The neoplasm typically occurs between the second and fourth decade of life, rarely before the first and after the fifth decade [7,12-14]. Its occurrence rate is reported by Kaffe et al. to be as little as 7% before the age of 10 years old and even less in children younger than 18 months [15]. Although known for their slow growth, the case of infant myxomas (younger than 2 years of age) is particular since they tend to grow faster and have a slight preference for the maxilla [15-17]. Long asymptomatic, maxillary myxomas present as a hardened, non-tender, paranasal mass, associated with nasal congestion and/or obstruction, diplopia, dysesthesia, tooth mobility or chewing difficulty, depending on the extension of the mass [16,18,19].

Imaging lacks conclusiveness in this matter and is useful for the differential diagnosis as it allows the elimination of lesions that are dangerous to biopsy such as endonasal hemangioma, meningocele or meningoencephalocele, glioma [20,21]. Radiographically, Barros et al [22] suggested a two-stage progression of radiologic patterns. In the initial stage, an osteoporotic appearance is characterized by more pronounced medullary spaces separated by thin bone septa, appearing as “soap bubble”, “tennis racket” or “honeycomb” patterns. The subsequent stage involves a destructive phase marked by substantial expansion, loss of internal locules, and perforation of the cortical bone, leading to invasion into the adjacent soft tissues. The margins can present as well-defined, poorly-defined or diffuse based on the lesion borders [15,23].

On a macroscopic level, they manifest as a sizable white-grayish mass with indistinct boundaries, containing a gelatinous component internally, and exhibiting a firm consistency [4,14,24]. Histologically, the myxoma is characterized by the presence of ovoid, stellate, and spindle cells scattered in abundant myxoid stroma and a network of reticular fibers [24-27] . Collagen is typically found in limited quantities; however, in certain instances characterized as myxofibroma, higher levels of collagen may be observed. There is no atypia, and the mitotic activity is low [11, 27-30]. It has a poor vascularization [31]. The presence of odontogenic epithelium is not essential for the diagnosis. This appearance poses a problem for the differential diagnosis with myxoid liposarcoma, rhabdomyosarcoma, fibroblastic or chondroid tumors. The range of potential diagnoses also encompasses ameloblastoma, odontogenic fibroma, giant cell granuloma, fibrous dysplasia, and dentigerous cyst in the differential diagnosis [25,30,32]. Immunohistochemically, the tumor cells express vimentin but do not express cytokeratin, Neuron-Specific Enolase (NSE), Glial Fibrillary Acidic Protein (GFAP), neurofilaments, desmin [5,6,17,19,27,33].

Myxoma exhibits resistance to chemotherapy and displays only limited responsiveness to radiotherapy [18,34-36]. Thus, surgery remains the treatment of choice. Because of its 25% rate of recurrence, some authors agree on wide excision with safety margins of 10-15mm, while others recommend the use of adjuvant cryotherapy. In some cases, enucleation or simple curettage procedures can be carried out [5,16,25,27,37,38]. Brian et al. [5] reported zero recurrence in maxillary myxomas resected without margins, based on an average follow-up period of eight and a half years. For large tumors, extensive resection with maxillectomy can be performed [7,39,40]. The selection of the surgical approach is contingent on the tumor's location and extent. Maintaining close follow-up during the initial 2 years post-excision is crucial. Some even argue for a minimum follow-up duration of 5 years to confirm disease-free status [41,42].