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| CASE REPORT |
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| Year : 2019 | Volume
: 8
| Issue : 2 | Page : 118-121 |
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Odontogenic myxoma- A rare case report
Kathoju Mounika, Surekha Ramulu, Guttikonda Venkateswara Rao, M Praveen Kumar
Department of Oral Pathology and Microbiology, Mamata Dental College, Khammam, Telangana, India
| Date of Submission | 26-Sep-2018 |
| Date of Acceptance | 27-Apr-2019 |
| Date of Web Publication | 30-Jul-2019 |
Correspondence Address:
Dr. Kathoju Mounika
Department of Oral Pathology and Microbiology, Mamata Dental College, Khammam - 507 002, Telangana
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/JDRNTRUHS.JDRNTRUHS_92_18
Odontogenic myxoma is benign, slow-growing, locally aggressive tumour arising from the ectomesenchyme of a developing tooth. It is the third most common odontogenic tumour. It occurs mainly in the 2nd and 3rd decades with slight predilection in females, and the mandible is more commonly involved than the maxilla. The lesion often grows without symptoms and presents as a painless swelling. Radiographic features are variable with a unilocular or multilocular radiolucency showing honeycomb, soap bubble or tennis racket pattern with cortical plate expansion. Histologically odontogenic myxoma shows loosely arranged stellate to spindle shaped cells interspersed in myxoid matrix. A case of odontogenic myxoma in 46- year-old female patient involving the right maxilla resulting in gross facial deformity within a span of 6 months is reported.
Keywords: Aggressive, anterior maxilla, odontogenic myxoma
How to cite this article:
Mounika K, Ramulu S, Rao GV, Kumar M P. Odontogenic myxoma- A rare case report. J NTR Univ Health Sci 2019;8:118-21 |
| Introduction | |  |
Odontogenic myxoma is an uncommon benign mesenchymal odontogenic tumour derived from primitive ectomesenchymal structures of the developing tooth including dental follicle, dental papilla or periodontal ligament.[1] Rudolph Virchow a German pathologist described the histologic features of myxofibroma in 1863. Thomas and Goldman in 1947 described myxomas of the jaws. Since then odontogenic myxoma has been a subject of continuous scientific debate.[2] It accounts for about 3-11% of all odontogenic tumors.[3]
The prevalence of odontogenic myxoma is 0.04% to 3.7% with slightly higher frequency reported in Asia, Europe, and American population. The preferred age group of odontogenic myxoma is 2nd and 3rd decades with slight predilection in females. The mandible is more frequently involved than the maxilla.[4] Clinically it is slow-growing neoplasm, infiltrative in growth and aggressive in nature with high recurrence rate.[5],[6],[7] Due to poor follow up and lack of reports a precise analysis of the recurrence rate is unclear. In view of its rarity, diagnostic and operative dilemma encountered while managing, the present case is here with reported.
| Case Report | | |
A 46 year old female patient presented to our dental hospital with a painful progressive swelling in the right side of maxilla since 6 months. Initially the swelling was small in size and gradually increased to the present size.
Extra oral examination revealed a diffuse swelling in right mid third of face obliterating the nasolabial fold extending anterioposteriorly from the ala of nose to the zygomatic bone, and superioinferiorly from the infraorbital region to the corner of the mouth measuring about 4 × 5 cms. Buccal cortical plates were expanded and there is no history of paraesthesia [Figure 1]a. Intraorally, swelling extended from the left maxillary incisor region to the right maxillary molar region posteriorly obliterating the buccal vestibule [Figure 1]b. | Figure 1: (a) Extraoral photograph showing diffuse swelling on right middle third of face (red circle). (b) Intraoral photograph showing obliteration of buccal vestibule
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Orthopantamograph revealed multilocular radiolucency involving the left maxillary premolar to right maxillary molar region and the CT image showed large expansile mass in the right maxilla with complete obliteration of the right maxillary sinus [Figure 2]a and [Figure 2]b. | Figure 2: (a) Orthopantomogram showing multilocular appearance in the maxilla (red circle). (b) Computed tomography scan showing lesion on right maxilla and obliterating right maxillary sinus
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Grossly, the excised mass was large with both hard and soft tissues. The hard tissue includes the extracted tooth i.e. from right premolar to left premolar. Soft tissue mass was white in color measuring about 6 × 4 cms with irregular borders, soft in consistency, and appeared as smooth, glistening, gelatinous, lobulated mass [Figure 3]a. Microscopically, haematoxylin and eosin (H and E) stained section revealed surface epithelium and tumour mass in connective tissue stroma separating them by grenz zone. Spindle - stellate shaped cells were loosely arranged in abundantly myxoid connective tissue stroma closely resembling the mesenchymal portion of developing tooth. Few areas showed moderately dense collagen fibres and strands of inactive odontogenic epithelium. Based on these histological features a diagnosis of odontogenic myxoma was given [Figure 3]b. | Figure 3: (a) Photomicrograph of the gross specimen of excised lesion. (b) Photomicrograph of Hematoxylin and Eosin stained tissue section showing stellate-shaped cells in the fibrillar stroma (40X)
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The mucoid nature was confirmed by positive reaction with Alcian blue staining and negative reaction with Periodic Acid Schiff (PAS) staining. Immunohistochemically markers such as vimentin and S100 were performed and revealed positivity for tumour cells [Figure 4]a, [Figure 4]b, [Figure 4]c, [Figure 4]d. | Figure 4: (a) Photomicrograph of tissue section showing positive reaction for Alcian blue (100X). (b) Photomicrograph of tissue section showing negative reaction for Periodic Acid- Schiff (100X). (c) Immunohistochemistry showing positivity for vimentin (40X). (d) Immunohistochemistry showing positivity for S100 (40X)
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| Discussion | | |
Odontogenic myxomas (OM) are rare benign mesenchymal odontogenic tumours arising from dental papilla, follicle or the periodontal ligament.[8],[9] Evidences supporting the odontogenic origin are (a) its occurrence is seen exclusive in the tooth-bearing areas of the jaws (b) occasional association with an unerupted tooth or a missing tooth (c) occurrence in younger individuals (d) histologic resemblance to dental papilla, and (e) occasional presence of odontogenic epithelial island.[10]
OM exhibits abundant extracellular production of ground substance and thin fibrils by the delicate mesenchymal cells that are spindle-stellate in shape. These undifferentiated mesenchymal cells are capable of fibroblastic differentiation. Depending upon the pattern of differentiation, the histological nature of the tumour varies. It can be either completely myxomatous in nature or with varying proportions of myxomatous and fibrous tissue. Some regard OM as a modified form of fibroma in which myxoid intracellular substance separates the connective tissue.[4],[5]
OM occurs between 10 to 30 years of age with female predilection.[3] It occurs most commonly in the posterior mandibular region [2] whereas in the present case it was seen in 46-years-old female patient involving anterior maxilla region. The majority of myxomas are almost always asymptomatic, although some patients present with progressive pain in lesions involving maxilla and maxillary sinus.[1],[11] Odontogenic myxomas of the maxilla is less frequent but behaves more aggressively than that of the mandible as it infiltrates through maxillary sinus. In the present case maxillary sinus obliteration was noted.
The radiographic characteristics of odontogenic myxomas are variable depending on developmental stage. Unilocular appearance is seen in the anterior maxilla of young children and multilocular radiolucency is observed in posterior maxilla of adults.[12],[13],[14] In our case multilocular appearance of the lesion was seen in the anterior maxilla. Zang et al.[10] classified radiographic appearances of odontogenic myxomas into six types- Type I-Unilocular, Type II-Multilocular (honey comb, soap bubble or tennis racquet pattern, Type III-Lesion located in alveolar bone, Type IV-Lesion involving the maxillary sinus, Type V-osteolytic destruction, Type VI-Combination of bone destruction and bone formation. Kaffe et al. in his radiographic study revealed correlation between size and locularity; unilocular lesions were smaller than 4 cms and multilocular lesions were larger than 4 cms and was in consistence with our case report, with the lesion being multilocular measuring about 7 cms in dimensions. Odontogenic myxomas should be included in differential diagnosis of solid ameloblastoma, central haemangioma and odontogenic keratocyst.[13]
MacDonald-Jankowski suggested that both CT and radiographs should be used in the investigation of odontogenic myxomas. CT assesses perforation and radiographs allow better assessment of the degree of definition of the lesion's margins with adjacent normal bone. In the present case, bony expansion in the anterior maxilla with thinning and erosion of cortical plate was observed under CT.[15]
Histologically the WHO (1992) defined OM as “A locally invasive neoplasm consisting of rounded and angular cells lying in an abundant mucoid stroma”.[2] They show loose myxoid stroma containing spindle- stellate and angular or round cells. Stroma shows very few fine fibrillar material with minimum vascularity. Calcifications may or may not be present. Inflammatory infiltration predominantly of lymphocytes and remnants of odontogenic epithelium are seen.[2],[9] In our case the histological features were similar to that of the literature. Epithelial islands are not commonly observed in the myxomas of jaws but in the present case epithelial islands were without calcifications. The mucoid nature of OM was confirmed with positive reaction with alcian blue and negative reaction with PAS stain. Alcian blue is a basic dye and has affinity for both sulphated – carboxylate acid mucopolysaccharides and glycoproteins in the mucoid stroma.[16]
An immunohistochemical panel is used to characterize the nature of the lesion. There are three types of cells discriminated in odontogenic myxoma they are: Spindle cells, stellate cells and hyaline cells. Antibodies directed against vimentin are used to identify mesenchymal cells, Spindle cells were positive for transferrin, ferritin, alpha 1-ACT, alpha 1-AT, S-100 protein and vimentin. Stellate cells were strongly positive for transferrin, alpha 1-AT, S-100 protein and vimentin. Hyaline cells reacted with alpha 1-ACT and alpha 1-AT.[15] In the present case immunohistochemical positivity with vimentin, and S100 supports the ectomesenchymal origin of odontogenic myxoma.
The aggressive nature of the lesion is well documented in literature. The absence of capsule and tendency of OM to permeate into marrow spaces makes enucleation and curettage difficult.[9] Small lesions have been successfully treated by enucleation but the larger lesions require segmental resection. In the present case segmental resection of lesion in the maxilla was performed with immediate reconstruction. The patient was on follow up for one year and no recurrence was noted till date.
| Conclusion | | |
Odontogenic myxoma is a slow growing benign, locally malignant tumour, notorious for recurrence. The greater variability in the clinical and radiological features of odontogenic myxoma emphasizes that it should be considered in differential diagnosis of both radiolucent and radiopaque lesions in both jaws. Due to unspecific nature of these lesions along with diagnostic and operative dilemmas related to myxomatous tumours, a sound knowledge with proper histopathological diagnosis is required for efficient management.
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.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]
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