Clinical outcome of patients with primary extranodal head and neck lymphoma (PEHNL) is not only influenced by histology and stage, but also by the site of presentation. However, it has not yet been clarified whether the reason for this difference is intrinsic to the location or depends on distribution of histopathology in different sites of disease. Therefore, the aim of this retrospective study was to evaluate the clinical outcome and prognostic factors of PEHNL patients with DLBCL according to the sites of presentation. From December 1990 to June 2004, 478 patients were referred to 14 cancer centres. This series included 253 males and 225 females, with a median age of 60 years (range, 14-93 years). Waldeyer’s ring (WR) was the most common site of presentation (n=297), followed by nose and paranasal sinuses (NPS) (n=48), thyroid (n=48) (T), salivary glands (SG) (n=38), combined sites (CS) (n=25) and oral cavity (n=22) (OC). The frequency of adverse features varied in different locations: WR had more stage II patients, while in thyroid cases advanced age (>60 yrs), female sex, bulky disease, poor ECOG-PS, elevated LDH and >1 adverse factors according to stage-modified IPI (MIPI) were more frequent. The commonest treatment was a short course of anthracycline-based chemotherapy (CHT) + involved field radiotherapy (IFRT). Forty-two percent of T patients also underwent surgery. Only 28/428 (6.5%) received CNS prophylaxis. The CR rate ranged from 79% (CS) to 95% (OC), while relapse was most common in SG (31%) and prevailed in distant sites (60%). Four patients (1 WR, 1 SG, 2 NPS), without CNS prophylaxis, relapsed in CNS (0.8%) and 4 WR patients (0.8%) in GI tract. After a median follow-up of 49 months (range 1-219 months), 5-yr OS, EFS and DFS were 72%, 59% and 74%, respectively. OS varied among different locations from 51% (T) to 89% (OC). It is noteworthy that CS patients did not fare worse than those with disease presenting in single sites. In all presentations with the exclusion of T patients, 5-yr EFS differed according to MIPI (MIPI 0-1, 68% vs. MIPI >1, 49%; p=0.0001) and CHT+IFRT (combined treatment) (CHT 43% vs. CHT+IFRT 71%; p=0.0001) as confirmed by Cox multivariate analysis (MIPI, p=0.012; combined treatment, p=0.0001). However, in thyroid involvement MIPI seems to be not predictive of survival due to a high mortality unrelated to disease. Moreover, T patients seem to benefit more from surgery in combination with chemotherapy and/or IFRT than from other treatments not including partial or complete thyroid resection (p=0.04). Conclusions. patients with DLBCL of different head and neck sites represent a heterogeneous group regarding clinical characteristics, prognostic factors and outcome. A low MIPI and a combined treatment with addition of radiotherapy influence the outcome favorably. Moreover, a very low rate of CNS recurrence suggests that CNS prophylaxis may not be mandatory for these patients.
Primary extranodal diffuse large B-cell lymphomas (DLBCL) arising in distinct sites of head and neck have different clinical characteristics and outcome / Cortelazzo, . S.; M., Mian; A., Rossi; E., Oldani; M. E., Cabrera; M., Federico; M., Bellei; S., Magrini; M., Buglione; Martelli, Maurizio; H., Gomez; A., Lopez Guillermo; E., Pogliani; F., Rossini; G., Gidano; A., Conconi; M., Busetto; C., Visco; R., Tsang; E., Zucca; A., Rambaldi; F., Cavalli. - In: HAEMATOLOGICA. - ISSN 0390-6078. - 92 (SUPPL. 1)(2007), pp. 321-321. ((Intervento presentato al convegno 12th Congress of the European-Hematology-Association tenutosi a Vienna, AUSTRIA nel JUN 07-10, 2007.