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{{ Infobox diagnosis | |||
| Name = {{PAGENAME}} | |||
| Image = Pigmented villonodular synovitis high mag.jpg | |||
| Width = | |||
| Caption = Diffuse tenosynovial giant-cell tumour. [[H&E stain]]. | |||
| 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 | |||
| Subtypes = generalized, localized (articular, extra-articular) | |||
| LMDDx = [[giant cell lesions]], others | |||
| Stains = | |||
| IHC = | |||
| EM = | |||
| Molecular = | |||
| IF = | |||
| Gross = pigmented, articular or extra-articular | |||
| Grossing = | |||
| Site = large [[joints]] - esp. knee or hip; occ. extra-articular | |||
| Assdx = | |||
| Syndromes = | |||
| Clinicalhx = | |||
| Signs = | |||
| Symptoms = | |||
| Prevalence = uncommon | |||
| Bloodwork = | |||
| Rads = | |||
| Endoscopy = | |||
| Prognosis = usually benign | |||
| Other = | |||
| ClinDDx = | |||
| Tx = | |||
}} | |||
'''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'''.<ref>{{Ref PBoD8|1247}}</ref> | |||
==General== | |||
*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> | |||
*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== | |||
*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/> | |||
Note: | |||
*Localized form - classically fat pad inferior to patella.<ref name=pmid23798766/> | |||
==Microscopic== | |||
Features:<ref>URL: [http://www.wheelessonline.com/ortho/pigmented_villonodular_synovitis http://www.wheelessonline.com/ortho/pigmented_villonodular_synovitis].</ref> | |||
*Subsynovial nodules composed of cells with: | |||
**Abundant cytoplasm. | |||
**Pale nuclei. | |||
*Multinucleated giant cells. | |||
*Hemosiderin-laden macrophages. | |||
*Foam cells. | |||
DDx - general for the site:<ref name=pmid17031677>{{Cite journal | last1 = Krenn | first1 = V. | last2 = Morawietz | first2 = L. | last3 = König | first3 = A. | last4 = Haeupl | first4 = T. | title = [Differential diagnosis of chronic synovitis]. | journal = Pathologe | volume = 27 | issue = 6 | pages = 402-8 | month = Nov | year = 2006 | doi = 10.1007/s00292-006-0866-6 | PMID = 17031677 }}</ref> | |||
*[[Synovial chondromatosis]]. | |||
*[[Gout]]. | |||
*[[Pseudogout]]. | |||
*[[Storage disorders]]. | |||
*[[Granuloma|Granulomatous inflammation]]. | |||
*Degenerative changes ([[osteoarthritis]]). | |||
*[[Rheumatic joint disease|Rheumatic disease]]. | |||
===Images=== | |||
<gallery> | |||
Image:Pigmented_villonodular_synovitis_low_mag.jpg | PVNS - low mag. (WC) | |||
Image:Pigmented_villonodular_synovitis_high_mag.jpg | PVNS - high mag. (WC) | |||
</gallery> | |||
www: | |||
*[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.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== | |||
*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== | |||
<pre> | |||
RIGHT FEMORAL HEAD AND JOINT CAPSULE, EXCISION: | |||
- DEGENERATIVE JOINT DISEASE. | |||
- DIFFUSE TENOSYNOVIAL GIANT-CELL TUMOUR (PIGMENTED VILLONODULAR SYNOVITIS). | |||
</pre> | |||
===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== | |||
*[[Chondro-osseous tumours]]. | |||
*[[Giant cell tumour of the tendon sheath]]. | |||
==References== | |||
{{Reflist|2}} | |||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
[[Category:Chondro-osseous tumours]] |