Familial Nasopharyngeal Carcinoma 6

272 Y. Guo and B. S. Glisson11.4 months is in question owing to short follow-up.Although response rate was low in this refractorypopulation, responses were durable and irinotecancould be further studied in this setting.21.2.6Long-Term SurvivalChemotherapy confers short-term palliative benefitfor the majority of patients with metastatic NPC.There is a very small subset of patients, however, whoobtain long-term disease control greater than 3–5years and apparent “cure,” most commonly with multimodaltreatment. In a series reported by Fandi et al.(2000), the most common site of metastatic disease inlong-term survivors was bone followed by lung.Radiation to involved bony sites and surgery, in thecase of pulmonary metastases, were frequently utilizedin addition to chemotherapy. Radiation can alsobe applied to aid in control of mediastinal metastases.Teo et al. evaluated prognostic factors in a case seriesof 289 patients with recurrent/metastatic NPC (Teoet al. 1996). Survival greater than 5 years was observedin four patients, all of whom were