|Year : 2023 | Volume
| Issue : 5 | Page : 104-108
Rapidly expanding chondromyxoid fibroma of the mandible: A case report of rare entity
Gargi Jadaun, Himanshu Gupta, Sayeeda Kharodia, Viral Gadhiya
Department of Oral and Maxillofacial Surgery, Pacific Dental College and Hospital, Udaipur, Rajasthan, India
|Date of Submission||26-Sep-2021|
|Date of Acceptance||04-Jan-2023|
|Date of Web Publication||04-Feb-2023|
Senior Lecturer, Department of Oral and Maxillofacial Surgery Pacific Dental College and Hospital, Udaipur - 313 024, Rajasthan
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Chondromyxoid fibroma is an unusual, benign bone tumour that is usually sited in the metaphyseal region of the long bones. It accounts for less than 1% of all bone tumours. It manifests predominantly in males in their second and third decades of life. Rarely, it occurs in the bones of the craniofacial skeleton. For small, lesions enucleation and curettage and for larger lesions, resection followed by are the treatment modalities available. Here, we present a case of chondromyxoid fibroma with respect to the right side of the mandible which was managed by surgical resection followed by reconstruction under general anaesthesia. Resection followed by reconstruction provides satisfactory outcomes, especially in cases with large lesions. Chondromyxoid fibroma is an asymptomatic, benign, slow-growing lesion but can rapidly expand and involve the greater area of bone. Thus, its detection at an early stage and treatment at the proper time can lead to less morbidity associated with the lesion and improved quality of life of the patient.
Keywords: Chondromyxoid fibroma, male, mandible, resection, surgical, tumour
|How to cite this article:|
Jadaun G, Gupta H, Kharodia S, Gadhiya V. Rapidly expanding chondromyxoid fibroma of the mandible: A case report of rare entity. J Oral Maxillofac Pathol 2023;27, Suppl S1:104-8
|How to cite this URL:|
Jadaun G, Gupta H, Kharodia S, Gadhiya V. Rapidly expanding chondromyxoid fibroma of the mandible: A case report of rare entity. J Oral Maxillofac Pathol [serial online] 2023 [cited 2023 Mar 21];27, Suppl S1:104-8. Available from: https://www.jomfp.in/text.asp?2023/27/5/104/369178
| Introduction|| |
Jaffe and Lichtenstein in 1948 were the first to describe chondromyxoid fibroma, a rare benign tumour of the bone. The World Health Organization (WHO) defines it as 'A benign tumour characterized by lobulated areas of spindle shaped or stellate cells with abundant myxoid or chondroid intercellular material separated by zones of more cellular tissue rich in spindle shaped or round cells with a varying number of multinucleated giant cells of different sizes.'
The present study reports a clinical case of extensive chondromyxoid fibroma involving the coronoid process and condyle on the right side of the mandible that was treated by marginal resection.
| Case Report|| |
A 13-year-old boy reported to our department with a chief complaint of swelling on the right side of the face since seven months. On clinical examination, swelling was diffuse. It had gradually increased to the present size and was not preceded by any trauma or toothache in that region. The swelling was asymptomatic; there was no history of pain or pus discharge. Past dental history and family history were negative. Routine blood investigations were advised to the patient and were within the normal limits.
On extraoral examination, a solitary well-defined swelling that was firm in consistency, non-tender and fixed, and approximately 15 × 12 cm in size, extending anteriorly from the left parasymphysis region to the right angle of the mandible posteriorly and superiorly from the right infraorbital margin to inferiorly involving the inferior border of the mandible was detected [Figure 1]. There was no lymphadenopathy. Mouth opening of the patient was 50 mm in size. No abnormalities were detected with respect to the temporomandibular joint (TMJ) bilaterally. Intraorally, the swelling extended from the right premolars to posteriorly up to the right condylar head. There was obliteration of the right buccal vestibule [Figure 2] and [Figure 3]. There was complete loss of sensation on the right side of the jaw. An orthopantomogram (OPG) was obtained, which revealed a well-defined unilocular lesion of 15 × 12 cm in size extending from the right first premolar to the subcondylar region on the right side [Figure 4]. There was displacement of all the teeth from 44 to 47. Inferior alveolar neurovascular bundles were also displaced up to the inferior border of the mandible. Computed tomography (CT) of the face was advised to establish the extent of the lesion, position of the neurovascular bundles, and to determine the status of bone. Contrast-enhanced computed tomography (CECT) scan revealed a unilocular thin-walled cystic lesion measuring 15–19 HU involving the right parasymphysis region and extending up to the right condylar head with thinning of the bone [Figure 5] and [Figure 6]. On the basis of clinical and radiological investigations, differential diagnoses of ameloblastoma, cementoblastoma, chondromyxoid fibroma, calcifying epithelial odontogenic tumour (CEOT), and ossifying fibroma were made.
|Figure 2: intraoral picture with obliteration of buccal vestibule on right side|
Click here to view
We subsequently proceeded with the provisional diagnosis of myxoid fibroma. Aspiration was negative. An incisional biopsy was performed from the right mandibular second premolar to the right canine region, and the specimen was sent for histopathological evaluation. Histopathological evaluation revealed various mesenchymal components like mature cartilage myxoid and fibrous tissue. There was no evidence of dysplastic/malignant changes or no ameloblastic epithelium were present. These findings were confirmatory of chondromyxoid fibroma.
After the final diagnosis of chondromyxoid fibroma, resection of the affected side of the mandible and placement of reconstruction plate under GA was the decided treatment plan. All required investigations were carried out and the surgery was planned.
Under general anaesthesia, the lesion was approached by an extraoral Risdon incision extending from the right angle of the mandible to the left parasymphysis region using electro-cautery. Full thickness flap was raised, dissection was carried out, and the tumour was exposed. The whole tumour was resected with around 5 mm of healthy margin. Hemi-mandibulectomy was performed followed by placement of reconstruction plate of size 2.4 mm and four screws of 10-mm length. Closure was done using 3-0 vicryl suture and staples [Figure 7]. The excised specimen was sent for the confirmation of histopathological diagnosis [Figure 8].
The histopathology evaluation revealed mesenchymal components like mature cartilage, myxoid, and fibrous tissue in the specimen which confirmed the diagnosis of chondromyxoid fibroma.
Postoperative period was uneventful and no recurrence has been noted on follow-up visits of up to two years [Figure 9].
| Discussion|| |
Chondromyxoid fibroma (CMF) is a benign chondroid and myxoid matrix-producing tumour that is sometimes seen in coalition with fibrous areas and is believed to be of cartilaginous origin. It represents less than 1% of all bone tumours and 2% of benign bone tumours.
The aetiology of CMF is unknown. According to Smith et al., 11 out of 14 of his subjects had non-random, clonal abnormalities of chromosome 6 involving band 6p25q13, which had not been associated with other bone tumours. Chromosome 6 has been involved in normal cartilaginous development, carrying genes BMP6, COL9A1, COL10A1, and IGF2. So non-random clonal abnormalities in chromosome 6 can be a cause for CMF.
CMF is most commonly found in the metaphysis around the knee in the proximal tibia, proximal fibula, or distal femur, and rarely in the small bones of the hands and feet. Twenty-five percent of cases occur in flat bones, including the skull and facial bones. The bone tumours in the craniofacial region are extremely rare: approximately 2% of cases involve a higher incidence of mandible (76%) than the maxilla (24%). CMF of the maxillofacial region is typically seen in patients in the second to third decade of life. The male-to-female ratio is 2:1, which is similar to our case.
According to Semnic et al., the lesion is always greater than 5 cm in diameter at presentation. In our case, the lesion was 10 × 10 cm in size. The clinical course ranges according to the area involved and is generally associated with a persistent history of non-specific symptoms, mainly pain and oedema; in our case, the patient had no history of pain. In most cases, CMF is slow-growing and has been observed on routine radiography for causes not directly linked to the symptoms caused by these lesions. However in our case, the lesion was rapidly growing and attained the present size in just seven months.
Conventional radiographs do not give much insight into the lesion. The CT findings of CMFs suggest that they have a lobulated outline with sharp margins and a sclerotic rim. The cortex of the bone is usually thin and expanded. One third of cases show radiographic evidence of soft-tissue extension. Most of the tumours are purely radiolucent. However, approximately 13% of tumours show some discrete areas of calcification. It manifests low signal intensity on T2-weighted images due to chondroid and myxoid tissue with an inhomogeneous pattern of enhancement. In our case, the lesion presented as a well-circumscribed, spherical, radiolucent mass with buccal cortical expansion and perforation.
Histopathologically, the tumour consists of lobulated or pseudo-lobulated regions of spindle-shaped or stellate cells with no distinct cytoplasmic boundary and abundant myxoid or often chondroid intercellular material separated by bands of more cellular tissue, rich in spindle-shaped or circular cells and entangled with a range of multinucleated giant cells of various sizes. These features were seen in our histology report that consisted of mature cartilage, myxoid, and fibrous tissue.
The differential diagnosis includes chondrosarcoma, chondroblastoma, giant cell tumour, aneurysmal bone cyst, non-ossifying fibroma, and enchondroma., Chondrosarcoma can be differentiated from CMF by the presence of mitosis, nuclear atypia, and an invasive growth pattern; the two are otherwise histologically very similar lesions.
Depending on the size of the lesion, two main treatment options are available, namely, curettage and en bloc resection, both of which have shown satisfactory outcomes. The tendency to recur after curettage was higher, especially in young children. Recurrence rate decreased when the lesion was treated with en bloc excision and bone grafting. Thus, the definitive management of a large lesion is en bloc surgical resection. In our case as well, since the lesion was extensive and there was expansion of the buccal cortical plate, resection followed by placement of reconstruction plate under general anaesthesia was the treatment plan carried out. The postoperative period was uneventful and no recurrence has been noted on two years of follow-up visits.
| Conclusion|| |
Chondromyxoid fibroma is an asymptomatic, benign, slow-growing lesion but can rapidly expand and involve the greater area of bone. Thus, the detection of the lesion at an early stage and its treatment at the proper time can lead to less morbidity associated with the lesion and improved quality of life of the patient.
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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], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9]