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.