| Identifier |
walsh_2016_s4_c2 |
| Title |
Growing Up Too Fast - Abstract |
| Creator |
Courtney E. Francis; Thomas T. Chia; Gordana Juric-Sekhar; Manuel Ferreira |
| Affiliation |
(CEF) (TTC) University of Washington/Ophthalmology Seattle, WA; (GJ) University of Washington/Pathology Seattle, WA; (MF) University of Washington/Neurosurgery Seattle, WA |
| Subject |
Ganglioglioma; Optic Pathway Glioma; Optic Tract; Precocious Puberty |
| Description |
The patient underwent craniotomy with cyst fenestration and partial resection of the tumor including biopsy. The pathology showed a ganglioglioma (WHO Grade I), with an infiltrative cellular neoplasm composed of glial cells containing irregular and hyperchromatic nuclei, along with dysplastic ganglion cells and neurons. Frequent eosinophilic granular bodies were present. Definite Rosenthal fibers, mitotic figures, necrosis and microvascular proliferation were not present. Immunohistochemistry was done and was positive for MAP-2 (microtubule associated protein-2), mGFAP (Glial Fibrillary Acidic Protein), Neurofilament 2F11. Ki-67 stained only 2% of cells. There was some focal reactivity for CD34. OncoPlex Single Gene analysis demonstrated positivity for BRAF p. V600E mutation. An outside institutional review of the slides agreed with the diagnosis. Following surgery, he had resolution of his right sided weakness. His right homonymous hemianopia remained unchanged. Treatment options of BRAF inhibitor versus radiation therapy were presented. He had no findings concerning for neurofibromatosis type 1. |
| History |
A 7 ½ year old boy presented in 2000 with precocious puberty (development of pubic hair, acne and rapid linear growth). Work-up included a brain MRI revealing a suprasellar mass, consistent with a visual pathway glioma. On ophthalmologic evaluation, he was noted to be 20/25 OU with a normal funduscopic exam. He was started on leuprorelin with improvement in his symptoms and followed with serial MRIs, showing slow increase in the size of the tumor. Two years after diagnosis, he was offered radiation therapy, but the family deferred treatment. Serial visual field testing showed a stable temporal defect in the right eye and mild nasal depression in the left eye, consistent with left optic tract involvement. He was lost to follow-up from August 2008 until June 2015 when he presented to the ER with 2 months of progressive right upper and lower extremity weakness. He denied any recent changes in his vision. Eye exam showed 20/20 acuity in both eyes, normal color vision, 2+ temporal pallor in both eyes and an incomplete right homonymous hemianopia on visual field testing. Repeat MRI showed a partially cystic and partially nodular enhancing mass, centered in the region of the left hypothalamus versus left optic tract, with associated infiltration of adjacent structures including the left thalamus and inferior left basal ganglia. A diagnostic procedure was performed. |
| Disease/Diagnosis |
Chiasmal/hypothalamic ganglioglioma. |
| Date |
2016-02 |
| References |
1. Rolston JD, Han SJ, Cotter JA, El-Sayed IH, Aghi MK. Gangliogliomas of the optic pathway. Journal of Clinical Neuroscience, 21, 2244-2249, 2014. 2. Liu GT, Galetta SL, Rorke LB, Bilaniuk LT, Vojta DD, et al. Gangliogliomas involving the optic chiasm. Neurology, 46, 1669-1673, 1996. 3. Shuangshoti S, Kirsch E, Bannan P, Fabian VA. Ganglioglioma of the optic chiasm: case report and review of the literature. American Journal of Neuroradiology, 21, 1486-1489, 2000. 4. Lang FF, Epstein FJ, Ransohoff J, Allen JC, Wisoff J, et al. Central nervous system gangliogliomas. Part 2: Clinical outcome. J Neurosurg, 79, 867-873, 1993. 5. Compton JJ, Issa Laack NN, Eckel LJ, Schomas DA, Giannini C, et al. Long-term outcomes for low-grade intracranial ganglioglioma: 30-year experience from the Mayo Clinic. J Neurosurg, 117, 825-830, 2012. |
| Language |
eng |
| Format |
application/pdf |
| Type |
Text |
| Source |
48th Annual Frank Walsh Society Meeting |
| Relation is Part of |
NANOS Annual Meeting 2016 |
| Collection |
Neuro-Ophthalmology Virtual Education Library: Walsh Session Annual Meeting Archives: https://novel.utah.edu/Walsh/ |
| Publisher |
North American Neuro-Ophthalmology Society |
| Holding Institution |
Spencer S. Eccles Health Sciences Library, University of Utah |
| Rights Management |
Copyright 2016. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
| ARK |
ark:/87278/s6x957wf |
| Setname |
ehsl_novel_fbw |
| ID |
179322 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s6x957wf |
