Diffuse Biarticular Pigmented Villonodular Synovitis

Diffuse biarticular pigmented villonodular synovitis is rare. In this paper, we will describe two histologically proved cases seen at the Duke University Medical Center and review the clinical, pathologic, and radiologic features of this lesion. In 1941, Jaffe, Lichtenstein, and Sutro (10) described the pathological evolution and natural history of a process involving the synovial lining of joints which had been known by various names, depending upon the major histologic manifestations. These lesions were termed in the literature chronic hemorrhagic villous synovitis, giant-cell fibro-hemangioma, fibro-hemosideric sarcoma, sarcoma fusigigantiocellulare, and benign polymorphocellular tumor of the synovial membrane. Since the lesion basically combined pigmentation and villous and nodular proliferation of the synovium, and seemed to be an inflammatory process rather than a true neoplasm, it was named pigmented villonodular synovitis by Jaffe and his associates. They also described similar lesions of the bursae and tendon sheaths which they termed pigmented villonodular bursitis and tenosynovitis. Although there are some 300 cases of pigmented villonodular synovitis now reported in the literature, only two other cases of the diffuse form have shown the histologic picture in more than one joint (3, 9). All of the other cases have been monoarticular. Kelikian and Lewis (12) mention bilateral knee involvement with pigmented villonodular synovitis in 3 siblings of one family; however, they do not state whether the diagnosis was made histologically or merely by arthrography. Clinical Manifestations Clark (6) estimates the incidence of pigmented villonodular synovitis to be 1 per cent of all patients presenting with joint complaints. It seems to be more common in females (3, 22) and occurs most often in the third through fifth decades of life, with the age in reported cases ranging from eleven (3) to seventy-five years (7). In our present series, the patients were fifty-six and sixty years old, respectively. By far the most common site of involvement is the knee joint (3, 22); however, there are reports of villonodular synovitis involving the hip (2, 3, 5, 22), ankle (3), tarsus, carpus (22), elbow (2), and shoulder (15), in decreasing order of frequency (3, 22). In the case of biarticular involvement reported by Greenfield and Wallace (9) both knees were involved. Byers et al. (3) reported biarticular pigmented villonodular synovitis of the ankles. In our patients, one showed involvement of both knees, while the other had involvement of one knee and a hip joint. The symptoms related to diffuse pigmented villonodular synovitis are usually insidious. The patient may notice a gradual swelling of a joint over several months or years. Occasional exacerbations of joint swelling are not uncommon, and the effusions regress slowly. In many instances, a history of trauma may be obtained.