Localised Langerhans cell histiocytosis of the hypothalamic-pituitary region: case report and literature review
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Langerhans cell histiocytosis (LCH) localised in the hypothalamic-pituitary region (HPR) is very rare, especially in adults. Diabetes insipidus (DI) is considered to be a hallmark of HPR LCH, while anterior pituitary abnormalities are usually seen as consequences of surgery, radiotherapy or chemotherapy.
We present a patient with localised HPR LCH with dominant anterior pituitary dysfunction and tumour mass effects but without DI. Seven years after surgery and local radiotherapy, she is stable. Control MRI shows no residual tumour growth and thorough physical examination is still without any signs of disease spread.
Anterior pituitary deficiency can appear without DI and not only as a consequence of LCH treatment. All patients with LCH should be screened for this endocrine abnormality so that appropriate substitution therapy may be provided.
KeywordsLangerhans cell histiocytosis Hypothalamic-pituitary region Anterior pituitary deficiency
Compliance with ethical standards
Procedures, data collection and presentation were approved by the hospital ethics committee (decision no.: 37232/1). Written informed consent was obtained from the patient to participate and for publication of this case report and any accompanying data.
Conflict of interest
The authors declare that they have no conflict of interest.
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