Odontogenic Myxoma in a Patient with MEN2A Syndrome

Abstract

Introduction

Odontogenic myxoma is considered to originate from the odontogenic ectomesenchyme. The tumor can involve any age group, but is predominantly seen in young adults. Here we present a patient with odontogenic myxoma with a history of multiple endocrine neoplasia type 2A (MEN2A). To the best of our knowledge, odontogenic myxoma has not been previously reported in a patient with a MEN2A history.

Case presentation

A 14-year-old female with a known history of MEN2A, presented with a painless bony expansion and radiolucency with fine residual bone trabeculae arranged at right angles to one another between teeth #4 and #5. Upon biopsy a diagnosis of odontogenic myxoma was rendered. MEN2A is an autosomal dominant syndrome characterized by clinically aggressive medullary thyroid carcinoma (MTC), pheochromocytoma, parathyroid hyperplasia, or adenoma. Therefore, as the standard of care for the MTC includes prophylactic total thyroidectomy, it was undertaken in this patient at an early age. For the odontogenic myxoma, the patient was treated with enucleation and curettage. At 2 years post-resection follow-up, the patient was disease-free.

Conclusion

It is unclear if the odontogenic myxoma in this patient was associated with previously diagnosed MEN2A syndrome or incidental, but the possibility of associated mesenchymal tissue tumors should be considered in patients with history of syndromic conditions. Although odontogenic myxoma is a benign lesion, because of its high recurrence rate and locally aggressive clinical behavior, periodic follow-up is necessary for at least 5 years post-treatment. Despite the high recurrence rate of 25% as reported in the literature, the prognosis largely remains good.