Background & Objective: Primary paranasal sinus lymphoma (PPSL) is a rare presentation of extranodal non-Hodgkin's lymphoma with a natural history distinct from other lymphomas. This study was to evaluate the clinical and pathologic characteristics, treatment outcomes and prognosis of PPSL.
Methods: The records of 14 PPSL patients, treated at Cancer Center of Sun Yat-sen University from 1994 to 2006, were analyzed.
Results: The primary involvement sites included the maxillary sinus (11 cases), ethmoid sinus (2 cases), and sphenoid sinus (1 case). All patients were at stage I-II (Ann Arbor system). According to the AJCC TNM staging system, most patients had advanced T3-T4 disease. Of the 14 patients, 12 had B-cell PPSL, 1 had T-cell PPSL, and 1 had unclassified PPSL. The most common type was diffuse large B-cell PPSL (6 cases, 42.9%). Two patients underwent total maxillectomy and 12 underwent local excision or biopsy. All patients received chemotherapy and 6 received radiotherapy after chemotherapy. Both 5-year overall and event-free survival rates were 78.6%, with a median survival of 59.5 months(range, 2-192 months).
Conclusions: PPSL is an uncommon presentation of lymphoma characterized by bulky local disease. Diffuse large B-cell lymphoma is the most common histologic type and the maxillary sinus is the most common original site of PPSL. A combined-modality approach with systemic chemotherapy and local-regional radiation is recommended for PPSL patients. The prognosis of PPSL is relatively good.
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