A 60-year-old female presented to her ophthalmologist with blurred vision in the right eye and unremarkable neurological exam. She was referred for brain imaging, which showed multiple lesions in both cerebral hemispheres. Biopsy of the right occipital lesion was elected, as it enhanced most on MRI.

Multifocal intracranial gangliogliomas are exceedingly rare tumors, especially in adults. These tumors present unique management barriers, as they are multifocal at the time of diagnosis, making resection more technically challenging.

In a review of Mansour et al., the average age at diagnosis was 19.2, and 80% of the cases had at least one lesion in the temporal lobe. Two studies opted for resection of intracranial tumors, whereas the remaining studies performed biopsy with conservative management and serial imaging. A biopsy was performed in all cases. They presented the first case of an intracranial multifocal ganglioglioma in a patient over 40 with lesions in the occipital lobe, corpus callosum and frontal lobe at presentation ¹⁾.

Narayan V, Savardekar AR, Mahadevan A, Arivazhagan A, Appaji L. Unusual Occurrence of Multifocal Desmoplastic Infantile Astrocytoma: A Case Report and Review of the Literature. Pediatr Neurosurg. 2017;52(3):173-180. doi: 10.1159/000455926. Epub 2017 Feb 22. Review. PubMed PMID: 28222441.

3: Wang JL, Hong CS, Otero J, Puduvalli VK, Elder JB. Genetic Characterization of a Multifocal Ganglioglioma Originating Within the Spinal Cord. World Neurosurg. 2016 Dec;96:608.e1-608.e4. doi: 10.1016/j.wneu.2016.09.063. Epub 2016 Sep 23. PubMed PMID: 27671879; PubMed Central PMCID: PMC5550327.

4: Jurkiewicz E, Grajkowska W, Nowak K, Kowalczyk P, Walecka A, Dembowska-Bagińska B. MR imaging, apparent diffusion coefficient and histopathological features of desmoplastic infantile tumors-own experience and review of the literature. Childs Nerv Syst. 2015 Feb;31(2):251-9. doi: 10.1007/s00381-014-2593-2. Epub 2014 Nov 22. Review. PubMed PMID: 25416471.

5: Allinson KS, O'Donovan DG, Jena R, Cross JJ, Santarius TS. Rosette-forming glioneuronal tumor with dissemination throughout the ventricular system: a case report. Clin Neuropathol. 2015 Mar-Apr;34(2):64-9. doi: 10.5414/NP300682. PubMed PMID: 25373141.

6: Gkekas N, Vyziotis A, Dimitriadi A, Koutsonikas G, Georgakoulias N. Multifocal low-grade gliomas: Adapting the optimal therapeutic management. Br J Neurosurg. 2015 Feb;29(1):94-96. doi: 10.3109/02688697.2014.957156. Epub 2014 Sep 12. PubMed PMID: 25215562.

7: Gurses I, Scheithauer BW. Inhibin-A immunoreactivity in nervous system lesions. Appl Immunohistochem Mol Morphol. 2012 May;20(3):277-84. doi: 10.1097/PAI.0b013e31823e5ba8. PubMed PMID: 22505009.

8: Andrychowski J, Taraszewska A, Czernicki Z, Jurkiewicz J, Netczuk T, Dabrowski P. Ten years observation and treatment of multifocal pilocytic astrocytoma. Folia Neuropathol. 2009;47(4):362-70. PubMed PMID: 20054789.

9: Uro-Coste E, Ssi-Yan-Kai G, Guilbeau-Frugier C, Boetto S, Bertozzi AI, Sevely A, Lolmede K, Delisle MB. Desmoplastic infantile astrocytoma with benign histological phenotype and multiple intracranial localizations at presentation. J Neurooncol. 2010 May;98(1):143-9. doi: 10.1007/s11060-009-0075-2. Epub 2009 Dec 11. PubMed PMID: 20012157.

10: Wang Y, Xiong J, Chu SG, Liu Y, Cheng HX, Wang YF, Zhao Y, Mao Y. Rosette-forming glioneuronal tumor: report of an unusual case with intraventricular dissemination. Acta Neuropathol. 2009 Dec;118(6):813-9. doi: 10.1007/s00401-009-0569-1. PubMed PMID: 19585134.

11: D'Incerti L. Morphological neuroimaging of malformations of cortical development. Epileptic Disord. 2003 Sep;5 Suppl 2:S59-66. Review. PubMed PMID: 14617422.

12: Gonçalves-Ferreira AJ, Herculano-Carvalho M, Pimentel J. Stereotactic biopsies of focal brainstem lesions. Surg Neurol. 2003 Oct;60(4):311-20; discussion 320. PubMed PMID: 14505847.

13: Uchida K, Nakayama H, Endo Y, Kai C, Tatewaki S, Yamaguchi R, Doi K, Tateyama S. Ganglioglioma in the thalamus of a puppy. J Vet Med Sci. 2003 Jan;65(1):113-5. PubMed PMID: 12576715.

14: al-Sarraj ST. Multifocal neurocytoma/ganglioglioma. Am J Surg Pathol. 1997 Feb;21(2):258-9. PubMed PMID: 9042299.

15: Prayson RA, Estes ML. Cortical dysplasia: a histopathologic study of 52 cases of partial lobectomy in patients with epilepsy. Hum Pathol. 1995 May;26(5):493-500. PubMed PMID: 7750933.

16: Leung SY, Gwi E, Ng HK, Fung CF, Yam KY. Dysembryoplastic neuroepithelial tumor. A tumor with small neuronal cells resembling oligodendroglioma. Am J Surg Pathol. 1994 Jun;18(6):604-14. PubMed PMID: 8179075.

17: Prayson RA, Estes ML, Morris HH. Coexistence of neoplasia and cortical dysplasia in patients presenting with seizures. Epilepsia. 1993 Jul-Aug;34(4):609-15. PubMed PMID: 8330569.