Abstract
Introduction
Diffuse leptomeningeal glioneuronal tumor is a new entity under the neuronal and mixed neuronal-glial tumors in the WHO 2016 updated classification and commonly found in children and adolescents. The initial diagnosis is challenging because of its non-specific radiologic feature and negative CSF cytology analysis.
Case summary
A 17 years male was presented with intractable headache subsequently followed by back pain and joint pain. MRI showed enhancement of arachnoid membrane at basal cistern, bilateral sylvian fissure and cerebral cistern with slight enlargement of ventricles. There were no evidences of infection in CSF and blood samples. Based on the duodenal biopsy and prodromal symptom of joint pain, the patient was suspected of having Whipple’s disease. Eleven months after the onset, a small mass lesion was observed at the anterior horn of right lateral ventricle. The histology was remarkable for anaplastic oligodendroglioma. Immunostainings revealed positivity for GFAP, Olig2, synaptophysin and negativity for IDH1 mutation, H3K27M. MIB1 labeling index was 40% and 1p19q FISH analysis showed only 1p deletion. Therefore, a final diagnosis of DLGNT was made.
Conclusion
DLGNT should be included as a differential diagnosis of patients with leptomeningeal-enhanced and high CSF protein level with normal white blood cell count.
Similar content being viewed by others
References
Louis DN, Perry A, Reifenberger G, von Deimling A, Figarella-Branger D, Cavenee WK et al (2016) The 2016 World Health Organization classification of tumors of the central nervous system: a summary. Acta Neuropathol (Berl). 131(6):803–820
Louis DN, Ohgaki H, Wiestler O, Cavenee WK, Ellison DW, Figarella-Branger D et al (2016) WHO classification of tumours of the central nervous system. Revised 4th ed. International Agency for Research on Cancer (IARC), Lyon
Rodriguez FJ, Perry A, Rosenblum MK, Krawitz S, Cohen KJ, Lin D et al (2012) Disseminated oligodendroglial-like leptomeningeal tumor of childhood: a distinctive clinicopathologic entity. Acta Neuropathol (Berl). 124(5):627–641
Polmeteer FE, Kernohan JW (1947) Meningeal gliomatosis: a study of forty-two cases. Arch Neurol Psychiatr 57(5):593–616
Reynolds RM, Boswell E, Hulette CM, Cummings TJ, Haglund MM, Boswell E et al (2011) Sudden death from diffuse leptomeningeal oligodendrogliomatosis. J Neurosurg Spine. 15(6):625–629
Ko MW, Turkeltaub PE, Lee EB, Gonatas NK, Volpe NJ, Moster ML et al (2009) Primary diffuse leptomeningeal gliomatosis mimicking a chronic inflammatory meningitis. J Neurol Sci 278(1–2):127–131
Armao DM, Stone J, Castillo M, Mitchell KM, Bouldin TW, Suzuki K (2000) Diffuse leptomeningeal oligodendrogliomatosis: radiologic/pathologic correlation. Am J Neuroradiol 21(6):1122–1126
Mathews MS, Paré LS, Kuo JV, Kim RC (2009) Primary leptomeningeal oligodendrogliomatosis. J Neuro-Oncol 94(2):275–278
Leep Hunderfund AN, Zabad RK, Aksamit AJ, Morris JM, Meyer FB, Thorell WE et al (2013) Diffuse anaplastic leptomeningeal oligodendrogliomatosis mimicking neurosarcoidosis. Neurol Clin Pract 3(3):261–265
Hervey-Jumper SL, Jumper M, Blaivas M, Parmar HA, Robertson PL, Maher CO (2010) Primary diffuse leptomeningeal oligodendroglioma. Pediatr Neurosurg 46(4):326–328
Bourne TD, Mandell JW, Matsumoto JA, Jane JA, Lopes MBS (2006) Primary disseminated leptomeningeal oligodendroglioma with 1p deletion. J Neurosurg Pediatr. 105(6):465–469
Lyle MR, Dolia JN, Fratkin J, Nichols TA, Herrington BL (2015) Newly identified characteristics and suggestions for diagnosis and treatment of diffuse leptomeningeal glioneuronal/neuroepithelial tumors a case report and review of the literature. Child Neurol Open 2(1):2329048X14567531
Ozkul A, Meteoglu I, Tataroglu C, Akyol A (2006) Primary diffuse leptomeningeal oligodendrogliomatosis causing sudden death. J Neuro-Oncol 81(1):75–79
Schniederjan MJ, Alghamdi S, Castellano-Sanchez A, Mazewski C, Brahma B, Brat DJ 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(5):763–771
Gilmer-Hill HS, Ellis WG, Imbesi SG, Boggan JE (2000) Spinal oligodendroglioma with gliomatosis in a child. Case report J Neurosurg 92(1 Suppl):109–113
Mittelbronn M, Wolff M, Bültmann E, Nägele T, Capper D, Beck R et al (2005) Disseminating anaplastic brainstem oligodendroglioma associated with allelic loss in the tumor suppressor candidate region D19S246 of chromosome 19 mimicking an inflammatory central nervous system disease in a 9-year-old boy. Hum Pathol 36(7):854–857
Rossi S, Rodriguez FJ, Mota RA, Tos APD, Paola FD, Bendini M et al (2009) Primary leptomeningeal oligodendroglioma with documented progression to anaplasia and t(1;19)(q10;p10) in a child. Acta Neuropathol (Berl). 118(4):575–577
Guppy KH, Akins PT, Moes GS, Prados MD (2009) Spinal cord oligodendroglioma with 1p and 19q deletions presenting with cerebral oligodendrogliomatosis. J Neurosurg Spine 10(6):557–563
Kessler BA, Bookhout C, Jaikumar S, Hipps J, Lee YZ (2015) Disseminated oligodendroglial-like leptomeningeal tumor with anaplastic progression and presumed extraneural disease: case report. Clin Imaging 39(2):300–304
Preuss M, Christiansen H, Merkenschlager A, Hirsch FW, Kiess W, Müller W et al (2015) Disseminated oligodendroglial cell-like leptomeningeal tumors: preliminary diagnostic and therapeutic results for a novel tumor entity. J Neuro-Oncol 124(1):65–74
Gardiman MP, Fassan M, Orvieto E, D’Avella D, Denaro L, Calderone M et al (2010) Diffuse leptomeningeal glioneuronal tumors: a new entity? Brain Pathol 20(2):361–366
Cho HJ, Myung JK, Kim H, Park C-K, Kim S-K, Chung CK et al (2015) Primary diffuse leptomeningeal glioneuronal tumors. Brain Tumor Pathol 32(1):49–55
Stark AM, Hugo HH, Mehdorn HM, Knerlich-Lukoschus F et al (2009) Acute hydrocephalus due to secondary Leptomeningeal dissemination of an anaplastic oligodendroglioma. Case Rep Med 2009:e370901
Gajjar A, Bhargava R, Jenkins JJ, Heideman R, Sanford RA, Langston JW et al (1995) Low-grade astrocytoma with neuraxis dissemination at diagnosis. J Neurosurg 83(1):67–71
Rodriguez FJ, Schniederjan MJ, Nicolaides T, Tihan T, Burger PC, Perry A (2015) High rate of concurrent BRAF-KIAA1549 gene fusion and 1p deletion in disseminated oligodendroglioma-like leptomeningeal neoplasms (DOLN). Acta Neuropathol (Berl) 129(4):609–610
Psarros TG, Swift D, Mulne AF, Burns DK (2005) Neurocytoma-like neoplasm of the thoracic spine in a 15-month-old child presenting with diffuse leptomeningeal dissemination and communicating hydrocephalus. J Neurosurg Pediatr 103(2):184–190
Yamamoto T, Komori T, Shibata N, Toyoda C, Kobayashi M (1996) Multifocal neurocytoma/gangliocytoma with extensive leptomeningeal dissemination in the brain and spinal cord. Am J Surg Pathol 20(3):363–370
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
All authors, none.
Rights and permissions
About this article
Cite this article
Karlowee, V., Kolakshyapati, M., Amatya, V.J. et al. Diffuse leptomeningeal glioneuronal tumor (DLGNT) mimicking Whipple’s disease: a case report and literature review. Childs Nerv Syst 33, 1411–1414 (2017). https://doi.org/10.1007/s00381-017-3405-2
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00381-017-3405-2