Secondary Non-Hodgkin's Lymphoma of the Larynx: Rarity and Incidence
Secondary non-Hodgkin's lymphoma involving the larynx is exceedingly rare, accounting for less than 1% of all laryngeal neoplasms, with primary laryngeal squamous cell carcinoma representing approximately 95% of laryngeal malignancies. 1, 2, 3
Epidemiologic Context
Primary laryngeal lymphomas (not secondary involvement) represent less than 1% of all laryngeal neoplasms, making secondary involvement even more uncommon 1, 2, 4
Among the rare cases of laryngeal lymphoma, the vast majority are primary extranodal presentations rather than secondary spread from systemic disease 2, 5
Non-Hodgkin's lymphoma overall accounts for approximately 4% of all new cancer cases in the United States, but laryngeal involvement—whether primary or secondary—remains an exceptional finding 6
In contrast, laryngeal squamous cell carcinoma is far more common, with an estimated 12,630 new cases annually in the United States 7
Anatomic Distribution When Laryngeal Involvement Occurs
When lymphoma does involve the larynx (primary or secondary), the supraglottic region is preferentially affected, with the subglottis being infrequently involved 5
NK/T-cell lymphomas, which are predominantly extranodal, can involve the upper aerodigestive tract including the larynx, but this represents a small subset of an already rare lymphoma subtype 7
Clinical Implications of Rarity
The extreme rarity creates diagnostic challenges, as clinicians have low index of suspicion and histopathologic confirmation is often difficult even with multiple biopsies 1, 2
Because of insufficient case numbers, no consensus exists for optimal management of laryngeal lymphoma, and treatment typically follows standard NHL protocols (such as R-CHOP chemotherapy ± involved-field radiation) rather than larynx-specific approaches 1, 2
Secondary laryngeal involvement by systemic lymphoma or leukemic infiltration carries a very poor prognosis, in contrast to localized primary laryngeal lymphomas which often present as stage IE/IIE disease with favorable outcomes after local radiation 5
Key Diagnostic Pitfall
When evaluating laryngeal masses, the overwhelming likelihood (>95%) is squamous cell carcinoma, not lymphoma 3
However, in patients with known systemic NHL presenting with new laryngeal symptoms, secondary involvement must be distinguished from a new primary squamous cell carcinoma, tumor-flare reaction from immunomodulatory therapy, or other etiologies through biopsy and systemic restaging 8