P IGMENTED villonodular synovi tis is not a new disease, as it was first described

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VOL. 110, No. DOUBLE-CONTRAST ARTHROGRAPHY IN THE DIAGNOSIS OF PIGMENTED VILLONODULAR SYNOVITIS OF THE KNEE* By LT. COLONEL RICHARD D. WOLFE, USAF, MC,f and MAJOR VINCENT J.

preoperatively, especially when it involves the knee.

1 VOL. 110, No. DOUBLE-CONTRAST ARTHROGRAPHY IN THE DIAGNOSIS OF PIGMENTED VILLONODULAR SYNOVITIS OF THE KNEE* By LT. COLONEL RICHARD D. WOLFE, USAF, MC,f and MAJOR VINCENT J. GIULIANO, USA, MC P IGMENTED villonodular synovi tis is not a new disease, as it was first described by Simon in i86, but even today, more than ioo years later, it is infrequently correctly diagnosed preoperatively, especially when it involves the knee. With the introduction of opaque contrast and then double-contrast knee arthrography, it would seem that more of these cases would be identified prior to surgery, but we were only able to find a total of 2 cases in which knee arthrography was used and was helpful in establishing the diagnosis. Seven of these used the double-contrast method, with 5 cases published by Bessler and Ruttiman and I by Czekala,1#{176} while Butt and McIntyre7 reported the only case in the English language literature. Others, each reporting single cases utilizing only positive contrast material, were Aye et al.,1 Rein et al.22 and Lindblom. 9 Brashear5 mentions i case in which the diagnosis was suggested by pneumoarthrography, while Keliki an and Lewis 7 presented an equivocal case studied also with pneumoarthrography. A recent case in which the preoperative diagnosis of pigmented villonodular synovitis of the knee was made with doublecontrast arthrographv motivated us to neport the 2 cases we have in the hope of stimulating interest in its use in the diagnosis ofsynovial tumors. REPORT OF CASES CASE I. A 25 year old white male was transferred to \Vilford Hall USAF Medical Center after initial care at Schilling AFB, Kansas. He had been well until 4 months prior to admission LACKLAND AFB, TEXAS when he noted swelling of his right knee. No other joints were involved and there was no history of trauma. The condition was diagnosed asprobablestrain,and rest and heat resulted in partial relief of the swelling. This recurred and when it became quite distended there was some pain with motion. A mass was easily palpable but was thought to be a lipoma. At surgery, it was thought to represent a muscle mass but the inadequate biopsy revealed only normal muscle and he was referred to our facility. On admission there was a recent but wellhealed lateral incision with diffuse swelling of the entire joint, most pronounced in the area ofthe suprapateilar bursa and laterally. Flexion was limited to 70#{176}by pain. The knee was otherwise stable and no local heat or erythema was noted. All laboratory studies, including sedimentation rate X 3, were normal. Plain roentgenograms of the knee revealed a normal bony architecture without osteoporosis and with the joint space maintained. A soft tissue mass was seen in the area of the suprapatellar bursa. Dou ble-con trast arthrography was performed, the details of which are not in the record, and these represen tative roen tgenograms were obtained (Fig. i, A-D). Aspiration at the time of arthrography revealed a frankly bloody effusion which showed no growth on culture. Although the roentgenograms are felt to be diagnostic of pigmented villonodular synovitis, this was not fully appreciated preoperatively for it was thought that this most likely represented a rhabdomyosarcoma with invasion of the synovium. At surgery an extremely large, fungating mass occupied the entire knee joint and extended into the thigh laterally. A total synovectomy was accomplished and this was followed by radiation therapy with s,6oo rads over a 2 week period. Pathologic examination S From the Department of Radiology, Wilford Hall USAF Medical Center, Lackland Air Force Base, Texas. t Present Address: Assistant Chief, Diagnostic Radiology Department, Mount Zion Hospital and Medical Center, s6oo Divisadero Street, San Francisco, California. Chief, General Medicine Service, Brooke Army Medical Center, Fort Sam Houston, Texas. 793

794 Richard D. Wolfe and Vincent J. Giuliano DECEMBER, 1970 revealed I I 5 grams of tissue, the largest piece being sx8xi. cm. with a shiny brown surface.

The postoperative recovery was slow and at discharge 4 months later he had a fibrous ankylosis of the knee with weakness and loss ofmotion. Further follow-up has not been possible. CASE II.

2 794 Richard D. Wolfe and Vincent J. Giuliano DECEMBER, 1970 revealed I I 5 grams of tissue, the largest piece being sx8xi. cm. with a shiny brown surface. Thehistologic appearance was quite typical of pigmented villonodular synovitis. The postoperative recovery was slow and at discharge 4 months later he had a fibrous ankylosis of the knee with weakness and loss ofmotion. Further follow-up has not been possible. CASE II. A 23 year old white female had noticed recurrent episodes of swelling and stiffness of the right knee for approximately year. She was in otherwise good health with no other joints involved. She recalled having fallen on her knees several times while ice skating prior to the onset of symptoms. On admission to Brooke Army General Hospital the only positive physical findings were limited to the right knee, which was slightly warm and diffusely tender, but only mildly so. There was a large suprapatellar effusion, but no definite mass could be palpated, even after aspiration of a large amount of blood-tinged synovial fluid. Laboratory studies were all normal including the sedimentation rate, the slide latex test for rheumatoid factor and the fluorescent antinuclear antibody test. Plain roentgenograms of the knees were negative except for an effusion. A double-contrast arthrogram was obtained utilizing 10 cc. of 75 per cent hypaque and 20 cc. of room air. The roentgenognams were diagnostic of a diffuse pigmented villonodular synovitis (Fig. 2, 1-D). At surgery a boggy synovial mass was identified in the suprapateliar region and a total synovectomy was accomplished through medial parapatellar and lateral posterior incisions. The mass was attached to the patella but not to the collateral ligamen ts. Pathologic examination revealed villous proliferation and hemosideninladen macrophages, considered typical of pigmented villonodular synovitis. No postoperative radiation therapy was given. DISCUSSION In 1941 Jaffe et al.14 reviewed the confusing literature and their own cases and recognized common gross and histopathologic characteristics which allowed them to group under a common name all of these processes which previously were reported under various names. They designated the condition as pigmented villonodular synovitis, bursitis or tenosynovitis and this has remained as the accepted term. The condition may be nodular or diffuse. If one excludes the nodular and quite characteristic phalangeal lesions, the vast majority are located in the knee and most of these are of the diffuse type. The less common nodular type of the knee is rarely if ever diagnosed prior to surgery, but instead presents as an internal derangement and frequently has episodes of locking. There are no reports of the use of arthrography in this localized type and it is doubtful ifit would be helpful other than in a negative manner. If no meniscal injuries were demonstrated then one would think of other possibilities for the etiology of the patient s symptoms. Gehweiler and Wilson 2 collected from the literature 300 cases of diffuse pigmented villonodular synovitis. The knee was by far the most common location and was then followed by the hip, ankle, tarsus, carpus, elbow and shoulder, in that order. They reported only the third and fourth cases of biopsy proven biarticular involvement with one of their cases having both knees involved and the other the right knee and right hip. Greenfield and Wallace13 in io presented a case with both knees, and Byers et al.8 in 1968 reported i case with both ankles involved. Kelikian and Lewis 7 in 1949 reported 3 siblings with biarticular involvement but there was no proof and these do not appear to represent this entity. Friedman and Schwartz reported a case with both knees affected but the involvement of the second knee occurred 10 years after the first and there was no biopsy of the second knee. Lewis 8 in 1947 described the typical patient with the diffuse process as a young adult with monarticular involvement and with an excessive amount of synovitis which may be smooth in outline and homogeneous in density but is especially diagnostic when it is nodular. He emphasized joint space symmetry with no indication of cartilage abnormality, normal appearing bones without osteoporosis and no

3 VOL. iso, No. Pigmented Villonodular Synovitis of Knee 795 I IG. I. Case I. DifFuse pigmented villonodulam synovitis of might knee. (ii) Soft tissue swelling of suprapatellam area laterally. (B) Anteroposterior view. (C) External rotation view. (D) Lateral view. Enlarged suprapatellar bursa almost completely filled with mass composed of recesses and villous pmolifemations. thigh or leg atrophy. When one adds a lack ofcalcification in the mass and a chronicity ofsymptoms to this, one has criteria which still apply to the vast majority of these patients. McMaster2 in 1960 reported 6 cases with bone changes. Three of these were in the hip, where previous cases had been reported by Breimer and Fneibergen,6 while his 3 cases in the knee were the first reported with bone changes in that joint. Since that time a few more cases have shown relatively nonspecific changes, although, unlike the hip, involvement of the knee remains the exception rather than the rule. There has been much discussion as to how the bone becomes involved but that is beyond the scope of this paper Neither of our pati en ts demons tnate(l noen tgenographi c evidence ofbone abnormality. ETIO LOGY Tile etiology of this process remains obscure, although most agree with Jaffe that it represents a benign inflammatory reaction to some as yet unidentified agent.

79() Richard D. Wolfe and Vincent J. Giuliano DECEMBER, 1970 11G. 2.

(A) Upright anteropostenior view. ( B) Internal rotation view.

Moderate enlargement of suprapatellar bursa with filling defects coated

4 79() Richard D. Wolfe and Vincent J. Giuliano DECEMBER, G. 2. Case II. Diffuse pigmented villonodular synovitis of right knee. (A) Upright anteropostenior view. ( B) Internal rotation view. (C) Lateral view. (D) Cross-table lateral view. Moderate enlargement of suprapatellar bursa with filling defects coated with positive contrast material. Note value of upright and crosstable lateral views to differentiate from bubbles or frothy fluid.

hemophiliac with findings closely simulating but not identical to that of pigmented villonodular synovitis.

5 VOL. Iso, No. Pigmented Villonodulan Synovitis of Knee 797 Many have incriminated bleeding into the joint, although only Dahlin (as cited by Chung and James9 in a personal communication) has seen a case in a hemophiliac with findings closely simulating but not identical to that of pigmented villonodular synovitis. Many interesting experimental studies have been only partially successful in producing lesions somewhat resembling this entity Wright27 in 1951 felt that this represented a neoplasm related to the ordinary malignant synovioma and he insisted that benign giant cell synovioma is a more appropriate designation. This was not widely accepted, although recently Jones et al. 6 again suggested this possible relationship. They based this conclusion on an article by Bliss and Reed3 in which cases of malignant giant cell tumor of tendon sheaths, 2 with metastases, were reported. There had been no previous cases suggesting malignancy or metastases and there have still been none of the diffuse type involving the larger joints. Bobechko and Kostuik4 recently reported 3 children with hemangiomas of the synovium. They suggested that these 2 conditions may be related and recommended arteniography in all adults with this diagnosis. Suffice it to say that the true etiology of this condition has not been definitely established! ARTHROGRAPHY AND ARTERIOGRAPHY Careful arthrography should be almost uniformly successful in demonstrating the fungating synovial lesions present. Some authors, however, have not found it of value, although in most cases only air was used. 3 5 We favor the double-contrast method for all arthrographies but especially when a synovial lesion is suspected. These will demonstrate the tremendous increase in the size of the articular cavity, will show the joint effusion and will demonstrate the synovial villi projecting into the suprapatellar bursa and posterior knee pouches. The coating by the positive contrast material and the distention from the air render an easy diagnosis possible when proper roentgenograms are obtained. A cross-table lateral and an upright views are of utmost importance in demonstrating these synovial abnormalities. During the actual injection of the positive contrast material in our second patient, she complained of pain and even cried, although she did not show a low threshold for pain when the needle was inserted. In several hundred previous studies done by one of the authors (RDW) this was never seen. Usually there is no pain but only slight discomfort from the mild distention. Even the burning encountered if some of the contrast material extravasates is never as severe as this was. It is probable that the large increase in surface area with its abnormal synovial lining is responsible for this irritative phenomenon and this may be a diagnostic point to watch in future arthrograms. Unfortunately there was no written comment regarding the arthrogram in the first patient. Arteniography was performed by Rein et al.22 but they concluded that the malignant type vascular changes seen rendered this oflittle diagnostic assistance. Its greatest value was in delineating the extent of the tumor. TREATMENT Total synovectomy with or without postoperative irradiation is generally accepted as the treatment of choice. Byers et al.8 in a large series of their own cases and in reviewing the literature found an extremely poor result with extensive synovectomy of the knee with a recurrence rate of 46 per cent. A complete cure with total synovectomy of the knee was obtained in only 17 per Cent. In 5 cases in which biopsy alone was done no further deterioration occurred. They treated 8 patients with radiation therapy and, although all showed regression of bone and soft tissue lesions, only 2 could be considered as having satisfactory results. Fortunately not all of the results in the literature are so dismal.

6 798 Richard 0. Wolfe and Vincent J. Giuliano DECEMBER, 197( SUMMARY Two patients with a histologically proven diagnosis of pigmented villonodular synovitis of the knee are reported. This diagnosis should be considered and an arthrogram obtained when a young adult presents with chronic swelling and stiffness of a single joint with minimal if any pain and with bogginess of the synovium. The condition is even further suspected when aspiration reveals a moderately large quantity of dark blood-containing effusion, and plain roentgenograms show a normal joint other than the effusion and occasionally some nodularity of the suprapatellar area. When the double-contrast method is used, preoperative arthrography should result in a diagnosis, as was the case in the 2 patients reported. Richard D. Wolfe, M.D. Mount Zion Hospital and Medical Center s6oo Divisademo Street San Francisco, California The authors wish to thank MSgt Eugene Szwarc who prepared the photographic prints and Mrs. Vera Ralston for help with the manuscript. REFERENCES I. AYE, R. C., DORR, T. W., and DREWRY, G. R. Arthnography of knee in office practice. Radiology, 1963, 8o, BESSLER, W., and R#{220}TTIMAN, A. Die R#{246}ntgensymptome den Synovitis villosa des Kniegelenks. Fortschr. a. d. Geb. d. R#{246}ntgenstrahlen U. d. Nuk/earmedizin, s 963, 99, BLISs, B. 0., and REED, R. J. Large cell sarcomas of tendon sheath; malignant giant cell tumors of tendon sheath. Am. 7. C/in. Path., 1968, 49, I. 4. BOBECHKO, W. P., and K05TuIK, J. P. Childhood villonodulan Synovitis. Canad. 7. Surg., 1968, II, BRASHEAR, H. R. Pigmented villonodular synovitis. South. M. 7., 3956, 49, BREIMER, C. W., and FREIBERGER, R. H. Bone lesions associated with villonodular synovitis. AM. J. ROENTGENOL., RAD. THERAPY & NU- CLEAR MED., 1958, 79, Burr, W. P., and MCINTYRE, J. L. Doublecontrast anthrography of knee. Radiology, 1969, 92, BYERS, P. D., Corron, R. E., DEACON, 0. W., LOWRY, M., NEWMAN, P. H., SsssoNs, H. A., and THOMSON, A. D. Diagnosis and treatment of pigmented villonodulan synovitis. 7. Bone & Joint Surg., 5968, so-b, CHUNG, S. M., and JANES, J. M. Diffuse pigmented villonodular synovitis of hip joint. 7. Bone & 7oint Surg., 1965, 47-A, CZEKALA, Z. Double contrast nadiologic diagnosis of pathological changes of certain pants of knee joint. Polish Rev. Radiol. & Nuclear Med., 5966, 20, I I. FRIEDMAN, M., and SCHWARTZ, E. E. Irradiation therapy of pigmented villonodulan synovitis Bull. Hosp. 7oint Dis., 5957, i8, GEHWEILER, J. A., and WILsoN, J. W. Diffuse bianticular pigmented villonodular synovitis. Radiology, 5969, 93, GREENFIELD, M. M., and WALLACE, K. M. Pigmented villonodular synovitis. Radiology, 5950, 54, JAFFE, H. L., LICHTENSTEIN, L., and SUTRO, C. J. Pigmented villonodulan synovitis, bunsitis, and tenosynovitis. Discussion of synovial and bunsal equivalents of tenosynovial lesion commonly denoted as xanthoma, xanthogranuloma, giant-cell tumor on myeloplaxoma of tendon sheath, with some consideration of this tendon sheath lesion itself. Arch. Path., 1945,31, Is. JELASO, D. V. Positive contrast anthrography of knee. AM. J. ROENTGENOL., RAD. THERAPY & NUCLEAR MED., 1968, 103, s6. JONES, F. E., SOULE, E. H., and COVENTRY, M. B. Fibrous xanthoma of synovium (giantcell tumor of tendon sheath, pigmented nodular synovitis). 7. Bone & Joint Surg., 5969, z-a, KELIKIAN, H., and LEWIS, E. K. Anthrognams. Radiology, 1949, 52, s8. LEWIS, R. W. Roentgen diagnosis of pigmented villonodular synovitis and synovial sarcoma ofkneejoint. Radio/ogy, 1947, 49, LINDBLOM, K. Anthnography of knee: noentgenognaphic and anatomical study. Acta radiol., 1948, Suppl. 7, MCCOLLUM, D. E., MussER, A. W., and RHANGOS, W. C. Experimental villonodular synovitis. South. M. 7., 5966, 59, MCMASTER, P. E. Pigmented villonodular synovitis with invasion of bone. 7. Bone & 7oint Surg., I 960, 42-A, s70-i I REIN, B. I., BILODEAU, L. P., and JOHANSON, P. Anthnognaphy and anteniography in pigmented villonodulan synovitis of knee. AM. J. ROENT-

7 \ OL 110, No. Pigmented Villonodular Synovitis of Knee 799 GENOL., RAD. THERAPY & NUCLEAR MED., 5964, 92, SANDERUD, A. Pigmented villonodular synovitis. Acta orthop. scandinav., 5954, 24, Scorn, P. M. Bone lesions in pigmented viilonodulan synovitis. 7. Bone & 7oint Surg., 1968, 50-B, SINGH, R., GREWAL, D. S., and CHAKRAVARTI, R. N. Experimental production of pigmented villonodulan synovitis in knee and ankle joints of rhesus monkeys. 7. Path., 1969, 98, SMITH, J. H., and PUGH, D. G. Roentgenographic aspects of articular pigmented villonodular synovitis. AM. J. ROENTOENOL., RAD. THERAPY & NUCLEAR MED., 5962, 87, 1146-I WRIGHT, C. J. Benign giant-cell synovioma: investigation of 85 cases. Brit. 7. Surg., 1951, 38, YOUNG, J. M., and HUDACEK, A. G. Experimental production of pigmented villonodular synovitis in dogs. Am. 7. Path., 5954, 30,

8 This article has been cited by: 1. J. Bruns, Th. Schubert, G. Eggers-Stroeder Pigmented villonodular synovitis in children. Archives of Orthopaedic and Trauma Surgery 112:3, [CrossRef] 2. W. P. Butt, G. Hardy, S. J. Ostlere Pigmented villonodular synovitis of the knee: computed tomographic appearances. Skeletal Radiology 19:3, [CrossRef] 3. Amy Beth Goldman Some miscellaneous joint diseases. Seminars in Roentgenology 17:1, [CrossRef] 4. William P. Docken Pigmented villonodular synovitis: A review with illustrative case reports. Seminars in Arthritis and Rheumatism 9:1, [CrossRef]

LOCALIZED NODULAR SYNOVITIS OF THE KNEE: A REPORT OF TWO CASES WITH ABNORMAL ARTHROGRAMS*

MARcH, i6 LOCALIZED NODULAR SYNOVITIS OF THE KNEE: A REPORT OF TWO CASES WITH ABNORMAL ARTHROGRAMS* ABSTRACT: By THOMAS G. GOERGEN, M.D.,t DONALD RESNICK, M.D.,t and GEM NIWAYAMA, M.D4 SAN DiEGO, CALIFORNIA