Unusual Site of Langerhans Cell Histiocytosis Involving Nasopharynx

Abstract

Langerhans cell histiocytosis (LCH) is a rare proliferative disorder that originates from myeloid-derived precursor dendritic cell, not the Langerhans cell in the skin. The etiology and subsequent development of LCH are idiopathic and not well understood. A 28-day full term baby girl referred her to high tertiary care center due to mass in nasopharynx for further management. Patient was intubated with normal size tube in low sitting mode, no facial dysmorphic feature, fiberoptic nasal scope showed patent nasal cavity bilaterally, mass was encountered at the lower end of nasopharynx with normal covering mucosa. MRI brain and sinuses with contrast demonstrates a single relatively well-defined mass, its epicenter seen at the nasopharynx posterior wall. Its showing extension to the adjacent osseous structures including C1 anterior arch, inferior clivus and bilateral occipital condyle. Transoral biopsy done in the operating room under general anesthesia which confirm the diagnosis of LCH. Case was discussed in the pediatric tumor board, and it was decided to start her on Vinblastine and prednisolone. Patient received Vinblastine 6 mg/m2 IV weekly bolus for 6 weeks, with systemic prednisone 40 mg/m2/day in three divided doses for 4 weeks and then tapered over the following 2 weeks. A follow-up FDG PET was performed for the whole body and MRI of head and neck Interval improvement of the nasopharyngeal and clival mass without significant residual, no enlarged or suspicious cervical lymph nodes. LCH is a rare condition and prevalent among early age group. Occur in any part of body including nasopharynx and oropharynx.