Abstract

Background

The Delphi consensus statements on the management of germ cell tumors (GCTs) failed to reach agreements on the statement that the cases with (i) pineal and neurohypophyseal bifocal lesion, (ii) with diabetes insipidus, and (iii) with negative tumor markers can be diagnosed as germinoma without histological verification. To answer this, multicenter retrospective analysis was performed.

Methods

A questionnaire on clinical findings, histological diagnosis, and details of surgical procedures was sent to 86 neurosurgical and 35 pediatrics departments in Japan.

Results

Fifty-one institutes reported 132 cases that fulfilled the 3 criteria. Tissue sampling was performed in 91 cases from pineal (n = 44), neurohypophyseal (n = 32), both (n = 6), and distant (n = 9) lesions. Histological diagnosis was established in 89 cases: pure germinoma or germinoma with syncytiotrophoblastic giant cells in 82 (92.1%) cases, germinoma and mature teratoma in 2 cases, and granulomatous inflammation in 2 cases. Histological diagnosis was not established in 2 cases. Although no tumors other than GCTs were identified, 3 (3.4%) patients had non-germinomatous GCTs (NGGCTs). None of the patients developed permanent complications after endoscopic or stereotactic biopsy. Thirty-nine patients underwent simultaneous procedure for acute hydrocephalus without permanent complications, and hydrocephalus was controlled in 94.9% of them.

Conclusion

All patients who fulfilled the 3 criteria had GCTs or granulomatous inflammation, but not other types of tumors. However, no fewer than 3.4% of the patients had NGGCTs. Considering the safety and the effects of simultaneous procedures for acute hydrocephalus, biopsy was recommended in such patients.

中文翻译：

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所谓的尿崩症双灶肿瘤和肿瘤标志物阴性：它们都是生殖细胞瘤吗？

 抽象的

 背景

关于生殖细胞肿瘤（GCT）管理的德尔菲共识声明未能就以下声明达成一致：（i）松果体和神经垂体双灶性病变，（ii）尿崩症，以及（iii）肿瘤标志物阴性的病例可以未经组织学验证即可诊断为生殖细胞瘤。为了回答这个问题，进行了多中心回顾性分析。

 方法

向日本 86 个神经外科和 35 个儿科发送了一份关于临床表现、组织学诊断和手术细节的调查问卷。

 结果

51 个机构报告了 132 例符合 3 个标准的病例。对 91 例松果体 ( n = 44)、神经垂体 ( n = 32)、两者 ( n = 6) 和远处 ( n = 9) 病变进行了组织取样。 89例均获得组织学诊断：单纯生殖细胞瘤或生殖细胞瘤伴合体滋养层巨细胞82例（92.1%），生殖细胞瘤及成熟畸胎瘤2例，肉芽肿性炎症2例。 2例组织学诊断未确诊。尽管没有发现除 GCT 以外的肿瘤，但 3 名 (3.4%) 患者患有非生殖细胞瘤 GCT (NGGCT)。内窥镜或立体定向活检后，没有患者出现永久性并发症。 39例急性脑积水患者同时接受手术，无永久性并发症，其中94.9%脑积水得到控制。

 结论

所有满足 3 个标准的患者均患有 GCT 或肉芽肿性炎症，但没有其他类型的肿瘤。然而，不少于 3.4% 的患者患有 NGGCT。考虑到急性脑积水同步手术的安全性和影响，建议对此类患者进行活检。