AMILOIDOSI PRIMITIVA DELLA LARINGE 'AMILODOMA'

The traditional classification of amyloidosis into a secondary form associated with multiple myeloma and a primitive form called systemic and isolated is not entirely satisfactory: it is often inaccurate, and fails to consider a possible genetic error in the metabolic mechanism that produces amyloid material. The chemical variability of amyloid composition from protein precursors within the reticulo-endothelial system is well exemplified by the absence of tryptophan and tyrosine in the amyloid material of endocrine polypeptide tumors, the material being called apudamyloid as opposed to immunoamyloid, the latter being reserved for other forms in which the two aminoacids just mentioned are present. Immunoamyloid is known to contain light polypeptide chains representing immunoglobulins; this fact supports the hypothesis already proposed in the past, that in the majority of clinical forms of amyloidosis, the accumulated material results from an altered or aberrant immune response, which may in some cases be genetically determined. In this light it seems to make sense to suggest that also in the isolated form, amyloidoma, antibody material or some fraction thereof may have accumulated locally as a result of the production of abnormal globulin complexes by the reticulo-endothelial mesenchyma, the latter operating under a more or less sustained stimulus; and that these elements, remaining fixed at the site of their production, constitute an isolated clone. Amyloidoma of the larynx might conceivably belong to this pathogenetic category. The tumor, however, is of rare occurrence, it is easily diagnosed, and with few exceptions it poses no particular problems of treatment, both because of its gross and histological features and in terms of its localization and clinical course, though the possibility of recurrence is not to be denied.