Blake Erin A, Sheridan Todd B, Wang Karen L, Takiuchi Tsuyoshi, Kodama Michiko, Sawada Kenjiro, Matsuo Koji
Division of Gynecologic Oncology, Department of Obstetrics and Gynecology, Los Angeles County Medical Center, University of Southern California, Los Angeles, CA, USA; Department of Obstetrics and Gynecology, University of Colorado, Denver, CO, USA.
Department of Pathology, Mercy Medical Center, Baltimore, MD, USA.
Eur J Obstet Gynecol Reprod Biol. 2014 Oct;181:163-70. doi: 10.1016/j.ejogrb.2014.07.050. Epub 2014 Aug 8.
Uterine tumor resembling ovarian sex-cord tumors (UTROSCT) is an extremely rare type of uterine tumor, and its clinical characteristics are not fully understood. A systematic literature search was conducted in PubMed and MEDLINE using the keywords, "uterine tumors resembling ovarian sex cord tumors", limited to case reports. Clinico-pathological characteristics and survival data were abstracted and evaluated for the analysis. Among 43 cases reporting UTROSCT, Type I (endometrial stromal tumors with sex cord-like elements, ESTSCLE) and Type II (classic UTROSCT) were reported in 5 (11.6%) and 17 (39.5%), respectively, and nearly half of reported UTROSCT did not subcategorize the histology pattern into Type I or II (unspecified, n=21, 48.8%). Mean age was 52.2. The two most common symptoms were postmenopausal vaginal bleeding (44.2%) and abnormal menstruation (39.5%). The majority underwent total hysterectomy with adnexectomy (65.1%) followed by hysterectomy alone (18.6%) and tumor resection alone (14.0%). Mean tumor size was 6.2cm, and extra-uterine spread was seen in 7.0%. By immunohistochemistry, calretinin expression was significantly correlated with CAM5.2, inhibin, and progesterone receptor expression (all, p<0.05). In survival analysis, disease-free survival (DFS) rates for all 43 cases at 1, 2, and 5 years for all cases were 97.0%, 92.7%, and 69.7%, respectively. Among recurrent cases, median time to recur was 24 months (range 9-48). Decreased DFS was significantly associated with pelvic pain (2-year rate, 81.8% versus 94.7%, p=0.006), histology subcategory (Type I versus II, 23.8% versus 100%, p=0.006), tumor size ≥10cm (75.0% versus 100%, p=0.046), cervical/extra-uterine metastasis (46.7% versus 100%, p=0.024), and lymphovascular space involvement (50% versus 100%, p=0.002). Treatment patterns were not statistically associated with DFS (hysterectomy, p=0.28; and adnexectomy, p=0.38). When histology patterns were examined, Type II disease was associated with less aggressive tumor behavior when compared to Type I disease: extra-uterine spread (Type I versus II, 40% versus 5.9%, p=0.007) and lymphovascular space invasion (50% versus 6.7%, p=0.012). Among 17 cases of Type II disease, disease recurrence was reported in 1 (5.9%) case at 3 years after the initial treatment. In conclusion, our study showed that UTROSCT was often not subcategorized. Because classic UTROSCT has a distinct clinical outcome and characteristic histological patterns when compared to ESTSCLE, distinguishing UTROSCT from ESTSCLE is an integral component of the diagnosis. While classic UTROSCT typically has a favorable prognosis, it has been known to develop a late recurrence. If risk factors for recurrence are absent, both hysterectomy and mass resection alone are possible options for management.
子宫肿瘤样卵巢性索肿瘤（UTROSCT）是一种极其罕见的子宫肿瘤，其临床特征尚未完全明确。在PubMed和MEDLINE数据库中进行了系统的文献检索，使用关键词“子宫肿瘤样卵巢性索肿瘤”，检索范围限于病例报告。提取并评估临床病理特征和生存数据以进行分析。在43例报告UTROSCT的病例中，I型（具有性索样成分的子宫内膜间质肿瘤，ESTSCLE）和II型（经典UTROSCT）分别有5例（11.6%）和17例（39.5%）报告，近一半报告的UTROSCT未将组织学模式分为I型或II型（未分类，n = 21，48.8%）。平均年龄为52.2岁。最常见的两种症状是绝经后阴道出血（44.2%）和月经异常（39.5%）。大多数患者接受了全子宫切除术加附件切除术（65.1%），其次是单纯子宫切除术（18.6%）和单纯肿瘤切除术（14.0%）。平均肿瘤大小为6.2cm，7.0%的患者出现子宫外扩散。通过免疫组织化学分析，钙视网膜蛋白表达与CAM5.2、抑制素和孕激素受体表达显著相关（均p<0.05）。在生存分析中，43例患者的1年、2年和5年无病生存率（DFS）分别为97.0%、92.7%和69.7%。在复发病例中，复发的中位时间为24个月（范围9 - 48个月）。DFS降低与盆腔疼痛显著相关（2年生存率，81.8%对94.7%，p = 0.006）、组织学亚类（I型对II型，23.8%对100%，p = 0.006）、肿瘤大小≥10cm（75.0%对100%，p = 0.046）、宫颈/子宫外转移（46.7%对100%，p = 0.024）和淋巴管间隙受累（50%对100%，p = 0.002）。治疗方式与DFS无统计学关联（子宫切除术，p = 0.28；附件切除术，p = 0.38）。当检查组织学模式时，与I型疾病相比，II型疾病的肿瘤行为侵袭性较小：子宫外扩散（I型对II型，40%对5.9%，p = 0.007）和淋巴管间隙侵犯（50%对6.7%，p = 0.012）。在17例II型疾病患者中，1例（5.9%）在初始治疗后3年报告疾病复发。总之，我们的研究表明UTROSCT常常未进行亚分类。由于经典UTROSCT与ESTSCLE相比具有独特的临床结局和特征性组织学模式，因此将UTROSCT与ESTSCLE区分开来是诊断的一个重要组成部分。虽然经典UTROSCT通常预后良好，但已知会出现晚期复发。如果不存在复发危险因素，单纯子宫切除术和肿块切除术都是可行的治疗选择。
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