Familial adenoid cystic carcinoma of sublingual salivary glands

Tumors of the sublingual salivary gland are extremely rare. Most of the sublingual tumors are malignant, adenoid cystic carcinoma (ACC) and mucoepidermoid carcinoma being the most common histological types. Here we report the first case of a familial occurrence of ACC in a family in which the father and daughter were treated for ACC of the sublingual salivary gland. A 75-year-old man was referred with a swelling of the floor of the mouth on his right side and diagnosed as ACC of sublingual salivary gland synchronous with adenocarcinoma of the lung. One year later, his daughter presented, at the age of 46 years, with a swelling of the floor of the mouth on her right side, which was also diagnosed as ACC. This is the first case of familial recurring ACC of sublingual salivary gland worldwide.