{"entity":{"id":"thymic-epithelial","kind":"cancer","name":"Thymoma and thymic carcinoma","aka":["Thymic epithelial tumours","TET","Thymus Cancer"],"tldr":"Thymoma and thymic carcinoma are rare tumours of the thymus gland in the chest. Thymomas grow slowly, often cause autoimmune diseases such as myasthenia gravis, and are usually cured by surgery; thymic carcinomas behave like other aggressive cancers and have few effective drugs.","summary":"Thymic epithelial tumours range from indolent thymomas (WHO types A, AB, B1-B3) to thymic carcinoma (type C, mostly squamous) and thymic neuroendocrine tumours. Thymomas have the lowest tumour mutational burden of any adult cancer (GTF2I L424H in ~40% of type A/AB) and are uniquely associated with paraneoplastic autoimmunity (myasthenia gravis in ~30%, pure red cell aplasia, hypogammaglobulinaemia/Good syndrome). Staging uses Masaoka-Koga and the TNM 8th edition (ITMIG/IASLC).\n\nComplete resection is the treatment for resectable disease, with post-operative radiotherapy for stage II-III thymoma with high-risk features and for thymic carcinoma. Unresectable disease is treated with induction chemotherapy (cisplatin-doxorubicin-cyclophosphamide, CAP, or carboplatin-paclitaxel for thymic carcinoma) followed by surgery or radiotherapy. Recurrent disease is treated with re-resection where possible, chemotherapy, octreotide plus prednisone for octreoscan-positive thymoma, and in thymic carcinoma with sunitinib or lenvatinib (REMORA). PD-1 inhibitors show activity in thymic carcinoma (pembrolizumab ~20% response) but cause severe immune-related adverse events, especially myocarditis and myositis, and are avoided in thymoma. Everolimus and KIT inhibitors (for the ~10% of thymic carcinomas with KIT mutations) are options.","asOf":"2026-09-08","wikipedia":"https://en.wikipedia.org/wiki/Thymoma","links":[{"label":"NCCN Guidelines: Thymomas and Thymic Carcinomas","url":"https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1469"},{"label":"ITMIG","url":"https://itmig.org/"},{"label":"NCI PDQ: thymoma and thymic carcinoma","url":"https://www.cancer.gov/types/thymoma/patient/thymoma-treatment-pdq"}],"tags":["gap-fill","thoracic","rare"],"related":[],"cancers":[],"sections":[],"technologies":["robotic-surgery","imrt-igrt","proton-therapy","kinase-inhibitors","checkpoint-inhibitor","cardio-oncology"],"targets":["kit","pd1","vegf"],"drugs":["cisplatin","doxorubicin","cyclophosphamide","carboplatin","paclitaxel","sunitinib","lenvatinib","pembrolizumab","everolimus","octreotide-lanreotide","imatinib"],"companies":["pfizer","eisai","merck"],"institutions":[],"pathways":[],"terms":["irae","tmb"],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"group":"thoracic","burden":"About 1.5-3 per million per year; the most common anterior mediastinal tumour in adults; a third of thymoma patients have myasthenia gravis.","subtypes":["Thymoma type A / AB (indolent)","Thymoma type B1 / B2 / B3","Thymic carcinoma (squamous, others)","Thymic neuroendocrine tumours (carcinoid, associated with MEN1)","Micronodular thymoma with lymphoid stroma"],"biomarkers":["WHO histotype and Masaoka-Koga / TNM stage","Completeness of resection (R0)","Acetylcholine-receptor antibodies (myasthenia screening before surgery)","GTF2I L424H (type A/AB thymoma)","KIT mutation (thymic carcinoma subset)","Octreotide scan positivity (somatostatin therapy)"],"standardOfCare":[{"setting":"Resectable (stage I-III)","approach":"Complete thymectomy (minimally invasive for small tumours) after myasthenia control; post-operative radiotherapy for stage III, R1/R2, or thymic carcinoma.","refs":["robotic-surgery","imrt-igrt"],"guideline":{"nccn":"Category 2A","version":"NCCN Guidelines: Thymomas and Thymic Carcinomas","url":"https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1469"}},{"setting":"Locally advanced unresectable","approach":"Induction chemotherapy (CAP or carboplatin-paclitaxel) then surgery if resectable, otherwise definitive radiotherapy ± chemotherapy.","refs":["cisplatin","doxorubicin","cyclophosphamide","carboplatin","paclitaxel","imrt-igrt"],"guideline":{"nccn":"Category 2A","version":"NCCN Guidelines: Thymomas and Thymic Carcinomas"}},{"setting":"Recurrent thymoma","approach":"Re-resection of pleural or local recurrence; chemotherapy; octreotide + prednisone if octreoscan-positive; everolimus.","refs":["octreotide-lanreotide","everolimus"],"guideline":{"nccn":"Category 2A","version":"NCCN Guidelines: Thymomas and Thymic Carcinomas"}},{"setting":"Recurrent thymic carcinoma","approach":"Sunitinib or lenvatinib (REMORA); pembrolizumab (with strict cardiac monitoring, not in thymoma); everolimus; KIT inhibitors for KIT-mutant disease.","refs":["sunitinib","lenvatinib","pembrolizumab","everolimus","imatinib"],"guideline":{"nccn":"Category 2A","version":"NCCN Guidelines: Thymomas and Thymic Carcinomas"}}],"stateOfArt":["Surgery cures most thymomas; the ITMIG global database and TNM staging (2017) standardised a field once defined by single-centre series.","Multikinase inhibitors are the only agents with prospective phase 2 evidence in thymic carcinoma.","Immunotherapy is a double-edged sword: responses in thymic carcinoma, but life-threatening myocarditis and myositis, and contraindication in thymoma.","Thymoma's near-absence of mutations and its autoimmune phenotype make it a model for understanding tolerance."],"history":[{"year":1939,"title":"Blalock: thymectomy improves myasthenia gravis","refs":[]},{"year":1981,"title":"Masaoka staging system","note":"Refined by Koga in 1994.","refs":[]},{"year":1999,"title":"WHO histologic classification of thymic epithelial tumours","refs":[]},{"year":2010,"title":"ITMIG founded; global retrospective database","refs":[]},{"year":2014,"title":"GTF2I mutation discovered in thymoma (Petrini, Nat Genet)","refs":[]},{"year":2015,"title":"Sunitinib active in thymic carcinoma (Thomas, Lancet Oncol)","refs":["sunitinib"]},{"year":2017,"title":"TNM 8th edition staging (IASLC/ITMIG)","refs":[]},{"year":2018,"title":"Pembrolizumab in thymic carcinoma: activity with severe irAEs (Giaccone, Lancet Oncol)","refs":["pembrolizumab"]},{"year":2020,"title":"REMORA: lenvatinib in thymic carcinoma","refs":["lenvatinib"]}],"pipeline":["lenvatinib","sunitinib","pembrolizumab","kc1036","eb-car30-nk"],"openProblems":["No randomised trials have ever been completed in thymic epithelial tumours.","Immunotherapy safety in a tumour that disturbs central tolerance.","Thymic carcinoma metastatic disease: median survival ~2-3 years.","Management of paraneoplastic syndromes alongside cancer therapy."]},"route":"/cancers/thymic-epithelial/","neighbours":{"technology":[{"id":"cardio-oncology","kind":"technology","name":"Cardio-oncology","route":"/technologies/cardio-oncology/"},{"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":"octreotide-lanreotide","kind":"drug","name":"Somatostatin analogues (octreotide, lanreotide)","route":"/drugs/octreotide-lanreotide/"},{"id":"sunitinib","kind":"drug","name":"Sunitinib","route":"/drugs/sunitinib/"}],"company":[{"id":"eisai","kind":"company","name":"Eisai","route":"/companies/eisai/"},{"id":"merck","kind":"company","name":"Merck & Co. (MSD)","route":"/companies/merck/"},{"id":"pfizer","kind":"company","name":"Pfizer (incl. Seagen)","route":"/companies/pfizer/"}],"term":[{"id":"irae","kind":"term","name":"Immune-related adverse events (irAEs)","route":"/terms/irae/"},{"id":"rare-cancers","kind":"term","name":"Rare cancers","route":"/terms/rare-cancers/"},{"id":"superior-vena-cava-obstruction","kind":"term","name":"Superior vena cava obstruction","route":"/terms/superior-vena-cava-obstruction/"},{"id":"tmb","kind":"term","name":"Tumour mutational burden (TMB)","route":"/terms/tmb/"}],"journal":[{"id":"lung-cancer-journal","kind":"journal","name":"Lung cancer","route":"/journals/lung-cancer-journal/"},{"id":"thoracic-cancer","kind":"journal","name":"Thoracic cancer","route":"/journals/thoracic-cancer/"}],"cancer":[{"id":"mediastinal-germ-cell-tumour","kind":"cancer","name":"Mediastinal germ cell tumour","route":"/cancers/mediastinal-germ-cell-tumour/"},{"id":"micronodular-thymoma","kind":"cancer","name":"Micronodular thymoma with lymphoid stroma","route":"/cancers/micronodular-thymoma/"},{"id":"thymic-carcinoma","kind":"cancer","name":"Thymic carcinoma","route":"/cancers/thymic-carcinoma/"},{"id":"thymoma","kind":"cancer","name":"Thymoma (WHO types A, AB, B1, B2 and B3)","route":"/cancers/thymoma/"},{"id":"thymoma-type-a-ab","kind":"cancer","name":"Type A and type AB thymoma","route":"/cancers/thymoma-type-a-ab/"},{"id":"thymoma-type-b1-b2","kind":"cancer","name":"Type B1 and type B2 thymoma","route":"/cancers/thymoma-type-b1-b2/"},{"id":"thymoma-type-b3","kind":"cancer","name":"Type B3 thymoma","route":"/cancers/thymoma-type-b3/"}],"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/"}]}}