Nasopharyngeal and laryngeal amyloidosis: a case report

Abstract

Amyloidosis is a clinical disorder caused by extracellular proteinaceous deposits of insoluble abnormal group of fibrils with characteristic microscopic, histochemical, and ultrastructural features. Amyloid is an extracellular amorphous eosinophilic protein, which displays apple-green birefringence with polarized light when stained with Congo red or shows metachromasia with crystal violet or methyl violet dyes. [1][2][3][4] Systemic and localized forms are the main clinical categories of the amyloidosis. Systemic amyloidosis can manifest as immunoglobulin light chain derivative (AL) amyloidosis, which is associated with primary systemic amyloidosis, multiple myeloma, or plasma cell dyscrasias. Amyloid associated (AA) amyloid occurs in patients with chronic inflammatory conditions or neoplastic disease. Familial amyloidosis is a rare manifestation of systemic amyloidosis which accounts for less than 2% of the cases.[5] In humans, more than 20 different, unrelated proteins are known to form amyloid fibrils. ÖzetK›rk dokuz yafl›ndaki kad›n hasta 4 y›ldan beri ilerleyen k›s›k ses, bo¤az›n›n arka taraf›nda yabanc› cisim hissi, kulak a¤r›s›, ses k›s›kl›¤› ve a¤›zdan soluma yak›nmalar›yla klini¤imize kabul edildi. Nazolarengoskopi orofarenkse do¤ru uzanan bir sa¤ nazofarengeal kitleyi gös-termekteydi. ‹ndirekt larengoskopi sa¤ ventrikül band›ndan kaynaklanan ve sa¤ vokal kordun üzerine do¤ru uzanan bir granülomatöz kitleyi ortaya ç›kard›. Granülomatöz kitle normal larenks mobilitesini bozmuyordu. Yutma zorlu¤u öyküsü yoktu. Kontrastl› bilgisayarl› tomografi nazofarenks ve glotisi tutan düzgün yüzeyli bir kitlenin var oldu¤unu gösterdi. Lezyonun eksizyonel biyopsisi için direkt larengoskopi planland›. Histopatolojik inceleme nazofarenks ve larenks amiloidozunu ortaya ç›kard›. ‹leri de¤erlendirmelerde sistemik amiloidoz saptanmad›. Bu olgu sunumunun amac› son derecede seyrek görülen izole primer nazofarenks ve larenks amiloidozu olgusunu sunmak ve güncel literatür bilgilerini kullanarak tart›flmakt›r.Anahtar sözcükler: Primer amiloidoz, nazofarenks, larenks. AbstractA 49-year-old woman admitted to our clinic due to a progressive hoarse voice for 4 years, foreign body sensation in the back of her throat, otalgia, hoarseness and mouth breathing. Nasolaryngoscopy demonstrated a right nasopharyngeal mass extending to oropharynx. Indirect laryngoscopy revealed a granulomatous lesion originating from right band ventricle and extending on the right vocal fold. The granulomatous lesion did not alter correct laryngeal mobility. There was no history of difficulty in swallowing. A contrast enhanced computed tomography showed a smooth mass involving nasopharynx and glottis. A direct laryngoscopy was planned for excisional biopsy of the lesion. Histopathological examination revealed nasopharyngeal and laryngeal amyloidosis. Further evaluations were negative for systemic amyloidosis. The aim of the report is to present an extremely rare case of isolated primary nasopharyngeal and laryngeal amyloidosis and discuss by using current lite...

Multifocal head and neck amyloidosis as a diagnostic clue of systemic lupus erythematosus (SLE)

“…Although the incidence is rare, multifocal amyloidosis should be taken as a hint of underlying occult disease predisposing to multiple amyloid protein deposition. [14–16]…”