Familial and sporadic cases of nasopharyngeal carcinoma in Taiwan

Abstract

Background: Nasopharyngeal carcinoma (NPC) has a striking geographic/ethnic distribution, with especially high rates among southern Chinese. Previous studies have indicated that a family history of NPC is associated with increased risk and noted familial clustering in low-risk populations.

Materials and methods: We investigated differences between sporadic and familial cases of NPC in a case-control study of 375 histologically confirmed NPC cases (99% response rate) and 328 age-, sex-, and geographically-matched controls (88% response rate). All participants answered a detailed risk factor interview and donated blood for EBV and CYP 2E1 testing.

Results: Subjects with a first degree relative with NPC had on odds ratio (OR) of 7.6 (95% confidence interval (CI) = 2.3-25), while those with a family history of any other cancer had only a slightly elevated risk of disease (OR = 1.4; 95% CI = .93-2.2). Of the cases, 25 (6.7%) were familial--having at least one first degree relative with NPC. No significant difference was seen between familial and sporadic cases with respect to sex, age, ethnicity, histology or stage. There was a nonsignificant (p = 0.16) increase in T1N2 tumors among familial cases, suggesting a more aggressive tumor. Family history of other cancers, EBV serologies, or the distribution of the RsaI c2 form of the allele of cytochrome P450 2E1 were also not significantly different between the two groups.

Conclusions: In conclusion, while genetic factors are likely to play an important role in NPC pathogenesis, our results provide little evidence that a familial form of NPC exists with characteristics notably distinct from sporadic cases.