Annals of Oncology Advance Access originally published online on February 23, 2006
Annals of Oncology 2006 17(5):794-800; doi:10.1093/annonc/mdl015
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© 2006 European Society for Medical Oncology
NK/T-cell lymphomas ‘nasal type’: an Italian multicentric retrospective survey
1 Istituto di Ematologia, Università Cattolica del Sacro Cuore, Roma; 2 Divisione di Ematologia, Ospedale di Cuneo; 3 Divisione di Ematologia, Ospedale di Montefiascone; 4 Istituto di Ematologia, Università di Verona; 5 Ematologia, Istituto Regina Elena Roma; 6 Divisone di Ematoncologia Medica, Istituto Europeo di Oncologia, Milano; 7 Istituto di Ematologia, Università di Palermo; 8 Istituto di Ematologia, Università ‘La Sapienza’, Roma; 9 Divisione di Ematologia, Ospedale Niguarda, Milano; 10 Divisione di Ematologia, Ospedale di Vicenza; 11 Reparto di Epidemiologia Clinica, Istituto Superiore della Sanità, Roma; 12 Istituto di Anatomia Patologica, Università Cattolica del Sacro Cuore, Roma, Italy
* Correspondence to: Dr L. Pagano, Istituto di Ematologia, Università Cattolica del Sacro Cuore, Largo Francesco Vito, 1, I-00168 Roma, Italy. Fax +39-063051343; E-mail: lpagano{at}rm.unicatt.it
Objective: To evaluate the clinical characteristics and outcome of NK/T-cell lymphoma ‘nasal type’ developed in Italian patients.
Patients: Between 1997 and 2004, 26 new cases of NK/T-cell lymphoma ‘nasal type’ were diagnosed in 10 Italian Hematology institutions.
Results: All patients were Caucasian, male/female ratio was 19/7, with a median age of 50 years (range 20–80). In 23 cases presentation at the onset was in the nasal cavity or adjacent structures, in two cases the lymphoma onset with skin lesions was followed successively by rhynopharyngeal dissemination, while the remaining case had bone marrow and lymph node involvement followed by oro-pharyngeal involvement. Regarding the stage of disease: 12 patients were in stage I; six in stage II; eight in stage IV. Diagnosis was based on the finding of a NK/T-cell phenotype at the histological and immunophenotypic examination of oropharyngeal or cutaneous lesions. All patients but one were treated with chemotherapy, alone in nine cases or associated to radiotherapy in 14 cases; two patients had chemotherapy, radiotherapy and surgery, while one patient underwent only surgery. Chemotherapy was anthracycline-based in 17 out of 25 cases. In those patients in whom radiotherapy was performed, radiation dosages ranged between 36 Gy and 47.5 Gy, with a median dosage of 40 Gy. Nine patients (34%) were responsive to the treatments: six patients obtained a complete remission and other three a partial remission. The remaining 17 patients resulted refractory or presented a limited response to therapy. The median disease-free survival was 14 months and the median overall survival time was 9 months.
Conclusion: The results of this retrospective survey confirmed that NK/T-cell lymphoma ‘nasal type’ is a very rare lymphoma in the Italian population, and it is characterized by a very bad prognosis. Due to the rarity of this disease, a standardized therapeutic approach is lacking. More data are needed to know the epidemiology of this kind of lymphoma in Europe.
Key words: NK/T cell, lymphoma treatment
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