TUMORS OF THE HAND AND FOkEARM 0749-0712/95 $0.00 + .20

GIANT CELL TUMORSOF TENDON SHEATH

Keith A. Glowacki/ MD/ and Arnold-Peter C. Weiss/ MD

Giant cell tumor of tendon sheath is a well-recognized histopathologic entity.1'3'4'6'12'14 It

is the second most common tumor of the

hand/ and although it can occur in other loca-tions of the body/ such occurrences are quiteuncommon.4'6r " Several authors have exam-

ined- the clinical and histologic parameters ofthis tumor/ and in fact there is no generalagreement as to the nomenclature of the le-sion.'1"6'"' "'15 In addition/ the cause of these

lesions as well as the optimal treatment re"gime is poorly understood at the presenttime.

Jaffe has termed these lesions a localizedpigmented villonodular synovitis based onthe histologic similarity to the diffuse form ofpigmented villonodular synovitis involvinglarger joints.6 He believed these to be variantsof the same process with a localized formfound more predominantly in the hand andupper extremity and the diffuse form seenmore commonly as a monoarticular arthritisin the lower extremity joints. Anatomically/the lesion is less frequently directly associatedwith joints when it presents in the upper ex"tremity and is always associated with the in-tracapsular region when presenting in thelower extremity.

Moore et al prefer the term localized nod-ular tenosynovitis" to try to more accuratelydescribe the clinical appearance of the tumorwhen it is noted in the hand." Other descrip-

five names that have been used to describethese lesions include fibrous xanthoma/ xan-

thoma of the synovium,. benign synovioma/and sclerosing hemangioma.3'6'81 11'13'15 Cer"

iauily the lack of definitive nomenclahire forthis particular tumor demonstrates that theexact pathologic nature is unknown. This tu-

mor is one that is frequently misdiagnosedand thought to represent a cyst. Commonly/it is only after presentation to and evaluationby a hand surgeon that tihe definitive diag-nosis of this lesion is made. This scenarioappears to be more due to the lack of wide-spread knowledge on this particular condi-tion than inappropriate examination. Al-though surgical excision is the most commonroute/ reported recurrence rates of 5% to 50%of primary excisions suggest that for severalreasons the present surgical treatment is notalways adequate.3'5'n-15

CLINICAL ISSUES

Although giant cell tumor of tendon sheathmay occur at any age/ it is most common

between the ages of 30 and 50 years, witha peak incidence in the fifth decade. A 3:2predominance is noted towards women. Typ-

ically the lesion occurs in the hands and fin-gers; it is second only to ganglions as themost common tumor of the hand. Giant cell

From the Deparhnent of Orthopaedics, Brown University School of M'edicine; and Division of Hand, Upper Extremity,and Mscrovascular Surgery/ Rhode Island Hospital/ Providence/ Rhode Island

HAND CLINICS

VOLUME 11 • NUMBER 2 • MAY 1995 245

246 GLOWACKI & WEISS

tumor of tendon sheath most commonly oc-

curs adjacent to the distal interphalangeal(DIP) joint and has a propensity to occur inthe index and long fingers (Figures 1A andB). The lesion is found along the volar aspectof the digit in approximately two thirds ofpatients who present with an easily palpableand definable lesion location. Other less com-mon sites of involvement mclude the foot/ankle/ knee and hip.4'8'11'15

On initial physical examiiiation/ the lesionsare noted to be firm/ tabulated/ nontendermasses firmly fixed to the deep tissues. Thetumor may involve all the structures of thedigit and include the tendon sheath/ volarplate/ capsular ligaments and joints. Whenthey are located dorsally in the digits, theyfrequently involve the joint itself or the tcndi-nous attachments to bone. The tumor is slow-

growing/ increasing in size only gradually fora long period of time/ and can remain dor-maiit as to size increase for several years at a

time. It is not uncommon to see a tumor

envelop the flexor or extensor tendons oreven the neurovascular bundles. A large re-

view of 115 cases demonstrates joint involve-ment by the tumor in only one fifth of allpatients." The giant cell hmior often extendsinto the flexor sheath to the vinculae and/rarely/ can erode the underlymg bone or joint.

Cartilage invasion and cystic bone disruptionhave not been generally described with thislesion. These two changes are characteristi-

cally noted in pigmented villonodular synovi-tis involving the toes/ knee and hip joints.6The lesion does not appear to be related totrauma despite its common location at theDIP joint of the digit. A strong associationbetween the presence of giant cell tumor andrheumatord arthritis has been reported.10 Nodocumented case of degencration of a benigngiant cell tumor of tcndon sheath into a ma-lignaiit form has been reported. Nevertheless/the lesion can be quite aggressive and behavein a low-grade malignant fashion: recurrenceafter surgical removal/ often requiring severalfurther procedures for eventual eradication/is reported in many patients.

On clinical presentation/ patients fre-quently note nodular swelling in the regionof the tumor. They commonly describe thelesion as slowly growing in size; very rarely/pain is associated with the growth. Numb-ness at the distal aspect of the finger is occa-sionally noted but if present is usually imld.Patients note occasional decreased range ofmotion/ especially if the lesion is in a palmarlocation/ and occasional snapping of un"known cause is also noted during digitalflexion. Duration of symptoms prior to pre-

Figure 1. (A & B)A multilobulated weli-circum-scribed mass is noted along Ihe dorsal aspectof the DIP joint in this 53-year-old woman.Some flattening of the nail plate was notedalong the radial aspect (A). Excision of the le-sion was undertaken through a transverse inci-sion with adequate Jocalization of the tumor.Extensive involvement of the extensor tendonwas noted requiring reconstruction and percu-taneous Kirschner wire pinning of the DIP jointduring healing (B).

GTANT CELL TUMORS OF TENDON SHEATH 247

sentation for treatment ranges from several

weeks to 30 years/ with an average of approx-imately 2 years in most reported studies.3' '

GROSS AND HISTOLOGICAPPEARANCE

Giant cell tumor of tendon sheath mostfrequently presents as a multilobular massthat is fairly well circumscribed and subcuta-neous. The villous structure and deep brownliemosiderin- pigmentation often, associated

with diffuse pigmented villonodular synovi-tis is seldom found m a localized multilobularform of giant cell tumor of tendon sheath.6'1()

The color of these lesions varies significantlybut is generally a fairly bright yellow withareas of discoloration turning to a brown hue.Tumors vary m color from entirely greyishbrown, to entirely yellow-orange. The color

of the lesions is affected by the degree ofhemosiderin and collagcn and the quantity ofhistiocytes present in the lesion.1 The nodulesrange in size from. 0.5 to 5.0 cm with a vari-

able degree of encapsulation from the sur-rounding tissue. Giant cell tumor of tendonsheath that occurs in association with largejoints is more difficult to diagnose becauselesion location is more frequently ii-ttra-articu-

lar and symptoms are generally nonspecificas far as localization is concerned. Ushijima etal have recognized the clinical and pathologicdifferences between digital and large-jointforms of this tumor.14

Giant cell tumor of tendon sheath in thedigits generally present as relatively small/firm and regular-app earing lesions. Lesionselsewhere, iiicluding the Jfeet/ are generallylarge. Tumors associated with the digits aregenerally surrounded by a thin fibrous cap-sule with very little invasion of the capsuleinto the lesion itself. Those lesions found inlarger joints are frequently covered by layersof synovial cells. The lesions "when isolated inthe digits do not appear to always have adefined association with the fiexor or extensortendon sheath. Nevertheless, there are fre-

quently small "staLks" of the tumor that ex-tend to the flexor tendon or extensor tendonsheath region/ and often if the mass is rela-lively large/ several little contiguous satellitelesions will be noted extending into the ten-don sheath and synovium. These satellite le-sions tend to be smaller in size than the maincentral lesion/ which is usually located in aneccentric location.

On microscopic examination/ the lesions

consist of variable portions of collagenizedstroma/ hemosiderin pigi'n.entation/ multinu"

cleated giant cells/ and the characteristic poly-hedral histiocyte (Fig. 2). Both diffuse andlocalized forms of this tumor contain activelyproliferating histiocytes and the large multi-nucleated giant cells. The origin and differen-tiation of these tumors has been extensivelydebated; recent histochemical studies demon-strate that the mononuclear cells and the mul-tmucleated giant cells represent osteoclastsand exhibit a phenotype consistent with thebone-marrow-derived monocytes and macro-

phages. Jaffe et al have pointed out an evolu-tion of these nodules from more cellular im-mature lesions to more advanced acellular

lesions with a hyalinized sh'oma.6 A recentstudy by Abdul-Karim et al compared thelocalized form of giant cell himor of tcndonsheath to a more diffuse form of this condi-tion and pigm.ented villonodular synovitis.1They concluded that all three lesions wereessentially similar from a hlstopathologic ba"sis but formed clinically distinct lesions. Theyproposed a spectrum ranging from a local-ized^ benign form to a more destructive dif-fuse form. A measure termed the //prolifera-

five index may assist in distinguishingbetween aggressive and benign lesions. Thediffuse form of this condition shows a morerapid proliferation and less controlled bio-logic behavior which should be consideredwhen managmg and counseling the patientabout the possibility of recurrence.

On rare occasion/ the histologlc featuresmay be confused with those of other softtissue tumor such as synovial sarcoma/ fi-

broma of tendon sheath and rhabdomyosar-coma. The nodular growth pattern/ occasionalpresence of mitotic figures and the propensityfor recurrence in certain lesions after inadc-

quate removal should all suggest not an in-flammatory process but a neoplastic one. Thehistologic pattern of the lesion and its sizecannot necessarily be correlated with recur-

rence rate. Wright et al stated that the moreimmature and cellular lesions demonstrateda higher recunence rate.15 Recurrence rate is

generally also associated with forms of thetumor that are more destructive to both softand hard tissue.

RADIOGRAPHIC APPEARANCE

Plain radiographic examination demon-strates a typical lesion of only a soft tissue

248 GLOWACKI & WEISS

Figure 2. Giant cell tumor of tendon sheath is characterized histoiogically by polyhedral histiocytes, hemosiderindeposition, and multinucleated giant cells (H & E, origina! magnification x 200).

mass in 50% of the cases. The lesion itselfdoes not appear to have a characteristic radio-

graphic appearance. When the lesion is lo-cated in direct contact with the bony cortex/a pressure phenomenon on the cortex can be

seen with some cases demonstrating in-

denting of the involved bone. No particularclinical diagnostic dilemma occurs with re-gards to benign versus malignant determina-tion when the himor appears in the handsand feet without bony erosion. The difficultyarises when radiographic changes are atypi"cal. Fortunately this is quite uncommon. Onrare occasion/ however/ a reaction may mimic

changes seen with a periosteal chondroma.

Intralesional calcifications may be presentand mimic synovial chondromatosis/ perios-teal chondroma or calcific tendinitis. Radio-graphically/ the differential diagnosis ofdigital lesions should include fibroma orchondroma of tendon sheath/ synovial chon-dromatosis/ synovial hemangioma/ foreignbody granuloma/ chronic tophaceous goutand periosteal chondroma/'9

These lesions can also be evaluated by MRimaging. MR scarming using both T-l and T-2 weighted images shows that giant cell tu-mors of tendon sheath have a signal intensitysimilar to that of pigmented villonodular syn-ovitis. There is a decreased signal on both theT-l and T-2 weighted images/ which is anuncommon appearance for extra-articular soft

tissue masses/ particularly when they occur

in the hands or feet.7 These findings maysuggest the diagnosis of giant cell tumor oftendon sheath. Pigmented villonodular syno-vitis does show^ a higher tendency towardsintralesional bleeding and greater depositionof hemosiderin often resulting in a more in-homogeneous appearance. Despite thesefindings/ diagnosis is generally still madebased on clinical examination grounds.6

TREATMENT

The optimal treatment for giant cell tumorof tendon sheafch is i.mknown. Based on pa-

tients who have had the lesions for a longtime/ it appears that growth is relatively slow/although progressive/ in the vast majority.Patients frequently present with distal symp-toms of neuropathy secondary to a compres-

sive phenomenon or with concerns regardingthe cosmetic appearance of the lesion itself.Rarely do functional consequences play a rolem electing treatment but occasionally tins isnoted with finger flexion. Excision of the Ie-sion is generally considered the treatment ofchoice; conservative management does not re-solve the condition.'*' n'13 Excision can be te-

dious because the presence of the mass isoften withhi the flexor tendon sheath or thesynovial joint. It is not uncommon that a par-tial excision of the sheafch or joint capsule isrequired to ensure complete removal of the

GIANT CELL TUMORS OF TENDON SHEATH 249

lesion. Although the lesion tends not to in-vade the soft tissues and can be shelled out/ ittends to have frequent satellites which makecareful dissection and exploration mandatory.Recurrence of giant cell tumor of tendonsheath is common despite meticulous re-moval; however/ malignant transformation ofthe lesion has not been reported even aftermultiple recurrences.2'4'5'n' !3 An association

with the development of arthritis at the distalinterphalangeal joint has been noted by Jonesct al." Occasionally with removal of the tu-mor/ debridement or fusion of the DIP jointis required in order to afford complete exci-sion of the lesion itself. On rare occasion theskin is also involved in the lesion and is besttaken with cxcision of the tumor/ requiringsecondary skin grafting.

OPERATIVE TECHNIQUE

In general/ operative approach to the lesionis directed towards the area of the major masseffect. For lesions along the volar aspect ofthe hand/ a Brunner-type mcision is most

commonly performed, whereas lesions oc-

curring in an eccentric fashion can often beexcised using a mid-lateral approach (Fig. 3).Lesions that occur along the dorsal aspect ofthe digit are usually approached through alongihidinal incision/ or if relatively localized/a transverse incision. Once the pseudocapsule

is localized about the lesion, careful dissec"tion in the soft tissues/ taking care not todirectly probe the lesion/ is important to tryto establish the boundaries of the tumor. Ascareful dissection of the surroundmg tendonsand soft tissues are undertaken/ a Freer eleva-

tor or other blunt probe is useful m trymg tomanipulate the lesion without achially prmc-turing its surface to see if one can "tease"

any satellites from underneath surroundmgtendons or other structures. It is frequentlypossible to completely remove these satellitesusing this "teasing technique rather than di-rect exposure of the entire site. A generoussoft tissue pathway is required prior to tryingto tease the lesion out from these nooks andcrannies or the surgeon risks leaving smallfragments behind. In general/ it is wise tolook around any adjacent tendons since satel-life lesions are sometimes seen that do notappear to be contiguous with tl^e mam tumor.These are often fairly small in size/ only sev-eral millimeters. For lesions exposed alongthe mid-lateral or volar aspect/ careful dissec-

tion of the digital neurovascular struchireshould be undertaken prior to excision/ andthe tumor can completely surround this mostimportant structure (Fig. 4).

For lesions that involve some bony inva-sion/ careful curettage after teasing the himorout of these areas is required. If any doubt ispresent/ it is better to undertake an extensivedebridement of the bone using a curette androngeur to ensure that no tumor is left be-hind. On rare occasions/ reconstruction of an

extensor or Hexor tendon may be required ifthe lesion has invaded these struchices (Fig.5A to f). This findrng is more frequently seensecondary to attenuation due to a pressurephenomenon rather than aggressive invasion.

The patient should be warned postopera-tively that lesions that are noted to have bothbony and tendinous invasion have a higherincidence of recurrence.

DISCUSSION

Giant cell hunor of tendon sheath/ alsoknown as localized nodular tenosynovitis/has long been included hi the general cate-gory of pigmented villonodular synovitis.Both are considered benign growths of polyg-onal or roimd histiocyte-like cells associatedwith multinuclear giant/ foam/ and hemosid-erin-laden cells. Most lesions produce one or

more discrete soft-tissue nodules/ usuallyalong the tendon sheath or small joints ofthe fingers and toes. Pigmented villonodularsynovitis is more commonly identified hi. a.diffusely proliferate synovial membrane andhas a darker pigmented appearance with orwithout nodular formation. Pigmented villo-nodular synovitis also more frequently in-valves the larger joints of the knee and hiprather than the digits and hand.4'6'"

Giant cell himor of tendon sheath is a be-nign condition and does not metastasize. Al-though there is a high incidence of jointinvolvement in lesions involvmg the digitsof the hand/ this is not universally the case.Various theories of pathogenesis and ctiologyhave been proposed but none codified.6'8'la 14

These lesions are characterized by some asinflammatory in nature and by others as rep-resenting a neoplastic source. The key ele"

ments of treatment involve a generous surgi-

cal exposure in appropriate tissue planes toallow protection of the neurovascular bundlesand flexor and extensor tendons. Careful ex-

posure working more on the adjacent tissues

250 GLOWACKI & WEISS

Figure 3. For volar lesions, aBrunner incision is ciassicallyused to allow access to both digi-tal neurovascular bundles beforeexcision of the tumor.

Figure 4. When giant cell tumor of tendonsheath is localized along the volar aspectof the distal finger, direct and circumferen-tial involvement of both digital neurovascu-lar structures is common. Vessel loops areplaced around both digital neurovascuiarstructures demonstrating the dose prox-imity of these two structures to the tumoritself.

GTANT CELL TUMORS OF TENDON SI-IEATH 251

Figure 5. A lateral radiograph of the right thumb in this 32-year-old man, who has had recurrence of his tumor, showscortical erosion at the base of the proximal phaianx dorsaliy (arrow) (A). During surgical exploration of this locallyaggressive giant cell tumor of tendon sheath, the main lesion can be seen to extend both on the radla! and ulnaraspects of the extensor tendon with attenuation of bolh the tendon and the sagittal hood (*, extensor tendon) (B).Because of the extensive Jnvoivement of the tumor in this particular patient, transection of the extensor tendon wasrequired to gain exposure to all the satellite lesions as well as the bony intra-articuiar extensions (C). With the extensortencton retracted distally, carefully piecemeai excision of the tumor down to its invasion of the bony cortex wasaccomplished.

tllustraft'on continued on following page

252 GLOWACKI & WEISS

Figure 5 (Continued). (D). Careful curettage of ail the small cavernous sinuses invaded by the tumor in the dorsalaspect of both the metacarpal and proximal phalanx was undertaken with removal of any tissue that appeared to behemosiderin stained (E). A gross photograph of this locally aggressive giant cell tumor of tendon sheath shows themultilobu!ated contour of the tumor as well as the variations and hemosiderin deposition throughout the tumor itself (F).

GIANT CELL TUMORS OF TENDON SHEATH 253

than on the lesion itself/ thereby avoidingpunchire and possible seeding of the tumorcells to other soft-tissue structures/ is vital. A

method of "teasing" the lesion from its vari-ous interstices to identify and remove aU sat-ellite lesions is quite helpful in its overaUexcision. Recurrence rates in the reported

literature range from 5% to 50%, but gener-ally less than 10%.2-4-6-8'n-12

References

1. Abdul-Karim FW/ El-Naggar AK/ Joyce MJ, et al:Tenosynoviaf giant ceU [umor: a clinicopathologicand flow cytomctoic DNA analysis. Human Path23:729-735, 1992

2, Ciattaglia G, Filosa G, Bugatti L: Gianf: ceil tumor oftendon sheath. Amer Acad Derm 4:728-29, 1991

3. Enzinger FM: Clear cell sarcoma of tcndons and apo-

neuroses: an analysis of 21 cases. Cancer 18:1163-74,1965

4. Froimson AT: Benign solid tumors. Hand Clin 3:213-17, 1987

5. Hubbard LF/ Burton RI: Malignant fibrous histiocy-ioma of the forearm. J Hand Surg 2:292-96, 1974

6. Jaffe HL, Liclitenstom HL, Elustro CJ: Pigmented vil-lonodular synovitis, bursitis and tenosynovitis. ArchPath 33:731-65, 1941

7. Iclinek }S, Kransdorf MJ/ Shmookler BN/ ct al: Giantcell tumor of the tendon sheath: MR findings in ninecases. AJR 162:919-22, 1994

8. Jones PE/ Souie EM, Covcnh-y M.B: Fibrous xanthomaof synovium (giant-cell tumor of tendon sheath/ pig-merited nodular synoviris): a study of 118 cases. JBone Joint Surg 51A:76-86/ 1969

9. Karasick D, Karasick S: Giant cell tumor of tendonsheath: spectrum of radiologic findings. Skel Rad21:219-24, 1992

10. MatE-hews RE/ Gould JS, Kashlan MB: Diffuse pig-mcntcd villonodular tcnosynovitis of the uh^arbursa—a case report. J Hand Surg 6:64-69, 1981

11. Moore JR, Weiland AJ, CurHs I<M: Localized nodulartenosynovitis: Experience with 115 cases, J HandSurg 9A:412-17, 1984

12. Spjut HJ^ DorAnan MD/ Fechncr RE, et al: Tumors ofBone and Cartilage. Washmglon DC, Armed ForcesInstitution of Pathology/ 1983, pp 400-410

13. Stewart MJ: Benign giant-cell synovioma and its rcla-tion to "xaiithoma." J Bone Joint Surg (Br) 30:522-27, 1948

14. Ushijma M, Hashimoto H/ TsuncyosM M/ et al: Giantcell hunor of the tendon sheath. Cancer 57:875-884,1986

15. Wright CJE: Benign giant-cell synovioma—an inves-tigation of 85 cases. Brit J Suig 38:257-271, 1951

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