Lymphocytic hypophysitis with central diabetes insipidus and consequent panhypopituitarism preceding a multifocal, intracranial germinoma in a prepubertal girl

We report the clinical course of a prepubertal girl with central diabetes insipidus (DI) and consequent panhypopituitarism evolving over a period of 10 years due to lymphocytic hypophysitis and subsequent germinoma. Two years after the diagnosis of central DI was established, MRI revealed a thickene...

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Main Authors: Bettendorf, Markus (Author) , Fehn, Marita (Author) , Grulich-Henn, Jürgen (Author) , Selle, Barbara (Author) , Darge, K. (Author) , Lüdecke, D. K. (Author) , Heinrich, Udo (Author) , Saeger, W. (Author)
Format: Article (Journal)
Language:English
Published: March 1999
In: European journal of pediatrics
Year: 1999, Volume: 158, Issue: 4, Pages: 288-292
ISSN:1432-1076
DOI:10.1007/s004310051074
Online Access:Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1007/s004310051074
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Author Notes:M. Bettendorf, M. Fehn, J. Grulich-Henn, B. Selle, K. Darge, D.K. Lüdecke, U.E. Heinrich, W. Saeger
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Summary:We report the clinical course of a prepubertal girl with central diabetes insipidus (DI) and consequent panhypopituitarism evolving over a period of 10 years due to lymphocytic hypophysitis and subsequent germinoma. Two years after the diagnosis of central DI was established, MRI revealed a thickened pituitary stalk. Later pituitary enlargement and increasing thickening of the pituitary stalk impinging on the optic chiasm required a trans-sphenoidal biopsy which disclosed active hypophysitis with lymphocytic infiltrates and necrosis. High dose dexamethasone treatment only temporarily halted the disease process. Therefore, stereotactic radiation therapy was performed as a rescue treatment and MRI findings almost reversed. However, the subsequent MRI showed multiple intracranial lesions identified histologically as a germinoma and a standard chemotherapy and radiation was performed.
Item Description:Gesehen am 15.12.2020
Physical Description:Online Resource
ISSN:1432-1076
DOI:10.1007/s004310051074