184-190
Clinicalrheumatology, 1991, 10, N ~ 2
Case Report
Pigmented Villonodular Synovitis of the Hip. A Case Report and Review of the Literature F. D E S C A M P S ,
E. Y A S I K , D. H A R D Y ,
M. L A F O N T A I N E ,
Ph. D E L I N C E .
Summary
A 73-year-old patient, whose hip was completely destroyed by pigmented villonodular synovitis, was successfully treated by a total prosthetic replacement of this articulation. The etiopathogenesis of the illness is uncertain. Its' localization in the hip is, fortunately, a rare occurrence. It can be responsible for frequently extensive articular destruction which is even more dramatic when it affects young patients, whose average age is 35. Only early diagnosis permits conservative surgical treatment : total synovectomy associated with curettage of the foyers of osteolysis and their filling with bone grafts. If the destructive lesions are too extensive, an arthrodesis or replacement arthroplasty are proposed.
Key words: Pigmented Villonodular Synovitis, Synovial Tumor, Total Hip Prosthesis.
INTRODUCTION Pigmented villonodular synovitis is a rare disease which may be responsible for very severe articular destruction. The recent observation of a particularly evolved case of pigmented viUonodular synovitis of the hip prompted us to review the existing literature concerning the epidemiology, the etiopathogenic hypotheses, the diagnostic techniques available as well as the current trends concerning treatment of the disease. CASE REPORT Mr. J.D., 73 years of age, consulted us for the first time in November 1987. He complained of left hip pain intermittently present over a period of two years. Despite physical therapy and medical treatment with anti-inflammatory drugs, the pain progressively increased and had become permanent six months before his consultation to such a point that walking was practically impossible. His medical history revealed that the patient had suffered a cerebrovascular accident at 63 years of age, from which he completely recovered. Daily administration of
Department of Orthopaedics and Traumatology,H6pital Universitaire Saint-Pierre, Brussels, Belgium.
diuretics controlled a hypertensive condition and he suffered from prostatic problems. On physical examination, all movements of the left hip were painful; but, the amplitude of these movements was almost normal. His walking perimeter was limited to a few steps. In the erect posture, he presented a 20 ~ flexion contracture of the hip and serious tilting of the pelvis. With the exception of the rectal examination, which revealed an apparently benign prostatic hypertrophy, the remainder of the physical examination was normal. Biological examination revealed an inflammatory syndrome: slight microcytic anemia, fibrinogen at 600 mg % (normal range = 200-400 mg %), and a sedimentation rate of 20 mm/h. Microscopic examination of his urine showed an important leucocyturia (+ + + + +) and the presence of Morganelle Morgani bodies. A radiological examination of the pelvis in July 1986 showed important coxarthrosis of the left hip and slight involvement of the right hip (Fig. 1). Recent radiological examination, in November 1987, revealed almost complete destruction of the left femoral head and neck associated with ascent of the femur. There were extensive cystic alterations at the acetabular level (Fig. 2). On the right side, there had been no radiological aggravation of the already present lesions (Fig. 3). Confronted with such recently evolutive and extensive bony destruction, several diagnostic hypotheses were
Pigmented villonodular synovitis of the hip
185
Fig. 1 : July 1986: On the left side, pinching of the superior pole with disappearance of the interarticular space. Presence of cystic cavities and subchondral sclerosis. On the right side, slight arthritic changes : osteophytosis of the superior external rim of the acetabulum with no articular pinching.
Fig. 2: November 1987: osteolysis of the left femoral neck and
Fig. 3: November 1987: right hip. No radiological evidence of
head with important ascension of the femur, cystic destruction of the acetabulum.
aggravation of the arthritic changes when compared to July 1986 examination.
186
F. Descamps, E. Yasik, D. Hardy, et.al.
Fig. 4: Pelvic CAT scan demonstrating the importance of the
destructive process of the right hip: disappearance of the femoral head and neck, cystic destruction of the acetabulum, thickening of the synovial membrane with articular effusion.
possible: primitive-bone or synovial tumor, metastatic cancer, bone tuberculosis, or rheumatoid arthritis. The diagnosis of rheumatoid arthritis was rapidly excluded due to the absence of the habitual markers of this disease, as well as the clinical presentation of the disease itself. Tuberculosis of the bone seemed improbable considering the slight reaction to tuberculine intradermally, good general physical condition of the patient, and normal chest X-ray. Despite the normality of the usual tumor markers, as well as acid phosphatases, the radiological images suggested tumoral destruction of the hip. Pelvic computerized axial tomogram (CAT), carried out with a high resolution and soft tissue window, revealed complete destruction of the left femoral head and neck. There was capsular thickening and an articular effusion (Fig. 4). On the right side, the only alteration seen was a posterior pinching of the interarticular space (Fig. 5). In addition, the prostate was hypertrophied and the bladder wall thickened. Intravenous urogram showed a marked prostatic impression, a diverticular aspect of the bladder wall as well as an important post-mictional residue. Hepatosplenic echogram, barium enema, and a colonoscopy to 50 cm were normal. A technetium bone scan showed slight hyperfixation of the tracer at the left hip and the right
Fig. 5: Pelvic CAT scan: on the left side, disappearance of the femoral head. On the right side, posterior articular pinching.
Prostatic hypertrophy.
Pigmented villonodular synovitis of the hip
187
then realized using a Harris cup of 54 mm (Zimmer) and a Landos T 12 prosthesis with a long neck (Landanger) (Fig. 6). Detailed anatomopathological examination showed a tissue with a thick, fibrous synovial wall presenting lymphocytic infiltration of variable intensity. Certain areas were rich in collagen, while others were richly vascularized and populated by fibroblasts. Several giant cells were found as well as numerous deposits of hemosiderine (Fig. 7). In one of the samples, there were agglomerates of foam cells. Thus, the diagnosis of pigmented villonodular synovitis was confirmed. The post-operative evolution was rapid and favorable. Ambulation with total weight-bearing was immediately authorized. The disappearance of pain was practically immediate and functional recuperation of the hip was very rapid. At this time, two years after the surgical procedure, the mobility of the hip is practically normal and the walking perimeter has reached 2 km. There are currently no clinical or radiological signs of local recurrence or involvement of other articulations.
DISCUSSION
Epidemiology Fig. 6: X-Ray of the left hip two years after surgical treatment. knee. The X-ray of the right knee was normal. Thyroid scintigram was normal. Finally, a cystoscopy associated with bladder and prostatic biopsy showed aspecific, chronic cystitis and benign nodular hyperplasia of the prostate. At the conclusion of these examinations, the retained diagnosis was that of a primitive tumor at the hip. Surgical treatment was carried out on December 16, 1987 under general anesthesia. The articular capsule had a normal aspect. Opening of the capsule revealed a thick yellow-grey granulation tissue invading the articular cavity from which the femoral head had disappeared. The synovial membrane was globally thickened. A neo-articulation had formed between the stump of the femoral neck and the roof of the acetabulum where there was localized sinking. The exuberant growth, as well as the synovial membrane, was sent to the laboratory for anatomopathological examination. The extemporaneous examination diagnosed pigmented villonodular synovitis. A total cemented hip arthroplasty was
The annual incidence of pigmented villonodular synovitis was estimated by Myers et al. in their epidemiological study (1) as being approximatively 1.8 cases per million. Except for this study, where Myers observed a higher incidence in the male sex (2/1 ratio), other authors had reported an equal incidence in both sexes. The disease manifests itself primarily in the third and fourth decades, but several cases have been reported in young children as well as in old people (1-4). Pigmented villonodular synovitis can occur at all synovial articulations as well as at the synovial sheaths of the fingers and the hands. It is this latter localization which is the most frequent (57% of all cases according to Rao et al.) (5). Among the larger articulations, the knee is the most often affected (80% of all cases) (1-3, 5-7). The hip is, in some series, the second most affected articulation (5-10% of all cases) followed by the ankle, the wrist, the shoulder, and the foot (1,3,5-7). Rare localizations, such as the vertebral column, have been reported (8). The disease is almost always mono-articular and multi-articular involvement has been described in only approximatively 10 cases.
188
F. Descarnps, E. Yasik, D. Hardy, et.al.
Fig. 7: Pigmented villonodular synovitis: anatomopathological aspect.
Etiopathogenesis Since its description in 1941 by Jaffe et al. (9), numerous hypotheses have been formulated : inflammatory response to an unknown agent (9), disorder of lipid metabolism (1), repeated micro-traumatisms associated with hemarthrosis (1). For Rao et al. (5), the disease resuits from a neoplastic-like proliferation of the fibroblasts and histiocytes of the synovial tissue. Currently, there exists no certainty concerning the etiology of pigmented villonodular synovitis. The pathogenesis of the osseous invasion and the formation of cystic cavities also remain uncertain. For Mc Master et al., invasion of bone tissue begins from an intra-articular lesion, a small cartilagenous erosion at the osteochondral junction (10). For Chung et al., the increase of intra-articular pressure, provoked by tumor growth, could be responsible for erosion of the cartilage (7). For Scott, finally, the tumoral growth reaches the deep tissues by following the trajectories of the nutritional blood vessels of bone tissue (11).
eral years of evolution. Most cases present episodes of pain, accompanied by limitation of movement, articular effusion or progressive tumefaction. Inflammatory signs are usually discrete or, more frequently, absent. Episodes of blocking are typical of involvement of the knee (1,3-5,10,12,13). Howie et al. (14) reported three cases where torsion of a lesion in the knee was responsible for acute pain and blocking. Tumefaction is often evident when the knee, the wrist or the ankle are involved (15,7,14). Often, involvement of the hip is accompanied only by a slight limitation of mobility or even normal mobility (2,3,7,10,15), as was seen in our patient. On the other hand, the evolution of disease was particularly rapid in our patient, and, in addition, the amplitude of the bone destruction was among the most important reported. The absence of pain on the right side as well as the stability of the radiological arthritic signs in the right hip permitted the authors to conclude that there was only unilateral articular involvement. This unilateral involvement has been seen in almost all of the cited cases of this disease.
Diagnosis
Complementary examinations
The evolution is habitually insidious and the diagnosis is usually made often after several months or even sev-
Except for the presence of hamatic articular fluids, the different authors have not been able to demonstrate any
Pigmented villonodular synovitis of the hip
significant anomalies in the different clinical tests carried out. The radiological anomalies of pigmented villonodular synovitis (PVS) are aspecific and can be seen in cases of bone tuberculosis or, more particularly, in synoviosarcoma. The radiological aspect, although highly suggestive of the disease in its evolved forms, does not allow a diagnosis with certainty. The most complete study of radiological anomalies is certainly that by Smith et al. (16), where he distinguished two forms of PVS. The first type is a localized form in which only a portion of the synovial is involved and where radiological anomalies are rarely observed. The second type is a diffuse form where the totality of the synovial is involved and where constant radiological abnormalities occur. The most frequently observed images are: thickening of the synovial tissue, bone erosions or cystic cavities bordered by sclerosis in nonosteoporotic bone without a decrease in the size of the interarticular space as seen in arthrosis. In our patient, older than the average age of most of the reported cases, in addition to these particular images, arthritic type alterations were added which temporarily masked and delayed the diagnosis. In the literature, we found only one patient where a technetium bone scan demonstrated a hyperfixation of the tracer molecule on the affected side (12). But here, the authors stressed the fact that this articulation had already been operated on and presented important arthritic lesions. In our patient, bone scan revealed a hyperfixation of the tracer in the affected hip. The other hip, however, which presented radiological signs of arthrosis, did not excessively fix the tracer molecule. There was also hyperfixation in the right knee of the patient, but, given his advanced age, an arthroscopy to look for lesions of PVS was not performed. In conclusion, it may be said that only anatomopathological examination permits the diagnosis of PVS with certainty. The characteristics of PVS are relatively well defined(i,5,6,9,10,12,17,18). One of the most remarkable aspects of the disease is the high concentration of hemosiderine in the diseased tissues. This could be responsible for the increased radiological density of the affected tissues. In a case of an affected knee, Rosenthal et al. (19) were able to evaluate, by means of a CAT scan, the concentration of iron in the tumoral mass, this being the first reported case in the literature. Although hemosiderine deposits were seen in our patient, their low con-
189
centration did not permit us to observe an increase in tissue density with CAT scan. Treatment
Pigmented villonodular synovitis presents many different clinical forms in diverse articulations. Because of its relative rarity, no prospective study has been carried out. The age of the patient, his autonomy and the seriousness of the destructive lesions determine the choice of treatment. In localized forms, local resection of the pathological tissues generally gives excellent results (2-5) with a very low recurrence rate. In more diffuse forms, most authors agree as to the necessity of a synovectomy as complete as possible. However, when synovectomy is the only therapeutic act realized, there is a very high rate of recurrence (46%) (1,2,3). This induces certain authors to directly prescribe radiotherapy in older patients or in cases of recurrence. Unfortunately, this adjuvant therapy is not without secondary effects (carcinogenic effects, articular ankylosis). For Mc Master (10) and Scott (11), synovectomy should be completed with careful curettage of the cystic cavities associated with bone grafts whenever the destructive lesions are not too important. Finally, if the lesions are too evolved, which is often the case in the hip or the shoulder, the authors report good results with replacement arthroplasty (2,3,4,6,7). Arthrodesis remains a debatable alternative in young patients (2,5,6,10). Salliere et al. (4) report satisfactory results in a child treated by synoviorthesis with osmic acid. They, therefore, propose this treatment for the recurrence of articular effusion after surgical synovectomy. In our 73-yearold patient, who presented extensive osteo-articular destruction, replacement arthroplasty of the entire articulation was the only therapeutic option. CONCLUSION Pigmented villonodular synovitis remains a rare illness whose etiopathogenesis is still uncertain. Its diagnosis remains one of exclusion and the therapeutic modalities must be decided according to the localization and the importance of the articular destruction, as well as the age of the patient.
Acknowledgements : The authors would like to thank Mrs Marianne Fouss for her secretarial assistance.
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REFERENCES
1.
Myers, B.W., Masi, T.A. Pigmented villonodular synovitis and tenosynovitis : A clinical epidemiologic study of 166 cases and literature review. Medecine 1980, 59, 223-238. 2. Byers, P.D., Cotton, R.E., Deacon, O.W., Lowy, M., Newman, P.H., Sissons, H.A., Thomson, A.D. The diagnosis and treatment of pigmented villonodular synovitis. J Bone Joint Surg 1968, 50-B, 290-305. 3. Johansson, J.E., Ajjoub, S., Coughlin, L.P., Wener, J.A., Cruess, R.L. Pigmented villonodular synovitis of joints. Clin Orthop 1982, 163, 159-166. 4. Salliere, D., Gaillard, F., Hulin, P.H., Carlioz, H., Daoud, D., Gougeon, J., Massias, P. Synovite villo-nodulaire de l'enfant. A propos de 4 observations. Rev Rhum 1984, 51, 33-36. 5. Rao, A.S., Vigorita, V.J. Pigmented villonodular synovitis (Giantcell tumor of the tendon sheath and synovial membrane). A review of eighty-one cases. J Bone Joint Surg 1984, 66-A, 76-94. 6. Dorwart, R.H., Genant, H.K., Johnston, W.H., Morris, J.M. Pigmented villonodular synovitis of synovial joints : clinical, pathologic and radiologic features. A J R 1984, 143, 877-885. 7. Chung, S.M.K., Janes, J.M. Diffuse pigmented villonodular synovitis of the hip joint. J Bone Joint Surg 1965, 47-A, 293-303. 8. Valat, J.P., Gatti, P., Meurice, J.C., Grangeponte, M.C., Monegier du Sorbier, C., Benatre, A., Castaing, J. Synovite villo-nodulaire du rachis. Un cas. Rev Rhum 1984, 51, 41-43. 9. Jaffe, H.L., Lichtenstein, L., Sutro, C.J. 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 myeloplaxoma of the tendon sheath, with some consideration of this tendon sheath lesion itself. Arch Pathol 1945, 31, 731-765. 10. Mc Master, P.E. pigmented villonodular synovitis with invasion of bone. Report of six cases. J Bone Joint Surg 1960, 42-A, 11701183.
11. Scott, P.M. Bone lesions in pigmented villonodular synovitis. J Bone Joint Surg 1968, 50-B, 306-311. 12. Kindblom, L.G., Gunterberg, B. Pigmented villonodular synovitis involving bone. Case report. J Bone Joint Surg 1978, 60-A, 830832. 13. Reginster, J.Y., Urbin-Choffray, D. Martens de Noordhout, B., Vandervoort, A., Franchimont, P. La synovite villo-nodulaire pigment6e du genou. Apropos de deux cas. Rev M6d Li6ge, 1985, 40, 506-509. 14. Howie, C.R., Smith, G.D., Christie, J., Gregg, P.J. Torsion of localised pigmented villonodular synovitis of the knee. J Bone Joint Surg 1985, 67-B, 564-566. 15. Van Rens, T.J.G. Pigmented villonodular synovitis of the hip joint. Acta Orthop Belg 1972, 38, 221-232. 16. Smith, J.H., Pugh, D.G. Roentgenographic aspects of articular pigmented villonodular synovitis. A J R 1962, 87, 1146-1156. 17. Flandry, F., Hughston, J.C. Villonodular synovitis. J Bone Joint Surg 1987, 69-A, 942-949. 18. Snook, G.A. Pigmented villonodular synovitis with bony invasions. A report of two cases. JAMA 1963, 184, 424-425. 19. Rosenthal, D.I., Aronow, S., Murray, W.T. Iron content of pigmented villonodular synovitis detected by computed tomography. Radiology, 1979, 133, 409-411.
Received: 26 February 1990; Revision-accepted: 31 August 1990 Correspondence to: Dr. Ph. DELINCE, H6pital Saint-Pierre, Rue Haute 322, B-1000 Bruxelles, Belgique.
Clinicalrheumatology, 1991, 10, N ~ 2
Case Report
Pigmented Villonodular Synovitis of the Hip. A Case Report and Review of the Literature F. D E S C A M P S ,
E. Y A S I K , D. H A R D Y ,
M. L A F O N T A I N E ,
Ph. D E L I N C E .
Summary
A 73-year-old patient, whose hip was completely destroyed by pigmented villonodular synovitis, was successfully treated by a total prosthetic replacement of this articulation. The etiopathogenesis of the illness is uncertain. Its' localization in the hip is, fortunately, a rare occurrence. It can be responsible for frequently extensive articular destruction which is even more dramatic when it affects young patients, whose average age is 35. Only early diagnosis permits conservative surgical treatment : total synovectomy associated with curettage of the foyers of osteolysis and their filling with bone grafts. If the destructive lesions are too extensive, an arthrodesis or replacement arthroplasty are proposed.
Key words: Pigmented Villonodular Synovitis, Synovial Tumor, Total Hip Prosthesis.
INTRODUCTION Pigmented villonodular synovitis is a rare disease which may be responsible for very severe articular destruction. The recent observation of a particularly evolved case of pigmented viUonodular synovitis of the hip prompted us to review the existing literature concerning the epidemiology, the etiopathogenic hypotheses, the diagnostic techniques available as well as the current trends concerning treatment of the disease. CASE REPORT Mr. J.D., 73 years of age, consulted us for the first time in November 1987. He complained of left hip pain intermittently present over a period of two years. Despite physical therapy and medical treatment with anti-inflammatory drugs, the pain progressively increased and had become permanent six months before his consultation to such a point that walking was practically impossible. His medical history revealed that the patient had suffered a cerebrovascular accident at 63 years of age, from which he completely recovered. Daily administration of
Department of Orthopaedics and Traumatology,H6pital Universitaire Saint-Pierre, Brussels, Belgium.
diuretics controlled a hypertensive condition and he suffered from prostatic problems. On physical examination, all movements of the left hip were painful; but, the amplitude of these movements was almost normal. His walking perimeter was limited to a few steps. In the erect posture, he presented a 20 ~ flexion contracture of the hip and serious tilting of the pelvis. With the exception of the rectal examination, which revealed an apparently benign prostatic hypertrophy, the remainder of the physical examination was normal. Biological examination revealed an inflammatory syndrome: slight microcytic anemia, fibrinogen at 600 mg % (normal range = 200-400 mg %), and a sedimentation rate of 20 mm/h. Microscopic examination of his urine showed an important leucocyturia (+ + + + +) and the presence of Morganelle Morgani bodies. A radiological examination of the pelvis in July 1986 showed important coxarthrosis of the left hip and slight involvement of the right hip (Fig. 1). Recent radiological examination, in November 1987, revealed almost complete destruction of the left femoral head and neck associated with ascent of the femur. There were extensive cystic alterations at the acetabular level (Fig. 2). On the right side, there had been no radiological aggravation of the already present lesions (Fig. 3). Confronted with such recently evolutive and extensive bony destruction, several diagnostic hypotheses were
Pigmented villonodular synovitis of the hip
185
Fig. 1 : July 1986: On the left side, pinching of the superior pole with disappearance of the interarticular space. Presence of cystic cavities and subchondral sclerosis. On the right side, slight arthritic changes : osteophytosis of the superior external rim of the acetabulum with no articular pinching.
Fig. 2: November 1987: osteolysis of the left femoral neck and
Fig. 3: November 1987: right hip. No radiological evidence of
head with important ascension of the femur, cystic destruction of the acetabulum.
aggravation of the arthritic changes when compared to July 1986 examination.
186
F. Descamps, E. Yasik, D. Hardy, et.al.
Fig. 4: Pelvic CAT scan demonstrating the importance of the
destructive process of the right hip: disappearance of the femoral head and neck, cystic destruction of the acetabulum, thickening of the synovial membrane with articular effusion.
possible: primitive-bone or synovial tumor, metastatic cancer, bone tuberculosis, or rheumatoid arthritis. The diagnosis of rheumatoid arthritis was rapidly excluded due to the absence of the habitual markers of this disease, as well as the clinical presentation of the disease itself. Tuberculosis of the bone seemed improbable considering the slight reaction to tuberculine intradermally, good general physical condition of the patient, and normal chest X-ray. Despite the normality of the usual tumor markers, as well as acid phosphatases, the radiological images suggested tumoral destruction of the hip. Pelvic computerized axial tomogram (CAT), carried out with a high resolution and soft tissue window, revealed complete destruction of the left femoral head and neck. There was capsular thickening and an articular effusion (Fig. 4). On the right side, the only alteration seen was a posterior pinching of the interarticular space (Fig. 5). In addition, the prostate was hypertrophied and the bladder wall thickened. Intravenous urogram showed a marked prostatic impression, a diverticular aspect of the bladder wall as well as an important post-mictional residue. Hepatosplenic echogram, barium enema, and a colonoscopy to 50 cm were normal. A technetium bone scan showed slight hyperfixation of the tracer at the left hip and the right
Fig. 5: Pelvic CAT scan: on the left side, disappearance of the femoral head. On the right side, posterior articular pinching.
Prostatic hypertrophy.
Pigmented villonodular synovitis of the hip
187
then realized using a Harris cup of 54 mm (Zimmer) and a Landos T 12 prosthesis with a long neck (Landanger) (Fig. 6). Detailed anatomopathological examination showed a tissue with a thick, fibrous synovial wall presenting lymphocytic infiltration of variable intensity. Certain areas were rich in collagen, while others were richly vascularized and populated by fibroblasts. Several giant cells were found as well as numerous deposits of hemosiderine (Fig. 7). In one of the samples, there were agglomerates of foam cells. Thus, the diagnosis of pigmented villonodular synovitis was confirmed. The post-operative evolution was rapid and favorable. Ambulation with total weight-bearing was immediately authorized. The disappearance of pain was practically immediate and functional recuperation of the hip was very rapid. At this time, two years after the surgical procedure, the mobility of the hip is practically normal and the walking perimeter has reached 2 km. There are currently no clinical or radiological signs of local recurrence or involvement of other articulations.
DISCUSSION
Epidemiology Fig. 6: X-Ray of the left hip two years after surgical treatment. knee. The X-ray of the right knee was normal. Thyroid scintigram was normal. Finally, a cystoscopy associated with bladder and prostatic biopsy showed aspecific, chronic cystitis and benign nodular hyperplasia of the prostate. At the conclusion of these examinations, the retained diagnosis was that of a primitive tumor at the hip. Surgical treatment was carried out on December 16, 1987 under general anesthesia. The articular capsule had a normal aspect. Opening of the capsule revealed a thick yellow-grey granulation tissue invading the articular cavity from which the femoral head had disappeared. The synovial membrane was globally thickened. A neo-articulation had formed between the stump of the femoral neck and the roof of the acetabulum where there was localized sinking. The exuberant growth, as well as the synovial membrane, was sent to the laboratory for anatomopathological examination. The extemporaneous examination diagnosed pigmented villonodular synovitis. A total cemented hip arthroplasty was
The annual incidence of pigmented villonodular synovitis was estimated by Myers et al. in their epidemiological study (1) as being approximatively 1.8 cases per million. Except for this study, where Myers observed a higher incidence in the male sex (2/1 ratio), other authors had reported an equal incidence in both sexes. The disease manifests itself primarily in the third and fourth decades, but several cases have been reported in young children as well as in old people (1-4). Pigmented villonodular synovitis can occur at all synovial articulations as well as at the synovial sheaths of the fingers and the hands. It is this latter localization which is the most frequent (57% of all cases according to Rao et al.) (5). Among the larger articulations, the knee is the most often affected (80% of all cases) (1-3, 5-7). The hip is, in some series, the second most affected articulation (5-10% of all cases) followed by the ankle, the wrist, the shoulder, and the foot (1,3,5-7). Rare localizations, such as the vertebral column, have been reported (8). The disease is almost always mono-articular and multi-articular involvement has been described in only approximatively 10 cases.
188
F. Descarnps, E. Yasik, D. Hardy, et.al.
Fig. 7: Pigmented villonodular synovitis: anatomopathological aspect.
Etiopathogenesis Since its description in 1941 by Jaffe et al. (9), numerous hypotheses have been formulated : inflammatory response to an unknown agent (9), disorder of lipid metabolism (1), repeated micro-traumatisms associated with hemarthrosis (1). For Rao et al. (5), the disease resuits from a neoplastic-like proliferation of the fibroblasts and histiocytes of the synovial tissue. Currently, there exists no certainty concerning the etiology of pigmented villonodular synovitis. The pathogenesis of the osseous invasion and the formation of cystic cavities also remain uncertain. For Mc Master et al., invasion of bone tissue begins from an intra-articular lesion, a small cartilagenous erosion at the osteochondral junction (10). For Chung et al., the increase of intra-articular pressure, provoked by tumor growth, could be responsible for erosion of the cartilage (7). For Scott, finally, the tumoral growth reaches the deep tissues by following the trajectories of the nutritional blood vessels of bone tissue (11).
eral years of evolution. Most cases present episodes of pain, accompanied by limitation of movement, articular effusion or progressive tumefaction. Inflammatory signs are usually discrete or, more frequently, absent. Episodes of blocking are typical of involvement of the knee (1,3-5,10,12,13). Howie et al. (14) reported three cases where torsion of a lesion in the knee was responsible for acute pain and blocking. Tumefaction is often evident when the knee, the wrist or the ankle are involved (15,7,14). Often, involvement of the hip is accompanied only by a slight limitation of mobility or even normal mobility (2,3,7,10,15), as was seen in our patient. On the other hand, the evolution of disease was particularly rapid in our patient, and, in addition, the amplitude of the bone destruction was among the most important reported. The absence of pain on the right side as well as the stability of the radiological arthritic signs in the right hip permitted the authors to conclude that there was only unilateral articular involvement. This unilateral involvement has been seen in almost all of the cited cases of this disease.
Diagnosis
Complementary examinations
The evolution is habitually insidious and the diagnosis is usually made often after several months or even sev-
Except for the presence of hamatic articular fluids, the different authors have not been able to demonstrate any
Pigmented villonodular synovitis of the hip
significant anomalies in the different clinical tests carried out. The radiological anomalies of pigmented villonodular synovitis (PVS) are aspecific and can be seen in cases of bone tuberculosis or, more particularly, in synoviosarcoma. The radiological aspect, although highly suggestive of the disease in its evolved forms, does not allow a diagnosis with certainty. The most complete study of radiological anomalies is certainly that by Smith et al. (16), where he distinguished two forms of PVS. The first type is a localized form in which only a portion of the synovial is involved and where radiological anomalies are rarely observed. The second type is a diffuse form where the totality of the synovial is involved and where constant radiological abnormalities occur. The most frequently observed images are: thickening of the synovial tissue, bone erosions or cystic cavities bordered by sclerosis in nonosteoporotic bone without a decrease in the size of the interarticular space as seen in arthrosis. In our patient, older than the average age of most of the reported cases, in addition to these particular images, arthritic type alterations were added which temporarily masked and delayed the diagnosis. In the literature, we found only one patient where a technetium bone scan demonstrated a hyperfixation of the tracer molecule on the affected side (12). But here, the authors stressed the fact that this articulation had already been operated on and presented important arthritic lesions. In our patient, bone scan revealed a hyperfixation of the tracer in the affected hip. The other hip, however, which presented radiological signs of arthrosis, did not excessively fix the tracer molecule. There was also hyperfixation in the right knee of the patient, but, given his advanced age, an arthroscopy to look for lesions of PVS was not performed. In conclusion, it may be said that only anatomopathological examination permits the diagnosis of PVS with certainty. The characteristics of PVS are relatively well defined(i,5,6,9,10,12,17,18). One of the most remarkable aspects of the disease is the high concentration of hemosiderine in the diseased tissues. This could be responsible for the increased radiological density of the affected tissues. In a case of an affected knee, Rosenthal et al. (19) were able to evaluate, by means of a CAT scan, the concentration of iron in the tumoral mass, this being the first reported case in the literature. Although hemosiderine deposits were seen in our patient, their low con-
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centration did not permit us to observe an increase in tissue density with CAT scan. Treatment
Pigmented villonodular synovitis presents many different clinical forms in diverse articulations. Because of its relative rarity, no prospective study has been carried out. The age of the patient, his autonomy and the seriousness of the destructive lesions determine the choice of treatment. In localized forms, local resection of the pathological tissues generally gives excellent results (2-5) with a very low recurrence rate. In more diffuse forms, most authors agree as to the necessity of a synovectomy as complete as possible. However, when synovectomy is the only therapeutic act realized, there is a very high rate of recurrence (46%) (1,2,3). This induces certain authors to directly prescribe radiotherapy in older patients or in cases of recurrence. Unfortunately, this adjuvant therapy is not without secondary effects (carcinogenic effects, articular ankylosis). For Mc Master (10) and Scott (11), synovectomy should be completed with careful curettage of the cystic cavities associated with bone grafts whenever the destructive lesions are not too important. Finally, if the lesions are too evolved, which is often the case in the hip or the shoulder, the authors report good results with replacement arthroplasty (2,3,4,6,7). Arthrodesis remains a debatable alternative in young patients (2,5,6,10). Salliere et al. (4) report satisfactory results in a child treated by synoviorthesis with osmic acid. They, therefore, propose this treatment for the recurrence of articular effusion after surgical synovectomy. In our 73-yearold patient, who presented extensive osteo-articular destruction, replacement arthroplasty of the entire articulation was the only therapeutic option. CONCLUSION Pigmented villonodular synovitis remains a rare illness whose etiopathogenesis is still uncertain. Its diagnosis remains one of exclusion and the therapeutic modalities must be decided according to the localization and the importance of the articular destruction, as well as the age of the patient.
Acknowledgements : The authors would like to thank Mrs Marianne Fouss for her secretarial assistance.
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Received: 26 February 1990; Revision-accepted: 31 August 1990 Correspondence to: Dr. Ph. DELINCE, H6pital Saint-Pierre, Rue Haute 322, B-1000 Bruxelles, Belgique.
