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CASE REPORT
Year : 2018  |  Volume : 45  |  Issue : 2  |  Page : 106-109

Chondromyxoid fibroma at an unusual site


1 Departments of Pathology, JN Medical College, KLE Academy of Higher Education and Research, Belagavi, Karnataka, India
2 Department of Consulatant Pathologist, Belagavi, Karnataka, India
3 Department of Orthopedics, Belagavi, Karnataka, India

Date of Web Publication10-Dec-2018

Correspondence Address:
Karthik Srevatsa
123 First Floor, 4th Main KGE Layout, New BEL Road, Bengaluru - 560 096, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jss.JSS_43_18

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  Abstract 


Chondromyxoid fibroma (CMF) is a rare tumor, accounting for <1% of all bone tumors. It generally affects the metaphysis of long bones of the lower limbs and involvement of the upper limb is rare. It can be confused with chondroblastoma and chondrosarcoma. Its recognition and differentiation from other tumors are of paramount importance. Here, we report a case of CMF involving the right ring finger.

Keywords: Benign rare tumor of bone, chondroblastoma, chondromyxoid fibroma, chondrosarcoma


How to cite this article:
Srevatsa K, Kangle RP, Kangle P, Haveri S. Chondromyxoid fibroma at an unusual site. J Sci Soc 2018;45:106-9

How to cite this URL:
Srevatsa K, Kangle RP, Kangle P, Haveri S. Chondromyxoid fibroma at an unusual site. J Sci Soc [serial online] 2018 [cited 2019 Mar 19];45:106-9. Available from: http://www.jscisociety.com/text.asp?2018/45/2/106/247157




  Introduction Top


Chondromyxoid fibroma (CMF) is an unusual bone tumor first described in 1948 by Jaffe and Lichtenstein.[1] CMF is a rare tumor, accounting for <1% of all bone tumors. CMF has a predilection for males, with a male-to-female ratio of about 1.5:1, and it preferentially affects young patients during the second or third decades of life. Individual cases, however, are widely seen in the later decades, and some cases are diagnosed in patients who aged 50 years and older.[1] It generally affects the metaphysis of long bones of the lower limbs and involvement of the upper limb is rare.


  Case Report Top


A 53 year old female patient presented with pain and swelling in the right ring finger along with a swelling in the palmar aspect since 3 months. The pain was dull aching and continuous patient did not give any history of trauma [Figure 1]. Physical examination revealed mild tenderness locally with overlying skin being normal. A radiographic examination showed an osteolytic, radiolucent, eccentric lesion in the metaphysis at the middle phalanx of the right ring finger [Figure 2].
Figure 1: Swelling in the ring finger near the middle phalanx

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Figure 2: X-ray showing osteolytic, radiolucent eccentric lesion in the metaphysis

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Magnetic resonance imaging (MRI) revealed a well-defined lesion in the middle phalanx which showed isointense signal on T1 and hyperintense signal on T2. The lesion was in proximity with the flexor tendon with mild erosion of the middle phalanx [Figure 3]. A possibility of ganglion cyst or fibroma was suggested.
Figure 3: Magnetic resonance imaging showing a hyperintense signal on T2

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The patient was operated, and swelling was excised. Gross examination of the swelling revealed a firm gray-white mass which was well delineated and measured 1.2 cm × 0.8 cm × 0.7 cm. The cut section was gray-white.

Microscopy revealed myxoid and chondroid areas separated by hypercellular mononuclear areas. The cells in the myxoid area were spindle shaped. The solid areas consist of oval mononuclear cells representing chondroblasts [Figure 4], [Figure 5], [Figure 6].
Figure 4: Low-power photomicrograph showing chondroid, fibromatous area along with areas of myxoid degeneration

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Figure 5: High-power photomicrograph showing fibromatous and chondroid area with myxoid degeneration

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Figure 6: High-power photomicrograph showing fibromatous area

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No evidence of mitosis in the section studied. Immunohistochemistry for S-100 was negative in the myxoid area, but cells showed cytoplasmic positivity for smooth muscle actin (SMA) [Figure 7] and [Figure 8].
Figure 7: Immunohistochemistry for S-100 negative

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Figure 8: Immunohistochemistry smooth muscle actin strongly positive

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  Discussion Top


CMF is a rare tumor, usually seen in the younger age group in the second and third decades, with a predilection toward males. CMFs are reported to involve the ribs, clavicle, sternum spine, and also bones of the hand and feet.[2]

Pain and slowly growing swelling are the chief complaints. Radiographic imaging usually reveals a lytic, oval, eccentric metaphyseal lesion with long axis parallel to the bone.[3],[4],[5] The intramedullary margin of the tumor is sharply defined with a sclerosed and scalloped border. MRI T1-weighted imaging has hypointense signal and on MRI T2 lesion usually shows a signal enhancement.[6] CMFs are metabolically active as revealed by fluorodeoxyglucose-positron emission tomography scan.[7] In small bones of the hands and feet, CMFs are usually central and uniformly expand the bone, but eccentric lesions can also occur [8] Gross examination of the CMF usually has a firm gray-white lobulated mass which is sharply demarcated and confined by intact periosteum. Areas of myxoid appearance can be seen, but generally, chondroid foci are not seen grossly. Microscopic examination typically has pseudolobulated architecture with myxomatous and chondroid areas separated by zones of hypercellular mononuclear tissues containing sparse giant cells.[9],[10] The lesion consists of a myxoid mesenchymal tissue that has undergone cartilaginous differentiation. These myxoid areas form a geographic or pseudolobulated pattern. The cellularity increases toward the periphery. Areas of solid cellularity are composed of plump, oval mononuclear cells representing chondroblasts with occasional giant cells seen in between the pseudolobules. The tumor cells within the pseudolobules are pleomorphic with hyperchromatism and multinucleation. Mitoses are rare.

Immunohistochemistry stain reveals that the cells of myxoid areas; cells in the hypercellular pseudosepta are weakly positive for S-100. Cells that are exhibit more chondroblastic differentiation are strongly positive for S-100. These features indicate that the CMFs are cartilaginous. The chondroblastic nature of cells in CMF is further confirmed by the expression of SOX9 protein.[11],[12],[13] Furthermore, CMFs may show focal SMA and multiple system atrophy positivity.

Genome-wide expression studies indicate that CMF has a profile distinct from that of other cartilage neoplasms such as chondroblastoma, enchondroma, and chondrosarcoma.[14]

Cytogenetically, CMFs are characterized by chromosome 6 aberrations that are heterogeneous. The commonly involved regions include 6p23-25, 6q12-15, and 6q23-27.[15],[16],[17],[18],[19]

Aberrations involving 6q13-21 were linked to the locally aggressive behavior of cartilage tumors, including CMF.[20]

The differential diagnosis of CMFs includes myxoid chondrosarcoma, CMF-like or chondroblastic osteosarcomas, and chondroblastomas on histology.

The myxoid chondrosarcomas share the lobular pattern, myxoid stroma, and peripheral cellularity with CMFs. However, chondrosarcomas are much more monotonous than CMFs and generally lack giant cells at the periphery of the lobules, and the lobules are usually rather large [21] and often show abundant free-flowing myxoid ground substance. Hyaline cartilage is more likely to be seen in chondrosarcomas.

Chondroblastic osteosarcomas have very large CMF-like areas and are difficult to differentiate from CMFs in a small biopsy sample. Mitoses and nuclear atypia are seen in osteosarcomas but not in CMFs. The diagnostic hallmark of osteosarcoma is the presence of osteoid production [22] which is not a feature in CMFs.

Grossly chondroblastomas arise from the epiphyseal ends of long bones. Chondroblasts are polygonal with eosinophilic cytoplasm. Calcification is a prominent feature in most of the chondroblastomas, which is unlikely in chondromyxoid fibroma.

Radiation therapy has been used to treat surgically inaccessible tumors. However, in rare instances, the development of high-grade sarcomas has been reported in association with this approach.[23]

Recurrences in CMFs are known to occur in 3%–22% of the cases sometimes even as late as 19 years.[24]

Malignant transformation of CMFs is a rare event and is reported to be 1%–2%,[25] which is the same as for any other benign bone tumor. In the series by Wu et al.,[10] two cases underwent malignant transformation, which was malignant fibrous histiocytoma in one and fibrosarcoma in the other. The latter had received radiotherapy.


  Conclusion Top


CMF is a rare, benign bone neoplasm. It can be confused with chondroblastoma and chondrosarcoma. Its recognition and differentiation from other tumors are of paramount importance.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Jaffe HL, Lichtenstein L. Chondromyxoid fibroma of bone; a distinctive benign tumor likely to be mistaken especially for chondrosarcoma. Arch Pathol (Chic) 1948;45:541-51.  Back to cited text no. 1
    
2.
Sakamoto A, Tanaka K, Matsuda S, Hosokawa A, Harimaya K, Yoshida T, et al. Chondromyxoid fibroma of the clavicle. J Orthop Sci 2006;11:533-6.  Back to cited text no. 2
    
3.
Feldman F, Hecht HL, Johnston AD. Chondromyxoid fibroma of bone. Radiology 1970;94:249-60.  Back to cited text no. 3
    
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Gherlinzoni F, Rock M, Picci P. Chondromyxoid fibroma. The experience at the Istituto Ortopedico Rizzoli. J Bone Joint Surg Am 1983;65:198-204.  Back to cited text no. 4
    
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Rahimi A, Beabout JW, Ivins JC, Dahlin DC. Chondromyxoid fibroma: A clinicopathologic study of 76 cases. Cancer 1972;30:726-36.  Back to cited text no. 5
    
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Kim HS, Jee WH, Ryu KN, Cho KH, Suh JS, Cho JH, et al. MRI of chondromyxoid fibroma. Acta Radiol 2011;52:875-80.  Back to cited text no. 6
    
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Hamada K, Tomita Y, Konishi E, Fujimoto T, Jin YF, Outani H, et al. FDG-PET evaluation of chondromyxoid fibroma of left ilium. Clin Nucl Med 2009;34:15-7.  Back to cited text no. 7
    
8.
Rozeman LB, Hameetman L, van Wezel T, Taminiau AH, Cleton-Jansen AM, Hogendoorn PC, et al. CDNA expression profiling of chondrosarcomas: Ollier disease resembles solitary tumours and alteration in genes coding for components of energy metabolism occurs with increasing grade. J Pathol 2005;207:61-71.  Back to cited text no. 8
    
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Kreicbergs A, Lönnquist PA, Willems J. Chondromyxoid fibroma. A review of the literature and a report on our own experience. Acta Pathol Microbiol Immunol Scand A 1985;93:189-97.  Back to cited text no. 9
    
10.
Wu CT, Inwards CY, O'Laughlin S, Rock MG, Beabout JW, Unni KK, et al. Chondromyxoid fibroma of bone: A clinicopathologic review of 278 cases. Hum Pathol 1998;29:438-46.  Back to cited text no. 10
    
11.
Dancer JY, Henry SP, Bondaruk J, Lee S, Ayala AG, de Crombrugghe B, et al. Expression of master regulatory genes controlling skeletal development in benign cartilage and bone forming tumors. Hum Pathol 2010;41:1788-93.  Back to cited text no. 11
    
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Horvai AE, Roy R, Borys D, O'Donnell RJ. Regulators of skeletal development: A cluster analysis of 206 bone tumors reveals diagnostically useful markers. Mod Pathol 2012;25:1452-61.  Back to cited text no. 12
    
13.
Konishi E, Nakashima Y, Iwasa Y, Nakao R, Yanagisawa A. Immunohistochemical analysis for sox9 reveals the cartilaginous character of chondroblastoma and chondromyxoid fibroma of the bone. Hum Pathol 2010;41:208-13.  Back to cited text no. 13
    
14.
Romeo S, Oosting J, Rozeman LB, Hameetman L, Taminiau AH, Cleton-Jansen AM, et al. The role of noncartilage-specific molecules in differentiation of cartilaginous tumors: Lessons from chondroblastoma and chondromyxoid fibroma. Cancer 2007;110:385-94.  Back to cited text no. 14
    
15.
Dadfarnia T, Velagaleti GV, Carmichael KD, Eyzaguirre E, Eltorky MA, Qiu S, et al. At(1;9)(q10;q10) translocation with additional 6q23 and 9q22 rearrangements in a case of chondromyxoid fibroma. Cancer Genet 2011;204:666-70.  Back to cited text no. 15
    
16.
Granter SR, Renshaw AA, Kozakewich HP, Fletcher JA. The pericentromeric inversion, inv (6)(p25q13), is a novel diagnostic marker in chondromyxoid fibroma. Mod Pathol 1998;11:1071-4.  Back to cited text no. 16
    
17.
Halbert AR, Harrison WR, Hicks MJ, Davino N, Cooley LD. Cytogenetic analysis of a scapular chondromyxoid fibroma. Cancer Genet Cytogenet 1998;104:52-6.  Back to cited text no. 17
    
18.
Romeo S, Duim RA, Bridge JA, Mertens F, de Jong D, Dal Cin P, et al. Heterogeneous and complex rearrangements of chromosome arm 6q in chondromyxoid fibroma: Delineation of breakpoints and analysis of candidate target genes. Am J Pathol 2010;177:1365-76.  Back to cited text no. 18
    
19.
Tallini G, Dorfman H, Brys P, Dal Cin P, De Wever I, Fletcher CD, et al. Correlation between clinicopathological features and karyotype in 100 cartilaginous and chordoid tumours. A report from the chromosomes and morphology (CHAMP) collaborative study group. J Pathol 2002;196:194-203.  Back to cited text no. 19
    
20.
Sawyer JR, Swanson CM, Lukacs JL, Nicholas RW, North PE, Thomas JR, et al. Evidence of an association between 6q13-21 chromosome aberrations and locally aggressive behavior in patients with cartilage tumors. Cancer 1998;82:474-83.  Back to cited text no. 20
    
21.
Keel SB, Bhan AK, Liebsch NJ, Rosenberg AE. Chondromyxoid fibroma of the skull base: A tumor which may be confused with chordoma and chondrosarcoma. A report of three cases and review of the literature. Am J Surg Pathol 1997;21:577-82.  Back to cited text no. 21
    
22.
Chow LT, Lin J, Yip KM, Kumta SM, Ahuja AT, King WW, et al. Chondromyxoid fibroma-like osteosarcoma: A distinct variant of low-grade osteosarcoma. Histopathology 1996;29:429-36.  Back to cited text no. 22
    
23.
Dorfman HD. Malignant transformation of benign bone lesions. In: Proceedings of the Seventh National Cancer Conference of the American Cancer Society. Philadelphia: Lippincott; 1973.  Back to cited text no. 23
    
24.
Mirra JM. Bone tumors. Diagnosis and Treatment. Philadelphia, Toronto: JB Lippincott; 1980.  Back to cited text no. 24
    
25.
Beggs IG, Stoker DJ. Chondromyxoid fibroma of bone. Clin Radiol 1982;33:671-9.  Back to cited text no. 25
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]



 

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