Pigmentierte villonoduläre Synovialitis (PVNS)

Zusammenfassung

Die pigmentierte villonoduläre Synovialitis (PVNS oder PVS) ist eine seltene benigne proliferative Erkrankung des Synovialgewebes der Gelenke und Sehnenscheiden. Man unterscheidet die diffuse, lokal aggressiv destruierend wachsende Verlaufsform von der nodulären, lokalisierten, eher verdrängend wachsenden Form. Die Diagnosestellung gelingt in der Regel zuverlässig mittels klinischem Befund, Röntgen und MRT, beweisend ist jedoch nur die Histologie. Die Therapie der Wahl besteht in der marginalen chirurgischen Resektion, befundabhängig als arthroskopische oder offene (Teno)synovialektomie durchgeführt, bei intraartikulär diffusem Befall kombiniert mit adjuvanter RSO. Rezidivraten liegen auch nach Kombinationstherapie im Bereich von fünf bis zehn Prozent.

Summary

Pigmented villonodular synovitis is a benign proliferative disorder of the synovium and tenosynovium that occurs in a diffuse, locally aggressive form and a localized nodular less agressive variant. Clinical examination, native radiographs and MRI are usually sufficient for a reliable diagnosis. The therapy of choice is marginal surgical resection, either by open or arthroscopic synovectomy, followed by radiotherapy for the intraarticular diffuse form. After combination therapy, recurrence rates of 5–10 % have to be expected.

Schlüsselwörter

Pigmentierte villonoduläre Synovialitis - tenosynovialer Riesenzelltumor - Synovialektomie - Radiosynoviorthese

Keywords

Pigmented villonodular synovitis - tenosynovial giant cell tumour - synovectomy

• Literatur

• 1 Myers BW, Masi AT. Pigmented villonodular synovitis: A clinical epidemiologic study of 166 cases and literature review. Medicine (Baltimore) 1980; 59: 223-238.
• 2 Tyler WK, Armando FV, Riley JW, Helaey JH. Pigmented villonodular synovitis. J Am Acad Orthop Surg 2006; 14: 276-385.
• 3 Garner HW, Ortiguera CJ, Nakleh RE. Pigement villonodular synovitis. RadioGraphics 2008; 28: 1519-1523.
• 4 Cheng XG, You YH, Liu W. et al. MRI features of pigmenetd villonodular synovitis (PVNS). Clin Rheumatol 2004; 23: 31-34.
• 5 Lucas DR. Tenosynovial Giant Cell Tumor. Arch Pathol Lab Med 2012; 136: 901-906.
• 6 Jain JK, Vidyasagar JVS, Sagar R. et al. Arthroscopic synovectomy in pigmented villonodular synovitis of the knee: clinical series and outcome. Int Orthop 2013; 37: 2363-2369.
• 7 Colman MW, Ye J, Weiss KR. et al. Does combined open and arthroscopic synovectomy for diffuse PVNS of the knee improve recurrence rates ?. Clin Orthop Relat Res 2013; 471: 883-890.
• 8 Shabat S, Kollender Y, Merimsky O. et al. The use of surgery and yttrium 90 in the management of extensive and diffuse pigmented villonodular synovitis of large joints. Rheumatology (Oxford) 2002; 41: 1113-1118.
• 9 Blanco CE, Leon HO, Guthrie TB. Combined partial arthroscopic synovectomy and radiation therapy for diffuse pigmented villonodular synovitis of the knee. Arthroscopy 2001; 12: 527-531.
• 10 Chen WM, Wu PK, Liu CL. Simultaneous anterior and posterior synovectoy for treating diffuse pigmented villonodular synovitis. Clin Orthop Relat Res 2012; 470: 1755-1762.
• 11 West RB, Rubin BP, Miller MA. et al. A landscape effect in tenosynovial giant-cell tumor from activation of CSF1 expression by a translocation in a minority of tumor cells. Proc Natl Acad Sci U S A 2006; 103: 690-695.
• 12 Ravi V, Wang WL, Lewis VO. Treatment of tenosynovial giant cell tumor and pigmented villonodular synovitis. Current Opinion in Oncology 2011; 23: 361-366.
• 13 Cassier PA, Gelderblom H, Stacchiotti S. et al. Efficacy of imatinib mesylate for the treatment of locally advanced and/or metastatic tenosynovial giant cell tumor/pigmented villonodular synovitis. Cancer 2012; 118: 1649-1655.