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Clinical Neuro-Oncology |
Departments of Pediatrics (A.T.R., H.A., K.W.B.), Surgery, Section of Pediatric Neurosurgery (J.C.W., P.A.G.), and Radiation Oncology (J.B.F.), University of Alabama at Birmingham, Birmingham, AL 35233; and Departments of Neurology and Pediatrics, Beth Israel Medical Center (J.C.A.), New York, NY 10128; USA
2 Address correspondence to Alyssa T. Reddy, 1600 7th Avenue South, ACC 512, The Children's Hospital of Alabama, Birmingham, AL 35233, USA (AReddy{at}peds.uab.edu).
Abstract
Treatment strategies for CNS germinoma are currently evolving. Current approaches include reducing the volume and dose of radiation by adding pre-irradiation chemotherapy. Very accurate staging is necessary with such an approach to prevent failures. Eight consecutive patients with pineal germinoma at one institution underwent endoscopic surgery for tumor biopsy, direct visualization of the third ventricular region, and third ventriculostomy for those with hydrocephalus. All patients were treated with 4 cycles of chemotherapy. Conformal field radiation therapy followed, with the dose to the tumor bed dependent on the response to chemotherapy. Patients who had MRI, endoscopic, or cerebrospinal fluid evidence of multicentric or disseminated disease also received craniospinal radiation. Six patients had diabetes insipidus (DI) at presentation. All 6 had tumor studding the floor of the third ventricle on endoscopic visualization, while only 4 of those patients had MRI evidence of disease in that region. All patients have completed therapy and are alive, with no evidence of disease at median follow-up of 31.5 months from diagnosis. Direct endoscopic visualization of the third ventricular region may be more sensitive than MRI for evaluating the presence of suprasellar disease and appears to add important information. This parameter should be added to the staging evaluation when feasible. In this series, the presence of DI was 100% predictive of suprasellar disease, even when the MRI was negative for involvement of that region. Patients should be evaluated for DI as part of the initial staging, and if it is present, the patients should be treated for suprasellar disease regardless of MRI findings.
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