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

• Verfasst von: Bettendorf, Markus [VerfasserIn]
• Verfasst von: Fehn, Marita [VerfasserIn]
• Verfasst von: Grulich-Henn, Jürgen [VerfasserIn]
• Verfasst von: Selle, Barbara [VerfasserIn]
• Verfasst von: Darge, K. [VerfasserIn]
• Verfasst von: Lüdecke, D. K. [VerfasserIn]
• Verfasst von: Heinrich, Udo [VerfasserIn]
• Verfasst von: Saeger, W. [VerfasserIn]
• Titel: Lymphocytic hypophysitis with central diabetes insipidus and consequent panhypopituitarism preceding a multifocal, intracranial germinoma in a prepubertal girl
• Verf.angabe: M. Bettendorf, M. Fehn, J. Grulich-Henn, B. Selle, K. Darge, D.K. Lüdecke, U.E. Heinrich, W. Saeger
• E-Jahr: 1999
• Jahr: March 1999
• Umfang: 5 S.
• Fussnoten: Gesehen am 15.12.2020
• Titel Quelle: Enthalten in: European journal of pediatrics
• Ort Quelle: Berlin : Springer Science & Business Media B.V., 1975
• Jahr Quelle: 1999
• Band/Heft Quelle: 158(1999), 4, Seite 288-292
• ISSN Quelle: 1432-1076
• DOI: doi:10.1007/s004310051074
• Datenträger: Online-Ressource
• Sprache: eng
• K10plus-PPN: 1742823556

Abstract

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.