Yasukawa Maya, Dainty Louis A, Sugarbaker Paul H
Department of Obstetrics and Gynecology, MedStar Washington Hospital Center, Washington, DC, USA.
Regional Director of Gynecologic Oncology at Georgetown Lombardi Comprehensive Cancer Center, Chief of Gynecologic Oncology Department at MedStar Washington Cancer, Washington, DC, USA.
Gynecol Oncol Rep. 2021 Mar 4;36:100741. doi: 10.1016/j.gore.2021.100741. eCollection 2021 May.
Uterine leiomyosarcoma (uLMS) is a rare aggressive malignant mesenchymal tumor with high risk of recurrence and poor prognosis regardless of stage. It is often diagnosed postoperatively following myomectomy, hysterectomy or supracervical hysterectomy for presumed benign disease. Primary surgery at the diagnosis of uLMS is considered to affect outcomes. If the tumor was morcellated, the oncologist will encounter special problems that require knowledgeable management of peritoneal metastases. We previously reported that six patients who successfully underwent cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) plus early postoperative intraperitoneal chemotherapy (EPIC) to manage the disease dissemination that must occur following morcellation. This is a study for long-term outcome of these patients. Six patients were treated with an absence of grade IV adverse events and no mortality. The median Peritoneal Cancer Index (PCI) was 18 and complete cytoreduction without peritoneal metastases visualized within the operative field at the completion of the surgical procedure (CC-0) was achieved in all patients. One patient was diagnosed leiomyomatosis peritonealis postoperatively. Among five patients who were confirmed uterine leiomyosarcoma, the 3-year overall survival was 40.0% and 5-year overall survival was 20.0% with the median follow-up of 18 months (range 5-73 months). The patient with PCI 0 at the time of CRS showed no evidence of disease (NED) at 73 months. We believe that prophylactic CRS contributed her favorable outcome. Therapeutic options for patients with uLMS post-morcellation are limited. Currently, CRS and HIPEC plus EPIC followed by adjuvant systemic chemotherapy may be considered an option for treatment. Further studies in a larger number of patients are needed.
子宫平滑肌肉瘤（uLMS）是一种罕见的侵袭性恶性间叶肿瘤，无论处于何阶段，其复发风险高且预后不良。它常在因疑似良性疾病而进行子宫肌瘤切除术、子宫切除术或次全子宫切除术后被诊断出来。在诊断为uLMS时进行的初次手术被认为会影响治疗结果。如果肿瘤被粉碎，肿瘤学家将面临特殊问题，需要对腹膜转移进行专业处理。我们之前报告过6例患者成功接受了细胞减灭术（CRS）、热灌注化疗（HIPEC）以及术后早期腹腔内化疗（EPIC），以处理粉碎术后必然发生的疾病播散。这是一项关于这些患者长期预后的研究。6例患者接受治疗，未出现IV级不良事件，也无死亡病例。腹膜癌指数（PCI）中位数为18，所有患者在手术结束时均实现了完全细胞减灭，手术视野内未见腹膜转移（CC-0）。1例患者术后被诊断为腹膜平滑肌瘤病。在5例确诊为子宫平滑肌肉瘤的患者中，3年总生存率为40.0%，5年总生存率为20.0%，中位随访时间为18个月（范围5 - 73个月）。CRS时PCI为0的患者在73个月时无疾病证据（NED）。我们认为预防性CRS促成了她良好的治疗结果。uLMS粉碎术后患者的治疗选择有限。目前，CRS、HIPEC加EPIC，随后进行辅助全身化疗可被视为一种治疗选择。需要对更多患者进行进一步研究。
