FOOT & ANKLE INTERNATIONAL

Copyright  2006 by the American Orthopaedic Foot & Ankle Society, Inc.

Differentiating Clinical and Radiographic Features of Enchondroma and

Secondary Chondrosarcoma in the Foot

Donald A. Gajewski, M.D.1 ; Jeffery B. Burnette, M.D.2 ; Mark D. Murphey, M.D.3 ; H. Thomas Temple, M.D.4
1,3 Washington, D.C.; 2 El Paso, TX; 4 Miami, FL

ABSTRACT warranted for cartilage bone tumors of the foot if they exceed
5 cm2 , or if they arise in the midfoot or hindfoot. In these cases,
Background: Enchondroma is the most common benign tumor we recommend either biopsy or close clinical followup.
of the bones of the foot. Chondrosarcoma in this area is rela-
tively rare with malignant transformation from enchondroma Key Words: Chondrosarcoma; Enchondroma; Foot
occurring rarely. In contrast to similar tumors in the appen-
dicular skeleton, it is difficult to distinguish between these two
tumors when they occur in the foot. Methods: We reviewed INTRODUCTION
the medical records and radiographs of all patients with
enchondroma and chondrosarcoma arising from enchondroma Enchondromas are frequent lesions of bone and most often
(secondary chondrosarcoma) from the radiologic archives at occur in the hands and feet. They arise from cartilaginous cell
the Armed Forces Institute of Pathology (AFIP) and identi- nests displaced from the physis during development.4 Often
fied those patients with tumors involving the bones of the foot. they are asymptomatic and usually are found incidentally or
There were 755 patients with enchondroma of which 34 (4.8%) after a pathologic fracture. In contrast, chondrosarcomas are
involved the foot; there were 340 patients with secondary chon- relatively rare tumors of the foot and most arise de novo with
drosarcoma and 14 (4.1%) involved the foot. We compared no apparent benign antecedent lesion.1,3,6,11,13,14 The differ-
clinical and radiographic features of both these lesions. We also
entiation between these tumors radiographically and histo-
compared interobserver differences not only for diagnosis but
also for the presence of scalloping, fracture, cortical destruc- logically has been described.1,3,7,9,10,11 A small percentage
tion, and mineralized matrix. Results: Size and location were of chondrosarcomas in the foot, however, arise from benign
statistically significant variables differentiating the two tumors lesions such as enchondromata.5,6 We hypothesized that it is
(p = 0.03). Enchondromas had a mean size of 2.7 cm2 . Lesions difficult to distinguish enchondroma from chondrosarcoma
that occurred in the hindfoot and midfoot were more likely to based on clinical and radiographic criteria.
be malignant compared to those in the forefoot. In comparing Murphey et al.8 demonstrated clinical and radiographic
interobserver reliability, most disagreement occurred regarding features that distinguish enchondroma from intramedullary
the presence or absence of matrix with the examiners concur- chondrosarcoma in the appendicular skeleton. In their study,
ring only 51% of the time. With regard to diagnosis, the exam- cartilage tumors of the hands and feet were excluded.
iners’ accuracy was 71% and 80%. Their accuracy increased
Although malignant tumors of the feet are rare, when
only to 83% when they agreed. Conclusion: Our findings suggest
that it is difficult to differentiate enchondroma from secondary
they do arise, the treatment often involves amputation or
chondrosarcoma in the foot. Concern for malignant change is partial amputation, and, therefore, it is critical to differen-
tiate between benign and malignant tumors. In our study,
1
Director, Musculoskeletal Oncology, Department of Orthopaedic Surgery and Reha- we present the largest reported series of secondary chon-
bilitation, Walter Reed Arm Medical Center, Washington, D.C.
2
drosarcomas arising from previous enchondromas of the foot.
Orthopaedic Surgery Service, William Beaumont Army Medical Center, El Paso, TX
3
Chief, Musculoskeletal Radiology, Department of Radiologic Pathology, Armed
Our purpose in this study was to identify observable differ-
Forces Institute of Pathology, Washington, D.C. ences between enchondroma and secondary chondrosarcoma
4
Professor, Orthopaedics and Pathology, Department of Orthopaedics and Rehabilita- arising from an enchondroma based on clinical history and
tion, University of Miami School of Medicine, Miami, FL
radiographs.
Corresponding Author:
Donald A. Gajewski, M.D.
Department of Orthopaedic Surgery and Rehabilitation MATERIALS AND METHODS
Walter Reed Army Medical Center
Washington, D.C. 20307
E-mail: Donald.gajewski@na.amedd.army.mil This is a retrospective study based on the review of clinical
For information on prices and availability of reprints, call 410-494-4994 X226 records and radiographs of 1095 patients in the radiologic
240
Foot & Ankle International/Vol. 27, No. 4/April 2006 ENCHONDROMA VS. CHONDROSARCOMA 241

archives of the Armed Forces Institute of Pathology (AFIP) of symptoms, including pain, swelling, or constitutional
in the diagnosis of enchondroma (n = 755) and chon- symptoms. Radiographs were evaluated by an experienced
drosarcoma arising from enchondroma (n = 340). There orthopaedic oncologist and musculoskeletal radiologist with-
were 34 enchondromas and 14 chondrosarcomas arising out any knowledge of the clinical history or final diag-
from enchondroma of the foot. Complete histories were nosis. They were evaluated for lesion size and location
not available for all patients. In those for whom histories (central vs. eccentric), as well as endosteal scalloping, patho-
were available, 12 (75%) patients with enchondroma and logic fracture, cortical remodeling (expansion of the normal
eight (75%) patients with secondary chondrosarcoma had contour), cortical destruction, soft-tissue extension, matrix
a history of pain. A mass was clinically evident in eight formation, and periosteal reaction. Endosteal scalloping and
of the patients with an enchondroma and six patients with matrix formation were defined by a classification described
secondary chondrosarcoma. No patient with enchondroma by Murphey et al.8
or chondrosarcoma complained of constitutional symptoms. Endosteal scalloping was classified by both extent and
Sixty-nine percent of men and 31% of women (average depth. Extent of endosteal scalloping was graded as grade
age 29.7 years) had enchondromas. Eighty-six percent of 1, involving less than one-third of the lesion extent; grade 2,
men and 14% of women (average age 38.6 years) had affecting between one-third and two-thirds of the lesion; and
secondary chondrosarcomas. The demographics, size, and grade 3, involving more than two-thirds of the lesion. Depth
most common locations of the two lesions are shown in of endosteal scalloping also was graded and determined
Table 1. by the most prominent location and classified as grade 1,
Pathologic material was reviewed and characterized by representing less than one-third of cortical thickness; grade
experienced orthopaedic pathologists. We analyzed all lesions 2, between one-third and two-thirds; grade 3, more than two-
located in the bones of the foot and included only those thirds; and grade 4, cortical penetration.
patients with solitary tumors, excluding those with Ollier’s Mineralized matrix was recorded as present or absent,
and Maffucci’s syndrome. subtle or obvious, as well as type (arc and rings, flocculent,
Clinical variables included patient age, gender, tumor loca- or stippled) and extent. Extent of the matrix was graded as
tion, history of local recurrence, and presence or absence grade 1, present in less than one-third of the lesion; grade 2,
present in more than one-third but less than two-thirds of the
Table 1: Demographics, lesion size, and location in lesion, and grade 3, present in more than two-thirds of the
patients with enchondroma compared to chondrosarcoma lesion.
arising from enchondroma Bivariate analyses were conducted to identify differences
between the groups. Specifically, Chi-square tests were run to
compare categorical variables and independent sample t-tests
Chondro-
were run to compare continuous variables. These analyses
Enchondroma sarcoma
were followed by multivariate regression analyses to assure
(n = 34) (n = 14)
accuracy of the bivariate analyses. The Kappa coefficient was
Patient demographics used to measure interobserver reliability.
Gender
Male 69 86 RESULTS
Female 31 14
Age (mean) 29.7 years 38.6 years
The mean size of enchondromas was 2.7 ± 2.7 cm2 with
Lesion size (mean)∗ 2.7 cm2 5.1 cm2 a range from 0.3 to 10.8 cm2 . The mean size of secondary
(SD = 2.7) (SD = 3.7) chondrosarcomas was 5.1 ± 3.7 cm2 , with a range from 1.1
Skeletal distribution to 10.6 cm2 . Three lesions in the malignant group (27.5, 35.8,
Distal phalanx 27% 14% and 112 cm2 ) were dropped from the analyses because they
Middle phalanx 3% 7% were clearly outside the range of most other lesions (more
Proximal phalanx 47% 43% than 3 standard deviations from the mean). Even when these
Metatarsal 15% 7% cases were excluded, size remained statistically significant
Midfoot∗ 0% 7% when comparing the two lesions (p = 0.03).
Hindfoot∗ 9% 21% There were 35 lesions confined to the phalanges. The
Osseous location most common site overall was the proximal phalanx with 16
Central 6 0 enchondromas and 6 chondrosarcomas. Most lesions were
Eccentric 94 100 eccentric. There were seven lesions in the midfoot and 30
lesions in the hindfoot. Lesions in these two locations were
∗p = 0.03 more likely to be malignant when compared to lesions in the
forefoot (p = 0.03).
242 GAJEWSKI ET AL. Foot & Ankle International/Vol. 27, No. 4/April 2006

Table 2: Radiographic features of enchondromas Table 3: Presence, type, character, and extent of matrix
compared to chondrosarcomas arising from in enchondroma compared to chondrosarcoma arising
enchondromas (numbers are percentages) from enchondroma (numbers are percentages) except
where indicated
Chondro-
Enchondroma sarcoma Enchondroma Chondrosarcoma
(n = 25) (n = 9)
Nearest physis
Open 6 0 Character
Closed 94 100 Subtle 76 56
Obvious 24 44
Pathologic fracture 24 15
Type
Periosteal reaction 18 15
Arcs and rings 80 67
Soft-tissue mass 15 39 Flocculent 16 22
Erosion into adjacent 20 20 Stippled 14 11
bone (percentage of
Extent
those with mass)
Grade 1 (<1/3 52 33
Cortical remodeling 82 92 of lesion)
Mild 79 75 Grade 2 24 33
Moderate 21 8 (1/3-2/3 of
Marked 0 17 lesion)
Cortical destruction 27 54 Grade 3 (>2/3 24 33
of lesion)

Radiographic findings are shown in Table 2. Only two

patients were radiographically immature and both had benign The examiners agreed on a diagnosis in 78% of the
lesions. Eight (24%) pathologic fractures were found in patients. Of those in which they agreed, they were accurate
patients with enchondromas compared to two (15%) in 83% of the time. Their accuracy independently was 71% and
patients with secondary chondrosarcomas. A soft-tissue mass 80%. They agreed on the presence or absence of matrix 51%
was seen in five patients (15%) with benign lesions and in of the time, 97% on scalloping, 87% on fracture, 80% on
five (39%) with malignant lesions. Cortical remodeling was cortical remodeling, and 84% on cortical destruction.
seen in most lesions whether benign or malignant. Cortical
destruction also was seen in both but was twice as common in DISCUSSION
secondary chondrosarcomas. With the numbers available, no
significant difference could be detected; however, there was a Enchondromas are relatively common lesions, accounting
trend towards chondrosarcomas presenting with a soft-tissue for about 10% of benign osseous tumors, with about half
mass and cortical destruction. found in the small bones of the hands or feet.4 In this series,
Character and extent of matrix and depth and extent of only 4.8% of enchondromas were located in the foot. This is
scalloping are shown in Tables 3 and 4, respectively. There most likely because this study is based on archival material
were no differences between enchondromas and secondary in which difficult cases were evaluated. Enchondromas are
chondrosarcomas. Matrix was evident on radiography in mostly asymptomatic and frequently are found incidentally
25 (74%) enchondromas and in nine (69%) secondary or after pathologic fracture. Chondrosarcomas, in contrast,
chondrosarcomas. Matrix was subtle in the benign lesions, rarely are found in the foot.2 Most of these are primary
whereas it was more obvious in malignant tumors. The most neoplasms with a small number arising from an antecedent
common matrix pattern in both lesions was an arcs-and- benign lesion.1,3,6,11,13,14 Unii12 reported 774 primary chon-
rings pattern. Enchondromas demonstrated grade 1 extent drosarcomas, of which 14 (1.8%) were located in the foot.
in 52% of cases, grade 2 in 24%, and grade 3 in 24%. There also were an additional 121 secondary chondrosar-
Chondrosarcomas were more evenly distributed, with 33% comas with none located in the foot. Of the 12 chondrosar-
of patients in each of the grades. Endosteal scalloping was comas in the foot reviewed by Cawte et al.1 one was thought
present in most patients (enchondroma n = 32; chondrosar- to have arisen from an enchondroma. Murari et al.6 found
coma n = 12). Although cortical penetration (grade 4) was only four secondary chondrosarcomas from enchondromas
more common with secondary chondrosarcomas, with the in a study of 255 benign and malignant tumors of the foot.
numbers available neither depth nor extent had any statistical Pachter and Alpert11 studied 14 chondrosarcomas of the feet
significance in differentiating the two lesions. of which two arose from enchondromas. Both patients in this
Foot & Ankle International/Vol. 27, No. 4/April 2006 ENCHONDROMA VS. CHONDROSARCOMA 243

diagnosis of chondrosarcoma. These criteria allowed distinc-

Table 4: Presence, depth, and extent of endosteal
tion of enchondroma and chondrosarcoma in over 90%
scalloping in enchondroma compared to chondrosarcoma
of patients. Interestingly, both studies excluded lesions in
arising from enchondroma (numbers are percentages)
the hands and feet, because they thought that malignances
except where indicated
were so exceptionally rare that diagnosis presented no major
problem.
Enchondroma Chondrosarcoma Radiographic features of chondrosarcoma of the foot have
(n = 32) (n = 12) been reported in few studies.1,3,9,10,11 Chondrosarcomas in
the foot are described as primarily lytic lesions that destroy
Depth the cortex. Soft-tissue masses were common. Ogose et al.9
Grade 1 (<1/3 19 8 studied 163 chondrosarcomas of the small bones of the hands
of cortex) and feet. Nineteen of these lesions were secondary, five of
Grade 2 16 8 which were solitary lesions. They found that cortical destruc-
(1/3-2/3 of tion and presence of a soft-tissue mass were essential to
cortex) differentiation between benign and malignant tumors. Cawte
Grade 3 (>2/3 34 33 et al.1 compared 12 chondrosarcomas and 12 enchondromas
of cortex) of the hands and feet. Both lesions expanded the cortex
Grade 4 31 50 and had a calcified matrix, but unique to chondrosarcomas
(cortical were the presence of periosteal reaction, soft-tissue mass, and
penetration) cortical destruction. They noted, however, that three of the
Extent chondrosarcomas lacked any radiologic signs of malignancy
Grade 1 (<1/3 22 25 as mentioned above. Our study does not support these find-
of lesion) ings. Although the incidence of periosteal reaction was equal
Grade 2 37 50 between the benign and malignant lesions, soft-tissue masses
(1/3-2/3 of and cortical destruction were more common in chondrosar-
lesion) comas; however, with the numbers available, no significant
Grade 3 (>2/3 41 25 difference could be detected. Even though a soft-tissue mass
of lesion) and cortical destruction are suspicious for malignancy, they
are not reliable discriminators between these two entities
(Figures 1 and 2). None of these other studies commented
series had multiple enchondromas (Ollier disease). In our on the significance of the location of the tumors in the foot.
material, we found an incidence of secondary chondrosar- We found that tumors occurring in the midfoot and hindfoot
coma in 9 (4.1%) of patients. There was histologic evidence were more likely to be malignant when compared to those
in each case to suggest that these lesions arose from a pre- in the forefoot (p = 0.03). This held true after multivariate
existing lesion. regression analysis.
The differentiation of enchondroma and chondrosarcoma Scalloping, which was a significant parameter in compar-
in the appendicular skeleton using only clinical and radio- ing enchondromas and chondrosarcomas in the appendicular
graphic variables is predictable. Mirra et al.7 reported 51
enchondromas and primary and secondary chondrosarcomas.
Clinically, they found that the patients with secondary chon-
drosarcomas were in general 12 years older than those with
pure benign solitary enchondromas. Further, they reported
that 97% of patients with malignancy had pain in contrast to
44% of patients with benign lesions. Radiographically, they
found the lesions to be easily differentiated. Enchondromas
showed no cortical thickening, bone expansion, or soft-tissue
mass, features that were common in chondrosarcomas. Their
margins were mostly sharp in contrast to the indistinct border
of the malignant lesions. Murphey et al.8 also showed that
multiple clinical and imaging parameters could demonstrate
the difference between enchondromas and chondrosarcomas
in the long bones. They found that pain, deep endosteal
scalloping, cortical destruction, presence of a soft-tissue
mass, periosteal reaction, and marked, heterogenic uptake Fig. 1: Benign enchondroma in a proximal phalanx with cortical destruction
of radionuclide on bone scintigraphy strongly suggested the and soft-tissue mass.
244 GAJEWSKI ET AL. Foot & Ankle International/Vol. 27, No. 4/April 2006

clinical suspicion should be used when evaluating patients

who present with presumed enchondromas of the foot, and if
the lesions are larger than 5 cm2 or occur in the midfoot or
hindfoot, the diagnosis of malignant degeneration should be
considered. We recommend biopsy or close clinical followup.

REFERENCES

1. Cawte, TG; Steiner, GC; Beltran, J; Dorfman, HD: Chondrosarcoma

of the short tubular bones of the hands and feet. Skeletal Radiol.
27:625 – 632, 1998.
2. Chou, LB; Malawer, MM: Analysis of surgical treatment of 33 foot
and ankle tumors. Foot Ankle Int. 15:175 – 181, 1994.
3. Dahlin, DC; Salvador, AH: Chondrosarcomas of bones of the hands
and feet — a study of 30 cases. Cancer 34:755 – 760, 1974.
4. Fechner, RE; Mills, SE: Tumors of the bones and joints. In Atlas of
Tumor Pathology, Fascicle 8, Third series, Armed Forces Institute of
Pathology, Washington, DC, pp 86 – 112, 1993.
Fig. 2: Secondary chondrosarcoma in a proximal phalanx with no obvious 5. Landry, MM; Sarma, DP: In situ chondrosarcoma of the foot arising
soft-tissue mass or cortical destruction. in a solitary enchondroma. J. Foot Surg. 29:324 – 326, 1990.
6. Murari, TM; Callaghan, JJ; Berrey, BH Jr; Sweet, DE: Primary
skeleton,8 was seen equally in both lesions in the foot. All benign and malignant osseous neoplasms of the foot. Foot Ankle
10:68 – 80, 1989.
grades of scalloping were seen in 34% of enchondromas 7. Mirra, JM; Gold, R; Downs, J; Eckardt, JJ: A new histologic
and 33% of secondary chondrosarcomas having grade 3 approach to the differentiation of enchondroma and chondrosarcoma
scalloping (more than two-thirds of the cortex). of the bones: a clinicopathologic analysis of 51 cases. Clin. Orthop.
Our examiners agreed on a diagnosis in 35 cases (78%). 201:214 – 237, 1985.
Of these lesions on which they agreed, seven were malignant 8. Murphey, MD; Flemming, DJ; Boyea, SR; et al.: Enchondroma
versus chondrosarcoma in the appendicular skeleton: differentiating
tumors. Two of these were thought to be benign by our exam- features. Radiographis 18:1213 – 1237, 1998.
iners, emphasizing the fact that differentiation of malignant 9. Ogose, A; Unni, KK; Swee, RG; et al.: Chondrosarcoma of the small
and benign lesions is difficult. bones of the hands and feet. Cancer 80:50 – 59, 1997.
Like all studies reporting tumors from a highly specialized 10. Ostrowski, ML; Spjut, HJ: Lesions of the bones of the hands and feet.
musculoskeletal pathology archive, we acknowledge that this Am. J. Surg. Path. 21:676 – 690, 1997.
11. Pachter, MR; Alpert, M: Chondrosarcoma of the foot skeleton.
study may be biased in terms of having a greater number J. Bone Joint Surg. 46-A:601 – 607, 1964.
of “unusual” or diagnostically difficult cases. Regardless, it 12. Unni, KK: Dahlin’s bone tumors. General aspects and data on 11,087
shows that differentiation between a purely benign enchon- cases, 5th edition, Lippincott-Raven, Philadelphia, pp. 71 – 92, 1996.
droma and a secondary chondrosarcoma arising from an 13. Wiss, DA: Chondrosarcoma of the first metatarsal. J. Surg. Oncol.
23:110 – 112, 1983.
enchondroma is difficult using clinical and radiographic
14. Wu, KK: Tumor review: secondary chondrosarcoma of the foot. J. Foot
parameters. A soft-tissue mass, cortical destruction, and Surg. 28:260 – 265, 1989.
deep endosteal scalloping can be seen in both benign and
malignant cartilage tumors of the foot. We concluded that