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

Abstract

<jats:sec> <jats:title>Object</jats:title> <jats:p>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.</jats:p></jats:sec> <jats:sec> <jats:title>Methods</jats:title> <jats:p>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.</jats:p></jats:sec> <jats:sec> <jats:title>Results</jats:title> <jats:p>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 &lt; 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.</jats:p></jats:sec> <jats:sec> <jats:title>Conclusions</jats:title> <jats:p>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.</jats:p></jats:sec>

Current concepts and future strategies in the management of intracranial germinoma

“…Involvement of basal ganglia may cause deficits in motor function [4]. There is still controversy as to whether bifocal tumor manifestations should be categorized as metastatic disease [5,6]. In the SIOP CNS GCT 96 study in bifocal tumor manifestation, no difference in tumor control could be detected between patients receiving local irradiation alone combined with chemotherapy and craniospinal irradiation [7].…”

Rare Presentation of Supratentorial Primitive Neuroectodermal Tumors Mimicking Bifocal Germ Cell Tumors

“…[2][3][4] Generally-although not exclusively-this term is used to describe the involvement of both the pineal and suprasellar region by a germ cell tumor (GCT), most commonly a germinoma. However, bifocal tumors have also been reported in patients with nongerminomatous GCTs 5 and with other histologies in other sites of the brain. 5,6 As bifocal pineal and suprasellar brain tumors are highly suggestive of central nervous system (CNS) GCT and have never been reported in other pediatric brain tumors, some authors have questioned the relevance of biopsying these bifocal lesions.…”

“…However, bifocal tumors have also been reported in patients with nongerminomatous GCTs 5 and with other histologies in other sites of the brain. 5,6 As bifocal pineal and suprasellar brain tumors are highly suggestive of central nervous system (CNS) GCT and have never been reported in other pediatric brain tumors, some authors have questioned the relevance of biopsying these bifocal lesions. 7 We report 2 cases of supratentorial primitive neuroectodermal tumor (PNET) that presented as bifocal tumors at the pineal and suprasellar regions.…”

The combination of a bifocal germinoma and arteriovenous malformations of the brain in children. Clinical observation and review of the literature

“…Метастатическое распространение, выявляемое при нейровизуализации или цитологическом исследова-нии ликвора, имеют 23-55 % бифокальных опухолей [15][16]. Корректное стадирование заболевания имеет решающее значение при инициальной диагностике [12].…”

“…В то же время неко-торые авторы предполагают более плохой прогноз для бифокальных гермином, так как рассматривают их с точки зрения метастатического распространения со-литарного опухолевого узла [15].…”