CALCIFYING APONEUROTIC FIBROMA : A REPORT OF THREE CASES

CASE REPORT CALCIFYING APONEUROTIC FIBROMA : A REPORT OF THREE CASES N. K. SFEROPOULOS, R. KOTAKIDOU 1 Three cses of clcifying poneurotic firom in n 8-yer-old oy, 43-yer-old womn nd 48-yerold mn re presented. The lesions involved the sustnce of the plmris longus tendon, the lterl spect of the foot t the se of the fifth mettrsl nd the fifth digit of the foot, respectively. The clinicl nd rdiologic findings, including ultrsound nd computed tomogrphy, re descried. Histologic fetures included chondroid tissue nd clcifiction within res of prolifertive plump firolsts in the younger ptient, while in the two dults the lesions were more shrply defined showing incresed clcifiction of dense connective tissue with less pronounced cellulrity nd no evidence of chondroid differentition. Keywords : clcifying poneurotic firom. Mots-clés : firome ponévrotique clcifint. INTRODUCTION Clcifying poneurotic firom (CAF) is n uncommon ut well-chrcterized enign firous tumor. It usully presents s slowly growing mss ttched to tendon or poneurosis commonly in the extremities of children nd young dults. Rpidity of growth seems to slow down, while tumor clcifiction ppers to increse with ge. The lesion hs strong propensity for locl invsion, nd the recurrence rte is pproximtely 50%. However, complete excision is dvisle when this cn e ccomplished without functionl compromise. Otherwise, more conservtive surgicl pproch is dvised (1, 4, 5). Three cses re presented illustrting the clinicl, rdiologic nd histologic fetures of this disese process. Cse 1 CASE REPORTS An 8-yer-old oy ws dmitted for evlution of firm, moile mss tht hd developed on the volr spect of his left wrist 18 months previously. The lesion hd grown recently nd cused mild pin nd tenderness more pronounced with wrist extension. There ws no history of trum nd no locl sign of infection. Routine lood tests nd plin rdiogrphs were norml. Ultrsound reveled sucutneous solid mss. Under generl nesthesi trnsverse incision ws mde cross the volr spect of the wrist etween flexor creses. The tumor ws locted within the sustnce of the plmris longus, so the tendon ws excised to mcroscopiclly helthy orders. Histologic exmintion indicted loulted nd poorly mrginted tumor mesuring 11 6 5 mm. There ws diffuse firolstic growth with liner rrngement of the rounded firolsts nd res of spotty clcifiction surrounded y chondrocytes. Ft infiltrtion s well s remnnts of tendon fiers were lso found (fig. 1). There ws no sign of nucler typi nd mitotic ctivity. Two yers lter no locl recurrence ws detected. Deprtments of Orthopedic Surgery nd 1 Pthology, G. Gennimts Hospitl, Thessloniki, Greece. Correspondence nd reprints : N. K. Sferopoulos, P. Ppgeorgiou 3, 546 35, Thessloniki, Greece. Act Orthopædic Belgic, Vol. 67-4 - 2001

CALCIFYING APONEUROTIC FIBROMA 413 Fig. 1. Cse 1. The lesion ws not well circumscried. It included ft infiltrtion nd remnnts of tendon fiers (). Plump firolsts nd focl clcifiction were lso evident (). Fig. 2. Cse 2. The lesion showed very well-delineted mrgins nd extensive clcifiction (). Myofirolst-like cells were noted round clcified res (). Cse 2 A 43-yer-old womn ws referred for 2-cm, nontender tumor in the lterl spect of the foot t the se of the fifth mettrsl. Since first noted, out 15 yers previously, it hd grown slowly nd cused some discomfort with shoes. There ws no history of trum. Routine lood tests were norml. Rdiogrphs showed clcified lesion close to the underlying one. Ultrsound evlution indicted sucutneous firous mss with diffuse hyperechoic res representing clcifiction. It ws removed under locl nesthesi. The lesion ws well-defined mss djcent to the peronel tendon. The histologic picture ws chrcterized y hevily collgenized component exhiiting extensive clcifiction. The cellulrity ws low with foci of myofirolst-like cells. There ws no sign of chondroid differentition (fig. 2). Nucler typi, mitotic ctivity nd locl tissue destruction were not encountered. Three yers lter there ws no recurrence. Cse 3 A 48-yer-old mn cme for evlution of 2-cm mss of the fifth digit of the right foot. It hd ppered out 12 yers previously, hd grown

414 N. K. SFEROPOULOS, R. KOTAKIDOU c Fig. 3. Cse 3. Extensive clcifiction of the lesion with n pprent clevge plne from the underlying one ws noted on rdiogrphs () nd CT-scn (). Histology reveled hevily collgenized component contining focl clcifiction resemling psmmom odies (c). slowly, nd cused some pin nd tenderness recently. Rdiogrphs of the lesion showed clcified lesion close to the proximl interphlngel joint ut with no ony destruction (fig. 3). A computed tomogrphy indicted hevily clcified mss on the plntr nd lterl spect of the fifth toe entirely seprted from one (fig. 3). The lesion ws removed under locl nesthesi. It ws firm, well-circumscried mss djcent to the flexor tendons. Microscopiclly, the mss consisted of dense firous strom with extensive foci of clcifiction. There ws no evidence of crtilginous metplsi (fig. 3c). After 2 1 / 2 yers the ptient hd no symptoms. DISCUSSION Clcifying poneurotic firom (CAF) is usully found superficilly in sucutneous tissue or in deeper musculofscil nd prskeletl tissue, while n intrtendinous lesion hs only een reported once in n dult (7). The ppernce of this firolstic tumor within the sustnce of the plmris longus tendon in our younger ptient my well indicte tht even in children tendon cn e involved prt from ft, fsci or poneurosis. The most constnt clinicl findings include slow-growing, nontender, firm, moile mss less thn 3 cm in dimeter in the distl portion of the extremities in children nd dolescents (4). However, there is only single report of CAF involving digit of the foot (9). Rdiogrphic fetures re lso nonpthognomonic. No clcifiction or only smudge-like rdiopcities my pper initilly. However, lesions tht hve een present for yers my exhiit lrge clcified res (1). Ultrsound exmintion excludes the more likely dignosis of gnglion indicting solid mss minly firous with foci of clcifiction. A computed tomogrphy lso revels clcified rther thn ossified lesion with visile clevge plne from one, which my led to the dignosis of pnniculitis ossificns, extrskeletl or prostel chondrom (1, 4, 5).

CALCIFYING APONEUROTIC FIBROMA 415 Definite dignosis is lwys sed on histologic findings nd sometimes on immunohistochemicl tests nd ultrstructurl studies (5, 6). A typicl microscopic picture ppered in our younger ptient. It ws mnifested s loulted mss with irregulr orders demonstrting plump firolsts, foci of clcifiction, nd ptches of chondroid differentition. Therefore, cler distinction from infntile or juvenile forms of firomtosis, firous hmrtom of infncy nd monophsic firous-type of synovil srcom could e esily mde (4). In our dult ptients histologic findings were lmost identicl. In oth cses extensive degenertive chnges were encountered i.e. firosis, clcifiction. Differentil dignosis from prostel osteochondrom nd extrskeletl chondrom ws sed on solute lck of crtilge formtion. On the other hnd, pnniculitis ossificns (firo-osseous pseudotumor) exhiits distinct minerliztion pttern with firolstic prolifertion showing vrying degrees of typi, while prostel (nodulr) fsciitis cn e redily distinguished y the presence of plump firolsts within myxoid ckground (3,5). Differentil dignosis from clcified soft tissue leiomyom is more complicted. In deep soft tissue leiomyoms regressive chnges re quite common, nd clcium my lso e lid down in distinct spherules reminiscent of psmmom odies. However, the min histologic chrcteristics of leiomyom i.e. cells with elongted, olong, fusiform, lunt-ended nuclei s well s prolifertion of smooth muscle fiers (2) were not found in our ptients. On the other hnd, the ppernce of cells closely resemling myofirolsts surrounding the clcified res is comptile with CAF (6, 8). The dignosis of CAF ws lso supported y recent studies where older lesions ppered well-demrcted nd less cellulr, with incresed collgen deposition etween the firolsts nd with more prominent degree of clcifiction suggesting mturtion of the process. Finlly, chondroid foci, lthough typiclly present, re not solute prerequisites for the dignosis of CAF (1, 4, 5). REFERENCES 1. Bogumill G. P., Fleegler E. J. Tumors of the Hnd nd Upper Lim, Churchill Livingstone, Edinurgh, 1993, pp. 239-240. 2. Bulmer J. H. Smooth muscle tumours of the lims. J. Bone Joint Surg., 1967, 49-B, 52-58. 3. Dupree W. B., Enzinger F. M. Firo-osseous pseudotumor of the digits. Cncer, 1986, 58, 2103-2109. 4. Enzinger F. M., Weiss S. W. Soft tissue tumors, Mosy, St Louis, 1995, pp. 256-261. 5. Fetsch J. F., Miettinen M. Clcifying poneurotic firom : A clinicopthologic study of 22 cses rising in uncommon sites. Hum. Pthol., 1998, 29, 1504-1510. 6. Iwski H., Kikuchi M., Eimoto T., Enjoji M., Yoh S., Skuri H. Juvenile poneurotic firom : An ultrstructurl study. Ultrstruct. Pthol., 1983, 4, 75-83. 7. Moskovich R., Posner M. A. Intrtendinous poneurotic firom. J. Hnd Surg. 1988, 13-A, 563-566. 8. Nvs-Plcios J. J. The firomtoses. An ulstrstructurl study of 31 cses. Pthol. Res. Prct., 1983, 176, 158-175. 9. Yee D. Y., Mott R. C., Nixon B. P. Clcifying poneurotic firom. J. Foot. Surg., 1991, 30, 279-283. SAMENVATTING N. K. SFEROPOULOS, R. KOTAKIDOU. Clcifierend poneurotisch firom (C.A.F.) : drie gevllen. Drie gevllen vn een clcifierend firoom vn de poneurosis worden eschreven ; het ging om een jongetje vn 8 jr, een 43-jrige vrouw en een 48-jrige mn ; de loclistie ws respectievelijk : de pees vn de plmris longus, de lterle voet ter hoogte vn mettrsl V sis en de V teen. Klinische en rdiogrfische (wronder Echo en CT) krkteristieken worden eschreven. Histologisch vindt men chondroid weefsel en verklking, middenin geieden vn prolifertie vn plompe firolsten ij het jongetje, ij de twee volwssenen drentegen, wren de letsels scherper egrensd en minder celrijk, ws er meer clcifictie vn het indweefsel, en ws er geen chondroide differentitie. RÉSUMÉ N. K. SFEROPOULOS, R. KOTAKIDOU. Firome ponévrotique clcifint : présenttion de 3 cs. Les uteurs ont oservé trois cs de firome ponévrotique clcifint chez un grçon de 8 ns, une femme de

416 N. K. SFEROPOULOS, R. KOTAKIDOU 43 ns et un homme de 48 ns. Les lésions étient loclisées u niveu du tendon du plmris longus dns un cs, à l se du 5 ème méttrsien dns le second et u niveu du petit orteil dns le 3 ème. Les uteurs présentent les spects cliniques et rdiologiques, en prticulier échogrphique et tomodensitométrique. Sur le pln histologique, ils ont oservé chez le ptient le plus jeune du tissu chondroïde et des clcifictions dns des zones de prolifértion ostéolstique ; chez les deux utres ptients, les lésions étient plus clirement définies et montrient un tissu conjonctif dense fortement clcifié, moins cellulire, sns signe de différentition chondroïde.