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Diffuse TGCT (also known as pigmented villonodular synovitis or PVNS) and
localized TGCT (also known as giant cell tumor of the tendon sheath or GCT-TS)
differ in tumor location, size, and characteristics.2

Click a tab below to learn about diffuse or localized TGCT symptoms and characteristics.

DIFFUSE TGCT
(PVNS)
LOCALIZED TGCT
(GCT-TS)
Symptoms and disease characteristics
Diffuse TGCT: 10% to 20% of all TGCT cases1,3
Diffused TGCT locations, shoulders, elbows, hips, knees, and ankles
Tumor locations
  • Mostly monoarticular
    and commonly seen
    in large joints4,5
  • The majority of
    tumors occur in
    the knee1,2,6
  • Can also occur in
    other joints, such as
    the hip, ankle, elbow,
    and shoulder2,7
The circles represent
common locations of
diffuse TGCT.2,7 The large
circle indicates the
joint affected most
commonly.1,2,6 Typically,
only one joint is
affected at a time.1
Symptoms
  • Common symptoms include pain, swelling,
    or limited motion in the joint; other symptoms
    such as locking and catching may occur2,8,9
  • Pain may be intermittent or sustained2,8,9
  • Tumors can interfere with joint function2,10,11
Lesion features
  • Diffuse tumors are usually large (>5 cm) and
    have poorly defined borders2,4
  • May be associated with degenerative joint
    disease and cystic lesions in adjacent bone2
  • May be intra-articular or extra-articular2
  • Extra-articular tumors may impinge and
    surround tendons and neurovascular
    structures,5 while intra-articular tumors
    can infiltrate adjacent soft tissue and
    erode bone4,12
Progression
  • Tumor development can progress slowly2
  • Patients often delay seeking medical
    consultation11,13
  • In one study, average delay in diagnosis was
    2.9 years (standard deviation: 3.7 years)6,*
  • After surgery, diffuse tumors recur up to 55%
    of the time3

Average delay in diagnosis in patients with TGCT, most of which
was diffuse, in a single-center retrospective study of 121 patients.6

Diffuse tenosynovial giant cell tumor (TGCT)
developing in a knee joint

TGCT developing in a knee joint
Working around the paina “If I know I’m going out Saturday night, I’m not going to go grocery shopping Saturday; I’m going grocery shopping on Sunday because [if I go shopping Saturday, then]
I can’t walk Saturday night.” —Woman with recurrent, diffuse TGCT in ankle

a The impact of TGCT is different for everyone; this information is not meant to be reflective of all patients with TGCT.

See where you fit in diagnosing TGCT. arrow-right
Symptoms and disease characteristics
Localized TGCT: 80% to 90% of all TGCT cases1,3
Localized TGCT locations, wrists, fingers, knees, and ankles.
Tumor locations
  • Mostly monoarticular
    and commonly seen
    in small joints2,7,14
  • The majority of
    tumors occur in
    the digits1,2
  • Can also occur in
    other joints, such as
    the wrist, ankle, foot,
    knee, elbow, or hip2
The circles represent
common locations of
localized TGCT.2 The
large circle indicates
the joint affected most
commonly.1,2 Typically,
only one joint is
affected at a time.1
Symptoms
  • Most common presenting symptom is
    painless swelling2
  • Pain may develop, especially with
    tumor growth15
  • Tumors can interfere with joint function11
Lesion features
  • Nodular, well circumscribed, at least partially
    encapsulated, and usually small2,16
  • Larger tumors can appear in large joints2
  • May be intra-articular or extra-articular6
  • Rarely, localized lesions lead to degenerative
    changes in the adjacent joint, exert pressure
    that causes erosion of nearby bone, or
    involve the skin2
Progression
  • Tumors can develop over a long period of time2
  • Patients may delay seeking medical treatment2,11
  • In one study, average delay before consulting
    a physician was 25 months (range: 2 months
    to 10 years) for TGCT of the finger/thumb and
    19 months (range: 3 to 48 months) for TGCT
    of the toe11,*
  • After surgery, localized tumors recur up to
    15% of the time3

In one retrospective analysis of 81 patients with TGCT, average
duration before patients with digit lesions consulted a physician.11

See where you fit in diagnosing TGCT. arrow-right

References

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  1. Mastboom MJL, Verspoor FGM, Verschoor AJ, et al; TGCT study group. Higher incidence rates than previously known in tenosynovial giant cell tumors: a nationwide study in The Netherlands. Acta Orthop. 2017;88(6):688-694. doi:10.1080/17453674.2017.1361126
  2. So-called fibrohistiocytic tumours. In: Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F, eds. WHO Classification of Tumours of Soft Tissue and Bone. 4th ed. International Agency for Research on Cancer; 2013:99-108. Bosman FT, Jaffe ES, Lakhani SR, Ohgaki H, eds. World Health Organization Classification of Tumours; vol 5.
  3. Ehrenstein V, Andersen SL, Qazi I, et al. Tenosynovial giant cell tumor: incidence, prevalence, patient characteristics, and recurrence. A registry-based cohort study in Denmark. J Rheumatol. 2017;44(10):1476-1483. doi:10.3899/jrheum.160816
  4. Lucas DR. Tenosynovial giant cell tumor: case report and review. Arch Pathol Lab Med. 2012;136(8):901-906. doi:10.5858/arpa.2012-0165-CR
  5. Ravi V, Wang W-L, Lewis VO. Treatment of tenosynovial giant cell tumor and pigmented villonodular synovitis. Curr Opin Oncol. 2011;23(4):361-366. doi:10.1097/CCO.0b013e328347e1e3
  6. Ottaviani S, Ayral X, Dougados M, Gossec L. Pigmented villonodular synovitis: a retrospective single-center study of 122 cases and review of the literature. Semin Arthritis Rheum. 2011;40(6):539-546. doi:10.1016/j.semarthrit.2010.07.005
  7. Brahmi M, Vinceneux A, Cassier PA. Current systemic treatment options for tenosynovial giant cell tumor/pigmented villonodular synovitis: targeting the CSF1/CSF1R axis. Curr Treat Options Oncol. Published online January 28, 2016. 2016;17(2):10. doi:10.1007/s11864-015-0385-x
  8. Gelhorn HL, Tong S, McQuarrie K, et al. Patient-reported symptoms of tenosynovial giant cell tumors. Clin Ther. Published online April 1, 2016. 2016;38(4):778-793. doi:10.1016/j.clinthera.2016.03.008
  9. Myers BW, Masi AT. Pigmented villonodular synovitis and tenosynovitis: a clinical epidemiologic study of 166 cases and literature review. Medicine (Baltimore). 1980;59(3):223-238.
  10. Verspoor FGM, van der Geest ICM, Vegt E, Veth RPH, van der Graaf WT, Schreuder HWB. Pigmented villonodular synovitis: current concepts about diagnosis and management. Future Oncol. 2013;9(10):1515-1531. doi:10.2217/fon.13.124
  11. Rao AS, Vigorita VJ. Pigmented villonodular synovitis (giant-cell tumor of the tendon sheath and synovial membrane): a review of eighty-one cases. J Bone Joint Surg Am. 1984;66(1):76-94.
  12. Asano N, Yoshida A, Kobayashi E, Yamaguchi T, Kawai A. Multiple metastases from histologically benign intraarticular diffuse-type tenosynovial giant cell tumor: a case report. Hum Pathol. 2014;45(11):2355-2358. http://dx.doi.org/10.1016/j.humpath.2014.06.025
  13. Shoji T, Yasunaga Y, Yamasaki T, et al. Transtrochanteric rotational osteotomy combined with intra-articular procedures for pigmented villonodular synovitis of the hip. J Orthop Sci. 2015;20(5):943-950. doi:10.1007/s00776-014-0563-x
  14. Hu Y, Kuang B, Chen Y, Shu J. Imaging features for diffuse-type tenosynovial giant cell tumor of the temporomandibular joint: a case report. Medicine. 2017;96(26):e7383. doi:10.1097/MD.0000000000007383
  15. Hsu CS, Hentz VR, Yao J. Tumours of the hand. Lancet Oncol. 2007;8(2):157-166. doi:10.1016/S1470-2045(07)70035-9
  16. Verspoor FGM, Zee AAG, Hannink G, van der Geest ICM, Veth RPH, Schreuder HWB. Long-term follow-up results of primary and recurrent pigmented villonodular synovitis. Rheumatology (Oxford). 2014;53(11):2063-2070. doi:10.1093/rheumatology/keu230