Localized Amyloidosis of the Nasal Mucosa: A Case Report and Review of the Literature

Amyloidosis is a disorder of protein folding in which various proteins automatically aggregate into a highly abnormal fibrillar conformation. Amyloidosis is classified into systemic and localized forms depending on whether the abnormal proteins deposited in several different organs or only a single organ. In localized amyloidosis of the head and neck regions, laryngeal amyloidosis is common; however, localized amyloidosis of the nose is extremely rare. We herein report a case of localized amyloidosis of the nose and review the relevant literature on localized sinonasal amyloidosis. A 41-year-old man presented with a history of severe nasal obstruction, which had persisted for two decades. Nasal endoscopy and imaging studies showed extensive thickening of the bilateral nasal mucosa and diffuse submucosal deposition of calcification. After histopathological and systemic examinations, he was diagnosed with localized amyloidosis of the nasal mucosa. Septoplasty and bilateral inferior turbinoplasty, which consisted of mucosal resection using an ultrasonic bone curette, was performed and his symptoms markedly improved. Localized sinonasal amyloidosis has a good prognosis and surgical resection should be selected as a first-line treatment; however, clinicians should recognize the high probability of recurrence.

Primary Laryngeal Amyloidosis and CO2 Laser as the Treatment Modality

Introduction Amyloidosis is brought about by intracellular and/or extracellular accumulation of insoluble abnormal amyloid fibrils that alters the normal function of the tissues. Localized laryngeal amyloidosis is a rare disease which lacks long-term follow-up studies. It is prone to recurrence; hence meticulous excision is required. We are doing this study to analyse clinical features of primary laryngeal amyloidosis, the subsites commonly found in, and the effectiveness of CO2 laser as treatment modality. Materials and Methods It is a retrospective study of 13 patients diagnosed as Primary Laryngeal Amyloidosis in between 2005 to 2018, where clinical features, histologic and immuno-histochemical patterns of the patients were evaluated. Systemic amyloidosis was ruled out by the non-appearance of Bence-Jones proteins in urine and serum electrophoresis examination. Systemic workups were pursued during the follow-up. The patients were followed up 3 monthly for the first year, then 6 monthly after that, for 3 years. The last patient who underwent the surgery was in the 2018 and had just finished his 2nd follow up, while the rest have been followed up for 3 years. Results Hoarseness was the most common complaint in all the patients. The subsites most common for amyloid deposition were seen in the true vocal cords followed by supraglottis, anterior commissure, ventricle and the subglottis. Microscopically, the amyloid was deposited within the submucosa surrounded by lymphoplamocytic infiltration. All cases were treated with microlaryngoscopic CO2 Laser excision. With the exception of one patient, the rest had no recurrence. Conclusion Primary Laryngeal Amyloidosis is an uncommon benign disease that has a predisposition for recurrence. With use of CO2 laser as the primary treatment modality, the percentage of recurrence has drastically reduced. Systemic involvement should be ruled out. A frequent follow-up of the patients is desirable for early detection of recurrences. Laser is a novel treatment of laryngeal amyloidosis.

A Case of Laryngeal Mucosa Associated Lymphoid Tissue Lymphoma Mimicking Laryngeal Amyloidosis

Laryngeal amyloidosis is a rare disorder characterized by localized amyloid deposition and carries an excellent prognosis. On the other hand, mucosa associated lymphoid tissue (MALT) lymphoma is the most common type of marginal zone lymphoma. MALT lymphoma has a poorer prognosis compared to laryngeal amyloidosis due to more frequent local recurrence, distant metastasis and malignant changes. Despite the differences, as both are found in the form of benign mass and have slow progression, accurate diagnosis is necessary. This report deals with the case of a patient with voice change accompanied by a mass, in which the initial biopsy did not include enough specimen for an accurate diagnosis. However, in the second attempt, an adequate sample was obtained, hence the final diagnosis of laryngeal MALT lymphoma. Based on our recent experience and a review of related literature, we report a case of laryngeal MALT lymphoma mimicking laryngeal amyloidosis.

Laryngotracheobronchial Amyloidosis: Patterns of Presentation and Management

Objective: To evaluate the pattern of presentation and management of laryngotracheobronchial amyloidosis at a tertiary care academic center over a 27 year period. Methods: In a retrospective review, the electronic medical record at a tertiary care academic center was queried for encounters with 3 laryngologists between 1996 and 2019 which included the ICD-9 or ICD-10 diagnosis of amyloidosis. Demographics, clinical presentation, referral diagnoses, medical history, family history, laboratory values, radiology studies, and treatment modalities of subjects were collated. Results were analyzed using standard univariate descriptive statistics. Results: Seventeen subjects were identified with an average age at diagnosis of 58 years (range 26-76 years). The most common amyloid type on biopsy was immunoglobulin light chain (AL) subtype. The most common location of laryngeal amyloid at diagnosis was the glottis and disease was more likely to be bilateral at the time of diagnosis in this location. Supraglottic disease more often had a unilateral presentation and had a tendency to spread to additional laryngeal subsites. Nearly 25% of subjects had associated systemic disease, including multiple myeloma, auto-immune disease, and familial ATTR mutation. Conclusions: The overall rate of associated systemic disease was low in our study cohort; however, it is higher than typically referenced in extant literature. Our cohort demonstrates that while laryngeal amyloidosis is a chronic condition, the behavior is generally indolent with a low treatment burden.

Localised laryngotracheal amyloidosis: a differential diagnosis not to forget

We present a case of multifocal laryngotracheal amyloidosis (LTA) in a 43-year-old man with persistent and progressive dysphonia and dyspnoea, and a first inconclusive histology. Although laryngeal amyloidosis accounts for fewer than 1% of all benign laryngeal tumours, it is in fact the most common site of amyloid deposition in the head, neck and respiratory tract. The clinical scenario is non-specific and diagnosis depends on a high degree of suspicion and on histology. Imaging is useful in mapping lesions, which are often more extensive than they appear during laryngoscopy. Despite being a benign entity, the prognosis is variable with a high-rate and long-latency recurrences, requiring long-term follow-up.