Acinic cell carcinoma (ACC) is a slow growing and low grade epithelial neoplasm of the salivary glands, in which few tumor cells exhibiting acinar cell differentiation that is characterized by presence of cytoplasmic zymogen secretory granules in the tumor cell. Most frequently ACC occur in parotid gland with the initial presentation somewhat at younger age than other parotid gland neoplasms and preferentially more in female with female: male ratio is 3:2 and median age at time of presentation is 52 years. “Fine needle aspiration cytology (FNAC)” examination may also be useful tool for confirmation of the diagnosis. The treatment of tumor is essentially by surgical excision of the gland. In histo-pathological examination; the most common presentation of tumor is solid sheet and nesting pattern of the growth followed by microcystic, papillary-cystic and follicular pattern . The purpose of the present case is to present the clinical, cytological and histopathological features of of ACC of the parotid glands of 18 years old male child which initially present as a benign cystic lesion of parotid gland than finally diagnosed as ACC with metastasized to regional lymph nodes. To conclude, ACC is frequently arising in parotid gland and metastasis to the intra-salivary gland lymph nodes is a very rare finding in young male. Intra-operative frozen section might be helpful in the diagnosis of parotid gland ACC because of low specificity and sensitivity of CT scan and FNAC examination of ACC. The histo-morphological examination of paraffin embedded H/E stained section is the main tool to explain the microscopic findings of ACC and to classify ACC of parotid gland. Specail stain like PAS, PAS-D and IHC examination might useful for diagnosis of ACC. Surgery with adjuvant post-operative radiation therapy is acceptable for final outcome of disease and the tumor should be managed uncompromisingly, despite of its slow growing and low malignant behaviour, due to its metastatic prospective which usually confirmed by distant metastasis and recurrence. Hence, these tumors require a long-term follow-up.
Key words: ACC: Acinic cell carcinoma