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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 10  |  Issue : 2  |  Page : 122-125

Fibromyxoma in an elderly female: A case report


1 Department Oral and Maxillofacial Surgery, Baba Jaswant Singh Dental College Hospital and Research Institute, Ludhiana, Punjab, India
2 Department Oral and Maxillofacial Pathology, Baba Jaswant Singh Dental College Hospital and Research Institute, Ludhiana, Punjab, India
3 Department Oral Medicine and Radiology, YMT Dental College and Hospital, Mumbai, Maharashtra, India

Date of Submission16-Dec-2019
Date of Decision13-Jan-2020
Date of Acceptance28-Apr-2020
Date of Web Publication16-Feb-2021

Correspondence Address:
Dr. Paramjot Kaur
Department Oral and Maxillofacial Surgery, Baba Jaswant Singh Dental College Hospital and Research Institute, Ludhiana, Punjab, Sector 40 Chandigarh Road, Ludhiana 141 010, Punjab
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijohs.ijohs_49_19

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  Abstract 


Odontogenic fibromyxoma is a slow-growing, locally invasive, nonmetastasizing neoplasm found exclusively in the bones of the facial skeleton. Although benign, it is locally aggressive making it difficult to treat. It commonly occurs in the second and third decades, and the mandible is involved more commonly than the maxilla. The lesion often grows without symptoms and presents as a painless swelling. The radiographic features are variable, and the diagnosis is therefore not easy in many cases. This article presents a rare case of odontogenic fibromyxoma occurring in the posterior mandible of a 75-year-old female patient.

Keywords: Fibromyxoma, mesenchymal tumor, myxoma, odontogenic


How to cite this article:
Kaur P, Bansal H, Kulkarni M. Fibromyxoma in an elderly female: A case report. Int J Oral Health Sci 2020;10:122-5

How to cite this URL:
Kaur P, Bansal H, Kulkarni M. Fibromyxoma in an elderly female: A case report. Int J Oral Health Sci [serial online] 2020 [cited 2021 Apr 22];10:122-5. Available from: https://www.ijohsjournal.org/text.asp?2020/10/2/122/309454




  Introduction Top


Rudolph Virchow first used the term “myxoma” in 1863 to describe abdominal and soft-tissue lesions. It was Thoma and Goldman in 1947 who described it as “a rare benign tumor of the tooth-bearing areas of the jaw bone.” Fibromyxomas are benign tumors containing a large amount of mature fibroblasts and connective tissue. They are similar to myxomas, as both myxomas and fibromyxomas exhibit a proliferation of primitive mesenchymal cells that produce an amorphous mucoid-rich intercellular matrix.

Odontogenic fibromyxoma (also called odontogenic myxofibroma) is a benign odontogenic tumor accounting for 3%–8% of all odontogenic tumors and cysts of the jaws. They are derived from mesenchymal tissues that may contain odontogenic epithelium. Fibromyxomas in the head-and-neck region originate either from the soft tissues or from the facial skeleton, although peripheral fibromyxomas are exceedingly rare.

Fibromyxomas are benign but aggressive mesenchymal tumors that are slow growing, expansile, and locally destructive. They usually occur in the second–third decades of life, rarely in children or adults over 50 years of age. They occur commonly in women with a mean age of 31 years and have a slight predilection for the posterior mandible. Odontogenic myxoma is a type of fibromyxoma in which the myxomatous element predominates.[1],[2],[3],[4],[5],[6]

Clinically, the tumor usually presents as a slow-growing, painless swelling. However, patients may be symptomatic and may present with pain and paresthesia in the advanced stages. Large lesions may lead to facial asymmetry. Displacement and mobility of teeth are relatively common. It may be associated with unerupted teeth. Cortical expansion can occur and large lesions can cause perforation. Radiographically, Odontogenic Fibromyxoma most commonly presents a unilocular or multilocular, well-defined radiolucency.

In this article, we report a rare case of an odontogenic fibromyxoma in a 75-year-old female patient diagnosed with diabetes mellitus and hypertension. We will review the clinical presentation, radiographic examination, histological features, differential diagnosis, and management of this rare neoplasm.


  Case Report Top


A 75-year-old female patient reported to our department with the chief complaint of a painless swelling in the right posterior region of the lower jaw for 3 years. Prior to history taking she was asked to sign a consent form. She was apparently asymptomatic 3 years back when she noticed facial asymmetry due to a small asymptomatic swelling in the lower right posterior region of the jaw. The swelling was initially small in size which increased at an alarming rate to involve the entire right side of the lower jaw. Her mouth opening was restricted due to which she experienced discomfort while eating her food. She also complained of paresthesia on the right side of the face. She was diabetic and hypertensive and was on medication for the same

Extraoral examination revealed the presence of a large solitary swelling in the lower right anterior and posterior region of the jaw measuring approximately 10 cm × 10.5 cm extending anteroposteriorly from the right commissure of the lip to the angle of the mandible and superoinferiorly from a horizontal line joining the right ala of the nose and pinna of the ear, 5 cm below the inferior border of the mandible. On palpation, the swelling was asymptomatic, afebrile, and firm to hard in consistency [Figure 1].
Figure 1: An expansile, painless, extraoral swelling involving the body and angle of the mandible in the lower right posterior region of the jaw

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Intraoral examination revealed an ill – defined exophytic, proliferative growth having irregular shape covering the alveolar ridge from the region of 43 extending posteriorly and causing lower right buccal vestibular obliteration and superiorly covering the crowns of the lower right posterior teeth. Overlying surface was erythematous with blackish discoloration and showed indentation marks. The mass was nontender and firm on palpation [Figure 2]. There was mild bleeding on probing. Based on the history and clinical features a provisional diagnosis of a benign odontogenic lesion was given. Malignancy was considered as a clinical differential diagnosis.
Figure 2: Exophytic soft-tissue mass involving the lower right vestibule and alveolus

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Radiographic investigations

Orthopantomogram

Orthopantomogram revealed a solitary, well-defined, multilocular radiolucent lesion in the lower right jaw involving the body and angle of the mandible, measuring approximately 8 cm × 6 cm extending antero-posteriorly from the distal of 43 to the distal of 47. The lesion had non-corticated borders. Internal structure showed the presence of linear radio-opaque septae arranged in a radiating pattern giving the “sunburst” appearance [Figure 3].
Figure 3: Orthopantomogram showing a well-defined multilocular radiolucency having radio-opaque septae arranged in a radiating pattern giving it the sunburst appearance

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Contrast-enhanced computed tomography (CECT)

Contrast-enhanced computed tomography revealed a well-defined osteolytic lesion involving the right body and angle of the mandible measuring approximately 8.5 cm × 4 cm in size. The internal structure of the lesion showed spiculated “frond like projections” having a large soft-tissue component in the right cheek. The internal trabecular pattern was described in 1980 by Eversole as lichen planus of the jaw bone.[4],[5] The fat planes between the lesion and surrounding fat and muscular structures were normal. No enlargement of the cervical lymph nodes was noted. Base of the tongue, floor of the mouth, sphenoid and maxillary sinuses, cerebellar hemispheres, nasopharynx, soft tissues of the neck, angle of the mandible, base of the skull, bilateral mastoid air cells, bilateral parotid glands, larynx, and vocal cords were normal. No areas of abnormal attenuation were seen in the thyroid gland. Major vessels of the neck appeared normal [Figure 4].
Figure 4: CECT image revealing a well-defined osteolytic lesion involving the right body and angle of the mandible showing spiculated frond like projections

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Lesions such as osteosarcoma, odontogenic myxoma and odontogenic fibromyxoma were considered as radiographic differential diagnosis.

Fine-needle aspiration cytology was done which revealed a serosanguinous aspirate which was suggestive of hemorrhage.

Intraoral incisional biopsy was taken from the buccal and vestibular mucosa, retromolar region, and alveolar mucoperiosteum of the same side of the mouth.

Histopathological examination revealed randomly arranged stellate, oval or spindle-shaped cells in a myxoid stroma along with variable amount of myofibroblasts and collagen fibres. These features were suggestive of an odontogenic fibromyxoma [Figure 5].
Figure 5: Photomicrograph showing randomly arranged stellate, oval or spindle-shaped cells in a myxoid stroma along with variable amount of myofibroblasts and collagen fibres

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Based on the histopathological report, immunohistochemistry for smooth muscle antibody (SMA)was advised which was SMA positive. The tumor cells in this lesion were reactive with antibodies directed against smooth muscle actin that ruled out desmoplastic fibromyxoma which is of myofibroblastic origin. Presence of stromal myofibroblasts confirmed the diagnosis as of an odontogenic fibromyxoma.

Hemimandibulectomy of the involved region of the mandible was advised. Excisional biopsy could not be performed as the patient did not report back for treatment.


  Discussion Top


Fibromyxomas are a rare variety of odontogenic tumours which are benign, intraosseous, and locally aggressive. They most commonly affect the facial skeleton and are restricted to teeth-bearing areas.

Thoma and Goldman in 1947 first described this rare neoplasm. According to the 1992 WHO Classification, the term is used along with odontogenic myxoma and myxofibroma. These are two forms of fibromyxomas recognized in the head-and-neck region derived from the facial skeleton and the facial soft tissues, respectively. Most Odontogenic Fibromyxomas (OF) are located intraosseously; however, rare peripheral occurrences in the long bones have been reported. These peripheral lesions demonstrate a higher tendency of recurrence and malignant transformation. In the pathogenesis of Odontogenic Fibromyxoma, dental papilla, dental follicle, and periodontal ligament tissue have been implicated as possible germ centers. Initial lesions are relatively small in size; however, they can progress rapidly into advanced lesions which may involve the entire jaw bone on the affected side associated with pain and paresthesia.

Myxomas can occur in the molar and premolar regions of the mandible and maxilla with 2:1 predilection in the mandible with cortical expansion and perforation.[1] In the present case, the evidence for its odontogenic origin arises from its location in the tooth-bearing areas of the mandible, its association with missing or unerupted 46 and 48, and the presence of odontogenic epithelium.[2]

In the present case report, the patient presented in the seventh decade of life,[2] unlike most cases which present in the fourth decade. The lesion was seen as an advanced, large, intraosseous growth with a proliferative soft-tissue component in the posterior mandible with associated pain and paresthesia.

Radiographically odontogenic fibromyxoma presents as a well-defined multilocular radioluscency with sclerotic borders. Internal structure usually shows curved and straight septae within the radiolucency giving it a multilocular appearance. In rare cases the septae maybe arranged in a radiating pattern giving the sunburst appearance very similar to that seen in osteosarcoma. Root resorption is a common feature along with expansion and perforation of buccal and lingual cortical plates. In the present case, the lesion was a well-defined multilocular one having non-corticated borders with presence of linear septae arranged in radiating pattern giving the “sunburst” appearance. Hence, radiographic differential diagnosis of osteosarcoma was considered.

Histopathologically, odontogenic fibromyxoma is marked by randomly arranged stellate, oval or spindle-shaped cells in a myxoid stroma along with variable amount of myofibroblasts and collagen fibres which were seen in the present case too.

Myofibroblasts (MFs) are fibroblasts with smooth muscle-like features characterized by the presence of a contractile apparatus. Normally, they are found in healing wounds, lymph nodes, blood vessels, uterine submucosa, intestinal villous core and lung septa and in pathologic conditions (reactive lesions, benign tumors, locally aggressive (borderline) fibromatoses and sarcomas showing MF differentiation). Stromal MFs have the potential to facilitate progression of neoplastic epithelial lesions that could contribute to their biological behavior. Most of the MFs express alpha-smooth muscle actin (α-SMA), which is the actin isoform typically found in vascular smooth muscle cells and coordinately regulated by TGF-β1. In the present case immunohistochemistry for smooth muscle antibody (SMA)was advised which was SMA positive

Treatment

These tumors are radioresistant, and hence, radiotherapy is never a choice of treatment. The rate of recurrences is attributed to the ability of the tumor to infiltrate into the surrounding bone. Incomplete removal of tumor is responsible for recurrence more than the biological behavior. Lesions of size >3 cm are considered for radical resections and bloc resections and tumors of lesser diameter are better treated by enucleation or curettage to preserve vital structures, maintain oral function, and to avoid recurrence.[2],[4] Literature suggests recurrence even after resection of large lesions. Thus, consideration was given to the benign nature of this asymptomatic, bulky painless growth that was causing facial disfigurement in a 75-year diabetic female. Our patient was not interested in hemimandibulectomy. She has been advised a periodic follow-up.[4]

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

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Brannon RB. Central odontogenic fibroma, myxoma (odontogenic myxoma, fibromyxoma), and central odontogenic granular cell tumor. Oral Maxillofac Surg Clin North Am 2004;16:359-74.  Back to cited text no. 1
    
2.
Joshi PS, Majati S, Hongal B, Agnihotri N. Fibromyxomas of the jaw. Clin Cancer Investig J 2015;4:737-40.  Back to cited text no. 2
  [Full text]  
3.
Godishala Swamy SR, Naag S, Bahl S, Priyadarshini E. Odontogenic myxoma: A causality dilemma – Report of a nonpareil case and review of literature. J Oral Maxillofac Pathol 2018;22:S2-6.  Back to cited text no. 3
    
4.
Kawase-Koga Y, Saijo H, Hoshi K, Takato T, Mori Y. Surgical management of odontogenic myxoma: A case report and review of the literature. BMC Res Notes 2014;7:214.  Back to cited text no. 4
    
5.
Kheir E, Stephen L, Nortje C, van Rensburg LJ, Titinchi F. The imaging characteristics of odontogenic myxoma and a comparison of three different imaging modalities. Oral Surg Oral Med Oral Pathol Oral Radiol 2013;116:492-502.  Back to cited text no. 5
    
6.
Alhousami T, Sabharwal A, Gupta S, Aguirre A, Park E, Kramer JM. Fibromyxoma of the Jaw: Case Report and Review of the Literature. Head Neck Pathol. 2018;12:44-51. doi:10.1007/s12105-017-0823-0.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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