Objective

To describe the rates of GCNIS-free and GCT-free pathology based on age at gonadal surgery and to describe long-term oncologic outcomes in patients with DSD who have GCNIS or GCT at the time of gonadal surgery.

Study design

A systematic review was conducted using MEDLINE to identify patients with DSD who underwent gonadal surgery. DSD diagnoses were stratified based on malignancy risk. GCNIS/GCT and GCT-free survival by age of gonadal surgery, RFS and OS were calculated using the Kaplan–Meier method, with groups compared using log-rank testing.

Results

386 articles from 1951 to 2017 were included (2037 patients). Median age at gonadal surgery was 17 years (y) (IQR 11–20), median follow-up was 60 months (m) (IQR 30–68.1). GCNIS/GCT- and GCT-free survival at the time of gonadal surgery was lowest for those in the high/intermediate risk group (p < 0.001) but decreased sharply around age 15y, regardless of risk category. 5y RFS and OS was similar for those with no GCNIS/GCT and GCNIS and was worse for those with GCT (p < 0.001).

Discussion

When patients undergo gonadal surgery, regardless of indication (i.e. prophylactic vs. tumor), it appears that GCTs are more commonly found when surgery is done around age 15 y or older, despite risk category. This is similar to ovarian and testicular GCTs. Patients with GCNIS can be reassured that long-term oncologic outcomes are excellent. While RFS and OS for GCTs are not as good as for ovarian and testicular GCTs (95%), they are still >80%. This similar trend was found in a COG review of 9 patients with DSD and ovarian GCT.

There were several limitations to this study. This is a retrospective analysis that included aa wide time frame of publications. The indication for surgical intervention was not addressed in the majority of publications. Thus these data provide pathologic outcomes based on age at gonadal surgery rather than the age at which GCNIS/GCT develops over a lifetime, if at all.

Conclusions

The risk of GCNIS or GCT at the time of gonadal surgery appears to increase with age, accelerating between 15 and 20y regardless of risk category. 5y RFS and OS for those with GCNIS is equivalent to those without GCNIS/GCT but is worse for those with GCT. These data may be used when counseling families on timing of gonadal surgery and quantification of outcomes should GCNIS or malignancy be identified.

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Summary Fig..

中文翻译：

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性别发育差异患者的恶性前和浸润性生殖细胞肿瘤的肿瘤学结局-系统评价。

目的

描述基于性腺手术年龄的无GCNIS和无GCT病理率，并描述性腺手术时患有GCNIS或GCT的DSD患者的长期肿瘤学结局。

学习规划

使用MEDLINE进行了系统的评价，以鉴定接受过性腺手术的DSD患者。根据恶性肿瘤风险对DSD诊断进行分层。使用Kaplan–Meier方法计算按性腺手术年龄，RFS和OS划分的GCNIS / GCT和无GCT生存率，并使用对数秩检验比较各组。

结果

纳入了1951年至2017年的386篇文章（2037例患者）。性腺手术的中位年龄为17岁（y）（IQR 11-20），中位随访时间为60个月（m）（IQR 30-68.1）。高危/中危组中，性腺手术时GCNIS / GCT和无GCT的存活率最低（p <0.001），但无论风险类别如何，在15岁左右急剧下降。没有GCNIS / GCT和GCNIS的5y RFS和OS相似，而没有GNIS的5y RFS和OS更差（p <0.001）。

讨论区

当患者进行性腺手术时，无论其适应症是什么（即预防还是肿瘤），尽管有风险类别，但似乎在15岁或更大的年龄进行手术时更常见于GCT。这类似于卵巢和睾丸GCT。可以放心，GCNIS患者的长期肿瘤学结局非常好。尽管GCT的RFS和OS不如卵巢和睾丸GCT（95％），但仍> 80％。在对9名DSD和卵巢GCT患者的COG复查中发现了类似的趋势。

这项研究有几个局限性。这是一项回顾性分析，其中涉及许多出版物。大多数出版物未提及手术干预的指征。因此，这些数据可根据性腺手术的年龄而不是一生中GCNIS / GCT发生的年龄提供病理结果。

结论

进行性腺手术时，GCNIS或GCT的风险似乎随着年龄的增长而增加，无论风险类别如何，其风险都会在15至20岁之间加速。具有GCNIS的用户的5年RFS和OS等同于不具有GCNIS / GCT的用户，但对于具有GCT的用户则更糟。在为家庭提供性腺手术时机咨询以及量化GCNIS或恶性肿瘤时对结果进行咨询时，可以使用这些数据。

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总结图。