Langerhans cell histiocytosis located in the spheno-orbital bone and the pons: illustrative case

Efecan Cekic; Ahmet Karagoz; Mustafa Sakar; Rabia Emel Senay; Kadriye Ebru Akar; Suheyla Bozkurt; Adnan Dagcinar

doi:10.3171/CASE22367

Langerhans cell histiocytosis located in the spheno-orbital bone and the pons: illustrative case

J Neurosurg Case Lessons. 2023 Jul 31;6(5):CASE22367. doi: 10.3171/CASE22367. Print 2023 Jul 31.

Authors

Efecan Cekic¹, Ahmet Karagoz¹, Mustafa Sakar¹, Rabia Emel Senay², Kadriye Ebru Akar³, Suheyla Bozkurt³, Adnan Dagcinar¹

Affiliations

¹ Departments of1Neurosurgery.
² 2Pediatric Hematology and Oncology Diseases, and.
³ 3Pathology, Faculty of Medicine, Marmara University, Istanbul, Turkey.

Abstract

Background: This is a case of aggressive Langerhans cell histiocytosis (LCH) with an atypical intracranial location.

Observations: In this report, the authors present the diagnosis and treatment of a 12-year-old male patient diagnosed with LCH. The patient was admitted to the emergency department with left-sided facial palsy, and a solid lesion with mass effect in the pons was found. A biopsy was performed via suboccipital craniotomy, and the diagnosis was LCH. A chemotherapy regimen was started since the LCH sample was the resistant type. The patient showed improvement in his neurological deficit following treatment.

Lessons: This rare localized and aggressive case's diagnosis process and treatment choices may apply to future cases.

Keywords: Langerhans cell histiocytosis; brainstem; neurosurgery; pediatric oncology; pons.