Peritoneal mesothelioma

Summary

Peritoneal mesothelioma (MONDO:0006362) is a disease (an umbrella term covering 5 Mondo subtypes) with 2 cohort genes and 18 clinical trials. Molecularly, EGFR Mutation confers sensitivity to Cytoreductive Surgery + Hyperthermic Intraperitoneal Chemotherapy in Peritoneal Mesothelioma (CIViC Level B); 1 further subtype–drug associations are mapped below. Top therapeutic interventions include mitomycin, sodium thiosulfate, and domvanalimab.

At a glance

• Umbrella term: 5 Mondo subtypes
• Cohort genes: 2
• Clinical trials: 18
• Precision-medicine evidence (CIViC): 2 subtype–drug associations

Clinical features

No curated clinical features (Orphanet) for this disease.

Identifiers

Disease identifiers

Field	Value
Canonical name	peritoneal mesothelioma
Mondo ID	MONDO:0006362
EFO	EFO:1000467
ICD-10-CM	C45.1
NCIT	C7633
SNOMED CT	109853004
UMLS	C1377610
MedGen	237171
Anatomy (UBERON)	UBERON:0002358
Is cancer (heuristic)	no

Also known as: mesothelioma of peritoneum · mesothelioma of the peritoneum · peritoneal mesothelioma · peritoneal mesothelioma (disease) · peritoneum mesothelioma

Data availability: 1 HPO phenotype.

Disease family

An umbrella term covering 5 Mondo subtypes.

Classification path: disease › human disease › disease by etiologic mechanism › cancer or benign tumor › neoplastic disease or syndrome › neoplasm › mesothelial neoplasm › mesothelioma › peritoneal mesothelioma

Related subtypes (6): benign mesothelioma, pleural mesothelioma, well differentiated papillary mesothelioma, adenomatoid tumor, malignant mesothelioma, well-differentiated papillary mesothelial tumour of the pleura

Subtypes (5): benign peritoneal mesothelioma, malignant peritoneal mesothelioma, peritoneal multicystic mesothelioma, peritoneal well differentiated papillary mesothelioma, peritoneal mesothelioma in situ

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

Mendelian disease overlap and somatic drivers

GenCC: 0 · Orphanet: 6 · OMIM-shared: 0 · Dual-evidence (GWAS+Mendelian): 0

Orphanet rare-disease linkage (cohort genes)

Gene	Orphanet ID	Rare disease
EGFR	Orphanet:251576	Gliosarcoma
EGFR	Orphanet:251579	Giant cell glioblastoma
NF2	Orphanet:2495	Meningioma
NF2	Orphanet:634475	Mosaic NF2-related schwannomatosis
NF2	Orphanet:637	Full NF2-related schwannomatosis
NF2	Orphanet:93921	Full schwannomatosis

Cohort genes → proteins

2 cohort genes, 2 distinct canonical proteins.

Evidence partition

Subset	Genes
civic_only	2

Cohort genes (full)

Symbol	HGNC	Ensembl	UniProt	Name	Evidence
EGFR	HGNC:3236	ENSG00000146648	P00533	Epidermal growth factor receptor	civic_evidence
NF2	HGNC:7773	ENSG00000186575	P35240	Merlin	civic_evidence

Cohort function summary

Lead sentence per gene, UniProt-curated.

Symbol	Protein name	Function (lead sentence)
EGFR	Epidermal growth factor receptor	Receptor tyrosine kinase binding ligands of the EGF family and activating several signaling cascades to convert extracellular cues into appropriate cellular responses.
NF2	Merlin	Probable regulator of the Hippo/SWH (Sav/Wts/Hpo) signaling pathway, a signaling pathway that plays a pivotal role in tumor suppression by restricting proliferation and promoting apoptosis.

Protein-family classification

Druggable: 1 · Difficult: 0 · Unknown: 1 · Druggable fraction: 0.5

Family distribution

Cohort families vs a genome-wide background (hypergeometric, BH-FDR; fold = observed/expected). Counts kept; sorted by enrichment, so the catch-all Other/Unknown bucket no longer leads.

Family	Genes	Fold	FDR
Kinase	1	13.9×	0.142
Other/Unknown	1	0.9×	0.805

Per-gene assignment

Symbol	Family	Druggable?	EC	InterPro (top 3)
EGFR	Kinase	yes	2.7.10.1	Rcpt_L-dom, Prot_kinase_dom, Ser-Thr/Tyr_kinase_cat_dom
NF2	Other/Unknown	no		FERM_domain, Ez/rad/moesin-like, Moesin_tail_sf

Expression context

Cohort genes with no expression data: 0.

2 cohort genes are a single-cell marker in ≥1 SCXA experiment.

Breadth distribution (Bgee present_calls)

Bucket	Genes
narrow (1-5 tissues)	0
moderate (6-20)	0
broad (>20)	2
unknown	0

Top tissues across cohort

Tissue	Cohort genes
gingiva	1
gingival epithelium	1
nipple	1
dorsal motor nucleus of vagus nerve	1
endometrium epithelium	1
stromal cell of endometrium	1

Per-gene tissue summary (top 30)

Symbol	Bgee breadth	FANTOM5 breadth	SCXA	Top tissues
EGFR	285	ubiquitous	marker	nipple, gingiva, gingival epithelium
NF2	283	ubiquitous	marker	endometrium epithelium, stromal cell of endometrium, dorsal motor nucleus of vagus nerve

Protein interactions among cohort

Intra-cohort edges: 0.

Hub genes (top 10 by interactor count)

Symbol	Interactor count
EGFR	18,421
NF2	3,208

Structural data

PDB: 2 · AlphaFold-only: 0 · No structure: 0

Cohort genes with PDB structures (top 30)

Symbol	UniProt	PDB entries
EGFR	P00533	388
NF2	P35240	6

Function

Pathway analysis

Distinct Reactome pathways touched by cohort: 46. Enrichment computed across 2 evidence-associated genes (2 with Reactome annotation).

Pathways by enrichment

Over-representation of cohort genes vs the genome-wide background (hypergeometric test, Benjamini-Hochberg FDR; fold = observed/expected over 2 annotated cohort genes). Counts and members are kept as ground-truth; sorted by enrichment.

Pathway	Cohort genes	Fold	FDR	Sample cohort genes
PLCG1 events in ERBB2 signaling	1	1427.5×	0.008	EGFR
PTK6 promotes HIF1A stabilization	1	815.7×	0.008	EGFR
Inhibition of Signaling by Overexpressed EGFR	1	634.4×	0.008	EGFR
EGFR interacts with phospholipase C-gamma	1	571.0×	0.008	EGFR
EGFR Transactivation by Gastrin	1	571.0×	0.008	EGFR
ERBB2 Activates PTK6 Signaling	1	407.9×	0.008	EGFR
GRB2 events in EGFR signaling	1	380.7×	0.008	EGFR
TFAP2 (AP-2) family regulates transcription of growth factors and their receptors	1	380.7×	0.008	EGFR
SHC1 events in EGFR signaling	1	356.9×	0.008	EGFR
Constitutive Signaling by EGFRvIII	1	356.9×	0.008	EGFR
ERBB2 Regulates Cell Motility	1	356.9×	0.008	EGFR
PI3K events in ERBB2 signaling	1	335.9×	0.008	EGFR
Signaling by ERBB2 ECD mutants	1	335.9×	0.008	EGFR
GAB1 signalosome	1	317.2×	0.008	EGFR
GRB2 events in ERBB2 signaling	1	317.2×	0.008	EGFR
Constitutive Signaling by Ligand-Responsive EGFR Cancer Variants	1	285.5×	0.008	EGFR
RHO GTPases activate PAKs	1	271.9×	0.008	NF2
Developmental Lineage of Mammary Gland Myoepithelial Cells	1	271.9×	0.008	EGFR
Signal transduction by L1	1	259.6×	0.008	EGFR
Respiratory syncytial virus (RSV) attachment and entry	1	248.3×	0.008	EGFR
SHC1 events in ERBB2 signaling	1	237.9×	0.008	EGFR
NOTCH3 Activation and Transmission of Signal to the Nucleus	1	237.9×	0.008	EGFR
Signaling by ERBB2 TMD/JMD mutants	1	237.9×	0.008	EGFR
Estrogen-dependent nuclear events downstream of ESR-membrane signaling	1	219.6×	0.009	EGFR
Signaling by ERBB2 KD Mutants	1	211.5×	0.009	EGFR
Downregulation of ERBB2 signaling	1	190.3×	0.009	EGFR
Signaling by ERBB2	1	173.0×	0.009	EGFR
EGFR downregulation	1	173.0×	0.009	EGFR
Signaling by EGFR	1	163.1×	0.010	EGFR
Fcgamma receptor (FCGR) dependent phagocytosis	1	139.3×	0.011	NF2

GO biological processes by enrichment

Over-representation of cohort genes vs the genome-wide background (hypergeometric test, Benjamini-Hochberg FDR; fold = observed/expected over 2 annotated cohort genes). Counts and members are kept as ground-truth; sorted by enrichment.

GO term	Cohort genes	Fold	FDR	Sample cohort genes
negative regulation of cardiocyte differentiation	1	8426.0×	0.005	EGFR
Schwann cell proliferation	1	2808.7×	0.005	NF2
regulation of gliogenesis	1	2808.7×	0.005	NF2
positive regulation of protein kinase C signaling	1	2808.7×	0.005	EGFR
morphogenesis of an epithelial fold	1	2106.5×	0.005	EGFR
response to UV-A	1	2106.5×	0.005	EGFR
regulation of peptidyl-tyrosine phosphorylation	1	1685.2×	0.005	EGFR
negative regulation of cell growth involved in contact inhibition	1	1685.2×	0.005	NF2
regulation of organelle assembly	1	1685.2×	0.005	NF2
salivary gland morphogenesis	1	1203.7×	0.005	EGFR
negative regulation of Schwann cell proliferation	1	1203.7×	0.005	NF2
regulation of hippo signaling	1	1203.7×	0.005	NF2
protein insertion into membrane	1	1053.2×	0.005	EGFR
regulation of protein localization to nucleus	1	1053.2×	0.005	NF2
ubiquitin-dependent endocytosis	1	936.2×	0.005	EGFR
positive regulation of early endosome to late endosome transport	1	936.2×	0.005	NF2
ERBB2-EGFR signaling pathway	1	842.6×	0.005	EGFR
positive regulation of protein localization to early endosome	1	842.6×	0.005	NF2
regulation of neural precursor cell proliferation	1	842.6×	0.005	NF2
cerebral cortex cell migration	1	766.0×	0.005	EGFR
negative regulation of osteoblast proliferation	1	766.0×	0.005	NF2
osteoblast proliferation	1	702.2×	0.005	NF2
regulation of stem cell proliferation	1	702.2×	0.005	NF2
ectoderm development	1	601.9×	0.006	NF2
eyelid development in camera-type eye	1	526.6×	0.006	EGFR
lens fiber cell differentiation	1	526.6×	0.006	NF2
negative regulation of cell-cell adhesion	1	495.6×	0.006	NF2
digestive tract morphogenesis	1	495.6×	0.006	EGFR
negative regulation of cell-matrix adhesion	1	443.5×	0.006	NF2
negative regulation of receptor signaling pathway via JAK-STAT	1	443.5×	0.006	NF2

Therapeutics

Drug target analysis

Approved (phase 4): 1 · Phase ≥3: 1 · Phased (≥1): 1 · Undrugged: 1

Druggability breadth: 1 of 2 evidence-associated genes (50%) have a ChEMBL target (buckets above are over the deeply-mined display cohort).

Genes with an approved drug

The molecule shown is one approved compound that hits the gene — not necessarily a drug of choice or one indicated for this disease.

Symbol	Example approved molecule
EGFR	LEVODOPA

Top cohort targets by molecule count

Symbol	Molecules	Max phase
EGFR	175	4
NF2	0	0

Drugs targeting cohort genes (top 30)

Molecule	Max phase	Targets in cohort
LEVODOPA	4	EGFR
CLOTRIMAZOLE	4	EGFR
ERLOTINIB HYDROCHLORIDE	4	EGFR
CISPLATIN	4	EGFR
PONATINIB	4	EGFR
AFATINIB	4	EGFR
CHROMIC CHLORIDE	4	EGFR
BACITRACIN	4	EGFR
ZINC CHLORIDE	4	EGFR
LAPATINIB DITOSYLATE	4	EGFR
VEMURAFENIB	4	EGFR
FEDRATINIB	4	EGFR
AXITINIB	4	EGFR
SORAFENIB	4	EGFR
DASATINIB ANHYDROUS	4	EGFR
NICLOSAMIDE	4	EGFR
SELUMETINIB	4	EGFR
TERFENADINE	4	EGFR
ALECTINIB	4	EGFR
NERATINIB	4	EGFR
IBRUTINIB	4	EGFR
AFATINIB DIMALEATE	4	EGFR
CABOZANTINIB	4	EGFR
DACOMITINIB	4	EGFR
DACOMITINIB ANHYDROUS	4	EGFR
CERITINIB	4	EGFR
VANDETANIB	4	EGFR
TRIBROMSALAN	4	EGFR
BOSUTINIB	4	EGFR
BITHIONOL	4	EGFR

Bioactivity and enzyme data

Enzyme cohort genes (≥1 EC): 1.

Cohort genes with ChEMBL bioactivity (full, sorted by assay count)

Symbol	Assays	Type breakdown
EGFR	6,531	Binding:6211, Functional:173, ADMET:138, Toxicity:9

Cohort enzymes (BRENDA EC)

Symbol	EC numbers	Names
EGFR	2.7.10.1	receptor protein-tyrosine kinase

Cohort genes with high screening signal

≥100 ChEMBL assays — a studied-ness signal; see Therapeutics for approved-drug status.

Symbol	ChEMBL assays
EGFR	6,531

Pharmacogenomics

Cohort genes with a PharmGKB record: 2; with CPIC/DPWG dosing guidelines: 0.

No cohort gene has a CPIC/DPWG genotype-guided dosing guideline (PharmGKB).

Chemical tractability of cohort targets

30 approved/phased compounds have measured bioactivity against a cohort gene (and aren’t yet in disease-level trials). This is a research / tractability signal, NOT a therapeutic recommendation — a bioactivity row often reflects off-target or screening binding (e.g. promiscuous kinase inhibitors against a cohort kinase), implying no disease mechanism.

Compound	Max phase	Cohort target (bioactivity)
LEVODOPA	4	EGFR
CLOTRIMAZOLE	4	EGFR
ERLOTINIB HYDROCHLORIDE	4	EGFR
CISPLATIN	4	EGFR
PONATINIB	4	EGFR
AFATINIB	4	EGFR
CHROMIC CHLORIDE	4	EGFR
BACITRACIN	4	EGFR
ZINC CHLORIDE	4	EGFR
LAPATINIB DITOSYLATE	4	EGFR
VEMURAFENIB	4	EGFR
FEDRATINIB	4	EGFR
AXITINIB	4	EGFR
SORAFENIB	4	EGFR
DASATINIB ANHYDROUS	4	EGFR
NICLOSAMIDE	4	EGFR
SELUMETINIB	4	EGFR
TERFENADINE	4	EGFR
ALECTINIB	4	EGFR
NERATINIB	4	EGFR
IBRUTINIB	4	EGFR
AFATINIB DIMALEATE	4	EGFR
CABOZANTINIB	4	EGFR
DACOMITINIB	4	EGFR
DACOMITINIB ANHYDROUS	4	EGFR
CERITINIB	4	EGFR
VANDETANIB	4	EGFR
TRIBROMSALAN	4	EGFR
BOSUTINIB	4	EGFR
BITHIONOL	4	EGFR

Druggability pyramid

Cohort genes binned by druggability tier (high → low):

Tier	Definition	Genes	Symbols
A	Approved (phase 4 drug)	1	EGFR
B	Phased (≥1) drug, not yet approved	0
C	Druggable family + PDB, no drug	0
D	Druggable family + AlphaFold only, no drug	0
E	Difficult family or no structure, no drug	1	NF2

Undrugged target profiles

1 cohort genes are undrugged. Ranked by ‘starting-point quality’ (assay depth + drugged-partner adjacency).

Symbol	ChEMBL assays	Drugged partners (top 3)
NF2	0	—

Clinical trials & evidence

Clinical trials

Clinical trials: 18.

Phase distribution (across all retrieved trials)

Phase	Trials
PHASE2	9
Not specified	6
PHASE1	3

Top trials by phase / activity

NCT	Phase	Status	Title
NCT06057935	PHASE2	RECRUITING	A Study of Additional Chemotherapy After Surgery for People With Malignant Peritoneal Mesothelioma
NCT06790706	PHASE2	RECRUITING	IMMUNORARE5: A National Platform of 5 Academic Phase II Trials Coordinated by Lyon University Hospital to Assess the Safety and the Efficacy of the IMMUNOtherapy With Domvanalimab + Zimberelimab Combination in Patients With Advanced RARE Cancers
NCT01160458	PHASE2	COMPLETED	Phase II Study of IMC-A12 in Patients With Mesothelioma Who Have Been Previously Treated With Chemotherapy
NCT02040142	PHASE2	COMPLETED	HIPEC for Peritoneal Carcinomatosis
NCT02588131	PHASE2	UNKNOWN	A Study of Tremelimumab Combined With the Anti-PD-L1 MEDI4736 Antibody in Malignant Mesothelioma (NIBIT-MESO-1)
NCT03875144	PHASE2	SUSPENDED	Treatment of Malignant Peritoneal Mesothelioma (MESOTIP)
NCT04462809	PHASE2	UNKNOWN	Efficacy of a Maintenance Treatment With TALAzoparib Following First Line Platinum-based Chemotherapy in Malignant MESOthelioma
NCT05041062	PHASE2	COMPLETED	A Study of Immunotherapy Drugs Nivolumab and Ipilimumab in Patients w/Resectable Malignant Peritoneal Mesothelioma
NCT05337735	PHASE2	SUSPENDED	A Phase II Clinical Trial to Evaluate Safety and Efficacy of XmAb20717 in Advanced Rare Cancers
NCT04847063	PHASE1	RECRUITING	Individual Response to Hyperthermic Intraperitoneal Chemotherapy (HIPEC) Treatment of Peritoneal Carcinomatosis From Peritoneal Mesothelioma or Atypical Mesothelial Proliferation or From Ovarian, Colorectal, or Appendiceal Histologies
NCT07277413	PHASE1	RECRUITING	A Study of IDE892 as Monotherapy and Combination in MTAP-deleted Advanced Solid Tumors
NCT03608618	PHASE1	TERMINATED	Intraperitoneal MCY-M11 (Mesothelin-targeting CAR) for Treatment of Advanced Ovarian Cancer and Peritoneal Mesothelioma
NCT01617382	Not specified	RECRUITING	Register With Patients in Which Hyperthermic Intra-Peritoneal Chemotherapy (HIPEC) Was Performed
NCT07330271	Not specified	RECRUITING	PTC-Guided Therapy for Peritoneal Mesothelioma
NCT01812148	Not specified	COMPLETED	Survival of Peritoneal Mesothelioma After Cytoreductive Surgery and Hyperthermic Intra-peritoneal Chemotherapy (HIPEC)
NCT02834169	Not specified	UNKNOWN	French National Registry of Rare Peritoneal Surface Malignancies
NCT02834234	Not specified	COMPLETED	Genomic Analysis of Peritoneal Mesothelioma by CGH Arrays
NCT03867578	Not specified	COMPLETED	Magnetic Resonance Imaging for Detection of Peritoneal Mesothelioma

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
MITOMYCIN	4	2
SODIUM THIOSULFATE	4	1
DOMVANALIMAB	3	1
ZIMBERELIMAB	3	1
CIXUTUMUMAB	2	1
VUDALIMAB	2	1
CHEMBL4071382	0	2
CHEMBL5175144	0	2
CHEMBL3764816	0	1
CHEMBL5724798	0	1
CHEMBL3753202	0	1

Precision-medicine subtype map (CIViC)

Drug × molecular subtype: 2 predictive associations from 2 curated evidence items.

Molecular subtype	Therapy	Effect	Level	CIViC
EGFR Mutation	Cytoreductive Surgery + Hyperthermic Intraperitoneal Chemotherapy	Sensitivity/Response	CIViC B	EID5923
NF2 Y177fs	Carboplatin + Cisplatin	Resistance	CIViC C	EID649