The enigma of bifocal germ cell tumors in the suprasellar and pineal regions: synchronous lesions or metastasis? Clinical article

The enigma of bifocal germ cell tumors in the suprasellar and pineal regions: synchronous lesions or metastasis? Clinical article
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DOI:
10.3171/2012.10.peds11487
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发表时间:
2013-02-01
影响因子:
1.9
通讯作者:
Wang, Kyu-Chang
Wang, Kyu-Chang
中科院分区:
医学3区
文献类型:
--
作者:
Phi, Ji Hoon;Kim, Seung-Ki;Wang, Kyu-Chang

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物体。颅内生殖细胞肿瘤(GCTS)常表现为鞍上区和松果体区的双灶性病变。这些双灶性GCTS的发病机制一直存在争议。双灶性GCTS可能是由同步肿瘤或肿瘤细胞从一个部位转移到另一个部位引起的。与双灶性GCTS相关的预后也是一个令人担忧的原因。作者构建了一个单一机构的患者队列,包括181名颅内GCTS患者。比较双灶性GCTS与鞍上型、松果体型GCTs的临床特点。23例(12.8%)出现双灶性GCTS。18例表现为双灶性生殖细胞瘤,5例表现为混合性生殖细胞瘤。年龄分布的分析和肿瘤大小的比较与双灶性GCTS的转移起源模型是一致的。11名患者(47.8%)在出现双灶性GCTS时发现了肿瘤播种。肿瘤种植与双灶性病变显著相关(p<0.001)。双灶性生殖细胞瘤患者的无事件生存期和总生存期明显短于单发灶生殖细胞瘤患者。双灶性GCTs并不局限于生殖细胞瘤,但包括混合性GCTs。作者推测,双灶性GCTS可能源于鞍上或松果体GCTS的转移性扩散。生殖细胞瘤的双灶性表现可能是一个预后不佳的迹象,应该提醒临床医生注意播散性疾病的可能性。(http://thejns.org/doi/abs/10.3171/2012.10.PEDS11487)
Object. Intracranial germ cell tumors (GCTs) frequently present with bifocal lesions in both the suprasellar and pineal areas. The pathogenesis of these bifocal GCTs has been the subject of controversy. Bifocal GCTs may be caused by synchronous tumors or by metastatic spread of tumor cells from one site to the other. The prognosis associated with bifocal GCTs has also been a cause of concern.Methods. The authors constructed a single-institution patient cohort comprising 181 patients with intracranial GCTs. The clinical characteristics of bifocal GCTs were compared with those of suprasellar and pineal GCTs.Results. Bifocal GCTs were observed in 23 patients (12.8%). Eighteen patients presented with bifocal GCTs that were diagnosed as germinomas, but 5 patients exhibited mixed GCTs. Analyses of age distributions and comparisons of tumor sizes were compatible with a model of a metastatic origin of bifocal GCTs. Eleven patients (47.8%) presenting with bifocal GCTs exhibited tumor seeding at presentation. Tumor seeding was significantly associated with bifocal lesions (p < 0.001). Patients with bifocal germinomas showed significantly shorter event-free survival and overall survival than did those presenting with germinomas from a single site of origin.Conclusions. Bifocal GCTs are not restricted to germinomas, as had been previously reported, but do include mixed GCTs. The authors hypothesize that bifocal GCTs may result from the metastatic spread of suprasellar or pineal GCTs. The bifocal presentation of germinomas may be a poor prognostic sign and should alert clinicians to the possibility of a disseminated disease. (http://thejns.org/doi/abs/10.3171/2012.10.PEDS11487)