Familial clustering of parotid gland lymphoepithelioma in North America.

OBJECTIVE To describe genetic susceptibility in the first familial clustering of parotid gland lymphoepithelioma reported in North America. DESIGN Retrospective study. SETTING Tertiary care institution with outreach to northern Quebec. METHODS Chart, family history, literature review, and c-kit assay. MAIN OUTCOME MEASURE C-kit assay. RESULTS Four family members of both sexes over two generations had lymphoepithelioma of the parotid gland, without involvement of the nasopharynx. Two c-kit assays were positive of the three cases that could be done. Association with Epstein-Barr virus has been demonstrated in the oncogenesis of lymphoepithelioma. CONCLUSION Genetic susceptibility and common familial environmental factors play a role in the development of parotid gland lymphoepithelioma. A complete family history should be obtained in all cases of lymphoepithelioma of the parotid gland.