Myoepithelial carcinoma of the hypopharynx

Primary myoepithelial carcinoma of the hypopharynx is an extremely rare malignant tumor that was first classified as a separate neoplasm by the World Health Organization in 1991. We present an unusual case arising in a 1-year-old boy. He underwent microsuspension direct laryngoscopy with organ preservation. The tumor cells were arranged in nests and lobules separated by thin fibrovascular septae in a background of fibromyxoid stroma. Immunohistochemically, the tumor was positive for S-100 protein, AE1/AE3, and synaptophysin. The tumor was diagnosed as myoepithelial carcinoma. During follow-up for 12 months, there was no clinical or radiological evidence of recurrence or distant metastasis.