Langerhans-Zell-Histiozytose der Orbita

Toddler with

left orbital swelling. Coronal CT with contrast of the orbits in bone (above left) and soft tissue (above right) windows show bony destruction in the superolateral aspect of the orbit caused by a soft tissue mass. Coronal T1 MRI without (below left) and with contrast (below right) of the orbits better show the soft tissue mass superolateral to the left orbit which enhances avidly.The diagnosis was Langerhans cell histiocytosis of the orbit.

MR imaging

features of orbital Langerhans cell Histiocytosis. Representative 3D reconstructions on CT data: it can show the lesion site very well. a: lesion located the superlateral roof of the right orbit, b: lesion located the superior roof of the right orbit

MR imaging

features of orbital Langerhans cell Histiocytosis. a–d. The different performances of orbital LCH lesions in T2WI:Axial (a) T2-weighted MR images show lesion located in the inferolateral right orbit with a clear border, and presenting hypointensity in a T2-weighted image with a low signal ring of the lesion edge;and coronal (b) T2-weighted MR images show lesion located in the right superolateral orbit with an irregular shape like a triangle and an unclear boundary, presenting hyper-hypointense mixed signals; Axial (c) T2WI show lesion located in the lateral wall of the left orbit presenting slightly hyperintense signals with a clear boundary. Axial (d) T2WI show lesion located in the superior wall of the right orbit presenting multiple cystic hyperintense signals near the edge of the lesion

MR imaging

features of orbital Langerhans cell Histiocytosis. a–d. The different performances in T1WI.Axial (a) T1-weighted MR images show isointensity, and the lesion performs diffuse thickening of the affected bone with ill-defined margins; Axial (b) T1WI show lesion located in the left lateral orbital wall presenting hypointensity and an unclear boundary; Coronal (c) T1-weighted MR images show mass located in the inferolateral left orbit presenting iso-hypointense mixed signals and an unclear boundary. Coronal (d) T1WI show lesion located in the superolateral right orbit performing isointensity with patchy hyperintensity inside

MR imaging

features of orbital Langerhans cell Histiocytosis. a-i. The different performances of orbital LCH lesions in enhanced T1-weighted images. Axial T1-weighted postcontrast images (a) demonstrate homogeneous enhancement; Coronal (b) and sagittal(c) T1-weighted postcontrast images represent the same patient with a lesion located in the superolateral of the left orbit, the lesion shaped like a triangle crumb presenting significant heterogeneous enhancement with some irregular low signal areas near the lesion edge. Coronal (d) and sagittal(e) T1-weighted postcontrast images represent the same patient, the lesion is located in the superolateral right orbit with an irregular triangle shape, an unclear boundary, and slight or moderate heterogeneous enhancement. Axial T1-weighted postcontrast images (f) demonstrate a lesion located in the lateral wall of left orbit and shows slight homogeneous enhancement around the center of the lesion. Axial (g) and Coronal (h) T1-weighted postcontrast images represent the same patient with a lesion showing flocculent enhancement in the central hypointense area, and the area surrounding the lesion showed more significant enhancement. Coronal (i) T1-weighted postcontrast images showing slight-moderate heterogeneous enhancement in the center of the lesion, and surrounded by a circular low signal

Preschooler

with new onset diabetes insipidus. 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.The diagnosis was Langerhans cell histiocytosis of the pituitary and Langerhans cell histiocytosis of the orbit.