Elena Ruíz Bravo-Burguillos1*, Jose Juan Pozo Kreilinger1, Jesús Manuel Muñoz Caro3 and Antonio Ríos Parra2
1Department of Pathology. Hospital Universitario La Paz. Paseo de la Castellana, 261. 28046 Madrid. Spain
2Department of Pathology. Hospital Universitario Fundación Jiménez Díaz. Avda. Reyes Católicos, 2. 28040 Madrid. Spain
3Department of Oral and Maxillofacial Surgery. Hospital Universitario La Paz. Paseo de la Castellana, 261. 28046 Madrid. Spain
Received: 08 January, 2016; Accepted: 15 January, 2016; Published: 21 January, 2016
Elena Ruíz Bravo-Burguillos. Department of Pathology. Hospital Universitario La Paz. Paseo de la Castellana, 261. 28034 Madrid. Spain, Tel: +34 91 727 73 00; Fax: +34 91 727 70 49; E-mail:
Bravo-Burguillos ER, Pozo Kreilinger JJ, Muñoz Caro JM, Parra AR (2016) Infant Odontogenic Myxoma: Case Report and Literature Review of a Specific Entity Recently Described. Arch Otolaryngol Rhinol 2(1): 006-008. DOI: 10.17352/2455-1759.000012
© 2015 Bravo-Burguillos ER, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Myxoma; Odontogenic tumour; Myxomatous tumour; Maxillary tumour; Infant; Children; Paediatric
Odontogenic myxomas are benign mesenchymal neoplasm most of them diagnosed in adults. They are uncommon in the paediatric population and exceptional in infants, with only 21 cases reported in the literature under the age of 2 years. We present a new case of infant odontogenic myxoma, that share the same clinical and radiological presentation with the cases described in the literature. They all presented with a painless paranasal swelling of short-term evolution, usually a few weeks duration (while in children or adults tumours usually develop slowly) and a well-defined, intraosseous, expansible lytic tumour of 3 cm average size in CT-scan examination. Most cases underwent enucleation and curettage with a very low rate of recurrences (4.76%). The aim of this article is to report a new case of this exceptional tumour, whose diagnosis was established at histologic examination. We focus on the importance to undergo a conservative approach in this infant population to minimize the surgical morbidity.
Odontogenic myxomas are intraosseous benign neoplasm most of them diagnosed in adults in the third decade of life . Their frequency varies in different parts of the world between 3-20% of all odontogenic tumours. Two thirds occur in the mandible and one third in the maxilla, usually associated with a tooth germ. This tumour is uncommon in the paediatric population and exceptional in infants (under 2 years) with only 21 cases described in the literature .
Radiographically, this tumour is unilocular, with well-defined and corticated borders and most of them grow slowly. As the myxoma grows, it develops a multilocular pattern and clinically is evident a painless swelling . The larger lesions have a soap-bubble radiographic image, similar to ameloblastoma and may present poorly defined borders, with root displacements or periosteal reactions.
Histologically, these lesions shows randomly orientated spindle or stellate-shaped cells set in a mucoid or myxoid matrix with a few delicate collagen fibers . The neoplasm tends to permeate into the adjacent bony trabeculae in a pseudo-malignant pattern.
This tendency of odontogenic myxomas to permeate into marrow spaces makes effective enucleation and curettage difficult. For small neoplasm, aggressive curettage may be adequate, but large lesions may require wide resection with free margins to prevent recurrences. Recurrence rates average about 25% and usually occur during the first 2 years after excision . However, the literature review of infant odontogenic myxomas shows a low rate of recurrences after conservative surgery (4.76%) .
These tumours do not metastasize and although malignant transformation to myxosarcoma has been reported is a rare event .
A healthy 21-month-old male, with no relevant personal or family medical history, presented to our hospital with a persistent swelling on the right side of his face after a minor trauma a few weeks before. The patient had persistent swelling that had no regressed. On physical examination there was an indurated, fixed 4cm mass in his right nasolabial groove, adjacent to the right anterior maxillary wall. The lesion did not enlarge with crying. The overlying skin was normal. Eye position and extra ocular motion were normal. An intraoral examination showed obliteration of the maxillary vestibule. Clinically the lesion looked like a mucocele.
In CT-scan examination there was a low-density lesion arising within the anterior medial aspect of the left maxillary bone with erosion of the maxillary sinus and the lateral nasal wall (Figure 1). There was separation of the mass from the nasal-lacrimal duct. Taking into account the history of trauma to the area, the CT scan concluded that the lesion was compatible with post-traumatic cyst.
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