Nasale en nasofaryngeale extranodale manifestatie van de ziekte van Rosai-Dorfman: een casusbespreking

Abstract

Rare nasal and nasopharyngeal manifestation of extranodal Rosai-Dorfman disease: a case report Rosai-Dorfman disease (RDD) is a rare histiocytic proliferative neoplastic process, characterized by a massive accumulation of histiocytes in lymph nodes. Extranodal involvement may also occur. However, isolated extranodal RDD (ENRDD) is relatively uncommon. Commonly affected extranodal sites are the skin, the central nervous system and the head and neck region. The most common otolaryngologic locations for extranodal manifestation are the sinonasal compartment and the pharynx. RDD patients with (para)nasal cavity masses present symptoms of progressive nasal obstruction, recurrent epistaxis, facial pain or hyposmia. The disease is characterized by a relapsing and remitting course that is usually self-limiting. Emergent molecular studies have shown an important role of the MAPK/ERK pathway. Histopathologic evaluation is the main diagnostic modality: the characteristic histiocytes are S-100+, CD68+ and CD1a- and show a variable frequency of emperipolesis. There are no guidelines regarding the optimal treatment strategy. RDD is a heterogeneous entity that can occur as an isolated disorder or in association with autoimmune, hereditary or malignant diseases. This article discusses the case of a patient with isolated ENRDD. A 32-year-old man presented with a left nasal mass, nasal septum involvement and a mass at the left nasopharynx. The diagnosis was made after histopathological examination. ENRDD is a rare, benign disease entity with a varied clinical profile. Consequently, RDD can present a diagnostic challenge and may lead to misdiagnosis.