Malignant Lymphoma

Primary non-Hodgkin's lymphoma (NHL) of the larynx is rare, accounting for less than 1% of all laryngeal neoplasms [8] and approximately 1% of all primary extra-nodal lymphomas [389]. By definition, the bulk of the disease should occur in the larynx [389]. About 65 cases have been reported in the English-language literature [8, 55, 70, 249].

The majority of laryngeal NHL are of B-cell type, especially diffuse large B-cell lymphoma, and extranodal marginal lymphoma of the MALT type. Rare cases of T-cell NHL have been reported, such as NK/T cell lymphoma of the nasal type, and peripheral T-cell lymphoma [389]. All regions of the larynx may be involved, with the exception of the extranodal marginal lymphoma of the MALT type, which has been described in the supraglottis only [70, 199], presumably because mucosa-asso-ciated lymphoid tissue has been found mostly in the su-praglottic region [199].

They present mostly at stage I or II and are limited to the larynx, with or without regional lymph node involvement. The most common symptoms are hoarseness, foreign body sensation, and airway obstruction.

The prognosis is favourable. Laryngeal NHL should be treated according to the histologic type of NHL. It usually responds well to radiation therapy. Systemic chemotherapy is indicated for recurrent or disseminated disease [8].

Rare examples of other sarcomas have been described in the larynx and hypopharynx, such as liposarcoma [136], osteosarcoma [220, 256, 332], angiosarcoma [214], synovial sarcoma [34], malignant fibrous histiocytoma [143], Kaposi's sarcoma [246, 325], leiomyosarcoma [202, 297], etc.

The aetiology of laryngeal sarcomas is unknown, although exposure to radiation [284] has been implicated as a possible aetiologic factor for osteosarcoma [332] and malignant fibrous histiocytoma [143], and infection with HIV for Kaposi's sarcoma [246, 325].

The larynx may be rarely involved in disseminated systemic lymphoma or leukaemia [163]. It can also be the primary site of a haematopoietic or lymphoid neoplasm. Extramedullary plasmacytoma seems to be the most common primary lymphoid neoplasm of the larynx. Various types of non-Hodgkin's lymphoma of B-cell type and T-cell type have also been reported in the larynx, as well as rare cases of granulocytic sarcoma and mast cell sarcoma [163].

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