Langerhans' cell histiocytosis of the temporal bone in an ad
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This report presents 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.

It reviews the pertinent imaging and correlate with histology and immunohistochemistry leading to the diagnosis.

A 40-year-old woman was admitted to our institution for increased thirst, polydipsia, and polyuria with associated dry mouth and dry eyes. Clinical symptoms were concerning for Sjogren's syndrome; although, antinuclear antibodies, anti-Ro/SSA, and anti-La/SSB antibodies were negative. Subsequent outpatient laboratory tests had found an elevated serum sodium level of 158 mEq/L (normal 135-145) with low urine osmolality. Further work-up for diabetes insipidus demonstrated a positive response after Vasopressin challenge. These findings conformed central diabetes insipidus.

A pituitary magnetic resonance image demonstrated a markedly thickened hypothalamic infundibulum. The infundibulum was deemed too difficult to biopsy and the patient was managed with desmopressin. The patient was readmitted 4 months later with right ear pain, A right mastoidectomy was performed which revealed avascular tissue filling the air cells and eroding the mastoid tip to the sigmoid sinus. An excisional biopsy of the right mastoid was obtained. The histologic findings confirmed the diagnosis of Langerhans’ cell histiocytosis.

Conclusively, Langerhans’ cell histiocytosis is a rare disease that is difficult to diagnose due to the variable clinical and radiologic findings. We hope to bring awareness of this disease process in the central nervous system in patients presenting with central diabetes insipidus who are found to have a thickened pituitary infundibulum. Consider this disease entity in the differential diagnosis of resorptive lesions in the mastoid process and temporal bone which spares the osseous labyrinth.

Source: https://www.sciencedirect.com/science/article/pii/S1930043319300822
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