Intracranial Langerhans cell Histiocytosis: A review

Abstract

Introduction: Intracranial Langerhans Cell Histiocytosis (LCH) is a rare disease caused by the proliferation and dissemination of antigen presenting cells in the skin. Few cases have been reported on to date and there is a lack of consensus on the most effective means of diagnosis and treatment of these lesions. This paper will serve as a review of this malady. Methods: MeSH database search was performed to include all relevant studies on intracranial LCH. Results: A total of 146 studies with 192 patients were included in our review. Men were more commonly affected and the average age of diagnosis was 31.6 and 28.0 in men and women respectively. CT and MRI were the most common imaging modalities. The majority of the case reports performed biopsies and the most common lesion location was the hypothalamus-pituitary axis. All studies used surgical resection, radiotherapy, chemotherapy or combination therapy as means of treatment, with resection plus chemotherapy being the most successful. 74 cases were successful in preventing recurrence. Conclusion: LCH rarely affects the brain as primary or secondary focus. Biopsy is required for precise diagnosis and exclusion of other intracranial lesions. There is no standard treatment for LCH of the central nervous system, but this review may serve as a guide to chronicle previous efficacious therapeutics strategies.

Keywords

• Intracranial
• Diabetes Insipidus
• Langerhans Cell Histiocytosis
• Human
• Rare Diseases