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Tenosynovial Giant Cell Tumor, Diffuse Type (CUI C0039106) Suggest changes to this concept
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Terms & Properties

Concept Unique Identifier (CUI): C0039106

NCI Thesaurus Code: C3401  (see NCI Thesaurus info)

Semantic Type: Neoplastic Process

NCIt Definition: A locally aggressive, diffusely infiltrating tumor, arising in the tendon sheath. It is composed of synovial-like mononuclear cells, hemosiderin-laden macrophages, foam cells, and inflammatory cells. Multinucleated osteoclast-like giant cells are usually present, although in a minority of cases they may be absent or rare. It predominantly affects young adults. Symptoms include joint swelling, pain, and joint effusion.

RADLEX Definition: Outgrowths of synovial membrane composed of villi and fibrous nodules characterized histologically by hemosiderin- and lipid-containing macrophages and multinucleated giant cells. It usually occurs in the knee. [MeSH]

GARD Definition: Pigmented villonodular synovitis is a disease in which the tissue lining a joint in the body begins to grow abnormally.  There are two types of pigmented villonodular synovitis: the nodular form (where the abnormal tissue growth forms a single bump) and the diffuse form (where the entire lining of the joint grows unusually).  The additional tissue in the joint can cause pain, limit movements or cause the joint to lock, and in some cases, can destroy the normal joint structure.  The knee is most commonly affected by this condition, though it can occur in other joints such as the hip, shoulder, elbow, ankle, or wrist.  The average age of diagnosis for this condition is 35 years. - this information is from GARD/ORDR/NCATS.

MSH Definition: Diffuse outgrowth arising from the SYNOVIAL MEMBRANE; SYNOVIAL BURSA; or TENDON sheath around the joint cavity, with extension to surrounding soft tissue. It is characterized by pigmented HEMOSIDERIN-containing MACROPHAGES; FOAM CELLS; and multinucleated GIANT CELLS. It usually occurs in the hands and feet, and around large joints, such as in the ankle and knee joints.

Synonyms & Abbreviations: (see Synonym Details)
Chronic haemorrhagic villous synovitis
Chronic hemorrhagic villous synovitis
Diffuse Giant Cell Neoplasm of Tendon Sheath
Diffuse Giant Cell Neoplasm of Tenosynovium
Diffuse Giant Cell Neoplasm of the Tenosynovium
Diffuse Giant Cell Tumor of Tendon Sheath
Diffuse Giant Cell Tumor of Tenosynovium
Diffuse Giant Cell Tumor of the Tenosynovium
Diffuse pigmented villonodular synovitis
Diffuse Tenosynovial Giant Cell Neoplasm
Diffuse Tenosynovial Giant Cell Tumor
Diffuse-type giant cell tumor
Pigmented Villonodular Synovitides
Pigmented villonodular synovitis (disorder)
Pigmented villonodular synovitis
Pigmentierte villonoduläre Synovitis
PVNS - Pigmented villonodular synovitis
PVNS
Synovitides, Pigmented Villonodular
Synovitis, Pigmented Villonodular [Disease/Finding]
Synovitis, Pigmented Villonodular
SYNOVITIS, VILLONODULAR, PIGMENTED
Tenosynovial giant cell tumor, diffuse (morphologic abnormality)
Tenosynovial Giant Cell Tumor, Diffuse Type
Tenosynovial giant cell tumor, diffuse
Tenosynovial giant cell tumour, diffuse
Villonodular Synovitides, Pigmented
Villonodular synovitis (pigmented), unspecified site
Villonodular synovitis (pigmented); Site unspecified
Villonodular synovitis (pigmented)
Villonodular Synovitis, Pigmented

External Source Codes: 
NCI Thesaurus Code C3401 (see NCI Thesaurus info)

Other Properties: Property Definitions
Name Value Source
ACTIVE 1 SNOMEDCT_US
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 SE SU TH UL UR VE VI MSH
CASE_SIGNIFICANCE_ID 900000000000017005 SNOMEDCT_US
CASE_SIGNIFICANCE_ID 900000000000448009 SNOMEDCT_US
CHARACTERISTIC_TYPE_ID 900000000000011006 SNOMEDCT_US
CTV3ID N092. SNOMEDCT_US
CTV3ID XUnKM SNOMEDCT_US
DATE_CREATED 2013-01-09T00:04:00 GARD
DATE_LAST_MODIFIED 10/09/2014 RADLEX
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 5412 GARD
DX 19910101 MSH
EFFECTIVE_TIME 20140731 SNOMEDCT_US
HAS_GARD_PAGE true GARD
HN 91(78); was see under SYNOVITIS 1978-90 MSH
IAN DEFAULT ICD10
ICD-O-3_CODE 9252/1 SNOMEDCT_US
ICD-O-3_Code 9251/0 NCI
IDENTIFIER_TYPE_ID 1 GARD
IS_ACTIVE true GARD
IS_RARE true GARD
IS_SPANISH false GARD
LAST_CHANGES_TO_CLASS syn RADLEX
MDA 19770301 MSH
MESH_DEFINITION Outgrowths of synovial membrane composed of villi and fibrous nodules characterized histologically by hemosiderin- and lipid-containing macrophages and multinucleated giant cells. It usually occurs in the knee. NDFRT
MESH_DUI D013586 NDFRT
MESH_NAME Synovitis, Pigmented Villonodular NDFRT
MESH_UI M0020965 NDFRT
MMR 20160208 MSH
MN C04.557.450.565.380.690.500 MSH
MN C05.550.870.445.500 MSH
MN C05.651.869.762.500 MSH
MODIFIER_ID 900000000000451002 SNOMEDCT_US
NDFRT_KIND DISEASE_KIND NDFRT
Neoplastic_Status Undetermined NCI
NUI N0000002880 NDFRT
ORDER_NO 16450 ICD10CM
ORDER_NO 16451 ICD10CM
PM 91; was see under SYNOVITIS 1978-90 MSH
RARE_DISEASE_URL http://rarediseases.info.nih.gov/gard/7396/pigmented-villonodular-synovitis/resources/1 GARD
RXAUI 3242919 RXNORM
RXAUI 3242920 RXNORM
RXCUI 1025078 RXNORM
SUBSET_MEMBER 446608001~MAPTARGET~9252/1 SNOMEDCT_US
SUBSET_MEMBER 447562003~CORRELATIONID~447561005 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPADVICE~ALWAYS M12.29 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPCATEGORYID~447637006 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~M12.29 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~CORRELATIONID~447561005 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~ALWAYS M12.20 | DESCENDANTS NOT EXHAUSTIVELY MAPPED SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPCATEGORYID~447637006 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPGROUP~1 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPPRIORITY~1 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~TRUE SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~M12.20 SNOMEDCT_US
SUBSET_MEMBER 900000000000497000~MAPTARGET~N092. SNOMEDCT_US
SUBSET_MEMBER 900000000000497000~MAPTARGET~XUnKM SNOMEDCT_US
SUBSET_MEMBER 900000000000508004~ACCEPTABILITYID~900000000000548007 SNOMEDCT_US
SUBSET_MEMBER 900000000000508004~ACCEPTABILITYID~900000000000549004 SNOMEDCT_US
SUBSET_MEMBER 900000000000509007~ACCEPTABILITYID~900000000000548007 SNOMEDCT_US
SUBSET_MEMBER 900000000000509007~ACCEPTABILITYID~900000000000549004 SNOMEDCT_US
TERMUI T000891250 MSH
TERMUI T039926 MSH
TH NLM (1978) MSH
TH NLM (2017) 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=C0039106

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