MCID: NNS018
MIFTS: 30

Nonsyndromic Paraganglioma

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

Aliases & Classifications for Nonsyndromic Paraganglioma

MalaCards integrated aliases for Nonsyndromic Paraganglioma:

Name: Nonsyndromic Paraganglioma 25
Extra-Adrenal Paraganglioma 71
Carotid Body Paraganglioma 71
Chemodectoma 25

Classifications:



External Ids:

UMLS 71 C0007279 C0030422

Summaries for Nonsyndromic Paraganglioma

Genetics Home Reference : 25 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. Some people develop a paraganglioma or pheochromocytoma as part of a hereditary syndrome that may affect other organs and tissues in the body. However, the tumors often are not associated with any syndromes, in which case the condition is called nonsyndromic paraganglioma or pheochromocytoma. Pheochromocytomas and some other paragangliomas are associated with ganglia of the sympathetic nervous system. The sympathetic nervous system controls the "fight-or-flight" response, a series of changes in the body due to hormones released in response to stress. Although most sympathetic paragangliomas are pheochromocytomas, some are found outside the adrenal glands, usually in the abdomen, and are called extra-adrenal paragangliomas. Most sympathetic paragangliomas, including pheochromocytomas, produce hormones called catecholamines, such as epinephrine (adrenaline) or norepinephrine. These excess catecholamines can cause signs and symptoms such as high blood pressure (hypertension), episodes of rapid heartbeat (palpitations), headaches, or sweating. Most paragangliomas are associated with ganglia of the parasympathetic nervous system, which controls involuntary body functions such as digestion and saliva formation. Parasympathetic paragangliomas, typically found in the head and neck, usually do not produce hormones. However, large tumors may cause signs and symptoms such as coughing, hearing loss in one ear, or difficulty swallowing. Although most paragangliomas and pheochromocytomas are noncancerous, some can become cancerous (malignant) and spread to other parts of the body (metastasize). Extra-adrenal paragangliomas become malignant more often than other types of paraganglioma or pheochromocytoma.

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

Related Diseases for Nonsyndromic Paraganglioma

Diseases in the Paraganglioma family:

Paragangliomas 4 Paragangliomas 1
Paragangliomas 2 Paragangliomas 3
Paragangliomas 5 Paragangliomas 6
Paragangliomas 7 Nonsyndromic Paraganglioma

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

(show top 50) (show all 79)
# Related Disease Score Top Affiliating Genes
1 abdominal chemodectomas with cutaneous angiolipomas 12.5
2 chemodectoma, intraabdominal, with cutaneous angiolipomas 12.3
3 non-secreting chemodectoma 12.2
4 paragangliomas 1 12.2
5 paraganglioma 11.9
6 glomus tumor 10.5
7 rare tumor 10.4
8 pheochromocytoma 10.3
9 adrenal gland pheochromocytoma 10.3
10 pericarditis 10.2
11 hemangioma 10.2
12 osteochondroma 10.2
13 ocular motor apraxia 10.1
14 horner's syndrome 10.1
15 goiter 10.1
16 neurilemmoma 10.1
17 cranial nerve palsy 10.1
18 paraplegia 10.1
19 adenoma 10.1
20 carotid body cancer 10.1
21 exophthalmos 10.1
22 back pain 10.1
23 dysphagia 10.1
24 hypoxia 10.1
25 thyroid carcinoma 10.1
26 hepatocellular carcinoma 9.9
27 lipomatosis, multiple 9.9
28 otitis media 9.9
29 sarcoidosis 1 9.9
30 thrombophilia due to thrombin defect 9.9
31 thyroid cancer, nonmedullary, 1 9.9
32 angiolipomatosis, familial 9.9
33 congenital heart defects, hamartomas of tongue, and polysyndactyly 9.9
34 myasthenia gravis 9.9
35 thymoma, familial 9.9
36 branchiootic syndrome 1 9.9
37 meningioma, radiation-induced 9.9
38 meningioma, familial 9.9
39 ataxia, combined cerebellar and peripheral, with hearing loss and diabetes mellitus 9.9
40 deficiency anemia 9.9
41 atrioventricular block 9.9
42 third-degree atrioventricular block 9.9
43 extra-adrenal pheochromocytoma 9.9
44 mucositis 9.9
45 pleomorphic lipoma 9.9
46 petrositis 9.9
47 spinal meningioma 9.9
48 hemopericardium 9.9
49 cardiac tamponade 9.9
50 cauda equina syndrome 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 12)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Epinephrine Approved, Vet_approved Phase 2 51-43-4 5816
2
Sirolimus Approved, Investigational Phase 2 53123-88-9 5284616 6436030 46835353
3
Everolimus Approved Phase 2 159351-69-6 6442177 70789204
4
Racepinephrine Approved Phase 2 329-65-7 838
5
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
6 Epinephryl borate Phase 2
7 Immunologic Factors Phase 2
8 Anti-Infective Agents Phase 2
9 Antifungal Agents Phase 2
10 Antibiotics, Antitubercular Phase 2
11 Immunosuppressive Agents Phase 2
12 Anti-Bacterial Agents Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Phase II Study of RAD001monotherapy in Patients With Unresectable Pheochromocytoma or Extra-adrenal Paraganglioma or Non-functioning Carcinoid Completed NCT01152827 Phase 2 RAD001
2 A Phase 2 Study of Pazopanib (GW786034) in Patients With Advanced and Progressive Malignant Pheochromocytoma or Paraganglioma Terminated NCT01340794 Phase 2 Pazopanib Hydrochloride

Search NIH Clinical Center for Nonsyndromic Paraganglioma

Genetic Tests for Nonsyndromic Paraganglioma

Anatomical Context for Nonsyndromic Paraganglioma

MalaCards organs/tissues related to Nonsyndromic Paraganglioma:

40
Adrenal Gland, Kidney, Lung, Testes, Cortex, Spinal Cord, Heart

Publications for Nonsyndromic Paraganglioma

Articles related to Nonsyndromic Paraganglioma:

(show top 50) (show all 150)
# Title Authors PMID Year
1
Prospective evaluation of 68Ga-DOTANOC positron emission tomography/computed tomography and 131I-meta-iodobenzylguanidine single-photon emission computed tomography/computed tomography in extra-adrenal paragangliomas, including uncommon primary sites and to define their diagnostic roles in current scenario. 61
31633647 2019
2
Ligament-Saving Laminoplasty for Intraspinal Tumor Excision: A Technical Note. 61
31103766 2019
3
Laparoscopic resection of retroperitoneal paraganglioma behind the Spiegel lobe in a kyphotic patient: A rare case report. 61
30015378 2019
4
68Ga-DOTATOC PET/CT in the localization of metastatic extra-adrenal paraganglioma and pheochromocytoma compared with 18F-DOPA PET/CT. 61
30630744 2019
5
Orbital paraganglioma in a dog. 61
30658369 2018
6
Immediate Clinical Success After Percutaneous Ablation of Extra-adrenal Paraganglioma. 61
29922859 2018
7
Diagnosis of paraganglioma as a pancreatic mass: A case report. 61
29882285 2018
8
Extra-adrenal paraganglioma masquerading as severe preeclampsia. 61
30018907 2018
9
Posterior Retroperitoneoscopic Resection of Extra-adrenal Paraganglioma Located in the Aorto-caval Space. 61
29330717 2018
10
Functional Ulnar Nerve Paraganglioma: First Documented Occurrence in the Extremity With Hitherto Undescribed Associated Extensive Glomus Cell Hyperplasia and Tumorlet Formation. 61
28697655 2018
11
Clinical evaluation and treatment of phaeochromocytoma. 61
29027806 2018
12
MR imaging features of benign retroperitoneal paragangliomas and schwannomas. 61
29301496 2018
13
68Ga-DOTA-TATE PET/CT for Molecular Imaging of Somatostatin Receptor Expression in Extra-adrenal Paraganglioma in a Case of Complete Carney Triad. 61
29076898 2017
14
Peripancreatic paraganglioma mimics pancreatic/gastrointestinal neuroendocrine tumor on fine needle aspiration: Report of two cases and review of the literature. 61
28560856 2017
15
Extra-adrenal pheochromocytoma at the organ of Zuckerkandl: a case report and literature review. 61
28491185 2017
16
Compared to 123I-MIBG SPECT/CT, 18F-DOPA PET/CT provides accurate tumor extent in patients with extra-adrenal paraganglioma. 61
28349331 2017
17
Challenging differential diagnosis of an extra-adrenal paraganglioma; the role of fine needle aspiration cytology. 61
28261927 2017
18
[Carney triad: Report of one case]. 61
28749001 2017
19
Pheochromocytoma of the Organ Zuckerkandl. 61
28414676 2017
20
A phase I clinical trial for [131I]meta-iodobenzylguanidine therapy in patients with refractory pheochromocytoma and paraganglioma: a study protocol. 61
28954983 2017
21
[Carney triad]. 61
28931294 2017
22
Malignant extra-adrenal pelvic paraganglioma in a paediatric patient. 61
28900474 2017
23
Functional extra-adrenal paraganglioma of the retroperitoneum giving thoracolumbar spine metastases after a five-year disease-free follow-up: a rare malignant condition with challenging management. 61
29255564 2017
24
An unusual case of incomplete Carney triad: an 18-year-old girl suffering from multiple benign tumors. 61
27867588 2016
25
Sudden Death and an Extra-Adrenal Paraganglioma. 61
31239903 2016
26
Imaging findings of malignant bilateral carotid body tumors: A case report and review of the literature. 61
27073498 2016
27
Pelvic paraganglioma: a rare and unusual clinical presentation of paraganglioma. 61
26740269 2016
28
Sporadic paraganglioma caused by de novo SDHB mutations in a 6-year-old girl. 61
26283294 2016
29
Primary pancreatic paraganglioma: a case report and literature review. 61
26801079 2016
30
A resected case of liver metastases from extra-adrenal retroperitoneal paraganglioma with von Recklinghausen's disease 16 years after the initial surgery. 61
26943413 2015
31
Carotid Body Tumor Presenting as Parotid Swelling Misdiagnosed as Pleomorphic Adenoma: A Rare Presentation. 61
26425073 2015
32
Multifocal Extra-Adrenal Paraganglioma - Case Report. 61
26351793 2015
33
Comparison of 131I-MIBG, 68Ga-DOTANOC PET/CT and 18F-FDG PET/CT scans in a patient with extra adrenal paraganglioma associated with SDH-B gene mutation. 61
25742231 2015
34
A case of carotid body tumor concomitant with carcinoid tumor. 61
25199739 2015
35
Combined PET/CT by 18F-FDOPA, 18F-FDA, 18F-FDG, and MRI correlation on a patient with Carney triad. 61
25423347 2015
36
Paraganglioma Presenting as Postpartum Fever of Unknown Origin. 61
26236513 2015
37
Gastric paraganglioma: a case report and a review of the literature. 61
25559752 2014
38
Extra-adrenal paraganglioma of the median nerve. 61
23750845 2014
39
Extra-adrenal paraganglioma presenting as acute chest pain mimicking acute myocardial infarction in a 10-year-old boy. 61
25373568 2014
40
Recurrent malignant pheochromocytoma with unusual omental metastasis: (68)Ga-DOTANOC PET/CT and (131)I-MIBG SPECT/CT scintigraphy findings. 61
25400380 2014
41
Rare extra-adrenal paraganglioma mimicking a painful Schwannoma: case report. 61
25285725 2014
42
Primary Retroperitoneal Inter-aorto-Caval Paraganglioma of the Organ of Zuckerkandl. 61
25177602 2014
43
Remission of a renal artery stenosis after laparoscopic removal of an extra adrenal paraganglioma. 61
25015630 2014
44
Cytomorphologic spectrum in aspirates of extra-adrenal paraganglioma. 61
25210234 2014
45
Non-functioning Aortocaval Paraganglioma Masquerading as Celiac Lymphnodal Metastasis. 61
25610296 2014
46
Unrecognized paraganglioma of the urinary bladder as a cause for basilar-type migraine. 61
23735539 2014
47
Anesthetic management of a patient with undiagnosed paraganglioma -a case report-. 61
24427467 2013
48
Multifocal extra-adrenal paraganglioma evaluated with different PET tracers: comparison between 18F-FDG, 18F-DOPA and 68Ga DOTANOC PET/CT. 61
23486329 2013
49
A retrospective comparison between 68Ga-DOTA-TOC PET/CT and 18F-DOPA PET/CT in patients with extra-adrenal paraganglioma. 61
24072345 2013
50
Extra-adrenal retroperitoneal paraganglioma in a dog. 61
24105380 2013

Variations for Nonsyndromic Paraganglioma

Cosmic variations for Nonsyndromic Paraganglioma:

9 (show top 50) (show all 113)
# Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)
Mutation CDS Mutation AA GRCh38 Location Conf
1 COSM88301868 VHL adrenal gland,adrenal gland,pheochromocytoma,benign c.389T>G p.V130G 3:10146562-10146562 4
2 COSM88294143 VHL adrenal gland,adrenal gland,pheochromocytoma,benign c.260T>C p.V87A 3:10142107-10142107 4
3 COSM88293303 VHL adrenal gland,adrenal gland,pheochromocytoma,benign c.475A>G p.K159E 3:10149798-10149798 4
4 COSM88288959 VHL adrenal gland,adrenal gland,pheochromocytoma,benign c.482G>A p.R161Q 3:10149805-10149805 4
5 COSM88296074 VHL adrenal gland,adrenal gland,pheochromocytoma,benign c.250G>C p.V84L 3:10142097-10142097 4
6 COSM88292246 VHL adrenal gland,adrenal gland,pheochromocytoma,benign c.227T>A p.F76Y 3:10142074-10142074 4
7 COSM88292324 VHL adrenal gland,adrenal gland,pheochromocytoma,benign c.496G>T p.V166F 3:10149819-10149819 4
8 COSM88305342 VHL adrenal gland,adrenal gland,pheochromocytoma,benign c.492G>T p.Q164H 3:10149815-10149815 4
9 COSM88292236 VHL adrenal gland,adrenal gland,pheochromocytoma,benign c.491A>G p.Q164R 3:10149814-10149814 4
10 COSM92347003 RET adrenal gland,adrenal gland,pheochromocytoma,benign c.2753T>C p.M918T 10:43121968-43121968 4
11 COSM92347226 RET adrenal gland,adrenal gland,pheochromocytoma,benign c.1900T>C p.C634R 10:43114500-43114500 4
12 COSM93656552 NF1 adrenal gland,adrenal gland,pheochromocytoma,benign c.1885G>A p.G629R 17:31225134-31225134 4
13 COSM93692982 NF1 adrenal gland,adrenal gland,pheochromocytoma,benign c.5665G>T p.E1889* 17:31330351-31330351 4
14 COSM93650970 NF1 adrenal gland,adrenal gland,pheochromocytoma,benign c.1466A>G p.Y489C 17:31214524-31214524 4
15 COSM93662933 NF1 adrenal gland,adrenal gland,pheochromocytoma,benign c.7582C>T p.Q2528* 17:31352381-31352381 4
16 COSM93654948 NF1 adrenal gland,adrenal gland,pheochromocytoma,benign c.7646C>G p.S2549* 17:31356490-31356490 4
17 COSM93650764 NF1 adrenal gland,adrenal gland,pheochromocytoma,benign c.7300C>T p.Q2434* 17:31349230-31349230 4
18 COSM93652933 NF1 adrenal gland,adrenal gland,pheochromocytoma,benign c.205-1G>C p.? 17:31159009-31159009 4
19 COSM93668028 NF1 adrenal gland,adrenal gland,pheochromocytoma,benign c.1722-1G>A p.? 17:31223443-31223443 4
20 COSM93647950 NF1 adrenal gland,adrenal gland,pheochromocytoma,benign c.1721+3A>T p.? 17:31221932-31221932 4
21 COSM93688082 NF1 adrenal gland,adrenal gland,pheochromocytoma,benign c.6855C>A p.Y2285* 17:31338739-31338739 4
22 COSM93680586 NF1 adrenal gland,adrenal gland,pheochromocytoma,benign c.226G>T p.E76* 17:31159031-31159031 4
23 COSM93687360 NF1 adrenal gland,adrenal gland,pheochromocytoma,benign c.3158C>A p.S1053* 17:31230886-31230886 4
24 COSM93653832 NF1 adrenal gland,adrenal gland,pheochromocytoma,benign c.2409+1G>A p.? 17:31227607-31227607 4
25 COSM88844097 MET adrenal gland,adrenal gland,pheochromocytoma,benign c.3029C>T p.T1010I 7:116771936-116771936 4
26 COSM88849124 MET adrenal gland,adrenal gland,pheochromocytoma,benign c.607T>A p.S203T 7:116699691-116699691 4
27 COSM88850455 MET adrenal gland,adrenal gland,pheochromocytoma,benign c.352A>T p.M118L 7:116699436-116699436 4
28 COSM112988851 HRAS adrenal gland,adrenal gland,pheochromocytoma,benign c.182A>G p.Q61R 11:533874-533874 4
29 COSM112988925 HRAS adrenal gland,adrenal gland,pheochromocytoma,benign c.181C>A p.Q61K 11:533875-533875 4
30 COSM112988832 HRAS adrenal gland,adrenal gland,pheochromocytoma,benign c.37G>C p.G13R 11:534286-534286 4
31 COSM95516578 H3-3A adrenal gland,adrenal gland,pheochromocytoma,benign c.103G>T p.G35W 1:226064454-226064454 4
32 COSM107493928 FGFR1 adrenal gland,adrenal gland,pheochromocytoma,benign c.1731C>A p.N577K 8:38417331-38417331 4
33 COSM86761750 EPAS1 adrenal gland,adrenal gland,pheochromocytoma,benign c.1104G>A p.M368I 2:46376608-46376608 4
34 COSM86761419 EPAS1 adrenal gland,adrenal gland,pheochromocytoma,benign c.1589C>A p.A530E 2:46380261-46380261 4
35 COSM86757527 EPAS1 adrenal gland,adrenal gland,pheochromocytoma,benign c.1595A>G p.Y532C 2:46380267-46380267 4
36 COSM86759134 EPAS1 adrenal gland,adrenal gland,pheochromocytoma,benign c.1592C>T p.P531L 2:46380264-46380264 4
37 COSM93530486 adrenal gland,adrenal gland,pheochromocytoma,benign c.226G>T p.E76* 17:31159031-31159031 4
38 COSM91331163 adrenal gland,adrenal gland,pheochromocytoma,benign c.182A>G p.Q61R 11:533874-533874 4
39 COSM120509060 adrenal gland,adrenal gland,pheochromocytoma,benign c.1466A>G p.Y489C 17:31214524-31214524 4
40 COSM92696795 adrenal gland,adrenal gland,pheochromocytoma,benign c.*688C>A p.? 8:38417331-38417331 4
41 COSM93535461 adrenal gland,adrenal gland,pheochromocytoma,benign c.3158C>A p.S1053* 17:31230886-31230886 4
42 COSM101951610 adrenal gland,adrenal gland,pheochromocytoma,benign c.182A>G p.Q61R 11:533874-533874 4
43 COSM109968573 adrenal gland,adrenal gland,pheochromocytoma,benign c.226G>T p.E76* 17:31159031-31159031 4
44 COSM109960979 adrenal gland,adrenal gland,pheochromocytoma,benign c.1466A>G p.Y489C 17:31214524-31214524 4
45 COSM93512112 adrenal gland,adrenal gland,pheochromocytoma,benign c.1885G>A p.G629R 17:31225134-31225134 4
46 COSM90654925 adrenal gland,adrenal gland,pheochromocytoma,benign c.260T>C p.V87A 3:10142107-10142107 4
47 COSM105721394 adrenal gland,adrenal gland,pheochromocytoma,benign c.182A>G p.Q61R 11:533874-533874 4
48 COSM93508888 adrenal gland,adrenal gland,pheochromocytoma,benign c.205-1G>C p.? 17:31159009-31159009 4
49 COSM90652830 adrenal gland,adrenal gland,pheochromocytoma,benign c.373G>T p.V125F 3:10149819-10149819 4
50 COSM111018308 adrenal gland,adrenal gland,pheochromocytoma,benign c.352A>T p.M118L 7:116699436-116699436 4

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
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
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32 ICD10
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39 LOVD
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43 MeSH
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56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
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70 Tocris
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72 UMLS via Orphanet
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