Primary Laryngeal Amyloidosis and its Response to Surgical Treatment
- 1. Department of Otorhinolaryngology, Vardhmann Mahavir Medical College and Safdarjung Hospital, India
ABSTRACT
We have reported two male patients presenting with hoarseness and stridor respectively to the outpatient department, with later requiring emergency tracheostomy as life saving procedure. Endoscopic excision biopsies of the laryngeal lesions were performed and histopathological examination revealed amyloidosis. In the first patient, endoscopic excision of the mass was done with cold knife and in the second, treated with staged endoscopic coblation followed by decannulation. There was no recurrence of disease in both patients at one year of follow up
KEYWORDS
Amyloidosis, Larynx, Stridor, Endoscopic coblation
CITATION
Pradhan P, Bhardwaj A, Venkatachalam VP (2015) Primary Laryngeal Amyloidosis and its Response to Surgical Treatment. Ann Otolaryngol Rhinol 2(9): 1062.
INTRODUCTION
Amyloidosis is characterized by the extracellular deposition of fibrillar proteins in various organs leading to organ failure [1]. It commonly affects individuals between 50 and 70 years of age and predominantly seen in males [2,3]. It can affect the respiratory tract diffusely from larynx to smallest bronchioles [4] and patients can have variable presentation according to the site of amyloid deposition. Laryngeal amyloidosis is a very rare benign lesion, which accounts for 0.2 to 1.2% of benign tumors of the larynx [5]. In the larynx, vocal cords are the commonest site to be affected [6]. Laryngoscopic examination usually reveals a diffuse mucosal thickening or subepithelial nodule; hence a deep biopsy is always required for diagnosis and to rule out malignancy. Histopathology is the gold standard for final diagnosis. There are various modalities of treatment options for laryngeal amyloidosis. Although carbon dioxide laser or cold knife excision is the standard treatment practiced [3], other treatments including corticosteroids, radiotherapy, chemotherapeautic agents like colchicine and melphalan have been tried for the treatment of laryngeal amyloidosis with variable results. In this case report, the first case was surgically treated using cold knife while in the second case, endoscopic coblation was used for complete excision of the mass. To the best of our knowledge, there are few case reports in the literature where coblation is being used for laryngeal pathology, but we have successfully used it as a complementary treatment modality to the standard treatment for laryngeal amyloidosis.
CASE 1
A 45 year old male patient presented to our Otorhinolaryngology outpatient clinic with history of hoarseness for 6 months. Patient was a non smoker, with no history of vocal abuse or chronic illness in the past. Systemic examination did not reveal any abnormality. Endoscopic evaluation revealed a polypoidal mass in the anterior one third of left false vocal cord. After general anesthesia, a self-retaining laryngoscope was introduced. Under endoscopic (0 degree and 30 degree endoscopes) vision, excision biopsy of the mass was done. Histopathological examination revealed submucosal deposition of acellular amorphous eosinophilic material that exhibited apple-green birefringence under polarized light with Congo red staining, which is consistent with the diagnosis of amyloidosis. Hematological investigations including complete blood counts, C-reactive protein, ESR, liver function tests, kidney function tests, rheumatologic tests, ophthalmological examination, ECG, echocardiography and serum protein, were carried out to rule out systemic amyloidosis and found to be within normal limits. No abnormal band was found in serum protein electrophoresis. Abdomen ultrasound and echocardiography were normal. All the above investigations ruled out any systemic involvement. Patient was on regular follow-up for one year after treatment and is asymptomatic without any recurrence.
CASE 2
A 50 year old male patient presented with dyspnoea and stridor of 6 days duration and hoarseness for 6 months. Emergency tracheostomy was done. He was a non smoker, non alcoholic, and there was no history of any chronic illness. Endoscopic picture demonstrated a smooth mass extending from anterior commissure to subglottis, almost occluding the airway. Both true vocal cords were mobile (Figure 1).
Figure 1: Endoscopic view of a polypoidal mass extending to subglottis.
Rest of tracheobronchial examination was found normal. Contrast enhanced CT scan of neck revealed a circumferential, non enhancing soft tissue mass, extending superiorly from the level of the vocal cord to the upper border of the tracheostomy (Figure 2).
Figure 2: CT scan image showing a non contrast soft tissue mass in the subglottis occluding the lumen.
There was no cervical lymphadenopathy. Subsequently endoscopic biopsy was taken under general anaesthesia. After non specific pathological findings of two consecutive biopsies, a deep biopsy was planned from the subglottic mass. Histopathological confirmed a diagnosis of amyloidosis (Figure 3).
Figure 3: Photomicrograph showing apple green birefringence under polarized light with Congo red staining (arrow) (H & E × 400).
Complete clinical, hematological and radiological investigations were carried out to exclude any systemic involvement. Patient was treated by staged endoscopic excision of amyloid mass using coblation technique. After general anesthesia, a self-retaining laryngoscope was introduced. Under endoscopic (0 degree and 30 degree endoscopes) vision, Plasma ablation system, EVac 70 Xtra Plasma Wand (Sunnyvale, CA, USA) was used to remove the laryngeal lesion with preservation of normal mucosa. Coblation wand was used to both cut and coagulate to achieve complete excision and adequate hemostasis. After three sittings of coblation the patient was decannulated. He was on close follow up for one year after treatment and is asymptomatic without recurrence.
DISCUSSION
Amyloidosis is a dysproteinemia in which a characteristic amorphous, eosinophilic extracellular deposit is seen in tissues of various organs. By its encroachment or pressure effects over adjacent cells, it causes organ dysfunction [4]. It can occur in a variety of clinical conditions demonstrating similar histopathological features [4]. According to the site of involvement, amyloidosis is classified as localized (limited to particular organ) or systemic amyloidosis (involving almost all tissues). Clinically systemic amyloidosis may be familial, primary (with or without myeloma) or secondary (associated with chronic inflammatory diseases like rheumatoid arthritis, tuberculosis etc). Amyloidosis involving the respiratory tract is usually of primary type and it can affect the whole length of the respiratory tract from larynx to bronchioles [4]. Patients usually present with non specific symptoms like hoarseness, cough, hemoptysis, dyspnoea, stridor of which hoarseness is the commonest presentation [7]. Proper endoscopic assessment of the mass is the initial step for diagnosis, which usually reveals mucosal hypertrophy or submucosal nodule. Laryngeal amyloidosis can mimic laryngeal malignancy (Table 1)
Table 1: Benign and malignant lesions of larynx.
| Benign lesions of Larynx | Malignant lesions of larynx |
|
Laryngeal papilloma |
Verrucous carcinoma |
and deep biopsy with histopathological examination becomes important in differentiating the two. Complete hematological and radiological workup is carried out to rule out systemic involvement. No general consensus exists with regard to optimal treatment resulting in a variety of modalities used in clinical practice to manage this disorder. Endoscopic surgical excision of mass by cold knife or CO2 laser is the mainstay of treatment [3]. Lang SM et al [8] treated tracheobronchial amyloidosis with endoscopic forceps debulking and external beam radiation. In case report of laryngeal amyloidosis by Wang Q et al [9], lesion was excised on staged laryngoscopy under general anesthesia. In study by Deviprasad D et al [10], biopsy proven laryngeal amyloidosis lesions were excised microendoscopically using KTP 532 laser in three patients. In study by Behnoud F et al [11], FVC mass was excised with a CO2 laser. Golombick T et al [12] administered curcumin to a patient with laryngeal amyloidosis and smoldering myeloma and found that their patient shown a lack of progression of his disease for a period of five years. A study conducted by Herbert and Krzysztof which included 10 patients of localized amyloidosis, demonstrated that medical management is ineffective for the treatment of amyloidosis [13]. In our first case, the mass was excised by cold instrumentation and in the second case; it was effectively removed by staged endoscopic coblation. Resection of lesion was done in staged sitting. In first sitting, maximum possible debulking was done and radical removal was not done so as to avoid any possible damage to the airway due to unidentified resected margins. Further sittings were done at 3 weeks interval to remove residual lesion, fibrotic tissue and granulation tissue. Patient was decannulated after third sitting.
Coblation uses bipolar waves to create a plasma field, which disassociates molecular bonds within soft tissues, providing haemostasis with minimal thermal damage and allowing for early wound healing [14,15]. Coblation can be safely used in microlaryngeal surgeries as an alternate to cold knife or laser. Both patients were on regular follow-up for one year after treatment and were asymptomatic. In a study including 16 patients of aerodigestive amyloidosis, Pribitkin and colleagues described the requirement of multiple surgical interventions for controlling the symptoms [16]. Patients need regular follow up, because sudden relapse has been reported even after long term treatment [17].
CONCLUSION
Laryngeal amyloidosis is a rare isolated benign lesion with variable presentation. High index of suspicion by clinician and pathologist is required as an initial step towards diagnosis. A deep biopsy is always advised for confirmation of diagnosis and to rule out malignancies. Although there are various treatment options offered to patients of laryngeal amyloidosis, staged endoscopic coblation of amyloid mass can provide satisfactory surgical outcome without recurrence of symptoms.
REFERENCES
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11. Behnoud F, Baghbanian N. Isolated laryngeal amyloidosis. Iran J Otorhinolaryngol. 2013; 25: 49-52.
13. Dedo HH, Izdebski K. Laryngeal amyloidosis in 10 patients. Laryngoscope. 2004; 114: 1742-1746.
