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Chondrosarcomas of the hands and feet: A case series and systematic review of the literature
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Chondrosarcomas of the hands and feet: A case series and systematic review of the literature Laura M. Fayad a,∗ , Shivani Ahlawat a , Muhammed Shayan Khan b , Edward McCarthy c a b c
The Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins University, Baltimore, MD, USA Khyber Medical College, Khyber Teaching Hospital, Peshawar, Pakistan Department of Pathology, Johns Hopkins University, Baltimore, MD, USA
a r t i c l e
i n f o
Article history: Received 7 April 2015 Received in revised form 19 June 2015 Accepted 24 June 2015 Keywords: Chondrosarcoma Hands Feet Radiography MRI Nuclear scintigraphy
a b s t r a c t Objective: To review the imaging features of chondrosarcomas (CS) of the hands and feet, with pathologic correlation. Materials and methods: For 24 histologically-confirmed CS of the hands (n = 14) and feet (n = 10), 23 studies were retrospectively reviewed by two musculoskeletal radiologists in consensus. Radiographs (n = 23), bone scintigrams (n = 2), and magnetic resonance (MR) (n = 7) images were evaluated for lesion location, cortical and medullary involvement, presence of perilesional signal abnormalities and soft tissue masses (STM). Pathologic specimens were reviewed for tumor grade (grade 1–3). Descriptive statistics were reported. Results: CS occurred in adults (age range 32–92) and most were located in the digits (22/23 (95.6%)) rather than tarsal/carpal bones (1/23 (4.4%)). For digital CS, 21/22 (95.45%) involved the epiphysis in addition to the metadiaphysis, 22/23 (95.6%) exhibited >2/3 endosteal scalloping, and 16/23 (69.5%) were expansile (>50% of the bone width). Pathologic fractures (7/23 (30.4%)) and STMs (16/23 (69.6%)) were frequent. By MR, perilesional abnormalities were common (bone marrow edema in 6/7 (85%), soft tissue edema in 5/7 (71.4%), STM in 7/7 (100%)). Following contrast administration (n = 6), there was solid (3/6 (50%)) or lobular (3/6 (50%)) enhancement. Bone scintigrams showed increased uptake on all phases (>anterior superior iliac spine (ASIS)). Pathology specimens revealed 17 grade 2 and 7 grade 3CS, with increased cellularity, necrosis and myxoid features. Conclusion: CS of the hands and feet have common distinctive features, including involvement of the endof-bone, significant endosteal scalloping, expansile appearance, a frequent STM, and intermediate to high grade histologic features. Additional aggressive imaging features are identified by MR (perilesional signal abnormalities) and bone scintigraphy (increased uptake > ASIS) that may be helpful clues to the diagnosis. © 2015 Published by Elsevier Ireland Ltd.
1. Introduction Chondrosarcomas (CS) are rare tumors of cartilage origin that account for about 9% of primary malignancies of bone [1]. While the most common sites of disease for CS include the pelvis, proximal femur and humerus [2], CS have only rarely been reported in the hands and feet, as the vast majority of intramedullary chondroid lesions in the peripheral skeleton are enchondromas [3]. Both primary [4–7] and secondary forms of CS [8–11] have been previously
∗ Corresponding author at: The Russell H. Morgan Department of Radiology and Radiological Science, The Johns Hopkins Medical Institutions, 601 N Wolfe Street, Baltimore, MD 21287, USA. Fax: +1 410 502 6454. E-mail address: [email protected] (L.M. Fayad).
reported, though malignant transformation of an enchondroma in the hands and feet is considered exceedingly rare [9,12]. The imaging features of CS of the hands and feet have been sparsely described with some inconsistent reports. For example, by radiography, Roberts and Price provided general descriptors of CS in the hand as expansile, destructive and occasionally associated with soft tissue masses (STMs) [13], and in the feet, Gajewski et al. described the radiographic features of secondary CS as similar to enchondromas [14]. Furthermore, a description of the crosssectional imaging features of CS of the hands and feet have been limited [15–17]. In addition, histologically, CS of the hands and feet are similar to CS elsewhere in the body [4,18], but nuclear atypia and other histologic features can be shared with enchondromas [19], lending great importance to the imaging appearance of a cartilage lesion in the hands and feet as a predictor of malignancy.
http://dx.doi.org/10.1016/j.ejrad.2015.06.026 0720-048X/© 2015 Published by Elsevier Ireland Ltd.
Please cite this article in press as: L.M. Fayad, et al., Chondrosarcomas of the hands and feet: A case series and systematic review of the literature, Eur J Radiol (2015), http://dx.doi.org/10.1016/j.ejrad.2015.06.026
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Therefore, the hypothesis of this study was that CS of the hands and feet have imaging features similar to enchondromas, but additional aggressive characteristics similar to those reported for CS in the central skeleton may be uncovered by cross-sectional imaging and bone scintigraphy over using radiography alone. The purpose of this study was to comprehensively describe the imaging characteristics of these rare lesions on radiography, as well as MR imaging and bone scintigraphy, their histologic grading, and provide a systematic review of all available reports of CS of the hands and feet in the literature for comparison.
grade was designated by the pathologist, with grade 1 showing cellularity and atypia with minimal myxoid change, grade 2 showing moderate increased cellularity and atypia with readily identifiable mitoses and limited necrosis, and grade 3 showing marked increased cellularity with pleomorphism, bizarre nuclei, numerous mitoses, prominent necrosis and myxoid change [19]. The pathologist had access to the imaging studies during interpretation of the pathology specimens.
2.5. Reader procedures 2. Materials and methods 2.1. Overview This retrospective study was approved by the institutional review board and informed consent was waived. The available imaging studies of CS of the hands and feet were reviewed by two musculoskeletal radiologists in consensus to identify imaging features by radiography, cross-sectional imaging and bone scintigraphy. The histologic features were reviewed by an experienced musculoskeletal pathologist. Descriptive statistics were reported. 2.2. Subject population Subjects were selected consecutively from a pathology database containing patients with histologically-proven resected bone tumors, over a 23 year period between June 1991 and July 2014 at one center. Inclusion criteria were any patient with a CS of the hands or feet who had an available histologic specimen for confirmation of the disease. Exclusion criteria were patients without available histology. One observer reviewed all available medical records on each patient and recorded the patient’s clinical features at presentation and availability of imaging studies. 2.3. Imaging studies Radiography in all subjects consisted of standard views, typically 2 views of the body part in question. Views of the hands and feet included postero-anterior views, and a lateral or additional oblique view. While 17 patients had images performed at only one time point, radiographic follow-up was available for 6 patients (mean 1834 days, range 11–3285 days). MR imaging studies were available in 7 subjects and were performed utilizing 1.5 Tesla systems (Siemens Medical Systems, General Electric Healthcare, Philips). MR imaging sequences consisted of spin echo T1-weighted images (TR/TE 450–650/20–28), fluid-sensitive sequences [(fat-suppressed T2 weighted (TR/TE 3000/4800/60–70) or short tau inversion recovery (STIR, TR/TE/TI 2500/60/160)] and T1-weighted gradient echo images obtained prior to and following the administration of an intravenous gadolinium-based contrast agent (TR/TE 60–60/5–8). Variable coils (quadrature, phased array or 5 inch surface coil), field of view (8–180 mm), section thickness (4–5 mm) and matrix (256 × 192, 256 × 256 or 512 × 256) were utilized. Technetium 99 whole-body bone scintigrams were available in two subjects, both performed as standard three-phase bone scans. 2.4. Pathologic interpretation All histologic specimens were reviewed by a pathologist specialized in the analysis of bone tumors, with experience of 40 years. Specimens were reviewed for the presence and degree of the following features: cellularity, atypia, necrosis, bone destruction and soft tissue invasion, polarity, mitoses and myxoid nodules. A tumor
Two experienced musculoskeletal radiologists (one with 14 years and one with 4 years post-residency experience) reviewed all imaging studies in consensus; radiographs, MR imaging sequences and bone scans (when available) were reviewed in the same session. The observers recorded the following features by radiography: involved bone of hand or foot, site of involvement in bone (epiphyses, metaphysis, diaphysis), percentage of bone width involvement (0–25%, 25–50%, 50–75%, >75%), percentage of bone length involvement (0–25%, 25–50%, 50–75%, >75%), percentage of endosteal scalloping (none, <2/3, at least 2/3), presence of expansion beyond the normal width of the bone (0–25%, 25–50%, 50–75%, >75% the width of the bone), matrix (lytic, sclerotic, mixed), mineralization percentage (none, 1–25%, 26–50%, 51–75%, >75% of lesion mineralization), as well as the presence or absence of a fracture or STM. For MR imaging, observers recorded the T1 and T2-weighted signal (hypointense, isointense, hyperintense to muscle), the T1and T2-weighted signal heterogeneity (homogeneous, <50% heterogeneity, >50% heterogeneity), the post-contrast pattern of enhancement (lobular, solid), the post-contrast T1-weighted signal heterogeneity (homogeneous, <50% heterogeneity, >50% heterogeneity), and the presence or absence of endosteal scalloping, perilesional bone marrow edema, periosteal reaction, perilesional soft tissue edema, perilesional contrast enhancement and a STM. For nuclear scintigraphy studies, the observers recorded whether there was uptake on each of the phases (perfusion, blood pool, delayed) and the degree of uptake on the delayed phase (similar or less than that of the anterior superior iliac spine (ASIS), or greater than that of the ASIS), similar to a prior report which showed 82% of CS of the appendicular skeleton having uptake greater than the ASIS, compared with only 21% of enchondromas [20].
2.6. Systematic review of the literature A search was made of articles in the peer-reviewed literature in PubMed that had been published since 1974, to identify reports of CS of the hands or feet. The search strategy included English language articles with the following search terms and keywords: chondrosarcoma, hand, digit, foot, as well as all the individual bones of the hands and feet. Articles on chondromas, chondromatosis, osteochondromas, and enchondromas as well as case reports in other languages were excluded.
2.7. Analysis The clinical and demographic features of the patients were tabulated and summarized. Descriptive statistics regarding the imaging features of CS by radiography, MR imaging and bone scintigraphy were reported, with categorization of the features by grade. A summary of the findings from a systematic review of the literature were also tabulated and features of CS uncovered in our study were compared with those that had been identified by review of the literature.
Please cite this article in press as: L.M. Fayad, et al., Chondrosarcomas of the hands and feet: A case series and systematic review of the literature, Eur J Radiol (2015), http://dx.doi.org/10.1016/j.ejrad.2015.06.026
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3. Results Twenty-four cases of CS of the hands or feet were identified, for which radiography was available in 23, MR imaging in 7 and threephase bone scintigrams in 2 subjects. Overall, there were more females than males (14 vs. 10, respectively), with the patients having a mean age of 58 years, and age range of 32–92 years, although a greater percentage of subjects who were above the age of 50 (16/24) were female (67% female (10/16) vs. 33% (6/16) males). Regarding the location CS, 14 were in the hands and 10 were in the feet, with the most common location being the phalanges in both the hands and feet (45.8% (11/24) in the distal phalanx, 33% (8/24) in the proximal phalanx, 13% (3/24) in the middle phalanx), and only 4% (1/24) in the first metatarsal and 4% (1/24) in a tarsal bone (calcaneus). Table 1 is a summary of the clinical and imaging features of the CS in this study. There were more grade 2 (n = 17) than grade3 (n = 7) lesions in the study population, and the most common presenting clinical feature in these cases was a palpable mass or deformity (73%), rather than pain (27%). By radiography, most CS were expansile lesions (expanding the width of bone at least 50%), involved the end of bone, exhibited endosteal scalloping greater than 2/3 the width of the cortex and 69.5% (16/23) had an associated STM. The lesions were either lytic or had a mixed lytic-sclerotic appearance, and the majority had a mineralized matrix visible by radiography. Patients presented with pathologic fractures in 34.8% (8/23). For subjects who had serial follow-up, an increase in the length of bone involvement, degree of endosteal scalloping, degree of expansion and a change in matrix and mineralization appearance occurred over years, presumably suggesting secondary transformation of an enchondroma. Table 2 is a summary of imaging features according to tumor grade; there were no substantial differences in the imaging appearance of grade 2 and 3 lesions. Figs. 1 and 2 are two examples from our case series. By MR imaging, most CS were homogeneously isointense or hyperintense to skeletal muscle on T1 weighted images, and fairly homogeneously hyperintense on fluidsensitive sequences. The majority presented with perilesional bone marrow edema, periosteal reaction, soft tissue edema and a STM. Following contrast administration, the pattern of enhancement varied between lobular and solid enhancement and there was associated perilesional contrast enhancement in the majority of cases. In 1/7 MR studies, a pathologic fracture was present, potentially contributing to perilesional signal abnormalities. By bone scintigraphy, there was notable uptake on all three phases, greater than that identified in the ASIS. Finally, a systematic review of the literature on the imaging of CS of the hands and feet, identified 136 reports of CS of the hands and feet, with a total of 105 case reports (66 in the hand, 39 in the feet), 10 reports in other languages and 21 case series [6,7,10,13–17,21–33]. Table 3 summarizes the case series results, which included a total of 414 reports of CS of the hands and feet. Overall, the clinical presentation of CS of the hands and feet included pain as a common symptom (43%), but many CS also presented with painless growth. There were more CS reported in the hands than feet (60% vs. 40%, respectively), average age was over 50 years, and there were slightly more males with CS than females (53% vs. 47%, respectively). CS commonly occurred in the phalanges (35.8%) or metacarpals (18.3%) of the hand and the calcaneus was a common site in the foot (24.5%). Aggressive features were generally present by radiography, including expansile lesions with extra-cortical extension and STMs. Available reports of MR imaging (n = 4/21 case series) were incomplete, and relayed limited features, including hyperintensity on T2-weighted imaging and a STM [15]. Regarding the treatment of CS, amputation was considered the treatment of choice. Recurrence was reported in 24 cases, typically in cases where there was a local resection or curettage performed and there were 28 reported cases of metastatic disease with
3
Table 1 Summary of clinical, histological and imaging characteristics of study population. Clinical historya
Pain 3/11 (27.2%) Mass/deformity 8/11 (72.8%)
Histologic grade
Grade 2 17/24 (70.8%) Grade 3 7/24 (29.2%)
Bone involved
Phalanx 22/24 (91.7%) Metatarsal 1/24 (4.2%) Flat bone 1/24 (4.2%)
LOCATION Digit • Epiphysis • Metaphysis • Diaphysis Tarsal bone (Calcaneus)
22/23 (95.7%) 21/22 (95.5%) 21/22 (95.5%) 18/22 (90%) 0/22 (90.9%)b 1/23 (4.3%)
Radiographic features (n = 23) Bone width involvement Bone length involvement
Endosteal scalloping presence fraction scalloped
Expansile
Matrix
Matrix–mineralization Presence Percentage
Pathologic fracture STM MRI features (n = 7) T1-weightedc Signal Heterogeneity T2-weighted Signal hyperintensity Heterogeneity
Perilesional bone marrow edema Periosteal reaction Extra-osseous soft tissue edema Endosteal scalloping Contrast–enhanced T1-weightedd Enhancement pattern
76–100% 21/23 (91.3%) 51–75% 1/23 (4.3%) 25–50% 1/23 (4.3%) 76–100% 14/23 (60.8%) 16/23 (69.5%)b 51–75% 5/23 (21.7%) 25–50% 4/23 (17.3%) 22/23 (95.65%) No scalloping 1/22 (4.5%) <2/3 0/22 (0%) >2/3 21/22 (95.4%) 22/22 (100%)b 76–100% 10/23 (43.5%) 13/23 (56.5%)b 51–75% 6/23 (26.1%) 25–50% 4/23 (17.3%) <25% 3/23 (13.0%) Lytic 11/23 (47.8%) Sclerotic 0/23 (9%) Mixed 12/23 (52.2%) 13/23 (56.5%)b 21/23 (91.3%) 75–100% 0/21 (0.0%) 50–75% 3/21 (14.2%) 25–50% 5/21 (23.8%) 6/21 (28.6%)b <25% 13/21 (61.9%) 7/23 (30.4%) 16/23 (69.5%)
Isointense 3/6 (50%) Hyperintense 3/6 (50%) ≤50% heterogeneity 3/6(50%) >50% heterogeneity 3/6(50%) 7/7 (100%) Homogeneous 1/7 (14.2%) ≤50% heterogeneous 5/7 (71.4%) >50% heterogeneous 1/7 (14.2%) 6/7 (85.7%) 3/7 (42.8%) 5/7 (71.4%) 7/7 (100%)
Perilesional enhancement
Lobular 3/6 (50%) Solid 3/6 (50%) Homogeneous 5/6 (83.3%) <50% heterogeneous 1/6 (16.6%) 5/6 (83.3%)
Bone scan (n = 2) Perfusion Blood pool Delayed phase
Positive uptake 2/2 (100%) Positive uptake 2/2 (100%) Uptake >ASIS 2/2 (100%)
Heterogeneity
a b c d
Clinical history was available in 11 cases. Findings after follow-up. In one case, T1 weighted images were not available for viewing. Contrast-enhanced sequences were available in 6/7 MRI scans
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4 Table 2 Imaging features of CS compared by histologic grade.
Grade 2 (n = 17)
Grade 3 (n = 7)
Radiography Bone width: Involves >75% width of bone Bone length: Involves >75% length of bone Endosteal scalloping Expansile: >75% width of bone Matrix–mineralization Fracture ST mass MRI (n = 7) T1 signala T1 heterogeneity T2 Hyperintense T2 heterogeneity Periosteal/bone marrow edema Soft tissue edema Contrast enhancementa Perilesional enhancement ST mass:
15/17 (88.2%) 10/17 (58.9%) 15/17 (88.2%) 7/17 (41.2%) 11/17 (64.7%) 5/17 (29.4%) 9/17 (59.2%) (n = 6/7) Isointense 2/5(40%) Hyperintense 3/5(60%) ≤50% hetero 3/5 (60%) >50% hetero 2/5 (40%) 6/6 (100%) Homogeneous 1/6(16.7%) ≤50% hetero 4/6(66.6%) >50% hetero 1/6(16.7%) 5/6 (83.3%) 4/6 (66.3%) 2/5 (40%) lobular 3/5(60%) solid 4/5 (80%) 6/6 (100%)
6/7 (85.7%) 4/7 (57.5%) 6/7 (85.7%) 3/7 (42.8%) 5/7 (71.4%) 2/7 (28.5%) 5/7 (71.4%) (n = 1/7) Isointense 1/1(100%) >50% hetero 1/1 (100%) 1/1 (100%) ≤50% hetero 1/1(100%) 1/1 (100%) 1/1 (100%) 1/1 (100%) lobular 1/1 (100%) 1/1 (100%)
BONE SCANb Positive uptake all 3 phases Positive uptake >ASIS
1/1 (100%) 1/1 (100%)
1/1 (100%) 1/1 (100%)
a b
n = 6 MR imaging studies with T1 imaging and contrast, 5 grade 2 and 1 grade 3. n = 2 bone scintigrams performed, 1 grade 2 and 1 grade 3.
Table 3 Systematic review of the literature: clinical, pathologic and imaging characteristics of patients with CS of the hands and feet. Reference
Mean age/sex
Clinical history
Site
Histologic features
Imaging features Radiographic
MRI
[27] (n = 6) [21] (n = 24)
Not provided Not provided
4/6 sec Mafucci or Ollier disease
6/6 Hands 13/24 Hands 11/24 Feet
None - cortical destruction - soft tissue extension
None None
[22] (n = 2)
52 years 2/2 F
2/2 pain
2/2Hands
Not provided 14 Gr 1 4 Gr 2 1 Gr 3 5 Unknown Gr 1–1 Gr 3 – 1
- lytic - cortical destruction - soft tissue extension
None
[14] (n = 8)
39 years 2/8 F
8/8 sec
8/8 Feet
None
- STM - cortical destruction
None
[15] (n = 2)
52 years 0/2 F
2/2 sec 0/2 pain 2/2 painless
2/2Hand
2 Gr 1
-
- Hypointense T1 - STM
[6] (n=12)
53 years 2/11 F
3/12 sec 8/12 pain 4/12 painless
12/12 Feet
2 Gr 1 9 Gr 2 1 Gr 3
- endosteal erosion None - cortical destruction–indistinct margins - matrix calcification–expansion - STM - joint extension - fracture
[6] (n = 23)
71 years 13/23 F
10/23 pain; 11/23 painless; 2/23 fracture
23/23 Hand
1 Gr 1 20 Gr 2 2 Gr 3
-
matrix calcification endosteal erosion cortical destruction expansion STM indistinct margins joint extension fracture
None
[24] (n = 35)
67 years 21/35 F
5/35 sec
27/35Hands 8/35 Feet
8 Gr 1 26 Gr 2 1 Gr 3
-
lytic lobulated mineralized ill-defined margins cortical destruction soft-tissue extension
None
lytic chondroid matrix cortical destruction STM
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Table 3 (Continued) Reference
Mean age/sex
Clinical history
Site
Histologic features
Imaging features Radiographic
MRI
[16] (n = 3)
50 years 0/3 F
3/3 painful mass
3/3 Feet
2 Gr 1–2
-
expansile lytic cortical thinning chondroid matrix
- lobular - high T2 signal intensity - septal contrast enhancement
[25] (n = 12)
55 years 7/12 F
12 sec 7/12 pain; 3/12 painless; 1/12 unknown; 1/12 injury
10/12Hands 2/12 Feet
5 Gr 1 6 Gr 2 1 Gr 3
-
expansion periosteal reaction cortical destruction STM mineralized
1/12 had MRI – no features not reported
[23] (n = 161)
53 years 77/161 F
19/161 sec 50/161 pain; 29/161 painless; Others not provided
88/161 Hands 75/161 Feet
116 Gr 1 44 Gr 2 3 Gr 3
-
endosteal erosion cortical destruction expansion STM chondroid mineralization
None
[26] (n = 13)
53 years 3/13 F
2/13 sec 3/8 pain 5/13 no history available
Hands and Feeta
9 Gr 1 2 Gr 2 2 Gr 3
- lytic - punctate calcification - irregular cortical destruction - soft tissue extension
None
[17] (n = 3)
59 years 2/3 F
3/3 painless mass
3/3Feet
2 Gr 1 2 Gr 2 Periosteal CS
- mineralized - cortically based
- marginal and septal enhancement
[28] (n = 14)
48 years 4/14 F
Not provided
4 Hands 10 Feet
Not provided
Not provided
[10] (n = 2)
59 years 2/2 F
2/2 sec
2/2 Hand
10 Gr 1 3 Gr 2 1 Gr 3 1/2 Gr 1 1/2 Gr 2
- lytic - mineralized - STM
None
[29] (n = 2)
57 years 1/2 F
Pain/mass-no details provided
2/2 Hand
2/2 Gr 2
- expansile - mineralized - fracture
None
[30] (n = 22)
43 9/22 F
6/22 sec
22/22 Feet
Gr not provided
Not provided
None
None
- Pain/swelling no details provided [33] (n = 18)
68 years 10/18 F
4/18 sec 13/18 pain
18/18Hand
Gr not provided
-
[31] (n = 3)
38 years 2/3 F
3/3 pain
3/3 Feet
Gr not provided
- marked cortical expansion - trabecular destruction
None
[13] (n = 19)
63 years 14/19 F
10/19 pain
19/19Hand
Gr not provided
-
large indistinct margins bone destruction soft-tissue invasion
None
[32] (n = 30)
50 years 13/30 F
4/30 sec 18/30 pain
16/30 Feet
12/30 Gr 1 15/30 Gr 2 3/30 Gr 3
-
expansile lytic mineralized cortical destruction soft tissue extension fracture
None
large indiscrete margins mineralized lytic soft tissue invasion fracture
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6 Table 3 (Continued) Reference
Mean age/sex
Clinical history
Site
Histologic features
Total n = 414a
54.2 years 48% F (184/384) 52% M (200/384)
59 sec 43.1% pain (127/295) 56.9% painless or unknown history (168/295)
60% Hand (229/383) 35.8% PP (82/229) 18.3% MC (42/229) 40% Feet (151/383) 24.5% Calcaneus (37/151)
55% Gr 1 (181/332) 40% Gr 2 (133/332 5% Gr 3 (18/332)
Imaging features Radiographic
MRI
Common features:
Common features (n = 4 with MRI)
- Expansile, - Lytic - Chondroid mineralization - Cortical destruction - STM
- Lobulated septal enhancement - STM
Gr = grade, PP = proximal phalanx, DP = distal phalanx, MC = metacarpal, MT = metatarsal, CS = chondrosarcoma, sec = secondary, M = male, F = female. a Information not available.
Fig. 1. 53 year old woman presented with pain and swelling of the long finger following trauma. (a–c) Anteroposterior, oblique and lateral radiographs of the right hand shows an expansile, lytic lesion (arrow) in the distal phalanx of the long finger involving the majority of the length and width of the affected bone. There is associated endosteal scalloping and minimal internal chondroid matrix (the latter best visualized on the lateral image). (d and e) Sagittal T1-weighted (TR/TE 650/22) and fluid sensitive (STIR, TR/TE/TI 2500/60/160) imaging shows a marrow-replacing lesion in the distal phalanx with slightly hyperintense T1 signal and diffusely hyperintense fluid signal (arrow). (f) Post-contrast, coronal fat suppressed T1-weighted image (TR/TE 650/22) through the hand shows heterogeneous “solid pattern” of internal enhancement (arrow). The patient underwent amputation of the distal phalanx and a portion of the middle phalanx (g). Photomicrograph obtained from the resected specimen shows a grade 2 chondrosarcoma with moderate atypia and binucleated chondrocytes.
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Fig. 2. 53 year old woman who presented with a palpable mass and deformity of the ring finger. (a and b) Anteroposterior and lateral views of the ring finger showing a markedly expansile lesion involving the entire length of the distal phalanx, with mineralization and soft tissue extension. (c) Photomicrograph shows a grade 3 chondrosarcoma with increased cellularity, atypia, and mitotic figures.
CS of the hands and feet. In our subject population, treatment of CS consisted of ray amputation (23/24); in the case of one patient with a calcaneal CS, excisional biopsy was performed and no records were otherwise available.
4. Discussion CS is a rare tumor in the hands and feet. Our study extends the work of prior reports with a comprehensive assessment of the multimodality imaging features, the histologic grade and clinical presentation of this disease. In our series, there were more women than men affected by this disease, and a predilection towards the female gender has also been noted in other investigations [13], although by systematic review of the literature, slightly more males (52%) were affected by this disorder. Regarding the age of presentation, two-thirds of the patients in our series were above the age of 50, in keeping with previously described reports of CS in the hands and feet. Regarding the location of CS in this series, the vast majority of cases in our series occurred in the phalanges, rather than the more proximal tubular bones or tarsal or carpal bones of the hands and feet, a distribution similar to several prior reports of CS in the hands and feet [6,7,21,23,25,32,33]. The propensity for benign enchondromas to occur in the phalanges as well as CS is in keeping with a common pathogenesis to these lesions; furthermore, a susceptibility to trauma in the more distal hands and feet, which may induce a pathologic fracture, may bring these lesions to presentation more easily than the tarsal or carpal bones. Of note, CS has also been reported to have a predilection for the calcaneus in the hindfoot [14,23], and there was one case located in the calcaneus in our series as well. Unlike CS elsewhere in the appendicular skeleton, a history of pain in our series of CS in the hands and feet was generally uncommon, but present in patients with pathologic fractures, as expected. Other reports have described a mixture of presenting clinical features, including pain as well as a painless presentation [6,7,23]; thus, pain is not a necessary feature of CS of the hands and feet, even though it has been described as a differentiator of enchondromas and CS elsewhere in the body [2].
Regarding the extent of osseous involvement, CS of the hands and feet involved the majority of the length of the bone (including the end of bone), and were not confined to the metadiaphysis as in other extremity CS [34]. Most cases presented with aggressive features similar to those reported CS features in the extremities outside the hands and feet, including significant endosteal scalloping greater than 2/3 of the cortex [2], and approximately 70% of our cases were associated with a STM. Overall, these findings suggest that CS of the hands and feet may have a later presentation, allowing for a greater extent of involvement of the bone and slightly greater involvement of the adjacent soft tissues, than CS in other parts of the appendicular skeleton. A series of 104 intramedullary CS similarly reported an associated STM in 79.8% (83/104) of cases, as seen by radiography [23], although cross-sectional imaging is more sensitive to the detection of a STM in patients with CS [35]. By cross-sectional imaging and bone scintigraphy, additional aggressive characteristics of CS were observed. By MR, as expected, CS of the hands and feet were hyperintense on fluid-sensitive sequences, and were of intermediate or high signal intensity on T1weighted imaging, as has been previously reported for CS outside the hands and feet [36]. The high proportion of periosteal reaction, perilesional bone and soft tissue edema, perilesional contrast enhancement and a STM may in part be explained by the presence of pathologic fractures, although even in cases without a fracture, these aggressive features were present. Finally, by scintigraphy, CS in this series showed significant uptake, greater than that in the ASIS, a feature common to CS found elsewhere in the appendicular skeleton [20]. Enchondromas are the most common benign bone lesions of the hands and feet [37], and the problem of distinguishing CS from the more frequent enchondromas of the hands and feet is challenging, as it is elsewhere in the body. Imaging is not only important for suggesting the presence of an aggressive cartilage lesion in the hands and feet, but also guides the histologic assessment of these lesions. In the small tubular bones of the hands and feet, the histologic threshold for the diagnosis of CS is much higher than that for CS elsewhere in the body. What might be regarded as a grade 1CS in the femur would not be considered CS histologically in the bones of the hands and feet. In particular, increased cellularity, atypical
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chondrocytes, bi-nucleated chondrocytes and even myxoid change are aggressive histologic features that can be associated with low grade cartilage lesions (including enchondromas) in the hands and feet, but are diagnostic of higher grade cartilage lesions outside the hands and feet [19,38]. Hence, aggressive imaging features suggesting a CS in the hands and feet in the setting of aggressive histologic features can appropriately guide the histologic diagnosis toward CS rather than enchondroma. In one of the cases in our study, features of an enchondroma with atypia were identified by initial histology (from a biopsy specimen), but subsequently evolved to a presumed secondary CS five and a half years later (after resection). Initially, the lesion showed typical radiographic features of an enchondroma, with subsequent development of aggressive imaging characteristics. In fact, in all cases of our study group, the imaging features of CS of the hands and feet were aggressive at the time of surgical resection, although it is unclear whether these presumed secondary CS in our series evolved from an enchondroma or were slow-growing non-aggressive appearing CS initially. Traditionally, low grade CS in the proximal appendicular skeleton (such as the humerus or femur) have been reported to resemble enchondromas by imaging [20,39,40], but in our series in the hands and feet, no grade 1CS was included and intermediate grade CS (grade 2) had features similar to higher grade lesions (significant expansion, perilesional edema and enhancement, and the presence of a STM), and were unlike traditionally-described enchondromas (which lack significant expansion, perilesional edema and a STM). Nevertheless, it is likely that some imaging features of CS reported here, such as deep endosteal scalloping and osseous expansion, overlap with features of some enchondromas. Finally, regarding the prognosis and treatment of CS of the hands and feet, some studies have termed CS of the digits as “non-metastising chondrosarcoma” because these lesions rarely metastasize as CS of the pelvis or the more proximal skeleton [41], although by systemic review of the literature, there were 28 reported cases of metastatic disease from CS of the hands and feet. Nevertheless, because CS of the hands and feet are rare, their natural history is not well understood. Morbidity in the form of recurrence may occur after surgical resection, especially if only a curettage is performed. Therefore, radical excision and amputation are generally recommended even in lower grade CS [2]. Our study has limitations, including a small sample. In our study, only 24 cases of CS of the digits were found over a long period (1983–2012), confirming the rarity of this disease; thus, the number of cases is not sufficient for statistical analysis. Also, as this was a retrospective study, the imaging protocols were not uniform, and follow-up of the patients was not available in many cases. Similarly, in performing a systematic review of the literature, imaging, histologic and clinical features were not reported uniformly, making comparisons between case reports and case series difficult. In conclusion, CS of the hands and feet have aggressive features that include significant endosteal scalloping, an expansile appearance with involvement of the end of the bone as well as the metadiaphysis. Additional aggressive imaging features are identified by MR (perilesional signal abnormalities and STMs) and bone scintigraphy (increased uptake > ASIS) that may be helpful clues to the diagnosis and can also guide the histologic assessment of these lesions.
[2]
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Contents lists available at ScienceDirect
European Journal of Radiology journal homepage: www.elsevier.com/locate/ejrad
Chondrosarcomas of the hands and feet: A case series and systematic review of the literature Laura M. Fayad a,∗ , Shivani Ahlawat a , Muhammed Shayan Khan b , Edward McCarthy c a b c
The Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins University, Baltimore, MD, USA Khyber Medical College, Khyber Teaching Hospital, Peshawar, Pakistan Department of Pathology, Johns Hopkins University, Baltimore, MD, USA
a r t i c l e
i n f o
Article history: Received 7 April 2015 Received in revised form 19 June 2015 Accepted 24 June 2015 Keywords: Chondrosarcoma Hands Feet Radiography MRI Nuclear scintigraphy
a b s t r a c t Objective: To review the imaging features of chondrosarcomas (CS) of the hands and feet, with pathologic correlation. Materials and methods: For 24 histologically-confirmed CS of the hands (n = 14) and feet (n = 10), 23 studies were retrospectively reviewed by two musculoskeletal radiologists in consensus. Radiographs (n = 23), bone scintigrams (n = 2), and magnetic resonance (MR) (n = 7) images were evaluated for lesion location, cortical and medullary involvement, presence of perilesional signal abnormalities and soft tissue masses (STM). Pathologic specimens were reviewed for tumor grade (grade 1–3). Descriptive statistics were reported. Results: CS occurred in adults (age range 32–92) and most were located in the digits (22/23 (95.6%)) rather than tarsal/carpal bones (1/23 (4.4%)). For digital CS, 21/22 (95.45%) involved the epiphysis in addition to the metadiaphysis, 22/23 (95.6%) exhibited >2/3 endosteal scalloping, and 16/23 (69.5%) were expansile (>50% of the bone width). Pathologic fractures (7/23 (30.4%)) and STMs (16/23 (69.6%)) were frequent. By MR, perilesional abnormalities were common (bone marrow edema in 6/7 (85%), soft tissue edema in 5/7 (71.4%), STM in 7/7 (100%)). Following contrast administration (n = 6), there was solid (3/6 (50%)) or lobular (3/6 (50%)) enhancement. Bone scintigrams showed increased uptake on all phases (>anterior superior iliac spine (ASIS)). Pathology specimens revealed 17 grade 2 and 7 grade 3CS, with increased cellularity, necrosis and myxoid features. Conclusion: CS of the hands and feet have common distinctive features, including involvement of the endof-bone, significant endosteal scalloping, expansile appearance, a frequent STM, and intermediate to high grade histologic features. Additional aggressive imaging features are identified by MR (perilesional signal abnormalities) and bone scintigraphy (increased uptake > ASIS) that may be helpful clues to the diagnosis. © 2015 Published by Elsevier Ireland Ltd.
1. Introduction Chondrosarcomas (CS) are rare tumors of cartilage origin that account for about 9% of primary malignancies of bone [1]. While the most common sites of disease for CS include the pelvis, proximal femur and humerus [2], CS have only rarely been reported in the hands and feet, as the vast majority of intramedullary chondroid lesions in the peripheral skeleton are enchondromas [3]. Both primary [4–7] and secondary forms of CS [8–11] have been previously
∗ Corresponding author at: The Russell H. Morgan Department of Radiology and Radiological Science, The Johns Hopkins Medical Institutions, 601 N Wolfe Street, Baltimore, MD 21287, USA. Fax: +1 410 502 6454. E-mail address: [email protected] (L.M. Fayad).
reported, though malignant transformation of an enchondroma in the hands and feet is considered exceedingly rare [9,12]. The imaging features of CS of the hands and feet have been sparsely described with some inconsistent reports. For example, by radiography, Roberts and Price provided general descriptors of CS in the hand as expansile, destructive and occasionally associated with soft tissue masses (STMs) [13], and in the feet, Gajewski et al. described the radiographic features of secondary CS as similar to enchondromas [14]. Furthermore, a description of the crosssectional imaging features of CS of the hands and feet have been limited [15–17]. In addition, histologically, CS of the hands and feet are similar to CS elsewhere in the body [4,18], but nuclear atypia and other histologic features can be shared with enchondromas [19], lending great importance to the imaging appearance of a cartilage lesion in the hands and feet as a predictor of malignancy.
http://dx.doi.org/10.1016/j.ejrad.2015.06.026 0720-048X/© 2015 Published by Elsevier Ireland Ltd.
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Therefore, the hypothesis of this study was that CS of the hands and feet have imaging features similar to enchondromas, but additional aggressive characteristics similar to those reported for CS in the central skeleton may be uncovered by cross-sectional imaging and bone scintigraphy over using radiography alone. The purpose of this study was to comprehensively describe the imaging characteristics of these rare lesions on radiography, as well as MR imaging and bone scintigraphy, their histologic grading, and provide a systematic review of all available reports of CS of the hands and feet in the literature for comparison.
grade was designated by the pathologist, with grade 1 showing cellularity and atypia with minimal myxoid change, grade 2 showing moderate increased cellularity and atypia with readily identifiable mitoses and limited necrosis, and grade 3 showing marked increased cellularity with pleomorphism, bizarre nuclei, numerous mitoses, prominent necrosis and myxoid change [19]. The pathologist had access to the imaging studies during interpretation of the pathology specimens.
2.5. Reader procedures 2. Materials and methods 2.1. Overview This retrospective study was approved by the institutional review board and informed consent was waived. The available imaging studies of CS of the hands and feet were reviewed by two musculoskeletal radiologists in consensus to identify imaging features by radiography, cross-sectional imaging and bone scintigraphy. The histologic features were reviewed by an experienced musculoskeletal pathologist. Descriptive statistics were reported. 2.2. Subject population Subjects were selected consecutively from a pathology database containing patients with histologically-proven resected bone tumors, over a 23 year period between June 1991 and July 2014 at one center. Inclusion criteria were any patient with a CS of the hands or feet who had an available histologic specimen for confirmation of the disease. Exclusion criteria were patients without available histology. One observer reviewed all available medical records on each patient and recorded the patient’s clinical features at presentation and availability of imaging studies. 2.3. Imaging studies Radiography in all subjects consisted of standard views, typically 2 views of the body part in question. Views of the hands and feet included postero-anterior views, and a lateral or additional oblique view. While 17 patients had images performed at only one time point, radiographic follow-up was available for 6 patients (mean 1834 days, range 11–3285 days). MR imaging studies were available in 7 subjects and were performed utilizing 1.5 Tesla systems (Siemens Medical Systems, General Electric Healthcare, Philips). MR imaging sequences consisted of spin echo T1-weighted images (TR/TE 450–650/20–28), fluid-sensitive sequences [(fat-suppressed T2 weighted (TR/TE 3000/4800/60–70) or short tau inversion recovery (STIR, TR/TE/TI 2500/60/160)] and T1-weighted gradient echo images obtained prior to and following the administration of an intravenous gadolinium-based contrast agent (TR/TE 60–60/5–8). Variable coils (quadrature, phased array or 5 inch surface coil), field of view (8–180 mm), section thickness (4–5 mm) and matrix (256 × 192, 256 × 256 or 512 × 256) were utilized. Technetium 99 whole-body bone scintigrams were available in two subjects, both performed as standard three-phase bone scans. 2.4. Pathologic interpretation All histologic specimens were reviewed by a pathologist specialized in the analysis of bone tumors, with experience of 40 years. Specimens were reviewed for the presence and degree of the following features: cellularity, atypia, necrosis, bone destruction and soft tissue invasion, polarity, mitoses and myxoid nodules. A tumor
Two experienced musculoskeletal radiologists (one with 14 years and one with 4 years post-residency experience) reviewed all imaging studies in consensus; radiographs, MR imaging sequences and bone scans (when available) were reviewed in the same session. The observers recorded the following features by radiography: involved bone of hand or foot, site of involvement in bone (epiphyses, metaphysis, diaphysis), percentage of bone width involvement (0–25%, 25–50%, 50–75%, >75%), percentage of bone length involvement (0–25%, 25–50%, 50–75%, >75%), percentage of endosteal scalloping (none, <2/3, at least 2/3), presence of expansion beyond the normal width of the bone (0–25%, 25–50%, 50–75%, >75% the width of the bone), matrix (lytic, sclerotic, mixed), mineralization percentage (none, 1–25%, 26–50%, 51–75%, >75% of lesion mineralization), as well as the presence or absence of a fracture or STM. For MR imaging, observers recorded the T1 and T2-weighted signal (hypointense, isointense, hyperintense to muscle), the T1and T2-weighted signal heterogeneity (homogeneous, <50% heterogeneity, >50% heterogeneity), the post-contrast pattern of enhancement (lobular, solid), the post-contrast T1-weighted signal heterogeneity (homogeneous, <50% heterogeneity, >50% heterogeneity), and the presence or absence of endosteal scalloping, perilesional bone marrow edema, periosteal reaction, perilesional soft tissue edema, perilesional contrast enhancement and a STM. For nuclear scintigraphy studies, the observers recorded whether there was uptake on each of the phases (perfusion, blood pool, delayed) and the degree of uptake on the delayed phase (similar or less than that of the anterior superior iliac spine (ASIS), or greater than that of the ASIS), similar to a prior report which showed 82% of CS of the appendicular skeleton having uptake greater than the ASIS, compared with only 21% of enchondromas [20].
2.6. Systematic review of the literature A search was made of articles in the peer-reviewed literature in PubMed that had been published since 1974, to identify reports of CS of the hands or feet. The search strategy included English language articles with the following search terms and keywords: chondrosarcoma, hand, digit, foot, as well as all the individual bones of the hands and feet. Articles on chondromas, chondromatosis, osteochondromas, and enchondromas as well as case reports in other languages were excluded.
2.7. Analysis The clinical and demographic features of the patients were tabulated and summarized. Descriptive statistics regarding the imaging features of CS by radiography, MR imaging and bone scintigraphy were reported, with categorization of the features by grade. A summary of the findings from a systematic review of the literature were also tabulated and features of CS uncovered in our study were compared with those that had been identified by review of the literature.
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3. Results Twenty-four cases of CS of the hands or feet were identified, for which radiography was available in 23, MR imaging in 7 and threephase bone scintigrams in 2 subjects. Overall, there were more females than males (14 vs. 10, respectively), with the patients having a mean age of 58 years, and age range of 32–92 years, although a greater percentage of subjects who were above the age of 50 (16/24) were female (67% female (10/16) vs. 33% (6/16) males). Regarding the location CS, 14 were in the hands and 10 were in the feet, with the most common location being the phalanges in both the hands and feet (45.8% (11/24) in the distal phalanx, 33% (8/24) in the proximal phalanx, 13% (3/24) in the middle phalanx), and only 4% (1/24) in the first metatarsal and 4% (1/24) in a tarsal bone (calcaneus). Table 1 is a summary of the clinical and imaging features of the CS in this study. There were more grade 2 (n = 17) than grade3 (n = 7) lesions in the study population, and the most common presenting clinical feature in these cases was a palpable mass or deformity (73%), rather than pain (27%). By radiography, most CS were expansile lesions (expanding the width of bone at least 50%), involved the end of bone, exhibited endosteal scalloping greater than 2/3 the width of the cortex and 69.5% (16/23) had an associated STM. The lesions were either lytic or had a mixed lytic-sclerotic appearance, and the majority had a mineralized matrix visible by radiography. Patients presented with pathologic fractures in 34.8% (8/23). For subjects who had serial follow-up, an increase in the length of bone involvement, degree of endosteal scalloping, degree of expansion and a change in matrix and mineralization appearance occurred over years, presumably suggesting secondary transformation of an enchondroma. Table 2 is a summary of imaging features according to tumor grade; there were no substantial differences in the imaging appearance of grade 2 and 3 lesions. Figs. 1 and 2 are two examples from our case series. By MR imaging, most CS were homogeneously isointense or hyperintense to skeletal muscle on T1 weighted images, and fairly homogeneously hyperintense on fluidsensitive sequences. The majority presented with perilesional bone marrow edema, periosteal reaction, soft tissue edema and a STM. Following contrast administration, the pattern of enhancement varied between lobular and solid enhancement and there was associated perilesional contrast enhancement in the majority of cases. In 1/7 MR studies, a pathologic fracture was present, potentially contributing to perilesional signal abnormalities. By bone scintigraphy, there was notable uptake on all three phases, greater than that identified in the ASIS. Finally, a systematic review of the literature on the imaging of CS of the hands and feet, identified 136 reports of CS of the hands and feet, with a total of 105 case reports (66 in the hand, 39 in the feet), 10 reports in other languages and 21 case series [6,7,10,13–17,21–33]. Table 3 summarizes the case series results, which included a total of 414 reports of CS of the hands and feet. Overall, the clinical presentation of CS of the hands and feet included pain as a common symptom (43%), but many CS also presented with painless growth. There were more CS reported in the hands than feet (60% vs. 40%, respectively), average age was over 50 years, and there were slightly more males with CS than females (53% vs. 47%, respectively). CS commonly occurred in the phalanges (35.8%) or metacarpals (18.3%) of the hand and the calcaneus was a common site in the foot (24.5%). Aggressive features were generally present by radiography, including expansile lesions with extra-cortical extension and STMs. Available reports of MR imaging (n = 4/21 case series) were incomplete, and relayed limited features, including hyperintensity on T2-weighted imaging and a STM [15]. Regarding the treatment of CS, amputation was considered the treatment of choice. Recurrence was reported in 24 cases, typically in cases where there was a local resection or curettage performed and there were 28 reported cases of metastatic disease with
3
Table 1 Summary of clinical, histological and imaging characteristics of study population. Clinical historya
Pain 3/11 (27.2%) Mass/deformity 8/11 (72.8%)
Histologic grade
Grade 2 17/24 (70.8%) Grade 3 7/24 (29.2%)
Bone involved
Phalanx 22/24 (91.7%) Metatarsal 1/24 (4.2%) Flat bone 1/24 (4.2%)
LOCATION Digit • Epiphysis • Metaphysis • Diaphysis Tarsal bone (Calcaneus)
22/23 (95.7%) 21/22 (95.5%) 21/22 (95.5%) 18/22 (90%) 0/22 (90.9%)b 1/23 (4.3%)
Radiographic features (n = 23) Bone width involvement Bone length involvement
Endosteal scalloping presence fraction scalloped
Expansile
Matrix
Matrix–mineralization Presence Percentage
Pathologic fracture STM MRI features (n = 7) T1-weightedc Signal Heterogeneity T2-weighted Signal hyperintensity Heterogeneity
Perilesional bone marrow edema Periosteal reaction Extra-osseous soft tissue edema Endosteal scalloping Contrast–enhanced T1-weightedd Enhancement pattern
76–100% 21/23 (91.3%) 51–75% 1/23 (4.3%) 25–50% 1/23 (4.3%) 76–100% 14/23 (60.8%) 16/23 (69.5%)b 51–75% 5/23 (21.7%) 25–50% 4/23 (17.3%) 22/23 (95.65%) No scalloping 1/22 (4.5%) <2/3 0/22 (0%) >2/3 21/22 (95.4%) 22/22 (100%)b 76–100% 10/23 (43.5%) 13/23 (56.5%)b 51–75% 6/23 (26.1%) 25–50% 4/23 (17.3%) <25% 3/23 (13.0%) Lytic 11/23 (47.8%) Sclerotic 0/23 (9%) Mixed 12/23 (52.2%) 13/23 (56.5%)b 21/23 (91.3%) 75–100% 0/21 (0.0%) 50–75% 3/21 (14.2%) 25–50% 5/21 (23.8%) 6/21 (28.6%)b <25% 13/21 (61.9%) 7/23 (30.4%) 16/23 (69.5%)
Isointense 3/6 (50%) Hyperintense 3/6 (50%) ≤50% heterogeneity 3/6(50%) >50% heterogeneity 3/6(50%) 7/7 (100%) Homogeneous 1/7 (14.2%) ≤50% heterogeneous 5/7 (71.4%) >50% heterogeneous 1/7 (14.2%) 6/7 (85.7%) 3/7 (42.8%) 5/7 (71.4%) 7/7 (100%)
Perilesional enhancement
Lobular 3/6 (50%) Solid 3/6 (50%) Homogeneous 5/6 (83.3%) <50% heterogeneous 1/6 (16.6%) 5/6 (83.3%)
Bone scan (n = 2) Perfusion Blood pool Delayed phase
Positive uptake 2/2 (100%) Positive uptake 2/2 (100%) Uptake >ASIS 2/2 (100%)
Heterogeneity
a b c d
Clinical history was available in 11 cases. Findings after follow-up. In one case, T1 weighted images were not available for viewing. Contrast-enhanced sequences were available in 6/7 MRI scans
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4 Table 2 Imaging features of CS compared by histologic grade.
Grade 2 (n = 17)
Grade 3 (n = 7)
Radiography Bone width: Involves >75% width of bone Bone length: Involves >75% length of bone Endosteal scalloping Expansile: >75% width of bone Matrix–mineralization Fracture ST mass MRI (n = 7) T1 signala T1 heterogeneity T2 Hyperintense T2 heterogeneity Periosteal/bone marrow edema Soft tissue edema Contrast enhancementa Perilesional enhancement ST mass:
15/17 (88.2%) 10/17 (58.9%) 15/17 (88.2%) 7/17 (41.2%) 11/17 (64.7%) 5/17 (29.4%) 9/17 (59.2%) (n = 6/7) Isointense 2/5(40%) Hyperintense 3/5(60%) ≤50% hetero 3/5 (60%) >50% hetero 2/5 (40%) 6/6 (100%) Homogeneous 1/6(16.7%) ≤50% hetero 4/6(66.6%) >50% hetero 1/6(16.7%) 5/6 (83.3%) 4/6 (66.3%) 2/5 (40%) lobular 3/5(60%) solid 4/5 (80%) 6/6 (100%)
6/7 (85.7%) 4/7 (57.5%) 6/7 (85.7%) 3/7 (42.8%) 5/7 (71.4%) 2/7 (28.5%) 5/7 (71.4%) (n = 1/7) Isointense 1/1(100%) >50% hetero 1/1 (100%) 1/1 (100%) ≤50% hetero 1/1(100%) 1/1 (100%) 1/1 (100%) 1/1 (100%) lobular 1/1 (100%) 1/1 (100%)
BONE SCANb Positive uptake all 3 phases Positive uptake >ASIS
1/1 (100%) 1/1 (100%)
1/1 (100%) 1/1 (100%)
a b
n = 6 MR imaging studies with T1 imaging and contrast, 5 grade 2 and 1 grade 3. n = 2 bone scintigrams performed, 1 grade 2 and 1 grade 3.
Table 3 Systematic review of the literature: clinical, pathologic and imaging characteristics of patients with CS of the hands and feet. Reference
Mean age/sex
Clinical history
Site
Histologic features
Imaging features Radiographic
MRI
[27] (n = 6) [21] (n = 24)
Not provided Not provided
4/6 sec Mafucci or Ollier disease
6/6 Hands 13/24 Hands 11/24 Feet
None - cortical destruction - soft tissue extension
None None
[22] (n = 2)
52 years 2/2 F
2/2 pain
2/2Hands
Not provided 14 Gr 1 4 Gr 2 1 Gr 3 5 Unknown Gr 1–1 Gr 3 – 1
- lytic - cortical destruction - soft tissue extension
None
[14] (n = 8)
39 years 2/8 F
8/8 sec
8/8 Feet
None
- STM - cortical destruction
None
[15] (n = 2)
52 years 0/2 F
2/2 sec 0/2 pain 2/2 painless
2/2Hand
2 Gr 1
-
- Hypointense T1 - STM
[6] (n=12)
53 years 2/11 F
3/12 sec 8/12 pain 4/12 painless
12/12 Feet
2 Gr 1 9 Gr 2 1 Gr 3
- endosteal erosion None - cortical destruction–indistinct margins - matrix calcification–expansion - STM - joint extension - fracture
[6] (n = 23)
71 years 13/23 F
10/23 pain; 11/23 painless; 2/23 fracture
23/23 Hand
1 Gr 1 20 Gr 2 2 Gr 3
-
matrix calcification endosteal erosion cortical destruction expansion STM indistinct margins joint extension fracture
None
[24] (n = 35)
67 years 21/35 F
5/35 sec
27/35Hands 8/35 Feet
8 Gr 1 26 Gr 2 1 Gr 3
-
lytic lobulated mineralized ill-defined margins cortical destruction soft-tissue extension
None
lytic chondroid matrix cortical destruction STM
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Table 3 (Continued) Reference
Mean age/sex
Clinical history
Site
Histologic features
Imaging features Radiographic
MRI
[16] (n = 3)
50 years 0/3 F
3/3 painful mass
3/3 Feet
2 Gr 1–2
-
expansile lytic cortical thinning chondroid matrix
- lobular - high T2 signal intensity - septal contrast enhancement
[25] (n = 12)
55 years 7/12 F
12 sec 7/12 pain; 3/12 painless; 1/12 unknown; 1/12 injury
10/12Hands 2/12 Feet
5 Gr 1 6 Gr 2 1 Gr 3
-
expansion periosteal reaction cortical destruction STM mineralized
1/12 had MRI – no features not reported
[23] (n = 161)
53 years 77/161 F
19/161 sec 50/161 pain; 29/161 painless; Others not provided
88/161 Hands 75/161 Feet
116 Gr 1 44 Gr 2 3 Gr 3
-
endosteal erosion cortical destruction expansion STM chondroid mineralization
None
[26] (n = 13)
53 years 3/13 F
2/13 sec 3/8 pain 5/13 no history available
Hands and Feeta
9 Gr 1 2 Gr 2 2 Gr 3
- lytic - punctate calcification - irregular cortical destruction - soft tissue extension
None
[17] (n = 3)
59 years 2/3 F
3/3 painless mass
3/3Feet
2 Gr 1 2 Gr 2 Periosteal CS
- mineralized - cortically based
- marginal and septal enhancement
[28] (n = 14)
48 years 4/14 F
Not provided
4 Hands 10 Feet
Not provided
Not provided
[10] (n = 2)
59 years 2/2 F
2/2 sec
2/2 Hand
10 Gr 1 3 Gr 2 1 Gr 3 1/2 Gr 1 1/2 Gr 2
- lytic - mineralized - STM
None
[29] (n = 2)
57 years 1/2 F
Pain/mass-no details provided
2/2 Hand
2/2 Gr 2
- expansile - mineralized - fracture
None
[30] (n = 22)
43 9/22 F
6/22 sec
22/22 Feet
Gr not provided
Not provided
None
None
- Pain/swelling no details provided [33] (n = 18)
68 years 10/18 F
4/18 sec 13/18 pain
18/18Hand
Gr not provided
-
[31] (n = 3)
38 years 2/3 F
3/3 pain
3/3 Feet
Gr not provided
- marked cortical expansion - trabecular destruction
None
[13] (n = 19)
63 years 14/19 F
10/19 pain
19/19Hand
Gr not provided
-
large indistinct margins bone destruction soft-tissue invasion
None
[32] (n = 30)
50 years 13/30 F
4/30 sec 18/30 pain
16/30 Feet
12/30 Gr 1 15/30 Gr 2 3/30 Gr 3
-
expansile lytic mineralized cortical destruction soft tissue extension fracture
None
large indiscrete margins mineralized lytic soft tissue invasion fracture
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6 Table 3 (Continued) Reference
Mean age/sex
Clinical history
Site
Histologic features
Total n = 414a
54.2 years 48% F (184/384) 52% M (200/384)
59 sec 43.1% pain (127/295) 56.9% painless or unknown history (168/295)
60% Hand (229/383) 35.8% PP (82/229) 18.3% MC (42/229) 40% Feet (151/383) 24.5% Calcaneus (37/151)
55% Gr 1 (181/332) 40% Gr 2 (133/332 5% Gr 3 (18/332)
Imaging features Radiographic
MRI
Common features:
Common features (n = 4 with MRI)
- Expansile, - Lytic - Chondroid mineralization - Cortical destruction - STM
- Lobulated septal enhancement - STM
Gr = grade, PP = proximal phalanx, DP = distal phalanx, MC = metacarpal, MT = metatarsal, CS = chondrosarcoma, sec = secondary, M = male, F = female. a Information not available.
Fig. 1. 53 year old woman presented with pain and swelling of the long finger following trauma. (a–c) Anteroposterior, oblique and lateral radiographs of the right hand shows an expansile, lytic lesion (arrow) in the distal phalanx of the long finger involving the majority of the length and width of the affected bone. There is associated endosteal scalloping and minimal internal chondroid matrix (the latter best visualized on the lateral image). (d and e) Sagittal T1-weighted (TR/TE 650/22) and fluid sensitive (STIR, TR/TE/TI 2500/60/160) imaging shows a marrow-replacing lesion in the distal phalanx with slightly hyperintense T1 signal and diffusely hyperintense fluid signal (arrow). (f) Post-contrast, coronal fat suppressed T1-weighted image (TR/TE 650/22) through the hand shows heterogeneous “solid pattern” of internal enhancement (arrow). The patient underwent amputation of the distal phalanx and a portion of the middle phalanx (g). Photomicrograph obtained from the resected specimen shows a grade 2 chondrosarcoma with moderate atypia and binucleated chondrocytes.
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Fig. 2. 53 year old woman who presented with a palpable mass and deformity of the ring finger. (a and b) Anteroposterior and lateral views of the ring finger showing a markedly expansile lesion involving the entire length of the distal phalanx, with mineralization and soft tissue extension. (c) Photomicrograph shows a grade 3 chondrosarcoma with increased cellularity, atypia, and mitotic figures.
CS of the hands and feet. In our subject population, treatment of CS consisted of ray amputation (23/24); in the case of one patient with a calcaneal CS, excisional biopsy was performed and no records were otherwise available.
4. Discussion CS is a rare tumor in the hands and feet. Our study extends the work of prior reports with a comprehensive assessment of the multimodality imaging features, the histologic grade and clinical presentation of this disease. In our series, there were more women than men affected by this disease, and a predilection towards the female gender has also been noted in other investigations [13], although by systematic review of the literature, slightly more males (52%) were affected by this disorder. Regarding the age of presentation, two-thirds of the patients in our series were above the age of 50, in keeping with previously described reports of CS in the hands and feet. Regarding the location of CS in this series, the vast majority of cases in our series occurred in the phalanges, rather than the more proximal tubular bones or tarsal or carpal bones of the hands and feet, a distribution similar to several prior reports of CS in the hands and feet [6,7,21,23,25,32,33]. The propensity for benign enchondromas to occur in the phalanges as well as CS is in keeping with a common pathogenesis to these lesions; furthermore, a susceptibility to trauma in the more distal hands and feet, which may induce a pathologic fracture, may bring these lesions to presentation more easily than the tarsal or carpal bones. Of note, CS has also been reported to have a predilection for the calcaneus in the hindfoot [14,23], and there was one case located in the calcaneus in our series as well. Unlike CS elsewhere in the appendicular skeleton, a history of pain in our series of CS in the hands and feet was generally uncommon, but present in patients with pathologic fractures, as expected. Other reports have described a mixture of presenting clinical features, including pain as well as a painless presentation [6,7,23]; thus, pain is not a necessary feature of CS of the hands and feet, even though it has been described as a differentiator of enchondromas and CS elsewhere in the body [2].
Regarding the extent of osseous involvement, CS of the hands and feet involved the majority of the length of the bone (including the end of bone), and were not confined to the metadiaphysis as in other extremity CS [34]. Most cases presented with aggressive features similar to those reported CS features in the extremities outside the hands and feet, including significant endosteal scalloping greater than 2/3 of the cortex [2], and approximately 70% of our cases were associated with a STM. Overall, these findings suggest that CS of the hands and feet may have a later presentation, allowing for a greater extent of involvement of the bone and slightly greater involvement of the adjacent soft tissues, than CS in other parts of the appendicular skeleton. A series of 104 intramedullary CS similarly reported an associated STM in 79.8% (83/104) of cases, as seen by radiography [23], although cross-sectional imaging is more sensitive to the detection of a STM in patients with CS [35]. By cross-sectional imaging and bone scintigraphy, additional aggressive characteristics of CS were observed. By MR, as expected, CS of the hands and feet were hyperintense on fluid-sensitive sequences, and were of intermediate or high signal intensity on T1weighted imaging, as has been previously reported for CS outside the hands and feet [36]. The high proportion of periosteal reaction, perilesional bone and soft tissue edema, perilesional contrast enhancement and a STM may in part be explained by the presence of pathologic fractures, although even in cases without a fracture, these aggressive features were present. Finally, by scintigraphy, CS in this series showed significant uptake, greater than that in the ASIS, a feature common to CS found elsewhere in the appendicular skeleton [20]. Enchondromas are the most common benign bone lesions of the hands and feet [37], and the problem of distinguishing CS from the more frequent enchondromas of the hands and feet is challenging, as it is elsewhere in the body. Imaging is not only important for suggesting the presence of an aggressive cartilage lesion in the hands and feet, but also guides the histologic assessment of these lesions. In the small tubular bones of the hands and feet, the histologic threshold for the diagnosis of CS is much higher than that for CS elsewhere in the body. What might be regarded as a grade 1CS in the femur would not be considered CS histologically in the bones of the hands and feet. In particular, increased cellularity, atypical
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chondrocytes, bi-nucleated chondrocytes and even myxoid change are aggressive histologic features that can be associated with low grade cartilage lesions (including enchondromas) in the hands and feet, but are diagnostic of higher grade cartilage lesions outside the hands and feet [19,38]. Hence, aggressive imaging features suggesting a CS in the hands and feet in the setting of aggressive histologic features can appropriately guide the histologic diagnosis toward CS rather than enchondroma. In one of the cases in our study, features of an enchondroma with atypia were identified by initial histology (from a biopsy specimen), but subsequently evolved to a presumed secondary CS five and a half years later (after resection). Initially, the lesion showed typical radiographic features of an enchondroma, with subsequent development of aggressive imaging characteristics. In fact, in all cases of our study group, the imaging features of CS of the hands and feet were aggressive at the time of surgical resection, although it is unclear whether these presumed secondary CS in our series evolved from an enchondroma or were slow-growing non-aggressive appearing CS initially. Traditionally, low grade CS in the proximal appendicular skeleton (such as the humerus or femur) have been reported to resemble enchondromas by imaging [20,39,40], but in our series in the hands and feet, no grade 1CS was included and intermediate grade CS (grade 2) had features similar to higher grade lesions (significant expansion, perilesional edema and enhancement, and the presence of a STM), and were unlike traditionally-described enchondromas (which lack significant expansion, perilesional edema and a STM). Nevertheless, it is likely that some imaging features of CS reported here, such as deep endosteal scalloping and osseous expansion, overlap with features of some enchondromas. Finally, regarding the prognosis and treatment of CS of the hands and feet, some studies have termed CS of the digits as “non-metastising chondrosarcoma” because these lesions rarely metastasize as CS of the pelvis or the more proximal skeleton [41], although by systemic review of the literature, there were 28 reported cases of metastatic disease from CS of the hands and feet. Nevertheless, because CS of the hands and feet are rare, their natural history is not well understood. Morbidity in the form of recurrence may occur after surgical resection, especially if only a curettage is performed. Therefore, radical excision and amputation are generally recommended even in lower grade CS [2]. Our study has limitations, including a small sample. In our study, only 24 cases of CS of the digits were found over a long period (1983–2012), confirming the rarity of this disease; thus, the number of cases is not sufficient for statistical analysis. Also, as this was a retrospective study, the imaging protocols were not uniform, and follow-up of the patients was not available in many cases. Similarly, in performing a systematic review of the literature, imaging, histologic and clinical features were not reported uniformly, making comparisons between case reports and case series difficult. In conclusion, CS of the hands and feet have aggressive features that include significant endosteal scalloping, an expansile appearance with involvement of the end of the bone as well as the metadiaphysis. Additional aggressive imaging features are identified by MR (perilesional signal abnormalities and STMs) and bone scintigraphy (increased uptake > ASIS) that may be helpful clues to the diagnosis and can also guide the histologic assessment of these lesions.
[2]
[3] [4]
[5]
[6] [7] [8]
[9]
[10]
[11]
[12] [13] [14]
[15]
[16]
[17]
[18]
[19] [20]
[21]
[22]
[23]
[24] [25] [26] [27]
[28] [29]
Acknowledgement [30]
None to report. [31]
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