gms | German Medical Science

61. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC) im Rahmen der Neurowoche 2010
Joint Meeting mit der Brasilianischen Gesellschaft für Neurochirurgie am 20. September 2010

Deutsche Gesellschaft für Neurochirurgie (DGNC) e. V.

21. - 25.09.2010, Mannheim

Atypical sellar teratoid rhabdoid tumors in adults – clinical, radiological and histopathological features in a series of 6 patients with a very rare cerebral pathology

Meeting Abstract

  • Kerim Beseoglu - Neurochirurgische Klinik, Heinrich-Heine-Universität Düsseldorf, Germany
  • Sevgi Sariakya-Seiwert - Neurochirurgische Klinik, Heinrich-Heine-Universität Düsseldorf, Germany
  • Stefan Macht - Abteilung Neuroradiologie, Institut für Radiologie, Heinrich-Heine-Universität Düsseldorf, Germany
  • Markus Riemenschneider - Institut für Neuropathologie, Heinrich-Heine-Universität Düsseldorf, Germany
  • Hans-Jakob Steiger - Neurochirurgische Klinik, Heinrich-Heine-Universität Düsseldorf, Germany
  • Daniel Hänggi - Neurochirurgische Klinik, Heinrich-Heine-Universität Düsseldorf, Germany

Deutsche Gesellschaft für Neurochirurgie. 61. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC) im Rahmen der Neurowoche 2010. Mannheim, 21.-25.09.2010. Düsseldorf: German Medical Science GMS Publishing House; 2010. DocP1758

DOI: 10.3205/10dgnc229, URN: urn:nbn:de:0183-10dgnc2292

Veröffentlicht: 16. September 2010

© 2010 Beseoglu et al.
Dieser Artikel ist ein Open Access-Artikel und steht unter den Creative Commons Lizenzbedingungen (http://creativecommons.org/licenses/by-nc-nd/3.0/deed.de). Er darf vervielfältigt, verbreitet und öffentlich zugänglich gemacht werden, vorausgesetzt dass Autor und Quelle genannt werden.


Gliederung

Text

Objective: Atypical teratoid rhabdoid tumors (AT/RT) are very rare tumors of early childhood usually arising in the posterior fossa between the age of 2 to 5 years. Here we describe two cases of adult sellar AT/RT treated in our department. In additional, the literature searched to analyze clinical, radiological and histopathological features.

Methods: We evaluated the clinical presentation and course, the radiological imaging, and the histopathological features of our two patients. Furthermore a search in the literature was conducted to identify a total of additional four cases with a sellar manifestation of an AT/RT.

Results: All six patients with sellar manifestation of AT/RT were females in their 3rd to 6th decade of life. Clinical presentation was typical for a large sellar tumor with beginning visual impairment and slight headache. The first radiological diagnosis was usually pituitary adenoma, MRI scan showing a heterogeneously enhancing intrasellar mass with suprasellar extension, invasion into the cavernous sinus was evident, the optic chiasm appeared elevated. Histological examination revealed rhabdoid cells with a primitive neuroectodermal component. Immunohistochemical examination showed typical inactivation of INI1 gene. After surgery and histological examination, all six patients were treated with radiation therapy. All patients died within 2 to 28 month after diagnosis usually after having developed either local recurrence or cerebral or spinal metastasis.

Conclusions: Sellar manifestation of AT/RT in adults occurs extremely rarely. If histological examination is inconclusive in an expected pituitary adenoma, clinicians should be aware of this entity. However, despite surgery and radiation therapy, the documented outcome is poor.