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.


METHODS
Retrospective study.


METHODS
Tertiary care institution with outreach to northern Quebec.


METHODS
Chart, family history, literature review, and c-kit assay.


METHODS
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.


CONCLUSIONS
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.
Vu
Thien-Tuong-Vi
TT
Department of Otolaryngology-Head and Neck Surgery, The Royal Victoria Hospital, McGill University, Montreal, Quebec.
Zeitouni
Anthony G
AG
Tsinalis
Pantelis
P
Foulkes
William D
WD
Hagr
Abdulrahman
A
D000328:Adult; D000368:Aged; D002294:Carcinoma, Squamous Cell / Q000235:genetics* / Q000473:pathology; D005190:Family; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D008214:Lymphocytes / Q000473:pathology; D008297:Male; D008875:Middle Aged; D010307:Parotid Neoplasms / Q000235:genetics* / Q000473:pathology; D011792:Quebec; D012189:Retrospective Studies