Intracranial Tumorous Lesion as the First Manifestation of LangerhansCell Histiocytosis: A Diagnostic and Therapeutic Challenge - Case Report

Abstract

Langerhans cell histiocytosis (LHC) is a rare proliferative disease of unknown etiology characterized by bone marrow-derived histiocyte-like cell proliferation. The disease is confirmed by electron microscopy or immunohistochemical reactivity of histiocytes to the CD1a receptor, langerin (CD207) and/or S100 protein. The clinical spectrum ranges from an acute disseminated disease to solitary or few chronic lesions of bone or other organs. Central nervous system invasion in LCH patients has rarely been reported, especially in the adult population. We report a case of an intracranial tumorous lesion as the first manifestation of LCH in a 23 year old Caucasian man. The patient presented a 2 year history of cognitive decline and a 7 year history of diabetes insipidus, diabetes mellitus, hypothyroidism and obesity. The histopathological findings of a lesion located in the hypothalamic region confirmed the diagnosis of LCH.