CHAPTER 52 PIGMENTED \TLLONODUIAR SYIIO\TTIS : Review of the Literature And Four Cases Robert B, Weinsteim, DPM Stepban J. LaPointe, DplI, pbD Mark Greenbauru, DPM Craig Camasta, DPM Thomas D. Caim, DPM Marc Sarnottt, DPM Introduction tumor of the synovial membrane, benign fibrous histiocyoma, chronic hemorrhagic villous synoviris, Pigmented villonodular synovitis (pVS), firsr fibro-hemangioma, fibro-hemosideric sarcoma, described by Jaffe et all in 1947, is a benign giant-cell fibro-hemangioma, giant-celi tumor of proliferation of synovium of unceftain etiology. the tendon sheath, myeloplaxoma, sarcoma Speculations have been made that suggest pVS may fusigigantiocellulare, synovial xanthoma, synovial be due to neoplastic process, inflammatory processl fibroendothelioma, synovial endothelioma, and repeated trauma, or metabolic disorder. Three forms xanthomatous giant-cell tumor.ar In their 7)47 paper, of PVS have been described depending on synovial Jaffe et al' proposed the terms pigmented involvement. These include nodular or localized pVS, villonodular synovitis, tenosynovitis and bursitis, diffuse PVS, and Giant Cell Tumor of the Tendon depending on the manifestation of the lesion. Sheath (GCTTS). Although different macroscopically Granowitz et a16 further subciassified the lesions these lesions appear to have the same histogenesis. based on clinical presentation into the more comlnon PVS usr-rally presenrs in the third to fifth decade of life diffuse form and the nodular, or localized form. and shows no predilection towards either sex. Patients generally present with monoarticuiar Etiology swelling out of proportion to discomfort and limitation of motion with no history of trauma to the The true etiology of pigmented villonodular synovi- area. Imaging studies including Roentgenography, tis is unknown. Speculation has centered around a CT particularly and MRI may play an impofianr role few theories, including neoplastic or inflammatory in the diagnosis and treatment of PVS. Recognition of processes, metabolic disorder, and chronic trauma. the clinical pVS and radiographic pathology of can Recurrence rates, high mitotic activity, and a distinct lead to earlier and biopsy possibly less infiltration of difference between lesional and healthy tissue the lesion.' Because pigmented villonodular synovitis suggest a neopiastic process.T Ray et al. have can cause joint bone and destruction and has a reported c),,togenetic clonaliry indicating origination tendency to recur, treatment consists of complete from a common stem cell in a case of pvS. This excision of the lesion and possible adjunctive hypothesis has been given further substantiation radiation therapy. from reports of metastatic disease originating from afiicular PVS.e 1'z PVS has been shown to have Terminology increased uptake of Technitium-99 without gallitm-57 citrate uptake which also lends evidence Chassaignac was the first to repofi a case of fibrous to a hyperplastic or neoplastic origin.13 However, xanthoma of synovium.3 prior to 794t, long-term survival rates and clinical course of pigmented villonodular synovitis had been described disease are not representative of a recurrent in the literature by various other names. These neoplasm. Inflammation, as discussed byJaffe et a1,, included benign or malignant polymorphocellular continues to be a prevailing theory. Histologic CFLAPTER 52 299 evidence has been reported that supports this Joint aspiration may yield a translucent straw-ye1low theory.'a Galloway et al" believed the lesions to be to bloody red or brown fluid. Bone involvement has a result of abnormal lipid metabolism. Hirohata et been repofied as a single or confluent cltister of al'o adr.anced this idea, implicating the foam cel1 as cyst-like areas bordered by a thin margin of the primary pathologic cell responsible for aL scierosis, mimicking a primary bone neoplasm.'6'" abnormal cholesterol and phospholipid synthesis. Classic microscopic description of the lesion Several studies have been carried ollt to examine includes subsynovial proliferation of large round, trauma as the etiologic agent.l Myers et al"' polyhedral or spindle shaped cells.* This micro- uncovered a somewhat common history of repeated scopic appearance along with immunophenotype trauma in patients who have PVS. Although a history studies present evidence that PVS is of synovial cell of trauma has been reported in a minoriry of cases, origin.'E The spindle shaped cells tend to appear in trauma remains a possible etiology for PVS.'S''9 areas of marked fibrogenesis.'e Some combination Interestingly, Wendt et al'o have sr,rggested that there of histiocyes, fibroblasts and hemosiderin-laden may be a genetic contribution to the development of macrophages and foam ce11s also has commonly this lesion in their report on a case of PVS in two been described.6 A distinct feature of PVS is the siblings and in multiple generations. intracellular and extra-cellular accumulation of hemosiderin and conseqLlent diffuse pigmentation Clinical presentation of the lesion site. PVS occurs most commonly in the knee, followed Imaging studies by the hip, ankle, carpal and tarsal bones, shoulder and the e1bow. PVS has been reporled in patients On plain film radiographs, two ollt of three cases ranging from 11 to 68 years o1d. PVS occurs in the show some sort of soft tissue density.'o third to fifth decades of life, t'ith approximateiy Approximately 50 percent shou,' some osseous equal distribution among males and females. The changes.3'r3' Excluding the knee greater than B0 mean age at presentation is in the fourth decade.'' percent of cases have demonstrated bony involve- The majority of patients usually present with symp- ment3O Classic findings of PVS include toms including marked localized monoafiicular monoafiicular involvement, presetwation of carti- swelling out of proportion to a mild or du1l pain. A lage, synovial swelling, absence of calcification palpable mass can sometimes be felt. Multiple joint within the swel1ing, and normal bony minetaliza- involvement has been reported.5"''a The time of tion of the affected ioint.3':Joint space narrowing, symptom onset to correct diagnosis of PVS of the just as polyarticular involvement, is an uncommon knee, wrist and ankle is approximately 10 months.'5 finding. If there is bone involvement one or more Laboratory stlldies including hemoglobulins, intraosseous cysts may be apparent, each delin- CBCs, sedimentation rates, urinalysis, Gram's stain eated by a thin rim of sclerosis and surrounding a and serum cholesterol are almost always normal.'e''5 bone of normai density." These defects may be lobulated. PVS may be difficult to distinguish from Clinical features tuberculosis, rheumatoid or osteoarthritis.5 Most authors conclude that radiographs alone should not pys.r'::3+ On gross examination of localized PVS, the be used in confirming the diagnosis sf synovium may appear from orange-yellow to red- Rosenthal et a!" have reported increased attenuation on computed tomography (CT) scan of dish-brown. The lesion is rypica1ly lobulated. The villi are often long and appear as tangled, matted a PVS lesion. The increased absorption is believed growth. masses, demonstrative of a istraggly-beardi eppear- to be due to the high iron content of the ance. Diffuse PVS may present individual or Afihrographic stucly of diffuse PVS may reveal project into an clustered nodules along the synovium with promi- multiple irregular filling defects that enlarged joint and are fixed to the joint capsule.a'3' nent pigmentation ranging from yellow tc) i red-brown.'The pigmented tissue may extend into Johansson et af report that athrograms provide are sub-sy'novial structures and the joint capsule." The little if any diagnostic aid. Arthrograms lesion. synovial membrane may be thickened and rubbery.' generally not indicated for this 300 CHAPTER 52 Angiography, although nonspecific, may be matosis, rheumatoid afihritis, hemangioma, chronic utilized for defining the extent of the lesion, dtie to traumatic synovitis, and fatty synovial tumor); the increasecl vascularity, presence of nunrerolls therefore, biopsy is necessary for proper arteriovenous shunts and irregular vessels com- diagnosis.3'? When associated witl-r hemorrhagic or monly seen w-ith Pvq.-rt,3(, The presentation of fine xanthochromatic joint efftrsions these hypointense caliber smooth walled arteries, Iate phase contrast lesions strongly suggest PVS.46 pooling in dilated vascular spaces ancl early visual- ization with this study slrggests synovial Treatment hemangioma rather than PVS.3. Rosenthal et al,rj have reported a case PVS of with little vascularity. Treatment generally depends on the type of lesions Lesions of this type are beiieved to be more present. The loczrlized form of PVS is sufliciently matllre, ancl demonstrate fibrosis as a more impor- treated by local excision of the tumor. Curettage of tant tisslre component.3s bone is performecl if there is bony involvement. Ultrasonograms can be utilized to evaluate the Recurrence rates are 1ow, and these patients anatomic dimensions and structure, density, and generally have a good prognosis. Bofion et a|; have vasculzrrity of PVS lesions. The use color of doppler successfr-rlly Lltilized arthroscopic synovectomy for a sonography has revealed hypoechoic synovial pro- lesion localized in the first metatarsophalangeal liferation and hypervascularity of a lesion involving joint. For afihroscopic approach to treating pVS the :rnkle joint.3e Ultrasouncl may also be r_rsefttl