Unusual sites of Rosai-Dorfman disease in children: 5 years' experience from tertiary care pediatric hemato-oncology center

Abstract

Rosai-Dorfman disease (RDD) is a rare histiocytic disorder, usually presenting with massive lymphadenopathy. The involvement of extra nodal sites, bone and nodal sites like mediastinum are rare. Clinical data of three cases of extra nodal RDD who were admitted in our Pediatric Hemato-oncology unit were analyzed in the last 5 years. The extra nodal RDD (maxillary sinus, nasal pharyngeal focus, and external auditory meatus) were diagnosed based on histopathology and positron emission tomography (PET) scan. They were treated with steroids, but were refractory hence requiring salvage chemotherapy. Currently all 3 of them are in complete remission. Extra nodal RDD are difficult to diagnose, and histopathology and radiology play an important role. Here we present three cases, of which two were steroid refractory which were treated with salvage chemotherapy and are in complete remission.

Manifestations, Diagnosis and Treatment of Osseous Rosai-Dorfman Disease: A Systematic Review

“…The number of lymph nodes involved varied among the patients. Multiple lymph nodes were found in a total of 4 studies, involving 6 39 , 4 34 , 3 52 and 2 54 nodal sites while 7 studies 34,40,45,46,50,51,56 reported only one lymph node involvement. The types of lymph nodes affected (Fig.…”

“…4). Out of the 53 cases studied, 10 displayed unusual symptoms 24,25,33,34,36,43,44,52,53 . Specifically, the symptoms related to the eyes were observed in 5 studies 24,33,34,43,53 , while ear-related symptoms were observed in 3 studies 24,34,36 and paresthesia was seen only in 1 study 44 .…”