Niedrig maligne endometriale Stromasarkome

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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