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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.
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).
Complete 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.
About 1.5-3 per million per year; the most common anterior mediastinal tumour in adults; a third of thymoma patients have myasthenia gravis.
Central tumours arise in the large airways, peripheral ones in the alveoli; both drain to hilar then mediastinal nodes, and the pleural lining is a separate cancer site.
Same organ: Mediastinal germ cell tumour, Pleuropulmonary blastoma (types I, Ir, II and III), Type A and type AB thymoma, Type B1 and type B2 thymoma, Type B3 thymoma, Micronodular thymoma with lymphoid stroma, Adenocarcinoma of the lung, Squamous cell carcinoma of the lung, Large cell carcinoma of the lung, Sarcomatoid carcinoma of the lung, Adenosquamous carcinoma of the lung, Invasive mucinous adenocarcinoma of the lung, Adenocarcinoma in situ and minimally invasive adenocarcinoma of the lung, Basaloid squamous cell carcinoma of the lung, Lymphoepithelial carcinoma of the lung, Pulmonary blastoma (adult), Non-small-cell lung cancer, Lung cancer (all types), Small-cell lung cancer, Mesothelioma, Pleural mesothelioma, Childhood lung and airway tumours (pleuropulmonary blastoma, tracheobronchial tumours), Inflammatory myofibroblastic tumour (IMT), EGFR-mutated non-small-cell lung cancer, ALK-positive non-small-cell lung cancer, KRAS G12C-mutant non-small-cell lung cancer, ROS1-positive non-small-cell lung cancer, MET exon 14 and MET-amplified non-small-cell lung cancer, RET fusion-positive non-small-cell lung cancer, BRAF V600E-mutant non-small-cell lung cancer, HER2-mutant non-small-cell lung cancer, NTRK fusion-positive non-small-cell lung cancer, PD-L1-high non-small-cell lung cancer without a driver mutation, Resectable stage I to III non-small-cell lung cancer, Unresectable stage III non-small-cell lung cancer, Limited-stage small-cell lung cancer, Extensive-stage small-cell lung cancer, Lung neuroendocrine tumours (typical and atypical carcinoid), Large cell neuroendocrine carcinoma of the lung, Thymoma (WHO types A, AB, B1, B2 and B3), Thymic carcinoma
Complete thymectomy (minimally invasive for small tumours) after myasthenia control; post-operative radiotherapy for stage III, R1/R2, or thymic carcinoma.
Induction chemotherapy (CAP or carboplatin-paclitaxel) then surgery if resectable, otherwise definitive radiotherapy ± chemotherapy.
Re-resection of pleural or local recurrence; chemotherapy; octreotide + prednisone if octreoscan-positive; everolimus.
Sunitinib or lenvatinib (REMORA); pembrolizumab (with strict cardiac monitoring, not in thymoma); everolimus; KIT inhibitors for KIT-mutant disease.
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Query for this cancer: (TITLE:"Thymoma and thymic carcinoma" OR ABSTRACT:"Thymoma and thymic carcinoma" OR TITLE:"Thymic epithelial tumours" OR ABSTRACT:"Thymic epithelial tumours" OR TITLE:"TET" OR ABSTRACT:"TET" OR TITLE:"Thymus Cancer" OR ABSTRACT:"Thymus Cancer") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Thymoma and thymic carcinoma, not a curated reading list.
Refined by Koga in 1994.
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Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
Fainting, near-fainting, or an irregular or racing heartbeat; several kinase inhibitors prolong the QT interval and the labels require ECG and electrolyte monitoring.
Sudden severe abdominal pain, a hard or very tender abdomen, or abdominal pain with vomiting and fever. Boxed warning for gastrointestinal perforation on bevacizumab.
Persistent headache with extreme tiredness, nausea, dizziness on standing or low blood pressure. Vomiting, severe weakness or collapse is adrenal crisis.
QT: both Sunitinib and Lenvatinib prolong the QT interval (known and known risk).. Avoid other QT-prolonging drugs where possible; check ECG and correct potassium and magnesium before and during treatment.
Cumulative dose: risk rises steeply above 400-550 mg/m² (see the anthracycline calculator).
See all on the product pages:CarboplatinCisplatinCyclophosphamideDoxorubicinImatinibLenvatinibPaclitaxel / nab-paclitaxelPembrolizumabSunitinib·Printable cards in the navigator
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