Siddachari Ravichand C, Chaukar Devendra A, Pramesh Conjeevaram S, Naresh Kikkeri N, de Souza Chris E, Dcruz Anil K
Department of Surgical Oncology, Division of Head and Neck Oncology, Tata Memorial Hospital, Mumbai, India.
J Otolaryngol. 2005 Feb;34(1):60-3. doi: 10.2310/7070.2005.03131.
Localized amyloidosis is characterized by the deposition of amyloid fibres in a particular site or organ system in the absence of systemic involvement. Patients with localized laryngeal amyloidosis usually present with long-standing hoarseness or dyspnea. The diagnosis is made by a high degree of suspicion on the basis of the history and a characteristic appearance on direct laryngoscopic examination. When such lesions are seen, an adequate deep punch biopsy should be obtained, and an experienced pathologist should be able to identify the lesion on routine staining. However, the slides should be stained with Congo red and examined with polarized light microscopy to establish the diagnosis. Following proper diagnosis and evaluation of the extent of disease, usually by computed tomographic scan, surgery is the treatment of choice. Preservation of the voice and airway should be the aim in all patients. Endoscopic carbon-dioxide laser excision of the mass should be the first line of therapy. Patients may require repeated removal of the amyloid deposits. The results of treatment are excellent.
局限性淀粉样变性的特征是在无全身受累的情况下,淀粉样纤维沉积于特定部位或器官系统。局限性喉淀粉样变性患者通常表现为长期声音嘶哑或呼吸困难。根据病史高度怀疑并结合直接喉镜检查的特征性表现做出诊断。当发现此类病变时,应进行足够深度的组织块活检,经验丰富的病理学家应能通过常规染色识别病变。然而,玻片应进行刚果红染色并通过偏振光显微镜检查以确诊。在通过计算机断层扫描等对疾病范围进行正确诊断和评估后,手术是首选治疗方法。所有患者的治疗目标都应是保留嗓音和气道。内镜下二氧化碳激光切除肿物应作为一线治疗方法。患者可能需要反复清除淀粉样沉积物。治疗效果极佳。