Role of surgery in gynaecological sarcomas.

Valentina Ghirardi,Francesco Guida,Giovanni Scambia,Barbara Costantini,Anna Fagotti,Nicolò Bizzarri,Carmine Vascone

doi:10.18632/oncotarget.26803

Abstract

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.

Highlights

Gynaecological sarcomas account for 3-4% of all gynaecological malignancies and have a poorer prognosis compared to gynaecological carcinomas
Gynaecological sarcomas account for approximately 3% to 4% of all gynaecological malignancies and are associated with poor outcomes compared with gynaecological carcinomas [1]
Gynaecological sarcomas represent a wide spectrum of neoplasms that, due to their rarity, are still www.oncotarget.com partially obscure

Summary

Introduction

Gynaecological sarcomas account for approximately 3% to 4% of all gynaecological malignancies and are associated with poor outcomes compared with gynaecological carcinomas [1]. Uterine sarcomas are approximately 83% of all gynaecological sarcomas. Leiomyosarcoma (uLMS) is the most common histological sub-type, reported in 52% of diagnoses [2], and contributing to a high proportion of death for uterine tumours. For all soft tissue sarcomas, surgery remains the standard of care [3]. We summarize current available evidences on the role of surgery for uterine, ovarian, cervical and vulval sarcomas in both primary and recurrent setting, to guide surgeons throughout the management of this largely obscure and aggressive disease

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