TY - JOUR
T1 - Pediatric suprasellar germ cell tumors
T2 - A clinical and radiographic review of solitary vs. bifocal tumors and its therapeutic implications
AU - Esfahani, Darian R.
AU - Alden, Tord
AU - Dipatri, Arthur
AU - Xi, Guifa
AU - Goldman, Stewart
AU - Tomita, Tadanori
N1 - Funding Information:
Funding: This research was partly supported by Yeager Professorship of Pediatric Neurosurgery (T.T.: endowed) and the Charles and Eleanor Clarke Pediatric Neurosurgery Fellowship (D.R.E.: recipient).
Publisher Copyright:
© 2020 by the authors. Licensee MDPI, Basel, Switzerland.
PY - 2020/9
Y1 - 2020/9
N2 - 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.
AB - 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.
KW - Bifocal germ cell tumor
KW - Dual germ cell tumor
KW - Germinoma
KW - Intracranial germ cell tumor
KW - Magnetic resonance imaging
KW - Non-germinoma germ cell tumor
KW - Overall survival
KW - Progression free survival
KW - Radiation
KW - Suprasellar tumor
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U2 - 10.3390/cancers12092621
DO - 10.3390/cancers12092621
M3 - Article
C2 - 32937871
AN - SCOPUS:85090842489
VL - 12
SP - 1
EP - 16
JO - Cancers
JF - Cancers
SN - 2072-6694
IS - 9
M1 - 2621
ER -