Langerhans Cell Histiocytosis of Pituitary

  • Etiology: Uncontrolled monoclonal proliferation of Langerhans cells
  • Imaging MRI:
    — Hypothalamic-pituitary region most commonly involved intracranial location
    — Absent neurohypophysis
    — Mass has similar imaging characteristics as germinoma but there may be large amount of surrounding edema
    — Skull base and calvarium should be evaluated for destructive lesions
    — T1WI post contrast: Enhancing thick hypothalamus and infundibulum
  • DDX:
  • Complications: Diabetes insipidus is common presenting symptom due to involvement of hypothalamus
  • Treatment:
  • Clinical:

Radiology Cases of Langerhans Cell Histiocytosis of Pituitary

MRI of Langerhans cell histiocytosis of the pituitary
Sagittal T1 MRI without contrast (above left) of the brain shows absence of the posterior pituitary bright spot. Sagittal (above right) and coronal T1 with contrast (below left) show a thick and enhancing pituitary stalk. Axial T1 with contrast (below right) again shows the thick enhancing pituitary stalk and enhancing lesions in both sphenoid wings and in the left orbit.