MCID: NNS018
MIFTS: 20

Nonsyndromic Paraganglioma

Categories: Cancer diseases, Genetic diseases, Rare diseases, Ear diseases, Endocrine diseases

Aliases & Classifications for Nonsyndromic Paraganglioma

MalaCards integrated aliases for Nonsyndromic Paraganglioma:

Name: Nonsyndromic Paraganglioma 24
Extra-Adrenal Paraganglioma 69
Carotid Body Paraganglioma 69
Chemodectoma 24

Classifications:



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Summaries for Nonsyndromic Paraganglioma

Genetics Home Reference : 24 Paraganglioma is a type of noncancerous (benign) tumor that occurs in structures called paraganglia. Paraganglia are groups of cells that are found near nerve cell bunches called ganglia. Paragangliomas are usually found in the head, neck, or torso. However, a type of paraganglioma known as pheochromocytoma develops in the adrenal glands. Adrenal glands are located on top of each kidney and produce hormones in response to stress. Most people with paraganglioma develop only one tumor in their lifetime.

MalaCards based summary : Nonsyndromic Paraganglioma, also known as extra-adrenal paraganglioma, is related to chemodectoma, intraabdominal, with cutaneous angiolipomas and abdominal chemodectomas with cutaneous angiolipomas. The drugs Epinephrine and Miconazole have been mentioned in the context of this disorder. Affiliated tissues include adrenal gland and kidney.

Related Diseases for Nonsyndromic Paraganglioma

Diseases in the Paraganglioma family:

Paragangliomas 4 Paragangliomas 1
Paragangliomas 2 Paragangliomas 3
Paragangliomas 5 Nonsyndromic Paraganglioma

Diseases related to Nonsyndromic Paraganglioma via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 11)
# Related Disease Score Top Affiliating Genes
1 chemodectoma, intraabdominal, with cutaneous angiolipomas 11.9
2 abdominal chemodectomas with cutaneous angiolipomas 11.9
3 non-secreting chemodectoma 11.9
4 paragangliomas 1 11.7
5 paraganglioma 11.4
6 glomus tumor 10.0
7 atrioventricular block 9.9
8 third-degree atrioventricular block 9.9
9 neuroendocrine tumor 9.9
10 ganglioneuroma 9.9
11 syncope 9.9

Graphical network of the top 20 diseases related to Nonsyndromic Paraganglioma:



Diseases related to Nonsyndromic Paraganglioma

Symptoms & Phenotypes for Nonsyndromic Paraganglioma

Drugs & Therapeutics for Nonsyndromic Paraganglioma

Drugs for Nonsyndromic Paraganglioma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 16)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Epinephrine Approved, Vet_approved Phase 2 51-43-4 5816
2
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
3
Everolimus Approved Phase 2 159351-69-6 6442177
4
Sirolimus Approved, Investigational Phase 2 53123-88-9 46835353 6436030 5284616
5 Racepinephrine Approved Phase 2 329-65-7
6 Anti-Bacterial Agents Phase 2
7 Epinephryl borate Phase 2
8 Anti-Infective Agents Phase 2
9 Antifungal Agents Phase 2
10 Immunosuppressive Agents Phase 2
11 Antibiotics, Antitubercular Phase 2
12
Dacarbazine Approved, Investigational Phase 1 4342-03-4 5351166
13
Temozolomide Approved, Investigational Phase 1 85622-93-1 5394
14
Vorinostat Approved, Investigational Phase 1 149647-78-9 5311
15 Alkylating Agents Phase 1
16 Histone Deacetylase Inhibitors Phase 1

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 RAD001 in Pheochromocytoma or Nonfunctioning Carcinoid Completed NCT01152827 Phase 2 RAD001
2 Pazopanib Hydrochloride in Treating Patients With Advanced or Progressive Malignant Pheochromocytoma or Paraganglioma Terminated NCT01340794 Phase 2 Pazopanib Hydrochloride
3 Vorinostat and Temozolomide in Treating Young Patients With Relapsed or Refractory Primary Brain Tumors or Spinal Cord Tumors Completed NCT01076530 Phase 1 vorinostat;temozolomide

Search NIH Clinical Center for Nonsyndromic Paraganglioma

Genetic Tests for Nonsyndromic Paraganglioma

Anatomical Context for Nonsyndromic Paraganglioma

MalaCards organs/tissues related to Nonsyndromic Paraganglioma:

38
Adrenal Gland, Kidney

Publications for Nonsyndromic Paraganglioma

Articles related to Nonsyndromic Paraganglioma:

(show all 28)
# Title Authors Year
1
68Ga-DOTA-TATE PET/CT for Molecular Imaging of Somatostatin Receptor Expression in Extra-adrenal Paraganglioma in a Case of Complete Carney Triad. ( 29076898 )
2017
2
Rare extra-adrenal paraganglioma mimicking a painful Schwannoma Case report. ( 25285725 )
2014
3
Extra-adrenal Paraganglioma Presenting as Acute Chest Pain Mimicking Acute Myocardial Infarction in a 10-Year-Old Boy. ( 25373568 )
2014
4
Extra-adrenal paraganglioma of the median nerve. ( 23750845 )
2013
5
Multifocal extra-adrenal paraganglioma evaluated with different PET tracers: comparison between 18F-FDG, 18F-DOPA and 68Ga DOTANOC PET/CT. ( 23486329 )
2013
6
A retrospective comparison between 68Ga-DOTA-TOC PET/CT and 18F-DOPA PET/CT in patients with extra-adrenal paraganglioma. ( 24072345 )
2013
7
Extra-adrenal paraganglioma of the prostate. ( 23766843 )
2013
8
Diagnostic tests and biomarkers for pheochromocytoma and extra-adrenal paraganglioma: from routine laboratory methods to disease stratification. ( 22180288 )
2012
9
Update on tumours of the adrenal cortex, phaeochromocytoma and extra-adrenal paraganglioma. ( 20718871 )
2011
10
Images for diagnosis. An unusual case of Carney triad with high level catecholamine-secreting but no existence of extra-adrenal paraganglioma. ( 20193497 )
2010
11
Pheochromocytomas and extra-adrenal paragangliomas detected by screening in patients with SDHD-associated head-and-neck paragangliomas. ( 19289533 )
2009
12
Local-regional recurrence of sporadic or syndromic abdominal extra-adrenal paraganglioma: incidence, characteristics, and outcome. ( 19958924 )
2009
13
Methylation of the p16INK4A promoter is associated with malignant behavior in abdominal extra-adrenal paragangliomas but not pheochromocytomas. ( 18509008 )
2008
14
Molecular and cellular biology of pheochromocytomas and extra-adrenal paragangliomas. ( 17525480 )
2006
15
RET is expressed but not mutated in extra-adrenal paragangliomas. ( 10878547 )
2000
16
Gastric stromal sarcoma, pulmonary chondroma, and extra-adrenal paraganglioma (Carney Triad): natural history, adrenocortical component, and possible familial occurrence. ( 10377927 )
1999
17
A family with hereditary extra-adrenal paragangliomas without evidence for mutations in the von Hippel-Lindau disease or ret genes. ( 9509062 )
1998
18
Immunohistochemical demonstration of chromogranin A, chromogranin B, and secretogranin II in extra-adrenal paragangliomas. ( 8058707 )
1994
19
[New histopathologic data of prognostic value in extra-adrenal paragangliomas. Study of 9 cases]. ( 8105157 )
1993
20
Catecholamine synthesizing enzymes in 70 cases of functioning and non-functioning phaeochromocytoma and extra-adrenal paraganglioma. ( 1353277 )
1992
21
Extra-adrenal paraganglioma and pulmonary chondroma: a case report and review of the literature. ( 2884350 )
1987
22
Carney's triad: gastric leiomyosarcoma, pulmonary chondroma and extra-adrenal paraganglioma in young females. ( 3028925 )
1986
23
Gastric epitheloid leiomyomas, pulmonary chondroma, non-functioning metastasizing extra-adrenal paraganglioma and myxoma: a variant of Carney's triad. Report of a patient. ( 3990171 )
1985
24
The triad of gastric epithelioid leiomyosarcoma, pulmonary chondroma, and functioning extra-adrenal paraganglioma: a five-year review. ( 6843355 )
1983
25
Pulmonary chondroma, extra-adrenal paraganglioma, and gastric leiomyosarcoma: Carney's triad. ( 6287121 )
1982
26
Gastric tumors in patients with pulmonary chondroma or extra-adrenal paraganglioma: an ultrastructural study. ( 6895164 )
1981
27
The triad of gastric epithelioid leiomyosarcoma, functioning extra-adrenal paraganglioma, and pulmonary chondroma. ( 367573 )
1979
28
The triad of gastric leiomyosarcoma, functioning extra-adrenal paraganglioma and pulmonary chondroma. ( 865533 )
1977

Variations for Nonsyndromic Paraganglioma

Cosmic variations for Nonsyndromic Paraganglioma:

9 (show all 42)
# Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)
Mutation CDS Mutation AA Conf
1 COSM236660 VHL adrenal gland,adrenal gland,pheochromocytoma,benign c.250G>C p.V84L 4
2 COSM144975 VHL adrenal gland,adrenal gland,pheochromocytoma,benign c.475A>G p.K159E 4
3 COSM14283 VHL adrenal gland,adrenal gland,pheochromocytoma,benign c.491A>G p.Q164R 4
4 COSM17662 VHL adrenal gland,adrenal gland,pheochromocytoma,benign c.492G>T p.Q164H 4
5 COSM18097 VHL adrenal gland,adrenal gland,pheochromocytoma,benign c.482G>A p.R161Q 4
6 COSM14321 VHL adrenal gland,adrenal gland,pheochromocytoma,benign c.227T>A p.F76Y 4
7 COSM6444479 VHL adrenal gland,adrenal gland,pheochromocytoma,benign c.260T>C p.V87A 4
8 COSM100047 VHL adrenal gland,adrenal gland,pheochromocytoma,benign c.389T>G p.V130G 4
9 COSM17982 VHL adrenal gland,adrenal gland,pheochromocytoma,benign c.496G>T p.V166F 4
10 COSM966 RET adrenal gland,adrenal gland,pheochromocytoma,benign c.1900T>C p.C634R 4
11 COSM965 RET adrenal gland,adrenal gland,pheochromocytoma,benign c.2753T>C p.M918T 4
12 COSM1710108 NF1 adrenal gland,adrenal gland,pheochromocytoma,benign c.7300C>T p.Q2434* 4
13 COSM330588 NF1 adrenal gland,adrenal gland,pheochromocytoma,benign c.2409+1G>A p.A776_Q803del 4
14 COSM330587 NF1 adrenal gland,adrenal gland,pheochromocytoma,benign c.205-1G>C p.R69fs*7 4
15 COSM220089 NF1 adrenal gland,adrenal gland,pheochromocytoma,benign c.1885G>A p.Q616fs*4 4
16 COSM329092 NF1 adrenal gland,adrenal gland,pheochromocytoma,benign c.1721+3A>T p.A548fs*13 4
17 COSM30670 NF1 adrenal gland,adrenal gland,pheochromocytoma,benign c.7646C>G p.S2549* 4
18 COSM329090 NF1 adrenal gland,adrenal gland,pheochromocytoma,benign c.226G>T p.E76* 4
19 COSM5885098 NF1 adrenal gland,adrenal gland,pheochromocytoma,benign c.5665G>T p.E1889* 4
20 COSM329093 NF1 adrenal gland,adrenal gland,pheochromocytoma,benign c.1722-1G>A p.? 4
21 COSM329089 NF1 adrenal gland,adrenal gland,pheochromocytoma,benign c.1466A>G p.Y489C 4
22 COSM327926 NF1 adrenal gland,adrenal gland,pheochromocytoma,benign c.3158C>A p.S1053* 4
23 COSM327927 NF1 adrenal gland,adrenal gland,pheochromocytoma,benign c.7582C>T p.Q2528* 4
24 COSM33676 NF1 adrenal gland,adrenal gland,pheochromocytoma,benign c.6855C>A p.Y2285* 4
25 COSM5946188 MET adrenal gland,adrenal gland,pheochromocytoma,benign c.352A>T p.M118L 4
26 COSM707 MET adrenal gland,adrenal gland,pheochromocytoma,benign c.3029C>T p.T1010I 4
27 COSM5967149 MET adrenal gland,adrenal gland,pheochromocytoma,benign c.607T>A p.S203T 4
28 COSM499 HRAS adrenal gland,adrenal gland,pheochromocytoma,benign c.182A>G p.Q61R 4
29 COSM496 HRAS adrenal gland,adrenal gland,pheochromocytoma,benign c.181C>A p.Q61K 4
30 COSM486 HRAS adrenal gland,adrenal gland,pheochromocytoma,benign c.37G>C p.G13R 4
31 COSM1732355 H3F3A adrenal gland,adrenal gland,pheochromocytoma,benign c.103G>T p.G35W 4
32 COSM19176 FGFR1 adrenal gland,adrenal gland,pheochromocytoma,benign c.1638C>A p.N546K 4
33 COSM6188660 EPAS1 adrenal gland,adrenal gland,pheochromocytoma,benign c.1592C>T p.P531L 4
34 COSM6196778 EPAS1 adrenal gland,adrenal gland,pheochromocytoma,benign c.1104G>A p.M368I 4
35 COSM6196613 EPAS1 adrenal gland,adrenal gland,pheochromocytoma,benign c.1589C>A p.A530E 4
36 COSM6188649 EPAS1 adrenal gland,adrenal gland,pheochromocytoma,benign c.1595A>G p.Y532C 4
37 COSM17988 VHL adrenal gland,NS,pheochromocytoma,benign c.341-1G>T p.? 3
38 COSM144972 VHL adrenal gland,NS,pheochromocytoma,benign c.245G>T p.R82L 3
39 COSM144971 VHL adrenal gland,NS,pheochromocytoma,benign c.244C>G p.R82G 3
40 COSM14311 VHL adrenal gland,NS,pheochromocytoma,benign c.499C>T p.R167W 3
41 COSM3402726 NF1 adrenal gland,NS,pheochromocytoma,benign c.1307C>A p.S436* 3
42 COSM6476262 EPAS1 adrenal gland,NS,pheochromocytoma,benign c.1601C>T p.P534L 3

Expression for Nonsyndromic Paraganglioma

Search GEO for disease gene expression data for Nonsyndromic Paraganglioma.

Pathways for Nonsyndromic Paraganglioma

GO Terms for Nonsyndromic Paraganglioma

Sources for Nonsyndromic Paraganglioma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
27 GO
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29 HGMD
30 HMDB
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70 UMLS via Orphanet
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