Abstract
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Non-Hodgkin's lymphoma (NHL) is rarely encountered in the larynx. We report another case of malignant lymphoma of larynx arising from mucosa-associated lymphoid tissue (MALT).
CASE REPORT
A 58-year-old Taiwanese woman was admitted to our hospital in August 1996 because of persistent hoarseness and a sensation of a lump in the throat for 2 months. Under indirect laryngoscopy, a right supraglottic, nonulcerative mass that was fish-flesh colored and about 1.5 cm in diameter was noted arising from the right aryepiglottic fold. Biopsy specimens revealed a nonulcerative mucosa infiltrated by a few atypical lymphocytes. The tumor persisted after supportive treatment, so direct laryngoscopy was performed again. Deep incision was done, and 6 pieces of tissue were taken, which revealed a submucosal tumor composed of small cleaved cells and large lymphoid cells with plasmacytoid differentiation. A thin grenz zone separated the main tumor from the overlying epithelium, but some atypical lymphoid cells infiltrated the overlying epithelium (Fig 1).
Immunohistochemistry study demonstrated B-cell lineage. CT scan showed a laryngeal mass at right aryepiglottic fold, and 1 enlarged regional lymph node was noted. No other foci of lymphoma were found. A primary, diffuse, small cleaved and large cell laryngeal lymphoma was diagnosed. The patient was treated with a total dose of 3000 cGy of radiation. After 2000 cGy was given, the symptoms disappeared. Follow-up laryngoscopy was performed 6 months later; no recurrent tumor was seen. The patient was alive and well without symptoms of recurrence 1 year after treatment.
DISCUSSION
Non-Hodgkin's lymphoma of the larynx is a rare condition. The median age at diagnosis is 58 years. 1 The major symptoms include a short history of hoarseness, dysphasia, and dysphonia, about 6 to 10.2 months before the diagnosis. 1 Two thirds of primary laryngeal lymphomas are on the left side, and most of the tumors are located in the supraglottic area, in particular the epiglottis and aryepiglottic folds. 1 Most tumors present as a polypoid, nonulcerated mass, which is the presentation of low-grade lymphoma. The tumor usually measures 1 to 3 cm in diameter at presentation. B-cell lymphomas appear to be much more common than T-cell lymphomas. Lymphomas that arise from MALT follow a low-grade malignant course 2 and have been found to recapitulate the cytologic features of MALT. These tumors contain centrocyte-like B cells with a phenotype similar to that of marginal zone B cells. MALT-type lymphoma of the larynx was first reported by Diebold et al. 3 The primary involvement of lymphoma of the larynx must be clearly distinguished from the disseminated lymphoma by complete evaluation and staging.
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Thin grenz zone separated the main tumor from the overlying epithelium, but some atypical lymphoid cell infiltrate the overlying epithelium.
These lymphomas are highly radiosensitive, so local radiotherapy is the most appropriate treatment. The prognosis is generally favorable. Chemotherapy is reserved for recurrent or disseminated disease. The larynx, which has little lymphoid tissue, may be involved secondarily in cases of disseminated malignant lymphoma, so clinical staging before and after treatment is necessary. The histopathologic picture of MALT, with only a few tumor cells involving the nonulcerative epithelium, emphasizes the need for a deep biopsy for accurate diagnosis of this tumor.