Uterinesarcomas include a number of rare tumours with different tumour biology, naturalhistory and response to treatment, comprise just 3-7% of total uterine malignanciesand about 1% of all gynaecologic cancers.
Although they cause important portionof women death due gynaecologic cancers. These tumours have aggressive behaviourand high recurrence rate, even when limited to the uterine corpus at the timeof diagnosis. The most common of uterine sarcomas is leiomyosarcoma. Theincidence of leiomyosarcoma is increased after age 50. Traditionally,carcinosarcomas were named as Malignant Mixed Mullerian tumour (MMMT), but inrecent classifications according to their pathologic structure and its behaviour,these tumours are classified as carcinomas.The rare groupof sarcomas is endometrial stromal sarcoma (ESS), which occurs in youngerwomen. In a medical study search from 2000 to 2017, all kinds of uterine sarcomas,pathologic diagnostic approaches, primary treatment and supportive treatment havebeen analysed.Lasthistological classification is based on FIGO 2009 and WHO.
According to suchclassification, sarcomas divided into three subtypes: leiomyosarcoma, endometrialstromal sarcoma and carcinosarcomas. Diagnosis of sarcomas before treatment anddistinguishing from benign myoma by current diagnostic methods is difficult.Pre-operative endometrial sampling identifies only 25% of sarcomas. It might bethe myometrial origin of tumour. Currently, MRI,ultrasound and PET scan may be used for the diagnosis of tumour. The goldstandard of treatment is complete and intact resection of tumour, including freemargins.
In advanced or recurrence disease, cytoreductive surgery followed bychemotherapy is the choice of treatment. If it is notpossible or there are further abdominal metastases, palliative chemotherapyshould be considered. Combination of Gemcitabine and Docetaxel are acceptablechoices. Recent studies are going to approve the effective role for targeted agentswith or without cytotoxic chemotherapy in these group of aggressive tumours.The only drugin this group has approval is pazopanib (Votrient). However, it did not achieveacceptable responses in phase I and II studies.
In regards to tumour biologyand inappropriate response to chemotherapy and radiotherapy, sarcomas have poorprognosis in all stages.