chodroblastoma

Chondroblastoma andChondromyxoid Fibroma

Abstract

Chondroblastoma and chondromyxoid fibroma are benign butlocally aggressive bone tumors. Chondroblastoma, a destructivelesion with a thin radiodense border, is usually seen in theepiphysis of long bones. Chondromyxoid fibroma presents as abigger, lucent, loculated lesion with a sharp sclerotic margin in themetaphysis of long bones. Although uncommon, these tumors canbe challenging to manage. They share similarities in pathology thatcould be related to their histogenic similarity. Very rarely,chondroblastoma may lead to lung metastases; however, themechanism is not well understood.

Chondroblastoma is a rare, be-nign bone tumor, usually lo-

cated in the epiphysis or apophysisof long bones. It was first describedby Kolodny in 1927 as a cartilage-containing giant cell tumor (GCT)but was better characterized by Cod-man in 1931, who believed it to bean “epiphyseal chondromatous giantcell tumor” involving the proximalhumerus.1 In 1942, Jaffe and Lich-tenstein,2 after a comprehensive re-view, included tumors in locationsother than the proximal humerusand designated the tumor as benignchondroblastoma of bone, that is, asa different, separate entity fromGCT. Historically, because of Cod-man’s great contribution, chondro-blastoma of the proximal humeruswas referred as “Codman tumor.”

Chondromyxoid fibroma (CMF), arare mixture of benign cartilage and fi-brous and myxoid tissue that generallydevelops in long bones of the lower ex-tremity, was described in 1948 by Jaffeand Lichtenstein.3 Prior to their de-scription, the lesion was thought tobe a myxoma of the bone, enchon-droma, or chondrosarcoma.

Epidemiology

Chondroblastoma represents 1% to2% of all primary bone tumors andapproximately 5% of benign bonetumors.4-6 The ratio of male to fe-male patients is approximately2:1.4,5,7-10 Although chondroblastomahas been reported in patients rangingin age from 2 to 73 years, most pa-tients are aged <20 years.7-9,11,12

The bone most affected by chon-droblastoma is the femur, followedby the humerus and tibia.7,9-12 Re-ports in the literature fluctuate be-tween identifying the proximal hu-merus and proximal femur as themost affected site.7-9,11,12 In the foot,chondroblastoma is located espe-cially in the talus and calcaneus, inan apophysis or near the articularsurface.13 Chondroblastoma can alsooccur in flat bones, such as the scap-ula, patella, sternum, and skullbones.5 The average age of patientswith chondroblastoma in small orflat bones is higher than that of pa-tients with chondroblastoma of longbones.6,13 About 0.5% to 1% ofchondroblastomas present on verte-

Camila B. R. De Mattos, MD

Chanika Angsanuntsukh, MD

Alexandre Arkader, MD

John P. Dormans, MD

From the Department ofOrthopaedic Surgery, The Children’sHospital of Philadelphia,Philadelphia, PA (Dr. De Mattos andDr. Dormans), the Department ofOrthopaedic Surgery, RamathibodiHospital, Mahidol University,Bangkok, Thailand(Dr. Angsanuntsukh), Children’sHospital Los Angeles, Los Angeles,CA (Dr. Arkader), and the Universityof Pennsylvania School of Medicine,Philadelphia (Dr. Dormans).

Dr. Arkader or an immediate familymember serves as a paid consultantto or is an employee of BiometTrauma. Dr. Dormans or animmediate family member serves asa board member, owner, officer, orcommittee member of the PediatricOrthopaedic Society of NorthAmerica, the Scoliosis ResearchSociety, the International Society ofOrthopaedic Surgery andTraumatology (SICOT) Foundation,SICOT USA, and the WorldOrthopaedic Concern. Neither of thefollowing authors or any immediatefamily member has receivedanything of value from or has stockor stock options held in acommercial company or institutionrelated directly or indirectly to thesubject of this article: Dr. De Mattosand Dr. Angsanuntsukh.

J Am Acad Orthop Surg 2013;21:225-233

http://dx.doi.org/10.5435/JAAOS-21-04-225

Copyright 2013 by the AmericanAcademy of Orthopaedic Surgeons.

Review Article

April 2013, Vol 21, No 4 225

brae, generally in the posterior ele-ments and/or the body.14

A prevalence of <0.5% of all bone tu-mors is reported in many series describ-ing CMF.6,15,16 There is a slight malepredominance.17 CMF most com-monly arises in young patients in thesecond or third decades of life.3,17,18

Most of these tumors are located inthe metaphysis of long bones withvariable distances from growth plate,mainly in lower extremities.17 Rarely,the lesion involves the epiphysis. Thediaphysis can be involved, especiallyin large tumors. A lesion in smallbones, such as phalanges, may in-volve the bone in its totality. Theproximal tibia is the most commonsite, comprising 28% to 52% of alllower extremity lesions in the litera-ture.17,19 This site is followed by theilium, ribs, distal femur, metatarsals,and distal tibia.17 In contrast tochondroblastoma, CMF is rarely en-countered in the humerus.15,17

Etiology

Although cytogenetic abnormalitiescan be highly specific for some tu-

mors, there is no single characteristicabnormality or chromosomal break-ing point specific for chondroblas-toma or CMF.

The histogenesis of chondroblas-toma and CMF is still uncertain. Ro-meo et al20 confirmed the active roleof cartilage-signaling molecules, bothIndian Hedgehog/parathyroid hor-mone–related protein (IHh/PTHrP)and fibroblast growth factor, indicat-ing that chondroblastoma is a neo-plasm that originates from a mesen-chymal cell committed towardchondrogenesis via active growthplate signaling pathways. This con-clusion supports the chondrogenicnature of this tumor and the close re-lationship between the physis andthe tumor.21 CMF has myofibroblas-tic differentiation in its “fibrous” ar-eas driven by transforming growthfactor β-1.22 A strong expression ofthe Sox9 gene, which is responsiblefor chondrocytic differentiation aswell as regulation of the expressionof cartilage-specific genes in maturechondrocytes, especially the synthe-sis of collagen type II, was found inboth chondroblastoma and CMF.23,24

This demonstrates that the expres-sion of Sox9 in these tumors is con-sistent with its commitment to theearly phases of cartilage differentia-tion, with chondroblastoma being amore “immature” tumor than CMFbecause of the greater presence ofpositive Sox9 in CMF cells.23

Pathology

Grossly, a chondroblastoma is agray-white tumor with yellowish ar-eas, usually because of calcification,which can be soft, rubbery, or fria-ble.2 Microscopically, chondroblas-toma reveals proliferation of mono-nuclear cells.10 The tumor ischaracterized by compact areas ofround, oval, or polygonal chondro-blasts with well-defined cytoplasmic

borders. The cells contain one or tworound, oval, slightly indented, oreven multilobulated nuclei with orwithout nucleoli.8 Occasional cellsmay have enlarged nuclei withoutnuclear atypia.4,25 The presence ofmitotic figures is scarce.2,4,25 Thereare scattered multinucleated osteo-clast-type giant cells among thechondroblasts.7,8 There may be fociof chondroid matrix formed by thechondroblasts.25 Dystrophic calcifi-cation is occasionally present andmay surround individual cells, givingthe classic “chicken wire” appear-ance7,8 (Figure 1), although this isnot mandatory for diagnosis.

In 15% to 32% of cases, chondro-blastoma may be associated withsecondary aneurysmal bone cyst(ABC).7-9,11 Although the reasons forthis association are unclear, hypothe-ses include mechanical stress,trauma, and hemorrhage.13 More ag-gressive chondroblastoma that cancause metastases or recurrence showsno difference in histology comparedwith less aggressive chondroblas-toma.25 The histology is equivalentto that of the primary site, and thepresence of atypical cells is rare.25

Grossly, CMF appears as lobulated,well-circumscribed, and sharply demar-cated from the adjacent bone marrow.The lesion is firm and white. The cutsurface shows a solid tumor mass thatis yellow, grayish-white, or blue-gray.6 Microscopic analysis of CMFreveals three components: myxoma-tous zones, fibrous zones, and fieldsthat appear chondroid. The classichistologic features of CMF are lob-ules of stellate or spindle-shaped cellsin abundant myxoid background orchondroid intercellular material.Scattered giant cells are found in ap-proximately 50% of cases, usually atthe edge of the lobules16,17 (Figure 2).These lobules have a hypocellularcenter and a condensation of the nu-clei toward the periphery, creating ahypercellular periphery. The inter-

Photomicrograph ofchondroblastoma. Note the diffuseand compact proliferation ofmononuclear cells with indentednuclei with abundant eosinophiliccytoplasm and distinct cell borders.There is presence of focalpericellular (ie, chicken wire)calcification (arrows) on the top leftcorner (hematoxylin-eosin, originalmagnification ×400).

Figure 1

Chondroblastoma and Chondromyxoid Fibroma

226 Journal of the American Academy of Orthopaedic Surgeons

lobular tissue is composed of oval orspindle-shaped cells.

Mitotic features are uncommonand are usually present in more con-centrated cellular interlobular areas.Atypical mitotic features are notfound, although some cells are largeand have irregularities in the size andshape of nuclei.17 Cellular atypia wasreported in 18% of cases by Wuet al;17 however, there was no changein the nucleocytoplasmic ratio. Le-sions in hands and feet are morelikely to have atypical cells.17

Myxoid stroma in CMF stainsuniformly and does not show exten-sive liquefaction, as is present inmyxoid foci of chondrosarcoma.Nevertheless, small foci of liquefac-tive changes are found in approxi-mately 30% of CMF cases.6

Cyst formation, necrosis, foamcells, foci of secondary ABC, andfrank hyaline cartilaginous areas areunusual findings.6,17 Calcificationsare present in approximately onethird of the lesions and appear asfine lacelike or plaquelike deposits.6

Histochemical analysis of chondro-blastoma and CMF demonstratesgreat positivity to collagen type IIand S-100 protein.8,10,24,26,27

Clinical Presentation

Chondroblastoma and CMF are usu-ally classified, using the Enneking be-nign bone tumor classification, asstage 2 (ie, active) or 3 (ie, aggres-sive). The delay between the onset ofsymptoms and diagnosis varies from<1 month to years. Although presen-tation of these tumors can be similar,chondroblastoma is typically morepainful than CMF.

Chondroblastoma normally has aninsidious presentation. Symptomsvary from mild to significant painand the presence of a soft-tissue massor even a pathologic fracture. Pa-tients with chondroblastoma may re-port pain lasting for several weeks,months, or even years. Pain with lo-cal tenderness in the involved boneand the adjacent joint is the most fre-quent complaint, followed by de-creased range of motion.4,5,8,10-12

Some patients attribute the pain totrauma, usually a minor or sports-related injury.7 Swelling or joint effu-sion, a limp (when the affected boneis in the lower extremity), and mus-cle wasting may also be seen.5,7,10,12 Apalpable mass is uncommon but may

be present, if the patient has hadsymptoms for several years, becauseof expansion of the lesion.28 Patho-logic fractures in long bones are notcommon at presentation, but whenthe lesion is in the foot, subchondralfracture is frequent and painful.13

CMF may be found incidentallybut more often presents with painthat is usually intermittent and notdistressing. The duration of symp-toms ranges from several months toyears. The second most common pre-sentation is local swelling, a lump, ora palpable mass. The patient maypresent with pain to palpation, andthe lump may slowly increase in size.The local swelling or lump is morecommon in tumor of the smallbones. Limping, limitation of adja-cent joint range of motion, andpathologic fracture are rare.3,6,15,19,29,30

Radiologic Evaluation

The classic radiographic appearanceof chondroblastoma is a well-definedeccentric oval or round lytic lesioninvolving the epiphysis adjacent toan open growth plate4 (Figure 3). Asharp sclerotic margin is often seen.

Photomicrograph of chondromyxoidfibroma. Note the stellate cells in amyxoid background. The stellatecells display mild atypia, butmitoses are rarely seen. The lesionhas a lobulated appearance, withalternating cellular and modemyxoid areas (hematoxylin-eosin,original magnification ×200).

Figure 2

AP (A) and AP with internal rotation (B) radiographs of a chondroblastoma ofthe proximal humerus of a 15-year-old girl who presented with right shoulderpain lasting 4 months. The lesion is located entirely in the epiphysis, islucent, and has thin, sclerotic borders.

Figure 3

Camila B. R. De Mattos, MD, et al

April 2013, Vol 21, No 4 227

At times, the lesion may be mottledor fuzzy or contain areas of calcifica-tion. Lesion size on radiograph var-ies, with most being <4 cm.2,5,8,11 Cal-cifications are found especially inskeletally immature patients.7

It is uncommon to find periostealbone formation on radiographs, butMRI usually depicts edema adjacentto the periosteum.31 In rare cases, es-pecially in older or neglected pa-tients, chondroblastoma may havean atypical presentation that clini-cally and radiographically mimics anaggressive process.5,28,32

The small percentage of chondro-blastomas with a secondary ABC inthe histology usually show differ-ences from regular chondroblastomaon radiograph, and these sometimeslead to confusion in the diagnosis.Cystic changes are seen more com-monly when the lesion is located inbone, such as the patella.13,33

Most chondroblastomas involvethe epiphysis of long bones.6 A smalllesion is usually confined to a part ofthe epiphysis or apophysis, althoughit may extend through the epiphysealplate21 (Figure 4). True metaphysealchondroblastoma is rare; most re-ported cases are of an extension

from the epiphyseal lesion.21 A fewcase reports of diaphyseal chondro-blastoma have appeared in the litera-ture.34 Chondroblastomas located onsmall bones may be more aggressive,with loss of cortical continuity andbony destruction.35

CT can help define the anatomiclimits of the lesion, especially the dis-tance to the growth plate and the re-lation of the lesion to subchondralbone. CT shows stipple calcificationof the cartilaginous matrix, whenpresent. In addition, CT is useful indelimiting lesions in unusual loca-tions as well as subchondral frac-tures not visible on plain radio-graphs.13,14

In a few cases in which MRI wasnot used in conjunction with radio-graphs and the clinical presentation,this modality led to misdiagnosis oroverestimation of tumor aggressive-ness.31 Chondroblastoma usually ishypointense on T1-weighted imagesand variably ranges from hypoin-tense to hyperintense on T2-weighted images, with or withoutperipheral lobulation and the associ-ated marrow and soft-tissue edemathat enhances after administration ofcontrast material31 (Figure 5). Bone

scan shows increased uptake but sel-dom is needed for diagnosis.21

CMF classically presents as a lyticradiolucent medullary lesion with athin sclerotic rim (Figure 6). In mostlesions, borders are sharp, with par-tial or complete effacement of thecortex.16,17,29,36 CMF tends to be ec-centrically located in the metaphysisof long bones. Rarely, or in advancedcases, the lesion crosses the growthplate into epiphysis or extends intothe diaphysis.6 In small bones, CMFgenerally occupies the entire width ofthe bone, causing thinning of corticesand fusiform expansion of the bone.The tumor typically has a scallopedborder that is well defined by a nar-row rim of sclerotic bone. Chronicbone reaction and cortical thickeningare commonly present. Unusual peri-osteal reaction has been re-ported.16,17,36 Pseudotrabeculation,that is, ridges of the sclerotic rim atthe edge of the lesion, is present.Gross and microscopic studies showthat there is no complete bony sep-tum.16

Unlike other cartilaginous tumors,calcification in CMF is unusual. Theprevalence of calcification in CMFranges from 2.4% to 16% of cases

Curettage and bone grafting of a chondroblastoma of the distal femur in an 11-year-old girl with chronic knee pain.A, Preoperative AP radiograph demonstrating a lytic lesion on the distal femur that extends from the epiphysis into themetaphysis. B, Sagittal T1-weighted magnetic resonance image demonstrating peripheral lobulation and associatedmarrow edema. C, Intraoperative AP fluoroscopic image demonstrating curettage and bone grafting of the lesionperformed through a cortical window, thereby avoiding damage to the unaffected surrounding physis. D, AP radiographmade at 7-month follow-up. The patient was asymptomatic, with no complications or recurrence.

Figure 4



radiologically, and from 6.8% to34% of cases histologically.16,36 Cal-cification presents more often in pa-tients aged >40 years and in flatbones.36 Pathologic fractures may befound but are unlikely.17 CT and

MRI are the preferred imaging mo-dalities. CT demonstrates cortical in-tegrity and calcification of the matrixwell. MRI shows low signal on T1-weighted images and increased signalon T2-weighted images and can help

with staging and preoperative plan-ning30 (Figure7).

Differential Diagnosis

The differential diagnosis for chon-droblastoma includes GCT, simplebone cyst, ABC, enchondroma, eo-sinophilic granuloma, fibrous dys-plasia, clear cell chondrosarcoma,subacute osteomyelitis (ie, Brodie ab-scess), and, when a subchondral cystis present, Legg-Calvé-Perthes dis-ease or osteochondritis dissecans.Tuberculosis can mimic the periartic-ular pain and bone lesion of chon-droblastoma and should be consid-ered, especially in developingcountries.6,8,21,25

The differential diagnosis for CMFincludes benign lesions such as GCT,simple bone cyst, ABC, enchondroma,eosinophilic granuloma, fibrous dys-plasia, osteoblastoma, osteofibrousdysplasia, and nonossifying fibro-mas.6,18 Malignant conditions thatmust be differentiated are low-gradechondrosarcoma and myxoid chon-drosarcomas.

Management

The natural history of these tumorsis not completely understood; todate, there has been no evidence ofpotential spontaneous healing.7 Sur-gical management is advised for bothtypes of tumors because no effectivemedical management is available.Both chondroblastoma and CMFgenerally have a favorable prognosiswhen identified and managed appro-priately.

The benchmark management ofchondroblastoma is curettage withbone grafting.8-12,25 The entire tumorshould be excised, with the surgeonfollowing meticulous oncologic crite-ria of a thorough intralesional exci-sion through a cortical and/or epi-physeal window (Figure 4), avoiding

The same patient as in Figure 3. Postcontrast T1-weighted fat-suppressedcoronal (A) and T2-weighted axial (B) magnetic resonance imagesdemonstrating heterogeneously increased signal. The bone marrow isnormal.

Figure 5

AP (A) and lateral (B) radiographs of the proximal tibia of a 22-year-old manwith chondromyxoid fibroma who presented with mild pain of the left kneelasting for 3 months. Note the mild expansion of the tumor and the scallopedborders defined by thin sclerotic bone. Biopsy was performed, and thediagnosis was confirmed. (Adapted and printed with permission fromDr. Olavo Pires de Carvalho, University of São Paulo, São Paulo, Brazil.)

Figure 6


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the growth plate with the help of in-traoperative fluoroscopy.9,10 Curet-tage through the physis with obliter-ation of part or all of the growthplate is an option in patients who arenear the end of skeletal growth.9

Intra-articular exposure should beconsidered to access all of the tumor,if necessary.

Lehner et al37 noted insufficient ev-idence supporting the use of adju-vant therapy. A high-speed burr isuseful, with caution exercised nearthe growth plate and subchondralbone.9,37 Electrocautery, phenol, ar-gon bean coagulation, and cryother-apy also may be used with cau-tion.5,9,11,37 Bone graft is the preferredmaterial to fill the cavitary defect af-ter curettage.9,10 In a series of 47 pa-tients, Ramappa et al11 reported norecurrence of tumor in 8 patientstreated with polymethyl methacry-late (PMMA). Radiotherapy is pro-scribed because of the increased risk

of malignant transformation.5,7,38

Management of a lesion in thefemoral head is challenging becauseof the difficulty of access—more so ifthe epiphysis is not fused.10,33 Thetraditional approach for this lesion isthrough the base of the femoral neckor the trochanter, although a directhip approach can also be used.7,12

Both techniques carry the risk ofspreading tumor into the femoralneck or the hip joint as well as ofdamaging the growth plate.10,33 Theuse of arthroscopy to visually inspectthe cavity following curettage via aminimally invasive approach, similarto core decompression, without com-promising the articular cartilage ofthe adjacent joint, has been success-ful, although reported in only casereports.33,39,40 A trapdoor procedurehas also been described, but it canresult in osteonecrosis and perma-nent damage to the cartilage.12,33,41

Radiofrequency ablation for chon-

droblastoma was recently describedin a small series.42 Results were bestwith small tumors (approximately1.5 cm) and when location of the tu-mor provided limited risk of me-chanical collapse of the adjacent ar-ticular surface. Limited data supportthis method of management, how-ever, so patients must be selectedcarefully.42

Some tumors can be widely ex-cised, especially in bones such as ribsand fibula.9,10 Lin et al9 reported norecurrence in all six patients inwhom chondroblastoma was treatedthrough en bloc resection.

Aggressive recurrences historicallytreated with amputation can now bemanaged with limb-sparing tech-niques and endoprosthetic recon-struction, if feasible.5,12

No clear guideline exists for fol-lowing patients with chondroblas-toma. The risk of late recurrence andlung metastases, although extremelylow, argues for prudent follow-up.Lin et al9 suggested following pa-tients on a yearly basis for at least 5years.9 Plain chest radiographs madepreoperatively and at the annual visitare recommended.

Because CMF is extremely rare,most published articles are from se-ries with patients who were treatedover several decades; thus, there are noultimate recommendations for manage-ment. The options include curettageand excision, with or without filling ofthe cavitary defect. Wide resection oren bloc excision is probably the bestmethod to avoid recurrence, but not alllocations allow the mechanical imbal-ance these procedures can cause, sobone grafting is advised.6,18 CMF canbe locally aggressive; thus, adjuvantssuch as PMMA are recommended (Fig-ure 8). Curettage alone has resulted ina rate of high recurrence in mostseries.16-18,29 Many authors reportthat bone grafting after excisionalcurettage reduces the recurrence rate,with some stating that the rate is

Sagittal T1-weighted fat-suppressed (A) and coronal T2-weightedpostcontrast (B) magnetic resonance images of a chondromyxoid fibroma ofthe distal phalanx of the great toe, demonstrating edema in the soft tissueand an expansive and solid lesion surrounded by a thin shell of residualbone. There is intralesional calcification with discrete contrast enhancementin the periphery of the lesion, characteristic of cartilaginous lesions.

Figure 7



similar to that observed after resec-tion.6,18 Use of PMMA as an adju-vant after excisional curettage re-portedly decreases the rate ofrecurrence.18

Complications

Recurrence of the lesion is the mostcommon complication following man-agement of chondroblastoma andCMF. Although growth disturbancesmay occur following the resection ofthese lesions because of their proxim-ity to the physis, major angular defor-mities and discrepancies are notcommon.9,11,12,33

Functional impairment, degenera-tive joint disease, and pathologicfractures can also result.11,33 Sunejaet al12 described a series of 40 pa-tients with chondroblastoma treatedwith curettage and bone graft withan average Musculoskeletal TumorSociety functional evaluation of94.2%. The higher-scoring patientshad lesions in more accessible areas.

Sarcomatous change has been re-ported in some series of CMF pa-tients, but the prevalence is verylow.6,17

Recurrence

The recurrence rates of chondroblas-toma vary from 5% to 40%; studyresults are inconclusive in determin-ing which patients have greaterchances of recurrence.9,10,12 Most re-cent series report rates of 8% to13%.9,10,12 The recurrence rate forCMF ranges from 20% to 25%.16,18

Some authors state that recurrencein chondroblastoma arises morecommonly in patients with an openepiphyseal plate, but others contra-dict this finding.7-12 Recurrence inskeletally immature patients can beexplained by inadequate curettagedone to avoid damage to the growthplate.7,10 The proximal femur andpelvis have higher rates of recur-rence, likely related to difficulty inaccessing these sites and obtaining

complete excision.7,9,11,12

Some authors have postulated thatpelvic chondroblastoma may bemore biologically aggressive thanother forms of chondroblastoma.5,9,11

Recurrences can occur between 5months to 7 years after the initialprocedure (average, 10 months fol-lowing diagnosis).7,9,12 Recurrence isnot related to one specific mode ofmanagement, tumor size, patient sex,or duration of follow-up.8,10,11 deSilva and Reid8 reported a statisti-cally significant relation between du-ration of symptoms and recurrence.They stated that patients with symp-toms of <6 months had a greaterchance of recurrence; however, toour knowledge, this has not been re-ported by other authors. Sunejaet al12 described a positive associa-tion of young age and higher recur-rence rate, although this, too, hasnot been noted by others.7,9 The as-sociation of chondroblastoma withABC was reported by Huvos andMarcove43 to have a higher recur-rence rate; this association was re-futed later by others.7,9,11,12 Recur-rence of chondroblastoma in the softtissue surrounding the treated lesionis believed to occur because of im-plantation or incomplete curettageand the subsequent growth of the re-sidual tumor cells, especially whenthe affected joint capsule wasopened.25 In sum, then, recurrence ofchondroblastoma depends funda-mentally on incomplete resection andbiologic aggressiveness.9

Lesions that contain enlarged andirregular nuclei or have a prominentmyxoid matrix are more likely to re-cur in CMF.16 The type of manage-ment, however, is the most importantfactor that affects the rate of recur-rence of this tumor. Curettage aloneresults in a very high recurrence ratein many series; Lersundi et al18 re-ported that a recurrence rate of 38%after curettage alone diminished to13% when the cavitary defect was

A, Intraoperative photograph demonstrating anterior access to the proximaltibia, allowing extensive curettage through a cortical window of thechondromyxoid fibroma shown in Figure 6. B, Intraoperative photographdemonstrating the cavity packed with polymethyl methacrylate. (Adapted andprinted with permission from Dr. Olavo Pires de Carvalho, University of SãoPaulo, São Paulo, Brazil.)

Figure 8


April 2013, Vol 21, No 4 231

filled with bone graft. Of the 29 pa-tients with CMF in their study, therewas no recurrence in the 3 who un-derwent curettage plus PMMA orthe 4 who were treated with wide re-section.

Metastasis

Metastases from chondroblastomacan arise from different primarysites. There is no reported relation ofmetastasis to previous surgery ornonsurgical treatment, tumor loca-tion, or patient age.25,44,45 The inci-dence of metastases associated withchondroblastoma is not known butis thought to be very low. Rodgersand Mankin46 described 2 patients of80 (2.5%) with chondroblastomastreated for metastases at their institu-tion. Selection bias can probably ex-plain this elevated rate; most authorsbelieve it to be <1%.45 To date, therehas been no published study on me-tastases from CMF.

The lung is by far the most commonsite of distant metastases. Bones differ-ent from those of the primary site, softtissue, the skin, and the liver are alsocited.11,25,44 The time reported for me-tastases to manifest clinically ranges5 months to 33 years (average, 8years) from the initial diagnosis ofchondroblastoma.25,46

Ostrowski et al47 reported evidenceof p53 mutation in one patient withchondroblastoma and metastases. Incontrast, Hasegawa et al27 found noevidence of p53 mutation in any offive patients with chondroblastomawithout metastases. The p53 muta-tion is a late event in tumorigenesisand is present in many high-gradesarcomas, including osteosarcomasand chondrosarcomas.47

Patients usually survive severalyears with metastatic lesions; theprognosis is better when the metasta-ses are resectable.25,44 There is no re-ported benefit from chemotherapy.25

Summary

Chondroblastoma and CMF are un-common benign bone tumors thatpresent with insidious bone pain.Chondroblastoma usually involvesthe epiphysis or apophysis of longbones; CMF is a metaphyseal tumor.The radiographic appearance ofchondroblastoma is of a lytic lesionwith sclerotic borders. CMF is alsoradiolucent with a sclerotic border,but it is usually larger than CMF andcan have a bubbly appearance. Peri-osteal reaction is uncommon forboth tumors. The chance of recur-rence of chondroblastoma is 8% to13% and, for CMF, 20% to 25%.Surgical management can be chal-lenging because, especially in youngpatients with chondroblastoma, theideal is to avoid the chance of recur-rence while preserving the integrityof the physis. Metastases are veryuncommon and have a good progno-sis when they are resectable. Addi-tional genetic studies can likely helpidentify the cause of metastases andexplain the nature of the aggressivechondroblastoma.

Acknowledgments

The authors would like to thankOlavo Pires de Carvalho, MD, andMarta E. Gutemberg, MD, for al-lowing the use of photographs oftheir cases for this article.

References

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2. Jaffe HL, Lichtenstein L: Benignchondroblastoma of bone: Areinterpretation of the so-calledcalcifying or chondromatous giant celltumor. Am J Pathol 1942;18(6):969-991.

3. Jaffe HL, Lichtenstein L: Chondro-myxoid fibroma of bone: A distinctivebenign tumor likely to be mistaken espe-cially for chondrosarcoma. Arch Pathol(Chic) 1948;45(4):541-551.

4. Schajowicz F, Gallardo H: Epiphysialchondroblastoma of bone: A clinico-pathological study of sixty-nine cases.J Bone Joint Surg Br 1970;52(2):205-226.

5. Dahlin DC, Ivins JC: Benignchondroblastoma: A study of 125 cases.Cancer 1972;30(2):401-413.

6. Unni KK, Inwards CY: Chondromyxoidfibroma, in Unni KK, Inwards CY, eds:Dahlin’s Bone Tumors, ed 6.Philadelphia, PA, Wolters Kluwer,Lippincott Williams & Wilkins, 2010, pp50-59.

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