131; MONDO:0005615","Xref__c":"C9349"},{"URL__c":"https://uts.nlm.nih.gov/uts/umls/concept/C0032131","Source__c":"C0032131","Xref__c":"C0032131"},{"URL__c":"https://www.ncbi.nlm.nih.gov/mesh/C010954","Source__c":"C0032131; MONDO:0005615","Xref__c":"D010954"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=1187107005","Source__c":"C0032131","Xref__c":"1187107005"},{"URL__c":"http://purl.obolibrary.org/obo/MONDO_0005615","Source__c":"GARD:0013446","Xref__c":"MONDO:0005615"},{"URL__c":"https://hpo.jax.org/browse/term/HP:0011857","Source__c":"C0032131","Xref__c":"HP:0011857"},{"URL__c":"https://evsexplore.semantics.cancer.gov/evsexplore/concept/ncit/C6932","Source__c":"C0032131","Xref__c":"C6932"}],"tags":{"Specialist":["Cancer - Oncologist","Neurology","Hematology"],"Disease Category":["Cancer","Neurology","Hematology"],"Account":["Peripheral Neuropathy"]},"synonyms":["plasmacytoma - disorder"," solitary myeloma"," solitary plasmacytoma"]}