A Rare Case of Muco-epidermoid Carcinoma Parotid in a 6-Year-Old Girl and Review of Literature

Abstract

Mucoepidermoid carcinoma (MEC) is the commonest malignant salivary gland tumor affecting the parotid gland in adults and children. In children and adolescents, there is a peak incidence in the second decade. We came across a 6-year-old girl with intermediate-grade MEC parotid gland, which is very unusual below 10 years of age. A global literature search revealed only 3 other similar cases in children below 10 years of age. She presented with a 2-year history of left parotid gradually increasing hard swelling involving the overlying skin and underlying sternocleidomastoid muscle which was confirmed on a contrast-enhanced computed tomography (CECT) scan of the face and neck as well as a core biopsy to be a MEC left parotid. The patient underwent a left radical parotidectomy sacrificing the main trunk of the facial nerve while carefully preserving its distal branches along with a left selective neck dissection (SND) followed by facial reanimation using primary neurorrhaphy. Histopathology confirmed an intermediate-grade MEC pT4aN2bMx with close deep lobe margin warranting adjuvant radiotherapy. Albeit very rare, salivary gland neoplasms may occur in children in the first decade of life. Appropriate planning regarding oncological resection with/without facial reanimation, appropriate rehabilitation followed by adjuvant treatment based on histopathology ensures a good prognosis.

Keywords: Facial reanimation; Mucoepidermoid carcinoma; Parotid malignancy; Pediatric mucoepidermoid carcinoma; Primary neurorrhaphy; Salivary gland tumors.

Fig. 1

A CECT showing a left parotid lesion involving both lobes of the parotid with widening of the stylo-mandibular tunnel (marked by red arrow). B Carefully dissected and preserved distal branches of the facial nerve, namely, marginal mandibular branch (yellow arrow), lower buccal branch (white arrow), upper buccal branch (blue arrow), and zygomatico-temporal branch (green arrow). C Reinnervated facial nerve function using spinal accessory nerve to innervate the marginal mandibular nerve. D Reinnervated masseteric nerve to innervate the zygomatico-temporal nerve branch/eye branch

Fig. 2

Branching patterns of the facial nerve