Familial clustering of nasopharyngeal carcinoma in a non-endemic geographical region. Report of two Hungarian cases and a review of the literature.

The aim of this study was to investigate the familial clustering of nasopharyngeal carcinoma (NPC) in a non-endemic geographical region on the basis of two case reports and a review of the literature. Following an upper respiratory infection, NPC (WHO type III) was detected in a 57-year-old female (Case 1) who presented with nasal symptoms and a year later in her 36-year-old son (Case 2) who presented with enlarged lymph nodes. After a full diagnostic work-up, cT2a cN0 cM0 (stage IIA; Case 1) and cT2a cN2 cM0 (stage III; Case 2) disease were identified, and telecobalt irradiation was administered to both patients. The mother achieved complete remission and has been disease-free during a 14-year follow-up period. After initial complete remission, the son experienced regional (cervical) and base of the skull relapses within 2 years, which were treated unsuccessfully by means of radical neck dissection, a second course of radiotherapy and chemotherapy. Epstein-Barr virus (EBV) was detected in pathology sections from both patients. The authors review 20 additional well-documented cases of familial clustering of NPC in non-endemic geographical regions from the English language literature. This clinical entity typically has WHO type III histology; it may occur following an upper respiratory tract infection, and EBV-related serological titers were elevated in all 20 investigated cases. No consequent promoting factors were identified. The present two cases and the review of the literature strongly suggest that familial clustering of NPC in non-endemic geographical areas may be related to EBV infections. The difference in outcome of our two cases may be explained by the fact that the disease in Case 2 was diagnosed 1 year later than that in Case 1 and hence at a more advanced stage.

[PET scan in patients with epipharyngeal tumors]. / Epipharynx-daganatok PET-vizsgálata.

Whole-body FDG PET examinations in 10 cases of epipharyngeal tumour (8 males, 2 females, mean age: 48 years) have been performed since January 1999. The PET examinations were aimed at the accurate staging, follow-up of the patients after the treatment, identification of recurrencies and localization of the unknown primary tumor. Functional imaging resulted in "upstaging" in 3 patients as compared to staging by the conventional diagnostic tools. Four additional patients with hyperplastic epipharyngeal tissue were investigated for occult primary cancer after negative results of multiple excisions, resulting in one case of primary epipharyngeal cancer. Correct staging, early detection of recurrencies, localization of occult primary tumor and the better post-therapeutic assessment of epipharyngeal masses all facilitate a more reasonable therapeutic approach, which may improve survival results.