sarcomas include a number of rare tumours with different tumour biology, natural
history and response to treatment, comprise just 3-7% of total uterine malignancies
and about 1% of all gynaecologic cancers. Although they cause important portion
of women death due gynaecologic cancers. These tumours have aggressive behaviour
and high recurrence rate, even when limited to the uterine corpus at the time
of diagnosis. The most common of uterine sarcomas is leiomyosarcoma. The
incidence of leiomyosarcoma is increased after age 50. Traditionally,
carcinosarcomas were named as Malignant Mixed Mullerian tumour (MMMT), but in
recent classifications according to their pathologic structure and its behaviour,
these tumours are classified as carcinomas.
The rare group
of sarcomas is endometrial stromal sarcoma (ESS), which occurs in younger
women. In a medical study search from 2000 to 2017, all kinds of uterine sarcomas,
pathologic diagnostic approaches, primary treatment and supportive treatment have
histological classification is based on FIGO 2009 and WHO. According to such
classification, sarcomas divided into three subtypes: leiomyosarcoma, endometrial
stromal sarcoma and carcinosarcomas. Diagnosis of sarcomas before treatment and
distinguishing from benign myoma by current diagnostic methods is difficult.
Pre-operative endometrial sampling identifies only 25% of sarcomas. It might be
the myometrial origin of tumour.
ultrasound and PET scan may be used for the diagnosis of tumour. The gold
standard of treatment is complete and intact resection of tumour, including free
margins. In advanced or recurrence disease, cytoreductive surgery followed by
chemotherapy is the choice of treatment.
If it is not
possible or there are further abdominal metastases, palliative chemotherapy
should be considered. Combination of Gemcitabine and Docetaxel are acceptable
choices. Recent studies are going to approve the effective role for targeted agents
with or without cytotoxic chemotherapy in these group of aggressive tumours.
The only drug
in this group has approval is pazopanib (Votrient). However, it did not achieve
acceptable responses in phase I and II studies. In regards to tumour biology
and inappropriate response to chemotherapy and radiotherapy, sarcomas have poor
prognosis in all stages.