Abstract
Background
Langerhans cell histiocytosis is a rare clonal myeloid disorder with heterogeneous clinical manifestations, predominantly affecting children. Osseous involvement is common; however, isolated localization to the clivus is exceptionally rare and poses significant diagnostic and therapeutic challenges due to its proximity to critical neurovascular structures. We present an illustrative pediatric case alongside a systematic review of the literature to better characterize this uncommon entity.Method
This study presents a rare case of a 3.5-year-old boy diagnosed with isolated clival Langerhans cell histiocytosis confirmed by histopathological and immunohistochemical evaluation. In addition, we systematically reviewed 11 published studies encompassing 11 patients with histologically confirmed isolated clival LCH. The systematic review adhered to PRISMA guidelines, with a comprehensive literature search conducted in PubMed and Scopus using predefined keywords related to Langerhans cell histiocytosis and clival involvement.Results
Eleven published cases met inclusion criteria. Most patients were pediatric, with a slight female predominance. Abducens nerve palsy and diplopia were the most frequent presenting symptoms. Imaging typically demonstrated osteolytic clival lesions that mimicked chordoma and other skull base tumors. Histopathology consistently showed characteristic LCH morphology with CD1a and S100 positivity. Management strategies varied and included biopsy, surgical resection, chemotherapy, and radiotherapy. Outcomes were favorable in most cases, with significant neurological improvement and no reported recurrences during follow-up.Conclusion
Isolated clival LCH is an exceptionally rare presentation that can radiologically mimic aggressive skull base neoplasms. Histopathological confirmation is essential. Early recognition and appropriate multidisciplinary management are associated with favorable outcomes.