{"entity":{"id":"rare-cancers","kind":"term","name":"Rare cancers","aka":["rare cancer"],"tldr":"Rare cancers are those with fewer than about 6 new cases per 100,000 people per year. Individually rare, together they are a quarter of all cancers and have worse survival because of late diagnosis, few trials and scattered expertise.","summary":"RARECARE (EU) defines rare cancers as incidence <6/100,000/year; there are ~200 such entities making up ~24% of European cancers, with 5-year survival ~48% versus ~63% for common cancers. Causes of the gap: diagnostic delay and misdiagnosis, absence of standard treatments, difficulty running trials (basket/umbrella designs, registries, real-world evidence help), and dispersed care. Responses: European Reference Networks (EURACAN, EuroBloodNet, PaedCan), centralisation (e.g. sarcoma centres), the NCI Rare Tumor Patient Engagement Network, Rare Cancers Europe, orphan-drug incentives and tumour-agnostic approvals (NTRK, MSI-H, RET, BRAF) that serve rare histologies. Paediatric cancers are all rare cancers.","asOf":"2026-09-08","wikipedia":"https://en.wikipedia.org/wiki/Rare_disease","links":[{"label":"RARECARE / RARECAREnet","url":"https://www.rarecarenet.eu/"},{"label":"Rare Cancers Europe","url":"https://www.rarecancerseurope.org/"},{"label":"NCI MyPART","url":"https://www.cancer.gov/pediatric-adult-rare-tumor"}],"tags":["gap-fill"],"related":["gist","adrenocortical","thymic-epithelial","salivary-gland","gestational-trophoblastic","bpdcn","appendiceal","uveal-melanoma","merkel-cell-carcinoma"],"cancers":[],"sections":[],"technologies":[],"targets":[],"drugs":[],"companies":[],"institutions":[],"pathways":[],"terms":["tumour-agnostic","basket-umbrella-platform","real-world-evidence"],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"category":"Epidemiology & prevention"},"route":"/terms/rare-cancers/","neighbours":{"cancer":[{"id":"adrenocortical","kind":"cancer","name":"Adrenocortical carcinoma","route":"/cancers/adrenocortical/"},{"id":"appendiceal","kind":"cancer","name":"Appendiceal cancer and pseudomyxoma peritonei","route":"/cancers/appendiceal/"},{"id":"bpdcn","kind":"cancer","name":"Blastic plasmacytoid dendritic cell neoplasm (BPDCN)","route":"/cancers/bpdcn/"},{"id":"chordoma","kind":"cancer","name":"Chordoma","route":"/cancers/chordoma/"},{"id":"dermatofibrosarcoma-protuberans","kind":"cancer","name":"Dermatofibrosarcoma protuberans","route":"/cancers/dermatofibrosarcoma-protuberans/"},{"id":"desmoid-tumour","kind":"cancer","name":"Desmoid tumour","route":"/cancers/desmoid-tumour/"},{"id":"epithelioid-sarcoma","kind":"cancer","name":"Epithelioid sarcoma","route":"/cancers/epithelioid-sarcoma/"},{"id":"esthesioneuroblastoma","kind":"cancer","name":"Esthesioneuroblastoma (olfactory neuroblastoma)","route":"/cancers/esthesioneuroblastoma/"},{"id":"gist","kind":"cancer","name":"Gastrointestinal stromal tumour (GIST)","route":"/cancers/gist/"},{"id":"gestational-trophoblastic","kind":"cancer","name":"Gestational trophoblastic neoplasia","route":"/cancers/gestational-trophoblastic/"},{"id":"hereditary-ppgl","kind":"cancer","name":"Hereditary pheochromocytoma and paraganglioma (SDHx, VHL, RET, NF1, MAX and TMEM127)","route":"/cancers/hereditary-ppgl/"},{"id":"inflammatory-myofibroblastic-tumour","kind":"cancer","name":"Inflammatory myofibroblastic tumour (IMT)","route":"/cancers/inflammatory-myofibroblastic-tumour/"},{"id":"merkel-cell-carcinoma","kind":"cancer","name":"Merkel cell carcinoma","route":"/cancers/merkel-cell-carcinoma/"},{"id":"metastatic-ppgl","kind":"cancer","name":"Metastatic pheochromocytoma and paraganglioma","route":"/cancers/metastatic-ppgl/"},{"id":"sinonasal","kind":"cancer","name":"Nasal cavity and paranasal sinus cancers (including esthesioneuroblastoma)","route":"/cancers/sinonasal/"},{"id":"nut-carcinoma","kind":"cancer","name":"NUT carcinoma (midline carcinoma with NUTM1 rearrangement)","route":"/cancers/nut-carcinoma/"},{"id":"parathyroid-carcinoma","kind":"cancer","name":"Parathyroid carcinoma","route":"/cancers/parathyroid-carcinoma/"},{"id":"pheochromocytoma-paraganglioma","kind":"cancer","name":"Pheochromocytoma and paraganglioma (PPGL)","route":"/cancers/pheochromocytoma-paraganglioma/"},{"id":"pituitary-tumours","kind":"cancer","name":"Pituitary tumours (pituitary neuroendocrine tumours) and pituitary carcinoma","route":"/cancers/pituitary-tumours/"},{"id":"salivary-gland","kind":"cancer","name":"Salivary gland cancers","route":"/cancers/salivary-gland/"},{"id":"sinonasal-undifferentiated-carcinoma","kind":"cancer","name":"Sinonasal undifferentiated carcinoma (SNUC) and SWI/SNF-deficient sinonasal carcinoma","route":"/cancers/sinonasal-undifferentiated-carcinoma/"},{"id":"tenosynovial-giant-cell-tumour","kind":"cancer","name":"Tenosynovial giant cell tumour (TGCT)","route":"/cancers/tenosynovial-giant-cell-tumour/"},{"id":"thymic-epithelial","kind":"cancer","name":"Thymoma and thymic carcinoma","route":"/cancers/thymic-epithelial/"},{"id":"uterine-sarcoma","kind":"cancer","name":"Uterine sarcoma","route":"/cancers/uterine-sarcoma/"},{"id":"uveal-melanoma","kind":"cancer","name":"Uveal melanoma","route":"/cancers/uveal-melanoma/"},{"id":"vascular-tumours","kind":"cancer","name":"Vascular tumours (angiosarcoma, epithelioid haemangioendothelioma, kaposiform haemangioendothelioma)","route":"/cancers/vascular-tumours/"}],"term":[{"id":"accrual","kind":"term","name":"Accrual and enrolment","route":"/terms/accrual/"},{"id":"basket-umbrella-platform","kind":"term","name":"Basket, umbrella, and platform trials","route":"/terms/basket-umbrella-platform/"},{"id":"centralisation","kind":"term","name":"Centralisation and high-volume centres","route":"/terms/centralisation/"},{"id":"off-label","kind":"term","name":"Off-label","route":"/terms/off-label/"},{"id":"orphan-drug","kind":"term","name":"Orphan drug","route":"/terms/orphan-drug/"},{"id":"orphan-drug-act","kind":"term","name":"Orphan Drug Act 1983","route":"/terms/orphan-drug-act/"},{"id":"orphan-designation","kind":"term","name":"Orphan drug designation","route":"/terms/orphan-designation/"},{"id":"real-world-evidence","kind":"term","name":"Real-world evidence","route":"/terms/real-world-evidence/"},{"id":"eu-orphan-regulation","kind":"term","name":"Regulation (EC) No 141/2000: EU orphan medicines","route":"/terms/eu-orphan-regulation/"},{"id":"eu-paediatric-regulation","kind":"term","name":"Regulation (EC) No 1901/2006: EU paediatric medicines","route":"/terms/eu-paediatric-regulation/"},{"id":"sarcoma-type","kind":"term","name":"Sarcoma (tissue type)","route":"/terms/sarcoma-type/"},{"id":"tumour-agnostic","kind":"term","name":"Tumour-agnostic (tissue-agnostic) approval","route":"/terms/tumour-agnostic/"}],"trial":[{"id":"combomatch","kind":"trial","name":"ComboMATCH (EAY191)","route":"/trials/combomatch/"},{"id":"dart-s1609","kind":"trial","name":"DART (SWOG S1609): nivolumab plus ipilimumab in rare tumours","route":"/trials/dart-s1609/"},{"id":"determine-trial","kind":"trial","name":"DETERMINE","route":"/trials/determine-trial/"},{"id":"determine-arm04","kind":"trial","name":"DETERMINE arm 04: trastuzumab with pertuzumab in HER2-amplified or mutated rare cancers","route":"/trials/determine-arm04/"},{"id":"i-predict","kind":"trial","name":"I-PREDICT","route":"/trials/i-predict/"},{"id":"mypathway","kind":"trial","name":"MyPathway","route":"/trials/mypathway/"},{"id":"nci-match","kind":"trial","name":"NCI-MATCH (EAY131)","route":"/trials/nci-match/"},{"id":"roar","kind":"trial","name":"ROAR (Rare Oncology Agnostic Research) basket: BRAF V600E biliary tract cancer cohort","route":"/trials/roar/"},{"id":"tapur","kind":"trial","name":"TAPUR (Targeted Agent and Profiling Utilization Registry)","route":"/trials/tapur/"},{"id":"winther","kind":"trial","name":"WINTHER","route":"/trials/winther/"}],"institution":[{"id":"garvan-institute","kind":"institution","name":"Garvan Institute of Medical Research / Kinghorn Cancer Centre","route":"/institutions/garvan-institute/"},{"id":"pumch","kind":"institution","name":"Peking Union Medical College Hospital","route":"/institutions/pumch/"}]}}