Case Report
Radiological Evaluation of a Chondromyxoid Fibroma
Austin Fletcher* and Megan Mastragostino
School of Medicine, Saint James School of Medicine, Anguilla Campus, USA
*Corresponding author: Austin Fletcher, Saint James School of Medicine, Anguilla Campus, USA
Published: 08 Sep, 2017
Cite this article as: Fletcher A, Mastragostino M.
Radiological Evaluation of a
Chondromyxoid Fibroma. Clin Oncol.
2017; 2: 1340.
Abstract
Chondromyxoid fibroma (CMF) is a very rare benign cartilaginous tumor representing less than
0.5% of all bone tumors while also being the rarest cartilaginous bone tumor. Common locations
of occurrence include the metaphysical region of the proximal tibia and distal femur. We report
a case of a 10-year-old female affected by a CMF of the left lower tibia. The radiological features
demonstrated by X-ray and Magnetic Resonance Imaging (MRI) are discussed.
Keywords: Chondromyxoid fibroma; Benign; Curettage; Bone graft; Autologous
Introduction
Chondromyxoid fibroma is a very rare benign cartilaginous tumor representing less than 0.5% of all bone tumors while also being the rarest cartilaginous bone tumor [1-5]. The most commonly affected area is the metaphysial region of the proximal tibia and the distal femur [6,7]. Rarer locations include the fibula, scapula, vertebrae and ribs [2]. The tumor primarily has a male sex distribution, usually presenting before age 30 [7]. We present a case of a CMF occurring in the distal tibia, differing from the most common presentation of the proximal tibia.
Case Report
A 10-year-old female presented with left lower leg pain that had progressively increased over the duration of 1 year. On physical examination, tenderness was elicited on palpation of the distal tibia. Routine laboratory testing was normal. X-Ray was performed and revealed a lytic lesion with sclerotic margins in the left lower tibia (Figure 1). MRI was subsequently performed, demonstrating a left lower tibia lesion with low signal intensity on T1 weighted images (Figure 2), and high signal intensity on T2 weighted images (Figure 3). Pathology of the tumor revealed a diagnosis of a CMF. She was treated with curettage and autologous bone grafting (Figure 4). Post operatively the left leg was immobilized with a plaster cast with restricted weight bearing (Figure 5). Follow up X-ray after 5 years demonstrates no tumor reoccurrence (Figure 6 and 7).
Figure 1
Figure 2
Figure 3
Figure 4
Figure 5
Discussion
CMF is the rarest benign cartilaginous bone tumor [5]. Although
benign the tumor is locally aggressive in nature [7]. Malignant
transformation is very rare [2]. The tumor occurs most commonly
in young adults and adolescents in the second and third decade of
life, with an age range between the ages of 10 to 30 [1,6]. However,
cases have been previously reported outside this range. Clinical
presentation is characterized by pain, swelling, and edema; most
commonly noted in the proximal metaphysis of the tibia [1]. The
proximal femur is also commonly affected [2]. Rarer locations
include the fibula, scapula, vertebrae and ribs [2]. Occasionally
clinical presentation may be absent and the tumor may be discovered
incidentally during radiography [1]. Tumors located in the distal
extremities such as the hands or feet may only present clinically with
signs of painless swelling [8]. Males have a higher incidence, with no
other demographic bias [2].
Imaging is crucial to the diagnosis of this tumor. On X-ray the
tumor appears as a space occupying radiolucent osteolytic lesion with
sharp and well-defined margins (Figure 1). The margins are noted
to be sclerotic (Figure 2) The tumor has no soft tissue component
(Figure 8). There is no matrix calcification or visible periosteal
reaction (Figure 4).
MRI demonstrates low signal intensity on T1 weighted images
(Figure 9). T2 weighted images demonstrate high signal intensity
(Figure 10 and 11). Post gadolinium contrast T1 weighted sagittal
view demonstrates diffuse heterogenous enhancement (Figure 12).
Post gadolinium contrast T1 weighted axial view demonstrates
peripheral nodular enhancement (Figure 13).
Treatment consists of curettage or en-bloc resection [3]. Curettage
alone has a higher recurrence rate compared to curettage combined
with bone grafting [3]. Recurrence rates as noted by Gherlinzoni F
et al. [6]. were noted to be as high as 80% with curettage alone and
dropping to 7% when combined with bone grafting. Long bone lesions
are preferably treated with either en bloc or curettage combined with
bone grafting as in our case.
Figure 6
Figure 7
Figure 8
Figure 9
Figure 10
Figure 11
Figure 12
Figure 13
Figure 14
Figure 15
Conclusion
Chondromyxoid fibromarepresents less than 0.5% of all bone tumors making it significantly rare as well as the rarest cartilaginous bone tumor. Common locations of occurrence include the metaphysical region of the proximal tibia and distal femur. We described a case of a 10-year-old female affected by a CMF of the left lower tibia which was treated with curettage and bone grafting. X-ray demonstrates a space occupying radiolucent osteolytic lesion with sharp and well defined sclerotic margins. MRI of the tumor revealed low signal intensity on T2 weighted images. T2 weighted images demonstrate high signal intensity. Post gadolinium contrast T1 weighted images demonstrated heterogenous enhancement. Follow up X-ray after 5 years demonstrates no tumor reoccurrence (Figure 14 and 15).
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