Simple hysterectomy with or without bilateral salpingo-oophorectomy is the mainstay of surgical management of uterine LMS. Ovaries can be preserved in a young patient with early-stage LMS. The risk of occult ovarian metastasis in early-stage disease is very low, reported to be less than 4 % [6–9]. Ovarian metastasis is generally associated with other extrauterine diseases [8]. A routine oophorectomy has not shown to provide survival advantage or reduce the risk of recurrence [5, 10–13]. Kapp et al. [5] in a Surveillance, Epidemiology, and End Results (SEER) study found no difference in disease-specific survival based on oophorectomy in women younger than 50 years and stage I or II disease. Similarly, in the study by Gadducci et al. [12] that included 126 women with uterine LMS all treated surgically, there was no difference in relapse rates among stage I women younger than 50 years who had ovarian tissue preserved compared to those who had bilateral salpingo-oophorectomy (relapse rates: 23.8 % vs. 33.3; p value = not significant). On the contrary, some retrospective studies have reported an adverse impact of oophorectomy on survival. Garg et al. [14] in a SEER data base analysis of 819 women with LMS showed that on multivariate analysis performance of salpingo-oophorectomy was a poor prognostic factor (p = 0.02) along with other factors, i.e., age, tumor size, and tumor grade. In a study of 208 women of LMS from Mayo Clinic, Giuntoli et al. [11] reported that oophorectomy, high grade, and advanced stage were associated with significantly worse DSS (disease-specific survival). So the current literature suggests that grossly normal ovaries can be conserved in a young patient with early-stage LMS without any detrimental effect on survival. Likewise, when the diagnosis of LMS is made after simple hysterectomy, a re-surgery for removal of the normal adnexa is not indicated.

Another controversial issue is the role of pelvic and para-aortic lymphadenectomy in early-stage LMS. Like soft tissue sarcomas at other sites, hematogenous spread is the primary route of metastasis for uterine LMS. Spread to retroperitoneal lymph nodes is infrequent and almost always associated with advanced disease. The risk of lymph nodal metastasis in early-stage disease is less than 3 %. Table 31.1 summarizes important studies that looked at the incidence of lymph-node metastasis in LMS [5, 7, 9, 11–13, 15–19]. Moreover, no therapeutic benefit has been reported from routine lymphadenectomy in women with early disease and clinically normal lymph nodes. Kapp et al. [5] retrospectively analyzed 1,396 women of uterine LMS; out of 348 women (24.9 %) who underwent lymphadenectomy, 23 (6.6 %) had lymph-node metastasis. All women with positive lymph nodes had advanced disease; 70 % were stage IV. The 5-year disease-specific survival was 64.2 % for women who had negative lymph nodes and 26 % with positive lymph nodes. The 5-year disease-specific survival rates were 75.8 %, 60.1 %, 44.9 %, and 28.7 % for stage I, stage II, stage III, and stage IV, respectively (P < 0.001). Lymphadenectomy did not show any impact on survival in this study. A subset analysis of early-stage disease (n = 1,079) revealed no significant difference in 3-year DSS in women who had lymphadenectomy (n = 291) compared to those who did not (n = 788) (69.7 versus 69.8 %, respectively; p = 0.90). In the Mayo Clinic study [11], lymphadenectomy was performed in 36 out of 208 women; 19 women had both pelvic and para-aortic lymph-node dissection. Four out of 36 (11 %) had positive lymph nodes. Extrauterine disease was present in three of four women with lymph-node involvement emphasizing that lymph-node involvement is generally associated with advanced disease. In another retrospective analysis of 37 women of uterine LMS who underwent lymphadenectomy, none of the women with stage I or II disease had positive nodes and all three women (8.1 %) with nodal metastases had clinically suspicious nodes [9]. Authors also suggested that even in women with gross extrauterine disease, the benefit of removing microscopically involved nodes is limited as most of these women have poor prognosis and die of distant metastasis. Therefore, the current evidence is not in favor of routine retroperitoneal lymph-node dissection in uterine LMS, and this procedure should be undertaken only if lymph nodes are grossly enlarged or in advanced disease as part of cytoreductive surgery.

Approximately 20 % of LMS women will have advanced disease at presentation. Management of these women should be individualized. Surgical cytoreduction should be considered in select cases with good performance status and in whom complete resection of tumor with acceptable morbidity seems to be feasible. The survival of women with complete tumor resection has shown to be better compared to those who had incomplete resection [20]. Lung is the most frequent site of hematogenous spread in uterine LMS. Resection of isolated or limited number of lung metastasis has shown to have a survival benefit and should be considered in appropriately selected women [21, 22].

Management of women diagnosed after surgery for benign lesion can be challenging. If the initial surgery was a myomectomy, a complete hysterectomy is recommended. However, if the initial surgery was a total hysterectomy and postoperative imaging study does not suggest residual disease, a re-surgery is not indicated. Management of women of LMS diagnosed after laparoscopic morcellation of presumed leiomyoma will be discussed later.