Langerhans cell histiocytosis of the head and neck in the pediatric population in a tertiary center: Clinical presentation, classification and treatment.

INTRODUCTION: Langerhans cell histiocytosis (LCH) is a rare disease of unknown etiology with a heterogeneous and unspecific clinical presentation. Any organ or system may be involved but the most frequent is the skeleton. The diagnostic gold standard is done through histopathology combined with immunohistochemistry in the correct clinical setting. Treatment is still controversial. The objective of this study is to present a series of pediatric patients in order to contribute to the diagnosis and treatment of LCH. MATERIAL AND METHODS: A longitudinal, descriptive and retrospective study was done in 20 pediatric patients histologically diagnosed of LCH int the last 10 years, in a tertiary center. A review of the medical history was done and data collection included: epidemiological data, clinical manifestations and classification, lesion characteristics, treatment employed, follow-up and evolution. RESULTS: The mean age presentation was 6.5 years (2-12). The most frequent disease presentation was a palpable skull tumor followed by headache. Other otorhinolaryngologic presenting symptoms were bloody otorrhea and post auricular swelling. Single-systems LCH with skull tumors was the most frequent classification. Treatment options (surgery, chemotherapy and corticosteroid infiltration) were indicated according to disease classification. CONCLUSION: LCH should be suspected in pediatric patients with a palpable cranial vault tumor, as well as in patients with chronic otorrhea with granulation tissue at the external ear canal. Management of LCH fundamentally includes chemotherapy and surgery, or the combination of both. High-risk organs lesions, including temporal bone lesions, and multi-system LCH are predictors of recurrence and should have a long-term follow-up.

An Iatrogenic Cholesteatoma of the Squamous Part of the Temporal Bone.

A cholesteatoma is a relatively common condition within the middle ear cavity, whereas a cholesteatoma of the squamous part of the temporal bone is an exceptionally rare entity. A case of an iatrogenic cholesteatoma located in the squamous part of the temporal bone is presented, which was revealed by an intermittent purulent discharge from an opening above the right ear 20 years after retroauricular myringoplasty. The diagnosis of an iatrogenic cholesteatoma is often made after several years of evolution, sometimes even at the stage of complications. This set the importance of the otoscopic and radiological regular monitoring of patients who have undergone a surgery of the temporal bone.