Pediatric Suprasellar Germ Cell Tumors: A Clinical and Radiographic Review of Solitary vs. Bifocal Tumors and Its Therapeutic Implications
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 th...
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| Format: | Article |
| Language: | English |
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MDPI AG
2020-09-01
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| Series: | Cancers |
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| Online Access: | https://www.mdpi.com/2072-6694/12/9/2621 |
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| author | Darian R. Esfahani Tord Alden Arthur DiPatri Guifa Xi Stewart Goldman Tadanori Tomita |
| author_facet | Darian R. Esfahani Tord Alden Arthur DiPatri Guifa Xi Stewart Goldman Tadanori Tomita |
| author_sort | Darian R. Esfahani |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-03-10T16:21:00Z |
| format | Article |
| id | doaj.art-e020788015494be6a0db5b7dafd49376 |
| institution | Directory Open Access Journal |
| issn | 2072-6694 |
| language | English |
| last_indexed | 2024-03-10T16:21:00Z |
| publishDate | 2020-09-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Cancers |
| spelling | doaj.art-e020788015494be6a0db5b7dafd493762023-11-20T13:40:38ZengMDPI AGCancers2072-66942020-09-01129262110.3390/cancers12092621Pediatric Suprasellar Germ Cell Tumors: A Clinical and Radiographic Review of Solitary vs. Bifocal Tumors and Its Therapeutic ImplicationsDarian R. Esfahani0Tord Alden1Arthur DiPatri2Guifa Xi3Stewart Goldman4Tadanori Tomita5Division of Pediatric Neurosurgery, Ann & Robert H. Lurie Children’s Hospital, Chicago, IL 60611, USADivision of Pediatric Neurosurgery, Ann & Robert H. Lurie Children’s Hospital, Chicago, IL 60611, USADivision of Pediatric Neurosurgery, Ann & Robert H. Lurie Children’s Hospital, Chicago, IL 60611, USADivision of Pediatric Neurosurgery, Ann & Robert H. Lurie Children’s Hospital, Chicago, IL 60611, USADivision of Hematology, Oncology, Neuro-Oncology & Stem Cell Transplantation, Ann & Robert H. Lurie Children’s Hospital, Chicago, IL 60611, USADivision of Pediatric Neurosurgery, Ann & Robert H. Lurie Children’s Hospital, Chicago, IL 60611, USASuprasellar 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.https://www.mdpi.com/2072-6694/12/9/2621intracranial germ cell tumorgerminomasuprasellar tumordual germ cell tumorbifocal germ cell tumornon-germinoma germ cell tumor |
| spellingShingle | Darian R. Esfahani Tord Alden Arthur DiPatri Guifa Xi Stewart Goldman Tadanori Tomita Pediatric Suprasellar Germ Cell Tumors: A Clinical and Radiographic Review of Solitary vs. Bifocal Tumors and Its Therapeutic Implications Cancers intracranial germ cell tumor germinoma suprasellar tumor dual germ cell tumor bifocal germ cell tumor non-germinoma germ cell tumor |
| title | Pediatric Suprasellar Germ Cell Tumors: A Clinical and Radiographic Review of Solitary vs. Bifocal Tumors and Its Therapeutic Implications |
| title_full | Pediatric Suprasellar Germ Cell Tumors: A Clinical and Radiographic Review of Solitary vs. Bifocal Tumors and Its Therapeutic Implications |
| title_fullStr | Pediatric Suprasellar Germ Cell Tumors: A Clinical and Radiographic Review of Solitary vs. Bifocal Tumors and Its Therapeutic Implications |
| title_full_unstemmed | Pediatric Suprasellar Germ Cell Tumors: A Clinical and Radiographic Review of Solitary vs. Bifocal Tumors and Its Therapeutic Implications |
| title_short | Pediatric Suprasellar Germ Cell Tumors: A Clinical and Radiographic Review of Solitary vs. Bifocal Tumors and Its Therapeutic Implications |
| title_sort | pediatric suprasellar germ cell tumors a clinical and radiographic review of solitary vs bifocal tumors and its therapeutic implications |
| topic | intracranial germ cell tumor germinoma suprasellar tumor dual germ cell tumor bifocal germ cell tumor non-germinoma germ cell tumor |
| url | https://www.mdpi.com/2072-6694/12/9/2621 |
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