Surgical management of primary central nervous system germ cell tumors

Proceedings from the Second International Symposium on Central Nervous System Germ Cell Tumors 

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The successful treatment of children with a primary CNS germ cell tumor can be greatly influenced by the neurosurgeon involved in the diagnostic and therapeutic care of these children. Variability in surgical philosophies no doubt exists due to the relatively infrequent incidence of these tumors, a lack of consensus regarding diagnostic and therapeutic approaches, and the advent of recent surgical innovations. Many of these issues were discussed at the Second International Symposium on Central Nervous System Germ Cell Tumors through presented abstracts and invited presentations. The neurosurgical aspects of these proceedings are summarized here in an effort to present the agreed-upon and debated issues that may confront the pediatric neurosurgeon.

Abbreviations used in this paper: AFP = alpha-fetoprotein; β−HCG = beta subunit-human chorionic gonadotropin; COG = Children's Oncology Group; ETV = endoscopic third ventriculostomy; GCT = germ cell tumor; NGGCT = nongerminomatous GCT.

Article Information

Address correspondence to: Mark M. Souweidane, M.D., Department of Neurological Surgery, Weill Medical College of Cornell University, 525 East 69th Street, Box 99, New York, New York 10021. email:

© AANS, except where prohibited by US copyright law.



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    A representative case of endoscopic tumor biopsy of a pineal region tumor. Left: Endoscopic view directed into the third ventricle from a left anterior approach. Choroid plexus is observed at the roof of the third ventricle. The tumor mass is observed situated posterior and inferior to the massa intermedia. Right: The tumor mass is undergoing sampling with cupped forceps.

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    Example of germinoma dissemination noted at the time of endoscopic tumor biopsy. A and B: Sagittal (A) and coronal (B) postcontrast MR imaging scans obtained in an adolescent patient with a pineal region tumor. Endoscopic third ventriculostomy and tumor biopsy were planned for a presumed germinoma. C: Endoscopic view from a right anterior approach with a view into the third ventricle. Ependymal deposits (arrow) believed to be disseminated tumor are situated on the lateral margin of the foramen of Monro.

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    Axial MR imaging scans obtained in a 15-year-old boy who presented with diabetes insipidus, confusion, and panhypopituitarism. Left: Image showing a heterogeneously enhancing suprasellar mass. The initial evaluation revealed serum β-HCG 1262 IU/L and AFP 56 ng/ml, and CSF β-HCG 5419 IU/L and AFP 43 ng/ml, with a normal spinal MR image and negative CSF cytology. Right: Image obtained after treatment with 5 cycles of chemotherapy (COG study ACNS 0122). Although the markers completely normalized, the MR imaging scan revealed a residual enhancing mass. Delayed second-look surgery resulted in a complete resection of a mature teratoma. The patient remains without evidence of disease following chemotherapy and craniospinal irradiation.


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