Primary acinic cell carcinoma in an adolescent female

Acinic cell carcinoma (ACC) is a rare, low-grade tumour, accounting for about 17% of all primary salivary gland malignancies in adults. ACC most commonly affects the parotid gland, predominating fifth and sixth decades of life and a female predilection. In the paediatric population, mucoepidermoid carcinoma is the most common parotid malignancy, followed by ACC.

We report a case of a 16-year-old female, presenting with a slow-growing right parotid lump. Clinical examination revealed a small right parotid nodule, grade I House-Brackmann score and cervical lymphadenopathy. Radiological investigations revealed a well-circumscribed lobulated lesion in the anterior right parotid gland and reactive lymph nodes. Surgical management included a right partial parotidectomy. Immunohistochemistry was negative for S100, positive with DOG-1 and widespread PAS-D-resistant granules throughout the tumour cells, confirming a diagnosis of ACC.

ACC is an important differential diagnosis to consider in salivary gland pathology in the female adolescent patient.