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 Table of Contents  
CASE REPORT
Year : 2016  |  Volume : 28  |  Issue : 4  |  Page : 465-469

Odontogenic myxoma: An archetypal presentation of a rare entity


Department of Oral Medicine and Radiology, Government Dental College, Thiruvananthapuram, Kerala, India

Date of Submission08-Mar-2016
Date of Acceptance30-Jan-2017
Date of Web Publication21-Feb-2017

Correspondence Address:
Dr. Anupama I Varun
Department of Oral Medicine and Radiology, Government Dental College, Thiruvananthapuram, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaomr.JIAOMR_25_16

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   Abstract 

Odontogenic myxoma is a benign, locally aggressive/destructive neoplasm arising from the mesenchymal portion of tooth germ, dental papilla, follicle, or periodontal ligament with an exclusive presentation in the tooth-bearing areas of the jaws. They usually present as an asymptomatic swelling in the second or third decade of life. This case report brings to the fore a classical presentation of odontogenic myxoma, with a brief review on its diverse features and diagnostic dilemmas.

Keywords: Benign, myxoma, odontogenic tumor


How to cite this article:
Varun AI, Ramachandran S, Rajasekharan A, Balan A. Odontogenic myxoma: An archetypal presentation of a rare entity. J Indian Acad Oral Med Radiol 2016;28:465-9

How to cite this URL:
Varun AI, Ramachandran S, Rajasekharan A, Balan A. Odontogenic myxoma: An archetypal presentation of a rare entity. J Indian Acad Oral Med Radiol [serial online] 2016 [cited 2021 Jan 18];28:465-9. Available from: https://www.jiaomr.in/text.asp?2016/28/4/465/200636


   Introduction Top


Odontogenic myxoma is a benign, unencapsulated head and neck tumor, which accounts for 3–6% of all odontogenic neoplasms.[1] The reported incidence and demographic data uncover wide variability in the clinical and radiological presentation of this tumor. It is an uncommon entity, and many a time the tumor makes it to a sizeable swelling before the patient seeks medical care. Because of the classic radiographic internal configuration, Eversole called it as “lichen planus of the jaw bone.”[2] According to the histologic classification of the World Health Organization, odontogenic myxoma originates from mesenchyme and/or odontogenic ectomesenchyme with or without odontogenic epithelium.[3]


   Case Report Top


A 35-year-old female patient reported to the Oral Medicine and Radiology outpatient department complaining of a swelling on the right lower gum for the past 4 years [Figure 1]. Patient gave a history of restoration of the right lower back tooth 5 years back. A round swelling of approximately 1 × 1 cm developed on the gum corresponding to the restored tooth 1 year after the procedure, which gradually enlarged to the present dimensions. No history of pain/paraesthesia was reported. Patient was under treatment for hypertension and nephrosclerosis.
Figure 1: Extraoral view

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Extraoral examination revealed a well-defined swelling on the right parasymphyseal region with normal overlying skin. Single submandibular lymph node measuring approximately 1 × 0.5 cm, which was mobile and nontender on palpation, was present on the right side. Intraorally, it was a well-defined swelling measuring approximately 3.5 × 3 cm with bosselated surface and normal overlying mucosa from 42 to 46 region involving buccal gingiva and vestibule [Figure 2]. It was a hard, nontender swelling with buccal and lingual cortical expansion [Figure 3]. Mandibular right second premolar had a fractured permanent restoration and was tender on percussion. Associated teeth (33 to 47) were vital on electrical pulp testing.
Figure 2: Intraoral view – swelling on the gingivo-buccal vestibule

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Figure 3: Intraoral view – mild lingual cortical expansion

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A provisional diagnosis of odontogenic tumor/fibrosseous lesion was made. Panoramic radiograph suggested a multilocular hazy radiolucency from 42 to 46 region with internal locules and poorly-defined margins. The intralesional trabeculations intersected at right angles imparting a honey comb pattern [Figure 4]a and [Figure 4]b. Intraoral periapical radiographs (IOPARs) showed a few straight, thin, sharp septae, and displacement of 44 [Figure 5]a and [Figure 5]b. Expansion of the mandible with thinning of the cortical plates and extension of the septa beyond the peripheral margin of the lesion at right angles imparting hairbrush/sunburst pattern was evident in the mandibular occlusal radiograph [Figure 6].
Figure 4: (a) Cropped panoramic view showing honeycomb pattern; (b) panoramic view

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Figure 5: (a) IOPAR showing hazy radiolucency with few straight septae; (b) IOPAR 43 region

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Figure 6: Occlusal view showing fish bone or hair brush pattern

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Computed tomography (CT) images of the mandible showed an expansile osteolytic lesion with a few straight internal septa and focal destruction of inner and outer cortices [Figure 7]. Radiographic differential diagnoses considered were odontogenic myxoma, ameloblastoma, central giant cell granuloma, and aneurysmal bone cyst. An incisional biopsy was done. Histopathology specimen showed moderately collagenous connective tissue stroma interspersed with bony trabeculae. Amid the bony trabeculae, the connective tissue was delicately collagenous with moderate cellularity (stellate cells) giving a myxomatous appearance. Within the connective tissue clustered odontogenic epithelial cells, osteoblast-lined bony trabeculae and osteocytic lacunae were seen [Figure 8]a and [Figure 8]b. All these features were supportive to conclude the final diagnosis as odontogenic myxoma. Surgical resection of the lesion with a safety margin of 1.5 cm followed by reconstruction was suggested as the treatment.
Figure 7: CT showing osteolytic lesion with few internal septae

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Figure 8: (a and b) Histopathological views

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   Discussion Top


Odontogenic myxoma was termed initially as “myxofibroma” by Rudolf Virchow in 1863 owing to its histologic similarity to the mucinous substance present in the umbilical cord. It was renamed as “odontogenic myxoma” later in 1947 by Thoma and Goldman.[4] The reported prevalence is 0.04–3.7%, with a slightly higher frequency (0.5–17.7%) in Asia, Europe and America. The penchant age group affected is 22.7 to 36.9 years, with a distinct female predilection. It is rarely seen in patients younger than 10 years and older than 50 years of age.[5] Mandible is frequently affected than maxilla and commonly involves premolar and molar areas.[6]

Odontogenic myxoma generally presents as an asymmetry of the jaw from a painless, slow growing lesion with possible loosening or displacement of teeth and cortical expansion. It is occasionally associated with missing or unerupted teeth.[5] Ulceration of the overlying oral mucosa occurs only when it interferes with dental occlusion. Rapid growth and infiltration into neighbouring soft tissue may occur. Expansion of the jaws in 74% of the cases has been reported by Kaffe et al.[7]

Only a few reviews with remarkable number of cases have been reported including 60 cases by Mosquida-Taylor et al. (1997), 64 cases by Lu et al. (1998), 33 cases by Simon et al. (2004), 25 cases by Li et al., and 30 cases by Claudia et al. (2007).[8] Most of these authors supported the occurrence in young adults and female predilection. Common anatomic site reported was mandible followed by maxilla except for Regezi et al. (1978) who documented an equal distribution. The characteristic features of the case reported here comply with the results registered in literature.

Substantiative evidence of the origin of this tumor from the mesenchyme of a developing tooth or the periodontal ligament include (1) almost exclusive occurrence in the tooth-bearing areas of the jaws, (2) occasional association with an unerupted or a missing tooth, (3) incidence in younger individuals, (4) histologic resemblance to dental mesenchyme (i.e., dental papilla, follicle or periodontal ligament), and (5) sporadic presence of odontogenic epithelial islands.[6] Two types of odontogenic myxoma are identified, tumors that arise exclusively in jawbones (the most common) and those derived from perioral soft tissues.[9]

Radiographically, it may display unilocular or multilocular radiolucency with well-defined corticated, well-defined noncorticated, poorly defined, or diffuse margins.[5] It is mostly seen in the premolar-molar areas of mandible and rarely in the condyle and ramus. Unilocular and multilocular variants are mostly located in the anterior and posterior jaw respectively.[3] Barros et al. described two internal structural patterns in the evolution of odontogenic myxoma.[10] In the first stage, an osteoporotic appearance is produced by prominent medullary spaces separated by remodelled residual bone. This is manifested as multilocular radiolucency with triangular, diamond, square, or rectangular locules described as soap bubble/honey comb pattern. The elongated, straight, thin, and lacy trabeculae intersect at a right angle, and many of them tend to form more angular locules resembling strings of a tennis racket or a step ladder. Worth was the first person to signify the term tennis racquet to the multilocular internal pattern.[11] The second stage (breakout/destructive) is marked with loss of internal locules, significant expansion, perforation of cortex and invasion. Appearance of septa beyond peripheral margin of the lesion provides hair brush/sunburst/fish bone pattern in some cases, which represents bony septa being carried into soft tissues with the tumor mass.[10] The case report presented here showed multilocular pattern with poorly-defined margins.

According to Peltola et al., root resorption is rare.[12] Odontogenic myxoma frequently scallops between the roots of adjacent teeth and tends to grow along the involved bone without same amount of expansion seen in other benign odontogenic tumors. They rarely cross the midline.[13] Imaging modalities such as CT and magnetic resonance imaging (MRI) (high tissue signal on T2 weighted MR images is characteristic) aid in establishing the intraosseous extent of the tumor, cortical perforation, soft tissue involvement, and accurate resection margins.[14]

Histopathology specimen typically shows loosely arranged spindle-shaped and stellate cells with long fibrillar processes that tend to intermesh in an abundant mucoid stroma. Presence of relatively large quantities of hyaluronic acid in the ground substance has significance in explaining its neoplastic behavior. Occasionally, the tumor may be interspersed with a variable number of tiny capillaries and strands of collagen. Sporadic presence of odontogenic epithelium remnants surrounded by a narrow zone of hyalinization is also noted.[14],[15]

Numerous lesions are considered as differential diagnosis of odontogenic myxoma such as central giant cell granuloma, central hemangioma, ameloblastoma, osteosarcoma, metastatic carcinoma, cherubism, giant cell lesion of hyperparathyroidism, and aneurysmal bone cyst. Absence of fine trabeculations within the locules and presence of destruction of mandibular angle differentiates ameloblastoma from odontogenic myxoma. Giant cell granulomas prefer a more anterior location and fine trabeculations are not seen within the lesions. If there is no history of renal disease and serum chemistry is normal, giant cell lesion of hyperparathyroidism can be ruled out from the differential diagnosis of odontogenic myxoma. Cherubism comes to clinical attention at an early age group and shows bilateral involvement in contrast to myxoma. Aneurysmal bone cyst is often associated with symptoms such as pain. Central hemangioma can be eliminated because aspiration of a myxoma is nonproductive. The possibility of a metastatic tumor should always be considered, especially in older patients though a primary tumor helps in differentiation. Area of bony expansion shows thin but intact outer cortex in odontogenic myxoma, while the cortex is lost in osteosarcoma.[2],[8],[11]

An exact diagnosis is necessary to plan appropriate surgical and adjuvant therapy. Recommended therapy can vary from a conservative curettage (with or without cautery) to resection with generous amount of surrounding bone to ensure removal of myxomatous tumor that infiltrates the adjacent marrow spaces.[11] Surgical specimen appears as a pale brownish gelatinous substance (tender coconut appearance), which is considered pathognomonic. Recurrence rates ranging from 10 to 33% have been reported in cases treated with simple excision and curettage.[3] Insidious local invasion into cancellous bone beyond the radiographically visible margins and absence of encapsulation are considered as reasons for the high recurrence.[11]


   Conclusion Top


Because of the provincially aggressive biologic nature, it is imperative to look into each and every report on odontogenic myxoma. This benign odontogenic tumor shows widely variant radiographic patterns, which compels the radiologist to consider a few differential diagnoses. Correlation of clinical, radiographic and histopathological features is a requisite to affirm the diagnosis of odontogenic myxoma. This case report intends to compare a classical presentation of odontogenic myxoma with the contrasts in published literature.

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.
Gupta S, Grover N, Kadam A, Gupta S, Sah K, Sunitha JD. Odontogenic myxoma. Natl J Maxillofac Surg 2013;4:81-3.  Back to cited text no. 1
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Moussa A, Achach T, Njim L, Yahia N Ben, Gassab E, Zakhama A. Odontogenic myxoma: A report of an unusual pediatric case. Int J Pediatr Otorhinolaryngol 2016;2:173-5.  Back to cited text no. 2
    
3.
Limdiwala P, Shah J. Odontogenic myxoma of maxilla: A review discussion with two case reports. Contemp Clin Dent 2015;6:131.  Back to cited text no. 3
[PUBMED]  Medknow Journal  
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Reddy S, Naag A, Kashyap B. Odontogenic myxoma: Report of two cases. Natl J Maxillofac Surg 2010;1:183.  Back to cited text no. 4
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Manne RK, Kumar V Suneel, Sarath PV, Anumula L, Mundlapudi S, Tanikonda R. Odontogenic myxoma of the mandible: A case report. Case Rep Dent 2012;2012:1-4.  Back to cited text no. 5
    
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Chaudhary Z, Sharma P, Gupta S, Mohanty S, Naithani M, Jain A. Odontogenic myxoma: Report of three cases and retrospective review of literature in Indian population. Contemp Clin Dent 2015;6:522.  Back to cited text no. 6
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7.
Kaffe I, Naor H, Buchner A. Clinical and radiological features of odontogenic myxoma of the jaws. Dentomaxillofacial Radiol 1997;26:299-303.  Back to cited text no. 7
    
8.
Noffke CEE, Raubenheimer EJ, Chabikuli NJ, Bouckaert MMR. Odontogenic myxoma: Review of the literature and report of 30 cases from South Africa. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2007;104:101-9.  Back to cited text no. 8
    
9.
Sharma N, Prashant D, Sharma S, Prashant V. Odontogenic myxoma of oral cavity: A case report. Int J Oral Maxillofac Pathol 2013;4:85-90.  Back to cited text no. 9
    
10.
Singaraju S, Wanjari SP, Parwani RN. Odontogenic myxoma of the maxilla: A report of a rare case and review of the literature. J Oral Maxillofac Pathol 2010;14:19-23.  Back to cited text no. 10
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Chrcanovic BR, Do Amaral MBF, Marigo HDA, Freire-Maia B. An expanded odontogenic myxoma in maxilla. Stomatologija 2010;12:122-8.  Back to cited text no. 11
    
12.
Mixoma EDU, Desplazamiento OC. Clinico-radiographic examination of odontogenic myxoma with displacement of unerupted upper third molar: Review of the literature. Int J Morphol 2011;29:930-3.  Back to cited text no. 12
    
13.
Li TJ, Sun LS, Luo HY. Odontogenic myxoma: A clinicopathologic study of 25 cases. Arch Pathol Lab Med 2006;130:1799-806.  Back to cited text no. 13
    
14.
Kheir E, Stephen L, Nortje C, Janse Van Rensburg L, 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. 14
    
15.
Rani V, Mahaboob Kadar M, Babu A, Sankari L, Krishnasamy G. Odontogenic myxoma diagnostic dilemma: A case report and review of literature. J Cranio-Maxillary Dis 2014;3:163-7.  Back to cited text no. 15
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]



 

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