Thymoma

Summary

Thymoma (MONDO:0006456) is a disease (an umbrella term covering 8 Mondo subtypes) and 64 clinical trials. Top therapeutic interventions include avelumab, belinostat, and bosutinib. A subtype of thymic epithelial neoplasm — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Prevalence: 1-9 / 1 000 000 (Europe) [Orphanet-validated]
• Umbrella term: 8 Mondo subtypes
• Phenotypes (HPO): 37
• Clinical trials: 64

Clinical features

Epidemiology

Prevalence records

2 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Annual incidence	1-9 / 1 000 000	0.14	Europe	Validated
Lifetime Prevalence	1-9 / 100 000	1.22	Europe	Validated

Signs & symptoms

Clinical features (HPO)

37 HPO clinical features (Orphanet curated; top 37 by frequency):

HPO ID	Term	Frequency
HP:0001824	Weight loss	Frequent (30-79%)
HP:0031378	Abnormal lymphocyte proliferation	Frequent (30-79%)
HP:0031409	Abnormal lymphocyte physiology	Frequent (30-79%)
HP:0100749	Chest pain	Frequent (30-79%)
HP:0001324	Muscle weakness	Occasional (5-29%)
HP:0001609	Hoarse voice	Occasional (5-29%)
HP:0001945	Fever	Occasional (5-29%)
HP:0002094	Dyspnea	Occasional (5-29%)
HP:0002664	Neoplasm	Occasional (5-29%)
HP:0002721	Immunodeficiency	Occasional (5-29%)
HP:0002960	Autoimmunity	Occasional (5-29%)
HP:0004313	Decreased circulating antibody level	Occasional (5-29%)
HP:0012735	Cough	Occasional (5-29%)
HP:0030166	Night sweats	Occasional (5-29%)
HP:0030208	Acetylcholine receptor antibody positivity	Occasional (5-29%)
HP:0045026	Abnormality of the mediastinum	Occasional (5-29%)
HP:0000099	Glomerulonephritis	Very rare (<1-4%)
HP:0000707	Abnormality of the nervous system	Very rare (<1-4%)
HP:0001370	Rheumatoid arthritis	Very rare (<1-4%)
HP:0001909	Leukemia	Very rare (<1-4%)
HP:0001915	Aplastic anemia	Very rare (<1-4%)
HP:0002725	Systemic lupus erythematosus	Very rare (<1-4%)
HP:0003326	Myalgia	Very rare (<1-4%)
HP:0005560	Imbalanced hemoglobin synthesis	Very rare (<1-4%)
HP:0007108	Demyelinating peripheral neuropathy	Very rare (<1-4%)
HP:0007378	Neoplasm of the gastrointestinal tract	Very rare (<1-4%)
HP:0012288	Neoplasm of head and neck	Very rare (<1-4%)
HP:0012410	Pure red cell aplasia	Very rare (<1-4%)
HP:0012539	Non-Hodgkin lymphoma	Very rare (<1-4%)
HP:0031041	Obstruction of the superior vena cava	Very rare (<1-4%)
HP:0100031	Neoplasm of the thyroid gland	Very rare (<1-4%)
HP:0100279	Ulcerative colitis	Very rare (<1-4%)
HP:0100324	Scleroderma	Very rare (<1-4%)
HP:0100526	Neoplasm of the lung	Very rare (<1-4%)
HP:0100527	Neoplasia of the pleura	Very rare (<1-4%)
HP:0100614	Myositis	Very rare (<1-4%)
HP:0100787	Prostate neoplasm	Very rare (<1-4%)

Identifiers

Disease identifiers

Field	Value
Canonical name	thymoma
Mondo ID	MONDO:0006456
EFO	EFO:1000581
MeSH	D013945
Orphanet	99867
DOID	DOID:3275
ICD-11	33869057
NCIT	C3411
SNOMED CT	444231005
UMLS	C0040100
MedGen	52743
GARD	0016922
MedDRA	10043670
Is cancer (heuristic)	no

Also known as: primary thymic epithelial neoplasm · primary thymic epithelial tumor · primary thymic epithelial tumour · THYM · thymoma · thymoma (disease)

Data availability: 1 HPO phenotype · 7 cell lines · 13 intOGen driver records.

Disease family

This is a subtype of thymic epithelial neoplasm. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by etiologic mechanism › cancer or benign tumor › neoplastic disease or syndrome › neoplasm › endocrine gland neoplasm › thymus neoplasm › thymic epithelial neoplasm › thymoma

Related subtypes (1): thymic carcinoma

Subtypes (8): encapsulated thymoma, thymoma type A, combined thymoma, invasive malignant thymoma, noninvasive malignant thymoma, thymoma, familial, thymoma type B, thymoma type AB

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

Drugs indicated for this disease

No approved or late-stage (phase ≥3) drug is indicated for this disease; the following are in earlier-phase trials only.

Earlier-phase candidates (phase 2, investigational — efficacy not yet established): Amrubicin, Avelumab, Belinostat, Bevacizumab, Bintrafusp Alfa, Buparlisib, Carboplatin, Cisplatin, Epirubicin, Erlotinib, Etoposide, Octreotide, Paclitaxel, Pasireotide, Pembrolizumab, Ramucirumab, Sacituzumab Govitecan, Saracatinib, Selinexor, Sunitinib.

Clinical trials & evidence

Clinical trials

Clinical trials: 64.

Phase distribution (across all retrieved trials)

Phase	Trials
Not specified	27
PHASE2	25
PHASE1	7
PHASE1/PHASE2	2
PHASE4	1
PHASE3	1
EARLY_PHASE1	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT06010277	PHASE4	UNKNOWN	Folinic Acid for Prevention of Pemetrexed-induced Toxicity
NCT02014805	PHASE3	UNKNOWN	Postoperative Conformal Radiotherapy for Stage II-III B Type Thymoma
NCT01025089	PHASE2	ACTIVE_NOT_RECRUITING	Chemotherapy Plus Cetuximab Followed by Surgical Resection in Patients With Locally Advanced or Recurrent Thymoma or Thymic Carcinoma
NCT03076554	PHASE2	ACTIVE_NOT_RECRUITING	A Pilot Study to Investigate the Safety and Clinical Activity of Avelumab (MSB0010718C) in Thymoma and Thymic Carcinoma After Progression on Platinum-Based Chemotherapy
NCT04417660	PHASE2	ACTIVE_NOT_RECRUITING	Bintrafusp Alfa (M7824) in Subjects With Thymoma and Thymic Carcinoma
NCT05104736	PHASE2	RECRUITING	PT-112 in Subjects With Thymoma and Thymic Carcinoma
NCT06248515	PHASE2	RECRUITING	A Phase II Trial of Sacituzumab Govitecan in Patients With Advanced Thymic Epithelial Tumors
NCT06730308	PHASE2	RECRUITING	Glucocorticoids Combined With Concurrent Chemoradiotherapy for Unresectable Thymoma
NCT06838910	PHASE2	RECRUITING	Tislelizumab Combined With Anlotinib as Second-line Therapy in Thymoma and Thymic Carcinoma
NCT06924619	PHASE2	RECRUITING	Neoadjuvant Radiotherapy for Locally Advanced Unresectable Thymoma
NCT00010257	PHASE2	COMPLETED	Carboplatin Combined With Paclitaxel in Treating Patients With Advanced Thymoma
NCT00198133	PHASE2	COMPLETED	Phase II Study of Alimta (Pemetrexed) Treatment of Advanced Thymoma and Thymic Carcinoma
NCT00332969	PHASE2	COMPLETED	Efficacy of Octreotide Treatment in Patients With Primary Inoperable Thymoma
NCT00369889	PHASE2	COMPLETED	A Study to Test the Safety and Efficacy of Erlotinib Plus Bevacizumab to Treat Advanced Thymoma and Thymic Cancer
NCT00387868	PHASE2	COMPLETED	Preoperative Treatment of Patients With High Risk Thymoma
NCT00589290	PHASE2	COMPLETED	Belinostat (PXD101) to Treat Tumors of the Thymus at an Advanced Stage
NCT00718809	PHASE2	TERMINATED	Saracatinib in Treating Patients With Relapsed or Refractory Thymoma or Thymic Cancer
NCT00818090	PHASE2	TERMINATED	Paclitaxel and Cisplatin for Thymic Neoplasm
NCT00965250	PHASE2	COMPLETED	Multicenter Phase II Study of IMC-A12 in Patients With Thymoma and Thymic Carcinoma Who Have Been Previously Treated With Chemotherapy
NCT01100944	PHASE1/PHASE2	TERMINATED	A Phase 1/2 Study of PXD101 (Belinostat) in Combination With Cisplatin, Doxorubicin and Cyclophosphamide in the First Line Treatment of Advanced or Recurrent Thymic, Malignancies
NCT01364727	PHASE2	COMPLETED	A Phase 2 Study of Amrubicin in Relapsed or Refractory Thymic Malignancies
NCT01621568	PHASE2	COMPLETED	Sunitinib for Advanced Thymus Cancer Following Earlier Treatment
NCT02062632	PHASE2	TERMINATED	Doxepin Hydrochloride in Treating Esophageal Pain in Patients With Thoracic Cancer Receiving Radiation Therapy to the Thorax With or Without Chemotherapy
NCT02220855	PHASE2	COMPLETED	A Study of BKM120 (Buparlisib) in Relapsed or Refractory Thymomas
NCT03193437	PHASE2	TERMINATED	Selinexor in Patients With Advanced Thymic Epithelial Tumor Progressing After Primary Chemotherapy
NCT03466827	PHASE2	UNKNOWN	Selinexor in Patients With Advanced Thymoma and Thymic Carcinoma
NCT03921671	PHASE2	UNKNOWN	Ramucirumab and Carbo-Paclitaxel for Untreated Thymic Carcinoma / B3 Thymoma With Carcinoma (RELEVENT)
NCT04371458	PHASE1/PHASE2	WITHDRAWN	Thymectomy With and Without Povidone-iodine Pleural Lavage in Stage IVA Thymic Malignancies
NCT05407649	PHASE2	UNKNOWN	Abscopal Effect of Radiotherapy in Combination With rhGM-CSF for Advanced Thymic Epithelial Tumours
NCT03295227	PHASE1	RECRUITING	Pembrolizumab in Treating Participants With Unresectable Thymoma or Thymic Cancer
NCT00921739	PHASE1	COMPLETED	Esophageal Sparing Intensity-modulated Radiation Therapy (IMRT) for Locally-Advanced Thoracic Malignancies
NCT01242072	PHASE1	UNKNOWN	Intravenous Palifosfamide-tris in Combination With Etoposide and Carboplatin in Patients With Malignancies
NCT01258868	PHASE1	TERMINATED	Tumor Cell Vaccines With ISCOMATRIX Adjuvant and Celecoxib in Patients Undergoing Resection of Lung and Esophageal Cancers and Malignant Pleural Mesotheliomas
NCT03023319	PHASE1	COMPLETED	Bosutinib in Combination With Pemetrexed in Patients With Selected Metastatic Solid Tumors
NCT03517488	PHASE1	COMPLETED	A Study of XmAb®20717 in Subjects With Selected Advanced Solid Tumors
NCT04375267	PHASE1	COMPLETED	177Lu-DOTA-TATE and Olaparib in Somatostatin Receptor Positive Tumours
NCT06086327	EARLY_PHASE1	UNKNOWN	Application of 68Ga-Pentixafor PET/CT for Thymoma
NCT01385722	Not specified	ENROLLING_BY_INVITATION	Molecular Analysis of Thoracic Malignancies
NCT01950572	Not specified	RECRUITING	Tissue Procurement and Natural History Study of Patients With Malignant Mesothelioma
NCT02633553	Not specified	RECRUITING	Adjuvant Radiotherapy for Stage II/III Thymoma After Complete Resection

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
AVELUMAB	4	1
BELINOSTAT	4	1
BOSUTINIB	4	1
DOXEPIN HYDROCHLORIDE	4	1
LEUCOVORIN	4	1
OCTREOTIDE	4	1
RAMUCIRUMAB	4	1
SACITUZUMAB GOVITECAN	4	1
SELINEXOR	4	1
AMRUBICIN	3	1
BINTRAFUSP ALFA	3	1
BUPARLISIB	3	1
SARACATINIB	3	1
VUDALIMAB	2	1
IMIFOPLATIN	1	1
CHEMBL3350037	0	1
CHEMBL4438584	0	1
CHEMBL1200462	0	1
CHEMBL4067978	0	1
FOLINIC ACID	0	1
PALIFOSFAMIDE TROMETHAMINE	-1	1