Involvement of the Larynx by Hemopoietic Neoplasms: An Investigation of Autopsy Cases and Review of the Literature

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Summary

Involvement of the larynx by hemopoietic tumors is generally considered a rare event and little is known about the associated clinicopathologic features. Laryngeal tissue removed at autopsy from 14 patients with known disseminated hematologic malignancies and at operation from one patient with multicentric malignant lymphoma of low-grade malignancy (MALToma) of the head and neck region was investigated. A systematic survey of the main clinicopathologic features of the published cases of hemopoietic tumors with laryngeal involvement was also performed. Primary involvement of the larynx by hemopoietic neoplasms must be clearly distinguished from secondary involvement by disseminated or leukemic tumors. Most of the primary tumors are localized lesions that may involve the regional lymph nodes (stages IE or IIE). Radiotherapy is the treatment of choice, and the prognosis is generally favorable. However, secondary involvement by disseminated or leukemic disease carries a very poor prognosis in most cases. Extramedullar plasmacytoma and non-Hodgkin's lymphoma (NHL), particularly B-cell lymphoma of high-grade malignancy, appear to be the most common hemopoietic tumors with primary laryngeal involvement, while primary tumors of myelogenous origin (granulocytic sarcoma and mast cell sarcoma) are extremely rare. Extramedullar plasmacytoma and NHL occur mainly in older persons and in men, are generally associated with a relatively short history of hoarseness and dysphagia, and exhibit preferential involvement of the supraglottic parts of the larynx, in particular the epiglottis and aryepiglottic folds. They are generally polypoid, non-ulcerated lesions. Secondary, usually asymptomatic, involvement of the mucosal layer and bone marrow of the larynx by leukemia, multiple myeloma, and disseminated lymphoma is almost certainly much more common than might be inferred from the small number of published cases.

While secondary involvement of the larynx by hematologic malignancies is much more common than generally thought, primary hemopoietic tumors are very rare here. These are usually localized neoplasms, and radiotherapy is the treatment of choice. To avoid inappropriate aggressive surgery it is necessary to be aware of the relatively characteristic macroscopic features of such tumors.

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    PD Dr. med. H.-P. Horny, Universität Tubingen, Institut für Pathologie, Liebermeisterstr. 8, D-72076 Tübingen, Germany, Tel: (0) 70 71-29 68 91, Fax: (0) 70 71-29 22 58

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