The natural course and optimal treatment for isolated hypothalamic Langerha
ns cell histiocytosis (LCH) are unknown. We describe an adult female in who
m total resection of a hypothalamic LCH granuloma was performed 12 years af
ter transphenoidal resection of a pituitary adenoma. A retrospective review
of the histological specimen of the first operation revealed CD1a positive
cells characteristic of LCH along with a plurihormonal adenoma 12 years ea
rlier. No other manifestations of LCH were found and MRI of the brain at th
e last follow-up 4 years after surgery did not show any recurrent or additi
onal lesion. The diagnosis of isolated hypothalamic LCH is only possible by
biopsy and our case demonstrates the feasability of a gross total resectio
n in certain cases.