Stadiengerechte Chirurgie von Saccus-endolymphaticus-Tumoren (ELST)

J. Schipper; W. Maier; S. Rosahl; A. Berlis; R. Laszig

doi:10.1055/s-2004-814395

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Laryngorhinootologie 2004; 83(8): 493-500
DOI: 10.1055/s-2004-814395

Otologie

Stadiengerechte Chirurgie von Saccus-endolymphaticus-Tumoren (ELST)

Tumour Staged Surgery of Endolymphatic Sac Tumors (ELST)J. Schipper^1, ⁴ , W. Maier^1, ⁴ , S. Rosahl^2, ⁴ , A. Berlis^3, ⁴ , R. Laszig^1, ⁴

¹Universitätsklinik für Hals-, Nasen- und Ohrenheilkunde und Poliklinik, Universitätsklinikum Freiburg (Direktor: Professor Dr. med. Dr. h.c. R. Laszig)
²Neurochirurgische Universitätsklinik, Neurozentrum, Universitätsklinikum Freiburg (Direktor: Professsor Dr. med. J. Zentner)
³Sektion Neuroradiologie, Neurozentrum, Universitätsklinikum Freiburg(Direktor: Professor Dr. med. M. Schumacher)
⁴Interdisziplinäres Zentrum für Schädelbasis- und Craniofaciale Chirurgie, Universitätsklinikum Freiburg

Further Information

Publication History

Eingegangen: 8. Januar 2004

Angenommen: 6. Februar 2004

Publication Date:
18 August 2004 (online)

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Zusammenfassung

Hintergrund: Saccus-endolymphaticus-Tumore (ELST) sind erst seit 1984 als eine eigene Tumorentität bekannt. Der ELST kann sowohl sporadisch solitär als auch heriditär im Rahmen eines Von-Hippel-Lindau (VHL)-Syndroms auftreten. Dieser Zusammenhang wurde erstmals 1992 beobachtet und wurde durch weitere molekulargenetische Analysen des VHL-Gens schließlich bestätigt. Zur Diagnostik und Therapie gibt es bislang keine übereinstimmende Vorstellungen.

Methode: Wir haben aufgrund eigener Erfahrungen und Literaturdaten erstmals den Versuch unternommen, diese Form von Tumoren zu klassifizieren: ELST-Typ A lokal begrenzt ohne Knochenarrosion des Felsenbeins und Infiltration des Subarachnoidalraumes, ELST-Typ B mit knöcherner Infiltration des Labyrinthblocks und klinischem Hörverlust sowie ELST-Typ C mit zusätzlicher Infiltration des Sinus sigmoideus sowie des Bulbus venae jugularis. Die präoperative Diagnostik erfolgt anhand definierter radiologischer Kriterien im CT und MR einschließlich MR-Angiographie.

Ergebnis: Entsprechend der von uns entwickelten Tumorklassifikation wurde bei 6 Patienten, davon zwei Patienten im Rahmen eines VHL-Syndroms, ein ELST stadiengerecht von transmastoidal, translabyrinthär bis infratemporal chirurgisch komplett saniert. In allen Tumorstadien konnte der N.VII und im Stadium ELST-Typ A auch der N.VIII erhalten werden. Bei einer Nachbeobachtungszeit von 4 bis 38 Monaten waren alle Patienten im Kontroll-MR ohne Lokalrezidiv. Schlussfolgerungen: Die von uns erarbeitete stadiengerechte Chirurgie von ELST erlaubt eine komplette Tumorsanierung bei geringster Morbidität. Im Gegensatz zu den antero- und retrosigmoidalen sowie suboccipitalen Zugangswegen erlaubt der transmastoidale Zugangsweg eine sichere Entfernung der Tumormatrix zum Ausschluss eines Lokalrezidivs.

Abstract

Background: Endolymphatic sac tumours (ELST) have only been known as own tumour entities since 1984. ELST might occur solitarily and sporadically as well as hereditary connected to von Hippel-Lindau disease (VHL). This connection has been observed in 1992 for the first time and confirmed by molecular genetic analyses of the VHL gen. There is no agreement yet concerning diagnostics and therapy.

Methods: Our attempt of classifying this type of tumour is the first one. According to our own experience and to literature, we suggest the following classification: ELST type A is locally confined without erosions of the temporal bone nor infiltration of the subarachnoidal area; ELST type B with bony infiltration of the labyrinth block and clinical hearing loss, and ELST type C with additional infiltration of the sigmoid sinus and the vein of jugular bulb. Preoperative diagnostics are performed according to defined radiological criteria in CT and MRI scans including MR-angiography.

Results: In 6 patients, including two with a VHL syndrome, ELST was completely sanitized by stage-compatible surgery, using translabyrintine to infratemporal approaches, according to the tumour classification that we developed. The VII^th nerve could be saved in all tumour stages, and in stage ELST type A the VIII^th nerve as well. All patients remained without local recurrence in MRI check during the observation period of 4 to 38 months.

Conclusion: Our stage-compatible surgery of ELST allows total tumor removal with minor morbidity. In contrast to the antero-, retrosigmoidal and suboccipital approaches, the tumour matrix can be safely removed via transmastoidal approach to exclude local recurrences.

Schlüsselwörter

Von-Hippel-Lindau-Syndrom - Morbidität - Tumorklassifikation - operativer Zugangsweg - Tumormatrix

Key words

Von Hippel-Lindau disease - morbidity - tumour classification - surgical approach - tumour matrix

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Priv.-Doz. Dr. med. Jörg Schipper

Universitätsklinik für Hals-, Nasen- und Ohrenheilkunde und Poliklinik · Universitätsklinikum Freiburg

Killianstraße 5 · 79106 Freiburg

Email: schipper@hno.ukl.uni-freiburg.de