Esfahani Darian R, Alden Tord, DiPatri Arthur, Xi Guifa, Goldman Stewart, Tomita Tadanori
Division of Pediatric Neurosurgery, Ann & Robert H. Lurie Children's Hospital, Chicago, IL 60611, USA.
Department of Neurosurgery, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA.
Cancers (Basel). 2020 Sep 14;12(9):2621. doi: 10.3390/cancers12092621.
Suprasellar germ cell tumors (S-GCTs) are rare, presenting in either solitary or multifocal fashion. In this study, we retrospectively examine 22 solitary S-GCTs and 20 bifocal germ cell tumors (GCTs) over a 30-year period and demonstrate clinical, radiographic, and prognostic differences between the two groups with therapeutic implications. Compared to S-GCTs, bifocal tumors were almost exclusively male, exhibited higher rate of metastasis, and had worse rates of progression free and overall survival trending toward significance. We also introduce a novel magnetic resonance (MR) imaging classification of suprasellar GCT into five types: a IIIrd ventricle floor tumor extending dorsally with or without an identifiable pituitary stalk (Type Ia, Ib), ventrally (Type III), in both directions (Type II), small lesions at the IIIrd ventricle floor extending to the stalk (Type IV), and tumor localized in the stalk (Type V). S-GCTs almost uniformly presented as Type I-III, while most bifocal GCTs were Type IV with a larger pineal mass. These differences are significant as bifocal GCTs representing concurrent primaries or subependymal extension may be treated with whole ventricle radiation, while cerebrospinal fluid (CSF)-borne metastases warrant craniospinal irradiation (CSI). Although further study is necessary, we recommend CSI for bifocal GCTs exhibiting high-risk features such as metastasis or non-germinomatous germ cell tumor histology.
鞍上生殖细胞肿瘤（S-GCTs）较为罕见，以单发或多发形式出现。在本研究中，我们回顾性研究了30年间的22例单发S-GCTs和20例双灶性生殖细胞肿瘤（GCTs），并展示了两组之间在临床、影像学及预后方面的差异及其治疗意义。与S-GCTs相比，双灶性肿瘤几乎均为男性，转移率更高，无进展生存率和总生存率更差，且有显著趋势。我们还引入了一种新的鞍上GCT磁共振（MR）成像分类，分为五种类型：第三脑室底部肿瘤向背侧延伸，有无可识别的垂体柄（Ia型、Ib型），向腹侧延伸（III型），双向延伸（II型），第三脑室底部小病变延伸至垂体柄（IV型），以及肿瘤局限于垂体柄（V型）。S-GCTs几乎均表现为I-III型，而大多数双灶性GCTs为IV型且松果体肿块较大。这些差异具有重要意义，因为代表同时性原发灶或室管膜下扩展的双灶性GCTs可能采用全脑室放疗，而脑脊液（CSF）播散性转移则需要进行全脑脊髓照射（CSI）。尽管有必要进一步研究，但我们建议对表现出转移或非生殖细胞瘤性生殖细胞肿瘤组织学等高危特征的双灶性GCTs采用CSI。
