Abstract
Diffuse leptomeningeal glioneuronal tumor (DLGNT) is a rare glioneuronal neoplasm with oligodendroglioma-like cells confined in the subarachnoid spaces. A great majority of DLGNT are histologically low grade. However, some tumors show features of anaplasia with increased mitotic and proliferative activity. Due to the limited number of patients and inadequate clinical follow-up reported to date, the WHO classification does not yet assign a distinct WHO grade to this entity. Polar spongioblastoma pattern, in which bipolar cells are arranged in parallel with palisading nuclei, remains poorly understood about the pathological process of forming this pattern. We experienced a case of 22-year-old man developing DLGNT with extensively distributed anaplastic changes involving polar spongioblastoma pattern and the secondary tumor invasion to brain parenchyma in 4½ years before the autopsy. Clinical and pathological courses of the patient are presented with radiological, histopathological, and genetic examinations. This is the first report demonstrating the immunohistological and genetic evaluation of a DLGNT with polar spongioblastoma pattern.
Similar content being viewed by others
References
Agamanolis DP, Katsetos CD, Klonk CJ et al (2012) An unusual form of superficially disseminated glioma in children: report of 3 cases. J Child Neurol 27:727–733
Aydemir F, Kardes O, Kayaselcuk F et al (2016) Massive calcified cerebellar pilocytic astrocytoma with rapid recurrence: a rare case. J Korean Neurosurg Soc 59:533–536
Bourne TD, Mandell JW, Matsumoto JA et al (2006) Primary disseminated leptomeningeal oligodendroglioma with 1p deletion. Case report. J Neurosurg 105:465–469
Cho HJ, Myung JK, Kim H et al (2015) Primary diffuse leptomeningeal glioneuronal tumors. Brain Tumor Pathol 32:49–55
Choi BH (1994) Role of the basement membrane in neurogenesis and repair of injury in the central nervous system. Microsc Res Tech 28:193–203
Cooper IS, Kernohan JW (1951) Heterotopic glial nests in the subarachnoid space; histopathologic characteristics, mode of origin and relation to meningeal gliomas. J Neuropathol Exp Neurol 10:16–29
Dahlrot RH, Hansen S, Jensen SS et al (2014) Clinical value of CD133 and nestin in patients with glioma: a population-based study. Int J Clin Exp Pathol 7:3739–3751
Deng MY, Sill M, Chiang J et al (2018) Molecularly defined diffuse leptomeningeal glioneuronal tumor (DLGNT) comprises two subgroups with distinct clinical and genetic features. Acta Neuropathol. https://doi.org/10.1007/s00401-018-1865-4
Fukushima S, Narita Y, Shinomiya A et al (2012) A case of unclassified high-grade glioma with polar spongioblastoma pattern. Neuropathology 32:604–610
Gardiman MP, Fassan M, Orvieto E et al (2010) Diffuse leptomeningeal glioneuronal tumors: a new entity? Brain Pathol 20:361–366
Huang Q, Zhang QB, Dong J et al (2008) Glioma stem cells are more aggressive in recurrent tumors with malignant progression than in the primary tumor, and both can be maintained long-term in vitro. BMC Cancer 8:304
Kakita A, Wakabayashi K, Su M et al (2001) Experimentally induced leptomeningeal glioneuronal heterotopia and underlying cortical dysplasia of the lateral limbic area in rats treated transplacentally with methylmercury. J Neuropathol Exp Neurol 60:768–777
Kessler BA, Bookhout C, Jaikumar S et al (2015) Disseminated oligodendroglial-like leptomeningeal tumor with anaplastic progression and presumed extraneural disease: case report. Clin Imaging 39:300–304
Kros JM, van der Weiden M, Zheng PP et al (2007) Intratumoral distribution of 1p loss in oligodendroglial tumors. J Neuropathol Exp Neurol 66:1118–1123
Langford LA, Camel MH (1987) Palisading pattern in cerebral neuroblastoma mimicking the primitive polar spongioblastoma. An ultrastructural study. Acta Neuropathol 73:153–159
Louis DN, Ohgaki H, Wiestler OD et al (2007) The 2007 WHO classification of tumours of the central nervous system. Acta Neuropathol 114:97–109
Louis DN, Perry A, Reifenberger G et al (2016) The 2016 World Health Organization classification of tumors of the central nervous system: a summary. Acta Neuropathol 131:803–820
Mekki-Dauriac S, Agius E, Kan P et al (2002) Bone morphogenetic proteins negatively control oligodendrocyte precursor specification in the chick spinal cord. Development 129:5117–5130
Nagaishi M, Tanaka Y, Iwatate K et al (2010) Polar spongioblastoma: a high-grade glioma that does not contain the IDH1 mutation or 1p/19q LOH. Neuropathology 30:547–552
Ozkul A, Meteoglu I, Tataroglu C et al (2007) Primary diffuse leptomeningeal oligodendrogliomatosis causing sudden death. J Neurooncol 81:75–79
Preuss M, Christiansen H, Merkenschlager A et al (2015) Disseminated oligodendroglial-like leptomeningeal tumors: preliminary diagnostic and therapeutic results for a novel tumor entity [corrected]. J Neurooncol 124:65–74
Rodriguez FJ, Perry A, Rosenblum MK et al (2012) Disseminated oligodendroglial-like leptomeningeal tumor of childhood: a distinctive clinicopathologic entity. Acta Neuropathol 124:627–641
Rodriguez FJ, Schniederjan MJ, Nicolaides T et al (2015) High rate of concurrent BRAF-KIAA1549 gene fusion and 1p deletion in disseminated oligodendroglioma-like leptomeningeal neoplasms (DOLN). Acta Neuropathol 129:609–610
Russell D, Cairns H (1947) Polar spongioblastoma. Arch Hist Norm Pathol 3:423–441
Schiffer D, Cravioto H, Giordana MT et al (1993) Is polar spongioblastoma a tumor entity? J Neurosurg 78:587–591
Schniederjan MJ, Alghamdi S, Castellano-Sanchez A et al (2013) Diffuse leptomeningeal neuroepithelial tumor: 9 pediatric cases with chromosome 1p/19q deletion status and IDH1 (R132H) immunohistochemistry. Am J Surg Pathol 37:763–771
Shibahara I, Sonoda Y, Shoji T et al (2015) Malignant clinical features of anaplastic gliomas without IDH mutation. Neuro Oncol 17:136–144
Steinberg GK, Shuer LM, Conley FK et al (1985) Evolution and outcome in malignant astroglial neoplasms of the cerebellum. J Neurosurg 62:9–17
Acknowledgements
The authors thank Drs Hideaki Yokoo and Junko Hirato (Japan Brain Tumor Reference Center) for their analysis of KIAA1549-BRAF fusion by FISH.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
The authors report no conflict of interest concerning the findings specified in this paper.
Rights and permissions
About this article
Cite this article
Yamasaki, T., Sakai, N., Shinmura, K. et al. Anaplastic changes of diffuse leptomeningeal glioneuronal tumor with polar spongioblastoma pattern. Brain Tumor Pathol 35, 209–216 (2018). https://doi.org/10.1007/s10014-018-0326-z
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10014-018-0326-z