Rosai-Dorfman disease presenting as laryngeal masses

Year 2008, Volume: 18 Issue: 2, 110 - 114, 18.04.2008

Serdar Ensari Adin Selçuk Hüseyin Dere Noelia Perez Serpil Dizbay Sak

Abstract

Extranodal lesions may be the sole manifestation of Rosai-Dorfman disease RDD . Although the head and neck region is one of the most common extranodal sites, laryngeal involvement is very rare. A 44-yearold woman presented with a complaint of progressive dyspnea. She had a three-year history of treatment for asthma and a history of operation for a nasal mass that afflicted her for 15 years and was diagnosed as rhinoscleroma. On physical examination, she had three subcutaneous lesions, in the left lower eyelid, right epicanthal area, and left forearm, respectively. No lymphadenopathy was present. Laryngoscopic examination revealed three solid, polypoid masses in the subglottic region, 1 cm in diameter. With a two-staged operation, the laryngeal masses were excised totally together with the subcutaneous lesions. Histological examination of all the specimens showed proliferation of histiocytes. Immunohistochemical staining revealed typical S-100 protein-positive histiocytes and emperipolesis. Both laryngeal and subcutaneous lesions were diagnosed as RDD. A re-evaluation of sections from the previous operation specimen of the nasal mass showed the same morphological features. The patient was healthy without recurrence, nine months following surgery.

Keywords

Diagnosis, differential, histiocytosis, sinus/ pathology, laryngeal diseases/pathology

Larenks kitleleri olarak kendini gösteren Rosai-Dorfman hastalığı

Abstract

Ekstranodal lezyonlar Rosai-Dorfman hastalığının RDH tek bulgusu olabilir. Baş-boyun bölgesi RDH’de en sık tutulan bölgelerden biri olmasına karşın, hasta- lığın larengeal tutulumu çok nadirdir. Kırk dört yaşında bir kadın hasta ilerleyici nefes darlığı yakınmasıyla başvurdu. Öyküsünden, astım için üç yıldır tedavi gör- düğü ve kendisini 15 yıldır etkileyen bir burun kitlesi nedeniyle ameliyat geçirdiği, bu lezyona da rinosklero- ma tanısı konduğu öğrenildi. Fizik muayenede, sol alt gözkapağında, sağ epikantal bölgede ve sol önkolda üç adet subkutan lezyon görüldü. Lenfadenopatiye rastlanmadı. Larengoskopik incelemede, subglottik bölgede, 1 cm çapında üç adet solid polipoid kitle gözlendi. Larenksteki kitleler ve subkutan lezyonlar iki aşamalı bir ameliyatla çıkarıldı. Histolojik incelemede örneklerin hepsinde histiosit proliferasyonu görüldü. İmmünhistokimyasal boyamada tipik S-100 proteini için pozitiflik gösteren histiositler ve emperipolez göz- lendi. Larenks kitleleri ve subkutan lezyonların tanısı RDH şeklinde kondu. Hastanın daha önce geçirmiş olduğu ameliyatta çıkarılan burun kitlesine ait kesit- lerin yeniden incelenmesi aynı morfolojik özellikleri gösterdi. Ameliyattan dokuz ay sonra hasta sağlıklıydı ve nüks yoktu

Keywords

Tanı, ayırıcı, histiositozis, sinüs/patoloji, larenks hastalıkları

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