NASAL-PARANASAL-ORONASOPHARYNGEAL LYMPHOMAS IN CHILDHOOD: The Role of Staging System on Prognosis

Most of the patients with nasal-paranasal and oropharyngeal-nasopharyngeal (NPONP) lymphomas had early-stage disease according to the Murphy system. But the survival rates are not correlated with the stages. Treatment results were analyzed to see the effects of the staging in NPONP lymphomas. Fifty-five children (median age 8 years, M/F: 4.5) with NPONP lymphoma were included in this study. The Murphy staging system was used at diagnosis and all cases were restaged according to the TNM system: primary tumor, regional lymph node, and metastasis. The survival rates were analyzed by grouping the patients according to the treatment and stages. The disease was located in Waldeyer's ring, the sinonasal region, and the nasopharynx in 45.4, 27.3, and 27.3% of patients, respectively. Thirty-nine patients had stage I or II disease according to the Murphy system. When the TNM system was used, 92% of these patients were upstaged to stage III-IV. Five-year overall and event-free survival rates were 52.1 and 50.4%, respectively, for the whole group. Five-year event-free survival rates for Murphy stage I, II, and III disease were 66.7, 56.9, and 45.4%, respectively. The rates for TNM stage III and IV patients were 64.3 and 43.8%. Treatment protocols were intensified in most of the early-stage disease treated with modified LSA2-L2 regimen and better survival rates were obtained in these patients. The intensification of the treatment by using intrathecal treatment and doxorubicin in patients with early-stage disease at NPONP location seems effective. In conclusion, the Murphy staging system is not suitable for the staging of NPONP lymphomas. It should be revised to predict the prognosis and decision-making for treatment.