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1. Erlic Z, Neumann HP: When should genetic testing be obtained in a patient with phaeochromocytoma or paraganglioma? Clin Endocrinol (Oxf); 2009 Mar;70(3):354-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] When should genetic testing be obtained in a patient with phaeochromocytoma or paraganglioma?
  • About 30% of phaeochromocytoma and paraganglioma patients harbour a germline mutation in one of the known susceptibility genes and in more than one-third of these patients there is no family history for these tumours.
  • Since several genes are involved in the genetics of phaeochromocytoma and paraganglioma, prioritizing which gene(s) to be tested first by using simple clinical information can reduce the efforts and costs of this analysis.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Genetic Testing. Germ-Line Mutation / genetics. Paraganglioma / genetics. Pheochromocytoma / genetics

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  • (PMID = 19067729.001).
  • [ISSN] 1365-2265
  • [Journal-full-title] Clinical endocrinology
  • [ISO-abbreviation] Clin. Endocrinol. (Oxf)
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / SDHD protein, human; EC 1.3.5.1 / SDHB protein, human; EC 1.3.99.1 / Succinate Dehydrogenase; EC 2.7.10.1 / Proto-Oncogene Proteins c-ret; EC 2.7.10.1 / RET protein, human; EC 6.3.2.19 / VHL protein, human; EC 6.3.2.19 / Von Hippel-Lindau Tumor Suppressor Protein
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2. Sawhney SA, Chapman AD, Carney JA, Gomersall LN, Dempsey OJ: Incomplete Carney triad--a review of two cases. QJM; 2009 Sep;102(9):649-53
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • 'Carney's triad' characteristically includes multifocal pulmonary chondroma, gastric stromal sarcoma and extra-adrenal paraganglioma.
  • [MeSH-major] Chondroma / diagnosis. Gastrointestinal Stromal Tumors / diagnosis. Liver Neoplasms / diagnosis. Lung Neoplasms / diagnosis. Paraganglioma, Extra-Adrenal / diagnosis

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  • (PMID = 19561114.001).
  • [ISSN] 1460-2393
  • [Journal-full-title] QJM : monthly journal of the Association of Physicians
  • [ISO-abbreviation] QJM
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Anti-Bacterial Agents; 0 / Antineoplastic Agents; 0 / Benzamides; 0 / Piperazines; 0 / Pyrimidines; 8A1O1M485B / Imatinib Mesylate
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3. Pacak K, Eisenhofer G, Ilias I: Diagnosis of pheochromocytoma with special emphasis on MEN2 syndrome. Hormones (Athens); 2009 Apr-Jun;8(2):111-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • For adrenal PHEOs, this method seems to be comparable to other functional modalities such as [18F]-fluorodopa ([18F]DOPA) PET or [123I]-metaiodobenzylguanidine ([123I]MIBG)scintigraphy.
  • [MeSH-major] Adrenal Gland Neoplasms / radionuclide imaging. Dihydroxyphenylalanine / analogs & derivatives. Fluorine Radioisotopes. Multiple Endocrine Neoplasia Type 2a / complications. Paraganglioma / pathology. Pheochromocytoma / radionuclide imaging

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  • (PMID = 19570738.001).
  • [ISSN] 1109-3099
  • [Journal-full-title] Hormones (Athens, Greece)
  • [ISO-abbreviation] Hormones (Athens)
  • [Language] eng
  • [Grant] United States / Intramural NIH HHS / / Z01 HD008735-08
  • [Publication-type] Journal Article; Research Support, N.I.H., Intramural; Review
  • [Publication-country] Greece
  • [Chemical-registry-number] 0 / Fluorine Radioisotopes; 2C598205QX / fluorodopa F 18; 63-84-3 / Dihydroxyphenylalanine
  • [Number-of-references] 38
  • [Other-IDs] NLM/ NIHMS750185; NLM/ PMC4713023
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4. Tahir M, Noor SJ, Herle A, Downing S: Right atrial paraganglioma: a rare primary cardiac neoplasm as a cause of chest pain. Tex Heart Inst J; 2009;36(6):594-7
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  • [Title] Right atrial paraganglioma: a rare primary cardiac neoplasm as a cause of chest pain.
  • The tumor was successfully resected, and the histopathologic features were consistent with paraganglioma.

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  • (PMID = 20069088.001).
  • [ISSN] 1526-6702
  • [Journal-full-title] Texas Heart Institute journal
  • [ISO-abbreviation] Tex Heart Inst J
  • [Language] ENG
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers
  • [Other-IDs] NLM/ PMC2801953
  • [Keywords] NOTNLM ; Angina pectoris / computed tomography / heart atria/pathology/surgery / heart neoplasms/diagnosis / paraganglioma/diagnosis/surgery
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5. Niveditha SR, Suguna BV, Krishnamurthy, Rajnikanth, Babu KG, Shukla AK: Cytologic features of malignant cystic pheochromocytoma: a case report. Acta Cytol; 2007 Mar-Apr;51(2):200-2
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Medulla / pathology. Carcinoma, Renal Cell / diagnosis. Cysts / pathology. Paraganglioma / diagnosis. Pheochromocytoma / diagnosis


6. Alzahrani AS, Alshaikh O, Faiyaz-Ul-Haque M, Abalkhail H, Al-Dayel F, Hindi HA: Multiple paraganglioma syndrome type 4 due to succinate dehydrogenase B mutation: diagnostic and therapeutic challenges of a skull base paraganglioma masquerading as nasopharyngeal cancer. Endocr Pract; 2010 May-Jun;16(3):452-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Multiple paraganglioma syndrome type 4 due to succinate dehydrogenase B mutation: diagnostic and therapeutic challenges of a skull base paraganglioma masquerading as nasopharyngeal cancer.
  • OBJECTIVE: To report a case of hereditary paraganglioma and describe the underlying genetic mutation and response to iodine 131 metaiodobenzylguanidine (MIBG) therapy.
  • During tumor staging, abdominal computed tomography showed a large, locally invasive left adrenal tumor.
  • MIBG scan showed uptake in the left adrenal gland and in the skull mass.
  • Biopsy of the nasopharyngeal mass confirmed the diagnosis of paraganglioma.
  • The patient underwent resection of the 13-cm pheochromocytoma in the left adrenal gland, with resection of part of the colon and kidney.
  • The nasopharyngeal paraganglioma was inoperable.
  • CONCLUSIONS: This case illustrates the diagnostic and therapeutic challenges of hereditary paraganglioma and the value of genetic testing.
  • [MeSH-major] Nasopharyngeal Neoplasms / diagnosis. Paraganglioma / diagnosis. Paraganglioma / genetics. Succinate Dehydrogenase / genetics

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  • (PMID = 20061288.001).
  • [ISSN] 1934-2403
  • [Journal-full-title] Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists
  • [ISO-abbreviation] Endocr Pract
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 35MRW7B4AD / 3-Iodobenzylguanidine; EC 1.3.99.1 / Succinate Dehydrogenase
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7. Allred JD, Mehta D, Courville KA, Aqel R: Mediastinal mass with blood supply from the coronary arteries. Clin Cardiol; 2009 Jul;32(7):E49
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [MeSH-major] Coronary Vessels / pathology. Mediastinal Neoplasms / blood supply. Paraganglioma, Extra-Adrenal / blood supply

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  • (PMID = 17803230.001).
  • [ISSN] 1932-8737
  • [Journal-full-title] Clinical cardiology
  • [ISO-abbreviation] Clin Cardiol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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8. Chetty R: Familial paraganglioma syndromes. J Clin Pathol; 2010 Jun;63(6):488-91
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Familial paraganglioma syndromes.
  • The familial paraganglioma-phaeochromocytoma syndromes are less well recognised, but as more genetic testing is performed, more cases belonging to this syndrome will be revealed.
  • Patients with SDHB mutations have a positive family history in 33% of cases, present with single tumours around 30 years of age and have extra-adrenal paragangliomas mainly in the abdomen and pelvis; 20% may also have phaeochromocytomas, and tumours in these patients have a great propensity to metastasise.
  • [MeSH-major] Neoplastic Syndromes, Hereditary / genetics. Paraganglioma / genetics. Pheochromocytoma / genetics

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  • (PMID = 20498024.001).
  • [ISSN] 1472-4146
  • [Journal-full-title] Journal of clinical pathology
  • [ISO-abbreviation] J. Clin. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] EC 1.3.99.1 / Succinate Dehydrogenase
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9. Rosing JH, Jeffrey RB, Longacre TA, Greco RS: Massive extra-adrenal retroperitoneal paraganglioma: pre-operative embolization and resection. Dig Dis Sci; 2009 Aug;54(8):1621-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Massive extra-adrenal retroperitoneal paraganglioma: pre-operative embolization and resection.
  • [MeSH-major] Embolization, Therapeutic / methods. Paraganglioma, Extra-Adrenal / surgery. Paraganglioma, Extra-Adrenal / therapy. Preoperative Care / methods. Retroperitoneal Neoplasms / surgery. Retroperitoneal Neoplasms / therapy

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  • (PMID = 19408117.001).
  • [ISSN] 1573-2568
  • [Journal-full-title] Digestive diseases and sciences
  • [ISO-abbreviation] Dig. Dis. Sci.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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10. Rossi GP, Seccia TM, Pessina AC: Secondary hypertension: the ways of management. Curr Vasc Pharmacol; 2010 Nov;8(6):753-68
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [MeSH-minor] Adrenal Gland Diseases / diagnosis. Adrenal Gland Diseases / physiopathology. Adrenal Gland Diseases / surgery. Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / genetics. Adrenal Gland Neoplasms / physiopathology. Adrenal Gland Neoplasms / surgery. Adult. Aged. Aged, 80 and over. Female. Humans. Hyperaldosteronism / diagnosis. Hyperaldosteronism / physiopathology. Hypertension, Renovascular / diagnosis. Hypertension, Renovascular / drug therapy. Hypertension, Renovascular / physiopathology. Hypertension, Renovascular / surgery. Male. Middle Aged. Paraganglioma / diagnosis. Paraganglioma / physiopathology. Pheochromocytoma / diagnosis. Pheochromocytoma / genetics. Pheochromocytoma / physiopathology. Pheochromocytoma / surgery. Prevalence. Young Adult

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  • (PMID = 20626342.001).
  • [ISSN] 1875-6212
  • [Journal-full-title] Current vascular pharmacology
  • [ISO-abbreviation] Curr Vasc Pharmacol
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United Arab Emirates
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11. Duet M, Herman P, Wassef M, Tran BH, Laredo JD: Bone metastases from head and neck paragangliomas: uncommon MR findings in an uncommon condition--report of three cases. J Magn Reson Imaging; 2006 Aug;24(2):428-33
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • These findings indicate that MRI should be included in the staging of paraganglioma patients with risk factors for malignancy.
  • [MeSH-major] Bone Neoplasms / secondary. Head and Neck Neoplasms / pathology. Magnetic Resonance Imaging / methods. Paraganglioma, Extra-Adrenal / secondary

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  • (PMID = 16786569.001).
  • [ISSN] 1053-1807
  • [Journal-full-title] Journal of magnetic resonance imaging : JMRI
  • [ISO-abbreviation] J Magn Reson Imaging
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Indium Radioisotopes; 0 / Iodine Radioisotopes; 0 / Radiopharmaceuticals; 35MRW7B4AD / 3-Iodobenzylguanidine; 51110-01-1 / Somatostatin; G083B71P98 / pentetreotide
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12. Huang J, Lemire BD: Mutations in the C. elegans succinate dehydrogenase iron-sulfur subunit promote superoxide generation and premature aging. J Mol Biol; 2009 Apr 3;387(3):559-69
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Mutations in the human SDHB, SDHC and SDHD genes are responsible for the development of paraganglioma and pheochromocytoma, tumors of the head and neck or the adrenal medulla, respectively.

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  • (PMID = 19233206.001).
  • [ISSN] 1089-8638
  • [Journal-full-title] Journal of molecular biology
  • [ISO-abbreviation] J. Mol. Biol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antioxidants; 0 / Caenorhabditis elegans Proteins; 0 / Herbicides; 0 / Protein Subunits; 0 / Reactive Oxygen Species; 11062-77-4 / Superoxides; EC 1.3.99.1 / Succinate Dehydrogenase; PLG39H7695 / Paraquat; PQ6CK8PD0R / Ascorbic Acid; WYQ7N0BPYC / Acetylcysteine
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13. Grossrubatscher E, Dalino P, Vignati F, Gambacorta M, Pugliese R, Boniardi M, Rossetti O, Marocchi A, Bertuzzi M, Loli P: The role of chromogranin A in the management of patients with phaeochromocytoma. Clin Endocrinol (Oxf); 2006 Sep;65(3):287-93
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Supranormal CgA concentrations have been recorded in patients with tumours of neuroectodermal origin such as phaeochromocytoma and paraganglioma.
  • [MeSH-major] Adrenal Gland Neoplasms / blood. Biomarkers, Tumor / blood. Chromogranin A / blood. Pheochromocytoma / blood

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  • (PMID = 16918946.001).
  • [ISSN] 0300-0664
  • [Journal-full-title] Clinical endocrinology
  • [ISO-abbreviation] Clin. Endocrinol. (Oxf)
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Catecholamines; 0 / Chromogranin A; 0J45DE6B88 / Normetanephrine; 5001-33-2 / Metanephrine
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14. Bayley JP, van Minderhout I, Hogendoorn PC, Cornelisse CJ, van der Wal A, Prins FA, Teppema L, Dahan A, Devilee P, Taschner PE: Sdhd and SDHD/H19 knockout mice do not develop paraganglioma or pheochromocytoma. PLoS One; 2009;4(11):e7987
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Sdhd and SDHD/H19 knockout mice do not develop paraganglioma or pheochromocytoma.
  • Mutations of SDHD, the first protein of intermediary metabolism shown to be involved in tumorigenesis, lead to the human tumors paraganglioma (PGL) and pheochromocytoma (PC).
  • No paraganglioma or other tumor development was seen in Sdhd KO mice followed for their entire lifespan, in sharp contrast to the highly penetrant phenotype in humans.
  • Heterozygous Sdhd KO mice did not show hyperplasia of paraganglioma-related tissues such as the carotid body or of the adrenal medulla, or any genotype-related pathology, with similar body and organ weights to wildtype mice.
  • [MeSH-major] Mutation. Paraganglioma / genetics. Pheochromocytoma / genetics. RNA, Untranslated / genetics. Succinate Dehydrogenase / genetics


15. Pawlu C, Bausch B, Neumann HP: Mutations of the SDHB and SDHD genes. Fam Cancer; 2005;4(1):49-54
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Mutations in the SDHB (1p35-36) and SDHD subunits (11q23) give rise to the paraganglioma syndromes (PGL), namely PGL 4 and PGL 1, and generate paraganglioma and pheochromocytoma.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Iron-Sulfur Proteins / genetics. Membrane Proteins / genetics. Mutation. Paraganglioma / genetics. Pheochromocytoma / genetics. Protein Subunits / genetics. Succinate Dehydrogenase / genetics

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  • (PMID = 15883710.001).
  • [ISSN] 1389-9600
  • [Journal-full-title] Familial cancer
  • [ISO-abbreviation] Fam. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / Iron-Sulfur Proteins; 0 / Membrane Proteins; 0 / Protein Subunits; 0 / SDHD protein, human; EC 1.3.5.1 / SDHB protein, human; EC 1.3.99.1 / Succinate Dehydrogenase
  • [Number-of-references] 26
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16. Pagni F, Galbiati E, Bono F, Di Bella C: Renal hilus paraganglioma: a case report and brief review. Pathologica; 2009 Apr;101(2):89-92
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  • [Title] Renal hilus paraganglioma: a case report and brief review.
  • Paraganglioma is a rare tumour that originates from any paraganglia.
  • Among extra-adrenal paraganglioma, renal hilus is a rare location.
  • The final diagnosis was renal hilus paraganglioma.
  • The paper shows the difficulty in diagnostic approaches to paraganglioma in this atypical site.
  • [MeSH-major] Kidney Neoplasms / pathology. Paraganglioma, Extra-Adrenal / pathology

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  • (PMID = 19886555.001).
  • [ISSN] 0031-2983
  • [Journal-full-title] Pathologica
  • [ISO-abbreviation] Pathologica
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
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17. Newton JD, Munir S, Bhindi R, Ormerod O: What a headache: rare neuroendocrine indication for cardiopulmonary bypass for severe left ventricular dysfunction and shock. Circ Heart Fail; 2008 Jul;1(2):143-5
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  • [MeSH-major] Cardiopulmonary Bypass / methods. Headache / etiology. Paraganglioma / complications. Shock / blood. Ventricular Dysfunction, Left / blood
  • [MeSH-minor] Adrenal Gland Neoplasms / complications. Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / surgery. Diagnosis, Differential. Follow-Up Studies. Humans. Laparotomy. Male. Middle Aged. Tomography, X-Ray Computed


18. López-Jiménez E, Gómez-López G, Leandro-García LJ, Muñoz I, Schiavi F, Montero-Conde C, de Cubas AA, Ramires R, Landa I, Leskelä S, Maliszewska A, Inglada-Pérez L, de la Vega L, Rodríguez-Antona C, Letón R, Bernal C, de Campos JM, Diez-Tascón C, Fraga MF, Boullosa C, Pisano DG, Opocher G, Robledo M, Cascón A: Research resource: Transcriptional profiling reveals different pseudohypoxic signatures in SDHB and VHL-related pheochromocytomas. Mol Endocrinol; 2010 Dec;24(12):2382-91
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  • The six major genes involved in hereditary susceptibility for pheochromocytoma (PCC)/paraganglioma (PGL) (RET, VHL, NF1, SDHB, SDHC, and SDHD) have been recently integrated into the same neuronal apoptotic pathway where mutations in any of these genes lead to cell death.
  • [MeSH-major] Cell Death / genetics. Paraganglioma / genetics. Pheochromocytoma / genetics. Succinate Dehydrogenase / genetics. Von Hippel-Lindau Tumor Suppressor Protein / genetics
  • [MeSH-minor] Adolescent. Adrenal Gland Neoplasms / genetics. Adrenal Gland Neoplasms / metabolism. Adult. Aged. Basic Helix-Loop-Helix Transcription Factors / genetics. Basic Helix-Loop-Helix Transcription Factors / metabolism. Child. Dioxygenases / genetics. Dioxygenases / metabolism. Humans. Hypoxia-Inducible Factor 1, alpha Subunit / genetics. Hypoxia-Inducible Factor 1, alpha Subunit / metabolism. Hypoxia-Inducible Factor-Proline Dioxygenases. Middle Aged. Neoplasms / genetics. Young Adult. von Hippel-Lindau Disease / genetics. von Hippel-Lindau Disease / metabolism

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  • (PMID = 20980436.001).
  • [ISSN] 1944-9917
  • [Journal-full-title] Molecular endocrinology (Baltimore, Md.)
  • [ISO-abbreviation] Mol. Endocrinol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Basic Helix-Loop-Helix Transcription Factors; 0 / HIF1A protein, human; 0 / Hypoxia-Inducible Factor 1, alpha Subunit; 0 / endothelial PAS domain-containing protein 1; EC 1.13.11.- / Dioxygenases; EC 1.14.11.29 / EGLN3 protein, human; EC 1.14.11.29 / Hypoxia-Inducible Factor-Proline Dioxygenases; EC 1.3.5.1 / SDHB protein, human; EC 1.3.99.1 / Succinate Dehydrogenase; EC 2.3.2.27 / Von Hippel-Lindau Tumor Suppressor Protein; EC 6.3.2.- / VHL protein, human
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19. Wen J, Li HZ, Ji ZG, Mao QZ, Shi BB, Yan WG: A decade of clinical experience with extra-adrenal paragangliomas of retroperitoneum: Report of 67 cases and a literature review. Urol Ann; 2010 Jan;2(1):12-6
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  • [Title] A decade of clinical experience with extra-adrenal paragangliomas of retroperitoneum: Report of 67 cases and a literature review.
  • OBJECT: The purpose was to highlight the diagnosis and treatment of extra-adrenal para-gangliomas, which often causes catecholamine hypersecretion and hypertension.
  • METHODS: 67 cases of extra-adrenal paraganglioma of retroperitoneum proven pathologically from 1999 to 2009 were reviewed and studied after operation.
  • There were nine cases assayed malignant paraganglioma by the follow-up.
  • CONCLUSIONS: 131-MIBG and octreotide have high sensitivity and accuracy in diagosing extra-adrenal paraganglioma.
  • Complete surgical excision is the treatment of choice for extra-adrenal paragangliomas as well as recurrent or metastatic disease, which could be resected laparoscopically.

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  • (PMID = 20842251.001).
  • [ISSN] 0974-7834
  • [Journal-full-title] Urology annals
  • [ISO-abbreviation] Urol Ann
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] India
  • [Other-IDs] NLM/ PMC2934586
  • [Keywords] NOTNLM ; Extra-adrenal paraganglioma / diagnosis / retroperitoneal tumor / treatment
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20. Horger M, Fritz J, Bares R, Müssig K: [Diagnosis of hyperfunctioning adrenal masses]. Rofo; 2006 Dec;178(12):1171-6
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  • [Title] [Diagnosis of hyperfunctioning adrenal masses].
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / secretion. Pheochromocytoma / diagnosis. Pheochromocytoma / secretion
  • [MeSH-minor] Adrenal Gland Diseases / diagnosis. Adrenal Gland Diseases / metabolism. Aldosterone / biosynthesis. Cushing Syndrome / diagnosis. Cysts / diagnosis. Diagnosis, Differential. Female. Humans. Hydrocortisone / biosynthesis. Male. Middle Aged. Paraganglioma / diagnosis. Paraganglioma / secretion

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  • (PMID = 17396301.001).
  • [ISSN] 1438-9029
  • [Journal-full-title] RöFo : Fortschritte auf dem Gebiete der Röntgenstrahlen und der Nuklearmedizin
  • [ISO-abbreviation] Rofo
  • [Language] ger
  • [Publication-type] Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 4964P6T9RB / Aldosterone; WI4X0X7BPJ / Hydrocortisone
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21. Chang H, Xu L, Mu Q: Primary functioning hepatic paraganglioma: a case report. Adv Ther; 2006 Sep-Oct;23(5):817-20
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  • [Title] Primary functioning hepatic paraganglioma: a case report.
  • An extra-adrenal pheochromocytoma is known as a paraganglioma.
  • This report describes a patient with a rare primary functioning hepatic paraganglioma that resulted in hypertension.
  • [MeSH-major] Hypertension / etiology. Liver Neoplasms / complications. Paraganglioma / complications

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  • (PMID = 17142218.001).
  • [ISSN] 0741-238X
  • [Journal-full-title] Advances in therapy
  • [ISO-abbreviation] Adv Ther
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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22. Schuller S, Van Israël N, Else RW: Third degree atrioventricular block and accelerated idioventricular rhythm associated with a heart base chemodectoma in a syncopal Rottweiler. J Vet Med A Physiol Pathol Clin Med; 2007 Dec;54(10):618-23
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  • [MeSH-major] Atrioventricular Block / veterinary. Dog Diseases / diagnosis. Heart Neoplasms / veterinary. Paraganglioma, Extra-Adrenal / veterinary

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  • (PMID = 18045349.001).
  • [ISSN] 0931-184X
  • [Journal-full-title] Journal of veterinary medicine. A, Physiology, pathology, clinical medicine
  • [ISO-abbreviation] J Vet Med A Physiol Pathol Clin Med
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
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23. Ahallal Y, Tazi MF, Elfatemi H, Znati K, Tazi E, Amarti A, El Fassi MJ, Moulay HF: Renal hilar pheochromocytoma: a case report. Cases J; 2009;2:6416
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  • Paraganglioma is a rare tumor arising from undifferentiated cells of the primitive neural crest.
  • These tumors are most commonly found in the adrenal gland, other localisations are also possible.

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  • (PMID = 19829802.001).
  • [ISSN] 1757-1626
  • [Journal-full-title] Cases journal
  • [ISO-abbreviation] Cases J
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24. Tran Ba Huy P, Duet M, Abulizi M, Crassard I, Guichard JP, Herman P: Skull base paraganglioma and intracranial hypertension. Arch Otolaryngol Head Neck Surg; 2010 Jan;136(1):91-4
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  • [Title] Skull base paraganglioma and intracranial hypertension.
  • [MeSH-major] Intracranial Hypertension / etiology. Paraganglioma, Extra-Adrenal / complications. Skull Base Neoplasms / complications

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  • (PMID = 20083786.001).
  • [ISSN] 1538-361X
  • [Journal-full-title] Archives of otolaryngology--head & neck surgery
  • [ISO-abbreviation] Arch. Otolaryngol. Head Neck Surg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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25. Boedeker CC, Neumann HP, Ridder GJ, Maier W, Schipper J: Paragangliomas in patients with mutations of the SDHD gene. Otolaryngol Head Neck Surg; 2005 Mar;132(3):467-70
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  • RESULTS: Five patients presented with head and neck paragangliomas, 1 patient with a thoracic paraganglioma, and 2 patients with intraabdominal paragangliomas.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Membrane Proteins / genetics. Mutation. Neoplasms, Multiple Primary / genetics. Paraganglioma / genetics. Pheochromocytoma / genetics

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  • (PMID = 15746863.001).
  • [ISSN] 0194-5998
  • [Journal-full-title] Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery
  • [ISO-abbreviation] Otolaryngol Head Neck Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Membrane Proteins; 0 / SDHD protein, human; EC 1.3.99.1 / Succinate Dehydrogenase
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26. Sandgren J, Diaz de Ståhl T, Andersson R, Menzel U, Piotrowski A, Nord H, Bäckdahl M, Kiss NB, Brauckhoff M, Komorowski J, Dralle H, Hessman O, Larsson C, Akerström G, Bruder C, Dumanski JP, Westin G: Recurrent genomic alterations in benign and malignant pheochromocytomas and paragangliomas revealed by whole-genome array comparative genomic hybridization analysis. Endocr Relat Cancer; 2010 Sep;17(3):561-79
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  • Pheochromocytomas and abdominal paragangliomas are adrenal and extra-adrenal catecholamine-producing tumours.
  • Our study revealed novel and narrow recurrent chromosomal regions of loss and gain at several autosomes, a prerequisite for identifying candidate tumour suppressor genes and oncogenes involved in the development of adrenal and extra-adrenal catecholamine-producing tumours.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Paraganglioma / genetics

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  • (PMID = 20410162.001).
  • [ISSN] 1479-6821
  • [Journal-full-title] Endocrine-related cancer
  • [ISO-abbreviation] Endocr. Relat. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
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27. Unal M, Polat A, Pata YS, Vayisoğlu Y, Yildiz A, Ismi O: Paraganglioma of the skull base: a case report. Auris Nasus Larynx; 2007 Sep;34(3):427-30
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  • [Title] Paraganglioma of the skull base: a case report.
  • Paragangliomas are rare benign neuroendocrine tumors derived from the extra-adrenal paraganglia of the autonomic nervous system.
  • Here, we described a new case of a 75-year-old woman with paraganglioma arising in the middle and posterior cranial fossa with extended destruction of the skull base and clivus.
  • [MeSH-major] Paraganglioma / diagnosis. Skull Base Neoplasms / diagnosis

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  • (PMID = 17331688.001).
  • [ISSN] 0385-8146
  • [Journal-full-title] Auris, nasus, larynx
  • [ISO-abbreviation] Auris Nasus Larynx
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Chromogranin A; 0 / S100 Proteins; 0 / Synaptophysin
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28. Lendvai N, Szabó I, Butz H, Beko G, Horányi J, Tarjányi M, Alföldi S, Szabó I, Rácz K, Patócs A: [Extra-adrenal pheochromocytoma associated to SDHD gene mutation]. Orv Hetil; 2009 Apr 5;150(14):645-9
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  • [Title] [Extra-adrenal pheochromocytoma associated to SDHD gene mutation].
  • Hereditary paraganglioma/pheochromocytoma syndrome is an autosomal dominantly inherited disease caused by germline mutation of the genes encoding subunits of the mitochondrial succinate dehydrogenase (SDH) enzyme involved in the mitochondrial respiratory chain.
  • Authors present the history of a patient with extra-adrenal pheochromocytoma who represents in Hungary the first genetically confirmed case of hereditary paraganglioma/pheochromocytoma syndrome due to disease-causing mutation of the SDHD gene.
  • Histological examination of the tumor proved extra-adrenal pheochromocytoma.
  • CONCLUSION: The presented patient represents the first case with hereditary paraganglioma/pheochromocytoma syndrome from Hungary, in whom genetic analysis identified a disease-causing mutation of the SDHD gene.
  • [MeSH-minor] Adrenal Gland Neoplasms / genetics. Adult. Base Sequence. DNA Mutational Analysis. Genetic Predisposition to Disease. Humans. Hungary. Male. Membrane Proteins / genetics. Molecular Sequence Data. Positron-Emission Tomography / methods. Tomography, X-Ray Computed. Von Hippel-Lindau Tumor Suppressor Protein / genetics

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  • (PMID = 19318336.001).
  • [ISSN] 0030-6002
  • [Journal-full-title] Orvosi hetilap
  • [ISO-abbreviation] Orv Hetil
  • [Language] hun
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Hungary
  • [Chemical-registry-number] 0 / Membrane Proteins; 0 / SDHC protein, human; 0 / SDHD protein, human; EC 1.3.5.1 / SDHB protein, human; EC 1.3.99.1 / Succinate Dehydrogenase; EC 6.3.2.19 / VHL protein, human; EC 6.3.2.19 / Von Hippel-Lindau Tumor Suppressor Protein
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29. Whitson BA, Tuttle TM: Laparoscopic resection of periaortic paragangliomas. Am Surg; 2005 May;71(5):450-4
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  • Paragangliomas are extra-adrenal pheochromocytomas that are often located in the retroperitoneum along the aorta.
  • Although laparoscopic resection is recommended for adrenal pheochromocytomas, few laparoscopic resections for paragangliomas have been reported.
  • We describe the presentation and imaging findings of four patients with periarotic paraganglioma.
  • [MeSH-major] Laparoscopy. Paraganglioma, Extra-Adrenal / surgery. Retroperitoneal Neoplasms / surgery

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  • (PMID = 15986981.001).
  • [ISSN] 0003-1348
  • [Journal-full-title] The American surgeon
  • [ISO-abbreviation] Am Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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30. Korpershoek E, Stobbe CK, van Nederveen FH, de Krijger RR, Dinjens WN: Intra-tumoral molecular heterogeneity in benign and malignant pheochromocytomas and extra-adrenal sympathetic paragangliomas. Endocr Relat Cancer; 2010 Sep;17(3):653-62
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  • [Title] Intra-tumoral molecular heterogeneity in benign and malignant pheochromocytomas and extra-adrenal sympathetic paragangliomas.
  • Pheochromocytomas (PCCs) and extra-adrenal sympathetic paragangliomas (sPGLs) are catecholamine-producing tumors occurring in the context of hereditary tumor syndromes, with known germline mutations, and as sporadic tumors.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Chromosomes, Human. Loss of Heterozygosity. Paraganglioma / genetics. Pheochromocytoma / genetics

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  • (PMID = 20488782.001).
  • [ISSN] 1479-6821
  • [Journal-full-title] Endocrine-related cancer
  • [ISO-abbreviation] Endocr. Relat. Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / DNA, Neoplasm
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31. Petersen J, Cooper G, Drew P, Silverstein B, Beave T: Paraganglioma resection requiring left atrial reconstruction. Clin Cardiol; 2010 Mar;33(3):E75-7
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  • [Title] Paraganglioma resection requiring left atrial reconstruction.
  • Paragangliomas, also known as extra-adrenal pheochromocytomas, arise from chromaffin cells within the autonomonic ganglia.
  • [MeSH-major] Heart Atria / surgery. Heart Neoplasms / surgery. Paraganglioma / surgery

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  • [Copyright] Copyright (c) 2009 Wiley Periodicals, Inc.
  • (PMID = 20127902.001).
  • [ISSN] 1932-8737
  • [Journal-full-title] Clinical cardiology
  • [ISO-abbreviation] Clin Cardiol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Catecholamines
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32. Müller U, Troidl C, Niemann S: SDHC mutations in hereditary paraganglioma/pheochromocytoma. Fam Cancer; 2005;4(1):9-12
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] SDHC mutations in hereditary paraganglioma/pheochromocytoma.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Adrenal Gland Neoplasms / physiopathology. Membrane Proteins / genetics. Paraganglioma / genetics. Paraganglioma / physiopathology. Pheochromocytoma / genetics. Pheochromocytoma / physiopathology

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  • (PMID = 15883704.001).
  • [ISSN] 1389-9600
  • [Journal-full-title] Familial cancer
  • [ISO-abbreviation] Fam. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] Netherlands
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  • [Number-of-references] 18
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33. Mannelli M, Castellano M, Schiavi F, Filetti S, Giacchè M, Mori L, Pignataro V, Bernini G, Giachè V, Bacca A, Biondi B, Corona G, Di Trapani G, Grossrubatscher E, Reimondo G, Arnaldi G, Giacchetti G, Veglio F, Loli P, Colao A, Ambrosio MR, Terzolo M, Letizia C, Ercolino T, Opocher G, Italian Pheochromocytoma/Paraganglioma Network: Clinically guided genetic screening in a large cohort of italian patients with pheochromocytomas and/or functional or nonfunctional paragangliomas. J Clin Endocrinol Metab; 2009 May;94(5):1541-7
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  • CONCLUSIONS: The frequency of the hereditary forms of pheochromocytoma/paraganglioma varies depending on the family history and the clinical presentation.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Paraganglioma / genetics. Pheochromocytoma / genetics

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  • (PMID = 19223516.001).
  • [ISSN] 1945-7197
  • [Journal-full-title] The Journal of clinical endocrinology and metabolism
  • [ISO-abbreviation] J. Clin. Endocrinol. Metab.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 9007-49-2 / DNA
  • [Investigator] Mariotti S; Montalto A; Parenti G; Ragghianti B; Zampetti B; Pacini F; Nassi R; Fagiano A; Milone F; Cordisco EL; Demattè S; Cecchini E; Bertola G; Giambona S; Milanese G; Bertuccio A; Pierani P; Fabrizzi B; Grego F; Piazza M; Trabalzini F; Toniato A; Boschin IM; Agliozzo E; Tiberio G; Nicolai P; Mulatero P; Sechi L
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34. Boulogianni G, Chourmouzi D, Sivitanidis E, Drevelegas AK: An incidental nonfunctioning mediastinal paraganglioma (2009: 11b). Aorticosympathetic paraganglioma. Eur Radiol; 2010 Feb;20(2):506-9
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  • [Title] An incidental nonfunctioning mediastinal paraganglioma (2009: 11b). Aorticosympathetic paraganglioma.
  • A rare case of an incidental nonfunctioning mediastinal paraganglioma is presented.
  • The diagnosis of aorticosympathetic paraganglioma was established by histological examination after surgical removal of the tumor.
  • [MeSH-major] Mediastinal Neoplasms / diagnostic imaging. Paraganglioma, Extra-Adrenal / diagnostic imaging

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35. Mannelli M: Management and treatment of pheochromocytomas and paragangliomas. Ann N Y Acad Sci; 2006 Aug;1073:405-16
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [MeSH-major] Adrenal Gland Neoplasms / therapy. Paraganglioma / therapy. Pheochromocytoma / therapy

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  • (PMID = 17102109.001).
  • [ISSN] 0077-8923
  • [Journal-full-title] Annals of the New York Academy of Sciences
  • [ISO-abbreviation] Ann. N. Y. Acad. Sci.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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36. Timmers HJ, Chen CC, Carrasquillo JA, Whatley M, Ling A, Havekes B, Eisenhofer G, Martiniova L, Adams KT, Pacak K: Comparison of 18F-fluoro-L-DOPA, 18F-fluoro-deoxyglucose, and 18F-fluorodopamine PET and 123I-MIBG scintigraphy in the localization of pheochromocytoma and paraganglioma. J Clin Endocrinol Metab; 2009 Dec;94(12):4757-67
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  • [Title] Comparison of 18F-fluoro-L-DOPA, 18F-fluoro-deoxyglucose, and 18F-fluorodopamine PET and 123I-MIBG scintigraphy in the localization of pheochromocytoma and paraganglioma.
  • CONTEXT: Besides (123)I-metaiodobenzylguanidine (MIBG), positron emission tomography (PET) agents are available for the localization of paraganglioma (PGL), including (18)F-3,4-dihydroxyphenylalanine (DOPA), (18)F-fluoro-2-deoxy-D-glucose ((18)F-FDG), and (18)F-fluorodopamine ((18)F-FDA).
  • PATIENTS: Fifty-two patients (28 males, 24 females, aged 46.8 +/- 14.2 yr): 20 with nonmetastatic PGL (11 adrenal), 28 with metastatic PGL (13 adrenal), and four in whom PGL was ruled out; 22 PGLs were of the succinate dehydrogenase subunit B (SDHB) genotype.

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  • (PMID = 19864450.001).
  • [ISSN] 1945-7197
  • [Journal-full-title] The Journal of clinical endocrinology and metabolism
  • [ISO-abbreviation] J. Clin. Endocrinol. Metab.
  • [Language] ENG
  • [Grant] United States / Intramural NIH HHS / /
  • [Publication-type] Journal Article; Research Support, N.I.H., Intramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 0Z5B2CJX4D / Fluorodeoxyglucose F18; 2C598205QX / fluorodopa F 18; 35MRW7B4AD / 3-Iodobenzylguanidine; 59043-70-8 / 6-fluorodopamine; 63-84-3 / Dihydroxyphenylalanine; VTD58H1Z2X / Dopamine
  • [Other-IDs] NLM/ PMC2795662
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37. Widimský J Jr, Zelinka T, Petrák O, Strauch B, Safarík L, Kasalický M, Vranková A, Holaj R: [Diagnostic and therapeutic procedures in pheochromocytoma: current trends]. Vnitr Lek; 2007 Apr;53(4):428-33
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Pheochromocytoma / diagnosis
  • [MeSH-minor] Humans. Hypertension / etiology. Multiple Endocrine Neoplasia Type 2a / diagnosis. Neurofibromatosis 1 / diagnosis. Paraganglioma / diagnosis. von Hippel-Lindau Disease / diagnosis. von Hippel-Lindau Disease / etiology

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  • (PMID = 17578179.001).
  • [ISSN] 0042-773X
  • [Journal-full-title] Vnitr̆ní lékar̆ství
  • [ISO-abbreviation] Vnitr Lek
  • [Language] cze
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Czech Republic
  • [Number-of-references] 25
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38. Havekes B, van der Klaauw AA, Weiss MM, Jansen JC, van der Mey AG, Vriends AH, Bonsing BA, Romijn JA, Corssmit EP: Pheochromocytomas and extra-adrenal paragangliomas detected by screening in patients with SDHD-associated head-and-neck paragangliomas. Endocr Relat Cancer; 2009 Jun;16(2):527-36
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  • [Title] Pheochromocytomas and extra-adrenal paragangliomas detected by screening in patients with SDHD-associated head-and-neck paragangliomas.
  • Pheochromocytomas and extra-adrenal paragangliomas were treated surgically after appropriate blockade.
  • Twenty-eight out of the 93 patients were included in our study and underwent additional imaging for pheochromocytomas/extra-adrenal paragangliomas.
  • In 11 out of the 28 patients intra-adrenal pheochromocytomas were found.
  • Extra-adrenal paragangliomas were discovered in eight patients.
  • The high prevalence of pheochromocytomas/extra-adrenal paragangliomas in patients with SDHD-associated HNP warrants regular screening for tumors in these patients.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Head and Neck Neoplasms / genetics. Paraganglioma, Extra-Adrenal / diagnosis. Pheochromocytoma / diagnosis. Succinate Dehydrogenase / genetics

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  • (PMID = 19289533.001).
  • [ISSN] 1351-0088
  • [Journal-full-title] Endocrine-related cancer
  • [ISO-abbreviation] Endocr. Relat. Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Catecholamines; EC 1.3.5.1 / SDHB protein, human; EC 1.3.99.1 / Succinate Dehydrogenase
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39. Laufer I, Edgar MA, Härtl R: Primary intraosseous paraganglioma of the sacrum: a case report. Spine J; 2007 Nov-Dec;7(6):733-8
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  • [Title] Primary intraosseous paraganglioma of the sacrum: a case report.
  • BACKGROUND CONTEXT: Paragangliomas are neuroendocrine tumors that most frequently arise in the adrenal medulla, carotid body, and glomus jugulare.
  • Operative biopsy revealed that this lesion was a paraganglioma.
  • PURPOSE: To report a rare case of an intrasacral paraganglioma that presented with back pain and radiculopathy and was treated with intralesional decompression, stabilization, and radiation.
  • The patient underwent initially an open biopsy of this lesion that revealed a paraganglioma.
  • [MeSH-major] Paraganglioma / pathology. Sacrum / pathology. Spinal Neoplasms / pathology

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  • (PMID = 17998133.001).
  • [ISSN] 1529-9430
  • [Journal-full-title] The spine journal : official journal of the North American Spine Society
  • [ISO-abbreviation] Spine J
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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40. Kimura N, Ishidate T, Kogawa T, Miura Y, Ishizaka M, Ogita M: A retroperitoneal sympathetic paraganglioma invading the duodenum and mimicking a submucosal tumor. Endocr Pathol; 2008;19(2):128-32
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  • [Title] A retroperitoneal sympathetic paraganglioma invading the duodenum and mimicking a submucosal tumor.
  • We report a case of an autopsy of unusual retroperitoneal sympathetic paraganglioma (SPG) that directly invaded the duodenum and showed expansive growth mimicking a submucosal tumor.
  • An autopsy revealed that the tumor was mainly in the retroperitoneum, measuring 7.5 x 9.5 cm, weighing 600 g and extending into the duodenum, adjacent to the pancreas but free of the adrenal glands.
  • For differential diagnosis, endocrine tumors of the duodenum or pancreas and extra-adrenal SPG were considered.
  • [MeSH-major] Autonomic Nervous System Diseases / pathology. Duodenal Neoplasms / secondary. Nervous System Neoplasms / pathology. Paraganglioma / secondary

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  • (PMID = 18438726.001).
  • [ISSN] 1046-3976
  • [Journal-full-title] Endocrine pathology
  • [ISO-abbreviation] Endocr. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Catecholamines; 0 / Ki-67 Antigen; EC 1.14.16.2 / Tyrosine 3-Monooxygenase; EC 1.14.17.1 / Dopamine beta-Hydroxylase; EC 2.1.1.28 / Phenylethanolamine N-Methyltransferase
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41. Cotesta D, Petramala L, Serra V, Pergolini M, Crescenzi E, Zinnamosca L, De Toma G, Ciardi A, Carbone I, Massa R, Filetti S, Letizia C: Clinical experience with pheochromocytoma in a single centre over 16 years. High Blood Press Cardiovasc Prev; 2009 Dec;16(4):183-93
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  • BACKGROUND: Pheochromocytoma and paraganglioma are rare tumours of neuroectodermal origin.
  • Pheochromocytoma occurs in 0.1-2% of people with hypertension, while the incidence rises to 4-5% in patients with incidental adrenal mass.
  • In 12% of patients, the pheochromocytoma was discovered during radiological images (adrenal incidentaloma).
  • The unilateral adrenal localization was observed in 78% of patients, bilateral in 15% and extra-adrenal in 7% of patients.

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  • (PMID = 23334910.001).
  • [ISSN] 1120-9879
  • [Journal-full-title] High blood pressure & cardiovascular prevention : the official journal of the Italian Society of Hypertension
  • [ISO-abbreviation] High Blood Press Cardiovasc Prev
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] New Zealand
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42. Archontovasilis F, Markogiannakis H, Dikoglou C, Drimousis P, Toutouzas KG, Theodorou D, Katsaragakis S: Paraganglioma of the greater omentum: Case report and review of the literature. World J Surg Oncol; 2007;5:87
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  • [Title] Paraganglioma of the greater omentum: Case report and review of the literature.
  • BACKGROUND: Extra-adrenal, intra-abdominal paraganglioma constitutes a rare neoplasm and, moreover, its location in the greater omentum is extremely infrequent.
  • Histopathology offered the diagnosis of benign greater omentum paraganglioma.
  • CONCLUSION: This is the second reported case of greater omentum paraganglioma.
  • Clinical and imaging data of patients with extra-adrenal, intra-abdominal paragangliomas are variable while many of them may be asymptomatic even when the lesion is quite large.

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  • (PMID = 17683569.001).
  • [ISSN] 1477-7819
  • [Journal-full-title] World journal of surgical oncology
  • [ISO-abbreviation] World J Surg Oncol
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43. Donckier JE, Michel L: Phaeochromocytoma: state-of-the-art. Acta Chir Belg; 2010 Mar-Apr;110(2):140-8
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  • Phaeochromocytomas are catecholamine-secreting tumours that arise from chromaffin cells of the adrenal medulla and extra-adrenal sites.
  • Extra-adrenal phaeochromocytomas are called paragangliomas.
  • A diagnosis of phaeochromocytoma is suspected by typical paroxysmal symptoms, unusual or refractory hypertension, discovery of an adrenal incidentaloma or a family history of phaeochromocytoma or paraganglioma, possibly associated with other genetic syndromes (multiple endocrine neoplasia type 2 A or B, neurofibromatosis type 1 and von Hippel-Lindau disease).
  • [MeSH-major] Adrenal Gland Neoplasms. Pheochromocytoma


44. Agaimy A, Hartmann A: [Hereditary and non-hereditary syndromic gastointestinal stromal tumours]. Pathologe; 2010 Oct;31(6):430-7
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  • In decreasing order of frequency, neurofibromatosis Recklinghausen (NF-1), Carney triad (gastric GIST, pulmonary chondroma and extra-adrenal paraganglioma), familial GIST syndromes resulting from germline mutations in c-Kit/PDGFRA and the Carney-Stratakis syndrome (hereditary GIST paraganglioma syndrome caused by germline mutations in the mitochondrial tumour suppressor gene pathway involving the succinate dehydrogenase subunits SDHD, SDHC and SDHB) represent the four most important GIST syndromes characterized to date.

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  • (PMID = 20848108.001).
  • [ISSN] 1432-1963
  • [Journal-full-title] Der Pathologe
  • [ISO-abbreviation] Pathologe
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Germany
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45. Schittenhelm J, Ebner FH, Harter P, Bornemann A: Symptomatic intraspinal oncocytic adrenocortical adenoma. Endocr Pathol; 2009;20(1):73-7
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  • The diagnosis of an oncocytic adrenal cortical adenoma was made.
  • Oncocytic tumors are rare neoplasms and they comprise non-functioning variants of adrenal cortical adenomas.
  • Immunohistochemistry excluded oncocytic paraganglioma, oncocytic meningioma, renal cell carcinoma, alveolar soft part sarcoma, and granular cell tumor.

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  • (PMID = 19039533.001).
  • [ISSN] 1046-3976
  • [Journal-full-title] Endocrine pathology
  • [ISO-abbreviation] Endocr. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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46. Kshirsagar AY, Dombale VD, Vinchurkar KM, Pareek VA: Retroperitoneal para-aortic paraganglioma. Int Surg; 2005 Jul-Aug;90(3):141-3
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Retroperitoneal para-aortic paraganglioma.
  • Paragangliomas or chemodectomas are neoplasms that arise from neural crest cells and histologically resemble their adrenal counterpart, the pheochromocytoma.
  • Seventy-one percent of the extra-adrenal paragangliomas are located in the superior or inferior paraaortic area.
  • [MeSH-major] Para-Aortic Bodies. Paraganglioma, Extra-Adrenal / surgery. Retroperitoneal Neoplasms / surgery

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  • (PMID = 16466001.001).
  • [ISSN] 0020-8868
  • [Journal-full-title] International surgery
  • [ISO-abbreviation] Int Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
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47. Srivastava A, Sinha T, Karan SC, Sandhu AS, Sethi GS, Talwar R, Narang V: Dispelling inhibition for laparoscopic surgery in younger children with Cushing's syndrome. Case report and literature review. Urol Int; 2006;76(3):283-4
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  • Laparoscopic adrenalectomy (LA) is now the gold standard for the treatment of small, benign adrenal tumors in adults.
  • The histopathology was reported as paraganglioma which is a very rare cause of CS.

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  • [Copyright] Copyright (c) 2006 S. Karger AG, Basel.
  • (PMID = 16601396.001).
  • [ISSN] 0042-1138
  • [Journal-full-title] Urologia internationalis
  • [ISO-abbreviation] Urol. Int.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Switzerland
  • [Number-of-references] 9
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48. Thomas D, Grant FD, Kwong R, Nose V, Di Carli MF, Dorbala S: Multimodality imaging of an unusual case of cardiac paraganglioma. J Nucl Cardiol; 2009 Jul-Aug;16(4):644-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Multimodality imaging of an unusual case of cardiac paraganglioma.
  • [MeSH-major] Heart Neoplasms / diagnosis. Paraganglioma / diagnosis. Paraganglioma, Extra-Adrenal / diagnosis. Paraganglioma, Extra-Adrenal / pathology

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  • (PMID = 19266248.001).
  • [ISSN] 1532-6551
  • [Journal-full-title] Journal of nuclear cardiology : official publication of the American Society of Nuclear Cardiology
  • [ISO-abbreviation] J Nucl Cardiol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 35MRW7B4AD / 3-Iodobenzylguanidine
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49. Gaal J, Burnichon N, Korpershoek E, Roncelin I, Bertherat J, Plouin PF, de Krijger RR, Gimenez-Roqueplo AP, Dinjens WN: Isocitrate dehydrogenase mutations are rare in pheochromocytomas and paragangliomas. J Clin Endocrinol Metab; 2010 Mar;95(3):1274-8
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  • CONTEXT: Paragangliomas and pheochromocytomas are neuroendocrine tumors that occur sporadically and in the context of inherited tumor syndromes including hereditary paraganglioma-pheochromocytoma syndrome and von Hippel-Lindau disease (VHL).
  • The paraganglioma-pheochromocytoma syndrome is caused by germline-inactivating mutations in the mitochondrial succinate dehydrogenase (SDH) genes SDHB, SDHC, SDHD, or SDHAF2, and VHL is the result of inactivating VHL gene mutations.
  • In SDH- and VHL-related paraganglioma and pheochromocytoma, hypoxia-inducible factor (HIF) stabilization has been described as the causal oncogenic event.
  • These findings suggest that inactivating IDH1 and IDH2 mutations might also play a role in paraganglioma and pheochromocytoma tumorigenesis, especially in non-SDH- or non-VHL-related tumors.
  • RESULTS: In one of 131 paragangliomas, a somatic heterozygous IDH1 p.Arg132Cys mutation was detected in a sporadic carotid paraganglioma diagnosed in a 61-yr-old woman.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Isocitrate Dehydrogenase / genetics. Mutation / genetics. Paraganglioma / genetics

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  • (PMID = 19915015.001).
  • [ISSN] 1945-7197
  • [Journal-full-title] The Journal of clinical endocrinology and metabolism
  • [ISO-abbreviation] J. Clin. Endocrinol. Metab.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] EC 1.1.1.41 / Isocitrate Dehydrogenase
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50. Núñez Lozano M, González Sarmiento R: [Genetic and molecular bases of paragangliomas]. Acta Otorrinolaringol Esp; 2009 Feb;60 Suppl 1:24-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Paragangliomas and pheochromocytomas are neuroendocrine tumors arising in the extraadrenal and adrenal medulla, respectively.
  • These tumors appear in certain familial syndromes, such as multiple endocrine neoplasia types 1 and 2, Von Hippel Lindau disease, neurofibromatosis type 1 and familial paraganglioma syndromes.
  • In the last few years, several studies have been performed of these genes in relation to correct diagnosis of paraganglioma and pheochromocytoma, as well as determination of germline mutations in familial and sporadic cases and its utility in genetic counselling in these patients.
  • [MeSH-major] Head and Neck Neoplasms / genetics. Paraganglioma / genetics

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  • (PMID = 19245772.001).
  • [ISSN] 0001-6519
  • [Journal-full-title] Acta otorrinolaringológica española
  • [ISO-abbreviation] Acta Otorrinolaringol Esp
  • [Language] spa
  • [Publication-type] English Abstract; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Spain
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51. Watanabe S, Kahara T, Seto C, Uchiyama A, Abo H, Ishikura K, Nakajima K, Usuda R: Utility of follow-up of 131I-MIBG scintigraphy to screen pheochromocytoma. Intern Med; 2009;48(18):1711-2
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  • [MeSH-major] 3-Iodobenzylguanidine. Adrenal Gland Neoplasms / radionuclide imaging. Iodine Radioisotopes. Pheochromocytoma / radionuclide imaging. Radiopharmaceuticals
  • [MeSH-minor] Humans. Male. Middle Aged. Paraganglioma / diagnosis. Tomography, X-Ray Computed

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  • (PMID = 19755781.001).
  • [ISSN] 1349-7235
  • [Journal-full-title] Internal medicine (Tokyo, Japan)
  • [ISO-abbreviation] Intern. Med.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Iodine Radioisotopes; 0 / Radiopharmaceuticals; 35MRW7B4AD / 3-Iodobenzylguanidine
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52. Pappas L, Seefelder C: Anesthetic consideration for radiofrequency ablation of a suspected paraganglioma metastasis in a child. Paediatr Anaesth; 2009 Sep;19(9):913-4
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  • [Title] Anesthetic consideration for radiofrequency ablation of a suspected paraganglioma metastasis in a child.
  • [MeSH-major] Adrenal Gland Neoplasms / secondary. Adrenal Gland Neoplasms / surgery. Anesthesia. Catheter Ablation. Paraganglioma / secondary. Paraganglioma / surgery. Retroperitoneal Neoplasms / pathology. Retroperitoneal Neoplasms / surgery

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  • (PMID = 19691700.001).
  • [ISSN] 1460-9592
  • [Journal-full-title] Paediatric anaesthesia
  • [ISO-abbreviation] Paediatr Anaesth
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] France
  • [Chemical-registry-number] EC 1.3.99.1 / Succinate Dehydrogenase
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53. Klein RD, Jin L, Rumilla K, Young WF Jr, Lloyd RV: Germline SDHB mutations are common in patients with apparently sporadic sympathetic paragangliomas. Diagn Mol Pathol; 2008 Jun;17(2):94-100
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  • In an effort to further investigate the role of these genes in malignant sympathetic paragangliomas and adrenal pheochromocytomas, we screened a series of tumors for mutations in SDHB and SDHD.
  • [MeSH-major] Germ-Line Mutation. Iron-Sulfur Proteins / genetics. Paraganglioma / genetics. Peripheral Nervous System Neoplasms / genetics. Succinate Dehydrogenase / genetics. Sympathetic Nervous System / pathology
  • [MeSH-minor] Adolescent. Adrenal Gland Neoplasms / genetics. Adrenal Gland Neoplasms / metabolism. Adrenal Gland Neoplasms / pathology. Adult. Child. DNA Mutational Analysis. DNA, Neoplasm / analysis. Female. Humans. Male. Middle Aged. Pheochromocytoma / genetics. Pheochromocytoma / metabolism. Pheochromocytoma / secondary

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  • (PMID = 18382370.001).
  • [ISSN] 1533-4066
  • [Journal-full-title] Diagnostic molecular pathology : the American journal of surgical pathology, part B
  • [ISO-abbreviation] Diagn. Mol. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DNA, Neoplasm; 0 / Iron-Sulfur Proteins; 0 / SDHD protein, human; EC 1.3.5.1 / SDHB protein, human; EC 1.3.99.1 / Succinate Dehydrogenase
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54. Prasad P, Kant JA, Wills M, O'Leary M, Lovvorn H 3rd, Yang E: Loss of heterozygosity of succinate dehydrogenase B mutation by direct sequencing in synchronous paragangliomas. Cancer Genet Cytogenet; 2009 Jul 15;192(2):82-5
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  • We report the presentation of three synchronous extra-adrenal abdominal paragangliomas in an adolescent boy who carries a germline mutation in the succinate dehydrogenase B (SDHB) gene.

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  • [Cites] Science. 2000 Feb 4;287(5454):848-51 [10657297.001]
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  • (PMID = 19596260.001).
  • [ISSN] 1873-4456
  • [Journal-full-title] Cancer genetics and cytogenetics
  • [ISO-abbreviation] Cancer Genet. Cytogenet.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / CA135695-02; United States / NCI NIH HHS / CA / K99 CA135695; United States / NCI NIH HHS / CA / K99 CA135695-02
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] EC 1.3.99.1 / Succinate Dehydrogenase
  • [Other-IDs] NLM/ NIHMS227742; NLM/ PMC2927361
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55. Szyfter W, Kopeć T, Kawczyński M: [Glomus caroticum, jugulare and vagale--problems in diagnosis and treatment]. Otolaryngol Pol; 2006;60(3):305-12
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  • Paraganglioma, being tumors arising from the paraganglion cells of the parasympathetic system, were first reported in man in 1935.
  • [MeSH-major] Carotid Body Tumor. Glomus Jugulare Tumor. Glomus Tympanicum Tumor. Paraganglioma, Extra-Adrenal

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  • (PMID = 16989440.001).
  • [ISSN] 0030-6657
  • [Journal-full-title] Otolaryngologia polska = The Polish otolaryngology
  • [ISO-abbreviation] Otolaryngol Pol
  • [Language] pol
  • [Publication-type] Case Reports; English Abstract; Journal Article; Review
  • [Publication-country] Poland
  • [Number-of-references] 34
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56. Solorzano CC, Lew JI, Wilhelm SM, Sumner W, Huang W, Wu W, Montano R, Sleeman D, Prinz RA: Outcomes of pheochromocytoma management in the laparoscopic era. Ann Surg Oncol; 2007 Oct;14(10):3004-10
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  • CT or MRI localized the adrenal lesion in all patients.
  • There were 92 adrenal pheochromocytomas and 9 paragangliomas.
  • Conversions to open procedures were performed in patients with 4 left, 2 right pheochromocytomas and 1 paraganglioma.
  • [MeSH-major] Adrenal Gland Neoplasms / surgery. Adrenalectomy. Laparoscopy. Pheochromocytoma / surgery
  • [MeSH-minor] 3-Iodobenzylguanidine. Adolescent. Adult. Aged. Aged, 80 and over. Child. Diagnosis, Differential. Female. Follow-Up Studies. Humans. Magnetic Resonance Imaging. Male. Middle Aged. Neoplasm Recurrence, Local / diagnosis. Paraganglioma / diagnosis. Paraganglioma / surgery. Retrospective Studies. Tomography, X-Ray Computed

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  • (PMID = 17690941.001).
  • [ISSN] 1068-9265
  • [Journal-full-title] Annals of surgical oncology
  • [ISO-abbreviation] Ann. Surg. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study
  • [Publication-country] United States
  • [Chemical-registry-number] 35MRW7B4AD / 3-Iodobenzylguanidine
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57. Hirai K, Ota J, Miura T: [Case of recurrent retroperitoneal malignant paraganglioma 25 years after surgery]. Hinyokika Kiyo; 2007 Oct;53(10):703-6
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  • [Title] [Case of recurrent retroperitoneal malignant paraganglioma 25 years after surgery].
  • Surgical resection was done under a diagnosis of recurrent pheochromocytoma, and the tumor was removed with other organs, including normal left adrenal gland that was confirmed histologically.
  • Histologically and immunohistochemically, the resectioned tumor was invasive malignant paraganglioma and diagnosed as the local recurrence or metastasis of previously resectioned retroperitoneal paraganglioma, which was believed to have grown slowly.
  • [MeSH-major] Neoplasm Recurrence, Local. Paraganglioma / surgery. Retroperitoneal Neoplasms / surgery

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  • (PMID = 18018586.001).
  • [ISSN] 0018-1994
  • [Journal-full-title] Hinyokika kiyo. Acta urologica Japonica
  • [ISO-abbreviation] Hinyokika Kiyo
  • [Language] jpn
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Japan
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58. Lee KY, Oh YW, Noh HJ, Lee YJ, Yong HS, Kang EY, Kim KA, Lee NJ: Extraadrenal paragangliomas of the body: imaging features. AJR Am J Roentgenol; 2006 Aug;187(2):492-504
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [MeSH-major] Paraganglioma, Extra-Adrenal / diagnosis

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  • (PMID = 16861555.001).
  • [ISSN] 1546-3141
  • [Journal-full-title] AJR. American journal of roentgenology
  • [ISO-abbreviation] AJR Am J Roentgenol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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59. Fliedner SM, Lehnert H, Pacak K: Metastatic paraganglioma. Semin Oncol; 2010 Dec;37(6):627-37
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  • [Title] Metastatic paraganglioma.
  • Paragangliomas (PGLs) are chromaffin cell tumors arising from ganglia; when arising in the adrenal gland they are called pheochromocytomas.
  • In recent years the opinion that metastatic disease is rare in PGL had to be revised, particularly in patients presenting with extra-adrenal PGL, with PGLs exceeding 5 cm in diameter, and/or those carrying an SDHB germline mutation.
  • [MeSH-major] Paraganglioma / secondary

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  • [Copyright] Published by Elsevier Inc.
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  • (PMID = 21167381.001).
  • [ISSN] 1532-8708
  • [Journal-full-title] Seminars in oncology
  • [ISO-abbreviation] Semin. Oncol.
  • [Language] eng
  • [Grant] United States / Intramural NIH HHS / / Z01 HD008735-07
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Transcription Factors; 0 / snail family transcription factors; EC 1.3.5.1 / SDHB protein, human; EC 1.3.99.1 / Succinate Dehydrogenase
  • [Other-IDs] NLM/ NIHMS252333; NLM/ PMC3018803
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60. Kuroda N, Tamura M, Ohara M, Hirouchi T, Mizuno K, Miyazaki E, Hayashi Y, Lee GH: Possible identification of third stromal component in extraadrenal paraganglioma: myofibroblast in fibrous band and capsule. Med Mol Morphol; 2008 Mar;41(1):59-61
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  • [Title] Possible identification of third stromal component in extraadrenal paraganglioma: myofibroblast in fibrous band and capsule.
  • Sustentacular and dendritic cells are known as the stromal components of extraadrenal paraganglioma.
  • The tumor was discovered near the right adrenal gland in the retroperitoneum.
  • Finally, we identified the third stromal component, namely, myofibroblasts, in the extraadrenal paraganglioma.
  • [MeSH-major] Paraganglioma, Extra-Adrenal / pathology. Retroperitoneal Neoplasms / pathology

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  • (PMID = 18470682.001).
  • [ISSN] 1860-1480
  • [Journal-full-title] Medical molecular morphology
  • [ISO-abbreviation] Med Mol Morphol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Actins; 0 / Calmodulin-Binding Proteins; 0 / HLA-DR Antigens
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61. Varma K, Jain S, Mandal S: Cytomorphologic spectrum in paraganglioma. Acta Cytol; 2008 Sep-Oct;52(5):549-56
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Cytomorphologic spectrum in paraganglioma.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Head and Neck Neoplasms / pathology. Mediastinal Neoplasms / pathology. Paraganglioma / pathology. Retroperitoneal Neoplasms / pathology

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  • (PMID = 18833816.001).
  • [ISSN] 0001-5547
  • [Journal-full-title] Acta cytologica
  • [ISO-abbreviation] Acta Cytol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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62. Horenstein MG, Hitchcock TA, Tucker JA: Dual CD117 expression in gastrointestinal stromal tumor (GIST) and paraganglioma of Carney triad: a case report. Int J Surg Pathol; 2005 Jan;13(1):87-92
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Dual CD117 expression in gastrointestinal stromal tumor (GIST) and paraganglioma of Carney triad: a case report.
  • We report a 36-year-old white woman with complete Carney triad, including metastatic gastric stromal tumor (GIST), pulmonary chondroma, and nonfunctioning extra-adrenal paraganglioma.
  • Immunohistochemistry was positive for CD34 and CD117 (c-kit) in the GIST, and positive for chromogranin and CD117 in the paraganglioma.
  • To our knowledge, this is the 21st complete Carney triad case reported and the first report of dual expression CD117 in both GIST and paraganglioma, a finding with intriguing pathogenetic implications related to the organization of the autonomic nervous system.
  • [MeSH-major] Chondroma / pathology. Gastrointestinal Stromal Tumors / pathology. Leiomyosarcoma / secondary. Lung Neoplasms / pathology. Paraganglioma, Extra-Adrenal / pathology. Proto-Oncogene Proteins c-kit / analysis

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  • (PMID = 15735861.001).
  • [ISSN] 1066-8969
  • [Journal-full-title] International journal of surgical pathology
  • [ISO-abbreviation] Int. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD34; 0 / Antineoplastic Agents; 0 / Biomarkers, Tumor; 0 / Chromogranins; EC 2.7.10.1 / Proto-Oncogene Proteins c-kit
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63. Santiago AH, Campbell BT, Estrada DE: Early presentation of familial paraganglioma with SDHB mutation in a 13 year old child and its mother. J Pediatr Endocrinol Metab; 2010 Apr;23(4):419-22
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Early presentation of familial paraganglioma with SDHB mutation in a 13 year old child and its mother.
  • These mutations are thought to be responsible for the familial paraganglioma syndrome.
  • We present the case of a 13 year-old boy with abdominal paraganglioma, whose mother also had a history of thoracic paraganglioma diagnosed at 14 years of age.
  • [MeSH-major] Paraganglioma, Extra-Adrenal / genetics. Succinate Dehydrogenase / genetics. Thoracic Neoplasms / genetics

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  • (PMID = 20583550.001).
  • [ISSN] 0334-018X
  • [Journal-full-title] Journal of pediatric endocrinology & metabolism : JPEM
  • [ISO-abbreviation] J. Pediatr. Endocrinol. Metab.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] EC 1.3.99.1 / Succinate Dehydrogenase
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64. Carney JA: Carney triad: a syndrome featuring paraganglionic, adrenocortical, and possibly other endocrine tumors. J Clin Endocrinol Metab; 2009 Oct;94(10):3656-62
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • BACKGROUND: Two young women, each with paraganglioma and gastric stromal tumor, were encountered in the middle 1970s.
  • One also had an adrenal cortical adenoma and the other pulmonary chondroma.
  • Five additional patients with gastric stromal tumor, paraganglioma, and pulmonary chondroma were found, and all were young women.
  • FOLLOW-UP: At follow-up, 80% of the patients were alive, two thirds with pulmonary chondroma, 25% with metastatic or residual gastric stromal tumor, and 5% with primary or metastatic paraganglioma.
  • Twenty percent of the patients were dead, usually from metastatic gastric stromal tumor, less frequently from metastatic paraganglioma.
  • [MeSH-major] Adenoma. Adrenal Cortex Neoplasms. Chondroma. Esophageal Neoplasms. Leiomyoma. Lung Neoplasms. Multiple Endocrine Neoplasia. Neoplastic Syndromes, Hereditary / pathology. Paraganglioma

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  • (PMID = 19723753.001).
  • [ISSN] 1945-7197
  • [Journal-full-title] The Journal of clinical endocrinology and metabolism
  • [ISO-abbreviation] J. Clin. Endocrinol. Metab.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 23
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65. Srirangalingam U, Walker L, Khoo B, MacDonald F, Gardner D, Wilkin TJ, Skelly RH, George E, Spooner D, Monson JP, Grossman AB, Akker SA, Pollard PJ, Plowman N, Avril N, Berney DM, Burrin JM, Reznek RH, Kumar VK, Maher ER, Chew SL: Clinical manifestations of familial paraganglioma and phaeochromocytomas in succinate dehydrogenase B (SDH-B) gene mutation carriers. Clin Endocrinol (Oxf); 2008 Oct;69(4):587-96
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Clinical manifestations of familial paraganglioma and phaeochromocytomas in succinate dehydrogenase B (SDH-B) gene mutation carriers.
  • 24% (6 of 25) of tumours were located in the adrenal and 76% (19 of 25) were extra-adrenal.
  • CONCLUSION: SDH-B mutation carriers develop disease early and predominantly in extra-adrenal locations.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Paraganglioma / diagnosis. Pheochromocytoma / diagnosis. Succinate Dehydrogenase / genetics

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  • (PMID = 18419787.001).
  • [ISSN] 1365-2265
  • [Journal-full-title] Clinical endocrinology
  • [ISO-abbreviation] Clin. Endocrinol. (Oxf)
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] EC 1.3.5.1 / SDHB protein, human; EC 1.3.99.1 / Succinate Dehydrogenase
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66. Szabados S, Varady E, Göbölös L: Cardiovascular flashlight. Paraganglioma of the aortopulmonary window. Eur Heart J; 2009 May;30(10):1286
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Cardiovascular flashlight. Paraganglioma of the aortopulmonary window.
  • [MeSH-major] Dyspnea / etiology. Mediastinal Neoplasms / diagnosis. Paraganglioma, Extra-Adrenal / complications

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  • (PMID = 19164332.001).
  • [ISSN] 1522-9645
  • [Journal-full-title] European heart journal
  • [ISO-abbreviation] Eur. Heart J.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
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67. Mohaupt MG, von Vigier RO: [Urinary hormone analysis]. Ther Umsch; 2006 Sep;63(9):559-64
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Diseases to be investigated by steroid hormone analysis are excess production of a typical or atypical mineralocorticoid active steroid hormones, the hormonal activity of adrenal or ovarian tumors, acne of unknown origin, hirsutism, a PCO-, an adrenogenital or a suspected Cushing syndrome.
  • Biogenic amines should be determined in suspected secondary or refractory arterial hypertension, in case of pheochromocytoma- or paraganglioma-associated symptoms or if a serotonin-producing tumor is suspected.

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  • (PMID = 17048168.001).
  • [ISSN] 0040-5930
  • [Journal-full-title] Therapeutische Umschau. Revue thérapeutique
  • [ISO-abbreviation] Ther Umsch
  • [Language] ger
  • [Publication-type] Case Reports; English Abstract; Journal Article; Review
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / Hormones
  • [Number-of-references] 11
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68. Braun S, Riemann K, Kupka S, Leistenschneider P, Sotlar K, Schmid H, Blin N: Active succinate dehydrogenase (SDH) and lack of SDHD mutations in sporadic paragangliomas. Anticancer Res; 2005 Jul-Aug;25(4):2809-14
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [MeSH-major] Membrane Proteins / genetics. Paraganglioma, Extra-Adrenal / enzymology. Paraganglioma, Extra-Adrenal / genetics. Succinate Dehydrogenase / genetics

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  • (PMID = 16080530.001).
  • [ISSN] 0250-7005
  • [Journal-full-title] Anticancer research
  • [ISO-abbreviation] Anticancer Res.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Greece
  • [Chemical-registry-number] 0 / Membrane Proteins; 0 / SDHD protein, human; EC 1.3.99.1 / Succinate Dehydrogenase
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69. Byrne CM, Daneshjoo R, Ma-Wyatt J, Cox M: Gastrointestinal haemorrhage as a presentation of Carney's triad. ANZ J Surg; 2007 Jan-Feb;77(1-2):88-90
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [MeSH-minor] Chondroma / radiography. Female. Gastrectomy. Humans. Lung Neoplasms / radiography. Middle Aged. Paraganglioma, Extra-Adrenal. Sarcoma / complications. Sarcoma / surgery. Syndrome. Tomography, X-Ray Computed

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  • (PMID = 17295830.001).
  • [ISSN] 1445-1433
  • [Journal-full-title] ANZ journal of surgery
  • [ISO-abbreviation] ANZ J Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Australia
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70. García JR, Minoves M, Bassa P, Fraile M: [Bone scintigraphy and (111)In-octreotide in the diagnosis of bone metastasis of chemodectoma]. Rev Esp Med Nucl; 2006 May-Jun;25(3):204-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [MeSH-major] Bone Neoplasms / radionuclide imaging. Bone Neoplasms / secondary. Carotid Body Tumor / secondary. Indium Radioisotopes. Octreotide / analogs & derivatives. Paraganglioma, Extra-Adrenal / radionuclide imaging. Paraganglioma, Extra-Adrenal / secondary. Radiopharmaceuticals

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  • (PMID = 16762278.001).
  • [ISSN] 0212-6982
  • [Journal-full-title] Revista española de medicina nuclear
  • [ISO-abbreviation] Rev Esp Med Nucl
  • [Language] spa
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Spain
  • [Chemical-registry-number] 0 / Indium Radioisotopes; 0 / Radiopharmaceuticals; 0 / indium-111-octreotide; RWM8CCW8GP / Octreotide
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71. Young WF Jr: Paragangliomas: clinical overview. Ann N Y Acad Sci; 2006 Aug;1073:21-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Paragangliomas are rare tumors that arise from extra-adrenal paraganglia.
  • The effective diagnosis and management of the paraganglioma patient involves the close collaboration of endocrinologists, endocrine surgeons, anesthesiologists, geneticists, laboratory specialists, radiologists, oncologists, and pathologists.
  • Paragangliomas are diagnosed in the following clinical settings: signs and symptoms related to catecholamine hypersecretion, mass effect symptoms (e.g., with head and neck paragangliomas), incidental finding on imaging, or family screening for hereditary paraganglioma.
  • Paragangliomas that hypersecrete catecholamines may cause signs and symptoms identical to those in patients with hyperfunctioning adrenal pheochromocytoma.
  • As many as 50% of paragangliomas are hereditary and may be associated with familial paraganglioma, neurofibromatosis Type 1, von Hippel-Lindau disease, the Carney triad, and, rarely, with multiple endocrine neoplasia Type 2.
  • Genetic testing should be considered in all patients with paraganglioma.
  • The treatment of choice for paraganglioma is surgical resection; most tumors are benign and can be excised totally.
  • [MeSH-major] Paraganglioma / physiopathology

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  • (PMID = 17102068.001).
  • [ISSN] 0077-8923
  • [Journal-full-title] Annals of the New York Academy of Sciences
  • [ISO-abbreviation] Ann. N. Y. Acad. Sci.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 21
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72. Ouaïssi M, Sieleznieff I, Pirrò N, Payan MJ, Chaix JB, Consentino B, Sastre B: [Retroperitoneal non-secreting paraganglioma. Apropos of a case]. Gastroenterol Clin Biol; 2007 Mar;31(3):307-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Retroperitoneal non-secreting paraganglioma. Apropos of a case].
  • These tumors are most commonly found in the adrenal gland but other locations are possible.
  • Histopathological examination revealed a non secreting paraganglioma in the left retroperitoneum.
  • [MeSH-major] Paraganglioma / diagnosis. Retroperitoneal Neoplasms / diagnosis

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  • (PMID = 17396092.001).
  • [ISSN] 0399-8320
  • [Journal-full-title] Gastroentérologie clinique et biologique
  • [ISO-abbreviation] Gastroenterol. Clin. Biol.
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
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73. Havekes B, Corssmit EP, Jansen JC, van der Mey AG, Vriends AH, Romijn JA: Malignant paragangliomas associated with mutations in the succinate dehydrogenase D gene. J Clin Endocrinol Metab; 2007 Apr;92(4):1245-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • RESULTS: Metastasis and/or local tumor invasion was documented 0 (n=2), 1, 18, and 30 yr after the initial diagnosis of paraganglioma.
  • Malignancy was proven by paraganglioma bone metastases (n=2), intrathoracic paraganglioma with lymph node metastases, locally invasive head-and-neck paraganglioma with destruction of the petrosal bone, and locally invasive paraganglioma of the bladder with lymph node metastases.
  • Four of the five patients developed catecholamine excess during follow-up due to intraadrenal paraganglioma (pheochromocytoma) (n=1), extra adrenal paraganglioma (n=2), and presumed subclinical disease (n=1).
  • [MeSH-major] Iron-Sulfur Proteins / genetics. Mutation. Paraganglioma / genetics. Succinate Dehydrogenase / genetics
  • [MeSH-minor] Adrenal Gland Neoplasms / genetics. Adult. Aged. Amino Acid Substitution. Carotid Body Tumor / genetics. Female. Humans. Male. Middle Aged. Skull Neoplasms / genetics. Temporomandibular Joint

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  • (PMID = 17227803.001).
  • [ISSN] 0021-972X
  • [Journal-full-title] The Journal of clinical endocrinology and metabolism
  • [ISO-abbreviation] J. Clin. Endocrinol. Metab.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Iron-Sulfur Proteins; EC 1.3.5.1 / SDHB protein, human; EC 1.3.99.1 / Succinate Dehydrogenase
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74. Dundee P, Clancy B, Wagstaff S, Briggs R: Paraganglioma: the role of genetic counselling and radiological screening. J Clin Neurosci; 2005 May;12(4):464-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Paraganglioma: the role of genetic counselling and radiological screening.
  • Genetic predisposition to develop paraganglioma can occur within known tumour syndromes and familial tumours tend to present at a younger age and at multiple sites compared to sporadic tumours.
  • We present a case of a 14-year-old boy with multiple paraganglioma and a strong family history of paraganglioma.
  • He suffered significant morbidity at resection of an extra-adrenal retroperitoneal tumour due to late diagnosis and was later unable to undergo excision of a head and neck paraganglioma due to its size and relation to neurovascular structures in the neck.
  • We review the current literature on suggested genetic counselling (psychological counselling and DNA analysis) and radiological screening guidelines and recommend that genetic counselling should be offered to all patients with a family history of paraganglioma from the age of 5 years.
  • Those positive for paternal paraganglioma locus gene should then undergo regular radiological screening with MRI.
  • [MeSH-major] Brain Neoplasms / genetics. Brain Neoplasms / pathology. Genetic Counseling. Magnetic Resonance Imaging. Paraganglioma / genetics

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  • (PMID = 15925783.001).
  • [ISSN] 0967-5868
  • [Journal-full-title] Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
  • [ISO-abbreviation] J Clin Neurosci
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Scotland
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75. Wen J, Li HZ, Ji ZG, Mao QZ, Shi BB, Yan WG: A case of large "silent" extra-adrenal retroperitoneal paraganglioma resected laparoscopically. Chin Med Sci J; 2010 Mar;25(1):61-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A case of large "silent" extra-adrenal retroperitoneal paraganglioma resected laparoscopically.

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  • (PMID = 20449957.001).
  • [ISSN] 1001-9294
  • [Journal-full-title] Chinese medical sciences journal = Chung-kuo i hsueh k'o hsueh tsa chih
  • [ISO-abbreviation] Chin. Med. Sci. J.
  • [Language] ENG
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] China
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76. Akl MN, Naidu SG, McCullough AE, Magtibay PM: Vaginal paraganglioma presenting as a pelvic mass. Surgery; 2010 Jan;147(1):169-71
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Vaginal paraganglioma presenting as a pelvic mass.
  • Paragangliomas are extra-adrenal neuroendocrine neoplasms derived from neural crest precursors.
  • We describe a case of vaginal paraganglioma managed with minimally invasive surgical techniques.
  • [MeSH-major] Paraganglioma, Extra-Adrenal / pathology. Paraganglioma, Extra-Adrenal / surgery. Vagina / pathology. Vaginal Neoplasms / pathology. Vaginal Neoplasms / surgery

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  • [Copyright] Copyright (c) 2010 Mosby, Inc. All rights reserved.
  • (PMID = 19744428.001).
  • [ISSN] 1532-7361
  • [Journal-full-title] Surgery
  • [ISO-abbreviation] Surgery
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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77. Nakamura F, Silva RA, dos Santos VP, Razuk Filho A, Caffaro RA: [Preoperative embolization of abdominal paraganglioma: case report]. Rev Col Bras Cir; 2010 Apr;37(2):159-61
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Preoperative embolization of abdominal paraganglioma: case report].
  • [Transliterated title] Embolização pré operatória no tratamento de paraganglioma abdominal: relato de caso.
  • Paragangliomas is a pheochromocytoma of extra adrenal localization.
  • The case report is a male, 55 years old who presented symptoms of adrenergic hyperstimulation associated to an abdominal mass diagnosed as paraganglioma by a biopsy.
  • [MeSH-major] Abdominal Neoplasms / therapy. Embolization, Therapeutic. Paraganglioma / therapy

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  • (PMID = 20549108.001).
  • [ISSN] 1809-4546
  • [Journal-full-title] Revista do Colégio Brasileiro de Cirurgiões
  • [ISO-abbreviation] Rev Col Bras Cir
  • [Language] por
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Brazil
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78. Dionne JM, Wu JK, Heran M, Murphy JJ, Jevon G, White CT: Malignant hypertension, polycythemia, and paragangliomas. J Pediatr; 2006 Apr;148(4):540-5
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  • [MeSH-major] Abdominal Neoplasms / complications. Hypertension, Malignant / etiology. Neoplasms, Multiple Primary / complications. Paraganglioma, Extra-Adrenal / complications. Polycythemia / complications

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  • (PMID = 16647420.001).
  • [ISSN] 0022-3476
  • [Journal-full-title] The Journal of pediatrics
  • [ISO-abbreviation] J. Pediatr.
  • [Language] eng
  • [Publication-type] Clinical Conference; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 11096-26-7 / Erythropoietin
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79. Cunningham SC, Suh HS, Winter JM, Montgomery E, Schulick RD, Cameron JL, Yeo CJ: Retroperitoneal paraganglioma: single-institution experience and review of the literature. J Gastrointest Surg; 2006 Sep-Oct;10(8):1156-63
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  • [Title] Retroperitoneal paraganglioma: single-institution experience and review of the literature.
  • Paragangliomas are rare tumors arising from extra-adrenal chromaffin cells.
  • [MeSH-major] Digestive System Surgical Procedures / methods. Paraganglioma. Retroperitoneal Neoplasms

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  • (PMID = 16966036.001).
  • [ISSN] 1091-255X
  • [Journal-full-title] Journal of gastrointestinal surgery : official journal of the Society for Surgery of the Alimentary Tract
  • [ISO-abbreviation] J. Gastrointest. Surg.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 25
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80. Chrisoulidou A, Kaltsas G, Ilias I, Grossman AB: The diagnosis and management of malignant phaeochromocytoma and paraganglioma. Endocr Relat Cancer; 2007 Sep;14(3):569-85
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  • [Title] The diagnosis and management of malignant phaeochromocytoma and paraganglioma.
  • Functional imaging using radiolabelled metaiodobenzylguanidine (MIBG) and more recently, (18)F-fluorodopamine and (18)F-fluorodopa positron emission tomography offer substantial sensitivity and specificity to correctly detect metastatic phaeochromocytoma and paraganglioma and helps identify patients suitable for treatment with radiopharmaceuticals.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / therapy. Paraganglioma / diagnosis. Paraganglioma / therapy. Pheochromocytoma / diagnosis. Pheochromocytoma / therapy

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  • (PMID = 17914089.001).
  • [ISSN] 1351-0088
  • [Journal-full-title] Endocrine-related cancer
  • [ISO-abbreviation] Endocr. Relat. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Radiopharmaceuticals
  • [Number-of-references] 159
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81. Bockenhauer D, Rees L, Neumann H, Foo Y: A sporadic case of paraganglioma undetected by urine metabolite screening. Pediatr Nephrol; 2008 Oct;23(10):1889-91
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  • [Title] A sporadic case of paraganglioma undetected by urine metabolite screening.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Homovanillic Acid / urine. Pheochromocytoma / diagnosis. Vanilmandelic Acid / urine

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  • [ISSN] 0931-041X
  • [Journal-full-title] Pediatric nephrology (Berlin, Germany)
  • [ISO-abbreviation] Pediatr. Nephrol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
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  • [Chemical-registry-number] 55-10-7 / Vanilmandelic Acid; X77S6GMS36 / Homovanillic Acid
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82. Skrivan J, Zvĕrina E, Kluh J, Chovanec M, Pádr R: Our experience with surgical treatment of tympanojugular pragangliomas. Prague Med Rep; 2010;111(1):25-34
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  • [MeSH-major] Ear Neoplasms / surgery. Glomus Jugulare Tumor / surgery. Paraganglioma, Extra-Adrenal / surgery

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  • (PMID = 20359435.001).
  • [ISSN] 1214-6994
  • [Journal-full-title] Prague medical report
  • [ISO-abbreviation] Prague Med Rep
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Czech Republic
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83. Tuncel A, Aslan Y, Han O, Horasanli E, Seckin S, Atan A: Laparoscopic resection of periadrenal paraganglioma mimicking an isolated adrenal hydatid cyst. JSLS; 2010 Oct-Dec;14(4):579-82
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  • [Title] Laparoscopic resection of periadrenal paraganglioma mimicking an isolated adrenal hydatid cyst.
  • We present the case of a 64-year-old female with a periadrenal paraganglioma.
  • These findings made us think it was an isolated adrenal hydatid cyst.
  • Histopathological examination was consistent with periadrenal paraganglioma.
  • We believe that the paraganglioma can mimic the radiological appearance of an isolated adrenal hydatid cyst, which should be taken into consideration during diagnosis.

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  • (PMID = 21605527.001).
  • [ISSN] 1086-8089
  • [Journal-full-title] JSLS : Journal of the Society of Laparoendoscopic Surgeons
  • [ISO-abbreviation] JSLS
  • [Language] ENG
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84. Fritzsche FR, Bode PK, Koch S, Frauenfelder T: Radiological and pathological findings of a metastatic composite paraganglioma with neuroblastoma in a man: a case report. J Med Case Rep; 2010;4:374
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  • [Title] Radiological and pathological findings of a metastatic composite paraganglioma with neuroblastoma in a man: a case report.
  • INTRODUCTION: Composite tumors of the adrenal medulla or paraganglia are extremely rare and present a diagnostic dilemma.
  • The final histopathological workup revealed a composite paraganglioma with neuroblastoma.

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  • (PMID = 21092109.001).
  • [ISSN] 1752-1947
  • [Journal-full-title] Journal of medical case reports
  • [ISO-abbreviation] J Med Case Rep
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC2997098
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85. Daramola OO, Shinners MJ, Levine SC: Secreting jugulotympanic paraganglioma with venous involvement into the thorax. Laryngoscope; 2008 Jul;118(7):1233-5
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  • [Title] Secreting jugulotympanic paraganglioma with venous involvement into the thorax.
  • Paragangliomas are highly vascular tumors that arise from chief cells in extra-adrenal paraganglia of the autonomic nervous system.
  • Patients with refractory hypertension and masses suspicious for paraganglioma should be examined for functional tumors.

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  • (PMID = 18425050.001).
  • [ISSN] 1531-4995
  • [Journal-full-title] The Laryngoscope
  • [ISO-abbreviation] Laryngoscope
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] X4W3ENH1CV / Norepinephrine
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86. Chew SL: Diagnosis: imaging of pheochromocytomas and paragangliomas. Nat Rev Endocrinol; 2010 Apr;6(4):193-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Diagnostic Imaging / methods. Paraganglioma / diagnosis. Pheochromocytoma / diagnosis

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  • [CommentOn] J Clin Endocrinol Metab. 2009 Dec;94(12):4757-67 [19864450.001]
  • (PMID = 20336165.001).
  • [ISSN] 1759-5037
  • [Journal-full-title] Nature reviews. Endocrinology
  • [ISO-abbreviation] Nat Rev Endocrinol
  • [Language] eng
  • [Publication-type] Comment; Comparative Study; News
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Contrast Media; 0 / Iodine Radioisotopes; 0Z5B2CJX4D / Fluorodeoxyglucose F18; 2C598205QX / fluorodopa F 18; 35MRW7B4AD / 3-Iodobenzylguanidine; 63-84-3 / Dihydroxyphenylalanine
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87. de Krijger RR, van Nederveen FH, Korpershoek E, Dinjens WN: New developments in the detection of the clinical behavior of pheochromocytomas and paragangliomas. Endocr Pathol; 2006;17(2):137-41
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  • Pheochromocytomas (PCC) are catecholamine-producing tumors that are, by definition, located in the adrenal medulla.
  • Extra-adrenal catecholamine-producing tumors are called paragangliomas (PGL), which should be distinguished from head and neck paragangliomas, which are of parasympathetic origin.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Paraganglioma / diagnosis. Pheochromocytoma / diagnosis

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  • (PMID = 17159246.001).
  • [ISSN] 1046-3976
  • [Journal-full-title] Endocrine pathology
  • [ISO-abbreviation] Endocr. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
  • [Number-of-references] 18
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88. Kasperlik-Zaluska AA, Roslonowska E, Slowinska-Srzednicka J, Otto M, Cichocki A, Cwikla J, Slapa R, Eisenhofer G: 1,111 patients with adrenal incidentalomas observed at a single endocrinological center: incidence of chromaffin tumors. Ann N Y Acad Sci; 2006 Aug;1073:38-46
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  • [Title] 1,111 patients with adrenal incidentalomas observed at a single endocrinological center: incidence of chromaffin tumors.
  • A majority of incidentally found adrenal tumors derive from the adrenal cortex.
  • The aim of our study was evaluation of the incidence of chromaffin tumors in a group of 1,111 patients with adrenal incidentalomas.
  • Chromaffin tumors were detected in 43 patients, 33 women, and 10 men aged 20-75 years: pheochromocytoma in 36 (malignant in 3); chromaffin cells hyperplasia in 2; paraganglioma in 3; ganglioneuroblastoma in 1; ganglioneuroma in 1; and schwannoma in 2.
  • Chromaffin tumors were detected in 4% (pheochromocytomas in 3%) of 1,111 patients with adrenal incidentalomas.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Chromaffin Cells / pathology

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  • (PMID = 17102070.001).
  • [ISSN] 0077-8923
  • [Journal-full-title] Annals of the New York Academy of Sciences
  • [ISO-abbreviation] Ann. N. Y. Acad. Sci.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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89. Todorovic M, Balint B, Suvajdzic N, Jevtic M, Pavlovic M, Petrovic M, Krstic M, Popovic V, Ivanovic B, Elezovic I, Milenkovic R, Colovic M: Triple-way therapeutic approach for paraganglioma-dependent erythrocytosis: drugs and surgery plus "multi-manner" apheresis. Med Oncol; 2008;25(2):148-53
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  • [Title] Triple-way therapeutic approach for paraganglioma-dependent erythrocytosis: drugs and surgery plus "multi-manner" apheresis.
  • Paragangliomas are tumors arising from the extra-adrenal paragangliar neural crest cells.
  • The sympathoadrenal neuroendocrine system consists of extra-adrenal paragangliar cellular layer along the paravertebral and para-aortic axis, and the adrenal medullae.
  • Paraganglioma should be included in the differential diagnosis of secondary erythrocytosis due to its possible ectopic erythropoietin (EPO) secretion.
  • Thus, in this report we present a 24-year-old female patient with onset of unregulated ectopic EPO secretion, and consecutive erythrocytosis followed by hypertension, secondary to paraganglioma of multifocal retroperitoneal localization.
  • Clinical, laboratory, and radiological investigations confirmed both an elevated EPO level and the presence of multiple paraganglioma.
  • [MeSH-major] Blood Component Removal. Paraganglioma / therapy. Polycythemia / therapy

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  • (PMID = 18488156.001).
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  • [ISO-abbreviation] Med. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 11096-26-7 / Erythropoietin
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90. Nozaki T, Iida H, Tsuritani S, Okumura A, Komiya A, Fuse H: Laparoscopic resection of retrocaval paraganglioma. J Laparoendosc Adv Surg Tech A; 2010 May;20(4):363-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Laparoscopic resection of retrocaval paraganglioma.
  • OBJECTIVES: Definitive treatment of paraganglioma is by surgical resection.
  • Laparoscopic resection of the paraganglioma presents a unique surgical challenge due to inherent risks associated with excessive catecholamine release during surgical manipulation and variable anatomic presentation.
  • In this report, we present our experience with the laparoscopic resection of the retrocaval paraganglioma.
  • METHODS: Between October 2001 and November 2007, 2 patients underwent the laparoscopic resection of a retrocaval paraganglioma.
  • Finally, the tumor and the right adrenal gland were extracted in an endobag.
  • CONCLUSIONS: To our knowledge, this is the first report of the laparoscopic resection of a retrocaval paraganglioma.
  • For the resection of a paraganglioma, in this location, laparoscopy has the advantage of precise visualization, which helps to resect the tumor completely.
  • Laparoscopic resection of a paraganglioma, despite the high level of compression of the IVC, is a feasible, reproducible technique with appropriate preoperative planning.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Adrenal Gland Neoplasms / surgery. Adrenalectomy. Laparoscopy. Paraganglioma / pathology. Paraganglioma / surgery

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  • (PMID = 20438308.001).
  • [ISSN] 1557-9034
  • [Journal-full-title] Journal of laparoendoscopic & advanced surgical techniques. Part A
  • [ISO-abbreviation] J Laparoendosc Adv Surg Tech A
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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91. Pham TH, Moir C, Thompson GB, Zarroug AE, Hamner CE, Farley D, van Heerden J, Lteif AN, Young WF Jr: Pheochromocytoma and paraganglioma in children: a review of medical and surgical management at a tertiary care center. Pediatrics; 2006 Sep;118(3):1109-17
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Pheochromocytoma and paraganglioma in children: a review of medical and surgical management at a tertiary care center.
  • METHODS: A retrospective chart review of the Mayo Clinic database from 1975 to 2005 identified 30 patients < 18 years of age with histologically confirmed pheochromocytoma or paraganglioma.
  • Nine patients (30%) had a genetic mutation or documented family history of pheochromocytoma or paraganglioma.
  • 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.
  • CONCLUSIONS: The incidence of malignant pheochromocytoma/paraganglioma was high in children (47%), particularly those with apparently sporadic disease, paraganglioma, and tumor diameters of > 6 cm.
  • Patients with a known genetic mutation or familial pheochromocytoma/paraganglioma were more likely to achieve resection with negative microscopic margins and had improved disease-specific mortality rates.
  • Surgical resection remains the treatment of choice for pheochromocytoma and paraganglioma.
  • [MeSH-major] Adrenal Gland Neoplasms / surgery. Paraganglioma / surgery. Pheochromocytoma / surgery


92. Castellani MR, Seghezzi S, Chiesa C, Aliberti GL, Maccauro M, Seregni E, Orunesu E, Luksch R, Bombardieri E: (131)I-MIBG treatment of pheochromocytoma: low versus intermediate activity regimens of therapy. Q J Nucl Med Mol Imaging; 2010 Feb;54(1):100-13
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Consequently, the increase of (131)I-MIBG activity, without reaching myeloablative levels, can be recommended for standard treatment of pheochromocytoma and paraganglioma patients.
  • [MeSH-major] 3-Iodobenzylguanidine / therapeutic use. Adrenal Gland Neoplasms / radiotherapy. Pheochromocytoma / radiotherapy. Radiation Dosage

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  • (PMID = 20168292.001).
  • [ISSN] 1824-4785
  • [Journal-full-title] The quarterly journal of nuclear medicine and molecular imaging : official publication of the Italian Association of Nuclear Medicine (AIMN) [and] the International Association of Radiopharmacology (IAR), [and] Section of the Society of Radiopharmaceutical Chemistry and Biology
  • [ISO-abbreviation] Q J Nucl Med Mol Imaging
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Iodine Radioisotopes; 35MRW7B4AD / 3-Iodobenzylguanidine
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93. Rufini V, Calcagni ML, Baum RP: Imaging of neuroendocrine tumors. Semin Nucl Med; 2006 Jul;36(3):228-47
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [MeSH-minor] 3-Iodobenzylguanidine / therapeutic use. ACTH Syndrome, Ectopic / etiology. ACTH Syndrome, Ectopic / radionuclide imaging. Adrenal Gland Neoplasms / radionuclide imaging. Adult. Carcinoma, Medullary / radionuclide imaging. Catecholamines / secretion. Child. Gastrointestinal Neoplasms / radionuclide imaging. Humans. Iodine Radioisotopes / therapeutic use. Middle Aged. Neuroblastoma / radionuclide imaging. Neuroblastoma / secondary. Octreotide. Paraganglioma / radionuclide imaging. Pheochromocytoma / radionuclide imaging. Positron-Emission Tomography. Radiopharmaceuticals / therapeutic use. Somatostatin / analogs & derivatives. Thyroid Neoplasms / radionuclide imaging

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  • (PMID = 16762613.001).
  • [ISSN] 0001-2998
  • [Journal-full-title] Seminars in nuclear medicine
  • [ISO-abbreviation] Semin Nucl Med
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Catecholamines; 0 / Iodine Radioisotopes; 0 / Radiopharmaceuticals; 35MRW7B4AD / 3-Iodobenzylguanidine; 51110-01-1 / Somatostatin; RWM8CCW8GP / Octreotide
  • [Number-of-references] 210
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94. Zelenov GG, Shchegolev AI, Kolokol'chikova EG: Ultrastructural characteristics of chemodectomas. Bull Exp Biol Med; 2008 Feb;145(2):266-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [MeSH-major] Head and Neck Neoplasms / ultrastructure. Paraganglioma, Extra-Adrenal / ultrastructure

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  • (PMID = 19023986.001).
  • [ISSN] 0007-4888
  • [Journal-full-title] Bulletin of experimental biology and medicine
  • [ISO-abbreviation] Bull. Exp. Biol. Med.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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95. Ercolino T, Becherini L, Valeri A, Maiello M, Gaglianò MS, Parenti G, Ramazzotti M, Piscitelli E, Simi L, Pinzani P, Nesi G, Degl'Innocenti D, Console N, Bergamini C, Mannelli M: Uncommon clinical presentations of pheochromocytoma and paraganglioma in two different patients affected by two distinct novel VHL germline mutations. Clin Endocrinol (Oxf); 2008 May;68(5):762-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Uncommon clinical presentations of pheochromocytoma and paraganglioma in two different patients affected by two distinct novel VHL germline mutations.
  • Extra-adrenal Pheos (paragangliomas, PGLs) are rare.
  • One patient was studied for the presence of an adrenal incidentaloma and the other for the presence of a neck tumour.
  • We identified two novel VHL point mutations: a L198V missense mutation in a 32-year-old female affected by a right adrenal compound and mixed tumour constituted by an epinephrine secreting Pheo, a ganglioneuroma and an adrenocortical adenoma, and a T152I missense mutation in a 24-year-old female affected by a left carotid body tumour.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Head and Neck Neoplasms / genetics. Paraganglioma / genetics. Pheochromocytoma / genetics. von Hippel-Lindau Disease / genetics


96. Ausania F, De-Giorgio F, Valerio L, Capelli A, Arena V: Neoplastic spread after trauma: natural death or manslaughter? Med Sci Law; 2010 Apr;50(2):100-2
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • We report an unusual death of a seemingly healthy 26-year-old man due to diffuse metastasization of adrenal paraganglioma caused by rupture of the primitive tumour with subsequent massive haemorrhage after a relatively minor trauma.
  • [MeSH-major] Accidents / legislation & jurisprudence. Adrenal Gland Neoplasms / pathology. Paraganglioma / pathology. Wounds, Nonpenetrating / complications

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  • (PMID = 20593602.001).
  • [ISSN] 0025-8024
  • [Journal-full-title] Medicine, science, and the law
  • [ISO-abbreviation] Med Sci Law
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
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97. Rodriguez FJ, Aubry MC, Tazelaar HD, Slezak J, Carney JA: Pulmonary chondroma: a tumor associated with Carney triad and different from pulmonary hamartoma. Am J Surg Pathol; 2007 Dec;31(12):1844-53
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  • The Carney triad is the clinical association of gastric stromal sarcomas, pulmonary cartilaginous tumors, and extra-adrenal paragangliomas.
  • [MeSH-minor] Adult. Diagnosis, Differential. Female. Gastrointestinal Stromal Tumors / pathology. Humans. Male. Middle Aged. Paraganglioma, Extra-Adrenal / pathology

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  • (PMID = 18043038.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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98. Yau JS, Li JK, Tam VH, Fung LM, Yeung CK, Chan KW, Lee KM, Lee KF, Cheung WS, Yeung VT, Yuen YP, Kwan WK: Phaeochromocytoma in the Hong Kong Chinese population. Hong Kong Med J; 2010 Aug;16(4):252-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Four (24%) had hereditary phaeochromocytoma/paraganglioma syndrome.
  • Approximately 65% of the patients had intra-adrenal tumours; 53% were on right side, 18% were bilateral.
  • CONCLUSION: Our series of patients with phaeochromocytomas commonly had a high frequency of normotension and extra-adrenal tumours.
  • A high index of clinical suspicion and appropriate biochemical investigations are necessary to make the diagnosis, especially for patients manifesting adrenal incidentaloma and extra-adrenal lesion.

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  • (PMID = 20683066.001).
  • [ISSN] 1024-2708
  • [Journal-full-title] Hong Kong medical journal = Xianggang yi xue za zhi
  • [ISO-abbreviation] Hong Kong Med J
  • [Language] ENG
  • [Publication-type] Journal Article
  • [Publication-country] China
  • [Chemical-registry-number] 0 / Catecholamines; 0 / Radiopharmaceuticals; 0TTZ664R7Z / Phenoxybenzamine; 35MRW7B4AD / 3-Iodobenzylguanidine
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99. Aupperle H, März I, Ellenberger C, Buschatz S, Reischauer A, Schoon HA: Primary and secondary heart tumours in dogs and cats. J Comp Pathol; 2007 Jan;136(1):18-26
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [MeSH-major] Cat Diseases / pathology. Dog Diseases / pathology. Heart Neoplasms / veterinary. Hemangiosarcoma / veterinary. Lymphoma, B-Cell / veterinary. Neurofibrosarcoma / veterinary. Paraganglioma, Extra-Adrenal / veterinary

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  • (PMID = 17270204.001).
  • [ISSN] 0021-9975
  • [Journal-full-title] Journal of comparative pathology
  • [ISO-abbreviation] J. Comp. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
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100. Qiao GB, Fang Y, Zeng WS, Peng LJ, Huang WJ: Images for diagnosis. An unusual case of Carney triad with high level catecholamine-secreting but no existence of extra-adrenal paraganglioma. Chin Med J (Engl); 2010 Feb 20;123(4):510-2
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Images for diagnosis. An unusual case of Carney triad with high level catecholamine-secreting but no existence of extra-adrenal paraganglioma.
  • [MeSH-major] Catecholamines / secretion. Lung Neoplasms / diagnosis. Paraganglioma, Extra-Adrenal / diagnosis. Stomach Neoplasms / diagnosis

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  • (PMID = 20193497.001).
  • [ISSN] 0366-6999
  • [Journal-full-title] Chinese medical journal
  • [ISO-abbreviation] Chin. Med. J.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] China
  • [Chemical-registry-number] 0 / Catecholamines
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