Laryngeal Lymphoma Treatment
Laryngeal lymphoma requires systemic chemotherapy with rituximab-based regimens (R-CHOP or R-COP) as the primary treatment, often combined with involved-field radiation therapy, rather than the surgical or radiation-based larynx-preservation strategies used for squamous cell laryngeal carcinoma.
Critical Distinction: Lymphoma vs. Squamous Cell Carcinoma
The provided guidelines 1 address squamous cell laryngeal carcinoma, not laryngeal lymphoma. These are fundamentally different diseases requiring completely different treatment approaches:
- Squamous cell carcinoma is treated with surgery, radiation, or chemoradiation with curative intent 1
- Laryngeal lymphoma is a systemic hematologic malignancy requiring chemotherapy-based treatment 2, 3
Standard Treatment for Laryngeal Lymphoma
Diffuse Large B-Cell Lymphoma (DLBCL) - Most Common Type
Primary treatment consists of:
- R-CHOP chemotherapy (rituximab + cyclophosphamide, doxorubicin, vincristine, prednisone) for 6-8 cycles, OR 2, 3
- 3 courses of R-CHOP followed by involved-field radiation therapy 2
- R-COP (without doxorubicin) may be substituted in elderly patients or those with cardiac dysfunction 2
The stage IE laryngeal DLBCL demonstrates complete resolution with R-CHOP plus rituximab therapy 3.
NK/T-Cell Lymphoma - Aggressive Variant
This subtype requires different management:
- Radiotherapy alone or combined with chemotherapy is superior to chemotherapy alone for early-stage disease 4
- SMILE regimen (dexamethasone, methotrexate, ifosfamide, L-asparaginase, etoposide) is used for advanced NK/T-cell lymphoma 5
- Radiation fields should cover the entire cervical area due to frequent local lymph node metastasis (approximately one-third of cases) 4
- Outcomes are generally poor, with prompt diagnosis and radiotherapy being critical for survival 4
Lymphoplasmacytic Lymphoma - Rare Variant
- Radiation therapy (30.6 Gy) combined with rituximab (8 cycles) achieves successful remission 6
Diagnostic Challenges
Multiple biopsies are frequently required before confirming the diagnosis of laryngeal lymphoma:
- Most patients require several biopsies over months due to difficulties interpreting pathologic findings 4
- Deep biopsy specimens are essential to identify the lymphoma subtype 3
- If laryngeal biopsies are non-diagnostic, lymph node biopsy from cervical nodes may establish the diagnosis 2
- The supraglottis is the most common laryngeal subsite involved 4
Airway Management Considerations
Aggressive airway monitoring is mandatory during treatment:
- Tracheostomy may be required if airway becomes compromised during chemotherapy or radiation 5
- Post-treatment sequelae include glottic stenosis, supraglottic scarring, and tracheal stenosis requiring dilation procedures 5
- Decannulation is possible after successful treatment and airway dilation 5
Common Pitfalls
Do not apply squamous cell carcinoma treatment algorithms to laryngeal lymphoma - the surgical and radiation-based larynx-preservation strategies described in the ASCO guidelines 1 are inappropriate for lymphoma, which requires systemic chemotherapy.
Do not delay treatment waiting for perfect tissue diagnosis - if clinical suspicion is high and initial biopsies are non-diagnostic, proceed with lymph node biopsy or repeat deep laryngeal biopsies rather than observing 2, 4.
Do not use single-modality radiation alone for DLBCL - systemic chemotherapy with rituximab is essential for this systemic disease 2, 3.