Successful treatment of disseminated Rosai-Dorfman disease with siltuximab

Rosai‐Dorfman disease presenting with solitary liver mass without lymphadenopathy: A case report

“…It is worth noting that in contrast to Langerhans cell histiocytosis, these cells are negative for CD1a and CD207 markers 4 . Although extranodal involvement in RDD is common and may occur in more than 40% of patients without lymphadenopathy occurrence, 6 intra‐abdominal involvement is uncommon with an incidence of 4% 9 . Of note, the GI manifestation mostly affects middle‐aged females 10 .…”

“…In general, RDD is defined as a self‐limited disease; however, it can be also permanent or seldom a life‐threatening condition. 6 The diagnosis of RDD mainly relies on histology and immunohistochemistry (IHC) assessments along with diagnostic imaging. IHC staining of the histiocytes are positive for S‐100, CD68, and CD163, while CD1a + cells are detected for Langerhans cell histiocytosis.…”

“…IHC staining of the histiocytes are positive for S‐100, CD68, and CD163, while CD1a + cells are detected for Langerhans cell histiocytosis. 6 In some forms of RDD, the mutation landscape of MAP2K1 , KRAS , NRAS , and ARAF genes in lesional tissues was also reported. 7 Although a large portion of patients experience spontaneous remission after a few months or years without receiving treatment, the rarity and unpredictability of RDD render a challenging treatment modality.…”

“…According to the results, of 64 patients with a mean age of 50 years, the most common presentation was subcutaneous masses (40%) with extranodal disease (92%) in comparison with nodal disease (classical form, 8%). In general, RDD is defined as a self‐limited disease; however, it can be also permanent or seldom a life‐threatening condition 6 . The diagnosis of RDD mainly relies on histology and immunohistochemistry (IHC) assessments along with diagnostic imaging.…”

“…The diagnosis of RDD mainly relies on histology and immunohistochemistry (IHC) assessments along with diagnostic imaging. IHC staining of the histiocytes are positive for S‐100, CD68, and CD163, while CD1a + cells are detected for Langerhans cell histiocytosis 6 . In some forms of RDD, the mutation landscape of MAP2K1 , KRAS , NRAS , and ARAF genes in lesional tissues was also reported 7 .…”