Wolf Benjamin, Kunert Christin, Horn Lars-Christian, Einenkel Jens
*Department of Obstetrics and Gynecology, Women and Children's Center, University Hospital Leipzig, Leipzig; †Department of Internal Medicine, Harzklinikum Wernigerode, Wernigerode; and ‡Institute for Pathology, University Hospital Leipzig, Leipzig, Germany.
Int J Gynecol Cancer. 2017 Jul;27(6):1064-1071. doi: 10.1097/IGC.0000000000001013.
Primary retroperitoneal mucinous tumors (PRMTs) are rare epithelial neoplasms arising in the retroperitoneal space whose pathogenesis is poorly understood. To date, only single-case reports and small case series on diagnosis and management of these tumors exist. The aim of this first-time meta-analysis was to provide more reliable data to inform the management of PRMTs.
Database searches were conducted to identify case reports and case series between the years of 1975 and 2015. Two cases of patients treated at our institution were also included. Diagnostic, radiographic, surgical, and follow-up data were gathered. Regression modeling was used to identify factors aiding preoperative diagnosis and affecting treatment. Survival analysis including Cox regression modeling was performed to provide insights into treatment effects on the prognosis of female patients with PRMTs.
One hundred forty-four cases of PRMTs were included in this analysis. Eighty-nine percent occurred in women. Most of the PRMTs were found to be malignant (53%). No preoperative factors predicting tumor dignity were identified by multiple regression analysis. Five-year disease specific survival of patients with benign and borderline tumors was excellent, reaching 100%, whereas it was only 68% for patients with malignant disease. Adjuvant surgical treatments including hysterectomy, salpingoophorectomy, and lymph node dissection were not associated with a statistically significant survival advantage. However, there was a nonsignificant association of hysterectomy with improved survival (hazard ratio, 0.42; 95% confidence interval, 0.09-2.07; P = 0.285). Adjuvant chemotherapy was associated with reduced survival (hazard ratio 3.791; 95% confidence interval, 1.509-9.526; P = 0.0046).
A reliable diagnosis of a PRMT can only be made by pathological examination of the tumor. Surgical excision is thus necessary to facilitate diagnosis while it also remains the mainstay of treatment. There is no conclusive evidence supporting the role of adjuvant surgical procedures or chemotherapy.
原发性腹膜后黏液性肿瘤（PRMTs）是起源于腹膜后间隙的罕见上皮性肿瘤，其发病机制尚不清楚。迄今为止，关于这些肿瘤诊断和治疗的仅有单病例报告和小病例系列。首次进行的这项荟萃分析旨在提供更可靠的数据，为PRMTs的治疗提供依据。
进行数据库检索，以识别1975年至2015年间的病例报告和病例系列。还纳入了在我们机构接受治疗的2例患者。收集诊断、影像学、手术和随访数据。采用回归模型确定有助于术前诊断和影响治疗的因素。进行生存分析，包括Cox回归模型，以深入了解治疗对PRMTs女性患者预后的影响。
本分析纳入了144例PRMTs。89%发生在女性。大多数PRMTs被发现是恶性的（53%）。多元回归分析未发现预测肿瘤良恶性的术前因素。良性和交界性肿瘤患者的5年疾病特异性生存率极佳，达到100%，而恶性疾病患者仅为68%。包括子宫切除术、输卵管卵巢切除术和淋巴结清扫术在内的辅助性手术治疗与统计学上显著的生存优势无关。然而，子宫切除术与生存改善之间存在不显著的关联（风险比，0.42；95%置信区间，0.09 - 2.07；P = 0.285）。辅助化疗与生存率降低相关（风险比3.791；95%置信区间，1.509 - 9.526；P = 0.0046）。
PRMTs的可靠诊断只能通过肿瘤的病理检查做出。因此，手术切除对于促进诊断是必要的，同时它仍然是主要的治疗方法。没有确凿证据支持辅助性手术或化疗的作用。
