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Familial clustering of parotid gland lymphoepithelioma in North America
Thien-Tuong-Vi Vu1, Anthony G Zeitouni, Pantelis Tsinalis
1Department of Otolaryngology-Head and Neck Surgery, The Royal Victoria Hospital, McGill University, Montreal, Quebec.
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.
