Localized Laryngeal Amyloidosis Research Articles

Surgical treatment of laryngeal amyloidosis: a systematic review.

The aim of this review was to study the surgical management of laryngeal amyloidosis and estimate the rate of recurrence after surgery. 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. 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 58years 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 1months to 15years and the duration of follow-up from 3months to 25years. The rate of recurrence was 28.4% (95% CI 24.5-32.6) and the timing of recurrences ranged from 3months to 10years. 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.

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

To analyze the factors related to postoperative recurrence in patients with localized laryngeal amyloidosis (LocLA) and to construct a nomogram prediction model (NPM). 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. 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. 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.

Клинико-морфологические особенности локального амилоидоза гортани

Introduction. Amyloidosis is a disease characterized by extracellular deposition of insoluble fibrils composed of an abnormal protein, amyloid. This protein builds up in various tissues and organs and interferes with their function leading to atrophy and sclerosis. Amyloidosis is usually a systemic disease. However, there are case reports of localized amyloidosis. Localized laryngeal amyloidosis is a rare condition accounting for approximately 15% of localized amyloidosis. The aim of the research was to describe morphological features of localized laryngeal amyloidosis. Materials and methods. We analyzed clinical findings, biopsy, and surgical specimens of 6 patients with localized laryngeal amyloidosis. Amyloid deposits were detected in tissue sections with Congo red stain with polarized light microscopy. We performed immunohistochemical analysis with monoclonal and polyclonal antibodies to detect different amyloid types. Results. Laryngeal amyloidosis was diagnosed in 4 male and 2 female patients aged from 44 to 62 (the mean age was 54 years). We found amyloid deposits in the interstitial spaces and tumor-like masses; in polarized light, amyloid showed a bright apple-green birefringence. Inflammatory cell infiltrate and multinucleated giant cells were visualized in most cases. Based on the immunohistochemical typing results, 4 patients had AL-kappa amyloidosis and 2 patients were diagnosed with AL-lambda amyloidosis. The disease recurrence was reported in 2 cases. Conclusion. In the head and neck area, the larynx is the most common site affected by localized amyloidosis. AL-kappa amyloidosis prevailed in our study, most of them being in men. In view of high recurrence rates, a long-term follow-up is needed after the amyloid deposit excision. Keywords: amyloidosis, larynx, amyloidoma, localized amyloidosis, immunohistochemistry

Primary Localized Amyloidosis of Larynx: A Case Report.

Primary localized laryngeal amyloidosis is a rare presentation of amyloid deposition. A case of 22years old male presented with progressive hoarseness of voice and supraglottic mass on laryngoscopy reported here. Complete excision of mass was done using Coblator wand. Histopathological examination and Congo red stain demonstrated AL amyloidosis of Larynx.

One Hundred Cases of Localized Laryngeal Amyloidosis - Evidence for Future Management.

To update the current understanding of localized laryngeal amyloidosis by analyzing the NHS National Amyloidosis Database and to further clarify the important ongoing management issues. Retrospective review, case series. Patients with laryngeal amyloid were identified from the database of the NHS National Amyloidosis Center, UCL, Royal Free Hospital, London between 2000 and 2017. Patient demographics and disease profile were collated, including the exact location of amyloid deposit, treatments if any, and progression of disease. One hundred and three patients with localized laryngeal amyloid where identified from the database, with a mean age of 54 at diagnosis and female to male ratio of 54:49. Three patients were excluded from further analysis due to limited database information. The majority of amyloid was found in either the supraglottis (44) or glottis (53) but all the laryngeal subsites were involved. One-third of the patients (34) had amyloid in more than one laryngeal subsite. No patients were found to progress to systemic amyloid, but many progressed locally to other subsites or further down the LTB tree (29%). Three patients were successfully treated with radiotherapy after other modalities had failed. This is the largest case series reported to date of localized laryngeal amyloidosis. It highlights the high incidence of multifocal disease and the significant proportion of patients who progressed, not to systemic amyloidosis but to more extensive localized amyloid. We recommend that in all cases of laryngeal amyloid, patients should undergo a thorough assessment of the upper and lower airways and have ongoing surveillance for at least 15 years. 4 Laryngoscope, 131:E1912-E1917, 2021.

Laryngeal amyloidosis: a case report

&lt;p class="abstract"&gt;Amyloidosis is a rare, benign, slowly progressive disease characterized by extracellular accumulation of&lt;strong&gt; &lt;/strong&gt;amyloid in different tissues of the body. It accounts for 0.2-1.2% of benign laryngeal tumors and usually presents as an isolated localized laryngeal amyloidosis, but can also be part of systemic amyloidosis. A 26 years old female with history of gradually developing, persistent hoarseness, and progressive&lt;strong&gt; &lt;/strong&gt;dyspnea since 1 year, worsened over the past three days. Outpatient Department based endoscopy showed bilateral mobile, thickened vocal cords with subglottic edematous thickness. Computed tomography scan showed symmetrical thickening of bilateral vocal cord, causing glottic narrowing about 70% on the AP view and about 50% on the lateral view and no cartilage invasion or lymphadenopathy. Microlaryngoscopy and biopsy of the specimen were performed and histopathology confirmed the diagnosis of amyloidosis with Congo red stain. Patient was managed by surgical excision of the mass and long term follow-up. To rule out systemic amyloidosis patient is referred to rheumatology clinic and hematology clinic for further evaluation and management. Histopathological examination of the involved tissue confirms the diagnosis, and long term follow up is mandatory in the management of amyloidosis.&lt;/p&gt;

Focus on localized laryngeal amyloidosis: management of five cases.

Amyloidosis is a group of idiopathic clinical syndromes caused by the deposition of insoluble fibrillar proteins (amyloid) in the extracellular matrix of organs and tissues. These deposits disrupt the function of the target organ. Amyloidosis can manifest as a systemic disease or a single-organ involvement (local form). Its etiology still remains unclear. Deposits of amyloid in the larynx are rare, accounting for between 0.2 and 1.2% of benign tumors of the larynx. In this retrospective study, we report the clinical aspects, diagnosis, treatment and follow-up of five female patients with localized laryngeal amyloidosis without systemic involvement. The patients were all treated successfully using microlaryngoscopy with CO2 laser or cold instruments. Prognosis is excellent; however, appropriate follow-up is an important part of the long-term management of this disease in order to prevent and control the possibility of local recurrence.

Localized subglottic laryngeal amyloidosis: a case report

Amyloidosis is a heterogeneous group of diseases characterized by accumulation of amyloid protein in different organs of the body. Localized affection of the larynx by amyloid deposits is a rare event. This is a case of localized laryngeal amyloidosis in a 24-year-old female patient presented with hoarseness of voice. The amyloid mass was occupying the anterior part of the subglottic region and reaching up between both the vocal folds. Complete excision was achieved by cold instruments. Localized laryngeal amyloidosis despite being rare should be kept in mind as one of the causes of mass lesions in the larynx.

A Case of Recurrent Localized Laryngeal Amyloidosis

Herein, we report a case of recurrent localized laryngeal amyloidosis in a patient who had been followed up for 15 years after the first treatment. A 63-year-old male was referred to our department with the chief complaints of hoarseness and dyspnea. Several examinations showed submucosal swelling of the subglottic area bilaterally. A biopsy specimen obtained by laryngomicrosurgery showed submucosal deposition of amyloid. Debulking surgery was performed via an opening of the trachea. However, 9 months after the first surgery, the patient presented to us again complaining of dyspnea. We performed revision surgery for a repeat debulking of the lesion via a laryngofissure. His postoperative course was good and his dyspnea improved. However, 13 years after the revision surgery, the patient presented yet again with dyspnea. Therefore, we performed a third surgery using the same procedure as in the previous operation. Despite the repeated recurrences, we performed debulking of the lesion, and not total resection. Since amyloidosis is a benign disorder, and the larynx has important functions, organ-preserving surgery is recommended in cases of laryngeal amyloidosis.

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.

Localized Laryngeal Amyloidosis

ABSTRACT Amyloidosis in head and neck is rare but a benign disease. Among the sites in head and neck, larynx is the most commonly involved. A 40-year-old female had presented with hoarseness of voice and dyspnea with a mass in false vocal cord, which was biopsied and diagnosed as amyloidosis. After excluding systemic diseases, final diagnosis of localized laryngeal amyloidosis was given. How to cite this article Baruah R, Khaund G, Kalita A. Localized Laryngeal Amyloidosis. Int J Phonosurg Laryngol 2016;6(1):32-34.

English

Primary localized laryngeal amyloidosis is an extremely rare condition of unknown etiology. It usually presents with hoarseness of voice. Dysphagia is seen in some cases. We present the case of a 28-year-old woman with primary localized laryngeal amyloidosis who presented with hoarseness of voice. There were no signs of any systemic disease in our patient and diagnosis was established histopathologically. She was treated surgically by micro laryngoscopy under general anesthesia and the mass was excised using a CO2 laser technology method.

Laryngeal amyloidosis, report of a new case and review of the literature

IntroductionAmyloidosis is related to the extracellular deposition in various tissues of an amorphous amyloid substance. Isolated laryngeal localization is very rare. ObservationWe report a case of localized laryngeal amyloidosis discovered during an isolated chronic dysphonia in a 45-year-old man, with no medical history, with no alcohol or tobacco intoxication. The histological and immunohistochemical study and further examinations confirmed the diagnosis of localized laryngeal amyloidosis without systemic location. The patient had a slight postoperative hoarseness that subsequently disappeared through speech therapy. DiscussionThe larynx remains a rare localization of amyloidosis. It is the most common form of amyloidosis of the upper aero digestive tract. Circumstances of discovery of laryngeal amyloidosis are dominated by hoarseness. Laryngoscopy may falsely suggest a neoplastic disease. Diagnosis is based upon histological examination with Congo red staining where amyloid deposits appear birefringent under polarized light. Surgery is the mainstay of treatment of symptomatic laryngeal amyloidosis. The survival of patients with laryngeal amyloidosis often exceeds ten years. ConclusionLaryngeal amyloidosis is essentially a local clinical form whose main symptom is dysphonia. The treatment is usually based on local endoscopic procedures but may require a laryngectomy in advanced forms, associated with colchicine. The prognosis is much better than systemic forms.

Primary Localized Laryngeal Amyloidosis (LA)

Abstract 4596 Background:Localized Laryngeal Amyloidosis (LA) is a rare condition. There is limited understanding of its natural history. Our aim is to report the clinical evolution of a patient diagnosed with localized LA. Methods:Case report. Results:In May 2002, a 43-year-old gentleman presented with hoarseness and a CT scan of the neck showed a thickening of the right vocal cord with a mass that measured 2.9 × 1.2 cm in widest diameter. The patient had this mass removed surgically and the pathology showed a positive Congo red staining and a positive birefringence under polarized light, diagnostic of amyloidosis. No evidence of systemic amyloidosis or an overt B-cell lymphoma was found in this patient.Patient lost follow-up until October 2008 when he was found to have soft tissue induration around the left false vocal cord area. The biopsy showed again amyloidosis. Conclusions:LA recurrences can manifest several years after initial diagnosis. Long-term follow-up is essential. Disclosures:No relevant conflicts of interest to declare.

A "Boxer Glove" Contoured Laryngeal Amyloidosis

Laryngeal amyloidosis is uncommon and poorly understood, with limited long-term studies. Although primary localized laryngeal amyloidosis is extremely rare, it frequently involves the ventricles and the false and true vocal cords. We present a 58-year-old woman with localized laryngeal amyloidosis who presented no symptoms. On indirect laryngoscopic examination, the mass involved both arytenoids only, and had a likeness to a "boxer glove." She was treated surgically by microlaryngoscopy under general anesthesia and the mass was excised using a carbon dioxide laser. We present this case and a review of literature.

A Rare Case of Hoarseness: Laryngeal Amyloidosis

This is the case of a 72 year old male who presented with a three year history of hoarseness. Two years prior to admission, he developed dyspnea, dysphagia and odynophagia. Three months prior to admission, the patient visited a private physician who noted a 3 cm diameter ill-defined laryngeal thickening and advised biopsy, hence the present consult. A biopsy and subsequent hemilaryngectomy were performed.&#x0D; Histologic sections from the mass show an abundant subepithelial deposition of an acellular, extracellular, eosinophilic and non-fibrillar matrix that surrounds atrophic mucous acini (Figs. 1, 2, 3). Scattered lymphocytes and foreign-body-type giant cells are noted. Congo-red histochemical stains show a characteristic salmon-pink reaction (Fig. 4) which on polarizing microscopy showed a characteristic apple-green birefringence. No evidence of malignancy is seen. The diagnosis was localized laryngeal amyloidosis.&#x0D; Laryngeal amyloidosis is rare, accounting for less than 1% of benign laryngeal tumors. Most are localized - not associated with deposits elsewhere in the body, and are primary – not associated with any known systemic or neoplastic diseases such as disseminated tuberculosis or certain lymphomas and multiple myeloma. Adults are usually affected although rarely, children can develop the condition. Almost all patients present with hoarseness, in addition to symptoms related to the size, extent and location of the deposits. The false vocal cord is often affected although multifocality within the larynx occurs in 15% of cases. 1, 3&#x0D; Careful histologic evaluation should exclude the presence of a coexistent laryngeal condition, particularly the malignant neoplasms stated above, as these would require more extensive management. Other differential diagnoses include vocal cord polyps and papillomas. 2, 3&#x0D; The prognosis for isolated laryngeal amyloidosis is excellent although occasional repeat surgeries may be necessary for recurrent disease. On the other hand, the prognosis of laryngeal amyloidosis associated with systemic or neoplastic conditions is dictated largely by the course of the coexisting disease processes. 1&#x0D; Although rare, laryngeal amyloidosis should not be overlooked in the differential diagnosis of hoarseness particularly in adults. An attempt to exclude a co-existing systemic or neoplastic disease should always be made.

Plasmocitoma extramedular de laringe. A propósito de un caso

Extramedullary plasmacytoma of the larynx and localized laryngeal amyloidosis are two entities that are extremely rare in children. We report the case of an 11-year-old child presenting with progressive dysphonia, with a diagnosis of extramedullary plasmacytoma and localized laryngeal amyloidosis. The treatment he received and subsequent follow-up were compared with the few cases found in the literature.

Laryngeal presentation of systemic apolipoprotein A‐I–derived amyloidosis

To study the clinical and pathological characteristics of two patients with laryngeal apolipoprotein A-I (apoA-I)-derived (AApoAI) amyloidosis with the apolipoprotein A-I variants Leu174Ser and Leu178Pro, respectively. The latter variant has not been associated with amyloid before. Descriptive report of two patients who presented with laryngeal amyloid presumed to be of localized AL type, but in who further assessments demonstrated systemic amyloidosis. The larynx was examined by videolaryngostroboscopy. The voice was analyzed with the GRBAS system, phonation times, and phonetography. Laryngeal biopsies were stained with Congo red and analyzed immunohistochemically. Organ function was assessed and tissue involvement by amyloid further determined by rectal biopsy, abdominal fat tissue aspirate, and serum amyloid P component scintigraphy. The appearance of the laryngeal amyloid was unusual in both patients, occurring as small, irregular floppy proliferations affecting the borders of both vocal folds. Amyloid was stained with antibodies to apoA-I and not with antibodies to immunoglobulin light chains. The 45-year-old woman with the previously described amyloidogenic apoA-I Leu174Ser variant had possible involvement by amyloid in joints, peripheral nerves, and heart. Whereas in the 67-year-old man with apoA-I Leu178Pro there was a clinical suggestion of autonomic and cardiac amyloid and histological corroboration of systemic amyloidosis in abdominal fat. Laryngeal symptoms may be the presenting feature of hereditary systemic AApoAI amyloidosis, and comprehensive investigations including apoA-I genotyping are warranted in patients who present with apparently localized laryngeal amyloidosis. The distinctive appearance of the amyloidotic vocal folds described here may further signal the possibility of hereditary AApoAI type.

Primary localized laryngeal amyloidosis presenting with hoarseness and dysphagia: a case report.

IntroductionPrimary localized laryngeal amyloidosis is an extremely rare condition. It usually presents with hoarseness, pain and/or difficulty in breathing.Case presentationWe present the case of a 23-year-old woman with primary localized laryngeal amyloidosis who presented with hoarseness and dysphagia.ConclusionA search of PubMed shows that dysphagia in patients with laryngeal amyloidosis has been reported only once, although this symptom is relatively common in other conditions presenting with laryngeal mass. There were no signs of any systemic disease in our patient and diagnosis was established histopathologically. She was treated surgically by microlaryngoscopy under general anesthesia and the mass was excised using a CO2 laser technology method.

Case 138: Idiopathic Spinal Cord Herniation

A 66-year-old woman presented with numbness in the anteromedial aspect of her right thigh and difficulty with right leg movement for the past year. She did not have back pain or lower limb pain, nor did she have a history of relevant trauma. At the time of presentation, her arterial hypertension and osteoarthritis were being treated. She had a history of localized laryngeal amyloidosis, which was found at 30 years of age and treated with laser excision and tracheostomy. Physical examination revealed mild spasticity and impaired pain and temperature sensation in the right leg. She underwent magnetic resonance (MR) imaging, myelography, and postmyelography computed tomography (CT) of the thoracic spine.