From the Guidelines
Extranodal natural killer (NK)/T-cell lymphoma, nasal type, is the primary lymphoma that can involve the nasopharynx, as it is more common in Asia and Central and South America, and is nearly always Epstein-Barr virus-positive 1. This lymphoma type is characterized by its aggressive clinical behavior, with a median survival of 27 months for patients presenting with only skin lesions, compared with 5 months for patients presenting with cutaneous and extracutaneous diseases 1.
Key Characteristics of Extranodal NK/T-cell Lymphoma
- More common in Asia and Central and South America
- Nearly always Epstein-Barr virus-positive
- Aggressive clinical behavior
- Median survival of 27 months for patients with only skin lesions, compared with 5 months for patients with cutaneous and extracutaneous diseases
Treatment Approach
Treatment for extranodal NK/T-cell lymphoma, nasal type, typically involves radiotherapy, with recommended radiation doses of 50 Gy to the initial lesion and a boost of 5–10 Gy to residual disease 1.
Importance of Early Diagnosis
Early diagnosis is crucial, as nasopharyngeal involvement can lead to local destruction and extension to adjacent structures, highlighting the need for prompt treatment and management.
Clinical Presentation
Patients with extranodal NK/T-cell lymphoma, nasal type, may present with symptoms such as nasal obstruction, epistaxis, hearing loss, or cranial nerve palsies, emphasizing the importance of a thorough diagnostic workup, including tissue biopsy with immunohistochemistry and molecular studies.
From the FDA Drug Label
Adult patients with Non-Hodgkin's Lymphoma (NHL) Previously untreated follicular, CD20-positive, B-cell NHL Previously untreated diffuse large B-cell, CD20-positive NHL Pediatric patients aged 6 months and older with mature B-cell NHL and mature B-cell acute leukemia (B-AL) Previously untreated, advanced stage, CD20-positive, diffuse large B-cell lymphoma (DLBCL), Burkitt lymphoma (BL), Burkitt-like lymphoma (BLL)
The FDA drug label does not answer the question of which lymphomas can involve the nasopharynx.
From the Research
Lymphomas Involving the Nasopharynx
The nasopharynx can be involved by various types of lymphomas, including:
- Diffuse large B cell lymphoma (DLBCL) 2, 3
- NK/T cell lymphoma (NKTCL) 2, 3, 4, 5, 6
- Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALToma) 2
- Small lymphocytic lymphoma 2
- Mantle cell lymphoma 2
- Post-transplant lymphoproliferative disorder (PTLD) 2
Characteristics of Lymphomas Involving the Nasopharynx
- Nasopharyngeal lymphoma is a rare primary malignancy of the head and neck 2
- The most common pathological diagnosis of nasopharyngeal lymphoma is DLBCL, followed by NKTCL 2
- EBV positivity is found in both NKTCL and DLBCL 2, 3
- NKTCL is often associated with a worse prognosis compared to other types of lymphomas 5, 6
- Radiotherapy and chemotherapy are common treatment modalities for lymphomas involving the nasopharynx 2, 4