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The article presents a clinical case with the detection of Langerhans cell histiocytosis of the temporal bone with a complex anatomical localization in a 3-year-old child. The computed tomography allowed to detect the mass lesion of the base of the pyramid in the right temporal bone, which made it possible to suggest indirectly the presence of a number of diseases in this patient, including histiocytosis of the temporal bone. In view of the absence of foci in other organs and systems, it was difficult to suspect the presence of histiocytosis. The surgical treatment of the detected temporal bone disease with extension to the posterior and middle cranial fossa was carried out, using neuromonitoring and microsurgical techniques. During the operation, it was possible to isolate and completely remove the lesion, intimately adherent to the dura mater of the middle cranial fossa and extending from the dura mater of the middle cranial fossa to the tegmen antri, attic, tegmen tympani and also to the region of prelabyrinthine and suprameatal cells, and wrapping ambos and stirrup. Destroying the lateral and partially posterior semicircular canals the tumor extended on the cochlear vestibule from the side of the lateral semicircular canal. The diagnosis of Langerhans cell histiocytosis was confirmed by histopathological and immunohistochemical studies. Thus, the interdisciplinary tactics of diagnostics and treatment applied in this clinical case allows to ensure good results from the operation. After surgical intervention with the removal of pathological tissue, the patient was sent to further treatment under the supervision of a hematologist-oncologist for the course of chemotherapy.

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