A Case of Primary Amyloidosis of Salivary Glands with Bronchial Amyloidosis

Abstract

Amyloidosis is a disease of unknown cause characterized by the accumulation of an amorphous, proteinaceous material in various organs and tissues of the body. In the oral region, tongues are most affected. Lips, cheeks, and salivary glands may also be affected, but few cases have been reported so far. We report a case of AL (amyloid light chain) amyloidosis in the left floor of mouth with bronchial amyloidosis. A 56-year-old woman was diagnosed with AL primary bronchial amyloidosis with no predisposing disease and was followed in the department of internal medicine. Three years after diagnosis, she noticed swelling of the left floor of her mouth.
Initially, it was 1cm in size, but showed rapid growth to about 3cm in 4 months. Enucleation of the mass revealed that this was the salivary gland, with diffuse and nodular amyloid deposition. The amyloid protein was identified as protein AL by immunohistochemical method and did not react to AA protein. There were no findings associated with multiple myeloma. Histological examinations showed this amyloid protein had the same composition as amyloid protein deposited in the tracheal tissues. This case seems specific in that amyloid deposition occured in the salivary gland and its size rapidly increased, resembling a tumor.