Giant Cell Tumors of Tendon Sheath
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TUMORS OF THE HAND AND FOkEARM 0749-0712/95 $0.00 + .20 GIANT CELL TUMORS OF TENDON SHEATH Keith A. Glowacki/ MD/ and Arnold-Peter C. Weiss/ MD Giant cell tumor of tendon sheath is a well- five names that have been used to describe recognized histopathologic entity.1'3'4'6'12'14 It these lesions include fibrous xanthoma/ xan- is the second most common tumor of the thoma of the synovium,. benign synovioma/ hand/ and although it can occur in other loca- and sclerosing hemangioma.3'6'81 11'13'15 Cer" tions of the body/ such occurrences are quite iauily the lack of definitive nomenclahire for uncommon.4'6r " Several authors have exam- this particular tumor demonstrates that the ined- the clinical and histologic parameters of exact pathologic nature is unknown. This tu- this tumor/ and in fact there is no general mor is one that is frequently misdiagnosed agreement as to the nomenclature of the le- and thought to represent a cyst. Commonly/ sion.'1"6'"' "'15 In addition/ the cause of these it is only after presentation to and evaluation lesions as well as the optimal treatment re" by a hand surgeon that tihe definitive diag- gime is poorly understood at the present nosis of this lesion is made. This scenario time. appears to be more due to the lack of wide- Jaffe has termed these lesions a localized spread knowledge on this particular condi- pigmented villonodular synovitis based on tion than inappropriate examination. Al- the histologic similarity to the diffuse form of though surgical excision is the most common pigmented villonodular synovitis involving route/ reported recurrence rates of 5% to 50% larger joints.6 He believed these to be variants of primary excisions suggest that for several of the same process with a localized form reasons the present surgical treatment is not found more predominantly in the hand and always adequate.3'5'n-15 upper extremity and the diffuse form seen more commonly as a monoarticular arthritis in the lower extremity joints. Anatomically/ CLINICAL ISSUES the lesion is less frequently directly associated with joints when it presents in the upper ex" Although giant cell tumor of tendon sheath tremity and is always associated with the in- may occur at any age/ it is most common tracapsular region when presenting in the between the ages of 30 and 50 years, with lower extremity. a peak incidence in the fifth decade. A 3:2 Moore et al prefer the term localized nod- predominance is noted towards women. Typ- ular tenosynovitis" to try to more accurately ically the lesion occurs in the hands and fin- describe the clinical appearance of the tumor gers; it is second only to ganglions as the when it is noted in the hand." Other descrip- most 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- Cartilage invasion and cystic bone disruption curs adjacent to the distal interphalangeal have not been generally described with this (DIP) joint and has a propensity to occur in lesion. These two changes are characteristi- the index and long fingers (Figures 1A and cally noted in pigmented villonodular synovi- B). The lesion is found along the volar aspect tis involving the toes/ knee and hip joints.6 of the digit in approximately two thirds of The lesion does not appear to be related to patients who present with an easily palpable trauma despite its common location at the and definable lesion location. Other less com- DIP joint of the digit. A strong association mon sites of involvement mclude the foot/ between the presence of giant cell tumor and ankle/ knee and hip.4'8'11'15 rheumatord arthritis has been reported.10 No On initial physical examiiiation/ the lesions documented case of degencration of a benign are noted to be firm/ tabulated/ nontender giant cell tumor of tcndon sheath into a ma- masses firmly fixed to the deep tissues. The lignaiit form has been reported. Nevertheless/ tumor may involve all the structures of the the lesion can be quite aggressive and behave digit and include the tendon sheath/ volar in a low-grade malignant fashion: recurrence plate/ capsular ligaments and joints. When after surgical removal/ often requiring several they are located dorsally in the digits, they further procedures for eventual eradication/ frequently involve the joint itself or the tcndi- is reported in many patients. nous attachments to bone. The tumor is slow- On clinical presentation/ patients fre- growing/ increasing in size only gradually for quently note nodular swelling in the region a long period of time/ and can remain dor- of the tumor. They commonly describe the maiit as to size increase for several years at a lesion as slowly growing in size; very rarely/ time. It is not uncommon to see a tumor pain is associated with the growth. Numb- envelop the flexor or extensor tendons or ness at the distal aspect of the finger is occa- even the neurovascular bundles. A large re- sionally noted but if present is usually imld. view of 115 cases demonstrates joint involve- Patients note occasional decreased range of ment by the tumor in only one fifth of all motion/ especially if the lesion is in a palmar patients." The giant cell hmior often extends location/ and occasional snapping of un" into the flexor sheath to the vinculae and/ known cause is also noted during digital rarely/ can erode the underlymg bone or joint. flexion. Duration of symptoms prior to pre- Figure 1. (A & B)A multilobulated weli-circum- scribed mass is noted along Ihe dorsal aspect of the DIP joint in this 53-year-old woman. Some flattening of the nail plate was noted along 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 tendon was noted requiring reconstruction and percu- taneous Kirschner wire pinning of the DIP joint during healing (B). GTANT CELL TUMORS OF TENDON SHEATH 247 sentation for treatment ranges from several On microscopic examination/ the lesions weeks to 30 years/ with an average of approx- consist of variable portions of collagenized imately 2 years in most reported studies.3' ' stroma/ hemosiderin pigi'n.entation/ multinu" cleated giant cells/ and the characteristic poly- hedral histiocyte (Fig. 2). Both diffuse and GROSS AND HISTOLOGIC localized forms of this tumor contain actively APPEARANCE proliferating histiocytes and the large multi- nucleated giant cells. The origin and differen- Giant cell tumor of tendon sheath most tiation of these tumors has been extensively frequently presents as a multilobular mass debated; recent histochemical studies demon- that is fairly well circumscribed and subcuta- strate that the mononuclear cells and the mul- neous. The villous structure and deep brown tmucleated giant cells represent osteoclasts liemosiderin- pigmentation often, associated and exhibit a phenotype consistent with the with diffuse pigmented villonodular synovi- bone-marrow-derived monocytes and macro- tis is seldom found m a localized multilobular phages. Jaffe et al have pointed out an evolu- form of giant cell tumor of tendon sheath.6'1() tion of these nodules from more cellular im- The color of these lesions varies significantly mature lesions to more advanced acellular but is generally a fairly bright yellow with lesions with a hyalinized sh'oma.6 A recent areas of discoloration turning to a brown hue. study by Abdul-Karim et al compared the Tumors vary m color from entirely greyish localized form of giant cell himor of tcndon brown, to entirely yellow-orange. The color sheath to a more diffuse form of this condi- of the lesions is affected by the degree of tion and pigm.ented villonodular synovitis.1 hemosiderin and collagcn and the quantity of They concluded that all three lesions were histiocytes present in the lesion.1 The nodules essentially similar from a hlstopathologic ba" range in size from. 0.5 to 5.0 cm with a vari- sis but formed clinically distinct lesions. They able degree of encapsulation from the sur- proposed a spectrum ranging from a local- rounding tissue. Giant cell tumor of tendon ized^ benign form to a more destructive dif- sheath that occurs in association with large fuse form. A measure termed the //prolifera- joints is more difficult to diagnose because five index may assist in distinguishing lesion location is more frequently ii-ttra-articu- between aggressive and benign lesions. The lar and symptoms are generally nonspecific diffuse form of this condition shows a more as far as localization is concerned. Ushijima et rapid proliferation and less controlled bio- al have recognized the clinical and pathologic logic behavior which should be considered differences between digital and large-joint when managmg and counseling the patient forms of this tumor.14 about the possibility of recurrence. Giant cell tumor of tendon sheath in the On rare occasion/ the histologlc features digits generally present as relatively small/ may be confused with those of other soft firm and regular-app earing lesions. Lesions tissue tumor such as synovial sarcoma/ fi- elsewhere, iiicluding the Jfeet/ are generally broma of tendon sheath and rhabdomyosar- large. Tumors associated with the digits are coma. The nodular growth pattern/ occasional generally surrounded by a thin fibrous cap- presence of mitotic figures and the propensity sule with very little invasion of the capsule for recurrence in certain lesions after inadc- into the lesion itself.