Gynaecological sarcomas account for 3-4% of all gynaecological malignancies and have a poorer prognosis compared to gynaecological carcinomas. Pivotal treatment for early-stage uterine sarcoma is represented by total hysterectomy. Whereas oophorectomy provides survival advantage in endometrial stromal sarcoma is still controversial. When the disease is confined to the uterus, systematic pelvic and para-aortic lymphadenectomy is not recommended. Removal of enlarged lymph-nodes is indicated in case of disseminated or recurrent disease, where debulking surgery is considered the standard of care. Fertility sparing surgery for uterine leiomyosarcoma is not supported by strong evidence, whilst available data on fertility sparing treatment for endometrial stromal sarcoma are more promising. For ovarian sarcomas, in the absence of specific data, it is reasonable to adapt recommendations existing for uterine sarcomas, also regarding the role of lymphadenectomy in both early and advanced stage disease. Specific recommendations on cervical sarcomas' surgery are lacking. Existing data on surgical approach vary from radical hysterectomy to fertility-preserving surgery in the form of trachelectomy or wide local excision, however no definite conclusions can be drafted on the recommended surgical approach. For vulval sarcomas, complete surgical excision with at least 2 cm of free margin is considered to be the primary treatment which is associated with good prognosis. The aim of this review is to provide highest quality evidence to guide gynaecologic oncologists throughout surgical management of gynaecological sarcomas.
BackgroundOvarian cancer (OC) is the leading cause of death among gynecological cancers, and the fifth leading cause of death in women among all cancers (1). Despite improvements in technology and the accuracy of radiological and laboratory diagnostic tests, around 60% of OC is actually diagnosed at an advanced stage, which therefore remains the main prognostic factor (1).The complete surgical removal of the disease, followed by platinum based chemotherapy, has still the greatest impact on survival for advanced OC (2,3), while a fertility sparing surgery (FSS) appears to be safe just in patients with low-grade stage IA (serous, endometrioid or mucinous expansile subtype) (4,5), and acceptable for stage IC1 tumors, where about 50% of recurrences are located in the remaining ovary and therefore suitable for subsequent surgery (6).
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