Pediatric Langerhans Cell Histiocytosis: An Aggressive Presentation

Abstract

Encountering a young child with an enlarging painless facial swelling often raises concerns in the treating physician about the possibility of a congenital lesion or an unfavorable pediatric tumor. We discuss a case of a female child who presented with multiple craniofacial swellings, which turned out to be Langerhans cell histiocytosis (LCH). She was subsequently diagnosed with multisystem LCH (MS-LCH) with risk-organ involvement, which included the craniofacial bones, skin, hemopoietic system, and liver. We analyze the various presentations and systemic complications of this rare pediatric tumor, LCH, with an aim to address the diagnostic dilemma associated with this great masquerader.

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

“…Regarding the immunohistochemical analysis, out of the 29 studies, 5 11,13,36,52,58 showed a positive result for CD1a, while the rest were negative. Cyclin d1 levels were reported in only 2 studies, with Adam et.…”

“…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 .…”