Pediatric langerhans cell histiocytosis of the temporal bone: A rare case report and review of literature

Abstract

Langerhans cell histiocytosis (LCH) is a rare cancer involving clonal proliferation of Langerhans cells (LCs) resembling epidermal dendritic cells. It can involve any organ or system. Temporal bone LCH is often confused with ear inflammatory lesions and malignant tumors. Diagnosis is based on clinical, radiological, and pathological findings. The definitive diagnosis is made on biopsy and by immunohistochemical demonstration of CD 1a and or Langerin positivity in the clonally neoplastic cells. The course of LCH is variable from spontaneous regression to repeated recurrences and death. The main form of treatment is chemotherapy. We describe a case of multifocal multisystem LCH in a 4-year-old child who presented with recurrent chronic suppurative otitis media and an external auditory canal polyp.