Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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May 19, 2016
Langerhans Cell Histiocytosis of the Hypothalamic Pituitary Axis
- Background:
- Langerhans cell histiocytosis (LCH) is a rare granulomatous reactive clonal monocyte-macrophage pathology that can involve multiple organ systems.
- Hypothalamic-pituitary axis involvement is well documented and is the most common intracranial manifestation of LCH.
- Clinical Presentation:
- Classic features of diabetes insipidus, including polydipsia, polyuria, and nocturia, are usual findings.
- Key Diagnostic Features:
- Thickening of the pituitary stalk greater than 3mm with contrast enhancement, lack of posterior pituitary high signal on T1WI
- Differential Diagnosis:
- Intracranial germinomas
- Sarcoidosis
- Leukemia
- Posttraumatic changes
- Tuberculosis
- Metastases
- Lymphocytic or drug induced hypophysitis also considered given the appropriate clinical setting
- Treatment:
- Vasopressin, systemic chemotherapy, targeted external beam radiotherapy
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