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DOI: 10.1055/s-2006-942118
© Georg Thieme Verlag Stuttgart · New York
Alveolar Rhabdomyosarcoma of the Clivus with Intrasellar Expansion: Case Report
Alveolares Rhabdomyosarkom der Sellaregion: Ein FallberichtPublication History
Publication Date:
01 December 2006 (online)
![](https://www.thieme-connect.de/media/zblneurochir/200604/lookinside/thumbnails/10.1055-s-2006-942118-1.jpg)
Abstract
Rhabdomyosarcomas are common tumors of the head and neck region in children. However, a primarily intracranial localization of this tumor entity is rare. We report on a 3-year-old boy presenting with double vision due to left VIth nerve palsy. No other neurological deficits were recognized by clinical inspection. MRI scans visualized an enhancing mass lesion in the upper clivus compressing the cavernous sinus and the pituitary gland. Transsphenoidal biopsy was performed and histopathological examination as well as molecular diagnostics confirmed the diagnosis of an alveolar rhabdomyosarcoma (ARMS). Staging identified a metastatic lesion in the fourth thoracic vertebra resulting in the diagnosis of stage IV disease. Treatment modality included stereotactic radio- and chemotherapy.
Zusammenfassung
Rhabdomyosarkome sind häufige Tumoren in der Kopf-Hals-Region bei Kindern. Im Gegensatz dazu ist die primäre intrakranielle Lokalisation dieser Tumorentität selten. Wir berichten über einen dreijährigen Jungen mit Doppelbildern aufgrund einer Abduzensparese auf der linken Seite. Klinisch fanden sich keine weiteren Defizite. Im MRT zeigte sich ein kontrastmittelaufnehmender raumfordernder Prozess im Bereich des Klivus mit Kompression des Chiasma opticum und der Hypophyse. Nach transsphenoidaler Biopsie ergab sowohl die histopathologische als auch die molekularbiologische Aufarbeitung des Gewebes die Diagnose eines alveolären Rhabdomyosarkomes (ARMS). Im Staging fand sich ein metastasensuspekter Befund in Höhe BWK 4, entsprechend einem Grad IV nach internationalem Staging. Die weitere Behandlung beinhaltete eine stereotaktische Radio- und Chemotherapie.
Key words
rhabdomyosarcoma - sella turcica - clivus
Schlüsselwörter
Rhabdomyosarkom - Sella turcica - Klivus
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Dr. med. J. Kreutzer
Neurochirurgische Klinik · Universität Erlangen-Nürnberg
Schwabachanlage 6
91054 Erlangen
Germany
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Email: juergen.kreutzer@nch.imed.uni-erlangen.de