Uterine sarcomas
Sarcomas account for 3-7% of all uterine cancers. New FIGO staging systems have been developed for uterine sarcomas.
• Carcinosarcomas are now considered and managed as high-grade endometrial carcinomas and no longer form part of uterine sarcomas (5).
• Leiomyosarcomas are staged using the new 2009 FIGO system (Table 63.5) (57). They are usually associated with a poor prognosis. Distant spread is common. Tumour size more than 5 cm, epithelioid-type histology, and severe atypia are histological features associated with distant metastasis (58).
• Adenosarcomas: adenosarcoma, in contrast to carcinosarcoma, is composed of benign glandular tissue and malignant stromal tissue and has a much better prognosis than carcinosarcoma. Adenosarcoma has its own staging system (Table 63.6). Total hysterectomy and bilateral salpingo-oophorectomy is often sufficient treatment. Deep myometrial invasion is a poor prognostic factor (59).
• Endometrial stromal tumours: endometrial stromal cancers are rare and account for less than 1% of uterine tumours. They are classified into two main histological subtypes: endometrial stromal sarcomas and undifferentiated sarcomas. The age of presentation is usually in the fourth and fifth decades and they present in a manner typical of sarcomas including abnormal uterine bleeding, lower abdominal pain, and an abdominal mass.
Endometrial stromal sarcomas are less aggressive than undifferentiated sarcomas but late recurrences are not uncommon. Stage is
Table 63.5 The 2009 FIGO staging of uterine leiomyosarcoma
| Stage I | Tumour confined to the corpus uteri |
| IA | Tumour <5 cm size |
| IB | Tumour >5 cm size |
| Stage II | Tumour extends to the pelvis |
| IIA | Adnexal involvement |
| IIB | Tumour extends to extrauterine pelvic tissue |
| Stage III | Tumour involved abdominal tissues (not just protruding into |
| IIIA | abdomen) |
| IIIB | 1 site |
| IIIC | >1 site Metastases to pelvic and/or para-aortic lymph nodes |
| Stage IV | Tumour invades bladder and/or rectum, and or distant |
| IVA | metastases |
| IVB | Tumour invasion of bladder and/or rectum Distant metastases |
Source data from FIGO Committee on Gynecologic Oncology, FIGO staging for uterine sarcomas.
Int J Gynecol Oncol 2009;104:179. doi:10.1016/j.ijgo.2008.12.009.| Stage I | Tumour confined to the corpus uteri |
| IA | Tumour confined to the endometrium/endocervix with no myometrial invasion |
| IB | Less than 50% myometrial invasion |
| IC | More than half myometrial invasion |
| Stage II | Tumour extends to the pelvis |
| IIA | Adnexal involvement |
| IIB | Tumour extends to extrauterine pelvic tissue |
| Stage III | Tumour involves abdominal tissues |
| IIIA | Involves one site |
| IIIB | More than one site involved |
| IIIC | Metastases to pelvic and/or para-aortic lymph nodes |
| Stage IV | Tumour invades bladder and/or rectum, and/or distant metastases |
| IVA | Tumour invading bladder or rectum |
| IVB | Distant metastases |
Source data from FIGO Committee on Gynecologic Oncology FIGO staging for uterine sarcomas. IntJ Gynecol Oncol 2009.504:179.. doi:10.1016/j.ijgo.2008.12.009.
the most important risk factor in these with a 90% 5-year survival rate for stage I disease. There is some argument for fertility-sparing management in highly selected women with endometrial stromal sarcoma and definitive surgery should be performed once childbearing is complete (60).
Undifferentiated sarcomas are highly aggressive tumours with poor survival rates.
Sixty per cent of patients present with stage III or IV disease (60).The mainstay of treatment of both types of stromal sarcoma remains hysterectomy. Bilateral salpingo-o ophorectomy is recommended in the endometrial stromal sarcoma group as these tumours often express oestrogen receptors and estrogen replacement therapy post surgery is not advisable (61). The role of adjuvant radiation and chemotherapy in the treatment of endometrial stromal sarcomas has not been established. The use of progestins or aromatase inhibitors may have a place in the treatment of endometrial stromal sarcomas.
Treatment of sarcomas
Surgery in uterine leiomyosarcomas
Lymph node and adnexal involvement are rare in leiomyosarcomas, therefore lymphadenectomy and bilateral salpingo-oophorectomy are not mandatory (62). Restaging surgery in patients with an incidental post-hysterectomy diagnosis of leiomyosarcoma is of no benefit.
Chemotherapy for leiomyosarcomas
The role of chemotherapy in patients with leiomyosarcomas is unclear irrespective of stage (63-65). Distant recurrences still occur in patients with early-stage disease whether they receive chemotherapy or not. Targeted treatments have not demonstrated any improved results as yet (66).
Radiotherapy in the treatment of uterine sarcomas
Establishing the role of radiotherapy in the treatment of uterine sarcomas is difficult as most studies have combined carcinosarcoma