Pediatric Odontogenic Myxoma of the Maxilla: A Case Report and Review of the Literature

Objective: Odontogenic myxoma (OM) is a rare, benign yet locally aggressive, neoplasm that originates from the odontogenic ectomesenchyme of a developing tooth. OM is most commonly a tumor of the mandible and diagnosed in the second to fourth decade. A similar, yet frequently considered distinct entity is sinonasal myxoma (SNM). SNM has an unclear etiology, but is often seen originating in the maxilla of pediatric patients. There are very few reports of true pediatric OM of the maxilla, without clear treatment recommendations.

Methods: A case report of a pediatric OM of the maxilla, which had clear continuity with a maxillary premolar tooth root, is presented. Due to the rarity of this tumor in this age group and in this location, a literature search was conducted to determine the optimal treatment and surveillance strategies.

Results: A 15-month-old ex-27-week preemie female presented to the emergency department with epiphora and left sided facial swelling. Imaging demonstrated an erosive mass extending through the anterior maxilla, orbital floor, and lamina papyracea. Biopsy confirmed OM. She subsequently underwent definitive resection, which included a partial maxillectomy and orbitotomy with preservation of periorbita, and extraction of the source tooth. This was performed via a sublabial incision. The 3-month post-operative CT shows no evidence of disease. At 4-month follow-up she is clinically without evidence of recurrence and has noted improvement in epiphora, facial asymmetry, and feeding tolerance. A comprehensive review of the literature reveals few cases of maxillary OM in patients younger than 18 months. There is no clear treatment recommendation, with complete bony resection including 1-cm margins, enucleation with peripheral ostectomy using cryotherapy, and enucleation and curettage of surrounding bone all reported. From the adult literature, total surgical resection is considered to confer the lowest rate of recurrence, which is cited to be as high as 10%. Myxomas are not considered responsive to adjuvant therapy.

Conclusion: Odontogenic myxomas are benign odontogenic neoplasms rarely seen in the pediatric population. While multiple treatment options exist, surgical resection is superior to curettage and enucleation. However, cosmetic and functional deficits associated with wide resection must be weighed against the high risk of recurrence. Patients should have long-term surveillance because of this. Nonetheless, long-term prognosis remains very good, without the need for adjuvant therapy.

Publication History

Article published online:
15 February 2022