MR of intracranial Langerhans cell histiocytosis

J C George1, M K Edwards, R R Smith

  • 1Department of Radiology, Indiana University School of Medicine, Indianapolis.

Insights

Langerhans cell histiocytosis (LCH) is a rare childhood brain disease. This case highlights LCH affecting dural venous sinuses and choroid plexus, detected via MRI.

Area of Science:

  • Neurology
  • Pediatric Oncology
  • Radiology

Background:

  • Langerhans cell histiocytosis (LCH) is a rare idiopathic histiocyte proliferation disorder primarily affecting children.
  • Intracranial involvement of LCH, particularly within the brain parenchyma, is uncommon.

Observation:

  • This report details a pediatric case of LCH with unusual intracranial presentation.
  • The LCH involved the dural venous sinuses and the choroid plexus, structures critical for cerebrospinal fluid dynamics and venous drainage.
  • Contrast-enhanced Magnetic Resonance Imaging (MRI) was utilized for detailed anatomical assessment.

Findings:

  • Contrast MRI effectively delineated the extent of LCH tumor involvement within the intracranial structures.
  • The imaging findings indicated a significant risk of venous sinus thrombosis due to tumor encroachment.

Implications:

  • This case underscores the importance of considering LCH in the differential diagnosis of pediatric intracranial masses, even with atypical locations.
  • Advanced imaging techniques like contrast MRI are crucial for accurate diagnosis, surgical planning, and risk stratification in such cases.
  • Early identification of venous sinus involvement is vital for preventing potentially life-threatening complications like thrombosis.

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