|Year : 2020 | Volume
| Issue : 2 | Page : 271-273
Chondromyxoid fibroma: Rare entity in a rare location
Sarita Asotra1, Pooja Chauhan2, Kanishk Gupta3
1 Department of Pathology, SLBSGMCH, Mandi, India
2 Department of Pathology, IGMC, Shimla, Himachal Pradesh, India
3 Department of Pathology, GIPMER, New Delhi, India
|Date of Submission||08-Jun-2020|
|Date of Decision||24-Jun-2020|
|Date of Acceptance||04-Aug-2020|
|Date of Web Publication||23-Dec-2020|
Dr. Pooja Chauhan
H No. 51, Housing Board Colony, Sanyard (PO), Talyahar, Mandi - 175 001, Himachal Pradesh
Source of Support: None, Conflict of Interest: None
Chondromyxoid fibroma (CMF) is a rare benign tumor affecting lower extremities with rare upper limb involvement. It is usually diagnosed by histology, and differentiating it from other benign and malignant neoplasms is of utmost importance for patient management. We present a case of CMF in an unusual location.
Keywords: Bone tumors, chondromyxoid, osteolytic
|How to cite this article:|
Asotra S, Chauhan P, Gupta K. Chondromyxoid fibroma: Rare entity in a rare location. Arch Med Health Sci 2020;8:271-3
| Introduction|| |
Chondromyxoid fibroma (CMF) is a rare tumor, accounting for <1% of all bone tumors, with a male-to-female ratio of about 1.5:1, and it occurs during the second or third decade of life though cases are widely seen in the later decades. It is known that approximately 75% of the total cases of CMF affect the bones of the lower extremities. In particular, it occurs most frequently in the tibia and femur around the knee joint. Thus far, its incidence in the hand has been described to be very rare. Most cases of CMF typically originate from the metaphysis and may then extend to the epiphysis and diaphysis. The diagnosis is usually made by radiology and histopathology because CMF is mostly myxoid and often has osteoclastic giant cells. However, malignant bone tumors can be histologically difficult to distinguish from CMF.
| Case Report|| |
A 43-year-old female patient presented to the orthopedic outpatient department with pain and swelling in the right thumb for 5 months. Physical examination revealed a firm immobile mass measuring 1.5 cm × 1 cm on the proximal phalanx of the thumb. X-rays showed an osteolytic lesion with a sclerotic border involving the proximal phalanx of the first digit [Figure 1]. Extensive curettage of the lesion was done, and the specimen was sent for histopathological evaluation. We received a single, gray-white, lobulated mass measuring 1.5 cm × 1 cm × 1 cm. Histopathological examination revealed lobules of chondromyxoid material showing stellate cells in myxoid background and benign cartilage surrounded by hypercellular fibrous stroma showing osteoclastic giant cells at the periphery of the lesion. The histopathological features were suggestive of CMF [Figure 2],[Figure 3],[Figure 4] immunohistochemical staining for S-100 was advised. Chondroid elements in CMF were S-100 positive.
|Figure 1: Osteolytic lesion with sclerotic border involving the middle phalanx|
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|Figure 2: (a-c) Lobules of chondromyxoid material showing stellate cells in myxoid background and benign cartilage surrounded by hypercellular fibrous stroma showing osteoclastic giant cells at the periphery of the lesion – features suggestive of chondromyxoid fibroma: H and E, ×100|
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|Figure 3: Benign cartilage surrounded by hypercellular fibrous stroma: H and E, ×100|
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| Discussion|| |
CMF was first described by Jaffe and Lichtenstein in 1948, and it is a rare, slow-growing, benign bone tumor of cartilaginous origin. It accounts for <1% of all the primary bone tumors and <2% of benign bone tumors. It classically occurs in the metaphyseal region of the long bones surrounding the knee but its occurance in other long bones, pelvis, ribs and small foot bones has been reported. The small bones of the hand, however, are rarely involved. The tumor is considered a physeal plate remnant and may involve the epiphysis, diaphysis, or both along with its metaphyseal origin. It may cause cortical expansion and destruction, but consistently respects the periosteal boundary. CMF occurs predominantly in persons between the ages of 10 and 30, rarely below 5 and above 60. There is a slight predominance of males over females, 55% as opposed to 45%. Clinically, CMFs are slow-growing and are commonly asymptomatic lesions that are discovered incidentally on radiography. When symptomatic, their clinical presentation is usually chronic local pain (85%), swelling (65%), restriction of motion, and, more rarely, pathological fracture. Radiographically, the lesion involves the diametaphyseal area of a limb bone and has a slightly sclerotic margin, giving the impression of a benign tumor. Occasionally, considerable reactive sclerosis may be present, particularly in later stages, accompanied by scalloping of the endosteal margins. Although the matrix of the tumor is largely cartilaginous, flecks of calcification are distinctly unusual (Turcotte, 1962).
The tumor resembles cartilage in naked eye. It glistens a white or tan color and appears almost translucent. It is firm and rubbery in consistency and forms well-circumscribed globules. Histopathologically, it is characterized by the multilobular arrangement of stellate or spindle-shaped cells in an abundant myxoid background or chondroid intracellular material. These lobules are composed of central hypocellular area and peripheral hypercellular area. Surgical excision is the first line of choice for CMF, for which only simple curettage is performed or a bone graft is used for filling the cavitary defect following curettage. Although variable depending on the reports, the recurrence is estimated at approximately 25%. Postoperatively, regular follow-up including radiography is necessary. A good prognosis of CMF has been documented. Cells that 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. Furthermore, CMFs may show focal SMA positivity.
Cytogenetically, CMFs are characterized by chromosome 6 aberrations that are heterogeneous. The commonly involved regions include 6p23–25, 6q12–15, and 6q23–27. Aberrations involving 6q13–21 are linked to the locally aggressive behavior of cartilage tumors, including CMF.
The malignant transformation of CMFs is a rare event and is reported to be 1%–2%, which is the same as for any other benign bone tumor. In the series by Wu et al., two cases underwent malignant transformation, which was malignant fibrous histiocytoma in one and fibrosarcoma in the other.
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 and often show abundant free-flowing myxoid ground substance. Hyaline cartilage is more likely to be seen in chondrosarcomas. Chondrosarcoma, usually seen in the 60–80-year-old group, is identified by its soft tissue and cortical invasion, but care should be exercised in making this diagnosis on histological grounds without clinical correlation. Prior to the recognition of CMF by Jaffe in 1948, four cases reported by Dahlin and two by Jaffe had been diagnosed as malignant tumors. 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, which is not a feature in CMFs.
| Conclusion|| |
CMF is a rare, benign bone neoplasm but can be confused with other malignant cartilaginous neoplasms, therefore its recognition and differentiation from other tumors is of importance.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]