A Case of Subglottic Marginal B-Cell Lymphoma With Laryngeal Amyloidosis.

Laryngeal mucosa-associated lymphoid tissue (MALT) is an extra-nodal margin zone B-cell lymphoma (MALT lymphoma) and a low-grade malignant lymphoma with a low incidence, the etiology of the condition remains obscure, and the process of differential diagnosis poses a significant challenge, so it is easy to miss diagnosis and misdiagnosis clinically. The present article presents a clinical case study of a patient who was diagnosed with subglottic MALT lymphoma, which was associated with laryngeal amyloidosis. The patient underwent a successful treatment regimen comprising carbon dioxide laser and radiotherapy. In addition, the article provides an overview of relevant literature that can aid in the diagnosis and management of this rare disease. The study is expected to contribute to the existing body of knowledge on the treatment of subglottic MALT lymphoma and laryngeal amyloidosis.

Laryngeal Amyloidosis: What is the Role of Imaging?

OBJECTIVE/HYPOTHESIS: To review the imaging findings of laryngeal amyloidosis and to identify radiological findings suggestive of this disease. STUDY DESIGN: Retrospective case series. METHODS: A retrospective chart review of patients with pathologically confirmed laryngeal amyloidosis was performed from 2009 to 2022. Clinical and demographic factors were collected. A fellowship-trained head and neck radiologist reviewed all computed tomography (CT) scans and magnetic resonance imaging (MRI) findings within this cohort. RESULTS: 12 patients were identified and a total of 36 imaging studies analyzed. Localized amyloidosis was found in the supraglottic region (n = 6), glottic region (n = 7), and subglottic region (n = 5); six patients had disease spanning two subsites. The most common finding on the CT scan was a homogeneous and well-defined submucosal soft tissue mass. Punctate calcifications were present in three cases. The presence of contrast enhancement was identified in the majority of patients who underwent MRI (4/5). MRI showed consistent signal intensity, hypointense, or isointense on both T1-weighted and T2-weighted images. Diffusion-weighted sequences were obtained in every patient and did not demonstrate diffusion restriction. CONCLUSION: This is the largest series searching for unifying imaging characteristics of laryngeal amyloidosis. This research suggests that characteristics from CT and MR provide both similar and unique features of laryngeal amyloidosis on imaging. Both modalities identify a submucosal mass. CT is the preferred modality to demonstrate punctate calcifications, while MRI identifies enhancement and altered signal characteristics. The main benefit of serial imaging is the correlation with patient symptoms, identification of the extent of disease, and assisting in delineating appropriate timing for surgery.

The subglottic involvement is an independent risk factor for recurrence of laryngeal amyloidosis.

OBJECTIVE: To analyze the risk factors for recurrence of laryngeal amyloidosis (LA). METHODS: The clinical data of patients with LA admitted in the Otolaryngology Head and Neck Surgery Department of the Second Affiliated Hospital of Xi'an Jiaotong University from August 2009 to June 2022 were analyzed retrospectively; then, the risk factors for recurrence and their impacts on the recurrence time were also analyzed. RESULTS: Of the 44 patients with LA, the majority (38 cases, 86.4%) only involved one anatomical region and the others (6 cases, 13.6%) involved two laryngeal regions concurrently. Overall, the glottic region was the most commonly affected area (28 cases, 63.6%), followed by the supraglottic region (16 cases, 36.4%) and subglottic region (6 cases, 13.6%). In addition, all the lesions were categorized as isolated nodule (31.8%), submucosal localized deposition (52.3%), and submucosal diffuse deposition (15.9%) according to their morphologies under electronic laryngoscope. Finally, six patients (13.6%) had recurrence after operation with a median recurrence time of 24.5 months, and subglottic involvement was confirmed to be an independent risk factor for recurrence of LA by univariate and multivariate logistic regression analyses (P < 0.05). Meanwhile, the patients with subglottic involvement presented as submucosal diffuse deposition had a considerable shorter recurrence time (t = 5.759, P = 0.005). CONCLUSION: The subglottic involvement is an independent risk factor for recurrence of LA.

Surgical treatment of laryngeal amyloidosis: a systematic review.

PURPOSE: The aim of this review was to study the surgical management of laryngeal amyloidosis and estimate the rate of recurrence after surgery. METHODS: A systematic review searching PubMed and EMBASE was performed. A qualitative synthesis of data regarding the surgical management of LA and a quantitative analysis of the recurrence rate after surgery was conducted. RESULTS: This systematic review included 14 retrospective studies, one of whom is retrospective controlled. A total of 515 subjects were included, the mean age ranged from 43.3 to 58 years with a male-to-female ratio of 1:1.3. All cases had a localized laryngeal amyloidosis. The supraglottic region was the most affected laryngeal site and multiple sites were commonly involved. Surgical treatment consists of endoscopic excision using laser, cold or powered instruments. Open surgery is required for severe primary case or revision surgery. Surgical complications such as granulomatosis scar tissue formation, tracheostomy, laryngotracheal stenosis, pneumothorax and concomitant malignancy were developed in 17.5% of patients. The time onset to diagnosis varied from 1 months to 15 years and the duration of follow-up from 3 months to 25 years. The rate of recurrence was 28.4% (95% CI 24.5-32.6) and the timing of recurrences ranged from 3 months to 10 years. CONCLUSION: The recurrence rate after primary surgery for laryngeal amyloidosis is high. A tailored surgical treatment based on the disease extension and a long-term follow up are recommended.

Pediatric Voice Disorders from the Perspective of Rare Diseases.

OBJECTIVE: This study aims to present rare pediatric voice disorders, which are usually reported as case reports in the literature, with their clinical features, treatment options, and prognosis, and to emphasize clinical findings that may be associated with rare diseases. METHODS: The clinical records of the pediatric patients presented with dysphonia were reviewed between 2014 and 2019. The terminology "rare disease" is used to describe diseases where the average prevalence thresholds are between 40 to 50 cases/100,000 people. Age, gender, symptoms, laryngeal examination findings, histopathological results, treatment modalities and follow-up results of rare laryngeal pathologies were reviewed. RESULTS: 274 children were diagnosed with and treated for voice disorders at our institution. Seven patients were identified with rare laryngeal pathologies. Four patients diagnosed with lipoid proteinosis, two patients with neurofibroma and one patient with amyloidosis. One patient was known to have neurofibromatosis type-1, while the others did not have any previously diagnosed diseases, dysphonia was the initial clinical presentation. CONCLUSION: There are some clues that bring to mind rare entities. Firstly, these patients generally present with various systemic manifestations. Secondly, if there is any discrepancy between voice quality and endoscopic laryngeal examination, the diagnosis should be reconsidered. Thirdly, in the case of the prolonged dysphonia recalcitrant to treatment, the clinician should reassess the treatment or the diagnosis.

A Nomogram Model for Predicting the Postoperative Recurrence of Localized Laryngeal Amyloidosis.

OBJECTIVE: To analyze the factors related to postoperative recurrence in patients with localized laryngeal amyloidosis (LocLA) and to construct a nomogram prediction model (NPM). METHODS: We collected the data for LocLA patients diagnosed from March 2000 to May 2019 and clinical characteristics data were extracted. Factors related to recurrence were analyzed using multivariate logistic regression. The NPM was constructed for predicting the recurrence risk of LocLA. The receiver operating characteristic (ROC) curve evaluated the distinguishing ability using the area under curve (AUC). The calibration curve was created to evaluate the consistency of the NPM. RESULTS: A total of 226 confirmed LocLA cases were included. One hundred seventy-five cases (77.4%) had localized single nodule, and 51 cases had more than one lesions. Sixty-three (27.9%) cases had no multinucleated giant cell (MGC) around amyloid, and 163 (72.1%) cases had MGC around amyloid. Multivariate logistic regression analysis showed that more than one lesions (odds ratio [OR] = 3.206 and 95% confidence interval [CI]: 1.492-6.888; P value: .003), subglottic involvement (OR = 2.926 and 95% CI: 1.300-6.585; P = .010), and no multinucleated giant cell (MGC) around amyloid (OR = 2.503 and 95% CI: 1.173-5.342; P = .018) had a statistically significant effect on postoperative LocLA recurrence (P < .05). The AUC of the ROC curve was 0.753 (95% CI: 0.667-0.832). The bias-corrected curve approached the ideal curve, with an average absolute error of 0.037. CONCLUSIONS: More than one lesions, subglottic involvement, and no MGC around amyloid are risk factors for postoperative recurrence of LocLA. The NPM constructed has good applicability.

Amyloidosis in the nasopharynx and larynx: a case report.

Amyloidosis is a disease caused by amyloid deposition in tissues or organs. According to the extent of the lesion, it can be divided into systemic amyloidosis and localized amyloidosis. Amyloidosis originating in the larynx accounts for approximately 0.5% to 1.0% of benign lesions of the larynx; such lesions are relatively rare and mostly localized. Nasopharyngeal amyloidosis combined with laryngeal amyloidosis is even rarer. We herein present a case involving a patient with amyloidosis in the nasopharynx and larynx who presented with a foreign body sensation and hoarseness in the pharynx. Electronic fiber laryngoscopy revealed a smooth neoplasm in the left nasopharynx and left vocal cord. The patient underwent surgical treatment, and the postoperative pathologic examination results suggested amyloidosis. Special staining performed using Congo red and crystal violet was positive, confirming amyloidosis. The patient recovered after surgery, and no recurrence was present at the 3- and 6-month follow-ups.

Long-term Follow-up After Radiation Therapy for Laryngeal Amyloidosis.

OBJECTIVES: Laryngeal amyloidosis (LA) is a rare disease characterized by extracellular protein deposition within the larynx. Treatment is difficult due to the frequently submucosal and multifocal nature of disease. The mainstay of treatment is surgical resection; however, recurrence rates are high. Recently, use of radiotherapy (RT), either alone or postoperatively, for LA has been adapted from the management of extramedullary plasmacytoma and has been shown to provide local disease control. Here, we describe the experience with adjuvant RT for LA at our center. STUDY DESIGN: Retrospective case series. METHODS: Retrospective study of patients with amyloidosis of the larynx, with or without other disease sites, seen at a tertiary academic center between 2011 and 2019. Outcomes included disease characteristics, recurrence rates, treatment modalities, and pre- and posttreatment voice handicap index (VHI)-10. RESULTS: Ten patients met eligibility criteria. Mean follow-up time for all patients was 62.0 ± 41.0 months; mean follow-up time after last treatment was 51 ± 55 months. All but one patient underwent surgical resection of disease. Seven patients underwent subsequent RT. Of these seven, six underwent RT at our institution; five received a dose of 45 Gray (Gy); and one received a dose of 20 Gy. All seven completed RT without toxicity-related interruption. Patients undergoing RT underwent 2.1 ± 1.3 surgical procedures prior to RT; no patients required surgery after RT. Mean pretreatment VHI-10 was 22.9 ± 8.1; mean posttreatment VHI-10 was 12.9 ± 13.3. CONCLUSION: RT after surgery for LA can provide good local control without unacceptable toxicity and may decrease the need for further surgery. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:1810-1815, 2021.

Risk factors for recurrence of laryngeal amyloidosis treated by microforceps and CO2 laser.

INTRODUCTION: Laryngeal amyloidosis is a benign, slowly progressive disease. The factors affecting the recurrence of LA have not been studied before for the rarity and incomplete understanding of this disease. To investigate the risk factors for the laryngeal amyloidosis treated by microforceps or carbon dioxide laser under microlaryngoscope, a retrospective review was conducted. MATERIALS AND METHODS: One hundred and four patients (42 male and 72 female, with an average age of 51.3 years) with laryngeal amyloidosis were identified. The cases were collected from January 1989 to May 2014 at the Eye, Ear, Nose, and Throat Hospital of Fudan University. RESULTS: Seventy-five patients complained of hoarseness, 36 patients complained of hoarseness and dyspnea, and 3 patients complained of foreign body sensation. All patients underwent surgical removal of the amyloid deposits (via microforceps, CO2 laser and tracheotomy). Thirty patients developed recurrences requiring further treatments. The duration from onset to the treatment and age affected the recurrence of laryngeal amyloidosis, the surgery method, stenotic degree and stenotic area of subglottic area and trachea did not affect the recurrence of the disease. CONCLUSION: Early diagnosis and treatment of laryngeal amyloidosis may reduce the recurrence of the disease. Regular follow-up is necessary to find any recurrence.

Curative effect of cold micro forceps and CO2 laser in the treatment of laryngeal amyloidosis.

Background: Amyloidosis is a benign, slowly progressive disease. However, the treatment of laryngeal amyloidosis (LA) has not been studied systematically.Objectives: To investigate the treatment results of cold micro forceps or carbon dioxide laser under microlaryngoscope in the treatment of LA.Methods: A retrospective review was conducted for the cases collected from January 1990 to March 2016 at our department.Results: One hundred and eighteen patients (44 males and 74 females, with an average age of 51.7 years) with LA were identified. The amyloidosis was not found in organs other than the larynges and tracheas. The patients most commonly presented with hoarseness and dyspnea. All patients underwent surgical removal of the amyloid deposits (via micro forceps or CO2 laser). Twenty-nine patients developed recurrences requiring further treatments. The mean interval from treatments to recurrences in these patients was 16.5 months. Five patients were treated more than once. No significant difference was found between cold micro forceps and CO2 laser methods in recurrences.Conclusions: LA generally behaves as a benign disease. Resection of LA either via cold micro forceps or CO2 laser under microlaryngoscope is both safe and effective methods. Regular follow-up with laryngoscopy is indicated for early diagnosis of recurrence.

[A case report of amyloidosis of the larynx in a 13-year-old girl]. / Sluchai amiloidoza gortani u 13-letnei devochki.

Amyloidosis of the larynx is rare in the pediatric age group. We present a clinical case of laryngeal amyloidosis manifesting as false vocal fold bulging in a thirteen-year-old girl with hoarseness and with initial suspicion of laryngeal tumor. Computed tomography and ultrasound scanning of the larynx were useful in determining the laryngeal process size along with fibro laryngoscopy. There was performed a microsurgical removal of the larynx formation. The results of Congo red staining of the formation were characteristic of amyloid. Systemic manifestations of the disease were not revealed.

Sleep disordered breathing and dysphonia in a pediatric patient - Laryngeal amyloidosis as an unusual diagnosis.

Primary laryngeal amyloidosis is an uncommon condition, and cases in the pediatric population are even rarer. We present a case of a nine year old female patient who presented with sleep disordered breathing and dysphonia to our outpatient clinic. The patient underwent Microlaryngoscopy and Bronchoscopy for diagnosis which identified a large soft tissue mass in the supraglottis. After Histological diagnosis was made, she had subtotal debridement of the mass and has maintained a good exercise tolerance with no airway compromise.

Isolated Laryngeal Amyloidosis Mimicking Laryngeal Cancer.

Amyloidosis is the deposition of an extracellular fibrillar protein in the tissues leading to organ dysfunction. Laryngeal amyloidosis is a rare phenomenon. We report a case of isolated laryngeal amyloidosis which was initially suspicious for laryngeal cancer on magnetic resonance imaging (MRI) but histopathology showed the presence of amyloid. Systemic workup was negative. The patient is being managed conservatively.

Can videolaryngoscopy be a first option in a patient with laryngeal amyloidosis? / ¿Puede la videolaringoscopia ser una primera opción en paciente con amiloidosis laríngea?

Amyloidosis is a term that involves a group of diseases characterised by deposition of extracellular monoclonal light-chain fibrillar immunoglobulin aggregates in the body, including many organs, with the larynx among them. A case is presented of a 78 year-old man who was referred to our institution for strangulated umbilical hernia treatment. He suffered from progressive hoarseness and dysphagia for 5months. He had a history of primary laryngeal amyloidosis. Awake intubation was performed successful with the King Vision® video-laryngoscopy. Sedation was achieved using a remifentanil infusion and midazolam. Haemorrhagic lesions are caused by deposition of amyloid in and around vessels, resulting in increased vascular fragility. Therefore, anaesthetists should take care in intubating the tracheas of these patients.

Laryngeal amyloidosis: diagnosis, pathophysiology and management.

BACKGROUND: Laryngeal amyloidosis represents approximately 1 per cent of all benign laryngeal lesions, and can cause variable symptoms depending on anatomical location and size. Treatment ranges from observation through to endoscopic microsurgery, laser excision and laryngectomy. OBJECTIVES: To highlight the diversity of presentations, increase awareness of paediatric amyloidosis and update the reader on current management. CASE SERIES: Five cases are illustrated. Four adult patients were female, and the one child, the second youngest in the literature, was male. Amyloid deposits were identified in all laryngeal areas, including the supraglottis, glottis and subglottis. Treatment consisted of balloon dilatation, endoscopic excision, laser cruciate incision, and resection with carbon dioxide laser, a microdebrider and coblation wands. CONCLUSION: Laryngeal amyloidosis remains a rare and clinically challenging condition. Diagnosis should be considered for unusual appearing submucosal laryngeal lesions. Treatment of this disease needs to be evaluated on a case-by-case basis and managed within an appropriate multidisciplinary team.

Long-term follow-up after surgery in localized laryngeal amyloidosis.

To study effectiveness of surgery and watchful waiting in localized laryngeal amyloidosis, retrospective case series. This retrospective study comprises all consecutive patients with localized laryngeal amyloidosis surgically treated in a tertiary hospital between 1994 and February 2016. Recurrence rate, revision surgery, progression to systemic amyloidosis, and changes in voice were monitored yearly. Eighteen patients were included. Seven women and eleven men had a median age 50 years (range 21-77 years) and median follow-up 6.4 years (2.4-17 years). Amyloid was located in subglottis (5), glottis (8), false vocal folds (8) and other supraglottic areas (5), in more than one laryngeal region (13) and bilaterally (12). Cold steel excision was used at the glottis; CO2 laser excision, sometimes assisted by microdebrider, at other laryngeal areas. Eleven patients needed revision surgery, ten within the first 4 years after surgical treatment. One patient needed his first revision surgery after 11 years. Five patients needed a second revision within 6 years after initial diagnosis. Two patients needed a third revision. Indications for first revision surgery were progression (8) with dysphonia (7), dyspnea (2), dysphagia (1), exclusion of malignancy (1), and aphonia (1). No patient developed systemic amyloidosis during follow-up. Although local progression of amyloid necessitates revision surgery once or twice in the first 4-6 years, progression slows down thereafter. Late progression, however, remains possible.

Supraglottic adenoid cystic carcinoma mimicking laryngeal amyloidosis: A case report.

Supraglottic adenoid cystic carcinoma (ACC) is extremely rare and may be misdiagnosed as laryngeal amyloidosis. The present report describes a case of supraglottic ACC, which went unrecognized until histopathological examination of the neoplasm 18 months after the first presentation. The present patient presented with progressive hoarseness for half a year and initially required partial resection. Following quick regional recurrence, the patient received a total laryngectomy while refusing radiotherapy. Adjuvant post-operational traditional Chinese medicine was accepted. Over 3 years' follow-up, there was no evidence of regional relapse or distant metastases. The present case is compared with a second case of supraglottic submucosal mass in which the signs, symptoms and examinations were similar to the first case, but that was diagnosed as laryngeal amyloidosis. Attention should be paid to submucosal masses in the larynx to prevent underlying malignancy and subsequent disease progression. Immunocytochemistry, such as p63 staining, is mandatory for making an early differential diagnosis of supraglottic ACC. Traditional Chinese medicine may be a useful adjuvant therapy for this rare disease.

Combined laryngocele secondary to localized laryngeal amyloidosis.

Laryngocele is a benign condition due to abnormal dilatation of the laryngeal saccule. Localized amyloidosis causing laryngocele is a rare entity with few reports in the literature. We present a young male patient with a large combined laryngocele secondary to laryngeal amyloidosis.

An Unexpected Cause of Hoarseness of Voice in a Healthy Teenager

Laryngeal amyloidosis is a rare cause of stridor in a healthy young adult. We report a case of localised laryngeal amyloidosis, including our MRI findings, which included a necrotic centre that has not previously been described. This case also highlights the need for a high index of clinical suspicion to achieve the correct histopathological interpretation