Non-Syndromic Optic Chiasm Mass in an Adult Gaurav Gupta, M.Sc. Stanford University School of Medicine MGH Neurosurgery Subinternship September 23, 2010
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
Work-up and Differential Diagnosis Primary glial neoplasm Germ cell tumor Primary CNS lymphoma Metastasis of unknown primary Isolated inflammatory process (e.g. neurosarcoidosis)
NF1: Age at Diagnosis Dermatology. 2008;216(4):347-8.
J Clin Neurosci. 2007 May;14(5):502-5.
Germ Cell Tumor J Neurooncol. 2009 Dec;95(3):437-43.
Surgical Approaches Minimally invasive Open Extended endoscopic endonasal Endoscopic transventricular Open Pterional Transorbital/subfrontal Subfrontal
Extended Endoscopic Endonasal Adv Tech Stand Neurosurg. 2008;33:151-99
Endoscopic Transventricular ETV J Neurosurg. 2010 Aug 13.
Craniotomy for Optic Chiasm Malignancy Medlock and Scott. Pediatr Neurosurg. 1997 Sep;27(3):129-36.
Gross Appearance at Biopsy
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
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. 2010 Oct;35(10):838-9.) 3.0T DTI Optic gliomas may be detected by decreases in fractional anisotropy (J Magn Reson Imaging. 2010 Jul;32(1):76-81.) Dynamic contrast-enhanced (DCE) MRI Clinically aggressive optic gliomas have significantly higher mean permeability values (Pediatr Radiol. 2008 38:1293–1299)
Questions