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DOI: 10.1055/s-2004-814391
Extranasopharyngeale Angiofibrome der Nasenhaupt- und -nebenhöhlen
Extranasopharyngeal Angiofibroma of the Nasal Cavity and Paranasal SinusesPublication History
Eingegangen: 21. November 2003
Angenommen: 10. Februar 2004
Publication Date:
14 May 2004 (online)
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Zusammenfassung
Hintergrund: Angiofibrome entwickeln sich typischerweise im Nasenrachenraum bei jungen männlichen Patienten in der Adoleszenz. Wegen des Gefäßreichtums sollten Biopsien nicht vorgenommen werden. Die korrekte Diagnose lässt sich mit hoher Sicherheit durch radiologische Verfahren stellen und stellt die Grundlage für die interdisziplinäre Therapieplanung. Ziel dieser Studie war es, die Inzidenz, klinischen Zeichen und Charakteristika sowie Komplikationen bei der Diagnostik und Therapie von extranasopharyngealen Angiofibromen der Nasenhaupt- und -nebenhöhlen festzustellen, bei denen der Nasopharynx explizit nicht betroffen war.
Patienten und Methode: Ausgehend von einem eigenen Fallbericht wurde eine umfassende Literaturrecherche unternommen.
Ergebnisse: Insgesamt konnten die Daten von 42 Patienten mit einem extranasopharyngealen Angiofibrom ausgewertet werden, 32 waren männlichen, 10 weiblichen Geschlechts. Die meisten Angiofibrome entwickelten sich vor dem 20. Lebensjahr (71,4 %; Altersdurchschnitt: 21,5 Jahre), der älteste Patient war 78 Jahre alt. Am häufigsten war die Kieferhöhle betroffen, seltener das Siebbeinzellsystem und die Nasenhaupthöhle, noch seltener isoliert die Keilbeinhöhle, in der Stirnhöhle fanden sich bisher keine Angiofibrome. Zwischen Beginn der Symptomatik (meist Epistaxis, nasale Obstruktion, Mittelgesichtsschwellung) und Diagnostik vergingen durchschnittlich 8,5 Monate. Blutungen traten nach 11 der insgesamt 20 Biopsien sowie während 10 Resektionen auf. Eine Hypervaskularisation konnte in drei von vier Angiographien nachgewiesen werden. Tödliche Verläufe ereigneten sich nicht.
Schlussfolgerungen: Das extranasopharyngeale Angiofibrom ist eine extrem seltene Differenzialdiagnose von Tumoren der Nasenhaupt- und -nebenhöhlen. Bei Hinweisen auf Gefäßreichtum in der Computer Tomographie sollte eine Angiographie indiziert werden, um im Falle einer Hypervaskularisation den Tumor superselektiv embolisieren zu können. Biopsien sollten prinzipiell erst nach abgeschlossener radiologischer Diagnostik und ausschließlich unter Vollnarkose erfolgen. Die Therapie der Wahl ist die vollständige Resektion dieser seltenen Entität.
Abstract
Background: Angiofibromas commonly arise in the nasopharynx in young male patients. Diagnosis is widely based on radiographic imaging to avoid ill-advised biopsy which may result in brisk bleeding. This study was undertaken to evaluate the incidence, clinical features and complications that may occur during the process of diagnosis and surgical therapy of angiofibromas outside the nasopharynx.
Methods and Patients: Case report of a 13-year-old female patient and review of the literature.
Results: Our patient received multi-agent chemotherapy elsewhere due to a misdiagnosed angiofibroma. Computed Tomography (CT) revealed a maxillary tumor which was repeatedly biopsied. Hypervascularity was excluded by arteriography and the lesion removed after lateral rhinotomy. The data of 42 patients were analyzed including our own case. 32 patients were male, 10 female. The majority became symptomatic aged 19 years or younger (71.4 %). The maxilla was most commonly affected (38 %), less frequently the ethmoid, nasal cavity or septum, beside others. In 38 patients, symptoms developed within 12 months or less (average: 8.5 months). Epistaxis, nasal obstruction and facial swelling were reported for most patients. Brisk bleeding occurred in 10 patients during tumor removal and resulted from biopsies in 11 of 20 patients. Angiography detected hypervascularity in 3 of 4 patients. There was no case with lethal outcome.
Conclusion: Extranasopharyngeal angiofibromas of the nasal cavity or paranasal sinuses should be included in the differential diagnosis of nasal tumors. Compared to nasopharyngeal angiofibromas, more female patients are involved, symptoms develop more quickly but hypervascularity is less common. Signs of questionable hypervascularity in Computed Tomography and Magnetic Resonance Imaging (MRI) should indicate arteriography prior to surgical procedures. Preoperative embolization of hypervascular lesions during arteriography will reduce the risk of brisk bleeding during biopsy or surgical tumor removal.
Schlüsselwörter
Extranasopharyngeales Angiofibrom - Kieferhöhlentumor - Angiographie - Embolisation - Caldwell-Luc - Karotisligatur
Key words
Extranasopharyngeal angiofibroma - maxillary tumor - arteriography - embolization - carotid ligation
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