Paraganglioma (Paragangliomas)

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)

Also Known As:

Networked: 2570 relevant articles (75 outcomes, 189 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Pheochromocytoma
2.	Neoplasms (Cancer)
3.	Carcinoid Tumor (Carcinoid)
4.	Thyroid Neoplasms (Thyroid Cancer)
5.	Neuroblastoma

Experts

1.	Pacak, Karel: 151 articles (01/2022 - 03/2002)
2.	Eisenhofer, Graeme: 60 articles (05/2022 - 01/2003)
3.	Gimenez-Roqueplo, Anne-Paule: 57 articles (11/2022 - 10/2002)
4.	Timmers, Henri J L M: 37 articles (04/2022 - 03/2007)
5.	Favier, Judith: 32 articles (11/2022 - 10/2002)
6.	Robledo, Mercedes: 32 articles (01/2022 - 08/2002)
7.	Taïeb, David: 31 articles (10/2021 - 05/2009)
8.	Amar, Laurence: 29 articles (01/2022 - 01/2005)
9.	Mannelli, Massimo: 26 articles (01/2022 - 08/2006)
10.	Burnichon, Nelly: 25 articles (05/2022 - 10/2007)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Paraganglioma:

1.	3-Iodobenzylguanidine (Iobenguane)IBA 09/01/1994 - "MRI is as effective as MIBG scintigraphy in detecting functioning paragangliomas. " 01/01/2016 - "I-123 metaiodobenzylguanidine (MIBG) scintigraphy has become the imaging study of choice for paragangliomas and has a sensitivity of ∼77% to 100% in detecting functional paragangliomas. " 05/01/1999 - "In this study, the influence of clinical features and tumour pathology on the results of 131I MIBG of patients with phaeochromocytomas and paragangliomas was examined. " 09/01/2022 - "Heterogeneous Head and Neck Paraganglioma With Distinct Features on 123 I-MIBG and 68 Ga-DOTATATE Images." 09/01/2022 - "The sensitivities of 68 Ga DOTATATE PET/CT, 18F-fluorodeoxyglucose positron emission tomography/computed tomography ( 18 F-FDG-PET/CT), 131 I-metaiodobenzylguanidine ( 131 I-MIBG) scintigraphy, and CECT/MRI for primary HNPGL, associated primary pheochromocytoma + sympathetic paraganglioma (PCC + sPGL), and metastatic lesions were analyzed. "
2.	Radioisotopes (Radionuclides)IBA 09/01/2016 - "A para-aortic paraganglioma was diagnosed during the patient's work-up with biochemical studies, computed tomography of abdomen and functional radioisotopes imaging and was eventually surgically resected. " 03/01/1985 - "Radionuclide thermographic studies were conducted in 16 neck paraganglioma patients for comprehensive diagnosis. " 09/01/2019 - "Diverse radionuclide imaging techniques are available for the diagnosis, staging, and follow-up of phaeochromocytoma and paraganglioma (PPGL). " 09/01/2019 - "European Association of Nuclear Medicine Practice Guideline/Society of Nuclear Medicine and Molecular Imaging Procedure Standard 2019 for radionuclide imaging of phaeochromocytoma and paraganglioma." 12/01/2012 - "Radionuclide imaging of phaeochromocytomas (PCCs) and paragangliomas (PGLs) involves various functional imaging techniques and approaches for accurate diagnosis, staging and tumour characterization. "
3.	CatecholaminesIBA 01/01/2023 - "Despite evidence of catecholamine excess in most patients with bladder paraganglioma, the majority are diagnosed after biopsy, indicating a need for improved diagnostic strategies in this patient population. " 01/01/2014 - "Medication-related interferences with measurements of catecholamines and their metabolites represent important causes of false-positive results during diagnosis of phaeochromocytomas and paragangliomas (PPGLs). " 01/01/2005 - "The aim of this study was to identify the prevalence of catecholamine excess and phaeochromocytomas in a well-defined population of people with hereditary head and neck paragangliomas. " 11/29/2023 - "We describe a pericardial paraganglioma with very unusual features: absence of catecholamine hypersecretion or compressive symptoms, located in the right atrium and apparently sporadic. " 10/30/2023 - "Conversely, the sympathetic paragangliomas are functional and produce catecholamine. "
4.	Peptide Receptors (Peptide Receptor)IBA 01/01/2015 - "Peptide receptor radio­nuclide therapy can be a therapeutic option in selected cases of head and neck paragangliomas." 03/01/2017 - "Peptide Receptor Radionuclide Treatment and (131)I-MIBG in the management of patients with metastatic/progressive phaeochromocytomas and paragangliomas." 01/01/2015 - "Peptide receptor radionuclide therapy (PRRT) is a very promising treatment option in neuroendocrine tumours, with good results, but there are only few reports regar­ding its use in paragangliomas. " 11/20/2011 - "Novel SDHD gene mutation (H102R) in a patient with metastatic cervical paraganglioma effectively treated by peptide receptor radionuclide therapy." 01/01/2019 - "Peptide Receptor Radionuclide Therapy as a Novel Treatment for Metastatic and Invasive Phaeochromocytoma and Paraganglioma."
5.	Octreotide (Sandostatin)FDA LinkGeneric 10/01/2003 - "These data suggest that octreotide can be useful in the treatment of inoperable paragangliomas." 06/01/2007 - "Contrast computed tomography and digital angiography still remain the gold standard reliable instruments for diagnosis despite the success of magnetic resonance imaging, magnetic resonance angiography and octreotide scintigraphy to detect head and neck paragangliomas. " 03/01/2000 - "On the basis of this series of patients, octreotide scintigraphy appears to be a reliable test to detect paragangliomas and may be quite helpful in preoperative planning." 03/01/2000 - "This study compared the results of preoperative octreotide scintigraphy with the histopathology in 21 patients who underwent surgery for presumed head and neck paragangliomas. " 09/01/2023 - "To report a dramatic and immediate clinical and biochemical response during treatment with octreotide in a patient with a functioning mesenteric paraganglioma (PGL). "
6.	Somatostatin (Somatotropin Release-Inhibiting Factor)IBA 01/01/2021 - "Metastatic Malignant Paraganglioma Presenting as a Neck Mass Treated with Radiolabeled Somatostatin Analog." 02/01/2009 - "Appropriately radiolabeled somatostatin analogs could potentially be used for the treatment of paragangliomas." 08/01/2005 - "Are somatostatin analogs therapeutic alternatives in the management of head and neck paragangliomas?" 10/01/2003 - "Case report: patient with multiple paragangliomas treated with long acting somatostatin analogue." 01/01/2021 - "Thus, patients with pheochromocytoma and paraganglioma, especially those with inoperable or metastatic diseases, depend on the clinical trials of somatostatin analogs. "
7.	NormetanephrineIBA 11/26/2020 - "The final pathology demonstrated that the intracardiac mass was a cardiac paraganglioma, and the patient had an increased level of normetanephrine in the blood. " 09/02/2020 - "She had elevated 24-hour urinary normetanephrine and was found to have a paraganglioma arising from the urinary bladder infiltrating the sigmoid colon. " 05/01/2012 - "Serum normetadrenaline was elevated in keeping with functional paragangliomas. " 01/01/2022 - "Sampling of blood in the supine position for diagnosis of pheochromocytoma and paraganglioma (PPGL) results in lower rates of false positives for plasma normetanephrine than seated sampling. " 01/01/2022 - "Blood tests showed high amounts of normetanephrine (35.1-45.4 nmol/L, ref <1.09 nmol/L) and a tumor biopsy confirmed the diagnosis of a paraganglioma. "
8.	Succinate Dehydrogenase (Fumarate Reductase)IBA 01/01/2021 - "The aim of this study was to assess whether radiologic features could differentiate between paragangliomas in the head and neck positive and negative for the succinate dehydrogenase mutation. " 12/01/2019 - "Exploring the link between tumour metabolism and succinate dehydrogenase deficiency: A 18 F-FDOPA PET/CT study in head and neck paragangliomas." 01/01/2019 - "The goal of this study was to detect and explore the mechanisms of the succinate dehydrogenase (SDH) complex subunit-related gene mutations in cases of multiple paraganglioma (PGL) in the head and neck. " 04/01/2018 - "Germline mutations in the succinate dehydrogenase B (SDHB) gene predispose to hereditary paraganglioma (PGL) syndrome type 4. The aim of this study was to evaluate the clinical characteristics and outcome of treatment strategies for patients with head and neck paraganglioma (HNPGL) carrying SDHB germline mutations. " 01/01/2018 - "A novel succinate dehydrogenase subunit B germline variant associated with head and neck paraganglioma in a Dutch kindred: A family-based study."
9.	MetanephrineIBA 01/01/2022 - "To analyze the correlation between metanephrine and normetanephrines (MNs) and subclinical myocardial injuries (SMI) diagnosed by low left ventricular global longitudinal strain (LV GLS) in patients with pheochromocytoma and paraganglioma (PPGL). " 11/01/2021 - "A rare metanephrine-predominant secretion phenotype of paraganglioma challenged an interpretation of imaging localization." 05/19/2020 - "Elevated serum metanephrine and uptake in 131iodine metaiodobenzylguanidine scan lead to the diagnosis of paraganglioma. " 11/01/2018 - "The challenge of improving the diagnostic yield from metanephrine testing in suspected phaeochromocytoma and paraganglioma." 01/28/2014 - "Based on imaging and blood metanephrine levels, the diagnosis of paraganglioma was made. "
10.	Edotreotide (SMT 487)IBA 05/01/2019 - "PET/CT showed a high Ga-DOTATOC uptake of the mass that was strongly consistent with paraganglioma diagnosis. " 12/01/2008 - "Nevertheless, DOTATOC appears to be a treatment option for surgically incurable paragangliomas, because toxicity is very low and especially the fact that long lasting remissions could be achieved justifies the treatment. " 10/01/2023 - "Multiple myeloma mimicking metastatic skull base paraganglioma in [68 Ga]Ga-DOTATOC-PET/CT and 2-[18F]FDG-PET/CT imaging." 12/01/2008 - "We evaluated the effectiveness and toxicity of radiolabeled DOTATOC in patients with metastatic paraganglioma and pheochromocytoma. " 12/01/2008 - "Radiolabeled DOTATOC in patients with advanced paraganglioma and pheochromocytoma."

Therapies and Procedures

1.	Therapeutics 01/01/1993 - "This system highlights the relative position of a vagal paraganglioma to the jugular foramen and is helpful in designing the proper therapy. " 10/01/2006 - "We report the feasibility and efficacy of percutaneous injection of Onyx (Micro Therapeutics, Irvine, Calif.), a nonadhesive liquid embolic agent, into an unusually located paraganglioma, followed by endovascular embolization with particles before surgery." 01/01/2022 - "In this study, we aim to evaluate the efficacy and safety of 225AC-DOTATATE targeted alpha therapy in advanced-stage paragangliomas (PGLs). " 04/01/2014 - "Seventeen studies concerning a total of 243 patients with malignant paraganglioma/phaeochromocytoma were treated with (131)I-MIBG therapy. " 04/01/2014 - "The main objective of this study was to perform a systematic review and meta-analysis assessing the effect of (131)I-MIBG therapy on tumour volume in patients with malignant paraganglioma/phaeochromocytoma. "
2.	Radiotherapy 06/01/2014 - "Conventional external beam radiation therapy is an effective and safe treatment option that achieves excellent local control; it should be considered as a first-line treatment of choice for head and neck paragangliomas." 10/01/2001 - "Paragangliomas of the head and neck may be treated successfully with surgery, radiation therapy, or stereotactic radiosurgery. " 09/01/2014 - "In the light of these results, together with the improved quality of life and a better knowledge of the natural history of this disease, many authors propose radiotherapy as first-line treatment for all glomus jugulare paragangliomas regardless of their size, particularly in patients with no preoperative deficits. " 03/01/2011 - "We believe that in patients affected by unresectable paragangliomas radiotherapy is a safe and effective alternative to surgery. " 06/01/2014 - "The role of wait-and-scan and the efficacy of radiotherapy in the treatment of temporal bone paragangliomas."
3.	Drug Therapy (Chemotherapy) 06/01/2017 - "To the best of our knowledge, this is the first report of a patient with a paraganglioma who developed CD after chemotherapy. " 06/01/2017 - "A 23-year-old man was diagnosed with a giant pelvic paraganglioma in September 2013, and a 6-month chemotherapy course was performed. " 08/05/2013 - "The patient is on palliative chemotherapy for malignant paraganglioma. " 01/01/2011 - "Subsequent biopsy revealed a paraganglioma that was treated with chemotherapy. " 10/01/2009 - "A male with metastatic paraganglioma received different chemotherapy regimens and 14 arterial embolizations with no or short-lasting clinical benefit. "
4.	Punctures 10/01/2004 - "Direct puncture embolization for paragangliomas: promising results but preliminary data." 07/16/2023 - "Direct percutaneous puncture versus transarterial embolization for head and neck paragangliomas: A systematic review and meta-analysis." 04/01/2022 - "Pre-operative paraganglioma embolization with SQUID-12 using a direct puncture and balloon-assisted technique is a safe and efficient method with few complications." 06/01/2020 - "Direct puncture embolization of carotid paragangliomas only using Squid® is a safe and relatively simple procedure that facilitates the subsequent surgery with minimal blood loss." 06/01/2020 - "Preoperative Squid embolization of carotid paragangliomas with direct puncture."
5.	Thyroidectomy 10/01/2019 - "Thyroid paraganglioma was diagnosed incidentally after total thyroidectomy. " 01/01/2018 - "Resection of the mass and total thyroidectomy were performed with confirmation of paraganglioma S100 positive and PTC. " 07/01/2016 - "A total thyroidectomy was performed, and a final diagnosis of paraganglioma was made. " 11/01/2002 - "The tumorous appearance of the lesion and some cytologic atypia led to a right lobe thyroidectomy, revealing a primary thyroid paraganglioma associated with capsular invasion; slight, focal cytologic atypia; and no vascular invasion. " 02/01/2013 - "The paraganglioma was treated with a total thyroidectomy followed by radiation therapy, and the patient was disease-free after more than 2 years of follow-up. "