When considering the incidence of malignancies that involve the head and neck, lymphoma is second only to squamous cell carcinoma.

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Introduction​

When considering the incidence of malignancies that involve the head and neck, lymphoma is second only to squamous cell carcinoma. This level of frequency is attributable primarily to disease involving Waldeyer's ring and cervical nodes. In contrast, primary disease arising in the larynx is unusual. Due to scarcity of lymphoid tissue in the glottis, most primary lymphomas arise either in the supraglottis or subglottis. Rapid enlargement of submucosal masses can result in relatively acute airway compromise. Primary Non-Hodgkin's lymphoma may be confined to the larynx or represent merely one manifestation of a systemic process. Initial management should include a biopsy specimen and airway stabilization, which may involve endoscopic debulking, tracheotomy, or nonsurgical management when possible. Various combinations of intravenous steroids, chemotherapy and external beam radiation therapy have been used successfully in sizable lesions compromising the airway [2]. In a solitary laryngeal lymphoma, radiation therapy may be the sole therapeutic modality necessary to achieve an excellent prognosis [3, 4].

 

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Case Report​

A 72-year-old male was referred to the Department of ENT with the complaints of pain left side of throat on swallowing liquids. There was no history of dysphagia, haemoptysis, weight loss, change of voice, dyspnoea. Individual was apparently alright till Jan 99 when he noticed pain while swallowing liquids which was gradually increasing. There is history of smoking hukka for past 50 years. No other associated medical complaint. No relevant family history.

ENT examination revealed edema over the ary-epiglottic fold left arytenoid, and a proliferative lesion involving left cord and ventricle. Neck examination did not reveal any gland. All relevant investigations were within normal limits.

CT scan of the larynx showed thickening of the left vocal cord (Fig. 1). Microlaryngeal surgery was done, arytenoids and left ary-epiglottic fold were found to be edematous. A small proliferative lesion was found over left cord extending into ventricle, it was removed and subjected for histopathological examination.

 

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Histopathological examination revealed-Non Hodgkin's Lymphoma (High Grade).

Discussion​

A case of primary Non-Hodgkin's lymphoma of the larynx is presented with a review of the literature. Non-Hodgkin's lymphomas with primary laryngeal presentation is very rare. The symptoms usually include dysphonia and progressive airway obstruction requiring tracheostomy. Laryngeal Non-Hodgkin's lymphomas are of B cell lineage and respond well to radiotherapy. Our patient had a high grade lymphoma of B cell lineage which showed a good response to radiotherapy. The role of chemotherapy and surgery is not yet established [6]. Primary localised laryngeal lymphoma is rare. The presenting symptoms are hoarseness of voice of long duration and increasing dyspnoea. Clinical and histological diagnosis is difficult and in some cases repeated biopsies are needed for conclusive diagnosis. Radiotherapy appears to be the treatment of choice for the disease. As in addition there may be head, neck or distant localisations, there should be a complete clinical evaluation before treatment, including lymphangiography, IV pyelography, liver and spleen scans, and total body CT scan. Although in a significant number of cases laryngeal lymphoma has been cured or has remained a localized disease, it is very likely that in some cases additional treatment for the tumour at other sites may be required. Lifelong follow-up and careful periodic evaluation by experts is imperative. Primary laryngeal lymphoma is a very rare entity, with fewer than 50 cases reported in the English literature in the past 60 years. Close scrutiny of some of these case reports reveal that the larynx was not always the only site of involvement, thereby diminishing the total number of patients with primary laryngeal lymphoma to fewer than 35. In view of the heterogeneity of histologies in this group of lymphomas, the variability in duration of response, and the significant number of patients who died of their disease, it is more likely that primary laryngeal lymphoma is an unusual presentation of non-Hodgkin's lymphoma than a separate disease entity. Despite the small number of patients in this study, the data would suggest that patients are best treated according to the histology of the lymphoma, rather than the limited stage and location of the disease [7].

The biologic behaviour of these tumors has many features in common with other extranodal lymphomas arising from mucosa-associated lymphoid tissue (e.g., a tendency to remain localised for long periods of time and to disseminate to other extranodal and unusual sites, with good response to radiotherapy for both the primary tumour and extranodal recurrences).

 

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Primary laryngeal lymphoma is a very rare entity, with fewer than 50 cases reported in the English literature in the past 60 years. Close scrutiny of some of these case reports reveal that the larynx was not always the only site of involvement, thereby diminishing the total number of patients with primary laryngeal lymphoma to fewer than 35.

No wonder the doctors said after my father is dead that he died from stomach cancer. Bloody stupid doctors should have known my father’s lymphoma is not the primary site since it’s so rare.

 

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. In a solitary laryngeal lymphoma, radiation therapy may be the sole therapeutic modality necessary to achieve an excellent prognosis [3, 4].

Bloody stupid doctors proposed surgery when radiation is sufficient.