Overview Modules Journal Club Procedure Log Interesting Cases Links Lectures Calendar NWSSP Home		Interesting Cases This page is a repository of interesting cases seen by practitioners at Northwest Spine and Sports Physicians, P.C. As cases are added, presentations will be offered in a downloadable PDF format. Case #1 – Pigmented Villonodular Synovitis (PVNS) of the Hip Spanier D, Harrast M. Am J Phys Med Rehabil. 2005 Feb;84(2):131-5 Case Presentation Image 1      A 39 year old male software programming executive who presented to an outpatient musculoskeletal clinic for evaluation of vague hip discomfort of approximately eight months duration. The patient reported vague “abnormal sensations” in the hip girdle region for a number of years, but had noticed a significant progression of his symptoms over the preceding eight months. He had difficulty describing the quality of his discomfort, but stated his symptoms were intermittent, usually in the right buttock or the groin, and occasionally radiating from the right groin into the anterior thigh to the knee (but never below). He attributed his symptoms to being more sedentary over the preceding year spending a lot of time working at his computer. He reported that walking routinely made his symptoms worse, and that stretching seemed to help. He had no specific treatment to this point other than taking ibuprofen 200mg bid as needed with some relief. He otherwise was a healthy male with no other past medical history. He previously was a competitive gymnast, and also enjoyed skate boarding, but denied any trauma from his activities or otherwise. His review of symptoms was negative except as noted in the HPI. He specifically denied any numbness or tingling, bowel or bladder disturbance, back pain, nocturnal symptoms, night sweats or unexplained weight loss. His family history was remarkable for thalassemia (although he was never tested) and Directed physical examination of the right hip demonstrated significant loss of range of motion. With the hip flexed to 90 degrees there was 5 degrees of internal rotation and 30 degrees of external rotation. On the contralateral side there was 20 degrees of internal rotation and 45 degrees of external rotation. Internal rotation on the right caused mild right buttock area symptoms, but no groin pain. Muscle testing was normal bilaterally with the exception of mild weakness noted in the right hip abductors. There was no groin pain with resisted hip adduction. There was no leg length discrepancy or pelvic obliquity. Lumbar spine range of motion was normal and pain free. Sacro-iliac joint provocative maneuvers were negative. There was no tenderness over the lumbar spine. Reflexes were present and symmetric at the patella, medial hamstrings and Achilles tendons bilaterally. Dural tension signs were negative. Proprioception was intact at the great toes bilaterally. Sensation was preserved to light touch and pinprick. Image 2      AP pelvis and right lateral hip plain films were performed in the office which demonstrated severe joint space narrowing, subchondral sclerosis and cysts all about the right hip. Also noted were multiple lucencies about the intertrochanteric region, femoral head and femoral neck which were suggestive of pigmented villonodular synovitis, though a neoplastic process could not be entirely ruled out (see image 1). Subsequent diagnostics included a bone scan that demonstrated active arthritis in the right hip but was otherwise normal. Serologic workup including serum protein electrophoresis, complete blood count, C-reactive protein, prostate specific antigen, and a basic chemistry panel were also completed and were all within normal limits except for mild anemia and microcytosis consistent with his family history of thalassemia. MRI of the right hip demonstrated innumerable low T1 and low T2 signal lesions about the right hip joint. The low signal lesions exhibited “blooming” on the gradient echo sequence consistent with hemosiderin (see image 2). These findings were consistent with and confirmed the diagnosis of pigmented villonodular synovitis.      The patient was referred to orthopedic surgery for further discussion of potential definitive treatment options – i.e., synovectomy versus total hip arthroplasty, and for the concern of pathologic fractures secondary to the large lesions noted in the femoral neck. He was first referred to a surgeon who specialized in joint replacement. That physician agreed that the radiographic findings were consistent with PVNS, however due to his young age and relatively mild symptoms, it was recommended to maximize the non-operative treatments of degenerative joint disease such as activity modification, use of an assistive device for ambulation, and judicious use of oral analgesics and non-steroidal anti-inflammatory medications. It was discussed that should non-operative treatment fail to provide lasting relief, surgical options might include total hip replacement. Discussion      The precise etiology of PVNS is still uncertain. Jaffe first proposed the name, described the main histopathologic features, and originally proposed an inflammatory etiology. This theory has not been uniformly supported. Others believe it is a neoplastic process such as a giant cell sarcoma arising near or inside the synovial space or tendon sheath. This theory is supported by the monoclonality and chromosomal abnormalities identified in these lesions. Other possible etiologies include trauma, disturbances of metabolism, and hemorrhage.      Histologically, the lesion of PVNS demonstrates dense infiltrates of polygonal or spindle cells with abundant cytoplasm and vesicular nuclei; some of the cells contain hemosiderin. Multinucleated giant cells are sometimes present, either scarcely or in large numbers. Aggregation of foamy cells can also be seen. Abundant production of collagen, fibrosis and hyalinization may be evident in patients with long-standing disease.      The clinical presentation of PVNS is often insidious. Patients seek medical consultation months or years after the onset of symptoms due to the often mild and slowly progressive nature of the disease process. At the time of presentation, patients often complain of monoartricular pain that has been present for a few months. The pain can be intermittent with pain free intervals, or may just wax and wane in timing. During a flare, the pain may be quite severe which may represent active hemorrhage into the joint. During these periods, the patient may report positional relief of pain (typical of a joint effusion, hemarthrosis or synovial process).      The differential diagnosis can be quite broad. Common things such as osteoarthritis (OA) need to be excluded. OA typically presents in older adults and affects multiple joints. Radiographically, polyarticular involvement is more suggestive of OA and helps to exclude the diagnosis of PVNS. Tuberculosis arthritis can also have a subtle presentation. Radiographs in this disease process demonstrate hallmark findings of juxta-articular osteoporosis and loss of articular cartilage which distinguish tuberculosis arthritis from PVNS. Other differential diagnosis includes synovial sarcoma, hemophilia, synovial hemangioma, and rheumatoid arthritis. Synovial sarcoma is typically extra-articular and presents with calcifications in 1/3 of patients. Hemophilia and synovial hemangioma may be associated with intraarticular bleeding and hemosiderin deposition, the former being excluded with laboratory testing. Rheumatoid synovitis and synovial hemangioma are difficult to differentiate from PVNS. Rheumatoid synovitis classically demonstrates thinner synovial proliferation with less hemosiderin deposition. It should be noted, that histiologic evidence of PVNS has been documented in the tissue of patients with rheumatoid arthrtitis. PVNS should be in the differential in any young patient who presents with arthritis, with or without a mass, involving a single major joint.      Blood tests are usually not helpful in making the diagnosis of PVNS, but may be helpful in excluding other diagnoses. Physical exam may demonstrate decreased ROM and possible effusion. Joint aspiration classically demonstrates a blood tinged or brown pigmented fluid. Microscopic analysis of joint fluid demonstrates large numbers of multinucleated foreign body giant cells with a large amount of coarsely granular yellow-brown hemosiderin pigment.      Radiographicallly, the classic appearance of PVNS on plain X-ray is soft tissue swelling or a mass about the joint, usually with joint space preservation, however the joint space will be destroyed as the disease progresses. Erosions and subchondral cysts are common, particularly when PVNS occurs in the hip, ankle, or elbow. Peri-articular osteoporosis is not usually seen. Intra-articular calcifications and osteophytes are rare. The effusion may appear dense on plain films or CT scan due to the high iron content. MRI will demonstrate the effusion and the hemosiderin laden soft tissue masses will be seen as areas of low signal intensity on T1 and T2 weighted sequences. This is more pronounced on the gradient echo than on spin-echo images. The size of the lesion may appear larger than the real lesion – so called “blooming phenomenon” – due to the magnetic susceptibility effect caused by the hemosiderin. The differential diagnosis based on magnetic resonance imaging includes hemophiliac arthropathy, soft tissue sarcoma, fibromatosis, synovial chondromatosis, septic arthritis, and inflammatory arthropathies.      No strict guidelines exist for the treatment of PVNS, however many authors agree that young patients without compromise of the articular cartilage should be treated with extensive synovectomy. Synovectomy may be performed open or arthroscopically. The arthroscopic approach is better tolerated and results in more rapid recovery for most patients, however open synovectomy has much lower recurrence rates, particularly in diffuse cases. Total joint arthroplasty may be combined with synovectomy when the joint cartilage is severely compromised.      Other treatments such as radiation therapy and radioiosotope synovectomy have been attempted with good result. Radiation therapy is usually uses in malignant tumors as definitive or adjuvant treatment. In PVNS it has been suggested when total synovectomy is not possible due to extensive disease or where recurrence and repeat surgery would lead to impaired function. Reported complications from radiation treatment have included joint stiffness, femoral fracture and impotence. Potential adverse affects of radiation therapy also include poor wound or graft healing and late radiation-induced sarcomas in young individuals. Since PVNS is a benign disorder radiation therapy should be considered with caution.      Intraarticular injection of yttrium-90 (90Y) has been described in the literature, and in small studies, it has been shown to be beneficial in treating the disease process and preventing recurrence, particularly when administered early in the course. Potential adverse effects include radionecrosis of the extraarticular soft tissues, febrile reaction, local painful reaction, and leakage of the radioactive material from the joint space to the entire body via the lymph nodes. With systemic leakage, there are also the possible risks of infertility and malignant changes with chromosomal damage to mitotic cells, although none of these have been reported. Selected References Enzinger, F. and S. Weiss, Benign Tumors and Tumorlike Lesions of Synovial Tissue, in Soft Tissue Tumors, F. Enzinger and S. Weiss, Editors. 1995, Mosby: St Louis. p. 742-749. Northmore-Ball, M.D., Young adults with arthritic hips. Bmj, 1997. 315(7103): p. 265-6. Hickman, J.M. and C.L. Peters, Hip pain in the young adult: diagnosis and treatment of disorders of the acetabular labrum and acetabular dysplasia. Am J Orthop, 2001. 30(6): p. 459-67. Rodriguez-Merchan, E.C., Coxarthrosis after traumatic hip dislocation in the adult. Clin Orthop, 2000(377): p. 92-8. Gelber, A.C., et al., Joint injury in young adults and risk for subsequent knee and hip osteoarthritis. Ann Intern Med, 2000. 133(5): p. 321-8. Adkins, S.B., 3rd and R.A. Figler, Hip pain in athletes. Am Fam Physician, 2000. 61(7): p. 2109-18. Sampathkumar, K., C. Rajasekhar, and M.J. Robson, Pigmented villonodular synovitis of lumbar facet joint: a rare cause of nerve root entrapment. Spine, 2001. 26(10): p. E213-5. Lee, J.H., et al., Extra-articular pigmented villonodular synovitis of the temporomandibular joint: case report and review of the literature. Int J Oral Maxillofac Surg, 2000. 29(6): p. 408-15. Jaffe, H., L. Lichtenstein, and C. Sutro, Pigmented villonodular synovitis, bursitis and tenosynovitis: A discussion of the synovial and bursal equivalents of the tenosynovial lesion commonly denoted as xanthoma, xanthogranuloma, giant cell tumor or myeolplaxoma of the tendon sheath itself. Arhc Pathol Lab Med, 1941. 31: p. 7310765.