CASE REPORT
Pediatric odontogenic fibromyxoma of the mandible: Case report
and review of the literature
Grace C. Haser, BA,1* Henry K. Su, BA,1 Juan C. Hernandez–Prera, MD,2 Azita S. Khorsandi, MD,3 Beverly Y. Wang, MD,4 Mark L. Urken, MD1,5
1
Department of Otolaryngology, Thyroid, Head, and Neck Cancer (THANC) Foundation, New York, New York, 2Department of Pathology, Mount Sinai, New York, New York,
3
Department of Radiology, Mount Sinai Beth Israel, New York, New York, 4Department of Pathology, Mount Sinai Beth Israel, New York, New York, 5Department of Otolaryngology
– Head and Neck Surgery, Mount Sinai Beth Israel, New York, New York.
Accepted 14 April 2015
Published online 18 July 2015 in Wiley Online Library (wileyonlinelibrary.com). DOI 10.1002/hed.24090
ABSTRACT: Background. Odontogenic fibromyxoma is an uncommon
benign tumor arising from the maxillofacial bones. Fibromyxomas are
rare in children under 10 years of age. Although this tumor is reported
most frequently in the mandible for the general population, it has rarely
been reported in the mandible in children <10 years of age.
Methods. We reviewed the 39 reported cases of odontogenic fibromyx-
oma in children under the age of 10. We add 1 case to the literature.
Results. This case represents the seventh case of odontogenic fibromyx-
oma of the mandible in a child under the age of 10 years reported in the
English literature.
INTRODUCTION
Odontogenic fibromyxoma is an uncommon benign tumor
of mesenchymal origin. It predominantly arises from the
maxillofacial bones and has a greater predilection for the
mandible compared to the maxilla.1 It represents 3% to
8% of odontogenic tumors and cysts.2 These fibromyxo-
mas occur most frequently during the second through
fourth decades of life and are rare in children under 10
years of age.3 Most patients with odontogenic fibromyxo-
mas present with painless swelling; however, in some
cases, patients may present with one or more symptoms,
including loosening of the teeth, impacted teeth, maloc-
clusion,4 and paresthesias of the mandibular nerve.1
Numerous diagnostic pitfalls exist for this unusual
entity. The radiologic appearance of odontogenic fibro-
myxoma is variable and overlaps with that of head and
neck malignancies.5 Histologically, odontogenic fibro-
myxomas may resemble other myxoid lesions,3 confound-
ing pathologic diagnosis.
Odontogenic fibromyxomas may behave in a locally
aggressive manner, and local recurrence after surgical
treatment is a concern.3 The optimal management strategy
remains controversial, with some authors advocating for a
more conservative surgical approach.6
*Corresponding author: G. C. Haser, Department of Otolaryngology, Thyroid
Head and Neck Cancer (THANC) Foundation, 10 Union Square East, Suite 5B,
New York, NY 10003. E-mail: ghaser@thancfoundation.org
Peter Andersen, MD, Section Editor
Conclusion. A case of pediatric odontogenic fibromyxoma in the mandi-
ble is described. Although rare in the pediatric population, odontogenic
fibromyxomas should be included as a differential diagnostic considera-
tion when evaluating tumors of the maxillofacial skeleton. Accurate path-
ologic diagnosis is critical to ensure proper management. V C 2015 Wiley
Periodicals, Inc. Head Neck 38: E25–E28, 2016
KEY WORDS: odontogenic myxoma, odontogenic fibromyxoma, pedi-
atric tumors, mandibular tumors, maxillary tumors
In this study, we describe a child who presented with
an odontogenic fibromyxoma in the mandible. Although
this tumor is reported most frequently in the mandible
compared to the maxilla in the general population, it has
rarely been reported in the mandible in children <10
years of age.
MATERIALS AND METHODS
This study received exemption from our local institu-
tional review board. We reviewed all clinical, radiologic,
and pathologic records associated with this case.
A literature review using the terms “myxoma,”
“fibromyxoma,” “pediatric,” “maxilla,” “mandible,” and
“jaws” were searched in PubMed. References from all
identified studies were also cross referenced. All studies
written in English that reported on odontogenic myxo-
mas/fibromyxomas in the head and neck region in chil-
dren under 10 years of age were included if they
specified age of patients, tumor location, management,
and follow-up information.
Literature review
A literature review revealed 39 cases of odontogenic
myxoma or fibromyxoma in the maxillofacial bones in
children <10 years old, of which 6 were located in the
mandible7–11 (Table 1), 32 were located in the max-
illa,6,7,12–30 and 1 was located in both the maxilla and the
mandible.31
HEAD & NECK—DOI 10.1002/HED JANUARY 2016 E25
HASER ET AL.

TABLE 1. Odontogenic myxoma/fibromyxoma of the mandible in children under 10 years of age.

Author (year) Patient age, y Sex Location of tumor Treatment Follow-up Recurrence
7
Ghosh (1973) 5 M Mandible Excision of tumor, hemimandibulectomy 25 y No
Ghosh (1973)7 2 M Mandible Excision of tumor, hemimandibulectomy 24 y No
Ataman (1993)8 7 M Mandible Curettage 1y No
Li (2006)9 7 M Mandible Enucleation and curettage 7y No
Shahoon (2009)10 8 M Mandible En bloc resection 3y No
Mauro (2013)11 6 M Mandible Enucleation and curettage 6 mo No

CASE REPORT fibromyxoid sarcoma. The biopsy was diagnostic for

A 6-year-old boy presented with a mass in the left
mandible with direct extension into the left neck, which
had reportedly been growing for 6 months. The patient
had no complaints of pain, fever, chills, or loss of
appetite.
An MRI of the neck revealed an expansile lesion of the
left hemimandible, extending from the level of the sym-
physis to the body with involvement of the proximal por-
tion of the left ramus of the mandible. The tumor
encompassed the course of the inferior alveolar nerve
canal and extended into the left submandibular space
without extension to the floor of the mouth. It remained
deep to the platysma muscle. None of the lymph nodes
identified in either side of the neck met the criteria for
significant adenopathy (Figure 1).
An incisional biopsy was performed and showed a spin-
dle cell proliferation in a myxoid background. The tumor
cells lacked pleomorphism with no overt mitotic activity.
Immunohistochemical stain showed that the tumor cells
were negative for smooth muscle actin, desmin, S-100
protein, and CD34. Interphase fluorescence in situ hybrid-
ization studies were negative for FUS gene rearrange-
ment, excluding the differential diagnosis of low grade
myxoma/fibromyxoma.
The patient underwent a hemimandibulectomy and
reconstruction with a fibular free flap. Because of the
location of the tumor, it was necessary to sacrifice the
inferior alveolar nerve, which was grafted with a sural
nerve interposition graft. The submandibular gland was
removed as part of the specimen. The fibular free flap
was held in place with a rigid fixation plate. The patient
tolerated the operation well.
The resection specimen showed a 5.8 cm well-
circumscribed, encapsulated mass arising from the mandib-
ular bone (Figure 2). The mass exhibited a gelatinous trans-
lucent cut surface with focal white rubbery areas. The
histological sections showed a spindle-to-stellate shaped
cell proliferation in a variable myxoid and collagenized
stroma. The morphology of the cells was bland and
revealed no significant nuclear pleomorphism or increased
mitotic activity. No tumor necrosis was present. Residual
bony trabeculae were observed. A small rest of the odonto-
genic epithelium was identified, confirming the diagnosis
of an odontogenic myxoma/fibromyxoma (Figure 3). Surgi-
cal resection margins were negative for tumor.
The patient’s rigid fixation plate was scheduled to be
removed 3 months after the initial surgery to ensure

FIGURE 1. MRI. Axial T2-weighted (A) and coronal contrast-enhanced (B) fat-suppressed images demonstrate an expansile, septated, enhancing
lesion of the body, and symphysis of the mandible (arrows). This lesion is hyperintense on T2-weighted images and shows enhancement on
administration of contrast. Low signal septation of this lesion is noted.

E26 HEAD & NECK—DOI 10.1002/HED JANUARY 2016


FIGURE 2. Lateral view of the resected left mandible. The mass is
well-circumscribed and encapsulated and arises from the man-
dibular bone.
proper growth and development. The patient will undergo
placement of dental implants at that time.
DISCUSSION
The incidence of odontogenic fibromyxoma is highest in
the second, third, and fourth decades of life.3 Odontogenic
fibromyxomas are rarely reported in children under 10
years or adults over 50 years.3 In a review of 164 cases of
odontogenic fibromyxoma between 1965 and 1995, Kaffe
et al5 reported only 12 cases (7.3%) in patients under 10
years old. Some studies have found these tumors more fre-
quently in adult women than in men, although others have
found roughly equal ratios.1 In a study of 10 pediatric
patients, Keszler et al2 could not establish a statistically sig-
nificant sex distribution. This tumor is presumed to be
FIGURE 3. (A) Medium magnification of the mandibular mass
shows a bland spindle cell proliferation in a myxoid background
(hematoxylin-eosin stain, original magnification 3200). (B) A
focus of odontogenic epithelium rest surrounded by tumor cells
(hematoxylin-eosin stain, original magnification 3400).
PEDIATRIC ODONTOGENIC FIBROMYXOMA OF THE MANDIBLE
odontogenic in origin because of the near exclusive inci-
dence in tooth-bearing regions of the maxillofacial bones.3
Thus, odontogenic fibromyxoma most commonly arises in
the molar and premolar regions of the mandible or max-
illa,3 although it has been found in other areas and even
other bones, such as the lateral skull base.32 It is more com-
mon in the mandible than in the maxilla5; however, our
review of the literature found only 6 cases of mandibular
odontogenic fibromyxoma in pediatric patients under 10
years of age compared with 32 cases of maxillary odonto-
genic fibromyxoma in the same population (Table 1).
Hence, the current case represents the seventh case reported
in the English literature. There was additionally 1 case of a
patient with odontogenic fibromyxomas in both the mandi-
ble and the maxilla.31 Other authors have noted that odon-
togenic fibromyxomas have occurred exclusively in the
maxilla in infants21,30; however, the basis of this observa-
tion is unclear. Moreover, given the small sample size of
this review, it is not possible to establish a trend.
Histologically, odontogenic fibromyxoma is composed
of randomly oriented, cytologically bland stellate-to-
spindle shaped cells embedded in the mucoid or myxoid
stroma. Some tumors exhibit a prominent collagenous
stoma and are designated as myxofibroma/fibromyxoma.
Nests of odontogenic epithelium may be present but are
not required for establishing the diagnosis.33 On gross
pathology, fibromyxomas are generally gelatinous, unen-
capsulated, and well-delineated masses.3 The radiographic
appearance is variable; however, most are radiolucent and
multilocular with well-defined borders.5
Because odontogenic fibromyxomas are rare and may
be misinterpreted as other benign myxoid tumors or as
malignant tumors, an accurate diagnosis is essential.
Patients with odontogenic fibromyxoma generally present
with painless swelling and/or deformity of the maxillofa-
cial region.4 Occasionally, patients present with mild
symptoms, most often involving dentition.4 This symp-
tomatology mimics ameloblastoma, central giant cell
granuloma, and hemangioma.34
There has been some debate about the surgical man-
agement for odontogenic fibromyxomas. Complete surgi-
cal resection is the standard approach for management of
this tumor, given the high recurrence rates after less
extensive procedures.3 However, Kansy et al6 have
argued for conservative approaches in children and
adults, except in incidences of recurrence. Because of the
aggressive nature of this tumor, a segmental mandibulec-
tomy with fibular free flap reconstruction was performed
in this case.
Although most clinically evident recurrences occur
within 2 years, in some patients, a recurrence was discov-
ered several years after surgery3; therefore, long-term fol-
low-up is recommended.
CONCLUSION
We have described a case of pediatric odontogenic
fibromyxoma in the mandible. Although rare in the pedi-
atric population, odontogenic fibromyxomas should be
included as a differential diagnostic consideration when
evaluating tumors of the maxillofacial skeleton. Accurate
pathologic diagnosis is critical to ensure proper
management.
HEAD & NECK—DOI 10.1002/HED JANUARY 2016 E27
HASER ET AL.
REFERENCES
1. Slootweg PJ, Wittkampf AR. Myxoma of the jaws. An analysis of 15 cases.
J Maxillofac Surg 1986;14:46–52.
2. Keszler A, Dominguez FV, Giannunzio G. Myxoma in childhood: an anal-
ysis of 10 cases. J Oral Maxillofac Surg 1995;53:518–521.
3. Brannon RB. Central odontogenic fibroma, myxoma (odontogenic myx-
oma, fibromyxoma), and central odontogenic granular cell tumor. Oral
Maxillofac Surg Clin North Am 2004;16:359–374.
4. Harder F. Myoxomas of the jaws. Int J Oral Surg 1978;7:148–155.
5. Kaffe I, Naor H, Buchner A. Clinical and radiological features of odonto-
genic myxoma of the jaws. Dentomaxillofac Radiol 1997;26:299–303.
6. Kansy K, Juergens P, Krol Z, et al. Odontogenic myxoma: diagnostic and
therapeutic challenges in paediatric and adult patients–a case series and
review of the literature. J Craniomaxillofac Surg 2012;40:271–276.
7. Ghosh BC, Huvos AG, Gerold FP, Miller TR. Myxoma of the jaw bones.
Cancer 1973;31:237–240.
8. Ataman M, Sarioglu T, Ayas K. Myxoma of the mandible. Int J Pediatr
Otorhinolaryngol 1993;27:183–186.
9. Li TJ, Sun LS, Luo HY. Odontogenic myxoma: a clinicopathologic study
of 25 cases. Arch Pathol Lab Med 2006;130:1799–1806.
10. Shahoon H, Esmaeili M, Nikhalat M, Farokhi E. Odontogenic fibromyx-
oma and odontogenic cyst in an eight year old boy: three-year follow-up.
J Dent Res Dent Clin Dent Prospects 2009;3:103–105.
11. Mauro A, Lipari L, Tortorici S, Leone A, Gerbino A, Buscemi M. Expres-
sion of MMP-2 and MMP-9 in odontogenic myxoma in a child: report of a
clinical case. Odontology 2013;101:233–238.
12. Dutz W, Stout AP. The myxoma in childhood. Cancer 1961;14:629–635.
13. Canalis RF, Smith GA, Konrad HR. Myxomas of the head and neck. Arch
Otolaryngol 1976;102:300–305.
14. Harris RJ, Garrow E, Spinnato G. Myxoma of the maxilla: report of case.
J Oral Surg 1977;35:70–73.
15. James DR, Lucas VS. Maxillary myxoma in a child of 11 months. A case
report. J Craniomaxillofac Surg 1987;15:42–44.
16. Leiberman A, Forte V, Thorner P, Crysdale W. Maxillary myxoma in chil-
dren. Int J Pediatr Otorhinolaryngol 1990;18:277–284.
17. Ang HK, Ramani P, Michaels L. Myxoma of the maxillary antrum in chil-
dren. Histopathology 1993;23:361–365.
18. Brewis C, Roberts DN, Malone M, Leighton SE. Maxillary myxoma: a rare
midfacial mass in a child. Int J Pediatr Otorhinolaryngol 2000;56:207–
209.
E28 HEAD & NECK—DOI 10.1002/HED JANUARY 2016
19. Fenton S, Slootweg PJ, Dunnebier EA, Mourits MP. Odontogenic myxoma
in a 17-month-old child: a case report. J Oral Maxillofac Surg 2003;61:
734–736.
20. Wachter BG, Steinberg MJ, Darrow DH, McGinn JD, Park AH. Odonto-
genic myxoma of the maxilla: a report of two pediatric cases. Int J Pediatr
Otorhinolaryngol 2003;67:389–393.
21. Rotenberg BW, Daniel SJ, Nish IA, Ngan BY, Forte V. Myxomatous
lesions of the maxilla in children: a case series and review of management.
Int J Pediatr Otorhinolaryngol 2004;68:1251–1256.
22. Prasannan L, Warren L, Herzog CE, Lopez–Camarillo L, Frankel L,
Goepfert H. Sinonasal myxoma: a pediatric case. J Pediatr Hematol Oncol
2005;27:90–92.
23. King TJ III, Lewis J, Orvidas L, Kademani D. Pediatric maxillary odonto-
genic myxoma: a report of 2 cases and review of management. J Oral Max-
illofac Surg 2008;66:1057–1062.
24. Iatrou IA, Theologie–Lygidakis N, Leventis MD, Michail–Strantzia C.
Sinonasal myxoma in an infant. J Craniofac Surg 2010;21:1649–1651.
25. Singaraju S, Wanjari SP, Parwani RN. Odontogenic myxoma of the max-
illa: a report of a rare case and review of the literature. J Oral Maxillofac
Pathol 2010;14:19–23.
26. Kaymakci M, Yazici S. Odontogenic myxoma of the maxilla. Clin Pract
2011;1:e136.
27. Rıos Y Valles–Valles D, Vera–Torres AM, Rodrıguez–Martınez HA,
Rodrıguez–Reyes AA. Periocular myxoma in a child. Case Rep Ophthal-
mol Med 2012;2012:739094.
28. Chen HH, Streubel SO, Durairaj VD. Odontogenic myxoma with orbital
involvement. Ophthal Plast Reconstr Surg 2013;29:e47–e49.
29. Zainine R, Mizouni H, El Korbi A, Beltaief N, Sahtout S, Besbes G. Maxil-
lary bone myxoma. Eur Ann Otorhinolaryngol Head Neck Dis 2014;131:
257–259.
30. Kadlub N, Mbou VB, Leboulanger N, et al. Infant odontogenic myxoma: a
specific entity. J Craniomaxillofac Surg 2014;42:2082–2086.
31. Kleiber GM, Skapek SX, Lingen M, Reid RR. Odontogenic myxoma of the
face: mimicry of cherubism. J Oral Maxillofac Surg 2014;72:2186–2191.
32. Klimo P Jr, Jha T, Choudhri AF, Joyner R, Michael LM 2nd. Fibromyxoma
of the lateral skull base in a child: case report. J Neurol Surg Rep 2013;74:
105–110.
33. Buchner A, Odell EW. Odontogenic myxoma/fibromyxoma. Lyon, France:
IARC Press; 2005.
34. Landa LE, Hedrick MH, Nepomuceno–Perez MC, Sotereanos GC. Recur-
rent myxoma of the zygoma: a case report. J Oral Maxillofac Surg 2002;
60:704–708.