|Year : 2020 | Volume
| Issue : 1 | Page : 47-50
Chondromyxoid fibroma of the femoral head: A rare case and review of literature
Mohammed Schezan Iqbal1, Debraj Sen2, Prasenjeet Singh3, Anjan Prabhakara4
1 Department of Orthopaedics, INHS Kalyani, Visakapatnam, Andhra Pradesh, India
2 Department of Radiodiagnosis, Armed Forces Medical College (AFMC), Pune, Maharashtra, India
3 Department of Orthopaedics, Military Hospital Agra, Agra, Uttar Pradesh, India
4 Department of Orthopaedics, Military Hospital Kirkee, Pune, Maharashtra, India
|Date of Submission||02-Sep-2019|
|Date of Acceptance||23-May-2020|
|Date of Web Publication||07-Jul-2020|
Dr. Mohammed Schezan Iqbal
Department of Orthopaedics, INHS Kalyani, Visakapatnam, Andhra Pradesh,
Source of Support: None, Conflict of Interest: None
Chondromyxoid fibroma (CMF) is one of the rarest benign cartilaginous tumors, accounting for less than 0.5% of bone tumors and less than 2% of benign bone tumors. Common locations of occurrence include the metaphyseal region of the proximal tibia and distal femur. The diagnosis of CMF rests upon its typical histological appearance like a lobular arrangement of stellate-shaped cells in a chondroid or myxoid background. We present a rare case of CMF in a 19-year-old male involving the head–neck junction of the proximal femur, which was managed by extended curettage and bone cementing, with excellent results on final follow-up.
Keywords: Benign, chondromyxoid fibroma, extended curettage, femoral head
|How to cite this article:|
Iqbal MS, Sen D, Singh P, Prabhakara A. Chondromyxoid fibroma of the femoral head: A rare case and review of literature. J Curr Oncol 2020;3:47-50
| Introduction|| |
Hip pain in young individuals can have varied causes. These could be minor sprains and strains to more serious causes like fractures, osteonecrosis of the femoral head, infections, arthritis, and developmental abnormalities like developmental dysplasia of the hip and tumors. Primary bone tumors (benign or malignant) of the hip are an uncommon cause of hip pain in the young. Chondromyxoid fibroma is the rarest benign cartilaginous tumor constituting less than 0.5% of all bone tumors. Here we present a case report of a chondromyxoid fibroma of the femoral head in a young adult.
| Case Presentation|| |
A 19-year-old male soccer player presented to our center with a history of dull, aching pain in the left hip of one and a half years duration, and a limp of 6 months duration. The pain was insidious in onset and gradually progressive. It was aggravated by exertion and relieved by rest. There was no swelling or sinuses. There was tenderness over the femoral triangle. The movements around the hip were terminally painful and restricted alongwith a fixed abduction deformity of 10°. A 1-cm apparent lengthening was present with no true limb length discrepancy.
The patient had initially reported at a different center where he was managed conservatively with rest and analgesics. The initial hip radiograph was normal. A second radiograph performed after a year revealed a solitary, well-demarcated, radiolucent lesion at the left femoral head–neck junction [Figure 1]. Noncontrast computerized tomography showed a well-defined, expansile, lytic lesion measuring 1.0 × 1.0 cm in size [Figure 2]A and B. There was an associated cortical breach. On magnetic resonance imaging (MRI), an irregular, peripherally enhancing, thick-walled cystic and lytic lesion measuring 2.5 × 2.0 cm in size was seen with surrounding marrow edema. A small cortical breach was also noted. A small cyst (1.5 × 1 cm) was seen adjacent to the cortical breach, associated with mild effusion [Figure 3]. A presumptive diagnosis of tuberculosis of the left hip had been made and the patient advised empirical anti-tubercular therapy, which he refused.
|Figure 1: AP radiograph of the pelvis with both hips with arrow depicting site of lesion|
Click here to view
At our center, a CT-guided biopsy from the lesion was performed. Histopathology showed a predominantly chondroid tissue having benign chondrocytes. No malignant chondrocytes, binucleation, atypia, or sarcomatous change was noted. No increase in osteoclastic giant cells, necrosis, or calcification was seen [Figure 4]. A diagnosis of a benign chondromatous lesion was made. The patient underwent an extended curettage and bone cementing of the lesion via safe surgical dislocation of the hip. Histopathology revealed predominantly chondroid material having benign chondrocytes. Ill-defined islands of spindle to stellate-shaped cells in an abundant myxoid to chondroid stroma separated by a more cellular stroma rich in spindle to rounded cells with mild pleomorphism along with fragments of sclerotic bone were also seen. No giant cells or granulomas were present [Figure 5]. A diagnosis of chondromyxoid fibroma was made.,
Postoperative recovery was uneventful, and the patient returned to full weight bearing ambulation in 2 months. The range of hip movements was full and pain free. There was no recurrence of the tumor noted on follow-up radiography [Figure 6].
| Discussion|| |
Jaffe and Lichtenstein first described chondromyxoid fibromata in 1948, noting that this benign entity was expected to be confused with chondrosarcoma. Since their first description, sporadic case reports and series have been reported in the literature and this tumor is presumed to be rare, constituting less than 0.5% of all bone tumors and less than 2% of benign bone tumors.
This tumor most commonly occurs in young adults in the second and third decades of life. Clinical features are characterized by pain and swelling, most commonly noted in the proximal tibial metaphysis. Rarer locations include the vertebrae, scapula, ribs, and fibula. Although malignant transformation is extremely rare, this tumor is known to be locally aggressive.
Patients may be asymptomatic and the tumor may be an incidental finding on a routine radiograph. Symptomatic patients with femoral lesions present with hip pain, groin discomfort, buttock pain radiating into the thigh, or rarely low back ache. Most patients have localized tenderness over the femoral triangle and fixed coronal plane deformity in the affected hip in long-standing cases, with an apparent limb length discrepancy.
On plain radiography, the tumor appears as a radiolucent, osteolytic lesion with sharp, well-defined, sclerotic margins. There is no visible periosteal reaction or matrix calcification. On MRI, the lesion shows low signal intensity lesion on T1-weighted images and high signal intensity on T2-weighted images. Contrast-enhanced images demonstrate diffuse heterogeneous or peripheral nodular enhancement.
Diagnosis of CMF primarily depends on its typical histological appearance, which includes a lobular pattern with stellate-shaped cells in a chondroid or myxoid background with hypercellular peripheries and hypocellular centers. Osteoclast-like giant cells are usually present at the lobular peripheries. Giant cells at the periphery of the chondroid lobules with plump, hyperchromatic nuclei with nuclear atypia are characteristic of CMF. Similar features were seen in our case.
The treatment options for CMF include simple curettage, extended curettage with phenol or liquid nitrogen application, and en bloc resection with bone grafting. The overall tendency to recur after initial curettage is reported to be 27%. However, extended curettage with bone grafting or bone cement has a 7% rate of local recurrence.
In conclusion, chondromyxoid fibroma is a rare, benign bone neoplasm that must be considered in patients complaining of hip or buttock pain with a proximal femoral lytic lesion.
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.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Yalniz E, Alicioglu B, Yalcin O, Yilmaz B. Non specific magnetic resonance features of chondromyxoid fibroma of the iliac bone. J Buon 2007;12:407-9.
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.
Scaglietti O, Stringa G. Myxoma of bone in childhood. J Bone Joint Surg Am 1961;43:67-80.
Fletcher A, Mastragostino M. Radiological evaluation of a chondromyxoid fibroma. J Radiol Oncol 2017;1:54-9.
Dahlin DC, Wells AH, Henderson ED. Chondromyxoid fibroma of bone. J Bone Joint Surg Am 1953;35:831-4.
Gherlinzoni F, Rock M, Picci P. Chondromyxoid fibroma. The experience at the Instituto Ortopedico Rizzoli. J Bone Joint Surg Am 1983;65:198-204.
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]