Isolated clival Langerhans cell histiocytosis in a pediatric patient: systematic review and illustrative case report.
Adam M Abdallah, Atef F Hulliel, Tala Y Dabash et al.
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Case in brief
A 3.5-year-old boy presented with isolated clival Langerhans cell histiocytosis, confirmed through histopathological and immunohistochemical evaluation. The case highlighted the challenges of diagnosis due to the lesion's proximity to critical neurovascular structures. Management involved a multidisciplinary approach, leading to significant neurological improvement without reported recurrences during follow-up.
What made this case unusual
The isolated localization of Langerhans cell histiocytosis to the clivus is exceptionally rare, complicating diagnosis as it can mimic aggressive skull base tumors.
Diagnostic / clinical pearl
Clinicians should consider isolated clival Langerhans cell histiocytosis in pediatric patients presenting with osteolytic lesions at the skull base, particularly when symptoms include cranial nerve palsies.
Why it matters
This case underscores the importance of histopathological confirmation in differentiating isolated clival LCH from other aggressive neoplasms, which can significantly impact management and outcomes.