{ "id": 18239, "label": "soft tissue sarcoma", "model": { "label": "Mondo Disease Ontology (MONDO)", "source": 0 }, "type_id": 0, "reference_id": "MONDO:0018078", "properties": { "xrefs": [ "DOID:0070663", "EFO:1001968", "GARD:0004898", "MEDGEN:1642116", "NCIT:C9306", "NORD:1953", "Orphanet:3394", "SCTID:424952003", "UMLS:C4551687" ], "synonyms": [ "malignant soft tissue tumor", "malignant soft tissue tumour", "connective tissue sarcoma", "malignant mesenchymal tumor", "malignant mesenchymal tumour", "non-Rhabdo. soft tissue sarcoma", "sarcoma of soft tissue", "sarcoma of the soft tissue", "soft part sarcoma", "soft tissue sarcoma" ], "definition": "A malignant neoplasm arising from muscle tissue, adipose tissue, blood vessels, fibrous tissue, or other supportive tissues excluding the bones." }, "isLeaf": false, "isRoot": false, "child_count": 46, "parents": [ { "id": 6816, "label": "sarcoma", "isLeaf": false, "isRoot": false, "parents": [ 6733 ], "type_id": 0, "properties": { "xrefs": [ "DOID:1115", "EFO:0000691", "ICD9:171", "ICD9:171.0", "ICD9:171.2", "ICD9:171.3", "ICD9:171.4", "ICD9:171.5", "ICD9:171.6", "ICD9:171.7", "ICD9:171.8", "ICD9:171.9", "ICDO:8800/3", "MEDGEN:224714", "MESH:D012509", "NCIT:C9118", "SCTID:424413001", "UMLS:C1261473" ], "synonyms": [ "mesenchymal tumor, malignant", "sarcoma", "sarcoma of soft tissue and bone", "sarcoma of the soft tissue and bone", "sarcoma, malignant", "tumor of soft tissue and skeleton", "tumour of soft tissue and skeleton" ], "definition": "A usually aggressive malignant neoplasm of the soft tissue or bone. It arises from muscle, fat, fibrous tissue, bone, cartilage, and blood vessels. Sarcomas occur in both children and adults. The prognosis depends largely on the degree of differentiation (grade) of the neoplasm. Representative subtypes are liposarcoma, leiomyosarcoma, osteosarcoma, and chondrosarcoma." }, "child_count": 22, "reference_id": "MONDO:0005089" }, { "id": 21548, "label": "malignant soft tissue neoplasm", "isLeaf": false, "isRoot": false, "parents": [ 6733, 7941 ], "type_id": 0, "properties": { "xrefs": [ "GARD:0027918", "MEDGEN:1632429", "NCIT:C4867", "SCTID:269469005", "UMLS:C4551686" ], "synonyms": [ "malignant neoplasm of soft tissue", "malignant neoplasm of the soft tissue", "malignant soft tissue neoplasm", "malignant soft tissue tumor", "malignant soft tissue tumour", "malignant tumor of soft tissue", "malignant tumor of the soft tissue", "malignant tumour of soft tissue", "malignant tumour of the soft tissue" ], "definition": "A malignant neoplasm arising exclusively from the soft tissues." }, "child_count": 6, "reference_id": "MONDO:0024637" } ], "children": [ { "id": 3444, "label": "esophagus sarcoma", "isLeaf": false, "isRoot": false, "parents": [ 8953, 18239 ], "type_id": 0, "properties": { "xrefs": [ "DOID:1114", "GARD:0022900", "MEDGEN:234233", "NCIT:C5341", "UMLS:C1333466" ], "synonyms": [ "esophageal sarcoma", "esophagus sarcoma", "sarcoma of esophagus", "sarcoma of oesophagus", "sarcoma of the esophagus", "sarcoma of the oesophagus", "sarcoma, esophagus", "sarcoma, oesophagus" ], "categories": [ { "ref": "MONDO:0004335", "name": "digestive system disorder" } ], "definition": "A malignant soft tissue neoplasm that arises from the esophagus. Representative examples include Kaposi sarcoma, leiomyosarcoma, rhabdomyosarcoma, and synovial sarcoma." }, "child_count": 4, "reference_id": "MONDO:0001204" }, { "id": 3596, "label": "bladder sarcoma", "isLeaf": true, "isRoot": false, "parents": [ 3431, 18239 ], "type_id": 0, "properties": { "xrefs": [ "DOID:11812", "GARD:0022928", "MEDGEN:87612", "NCIT:C4669", "SCTID:278046008", "UMLS:C0349666" ], "synonyms": [ "bladder sarcoma", "sarcoma of bladder", "sarcoma of the bladder", "sarcoma of the urinary bladder", "sarcoma of urinary bladder", "urinary bladder sarcoma" ], "categories": [ { "ref": "MONDO:0002118", "name": "urinary system disorder" } ], "definition": "A malignant mesenchymal cell neoplasm that affects the urinary bladder." }, "child_count": 0, "reference_id": "MONDO:0001374" }, { "id": 3609, "label": "penile sarcoma", "isLeaf": true, "isRoot": false, "parents": [ 3554, 18239 ], "type_id": 0, "properties": { "xrefs": [ "DOID:11838", "GARD:0022932", "MEDGEN:65960", "NCIT:C7730", "UMLS:C0238352" ], "synonyms": [ "penile sarcoma", "penis sarcoma", "sarcoma of penis", "sarcoma of the penis" ], "categories": [ { "ref": "MONDO:0005039", "name": "reproductive system disorder" } ], "definition": "A malignant soft tissue neoplasm that arises from the penis. Representative examples include Kaposi sarcoma, leiomyosarcoma, and angiosarcoma." }, "child_count": 0, "reference_id": "MONDO:0001387" }, { "id": 3639, "label": "trachea sarcoma", "isLeaf": true, "isRoot": false, "parents": [ 3628, 18239 ], "type_id": 0, "properties": { "xrefs": [ "DOID:12002", "GARD:0022942", "MEDGEN:237016", "NCIT:C6050", "UMLS:C1336774" ], "synonyms": [ "sarcoma of the trachea", "sarcoma of trachea", "trachea sarcoma", "tracheal sarcoma" ], "categories": [ { "ref": "MONDO:0005087", "name": "respiratory system disorder" } ], "definition": "A rare malignant soft tissue neoplasm that arises from the trachea." }, "child_count": 0, "reference_id": "MONDO:0001418" }, { "id": 3709, "label": "retroperitoneal sarcoma", "isLeaf": false, "isRoot": false, "parents": [ 7537, 18239 ], "type_id": 0, "properties": { "xrefs": [ "DOID:12341", "GARD:0022958", "MEDGEN:107937", "NCIT:C4832", "SCTID:307219002", "UMLS:C0585129" ], "synonyms": [ "retroperitoneal sarcoma", "retroperitoneal space sarcoma", "sarcoma of retroperitoneal space" ], "definition": "A sarcoma involving a retroperitoneal space." }, "child_count": 2, "reference_id": "MONDO:0001501" }, { "id": 3943, "label": "paranasal sinus sarcoma", "isLeaf": true, "isRoot": false, "parents": [ 2879, 18239 ], "type_id": 0, "properties": { "xrefs": [ "DOID:1362", "GARD:0023005", "MEDGEN:233842", "NCIT:C6849", "UMLS:C1335342" ], "synonyms": [ "accessory sinus sarcoma", "paranasal sinus sarcoma", "sarcoma of accessory sinus", "sarcoma of paranasal sinus", "sarcoma of the accessory sinus", "sarcoma of the paranasal sinus" ], "categories": [ { "ref": "MONDO:0002081", "name": "musculoskeletal system disorder" }, { "ref": "MONDO:0003900", "name": "connective tissue disorder" }, { "ref": "MONDO:0005071", "name": "nervous system disorder" }, { "ref": "MONDO:0005087", "name": "respiratory system disorder" }, { "ref": "MONDO:0024623", "name": "otorhinolaryngologic disease" } ], "definition": "A malignant soft tissue neoplasm that arises from the paranasal sinus." }, "child_count": 0, "reference_id": "MONDO:0001758" }, { "id": 4252, "label": "pancreas sarcoma", "isLeaf": true, "isRoot": false, "parents": [ 11035, 18239 ], "type_id": 0, "properties": { "xrefs": [ "DOID:1796", "GARD:0023066", "MEDGEN:202112", "NCIT:C5715", "UMLS:C1096346" ], "synonyms": [ "pancreas sarcoma", "pancreatic sarcoma", "sarcoma of pancreas", "sarcoma of the pancreas" ], "categories": [ { "ref": "MONDO:0004335", "name": "digestive system disorder" }, { "ref": "MONDO:0005151", "name": "endocrine system disorder" } ], "definition": "A rare malignant soft tissue neoplasm that occurs primarily in the pancreas." }, "child_count": 0, "reference_id": "MONDO:0002117" }, { "id": 4273, "label": "vagina sarcoma", "isLeaf": false, "isRoot": false, "parents": [ 3623, 18239 ], "type_id": 0, "properties": { "xrefs": [ "DOID:1901", "GARD:0023074", "MEDGEN:65971", "NCIT:C7737", "UMLS:C0238519" ], "synonyms": [ "sarcoma of the vagina", "sarcoma of vagina", "vagina sarcoma", "vaginal sarcoma" ], "categories": [ { "ref": "MONDO:0005039", "name": "reproductive system disorder" } ], "definition": "A malignant mesenchymal neoplasm that arises from the vagina. Representative examples include botryoid-type embryonal rhabdomyosarcoma, leiomyosarcoma, and endometrioid stromal sarcoma." }, "child_count": 6, "reference_id": "MONDO:0002140" }, { "id": 4275, "label": "undifferentiated pleomorphic sarcoma", "isLeaf": false, "isRoot": false, "parents": [ 7165, 18239, 20297 ], "type_id": 0, "properties": { "xrefs": [ "DOID:1907", "EFO:1001972", "GARD:0006963", "HGNC:16982", "ICD9:171.9", "ICDO:8830/3", "MEDGEN:87248", "MESH:D051677", "MedDRA:10025552", "NCIT:C114541", "NCIT:C4247", "ONCOTREE:MFH", "Orphanet:2023", "SCTID:443439001", "UMLS:C0334463" ], "synonyms": [ "MFH", "Storiform-pleomorphic MFH", "Storiform-pleomorphic fibrous histiocytoma", "Storiform-pleomorphic malignant fibrous histiocytoma", "UPS", "Unclassified Pleomorphic sarcoma (formerly \"MFH\")", "Unclassified Pleomorphic sarcoma (formerly \"malignant fibrous histiocytoma\")", "adult malignant fibrous histiocytoma", "adult unclassified pleomorphic sarcoma", "adult undifferentiated pleomorphic sarcoma", "fibrous histiocytoma, malignant", "fibrous histiocytoma, malignant (morphologic abnormality)", "fibroxanthosarcoma", "fibroxanthosarcoma (morphologic abnormality)", "histiocytoma, fibrous, malignant", "malignant fibrous cytoma", "malignant fibrous histiocytoma", "malignant fibrous histiocytoma of soft tissue and bone", "malignant fibrous histiocytoma of the soft tissue and bone", "malignant fibroxanthoma", "unclassified pleomorphic sarcoma", "undifferentiated pleomorphic sarcoma", "undifferentiated pleomorphic soft tissue sarcoma", "malignant fibrohistiocytic tumors", "malignant fibrohistiocytic tumours", "undifferentiated pleomorphic sarcoma/malignant fibrous histiocytoma/high-grade spindle cell sarcoma" ], "categories": [ { "ref": "MONDO:0002081", "name": "musculoskeletal system disorder" }, { "ref": "MONDO:0003900", "name": "connective tissue disorder" } ], "definition": "An undifferentiated soft tissue sarcoma characterized by the presence of a pleomorphic malignant cellular infiltrate. It is also known as malignant fibrous histiocytoma." }, "child_count": 9, "reference_id": "MONDO:0002142" }, { "id": 4336, "label": "central nervous system sarcoma", "isLeaf": false, "isRoot": false, "parents": [ 4753, 18239 ], "type_id": 0, "properties": { "xrefs": [ "DOID:2133", "GARD:0023088", "MEDGEN:272444", "NCIT:C5153", "UMLS:C1332892" ], "synonyms": [ "CNS sarcoma", "central nervous system sarcoma", "sarcoma of CNS", "sarcoma of central nervous system", "sarcoma of the CNS", "sarcoma of the central nervous system" ], "categories": [ { "ref": "MONDO:0005071", "name": "nervous system disorder" } ], "definition": "A sarcoma that arises from the central nervous system." }, "child_count": 24, "reference_id": "MONDO:0002217" }, { "id": 4344, "label": "ovarian sarcoma", "isLeaf": false, "isRoot": false, "parents": [ 9481, 18239 ], "type_id": 0, "properties": { "xrefs": [ "DOID:2146", "GARD:0023091", "MEDGEN:128881", "NCIT:C8267", "SCTID:423627007", "UMLS:C0280746" ], "synonyms": [ "ovarian sarcoma", "ovary sarcoma", "sarcoma of ovary", "sarcoma of the ovary" ], "categories": [ { "ref": "MONDO:0005039", "name": "reproductive system disorder" }, { "ref": "MONDO:0005151", "name": "endocrine system disorder" } ], "definition": "A rare, aggressive malignant mesenchymal neoplasm that arises from the ovary. The prognosis is poor." }, "child_count": 8, "reference_id": "MONDO:0002225" }, { "id": 4487, "label": "liver sarcoma", "isLeaf": false, "isRoot": false, "parents": [ 4735, 18239 ], "type_id": 0, "properties": { "xrefs": [ "DOID:270", "GARD:0023130", "MEDGEN:87522", "NCIT:C4437", "SCTID:254601002", "UMLS:C0345906" ], "synonyms": [ "hepatic sarcoma", "liver sarcoma", "sarcoma of liver", "sarcoma of the liver" ], "categories": [ { "ref": "MONDO:0004335", "name": "digestive system disorder" }, { "ref": "MONDO:0005151", "name": "endocrine system disorder" } ], "definition": "A malignant soft tissue neoplasm that arises from the liver. Representative examples include angiosarcoma, undifferentiated (embryonal) sarcoma, rhabdomyosarcoma, and leiomyosarcoma." }, "child_count": 14, "reference_id": "MONDO:0002397" }, { "id": 4514, "label": "lung sarcoma", "isLeaf": false, "isRoot": false, "parents": [ 10167, 18239 ], "type_id": 0, "properties": { "xrefs": [ "DOID:2784", "GARD:0023135", "MEDGEN:109336", "NCIT:C4860", "UMLS:C0598790" ], "synonyms": [ "lung sarcoma", "pulmonary sarcoma", "sarcoma of lung", "sarcoma of the lung" ], "categories": [ { "ref": "MONDO:0005087", "name": "respiratory system disorder" } ], "definition": "A malignant mesenchymal neoplasm that arises from the lung. Representative examples include Kaposi sarcoma, leiomyosarcoma, and synovial sarcoma." }, "child_count": 2, "reference_id": "MONDO:0002426" }, { "id": 4531, "label": "laryngeal sarcoma", "isLeaf": false, "isRoot": false, "parents": [ 4451, 18239 ], "type_id": 0, "properties": { "xrefs": [ "DOID:2877", "GARD:0023139", "MEDGEN:233610", "NCIT:C6020", "UMLS:C1334377" ], "synonyms": [ "laryngeal sarcoma", "larynx sarcoma", "sarcoma of larynx", "sarcoma of the larynx" ], "categories": [ { "ref": "MONDO:0005087", "name": "respiratory system disorder" } ], "definition": "A rare malignant soft tissue neoplasm that arises from the larynx." }, "child_count": 4, "reference_id": "MONDO:0002448" }, { "id": 4569, "label": "breast sarcoma", "isLeaf": false, "isRoot": false, "parents": [ 8659, 18239 ], "type_id": 0, "properties": { "xrefs": [ "DOID:3017", "GARD:0023149", "MEDGEN:87613", "NCIT:C4670", "ONCOTREE:PBS", "SCTID:278050001", "UMLS:C0349667" ], "synonyms": [ "breast sarcoma", "sarcoma of breast", "sarcoma of the breast", "PBS" ], "categories": [ { "ref": "MONDO:0002657", "name": "breast disorder" } ], "definition": "A malignant mesenchymal neoplasm that arises from the breast. Representative examples include angiosarcoma, liposarcoma, leiomyosarcoma, rhabdomyosarcoma, and extraskeletal osteosarcoma." }, "child_count": 14, "reference_id": "MONDO:0002490" }, { "id": 4672, "label": "extraosseous osteosarcoma", "isLeaf": false, "isRoot": false, "parents": [ 11016, 18239 ], "type_id": 0, "properties": { "xrefs": [ "DOID:3357", "GARD:0023192", "ICD9:170.9", "MEDGEN:163186", "NCIT:C8810", "SCTID:404077005", "UMLS:C0855052" ], "synonyms": [ "extraosseous osteosarcoma", "extraskeletal osteogenic sarcoma", "extraskeletal osteosarcoma", "osteosarcoma, extraskeletal, malignant", "soft tissue osteosarcoma" ], "definition": "An osteosarcoma arising from the soft tissue." }, "child_count": 14, "reference_id": "MONDO:0002621" }, { "id": 4765, "label": "rhabdoid tumor", "isLeaf": false, "isRoot": false, "parents": [ 7212, 18239 ], "type_id": 0, "properties": { "xrefs": [ "DOID:3672", "EFO:0005701", "GARD:0007572", "ICDO:8963/3", "MEDGEN:64646", "MESH:D018335", "NANDO:2200057", "NCIT:C3808", "ONCOTREE:MRT", "Orphanet:69077", "UMLS:C0206743" ], "synonyms": [ "malignant rhabdoid tumor", "rhabdoid sarcoma", "rhabdoid tumor", "rhabdoid cancer" ], "definition": "An aggressive malignant embryonal neoplasm usually occurring during childhood. It is characterized by the presence of large cells with abundant cytoplasm, large eccentric nucleus, and a prominent nucleolus and it is associated with abnormalities of chromosome 22. It can arise from the central nervous system, kidney, and the soft tissues. The prognosis is poor." }, "child_count": 10, "reference_id": "MONDO:0002728" }, { "id": 4866, "label": "mediastinum sarcoma", "isLeaf": false, "isRoot": false, "parents": [ 18239, 22973 ], "type_id": 0, "properties": { "xrefs": [ "DOID:4050", "GARD:0023270", "MEDGEN:233687", "NCIT:C6606", "UMLS:C1334678" ], "synonyms": [ "mediastinal sarcoma", "mediastinum sarcoma", "sarcoma of mediastinum", "sarcoma of the mediastinum" ], "definition": "A rare sarcoma that arises from the mediastinum. Examples include liposarcoma, leiomyosarcoma, and angiosarcoma." }, "child_count": 14, "reference_id": "MONDO:0002852" }, { "id": 4868, "label": "prostate sarcoma", "isLeaf": false, "isRoot": false, "parents": [ 9621, 18239 ], "type_id": 0, "properties": { "xrefs": [ "DOID:4054", "GARD:0023272", "MEDGEN:116060", "NCIT:C7731", "UMLS:C0238393" ], "synonyms": [ "prostate gland sarcoma", "prostate sarcoma", "sarcoma of prostate", "sarcoma of prostate gland", "sarcoma of the prostate" ], "categories": [ { "ref": "MONDO:0005039", "name": "reproductive system disorder" } ], "definition": "A rare malignant soft tissue neoplasm that arises from the prostate gland. Representative examples include leiomyosarcoma, rhabdomyosarcoma, and stromal sarcoma." }, "child_count": 8, "reference_id": "MONDO:0002854" }, { "id": 4871, "label": "gallbladder sarcoma", "isLeaf": false, "isRoot": false, "parents": [ 7085, 18239 ], "type_id": 0, "properties": { "xrefs": [ "DOID:4058", "GARD:0023274", "MEDGEN:232227", "NCIT:C5736", "UMLS:C1333757" ], "synonyms": [ "gall bladder sarcoma", "gallbladder sarcoma", "malignant mesenchymal tumour of gallbladder", "sarcoma of gall bladder", "sarcoma of gallbladder", "sarcoma of the gallbladder" ], "categories": [ { "ref": "MONDO:0004335", "name": "digestive system disorder" } ], "definition": "A malignant soft tissue neoplasm that arises from the gallbladder. Representative examples include Kaposi sarcoma, leiomyosarcoma, and rhabdomyosarcoma." }, "child_count": 6, "reference_id": "MONDO:0002857" }, { "id": 4875, "label": "testis sarcoma", "isLeaf": false, "isRoot": false, "parents": [ 7111, 18239 ], "type_id": 0, "properties": { "xrefs": [ "DOID:4062", "GARD:0023278", "MEDGEN:234943", "NCIT:C6359", "UMLS:C1336727" ], "synonyms": [ "sarcoma of testis", "sarcoma of the testis", "testicular sarcoma", "testis sarcoma" ], "categories": [ { "ref": "MONDO:0005039", "name": "reproductive system disorder" }, { "ref": "MONDO:0005151", "name": "endocrine system disorder" } ], "definition": "A sarcoma that arises from the testis. The majority of cases arise from teratomas or spermatocytic seminomas." }, "child_count": 2, "reference_id": "MONDO:0002861" }, { "id": 4879, "label": "anus sarcoma", "isLeaf": false, "isRoot": false, "parents": [ 4054, 4294, 18239 ], "type_id": 0, "properties": { "xrefs": [ "DOID:4067", "GARD:0023282", "MEDGEN:233982", "NCIT:C5611", "UMLS:C1332277" ], "synonyms": [ "anal sarcoma", "anus sarcoma", "sarcoma of anus", "sarcoma of the anus" ], "categories": [ { "ref": "MONDO:0004335", "name": "digestive system disorder" } ], "definition": "A malignant soft tissue neoplasm arising from the anus. Representative examples include leiomyosarcoma, rhabdomyosarcoma, and Kaposi sarcoma." }, "child_count": 6, "reference_id": "MONDO:0002865" }, { "id": 4932, "label": "clear cell sarcoma", "isLeaf": false, "isRoot": false, "parents": [ 18239 ], "type_id": 0, "properties": { "xrefs": [ "DOID:4233", "EFO:0008498", "GARD:0023304", "ICD9:171.9", "ICDO:9044/3", "MEDGEN:104909", "MESH:D018227", "NANDO:2200062", "NCIT:C3745", "ONCOTREE:CCS", "SCTID:402561003", "UMLS:C0206651" ], "synonyms": [ "chordoid sarcoma", "clear cell sarcoma (morphologic abnormality)", "clear cell sarcoma - not kidney", "clear cell sarcoma of soft parts", "clear cell sarcoma of soft tissue", "clear cell sarcoma/malignant melanoma of soft parts (excluding clear cell sarcoma of the kidney)", "malignant melanoma of the soft parts", "melanoma, malignant, of soft parts" ], "definition": "A rare malignant neoplasm with melanocytic differentiation characterized by the presence of polygonal or spindle shaped clear cells. This sarcoma usually affects the tendons and aponeuroses and is associated with a poor prognosis due to recurrences and metastases." }, "child_count": 1, "reference_id": "MONDO:0002926" }, { "id": 4936, "label": "kidney sarcoma", "isLeaf": false, "isRoot": false, "parents": [ 4465, 18239 ], "type_id": 0, "properties": { "xrefs": [ "DOID:4242", "GARD:0023305", "ICD9:189.0", "MEDGEN:87548", "NCIT:C4525", "SCTID:254918001", "UMLS:C0346251" ], "synonyms": [ "kidney sarcoma", "renal sarcoma", "sarcoma of kidney", "sarcoma of the kidney" ], "categories": [ { "ref": "MONDO:0002118", "name": "urinary system disorder" } ], "definition": "A sarcoma involving a kidney." }, "child_count": 14, "reference_id": "MONDO:0002930" }, { "id": 5021, "label": "thyroid sarcoma", "isLeaf": false, "isRoot": false, "parents": [ 4245, 18239 ], "type_id": 0, "properties": { "xrefs": [ "DOID:4515", "GARD:0023335", "MEDGEN:234950", "NCIT:C6041", "UMLS:C1336756" ], "synonyms": [ "sarcoma of the thyroid", "sarcoma of the thyroid gland", "sarcoma of thyroid", "sarcoma of thyroid gland", "thyroid gland sarcoma", "thyroid sarcoma" ], "categories": [ { "ref": "MONDO:0005151", "name": "endocrine system disorder" } ], "definition": "A malignant soft tissue neoplasm primarily involving the thyroid gland." }, "child_count": 2, "reference_id": "MONDO:0003028" }, { "id": 5289, "label": "heart sarcoma", "isLeaf": false, "isRoot": false, "parents": [ 3568, 18239 ], "type_id": 0, "properties": { "xrefs": [ "DOID:5262", "GARD:0023469", "MEDGEN:68657", "NCIT:C7723", "UMLS:C0238152" ], "synonyms": [ "Cardiac sarcoma", "cardiac sarcoma", "heart sarcoma", "sarcoma of heart", "sarcoma of the heart" ], "categories": [ { "ref": "MONDO:0004995", "name": "cardiovascular disorder" } ], "definition": "A malignant soft tissue neoplasm that arises from the heart. The majority of cases are angiosarcomas." }, "child_count": 6, "reference_id": "MONDO:0003354" }, { "id": 5296, "label": "small intestinal sarcoma", "isLeaf": false, "isRoot": false, "parents": [ 3225, 18239 ], "type_id": 0, "properties": { "xrefs": [ "DOID:5272", "GARD:0023475", "MEDGEN:233455", "NCIT:C5335", "UMLS:C1336007" ], "synonyms": [ "sarcoma of small bowel", "sarcoma of small intestine", "sarcoma of the small bowel", "sarcoma of the small intestine", "sarcoma, small intestinal", "small bowel sarcoma", "small intestinal sarcoma", "small intestine sarcoma" ], "categories": [ { "ref": "MONDO:0004335", "name": "digestive system disorder" } ], "definition": "A malignant soft tissue neoplasm that arises from the small intestine. Representative examples include leiomyosarcoma, angiosarcoma, and Kaposi sarcoma." }, "child_count": 4, "reference_id": "MONDO:0003361" }, { "id": 6788, "label": "leiomyosarcoma", "isLeaf": false, "isRoot": false, "parents": [ 4931, 17662, 18239 ], "type_id": 0, "properties": { "xrefs": [ "DOID:1967", "EFO:0000564", "GARD:0006880", "ICD9:171.9", "ICDO:8890/3", "MEDGEN:9711", "MESH:D007890", "MedDRA:10024189", "NANDO:2200064", "NCIT:C3158", "NORD:1356", "ONCOTREE:LMS", "Orphanet:64720", "SCTID:443719001", "UMLS:C0023269" ], "synonyms": [ "Leiomyosarcomas", "leiomyosarcoma", "leiomyosarcoma (excluding uterine leiomyosarcoma)", "leiomyosarcoma - not uterine", "leiomyosarcoma, malignant" ], "categories": [ { "ref": "MONDO:0002081", "name": "musculoskeletal system disorder" } ], "definition": "An uncommon, aggressive malignant smooth muscle neoplasm, usually occurring in post-menopausal women. It is characterized by a proliferation of neoplastic spindle cells. Morphologic variants include epithelioid, granular cell, inflammatory and myxoid leimyosarcomas." }, "child_count": 93, "reference_id": "MONDO:0005058" }, { "id": 6790, "label": "liposarcoma", "isLeaf": false, "isRoot": false, "parents": [ 4839, 18239 ], "type_id": 0, "properties": { "xrefs": [ "DOID:3382", "EFO:0000569", "GARD:0006913", "ICD9:171.9", "ICDO:8850/3", "MEDGEN:44177", "MESH:D008080", "MedDRA:10024627", "NANDO:2200065", "NCIT:C3194", "NORD:1925", "ONCOTREE:LIPO", "Orphanet:69078", "SCTID:254829001", "UMLS:C0023827" ], "synonyms": [ "lip sarcoma", "liposarcoma", "liposarcoma, malignant", "sarcoma of lip" ], "categories": [ { "ref": "MONDO:0003900", "name": "connective tissue disorder" } ], "definition": "A usually painless malignant tumor that arises from adipose tissue. Microscopically, it may contain a spectrum of neoplastic adipocytes ranging from lipoblasts to pleomorphic malignant adipocytes. Morphologic variants include: well differentiated, dedifferentiated, pleomorphic, and myxoid liposarcoma. The metastatic potential is higher in less differentiated tumors." }, "child_count": 38, "reference_id": "MONDO:0005060" }, { "id": 6886, "label": "fibrosarcoma", "isLeaf": false, "isRoot": false, "parents": [ 7762, 18239 ], "type_id": 0, "properties": { "xrefs": [ "DOID:3355", "EFO:0002087", "GARD:0002327", "HP:0100244", "ICD9:171.9", "ICDO:8810/3", "MEDGEN:5178", "MESH:D005354", "MedDRA:10016632", "NANDO:2200060", "NCIT:C3043", "ONCOTREE:FIBS", "Orphanet:2030", "SCTID:443250000", "UMLS:C0016057" ], "synonyms": [ "fibrocytic tumor", "fibrocytic tumour", "fibrosarcoma", "fibrosarcoma (disease)", "fibrosarcoma (excluding infantile fibrosarcoma)", "fibrosarcoma - not infantile", "fibrosarcoma, malignant", "malignant fibromatous neoplasm" ], "categories": [ { "ref": "MONDO:0003900", "name": "connective tissue disorder" } ], "definition": "A malignant mesenchymal fibroblastic neoplasm affecting the soft tissue and bone." }, "child_count": 22, "reference_id": "MONDO:0005164" }, { "id": 6928, "label": "rhabdomyosarcoma", "isLeaf": false, "isRoot": false, "parents": [ 18239 ], "type_id": 0, "properties": { "xrefs": [ "DOID:3247", "EFO:0002918", "GARD:0011951", "HP:0002859", "ICD9:171.9", "ICDO:8900/3", "MEDGEN:20561", "MESH:D012208", "MedDRA:10039022", "NANDO:2200056", "NCIT:C3359", "ONCOTREE:RMS", "Orphanet:780", "SCTID:302847003", "UMLS:C0035412" ], "synonyms": [ "rhabdomyosarcoma", "rhabdomyosarcoma (disease)", "rhabdomyosarcoma, malignant" ], "definition": "A rare aggressive malignant mesenchymal neoplasm arising from skeletal muscle. It usually occurs in children and young adults. Only a small percentage of tumors arise in the skeletal muscle of the extremities. The majority arise in other anatomical sites." }, "child_count": 20, "reference_id": "MONDO:0005212" }, { "id": 6930, "label": "vulva sarcoma", "isLeaf": false, "isRoot": false, "parents": [ 3735, 18239 ], "type_id": 0, "properties": { "xrefs": [ "DOID:2096", "GARD:0024165", "MEDGEN:65972", "NCIT:C40317", "SCTID:254897006", "UMLS:C0238525" ], "synonyms": [ "mammalian vulva sarcoma", "sarcoma of mammalian vulva", "vulvar sarcoma" ], "categories": [ { "ref": "MONDO:0005039", "name": "reproductive system disorder" } ], "definition": "A malignant mesenchymal neoplasm that arises from the vulva. Representative examples include childhood botryoid-type embryonal rhabdomyosarcoma, alveolar soft part sarcoma, and leiomyosarcoma." }, "child_count": 10, "reference_id": "MONDO:0005214" }, { "id": 7802, "label": "intimal sarcoma", "isLeaf": true, "isRoot": false, "parents": [ 4933, 18239 ], "type_id": 0, "properties": { "xrefs": [ "EFO:1000305", "GARD:0024348", "MEDGEN:317791", "NCIT:C53677", "ONCOTREE:INTS", "UMLS:C1708550" ], "synonyms": [ "INTS" ], "definition": "A malignant neoplasm arising from the large blood vessels. It is characterized by the presence of tumor cells that grow within the lumen of the blood vessels. The intraluminal tumor growth may result in vascular obstruction and spread of tumor emboli to peripheral organs. The prognosis is usually poor." }, "child_count": 0, "reference_id": "MONDO:0006255" }, { "id": 7932, "label": "skin sarcoma", "isLeaf": false, "isRoot": false, "parents": [ 5298, 18239 ], "type_id": 0, "properties": { "xrefs": [ "DOID:2687", "EFO:1000531", "GARD:0024403", "MEDGEN:208989", "NCIT:C5585", "UMLS:C0856900" ], "synonyms": [ "cutaneous sarcoma", "sarcoma of skin", "sarcoma of the skin", "sarcoma of zone of skin", "skin sarcoma", "zone of skin sarcoma" ], "categories": [ { "ref": "MONDO:0002051", "name": "integumentary system disorder" } ], "definition": "A sarcoma that arises from the skin. Representative examples include Kaposi sarcoma, angiosarcoma, lymphangiosarcoma, liposarcoma, and leiomyosarcoma." }, "child_count": 10, "reference_id": "MONDO:0006414" }, { "id": 8305, "label": "myxosarcoma", "isLeaf": true, "isRoot": false, "parents": [ 18239 ], "type_id": 0, "properties": { "xrefs": [ "DOID:4136", "EFO:1001056", "GARD:0024492", "ICDO:8840/3", "MEDGEN:6508", "MESH:D009236", "NCIT:C3255", "UMLS:C0027155" ], "synonyms": [ "myxosarcoma", "myxosarcoma (morphologic abnormality)", "myxosarcoma, malignant" ], "definition": "An infiltrating malignant soft tissue neoplasm characterized by the presence of immature undifferentiated cells and abundant myxoid stroma formation." }, "child_count": 0, "reference_id": "MONDO:0006863" }, { "id": 11594, "label": "synovial sarcoma", "isLeaf": false, "isRoot": false, "parents": [ 5714, 18239 ], "type_id": 0, "properties": { "xrefs": [ "DOID:5485", "EFO:0001376", "GARD:0007721", "HP:0012570", "ICD9:171.9", "ICDO:9040/3", "MEDGEN:21050", "MESH:D013584", "MedDRA:10042863", "NANDO:2200061", "NCIT:C3400", "OMIM:300813", "ONCOTREE:SYNS", "Orphanet:3273", "SCTID:302851001", "UMLS:C0039101" ], "synonyms": [ "SS", "Synovialosarcoma", "sarcoma, synovial, malignant", "synovial sarcoma", "synovial sarcoma (disease)", "sarcoma, synovial" ], "definition": "Synovial sarcoma is an aggressive soft tissue sarcoma, occurring most commonly in adolescents and young adults (15 to 40 years), usually localized near the large joints of the extremities but also in the head and neck, mediastinum and viscera (lung, kidney etc), clinically presenting as a deep seated swelling or a painful mass often with an initial indolent course and is characterized by its local invasiveness and a propensity to metastasize. The origin of synovial sarcoma is likely from multipotent mesenchymal cells and not synovium (contrary to its name)." }, "child_count": 10, "reference_id": "MONDO:0010434" }, { "id": 12747, "label": "alveolar soft part sarcoma", "isLeaf": false, "isRoot": false, "parents": [ 5714, 18239 ], "type_id": 0, "properties": { "xrefs": [ "DOID:4239", "EFO:0007143", "GARD:0005654", "HP:0012218", "ICD9:171.9", "ICDO:9581/3", "MEDGEN:61652", "MESH:D018234", "MedDRA:10001882", "NANDO:2200063", "NCIT:C3750", "NCIT:C7943", "NORD:760", "OMIM:606243", "ONCOTREE:ASPS", "Orphanet:163699", "SCTID:404056007", "UMLS:C0206657" ], "synonyms": [ "ASPS", "adult alveolar soft Part sarcoma", "adult alveolar soft part sarcoma", "adult alveolar soft-Part sarcoma", "alveolar soft Part sarcoma", "alveolar soft part sarcoma", "alveolar soft part sarcoma (disease)", "alveolar soft-part sarcoma", "childhood alveolar soft part sarcoma", "paediatric alveolar soft Part sarcoma", "pediatric alveolar soft Part sarcoma", "alveolar soft PART sarcoma", "alveolar soft tissue sarcoma", "alveolar soft-tissue sarcoma" ], "definition": "An alveolar soft part sarcoma occurring in adults. The most common site of involvement is the extremity, particularly the deep soft tissues of the thigh." }, "child_count": 4, "reference_id": "MONDO:0011655" }, { "id": 13865, "label": "extraskeletal myxoid chondrosarcoma", "isLeaf": false, "isRoot": false, "parents": [ 5714, 18239 ], "type_id": 0, "properties": { "xrefs": [ "DOID:6496", "GARD:0017105", "ICD9:171.9", "MEDGEN:220892", "MESH:C563195", "NCIT:C27502", "OMIM:612237", "ONCOTREE:EMCHS", "Orphanet:209916", "SCTID:404079008", "UMLS:C1275278" ], "synonyms": [ "extraosseous chondrosarcoma", "extraskeletal chondrosarcoma", "myxoid extraosseous chondrosarcoma", "myxoid extraskeletal chondrosarcoma", "EMC", "chondrosarcoma, extraskeletal myxoid" ], "definition": "A rare malignant soft tissue neoplasm of uncertain differentiation, characterized by the presence of chondroblast-like cells in a myxoid stroma and a multinodular growth pattern. The most common sites of involvement are the deep soft tissues of the extremities, particularly the thigh. It usually presents as an enlarging soft tissue mass. Patients may have long survivals, but local recurrences and metastases occur in approximately half of the cases. The most common site of metastasis is the lungs." }, "child_count": 2, "reference_id": "MONDO:0012825" }, { "id": 17394, "label": "angiosarcoma", "isLeaf": false, "isRoot": false, "parents": [ 4233, 18239 ], "type_id": 0, "properties": { "xrefs": [ "DOID:0001816", "EFO:0003968", "GARD:0020900", "HP:0200058", "ICD9:171.9", "ICDO:9120/3", "MEDGEN:42385", "MESH:D006394", "MedDRA:10002476", "NCIT:C3088", "ONCOTREE:ANGS", "Orphanet:263413", "SCTID:403977003", "UMLS:C0018923" ], "synonyms": [ "angiosarcoma", "angiosarcoma (disease)", "blood vessel sarcoma", "hemangiosarcoma", "hemangiosarcoma, malignant", "malignant angioendothelioma", "malignant hemangioendothelioma", "sarcoma of blood vessel", "vascular sarcoma" ], "categories": [ { "ref": "MONDO:0004995", "name": "cardiovascular disorder" } ], "definition": "A malignant tumor arising from the endothelial cells of the blood vessels. Microscopically, it is characterized by frequently open vascular anastomosing and branching channels. The malignant cells that line the vascular channels are spindle or epithelioid and often display hyperchromatic nuclei. Angiosarcomas most frequently occur in the skin and breast. Patients with long-standing lymphedema are at increased risk of developing angiosarcoma." }, "child_count": 38, "reference_id": "MONDO:0016982" }, { "id": 17696, "label": "epithelioid sarcoma", "isLeaf": false, "isRoot": false, "parents": [ 18239 ], "type_id": 0, "properties": { "xrefs": [ "DOID:6193", "GARD:0010181", "ICDO:8804/3", "MEDGEN:104753", "MedDRA:10015099", "NCIT:C3714", "ONCOTREE:EPIS", "Orphanet:293202", "UMLS:C0205944" ], "synonyms": [ "ES", "epithelioid cell sarcoma", "epithelioid sarcoma" ], "definition": "An aggressive malignant neoplasm of uncertain differentiation, characterized by the presence of epithelioid cells forming nodular patterns. The nodules often undergo central necrosis, resulting in a pseudogranulomatous growth pattern. It usually occurs in young adults. The most common sites of involvement are the extremities (distal-type epithelioid sarcoma), and less frequently the pelvis, perineum, and genital organs (proximal-type epithelioid sarcoma)." }, "child_count": 4, "reference_id": "MONDO:0017387" }, { "id": 18389, "label": "extraskeletal Ewing sarcoma", "isLeaf": true, "isRoot": false, "parents": [ 13857, 18239, 20284 ], "type_id": 0, "properties": { "xrefs": [ "DOID:4232", "GARD:0017600", "MEDGEN:76053", "NCIT:C7135", "Orphanet:370334", "UMLS:C0279980", "icd11.foundation:489162074" ], "synonyms": [ "EOE", "Extra-osseous Ewing's sarcoma", "extraosseous Ewing sarcoma", "extraosseous Ewing tumor", "extraosseous Ewing tumour", "extraosseous Ewing's sarcoma", "extraosseous Ewing's tumor", "extraosseous Ewing's tumour", "extraskeletal Ewing sarcoma", "extraskeletal Ewing tumor", "extraskeletal Ewing tumour", "extraskeletal Ewing's sarcoma", "extraosseous Ewing's sarcoma/peripheral primitive neuroectodermal tumour" ], "definition": "A rare malignant neoplasm of the soft tissues. It is typically a disease of children and young adults. Most commonly occurs in the paravertebral region, chest wall, pelvis and lower extremities. Treatment includes local excision with consideration for post-operative chemotherapy and/or radiotherapy." }, "child_count": 0, "reference_id": "MONDO:0018270" }, { "id": 18726, "label": "SMARCA4-deficient sarcoma of thorax", "isLeaf": true, "isRoot": false, "parents": [ 2875, 18239, 20056 ], "type_id": 0, "properties": { "xrefs": [ "DOID:0080532", "GARD:0021943", "MEDGEN:1806595", "NCIT:C183115", "Orphanet:466962", "UMLS:C5554190" ], "synonyms": [ "SMARCA4-deficient thoracic sarcoma" ], "categories": [ { "ref": "MONDO:0005087", "name": "respiratory system disorder" } ] }, "child_count": 0, "reference_id": "MONDO:0018761" }, { "id": 19072, "label": "myxofibrosarcoma", "isLeaf": true, "isRoot": false, "parents": [ 18239 ], "type_id": 0, "properties": { "xrefs": [ "DOID:0080534", "GARD:0018941", "ICDO:8811/3", "MEDGEN:811359", "MedDRA:10066948", "NCIT:C6496", "ONCOTREE:MFS", "Orphanet:79105", "SCTID:253042009", "UMLS:C3714524", "icd11.foundation:405689402" ], "synonyms": [ "fibromyxosarcoma", "myxofibrosarcoma", "myxoid MFH", "myxoid fibrous histiocytoma", "myxoid malignant fibrous histiocytoma", "MFS" ], "definition": "A malignant fibroblastic neoplasm arising from the soft tissue. It is characterized by the presence of spindle-shaped cells, cellular pleomorphism, thin-walled blood vessels, fibrous septa, and myxoid stroma." }, "child_count": 0, "reference_id": "MONDO:0019202" }, { "id": 19207, "label": "desmoplastic small round cell tumor", "isLeaf": true, "isRoot": false, "parents": [ 8399, 18239 ], "type_id": 0, "properties": { "xrefs": [ "DOID:6785", "EFO:1000895", "GARD:0006265", "HGNC:12796", "ICDO:8806/3", "MEDGEN:83833", "MESH:D058405", "MedDRA:10064581", "MedDRA:10064587", "NANDO:2200059", "NCIT:C8300", "ONCOTREE:DSRCT", "Orphanet:83469", "UMLS:C0281508" ], "synonyms": [ "DSRCT", "Desmoplas. small round cell tumor", "Desmoplas. small round cell tumour", "Desmoplastic small round cell tumor", "Desmoplastic small round cell tumour", "Desmoplastic small round-cell neoplasm", "Desmoplastic small round-cell tumor", "Desmoplastic small round-cell tumour", "Polyphenotypic small round cell tumor", "Polyphenotypic small round cell tumour", "desmoplastic small round cell tumor", "desmoplastic small-round-cell tumor", "desmoplastic small-round-cell tumour" ], "definition": "Desmoplastic small round cell tumor (DSRCT) is an aggressive soft tissue cancer that typically arises in serous lined surfaces of the abdominal or pelvic peritoneum, and spreads to the omentum, lymph nodes and hematogenously disseminates especially to the liver. Extraserous primary location has been reported in exceptional cases." }, "child_count": 0, "reference_id": "MONDO:0019373" }, { "id": 20074, "label": "undifferentiated round cell sarcoma", "isLeaf": true, "isRoot": false, "parents": [ 18239 ], "type_id": 0, "properties": { "xrefs": [ "GARD:0025204", "MEDGEN:885673", "NCIT:C121799", "UMLS:C4048304" ], "synonyms": [ "undifferentiated round cell sarcoma" ], "definition": "An undifferentiated soft tissue sarcoma characterized by the presence of uniform round or ovoid malignant cells with a high nuclear to cytoplasmic ratio." }, "child_count": 0, "reference_id": "MONDO:0020661" }, { "id": 23339, "label": "stromal sarcoma", "isLeaf": false, "isRoot": false, "parents": [ 18239 ], "type_id": 0, "properties": { "xrefs": [ "GARD:0025899", "ICDO:8935/3", "MEDGEN:235091", "NCIT:C6926", "UMLS:C1370723" ], "synonyms": [ "stromal sarcoma", "stromal sarcoma, malignant", "stromal tumor, malignant" ], "definition": "A malignant neoplasm characterized by the presence of atypical mesenchymal-stromal cells. Representative examples include endometrial stromal sarcoma and prostate stromal sarcoma." }, "child_count": 1, "reference_id": "MONDO:0044337" } ], "roots": [ { "id": 6816, "label": "sarcoma" }, { "id": 21548, "label": "malignant soft tissue neoplasm" } ] }