So-called bifocal tumors with diabetes insipidus and negative tumor markers: are they all germinoma?
So-called bifocal tumors with diabetes insipidus and negative tumor markers: are they all germinoma?
复制标题
DOI：
10.1093/neuonc/noaa199
复制
发表时间：
2021-02-01
期刊：
影响因子：
15.9
通讯作者：
Nishikawa, Ryo
中科院分区：
文献类型：
--
作者：
Kanamori, Masayuki;Takami, Hirokazu;Nishikawa, Ryo
背景资料。关于生殖细胞肿瘤(GCTS)治疗的德尔福共识声明未能就以下声明达成一致：(I)松果体和神经垂体部双灶性病变，(Ii)尿崩症，(Iii)肿瘤标志物阴性的病例可以在没有组织学验证的情况下被诊断为生殖细胞瘤。为了回答这个问题，进行了多中心的回顾分析。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、组织诊断和手术细节的调查问卷。51个研究所报告了132例符合3个标准的病例。91例分别从松果体(n=44)、神经垂体部(n=32)、两者(n=6)和远处(n=9)病变进行了组织取样。组织学确诊89例，其中单纯生殖细胞瘤或生殖细胞瘤伴合体滋养细胞巨细胞82例(92.1%)，生殖细胞瘤和成熟畸胎瘤2例，肉芽肿性炎症2例。2例组织学诊断不明确。尽管未发现其他肿瘤，但有3例(3.4%)患者有非生殖细胞瘤性GCT(NGGCT)。无一例患者在内窥镜或立体定向活检后出现永久性并发症。39例急性脑积水同期手术，无永久性并发症，94.9%脑积水得到控制。所有符合3个标准的患者都有GCTS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期手术的安全性和效果，建议对此类患者进行活组织检查。
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.