Idiopathic Isolated Nasal Amyloidosis: Report of a Rare Case with Review of Literature

Abstract

Deposition of amyloid in localized form is rare and most often involves head and neck region. Localized amyloidosis carries good prognosis with surgical debridement offering symptomatic benefit. We present an adolescent male presenting with localized sinonasal amyloidosis managed previously with conservative surgical debridement. The patient was symptomatic from early childhood and the previous histopathological reports were non-specific inflammation. A repeat biopsy revealed features of amyloidosis and retrospective review of past slides revealed the same. On explaining the course of the disease and prognosis, the patient opted for regular follow up. A pertinent comprehensive literature review of the condition is discussed.

Clinical Profile of Patients with Head and Neck Amyloidosis: A Single-Institution Retrospective Chart Review

“…2 shows the non-contrast computed tomography (CT) scan findings of the patient with sinonasal amyloidosis. This patient has been previously reported in another study 6 from our institute. The ratio of female to male patients was of 1.3:1.…”

Sinonasal localised amyloidosis: an uncommon location

“…Evidence regarding localised amyloidosis management is sparse, but it usually involves surgical excision, radiotherapy or clinical surveillance. 4…”

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

“…We only identified 21 case reports of localized amyloidosis originating from the nasal cavity and paranasal sinuses that included detailed patient information in 20 Englishlanguage articles published from 1990 to date (7,(14)(15)(16)(17)(18)(19)(20)(21)(22)(23)(24)(25)(26)(27)(28)(29)(30)(31)(32). The clinical characteristics of the reported cases localized sinonasal amyloidosis, including our own, are shown in Table 1.…”

“…Amyloidosis is a rare disease, with ∼1,275-3,200 new cases per year occurring annually in the United States (1,6). The most common subtype, AL amyloidosis has an incidence of 1 case per 100,000 person-years in Western countries (1,6,7).…”

“…Among localized amyloidosis foci in the head and neck region, the larynx is the most frequently involved site (61%), followed by the oropharynx (23%), trachea (9%), orbit (4%), and only 3% of cases are reported to involve the nasopharynx and sinonasal tract (11,12). In 2019, only 19 cases of truly idiopathic primary localized sinonasal amyloidosis were documented (7).…”