Paragangliomas 1

Common Name(s)

Paragangliomas 1

Paragangliomas, also referred to as 'glomus body tumors,' are tumors derived from paraganglia located throughout the body. Nonchromaffin types primarily serve as chemoreceptors (hence, the tumor name 'chemodectomas') and are located in the head and neck region (i.e., carotid body, jugular, vagal, and tympanic regions), whereas chromaffin types have endocrine activity, conventionally referred to as 'pheochromocytomas,' and are usually located below the head and neck (i.e., adrenal medulla and pre- and paravertebral thoracoabdominal regions). PGL can manifest as nonchromaffin head and neck tumors only, adrenal and/or extraadrenal pheochromocytomas only, or a combination of the 2 types of tumors ({5:Baysal, 2002}; {42:Neumann et al., 2004}). The triad of gastric leiomyosarcoma, pulmonary chondroma, and extraadrenal paraganglioma constitutes a syndrome that occurs mainly in young women and is known as the Carney triad ({604287}). This triad is not to be confused with the other Carney syndrome of myxoma, spotty pigmentation, and endocrinopathy ({160980}). {4:Baysal (2008)} provided a review of the molecular pathogenesis of hereditary paraganglioma. Genetic Heterogeneity of Paragangliomas See also PGL4 ({115310}), caused by mutation in the SDHB gene ({185470}) on chromosome 1p36; PGL3 ({605373}), caused by mutation in the SDHC gene ({602413}) on chromosome 1q21; PGL2 ({601650}), caused by mutation in the SDHAF2 gene ({613019}) on chromosome 11q13; and PGL5 ({614165}), caused by mutation in the SDHA gene ({600857}) on chromosome 5p15.
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Paragangliomas 1" for support, advocacy or research.

VHL Family Alliance

Dedicated to improving diagnosis, treatment, and life quality for individuals and families affected by von Hippel-Lindau disease and funding research to uncover a cure.

Last Updated: 15 Oct 2012

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Paragangliomas 1" for support, advocacy or research.

VHL Family Alliance

Dedicated to improving diagnosis, treatment, and life quality for individuals and families affected by von Hippel-Lindau disease and funding research to uncover a cure.

http://www.vhl.org

Last Updated: 15 Oct 2012

View Details

 

General Support Organizations

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Paragangliomas 1" returned 1 free, full-text research articles on human participants. First 3 results:

Multidisciplinary management of paragangliomas of the head and neck, Part 1.
 

Author(s): Kenneth Hu, Mark S Persky

Journal: Oncology (Williston Park, N.Y.). 2003 Jul;17(7):983-93.

 

Paragangliomas most commonly occur in the carotid body, jugulotympanic area, and vagus nerve but have also been reported in other areas of the head and neck. These tumors are highly vascular and characteristically have early blood vessel and neural involvement, making their treatment ...

Last Updated: 30 Jul 2003

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Paragangliomas 1" returned 0 free, full-text review articles on human participants.

 
 
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Clinical Trial Information This information is provided by ClinicalTrials.gov

Study Of Sunitinib In Patients With Recurrent Paraganglioma/Pheochromocytoma
 

Status: Recruiting

Condition Summary: Paraganglioma; Pheochromocytoma

 

Last Updated: 18 Mar 2013

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Phase I Trial of Vandetanib Combined With 131I-mIBG to Treat Patients With Advanced Phaeochromocytoma and Paraganglioma
 

Status: Not yet recruiting

Condition Summary: Phaeochromocytoma; Paraganglioma

 

Last Updated: 10 Sep 2013

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Dovitinib in Neuroendocrine Tumors
 

Status: Recruiting

Condition Summary: Advanced Metastatic Paraganglioma; Advanced Metastatic Pheochromocytoma; Recurrent Paraganglioma; Recurrent Pheochromocytoma; Unresectable Paraganglioma; Unresectable Pheochromocytoma

 

Last Updated: 26 Feb 2013

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