Pigmented villonodular synovitis
Pigmented Villonodular Synovitis: A Retrospective Single-Center Study of 122 Cases and Review of the Literature

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Objectives

Pigmented villonodular synovitis (PVNS) is a rare but disabling disease. The objective was to describe the clinical presentation and outcomes of PVNS according to its localization.

Methods

Retrospective, systematic study of all cases of biopsy-proven PVNS followed in 1 tertiary-care center specialized in isotopic synoviorthesis. Cases were selected by keyword. Collected data included disease localization, therapeutic modalities, and outcomes.

Results

A total of 122 cases (mean age 33.0 ± 13.1 years, 58% female, 89% diffuse form) of histologically confirmed PVNS were analyzed with a mean follow-up of 5.8 ± 4.3 years (707 patient-years total). The main localizations were the knee (75%) and ankle (16%). Clinical presentation included joint pain (80%) and joint effusion (79%) with hemarthrosis (75% of analyzed articular fluid). The mean delay before diagnosis was 2.9 ± 3.7 years. Magnetic resonance imaging was helpful for diagnosis in 83%. Surgical synovectomy was initially performed in 98% of cases and was often associated with isotopic synoviorthesis (knee: 57%; other localizations: 74%). In patients with a diffuse form treated at first line by surgery followed by isotopic synoviorthesis, the relapse rate was 30% (knee) and 9% (other localizations), respectively, with a mean delay before relapse of 2.6 ± 2.4 and 2.4 ± 0.9 years, respectively.

Conclusions

PVNS occurs in young adults, mainly in the knee joint; joint pain and effusion with hemarthrosis are the most frequent signs. Relapse is frequent, in particular, for diffuse knee PVNS; the usefulness of isotopic synoviorthesis remains to be confirmed.

Section snippets

Design

The design of the present study was a systematic monocentric retrospective analysis.

Patients

The patients in the present study included all cases of biopsy-proven PVNS seen in 1 center either as outpatients or inpatients, between June 1991 and October 2008. Histology was analyzed where the biopsy was performed (outside or in our hospital).

Patient Selection

Cases were selected by the keyword “PVNS” in the computerized database. A total of 133 patients were screened. Twelve were excluded: 10 had no PVNS (5 undetermined

Patient Selection

A total of 121 patients were screened. As 1 patient had 2 localizations (knee and ankle) of PVNS, the total number of cases was 122 for 121 patients. All 122 cases of histologically confirmed PVNS were analyzed. To confirm the diagnosis of PVNS, histology was analyzed by the local pathologist after biopsy in all cases and showed characteristic features of PVNS: brownish, villous, and nodular growths containing groups of histiocytes with fibrous stroma, hemosiderin deposits, giant cells, and

Discussion

This retrospective study is the largest series of PVNS affecting mainly large joints reported in the literature. Our study confirmed that PVNS occurs mainly in young adults (mean age: 33 years) both male and female. Clinical and demographic data of the present and previous clinical studies are described in Table 3 (1, 7, 10, 11, 20, 21, 22, 23, 24). In the present study, the mean delay before diagnosis was high (2.9 years), probably because clinical signs are nonspecific. Pain (80%) and/or

Acknowledgments

We thank Dr Brière and and Karine Emengeard for patient selection.

References (41)

  • B.W. Myers et al.

    Pigmented villonodular synovitis and tenosynovitis: a clinical epidemiologic study of 166 cases and literature review

    Medicine (Baltimore)

    (1980)
  • R.J. Sakkers et al.

    X-chromosome inactivation in patients who have pigmented villonodular synovitis

    J Bone Joint Surg Am

    (1991)
  • J.A. Fletcher et al.

    Trisomy 5 and trisomy 7 are nonrandom aberrations in pigmented villonodular synovitis: confirmation of trisomy 7 in uncultured cells

    Genes Chromosomes Cancer

    (1992)
  • P.F. Choong et al.

    Pigmented villonodular synovitis

    Monoclonality and metastasis—a case for neoplastic origin? Acta Orthop Scand

    (1995)
  • H.S. Schwartz et al.

    Pigmented villonodular synovitisA retrospective review of affected large joints

    Clin Orthop Relat Res

    (1989)
  • M.D. Murphey et al.

    Pigmented villonodular synovitis: radiologic-pathologic correlation

    Radiographics

    (2008)
  • P.D. Byers et al.

    The diagnosis and treatment of pigmented villonodular synovitis

    J Bone Joint Surg Br

    (1968)
  • J.E. Johansson et al.

    Pigmented villonodular synovitis of joints

    Clin Orthop Relat Res

    (1982)
  • F.C. Flandry et al.

    Surgical treatment of diffuse pigmented villonodular synovitis of the knee

    Clin Orthop Relat Res

    (1994)
  • K.R. Chin et al.

    Treatment of advanced primary and recurrent diffuse pigmented villonodular synovitis of the knee

    J Bone Joint Surg Am

    (2002)

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