Adult multifocal pigmented villonodular synovitis--clinical review

Abstract

Pigmented villonodular synovitis (PVNS) is a rare, benign proliferative disease of the synovial tissue that affects a single joint or a tendon sheath. Data from the literature present only a few cases of multifocal PVNS. This paper presents multifocal PVNS in the adult. This disease can affect bilateral shoulders, hips and knees. The diagnosis may be delayed by the slow evolution of the disease (up to ten years); some patients may be seen with late-stage degenerative joints, serious complications, painful and functionally uncompensated, with significant locomotion deficit. PVNS requires a radical treatment with prosthetic arthroplasty associated with synovectomy. Complex imaging (X-Rays, magnetic resonance imaging (MRI), ultrasound) and macroscopic appearance of the lesions during surgery confirms the clinical diagnosis of multifocal PVNS with secondary bone lesions. Histology marks the final diagnosis of multifocal PVNS. The postoperative results are good, with recovery in functional parameters of the joints with endoprosthesis.

Fig. 1

Radiography of the shoulder, showing large cortical erosions and deformity, large cephalic geodes, glenoid erosion, narrowed articular space

Fig. 2

Radiography of the pelvis, showing pseudocystic images on the acetabulum and right femoral neck outside the bearing area, narrowed articular space

Fig. 3

Magnetic resonance imaging (MRI) of the shoulder demonstrates synovial hypertrophy, diffuse oedema of the humeral head and of the glenoid, small marginal erosions and large subchondral cysts on both articular surfaces, larger on the superior part of the humeral head (22 mm), effusion in the subacromial bursa

Fig. 4

Magnetic resonance imaging (MRI) of the pelvis, showing oedema on the right femoral head and neck, and on the acetabulum; small marginal erosions and large subchondral cysts on both articular surfaces, hypertrophy of the synovia with T2 hyper signal on the left, as well as small marginal erosions with subchondral cysts on the femoral head and acetabulum

Fig. 5

Microscopic presentation: synovial hypertrophy with villositary structure