Familial occurrence of acinic cell carcinoma of the parotid gland

We report the familial occurrence of acinic cell carcinoma involving the pa rotid gland, the first such report of which we are aware. The familial occu rrence of any salivary gland neoplasm is rare. Several reports are present in the literature, including pleomorphic adenoma, Warthin tumor, carcinoma of the submandibular gland, and malignant lymphoepithelial lesion. We repor t the case of a 35-year-old man who underwent excision of a left parotid gl and acinic cell carcinoma. Eight years later, his daughter presented at the age of 16 years with a nontender parotid gland mass that was excised and f ound also to be acinic cell carcinoma. The histologic features of both neop lasms were typical of acinic cell carcinoma. While this may represent a coi ncidental event, the possibility that this familial occurrence is a manifes tation of common genetic or environmental risk cannot be excluded.