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Tenosynovial Giant Cell Tumor

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Other Names

  • Pigmented Villonodular Synovitis
  • Tenosynovial Giant Cell Tumor

Background

  • This page refers to Tenosynovial Giant Cell Tumor (TGCT), sometimes referred to as Pigmented Villonodular Synovitis

History

  • In 1852 a lesion in the flexor tendon sheath of a finger was described as “Cancer de la gaine des tendons”.[1]
  • In 1941 the current terminology of “pigmented villonodular synovitis,” was established [2]
  • Later the subtypes of localized and diffuse were defined and currently recognized.

Epidemiology

  • Very rare pathology incidence, 1/1,800,000, Localized >Diffuse [3]
  • Localized form presents more commonly in 4th or 5th decade (30-50).[4]
  • Diffuse form presents a little earlier (<40 years). [4]

Pathophysiology

  • General
    • Combination of proliferative process of synovial tissue with hemosiderin deposition
    • Results from the proliferation of stromal cells, accumulation of mononuclear cells and multinuclear giant cells[5]
    • Note, these are fully differentiated osteoclasts
    • Historically considered to be an inflammatory process, more recent evidence in the literature to suggest a neoplastic origin [6]

Terminology

  • Tenosynovial Giant Cell Tumor vs Pigmented Villonodular Synovitis
    • The histological similarities were noted and terminology between benign soft-tissue tumor of tendon sheaths and pigmented villonodular synovitis (PVNS) were unified in 2007. [7]
    • Currently, localized pigmented villonodular synovitis and tenosynovial giant cell tumor (TGCT) are interchangeably used in the literature. [6]

Risk Factors

  • Females (2:1)
    • Localized type: predominance is slight in diffuse type[4]

Differential Diagnosis

  • Rheumatoid Arthritis
  • Synovial sarcoma
  • Scarring/capsulitis
  • Synovial chondromatosis
  • Lipoma arborescens
  • Joint effusion

Clinical Features

  • History
    • Localized type
      • Most commonly presents in the hands (85% of cases)
      • Few cases described within the knee, ankle or rarely other large joints[4]
    • Diffuse type
      • Intraarticular
      • Knee 75% of cases, then hip, ankle, shoulder
      • Rarely elbow, temporomandibular, spinal inter-apophyseal joints[4]
  • Physical Exam
    • No pathomnemonic findings specific to TGCT
    • Monoarticular
    • Visible nodule or mass
    • Skin tension
    • Restricted range of motion
    • Mechanical symptoms (clicks, joint locking, triggering, etc.)
    • Joint effusion or edema

Evaluation

Cytology

  • Fine Needle Aspiration[8]
    • Necessary to confirm diagnosis
    • May be beneficial in surgical planning

Radiographs

  • Findings[9]
    • Soft tissue densities within the joint
    • Soft tissue swelling
    • Joint Effusion

Ultrasound

  • Findings
    • Hypervascularized mass may be identified with doppler evaluation

CT

  • Optimal for evaluating bone quality
  • Findings
    • Soft tissue densities
    • Joint effusion

MRI

  • Imaging modality of choice, necessary for surgical planning
  • Findings
    • Homogenous uptake of mass-like synovial proliferation with lobulated margins
    • May demonstrate blooming

Management

Nonoperative

  • General[4] [6]
    • Never urgent to initiate treatment
    • Observation with serial follow-ups every 6-12 months
    • There are no evidence-based guidelines for observation or follow-up
  • Radiation Therapy
    • Treatment is usually reserved for recurrent cases due to the increased associated risks
  • Pharmacotherapy
    • Imatinib and other targeted therapies have shown positive responses
    • However are not used due to high costs and higher risks for a benign disease

Operative

  • Indications[6]
    • Localized is more responsive to local or arthroscopic excisions and are preferred due to decreased risks of arthritic changes
    • Diffuse typically requires open excisions to decrease recurrence rates versus arthroscopic excision
  • Technique
    • Open
    • Arthroscopic

Rehab and Return to Play

Rehabilitation

  • Case specific, no clear guidelines

Return to Play/ Work

  • General
    • No clear guidelines
    • Most cases only require post surgical recovery periods
    • Patients are then able to continue with prior activities without restrictions

Complications and Prognosis

Prognosis

  • Benign disease which is rarely lethal [4]

Complications

  • Recurrence
    • Rates can be as high as 33% with complete surgical resection
    • 55% with incomplete resection. [4] [6]
  • Bone erosion
    • More common in diffuse type [4] [6]

See Also

References

  1. ↑ Chassaignac EP. Cancer de la gaine des tendons. GazHop CivMilit. 1852;25:185-6. French.
  2. ↑ Jaffe HL, Lichtenstein L, Sutro CJ. Pigmented villonodular synovitis, bursitis, and tenosynovitis: a discussion of the synovial and bursal equivalents of tenosynovial lesion commonly denoted as xanthoma, xanthogranuloma, giant cell tumor or myeloplaxoma of tendon sheath with some consideration of this tendon sheath lesion itself. Arch Pathol (Chic). 1941;31:731-65
  3. ↑ Myers BW, Masi AT, Feigenbaum SL. Pigmented villonodular synovitis and tenosynovitis. A clinical epidemiologic study of 166 cases and literature review. Medicine (Baltimore) 1980;59:223–38.
  4. ↑ 4.0 4.1 4.2 4.3 4.4 4.5 4.6 4.7 4.8 Gouin, F., and T. Noailles. "Localized and diffuse forms of tenosynovial giant cell tumor (formerly giant cell tumor of the tendon sheath and pigmented villonodular synovitis)." Orthopaedics & Traumatology: Surgery & Research 103.1 (2017): S91-S97.
  5. ↑ Robert M, Farese H, Miossec P. Update on Tenosynovial Giant Cell Tumor, an Inflammatory Arthritis With Neoplastic Features. Front Immunol. 2022 Feb 21;13:820046. doi: 10.3389/fimmu.2022.820046. PMID: 35265077; PMCID: PMC8899011.
  6. ↑ 6.0 6.1 6.2 6.3 6.4 6.5 Stephan SR, Shallop B, Lackman R, Kim TWB, Mulcahey MK. Pigmented Villonodular Synovitis: A Comprehensive Review and Proposed Treatment Algorithm. JBJS Rev. 2016 Jul 19;4(7):e3. doi: 10.2106/JBJS.RVW.15.00086. PMID: 27509331.
  7. ↑ Rubin BP. Tenosynovial giant cell tumor and pigmented villonodular synovitis: a proposal for unification of these clinically distinct but histologically and genetically identical lesions. Skeletal Radiol. 2007 Apr;36(4):267-8.
  8. ↑ Suresh SS, Zaki H. Giant cell tumor of tendon sheath: case series and review of literature. J Hand Microsurg. 2010 Dec;2(2):67-71. doi: 10.1007/s12593-010-0020-9. Epub 2010 Nov 18. PMID: 22282671; PMCID: PMC3122708.
  9. ↑ Iqbal, S. (2021, October 28). Pigmented villonodular synovitis. Radiology Reference Article | Radiopaedia.Org. https://radiopaedia.org/articles/pigmented-villonodular-synovitis?lang=us
Created by:
John Kiel on 14 June 2019 10:22:28
Last edited:
19 August 2022 04:36:55
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