Paraganglioma
diseaseOn this page
Also known as chemodectomaneoplasm of paraganglionneoplasm of the paraganglionparagangliomasparaganglion neoplasmparaganglion tumorparaganglion tumourParaganglionic neoplasmParaganglionic tumorParaganglionic tumourtumor of paragangliontumor of the paragangliontumour of paragangliontumour of the paraganglion
Summary
Paraganglioma (MONDO:0000448) is a disease (an umbrella term covering 12 Mondo subtypes) with 3 cohort genes and 77 clinical trials. The dominant Reactome pathway is Maturation of TCA enzymes and regulation of TCA cycle (3 cohort genes). Top therapeutic interventions include phenoxybenzamine, edotreotide gallium ga-68, and cabozantinib.
At a glance
- Umbrella term: 12 Mondo subtypes
- Cohort genes: 3
- ClinVar variants: 7
- Clinical trials: 77
Clinical features
No curated clinical features (Orphanet) for this disease.
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | paraganglioma |
| Mondo ID | MONDO:0000448 |
| EFO | EFO:1000453 |
| MeSH | D010235 |
| DOID | DOID:0050773 |
| NCIT | C3308 |
| SNOMED CT | 127027008 |
| UMLS | C0030421 |
| MedGen | 10571 |
| GARD | 0022770 |
| Anatomy (UBERON) | UBERON:0002410 |
| Is cancer (heuristic) | no |
Also known as: chemodectoma · neoplasm of paraganglion · neoplasm of the paraganglion · paraganglioma · paragangliomas · paraganglion neoplasm · paraganglion tumor · paraganglion tumour · Paraganglionic neoplasm · Paraganglionic tumor · Paraganglionic tumour · tumor of paraganglion · tumor of the paraganglion · tumour of paraganglion · tumour of the paraganglion
Data availability: 7 ClinVar variants · 11 intOGen driver records.
Disease family
An umbrella term covering 12 Mondo subtypes.
Classification path: disease › human disease › disease by body system or component › nervous system disorder › central nervous system disorder › autonomic nervous system disorder › autonomic nervous system neoplasm › paraganglioma
Related subtypes (2): sympathetic neurilemmoma, ganglioneuroma
Subtypes (12): head and neck paraganglioma, pheochromocytoma/paraganglioma syndrome 4, pheochromocytoma/paraganglioma syndrome 1, pheochromocytoma/paraganglioma syndrome 2, pheochromocytoma/paraganglioma syndrome 3, pheochromocytoma/paraganglioma syndrome 5, sporadic pheochromocytoma/secreting paraganglioma, non-secreting paraganglioma, parasympathetic paraganglioma, sympathetic paraganglioma, pheochromocytoma/paraganglioma syndrome 6, pheochromocytoma/paraganglioma syndrome 7
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
ClinVar germline variants
7 retrieved; paginated sample, class counts are floors:
3 pathogenic/likely pathogenic, 2 conflicting classifications of pathogenicity, 1 benign/likely benign, 1 likely pathogenic
| ClinVar | Variant (HGVS) | Gene | Classification | Review |
|---|---|---|---|---|
| 239647 | NM_004168.4(SDHA):c.1432_1432+1del | SDHA | Pathogenic/Likely pathogenic | criteria provided, multiple submitters, no conflicts |
| 1687624 | NM_003000.3(SDHB):c.206G>T (p.Gly69Val) | SDHB | Pathogenic/Likely pathogenic | criteria provided, multiple submitters, no conflicts |
| 231390 | NM_003002.4(SDHD):c.147dup (p.His50fs) | SDHD | Pathogenic/Likely pathogenic | criteria provided, multiple submitters, no conflicts |
| 428929 | NM_003000.3(SDHB):c.744C>G (p.Asn248Lys) | SDHB | Likely pathogenic | criteria provided, multiple submitters, no conflicts |
| 472322 | NM_004168.4(SDHA):c.1334C>T (p.Ser445Leu) | SDHA | Conflicting classifications of pathogenicity | criteria provided, conflicting classifications |
| 1687625 | NM_003002.4(SDHD):c.242C>G (p.Pro81Arg) | SDHD | Conflicting classifications of pathogenicity | criteria provided, conflicting classifications |
| 36768 | NM_003000.3(SDHB):c.32G>A (p.Arg11His) | SDHB | Benign/Likely benign | criteria provided, multiple submitters, no conflicts |
Genes & proteins
Mendelian disease overlap and somatic drivers
GenCC: 0 · Orphanet: 19 · OMIM-shared: 0 · Dual-evidence (GWAS+Mendelian): 0
Orphanet rare-disease linkage (cohort genes)
| Gene | Orphanet ID | Rare disease |
|---|---|---|
| SDHB | Orphanet:139411 | Carney triad |
| SDHB | Orphanet:201 | Cowden syndrome |
| SDHB | Orphanet:276621 | Sporadic pheochromocytoma/secreting paraganglioma |
| SDHB | Orphanet:29072 | Hereditary pheochromocytoma-paraganglioma |
| SDHB | Orphanet:3208 | Isolated succinate-CoQ reductase deficiency |
| SDHB | Orphanet:44890 | Gastrointestinal stromal tumor |
| SDHB | Orphanet:97286 | Carney-Stratakis syndrome |
| SDHA | Orphanet:139411 | Carney triad |
| SDHA | Orphanet:154 | Familial isolated dilated cardiomyopathy |
| SDHA | Orphanet:29072 | Hereditary pheochromocytoma-paraganglioma |
| SDHA | Orphanet:3208 | Isolated succinate-CoQ reductase deficiency |
| SDHA | Orphanet:44890 | Gastrointestinal stromal tumor |
| SDHA | Orphanet:97286 | Carney-Stratakis syndrome |
| SDHD | Orphanet:100093 | Carcinoid syndrome |
| SDHD | Orphanet:201 | Cowden syndrome |
| SDHD | Orphanet:276621 | Sporadic pheochromocytoma/secreting paraganglioma |
| SDHD | Orphanet:29072 | Hereditary pheochromocytoma-paraganglioma |
| SDHD | Orphanet:3208 | Isolated succinate-CoQ reductase deficiency |
| SDHD | Orphanet:97286 | Carney-Stratakis syndrome |
Cohort genes → proteins
3 cohort genes, 3 distinct canonical proteins.
Evidence partition
| Subset | Genes |
|---|---|
| multi_evidence | 3 |
Cohort genes (full)
| Symbol | HGNC | Ensembl | UniProt | Name | Evidence |
|---|---|---|---|---|---|
| SDHB | HGNC:10681 | ENSG00000117118 | P21912 | Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial | clinvar,civic_evidence |
| SDHA | HGNC:10680 | ENSG00000073578 | P31040 | Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial | clinvar |
| SDHD | HGNC:10683 | ENSG00000204370 | O14521 | Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial | clinvar |
Cohort function summary
Lead sentence per gene, UniProt-curated.
| Symbol | Protein name | Function (lead sentence) |
|---|---|---|
| SDHB | Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial | Iron-sulfur protein (IP) subunit of the succinate dehydrogenase complex (mitochondrial respiratory chain complex II), responsible for transferring electrons from succinate to ubiquinone (coenzyme Q). |
| SDHA | Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial | Flavoprotein (FP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q). |
| SDHD | Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial | Membrane-anchoring subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q). |
Protein-family classification
Druggable: 1 · Difficult: 0 · Unknown: 2 · Druggable fraction: 0.33
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 |
|---|---|---|---|
| Enzyme (other) | 1 | 4.0× | 0.460 |
| Other/Unknown | 2 | 1.2× | 0.587 |
Per-gene assignment
| Symbol | Family | Druggable? | EC | InterPro (top 3) |
|---|---|---|---|---|
| SDHB | Enzyme (other) | yes | 1.3.5.1 | 2Fe-2S_ferredoxin-type, Succ_DH/fum_Rdtase_Fe-S, 2Fe2S_fd_BS |
| SDHA | Other/Unknown | no | FRD_SDH_FAD_BS, FAD-dep_OxRdtase_2_FAD-bd, Succ_DH_flav_su_fwd | |
| SDHD | Other/Unknown | no | CybS, SQR/QFR_C/D |
Expression context
Cohort genes with no expression data: 0.
3 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) | 3 |
| unknown | 0 |
Top tissues across cohort
| Tissue | Cohort genes |
|---|---|
| apex of heart | 2 |
| heart left ventricle | 2 |
| cardiac ventricle | 1 |
| mucosa of transverse colon | 1 |
| jejunal mucosa | 1 |
| jejunum | 1 |
| rectum | 1 |
Per-gene tissue summary (top 30)
| Symbol | Bgee breadth | FANTOM5 breadth | SCXA | Top tissues |
|---|---|---|---|---|
| SDHB | 293 | ubiquitous | marker | heart left ventricle, cardiac ventricle, apex of heart |
| SDHA | 143 | ubiquitous | marker | apex of heart, heart left ventricle, mucosa of transverse colon |
| SDHD | 287 | ubiquitous | marker | jejunal mucosa, rectum, jejunum |
Protein interactions among cohort
Intra-cohort edges: 3.
Hub genes (top 10 by interactor count)
| Symbol | Interactor count |
|---|---|
| SDHA | 6,141 |
| SDHB | 5,471 |
| SDHD | 2,229 |
Intra-cohort edges
| A | B | Sources |
|---|---|---|
| SDHA | SDHB | biogrid_interaction, intact, string_interaction |
| SDHA | SDHD | string_interaction |
| SDHB | SDHD | biogrid_interaction, string_interaction |
Structural data
PDB: 3 · AlphaFold-only: 0 · No structure: 0
Cohort genes with PDB structures (top 30)
| Symbol | UniProt | PDB entries |
|---|---|---|
| SDHB | P21912 | 6 |
| SDHA | P31040 | 5 |
| SDHD | O14521 | 2 |
Function
Pathway analysis
Distinct Reactome pathways touched by cohort: 5. Enrichment computed across 3 evidence-associated genes (3 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 3 annotated cohort genes). Counts and members are kept as ground-truth; sorted by enrichment.
| Pathway | Cohort genes | Fold | FDR | Sample cohort genes |
|---|---|---|---|---|
| Maturation of TCA enzymes and regulation of TCA cycle | 3 | 571.0× | 2e-08 | SDHB, SDHA, SDHD |
| Citric acid cycle (TCA cycle) | 3 | 423.0× | 3e-08 | SDHB, SDHA, SDHD |
| Respiratory electron transport | 3 | 95.2× | 2e-06 | SDHB, SDHA, SDHD |
| Aerobic respiration and respiratory electron transport | 2 | 59.0× | 5e-04 | SDHB, SDHA |
| Metabolism | 2 | 7.7× | 0.021 | SDHB, SDHA |
GO biological processes by enrichment
Over-representation of cohort genes vs the genome-wide background (hypergeometric test, Benjamini-Hochberg FDR; fold = observed/expected over 3 annotated cohort genes). Counts and members are kept as ground-truth; sorted by enrichment.
| GO term | Cohort genes | Fold | FDR | Sample cohort genes |
|---|---|---|---|---|
| mitochondrial electron transport, succinate to ubiquinone | 3 | 3370.4× | 1e-10 | SDHB, SDHA, SDHD |
| tricarboxylic acid cycle | 3 | 510.7× | 3e-08 | SDHB, SDHA, SDHD |
| proton motive force-driven mitochondrial ATP synthesis | 3 | 263.3× | 2e-07 | SDHB, SDHA, SDHD |
| succinate metabolic process | 2 | 2246.9× | 5e-07 | SDHB, SDHA |
| respiratory electron transport chain | 2 | 561.7× | 7e-06 | SDHB, SDHA |
| regulation of catecholamine secretion | 1 | 5617.3× | 3e-04 | SDHD |
| aerobic respiration | 1 | 82.6× | 0.016 | SDHB |
| cellular response to hypoxia | 1 | 40.4× | 0.028 | SDHD |
| nervous system development | 1 | 15.3× | 0.064 | SDHA |
Therapeutics
Drugs indicated for this disease
0 approved, 16 in late-stage (phase 3) trials. Disease-direct ChEMBL indications, not inferred from the associated-gene cohort below.
| Drug | Development status |
|---|---|
| Albumin Human | Phase 3 (in late-stage trials) |
| Aminolevulinic Acid | Phase 3 (in late-stage trials) |
| Ascorbic Acid | Phase 3 (in late-stage trials) |
| Bevacizumab | Phase 3 (in late-stage trials) |
| Carboplatin | Phase 3 (in late-stage trials) |
| Carmustine | Phase 3 (in late-stage trials) |
| Dextroamphetamine | Phase 3 (in late-stage trials) |
| Dianhydrogalactitol | Phase 3 (in late-stage trials) |
| Dordaviprone | Phase 3 (in late-stage trials) |
| Doxazosin | Phase 3 (in late-stage trials) |
| Lomustine | Phase 3 (in late-stage trials) |
| Paclitaxel | Phase 3 (in late-stage trials) |
| Phenoxybenzamine | Phase 3 (in late-stage trials) |
| Temozolomide | Phase 3 (in late-stage trials) |
| Tranexamic Acid | Phase 3 (in late-stage trials) |
| Vorasidenib | Phase 3 (in late-stage trials) |
Earlier-phase candidates (phase 2, investigational — efficacy not yet established): ANG1005, ANTINEOPLASTON A10, Abemaciclib, Afatinib, Avelumab, Axitinib, Azacitidine, Bortezomib, Capecitabine, Celecoxib, Cilengitide, Cisplatin, Copper, Crenolanib, Dasatinib Anhydrous, Disulfiram, Doxorubicin, Edotreotide, Epacadostat, Etoposide, Everolimus, Infigratinib, Influenza Virus Vaccine, Iobenguane, Irinotecan, Ivosidenib, Keyhole Limpet Hemocyanin, Lacosamide, Lanreotide, Lapatinib, Lenvatinib, Levetiracetam, Marizomib, Mirtazapine, Nilotinib, Nimotuzumab, Nivolumab, PEGINTERFERON ALFA-2B, Pazopanib, Pembrolizumab, Penpulimab, Retifanlimab, Rivoceranib, Selinexor, Sirolimus, Tetanus Toxoid, Thalidomide, Tipifarnib, Vinblastine, Vismodegib.
Drug target analysis
Approved (phase 4): 1 · Phase ≥3: 1 · Phased (≥1): 1 · Undrugged: 2
Druggability breadth: 2 of 3 evidence-associated genes (67%) 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 |
|---|---|
| SDHA | LINEZOLID |
Top cohort targets by molecule count
| Symbol | Molecules | Max phase |
|---|---|---|
| SDHA | 1 | 4 |
| SDHB | 0 | 0 |
| SDHD | 0 | 0 |
Drugs targeting cohort genes (top 30)
| Molecule | Max phase | Targets in cohort |
|---|---|---|
| LINEZOLID | 4 | SDHA |
Bioactivity and enzyme data
Enzyme cohort genes (≥1 EC): 1.
Cohort genes with ChEMBL bioactivity (full, sorted by assay count)
| Symbol | Assays | Type breakdown |
|---|---|---|
| SDHB | 4 | Binding:4 |
| SDHA | 3 | Binding:3 |
Cohort enzymes (BRENDA EC)
| Symbol | EC numbers | Names |
|---|---|---|
| SDHB | 1.3.5.1 | succinate dehydrogenase |
Pharmacogenomics
Cohort genes with a PharmGKB record: 3; with CPIC/DPWG dosing guidelines: 0.
No cohort gene has a CPIC/DPWG genotype-guided dosing guideline (PharmGKB).
Chemical tractability of cohort targets
1 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) |
|---|---|---|
| LINEZOLID | 4 | SDHA |
Druggability pyramid
Cohort genes binned by druggability tier (high → low):
| Tier | Definition | Genes | Symbols |
|---|---|---|---|
| A | Approved (phase 4 drug) | 1 | SDHA |
| B | Phased (≥1) drug, not yet approved | 0 | |
| C | Druggable family + PDB, no drug | 1 | SDHB |
| D | Druggable family + AlphaFold only, no drug | 0 | |
| E | Difficult family or no structure, no drug | 1 | SDHD |
Undrugged target profiles
2 cohort genes are undrugged. Ranked by ‘starting-point quality’ (assay depth + drugged-partner adjacency).
| Symbol | ChEMBL assays | Drugged partners (top 3) |
|---|---|---|
| SDHB | 4 | SDHA |
| SDHD | 0 | SDHA |
Clinical trials & evidence
Clinical trials
Clinical trials: 77.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| Not specified | 34 |
| PHASE2 | 25 |
| PHASE1/PHASE2 | 7 |
| PHASE1 | 5 |
| PHASE4 | 2 |
| PHASE3 | 2 |
| EARLY_PHASE1 | 2 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT04081701 | PHASE4 | RECRUITING | 68-Ga DOTATATE PET/MRI in the Diagnosis and Management of Somatostatin Receptor Positive CNS Tumors. |
| NCT05702944 | PHASE4 | RECRUITING | The Effect and Safety of Omitting Preoperative Alpha-adrenergic Blockade for Normotensive Pheochromocytoma |
| NCT01373736 | PHASE3 | UNKNOWN | 123I-MIBG Scintigraphy in Patients Being Evaluated for Neuroendocrine Tumors |
| NCT03176693 | PHASE3 | COMPLETED | Preoperative Alpha Blockade for Pheochromocytoma |
| NCT02721732 | PHASE2 | ACTIVE_NOT_RECRUITING | Pembrolizumab in Treating Patients With Rare Tumors That Cannot Be Removed by Surgery or Are Metastatic |
| NCT02834013 | PHASE2 | ACTIVE_NOT_RECRUITING | Nivolumab and Ipilimumab in Treating Patients With Rare Tumors |
| NCT03206060 | PHASE2 | RECRUITING | Lu-177-DOTATATE (Lutathera) in Therapy of Inoperable Pheochromocytoma/ Paraganglioma |
| NCT03839498 | PHASE2 | ACTIVE_NOT_RECRUITING | Study of Axitinib (AG-013736) With Evaluation of the VEGF-pathway in Pheochromocytoma/Paraganglioma |
| NCT03946527 | PHASE2 | ACTIVE_NOT_RECRUITING | LAnreotide in Metastatic Pheochromocytoma / PARAganglioma (LAMPARA) |
| NCT04394858 | PHASE2 | ACTIVE_NOT_RECRUITING | Testing the Addition of an Anticancer Drug, Olaparib, to the Usual Chemotherapy (Temozolomide) for Advanced Neuroendocrine Cancer |
| NCT04711135 | PHASE2 | ACTIVE_NOT_RECRUITING | Study to Evaluate Safety and Dosimetry of Lutathera in Adolescent Patients With GEP-NETs and PPGLs |
| NCT05142241 | PHASE2 | ACTIVE_NOT_RECRUITING | Testing the Combination of Anti-Cancer Drugs Talazoparib and Temozolomide in Patients With Advanced Stage Rare Cancers, RARE 2 Trial |
| NCT05636618 | PHASE1/PHASE2 | RECRUITING | Targeted Alpha-Particle Therapy for Advanced Somatostatin Receptor Type 2 (SSTR2) Positive Tumors |
| NCT06045260 | PHASE2 | RECRUITING | Receptor Radionuclide Therapy With 177Lu-DOTATOC |
| NCT06121271 | PHASE2 | NOT_YET_RECRUITING | Trial of Lu-177 DOTATATE (Lutathera®) in Unlicensed Indications |
| NCT06427798 | PHASE1/PHASE2 | RECRUITING | Somatostatin-Receptors (SSTR)-Agonist [212Pb]VMT-alpha-NET in Metastatic or Inoperable SSTR+ Gastrointestinal Neuroendocrine Tumor and Pheochromocytoma/Paraganglioma Previously Treated With Systemic Targeted Radioligand Therapy |
| NCT06607692 | PHASE1/PHASE2 | RECRUITING | Study in Children and Adolescents of 177Lu-DOTATATE (Lutathera®) Combined With the PARP Inhibitor Olaparib for the Treatment of Recurrent or Relapsed Solid Tumours Expressing Somatostatin Receptor (SSTR) (LuPARPed). |
| NCT00458952 | PHASE1/PHASE2 | COMPLETED | Phase 1 Study of Iobenguane (MIBG) I 131 in Patients With Malignant Pheochromocytoma/Paraganglioma |
| NCT00843037 | PHASE2 | COMPLETED | Study Of Sunitinib In Patients With Recurrent Paraganglioma/Pheochromocytoma |
| NCT00874614 | PHASE2 | UNKNOWN | A Study Evaluating Ultratrace Iobenguane I131 in Patients With Malignant Relapsed/Refractory Pheochromocytoma/Paraganglioma |
| NCT01340794 | PHASE2 | TERMINATED | Pazopanib Hydrochloride in Treating Patients With Advanced or Progressive Malignant Pheochromocytoma or Paraganglioma |
| NCT01413503 | PHASE2 | COMPLETED | A Phase II Study of 131I- Metaiodobenzylguanidine (MIBG) for Treatment of Metastatic or Unresectable Pheochromocytoma and Related Tumors |
| NCT01560260 | PHASE2 | COMPLETED | Linsitinib in Treating Patients With Gastrointestinal Stromal Tumors |
| NCT01635907 | PHASE2 | COMPLETED | Dovitinib in Neuroendocrine Tumors |
| NCT01967576 | PHASE2 | COMPLETED | Phase II Study of Axitinib (AG-013736) With Evaluation of the VEGF-pathway in Metastatic, Recurrent or Primary Unresectable Pheochromocytoma/Paraganglioma |
| NCT02177773 | PHASE1/PHASE2 | TERMINATED | GA-68 DOTA-TOC of Somatostatin Positive Malignancies |
| NCT02302833 | PHASE2 | COMPLETED | Cabozantinib S-malate in Treating Patients With Metastatic Pheochromocytomas or Paragangliomas That Cannot Be Removed by Surgery |
| NCT03165721 | PHASE2 | TERMINATED | A Phase II Trial of the DNA Methyl Transferase Inhibitor, Guadecitabine (SGI-110), in Children and Adults With Wild Type GIST,Pheochromocytoma and Paraganglioma Associated With Succinate Dehydrogenase Deficiency and HLRCC-associated Kidney Cancer |
| NCT03923257 | PHASE1/PHASE2 | WITHDRAWN | Dosimetry Guided PRRT With 177Lu-DOTATATE in Children and Adolescents |
| NCT04106843 | PHASE2 | WITHDRAWN | Radioactive Drug (177Lu-DOTATATE) for the Treatment of Locally Advanced, Metastatic, or Unresectable Rare Endocrine Cancers |
| NCT04187404 | PHASE1/PHASE2 | TERMINATED | A Novel Therapeutic Vaccine (EO2401) in Metastatic Adrenocortical Carcinoma, or Malignant Pheochromocytoma/Paraganglioma |
| NCT04276597 | PHASE2 | WITHDRAWN | Phase-II Study of Lu177DOTATOC in Adults With STTR(+)Pulmonary, Pheochromocytoma, Paraganglioma, Unknown Primary, Thymus NETs (PUTNET), or Any Other Non-.GEP-NET. |
| NCT04400474 | PHASE2 | COMPLETED | Trial of Cabozantinib Plus Atezolizumab in Advanced and Progressive Neoplasms of the Endocrine System. The CABATEN Study |
| NCT05883085 | PHASE2 | UNKNOWN | A Study on the Safety and Effectiveness of Anlotinib for Neoadjuvant Treatment of PPGL |
| NCT05885386 | PHASE2 | UNKNOWN | A Study on the Safety and Effectiveness of Temozolomide for Neoadjuvant Treatment of PPGL |
| NCT06233903 | PHASE2 | WITHDRAWN | 18F-mFBG Expression in Neural Crest Tumors and Organs Innervated by the Sympathetic Nervous System |
| NCT04119024 | PHASE1 | RECRUITING | Gene Modified Immune Cells After Conditioning Regimen for the Treatment of Stage IIIC or IV Melanoma or Metastatic Solid Tumors |
| NCT00339131 | PHASE1 | COMPLETED | ULTRATRACE Iobenguane I 131 in Patients With Malignant Pheochromocytoma, Paraganglioma, or Metastatic Carcinoid |
| NCT01155258 | PHASE1 | COMPLETED | Temsirolimus and Vinorelbine Ditartrate in Treating Patients With Unresectable or Metastatic Solid Tumors |
| NCT01204476 | PHASE1 | COMPLETED | Cixutumumab, Everolimus, and Octreotide Acetate in Treating Patients With Advanced Low to Intermediate Grade Neuroendocrine Carcinoma |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| PHENOXYBENZAMINE | 4 | 6 |
| EDOTREOTIDE GALLIUM GA-68 | 4 | 5 |
| CABOZANTINIB | 4 | 3 |
| DOXAZOSIN | 4 | 3 |
| LUTETIUM OXODOTREOTIDE LU-177 | 4 | 3 |
| AXITINIB | 4 | 2 |
| IOBENGUANE I 131 | 4 | 2 |
| LANREOTIDE | 4 | 1 |
| OCTREOTIDE ACETATE | 4 | 1 |
| PAZOPANIB HYDROCHLORIDE | 4 | 1 |
| PIFLUFOLASTAT F18 | 4 | 1 |
| VANDETANIB | 4 | 1 |
| FLORBENGUANE F18 | 3 | 2 |
| CATEQUENTINIB HYDROCHLORIDE | 3 | 1 |
| DOVITINIB | 3 | 1 |
| GUADECITABINE | 3 | 1 |
| LINSITINIB | 3 | 1 |
| LUTETIUM LU177 EDOTREOTIDE | 2 | 2 |
| CIXUTUMUMAB | 2 | 1 |
| TIGOZERTINIB | 2 | 1 |
| CHEMBL3939307 | 0 | 2 |
| CHEMBL275117 | 0 | 1 |
| CHEMBL4517714 | 0 | 1 |
| CHEMBL5405436 | 0 | 1 |
| CHEMBL4578973 | 0 | 1 |
| CHEMBL3764816 | 0 | 1 |
| CHEMBL5724798 | 0 | 1 |
| CHEMBL5572229 | 0 | 1 |
Related Atlas pages
- Cohort genes: SDHB, SDHA, SDHD
- Drugs: Phenoxybenzamine, EDOTREOTIDE GALLIUM GA-68, Cabozantinib, Doxazosin, LUTETIUM OXODOTREOTIDE LU-177, Axitinib, IOBENGUANE I 131, Lanreotide, Octreotide Acetate, Pazopanib, PIFLUFOLASTAT F18, Vandetanib, FLORBENGUANE F18, Catequentinib, Dovitinib, Guadecitabine, Linsitinib