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Tenosynovial giant cell tumor, diffuse type

MedGen UID:
11691
Concept ID:
C0039106
Neoplastic Process
Synonym: Pigmented villonodular synovitis
SNOMED CT: Pigmented villonodular synovitis (95412009); Chronic hemorrhagic villous synovitis (95412009); Tenosynovial giant cell tumor, diffuse (703703002); Pigmented villonodular synovitis (703703002); PVNS - Pigmented villonodular synovitis (95412009)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0024686
Orphanet: ORPHA66627

Definition

A rare benign proliferative disorder of the synovial membrane primarily affecting young adults (with a peak age of onset in the second to fourth decade of life) characterized by proliferative, locally invasive tumor-like lesions, usually involving a single joint, tendon sheath or bursa (most commonly the joints of the knee and hip and rarely others such as the ankle, shoulder and temporomandibular joints). It presents with pain and limitation of motion along with swelling, heat and tenderness over the involved joint, eventually leading to arthritic degeneration and significant locomotor deficit, if left untreated. PVNS can recur in patients even after treatment. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVTenosynovial giant cell tumor, diffuse type
Follow this link to review classifications for Tenosynovial giant cell tumor, diffuse type in Orphanet.

Professional guidelines

PubMed

Palmerini E, Staals EL
Curr Opin Oncol 2022 Jul 1;34(4):322-327. doi: 10.1097/CCO.0000000000000853. PMID: 35837703

Recent clinical studies

Etiology

Helming A, Hansford B, Beckett B
Skeletal Radiol 2022 May;51(5):1085-1091. Epub 2021 Sep 29 doi: 10.1007/s00256-021-03924-3. PMID: 34586485

Diagnosis

Helming A, Hansford B, Beckett B
Skeletal Radiol 2022 May;51(5):1085-1091. Epub 2021 Sep 29 doi: 10.1007/s00256-021-03924-3. PMID: 34586485
Hamza A, Gidley PW, Learned KO, Hanna EY, Bell D
Head Neck 2020 Aug;42(8):1859-1873. Epub 2020 Feb 10 doi: 10.1002/hed.26106. PMID: 32040228
Harvey JP, Suster DI, Raskin KA, Nielsen GP, Bredella MA
Skeletal Radiol 2019 Jun;48(6):965-969. Epub 2018 Sep 11 doi: 10.1007/s00256-018-3065-7. PMID: 30206676
Kimura T, Nishisho T, Sakai T, Miyagi R, Takao S, Iwamoto S, Higashino K, Takata Y, Goda Y, Toki S, Sairyo K
Spine (Phila Pa 1976) 2015 Jun 15;40(12):E735-9. doi: 10.1097/BRS.0000000000000923. PMID: 26067150
Horvath RR, Bostanche J, Altman MI
J Am Podiatr Med Assoc 1993 Apr;83(4):231-3. doi: 10.7547/87507315-83-4-231. PMID: 8386232

Therapy

Helming A, Hansford B, Beckett B
Skeletal Radiol 2022 May;51(5):1085-1091. Epub 2021 Sep 29 doi: 10.1007/s00256-021-03924-3. PMID: 34586485

Prognosis

Kimura T, Nishisho T, Sakai T, Miyagi R, Takao S, Iwamoto S, Higashino K, Takata Y, Goda Y, Toki S, Sairyo K
Spine (Phila Pa 1976) 2015 Jun 15;40(12):E735-9. doi: 10.1097/BRS.0000000000000923. PMID: 26067150

Clinical prediction guides

Kimura T, Nishisho T, Sakai T, Miyagi R, Takao S, Iwamoto S, Higashino K, Takata Y, Goda Y, Toki S, Sairyo K
Spine (Phila Pa 1976) 2015 Jun 15;40(12):E735-9. doi: 10.1097/BRS.0000000000000923. PMID: 26067150

Supplemental Content

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