Paraganglioma
Recent clinical, regulatory, research and industry developments relating to this disease.
The Immune Landscape of Pheochromocytoma and Paraganglioma: Current Advances and Perspectives.
What's happening now
An analyst briefing on current research, clinical, regulatory and industry activity surrounding this disease.
- 4 clinical trials expected to report results, the earliest in Q1 2027.
- Q1 2027Exploratory Phase II Study of LAnreotide in Metastatic Pheochromocytoma/PARAganglioma (LAMPARA)
- 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
Clinical MilestonesViewHide
- 2026-09-03A 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
- 2026-04-24Lu-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
- 2026-09-03ClinicalA 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
- 2026-04-24ClinicalLu-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.
Research activity
Key research shaping understanding of this disease, combining the latest publications with the most influential evidence.
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
Associated genes
Genes associated with this disease in the canonical knowledge graph (Open Targets evidence). Number shows the association score.
Disease biology
Proteins whose encoding gene is associated with this disease, reached through the canonical gene→disease graph. Number shows the gene's association score.
Reference
Authoritative identity, definition & identifiers.
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)
- 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.