{"Name":"Adenoid cystic carcinoma","DiseaseID__c":"GARD:0005743","id":5743,"encodedName":"adenoid-cystic-carcinoma","IsDeleted":false,"Disease_Name_Full__c":"Adenoid cystic carcinoma","Xref_IDs__c":"11671000; C0010606; C2970; D003528; DOID:0080202; MEDGEN:41382; MONDO:0004971","USA_Estimate__c":null,"No_of_Specialist_Tagsa__c":0,"No_of_ClinGen_records__c":0,"No_of_GeneReviews__c":0,"No_of_HHS_records__c":0,"World_Estimate__c":null,"No_of_HRSA_records__c":0,"Evidence_Based_Score__c":0,"No_of_Disease_Descriptions__c":2,"Disease_Characteristics_Score__c":2,"No_of_Age_at_Onset__c":0,"Description_Source__c":"MONDO:0004971","Disease_Description__c":"A malignant tumor arising from the epithelial cells. Microscopically, the neoplastic epithelial cells form cylindrical spatial configurations (cribriform or classic type of adenoid cystic carcinoma), cordlike structures (tubular type of adenoid cystic carcinoma), or solid structures (basaloid variant of adenoid cystic carcinoma). Adenoid cystic carcinomas mostly occur in the salivary glands. Other primary sites of involvement include the lacrimal gland, the larynx, and the lungs. Adenoid cystic carcinomas spread along nerve sheaths, resulting in severe pain, and they tend to recur. Lymph node metastases are unusual; hematogenous tumor spread is characteristic.","GARD_Name__c":"Adenoid cystic carcinoma","GARD_Synonym__c":"adenocarcinoma, cylindroid; adenocystic carcinoma; adenoid cystic cancer; cylindroid adenocarcinoma; cylindroma; cylindroma (except cylindroma of skin, m-82000)","Curated_Disease_Description_Source__c":"GARD:0005743","Curated_Disease_Description__c":"Adenoid cystic carcinoma (ACC) is a rare form of adenocarcinoma, a type of cancer that begins in glandular tissues. It most commonly arises in the major and minor salivary glands of the head and neck. It can also occur in the breast, uterus, or other locations in the body. Symptoms depend on the tumor's location. Salivary gland tumors may cause painless masses in the mouth or face. Tumors of the lacrimal gland may cause a bulging eye or changes in vision. Those affecting the windpipe or voice box may cause respiratory symptoms or changes in speech, respectively. Advanced tumors may cause pain and/or nerve paralysis, as ACC often spreads along the nerves. It may also spread through the bloodstream. It spreads to the lymph nodes in about 5% to 10% of cases. The cause of ACC is currently unknown. It typically does not run in families.","Curated_USA_Estimate_Source__c":null,"Curated_USA_Estimate__c":null,"Age_at_Onset_Snippet_Text__c":null,"SourceID__c":null,"Deprecated__c":"No","Disease_Concept_Type__c":"Rare Disease Grouping","MONDO_ID__c":"MONDO:0004971","ORPHANET_ID__c":null,"Replaced_By_ID__c":null,"Display_Spanish_Disease_Name__c":null,"Spanish_Description_Source__c":null,"Spanish_Description__c":null,"Spanish_Disease_Name__c":null,"Spanish_GARD_Synonym__c":null,"Category_Linearization__c":null,"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":"Adenoid cystic carcinoma (ACC) is a rare form of adenocarcinoma, a type of cancer that begins in glandular tissues. It most commonly arises in the major and minor salivary glands of the head and neck. It can also occur in the breast, uterus, or other locations in the body. Symptoms depend on the tumor's location. Salivary gland tumors may cause painless masses in the mouth or face. Tumors of the lacrimal gland may cause a bulging eye or changes in vision. Those affecting the windpipe or voice box may cause respiratory symptoms or changes in speech, respectively. Advanced tumors may cause pain and/or nerve paralysis, as ACC often spreads along the nerves. It may also spread through the bloodstream. It spreads to the lymph nodes in about 5% to 10% of cases. The cause of ACC is currently unknown. It typically does not run in families.","Curated_Disease_Description_Source__c":"GARD:0005743","GARD_Synonym__c":"adenocarcinoma, cylindroid; adenocystic carcinoma; adenoid cystic cancer; cylindroid adenocarcinoma; cylindroma; cylindroma (except cylindroma of skin, m-82000)","Name":"Adenoid cystic carcinoma","estimateUsa":""}],"Organization_Supported_Diseases__c":[{"Account_Name__c":"American Cancer Society","Website__c":"https://www.cancer.org"},{"Account_Name__c":"Adenoid Cystic Carcinoma Research Foundation","Website__c":"https://www.accrf.org/"},{"Account_Name__c":"Adenoid Cystic Carcinoma Organization International","Website__c":"https://www.accoi.org/"},{"Account_Name__c":"The Oral Cancer Foundation","Website__c":"https://oralcancerfoundation.org/"}],"GARD_Disease_Tag__c":[{"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"}],"External_Identifier_Disease__c":[{"URL__c":"https://www.ncbi.nlm.nih.gov/mesh/C003528","Source__c":"C0010606; MONDO:0004971","Xref__c":"D003528"},{"URL__c":"https://uts.nlm.nih.gov/uts/umls/concept/C0010606","Source__c":"C0010606","Xref__c":"C0010606"},{"URL__c":"https://www.ebi.ac.uk/ols4/ontologies/doid/classes?obo_id=DOID%3A0080202","Source__c":"MONDO:0004971","Xref__c":"DOID:0080202"},{"URL__c":"https://evsexplore.semantics.cancer.gov/evsexplore/concept/ncit/C2970","Source__c":"C0010606; MONDO:0004971","Xref__c":"C2970"},{"URL__c":"https://www.ncbi.nlm.nih.gov/medgen/?term=41382","Source__c":"C0010606","Xref__c":"MEDGEN:41382"},{"URL__c":"http://purl.obolibrary.org/obo/MONDO_0004971","Source__c":"GARD:0005743","Xref__c":"MONDO:0004971"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=302827006","Source__c":"C0010606","Xref__c":"302827006"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=11671000","Source__c":"C0010606","Xref__c":"11671000"}],"tags":{"Disease Category":["Cancer"]},"synonyms":["adenocarcinoma, cylindroid"," adenocystic carcinoma"," adenoid cystic cancer"," cylindroid adenocarcinoma"," cylindroma"," cylindroma (except cylindroma of skin, m-82000)"]}