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.
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。