Laryngeal Swelling – 'Thinking outside the box'

Introduction:

Amyloidosis is a rare, benign, slowly progressive condition that is typically characterized by a localized deposition of proteins in a variety of organs and tissues. Disease manifestation can be seen in various areas of the head and neck like the pharynx, the paranasal sinuses, the larynx and the trachea, why patients often first present to the otolaryngologist due to acute dyspnea. However, further diagnostics and will mainly be performed by the hemato-oncologist. This case report gives an overview on this rare clinical disease and describes the interdisciplinary diagnostic and therapeutic regimen.

Case presentation:

The 70 year old, female patient presented to our outpatient department with hoarseness, dyspnea and inspiratory stridor. She had suffered from thyroid papillary cancer in the past and showed vocal cord palsy. The endolarynx was swollen with some granulation tissue. Computed tomography showed similar findings without the presence of a defined laryngeal mass. The initial panendoscopy under general anaesthesia showed no pathological findings. Another microlaryngoscopy with larger biopsies revealed the amyloid deposition. Because of acute dyspnea a surgical tracheotomy had to be performed.

Discussion:

The histological examination revealed an Amyloid deposition with lymphoplasmacytic infiltration and showed further deposition in terms of Light chain deposition disease (LCDD). This form of disease can manifest locally but may also affect other organs such as the heart, kidney and liver. Beside blood tests a complete and thorough workup should be made to plan the systemic therapies which usually include some form of chemotherapy.

Publication History

Publication Date:
12 June 2019 (online)