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Craniofacial and Intracranial Manifestations of Langerhans Cell Histiocytosis: Report of Findings in 100 Patients

Nicholas D'Ambrosio1,2, Stephanie Soohoo1, Craig Warshall1, Alan Johnson1 and Sasan Karimi2

1 Department of Radiology, Schneider Children's Hospital, Long Island Jewish Medical Center, New Hyde Park, NY 11040.
2 Department of Radiology, Memorial Sloan-Kettering Cancer Center, 1275 York Ave., New York, NY 10065.


Figure 1
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  		Fig. 1A —Two different 4-year-old girls with multiple palpable scalp abnormalities. Lateral skull radiograph shows two well-defined lytic lesions (arrows) with characteristic beveled edges.

 

Figure 2
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  		Fig. 1B —Two different 4-year-old girls with multiple palpable scalp abnormalities. Axial CT of different patient from A shows unequal destruction of outer table (arrowhead) and inner table (arrow), which produces beveled-edge appearance.

 

Figure 3
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  		Fig. 2A —3-year-old girl with palpable scalp soft-tissue mass. Axial MR images of brain show well-circumscribed calvarial lesion (arrows), which is isointense on T1 (A), enhances almost homogeneously after gadolinium administration (B), and is hyperintense on T2 (C). Arrowhead in C denotes dense sclerotic central focus, which corresponds to button sequestrum found on CT scan (not shown). Note generalized brain atrophy, which is associated finding in Langerhans cell histiocytosis.

 

Figure 4
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  		Fig. 2B —3-year-old girl with palpable scalp soft-tissue mass. Axial MR images of brain show well-circumscribed calvarial lesion (arrows), which is isointense on T1 (A), enhances almost homogeneously after gadolinium administration (B), and is hyperintense on T2 (C). Arrowhead in C denotes dense sclerotic central focus, which corresponds to button sequestrum found on CT scan (not shown). Note generalized brain atrophy, which is associated finding in Langerhans cell histiocytosis.

 

Figure 5
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  		Fig. 2C —3-year-old girl with palpable scalp soft-tissue mass. Axial MR images of brain show well-circumscribed calvarial lesion (arrows), which is isointense on T1 (A), enhances almost homogeneously after gadolinium administration (B), and is hyperintense on T2 (C). Arrowhead in C denotes dense sclerotic central focus, which corresponds to button sequestrum found on CT scan (not shown). Note generalized brain atrophy, which is associated finding in Langerhans cell histiocytosis.

 

Figure 6
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  		Fig. 3A —2-year-old boy with recurrent otitis media and aural discharge. Axial CT bone (A) and soft-tissue (B) window images show extensive punched out, lytic lesion with associated soft-tissue mass involving right sphenoid bone, extending into middle cranial fossa (arrows).

 

Figure 7
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  		Fig. 3B —2-year-old boy with recurrent otitis media and aural discharge. Axial CT bone (A) and soft-tissue (B) window images show extensive punched out, lytic lesion with associated soft-tissue mass involving right sphenoid bone, extending into middle cranial fossa (arrows).

 

Figure 8
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  		Fig. 4A —4-year-old girl with right ear pain and discharge. Axial T1-weighted MR image (A) shows isointense lesion in right temporal bone (arrow), which enhances after gadolinium administration (B). At biopsy, this lesion was proven to be Langerhans cell histiocytosis.

 

Figure 9
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  		Fig. 4B —4-year-old girl with right ear pain and discharge. Axial T1-weighted MR image (A) shows isointense lesion in right temporal bone (arrow), which enhances after gadolinium administration (B). At biopsy, this lesion was proven to be Langerhans cell histiocytosis.

 

Figure 10
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  		Fig. 5A —16-year-old boy who presented with left-sided sixth-nerve palsy. Coronal reformatted CT image shows extensive opacification and dense sclerosis involving left mastoids (arrow).

 

Figure 11
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  		Fig. 5B —16-year-old boy who presented with left-sided sixth-nerve palsy. Axial bone algorithm CT image shows involvement of petrous apex (arrow).

 

Figure 12
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  		Fig. 5C —16-year-old boy who presented with left-sided sixth-nerve palsy. After IV contrast administration, there is marked abnormal enhancement of left cavernous sinus (arrow).

 

Figure 13
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  		Fig. 5D —16-year-old boy who presented with left-sided sixth-nerve palsy. Histologic specimen from mastoid biopsy shows Langerhans cells (arrows), which stain positively for S-100 and CD1a. Top image = H and E, x600. Bottom images are immunohistochemical stains with a brown chromogen. (Courtesy of Dr. Morris Edelman, New Hyde Park, NY)

 

Figure 14
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  		Fig. 6A —2-year-old boy who presented with hoarseness and difficulty swallowing. Axial CT bone (A) and soft-tissue (B) window images show large, lytic lesion centered in right occipital bone, involving jugular foramen (arrows).

 

Figure 15
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  		Fig. 6B —2-year-old boy who presented with hoarseness and difficulty swallowing. Axial CT bone (A) and soft-tissue (B) window images show large, lytic lesion centered in right occipital bone, involving jugular foramen (arrows).

 

Figure 16
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  		Fig. 6C —2-year-old boy who presented with hoarseness and difficulty swallowing. Axial T1-weighted MR images without (C) and with (D) gadolinium enhancement of skull base show large isointense jugular foramen lesion (arrows), which enhances avidly after gadolinium administration.

 

Figure 17
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  		Fig. 6D —2-year-old boy who presented with hoarseness and difficulty swallowing. Axial T1-weighted MR images without (C) and with (D) gadolinium enhancement of skull base show large isointense jugular foramen lesion (arrows), which enhances avidly after gadolinium administration.

 

Figure 18
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  		Fig. 6E —2-year-old boy who presented with hoarseness and difficulty swallowing. Coronal T1-weighted MR images without (E) and with (F) gadolinium enhancement of skull base show large isointense jugular foramen lesion (arrows), which enhances avidly after gadolinium administration.

 

Figure 19
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  		Fig. 6F —2-year-old boy who presented with hoarseness and difficulty swallowing. Coronal T1-weighted MR images without (E) and with (F) gadolinium enhancement of skull base show large isointense jugular foramen lesion (arrows), which enhances avidly after gadolinium administration.

 

Figure 20
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  		Fig. 7A —8-year-old boy with rapidly progressive frontal swelling. Lateral skull radiograph windowed to accentuate soft tissues shows prominent soft-tissue swelling in frontal region (arrowhead). Note underlying frontal bone with eroded cortices.

 

Figure 21
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  		Fig. 7B —8-year-old boy with rapidly progressive frontal swelling. Axial CT bone (B) and soft-tissue (C) window images show lytic punched out lesion arising from frontal sinus (arrows), with associated large soft-tissue mass.

 

Figure 22
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  		Fig. 7C —8-year-old boy with rapidly progressive frontal swelling. Axial CT bone (B) and soft-tissue (C) window images show lytic punched out lesion arising from frontal sinus (arrows), with associated large soft-tissue mass.

 

Figure 23
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  		Fig. 7D —8-year-old boy with rapidly progressive frontal swelling. Gadolinium-enhanced axial MRI shows Langerhans cell histiocytosis extension into epidural space (arrow).

 

Figure 24
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  		Fig. 8A —23-year-old man with gingival bleeding. Lateral oblique radiograph of left mandible shows expansile, lytic lesion in superior alveolar ridge (arrows), resulting in "floating teeth."

 

Figure 25
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  		Fig. 8B —23-year-old man with gingival bleeding. Axial CT image shows expansile, lytic lesion, with associated periosteal reaction (arrow).

 

Figure 26
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  		Fig. 9 —3-year-old girl with gingival swelling and bleeding. Coronal reformatted CT image shows soft-tissue mass arising from hard palate (arrow).

 

Figure 27
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  		Fig. 10A —3-year-old boy who presented with periorbital swelling and proptosis. Axial CT soft-tissue (A) and bone (B) images show large mass eroding lateral wall of left orbit and greater wing of sphenoid bone (arrows).

 

Figure 28
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  		Fig. 10B —3-year-old boy who presented with periorbital swelling and proptosis. Axial CT soft-tissue (A) and bone (B) images show large mass eroding lateral wall of left orbit and greater wing of sphenoid bone (arrows).

 

Figure 29
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  		Fig. 10C —3-year-old boy who presented with periorbital swelling and proptosis. Coronal T1-weighted MR images, without and with gadolinium enhancement better delineate borders of this lesion (arrows), located in lateral orbital wall.

 

Figure 30
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  		Fig. 10D —3-year-old boy who presented with periorbital swelling and proptosis. Coronal T1-weighted MR images, without and with gadolinium enhancement better delineate borders of this lesion (arrows), located in lateral orbital wall.

 

Figure 31
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  		Fig. 11A —16-year-old boy with central diabetes insipidus. Sagittal T1-weighted image shows thickened pituitary stalk (arrow), indicating infiltration of infundibulum by Langerhans cell histiocytosis. Note absent normal posterior pituitary bright spot, common finding in Langerhans cell histiocytosis patients with diabetes insipidus.

 

Figure 32
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  		Fig. 11B —16-year-old boy with central diabetes insipidus. Coronal gadolinium-enhanced MR image shows thickening and enhancement of infundibulum (arrow).

 

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