tumorlike lesions and benign tumors of the hand and...

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Vol 11, No 2, March/April 2003 129 Lesions of the hand and wrist may originate in either soft tissues or bone. They can be divided into two groups, tumorlike lesions and true neoplasms, with the latter subdi- vided into benign and malignant tumors. Although there are a rela- tively large number of lesions and subtle variations, established princi- ples of tumor management provide a logical and systematic approach to both diagnosis and treatment. Col- laboration with a musculoskeletal radiologist and a pathologist is fre- quently important for arriving at the correct diagnosis and applying the proper treatment. Although many of these lesions can occur in other parts of the body, their presentation and treatment may differ in the hand and wrist. Classification Benign neoplasms have been clini- cally classified into three types: latent, active, and locally aggres- sive. 1 Latent tumors either remain unchanged or heal spontaneously and therefore may not require treat- ment other than observation. An example is a soft-tissue heman- gioma undergoing involution. Ac- tive tumors continue to grow but are constrained by anatomic bound- aries. They usually require surgery, either by intralesional or marginal excision. Common examples are enchondromas and lipomas. Lo- cally aggressive tumors continue to grow beyond their natural anatomic boundaries; an example is a giant cell tumor of bone that destroys the cortex and extends into adjacent soft tissues. General Principles On initial evaluation, any lesion is more likely to be a common rather than a rare condition. The most com- mon soft-tissue lesion in the hand and wrist is a ganglion; the most common bone tumor is an enchon- droma. An unusual presentation of a common lesion is more frequent than the occurrence of a rare lesion. However, errors can be made when a seemingly innocent-appearing mass is not appropriately evaluated. Evaluation begins with a detailed history that includes any pertinent medical conditions or events (eg, renal disease, parathyroid disease, prior malignancies) and a family history of similar lesions. The his- tory also should include informa- tion concerning the lesion’s rate of growth, changes in consistency or color, associated pain or neurologic symptoms, and any prior trauma to the area. Rapid growth, night pain, and/or increase in pain should raise the suspicion of a malignant tumor, although such symptoms also may occur with benign lesions. During the clinical examination, the location of the lesion should be carefully documented using ana- tomic landmarks as references. A sketch of the hand and wrist depict- ing the location and dimensions of the mass is often helpful as a refer- ence for future examinations, when Dr. Plate is Assistant Professor, New York University School of Medicine, and Assistant Attending Physician, Hand Service, NYU– Hospital for Joint Diseases, New York, NY. Dr. Lee is Clinical Instructor, Lenox Hill Hospital, New York. Dr. Steiner is Professor of Surgical Pathology, New York University School of Medi- cine, and Chairman, Department of Pathology, NYU–Hospital for Joint Diseases. Dr. Posner is Clinical Professor, Orthopedic Surgery, New York University School of Medicine, and Chief of Hand Service, NYU–Hospital for Joint Diseases. Reprint requests: Dr. Posner, 2 East 88th Street, New York, NY 10128. Copyright 2003 by the American Academy of Orthopaedic Surgeons. Abstract A broad spectrum of tumorlike lesions and neoplasms can occur in the hand and wrist, although with somewhat less frequency than in other parts of the body. A thorough understanding of the differential diagnosis of these lesions and a comprehensive strategy for evaluation are central for effective care. Plain radio- graphs are diagnostic for most bony lesions, whereas magnetic resonance imag- ing may be necessary to help differentiate a benign soft-tissue lesion from the rare malignant neoplasm. In spite of the complex anatomy, adherence to proper oncologic principles most often will lead to a satisfactory outcome. J Am Acad Orthop Surg 2003;11:129-141 Tumorlike Lesions and Benign Tumors of the Hand and Wrist Ann-Marie Plate, MD, Steven J. Lee, MD, German Steiner, MD, and Martin A. Posner, MD

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Vol 11, No 2, March/April 2003 129

Lesions of the hand and wrist mayoriginate in either soft tissues orbone. They can be divided into twogroups, tumorlike lesions and trueneoplasms, with the latter subdi-vided into benign and malignanttumors. Although there are a rela-tively large number of lesions andsubtle variations, established princi-ples of tumor management providea logical and systematic approach toboth diagnosis and treatment. Col-laboration with a musculoskeletalradiologist and a pathologist is fre-quently important for arriving at thecorrect diagnosis and applying theproper treatment. Although manyof these lesions can occur in otherparts of the body, their presentationand treatment may differ in thehand and wrist.

Classification

Benign neoplasms have been clini-cally classified into three types:latent, active, and locally aggres-sive.1 Latent tumors either remainunchanged or heal spontaneouslyand therefore may not require treat-

ment other than observation. Anexample is a soft-tissue heman-gioma undergoing involution. Ac-tive tumors continue to grow butare constrained by anatomic bound-aries. They usually require surgery,either by intralesional or marginalexcision. Common examples areenchondromas and lipomas. Lo-cally aggressive tumors continue togrow beyond their natural anatomicboundaries; an example is a giantcell tumor of bone that destroys thecortex and extends into adjacent softtissues.

General Principles

On initial evaluation, any lesion ismore likely to be a common ratherthan a rare condition. The most com-mon soft-tissue lesion in the handand wrist is a ganglion; the mostcommon bone tumor is an enchon-droma. An unusual presentation of acommon lesion is more frequentthan the occurrence of a rare lesion.However, errors can be made when aseemingly innocent-appearing massis not appropriately evaluated.

Evaluation begins with a detailedhistory that includes any pertinentmedical conditions or events (eg,renal disease, parathyroid disease,prior malignancies) and a familyhistory of similar lesions. The his-tory also should include informa-tion concerning the lesion’s rate ofgrowth, changes in consistency orcolor, associated pain or neurologicsymptoms, and any prior trauma tothe area. Rapid growth, night pain,and/or increase in pain should raisethe suspicion of a malignant tumor,although such symptoms also mayoccur with benign lesions.

During the clinical examination,the location of the lesion should becarefully documented using ana-tomic landmarks as references. Asketch of the hand and wrist depict-ing the location and dimensions ofthe mass is often helpful as a refer-ence for future examinations, when

Dr. Plate is Assistant Professor, New YorkUniversity School of Medicine, and AssistantAttending Physician, Hand Service, NYU–Hospital for Joint Diseases, New York, NY. Dr.Lee is Clinical Instructor, Lenox Hill Hospital,New York. Dr. Steiner is Professor of SurgicalPathology, New York University School of Medi-cine, and Chairman, Department of Pathology,NYU–Hospital for Joint Diseases. Dr. Posner isClinical Professor, Orthopedic Surgery, New YorkUniversity School of Medicine, and Chief of HandService, NYU–Hospital for Joint Diseases.

Reprint requests: Dr. Posner, 2 East 88th Street,New York, NY 10128.

Copyright 2003 by the American Academy ofOrthopaedic Surgeons.

Abstract

A broad spectrum of tumorlike lesions and neoplasms can occur in the hand andwrist, although with somewhat less frequency than in other parts of the body.A thorough understanding of the differential diagnosis of these lesions and acomprehensive strategy for evaluation are central for effective care. Plain radio-graphs are diagnostic for most bony lesions, whereas magnetic resonance imag-ing may be necessary to help differentiate a benign soft-tissue lesion from therare malignant neoplasm. In spite of the complex anatomy, adherence to properoncologic principles most often will lead to a satisfactory outcome.

J Am Acad Orthop Surg 2003;11:129-141

Tumorlike Lesions and Benign Tumors of the Hand and Wrist

Ann-Marie Plate, MD, Steven J. Lee, MD, German Steiner, MD, and Martin A. Posner, MD

changes in size or configuration areevaluated. The color of the overlyingskin, mobility of the mass, move-ment with adjacent tendons, andconsistency (eg, firm, soft, lobulated,cystic) also should be documented.Some lesions may be pulsatile, havea thrill or bruit, or increase in sizewith dependency of the hand (grav-ity-induced filling).

Conventional radiographs alwaysshould be obtained, even for soft-tissue masses. They may show cal-cific densities within the lesion, suchas phleboliths in a hemangioma, orchanges in the cortex of the bonebecause of pressure from the overly-ing mass. In complex lesions forwhich plain radiographs are notdiagnostic, computed tomography(CT) can be used to visualize bonydetails. CT images are obtained in 2-mm slices, together with coronaland sagittal reconstruction. Mag-netic resonance imaging (MRI) isuseful to determine the extent andcharacteristics of soft-tissue lesions.For vascular lesions, magnetic reso-nance angiography (MRA) and/orconventional angiography are com-monly used. Bone scans are helpfulif other sites of involvement are sus-pected.

Surgical Principles

Useful diagnostic tests include nee-dle aspiration of a soft-tissue cystsuch as a ganglion, or core needlebiopsy (with radiographic guid-ance) for some bone lesions, such as giant cell tumors of bone andaneurysmal bone cysts. Excisionalbiopsies can be safely performed forsmall tumors (<2 cm) and for somelarger tumors (such as lipomas) thathave both the clinical and radio-graphic features of benign lesions.For most tumors or when the diag-nosis is in doubt, an incisional biopsyshould be done before excision.

The biopsy is the final step in theworkup that establishes the defini-

tive diagnosis, and the importanceof adhering to strict principles whenperforming this procedure cannotbe overstated. If there is any possi-bility that the lesion could be malig-nant, only the physician who willperform the definitive surgeryshould perform an open biopsy.Improperly performed biopsieshave resulted in significant compli-cations, often requiring alterationsin the preferred course of treatmentthat may result in a compromisedoutcome.2 Use of a compressivebandage to exsanguinate the limbshould be avoided because of therisk of spreading tumor cells.Instead, the limb is elevated for several minutes before tourniquetinflation. Longitudinal incisions arepreferred to transverse incisionsbecause they are more easily incor-porated into a definitive resection.The surgical approach should bethrough the most direct route and, ifpossible, through a single anatomiccompartment to minimize tumorcell contamination of surroundingtissues. Meticulous hemostasis andclosure of tissue planes also reducethe risk of tumor spread. Becauseinfections can imitate virtually anytumor, cultures of the biopsy speci-men are advisable.3

With a skilled musculoskeletalpathologist, a frozen section of thebiopsy specimen is often sufficientfor definitive diagnosis, and excisionof the entire lesion often can be done at the same surgery. However,when the diagnosis is in doubt,definitive treatment should be de-layed until the permanent sectionsare reviewed. Even when frozen sec-tions are inconclusive, they are help-ful because they determine whetheradequate tissue is present to allowdiagnosis with the permanent sec-tions. Communication with thepathologist is critically important,particularly when there is a patho-logic fracture, because the presenceof fracture callus may confuse thediagnosis.

Tumorlike Lesions of Soft Tissues

Ganglion

A ganglion is the most commonsoft-tissue mass occurring in thehand and wrist. Although the exactetiology is unknown, mucoid degen-eration of collagen tissue is the mostlikely cause. The tendency for theselesions to fluctuate in size may bethe result of a one-way valve mecha-nism.4 Although a ganglion candevelop at any joint or tendonsheath, the most common locationsin order of frequency are the wrist,flexor tendon sheaths of digits (reti-nacular cysts), and distal interpha-langeal joints (mucous cysts). In thewrist, most lesions are situated dor-sally and originate from the scapho-lunate joint. When they appear onthe volar surface, they usually arisefrom the radioscaphoid or scapho-trapezial joint. Ganglia also canarise from other joints, such as thedistal radioulnar and ulnocarpaljoints. The typical presentation is asmooth, firm mass that is sometimestender and painful. When suffi-ciently large, the ganglion will trans-illuminate. A volar radial ganglionof the wrist can cause compressionof the median nerve in the carpalcanal; a volar ulnar ganglion cancause compression of the ulnarnerve in Guyon’s canal.

Nonsurgical treatment, includingaspiration and a corticosteroid injec-tion of the lesion, or simply disrupt-ing the mass with multiple punc-tures, has a recurrence rate of 13% to100%.5 Although recurrence afteraspiration is high, the procedure canrelieve pain and is diagnostic whena viscous, jellylike clear mucin isobtained; thus, one attempt at aspi-ration can be justified. However, as-piration of a radial volar wrist gan-glion should be avoided because ofrisk of injury to the radial artery,which is usually in intimate contactwith the mass.

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Journal of the American Academy of Orthopaedic Surgeons130

Ganglia always should be excisedat their origin to reduce the risk ofrecurrence. Although most sur-geons think that the capsule shouldbe left open, some advocate closure.After excision of a volar ganglion,the wrist should be immobilized inslight extension for 7 to 10 days;after excision of a dorsal ganglion,the wrist should be immobilized in aslight flexion to avoid a capsulodesiseffect that can result from postopera-tive scarring.

A mucoid cyst is associated withsome degree of degenerative arthri-tis of the underlying distal interpha-langeal joint and the presence of anosteophyte that may or may not beevident on conventional radio-graphs. When the cyst is small (sev-eral millimeters in diameter), notreatment is necessary. A cortico-steroid injection is generally avoidedbecause it can cause further thin-ning of the overlying skin, whichcan easily tear and lead to a jointinfection. When the skin is alreadyvery thin, cyst excision is warranted,and removing the osteophyte re-duces the risk of recurrence to about10%.6 Care must be taken to avoidinjury to the germinal matrix. Whenthe skin overlying a large cyst is ex-tremely thin, it should be excisedtogether with the cyst. Coveragewith a skin graft is usually neces-sary; an excellent donor area for afull-thickness graft is the thenarcrease of the palm. An ellipticalgraft can be harvested from this siteleaving little, if any, visible scar.

Epidermal Inclusion CystEpidermal inclusion cysts are the

third most common mass of thehand, following ganglia and giantcell tumors of the tendon sheath.4Epidermal inclusion cysts resultfrom penetrating trauma, with de-position of keratin-producingepithelial cells into the soft tissues.Consequently, they are most com-monly found on the tactile surfacesof the digits, where they are slow

growing, firm, and usually painless.When large, an inclusion cyst fre-quently will cause pressure erosionof the underlying phalanx. In someinstances, the erosion is so severethat the bone is markedly weakened.Treatment requires not only excisionof the cyst but also a bone graft torestore skeletal stability. Recurrenceafter surgery is uncommon.

Foreign-Body GranulomaThe lesion may be difficult to dif-

ferentiate clinically from an epider-mal inclusion cyst, especially whenit is on the tactile surface of a digit.Conventional radiographs can aidin the differentiation when the for-eign material is radiodense. Treat-ment is determined by the accessi-bility of the lesion, and usually onlysymptomatic lesions are excised.

Fibromatosis/CalcifyingAponeurotic Fibroma

Dupuytren’s disease is a fibro-matosis involving the palmar fasciaof the hand that may cause fingercontractures. However, the diagno-sis may not be evident with the ini-tial presentation of nodules, whichgenerally are nontender althoughthey are sometimes transientlypainful in the early stage of the dis-ease. The nodules contain contractilemyofibroblasts and are commonlyassociated with dimpling of the over-lying skin. Knuckle pads (Garrod’snodes), another form of fibromatosis,are found on the dorsum of the prox-imal interphalangeal joints. Theyoccur in 20% to 40% of patients withDupuytren’s disease7 and are morecommon when the other diatheses ofthe disease, such as Peyronie’s dis-ease (penile involvement) and/orLedderhose’s disease (plantar fasciainvolvement), are present. Knucklepads almost never require excision,and treatment of Dupuytren’s nod-ules in the absence of any joint con-tracture also is nonsurgical. Cortico-steroid injections may provide somesymptomatic relief for a painful nod-

ule. The pain is more likely to besecondary to a tenosynovitis of theunderlying flexor tendon sheathrather than a result of the noduleitself. If pain persists and the accura-cy of the diagnosis is in doubt, biop-sy is warranted.8

Calcifying aponeurotic fibroma(juvenile aponeurotic fibroma) is arare tumor that was originally re-ported in infants and young chil-dren. More recently it also has beenreported in adults and thus is moreappropriately referred to as calcify-ing rather than juvenile.9 It usuallypresents as a slow-growing, pain-less, nontender mass. Radiographstypically show a soft-tissue masswith fine, granular calcifications. Atsurgery, the tumor is a firm, graymass with poorly defined borders,giving it an ominous appearance.Histologically, the tumor consists offibrous tissue containing foci ofchondroid metaplasia and areas ofcalcification. Treatment requireswide resection. The recurrence rateis high and may exceed 50%, but itdecreases with age, as does its rateof growth. Because malignant trans-formation has not been reported, re-currence is treated with observationor, when the lesion is symptomatic,with repeat resection.10

Gout/Tophaceous PseudogoutGout is caused by either overpro-

duction or underexcretion of uricacid, resulting in precipitation ofmonosodium urate crystals withinsynovial or tenosynovial tissues.The crystals generally elicit anintense inflammatory response char-acterized by marked swelling, ery-thema, and pain. The appearance ofgout can be mistaken for infection,rheumatoid arthritis, or even neo-plasm. Gouty flexor tenosynovitisin a finger can be confused with asuppurative tenosynovitis. Goutytenosynovitis also can affect the flex-or tendons more proximally in thecarpal canal and the extensor ten-dons under the extensor retinacu-

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lum. In chronic disease, tophaceousdeposits are common, affectingmetacarpophalangeal and interpha-langeal joints as well as carpal joints.The diagnosis is confirmed by theidentification of needle-shaped, neg-ative-birefringent crystals on joint ortenosynovial aspiration.

An acute attack is treated withcolchicine and anti-inflammatorymedication. Treatment of painfulchronic tophi is more problematicbecause they are not likely to resolvewith medication, and complete exci-sion usually is not feasible becausethey are not encapsulated. However,tophi can be debulked, which is indi-cated for impending skin break-down, for pain relief, to controldrainage or infection, and to improvefunction and cosmesis. Frequently,more extensive procedures are neces-sary, such as excision of necrotic ten-dons, arthroplasty, and arthrodesis.In some cases, amputation of a se-verely affected finger is required.

Tophaceous pseudogout (tumoralcalcium pyrophosphate depositiondisease) is characterized by deposi-tion of calcium pyrophosphate intumorlike masses.11,12 Pseudogoutcrystals, unlike the monosodiumcrystals of gout, are rhomboid-shaped and weakly positive birefrin-gent under polarized light micros-copy. Radiographically, the lesionpresents as a soft-tissue mass withcalcification and occasional bone ero-sion. Wrist involvement is commonand is characterized by calcificationof the triangular fibrocartilage com-plex. Like gout, pseudogout cancause severe inflammation. Treat-ment for the acute flare-up is rest,immobilization of the inflamed joint,and anti-inflammatory medication.Treatment for a large pseudogoutlesion is the same as that for a goutytophus (debulking or more extensiveprocedures).

Vascular AneurysmsA true vascular aneurysm is differ-

entiated from a false or pseudo-

aneurysm by the presence of all threelayers of the arterial wall—endothe-lium, tunica media, and tunica ad-ventitia. True aneurysms result fromrepetitive blunt trauma that causes aweakening of the vessel wall andprogressive dilation of its three com-ponents. They usually involve theulnar artery in the hypothenar areaof the palm and occur in individualswho use that area as a hammer (hypo-thenar hammer syndrome).

A false aneurysm develops as aconsequence of a penetrating injurythat causes a partial laceration to the arterial wall. The hematoma atthe site of injury organizes, recana-lizes, and forms an outer wall for theinjured vessel. The endothelium,the only layer of the original arterythat remains, communicates withthe new outer wall cavity. The me-dial and adventitial layers of theoriginal artery are not part of thewall of a false aneurysm. The inter-val between injury and aneurysmcan range from weeks to years.Although trauma is the most com-mon cause of both true and falseaneurysms, they also can be causedby infection, atherosclerosis, arteri-tis, tumor infiltration, and metabolicdisorders.13

The most common presentingcomplaint is a painless mass that isnot always pulsatile. Obtaining acomplete history from the patient isimportant because prior trauma tothe area can indicate the possibilityof the lesion. Additional symptomsand clinical signs may result fromthe aneurysm’s compressing adja-cent structures (particularly nerves)or from the shedding of emboli intothe fingertips. Although angiog-raphy remains the most effectivemeans of diagnosis, pulse volumerecordings, duplex scanning, andDoppler ultrasound recordings alsoprovide valuable information.

Treatment is the same for trueand false aneurysms. Because thenatural history for each is a progres-sive increase in size with possible

thrombosis and shedding of emboli,resection is recommended. At sur-gery, a true aneurysm is usuallymore uniform in shape than a falseaneurysm, which tends to have asac-like appearance. The decision toligate the proximal and distal endsof the artery or to repair the artery isdetermined by the adequacy of col-lateral circulation. In patients withinadequate collateral circulation, theartery is repaired either end-to-endor, when the gap is large, with aninterpositional vein graft.

Vascular MalformationVascular malformations result

from errors in development of thevascular system during the fourththrough tenth fetal weeks. Thesemalformations are present at birth,although they may not become clini-cally evident until adulthood. Vas-cular malformations generally areclassified as low-flow (capillary,venous, lymphatic) or high-flow (ar-terial) lesions.14 Clinically differenti-ating a vascular malformation from ahemangioma is not always possible.One major difference is that a heman-gioma often will spontaneously invo-lute, whereas a vascular malforma-tion will not.

Venous malformations are themost common of low-flow lesions.They present as a blue swelling or a mass that increases in size withthe hand dependent and decreaseswith the hand elevated. Patientstypically present because of a cos-metic deformity and an uncomfort-able, heavy feeling in the hand,especially when it is in a dependentposition. They also may complainof pain caused by either local neu-rovascular compression or throm-bophlebitis. Imaging studies suchas MRI, MRA, and conventionalangiography can delineate theextent of the lesion. Surgery is indi-cated to relieve pain when nonsur-gical measures such as compressiongarments, elevation, and pain med-ications are unsuccessful, as well as

Tumorlike Lesions and Benign Tumors of the Hand and Wrist

Journal of the American Academy of Orthopaedic Surgeons132

to improve the aesthetic appearanceof the area. Recurrence after com-plete excision is uncommon, butwhen the lesion is diffuse andextensive, complete excision fre-quently is not feasible. In suchcases, staged resections or simplydebulking the lesion usually willprovide temporary symptomaticrelief. Repeated debulkings aresometimes necessary, and in severecases, amputation of a digit may berequired.

Tumorlike Lesions of Bone

Cystic LesionsIn order of relative frequency,

radiographically apparent cysts ofthe wrist and hand are intraosseousganglions, aneurysmal bone cysts,and unicameral bone cysts. As agroup, these lesions are far morecommon outside the hand and wrist.

An intraosseous ganglion is themost common bony cystic lesion ofthe hand and wrist, usually occur-ring in a carpal bone (Fig. 1). It is farless common than a soft-tissue gan-glion, although the histologic charac-teristics are identical. The etiology ofan intraosseous ganglion remainsunknown, and controversy contin-ues concerning the existence of aconnection between lesion and joint.For the symptomatic cyst, treatmentis curettage and grafting.

An aneurysmal bone cyst is abenign, locally aggressive lesion ofunknown etiology that clinicallybehaves similarly in the hand andwrist as elsewhere in the body.Aneurysmal bone cysts involving thehand account for approximately 5%of cases throughout the body. Theyare more common in metacarpalsthan in phalanges.15 Adolescents andyoung adults are more commonlyaffected. Conventional radiographsshow an expansile, lytic lesion withcortical destruction. Fluid-filled lev-els on MRI can confirm the diagnosis.Treatment depends on the location of

the lesion and the extent of bone de-struction. Curettage and packingwith either autogenous bone graft or, more recently, graft substitutesand/or allografts, is usually suffi-cient. When an aneurysmal bonecyst destroys the entire cortical shellof the bone, a primary amputationcan be considered. That is more suit-able for a lesion in a distal phalanx asan amputation at that site causes lessfunctional impairment than would amore proximal amputation. Recur-rences are common (up to 50%) andare treated with repeat curettage.

A unicameral bone cyst is rare inthe hand and wrist. However, theclinical and radiographic features ofthese cysts are similar to those in theproximal humerus and femur. Theyusually are discovered either as anincidental finding on radiographstaken for an unrelated problem orafter a pathologic fracture. A varietyof treatments has been recommend-ed, including observation, aspirationand injection of steroids into thelesion, and curettage and graftingwith autogenous graft or bone sub-stitute material.

Giant Cell ReparativeGranuloma

Giant cell reparative granuloma isa benign, reactive, intraosseous lesionof unknown etiology that developsin the metaphyseal/diaphyseal areaof small tubular bones. The lesiondoes not cross an open epiphysealplate, although in skeletally maturepatients it can involve the epiphy-seal end of the bone. Clinically,most patients are young (10 to 25years) and present with pain,swelling, and tenderness followingminor trauma. Radiographically,the lesion appears expansile andradiolucent with cortical thinning(Fig. 2). Microscopically, a giant cellreparative granuloma is composedof a fibrous stroma with spindle-shaped fibroblasts and multinucleat-ed giant cells arranged in a patchydistribution. There are also areas ofmetaplastic bone formation andhemorrhage. The lesion may be dif-ficult to differentiate from other lyticlesions containing giant cells, suchas aneurysmal bone cysts, giant celltumors, and brown tumors of hyper-parathyroidism. Treatment is curet-tage and grafting.16 Recurrencesrange in frequency from 20% to 40%and are treated similarly.

Brown TumorBrown tumor is a common le-

sion associated with both primaryhyperparathyroidism (parathyroidadenoma) and secondary hyper-parathyroidism (chronic renal dis-ease with inability to excrete phos-phate). Both conditions result in anoverproduction of parathyroid hor-mone that causes increased osteo-clastic activity, leading to boneresorption and trabecular fibrosis(osteitis fibrosa cystica). Browntumors develop in areas of exces-sive bone resorption and hemor-rhage, and they are commonly seenin distal phalanges. Radiographicfeatures include endosteal resorp-tion and scalloping associated withan intraosseous lytic lesion. The

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Vol 11, No 2, March/April 2003 133

Figure 1 Posteroanterior radiograph of anintraosseous ganglion in a lunate bone(arrows). MRI may be required to differ-entiate it from osteonecrosis (Kienböck’sdisease).

diagnostic workup should includea complete screening panel to de-termine the nature of metabolicdysfunction.

Reactive Bone Surface LesionsReactive bone surface lesions

include florid reactive periostitis,bizarre parosteal osteochondroma-tous proliferation (Nora’s lesion),and acquired osteochondroma (tur-ret exostosis). These lesions aremost commonly found in the pha-langes of adults (25 to 45 years).Although each lesion presents as adifferent clinical pathologic entity, asingle unifying etiology has beenproposed.17 Each lesion may repre-sent a different stage in the matura-tion and organization of subperi-

osteal hemorrhage that follows aninitial traumatic event.

Florid reactive periostitis, thefirst stage in the process, appears asan ill-defined density arising fromthe surface of the bone. The lesion,containing varying amounts of cal-cific material, will sometimes growrapidly over days or weeks. Micro-scopically, it resembles early frac-ture callus with active proliferationof cartilage cells and fibroblasts.

Bizarre parosteal osteochondro-matous proliferation (Nora’s lesion)is the next stage in the maturationprocess. Radiographically, there is aclearly defined, cap-shaped lesionattached to the surface of the bone,containing a patchy or linear patternof mineralization. Histologic sec-tions show periosteal lamellar bone,irregularly covered with hyper-cellular cartilage (Fig. 3). There isusually atypia of the cartilage cellsthat can be mistakenly diagnosed asa malignancy if the pathologist isunaware of the clinical and radio-graphic features of the lesion.

Acquired osteochondroma orturret exostosis is the end stage ofthe maturation process. Radio-graphically, the lesion has a well-developed pattern of linear mineral-ization at its base that is fused withthe cortex of the underlying bone.A subungual exostosis is a turret exostosis that arises from a distalphalanx.

Benign Tumors of SoftTissues

Giant Cell Tumor of the Tendon Sheath

A giant cell tumor of the tendonsheath is the second most commonlesion of the hand and wrist. It isalso referred to as localized nodulartenosynovitis, pigmented villo-nodular tenosynovitis, fibrous xan-thoma, and benign synovioma. Agiant cell tumor of the tendonsheath presents as a slow-growing,firm, nontender fixed mass with apredilection for the radial three dig-its, particularly in the area of thedistal interphalangeal joints. Thetumor is often multinodular and hasa propensity to involve the neigh-boring joint. Radiographs mayappear normal or show a soft-tissuemass. Long-standing lesions adja-cent to bone may cause pressureerosion of the cortex; rarely is thereany bony invasion (Fig. 4, B).

Histologically, the tumor is simi-lar to intra-articular pigmented vil-lonodular synovitis, although ittends to be more solid and nodular.Treatment is excision of the tumor.The surgical dissection should bemeticulous to ensure that any exten-sion of tumor into the joint is re-moved (4, C). This will notably re-duce a recurrence rate that is reportedto range as high as 50%. Recur-rences also are treated with local

Tumorlike Lesions and Benign Tumors of the Hand and Wrist

Journal of the American Academy of Orthopaedic Surgeons134

Figure 2 Posteroanterior radiograph of agiant cell reparative granuloma (arrow) inthe diaphyseal portion of the fifth meta-carpal in a 14-year-old boy who had hadtrauma to the hand 5 months earlier. Thelesion is lytic with mild expansion and cor-tical thinning. (Reprinted with permissionfrom Toolan BC, Steiner GC, Kenan S:Tumor-like lesions, in Spivak JM, DiCesare PE, Feldman DS, Koval KJ, RokitoAS, Zuckerman JD [eds]: Orthopaedics: AStudy Guide. New York, NY: McGraw-Hill,1999, p 286.)

Figure 3 Lateral radiograph (A) and intraoperative photograph (B) of bizarre parostealosteochondromatous proliferation (Nora’s lesion).

A B

excision,18,19 although the lesionscan be quite invasive.

Fibroma of the Tendon SheathA fibroma of the tendon sheath is

a circumscribed tumor, rarely >2 cmin diameter, attached to the tendonsheaths of digits. The thumb is themost commonly involved digit. His-tologically, the tumor resembles a giant cell tumor of the tendonsheath but with much less cellulari-ty and without xanthoma cells orgiant cells.20

LipomaThese lesions are composed of

mature adipose tissue and clinicallyare usually soft and nontender,although they may feel firm withindistinct borders when locatedbeneath muscle or fascia. A lipomacan grow to an unusually large sizeand still be asymptomatic. On MRI,lipomas are homogeneous, well-cir-cumscribed masses with signal

intensities similar to those of normalfat. At surgery, the tumor usuallycan be easily separated from sur-rounding structures by blunt dissec-tion. Care must be taken to identifyand protect neurovascular structuresthat are often displaced by the tu-mor. Recurrence after marginalexcision is rare.

HemangiomaA hemangioma consists of pro-

liferating blood vessels and usuallyappears within the first few yearsof life. Typically, the tumor has arapidly growing proliferativephase that may last up to 1 year,followed by an involutional phaseduring which the tumor graduallyfades and regresses. Approxi-mately 50% of hemangiomas invo-lute by age 5 years and 70% by 7years.21 Tumors that do not pre-sent within the first few years oflife are less likely to spontaneouslyinvolute.22

The clinical appearance of ahemangioma varies depending onits location. When superficial, it hasa circumscribed appearance, butwhen it is deep, it may be difficultto distinguish from a venous mal-formation. MRA and/or angiogra-phy are helpful in establishing thediagnosis. Treatment for tumorsthat appear during infancy consistsof observation and reassuring theparents that it is likely to involuteby age 7 years. Hemangiomas aresometimes complicated by bleeding,ulceration, infection, or a coagu-lopathy. Bleeding and ulcerationare fairly common and are treatedby compression of the lesion andlocal wound care. Infections gener-ally respond rapidly to antibiotics.The coagulopathy is a thrombocy-topenia secondary to platelet trap-ping in the tumor (Kasabach-Merrittsyndrome). This rare conditionusually is associated with largehemangiomas in major musclegroups outside the hand.

Surgery is reserved for tumorsthat do not undergo spontaneousinvolution and remain sympto-matic, that cause functional impair-ment, or that are aesthetically dis-pleasing. Recurrence is related tothe size, location, and degree of soft-tissue infiltration of the originallesion, as well as to the complete-ness of the primary excision. Re-currence of hemangiomas in thehand is very low, with a reportedincidence of 2%.22

Glomus TumorA glomus body is an apparatus

regulating normal blood flow andtemperature, located in the dermalreticular layer of the skin. Glomusbodies are situated throughout thebody, but they are most common insubungual areas, the lateral aspectsof digits, and the palm. A glomustumor is a benign tumor that con-tains modified perivascular smoothmuscle cells, a component of a glo-mus body. The classic clinical pic-

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A B

C

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Figure 4 A, Giant cell tumor of the tendon sheath. B, Posteroanterior radiograph. Themass had been present for several years and caused a pressure erosion of the underlyingproximal phalanx (arrow). C, At surgery, the nodular lesion was found to be attached tothe tendon, and there was a small intracapsular extension into the proximal interpha-langeal joint (tip of probe). D, Photomicrograph showing sheets of round to ovoidmononuclear cells and osteoclast-like giant cells (arrow) with thin bands of collagenousstroma (hematoxylin-eosin, original magnification ×125).

ture is a blue-red subungual lesionaccompanied by the symptom triadof cold hypersensitivity, paroxysmalpain, and exquisite point tenderness.The history and physical examina-tion usually are sufficient to makethe diagnosis, and surgical excisionof the tumor is curative. When thetumor is subungual, the nail is re-moved and a longitudinal incision ismade in the nail bed. The tumorthen can be identified and excised.The nail bed usually can be repaired,but if this is not feasible, a graft fromeither an adjacent area of the samenail bed or from the nail bed of a toecan be harvested. The nail is thenreplaced because it serves as anexcellent biologic dressing. Multipleglomus tumors occur in approxi-mately 10% of patients.20

Schwannoma (Neurilemoma)and Neurofibroma

A schwannoma, often referred toas a neurilemoma, is a benign nervetumor composed almost entirely ofSchwann cells. The tumor consistsof hypercellular (Antoni A cell) andhypocellular (Antoni B cell) areas;the nuclei of the spindle cells have apalisading arrangement referred toas Verocay bodies. Most schwanno-mas are asymptomatic, but somecan cause neurologic deficits result-ing from compression of nervefibers. When superficial, the tumormay be palpated, and there is oftena Tinel sign with percussion over it.A schwannoma typically is well cir-cumscribed and eccentrically locat-ed on a peripheral nerve. The fasci-cles of the nerve do not enter thetumor but are splayed over it. Withlarge tumors, there may be com-pression of the fascicles, resulting in some neurologic deficit. Mostschwannomas are solitary lesions,but multiple lesions within a singlenerve or nerve trunk do occur. Thetumor is excised using magnifica-tion and microsurgical techniques toreduce the risk of iatrogenic injuryto the nerve fibers. Malignant trans-

formation of a schwannoma is ex-tremely rare.23,24

Clinically, solitary neurofibromasbehave in a fashion similar to that ofa schwannoma, but with severalimportant differences. Althoughboth tumors arise from Schwanncells, neurofibromas contain peri-neural cells, fibroblasts, and mucoidmaterial. They also are more likely tobe associated with multiple lesions, acondition referred to as neurofibro-matosis or von Recklinghausen’s dis-ease. Unlike a neurilemoma, a neu-rofibroma is intimately connectedwith the nerve fascicles, and it is usu-ally not possible to separate the twosurgically. This is not a problemwhen the tumor is subcutaneous andpresents as a firm, circumscribednodule; then it is simply excised, andoften the diagnosis is not made untilmicroscopic sections of the lesion arestudied. However, treatment for theneurofibroma of a large nerve, suchas the median or ulnar nerve, is moreproblematic. To remove the tumor,the involved nerve segment isexcised, followed by end-to-endrepair or interposition of nerve graftswhen the tumor is large. Becausesuch treatment results in some per-manent neurologic deficit, it isreserved for the lesion that is verysymptomatic or demonstrates malig-nant characteristics, such as rapidgrowth or increasing pain. If excisionis not done, an intralesional biopsy iswarranted for definitive diagnosis. Asimilar treatment protocol is followedwith multiple tumors. Whereas thepotential for malignant degenerationof a solitary neurofibroma is rare,malignant degeneration in neurofi-bromatosis has been reported to be ashigh as 15%.23,25 This percentage isfor lesions in all locations; no specificfigures are available for lesions isolat-ed in the hand.

Extraosseous Chondroma andSynovial Chondromatosis

Extraosseous chondromas areslow-growing, painless, nontender

masses, most commonly seen in thefingers. They are usually firm andwell demarcated and rarely exceed3 cm in diameter. Radiographs fre-quently show focal or diffuse calcifi-cation within the lesion and, occa-sionally, pressure erosion on theunderlying bone. Histologically,the tumor consists of mature lobu-lated hyaline cartilage with occa-sional areas of fibrosis and/or myx-oid material.26 In approximatelyone third of cases, calcification is sosevere that the cartilaginous basis ofthe tumor is obscured and the histo-logic appearance can mimic tumoralcalcinosis.

Synovial chondromatosis differsfrom chondromas by its occurrencein joints or tendon sheaths (Fig. 5). It develops through cartilaginousmetaplasia of synovial tissue. Tu-mors that arise from the synovialmembrane of joints also can have anextra-articular tenosynovial compo-nent. Typically, multiple cartilagi-nous foci calcify into nodules that ap-pear as radiodense lesions on radio-graphs (Fig. 5). MRI is often usefulbecause some lesions are not calci-fied and cannot be visualized on con-ventional radiographs. Commonsymptoms include pain, swelling,and decreased mobility of the in-volved joint. Treatment consists ofexcision of the cartilaginous bodiesand the involved synovium, eitherarthroscopically, when the tumor isin the wrist joint, or as an open pro-cedure. An open surgical techniqueis necessary when there is an extra-articular component. Recurrencesare rare.27

LeiomyomaA leiomyoma is a smooth muscle

tumor that occasionally occurs inthe subcutaneous tissues of thehand and digits.28 The tumor iswell circumscribed, gray-white, andusually pain free. Angiomyoma,also referred to as a vascular leio-myoma or angioleiomyoma, is avascular variant of a leiomyoma. It

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is sometimes calcified and usually ispainful.

Granular Cell TumorA granular cell tumor usually

presents as a small, poorly circum-scribed nodule in subcutaneous tis-sues. The tumor is most commonlyfound in African-American womenin their fourth through sixthdecades. Rarely is the correct diag-nosis made before microscopicexamination of the biopsy speci-men. These tumors were originallyreferred to as granular cell myoblas-tomas, but because it is generallyaccepted that they arise from neuraltissue, “myoblastoma” has beendropped from the description.

Benign Tumors of Bone

Cartilage-Forming TumorsCartilaginous tumors are the

most common type of primary bonetumor in the hand. Some are foundincidentally; others present withpain, swelling, limited range of mo-tion, and sometimes a pathologicfracture. Cartilage-forming tumorsinclude enchondromas, periostealchondromas, and osteochondromas.(Chondroblastomas and chondro-myxoid fibromas are exceedinglyrare in the hand and wrist.)

The enchondroma is the mostcommon primary bone tumor in thehand, and it represents approximate-ly 40% of cases throughout the

body.29 In the hand, enchondromasusually occur in proximal phalanges,followed in frequency by metacarpalsand middle phalanges. Radiographi-cally, the lesion is centrally located,well circumscribed, radiolucent, andoften associated with punctate calcifi-cations. Medullary expansion andcortical thinning are frequent (Fig. 6).The histologic characteristics ofenchondromas in the hand are some-times different from those of enchon-dromas at other sites in the body. Inthe hand, there is often greater cellu-larity and atypia, which are not a con-cern if the clinical examination andradiographs are consistent with abenign lesion. Frozen sections of thetumor at surgery are therefore unnec-

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A B

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Figure 5 A, Synovial chondromatosis in the metacarpophalangeal joint of the little finger, presenting as a hard, irregular mass in thehypothenar eminence. B, Palmar radiograph showing erosion of the metacarpal head and radiodensities in the soft tissues. C, Tumor tis-sue within and outside the joint (arrow). D, Photomicrograph showing cellular metaplastic nodules of hyaline cartilage arising in synovi-um (hematoxylin-eosin, original magnification ×80).

essary unless radiographs suggest a malignant tumor. If there is anydoubt, definitive treatment should bedeferred until the permanent sectionsare available.

Treatment is curettage of the tu-mor and packing with autogenouscancellous bone, bone graft substi-tute, and/or allograft. When autog-enous bone is used, the donor areafor the graft should be segregatedfrom the tumor site by using sepa-rate instruments and differentgloves. If the pathologic fracturesite is unstable, it is preferable todefer surgery for several weeksuntil the fracture becomes stable; ifthere is no instability, surgery neednot be delayed. Recurrence is rareand usually is the result of an in-complete curettage. Treatment isrepeat curettage and bone grafting.

Malignant transformation of asolitary enchondroma into a chon-drosarcoma or osteosarcoma is rare.

However, with multiple enchondro-matosis (Ollier’s disease), malignanttransformation has been reportedto develop in 25% of cases, and inMaffucci’s syndrome (multiple en-chondromatosis with multiple he-mangiomas or multiple lymphan-giomas), the incidence of malignanttransformation is even higher.30

Periosteal chondromas are carti-lage tumors that usually involvelong bones, such as the femur andhumerus. In the hand, they mostcommonly occur on the cortical sur-faces of phalanges in young patients(10 to 25 years). Radiographs showcortical scalloping. These tumorsoccur far less frequently than doenchondromas, but their histologicappearance is sometimes more ag-gressive. Treatment is surgical exci-sion, accomplished either intralesion-ally or by en bloc resection.

Osteochondromas are corticalbony prominences with cartilagi-nous caps that arise in continuitywith the intramedullary canal of thebone. Solitary osteochondromas arecommon in the upper extremity, butthey are rare in the hand.12 They aremost frequently seen in multipleosteochondromatosis. When a soli-tary osteochondroma does occur, itusually involves a proximal phalanx(Fig. 7). Excision is reserved forlarge lesions that interfere with digi-tal function or are cosmetically unac-ceptable. Malignant degeneration ina solitary lesion in the hand is ex-tremely rare.

Bone-Forming TumorsOsteoid osteomas and osteoblas-

tomas are benign bone-formingtumors that generally become symp-tomatic in the second and thirddecades of life. Pain and local ten-

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A B

Figure 6 A, Posteroanterior radiograph of an enchondroma showing cortical expansion,endosteal scalloping, and stippled calcifications. (Reprinted with permission from SteinerGC: Benign cartilage tumors, in Taveras JM, Ferrucci JT [eds]: Radiology. Philadelphia,PA: Lippincott, 1986, p 7.) B, Photomicrograph showing a hyaline cartilage tumor com-posed of chondrocytes with small uniform nuclei lying within lacunae (hematoxylin-eosin,original magnification ×120).

A B

Figure 7 Lateral radiograph (A) and intraoperative photograph (B) of an osteochondromaarising from the head of a proximal phalanx, an unusual site for the tumor. The corticaland cancellous bone of both tumor and phalanx are in continuity, in contradistinction to aNora lesion (Fig. 3).

derness are the most common com-plaints. Pain tends to be more se-vere at night and usually is relievedby nonsteroidal anti-inflammatorymedication (NSAIDs). The painprobably is related to the high levelsof prostaglandin E2 and prostacyclinfound in the tumor.31

Osteoid osteomas also can bepainless, especially when they occurin the fingers. Most lesions occur inproximal phalanges. Local swellingis usually the most important clini-cal sign in such cases, and it canmimic an inflammatory process.32

The classic radiographic appearanceis a small, central, radiolucent lesionor nidus <1 cm in diameter sur-rounded by an area of reactive scle-rosis (Fig. 8). In approximately 25%of cases, a nidus is not seen on con-ventional radiographs. In these situ-ations, CT can help to confirm thediagnosis. Treatment is surgicalexcision of the nidus. Persistence ofpain postoperatively is more com-mon with osteoid osteoma lesions inthe hand and wrist than elsewhere,probably because of incompleteexcision of the nidus. In some cases,the nidus is very small and can bemissed at surgery. Because someosteoid osteomas eventually “burnout,” nonsurgical treatment is anoption in patients who respondfavorably to NSAIDs.

An osteoblastoma is a rare bone-forming tumor that is histologicallysimilar to an osteoid osteoma, butwith several differences. Osteo-blastomas are larger (usually ≥2 cmin diameter), generally do not re-spond to NSAIDs, and tend to in-crease in size. Resection is usuallycurative. With local bone destruc-tion or a recurrence, marginal resec-tion followed by reconstruction isindicated.

Giant Cell Tumor of BoneGiant cell tumor of bone is a rare

benign lesion that should be distin-guished from other giant cell le-sions, such as giant cell reparative

granuloma, aneurysmal bone cyst,and brown tumor of hyperparathy-roidism. Giant cell tumors in thesmall tubular bones of the handrepresent only 2% to 5% of all suchtumors, but they are associatedwith a higher postoperative recur-rence rate than are similar tumorselsewhere in the body.33 The mostcommon presenting complaints are

pain and swelling. Radiographstypically show an aggressive-appearing, radiolucent, expansilelesion with indistinct borders in-volving epiphyseal bone. Fre-quently, there is cortical destructionand extension into the soft tissues(Fig. 9). Some giant cell tumors arenonepiphyseal in location andshow nonspecific radiographic fea-

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Figure 8 A, Osteoid osteoma of the proximal phalanx of the middle finger with localswelling and tenderness. B, Posteroanterior radiograph showing a radiolucent lesion ornidus surrounded by sclerotic bone. C, Photomicrograph of the nidus showing irregulartrabeculae of woven bone (arrow) bordered by osteoblasts (left of the arrow) and osteo-clasts, separated by blood vessels (hematoxylin-eosin, original magnification ×120).

A B

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tures similar to those of giant cellreparative granulomas and aneu-rysmal bone cysts.

Treatment can be problematicbecause of the tumor’s aggressivenature. Although the recurrencerate after curettage and grafting ishigh, one attempt is still a reason-

able surgical approach. For recur-rent tumor, en bloc resection andbridging the defect with autograft,allograft, and/or bone graft substi-tute is necessary. For a tumor thatcauses extensive bone destructionand extends into the soft tissues,particularly when it involves a pha-

lanx, partial or total amputation ofthe finger may be required.

Summary

The diagnosis and effective treat-ment of tumorlike lesions andtumors of the hand and wrist requirethe collaboration of the orthopaedicsurgeon, radiologist, and patholo-gist. The first steps in the diagnosticworkup are a complete history andclinical examination. Radiographsare useful even when the lesion is inthe soft tissues because radiodensi-ties may provide valuable clues tothe nature of the lesion. There maybe phleboliths in the lesion (vasculartumor), or the lesion may containareas of mineralization in the form ofcalcification (cartilage matrix) oractual bone. More specialized imag-ing studies, such as CT, MRI, MRA,and angiography, also may be indi-cated. Oncologic surgical principlesalways should be followed, andwhenever there is any doubt as tothe nature of the lesion, an incisionalbiopsy should be done before pro-ceeding with the definitive surgicalprocedure.

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