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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)
MeSH Review


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Disease relevance of Paraganglioma


High impact information on Paraganglioma


Chemical compound and disease context of Paraganglioma


Biological context of Paraganglioma


Anatomical context of Paraganglioma


Gene context of Paraganglioma

  • Germline SDHD mutations were originally described in hereditary paraganglioma, a dominantly inherited disorder characterized by vascular tumors in the head and the neck, most frequently at the carotid bifurcation [23].
  • In addition, germline SDHD and SDHB mutations may cause phaeochromocytoma susceptibility with or without associated head and neck paragangliomas [24].
  • E was found in a few neuroendocrine tissues outside of the adrenal, including cardiac paragangliomas, and the enzyme PNMT was localized in some of these neoplasms [25].
  • Since 2000 the genes for succinatedehydrogenase subunits SDHB, SDHC, and SDHD have been identified for paraganglioma syndromes type 4, type 3, and type 1 respectively [26].
  • Here we introduce all reported paraganglioma and pheochromocytoma related sequence variations in these genes, in addition to all reported mutations of SDHA [27].
  • SDHD mutations (D92Y) are associated with malignant paragangliomas and catecholamine excess with remarkable interindividual variations despite the same mutation [28].

Analytical, diagnostic and therapeutic context of Paraganglioma


  1. Early-onset renal cell carcinoma as a novel extraparaganglial component of SDHB-associated heritable paraganglioma. Vanharanta, S., Buchta, M., McWhinney, S.R., Virta, S.K., Peçzkowska, M., Morrison, C.D., Lehtonen, R., Januszewicz, A., Järvinen, H., Juhola, M., Mecklin, J.P., Pukkala, E., Herva, R., Kiuru, M., Nupponen, N.N., Aaltonen, L.A., Neumann, H.P., Eng, C. Am. J. Hum. Genet. (2004) [Pubmed]
  2. Vasoactive intestinal peptide/pituitary adenylate cyclase-activating peptide receptor subtypes in human tumors and their tissues of origin. Reubi, J.C., Läderach, U., Waser, B., Gebbers, J.O., Robberecht, P., Laissue, J.A. Cancer Res. (2000) [Pubmed]
  3. The coexistence of renal artery stenosis and pheochromocytoma. Hill, F.S., Jander, H.P., Murad, T., Diethelm, A.G. Ann. Surg. (1983) [Pubmed]
  4. Analysis of the SDHD gene, the susceptibility gene for familial paraganglioma syndrome (PGL1), in pheochromocytomas. Aguiar, R.C., Cox, G., Pomeroy, S.L., Dahia, P.L. J. Clin. Endocrinol. Metab. (2001) [Pubmed]
  5. Somatostatin receptor imaging, therapy and new strategies in patients with neuroendocrine tumours. Slooter, G.D., Mearadji, A., Breeman, W.A., Marquet, R.L., de Jong, M., Krenning, E.P., van Eijck, C.H. The British journal of surgery. (2001) [Pubmed]
  6. Mutations in SDHC cause autosomal dominant paraganglioma, type 3. Niemann, S., Müller, U. Nat. Genet. (2000) [Pubmed]
  7. Mutations in SDHD, a mitochondrial complex II gene, in hereditary paraganglioma. Baysal, B.E., Ferrell, R.E., Willett-Brozick, J.E., Lawrence, E.C., Myssiorek, D., Bosch, A., van der Mey, A., Taschner, P.E., Rubinstein, W.S., Myers, E.N., Richard, C.W., Cornelisse, C.J., Devilee, P., Devlin, B. Science (2000) [Pubmed]
  8. Carboplatin chemotherapy for malignant paraganglioma. Cairnduff, F., Smith, I.E. Lancet (1986) [Pubmed]
  9. Mitochondrial succinate is instrumental for HIF1alpha nuclear translocation in SDHA-mutant fibroblasts under normoxic conditions. Brière, J.J., Favier, J., Bénit, P., El Ghouzzi, V., Lorenzato, A., Rabier, D., Di Renzo, M.F., Gimenez-Roqueplo, A.P., Rustin, P. Hum. Mol. Genet. (2005) [Pubmed]
  10. The ubiquinone-binding site of the Saccharomyces cerevisiae succinate-ubiquinone oxidoreductase is a source of superoxide. Guo, J., Lemire, B.D. J. Biol. Chem. (2003) [Pubmed]
  11. Benign paragangliomas: clinical presentation and treatment outcomes in 236 patients. Erickson, D., Kudva, Y.C., Ebersold, M.J., Thompson, G.B., Grant, C.S., van Heerden, J.A., Young, W.F. J. Clin. Endocrinol. Metab. (2001) [Pubmed]
  12. Biochemical and clinical manifestations of dopamine-producing paragangliomas: utility of plasma methoxytyramine. Eisenhofer, G., Goldstein, D.S., Sullivan, P., Csako, G., Brouwers, F.M., Lai, E.W., Adams, K.T., Pacak, K. J. Clin. Endocrinol. Metab. (2005) [Pubmed]
  13. Octreotide scintigraphy for the detection of paragangliomas. Kwekkeboom, D.J., van Urk, H., Pauw, B.K., Lamberts, S.W., Kooij, P.P., Hoogma, R.P., Krenning, E.P. J. Nucl. Med. (1993) [Pubmed]
  14. Localization and treatment of familial malignant nonfunctional paraganglioma with iodine-131 MIBG: report of two cases. Khafagi, F., Egerton-Vernon, J., van Doorn, T., Foster, W., McPhee, I.B., Allison, R.W. J. Nucl. Med. (1987) [Pubmed]
  15. Founder effect at PGL1 in hereditary head and neck paraganglioma families from the Netherlands. van Schothorst, E.M., Jansen, J.C., Grooters, E., Prins, D.E., Wiersinga, J.J., van der Mey, A.G., van Ommen, G.J., Devilee, P., Cornelisse, C.J. Am. J. Hum. Genet. (1998) [Pubmed]
  16. Somatic loss of maternal chromosome 11 causes parent-of-origin-dependent inheritance in SDHD-linked paraganglioma and phaeochromocytoma families. Hensen, E.F., Jordanova, E.S., van Minderhout, I.J., Hogendoorn, P.C., Taschner, P.E., van der Mey, A.G., Devilee, P., Cornelisse, C.J. Oncogene (2004) [Pubmed]
  17. SDHD mutations in head and neck paragangliomas result in destabilization of complex II in the mitochondrial respiratory chain with loss of enzymatic activity and abnormal mitochondrial morphology. Douwes Dekker, P.B., Hogendoorn, P.C., Kuipers-Dijkshoorn, N., Prins, F.A., van Duinen, S.G., Taschner, P.E., van der Mey, A.G., Cornelisse, C.J. J. Pathol. (2003) [Pubmed]
  18. Mutations in the SDHB gene are associated with extra-adrenal and/or malignant phaeochromocytomas. Gimenez-Roqueplo, A.P., Favier, J., Rustin, P., Rieubland, C., Crespin, M., Nau, V., Khau Van Kien, P., Corvol, P., Plouin, P.F., Jeunemaitre, X. Cancer Res. (2003) [Pubmed]
  19. Immunocytochemical demonstration of IGF-II immunoreactivity in human phaeochromocytoma and extra-adrenal abdominal paraganglioma. Suzuki, T., Watanabe, K., Sugino, T., Tanigawa, T., Satoh, S. J. Pathol. (1992) [Pubmed]
  20. Novel mutations in the SDHD gene in pedigrees with familial carotid body paraganglioma and sensorineural hearing loss. Badenhop, R.F., Cherian, S., Lord, R.S., Baysal, B.E., Taschner, P.E., Schofield, P.R. Genes Chromosomes Cancer (2001) [Pubmed]
  21. Skull base tumors: gadodiamide injection--enhanced MR imaging--drop-out effect in the early enhancement pattern of paragangliomas versus different tumors. Vogl, T.J., Mack, M.G., Juergens, M., Bergman, C., Grevers, G., Jacobsen, T.F., Lissner, J., Felix, R. Radiology. (1993) [Pubmed]
  22. Pancreatic somatostatin-secreting gangliocytic paraganglioma with lymph node metastases. Tomic, S., Warner, T. Am. J. Gastroenterol. (1996) [Pubmed]
  23. Gene mutations in the succinate dehydrogenase subunit SDHB cause susceptibility to familial pheochromocytoma and to familial paraganglioma. Astuti, D., Latif, F., Dallol, A., Dahia, P.L., Douglas, F., George, E., Sköldberg, F., Husebye, E.S., Eng, C., Maher, E.R. Am. J. Hum. Genet. (2001) [Pubmed]
  24. The pressure rises: update on the genetics of phaeochromocytoma. Maher, E.R., Eng, C. Hum. Mol. Genet. (2002) [Pubmed]
  25. Immunohistochemical localization of epinephrine, norepinephrine, catecholamine-synthesizing enzymes, and chromogranin in neuroendocrine cells and tumors. Lloyd, R.V., Sisson, J.C., Shapiro, B., Verhofstad, A.A. Am. J. Pathol. (1986) [Pubmed]
  26. New genetic causes of pheochromocytoma: current concepts and the clinical relevance. Neumann, H.P., Cybulla, M., Shibata, H., Oya, M., Naruse, M., Higashihara, E., Terachi, T., Ling, H., Takami, H., Shuin, T., Murai, M. The Keio journal of medicine. (2005) [Pubmed]
  27. The SDH mutation database: an online resource for succinate dehydrogenase sequence variants involved in pheochromocytoma, paraganglioma and mitochondrial complex II deficiency. Bayley, J.P., Devilee, P., Taschner, P.E. BMC Med. Genet. (2005) [Pubmed]
  28. Malignant paragangliomas associated with mutations in the succinate dehydrogenase D gene. Havekes, B., Corssmit, E.P., Jansen, J.C., van der Mey, A.G., Vriends, A.H., Romijn, J.A. J. Clin. Endocrinol. Metab. (2007) [Pubmed]
  29. Gross SDHB deletions in patients with paraganglioma detected by multiplex PCR: a possible hot spot? Cascón, A., Montero-Conde, C., Ruiz-Llorente, S., Mercadillo, F., Letón, R., Rodríguez-Antona, C., Martínez-Delgado, B., Delgado, M., Díez, A., Rovira, A., Díaz, J.A., Robledo, M. Genes Chromosomes Cancer (2006) [Pubmed]
  30. Primary paraganglioma strictly confined to the liver and mimicking hepatocellular carcinoma: an immunohistochemical and in situ hybridization study. Corti, B., D'Errico, A., Pierangeli, F., Fiorentino, M., Altimari, A., Grigioni, W.F. Am. J. Surg. Pathol. (2002) [Pubmed]
  31. Decreased expression of neuropeptides in malignant paragangliomas: an immunohistochemical study. Linnoila, R.I., Lack, E.E., Steinberg, S.M., Keiser, H.R. Hum. Pathol. (1988) [Pubmed]
  32. Paraganglioma of the cauda equina. A case report and review of the literature. Bak, J., Olsson, Y., Grimelius, L., Spännare, B. APMIS (1996) [Pubmed]
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