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Non-Syndromic Optic Chiasm Mass in an Adult
Gaurav Gupta, M.Sc. Stanford University School of Medicine MGH Neurosurgery Subinternship September 23, 2010
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Case Presentation ID: 42 yo M with optic chiasm mass
HPI: 40 lb weight loss over 6 months; loss of libido, fatigue, polydipsia/polyuria X 4 months; blurred vision X 6 weeks No significant PMH, no medications, FH/SH noncontributory No history of radiation MRI 2005: “partially empty sella”, no other abnormalities Exam: Dense bitemporal hemianopsia
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Work-up and Differential Diagnosis
Primary glial neoplasm Germ cell tumor Primary CNS lymphoma Metastasis of unknown primary Isolated inflammatory process (e.g. neurosarcoidosis)
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NF1: Age at Diagnosis Dermatology. 2008;216(4):347-8.
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J Clin Neurosci. 2007 May;14(5):502-5.
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Germ Cell Tumor J Neurooncol Dec;95(3):
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Surgical Approaches Minimally invasive Open
Extended endoscopic endonasal Endoscopic transventricular Open Pterional Transorbital/subfrontal Subfrontal
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Extended Endoscopic Endonasal
Adv Tech Stand Neurosurg. 2008;33:151-99
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Endoscopic Transventricular
ETV J Neurosurg Aug 13.
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Craniotomy for Optic Chiasm Malignancy
Medlock and Scott. Pediatr Neurosurg Sep;27(3):
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Gross Appearance at Biopsy
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Pathology In initial stain of tissue samples, < 50% of cells took up GFAP, CD45, p53, Ki-67, IDH-1, and CD68 These will be re-stained along with PLAP (placental alkaline phosphatase), Oct4, c-kit/CD117
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Emerging Diagnostic Modalities
PET-CT co-registered with MR spectroscopy Increase in SUV and choline/creatine ratio distinguish high-grade from low-grade optic glioma in NF1 patients (Clin Nucl Med Oct;35(10):838-9.) 3.0T DTI Optic gliomas may be detected by decreases in fractional anisotropy (J Magn Reson Imaging Jul;32(1):76-81.) Dynamic contrast-enhanced (DCE) MRI Clinically aggressive optic gliomas have significantly higher mean permeability values (Pediatr Radiol :1293–1299)
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Questions
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