Familial occurrence of pleomorphic adenoma

Pleomorphic adenoma is the most common neoplasm found in the parotid gland. The familial occurrence of any salivary gland neoplasm is rare. There are 8 previous reports in the literature of familial occurrence of salivary gland tumors. Two of these report the occurrence of pleomorphic adenoma of the parotid gland in siblings; all 4 patients were adults. We report here an 11-year-old male who presented with a left parotid mass and underwent parotidectomy. The mass proved to be a pleomorphic adenoma. His sister presented 9 years later at the age of 15 years with a similar mass also in the left parotid area. She underwent superficial parotidectomy which revealed the mass to be a pleomorphic adenoma. We believe these are the youngest siblings reported to date in which familial pleomorphic adenoma has occurred.