Which conditions are included in the differential diagnoses of Langerhans cell histiocytosis (LCH) with hypothalamic and pituitary lesions?

Updated: Sep 16, 2020
  • Author: Cameron K Tebbi, MD; Chief Editor: Vikramjit S Kanwar, MBBS, MBA, MRCP(UK), FAAP  more...
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Answer

In patients with pituitary and hypothalamic lesions, other causes of diabetes insipidus, such as adenomas, craniopharyngioma, sellar chondromas, meningiomas, gangliocytomas, hypothalamic and optic gliomas, and germ-cell tumors, should be considered.

Involvement of hypothalamic-pituitary region and neurodegenerative changes in the cerebellum, basal ganglia, and pons are seen in Langerhans cell histiocytosis. [226] Intracerebral Langerhans cell histiocytosis is rare, but a case report describes involvement of temporal lobe. [227]

Genetic syndromes with chromosomal disorders, such as multiple endocrine neoplasia type 1 (MEN-1), familial acromegaly, McCune-Albright syndrome, and Carney syndrome, can also be associated with pituitary tumors.


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