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: 1166 relevant articles (37 outcomes, 74 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Pheochromocytoma
2.	Neoplasms (Cancer)
3.	Carcinoid Tumor (Carcinoid)
4.	Carcinoma (Carcinomatosis)
5.	Hemorrhage

Experts

1.	Pacak, Karel: 56 articles (12/2015 - 03/2002)
2.	Gimenez-Roqueplo, Anne-Paule: 33 articles (01/2016 - 10/2002)
3.	Eisenhofer, Graeme: 22 articles (06/2015 - 07/2003)
4.	Plouin, Pierre-François: 18 articles (04/2014 - 10/2002)
5.	Favier, Judith: 17 articles (06/2015 - 10/2002)
6.	Bayley, Jean-Pierre: 16 articles (12/2015 - 01/2005)
7.	Neumann, Hartmut P H: 16 articles (04/2015 - 05/2003)
8.	Amar, Laurence: 14 articles (02/2015 - 01/2005)
9.	Adams, Karen T: 13 articles (06/2015 - 04/2005)
10.	Burnichon, Nelly: 12 articles (01/2016 - 10/2007)

Drugs and Biologics

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

1.	3-Iodobenzylguanidine (Iobenguane)IBA 08/01/2006 - "Stable disease and improved health-related quality of life (HRQoL) following fractionated low dose 131I-metaiodobenzylguanidine (MIBG) therapy in metastatic paediatric paraganglioma: observation on false "reverse" discordance during pre-therapy work up and its implication for patient selection for high dose targeted therapy." 09/01/1994 - "MRI is as effective as MIBG scintigraphy in detecting functioning 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. " 12/01/1996 - "We compared iodine-131 metaiodobenzylguanidine (MIBG), computed tomography (CT) and magnetic resonance (MR) imaging studies in 40 patients with suspected or proven paragangliomas. " 12/01/2015 - "MIBG uptake in malignant paragangliomas was lower than in malignant phaeochromocytomas (36% vs. 100%, P = 0.002) and disease stabilisation was achieved in 29% and 86% of patients respectively. "
2.	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. " 12/01/2013 - "Effects of octreotide therapy in progressive head and neck paragangliomas: case series."
3.	CatecholaminesIBA 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). " 02/01/2014 - "Skull base and head and neck region paragangliomas (SHN-PGs) are almost always derived from parasympathetic tissue and rarely secrete catecholamines. " 01/01/2014 - "Measurements of catecholamines suggested the diagnosis of paraganglioma. " 01/01/2012 - "Preoperative embolization is a useful and safe procedure which may reduce the risk of catecholamines release at the time of surgical excision in large pelvic extra-adrenal sympathetic paraganglioma." 01/01/2012 - "Malignant paragangliomas derive from sympathetic tissue and secrete catecholamines. "
4.	Somatostatin (Somatotropin Release-Inhibiting Factor)IBA 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." 07/01/1996 - "Long-term therapy with somatostatin analogues of catecholamine-secreting (malignant) paragangliomas and phaeochromocytomas has not shown clinical benefits." 02/01/2009 - "Head and neck paragangliomas are neuroendocrine tumors that express somatostatin type 2 receptors and can consequently be visualized through nuclear imaging techniques, using radionuclide-labelled somatostatin analogs, specifically 111In-pentetreotide. "
5.	Succinate Dehydrogenase (Fumarate Reductase)IBA 05/01/2013 - "The aim of the present study was to review treatment results in patients with paraganglioma (PGL) of the neck presenting as carotid body tumour, long-term follow-up and relevance of genetic testing for succinate dehydrogenase (SDH)-gene mutations. " 05/01/2013 - "Germline mutations in succinate dehydrogenase (SDH) genes predispose carriers for developing paragangliomas, and studies on their quality of life (QoL) are scarce. " 08/01/2011 - "The purpose of this study was to investigate the somatic mutations of human mitochondria succinate dehydrogenase subunit B (SDHB) in sporadic paragangliomas. " 06/01/2009 - "Several studies have shown that patients with succinate dehydrogenase subunit B (SDHB) mutations have a very high risk for developing malignant paragangliomas. " 01/01/2016 - "About 70% paragangliomas are sporadic, 30% are hereditary, having identifiable germline mutations of Succinate Dehydrogenase enzyme (SDH). "
6.	Somatostatin Receptors (Somatostatin Receptor)IBA 12/01/2002 - "In this study, the clinical value of somatostatin receptor imaging was assessed in 19 patients who underwent somatostatin receptor scintigraphy because of known or suspected paraganglioma of the head and neck region. " 02/01/2001 - "Somatostatin receptor imaging (SRI) has a high sensitivity for localizing head and neck paragangliomas, but studies of intraabdominal PCCs are rare. " 09/01/2015 - "A high percentage of paragangliomas express somatostatin receptors that can be utilized for targeted radioisotope therapy. " 08/01/2015 - "This report presents a case of a 13-year-old boy with urinary bladder paraganglioma and the role of somatostatin receptor imaging with simultaneous positron emission tomography magnetic resonance (PET/MR). " 11/01/2014 - "Pre-operative ⁶⁸Ga-DOTANOC somatostatin receptor PET/CT imaging demonstrating multiple synchronous lesions in a patient with head and neck paraganglioma."
7.	Gadolinium DTPA (Magnevist)FDA Link 01/01/1994 - "Contrast enhancement with gadopentetate dimeglumine has been advocated to increase the efficacy of MR imaging for paragangliomas of the head and neck. " 01/01/1994 - "The results of this study indicate that, in general, the use of gadopentetate dimeglumine is not necessary for the detection of head and neck paragangliomas. " 01/01/1988 - "[Magnetic resonance tomographic studies of paragangliomas of the glomus caroticum and glomus jugulare using Gd-DTPA]." 01/01/1998 - "On T1-weighted images, paraganglioma is isointense relative to spinal cord and shows moderate to intense enhancement after Gd-DTPA application, usually with heterogeneous texture. " 09/01/1989 - "Paragangliomas of the jugular bulb and carotid body: MR imaging with short sequences and Gd-DTPA enhancement."
8.	Islet cell tumor syndromeIBA 12/01/2011 - "Although a large kindred with familial pheochromocytoma (Pheo) and paraganglioma (PGL) was discovered in 1962 and later found to represent von Hippel-Lindau (VHL) type 2A (mutation Y112H), the phenotype lacks current characterization. " 01/01/2006 - "In conclusion, these data indicate that germline mutations of SDHB and SDHC play a minor role in sporadic head and neck paraganglioma and further underline the importance of germline SDHB mutations in cases of familial pheochromocytoma-paraganglioma." 12/01/2004 - "The final diagnosis was familial pheochromocytoma/paraganglioma type 1 (OMIM 168000). " 08/01/1998 - "Laparoscopic adrenal sparing surgery (4 cases, 2 bilateral) and bilateral adrenalectomy (1) were performed for multiple familial pheochromocytoma, and all paragangliomas were excised simultaneously. " 09/01/2006 - "Logistic analysis showed that statistically significant risk factors for malignancy were (1) paraganglioma, (2) apparently sporadic, as opposed to familial, pheochromocytoma or paraganglioma, and (3) tumor size of > 6 cm. Surgical resection was performed for 28 patients (93%), with perioperative mortality and major morbidity rates of 0% and 10%, respectively. "
9.	glucuronyl glucosamine glycan sulfate (Vessel)IBA 02/01/2015 - "Paraganglioma tends to grow slowly but can compress adjacent vessel and nerve. " 06/01/2014 - "An intrapericardial paraganglioma with embolization of a large vessel from the left coronary artery." 05/01/2010 - "Two neuroradiologists evaluated the overall image quality, the presence of a paraganglioma, the maximum diameter, as well as the vessel invasion. " 05/01/2001 - "Radiologic features demonstrating a vascular mass with a dominant feeder vessel by the superior or inferior thyroid artery may help in the clinical diagnosis of paragangliomas of the larynx. " 04/01/2014 - "Angiography may help to clarify the vessel architecture for reducing intraoperative bleeding when primary intracerebellar paraganglioma is considered."
10.	Vesicular Monoamine Transport Proteins (Vesicular Monoamine Transporter)IBA 06/01/2010 - "6-18F-fluoro-L-dihydroxyphenylalanine positron emission tomography is superior to 123I-metaiodobenzyl-guanidine scintigraphy in the detection of extraadrenal and hereditary pheochromocytomas and paragangliomas: correlation with vesicular monoamine transporter expression." 08/01/2006 - "The aims of this study were to investigate the quantitative expression of vesicular monoamine transporters (VMAT 1, 2) and all five SSTRs in malignant pheochromocytoma/paraganglioma (PC/PG) to evaluate the possibilities for tumor-specific radionuclide therapy. " 07/01/2012 - "We provide a classifying rule for differential diagnosis that combines negativity or low staining for calcitonin gene-related protein (histologic score, <10) or calcitonin (histologic score, <50) together with positivity of any of NADH dehydrogenase 1 alpha subcomplex, 4-like 2; cytochrome c oxidase subunit IV isoform 2; or vesicular monoamine transporter 2 to predict paragangliomas, showing a prediction error of 0%. " 04/01/2011 - "Expression of somatostatin receptors, dopamine D₂ receptors, noradrenaline transporters, and vesicular monoamine transporters in 52 pheochromocytomas and paragangliomas."

Therapies and Procedures

1.	Radiotherapy 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." 01/01/2014 - "Aim of the study is to evaluate the results of postoperative radiotherapy of paragangliomas, prognostic factors and causes of treatment failure. " 08/01/2013 - "Jugular and vagal paragangliomas: Systematic study of management with surgery and radiotherapy."
2.	Drug Therapy (Chemotherapy) 01/01/2013 - "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. " 02/01/2009 - "The role of chemotherapy is restricted to malignant paragangliomas. " 09/01/2007 - "Malignant paraganglioma patients who could not meet complete resection needed chemotherapy for promising survival. "
3.	Thyroidectomy 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. " 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. " 02/01/2014 - "Out of the 22 eligible patients for the study, various laparoscopic surgeries performed were - Fundoplication (4), Cystogastrostomy (3), Endoscopic thyroidectomy (7), Thoracoscopic Thyroidectomy (2), Adrenalectomy (5) and Retroperitoneal paraganglioma excision (1). " 04/01/1986 - "Six patients underwent total thyroidectomy and two patients, one with paraganglioma and one with hemangiopericytoma, had a hemithyroidectomy. "
4.	Adjuvant Radiotherapy 01/01/2013 - "Currently, the combination of curative surgical resection and secondary adjuvant radiotherapy is the gold standard for treating symptomatic malignant paragangliomas. " 09/01/1996 - "In 3 malignant paragangliomas (23.0%) adjuvant radiotherapy of 50-60 Gy was administered to the tumor site after surgery. "
5.	Therapeutics 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." 05/01/2012 - "Metastatic pheochromocytoma and paraganglioma: focus on therapeutics."