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Paraganglioma (CUI C0030421) Suggest changes to this concept
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Terms & Properties

Concept Unique Identifier (CUI): C0030421

NCI Thesaurus Code: C3308  (see NCI Thesaurus info)

Semantic Type: Neoplastic Process

NCIt Definition: A benign or malignant neoplasm arising from paraganglia located along the sympathetic or parasympathetic nerves. Infrequently, it may arise outside the usual distribution of the sympathetic and parasympathetic paraganglia. Tumors arising from the adrenal gland medulla are called pheochromocytomas. Morphologically, paragangliomas usually display a nesting (Zellballen) growth pattern. There are no reliable morphologic criteria to distinguish between benign and malignant paragangliomas. The only definitive indicator of malignancy is the presence of regional or distant metastases.

RADLEX Definition: A neural crest tumor usually derived from the chromoreceptor tissue of a paraganglion, such as the carotid body, or medulla of the adrenal gland (usually called a chromaffinoma or pheochromocytoma). It is more common in women than in men. (Stedman, 25th ed; from Segen, Dictionary of Modern Medicine, 1992) [MeSH]

HPO Definition: A carotid body tumor (also called paraganglionoma or chemodectoma) is a tumor found in the upper neck at the branching of the carotid artery. They arise from the chemoreceptor organ (paraganglion) located in the adventitia of the carotid artery bifurcation. [HPO:probinson]

NCI-GLOSS Definition: A rare, usually benign tumor that develops from cells of the paraganglia. Paraganglia are a collection of cells that came from embryonic nervous tissue, and are found near the adrenal glands and some blood vessels and nerves. Paragangliomas that develop in the adrenal gland are called pheochromocytomas. Those that develop outside of the adrenal glands near blood vessels or nerves are called glomus tumors or chemodectomas.

MSH Definition: A neural crest tumor usually derived from the chromoreceptor tissue of a paraganglion, such as the carotid body, or medulla of the adrenal gland (usually called a chromaffinoma or pheochromocytoma). It is more common in women than in men. (Stedman, 25th ed; from Segen, Dictionary of Modern Medicine, 1992)

Synonyms & Abbreviations: (see Synonym Details)
Carotid body tumors
Gangliocytic paraganglioma (disorder)
Gangliocytic paraganglioma (morphologic abnormality)
Gangliocytic Paragangliomas
Gangliocytic paraganglioma
Neoplasm of paraganglion (disorder)
Neoplasm of Paraganglion
Neoplasm of the Paraganglion
Paraganglioma (disorder)
Paraganglioma (morphologic abnormality)
Paraganglioma [Disease/Finding]
Paraganglioma, Gangliocytic
Paraganglioma, NOS
Paragangliomas 1
Paragangliomas familial 1
Paragangliomas, Familial, 1
Paragangliomas, Gangliocytic
Paragangliomas
Paragangliomata
paraganglioma
Paragangliom
Paraganglion neoplasm NOS
Paraganglion neoplasm
Paraganglion Tumor
Paraganglionic Neoplasm
Paraganglionic Tumor
PGL 1
PGL1
PGL
SDHD-Related Hereditary Paraganglioma-Pheochromocytoma Syndrome (Paragangliomas 1)
Tumor of Paraganglion
Tumor of the Paraganglion

External Source Codes: 
NCI Thesaurus Code C3308 (see NCI Thesaurus info)

Other Properties: Property Definitions
Name Value Source
ACTIVE 1 SNOMEDCT_US
AN /blood supply /chem /second /secret /ultrastruct permitted; coord IM with precoord organ/neopl heading (IM) MSH
AQL BL BS CF CH CI CL CN CO DG DH DI DT EC EH EM EN EP ET GE HI IM ME MI MO NU PA PC PP PS PX RH RT SC SE SU TH UL UR VE VI MSH
CASE_SIGNIFICANCE_ID 900000000000448009 SNOMEDCT_US
CHARACTERISTIC_TYPE_ID 900000000000011006 SNOMEDCT_US
CODE_REF (C17.0) ICDO
Contributing_Source CTRP NCI
CTV3ID BBDE. SNOMEDCT_US
CTV3ID X77oB SNOMEDCT_US
CTV3ID Xa99M SNOMEDCT_US
CTV3ID XU6sB SNOMEDCT_US
CTV3ID XULIb SNOMEDCT_US
DATE_CREATED 2013-01-09T00:04:00 GARD
DATE_FIRST_PUBLISHED 2009-03-31 PDQ
DATE_LAST_MODIFIED 2009-10-19 PDQ
DATE_LAST_MODIFIED 2013-01-09T00:04:00 GARD
DC 1 MSH
DEFINITION_STATUS_ID 900000000000073002 SNOMEDCT_US
DEFINITION_STATUS_ID 900000000000074008 SNOMEDCT_US
DISEASE_IDENTIFIER_ID 5217 GARD
DISEASE_IDENTIFIER_ID 5218 GARD
DISEASE_IDENTIFIER_ID 5219 GARD
DISEASE_IDENTIFIER_ID 5220 GARD
DISEASE_IDENTIFIER_ID 5221 GARD
DX 19660101 MSH
EFFECTIVE_TIME 20020131 SNOMEDCT_US
EFFECTIVE_TIME 20030731 SNOMEDCT_US
GENELOCUS ,1,1,q,2,3, OMIM
GENESYMBOL CWS3 OMIM
GENESYMBOL PGL1 OMIM
GENESYMBOL SDHD OMIM
HAS_GARD_PAGE true GARD
ICD-O-3_Code 8680/1 NCI
ICD-O-3_CODE 8680/1 SNOMEDCT_US
ICD-O-3_CODE 8683/0 SNOMEDCT_US
IDENTIFIER_TYPE_ID 1 GARD
IS_ACTIVE true GARD
IS_RARE true GARD
IS_SPANISH false GARD
LAST_REVIEW_DATE 2009-11-30T00:00:00 GARD
MDA 19990101 MSH
MESH_DEFINITION A neural crest tumor usually derived from the chromoreceptor tissue of a paraganglion, such as the carotid body, or medulla of the adrenal gland (usually called a chromaffinoma or pheochromocytoma). It is more common in women than in men. (Stedman, 25th ed; from Segen, Dictionary of Modern Medicine, 1992) NDFRT
MESH_DUI D010235 NDFRT
MESH_NAME Paraganglioma NDFRT
MESH_UI M0015869 NDFRT
MIMTYPE 3 OMIM
MIMTYPEMEANING Phenotype description, molecular basis known. OMIM
MIMTYPEVALUE pound OMIM
MMR 20130708 MSH
MN C04.557.465.625.650.700 MSH
MN C04.557.580.625.650.700 MSH
MODIFIER_ID 900000000000451002 SNOMEDCT_US
NCI_THESAURUS_CODE C3308 PDQ
NDFRT_KIND DISEASE_KIND NDFRT
Neoplastic_Status Undetermined NCI
NUI N0000002299 NDFRT
ORIG_STY Neoplasm diagnosis PDQ
PRIMARY_PATH 10061332$10014712$10014713$10029104$Paraganglion neoplasm$Endocrine neoplasms malignant and unspecified NEC$Endocrine neoplasms malignant and unspecified$Neoplasms benign, malignant and unspecified (incl cysts and polyps) MDR
PRIMARY_SOC 10029104 MDR
PT_IN_VERSION 10.0 MDR
PT_IN_VERSION 10.1 MDR
PT_IN_VERSION 11.0 MDR
PT_IN_VERSION 11.1 MDR
PT_IN_VERSION 12.0 MDR
PT_IN_VERSION 12.1 MDR
PT_IN_VERSION 13.0 MDR
PT_IN_VERSION 13.1 MDR
PT_IN_VERSION 14.0 MDR
PT_IN_VERSION 14.1 MDR
PT_IN_VERSION 15.0 MDR
PT_IN_VERSION 15.1 MDR
PT_IN_VERSION 16.0 MDR
PT_IN_VERSION 16.1 MDR
PT_IN_VERSION 17.0 MDR
PT_IN_VERSION 17.1 MDR
PT_IN_VERSION 18.0 MDR
PT_IN_VERSION 18.1 MDR
PT_IN_VERSION 19.0 MDR
PT_IN_VERSION 19.1 MDR
PT_IN_VERSION 20.0 MDR
PT_IN_VERSION 20.1 MDR
PT_IN_VERSION 8.0 MDR
PT_IN_VERSION 8.1 MDR
PT_IN_VERSION 9.0 MDR
PT_IN_VERSION 9.1 MDR
RARE_DISEASE_URL http://rarediseases.info.nih.gov/gard/7324/paragangliomas-1/resources/1 GARD
REF HPO:skoehler HPO
RXAUI 3217854 RXNORM
RXAUI 3217855 RXNORM
RXAUI 3217860 RXNORM
RXAUI 5039783 RXNORM
RXAUI 5039785 RXNORM
RXAUI 5039786 RXNORM
RXCUI 1022619 RXNORM
SID HP:0002670 HPO
SID HP:0003004 HPO
SMQ_TERM_ADDVERSION 10.0 MDR
SMQ_TERM_ADDVERSION 16.1 MDR
SMQ_TERM_CAT A MDR
SMQ_TERM_LEVEL 4 MDR
SMQ_TERM_LEVEL 5 MDR
SMQ_TERM_LMVERSION 20.0 MDR
SMQ_TERM_SCOPE 2 MDR
SMQ_TERM_STATUS A MDR
SMQ_TERM_WEIGHT 0 MDR
SNOMED_CID 127027008 NDFRT
SNOMED_CID 253029009 NDFRT
SNOMED_CID 302833002 NDFRT
SUBSET_MEMBER 446608001~MAPTARGET~8680/1 SNOMEDCT_US
SUBSET_MEMBER 446608001~MAPTARGET~8683/0 SNOMEDCT_US
SUBSET_MEMBER 447562003~CORRELATIONID~447561005 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPADVICE~ALWAYS D13.2 | MAPPED FOLLOWING WHO GUIDANCE | POSSIBLE REQUIREMENT FOR MORPHOLOGY CODE SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPADVICE~ALWAYS D44.7 | POSSIBLE REQUIREMENT FOR MORPHOLOGY CODE SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPADVICE~MAP SOURCE CONCEPT CANNOT BE CLASSIFIED WITH AVAILABLE DATA SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPCATEGORYID~447637006 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPCATEGORYID~447638001 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPGROUP~1 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPPRIORITY~1 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPRULE~TRUE SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPTARGET~D13.2 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPTARGET~D44.7 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPTARGET~ SNOMEDCT_US
SUBSET_MEMBER 6011000124106~CORRELATIONID~447561005 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~ALWAYS D13.2 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~ALWAYS D44.7 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~ALWAYS D49.7 | DESCENDANTS NOT EXHAUSTIVELY MAPPED SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~IF GANGLIOCYTIC PARAGANGLIOMA CHOOSE D13.2 | MAP OF SOURCE CONCEPT IS CONTEXT DEPENDENT SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~IF HEREDITARY PHEOCHROMOCYTOMA AND PARAGANGLIOMA CHOOSE D35.00 | CONSIDER ADDITIONAL CODE TO IDENTIFY SPECIFIC CONDITION OR DISEASE | CONSIDER LATERALITY SPECIFICATION | MAP OF SOURCE CONCEPT IS CONTEXT DEPENDENT SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~IF HEREDITARY PHEOCHROMOCYTOMA AND PARAGANGLIOMA CHOOSE D44.7 | MAP OF SOURCE CONCEPT IS CONTEXT DEPENDENT SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~MAP SOURCE CONCEPT CANNOT BE CLASSIFIED WITH AVAILABLE DATA SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPCATEGORYID~447637006 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPCATEGORYID~447638001 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPCATEGORYID~447639009 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPGROUP~1 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPGROUP~2 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPPRIORITY~1 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPPRIORITY~2 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPPRIORITY~3 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~IFA 253029009 | Gangliocytic paraganglioma | SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~IFA 716857003 | Hereditary pheochromocytoma and paraganglioma | SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~OTHERWISE TRUE SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~TRUE SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~D13.2 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~D35.00 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~D44.7 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~D49.7 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~ SNOMEDCT_US
SUBSET_MEMBER 900000000000497000~MAPTARGET~BBDE. SNOMEDCT_US
SUBSET_MEMBER 900000000000497000~MAPTARGET~X77oB SNOMEDCT_US
SUBSET_MEMBER 900000000000497000~MAPTARGET~Xa99M SNOMEDCT_US
SUBSET_MEMBER 900000000000497000~MAPTARGET~XU6sB SNOMEDCT_US
SUBSET_MEMBER 900000000000497000~MAPTARGET~XULIb SNOMEDCT_US
SUBSET_MEMBER 900000000000508004~ACCEPTABILITYID~900000000000548007 SNOMEDCT_US
SUBSET_MEMBER 900000000000509007~ACCEPTABILITYID~900000000000548007 SNOMEDCT_US
TERM_LEVEL 4 ICDO
TERMUI T030259 MSH
TERMUI T030260 MSH
TERMUI T811807 MSH
TERMUI T811809 MSH
TERMUI T811810 MSH
TH GHR (2014) MSH
TH NLM (1966) MSH
TH NLM (1994) MSH
TH OMIM (2013) MSH
TH ORD (2010) MSH
TYPE_ID 900000000000003001 SNOMEDCT_US
TYPE_ID 900000000000013009 SNOMEDCT_US

Additional Concept Data:  (none)

URL to Bookmark: https://ncim-stage.nci.nih.gov/ncimbrowser/ConceptReport.jsp?dictionary=NCI Metathesaurus&code=C0030421

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