Pigmented villonodular synovitis

Abstract

Pigmented villonodular synovitis (PVNS) is a benign disease that rarely undergoes malignant transformation. There are two types of disease: localized (nodular tenosynovitis) and di used (pigmented villonodular synovitis/tenosynovitis) with intra- or extra-articular locations. The second one is limited to synovium of the burse (PVNB) or tendon sheath (PVNTS). The intraarticular lesions are usually located in the knee, hip, ankle and elbow joints. Histologically, PVNS is a tenosynovial giant cell tumor, characterized by proliferation of two types of mononuclear cells - predominantly small, histiocyte-like cells and larger cells with dense cytoplasm, reniform or lobulated nucleus, with accompanying multinucleated giant cells and macrophages overloaded with hemosiderin that give typical image on MRI - currently selected as a gold standard for its diagnosis. The classic X-ray and CT are non-specific but similar to ultrasound should be used to evaluate disease progression and treatment response if radiotherapeutic and pharmacological methods were selected for treatment. An open arthroscopic surgery could also be applied in selected cases.

Keywords: PVNS; diagnosis; epidemiology; pigmented villonodular synovitis; tenosynovial giant cell tumor.