Chondroblastoma and Chondromyxoid Fibroma

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Article in The Journal of the American Academy of Orthopaedic Surgeons · April 2013 DOI: 10.5435/JAAOS-21-04-225 · Source: PubMed

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Abstract Camila B. R. De Mattos, MD Chondroblastoma and chondromyxoid ﬁbroma are benign but Chanika Angsanuntsukh, MD locally aggressive bone tumors. Chondroblastoma, a destructive lesion with a thin radiodense border, is usually seen in the Alexandre Arkader, MD epiphysis of long bones. Chondromyxoid ﬁbroma presents as a John P. Dormans, MD bigger, lucent, loculated lesion with a sharp sclerotic margin in the metaphysis of long bones. Although uncommon, these tumors can be challenging to manage. They share similarities in pathology that could be related to their histogenic similarity. Very rarely, From the Department of chondroblastoma may lead to lung metastases; however, the Orthopaedic Surgery, The Children’s Hospital of Philadelphia, mechanism is not well understood. Philadelphia, PA (Dr. De Mattos and Dr. Dormans), the Department of Orthopaedic Surgery, Ramathibodi hondroblastoma is a rare, be- Hospital, Mahidol University, Epidemiology Bangkok, Thailand Cnign bone tumor, usually lo- (Dr. Angsanuntsukh), Children’s cated in the epiphysis or apophysis Hospital Los Angeles, Los Angeles, Chondroblastoma represents 1% to of long bones. It was first described CA (Dr. Arkader), and the University 2% of all primary bone tumors and of Pennsylvania School of Medicine, by Kolodny in 1927 as a cartilage- approximately 5% of benign bone Philadelphia (Dr. Dormans). containing giant cell tumor (GCT) tumors.4-6 The ratio of male to fe- Dr. Arkader or an immediate family but was better characterized by Cod- male patients is approximately member serves as a paid consultant man in 1931, who believed it to be 4,5,7-10 to or is an employee of Biomet 2:1. Although chondroblastoma an “epiphyseal chondromatous giant Trauma. Dr. Dormans or an has been reported in patients ranging cell tumor” involving the proximal immediate family member serves as in age from 2 to 73 years, most pa- a board member, owner, officer, or 1 humerus. In 1942, Jaffe and Lich- tients are aged <20 years.7-9,11,12 committee member of the Pediatric tenstein,2 after a comprehensive re- Orthopaedic Society of North The bone most affected by chon- view, included tumors in locations America, the Scoliosis Research droblastoma is the femur, followed Society, the International Society of other than the proximal humerus by the humerus and tibia.7,9-12 Re- Orthopaedic Surgery and and designated the tumor as benign Traumatology (SICOT) Foundation, ports in the literature fluctuate be- chondroblastoma of bone, that is, as SICOT USA, and the World tween identifying the proximal hu- Orthopaedic Concern. Neither of the a different, separate entity from merus and proximal femur as the following authors or any immediate GCT. Historically, because of Cod- most affected site.7-9,11,12 In the foot, family member has received man’s great contribution, chondro- anything of value from or has stock chondroblastoma is located espe- or stock options held in a blastoma of the proximal humerus cially in the talus and calcaneus, in commercial company or institution was referred as “Codman tumor.” an apophysis or near the articular related directly or indirectly to the 13 subject of this article: Dr. De Mattos Chondromyxoid fibroma (CMF), a surface. Chondroblastoma can also and Dr. Angsanuntsukh. rare mixture of benign cartilage and fi- occur in flat bones, such as the scap- brous and myxoid tissue that generally J Am Acad Orthop Surg 2013;21: ula, patella, sternum, and skull 5 225-233 develops in long bones of the lower ex- bones. The average age of patients tremity, was described in 1948 by Jaffe with chondroblastoma in small or http://dx.doi.org/10.5435/ 3 JAAOS-21-04-225 and Lichtenstein. Prior to their de- flat bones is higher than that of pa- scription, the lesion was thought to tients with chondroblastoma of long Copyright 2013 by the American 6,13 Academy of Orthopaedic Surgeons. be a myxoma of the bone, enchon- bones. About 0.5% to 1% of droma, or chondrosarcoma. chondroblastomas present on verte-

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Figure 1 mors, there is no single characteristic borders. The cells contain one or two abnormality or chromosomal break- round, oval, slightly indented, or ing point specific for chondroblas- even multilobulated nuclei with or toma or CMF. without nucleoli.8 Occasional cells The histogenesis of chondroblas- may have enlarged nuclei without toma and CMF is still uncertain. Ro- nuclear atypia.4,25 The presence of meo et al20 confirmed the active role mitotic figures is scarce.2,4,25 There of cartilage-signaling molecules, both are scattered multinucleated osteo- Indian Hedgehog/parathyroid hor- clast-type giant cells among the mone–related protein (IHh/PTHrP) chondroblasts.7,8 There may be foci and fibroblast growth factor, indicat- of chondroid matrix formed by the ing that chondroblastoma is a neo- chondroblasts.25 Dystrophic calcifi- Photomicrograph of chondroblastoma. Note the diffuse plasm that originates from a mesen- cation is occasionally present and and compact proliferation of chymal cell committed toward may surround individual cells, giving mononuclear cells with indented chondrogenesis via active growth the classic “chicken wire” appear- nuclei with abundant eosinophilic ance7,8 (Figure 1), although this is cytoplasm and distinct cell borders. plate signaling pathways. This con- There is presence of focal clusion supports the chondrogenic not mandatory for diagnosis. pericellular (ie, chicken wire) nature of this tumor and the close re- In 15% to 32% of cases, chondro- calciﬁcation (arrows) on the top left lationship between the physis and blastoma may be associated with corner (hematoxylin-eosin, original 21 magniﬁcation ×400). the tumor. CMF has myofibroblas- secondary aneurysmal bone cyst tic differentiation in its “fibrous” ar- (ABC).7-9,11 Although the reasons for eas driven by transforming growth this association are unclear, hypothe- brae, generally in the posterior ele- factor β-1.22 A strong expression of ses include mechanical stress, ments and/or the body.14 the Sox9 gene, which is responsible trauma, and hemorrhage.13 More ag- A prevalence of <0.5% of all bone tu- for chondrocytic differentiation as gressive chondroblastoma that can mors is reported in many series describ- well as regulation of the expression cause metastases or recurrence shows ing CMF.6,15,16 There is a slight male of cartilage-specific genes in mature no difference in histology compared predominance.17 CMF most com- chondrocytes, especially the synthe- with less aggressive chondroblas- monly arises in young patients in the sis of collagen type II, was found in toma.25 The histology is equivalent second or third decades of life.3,17,18 both chondroblastoma and CMF.23,24 to that of the primary site, and the Most of these tumors are located in This demonstrates that the expres- presence of atypical cells is rare.25 the metaphysis of long bones with sion of Sox9 in these tumors is con- Grossly, CMF appears as lobulated, variable distances from growth plate, sistent with its commitment to the well-circumscribed, and sharply demar- mainly in lower extremities.17 Rarely, early phases of cartilage differentia- cated from the adjacent bone marrow. the lesion involves the epiphysis. The tion, with chondroblastoma being a The lesion is firm and white. The cut diaphysis can be involved, especially more “immature” tumor than CMF surface shows a solid tumor mass that in large tumors. A lesion in small because of the greater presence of is yellow, grayish-white, or blue- bones, such as phalanges, may in- positive Sox9 in CMF cells.23 gray.6 Microscopic analysis of CMF volve the bone in its totality. The reveals three components: myxoma- proximal tibia is the most common tous zones, fibrous zones, and fields site, comprising 28% to 52% of all Pathology that appear chondroid. The classic lower extremity lesions in the litera- histologic features of CMF are lob- ture.17,19 This site is followed by the Grossly, a chondroblastoma is a ules of stellate or spindle-shaped cells ilium, ribs, distal femur, metatarsals, gray-white tumor with yellowish ar- in abundant myxoid background or and distal tibia.17 In contrast to eas, usually because of calcification, chondroid intercellular material. chondroblastoma, CMF is rarely en- which can be soft, rubbery, or fria- Scattered giant cells are found in ap- countered in the humerus.15,17 ble.2 Microscopically, chondroblas- proximately 50% of cases, usually at toma reveals proliferation of mono- the edge of the lobules16,17 (Figure 2). Etiology nuclear cells.10 The tumor is These lobules have a hypocellular characterized by compact areas of center and a condensation of the nu- Although cytogenetic abnormalities round, oval, or polygonal chondro- clei toward the periphery, creating a can be highly specific for some tu- blasts with well-defined cytoplasmic hypercellular periphery. The inter-

226 Journal of the American Academy of Orthopaedic Surgeons Camila B. R. De Mattos, MD, et al

Figure 2 Figure 3

Photomicrograph of chondromyxoid ﬁbroma. Note the stellate cells in a myxoid background. The stellate cells display mild atypia, but mitoses are rarely seen. The lesion AP (A) and AP with internal rotation (B) radiographs of a chondroblastoma of has a lobulated appearance, with the proximal humerus of a 15-year-old girl who presented with right shoulder alternating cellular and mode pain lasting 4 months. The lesion is located entirely in the epiphysis, is myxoid areas (hematoxylin-eosin, lucent, and has thin, sclerotic borders. original magniﬁcation ×200). lobular tissue is composed of oval or be present, if the patient has had spindle-shaped cells. Clinical Presentation symptoms for several years, because Mitotic features are uncommon of expansion of the lesion.28 Patho- Chondroblastoma and CMF are usu- and are usually present in more con- logic fractures in long bones are not ally classified, using the Enneking be- centrated cellular interlobular areas. common at presentation, but when nign bone tumor classification, as Atypical mitotic features are not the lesion is in the foot, subchondral stage 2 (ie, active) or 3 (ie, aggres- found, although some cells are large fracture is frequent and painful.13 sive). The delay between the onset of and have irregularities in the size and CMF may be found incidentally 17 symptoms and diagnosis varies from shape of nuclei. Cellular atypia was but more often presents with pain <1 month to years. Although presen- reported in 18% of cases by Wu that is usually intermittent and not 17 tation of these tumors can be similar, et al; however, there was no change distressing. The duration of symp- chondroblastoma is typically more in the nucleocytoplasmic ratio. Le- toms ranges from several months to painful than CMF. sions in hands and feet are more years. The second most common pre- 17 likely to have atypical cells. Chondroblastoma normally has an sentation is local swelling, a lump, or Myxoid stroma in CMF stains insidious presentation. Symptoms a palpable mass. The patient may uniformly and does not show exten- vary from mild to significant pain present with pain to palpation, and sive liquefaction, as is present in and the presence of a soft-tissue mass the lump may slowly increase in size. myxoid foci of chondrosarcoma. or even a pathologic fracture. Pa- The local swelling or lump is more Nevertheless, small foci of liquefac- tients with chondroblastoma may re- common in tumor of the small tive changes are found in approxi- port pain lasting for several weeks, bones. Limping, limitation of adja- 6 mately 30% of CMF cases. months, or even years. Pain with lo- cent joint range of motion, and Cyst formation, necrosis, foam cal tenderness in the involved bone pathologic fracture are rare.3,6,15,19,29,30 cells, foci of secondary ABC, and and the adjacent joint is the most fre- frank hyaline cartilaginous areas are quent complaint, followed by de- unusual findings.6,17 Calcifications creased range of motion.4,5,8,10-12 Radiologic Evaluation are present in approximately one Some patients attribute the pain to third of the lesions and appear as trauma, usually a minor or sports- The classic radiographic appearance fine lacelike or plaquelike deposits.6 related injury.7 Swelling or joint effu- of chondroblastoma is a well-defined Histochemical analysis of chondro- sion, a limp (when the affected bone eccentric oval or round lytic lesion blastoma and CMF demonstrates is in the lower extremity), and mus- involving the epiphysis adjacent to great positivity to collagen type II cle wasting may also be seen.5,7,10,12 A an open growth plate4 (Figure 3). A and S-100 protein.8,10,24,26,27 palpable mass is uncommon but may sharp sclerotic margin is often seen.

April 2013, Vol 21, No 4 227 Chondroblastoma and Chondromyxoid Fibroma

Figure 4

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 the metaphysis. B, Sagittal T1-weighted magnetic resonance image demonstrating peripheral lobulation and associated marrow edema. C, Intraoperative AP ﬂuoroscopic image demonstrating curettage and bone grafting of the lesion performed through a cortical window, thereby avoiding damage to the unaffected surrounding physis. D, AP radiograph made at 7-month follow-up. The patient was asymptomatic, with no complications or recurrence.

At times, the lesion may be mottled from the epiphyseal lesion.21 A few scan shows increased uptake but sel- or fuzzy or contain areas of calcifica- case reports of diaphyseal chondro- dom is needed for diagnosis.21 tion. Lesion size on radiograph var- blastoma have appeared in the litera- CMF classically presents as a lytic ies, with most being <4 cm.2,5,8,11 Cal- ture.34 Chondroblastomas located on radiolucent medullary lesion with a cifications are found especially in small bones may be more aggressive, thin sclerotic rim (Figure 6). In most skeletally immature patients.7 with loss of cortical continuity and lesions, borders are sharp, with par- It is uncommon to find periosteal bony destruction.35 tial or complete effacement of the bone formation on radiographs, but CT can help define the anatomic cortex.16,17,29,36 CMF tends to be ec- MRI usually depicts edema adjacent limits of the lesion, especially the dis- centrically located in the metaphysis to the periosteum.31 In rare cases, es- tance to the growth plate and the re- of long bones. Rarely, or in advanced pecially in older or neglected pa- lation of the lesion to subchondral cases, the lesion crosses the growth tients, chondroblastoma may have bone. CT shows stipple calcification plate into epiphysis or extends into an atypical presentation that clini- of the cartilaginous matrix, when the diaphysis.6 In small bones, CMF cally and radiographically mimics an present. In addition, CT is useful in generally occupies the entire width of aggressive process.5,28,32 delimiting lesions in unusual loca- the bone, causing thinning of cortices The small percentage of chondro- tions as well as subchondral frac- and fusiform expansion of the bone. blastomas with a secondary ABC in tures not visible on plain radio- The tumor typically has a scalloped the histology usually show differ- graphs.13,14 border that is well defined by a nar- ences from regular chondroblastoma In a few cases in which MRI was row rim of sclerotic bone. Chronic on radiograph, and these sometimes not used in conjunction with radio- bone reaction and cortical thickening lead to confusion in the diagnosis. graphs and the clinical presentation, are commonly present. Unusual peri- Cystic changes are seen more com- this modality led to misdiagnosis or osteal reaction has been re- monly when the lesion is located in overestimation of tumor aggressive- ported.16,17,36 Pseudotrabeculation, bone, such as the patella.13,33 ness.31 Chondroblastoma usually is that is, ridges of the sclerotic rim at Most chondroblastomas involve hypointense on T1-weighted images the edge of the lesion, is present. the epiphysis of long bones.6 A small and variably ranges from hypoin- Gross and microscopic studies show lesion is usually confined to a part of tense to hyperintense on T2- that there is no complete bony sep- the epiphysis or apophysis, although weighted images, with or without tum.16 it may extend through the epiphyseal peripheral lobulation and the associ- Unlike other cartilaginous tumors, plate21 (Figure 4). True metaphyseal ated marrow and soft-tissue edema calcification in CMF is unusual. The chondroblastoma is rare; most re- that enhances after administration of prevalence of calcification in CMF ported cases are of an extension contrast material31 (Figure 5). Bone ranges from 2.4% to 16% of cases

228 Journal of the American Academy of Orthopaedic Surgeons Camila B. R. De Mattos, MD, et al

Figure 5 with staging and preoperative plan- ning30 (Figure7).

Differential Diagnosis

The differential diagnosis for chondroblastoma includes GCT, simple bone cyst, ABC, enchondroma, eosinophilic granuloma, fibrous dysplasia, clear cell chondrosarcoma, subacute osteomyelitis (ie, Brodie ab- scess), and, when a subchondral cyst is present, Legg-Calvé-Perthes disease or osteochondritis dissecans. Tuberculosis can mimic the periartic- The same patient as in Figure 3. Postcontrast T1-weighted fat-suppressed ular pain and bone lesion of chon- coronal (A) and T2-weighted axial (B) magnetic resonance images demonstrating heterogeneously increased signal. The bone marrow is droblastoma and should be consid- normal. ered, especially in developing countries.6,8,21,25 The differential diagnosis for CMF Figure 6 includes benign lesions such as GCT, simple bone cyst, ABC, enchondroma, eosinophilic granuloma, fibrous dysplasia, osteoblastoma, osteofibrous dysplasia, and nonossifying fibro- mas.6,18 Malignant conditions that must be differentiated are low-grade chondrosarcoma and myxoid chondrosarcomas.

Management

The natural history of these tumors is not completely understood; to date, there has been no evidence of potential spontaneous healing.7 Sur- gical management is advised for both types of tumors because no effective medical management is available. AP (A) and lateral (B) radiographs of the proximal tibia of a 22-year-old man Both chondroblastoma and CMF with chondromyxoid fibroma who presented with mild pain of the left knee lasting for 3 months. Note the mild expansion of the tumor and the scalloped generally have a favorable prognosis borders defined by thin sclerotic bone. Biopsy was performed, and the when identified and managed appro- diagnosis was confirmed. (Adapted and printed with permission from priately. Dr. Olavo Pires de Carvalho, University of São Paulo, São Paulo, Brazil.) The benchmark management of chondroblastoma is curettage with radiologically, and from 6.8% to MRI are the preferred imaging mo- bone grafting.8-12,25 The entire tumor 34% of cases histologically.16,36 Cal- dalities. CT demonstrates cortical in- should be excised, with the surgeon cification presents more often in pa- tegrity and calcification of the matrix following meticulous oncologic crite- tients aged >40 years and in flat well. MRI shows low signal on T1- ria of a thorough intralesional exci- bones.36 Pathologic fractures may be weighted images and increased signal sion through a cortical and/or epi- found but are unlikely.17 CT and on T2-weighted images and can help physeal window (Figure 4), avoiding

April 2013, Vol 21, No 4 229 Chondroblastoma and Chondromyxoid Fibroma

Figure 7 droblastoma was recently described in a small series.42 Results were best with small tumors (approximately 1.5 cm) and when location of the tumor provided limited risk of mechanical collapse of the adjacent articular surface. Limited data support this method of management, however, so patients must be selected carefully.42 Some tumors can be widely excised, especially in bones such as ribs and fibula.9,10 Lin et al9 reported no recurrence in all six patients in whom chondroblastoma was treated through en bloc resection. Aggressive recurrences historically treated with amputation can now be managed with limb-sparing techniques and endoprosthetic recon- 5,12 Sagittal T1-weighted fat-suppressed (A) and coronal T2-weighted struction, if feasible. postcontrast (B) magnetic resonance images of a chondromyxoid ﬁbroma of No clear guideline exists for fol- the distal phalanx of the great toe, demonstrating edema in the soft tissue lowing patients with chondroblas- and an expansive and solid lesion surrounded by a thin shell of residual toma. The risk of late recurrence and bone. There is intralesional calciﬁcation with discrete contrast enhancement in the periphery of the lesion, characteristic of cartilaginous lesions. lung metastases, although extremely low, argues for prudent follow-up. Lin et al9 suggested following pa- the growth plate with the help of in- of malignant transformation.5,7,38 tients on a yearly basis for at least 5 traoperative fluoroscopy.9,10 Curet- Management of a lesion in the years.9 Plain chest radiographs made tage through the physis with obliter- femoral head is challenging because preoperatively and at the annual visit ation of part or all of the growth of the difficulty of access—more so if are recommended. plate is an option in patients who are the epiphysis is not fused.10,33 The Because CMF is extremely rare, near the end of skeletal growth.9 traditional approach for this lesion is most published articles are from se- Intra-articular exposure should be through the base of the femoral neck ries with patients who were treated considered to access all of the tumor, or the trochanter, although a direct over several decades; thus, there are no if necessary. hip approach can also be used.7,12 ultimate recommendations for manage- Lehner et al37 noted insufficient ev- Both techniques carry the risk of ment. The options include curettage idence supporting the use of adju- spreading tumor into the femoral and excision, with or without filling of vant therapy. A high-speed burr is neck or the hip joint as well as of the cavitary defect. Wide resection or useful, with caution exercised near damaging the growth plate.10,33 The en bloc excision is probably the best the growth plate and subchondral use of arthroscopy to visually inspect method to avoid recurrence, but not all bone.9,37 Electrocautery, phenol, ar- the cavity following curettage via a locations allow the mechanical imbal- gon bean coagulation, and cryother- minimally invasive approach, similar ance these procedures can cause, so apy also may be used with cau- to core decompression, without com- bone grafting is advised.6,18 CMF can tion.5,9,11,37 Bone graft is the preferred promising the articular cartilage of be locally aggressive; thus, adjuvants material to fill the cavitary defect af- the adjacent joint, has been success- such as PMMA are recommended (Fig- ter curettage.9,10 In a series of 47 pa- ful, although reported in only case ure 8). Curettage alone has resulted in tients, Ramappa et al11 reported no reports.33,39,40 A trapdoor procedure a rate of high recurrence in most recurrence of tumor in 8 patients has also been described, but it can series.16-18,29 Many authors report treated with polymethyl methacry- result in osteonecrosis and perma- that bone grafting after excisional late (PMMA). Radiotherapy is pro- nent damage to the cartilage.12,33,41 curettage reduces the recurrence rate, scribed because of the increased risk Radiofrequency ablation for chon- with some stating that the rate is

230 Journal of the American Academy of Orthopaedic Surgeons Camila B. R. De Mattos, MD, et al

7,9,11,12 Figure 8 complete excision. Some authors have postulated that pelvic chondroblastoma may be more biologically aggressive than other forms of chondroblastoma.5,9,11 Recurrences can occur between 5 months to 7 years after the initial procedure (average, 10 months following diagnosis).7,9,12 Recurrence is not related to one specific mode of management, tumor size, patient sex, or duration of follow-up.8,10,11 de Silva and Reid8 reported a statisti- cally significant relation between duration of symptoms and recurrence. They stated that patients with symptoms of <6 months had a greater chance of recurrence; however, to A, Intraoperative photograph demonstrating anterior access to the proximal our knowledge, this has not been re- tibia, allowing extensive curettage through a cortical window of the ported by other authors. Suneja chondromyxoid ﬁbroma shown in Figure 6. B, Intraoperative photograph 12 demonstrating the cavity packed with polymethyl methacrylate. (Adapted and et al described a positive associa- printed with permission from Dr. Olavo Pires de Carvalho, University of São tion of young age and higher recur- Paulo, São Paulo, Brazil.) rence rate, although this, too, has not been noted by others.7,9 The association of chondroblastoma with similar to that observed after resec- Sarcomatous change has been re- ABC was reported by Huvos and tion.6,18 Use of PMMA as an adju- ported in some series of CMF pa- Marcove43 to have a higher recur- vant after excisional curettage re- tients, but the prevalence is very rence rate; this association was re- portedly decreases the rate of low.6,17 futed later by others.7,9,11,12 Recur- recurrence.18 rence of chondroblastoma in the soft Recurrence tissue surrounding the treated lesion Complications is believed to occur because of im- The recurrence rates of chondroblas- plantation or incomplete curettage Recurrence of the lesion is the most toma vary from 5% to 40%; study and the subsequent growth of the re- common complication following man- results are inconclusive in determin- sidual tumor cells, especially when agement of chondroblastoma and ing which patients have greater the affected joint capsule was CMF. Although growth disturbances chances of recurrence.9,10,12 Most re- opened.25 In sum, then, recurrence of may occur following the resection of cent series report rates of 8% to chondroblastoma depends funda- these lesions because of their proxim- 13%.9,10,12 The recurrence rate for mentally on incomplete resection and ity to the physis, major angular defor- CMF ranges from 20% to 25%.16,18 biologic aggressiveness.9 mities and discrepancies are not Some authors state that recurrence Lesions that contain enlarged and common.9,11,12,33 in chondroblastoma arises more irregular nuclei or have a prominent Functional impairment, degenera- commonly in patients with an open myxoid matrix are more likely to re- tive joint disease, and pathologic epiphyseal plate, but others contra- cur in CMF.16 The type of manage- fractures can also result.11,33 Suneja dict this finding.7-12 Recurrence in ment, however, is the most important et al12 described a series of 40 pa- skeletally immature patients can be factor that affects the rate of recur- tients with chondroblastoma treated explained by inadequate curettage rence of this tumor. Curettage alone with curettage and bone graft with done to avoid damage to the growth results in a very high recurrence rate an average Musculoskeletal Tumor plate.7,10 The proximal femur and in many series; Lersundi et al18 re- Society functional evaluation of pelvis have higher rates of recur- ported that a recurrence rate of 38% 94.2%. The higher-scoring patients rence, likely related to difficulty in after curettage alone diminished to had lesions in more accessible areas. accessing these sites and obtaining 13% when the cavitary defect was

April 2013, Vol 21, No 4 231 Chondroblastoma and Chondromyxoid Fibroma filled with bone graft. Of the 29 pa- those published within the past 5 tients with CMF in their study, there Summary years. was no recurrence in the 3 who un- Chondroblastoma and CMF are un- 1. Codman EA: The Classic: Epiphyseal derwent curettage plus PMMA or common benign bone tumors that chondromatous giant cell tumors of the the 4 who were treated with wide re- upper end of the humerus. Surg Gynecol present with insidious bone pain. Obstet.1931;52:543. Clin Orthop Relat section. Chondroblastoma usually involves Res 2006;450:12-16. the epiphysis or apophysis of long 2. Jaffe HL, Lichtenstein L: Benign bones; CMF is a metaphyseal tumor. chondroblastoma of bone: A Metastasis reinterpretation of the so-called The radiographic appearance of calcifying or chondromatous giant cell tumor. Am J Pathol 1942;18(6):969-991. Metastases from chondroblastoma chondroblastoma is of a lytic lesion 3. Jaffe HL, Lichtenstein L: Chondro- can arise from different primary with sclerotic borders. CMF is also radiolucent with a sclerotic border, myxoid fibroma of bone: A distinctive sites. There is no reported relation of benign tumor likely to be mistaken espe- metastasis to previous surgery or but it is usually larger than CMF and cially for chondrosarcoma. Arch Pathol (Chic) 1948;45(4):541-551. nonsurgical treatment, tumor loca- can have a bubbly appearance. Peri- tion, or patient age.25,44,45 The inci- osteal reaction is uncommon for 4. Schajowicz F, Gallardo H: Epiphysial both tumors. The chance of recur- chondroblastoma of bone: A clinico- dence of metastases associated with pathological study of sixty-nine cases. chondroblastoma is not known but rence of chondroblastoma is 8% to J Bone Joint Surg Br 1970;52(2):205- 226. is thought to be very low. Rodgers 13% and, for CMF, 20% to 25%. and Mankin46 described 2 patients of Surgical management can be chal- 5. Dahlin DC, Ivins JC: Benign lenging because, especially in young chondroblastoma: A study of 125 cases. 80 (2.5%) with chondroblastomas Cancer 1972;30(2):401-413. treated for metastases at their institu- patients with chondroblastoma, the ideal is to avoid the chance of recur- 6. Unni KK, Inwards CY: Chondromyxoid tion. Selection bias can probably ex- fibroma, in Unni KK, Inwards CY, eds: rence while preserving the integrity Dahlin’s Bone Tumors,ed6. plain this elevated rate; most authors Philadelphia, PA, Wolters Kluwer, 45 of the physis. Metastases are very believe it to be <1%. To date, there Lippincott Williams & Wilkins, 2010, pp has been no published study on me- uncommon and have a good progno- 50-59. sis when they are resectable. Addi- tastases from CMF. 7. Springfield DS, Capanna R, Gherlinzoni tional genetic studies can likely help F, Picci P, Campanacci M: Chondro- The lung is by far the most common identify the cause of metastases and blastoma: A review of seventy cases. site of distant metastases. Bones differ- J Bone Joint Surg Am 1985;67(5):748- explain the nature of the aggressive ent from those of the primary site, soft 755. chondroblastoma. tissue, the skin, and the liver are also 8. de Silva MV, Reid R: Chondroblastoma: 11,25,44 Varied histologic appearance, potential cited. The time reported for me- diagnostic pitfalls, and clinicopathologic tastases to manifest clinically ranges Acknowledgments features associated with local recurrence. 5 months to 33 years (average, 8 Ann Diagn Pathol 2003;7(4):205-213. years) from the initial diagnosis of The authors would like to thank 9. Lin PP, Thenappan A, Deavers MT, 25,46 Lewis VO, Yasko AW: Treatment and chondroblastoma. Olavo Pires de Carvalho, MD, and prognosis of chondroblastoma. Clin 47 Ostrowski et al reported evidence Marta E. Gutemberg, MD, for al- Orthop Relat Res 2005;438:103-109. of p53 mutation in one patient with lowing the use of photographs of 10. Sailhan F, Chotel F, Parot R; SOFOP: chondroblastoma and metastases. In their cases for this article. Chondroblastoma of bone in a pediatric 27 population. J Bone Joint Surg Am 2009; contrast, Hasegawa et al found no 91(9):2159-2168. evidence of p53 mutation in any of 11. Ramappa AJ, Lee FY, Tang P, Carlson five patients with chondroblastoma References JR, Gebhardt MC, Mankin HJ: without metastases. The p53 muta- Chondroblastoma of bone. J Bone Joint tion is a late event in tumorigenesis Evidence-based Medicine: Levels of Surg Am 2000;82(8):1140-1145. and is present in many high-grade evidence are described in the table of 12. Suneja R, Grimer RJ, Belthur M, et al: contents. In this article, references Chondroblastoma of bone: Long-term sarcomas, including osteosarcomas results and functional outcome after and chondrosarcomas.47 1-5, 7-12, 14-24, 26, 27, 30, 31, 33, intralesional curettage. J Bone Joint Surg Br 2005;87(7):974-978. Patients usually survive several 35-39, and 42 are level IV studies. years with metastatic lesions; the References 25, 29, 32, 34, 40, 41, 13. Fink BR, Temple HT, Chiricosta FM, and 43-45 are level V expert opin- Mizel MS, Murphey MD: Chondro- prognosis is better when the metasta- blastoma of the foot. Foot Ankle Int ses are resectable.25,44 There is no re- ion. 1997;18(4):236-242. 25 ported benefit from chemotherapy. References printed in bold type are 14. Ilaslan H, Sundaram M, Unni KK:

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