U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Tenosynovial giant cell tumor, diffuse type

MedGen UID:
11691
Concept ID:
C0039106
Neoplastic Process
Synonym: Pigmented villonodular synovitis
SNOMED CT: Tenosynovial giant cell tumor, diffuse (703703002); Pigmented villonodular synovitis (703703002); Diffuse tenosynovial giant cell tumor (1295265008); Pigmented villonodular synovitis (1295265008)
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

Naja A, Abboud A, Masrouha K, Ghert M, Saghieh S
Bull Hosp Jt Dis (2013) 2024 Sep;82(3):178-185. PMID: 39150871
Palmerini E, Staals EL
Curr Opin Oncol 2022 Jul 1;34(4):322-327. doi: 10.1097/CCO.0000000000000853. PMID: 35837703

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Soft Tissue Sarcoma, 2024

Recent clinical studies

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
Gounder MM, Thomas DM, Tap WD
J Clin Oncol 2018 Jan 10;36(2):202-209. Epub 2017 Dec 8 doi: 10.1200/JCO.2017.75.8482. PMID: 29220303Free PMC Article
Cannarile MA, Weisser M, Jacob W, Jegg AM, Ries CH, Rüttinger D
J Immunother Cancer 2017 Jul 18;5(1):53. doi: 10.1186/s40425-017-0257-y. PMID: 28716061Free PMC Article
Gelhorn HL, Tong S, McQuarrie K, Vernon C, Hanlon J, Maclaine G, Lenderking W, Ye X, Speck RM, Lackman RD, Bukata SV, Healey JH, Keedy VL, Anthony SP, Wagner AJ, Von Hoff DD, Singh AS, Becerra CR, Hsu HH, Lin PS, Tap WD
Clin Ther 2016 Apr;38(4):778-93. Epub 2016 Apr 1 doi: 10.1016/j.clinthera.2016.03.008. PMID: 27041409Free PMC Article
Ravi V, Wang WL, Lewis VO
Curr Opin Oncol 2011 Jul;23(4):361-6. doi: 10.1097/CCO.0b013e328347e1e3. PMID: 21577109

Prognosis

Wang DD, Luo HY, Guo CB, Meng JH
Int J Oral Maxillofac Surg 2020 Jul;49(7):882-888. Epub 2020 Jan 31 doi: 10.1016/j.ijom.2019.10.022. PMID: 32014315
Machado V, San-Julián M
Rev Esp Cir Ortop Traumatol (Engl Ed) 2020 May-Jun;64(3):199-205. Epub 2020 Jan 21 doi: 10.1016/j.recot.2019.12.001. PMID: 31980414
Gounder MM, Thomas DM, Tap WD
J Clin Oncol 2018 Jan 10;36(2):202-209. Epub 2017 Dec 8 doi: 10.1200/JCO.2017.75.8482. PMID: 29220303Free PMC Article
Ravi V, Wang WL, Lewis VO
Curr Opin Oncol 2011 Jul;23(4):361-6. doi: 10.1097/CCO.0b013e328347e1e3. PMID: 21577109
Tyler WK, Vidal AF, Williams RJ, Healey JH
J Am Acad Orthop Surg 2006 Jun;14(6):376-85. doi: 10.5435/00124635-200606000-00007. PMID: 16757677

Clinical prediction guides

Palmerini E, Healey JH, Bernthal NM, Bauer S, Schreuder H, Leithner A, Martin-Broto J, Gouin F, Lopez-Bastida J, Gelderblom H, Staals EL, Mercier F, Laeis P, Ye X, van de Sande M
Oncologist 2023 Jun 2;28(6):e425-e435. doi: 10.1093/oncolo/oyad011. PMID: 36869793Free PMC Article
Nguyen JC, Biko DM, Nguyen MK, Othman S, Weber KL, Ganley TJ, Arkader A
Pediatr Radiol 2021 Mar;51(3):441-449. Epub 2020 Nov 6 doi: 10.1007/s00247-020-04861-4. PMID: 33156428
Wang DD, Luo HY, Guo CB, Meng JH
Int J Oral Maxillofac Surg 2020 Jul;49(7):882-888. Epub 2020 Jan 31 doi: 10.1016/j.ijom.2019.10.022. PMID: 32014315
Machado V, San-Julián M
Rev Esp Cir Ortop Traumatol (Engl Ed) 2020 May-Jun;64(3):199-205. Epub 2020 Jan 21 doi: 10.1016/j.recot.2019.12.001. PMID: 31980414
Gounder MM, Thomas DM, Tap WD
J Clin Oncol 2018 Jan 10;36(2):202-209. Epub 2017 Dec 8 doi: 10.1200/JCO.2017.75.8482. PMID: 29220303Free PMC Article

Recent systematic reviews

Panciera A, Colangelo A, Di Martino A, Ferri R, Bulzacki Bogucki BD, Cecchin D, Brunello M, Benvenuti L, Digennaro V
Musculoskelet Surg 2024 Jun;108(2):145-152. Epub 2023 Jun 20 doi: 10.1007/s12306-023-00793-y. PMID: 37338752Free PMC Article
Levy DM, Haughom BD, Nho SJ, Gitelis S
Am J Orthop (Belle Mead NJ) 2016 Jan;45(1):23-8. PMID: 26761914
Safaee M, Oh T, Sun MZ, Parsa AT, McDermott MW, El-Sayed IH, Bloch O
Head Neck 2015 Aug;37(8):1213-24. Epub 2014 Jul 11 doi: 10.1002/hed.23717. PMID: 24764167
Jennings F, Lambert E, Fredericson M
Sports Med 2008;38(11):917-30. doi: 10.2165/00007256-200838110-00003. PMID: 18937522

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Soft Tissue Sarcoma, 2024

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...