Diagnosis Issues and Management of Nasopharyngeal Non-Hodgkin Lymphomas

Background: Non-Hodgkin lymphomas (NHL) comprise a variety of lymphoproliferative malignancies with certain differences related to the morphological, clinical, immunohistochemical and hematological patterns, as well as the results of treatment. The patients with generalized and relapsed nasopharyngeal NHL experience marked disease burden and unfavorable impact on their life quality and working capacity.

Objective: The aim of the study was to characterize the diagnosis issues of NHL with primary involvement of the nasopharynx and evaluate the short- and long-term results of management options.

Materials and methods: This analytical and cohort study included 66 patients with different stages of nasopharyngeal NHL, who were managed at the Institute of Oncology from Moldova between 2014-2021. The diagnosis was confirmed by cytological, histopathological and immunohistochemical examinations. The histological types of NHL were verified and distinguished according to the 2017 Revision of WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues. The patients treatment, follow-up and researches were realized at the comprehensive cancer center. The study was related to the hospitalized care. The patients age ranged between 19-85 years (average age - 58.4±2,14 years). Males were 28 (42%), females - 38 (58%). Stage I NHL was diagnosed in 10 (15.2%) cases, stage II - in 36 (54.5%), stage III - in 8 (12.1%) and stage IV - in 12 (18.2%). The eligible NHL patients underwent combined chemotherapy (CChT) regimens (CVChlP, R-CVChlP, CHOP and R-CHOP), associated with radiotherapy locoregional treatment in cases of bulky disease or residual tumor masses. The ECOG-WHO score and complete response (CR) rate assessed the short-term results. The long-term results were asserted by the overall one- and 5-year survival.

Results: Primary nasopharyngeal NHL occurred commonly in females (58%) and in patients over 60 years (42.4%). The ECOG-WHO score accounted 1-3 at diagnosis. The aggressive NHL were diagnosed mostly (76.1%) in stage I and II due to the earlier developed disease burden. The primary tumor site was localized in the palatine tonsils in 22 (33.3%) patients, in 33 (50%) patients in the pharyngeal tonsil, in 2 (3%) patients in the lingual tonsil. The palatine and pharyngeal tonsils were concomitantly involved in 9 (13.7%) patients. Palatine tonsil involvement occurred mostly in patients over 60 years old, and pharyngeal tonsil involvement - in patients of 40-59 years. CR was achieved in 10 (100%) cases with stage I after combined chemotherapy (CChT) and radiotherapy locoregional treatment. CR occurred in 21 (67.7%), partial response (PR) - in 7 (22.6%) and response failure (RF) - in 3 (9.7%) in stage II NHL after CChT and radiotherapy locoregional treatment. In stage II NHL treated with CChT along, CR was achieved in 1 (25%), PR - in 2 (50%) and RF in 1 (25%). In stage III treated with CChT and radiotherapy locoregional treatment, CR was registered in 1 (20%), PR - in 2 (40%) and RF - in 2 (40%). PR occurred in 2 (66.7%), RF - in 1 (33.3%) in stage III managed with CChT alone. In stage IV NHL, CR was obtained 1 (11.1%) case, PR - in 5 (55.6%), RF - in 3 (33.3%) after CChT and radiotherapy locoregional treatment. PR occurred in 1 (33.3%), RF - in 2 (66.7%) cases in stage IV patients managed with CChT alone. No significant differences of CR rate were found in stage III (12,5%) and stage IV (8.3%) NHL (P>0.05). Irrespective of the stage, the highest CR rate was registered after CChT and radiotherapy locoregional treatment (97.1% of all cases), as compared to CChT alone (2.9% of all cases). The ECOG-WHO score reached 0-1 under the management with CChT and radiotherapy locoregional treatment in all cases with CR and PR. The overall survival was 79.9% at one year and 34.5% at 5 years. One- and 5-year survival proved to be significantly higher în stage I and II NHL - 96.1% and 64.2% respectively. One- and 5-year survival was 79.9% and 34.5% in stage III and IV NHL.

Conclusions: Primary nasopharyngeal NHL were outlined by the predominant involvement of females, patients over 60 years and frequent site in the pharyngeal tonsil. The aggressive NHL were revealed commonly in stage I and II due to the progressive disease burden. The rates of the indolent and aggressive NHL turned out to be statistically equal in stage III and IV. The response and overall survival rates proved to be superior after R-CHOP regimen followed by the radiotherapy locoregional treatment.