{"entity":{"id":"nasopharyngeal","kind":"cancer","name":"Nasopharyngeal carcinoma","aka":["NPC"],"tldr":"A cancer at the back of the nose caused largely by the Epstein-Barr virus and common in southern China and Southeast Asia. Radiation cures most early cases; adding chemotherapy and, recently, PD-1 immunotherapy has improved outcomes in advanced disease, and a blood test for viral DNA can detect it early.","summary":"Nasopharyngeal carcinoma (NPC) in endemic regions is EBV-associated non-keratinising carcinoma with distinct biology (NF-κB pathway alterations, immune-rich stroma, low TMB), while sporadic Western cases include keratinising HPV- or smoking-related tumours. Plasma EBV DNA is a diagnostic, prognostic and surveillance marker, and population screening with EBV DNA detected early-stage cancers in Hong Kong (Chan, NEJM 2017).\n\nIMRT is the backbone: radiotherapy alone for stage I, concurrent cisplatin-radiotherapy for stage II-IVA (Intergroup 0099), with induction gemcitabine-cisplatin improving survival in locoregionally advanced disease (Zhang, NEJM 2019) and adjuvant metronomic capecitabine adding benefit in high-risk patients (Chen, Lancet 2021). Recurrent or metastatic disease is treated with gemcitabine-cisplatin plus a PD-1 inhibitor: toripalimab (JUPITER-02; FDA approval 2023, the first US approval for NPC), camrelizumab (CAPTAIN-1st), tislelizumab (RATIONALE-309) and penpulimab (FDA 2025). Nasopharyngectomy (endoscopic) and re-irradiation are options for local recurrence. Late toxicities of radiotherapy (xerostomia, hearing loss, temporal-lobe necrosis, carotid stenosis) drive de-escalation trials guided by EBV DNA.","asOf":"2026-09-08","wikipedia":"https://en.wikipedia.org/wiki/Nasopharyngeal_carcinoma","links":[{"label":"CSCO/ASCO NPC guideline (JCO 2021)","url":"https://doi.org/10.1200/JCO.20.03237"},{"label":"EBV DNA screening (NEJM 2017)","url":"https://doi.org/10.1056/NEJMoa1701717"},{"label":"NCI PDQ: nasopharyngeal cancer","url":"https://www.cancer.gov/types/head-and-neck/patient/adult/nasopharyngeal-treatment-pdq"}],"tags":["gap-fill","head-and-neck","viral"],"related":[],"cancers":[],"sections":[],"technologies":["imrt-igrt","proton-therapy","checkpoint-inhibitor","platinum","liquid-biopsy","tors","tcr-t","acupuncture-xerostomia"],"targets":["pd1","pdl1","egfr","vegf"],"drugs":["toripalimab","camrelizumab","tislelizumab","penpulimab","gemcitabine-cisplatin","cisplatin","nivolumab","pembrolizumab"],"companies":["akeso","hengrui","beone"],"institutions":["sysucc"],"pathways":["pd1-checkpoint"],"terms":["ctdna","re-irradiation"],"trials":["taishan-301","nct05294172","nct06976190","nct06118333","nct04974398"],"people":[],"bottlenecks":[],"keyPapers":["paper-chan-n-engl-j-med"],"journals":["tumour-virus-research"],"dependsOn":[],"notes":[],"group":"head and neck","burden":"About 120,000 cases per year worldwide, ~70% in East and Southeast Asia (Guangdong incidence 20-30 per 100,000 versus <1 in the West); strongly linked to Epstein-Barr virus.","subtypes":["Non-keratinising undifferentiated (EBV-associated, endemic; WHO type III)","Non-keratinising differentiated (type II)","Keratinising squamous (type I; sporadic, smoking)","HPV-associated NPC (non-endemic, oropharynx-like)"],"biomarkers":["Plasma EBV DNA (screening, staging, post-treatment risk)","EBER in situ hybridisation on biopsy","TNM (AJCC 8th) stage","PD-L1 (not required for PD-1 therapy)","Post-radiotherapy detectable EBV DNA (adjuvant therapy selection)","Family history / HLA (risk)"],"standardOfCare":[{"setting":"Stage I","approach":"IMRT alone (70 Gy) to nasopharynx and elective neck.","refs":["imrt-igrt"],"guideline":{"nccn":"Category 2A","version":"NCCN Guidelines: Head and Neck (Nasopharynx); CSCO/ASCO NPC guideline","url":"https://doi.org/10.1200/JCO.20.03237"}},{"setting":"Stage II-IVA","approach":"Induction gemcitabine-cisplatin ×3 then concurrent cisplatin-IMRT (for stage III-IVA); concurrent chemoradiation alone for stage II; adjuvant capecitabine for high-risk (detectable post-RT EBV DNA, N2-3).","refs":["gemcitabine-cisplatin","cisplatin","imrt-igrt"],"guideline":{"nccn":"Category 1 (induction GP)","esmoMcbs":"A","version":"CSCO/ASCO NPC guideline 2021"}},{"setting":"Recurrent/metastatic, first line","approach":"Gemcitabine-cisplatin + PD-1 inhibitor (toripalimab, camrelizumab, tislelizumab or penpulimab), then PD-1 maintenance.","refs":["toripalimab","camrelizumab","tislelizumab","penpulimab","gemcitabine-cisplatin"],"guideline":{"nccn":"Category 1 (toripalimab + GP)","esmoMcbs":"4","version":"NCCN Guidelines: Head and Neck"}},{"setting":"Local recurrence","approach":"Endoscopic or open nasopharyngectomy for resectable rT1-3 (better survival than re-irradiation, Liu Lancet Oncol 2021); hyperfractionated re-IMRT otherwise.","refs":["imrt-igrt","tors"]}],"stateOfArt":["Plasma EBV DNA is the most mature liquid biopsy in oncology: it screens, stages, guides adjuvant therapy and detects relapse.","PD-1 inhibitors with GP chemotherapy roughly doubled PFS in metastatic disease; toripalimab was the first FDA approval for NPC and among the first for a Chinese-developed PD-1 antibody.","Induction GP and adjuvant capecitabine are the standard for locoregionally advanced disease.","De-escalation (omitting concurrent cisplatin in low-risk stage II, reducing neck irradiation) is being validated in trials led from Guangzhou."],"history":[{"year":1921,"title":"Regaud and Schmincke describe lymphoepithelioma","refs":[]},{"year":1966,"title":"EBV antibodies linked to NPC (Old et al.)","refs":[]},{"year":1998,"title":"Intergroup 0099: concurrent cisplatin-RT improves survival (Al-Sarraf)","refs":["cisplatin"]},{"year":1999,"title":"Plasma EBV DNA quantified as a tumour marker (Lo, Cancer Res)","refs":[]},{"year":2017,"title":"EBV DNA screening detects early NPC in 20,000 men (Chan, NEJM)","refs":[]},{"year":2019,"title":"Induction gemcitabine-cisplatin improves survival (Zhang, NEJM)","refs":["gemcitabine-cisplatin"]},{"year":2021,"title":"JUPITER-02, CAPTAIN-1st, RATIONALE-309: PD-1 + GP first line","refs":["toripalimab","camrelizumab","tislelizumab"]},{"year":2023,"title":"Toripalimab: first FDA approval for NPC","refs":["toripalimab"]},{"year":2025,"title":"Penpulimab approved (FDA)","refs":["penpulimab"]}],"pipeline":["toripalimab","penpulimab","camrelizumab","tislelizumab","proton-therapy","yl201","kl-a167","mrg003","hmbd-001"],"openProblems":["Radiation late effects in a disease cured young.","Western access to PD-1 inhibitors studied in Asia; regulatory lag.","EBV-targeted therapy (vaccines, EBV-specific T cells) still investigational.","Distant metastasis is the main way treatment fails after chemoradiation; plasma EBV DNA now identifies who needs more."]},"route":"/cancers/nasopharyngeal/","neighbours":{"technology":[{"id":"acupuncture-xerostomia","kind":"technology","name":"Acupuncture for dry mouth after head and neck radiotherapy","route":"/technologies/acupuncture-xerostomia/"},{"id":"checkpoint-inhibitor","kind":"technology","name":"Immune checkpoint inhibitors","route":"/technologies/checkpoint-inhibitor/"},{"id":"imrt-igrt","kind":"technology","name":"IMRT / IGRT (modern external beam)","route":"/technologies/imrt-igrt/"},{"id":"liquid-biopsy","kind":"technology","name":"Liquid biopsy (ctDNA)","route":"/technologies/liquid-biopsy/"},{"id":"platinum","kind":"technology","name":"Platinum agents","route":"/technologies/platinum/"},{"id":"proton-therapy","kind":"technology","name":"Proton therapy","route":"/technologies/proton-therapy/"},{"id":"rejuv-rehab-scar-contracture","kind":"technology","name":"Scars, stiffness and contracture: shoulders, necks and jaws","route":"/technologies/rejuv-rehab-scar-contracture/"},{"id":"rejuv-rehab-swallowing","kind":"technology","name":"Swallowing therapy around head and neck radiotherapy","route":"/technologies/rejuv-rehab-swallowing/"},{"id":"tcr-t","kind":"technology","name":"TCR-T cell therapy","route":"/technologies/tcr-t/"},{"id":"rejuv-paed-pituitary-and-puberty","kind":"technology","name":"The pituitary, puberty and the hypothalamic axis after cranial radiotherapy in childhood","route":"/technologies/rejuv-paed-pituitary-and-puberty/"},{"id":"tors","kind":"technology","name":"Transoral robotic surgery (TORS)","route":"/technologies/tors/"},{"id":"virus-specific-t-cells","kind":"technology","name":"Virus-specific T cells","route":"/technologies/virus-specific-t-cells/"}],"target":[{"id":"arid2","kind":"target","name":"ARID2","route":"/targets/arid2/"},{"id":"casp8","kind":"target","name":"CASP8","route":"/targets/casp8/"},{"id":"cntrl","kind":"target","name":"CNTRL","route":"/targets/cntrl/"},{"id":"cyld","kind":"target","name":"CYLD","route":"/targets/cyld/"},{"id":"dsp","kind":"target","name":"DSP","route":"/targets/dsp/"},{"id":"egfr","kind":"target","name":"EGFR","route":"/targets/egfr/"},{"id":"fat1","kind":"target","name":"FAT1","route":"/targets/fat1/"},{"id":"kat6b","kind":"target","name":"KAT6B","route":"/targets/kat6b/"},{"id":"keap1","kind":"target","name":"KEAP1","route":"/targets/keap1/"},{"id":"myh11","kind":"target","name":"MYH11","route":"/targets/myh11/"},{"id":"nutm2d","kind":"target","name":"NUTM2D","route":"/targets/nutm2d/"},{"id":"pd1","kind":"target","name":"PD-1","route":"/targets/pd1/"},{"id":"pdl1","kind":"target","name":"PD-L1","route":"/targets/pdl1/"},{"id":"spen","kind":"target","name":"SPEN","route":"/targets/spen/"},{"id":"tet1","kind":"target","name":"TET1","route":"/targets/tet1/"},{"id":"usp9x","kind":"target","name":"USP9X","route":"/targets/usp9x/"},{"id":"vegf","kind":"target","name":"VEGF / VEGFR","route":"/targets/vegf/"},{"id":"zeb1","kind":"target","name":"ZEB1","route":"/targets/zeb1/"},{"id":"znf331","kind":"target","name":"ZNF331","route":"/targets/znf331/"},{"id":"znf521","kind":"target","name":"ZNF521","route":"/targets/znf521/"},{"id":"znf626","kind":"target","name":"ZNF626","route":"/targets/znf626/"},{"id":"znf680","kind":"target","name":"ZNF680","route":"/targets/znf680/"},{"id":"znf721","kind":"target","name":"ZNF721","route":"/targets/znf721/"},{"id":"znf780a","kind":"target","name":"ZNF780A","route":"/targets/znf780a/"},{"id":"znf90","kind":"target","name":"ZNF90","route":"/targets/znf90/"},{"id":"znf93","kind":"target","name":"ZNF93","route":"/targets/znf93/"}],"drug":[{"id":"camrelizumab","kind":"drug","name":"Camrelizumab","route":"/drugs/camrelizumab/"},{"id":"cisplatin","kind":"drug","name":"Cisplatin","route":"/drugs/cisplatin/"},{"id":"gemcitabine","kind":"drug","name":"Gemcitabine","route":"/drugs/gemcitabine/"},{"id":"gemcitabine-cisplatin","kind":"drug","name":"Gemcitabine + cisplatin","route":"/drugs/gemcitabine-cisplatin/"},{"id":"h101-oncolytic-adenovirus","kind":"drug","name":"H101 oncolytic adenovirus","route":"/drugs/h101-oncolytic-adenovirus/"},{"id":"hmbd-001","kind":"drug","name":"HMBD-001","route":"/drugs/hmbd-001/"},{"id":"kl-a167","kind":"drug","name":"KL-A167","route":"/drugs/kl-a167/"},{"id":"mrg003","kind":"drug","name":"MRG003","route":"/drugs/mrg003/"},{"id":"nivolumab","kind":"drug","name":"Nivolumab","route":"/drugs/nivolumab/"},{"id":"pembrolizumab","kind":"drug","name":"Pembrolizumab","route":"/drugs/pembrolizumab/"},{"id":"penpulimab","kind":"drug","name":"Penpulimab","route":"/drugs/penpulimab/"},{"id":"pilocarpine","kind":"drug","name":"Pilocarpine","route":"/drugs/pilocarpine/"},{"id":"tislelizumab","kind":"drug","name":"Tislelizumab","route":"/drugs/tislelizumab/"},{"id":"toripalimab","kind":"drug","name":"Toripalimab","route":"/drugs/toripalimab/"},{"id":"yl201","kind":"drug","name":"YL201","route":"/drugs/yl201/"}],"company":[{"id":"akeso","kind":"company","name":"Akeso","route":"/companies/akeso/"},{"id":"beone","kind":"company","name":"BeOne Medicines (formerly BeiGene)","route":"/companies/beone/"},{"id":"hengrui","kind":"company","name":"Jiangsu Hengrui Pharmaceuticals","route":"/companies/hengrui/"},{"id":"lepu-biopharma","kind":"company","name":"Lepu Biopharma","route":"/companies/lepu-biopharma/"},{"id":"shanghai-sunway-biotech","kind":"company","name":"Shanghai Sunway Biotech","route":"/companies/shanghai-sunway-biotech/"}],"institution":[{"id":"sysucc","kind":"institution","name":"Sun Yat-sen University Cancer Center","route":"/institutions/sysucc/"}],"pathway":[{"id":"oncogenic-viruses","kind":"pathway","name":"Oncogenic viruses","route":"/pathways/oncogenic-viruses/"},{"id":"pd1-checkpoint","kind":"pathway","name":"PD-1 / PD-L1 immune checkpoint & T-cell activation","route":"/pathways/pd1-checkpoint/"}],"term":[{"id":"ctdna","kind":"term","name":"Circulating tumour DNA (ctDNA)","route":"/terms/ctdna/"},{"id":"plasma-ebv-dna","kind":"term","name":"Plasma EBV DNA","route":"/terms/plasma-ebv-dna/"},{"id":"re-irradiation","kind":"term","name":"Re-irradiation","route":"/terms/re-irradiation/"}],"trial":[{"id":"nct06839066","kind":"trial","name":"A Phase II Study to Evaluate HLX43 in Subjects With Recurrent/Metastatic Nasopharyngeal Carcinoma Failed or Intolerance to Second-line Therapy","route":"/trials/nct06839066/"},{"id":"taishan-301","kind":"trial","name":"A Phase III Study of YL201 in Recurrent or Metastatic Nasopharyngeal Carcinoma(TAISHAN-301)","route":"/trials/taishan-301/"},{"id":"nct06118333","kind":"trial","name":"A Study Comparing BL-B01D1 With Physician's Choice of Chemotherapy in Patients With Recurrent or Metastatic Nasopharyngeal Carcinoma(PANKU-NPC01)","route":"/trials/nct06118333/"},{"id":"nct06475300","kind":"trial","name":"A Study of BL-B01D1+PD-1 Monoclonal Antibody in Patients With Locally Advanced or Metastatic Non-small Cell Lung Cancer, Nasopharyngeal Carcinoma and ","route":"/trials/nct06475300/"},{"id":"nct06976190","kind":"trial","name":"A Study of MRG003 in Combination With Pucotenlimab Versus Chemotherapy in the Treatment of Patients With Recurrent or Metastatic Nasopharyngeal Carcin","route":"/trials/nct06976190/"},{"id":"nct04974398","kind":"trial","name":"A Study of Penpulimab (AK105) in the First-line Treatment of Recurrent or Metastatic Nasopharyngeal Carcinoma","route":"/trials/nct04974398/"},{"id":"nct05956587","kind":"trial","name":"A Study of SI-B003 and BL-B01D1+SI-B003 in Patients With Locally Advanced or Metastatic Non-small Cell Lung Cancer, Nasopharyngeal Carcinoma and Other","route":"/trials/nct05956587/"},{"id":"nct07258979","kind":"trial","name":"A Study of YL201 in Combination With Toripalimab and With or Without Cisplatin in Nasopharyngeal Carcinoma.","route":"/trials/nct07258979/"},{"id":"nct05126719","kind":"trial","name":"A Study to Evaluate the Efficacy and Safety of MRG003 in Patients With Recurrent Metastatic Nasopharyngeal Carcinoma","route":"/trials/nct05126719/"},{"id":"nct05261750","kind":"trial","name":"Autologous Dendritic Cells and Allogenic Dendritic Secretomes for Patients With Advanced Nasopharyngeal Cancer","route":"/trials/nct05261750/"},{"id":"nct07038343","kind":"trial","name":"AVENTINE-1: Study of AVZO-1418 as a Single Agent and in Combination Therapy in Patients With Locally Advanced or Metastatic Solid Tumors (AVZO-1418-1001)","route":"/trials/nct07038343/"},{"id":"captain-1st","kind":"trial","name":"CAPTAIN-1st","route":"/trials/captain-1st/"},{"id":"continuum","kind":"trial","name":"CONTINUUM","route":"/trials/continuum/"},{"id":"nct01086488","kind":"trial","name":"Foscan®-Mediated Photodynamic Therapy Versus Brachytherapy in Patients With Nasopharyngeal Carcinoma","route":"/trials/nct01086488/"},{"id":"jupiter-02","kind":"trial","name":"JUPITER-02","route":"/trials/jupiter-02/"},{"id":"nct05294172","kind":"trial","name":"KL-A167 Injection Combined With Cisplatin and Gemcitabine vs Placebo Combined With Cisplatin and Gemcitabine in the Treatment of Recurrent or Metastatic Nasopharyngeal Carcinoma","route":"/trials/nct05294172/"}],"paper":[{"id":"paper-chan-n-engl-j-med","kind":"paper","name":"Analysis of Plasma Epstein-Barr Virus DNA to Screen for Nasopharyngeal Cancer","route":"/key-papers/paper-chan-n-engl-j-med/"},{"id":"paper-continuum-sintilimab-nasopharyngeal-liu-lancet-2024","kind":"paper","name":"CONTINUUM: induction-concurrent chemoradiotherapy with or without sintilimab in locoregionally advanced nasopharyngeal carcinoma","route":"/key-papers/paper-continuum-sintilimab-nasopharyngeal-liu-lancet-2024/"}],"journal":[{"id":"tumour-virus-research","kind":"journal","name":"Tumour virus research","route":"/journals/tumour-virus-research/"}],"cancer":[{"id":"extranodal-nk-t-cell-lymphoma","kind":"cancer","name":"Extranodal NK/T-cell lymphoma","route":"/cancers/extranodal-nk-t-cell-lymphoma/"},{"id":"locoregionally-advanced-nasopharyngeal-carcinoma","kind":"cancer","name":"Locoregionally advanced nasopharyngeal carcinoma (stage III to IVA)","route":"/cancers/locoregionally-advanced-nasopharyngeal-carcinoma/"},{"id":"lymphoepithelial-carcinoma-lung","kind":"cancer","name":"Lymphoepithelial carcinoma of the lung","route":"/cancers/lymphoepithelial-carcinoma-lung/"},{"id":"rare-childhood-cancers","kind":"cancer","name":"Rare cancers of childhood (NCI PDQ umbrella)","route":"/cancers/rare-childhood-cancers/"},{"id":"recurrent-metastatic-nasopharyngeal-carcinoma","kind":"cancer","name":"Recurrent and metastatic nasopharyngeal carcinoma","route":"/cancers/recurrent-metastatic-nasopharyngeal-carcinoma/"}],"person":[{"id":"anthony-chan","kind":"person","name":"Anthony T. C. Chan","route":"/people/anthony-chan/"},{"id":"goh-boon-cher","kind":"person","name":"Goh Boon Cher","route":"/people/goh-boon-cher/"}],"collection":[{"id":"csco-guidelines","kind":"collection","name":"CSCO guidelines (China)","route":"/collections/csco-guidelines/"}]}}