Primary non-Hodgkin's lymphoma of the larynxNK Nerurkar, A Sarkar
Voice and Swallowing Centre, Bombay Hospital & Medical Research Centre, Mumbai, Maharashtra, India
Correspondence Address: Source of Support: None, Conflict of Interest: None DOI: 10.4103/jpgm.jpgm_290_22
Source of Support: None, Conflict of Interest: None
Primary non-Hodgkin's Lymphoma (NHL) of the larynx is rare, accounting for less than 1% of laryngeal neoplasms with fewer than 100 cases reported in the literature. Although NHL usually occurs within lymph nodes, 25% of the cases may arise in normal non-lymphoid tissues. Laryngeal NHL is likely to develop in the supraglottic and ventricular lymphoid collections due to the scarcity of lymphoid tissues in the glottis. We present a case of primary glottic NHL that suffered diagnostic and treatment delays due to the COVID-19 pandemic.
A 62-year-old male complaining of a change in voice and foreign body sensation while swallowing, since one year, was referred to our voice center. He had uncontrolled type-2 diabetes mellitus and routinely used inhaled steroids for bronchial asthma. The patient had been variably treated for bacterial, viral, and fungal laryngitis by four different otolaryngologists earlier. Video laryngostroboscopy revealed irregular red-white patchy lesions over bilateral vocal folds and a large smooth-surfaced bulge in the inter-arytenoid region [Figure 1]a with moderate amplitude of mucosal waves of both vocal folds. Narrow band imaging (NBI) revealed occasional intraepithelial papillary capillary loop (IPCL) pattern/vertical pattern as per the European Laryngeal Society classification of vascularity [Figure 1]b. The patient was advised excision of the lesions, but due to the pandemic-related concerns, he followed up only after eight months. The high-resolution computed tomography neck scan was unremarkable with no involvement of soft tissues or cartilages.
Intraoperative imaging confirmed red and white areas on both vocal folds [Figure 1]c with occasional IPCLs on Spectra-A imaging. Laser-assisted biopsy of bilateral vocal fold lesions was performed, following subepithelial infiltration, leaving the anterior commissure untouched [Figure 1]d.
Fungal culture reported growth of Aspergillus fumigatus, and mycobacteria growth indicator tube (MGIT) did not reveal the growth of Mycobacterium tuberculosis. Histopathology examination [Figure 2]a and [Figure 2]b suggested atypical lymphoid proliferation in both vocal folds, and immunohistochemistry confirmed NHL with large B-cell type tumor cells expressing CD20, CD30 and MUM-1. Systemic involvement was excluded by whole-body positron emission tomography-computed tomography scan, hepatic and renal function tests. Thereafter, he was advised chemotherapy followed by radiotherapy by the oncologist for the high-grade laryngeal NHL. Upon the latest follow-up (six months after completing treatment), the patient is faring well with a good, serviceable voice.
Primary laryngeal NHL is reported more frequently in men in a ratio of 3.4:1. Clinical appearance may be as non-ulcerated submucosal masses occurring commonly in the supraglottis, particularly the aryepiglottic folds that are known to have a rich lymphatic network. Involvement of supraglottis is likely to cause ventricular phonation resulting in a change in voice. The laryngoscopy appearance resulted in the patient receiving treatment for fungal laryngitis and reflux at various centers. Both long-term uncontrolled diabetes mellitus and steroid use lead to an immunocompromised state, which may aid in pathogenesis of lympho-proliferative disorders like NHL. There is growing evidence linking insulin and insulin-like growth factor- 1 (IGF-I) to cell proliferation, lack of apoptosis in cells and cell metastasis phenomena involved in the development and progression of malignancies.
Prior to surgery, anesthetic management was carefully planned. We used a 5.5 mm size laser-safe cuffed micro-laryngeal endotracheal tube to secure the airway and prevent leakage of anesthetic gas or oxygen while providing ventilation. Endotracheal tube cuff was filled with methylene blue tinted saline to alert in case of rupture of cuff. When using the laser, inspired oxygen concentration was reduced to <30%, and nitrous oxide was discontinued. CO2 laser microflap excision of the glottic lesions was performed using acublade in scanning mode, ensuring that the anterior commissure remains untouched. In case of the inter-arytenoid lesion, tissue was excised unilaterally to prevent cicatrization and posterior glottic webbing.
Treatment of NHL includes radiation and/or chemotherapy and/or immunotherapy depending upon the histological grade of lesion. Rituximab and CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) regimen is usually the standard treatment choice for diffuse large B-cell NHL. Laryngeal NHL patients with T-cell type have a worse prognosis than those with B-cell lymphoma. In the present patient, the B-cell type expressed CD30 positivity which is rare. Hu et al. have reported in 2013 that CD30 expression identifies a novel subgroup of diffuse large B-cell lymphoma patients with superior clinical outcomes. Although the prognosis of localized B-cell type laryngeal NHL is favorable, life-long follow-up is indicated for early detection of recurrence.
Declaration of patient consent
The authors certify that appropriate patient consent was obtained.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
[Figure 1], [Figure 2]