Temporal Bone Histiocytosis in Childhood: A Case Report
Introduction: Langerhans cells histiocytosis (LCH) is a rare disease characterized by the proliferation of monocytics and macrophagics cells. In children, presents incidence of 2 to 5 cases/million/y. The natural history varies from a benign disorder with spontaneous resolution to progressive fatal disease. About 60% of cases manifest with head and neck symptoms and temporal bone is involved in 4 to 25%.
Objective: This study aims to report a case of a pediatric patient with histiocytosis in the temporal bone with good clinical outcome after chemotherapy.
Case Report: A 3-year-old male patient Presented with polipoids lesion in the left ear for 15 days, associated with otorrhea, otalgia, and hearing loss on the left. Otoscopy revealed polyp occupying 90% of the external auditory canal (EAC). CT with erosion in the left temporal bone and soft tissue content involving the ossicles and mastoid. Brain magnetic resonance images shows mass lesion with an epicenter in the left temporal bone, invasion of external and middle ear, and middle cranial fossa. Incisional biopsy showed an inflammatory lymphocytic infiltration and positive immunohistochemical marker CD1. The patient was referred to the use of chemotherapy and the framework is evolving positively.
Conclusion: The pathogenesis of LCH does not allow a uniform therapeutic approach. So, it is important to disclose this disease, especially among professionals in the pediatric area. Through more prospective studies we will improve the clinical management of the patients, allowing earlier treatment and less chance of sequels.