MCID: PRG013
MIFTS: 49

Paraganglioma malady

Summaries for Paraganglioma

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63Wikipedia, 32MalaCards
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Wikipedia:63 A paraganglioma is rare neuroendocrine neoplasm that may develop at various body sites (including the... more...

MalaCards: Paraganglioma is related to pheochromocytoma and hereditary paraganglioma-pheochromocytoma syndromes. An important gene associated with Paraganglioma is SDHAF2 (succinate dehydrogenase complex assembly factor 2), and among its related pathways are Glycolysis and gluconeogenesis (short map) and Citric acid cycle (TCA cycle). The compounds UBIQUINONE-1 and 2-Hexyloxy-6-Hydroxymethyl-Tetrahydro-Pyran-3,4,5-Triol have been mentioned in the context of this disorder. Affiliated tissues include thyroid, testes and lymph node, and related mouse phenotype endocrine/exocrine gland.

Aliases & Classifications for Paraganglioma

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8Disease Ontology, 10DISEASES, 44Novoseek, 60UMLS, 34MeSH
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Aliases & Descriptions:

paraganglioma 8 10 44 60


External Ids:

Disease Ontology8 DOID:0050773
MeSH34 D010235

Related Diseases for Paraganglioma

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17GeneCards, 18GeneDecks
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Diseases in the Paragangliomas 4 family:

paraganglioma Paragangliomas 1
Paragangliomas 2 Paragangliomas 3
Nonsyndromic Paraganglioma Paragangliomas 5

Diseases related to Paraganglioma via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 215)
idRelated DiseaseScoreTop Affiliating Genes
1pheochromocytoma31.4SDHB, VHL, RET, SDHD
2hereditary paraganglioma-pheochromocytoma syndromes30.9SDHC, SDHB, SDHAF2, SDHD
3phaeochromocytoma30.9SDHC, SDHB, SDHD, RET, CHGA
4hypertension30.6PNMT
5chondroma30.5SDHD, SDHB, SDHC
6ganglioneuroma30.5SYP, CHGA, RET, ENO2, S100B, SCG2
7paraganglioma and gastric stromal sarcoma30.5SDHB, SDHD, SDHC
8sarcoma30.4S100B, SSTR2, ENO2
9glomus tumor30.3SDHD, VHL, SYP, SDHB
10gastrointestinal stromal tumor30.3ENO2, SDHB, S100B, SDHA, SDHC
11thyroid medullary carcinoma30.3ENO2, SSTR2, MEN1, CHGA, CHGB, VHL
12adenoma30.3SCG2, CHGA, VHL, MEN1, CHGB, SYP
13neuroendocrine tumor30.3CHGA, SSTR2
14von hippel-lindau disease30.3SDHC, SDHB, SDHD, TMEM127, RET, MEN1
15paragangliomas 130.3SDHB, CHGA, RET
16neuroblastoma30.2ENO2, SYP
17hepatitis30.2GAL
18carotid body tumor30.1SDHC, SDHB, SDHD
19thyroid cancer30.1CHGA, RET
20papillary carcinoma30.1CHGA, SSTR2, SYP, RET
21pituitary adenoma30.1RET, SSTR2, CHGA, SYP, MEN1, GAL
22somatostatinoma30.0SSTR2, CHGA
23ganglioneuroblastoma30.0ENO2, S100B, SYP
24carney triad29.9SDHD, SDHB, SDHC
25neurofibromatosis29.9SDHC, SDHB, SDHD, ENO2, RET, MEN1
26parathyroid adenoma29.9CHGB, CHGA, MEN1, RET
27neuroendocrine carcinoma29.9S100B, CHGA, SCG2, MEN1, SYP, ENO2
28meningioma29.9SYP, S100B, SSTR2, MEN1
29melanoma29.9ENO2, S100B, GAL, SSTR2
30hemangioma29.7RET, MEN1, VHL, SDHD, SDHAF2, SDHC
31granular cell tumor29.7S100B, SYP, ENO2
32cushing's syndrome29.7RET, CHGA, SYP, MEN1
33merkel cell carcinoma29.7SYP, CHGA, ENO2
34peripheral primitive neuroectodermal tumor29.7SCG2, S100B, CHGA, ENO2
35adenocarcinoma29.7CHGA, SSTR2, VHL, SCG2, GAL, MEN1
36vipoma29.7CHGA, MEN1
37thyroiditis10.5
38duodenitis10.4
39mediastinitis10.4
40cervicitis10.3
41laryngitis10.3
42pancreatitis10.3
43nonsyndromic paraganglioma10.3
44paragangliomas 410.3
45polycythemia10.2
46paragangliomas 210.2
47paragangliomas 310.2
48leiomyosarcoma10.2
49paragangliomas 1, with or without deafness10.2
50gastric leiomyosarcoma10.2

Graphical network of the top 20 diseases related to Paraganglioma:



Diseases related to paraganglioma

Clinical Features for Paraganglioma

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Drugs & Therapeutics for Paraganglioma

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5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-RT
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Approved drugs:

Search CenterWatch for Paraganglioma

Drug clinical trials:

Search ClinicalTrials for Paraganglioma

Search NIH Clinical Center for Paraganglioma

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Genetic Tests for Paraganglioma

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Anatomical Context for Paraganglioma

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32MalaCards
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MalaCards organs/tissues related to Paraganglioma:

32
Thyroid, Testes, Lymph node, Pituitary, Liver, Bone, Lung, Prostate, Eye, Ovary, Kidney, Heart, Spinal cord, Adrenal cortex, Appendix, Trachea, Tonsil, Brain, Cortex, Uterus, Cerebellum, Breast, Pancreas

Animal Models for Paraganglioma or affiliated genes

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36MGI
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MGI Mouse Phenotypes related to Paraganglioma:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:000537910.4SDHD, RET, CHGB, CHGA, MEN1, GAL

Publications for Paraganglioma

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50PubMed
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Articles related to Paraganglioma:

(show top 50)    (show all 844)
idTitleAuthorsYear
1
Paraganglioma presenting with marked proteinuria: a case report. (23154520)
2014
2
Sympathetic paraganglioma in a patient with unrepaired tetralogy of Fallot: a case report and review of the literature. (23150681)
2013
3
First Experience With Image-guided Resection of Paraganglioma. (24217546)
2013
4
Metastatic paraganglioma presenting as a primary shoulder mass. (23722292)
2013
5
Anesthetic management for resection of hepatic paraganglioma metastatic from the donor organ in an orthotopic liver transplant recipient: a case report. (23498826)
2013
6
Iodine-131 metaiodobenzylguanidine (I-131 MIBG) diagnosis and therapy of pheochromocytoma and paraganglioma: current problems, critical issues and presentation of a sample case. (23822990)
2013
7
Mosaicism in HIF2A-related polycythaemia-paraganglioma syndrome. (24276449)
2013
8
An unusual case of paraganglioma. (24381898)
2013
9
Endobronchial Gangliocytic Paraganglioma: Not All Keratin-Positive Endobronchial Neuroendocrine Neoplasms are Pulmonary Carcinoids. (23912549)
2013
10
Radiology quiz case 1: glomus facialis paraganglioma. (23329103)
2013
11
Prevalence and spectrum of SDHx mutations in pheochromocytoma and paraganglioma in patients from Belgium: an update. (22566194)
2012
12
Paraganglioma of urinary bladder: an unusual presentation. pitfalls in diagnosis and treatment. (22352107)
2012
13
Malignant paraganglioma of the mesentery: a case report and review of literature. (22360863)
2012
14
Diagnostic performance of 18F-dihydroxyphenylalanine positron emission tomography in patients with paraganglioma: a meta-analysis. (22358431)
2012
15
High (18)F-FDG uptake in sporadic paraganglioma of the retroperitoneum may be related to intra-tumor haemorrhage and macrophages. (23293798)
2012
16
Surgical treatment of an interatrial septal paraganglioma. (23087305)
2012
17
EANM 2012 guidelines for radionuclide imaging of phaeochromocytoma and paraganglioma. (22926712)
2012
18
Staging and functional characterization of pheochromocytoma and paraganglioma by 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography. (22517990)
2012
19
Peptide receptor radionuclide therapy (PRRT) with 177Lu-DOTATATE in individuals with neck or mediastinal paraganglioma (PGL). (22566197)
2012
20
Paraganglioma of the cauda equina: a report of two cases with unusual histopathological features. (22192703)
2012
21
Aorticopulmonary paraganglioma. (22173680)
2011
22
A supratentorial primary parenchymal paraganglioma. (21565510)
2011
23
SOLITARY PARAGANGLIOMA OF THE HYPOGLOSSAL NERVE: CASE REPORT. (21242835)
2011
24
Minimally invasive treatment of metastatic pheochromocytoma and paraganglioma: efficacy and safety of radiofrequency ablation and cryoablation therapy. (21856504)
2011
25
Delayed intracranial and bony metastasis of paraganglioma. (21697977)
2011
26
Images for diagnosis. An unusual case of Carney triad with high level catecholamine-secreting but no existence of extra-adrenal paraganglioma. (20193497)
2010
27
Childhood phaeochromocytoma and paraganglioma: 100% incidence of genetic mutations and 100% survival. (20152357)
2010
28
Laryngeal paraganglioma: report of an unusual entity. (21086280)
2010
29
Exercise-induced nausea and vomiting: another sign and symptom of pheochromocytoma and paraganglioma. (20960160)
2010
30
Low penetrance of a SDHB mutation in a large Dutch paraganglioma family. (20540712)
2010
31
Rare presentation of familial paraganglioma without evidence of mutation in the SDH, RET and VHL genes: towards further genetic heterogeneity. (19145771)
2009
32
Composite paraganglioma-ganglioneuroma in the retroperitoneum. (19889235)
2009
33
Molecular characterization of novel germline deletions affecting SDHD and SDHC in pheochromocytoma and paraganglioma patients. (19546167)
2009
34
Cardiovascular flashlight. Paraganglioma of the aortopulmonary window. (19164332)
2009
35
Head and neck paragangliomas in von Hippel-Lindau disease and multiple endocrine neoplasia type 2. (19336503)
2009
36
Paraganglioma-like dermal melanocytic tumor: a case report with particular features. (20126591)
2009
37
When should genetic testing be obtained in a patient with phaeochromocytoma or paraganglioma? (19067729)
2009
38
The succinate dehydrogenase genetic testing in a large prospective series of patients with paragangliomas. (19454582)
2009
39
Diagnosis and management of hereditary paraganglioma syndrome due to the F933>X67 SDHD mutation. (19072999)
2009
40
The SDH mutation database: an online resource for succinate dehydrogenase sequence variants involved in pheochromocytoma, paraganglioma and mitochondrial complex II deficiency. (16288654)
2005
41
Paraganglioma in the area of the head and neck. A review of molecular genetic research]. (14740109)
2004
42
Hereditary paraganglioma due to the SDHD M1I mutation in a second Chinese family: a founder effect? (12782822)
2003
43
Calcified filum terminale paraganglioma causing superficial siderosis. (9609190)
1998
44
Paraganglioma-Like Adenomas of the Thyroid (PLAT): Incidental Lesions with Unusual Features in a Patient with Nodular Goiter. (12114635)
1997
45
Immunohistochemical characterization of a case of duodenal gangliocytic paraganglioma. (8893232)
1996
46
Immunohistochemical demonstration of chromogranin A, chromogranin B, and secretogranin II in extra-adrenal paragangliomas. (8058707)
1994
47
A comparative immunohistochemical study of phaeochromocytomas and paragangliomas. (8358154)
1993
48
Hereditary Paraganglioma-Pheochromocytoma Syndromes (20301715)
1993
49
Gastric tumors in patients with pulmonary chondroma or extra-adrenal paraganglioma: an ultrastructural study. (6895164)
1981
50
Extraadrenal paraganglioma, pulmonary chondroma, and gastric leiomyoblastoma: triad in young females. (106655)
1979

Genetic Variations for Paraganglioma

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Expression for genes affiliated with Paraganglioma

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1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Paraganglioma

Search GEO for disease gene expression data for Paraganglioma.

Pathways for genes affiliated with Paraganglioma

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12EMD Millipore, 37NCBI BioSystems Database, 53Reactome, 29KEGG, 4Cell Signaling Technology
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Pathways related to Paraganglioma according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
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10.5SDHA, SDHC, SDHB, SDHD
2
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10.5SDHA, SDHC, SDHB, SDHD
3
Hide members
10.5SDHD, SDHB, SDHC, SDHA
4
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10.5ENO2, SDHD, SDHB, SDHC, SDHA
5
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10.4SDHA, SDHC, SDHB, SDHD, ENO2, PNMT
610.4VHL, S100B, SYP, RET, ENO2

Compounds for genes affiliated with Paraganglioma

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11DrugBank, 44Novoseek, 28IUPHAR, 24HMDB, 49PharmGKB, 59Tocris Bioscience
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Compounds related to Paraganglioma according to GeneCards/GeneDecks:

(show top 50)    (show all 80)
idCompoundScoreTop Affiliating Genes
1UBIQUINONE-11110.9SDHD, SDHA, SDHB, SDHC
22-Hexyloxy-6-Hydroxymethyl-Tetrahydro-Pyran-3,4,5-Triol1110.9SDHA, SDHC, SDHB, SDHD
3succinate4410.9SDHB, SDHC, SDHA, SDHD, RET, VHL
4fumarate44 1111.9SDHB, SDHD, SDHC, VHL
5secretoneurin4410.9CHGB, CHGA, GAL, SCG2
6s 1004410.9S100B, ENO2, CHGA, SYP
7mibg4410.9SDHB, CHGA, ENO2, SDHD
8succinic acid28 11 2412.9SDHA, SDHB, SDHD, SDHC
9l-amino acid4410.9SYP, PNMT, ENO2, CHGA, S100B
10iron-sulfur4410.9SDHD, SDHB, SDHC, SDHA
11dopamine44 28 11 2413.9SDHB, CHGB, ENO2, PNMT, SSTR2, CHGA
12ubiquinone4410.9SDHB, SDHD, SDHA, SDHC
13forskolin44 49 1112.8MAX, GAL, SSTR2, ENO2
14iron44 2411.8SDHB, SDHC, SDHD, GAL, S100B, VHL
15catecholamine4410.8ENO2, RET, PNMT, CHGA, SDHD, SDHB
16norepinephrine44 11 2412.8SDHB, ENO2, PNMT, CHGB, CHGA, SYP
17acth4410.8CHGB, SYP, CHGA, GAL, S100B, SSTR2
185-hydroxytryptamine4410.8SCG2, SYP, CHGA, GAL, PNMT, ENO2
19vegf4410.8MEN1, SDHC, SDHB, ENO2, RET, SSTR2
20smcc4410.8SYP, CHGA, ENO2
21glycogen44 2411.8SDHB, ENO2, VHL, CHGA, S100B, SYP
22111in-pentetreotide4410.8SSTR2, CHGA, SYP
23histidine4410.8VHL, SDHB, PNMT, CHGA, SYP, S100B
24acetylcholine44 49 28 11 2414.8S100B, CHGA, SYP, GAL, SCG2, PNMT
25tyrosine4410.8S100B, VHL, ENO2, MEN1, PNMT, RET
26dexamethasone44 49 28 1113.8ENO2, PNMT, GAL, SCG2, S100B, CHGB
27oxygen44 2411.8ENO2, SDHA, VHL, S100B, CHGA, SDHD
28histamine44 28 2412.8CHGA, SYP, SSTR2, PNMT, SCG2, GAL
29formaldehyde44 2411.8CHGA, GAL, SYP, ENO2, S100B
30choline44 11 2412.8ENO2, S100B, SYP, GAL, RET
31calcium44 49 11 2413.7SDHB, ENO2, SSTR2, CHGA, CHGB, RET
32potassium44 11 2412.7S100B, GAL, SYP, CHGB, ENO2, VHL
33octreotide44 59 28 1113.7CHGA, SSTR2, GAL, ENO2
34glucose4410.7SDHB, SSTR2, GAL, SCG2
35testosterone44 59 11 2413.7CHGA, SYP, RET, ENO2, SDHB, S100B
36paraffin4410.7ENO2, RET, SSTR2, CHGA, SYP, S100B
37hematoxylin4410.7ENO2, CHGA, SYP, S100B
38gaba4410.7GAL, CHGA, SYP, S100B, ENO2
39amine4410.7SYP, CHGA, PNMT, ENO2
40etoposide44 49 59 1113.7S100B, GAL, CHGA
41nitric oxide44 11 2412.7CHGA, GAL, S100B, SYP, ENO2, VHL
42adenylate4410.6SSTR2, GAL, CHGA, S100B, ENO2, SCG2
43b 7234410.6CHGA, SYP, S100B
44cyclic amp44 2411.6S100B, SCG2, GAL, RET, SYP, CHGA
45retinoic acid44 2411.5CHGA, SYP, RET, SDHC, S100B, ENO2
46atp44 2811.5SDHB, ENO2, RET, S100B, CHGA, GAL
47edss4410.4ENO2, S100B
48serine4410.4RET, VHL, CHGB, MEN1, S100B, GAL
49estracyt4410.4CHGA, ENO2
50hydrogen44 2411.1PNMT, SYP, GAL, S100B

GO Terms for genes affiliated with Paraganglioma

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16Gene Ontology
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Cellular components related to Paraganglioma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1mitochondrial respiratory chain complex IIGO:00574910.5SDHA, SDHC, SDHB, SDHD
2mitochondrionGO:00573910.5SDHA, SDHC, SDHB, SDHAF2, SDHD, VHL
3secretory granuleGO:03014110.3SCG2, GAL, CHGA, CHGB
4mitochondrial inner membraneGO:00574310.2TIMM8B, SDHD, SDHB, SDHC, SDHA

Biological processes related to Paraganglioma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1tricarboxylic acid cycleGO:00609910.5SDHA, SDHC, SDHB, SDHD
2succinate metabolic processGO:00610510.5SDHA, SDHB
3respiratory electron transport chainGO:02290410.5SDHD, SDHB, SDHC, SDHA
4cellular metabolic processGO:04423710.4SDHD, SDHB, SDHC, SDHA
5MAPK cascadeGO:00016510.1RET, MEN1, SCG2

Molecular functions related to Paraganglioma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1ubiquinone bindingGO:04803910.5SDHB, SDHD
2succinate dehydrogenase (ubiquinone) activityGO:00817710.2SDHA, SDHB

Products for genes affiliated with Paraganglioma

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Sources for Paraganglioma

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet