Difference between revisions of "Diffuse tenosynovial giant-cell tumour"

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{{ Infobox diagnosis
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Name      = {{PAGENAME}}
| Image      =  
| Image      = Pigmented villonodular synovitis high mag.jpg
| Width      =
| Width      =
| Caption    =  
| Caption    = Diffuse tenosynovial giant-cell tumour. [[H&E stain]].
| Synonyms  =
| Synonyms  = pigmented villonodular synovitis (PVNS) - old term
| Micro      = nodules composed of cells with abundant cytoplasm & pale nuclei, multinucleated giant cells, hemosiderin-laden macrophages, foam cells
| Micro      = nodules composed of cells with abundant cytoplasm & pale nuclei, multinucleated giant cells, hemosiderin-laden macrophages, foam cells
| Subtypes  =
| Subtypes  = generalized, localized (articular, extra-articular)
| LMDDx      = [[giant cell lesions]], others
| LMDDx      = [[giant cell lesions]], others
| Stains    =
| Stains    =
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| Molecular  =
| Molecular  =
| IF        =
| IF        =
| Gross      = pigmented
| Gross      = pigmented, articular or extra-articular
| Grossing  =
| Grossing  =
| Site      = large [[joints]] - esp. knee or hip; occ. extra-articular
| Site      = large [[joints]] - esp. knee or hip; occ. extra-articular
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| Signs      =
| Signs      =
| Symptoms  =
| Symptoms  =
| Prevalence =  
| Prevalence = uncommon
| Bloodwork  =
| Bloodwork  =
| Rads      =
| Rads      =
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==General==
==General==
*Course: benign.
*Usually benign.
**Occasionally malignant.<ref name=pmid22827766>{{Cite journal  | last1 = Kondo | first1 = R. | last2 = Akiba | first2 = J. | last3 = Hiraoka | first3 = K. | last4 = Hisaoka | first4 = M. | last5 = Hashimoto | first5 = H. | last6 = Kage | first6 = M. | last7 = Yano | first7 = H. | title = Malignant diffuse-type tenosynovial giant cell tumor of the buttock. | journal = Pathol Int | volume = 62 | issue = 8 | pages = 559-64 | month = Aug | year = 2012 | doi = 10.1111/j.1440-1827.2012.02838.x | PMID = 22827766 }}</ref><ref name=pmid18301053>{{Cite journal  | last1 = Li | first1 = CF. | last2 = Wang | first2 = JW. | last3 = Huang | first3 = WW. | last4 = Hou | first4 = CC. | last5 = Chou | first5 = SC. | last6 = Eng | first6 = HL. | last7 = Lin | first7 = CN. | last8 = Yu | first8 = SC. | last9 = Huang | first9 = HY. | title = Malignant diffuse-type tenosynovial giant cell tumors: a series of 7 cases comparing with 24 benign lesions with review of the literature. | journal = Am J Surg Pathol | volume = 32 | issue = 4 | pages = 587-99 | month = Apr | year = 2008 | doi = 10.1097/PAS.0b013e318158428f | PMID = 18301053 }}</ref><ref name=pmid10757395>{{Cite journal  | last1 = Somerhausen | first1 = NS. | last2 = Fletcher | first2 = CD. | title = Diffuse-type giant cell tumor: clinicopathologic and immunohistochemical analysis of 50 cases with extraarticular disease. | journal = Am J Surg Pathol | volume = 24 | issue = 4 | pages = 479-92 | month = Apr | year = 2000 | doi =  | PMID = 10757395 }}</ref>
**Occasionally malignant.<ref name=pmid22827766>{{Cite journal  | last1 = Kondo | first1 = R. | last2 = Akiba | first2 = J. | last3 = Hiraoka | first3 = K. | last4 = Hisaoka | first4 = M. | last5 = Hashimoto | first5 = H. | last6 = Kage | first6 = M. | last7 = Yano | first7 = H. | title = Malignant diffuse-type tenosynovial giant cell tumor of the buttock. | journal = Pathol Int | volume = 62 | issue = 8 | pages = 559-64 | month = Aug | year = 2012 | doi = 10.1111/j.1440-1827.2012.02838.x | PMID = 22827766 }}</ref><ref name=pmid18301053>{{Cite journal  | last1 = Li | first1 = CF. | last2 = Wang | first2 = JW. | last3 = Huang | first3 = WW. | last4 = Hou | first4 = CC. | last5 = Chou | first5 = SC. | last6 = Eng | first6 = HL. | last7 = Lin | first7 = CN. | last8 = Yu | first8 = SC. | last9 = Huang | first9 = HY. | title = Malignant diffuse-type tenosynovial giant cell tumors: a series of 7 cases comparing with 24 benign lesions with review of the literature. | journal = Am J Surg Pathol | volume = 32 | issue = 4 | pages = 587-99 | month = Apr | year = 2008 | doi = 10.1097/PAS.0b013e318158428f | PMID = 18301053 }}</ref><ref name=pmid10757395>{{Cite journal  | last1 = Somerhausen | first1 = NS. | last2 = Fletcher | first2 = CD. | title = Diffuse-type giant cell tumor: clinicopathologic and immunohistochemical analysis of 50 cases with extraarticular disease. | journal = Am J Surg Pathol | volume = 24 | issue = 4 | pages = 479-92 | month = Apr | year = 2000 | doi =  | PMID = 10757395 }}</ref>
*''[[Giant cell tumour of the tendon sheath]]'' is considered to be the soft-tissue counterpart of PVNS.<ref>URL: [http://emedicine.medscape.com/article/1253223-overview http://emedicine.medscape.com/article/1253223-overview]. Accessed on: 6 January 2011.</ref>
*Can be thought of as the large joint version of [[giant cell tumour of the tendon sheath]].<ref name=Ref_DCHH341>{{Ref DCHH|341}}</ref>
 
===Classification===
Subclassified - clinical:<ref name=pmid10725067>{{Cite journal  | last1 = Perka | first1 = C. | last2 = Labs | first2 = K. | last3 = Zippel | first3 = H. | last4 = Buttgereit | first4 = F. | title = Localized pigmented villonodular synovitis of the knee joint: neoplasm or reactive granuloma? A review of 18 cases. | journal = Rheumatology (Oxford) | volume = 39 | issue = 2 | pages = 172-8 | month = Feb | year = 2000 | doi =  | PMID = 10725067 }}
</ref>
*Generalized PVNS.
*Localized PVNS.<ref name=pmid12876042>{{Cite journal  | last1 = Huang | first1 = GS. | last2 = Lee | first2 = CH. | last3 = Chan | first3 = WP. | last4 = Chen | first4 = CY. | last5 = Yu | first5 = JS. | last6 = Resnick | first6 = D. | title = Localized nodular synovitis of the knee: MR imaging appearance and clinical correlates in 21 patients. | journal = AJR Am J Roentgenol | volume = 181 | issue = 2 | pages = 539-43 | month = Aug | year = 2003 | doi = 10.2214/ajr.181.2.1810539 | PMID = 12876042 }}</ref>
**Articular.
**Extra-articular.


==Gross==
==Gross==
*Typically knee or hip.<ref name=pmid10524485>{{Cite journal  | last1 = Frassica | first1 = FJ. | last2 = Bhimani | first2 = MA. | last3 = McCarthy | first3 = EF. | last4 = Wenz | first4 = J. | title = Pigmented villonodular synovitis of the hip and knee. | journal = Am Fam Physician | volume = 60 | issue = 5 | pages = 1404-10; discussion 1415 | month = Oct | year = 1999 | doi =  | PMID = 10524485 | URL = http://www.aafp.org/afp/1999/1001/p1404.html }}</ref>
*Typically knee or hip.<ref name=pmid10524485>{{Cite journal  | last1 = Frassica | first1 = FJ. | last2 = Bhimani | first2 = MA. | last3 = McCarthy | first3 = EF. | last4 = Wenz | first4 = J. | title = Pigmented villonodular synovitis of the hip and knee. | journal = Am Fam Physician | volume = 60 | issue = 5 | pages = 1404-10; discussion 1415 | month = Oct | year = 1999 | doi =  | PMID = 10524485 | URL = http://www.aafp.org/afp/1999/1001/p1404.html }}</ref>
*May be extra-articular.<ref name=pmid10757395/>
*May be extra-articular.<ref name=pmid10757395/>
Note:
*Localized form - classically fat pad inferior to patella.<ref name=pmid23798766/>


==Microscopic==
==Microscopic==
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*[http://path.upmc.edu/cases/case251/micro.html PVNS - several images (upmc.edu)].
*[http://path.upmc.edu/cases/case251/micro.html PVNS - several images (upmc.edu)].
*[http://www.webpathology.com/image.asp?case=357&n=2 PVNS (webpathology.com)].
*[http://www.webpathology.com/image.asp?case=357&n=2 PVNS (webpathology.com)].
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3687912/figure/F4/ Localized nodular synovitis (nih.gov)].<ref name=pmid23798766>{{Cite journal  | last1 = Park | first1 = JH. | last2 = Ro | first2 = KH. | last3 = Lee | first3 = DH. | title = Localized nodular synovitis of the infrapatellar fat pad. | journal = Indian J Orthop | volume = 47 | issue = 3 | pages = 313-6 | month = May | year = 2013 | doi = 10.4103/0019-5413.111514 | PMID = 23798766 }}</ref>
==IHC==
*May be desmin positive.<ref name=pmid9796719>{{Cite journal  | last1 = Folpe | first1 = AL. | last2 = Weiss | first2 = SW. | last3 = Fletcher | first3 = CD. | last4 = Gown | first4 = AM. | title = Tenosynovial giant cell tumors: evidence for a desmin-positive dendritic cell subpopulation. | journal = Mod Pathol | volume = 11 | issue = 10 | pages = 939-44 | month = Oct | year = 1998 | doi =  | PMID = 9796719 }}</ref>
**May lead one to think [[rhabdomyosarcoma]].


==Molecular==
==Molecular==
*Clonal - overexpresses CSF1.<ref name=pmid22849738>{{Cite journal  | last1 = Lucas | first1 = DR. | title = Tenosynovial giant cell tumor: case report and review. | journal = Arch Pathol Lab Med | volume = 136 | issue = 8 | pages = 901-6 | month = Aug | year = 2012 | doi = 10.5858/arpa.2012-0165-CR | PMID = 22849738 }}</ref>
*Clonal - overexpresses CSF1.<ref name=pmid22849738>{{Cite journal  | last1 = Lucas | first1 = DR. | title = Tenosynovial giant cell tumor: case report and review. | journal = Arch Pathol Lab Med | volume = 136 | issue = 8 | pages = 901-6 | month = Aug | year = 2012 | doi = 10.5858/arpa.2012-0165-CR | PMID = 22849738 }}</ref>
**Possible treatment with CSF1-R inhibitor Pexidartinib.<ref>{{Cite journal  | last1 = Tap | first1 = WD. | last2 = Gelderblom | first2 = H. | last3 = Palmerini | first3 = E. | last4 = Desai | first4 = J. | last5 = Bauer | first5 = S. | last6 = Blay | first6 = JY. | last7 = Alcindor | first7 = T. | last8 = Ganjoo | first8 = K. | last9 = Martín-Broto | first9 = J. | title = Pexidartinib versus placebo for advanced tenosynovial giant cell tumour (ENLIVEN): a randomised phase 3 trial. | journal = Lancet | volume = 394 | issue = 10197 | pages = 478-487 | month = 08 | year = 2019 | doi = 10.1016/S0140-6736(19)30764-0 | PMID = 31229240 }}</ref>


==Sign out==
==Sign out==

Latest revision as of 09:54, 10 October 2019

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]

General

Classification

Subclassified - clinical:[6]

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

Gross

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

Note:

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

Microscopic

Features:[10]

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

DDx - general for the site:[11]

Images

  • PVNS - low mag. (WC)

  • PVNS - high mag. (WC)

www:

IHC

Molecular

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

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RIGHT FEMORAL HEAD AND JOINT CAPSULE, EXCISION:
- DEGENERATIVE JOINT DISEASE.
- DIFFUSE TENOSYNOVIAL GIANT-CELL TUMOUR (PIGMENTED VILLONODULAR SYNOVITIS).

Micro

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

References

  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.
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