T/NK “Nasal Type” Lymphomas: An Italian Cooperative Retrospective Survey.
Authors: Pagano Livio , Gallamini Andrea , Trapè Giulio , Mattei Daniele , Di Bona Eros , Iannitto Emilio , Spadea Antonio , Rossi Elena , Tartaglione Raffaele , Martelli Maurizio , Nosari Anna Maria , Petti Concetta , Falcucci Paolo , Larocca Luigi Maria , Leone Giuseppe 
Published: April 2020

Abstract
T/NK “nasal type” lymphoma is a neoplasm that shows a peculiar geographical distribution. In fact most of the reported cases come from Far Eastern countries such as Hong Kong, and Central America, particularly from Mexico; this kind of lymphoma occurrs rarely in western countries.
Between 1997 and 2004, 19 new cases of T/NK nasal type lymphoma were diagnosed in 7 Italian Hematology Division. Patients characteristics were as follows: all caucasian; 14 men and 5 women; median age 53 years (range 20–80). The presentation at the onset was in the nasal cavity or adjacent structures in 15 cases, who presented symptoms like nasal obstruction, dysphagia, orbital edema; in 2 cases, skin lesions were the first sign of disease, followed by oropharingeal involvement; in 1 case, the patient was studied because of migrating bone pain, and in the last patient lymphoma presented as a right leg tumor. Considering the stage of disease, 10 patients presented a I stage (in 8 cases IA, 1 IB, and 1 IE); 3 patients were in stage II (2 IIB, 1 IIE); 6 patients presented an advanced stage disease (3 IVA, 3 IVB). Thus, early stage patients were 13, while advanced stages were 6. The median time from the onset of signs, as skin lesions, or symptoms, as nasal obstruction to diagnosis was 3 months, ranging between 1 and 24.
Diagnosis was based on the finding of a T/NK phenotype by the histological examination of oropharingeal or cutaneous lesions in 17 cases, bioptical examination of the leg tumor in another patient, and by a bone marrow biopsy in the patient who presented bone pain.
All patients were treated with chemotherapy alone (8 cases), or associated to radiotherapy (10 cases); 1 patient underwent chemotherapy, radiotherapy, and surgery. Chemotherapy regimens usually adopted contained anthracyclines (11 cases); in the other cases patients were treated with CVP (2 cases), low dosages of cyclophosphamide (1), cyclophosphamide-vinblastine-mithoxantrone-bleomicine (1), DHAP (3). Radiation dosages ranged between 27.5 and 47.5 Gy, with a median dosage of 40 Gy.
Eight patients reached a clinical improvement, while 11 patients resulted refractory or presented a limited response to therapy The overall median survival time was 5 months (range from 1 to 48): Eleven patients died within 14 months from diagnosis, and the median survival time of this group was 6 months.
At last follow-up (June 2004) 8 patients are still alive, with a time from diagnosis ranging beteween 2 and 48 months. The preliminary results of this retrospective survey confirmed that T/NK nasal type lymphoma is a very rare lymphoma in italian population, and it is characterized by a very bad prognosis. The rarity of this disease cause a lacking of a standardized therapeutic approaches. More data are needed to know the epidemiology of this kind of lymphoma in Europe.