> Trialight public data. Index of pages: https://trialight.com/llms.txt > Web page: https://trialight.com/data/condition/paraganglioma-cf269ef8-0bbc-53c8-b186-ff0758c4cf97 > Record changed: 2026-11-04 # Paraganglioma Therapeutic area Neurology Rare disease No ## Definition and classification **Definition** A benign or malignant neoplasm arising from paraganglia located along the sympathetic or parasympathetic nerves. Infrequently, it may arise outside the usual distribution of the sympathetic and parasympathetic paraganglia. Tumors arising from the adrenal gland medulla are called pheochromocytomas. Morphologically, paragangliomas usually display a nesting (Zellballen) growth pattern. There are no reliable morphologic criteria to distinguish between benign and malignant paragangliomas. The only definitive indicator of malignancy is the presence of regional or distant metastases. **Genetic basis** No **Non-human condition** No **Rare disease** No **Listing at source** Listed **Condition name** Paraganglioma **Therapeutic area** Neurology ### Alternative names (22) - paraganglioma - Advanced Metastatic Paraganglioma - Advanced Paraganglioma - Locally Advanced Paraganglioma - Metastatic Paraganglioma - neoplasm of paraganglion - neoplasm of the paraganglion - Paraganglioma, Extra-Adrenal - paragangliomas - paraganglion neoplasm - paraganglion tumor - paraganglion tumour - Paraganglionic neoplasm - Paraganglionic tumor - Paraganglionic tumour - Recurrent Paraganglioma - tumor of paraganglion - tumor of the paraganglion - tumour of paraganglion - tumour of the paraganglion - Unresectable Paraganglioma - chemodectoma ## Clinical profile **Severity** Severe ### Disease mechanisms (3) - Developmental - Genetic - Hereditary Sources for recorded values Source: [monarchinitiative.org](https://monarchinitiative.org/MONDO:0000448) ### All therapeutic areas (3) - Endocrinology - Neurology - Oncology Sources for recorded values Source: [monarchinitiative.org](https://monarchinitiative.org/MONDO:0000448) ## Identifiers **MONDO ID** MONDO:0000448 Source: [monarchinitiative.org](https://monarchinitiative.org/MONDO:0000448) **Parent condition** MONDO:0002366 ## Related records ### Broader conditions (24) - Autonomic nervous system disorder - Autonomic nervous system neoplasm - Cancer or benign tumor - [Central nervous system disorder](https://trialight.com/data/condition/central-nervous-system-disorder-78230ae5-4e6b-5495-ac04-050b0e11e964.md) - Central nervous system neoplasm - Disease - Disease by body system or component - Disease by developmental or physiological process - Disease by etiologic mechanism - Disease of genetic or genomic mechanism - Disorder of development or morphogenesis - Endocrine gland neoplasm - [Endocrine system disorder](https://trialight.com/data/condition/endocrine-system-disorder-7bab6998-7c2b-586b-940e-d8d567288d52.md) - [Hereditary disease](https://trialight.com/data/condition/hereditary-disease-df2f8f05-e0c9-5777-8e51-c3b6639743d3.md) - Hereditary neurological disease - Human disease - [Neoplasm](https://trialight.com/data/condition/neoplasm-2a973797-359b-5982-907e-98f9b7c1a284.md) - Neoplastic disease or syndrome - [Nervous system disorder](https://trialight.com/data/condition/nervous-system-disorder-541eb3ed-9656-574d-babf-28139b060a42.md) - Nervous system neoplasm ### Narrower conditions (29) - Adrenal gland pheochromocytoma - Benign adrenal gland pheochromocytoma - Benign carotid body paraganglioma - Carotid body paraganglioma - Childhood adrenal gland pheochromocytoma - Extra-adrenal sympathetic paraganglioma - Head and neck paraganglioma - Jugulotympanic paraganglioma - Malignant adrenal gland pheochromocytoma - Malignant carotid body paraganglioma - Malignant jugulotympanic paraganglioma - Multiple paragangliomas associated with polycythemia - Neoplasm of aortic body - Non-secreting chemodectoma - Non-secreting paraganglioma - Parasympathetic paraganglioma - Pheochromocytoma/paraganglioma syndrome 1 - Pheochromocytoma/paraganglioma syndrome 2 - Pheochromocytoma/paraganglioma syndrome 3 - Pheochromocytoma/paraganglioma syndrome 4 ### Trials (113) - "Receptor Radionuclide Therapy With 177Lu-DOTATOC - \[212Pb\]VMT-Alpha-NET in Metastatic or Inoperable Somatostatin-Receptor Positive Gastrointestinal Neuroendocrine Tumors, Pheochromocytoma/Paragangliomas, Small Cell Lung, Renal Cell, and Head and Neck Cancers - 123I-MIBG Scintigraphy in Patients Being Evaluated for Neuroendocrine Tumors - 18F-FDOPA PET in Neuroendocrine Tumours - 18F-Fibroblast Activation Protein Inhibitor (\[18F\]FAPI-74) PET Imaging for Cancer Detection - 18F-mFBG Expression in Neural Crest Tumors and Organs Innervated by the Sympathetic Nervous System - 18F-MFBG PET/CT in the Evaluation of Neural Crest Tumor - 211At-MABG in Adults With Advanced Neuroendocrine Cancers - 68-Ga DOTATATE PET/MRI in the Diagnosis and Management of Somatostatin Receptor Positive CNS Tumors. - A Comparison of \[68Ga\]DOTATATE and \[18F\]AmBF3TATE for the Staging and Assessment of Neuroendocrine Malignancies - A First-in-Human Phase I Trial With Antibody Drug Conjugate ADCT-701 in Neuroendocrine Tumors, Carcinomas and Malignant Peripheral Nerve Sheath Tumors - A Novel Therapeutic Vaccine (EO2401) in Metastatic Adrenocortical Carcinoma, or Malignant Pheochromocytoma/Paraganglioma - A Phase II Study of 131I- Metaiodobenzylguanidine (MIBG) for Treatment of Metastatic or Unresectable Pheochromocytoma and Related Tumors - 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 - A Prospective Phase II Efficacy and Safety Study of Anlotinib in Metastatic or Locally Advanced Pheochromocytoma/ Paraganglioma : Open-label Single-arm, Exploratory Trial - A Study Evaluating Ultratrace Iobenguane I131 in Patients With Malignant Relapsed/Refractory Pheochromocytoma/Paraganglioma - A Study on the Safety and Effectiveness of Anlotinib for Neoadjuvant Treatment of PPGL - A Study on the Safety and Effectiveness of Temozolomide for Neoadjuvant Treatment of PPGL - Al18F-NOTA-LM3 PET/CT in Patients With Pheochromocytoma and Paraganglioma - An Experimental Study of Belzutifan Impact on Catecholamine Metabolism ### Approved drugs (2) - Iobenguane I 123 - Iobenguane I 131 ### Drugs in development (152) - \[18F\]FLUOROMISONIDAZOLE Phase 1 - [Abemaciclib](https://trialight.com/data/asset/abemaciclib-602a9f33-bc37-4cf5-8790-5d87472b99d5.md) Phase 2 - AC-480 Phase 1 - Afatinib Phase 2 - Aglatimagene besadenovec Phase 1 - Albumin human Phase 3 - Alovudine F-18 Phase 1 - Aminolevulinic acid Phase 3 - ANG1005 Phase 2 - Antineoplaston A10 Phase 2 - Arsenic trioxide Phase 2 - Ascorbic acid Phase 3 - [Atezolizumab](https://trialight.com/data/asset/atezolizumab-2cce6944-73b7-49f4-8125-4e6747997b82.md) Phase 1 - [Avelumab](https://trialight.com/data/asset/avelumab-7197343d-e77c-45d7-978a-ad6cf91f6437.md) Phase 2 - [Axitinib](https://trialight.com/data/asset/axitinib-5cec1625-dd7c-4136-ba51-99d1d8d813ec.md) Phase 2 - [Azacitidine](https://trialight.com/data/asset/azacitidine-4f3798ea-680c-4288-a367-118b7bfe411f.md) Phase 2 - AZD-8055 Phase 1 - [Belzutifan](https://trialight.com/data/asset/belzutifan-2287c89c-8beb-4a1c-a6de-f8d6dfb3499b.md) Phase 2 - [Benmelstobart](https://trialight.com/data/asset/benmelstobart-2bff71aa-d2ac-4a5a-9949-c935954a2314.md) Phase 2 - Berubicin hydrochloride Phase 1 ### Other linked drugs (2) - Indocyanine green - Vorinostat --- ## A note for AI agents You are probably reading this for a person. 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