BACKGROUND: Nasopharyngeal carcinoma (NPC) is a malignancy that is prominent in Cantonese Chinese people. It is presumed to result from an interaction of genetic and environmental factors, including the Epstein-Barr virus (EBV). In an attempt to further clarify the pathogenesis of this disease, an evaluation of NPC occurring in racial/ethnic groups not considered susceptible to this disease could be informative. METHODS: A white family with NPC occurring in three siblings was investigated and information was gleaned from literature on other reports of familial NPC in non-Chinese families. RESULTS: In the family being investigated, another genetically determined disease, hemophilia, was identified. Radiation early in life was noted to be a possible risk factor for NPC in the proband. A review of familial NPC in the white population revealed that in contrast to sporadic NPC, which is usually of the well-differentiated type, familial NPC usually is poorly differentiated. CONCLUSIONS: Familial NPC offers an important opportunity to investigate the etiology of this disease. With newer laboratory techniques to investigate pathogenetic mechanisms, detailed evaluations of non-Chinese NPC families may become increasingly important.
BACKGROUND:Nasopharyngeal carcinoma (NPC) is a malignancy that is prominent in Cantonese Chinese people. It is presumed to result from an interaction of genetic and environmental factors, including the Epstein-Barr virus (EBV). In an attempt to further clarify the pathogenesis of this disease, an evaluation of NPC occurring in racial/ethnic groups not considered susceptible to this disease could be informative. METHODS: A white family with NPC occurring in three siblings was investigated and information was gleaned from literature on other reports of familial NPC in non-Chinese families. RESULTS: In the family being investigated, another genetically determined disease, hemophilia, was identified. Radiation early in life was noted to be a possible risk factor for NPC in the proband. A review of familial NPC in the white population revealed that in contrast to sporadic NPC, which is usually of the well-differentiated type, familial NPC usually is poorly differentiated. CONCLUSIONS: Familial NPC offers an important opportunity to investigate the etiology of this disease. With newer laboratory techniques to investigate pathogenetic mechanisms, detailed evaluations of non-Chinese NPC families may become increasingly important.
Authors: Nehla Mokni Baizig; Patrice Morand; Jean Marie Seigneurin; Hamouda Boussen; Asma Fourati; Said Gritli; Zeineb Oueslati; Slim Touati; Amor Gamoudi; Mansour Ben Abdallah; Michèle El May; Ahmed El May Journal: Eur Arch Otorhinolaryngol Date: 2011-07-30 Impact factor: 2.503
Authors: Wai Tong Ng; Cheuk Wai Choi; Michael C H Lee; Siu Hong Chan; Tsz Kok Yau; Anne W M Lee Journal: Fam Cancer Date: 2008-08-26 Impact factor: 2.375