Nasopharyngeal Lymphoma: A 22-Year Review of 35 Cases

Abstract

Nasopharyngeal (NP) lymphoma is a rare primary malignancy of the head and neck and represents a minority of malignancies originating from the nasopharynx. For this reason, there are limited data regarding epidemiologic and treatment outcomes. This is a retrospective review of patients diagnosed with NP lymphoma from 1995 to 2017 at a tertiary medical center. The patients’ demographic data, clinical presentations, treatment modalities, Epstein–Barr virus (EBV)-encoded small RNA (EBER) staining, and outcomes were investigated. We considered a total of 35 patients, including 20 males and 15 females, diagnosed with NP lymphoma. The age ranged from 17 to 88 years (mean = 59.6). The common presentations were nasal obstruction, epistaxis, and neck mass. In our study, the most common pathological diagnosis of NP lymphoma was diffuse large B cell lymphoma (DLBCL) (n = 17), followed by NK/T cell lymphoma (NKTCL) (n = 9). Other pathologic diagnoses included extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALToma), small lymphocytic lymphoma, mantle cell lymphoma. There were 13 cases showing EBER positivity, including 7 cases of NKTCL, 5 cases of DLBCL, and 1 case of post-transplant lymphoproliferative disorder (PTLD). Most patients received chemotherapy alone, while some patients received both chemotherapy and radiotherapy. Seven patients had local recurrence, and fewer than half of the patients (n = 16) were alive at the time of the study (mean follow-up duration: 54.4 months). The five-year overall survival was 50.4%. NP lymphoma is very rare, and the most common pathologic type is DLBCL. EBER positivity is found in both NKTCL and DLBCL. Identifying more effective therapeutic agents is extremely important to improve patients’ survival.

Nasopharyngeal B-cell lymphoma with pan-hypopituitarism and oculomotor nerve palsy: a case report and review of the literature

“…Diffuse large B cell lymphoma was the most common pathological diagnosis of nasopharyngeal ( n = 17), followed by NK/T cell lymphoma ( n = 9). Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue, mantle cell lymphoma, and small lymphocytic lymphoma was the other pathologic diagnoses [ 5 ]. To compare our patient with Hsueh et al study, our patient had leukopenia and a longer duration from initial symptoms (i.e., diplopia) to diagnosis (about 9 months) and similar age and LDH levels.…”

“…There are limited data regarding epidemiologic and treatment outcomes of NH lymphoma [ 5 ]. The treatment of localized disease (stage I, II and non-bulky disease) with activity index less than 2 and normal LDH level is based on the CHOP regimen (3 to 4 cycles) plus Rituximab (if CD20 positive in immunochemistry).…”

“…In most cases, NPL appears with nasal manifestations, including epistaxis, nasal obstruction, and purulent rhinorrhea. However, it can present with a neck mass, headache, and B symptoms (i.e., weight loss, night sweats, and fever), less commonly [ 5 ].…”

Nasopharyngeal diffuse large B-cells lymphoma causing acute airway obstruction amid COVID-19 crisis: an anaesthetist’s nightmare

“…The expression of EBV-encoded small RNAs is controversial in DLBCL. 5 In our patient, staining for EBV was not done due to financial constraints and the outcome of the results would not change the future management. The principles of management for patients with NP lymphoma include symptomatic surgical resection of tumour, commencement of arthracycline chemotherapy and radiotherapy.…”

“…NP lymphoma is an extremely rare extranodal haematological malignancy. 5 There are two subtypes of NP lymphoma—Hodgkin lymphoma and non-Hodgkin’s lymphoma (NHL). The latter is the most common among Asians amounting to about 90% of lymphoma cases.…”