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DOI: 10.1055/s-2003-42141
Georg Thieme Verlag Stuttgart · New York
Niedrig maligne endometriale Stromasarkome
Low-Grade Endometrial Stromal SarcomasPublication History
Eingang Manuskript: 23. Januar 2003
Eingang revidiertes Manuskript: 22. März 2003
Akzeptiert: 2. April 2003
Publication Date:
16 September 2003 (online)
Zusammenfassung
Niedrig maligne endometriale Stromasarkome (NMESS) sind seltene sexualhormonabhängige Tumoren zumeist prämenopausaler Frauen. Sie kommen im Uterus und im Ovar, sehr selten auch in anderer Lokalisation vor. Die primäre Therapie ist chirurgisch. Standardoperation ist eine abdominale Hysterektomie mit bilateraler Salpingoophorektomie. Adjuvant ist in Abhängigkeit der Expression von Hormonrezeptoren eine Hormontherapie indiziert. Erfahrungen liegen mit Gestagenen und GNRH-Analoga vor. Der Benefit einer postoperativen Bestrahlung auf das Gesamtüberleben der Patientinnen ist bisher nicht bewiesen, ein effektives Chemotherapieregime nicht bekannt. Wichtigster Prognosefaktor ist das Tumorstadium. Bis zu 50 % aller NMESS rezidivieren. Die hohe Rezidivrate wird durch während der Operation zurückgelassene Tumorreste, die von der körpereigenen humoralen Abwehr nicht zerstört werden können, erklärt. Eine der anatomischen Ausbreitung angepasste Radikalität der operativen Behandlung ist deshalb ebenso notwendig wie eine lebenslange Tumornachsorge der Patientinnen.
Abstract
Low-grade endometrial stromal sarcomas (LGESS) are rare steroid-hormone dependent neoplasms, most of which arise in the uterus and ovaries of premenopausal women. If an LGESS is diagnosed preoperatively, hysterectomy with bilateral salpingoophorectomy is considered the standard therapy. For prognosis, tumor stage is the most important factor. Patients with LGESS suffer from a high recurrence rate up to 50 % which is believed to result from residual microscopic tumor islands. Adjuvant hormonal therapy may be indicated if the LGESS expresses hormone receptors. First promising results have been obtained with gestagen and GNRH-analoga therapy. Radiation therapy has no beneficial effect on overall survival, and an effective chemotherapy schema does not exist. Optimal treatment of patients is radical surgery and life-long oncological follow-up.
Schlüsselwörter
Uterus - Endometrium - Sarkom - Östrogenrezeptor - Progesteronrezeptor - Hormontherapie
Key words
Uterus - endometrium - sarcoma - oestrogen receptor - progesterone receptor - hormonal therapy
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Ao. Univ.-Prof. Dr. Olaf Reich
Klinische Abteilung für Gynäkologie und Geburtshilfe der Geburtshilflich-gynäkologischen Universitätsklinik Graz
Auenbruggerplatz 14
8036 Graz
Österreich
Email: olaf.reich@kfunigraz.ac.at