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Disease
Paraganglioma
Active therapeutic pipelineEmerging research
1
Publications
21
Clinical trials
2024
Latest publication
Latest activity
betaRecent clinical, regulatory, research and industry developments relating to this disease.
The Immune Landscape of Pheochromocytoma and Paraganglioma: Current Advances and Perspectives.
Research2024-07-01Endocrine reviews
What's happening now
An analyst briefing on current research, clinical, regulatory and industry activity surrounding this disease.
Executive briefingUpdating summary…Momentum: Moderate
Key developments
- 5 clinical trials expected to report results, the earliest in Q1 2027.
Upcoming Milestones
- Q1 2027Exploratory Phase II Study of LAnreotide in Metastatic Pheochromocytoma/PARAganglioma (LAMPARA)
- Q1 2028A Prospective, Multi-Institutional Phase II Trial Evaluating Temozolomide vs. Temozolomide and Olaparib for Advanced Pheochromocytoma and Paraganglioma
- Q4 2028A Phase 2 Study of ONC206 in Advanced Pheochromocytoma and Paraganglioma
- Q4 2029A Phase 2 Study to Evaluate the Efficacy and Safety of Belzutifan (MK-6482, Formerly PT2977) Monotherapy in Participants With Advanced Pheochromocytoma/Paraganglioma (PPGL), Pancreatic Neuroendocrine Tumor (pNET), Von Hippel-Lindau (VHL) Disease-Associated Tumors, Advanced Gastrointestinal Stromal Tumor (wt GIST), or Advanced Solid Tumors With HIF-2α Related Genetic Alterations
- Q1 2030Lu-177-DOTATATE (Lutathera) in Therapy of Inoperable Pheochromocytoma/ Paraganglioma
Major developments
Upcoming trial readoutHigh impact
Results expected Q4 20292026-07-08
Upcoming trial readoutHigh impact
Results expected Q4 20282026-06-10
Clinical MilestonesViewHide
Results posted
Results expected
- 2026-07-08A Phase 2 Study to Evaluate the Efficacy and Safety of Belzutifan (MK-6482, Formerly PT2977) Monotherapy in Participants With Advanced Pheochromocytoma/Paraganglioma (PPGL), Pancreatic Neuroendocrine Tumor (pNET), Von Hippel-Lindau (VHL) Disease-Associated Tumors, Advanced Gastrointestinal Stromal Tumor (wt GIST), or Advanced Solid Tumors With HIF-2α Related Genetic AlterationsResults expected Q4 2029
- 2026-06-10A Phase 2 Study of ONC206 in Advanced Pheochromocytoma and ParagangliomaResults expected Q4 2028
- 2026-05-13A Prospective, Multi-Institutional Phase II Trial Evaluating Temozolomide vs. Temozolomide and Olaparib for Advanced Pheochromocytoma and ParagangliomaResults expected Q1 2028
- 2026-04-24Lu-177-DOTATATE (Lutathera) in Therapy of Inoperable Pheochromocytoma/ ParagangliomaResults expected Q1 2030
- 2026-03-02Exploratory Phase II Study of LAnreotide in Metastatic Pheochromocytoma/PARAganglioma (LAMPARA)Results expected Q1 2027
Activity timeline6
- 2026-07-08ClinicalA Phase 2 Study to Evaluate the Efficacy and Safety of Belzutifan (MK-6482, Formerly PT2977) Monotherapy in Participants With Advanced Pheochromocytoma/Paraganglioma (PPGL), Pancreatic Neuroendocrine Tumor (pNET), Von Hippel-Lindau (VHL) Disease-Associated Tumors, Advanced Gastrointestinal Stromal Tumor (wt GIST), or Advanced Solid Tumors With HIF-2α Related Genetic AlterationsResults expected Q4 2029
- 2026-06-10ClinicalA Phase 2 Study of ONC206 in Advanced Pheochromocytoma and ParagangliomaResults expected Q4 2028
- 2026-05-13ClinicalA Prospective, Multi-Institutional Phase II Trial Evaluating Temozolomide vs. Temozolomide and Olaparib for Advanced Pheochromocytoma and ParagangliomaResults expected Q1 2028
- 2026-04-24ClinicalLu-177-DOTATATE (Lutathera) in Therapy of Inoperable Pheochromocytoma/ ParagangliomaResults expected Q1 2030
- 2026-03-02ClinicalExploratory Phase II Study of LAnreotide in Metastatic Pheochromocytoma/PARAganglioma (LAMPARA)Results expected Q1 2027
- 2026-02-12ClinicalA Multicenter Open-label Study to Evaluate Safety and Dosimetry of Lutathera in Adolescent Patients With Somatostatin Receptor Positive Gastroenteropancreatic Neuroendocrine (GEP-NET) Tumors, Pheochromocytoma and Paragangliomas (PPGL)Results posted
Clinical trials
The current development programme across all trial phases.
Clinical programme
21
6
0
6
Recruiting
Recently completed
Research activity
Key research shaping understanding of this disease, combining the latest publications with the most influential evidence.
Most influential
Major themes4
- Adrenal Gland Neoplasms1
- Paraganglioma1
- Pheochromocytoma1
- Tumor Microenvironment1
Leading journals1
- Endocrine reviews1
Leading researchers7
- Calsina B1
- Clifton-Bligh R1
- Hadrava Vanova K1
- Pacak K1
- Robledo M1
- Taïeb D1
- Uher O1
Affiliations (unnormalised)6
- Cancer Genetics Laboratory1
- Eunice Kennedy Shriver National Institute of Child Health and Human Development1
- Familiar Cancer Clinical Unit1
- Institute of Health Carlos III (ISCIII)1
- Royal North Shore Hospital1
- Spanish National Cancer Research Centre (CNIO)1
Reference
Authoritative identity, definition & identifiers.
Defined in MeSH
A neural crest tumor usually derived from the chromoreceptor tissue of a paraganglion, such as the carotid body, or medulla of the adrenal gland (usually called a chromaffinoma or pheochromocytoma). It is more common in women than in men. (Stedman, 25th ed; from Segen, Dictionary of Modern Medicine, 1992)
Identifiers
References & data sources
- Disease identity & definition — NLM Medical Subject Headings (MeSH), public domain
- Clinical trials — ClinicalTrials.gov (U.S. National Library of Medicine)
- Research activity — Europe PMC (EMBL-EBI) + OpenAlex-derived paper links
- Related entities are derived from literature co-mention (studied together) — associative, not causal.