RESUMO
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.
