Uterine sarcomas are uncommon aggressive mesenchymal tumours, which comprise only about 3% of all uterine malignancies [D'Angelo and Prat, 2010]. The incidence of uterine sarcomas varies between 0.5 and 3.3 cases per 100,000 women per year [Harlow et al. 1986]. Uterine sarcomas include different histological entities. The most frequent type is leiomyosarcoma (LMS) in about 60% of cases, followed by endometrial stromal tumours (ESS), undifferentiated uterine sarcomas (UUS), and pure heterologous sarcomas. Mixed epithelial and mesenchymal tumours are adenosarcoma (with and without sarcomatous component) and carcinosarcoma (mixed mullerian tumours). Carcinosarcoma are of epithelial origin, as shown by in vitro data, immunohistochemical and molecular studies [Amant et al. 2005]. Therefore, uterine carcinosarcoma are counted as undifferentiated epithelial uterine carcinoma and should not be classified into the sarcoma group.

The cornerstone of the treatment in LMS is surgery. The resection of the localized disease by hysterectomy is regarded as gold standard. Total abdominal hysterectomy and bilateral salpingooophorectomy is considered to be the standard surgical treatment [Vrzic-Petronijevic et al. 2006; Ramondetta L et al. 2006; Gadducci A et al. 2008, Zivanovic et al. 2009]. Pelvic and para-aortic lymphadenectomy is not routinely indicated. The incidence of lymphatic spread is only about 3% in early stage uterine LMS [Gadducci et al. 2008; Vrzic-Petronijevic et al. 2006; Giuntoli et al. 2003; Leitao et al. 2003]. However, lymph-node involvement is often present in advanced disease. Ovarian preservation can be considered in premenopausal patients with early stage LMS of the uterus [Gadducci A et al. 1996a]. Many LMS are diagnosed after surgical intervention of presumed leiomyoma or hysterectomy. Morcellation of the tumour or uterus in total, for example, during laparoscopic assisted supracervical hysterectomy increases the rate of the abdominopelvic dissemination causing an iatrogenic advanced stage disease. This translates to a worse progression-free survival (PFS) and overall survival (OS). Thus, before performing surgery with morcellation, women have to be informed in detail about the possibility of tumour dissemination and prognosis deterioration via iatrogenic advanced stage disease [Park et al. 2011].