Non-Syndromic Non-Syndromic Optic Chiasm Mass Optic Chiasm Mass

in an Adultin an AdultGaurav Gupta, M.Sc.Gaurav Gupta, M.Sc.

Stanford University School of Stanford University School of MedicineMedicine

MGH Neurosurgery MGH Neurosurgery SubinternshipSubinternship

September 23, 2010September 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 Extended endoscopic endonasal Endoscopic transventricular

Open Pterional Transorbital/subfrontal Subfrontal

Extended Endoscopic Endonasal

Adv Tech Stand Neurosurg. 2008;33:151-99

Endoscopic Transventricular

J Neurosurg. 2010 Aug 13.

ETV

Medlock and Scott. Pediatr Neurosurg. 1997 Sep;27(3):129-36.

Craniotomy for Optic Chiasm Malignancy

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