Rare Development of Primary Parotid Gland Epithelial-Myoepithelial Carcinoma in a Child

Abstract

Salivary gland tumors are uncommon in children. They consist of variable histopathological subtypes of benign and malignant tumors. EMC is a discrete entity among the WHO classification of salivary gland tumors since 1991. EMC is considered a low-grade malignant salivary gland tumor arising from intercalated ducts. Typically, it affects an adult female individual. Surgical resection with a negative margin is the mainstay treatment option. EMC has a potential for metastasis with a high rate of recurrence. Based on the available English literature, two cases of EMC diagnosed in a pediatric age group have been reported. Therefore, we describe the third EMC that developed in the left parotid gland of a young child. The diagnosis of EMC was established through histopathological examination of the total parotidectomy specimen and neck lymph node dissection, together with ancillary studies. Later, the patient suffered from cervical lymph node enlargement due to metastasis in which FNAB was taken. Metastasis from the known EMC was suspected with cytomorphological features in smears and cell block. Immunohistochemistry markers for the biphasic components were supportive of EMC. Due to advanced disease, the patient necessitated a concomitant treatment of radiochemotherapy. Besides, there was radiological evidence of bilateral multiple lung metastatic nodules. However, a biopsy was not sent for pathological confirmation.