Diffuse tenosynovial giant-cell tumour

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Diffuse tenosynovial giant-cell tumour
Diagnosis in short

Diffuse tenosynovial giant-cell tumour. H&E stain.

Synonyms pigmented villonodular synovitis (PVNS) - old term

LM nodules composed of cells with abundant cytoplasm & pale nuclei, multinucleated giant cells, hemosiderin-laden macrophages, foam cells
Subtypes generalized, localized (articular, extra-articular)
LM DDx giant cell lesions, others
Gross pigmented, articular or extra-articular
Site large joints - esp. knee or hip; occ. extra-articular

Prevalence uncommon
Prognosis usually benign

Diffuse tenosynovial giant-cell tumour is relatively common mostly benign chondro-osseous tumour of the large joints.

It is also known as tenosynovial giant-cell tumour, diffuse type. Previously, it was known as pigmented villonodular synovitis, abbreviated PVNS.[1]



Subclassified - clinical:[6]

  • Generalized PVNS.
  • Localized PVNS.[7]
    • Articular.
    • Extra-articular.


  • Typically knee or hip.[8]
  • May be extra-articular.[4]


  • Localized form - classically fat pad inferior to patella.[9]



  • Subsynovial nodules composed of cells with:
    • Abundant cytoplasm.
    • Pale nuclei.
  • Multinucleated giant cells.
  • Hemosiderin-laden macrophages.
  • Foam cells.

DDx - general for the site:[11]





  • Clonal - overexpresses CSF1.[13]
    • Possible treatment with CSF1-R inhibitor Pexidartinib.[14]

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The soft tissue sections show nodules with abundant hemosiderin-laden macrophages and multinucleated giant cells. Nuclear atypia is not identified. Mitotic activity is not apparent.

See also


  1. Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 1247. ISBN 978-1416031215.
  2. Kondo, R.; Akiba, J.; Hiraoka, K.; Hisaoka, M.; Hashimoto, H.; Kage, M.; Yano, H. (Aug 2012). "Malignant diffuse-type tenosynovial giant cell tumor of the buttock.". Pathol Int 62 (8): 559-64. doi:10.1111/j.1440-1827.2012.02838.x. PMID 22827766.
  3. Li, CF.; Wang, JW.; Huang, WW.; Hou, CC.; Chou, SC.; Eng, HL.; Lin, CN.; Yu, SC. et al. (Apr 2008). "Malignant diffuse-type tenosynovial giant cell tumors: a series of 7 cases comparing with 24 benign lesions with review of the literature.". Am J Surg Pathol 32 (4): 587-99. doi:10.1097/PAS.0b013e318158428f. PMID 18301053.
  4. 4.0 4.1 Somerhausen, NS.; Fletcher, CD. (Apr 2000). "Diffuse-type giant cell tumor: clinicopathologic and immunohistochemical analysis of 50 cases with extraarticular disease.". Am J Surg Pathol 24 (4): 479-92. PMID 10757395.
  5. Tadrous, Paul.J. Diagnostic Criteria Handbook in Histopathology: A Surgical Pathology Vade Mecum (1st ed.). Wiley. pp. 341. ISBN 978-0470519035.
  6. Perka, C.; Labs, K.; Zippel, H.; Buttgereit, F. (Feb 2000). "Localized pigmented villonodular synovitis of the knee joint: neoplasm or reactive granuloma? A review of 18 cases.". Rheumatology (Oxford) 39 (2): 172-8. PMID 10725067.
  7. Huang, GS.; Lee, CH.; Chan, WP.; Chen, CY.; Yu, JS.; Resnick, D. (Aug 2003). "Localized nodular synovitis of the knee: MR imaging appearance and clinical correlates in 21 patients.". AJR Am J Roentgenol 181 (2): 539-43. doi:10.2214/ajr.181.2.1810539. PMID 12876042.
  8. Frassica, FJ.; Bhimani, MA.; McCarthy, EF.; Wenz, J. (Oct 1999). "Pigmented villonodular synovitis of the hip and knee.". Am Fam Physician 60 (5): 1404-10; discussion 1415. PMID 10524485.
  9. 9.0 9.1 Park, JH.; Ro, KH.; Lee, DH. (May 2013). "Localized nodular synovitis of the infrapatellar fat pad.". Indian J Orthop 47 (3): 313-6. doi:10.4103/0019-5413.111514. PMID 23798766.
  10. URL: http://www.wheelessonline.com/ortho/pigmented_villonodular_synovitis.
  11. Krenn, V.; Morawietz, L.; König, A.; Haeupl, T. (Nov 2006). "[Differential diagnosis of chronic synovitis].". Pathologe 27 (6): 402-8. doi:10.1007/s00292-006-0866-6. PMID 17031677.
  12. Folpe, AL.; Weiss, SW.; Fletcher, CD.; Gown, AM. (Oct 1998). "Tenosynovial giant cell tumors: evidence for a desmin-positive dendritic cell subpopulation.". Mod Pathol 11 (10): 939-44. PMID 9796719.
  13. Lucas, DR. (Aug 2012). "Tenosynovial giant cell tumor: case report and review.". Arch Pathol Lab Med 136 (8): 901-6. doi:10.5858/arpa.2012-0165-CR. PMID 22849738.
  14. Tap, WD.; Gelderblom, H.; Palmerini, E.; Desai, J.; Bauer, S.; Blay, JY.; Alcindor, T.; Ganjoo, K. et al. (08 2019). "Pexidartinib versus placebo for advanced tenosynovial giant cell tumour (ENLIVEN): a randomised phase 3 trial.". Lancet 394 (10197): 478-487. doi:10.1016/S0140-6736(19)30764-0. PMID 31229240.