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NEUROIMAGES
Year : 2019  |  Volume : 67  |  Issue : 2  |  Page : 615-616

Imaging in diffuse leptomeningeal glioneuronal tumor


Department of Neurosurgery and Gamma Knife, All India Institute of Medical Sciences, New Delhi, India

Date of Web Publication13-May-2019

Correspondence Address:
Dr. Amol Raheja
Department of Neurosurgery, Neurosciences Centre, All India Institute of Medical Sciences, New Delhi - 110 029
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.258051

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How to cite this article:
Ghoniya R, Raheja A. Imaging in diffuse leptomeningeal glioneuronal tumor. Neurol India 2019;67:615-6

How to cite this URL:
Ghoniya R, Raheja A. Imaging in diffuse leptomeningeal glioneuronal tumor. Neurol India [serial online] 2019 [cited 2019 May 24];67:615-6. Available from: http://www.neurologyindia.com/text.asp?2019/67/2/615/258051




Diffuse leptomeningeal glioneuronal tumor (DLGNT) was first described as a new entity in 2010;[1] recently, it has been included in the World Health Organization classification of brain tumors, in the 2016 revision.[2] It has been primarily described in the pediatric population.[1],[3],[4] We report imaging findings of this rare condition in a 36-year old female patient, who presented with symptomatic hydrocephalus and a single episode of generalized tonic clonic seizures for which she required a ventriculoperitoneal shunt. She later developed bilateral cerebellar signs and spastic paraparesis with early bladder and bowel involvement, a surrogate clinical presentation for an intramedullary pathology. Imaging of this patient serves as an interesting outlook into this rare entity. Magnetic resonance imaging of brain and spine demonstrated multiple T1 hypointense, T2 hyperintense, fluid attenuated inversion recovery (FLAIR) hypointense, nonenhancing subpial cystic lesions along the cerebellar folia and spinal cord [Figure 1] and [Figure 2], giving a presumptive diagnosis of DLGNT based on its classical radiological signs. The largest lesion can be seen in the subpial location of D9–D10 spinal cord without any leptomeningeal enhancement [Figure 1] and [Figure 2]. The unique aspects of this DLGNT case include an adult-onset presentation, hydrocephalus with seizure as the primary presenting complaint, and lack of leptomeningeal contrast enhancement.
Figure 1: (a) T1W axial brain MRI showing hypointense lesions along cerebellar folia bilaterally (arrow), which are hyperintense on T2W image (arrow) (b). (c) These lesions do not enhance on postcontrast images. (d) They remain hypointense on fluid attenuated inversion recovery (FLAIR) sequence (arrow). (e) A large subpial dorsal cord lesion (horizontal arrow) with smaller cystic lesions located anterolaterally (vertical arrow) are also seen on T2W axial image

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Figure 2: (a) T2W sagittal MRI brain image showing multiple subpial hyperintense lesions along the cerebellar folia (arrow), as well as in the cervicodorsal spinal cord (b). (c) In addition to the multiple subpial lesions (arrow) along the cervicodorsal spinal cord, there is a large subpial (arrowhead) lesion in the lower dorsal cord at the D9–D10 vertebral level. (d) The cystic subpial lesions extend up to the tip of conus (arrow). (e) Postcontrast image of the whole spine showing the nonenhancing nature of the leptomeninges, and the multiple subpial lesions as well as the intramedullary lesion

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  References Top

1.
Gardiman MP, Fassan M, Orvieto E, D'Avella D, DenaroL, Caldero M, et al. Diffuse leptomeningeal glioneuronal tumors: A new entity? Brain Pathol 2010;20:361-6.  Back to cited text no. 1
    
2.
Louis DN, Perry A, Reifenberger G, von Deimling A, Figarella-Branger D, Cavenee WK, et al. The 2016 World Health Organization classification of tumors of the central nervous system: A summary. Acta Neuropathol 2016;131:803-20.  Back to cited text no. 2
    
3.
Rodriguez FJ, Perry A, Rosenblum MK, Krawitz S, Cohen KJ, Lin D, et al. Disseminated oligodendroglial-like leptomeningeal tumor of childhood: A distinctive clinicopathologic entity. Acta Neuropathol 2012;124:627-41.  Back to cited text no. 3
    
4.
Santosh V, Sravya P, Gupta T, Muzumdar D, Chacko G, Suri V, et al. ISNO consensus guidelines for practical adaptation of the WHO 2016 classification of adult diffuse gliomas. Neurol India 2019;67:173-82.  Back to cited text no. 4
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