Abstract：
Ovarian tumors in the pediatric population are rare. The incidence and frequency of subtypes differ between children and adults. Although not all tumors are aggressive, they may still lead to morbidity. The goal of this study was a comprehensive review of malignant ovarian tumors in children and adolescents diagnosed and registered in Sweden.
Individuals were identified through a search in the National Cancer Register, limited for ages 0-19, years 1970-2014. Stored tumor diagnostic material from regional biobanks was retrieved and reviewed.
The study includes 345 individuals with ovarian tumors and 70.7% of them were between 15 and 19 years at time of diagnosis. No differences in incidence over time or geographic location were identified. The average follow-up time was 21.2 years and 5-year survival was 88.4%. Survival was similar in the different time periods, except for 1970-1979. Review was possible for 260 cases, resulting in 85 epithelial tumors, 121 GCTs, 47 SCSTs and 7 others. For age 0-4 years SCSTs dominated (85.7%), for 5-9- and 10-14-years GCTs dominated (70,8% and 75.0% respectively), and for age 15-19 years epithelial tumors dominated (43.8%). There was a strong agreement between review diagnosis and original diagnosis (Cohen\'s κ 0.944). Differentiating between entities within the sex cord-stromal group posed the biggest diagnostic challenge.
Ovarian tumors in children and adolescents are rare and distinct from their adult counterparts regarding incidence and frequency. There was a strong concurrence between original and review diagnoses. The greatest diagnostic difficulty was subtyping of epithelial tumors and differentiating between tumors within the SCST group.
Individuals were identified through a search in the National Cancer Register, limited for ages 0-19, years 1970-2014. Stored tumor diagnostic material from regional biobanks was retrieved and reviewed.
The study includes 345 individuals with ovarian tumors and 70.7% of them were between 15 and 19 years at time of diagnosis. No differences in incidence over time or geographic location were identified. The average follow-up time was 21.2 years and 5-year survival was 88.4%. Survival was similar in the different time periods, except for 1970-1979. Review was possible for 260 cases, resulting in 85 epithelial tumors, 121 GCTs, 47 SCSTs and 7 others. For age 0-4 years SCSTs dominated (85.7%), for 5-9- and 10-14-years GCTs dominated (70,8% and 75.0% respectively), and for age 15-19 years epithelial tumors dominated (43.8%). There was a strong agreement between review diagnosis and original diagnosis (Cohen\'s κ 0.944). Differentiating between entities within the sex cord-stromal group posed the biggest diagnostic challenge.
Ovarian tumors in children and adolescents are rare and distinct from their adult counterparts regarding incidence and frequency. There was a strong concurrence between original and review diagnoses. The greatest diagnostic difficulty was subtyping of epithelial tumors and differentiating between tumors within the SCST group.
摘要：
目的：卵巢肿瘤在儿童人群中是罕见的。儿童和成人之间亚型的发生率和频率不同。尽管并非所有肿瘤都具有侵袭性，它们仍然可能导致发病。这项研究的目的是全面审查在瑞典诊断和注册的儿童和青少年的恶性卵巢肿瘤。
方法：通过国家癌症登记处的搜索确定个体，仅限0-19岁，1970-2014年。检索并审查了来自区域生物库的存储的肿瘤诊断材料。
结果：该研究包括345例卵巢肿瘤患者，其中70.7%的患者在诊断时年龄在15至19岁之间。没有发现发病率随时间或地理位置的差异。平均随访时间为21.2年，5年生存率为88.4%。不同时期的生存率相似,除了1970-1979年。260例可以进行审查，导致85个上皮肿瘤，121个GCT,47个SCSTs和其他7个。对于0-4岁的SCST占主导地位(85.7%)，在5-9年和10-14年的GCT中占主导地位（分别为70,8%和75.0%），年龄为15-19岁的上皮性肿瘤占主导地位（43.8％）。复查诊断和原始诊断之间有很强的一致性(科恩κ0.944)。区分性索基质组内的实体构成了最大的诊断挑战。
结论：儿童和青少年卵巢肿瘤是罕见的，在发病率和频率方面与成人卵巢肿瘤不同。原始诊断和审查诊断之间存在很强的一致性。最大的诊断困难是上皮肿瘤的分型和SCST组中肿瘤的区分。
方法：通过国家癌症登记处的搜索确定个体，仅限0-19岁，1970-2014年。检索并审查了来自区域生物库的存储的肿瘤诊断材料。
结果：该研究包括345例卵巢肿瘤患者，其中70.7%的患者在诊断时年龄在15至19岁之间。没有发现发病率随时间或地理位置的差异。平均随访时间为21.2年，5年生存率为88.4%。不同时期的生存率相似,除了1970-1979年。260例可以进行审查，导致85个上皮肿瘤，121个GCT,47个SCSTs和其他7个。对于0-4岁的SCST占主导地位(85.7%)，在5-9年和10-14年的GCT中占主导地位（分别为70,8%和75.0%），年龄为15-19岁的上皮性肿瘤占主导地位（43.8％）。复查诊断和原始诊断之间有很强的一致性(科恩κ0.944)。区分性索基质组内的实体构成了最大的诊断挑战。
结论：儿童和青少年卵巢肿瘤是罕见的，在发病率和频率方面与成人卵巢肿瘤不同。原始诊断和审查诊断之间存在很强的一致性。最大的诊断困难是上皮肿瘤的分型和SCST组中肿瘤的区分。
