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儿童人群中的卵巢表面上皮性肿瘤:发病率、组织学亚型和自然病史。

Ovarian surface epithelial neoplasms in the pediatric population: incidence, histologic subtype, and natural history.

机构信息

Department of Pathology, Stanford University School of Medicine, Stanford, CA 94305, USA.

出版信息

Am J Surg Pathol. 2013 Apr;37(4):548-53. doi: 10.1097/PAS.0b013e318273a9ff.

DOI:10.1097/PAS.0b013e318273a9ff
PMID:23388124
Abstract

Surface epithelial neoplasms account for a small but significant proportion of pediatric ovarian tumors. The overall incidence, prevalence of histologic subtypes, and natural history of these neoplasms has not been thoroughly evaluated. A retrospective review of the pathology archives of Stanford University School of Medicine yielded 69 surface epithelial ovarian tumors in 64 pediatric patients 18 years of age or younger from 1974 to 2010. Tumors comprised benign (57.8%), borderline/low malignant potential (LMP) (37.5%), and malignant (4.7%) subgroups and exhibited serous, mucinous, and mixed histology; there were no clear cell, pure endometrioid, or transitional (Brenner) tumors. In addition, no high-grade carcinomas were identified. Clinical follow-up data were available in a subset of patients (maximum follow-up, 22 y). Similar numbers of recurrences were found in each of the 3 subgroups. However, overall survival was 100% for benign and borderline/LMP tumors and 50% for carcinomas. The type of surgical management and the use of chemotherapy varied; 2 patients with borderline/LMP tumors were treated by sterilizing procedures and/or chemotherapy. These data suggest that surface epithelial neoplasms comprise a small but significant proportion of ovarian tumors in the pediatric population, and they exhibit a marked preponderance for benign, borderline, and low-grade malignant subgroups. In contrast to their adult counterpart, high-grade serous carcinoma in children is extraordinarily rare and not seen in this series. Given this difference, uniform treatment modalities with consideration for ovarian conservation and fertility preservation should be rigorously adopted in any pediatric patient with a suspected ovarian surface epithelial neoplasm.

摘要

表面上皮性肿瘤占小儿卵巢肿瘤的一小部分,但具有重要意义。这些肿瘤的总体发生率、组织学亚型的流行率和自然病史尚未得到彻底评估。斯坦福大学医学院病理学档案的回顾性研究显示,1974 年至 2010 年间,64 例年龄在 18 岁以下的小儿患者中有 69 例存在卵巢表面上皮性肿瘤。肿瘤包括良性(57.8%)、交界性/低恶性潜能(LMP)(37.5%)和恶性(4.7%)亚组,并表现出浆液性、黏液性和混合性组织学;没有透明细胞、纯子宫内膜样或过渡性(Brenner)肿瘤。此外,没有发现高级别癌。在一部分患者中可获得临床随访数据(最长随访时间为 22 年)。在这 3 个亚组中,复发的例数相似。然而,良性和交界性/LMP 肿瘤的总生存率为 100%,而癌的总生存率为 50%。手术管理类型和化疗的使用有所不同;2 例交界性/LMP 肿瘤患者采用绝育手术和/或化疗进行治疗。这些数据表明,表面上皮性肿瘤构成小儿卵巢肿瘤的一小部分,但具有显著的良性、交界性和低级别恶性倾向。与成人相比,儿童的高级别浆液性癌极为罕见,在本系列中未见。鉴于这种差异,对于任何疑似卵巢表面上皮性肿瘤的儿科患者,都应严格采用统一的治疗模式,同时考虑卵巢保留和生育力保存。

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