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Thymoma and thymic carcinoma

aka Thymic epithelial tumours, TET

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.

State of the art today

  • 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.
Who it affects

About 1.5-3 per million per year; the most common anterior mediastinal tumour in adults; a third of thymoma patients have myasthenia gravis.

Where the cases are

Cases by country · GLOBOCAN 2022
All countries →

No country-level case numbers. This cancer is not mapped to a GLOBOCAN site.

Standard of care

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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.

NCCN · Category 2A
Robotic & minimally invasive surgeryIMRT / IGRT (modern external beam)
Locally advanced unresectable

Induction chemotherapy (CAP or carboplatin-paclitaxel) then surgery if resectable, otherwise definitive radiotherapy ± chemotherapy.

NCCN · Category 2A
CisplatinDoxorubicinCyclophosphamideCarboplatinPaclitaxel / nab-paclitaxelIMRT / IGRT (modern external beam)
Recurrent thymoma

Re-resection of pleural or local recurrence; chemotherapy; octreotide + prednisone if octreoscan-positive; everolimus.

NCCN · Category 2A
Somatostatin analogues (octreotide, lanreotide)Everolimus
Recurrent thymic carcinoma

Sunitinib or lenvatinib (REMORA); pembrolizumab (with strict cardiac monitoring, not in thymoma); everolimus; KIT inhibitors for KIT-mutant disease.

NCCN · Category 2A
SunitinibLenvatinibPembrolizumabEverolimusImatinib

Subtypes & biomarkers

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Subtypes
Biomarkers clinicians test

Target prevalence in this cancer

History

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  1. 1939Blalock: thymectomy improves myasthenia gravis
  2. 1981Masaoka staging system

    Refined by Koga in 1994.

  3. 1999WHO histologic classification of thymic epithelial tumours
  4. 2010ITMIG founded; global retrospective database
  5. 2014GTF2I mutation discovered in thymoma (Petrini, Nat Genet)
  6. 2015Sunitinib active in thymic carcinoma (Thomas, Lancet Oncol)
    Sunitinib
  7. 2017TNM 8th edition staging (IASLC/ITMIG)
  8. 2018Pembrolizumab in thymic carcinoma: activity with severe irAEs (Giaccone, Lancet Oncol)
    Pembrolizumab
  9. 2020REMORA: lenvatinib in thymic carcinoma
    Lenvatinib

Pipeline

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ApprovedSmall-molecule multi-kinase inhibitor (VEGFR, FGFR, PDGFR, RET, KIT)
Lenvatinib · Lenvima
ApprovedSmall-molecule multi-kinase inhibitor (VEGFR, PDGFR, KIT)
Sunitinib · Sutent
ApprovedMonoclonal antibody (anti-PD-1)
Pembrolizumab · Keytruda / Keytruda Qlex (SC)

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.

Trials

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Recruiting now (live from ClinicalTrials.gov)

Recruiting trials near you · live from ClinicalTrials.gov
Thymoma and thymic carcinoma
condition: Thymoma and thymic carcinoma
Open on ClinicalTrials.gov →

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Expert centres

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Where the expertise is

Centres linked to this cancer in OnCo

Seeking a second opinion: ask your oncologist for a referral to a high-volume centre; most accept records and pathology by mail or telehealth. In the US, use the NCI's Find a Cancer Center tool or the nonprofit Cancer Commons, which navigates options for advanced cancers at no cost.

Questions to ask

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Bring to your appointment

Questions to ask your oncologist about Thymoma and thymic carcinoma

Generated from this cancer's standard of care, biomarkers, and pipeline · 16 questions

Newly diagnosed

  1. What is my exact diagnosis, stage, and grade, and which tests established them?
    Why: Everything else follows from an accurate stage and subtype.
  2. Which biomarkers have been tested on my tumour (for example WHO histotype and Masaoka-Koga / TNM stage, Completeness of resection, Acetylcholine-receptor antibodies, GTF2I L424H, KIT mutation), and what were the results?
    Why: These results decide eligibility for targeted therapy, immunotherapy, and trials.
  3. Which subtype is my cancer, and does that change the recommended treatment?
    Why: Recognised subtypes for this cancer include Thymoma type A / AB, Thymoma type B1 / B2 / B3, Thymic carcinoma.
  4. Is germline (inherited) genetic testing recommended for me or my family?
    Why: Inherited variants can change treatment and matter for relatives.

Resectable (stage I-III)

  1. For my situation (resectable (stage i-iii)), which of the standard options do you recommend and why?
    Why: Guideline options include: Complete thymectomy (minimally invasive for small tumours) after myasthenia control; post-operative radiotherapy for stage III, R1/R2, or thymic carcinoma.

Locally advanced unresectable

  1. For my situation (locally advanced unresectable), which of the standard options do you recommend and why?
    Why: Guideline options include: Induction chemotherapy (CAP or carboplatin-paclitaxel) then surgery if resectable, otherwise definitive radiotherapy ± chemotherapy.
  2. Am I a candidate for Cisplatin, Doxorubicin, Cyclophosphamide or related drugs, and what side effects should I expect?
    Why: Knowing the expected toxicities helps you plan work, family, and supportive care.

Recurrent thymoma

  1. For my situation (recurrent thymoma), which of the standard options do you recommend and why?
    Why: Guideline options include: Re-resection of pleural or local recurrence; chemotherapy; octreotide + prednisone if octreoscan-positive; everolimus.
  2. Am I a candidate for Somatostatin analogues (octreotide, lanreotide), Everolimus, and what side effects should I expect?
    Why: Knowing the expected toxicities helps you plan work, family, and supportive care.

Recurrent thymic carcinoma

  1. For my situation (recurrent thymic carcinoma), which of the standard options do you recommend and why?
    Why: Guideline options include: Sunitinib or lenvatinib (REMORA); pembrolizumab (with strict cardiac monitoring, not in thymoma); everolimus; KIT inhibitors for KIT-mutant disease.
  2. Am I a candidate for Sunitinib, Lenvatinib, Pembrolizumab or related drugs, and what side effects should I expect?
    Why: Knowing the expected toxicities helps you plan work, family, and supportive care.

Any stage

  1. Are there clinical trials I could join, for example of Lenvatinib, Sunitinib, Pembrolizumab?
    Why: Trials are how the next standard of care is set; asking early keeps options open.
  2. Would a second opinion at a high-volume centre change anything, and can you help arrange it?
    Why: Rare or high-stakes decisions benefit from a centre that treats many similar patients.
  3. What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?
    Why: Supportive care improves quality of life and helps patients complete treatment.
  4. I read that “No randomised trials have ever been completed in thymic epithelial tumours”. How does that affect my plan?
    Why: Open problems are where trials and second opinions matter most.
  5. I read that “Immunotherapy safety in a tumour that disturbs central tolerance”. How does that affect my plan?
    Why: Open problems are where trials and second opinions matter most.

Print this page for your appointment (your browser's print command). These prompts are for discussion; your clinical team knows your case.

Everything relevant

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Direct links plus the targets, companies, and technologies of this cancer's products.

technologies

11
Allogeneic stem cell transplantationAnti-angiogenic therapyCAR-T cell therapyCardio-oncologyCytotoxic chemotherapyImmune checkpoint inhibitorsIMRT / IGRT (modern external beam)Platinum agentsProton therapyRobotic & minimally invasive surgerySmall-molecule kinase inhibitors

targets

8
AKTFGFR2KITPD-1PIK3CA / PI3K-alphaRETSomatostatin receptor 2VEGF / VEGFR

drugs

11
ApprovedCytotoxic chemotherapy (platinum)
Carboplatin
ApprovedCytotoxic chemotherapy (platinum)
Cisplatin · Platinol (generic)
Not mapped hereAlkylating agent (oxazaphosphorine prodrug)
Cyclophosphamide · Cytoxan / Endoxan
ApprovedCytotoxic chemotherapy (anthracycline)
Doxorubicin · Adriamycin
ApprovedSmall-molecule mTOR inhibitor
Everolimus · Afinitor
ApprovedSmall-molecule kinase inhibitor (BCR-ABL, KIT, PDGFRA)
Imatinib · Gleevec
ApprovedSmall-molecule multi-kinase inhibitor (VEGFR, FGFR, PDGFR, RET, KIT)
Lenvatinib · Lenvima
ApprovedCytotoxic chemotherapy (taxane)
Paclitaxel / nab-paclitaxel · Taxol / Abraxane
ApprovedMonoclonal antibody (anti-PD-1)
Pembrolizumab · Keytruda / Keytruda Qlex (SC)
ApprovedPeptide hormone analogue (SSTR2 agonist)
Somatostatin analogues (octreotide, lanreotide) · Sandostatin LAR, Somatuline Depot
ApprovedSmall-molecule multi-kinase inhibitor (VEGFR, PDGFR, KIT)
Sunitinib · Sutent

companies

5
EisaiIpsenMerck & Co. (MSD)NovartisPfizer (incl. Seagen)

terms

3
Immune-related adverse events (irAEs)Rare cancersTumour mutational burden (TMB)

Latest papers

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Latest papers · live from Europe PMC
Open in Europe PMC

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") 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.

Connected

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technologies

6
Cardio-oncologyImmune checkpoint inhibitorsIMRT / IGRT (modern external beam)Proton therapyRobotic & minimally invasive surgerySmall-molecule kinase inhibitors

targets

3
KITPD-1VEGF / VEGFR

drugs

11
ApprovedCytotoxic chemotherapy (platinum)
Carboplatin
ApprovedCytotoxic chemotherapy (platinum)
Cisplatin · Platinol (generic)
Not mapped hereAlkylating agent (oxazaphosphorine prodrug)
Cyclophosphamide · Cytoxan / Endoxan
ApprovedCytotoxic chemotherapy (anthracycline)
Doxorubicin · Adriamycin
ApprovedSmall-molecule mTOR inhibitor
Everolimus · Afinitor
ApprovedSmall-molecule kinase inhibitor (BCR-ABL, KIT, PDGFRA)
Imatinib · Gleevec
ApprovedSmall-molecule multi-kinase inhibitor (VEGFR, FGFR, PDGFR, RET, KIT)
Lenvatinib · Lenvima
ApprovedCytotoxic chemotherapy (taxane)
Paclitaxel / nab-paclitaxel · Taxol / Abraxane
ApprovedMonoclonal antibody (anti-PD-1)
Pembrolizumab · Keytruda / Keytruda Qlex (SC)
ApprovedPeptide hormone analogue (SSTR2 agonist)
Somatostatin analogues (octreotide, lanreotide) · Sandostatin LAR, Somatuline Depot
ApprovedSmall-molecule multi-kinase inhibitor (VEGFR, PDGFR, KIT)
Sunitinib · Sutent

companies

3
EisaiMerck & Co. (MSD)Pfizer (incl. Seagen)

terms

3
Immune-related adverse events (irAEs)Rare cancersTumour mutational burden (TMB)