|Year : 2020 | Volume
| Issue : 2 | Page : 192-195
”Myxoma of the Jaws”: Report of an atypical presentation and review of literature
Giridhar S Naidu1, Ramanpal S Makkad1, Gopakumar R Nair2, Ravleen Nagi3
1 Department of Oral Medicine and Radiology, New Horizon Dental College and Research Institute, Sakri, Bilaspur, Chhattisgarh, India
2 Department of Oral Medicine and Radiology, KD Dental College and Hospital, Mathura, Uttar Pradesh, India
3 Department of Oral Medicine and Radiology, Swami Devi Dyal Hospital and Dental College, Panchkula, Haryana, India
|Date of Submission||29-Feb-2020|
|Date of Decision||13-Apr-2020|
|Date of Acceptance||25-Apr-2020|
|Date of Web Publication||27-Jun-2020|
Dr. Giridhar S Naidu
Department of Oral Medicine and Radiology, New Horizon Dental College and Research Institute, Sakri, Bilaspur, Chhattisgarh
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Oral lesions of myxomatous origin are relatively rare and include nerve sheath myxomas, soft tissue myxomas, oral focal mucinosis, and odontogenic myxomas. These lesions have a common histologic appearance of abundant myxoid ground substance. A 61-year-old edentulous female presented with nasal stuffiness since the past 4 months. Clinical examination revealed an asymptomatic intraoral swelling in the edentulous maxillary ridge which had ulcerated. Plain film radiography and CT showed an expansile osteolytic lesion involving the right maxillary sinus. A partial maxillectomy was performed and direct microscopy showed a predominant embryonic mesenchymal stroma with fibroblasts and calcifications suggestive of a myxoma. The central myxoma is hypothesized to originate from the odontogenic apparatus due to its almost exclusivity to the jaw bones. However, when a central myxoma occurs in an edentulous jaw at an age when the odontogenic apparatus is apparently nonfunctional, explaining the genesis of this unique tumor based on present understanding becomes difficult. We present one such case while reviewing the existing literature on the present concepts of pathogenesis.
Keywords: Computed tomography, jaw myxoma, maxillary sinus, resection
|How to cite this article:|
Naidu GS, Makkad RS, Nair GR, Nagi R. ”Myxoma of the Jaws”: Report of an atypical presentation and review of literature. J Indian Acad Oral Med Radiol 2020;32:192-5
|How to cite this URL:|
Naidu GS, Makkad RS, Nair GR, Nagi R. ”Myxoma of the Jaws”: Report of an atypical presentation and review of literature. J Indian Acad Oral Med Radiol [serial online] 2020 [cited 2022 Jan 27];32:192-5. Available from: https://www.jiaomr.in/text.asp?2020/32/2/192/288134
| Introduction|| |
Myxomas are a heterogenous group of soft tissue tumors which have a common histologic appearance of abundant myxoid ground substance., The modified World Health Organization (WHO) classification of odontogenic tumors defines odontogenic myxoma (OM) as an intraosseous neoplasm characterized by stellate and spindle-shaped cells embedded in an abundant myxoid or mucoid extracellular matrix. Jaw myxomas often involve posterior mandible as compared to other intraoral sites, and around 3–6% cases are odontogenic in origin., Despite several reported cases, the origin of jaw myxomas is still debatable. The dental papilla, the follicle, and the periodontal ligament have all been implicated as possible sites of origin. This is due to the close histological similarity of the tumor to the dental papilla., Such a simplistic assumption cannot account for a certain group of cases which present in unusual sites like the condyle  or an edentulous jaw at an age when the odontogenic apparatus is presumably nonfunctional. We present such a unique case of maxillary myxoma in a 61-year-old completely edentulous female patient with symptoms of nasal stuffiness, and difficulty in breathing since 4 months with reviewing the existing literature.
| Case Report|| |
A 61-year-old female was referred to our hospital for treatment of a maxillary swelling. She presented with nasal stuffiness in the right nostril and difficulty in breathing since 4 months. There was no history of associated pain, discharge, or bleeding from the nostrils. The patient had earlier consulted an otolaryngologist where an intraoral biopsy was taken; the reports of which were unavailable. She was otherwise systemically healthy and had undergone total extraction 4 years back and was a complete denture wearer since the same period.
Clinical examination showed completely edentulous maxillary and mandibular arches. A bicortical, nontender, firm to hard, expansion of the edentulous maxillary arch in the right posterior quadrant measuring 2 × 3 cm was noted. There was obliteration of the adjacent buccal sulcus and a diffuse enlargement in the palatal aspect of edentulous ridge. An ulcerated area with slightly raised margins measuring 2 cm representative of the biopsy site was noted on the residual alveolar ridge in relation to the maxillary right first molar [Figure 1]. A well-defined, nontender, firm mass extruded from the same region and was covered with necrotic slough. There was no associated discharge or bleeding on provocation present from either the ulcerated area or the mass. A provisional diagnosis of benign antral tumor with an inverted papilloma or an antral polyp was considered first. In the differential diagnosis, adenoma, antral mucocele, low-grade mucoepidermoid carcinoma, and ossifying fibroma were considered.
|Figure 1: Intraoral examination reveals bicortical expansion of the edentulous maxillary arch in the right posterior quadrant|
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Conventional radiography showed a hazy radiolucent lesion in the right posterior maxilla with minimal buccal cortical expansion in the occlusal and periapical radiographs. The panoramic radiograph showed completely edentulous jaws with partial discontinuity of the floor of the right maxillary sinus in the maxillary molar region. The floor of the sinus appeared to have been raised and discontinuous in areas [Figure 2]. A Waters' view showed uniform opacification of the right maxillary sinus with faintly defined lateral and medial walls [Figure 3]. Axial and coronal computed tomography (CT) images of the osteomeatal complex showed a homogenously dense expansile lesion involving the entire right maxillary antrum measuring 4.4 × 3.6 × 4.8 cm in size. It had extended into the right nasal cavity and posteriorly into the right ethmoidal sinus. The density of the lesion was less than muscle tissue [Figure 4]a, [Figure 4]b.
|Figure 2: Panoramic radiograph shows completely edentulous jaws with partial discontinuity of the floor of the right maxillary sinus in maxillary molar region|
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|Figure 3: Waters' view shows uniform opacification of right maxillary sinus|
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|Figure 4: a) Axial and b) coronal CT images of the osteomeatal complex shows homogenously dense expansile lesion involving the entire right maxillary antrum measuring 4.4 × 3.6 × 4.8 cm in size and density of lesion less than muscle tissue|
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Considering the extent of the lesion and its relatively well-encapsulated margins, a partial maxillectomy of right maxilla with tumor excision was carried out under general anesthesia. On gross appearance, the tumor mass was well encapsulated, measuring 4 × 5 cm in size [Figure 5]. Microscopy showed hypocellular loose connective tissue with delicate and haphazardly arranged collagen fibers. In few areas, connective tissue cells were noted with indistinct cytoplasm and wavy nuclei. Epithelium was not present anywhere. Areas of round to ovoid concentric hematoxiphillic lamellar calcified masses (cementicle like) were seen dispersed throughout the section [Figure 6]a, [Figure 6]b. The findings were suggestive of an OM and the presence of calcification was an additional finding. The patient was apparently healthy till 6 months after the surgery after which she did not report back and was lost for follow up.
|Figure 5: Gross-excised tumor mass which was well encapsulated, measuring 4 × 5 cm in size|
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|Figure 6: Microscopic examination a) 10x and b) 40 × (Hematoxylin and Eosin) stained section reveals hypocellular loose embryonic tissue with delicate and haphazardly arranged collagen fibers, few areas of connective tissue cells with indistinct cytoplasm and wavy nuclei, no evidence of epithelium, and areas of round to ovoid concentric calcified masses|
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| Discussion|| |
The jaw/odontogenic myxoma has been described as an uncommon lesion rarely occurring before age 10 or after 50 years of age with no apparent sex predilection, frequently located in the mandible in two third of the cases and in the maxilla in one-third of the cases. In the maxilla, the alveolar and zygomatic processes are the most common sites of occurrence, with frequent invasion of the tumor into the maxillary antrum., Our patient was a 61-year-old female who presented with painless bicortical enlargement of the edentulous maxillary arch in the right posterior quadrant. Radiographically, it appears as an unilocular or multilocular radiolucency with well-defined borders, giving a “honey- comb”, soap bubble” appearance or trabeculae intersect at right angles to form angular locules giving a “tennis racquet” appearance, but maxillary lesions are often radiopaque as seen in our case.
One of the rare findings of our case is the occurrence of tumor in the edentulous jaw, where its odontogenic origin becomes difficult to establish. In circumspection, the lack of earlier radiographic records could be a reason enough to dispute the fact that the lesion could have developed in the dentulous jaw of the patient and may be the reason for edentulism. Another school of thought is that the presence of myxoma tissue with few odontogenic cells microscopically favors the diagnosis of OM, but does not prove its origin from the odontogenic apparatus. Other mesenchymal tissue can give rise to myxoma like cells, and since in edentulous jaw there is no odontogenic apparatus and further any remaining odontogenic cell rests may undergo epithelial metaplasia, the potential to cause tumor is less.,
Various theories have been proposed for the origin of jaw myxomas. They are generally believed to arise from the derivatives of neural crest ectomesenchyme, i.e. dental papilla and dental follicle. Some researchers have suggested that myxoma cells develop as a result of odontogenic epithelial induction. Similar to present case, Kim J  found calcifications in 37% dental follicles that attribute to an abnormal inductive phenomenon but whether these calcifications are a product of induction or dystrophic in nature remain unresolved.
Two cases reported of maxillary myxoma involving edentulous region have been found by our literature search. De Mello et al. observed cortical expansion in the edentulous region in a 23-year-old female in whom the right maxillary first molar was removed 12 months back. Axial and coronal views of CT showed tumor extending throughout the entire walls of the maxillary sinus superiorly till the orbital floor. Mileto et al. reported another case of soft tissue myxoma in the right upper edentulous canine region in a 54-year-old male patient, gradually progressing in size asymptomatically since 10 years after extraction of the tooth. Definitive diagnosis of OM was established by histopathological features of stellate, fusiform, or rounded shaped cells and small islands of odontogenic epithelial remains in the myxoid matrix.
Maxillary myxomas are aggressive, radio resistant, and therefore wide surgical excision is the treatment of choice. Various conservative treatment options such as enucleation, curettage, and cryosurgery have shown high recurrence rates. In the present case, considering the extent of the lesion and well-encapsulated margins, partial maxillectomy of the right maxilla with tumor excision was carried out under general anesthesia and no recurrence of the lesion was reported after 6 months postoperative follow up.
| Conclusion|| |
To conclude, we present a case of potential interest due to its unusual presentation in the edentulous jaw at an age when odontogenic apparatus presumably ceases to develop and function does pose questions regarding the origin of the tumor cell. Therefore, a suggestion in hindsight would be that future research should be conducted to prove their “odontogenic origin” conclusively.
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
Conflicts of interest
There are no conflicts of interest.
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