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Case Reports
. 2019 May 2;14(7):847-850.
doi: 10.1016/j.radcr.2019.03.040. eCollection 2019 Jul.

Langerhans' cell histiocytosis of the temporal bone in an adult with central diabetes insipidus

Amanda Allen  1 , Elza Matrova  2 , Burce Ozgen  3 , Miriam Redleaf  4 , Rajyasree Emmadi  2 , Nitu Saran  3
Affiliations
Case Reports

Langerhans' cell histiocytosis of the temporal bone in an adult with central diabetes insipidus

Amanda Allen et al. Radiol Case Rep. .

Abstract

We present a case of Langerhans' cell histiocytosis in a 40-year-old woman presenting with central diabetes insipidus and right ear pain. As this disease process is often clinically challenging, the presence of certain imaging findings should raise the possibility of this diagnosis. We review the pertinent imaging and correlate with histology and immunohistochemistry leading to the diagnosis.

Keywords: Adult; CT temporal bone; Histology; Langerhans cell histiocytosis; MRI.

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Figures

Fig 1
Fig. 1
Sagittal T1-weighted contrast-enhanced image of the pituitary MRI demonstrating marked thickening of the pituitary infundibulum.
Fig 2
Fig. 2
Axial section from high resolution CT of the temporal bone demonstrating opacification of the right mastoid air cells with focal bony resorption at the posterior cortex (arrow).
Fig 3
Fig. 3
Axial T2-weighted MRI of the temporal bone reveals mixed intensity signal changes within the right mastoid air cells. Note that the right petrous apex has also increased T2-weighted signal.
Fig 4
Fig. 4
Coronal T1-weighted contrast-enhanced fat saturated image of the right temporal bone demonstrating heterogeneous enhancement within the right mastoid air cells with accompanying subperiosteal soft tissue thickening and enhancement beneath the mastoid tip (arrow).
Fig 5
Fig. 5
Bony lesion shows infiltration of polygonal cells with eosinophilic cytoplasm and oval nuclei (Langerhans cells); admixed with numerous eosinophils and lymphocytes; ×100 (H&E).
Fig 6
Fig. 6
Cells with nuclei containing linear grooves (arrow), typical cytologic feature of Langerhans cells; ×200 (H&E).
Fig 7
Fig. 7
Multinucleated form (circled); ×200 (H&E).
Fig 8
Fig. 8
Immunohistochemistry showing presence of Langerhans cell CD1a antigen. CD1a; ×200.
Fig 9
Fig. 9
Immunohistochemistry showing presence of Langerhans cell CD68 protein; ×200.
Fig 10
Fig. 10
Immunohistochemistry showing presence of Langerhans cell S100 protein; ×200.
See this image and copyright information in PMC

References

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