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Contemporary Clinical Dentistry | Jan-Mar 2015 | Vol 6 | Issue 1
131
Odontogenic myxoma of maxilla: A review discussion with two case reports
P L, J S
Abstract
Odontogenic myxoma (OM) is a rare entity of slowly growing benign neoplasm of ectomesenchymal origin, comprising of
3–6% of all odontogenic tumors that histologically presenting spindle-shaped, stellate and round cells within loosely arranged
myxomatous tissue stroma. OM originates from the dental papilla, follicle or periodontal ligament with an exclusive location in the
tooth-bearing areas of the jaws, association with missing or unerupted teeth. Clinically and radiographically the reported incidence
and demographic information of this tumor has wide variability. Most common clinical variant is associated with the impacted
tooth and shows local invasion with destruction of adjacent structures and displacement of teeth. Radiographically, common
manifestations are multilocular radiolucent areas with well-defi ned borders and typical soap bubble or tennis racket appearances.
This paper presents two rare case reports of OM of maxilla along with review discussion.
Keywords: Fibromyxoma, myxofi broma, myxoma, odontogenic myxoma, tennis racket appearance
Department of Oral Medicine and Radiology, Government Dental
College and Hospital, Ahmedabad, Gujarat, India
Correspondence: Dr. Piyush Limdiwala,
A-401, Savvy Solaris, B/H D-Mart, Opp.
Auda Garden, Motera, Ahmedabad - 380 005, Gujarat, India.
E-mail: drpiyushlimdiwala@gmail.com
Introduction
Myxomas are benign, slow-growing and locally aggressive
mesenchymal origin neoplasm.[1] Myxomas can be found in
various body parts such as skin, subcutaneous tissues, and
heart. Myxomas of head and neck region are rare.[1,2] Two
forms of myxomas have been identified those derived from
“facial bones” and they are either osteogenic myxoma or
odontogenic myxoma (OM) and another derived “soft tissue”
like perioral soft tissue, parotid gland, ear or larynx.[2,3] OM
represents an uncommon benign neoplasm comprising of
3–6% of all odontogenic tumors,[2-5] most commonly arising
in mandible (66.4%) followed by maxilla (33.6%).[2,3] According
to World Health Organization (WHO), OM is classified as a
benign tumor of ectomesenchymal origin with or without
odontogenic epithelium.[3,5,6]
Odontogenic myxomas are asymptomatic. Pain, paresthesia or
asymmetries occur only when they take on larger sizes. Their
growth is usually slow; however, they are locally aggressive.
They may cause divergence of root, resorption, tooth shifting
or movement. When involving maxilla, OM can expand inside
the maxillary sinus and are then diagnosed later only after
having grown to larger sizes.[5]
Radiographically, the tumor presents as a unilocular or
multilocular radiolucent lesion with well-defined borders with
fine bony trabeculae within its interior structure expressing a
“honeycombed”, “soap bubble” or “tennis racket” appearance.
Unilocular appearance may be seen more commonly in
children and in the anterior parts of jaws. Displacement of
teeth is a relatively common finding, root resorption is rarely
seen and the tumor is often scalloped between roots.[1,3,6]
A histological characteristic of this tumor resembles the
mesenchymal portion of a tooth in development. OM consists
of rounded, spindled, fusiforms and star cells arranged in a
loose, abundant myxoid stroma with few collagen fibrils. Small
islands of apparently inactive epithelial odontogenic rests
may be scattered through the myxoid substance without any
capsule, and they are important to establish the diagnosis.[7]
Because of its high rate of recurrence, especially due to its
gelatinous and mucous aspect and having no capsule, surgical
treatment through bone resection is the most indicated
treatment modality, followed by long-term follow up.[5]
This paper presents two cases of OM of maxilla with different
clinical and radiographic pictures. The purpose of the
present paper is to increase cognition of the radiographic
appearances of OM and to provide diagnostic references for
better diagnosis.
Case Reports
Case I
A 36-year-old Hindu male patient presented with the chief
complaint of painless swelling on 23 tooth region since 1-year.
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Limdiwala and Shah: Odontogenic myxoma
Contemporary Clinical Dentistry | Jan-Mar 2015 | Vol 6 | Issue 1 132
Patient had undergone for excision for the same type of
lesion at private clinic before 3 years. Since then, patient was
normal, except peanut size swelling which remained present
after surgery, but since 2–3 months, patient noticed increased
in size of the swelling, and it reached up to present size.
Intraoraly, well-defined around 2 cm in size, oval shaped
swelling was present at 23 tooth region. Swelling was soft,
nonfluctuant, nontender and without any discharge. Distal
displacement of 22 was present. No pain or discoloration
was found in any tooth. Medical history was not significant.
Vitality test was done and 22, 23 and 24 was found
vital [Figure 1].
The patient underwent radiographic investigations that
suggested radiolucent area around 3 cm × 2 cm in size at
interdental and periapical area of 22 and 23 region. A thin
sclerotic bone reaction was seen at only superior border, rest
of the area was blending to adjacent bone. Internal structure
showed hazy radiolucency with one to two thin septa
crossing lesion centrally. Mesial displacement of 22 was seen
without any root resorption [Figure 2]. Clinico-radiographic
features and history of recurrence was suggestive of either
odontogenic keratocyst or lateral periodontal cyst. A routine
hematological investigation was done before surgery. As
negative aspiration was found, lesion was totally excised
and specimen sent to the pathology department [Figure 3].
Histologically, section showed abundance of fusiform, stellate
and round cells in myxoid tissue [Figure 4] and was diagnosed
as myxoma.
Case II
A 28-year-old Hindu female patient came with painless
swelling in right posterior maxilla since 1 year and 3 months.
No history of pain, fever or trauma was found. Initially
swelling was small in size and remained static for 1 year. Since
last 3 months, swelling was rapidly increasing in size causing
facial asymmetry without any pain or any other associated
symptoms. Patient went to local private practitioner where
she had been prescribed antibiotics, but the size of swelling
Figure 1: Intraoral photograph of case 1 showing dome shaped
swelling present at 22.23 region with distal displacement of 22
Figure 2: Maxillary occlusal radiograph of case 1 showing
hazy radiolucency with one to two thin septa crossing lesion
centrally at 22,23 region
Figure 3: Gross specimen of excised soft tissue mass of case 1
Figure 4: Histological picture of case 1 showing abundance of
fusiform, stellate and round cells in myxoid tissue
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was not reduced. Finally, patient came to our hospital with
painless asymptomatic swelling in the right maxilla.
Extraorally, swelling was around 4 cm × 3 cm in size at right
maxilla with obliteration of nasolabial fold. Swelling was
diffuse, nontender, hard in consistency, without any discharge.
No history of nasal discharge, difficulty in breathing,
paresthesia or anesthesia on the affected site. Overlying
skin was normal in color with regional lymphadenopathy.
No deviation of the lateral wall of the nose or no difficulty
in mastication or trismus was found [Figure 5]. Intraoraly,
swelling was well defined around 4 cm × 3 cm in size,
extending from 13 to 18 teeth region with expansion of both
cortical plates around 3 cm buccally and 2 cm palatally up to
midline of palate. On palpation, swelling was hard, nontender,
nonfluctuant without any discharge. Overlying mucosa was
slightly red and inflamed. Tooth 17 was grossly carious with
displacement of 16 palatally and 18 distally [Figure 6].
Maxillary occlusal radiograph showed a mixed
radiopaque-radiolucent area with a granular appearance
and ill-defined boundaries. No root resorption was seen
of adjacent teeth. Orthopantomogram showed around
4 cm × 3 cm mixed radiopaque-radiolucent lesion at
Figure 5: Extraoral photograph of case 2 showing swelling on
right maxilla with obliteration of nasolabial fold
Figure 6: Intraoral photograph of case 2 showing swelling at
posterior maxilla of 14–18 region with buccal cortical plate
expansion
Figure 7: Orthopantomogram of case 2 showing poorly defi ned
mixed radiolucent radiopaque area at right posterior maxilla with
root piece of 17 and without root resorption of involved teeth
Figure 8: Computed tomography image of case 2 showed a
well-defi ned expansile mass with calcifi ed matrix arising from
the alveolar process of maxilla, extending into the maxillary
sinus with destruction of the posterolateral wall of the sinus
Figure 9: Histological picture of case 2 showing typical spindle-
shaped cells in a myxomatous stroma
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periapical area of right maxillary molars approaching
maxillary sinus. Lesion was having poorly differentiated
boundaries. Internally, irregularly arranged thin trabeculae
with bone resorption were present. No resorption of teeth
found [Figure 7]. Computed tomography (CT) images
showed a well-defined expansile mass with calcified matrix
around 4.4 cm × 2.9 cm in size, arising from the alveolar
process of maxilla, extending into maxillary sinus with
destruction of posterolateral wall of sinus [Figure 8]. Benign
odontogenic tumor, that is, central giant cell granuloma,
fibro-osseous lesion, and OM were considered in the
differential diagnosis.
Routine hematological investigations showed only slight
eosinophilia. Incisional biopsy was performed, and it was
diagnosed as myxofibroma [Figure 9]. Tumor was totally
excised with surgical curettage and specimen was sent to
the pathology department.
Discussion
Rudolf Virchow coined the term myxofibroma in 1863,[2-5]
for a group of tumors that had histologic resemblance to
the mucinous substance of the umbilical cord.[2,3,5] OM was
first mentioned in the literature by Thoma and Goldman in
1947.[5,8,9] In 1948, Stout redefined the histologic criteria for
myxomas as true neoplasms that do not metastasize and
exclude the presence of recognizable cellular components
of other mesenchymal tissues, especially chondroblasts,
lipoblasts, and rhabdomyoblasts.[2,3]
In 1992, WHO classified OM for histological typing
of odontogenic tumors: “A benign tumor, which is of
ectomesenchymal origin with or without the presence of
odontogenic epithelium.[3,5] Traditionally, OM is mainly
circumstantial, involving teeth bearing areas associated
with unerupted tooth with histologically resemblance to
dental mesenchyme (dental papilla, follicle or periodontal
ligament) and the sporadic presence of islands of odontogenic
epithelium.[3] The Mucoid tumors of soft tissue represent
significant differences in biologic behavior, ranging from
harmless to malignant neoplasms. As an osseous entity,
however, OMs of the jaws are considered slow-growing
tumors with the potential for extensive bone destruction,
cortical expansion, and a relatively high recurrence rate.[10]
In presented paper, both cases had different clinical picture
and biological behavior. First case had slow growing lesion
with asymptomatic condition but with a recurrent history
and another case had faster growth, facial asymmetry, the
destructive and expansile nature, affecting surrounding vital
structures resembling malignant tendency.
The tumor occurs across an age group that varies from 22.7
to 36.9 years.[6,9] It is rarely seen in patients younger than
10 years and older than 50 years of age.[2,5,6,9] According
to Farman et al., suggested mean age of maxillary OM for men
was 29.2 years and 35.3 years for women while in mandibular
OM in men occur at mean age of 25.8 years and 29.3 years
for women.[11] Gunahan et al. and Regazi et al. reported a
distinct predominance in females (64–95%) and a predilection
for the mandible.[12,13] Keszler et al. noted that 8% of OMs
occurred in children of <16 years.[14,15] Posterior Mandible
is more frequently affected than the maxilla.[3-6] According to
Reichart and Philipsen, mandibular myxomas accounted for
66.4%, with 33.6% in the maxilla. Whereas 65.1% of mandibular
cases located in molar-premolar region, 73.8% were located
in same areas of the maxilla.[2,3,16] In presented, both cases,
one patient was male with 36 years of age with lesion found
on anterior maxilla and another was female with 28 years of
age and lesion was found in the posterior maxilla.
Most often OMs are first noticed as a result a slowly growing
increasing swelling or asymmetry of the affected jaw.[3]
Growth may be rapid with infiltration of neighboring soft
tissue structures. OM of the maxilla may be asymptomatic
or on examination, it may present as heaviness, swelling
of cheek or palate, malocclusion or loosening of teeth.[16]
Displacement of teeth has been registered in 9.5% of the
cases. Lesions are generally painless, and ulceration of the
overlying oral mucosa occurs only when the tumor interferes
with dental occlusion. When the maxillary sinus is involved,
OM often fills the entire antrum.[2,3,16] They may still involve the
palate, orbit, and nasal cavity, causing symptoms associated
with this structures.[4,5] In severe cases, nasal obstruction
or exophthalmus may be the leading symptoms.[2,3,16] In the
mandible, OM may involve the neurovascular bundle in the
mandibular canal.[17] Our both cases were of maxilla in which
first case was totally asymptomatic with slow growth, and
another had all classical features accordance to review.
Radiographically, larger multilocular OM is more common
in the posterior areas of the jaws and unilocular lesions
are mostly located in the anterior.[3,6] The radiographic
tumor margins may be either well-defined or poorly
defined.[17] On conventional radiographs, OM presented
varying radiographic appearances, ranging from unilocular to
multilocular (including honeycomb, soap bubble and tennis
racquet patterns); with involvement of local alveolar bone or
maxillary sinus and sometimes osteolytic destruction with or
without osteogenesis.[15] The tennis racket appearance where
the bony septae appear as triangular, square or rectangular
compartments with very fine trabeculation within them is
the most common.[17] Many review suggest that there may
be no correlation between the borders of the lesion and the
amount of bony trabeculae within the lesion, but maxillary
tumors were more likely to be ill defined in nature compared
with mandibular lesions.[10]
According to some studies, root displacement rather
than resorption is the rule of jaw myxomas.[3,6,9,10,17]
Radiographically, OM may present similar features of
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an ameloblastoma or a central giant cell granuloma or
sometimes may mimic an osteosarcoma. These facts do pose
potential difficulties in reaching a proper diagnosis merely
on radiographic studies. A biopsy is, therefore, necessary to
ascertain an accurate diagnosis.[9,10]
Recently, CT and magnetic resonance imaging examinations
were applied in many case reports and it may present as
osteolytic expansile lesions with mild enhancement of the
solid portion of the mass in the myxoma of the mandible
or bony expansion and thinning of cortices with strong
enhancement of the mass lesion in the anterior maxilla. In
the case of maxillary sinus, bone destruction and thinning and
strands of fine lace like density representing ossifications may
be seen.[2,3] Our second case showed internal calcifications
with destruction of the posterolateral wall of maxillary
sinus whereas first case had only soft tissue mass with
mild osteolytic areas in the anterior maxilla. Furthermore,
lesion was small, unilocular with fine one to two straight
septa internally, involvement of local alveolar bone, poorly
defined boundaries and only displacement of teeth without
resorption, while second case was of posterior maxilla
with larger in size, multilocular variety and involvement of
maxillary sinus. Owing to the existence of the maxillary sinus,
part of the lesion image would overlap it. This influenced
the image definition. Therefore, the lesion image may be
obscure and may often be easily mistaken for other tumors
or sometimes malignancy.
Differential diagnoses include ameloblastoma, odontogenic
keratocyst, central giant cell granuloma, intraosseous
hemangioma, aneurysmal bone cyst, glandular odontogenic
cyst, cherubism, fibrous dysplasia, metastatic tumor in
multilocular cases and in cases of unilocular lesions,
periapical, lateral, periodontal and simple bone cysts.[4,5,9,17]
In older patients, the possibility of a malignancy should not
be ruled out.[16] The radiographic appearance of unilocular
type of OM was similar to that of the odontogenic cyst
and unilocular ameloblastoma, but the fine bone septa can
sometimes be seen in the unilocular type of OM and the
latter two lack this feature.[15] In our first case, lesion was
small with fine one or two septa internally.
Microscopically, it resembles the mesenchymal portion of
a tooth in development.[7] The lesion is not encapsulated
and exhibits the abundant extracellular myxomatous stroma
of ground substance and thin fibrils characterized by a
proliferation of a few rounded cells, fusiforms or star cells
and spindle-shaped cells. It may have a complete myxomatous
tissue or varying proportions of myxomatous and fibrous tissue.
In the latter case it can be designated either as odontogenic
fibromyxoma, in which the myxomatous element predominates;
or odontogenic myxofibroma, with a predominance of fibrous
tissue.[3,6] Small islands of odontogenic epithelial tissue can be
found scattered in stroma, sometimes being surrounded by
a narrow zone of hyalinization.[2,6] Immunocytochemicallly, all
tumor cells were found to be positive for vimentin and muscle
specific actin and negative for desmin, neuron-specific enolase,
glial fibrillary acid protein, and S100.[2,3,10] Histologic features
of our both cases were accordance to review.
Recommended therapy varies from curettage to radical
excision. Complete surgical removal can be difficult as the
lesion is not encapsulated especially in the maxilla because
the myxomatous tissue infiltrates adjacent bone tissue as
well as close proximity of vital structures and more complex
anatomy.[1,10] The prime reason for recurrence is thought to
be related to incomplete removal rather than the intrinsic
biologic behavior of the tumor.[10] These characteristics
may explain the high rate of recurrence of myxomas, which
ranges from 10% to 33% with an average of 25%.[1,9,10] Although
there are few studies regarding this, radiotherapy and
chemotherapy appear to be ineffective in controlling the
recurrent lesions. It is suggested that patients be followed
closely for at least 2 years because this is the most likely
time for recurrence.[10] Our first case had a recurrence history.
Previous incomplete surgical removal may be probable cause.
Summary and Conclusion
Two rare cases of OM of maxilla were presented. One was with
unilocular variety in the anterior region and another large
lesion with multilocularity and aggressiveness. In respect of
biological behavior and extensiveness of such lesion, better
knowledge, correlation of clinico-radiographic appearance
with histologic counterpart are mandatory for such lesions
to avoid controversies and to reach the final diagnosis and
to prevent further recurrences.
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How to cite this article: Limdiwala P, Shah J. Odontogenic myxoma of
maxilla: A review discussion with two case reports. Contemp Clin Dent
2015;6:131-6.
Source of Support: Nil. Confl ict of Interest: None declared.
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