2017 Review

Journal of Dental Sciences (2017) 12, 417e420
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Case Report
Myxofibroma of the maxilla, current

concepts, and differential diagnosis
Abdulkadir Burak Cankaya a*, Mehmet Ali Erdem a, Bilge Bilgic b,
Deniz Firat a
a
Department of Oral Surgery, Faculty of Dentistry, Istanbul University, Istanbul, Turkey
b
Department of Pathology, Faculty of Medicine, Istanbul University, Istanbul, Turkey
Received 19 August 2011; Final revision received 22 September 2011

Available online 27 July 2013
KEYWORDS Abstract Odontogenic myxomas represent a small portion of all odontogenic tumors. A myx-
maxilla; oma of the bone is a rare lesion that occurs almost exclusively in the jaws. An odontogenic
myxofibroma; myxoma has a variable clinical and radiological appearance, and it should be considered in
odontogenic tumor the differential diagnosis of radiolucent and mixed radiolucenteradiopaque lesions of both
jaws in all age groups. Myxomas consist of an accumulation of mucoid ground substance with
little collagen, the amount of which determines whether it is called a myxofibroma. This paper
presents the case of a 39-year-old male with a solid whitish red, nonulcerative, nontender
expansion of both the buccal and palatal sides of the right upper alveolar bone. Results of a
radiological examination revealed a unilocular radiolucency with cortical expansion and
displacement of both the right upper second premolar and the first molar. The lesion was
totally excised, and the histopathological examination showed a myxofibroma. Healing was un-
eventful, and there was no recurrence 12 months after surgical excision. Complete removal of
the tumor, leaving no remnants attached to the soft tissue or bone, should be considered
because of the well-known potential of myxofibromas to recur.
ª 2013 Association for Dental Sciences of the Republic of China. Publishing services by Elsevier
B.V. This is an open access article under the CC BY-NC-ND license (http://creativecommons.
org/licenses/by-nc-nd/4.0/).
Introduction
Myxomas are tumors usually seen in the left atrium of the

heart, skin, subcutaneous tissues, and centrally in the
* Corresponding author. Department of Oral Surgery, Faculty of bones.1 According to the histological classification of
Dentistry, Istanbul University, Istanbul, Turkey. odontogenic tumors by Pindborg and Kramer, myxomas and
E-mail address: [email protected] (A.B. Cankaya). myxofibromas are benign tumors that infiltrate and consist
https://doi.org/10.1016/j.jds.2013.06.001
1991-7902/ª 2013 Association for Dental Sciences of the Republic of China. Publishing services by Elsevier B.V. This is an open access article under
the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
418 A.B. Cankaya et al
wholly or partly of a myxoid stroma containing loosely ar-

ranged fusiform and stellate cells with more or less long
anastomosing processes.2 The World Health Organization
classifies these tumors as benign odontogenic neoplasms
consisting of rounded and angular cells lying in an abundant
myxoid stroma.3
Myxomas represent 3e6% of all odontogenic tumors.
These slow-growing tumors consist of an accumulation of
mucoid ground substance with little collagen, the amount
of which determines whether they are called myxofi-
bromas. A myxofibroma of the bone is a rare lesion, which
occurs almost exclusively in the jaws.3,4 In some cases, a
myxoma is an aggressive tumor capable of extensive local Figure 1 Preoperative panoramic view of the lesion showing
infiltration and bone destruction, and it can spread into a typically well-circumscribed, unilocular radiolucency that
adjacent soft tissues. Although the mandible and maxilla caused displacement of the adjacent tooth roots.
are the two most common sites of head and neck myxomas,
they have also been reported in the parotid glands, nasal Under a local anesthesia, the lesion was removed with a
cavity, paranasal sinuses, nasopharynx, and eyelids. The margin of healthy tissue. Both the second premolar and the
mandible is involved more often than the maxilla, and most molar were extracted. Healing was uneventful, and there
reports show a slight predilection for females.5,6 The angle was no recurrence after 12 months (Fig. 3).
of the jaw, ramus, and adjacent molar region are most The gross pathologic examination revealed an unen-
commonly affected. The anterior mandible is involved less capsulated mass with the surrounding condensed tissue
frequently than the posterior mandible and ramus.4 Myx- often mistaken for a capsule. The resected tumor was a
omas usually occur in the 2nde4th decades of life, with a smooth, glistening, mucoid, or gelatinous lobulated mass.
peak in the 3rd decade. Because they are benign tumors Its color varied from whitish white to yellow.
with a slowly progressive course, surgical options vary from A microscopic examination showed a myxomatous tissue
conservative approaches, such as curettage or enucleation, structure composed of loosely arranged spindle cells and
to more aggressive lesions requiring a local anesthetic or en small hyperchromatic stellate cells surrounded and sepa-
bloc resection. rated by an abundant myxomatous ground substance
This article presents a case of a myxofibroma, briefly (Fig. 4). The tumor contained islands of cytokeratin (CK
reviews the pertinent literature, and suggests possible 1e3)-positive odontogenic epithelium (Fig. 5). The histo-
steps for the differential diagnosis. pathological diagnosis was a myxofibroma.
Discussion
Case presentation
When a myxofibroma is seen in the jaw, it is presumed to be
A 39-year-old male was referred to the Department of Oral associated with the dental anlage because of its close
and Maxillofacial Surgery, Faculty of Dentistry, Istanbul similarity to the mesenchymal portion of the tooth germ
University, Istanbul, Turkey, for evaluation of an expansion (i.e., the dental follicle, papillae, or periodontal ligament).
in the posterior right maxilla. The mass was slowly growing,
and the patient was referred to our hospital by a private
dentist.
The clinical examination revealed a solid, whitish red,
nonulcerative, nontender fixed swelling that had a hard
consistency and measured 2 cm 2 cm. The swelling was
located in the right posterior maxilla, and involved a part of
the buccal mucosa that approached both the buccal and
palatal sides of the alveolar bone. The texture and color of
the overlying skin were normal. The patient could recall no
pertinent traumatic or medical history.
A radiological examination showed a well-defined pear-
shaped unilocular radiolucency with cortical expansion and
displacement of both the premolar and the molar. The
radiolucency had clearly defined borders. The superior
surface was slightly scalloped and had displaced the floor of
the sinus (Fig. 1). No destruction of the root of the premolar
or the molar was seen. An axial computed tomographic
examination showed both buccal and palatal expansion of
the lesion extending from the first premolar to the second
molar and the exact borders (Fig. 2). The regional lymph Figure 2 Axial computed tomographic scan of the lesion
nodes were not palpable. showing buccal and palatal expansion.
Myxofibroma of the maxilla 419
Figure 3 Postoperative view of the lesion showing no

recurrence.
However, there are different theories regarding the role of

the odontogenic epithelial component frequently found
within myxomatous stroma on histological examinations.
Many investigators discussed whether the origin of a myx- Figure 5 Islands of odontogenic epithelium in myxomatous
ofibroma of the jaws is osteogenic or odontogenic.6,7 areas of the lesion (cytokeratin 1e3: 100).
Myxofibromas of the jaws have a better prognosis than
myxofibromas of the skeletal long bones. Most researchers racquet appearance with a well-defined or diffuse margin.
believe that a myxofibroma of the jaw should be considered They may resemble a hemangioma, ameloblastoma, central
a benign odontogenic tumor.8 giant cell tumor, or chondroma.8
Myxofibromas of the head and neck are rare. Myxofi- According to some studies, root displacement rather
bromas can be divided into central and peripheral types,3 than resorption is the rule for jaw myxofibromas. In our
with the peripheral type being rarer. It is important to case, neither the premolar nor the molar was mobile. In
determine the type, because it is correlated with the some cases, resorption of tooth roots can be observed to
treatment. Myxofibromas grow slowly without causing any varying extents,9,10 but no resorption was seen in our case.
symptoms. Because they enlarge painlessly, they can reach a There was only a painless swelling for 5 months.
considerable size prior to being noticed. Lesions can expand Radical surgery, excision, and enucleation followed by
the bone, but they perforate the cortex only if they reach a curettage of the surrounding bony tissue have all been
great size. They are locally aggressive lesions, which tend to recommended.10,11 The recommended therapy is local
recur if they are treated too conservatively. Central types conservative enucleation with adequate margins, the
can proliferate in the jaw causing bulging of the bone cortex extent of which depends on the size and location of the
and ultimately breaking through into the surrounding soft tumor, because the tumor is not encapsulated and its
tissue. These tumors grow asymptomatically, and loosened myxomatous tissue infiltrates the surrounding bony tissue
teeth may draw attention to the lesions. Otherwise, they are without causing its immediate destruction. Consequently,
usually diagnosed during routine dental examinations. conservative treatment may result in recurrence.7 To avoid
Radiologically, the appearance of the lesions can range from recurrence, after removing the tumor, we performed
a unilocular radiolucency to multilocular soap-bubble curettage, extracted the premolar and the molar, and
radiolucency, or they may have a honeycomb or tennis smoothed off the surrounding bone, which had a burr.
There has been no recurrence for approximately 12 months.
A conservative approach may be used for smaller lesions to
preserve function, reserving more radical surgery for re-
currences and larger lesions.
It remains unclear whether the origin of the lesion is
odontogenic. However, the presence of odontogenic epithe-
lium suggests that this tumor has an odontogenic origin.9 We
observed odontogenic epithelial islands scattered in the
tumor, and they were thought to be truly odontogenic.
The differential diagnosis involves any lesion with myx-
oid change. Depending on the location, soft-tissue myxofi-
bromas must be distinguished from nerve sheath tumors,
oral focal mucinosis, and pleomorphic adenomas. Nerve
sheath tumors are positive for S-100 protein, while myx-
omas are negative for this marker. Whether oral focal
mucinosis and myxofibromas are separate and distinct le-
Figure 4 Loosely arranged characteristic spindle and small sions is debatable. Those that separate the two indicated
hyperchromatic satellite cells with poorly defined cytoplasm that oral focal mucinosis, in contrast to myxofibromas, is
lying in a sparsely to mildly vascular stroma (Hematoxylin and usually approximately 1 cm in size, contains few if any
Eosin: 100). reticulin fibers, and is a noninfiltrative, circumscribed
420 A.B. Cankaya et al
lesion. Pleomorphic adenomas may at times be myxoid. 4. Sivakumar G, Kavitha B, Saraswathi TR, Sivapathasundharam B.
The presence of an epithelial ductal component and Odontogenic myxoma of maxilla. Indian J Dent Res 2008;19:
myxochondroid stroma separate them from true 62e5.
myxofibromas.12 5. Simon EN, Merkx MA, Vuhahula E, Ngassapa D, Stoelinga PJ.
Odontogenic myxoma: a clinicopathological study of 33 cases.
In conclusion, a myxofibroma is not an immediate threat
Int J Oral Maxillofac Surg 2004;33:333e7.
to life, but it is infiltrative, locally invasive, and tends to 6. DeFatta RJ, Verret DJ, Ducic Y, Carrick K. Giant myxomas of
recur if excision or curettage is incomplete. Complete the maxillofacial skeleton and skull base. Otolaryngol Head
removal of the tumor, leaving no remnants attached to the Neck Surg 2006;134:931e5.
soft tissue or bone, should be considered. Whichever sur- 7. Lo Muzio L, Nocini P, Favia G, Procaccini M, Mignogna MD.
gical approach is chosen, the patient should be observed Odontogenic myxoma of the jaws: a clinical, radiologic,
over a long term, because of the well-known potential of immunohistochemical, and ultrastructural study. Oral Surg
myxofibromas to recur. Oral Med Oral Pathol Oral Radiol Endod 1996;82:426e33.
8. Regezi JA, Schibba J. Oral Pathology: Clinical Pathologic Cor-
relations, 3th ed. Philadelphia, PA: WB Saunders, 1999:186e7
References [Chapter 7(Connective Tissue Lesions)].
9. Abiose BO, Ajagbe HA, Thomas O. Fibromyxomas of the
1. Sumi Y, Miyaishi O, Ito K, Ueda M. Magnetic resonance imaging jawbonesda study of ten cases. Br J Oral Maxillofac Surg
of myxoma in the mandible: a case report. Oral Surg Oral Med 1987;25:415e21.
Oral Pathol Oral Radiol Endod 2000;90:671e6. 10. White CS, Pharaoh JM. Oral Radiology, 5th ed. Missouri: Mosby,
2. Kramer IR, Pindborg JJ, Shear M. The World Health Organiza- 2004:433e4 [Chapter 21(Benign Tumors of the Jaws)].
tion histological typing of odontogenic tumours. Introducing 11. Li TJ, Sun LS, Luo HY. Odontogenic myxoma: a clinicopatho-
the second edition. Eur J Cancer B Oral Oncol 1993;29B: logic study of 25 cases. Arch Pathol Lab Med 2006;130:
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3. Nakano Y, Yamamoto H, Shiozaki H, et al. Peripheral myxoma 12. Medina JE. In: Barnes L, ed. Surgical Pathology of the Head and
of the right maxilla: a case and review of the Japanese liter- Neck, vols. 1 and 2. New York: Marcel Dekker, Odontogenic
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Journal of Dental Sciences (2017) 12, 417e420

Available online at www.sciencedirect.com

journal homepage: www.e-jds.com

Myxofibroma of the maxilla, current

Received 19 August 2011; Final revision received 22 September 2011

Myxomas are tumors usually seen in the left atrium of the

wholly or partly of a myxoid stroma containing loosely ar-

Figure 3 Postoperative view of the lesion showing no

However, there are different theories regarding the role of

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