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DOI: 10.1055/s-2005-832313
© Georg Thieme Verlag Stuttgart · New York
Epitheloides Leiomyosarkom der Mundhöhle
im Kindesalter: Fallbericht und Literaturübersicht
Epitheloid Leiomyosarcoma of the Mouth in Childhood
Publication History
Publication Date:
12 May 2005 (online)
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Zusammenfassung
Es wird über ein sechsjähriges Mädchen mit einem Weichteiltumor im anterioren Unterkieferbereich berichtet. Der Tumor erwies sich histologisch als hochmalignes epitheloides Leiomyosarkom (LMS). Die Behandlung erfolgte kombiniert chemotherapeutisch und chirurgisch nach den Richtlinien des CWS 96-Studienprotokolls. Bereits drei Monate postoperativ wurde eine erneute Tumorprogression festgestellt, die trotz weiterer interdisziplinärer Therapiemaßnahmen nach einem Krankheitsverlauf von insgesamt 16 Monaten zum Tod der Patientin führte. Das LMS der Mundhöhle stellt im Kindesalter eine Rarität dar. Die Diagnose wird histologisch und immunhistochemisch gestellt. Beim vorliegenden Fall war sie zunächst durch die fehlende Expression von SM-Aktin erschwert. Die Entscheidung zur neoadjuvanten Chemotherapie brachte keinen therapeutischen Gewinn, eine Tumorremission konnte nicht erzielt werden, so dass ein radikalchirurgisches Vorgehen nicht zu vermeiden war. Die Prognose der oralen Leiomyosarkome ist bei Kindern aufgrund der Seltenheit schwierig einzuschätzen, besonders ungünstig scheint sie bei gering differenzierten Tumoren und Befall des Knochens zu sein. Der vorgestellte Fall bestätigt dies.
Abstract
A six-year-old girl is presented with an increasing mass involving the anterior vestibule and the floor of the mouth. Histologic and immunohistochemical examination revealed a poorly differentiated epitheloid leiomyosarcoma with destruction of the mandible. According to the CWS-96-study the patient underwent preoperative chemotherapy followed by complete resection of the mandibular body with the surrounding soft tissues. Recurrent tumor became evident only three months later on. The further treatment consisted of tumor resection, oral chemotherapy and irradiation. Nevertheless tumor control could not be achieved. The patient died of progressive disease 16 months after diagnosis. Leiomyosarcoma is extremely uncommon in childhood, especially with localisation in the oral cavity. Diagnosis is based on histologic examination and immunohistochemistry. In the presented case the lack of smooth muscle actin expression made diagnosis difficult. The preoperative chemotherapy could not achieve reduction of tumor size, so that extensive surgery became necessary. Estimation of the prognosis of the oral leiomyosarcomas in childhood is difficult. High grade tumours and involvement of bone seem to be associated with bad clinical outcome. The presented case is confirming that.
Schlüsselwörter
Leiomyosarkom - Mundhöhle - Kindheit
Key words
Leiomyosarcoma - oral cavity - childhood
Literatur
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Dr. Dr. Dirk Gülicher
Klinik für Mund-, Kiefer- und Gesichtschirurgie · Otto-von-Guericke-Universität
Leipziger Straße 44
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