{"entity":{"id":"thymic-carcinoma","kind":"cancer","name":"Thymic carcinoma","aka":["Thymic squamous cell carcinoma","Type C thymoma (obsolete)","Thymic carcinoma","Malignant thymic epithelial tumour"],"tldr":"Thymic carcinoma is the aggressive kind of thymic epithelial tumour, a cancer of the thymus that behaves like carcinoma elsewhere and has often spread when found. Surgery is attempted when possible, carboplatin with paclitaxel is the usual chemotherapy, and sunitinib, lenvatinib and the PD-1 antibody pembrolizumab have shown responses in trials, with lenvatinib approved in Japan.","summary":"Thymic carcinoma differs from thymoma in every way that matters: its cells are overtly malignant, it lacks the immature T lymphocytes and the organotypic features of thymoma, it is rarely associated with myasthenia gravis, and it presents with local invasion, pleural spread or distant metastases in most patients. Squamous cell carcinoma is the commonest subtype, followed by lymphoepithelioma-like, basaloid, mucoepidermoid, sarcomatoid and NUT carcinoma (covered on its own page); thymic neuroendocrine carcinomas are classified separately. KIT mutations occur in about a tenth of thymic carcinomas, mostly squamous, and respond to imatinib in case series, and TP53, CDKN2A and epigenetic regulator mutations are common; PD-L1 is often highly expressed.\n\nComplete resection is attempted for localised disease, followed by postoperative radiotherapy, and chemotherapy is given before surgery for borderline tumours. For advanced disease the guidelines favour carboplatin and paclitaxel, which produced responses in about a fifth of patients with thymic carcinoma in prospective series, with CAP or platinum-etoposide as alternatives. After platinum, three phase 2 trials define the options: sunitinib (Lancet Oncology 2015) produced responses in about a quarter of 23 patients with thymic carcinoma; lenvatinib in the Japanese REMORA trial (Lancet Oncology 2020) produced responses in 38 percent of 42 patients and was approved in Japan in 2021; and pembrolizumab (Lancet Oncology 2018) produced responses in 22.5 percent of 40 patients with durable benefit but severe immune-related adverse events, including myocarditis, in 15 percent, a rate high enough that PD-1 antibodies are used only with monitoring and are avoided in thymoma. Newer agents in trials include the multikinase inhibitor KC1036, the TROP2 antibody-drug conjugate sacituzumab tirumotecan, ramucirumab with carboplatin and paclitaxel, and CAR-NK cells against CD30 or mesothelin.","asOf":"2026-09-18","wikipedia":"https://en.wikipedia.org/wiki/Thymic_carcinoma","links":[{"label":"REMORA lenvatinib (Lancet Oncology 2020)","url":"https://doi.org/10.1016/S1470-2045(20)30162-5"},{"label":"Pembrolizumab in thymic carcinoma (Lancet Oncology 2018)","url":"https://doi.org/10.1016/S1470-2045(18)30062-7"},{"label":"Wikipedia","url":"https://en.wikipedia.org/wiki/Thymic_carcinoma"}],"tags":["subtype-page","thoracic"],"related":["thymoma","thymic-epithelial","extrapulmonary-nec"],"cancers":[],"sections":[],"technologies":["robotic-surgery","imrt-igrt","proton-therapy","kinase-inhibitors","checkpoint-inhibitor","cytotoxic-chemotherapy"],"targets":["kit","pd1","vegf"],"drugs":["carboplatin","paclitaxel","cisplatin","doxorubicin","cyclophosphamide","sunitinib","lenvatinib","pembrolizumab","everolimus","imatinib"],"companies":[],"institutions":[],"pathways":[],"terms":["irae","tmb","staging-systems"],"trials":["nct05683886","nct07324629","nct07598955"],"people":[],"bottlenecks":[],"keyPapers":["paper-remora-lenvatinib-thymic-carcinoma-lancet-oncol-2020","paper-giaccone-pembrolizumab-thymic-carcinoma-lancet-oncol-2018","paper-esmo-thymic-epithelial-tumours-guideline-ann-oncol-2015","paper-who-2021-thymus-mediastinum-classification-marx-jto-2022"],"journals":[],"dependsOn":[],"notes":[],"group":"thoracic","burden":"About a fifth of thymic epithelial tumours; usually found at an advanced stage with invasion of the mediastinum or spread to pleura, nodes and distant organs, and rarely tied to myasthenia gravis.","subtypes":["Thymic squamous cell carcinoma (the commonest form; KIT mutations in a subset)","Lymphoepithelioma-like thymic carcinoma (EBV-associated in some)","Basaloid, mucoepidermoid and adenocarcinoma of the thymus","Sarcomatoid thymic carcinoma","Resectable thymic carcinoma (surgery and postoperative radiotherapy)","Advanced or metastatic thymic carcinoma (carboplatin-paclitaxel, then kinase inhibitors or pembrolizumab)"],"biomarkers":["Histological subtype and CD5, CD117 (KIT) and p63 immunohistochemistry","TNM and Masaoka-Koga stage","Completeness of resection","KIT mutation (imatinib-responsive subset)","PD-L1 expression (high in many; not required for pembrolizumab)","Baseline troponin and autoimmune screen before PD-1 therapy"],"standardOfCare":[{"setting":"Resectable disease","approach":"Complete resection with thymectomy and involved structures; postoperative radiotherapy for all stages of thymic carcinoma; chemotherapy considered after incomplete resection.","refs":["robotic-surgery","imrt-igrt","proton-therapy"],"guideline":{"version":"NCCN Guidelines: Thymomas and Thymic Carcinomas","url":"https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1469"}},{"setting":"Borderline resectable","approach":"Induction chemotherapy (carboplatin-paclitaxel or CAP) followed by surgery or radiotherapy according to response.","refs":["carboplatin","paclitaxel","cisplatin","doxorubicin","cyclophosphamide"],"guideline":{"version":"NCCN Guidelines: Thymomas and Thymic Carcinomas","url":"https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1469"}},{"setting":"Advanced, first line","approach":"Carboplatin and paclitaxel; CAP or platinum-etoposide as alternatives.","refs":["carboplatin","paclitaxel","cytotoxic-chemotherapy"],"guideline":{"version":"NCCN Guidelines: Thymomas and Thymic Carcinomas","url":"https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1469"}},{"setting":"After platinum","approach":"Sunitinib; lenvatinib (approved in Japan, REMORA); pembrolizumab with cardiac and autoimmune monitoring; everolimus; imatinib for KIT-mutant tumours.","refs":["sunitinib","lenvatinib","pembrolizumab","everolimus","imatinib","irae","kinase-inhibitors","checkpoint-inhibitor"],"guideline":{"version":"NCCN Guidelines: Thymomas and Thymic Carcinomas","url":"https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1469"}},{"setting":"Trials","approach":"KC1036, sacituzumab tirumotecan, CAR-NK cells, ramucirumab combinations.","refs":["nct05683886","nct07324629","nct07598955","kc1036","eb-car30-nk"],"guideline":{"version":"NCCN Guidelines: Thymomas and Thymic Carcinomas","url":"https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1469"}}],"stateOfArt":["Carboplatin and paclitaxel is the accepted first-line regimen on prospective phase 2 evidence.","Lenvatinib is the first drug approved anywhere specifically for thymic carcinoma.","Pembrolizumab gives durable responses in a fifth of patients but at a real risk of myocarditis."],"history":[{"year":1999,"title":"WHO classification separates thymic carcinoma (then type C) from thymoma","refs":[]},{"year":2004,"title":"KIT mutations and imatinib responses described in thymic carcinoma","refs":["imatinib"]},{"year":2015,"title":"Sunitinib phase 2 reports responses in thymic carcinoma after platinum","refs":["sunitinib"]},{"year":2018,"title":"Pembrolizumab phase 2 in thymic carcinoma: 22.5 percent response, 15 percent severe immune toxicity","refs":["pembrolizumab","irae"]},{"year":2020,"title":"REMORA: lenvatinib 38 percent response rate in thymic carcinoma","refs":["lenvatinib"]},{"year":2021,"title":"Lenvatinib approved in Japan for unresectable thymic carcinoma","refs":["lenvatinib"]}],"pipeline":["lenvatinib","sunitinib","pembrolizumab","kc1036","eb-car30-nk","nct07324629","nct05683886"],"openProblems":["No randomised trial has compared any two regimens in thymic carcinoma.","The sequence of kinase inhibitors and immunotherapy is unstudied.","Predicting which patients will develop myocarditis on PD-1 antibodies is not yet possible.","Long-term survival in metastatic disease remains poor."],"parent":"thymic-epithelial"},"route":"/cancers/thymic-carcinoma/","neighbours":{"cancer":[{"id":"extrapulmonary-nec","kind":"cancer","name":"Extrapulmonary neuroendocrine carcinoma","route":"/cancers/extrapulmonary-nec/"},{"id":"thymoma","kind":"cancer","name":"Thymoma (WHO types A, AB, B1, B2 and B3)","route":"/cancers/thymoma/"},{"id":"thymic-epithelial","kind":"cancer","name":"Thymoma and thymic carcinoma","route":"/cancers/thymic-epithelial/"}],"technology":[{"id":"cytotoxic-chemotherapy","kind":"technology","name":"Cytotoxic chemotherapy","route":"/technologies/cytotoxic-chemotherapy/"},{"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":"proton-therapy","kind":"technology","name":"Proton therapy","route":"/technologies/proton-therapy/"},{"id":"robotic-surgery","kind":"technology","name":"Robotic & minimally invasive surgery","route":"/technologies/robotic-surgery/"},{"id":"kinase-inhibitors","kind":"technology","name":"Small-molecule kinase inhibitors","route":"/technologies/kinase-inhibitors/"}],"target":[{"id":"kit","kind":"target","name":"KIT","route":"/targets/kit/"},{"id":"pd1","kind":"target","name":"PD-1","route":"/targets/pd1/"},{"id":"vegf","kind":"target","name":"VEGF / VEGFR","route":"/targets/vegf/"}],"drug":[{"id":"carboplatin","kind":"drug","name":"Carboplatin","route":"/drugs/carboplatin/"},{"id":"cisplatin","kind":"drug","name":"Cisplatin","route":"/drugs/cisplatin/"},{"id":"cyclophosphamide","kind":"drug","name":"Cyclophosphamide","route":"/drugs/cyclophosphamide/"},{"id":"doxorubicin","kind":"drug","name":"Doxorubicin","route":"/drugs/doxorubicin/"},{"id":"eb-car30-nk","kind":"drug","name":"EB-CAR30-NK","route":"/drugs/eb-car30-nk/"},{"id":"everolimus","kind":"drug","name":"Everolimus","route":"/drugs/everolimus/"},{"id":"imatinib","kind":"drug","name":"Imatinib","route":"/drugs/imatinib/"},{"id":"kc1036","kind":"drug","name":"KC1036","route":"/drugs/kc1036/"},{"id":"lenvatinib","kind":"drug","name":"Lenvatinib","route":"/drugs/lenvatinib/"},{"id":"paclitaxel","kind":"drug","name":"Paclitaxel / nab-paclitaxel","route":"/drugs/paclitaxel/"},{"id":"pembrolizumab","kind":"drug","name":"Pembrolizumab","route":"/drugs/pembrolizumab/"},{"id":"sunitinib","kind":"drug","name":"Sunitinib","route":"/drugs/sunitinib/"}],"term":[{"id":"staging-systems","kind":"term","name":"Disease-specific staging and risk systems (FIGO, Ann Arbor, IPI, R-ISS, ELN, IMDC)","route":"/terms/staging-systems/"},{"id":"irae","kind":"term","name":"Immune-related adverse events (irAEs)","route":"/terms/irae/"},{"id":"tmb","kind":"term","name":"Tumour mutational burden (TMB)","route":"/terms/tmb/"}],"trial":[{"id":"nct05683886","kind":"trial","name":"A Study of KC1036 in Patients with Advanced Thymic Tumors","route":"/trials/nct05683886/"},{"id":"nct07324629","kind":"trial","name":"Sacituzumab Tirumotecan in Participants With Locally Advanced or Metastatic Thymic Carcinoma","route":"/trials/nct07324629/"},{"id":"nct07598955","kind":"trial","name":"Target-Selected CAR-NK Cells (CD30, CD5, or Mesothelin) for Relapsed/Refractory B2 Thymoma or Thymic Carcinoma","route":"/trials/nct07598955/"}],"paper":[{"id":"paper-giaccone-pembrolizumab-thymic-carcinoma-lancet-oncol-2018","kind":"paper","name":"Pembrolizumab in patients with thymic carcinoma: a single-arm phase 2 study","route":"/key-papers/paper-giaccone-pembrolizumab-thymic-carcinoma-lancet-oncol-2018/"},{"id":"paper-remora-lenvatinib-thymic-carcinoma-lancet-oncol-2020","kind":"paper","name":"REMORA: lenvatinib in advanced or metastatic thymic carcinoma","route":"/key-papers/paper-remora-lenvatinib-thymic-carcinoma-lancet-oncol-2020/"},{"id":"paper-who-2021-thymus-mediastinum-classification-marx-jto-2022","kind":"paper","name":"The 2021 WHO classification of tumours of the thymus and mediastinum: what is new in thymic epithelial, germ cell and mesenchymal tumours","route":"/key-papers/paper-who-2021-thymus-mediastinum-classification-marx-jto-2022/"},{"id":"paper-esmo-thymic-epithelial-tumours-guideline-ann-oncol-2015","kind":"paper","name":"Thymic epithelial tumours: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up","route":"/key-papers/paper-esmo-thymic-epithelial-tumours-guideline-ann-oncol-2015/"}]}}