# Thymoma and thymic carcinoma

Source: https://onco.cc/cancers/thymic-epithelial/  
OnCo record `thymic-epithelial` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

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).

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.

## Fields

- Kind: Cancer
- Last checked: 2026-09-08
- Also known as: Thymic epithelial tumours; TET; Thymus Cancer
- Tags: gap-fill; thoracic; rare
- 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)

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/thymic-epithelial/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/thymic-epithelial/#overview [2 subtypes, 4 state-of-the-art points]
- What it is (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/thymic-epithelial/#what-it-is [7 subtypes]
- Finding it (on the hub): How it shows itself, how it is confirmed, what screening exists, and the biomarkers clinicians test for. https://onco.cc/cancers/thymic-epithelial/#finding-it [6 biomarkers]
- Treating it (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/thymic-epithelial/#treating-it [4 settings, 4 decisions with options]
- Evidence (on the hub): Trials recruiting now, the landmark trials, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/thymic-epithelial/#evidence [3 trials, 9 milestones]
- The science (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/thymic-epithelial/#science [9 targets]
- Where you are (own page): Cases by country, the UK and NHS pathway and other country lenses, and the expert centres with trials on record. https://onco.cc/cancers/thymic-epithelial/where-you-are/
- Living with it (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/thymic-epithelial/#living-with-it [16 questions, 6 red cards]
- What is coming (own page): Everything in development, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/thymic-epithelial/coming/ [13 medicines, 3 trials, 4 open problems]
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/thymic-epithelial/data/ [51 connected records]

## Standard of care

- Resectable (stage I-III): Complete thymectomy (minimally invasive for small tumours) after myasthenia control; post-operative radiotherapy for stage III, R1/R2, or thymic carcinoma. ([Robotic & minimally invasive surgery](https://onco.cc/technologies/robotic-surgery/), [IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/))
- Locally advanced unresectable: Induction chemotherapy (CAP or carboplatin-paclitaxel) then surgery if resectable, otherwise definitive radiotherapy ± chemotherapy. ([Cisplatin](https://onco.cc/drugs/cisplatin/), [Doxorubicin](https://onco.cc/drugs/doxorubicin/), [Cyclophosphamide](https://onco.cc/drugs/cyclophosphamide/), [Carboplatin](https://onco.cc/drugs/carboplatin/), [Paclitaxel / nab-paclitaxel](https://onco.cc/drugs/paclitaxel/), [IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/))
- Recurrent thymoma: Re-resection of pleural or local recurrence; chemotherapy; octreotide + prednisone if octreoscan-positive; everolimus. ([Somatostatin analogues (octreotide, lanreotide)](https://onco.cc/drugs/octreotide-lanreotide/), [Everolimus](https://onco.cc/drugs/everolimus/))
- Recurrent thymic carcinoma: Sunitinib or lenvatinib (REMORA); pembrolizumab (with strict cardiac monitoring, not in thymoma); everolimus; KIT inhibitors for KIT-mutant disease. ([Sunitinib](https://onco.cc/drugs/sunitinib/), [Lenvatinib](https://onco.cc/drugs/lenvatinib/), [Pembrolizumab](https://onco.cc/drugs/pembrolizumab/), [Everolimus](https://onco.cc/drugs/everolimus/), [Imatinib](https://onco.cc/drugs/imatinib/))

## State of the art

- 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.

## Open problems

- 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.

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Thymoma
- NCCN Guidelines: Thymomas and Thymic Carcinomas: https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1469
- ITMIG: https://itmig.org/
- NCI PDQ: thymoma and thymic carcinoma: https://www.cancer.gov/types/thymoma/patient/thymoma-treatment-pdq

## Connected records

- technologies: [Cardio-oncology](https://onco.cc/technologies/cardio-oncology/), [Immune checkpoint inhibitors](https://onco.cc/technologies/checkpoint-inhibitor/), [IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/), [Proton therapy](https://onco.cc/technologies/proton-therapy/), [Robotic & minimally invasive surgery](https://onco.cc/technologies/robotic-surgery/), [Small-molecule kinase inhibitors](https://onco.cc/technologies/kinase-inhibitors/)
- targets: [KIT](https://onco.cc/targets/kit/), [PD-1](https://onco.cc/targets/pd1/), [VEGF / VEGFR](https://onco.cc/targets/vegf/)
- drugs: [Carboplatin](https://onco.cc/drugs/carboplatin/), [Cisplatin](https://onco.cc/drugs/cisplatin/), [Cyclophosphamide](https://onco.cc/drugs/cyclophosphamide/), [Doxorubicin](https://onco.cc/drugs/doxorubicin/), [EB-CAR30-NK](https://onco.cc/drugs/eb-car30-nk/), [Everolimus](https://onco.cc/drugs/everolimus/), [Imatinib](https://onco.cc/drugs/imatinib/), [KC1036](https://onco.cc/drugs/kc1036/), [Lenvatinib](https://onco.cc/drugs/lenvatinib/), [Paclitaxel / nab-paclitaxel](https://onco.cc/drugs/paclitaxel/), [Pembrolizumab](https://onco.cc/drugs/pembrolizumab/), [Somatostatin analogues (octreotide, lanreotide)](https://onco.cc/drugs/octreotide-lanreotide/), [Sunitinib](https://onco.cc/drugs/sunitinib/)
- companies: [Eisai](https://onco.cc/companies/eisai/), [Merck & Co. (MSD)](https://onco.cc/companies/merck/), [Pfizer (incl. Seagen)](https://onco.cc/companies/pfizer/)
- terms: [Immune-related adverse events (irAEs)](https://onco.cc/terms/irae/), [Rare cancers](https://onco.cc/terms/rare-cancers/), [Tumour mutational burden (TMB)](https://onco.cc/terms/tmb/)
- journals: [Lung cancer](https://onco.cc/journals/lung-cancer-journal/), [Thoracic cancer](https://onco.cc/journals/thoracic-cancer/)
- cancers: [Mediastinal germ cell tumour](https://onco.cc/cancers/mediastinal-germ-cell-tumour/), [Micronodular thymoma with lymphoid stroma](https://onco.cc/cancers/micronodular-thymoma/), [Thymic carcinoma](https://onco.cc/cancers/thymic-carcinoma/), [Thymoma (WHO types A, AB, B1, B2 and B3)](https://onco.cc/cancers/thymoma/), [Type A and type AB thymoma](https://onco.cc/cancers/thymoma-type-a-ab/), [Type B1 and type B2 thymoma](https://onco.cc/cancers/thymoma-type-b1-b2/), [Type B3 thymoma](https://onco.cc/cancers/thymoma-type-b3/)
- trials: [A Study of KC1036 in Patients with Advanced Thymic Tumors](https://onco.cc/trials/nct05683886/), [Sacituzumab Tirumotecan in Participants With Locally Advanced or Metastatic Thymic Carcinoma](https://onco.cc/trials/nct07324629/), [Target-Selected CAR-NK Cells (CD30, CD5, or Mesothelin) for Relapsed/Refractory B2 Thymoma or Thymic Carcinoma](https://onco.cc/trials/nct07598955/)

---
JSON: https://onco.cc/api/v1/entities/thymic-epithelial.json