Fertility-Sparing Surgery in Uterine Leiomyosarcoma

Abstract & Commentary

Synopsis: Selected cases of uterine leiomyosarcoma may be conservatively managed in young nulliparous women desiring pregnancy.

Source: Lissoni A, et al. Gynecol Oncol 1998;70:348-350.

Lissoni and associates have reported eight patients with a diagnosis of uterine leiomyosarcoma between 1982 and 1996 who were managed conservatively after myomectomy. After myomectomy, surveillance consisted of physical examination, vaginal sonography, and hysteroscopy every three months for the first two years and every six months thereafter. Median age was 29 years (range, 19-32 years). All were nulliparous. The tumor was confined to the myoma in all patients. The mean mitotic count of the leiomyosarcomas was 6/10 high-power fields, with a range of 5-33. At a median follow-up of 42 months, three pregnancies were reported. Two patients had a spontaneous delivery at term. The third patient had a diagnosis of recurrent disease at the time of cesarean section. Despite further surgery and chemotherapy, she died of disseminated tumor 26 months after diagnosis. The remaining seven patients are alive and well. Two patients underwent a second surgical procedure after diagnosis of leiomyosarcoma, 24 and 16 months after primary surgery; both were found to have leiomyomas. Lissoni et al conclude that selected cases of uterine leiomyosarcoma might be managed conservatively in young nulliparous women desiring pregnancy. They emphasized that a strict follow-up is mandatory, and, at completion of childbearing, a hysterectomy could be considered.

Comment by David M. Gershenson, MD

This is a provocative report of fertility-sparing surgery after diagnosis of leiomyosarcoma treated with myomectomy. Lissoni et al are clearly pushing the envelope in terms of what is considered standard care. Leiomyosarcoma is an extremely rare malignancy, accounting for only about 25% of all uterine sarcomas. It is found arising in a benign leiomyoma in less than 1% of cases. It should be noted that the histopathologic definition of leiomyosarcoma has changed recently, with more emphasis placed on the presence of atypia and necrosis than on mitotic count alone. Standard treatment for high-grade leiomyosarcomas consists of abdominal hysterectomy and bilateral salpingo-oophorectomy. Approximately 50% of patients whose tumor is apparently confined to the uterus will ultimately develop recurrence. There is no known effective adjuvant therapy. Pelvic irradiation probably reduces the incidence of pelvic recurrence but has no effect on survival. Chemotherapy is relatively ineffective in treating metastatic disease. As emphasized by Lissoni et al, in considering a conservative approach after myomectomy, the patient and her family must be advised of the risks. Unfortunately, the precise risk of recurrence is unknown. Lissoni et al refer to two other series detailing 11 cases of conservative management after myomectomy for leiomyosarcoma; only one patient recurred and six pregnancies were documented. In the present series, one patient recurred and died. Therefore, based on small numbers, one could estimate the risk of recurrence in such patients as 10-15%. However, it is important to underscore the fact that recurrence almost always equals death. In addition, it would seem advisable to retrospectively review the pathology from the world experience using modern criteria. In the interim, extreme caution is recommended until more experience is reported.