Abstract
Introduction
Diffuse leptomeningeal glioneuronal tumors (DLGT) have been recognized in the most recent WHO classification as a distinct entity.
Objective
We describe seven pediatric cases of DLGT and the responses to therapy and outcome.
Methods
We conducted a retrospective review of charts from 1985 to 2013.
Results
DBS is an effective therapeutic modality for intractable TLE, particularly in patients with lateralized EEG A total of seven patients were identified. Median age at diagnosis was 3 years. Three months was the median time from symptom development to diagnosis. Common MRI findings included diffuse leptomeningeal thickening, nodularity, or coating of the subarachnoid or ependymal surfaces. The leptomeningeal lesions often appear cystic and contrast enhancement was variable. Six patients had leptomeningeal involvement of the brain and spine. All patients had a negative CSF cytology. Biopsies demonstrated thickened meninges infiltrated by a monotonous population of oligodendrocyte-like cells. Immunohistochemistry revealed variable features of neuronal and/or glial differentiation. All samples were negative for BRAF V600E mutation by immunohistochemistry. Therapy included one patient treated with craniospinal irradiation followed by vincristine, etoposide, cyclophosphamide, and cisplatin with stable disease for 164 months. Six patients received carboplatin and vincristine with a median duration of response of 20+ months (15–122+). Three patients received temozolomide upfront and progressed at 3, 4, and 27 months. No patients demonstrated complete or partial responses to any chemotherapy regimens. Progression-free survival ranged from 3 to 164+ months; 4/7 patients remained free of progression. All patients are alive.
Conclusions
DLGT are rare tumors that lack imaging responses; however, there was clinical/ symptom improvement in 100% of the patients. A better understanding of the tumor biology is necessary to improve the diagnosis and treatment of this rare disease.
Similar content being viewed by others
References
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 124(5):627–641. https://doi.org/10.1007/s00401-012-1037-x
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. https://doi.org/10.1097/PAS.0b013e31827bf4cc
Mazloom A, Hodges JC, Teh BS, Chintagumpala M, Paulino AC (2012) Outcome of patients with pilocytic astrocytoma and leptomeningeal dissemination. Int J Radiat Oncol Biol Phys 84(2):350–354. https://doi.org/10.1016/j.ijrobp.2011.12.044
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. https://doi.org/10.1111/j.1750-3639.2009.00285.x
Perilongo G, Gardiman M, Bisaglia L, Rigobello L, Calderone M, Battistella A et al (2002) Spinal low-grade neoplasms with extensive leptomeningeal dissemination in children. Childs Nerv Syst 18(9–10):505–512
Stodberg T, Deniz Y, Esteitie N, Jacobsson B, Mousavi-Jazi M, Dahl H et al (2002) A case of diffuse leptomeningeal oligodendrogliomatosis associated with HHV-6 variant A. Neuropediatrics 33(5):266–270. https://doi.org/10.1055/s-2002-36739
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 131(6):803–820. https://doi.org/10.1007/s00401-016-1545-1
Preuss M, Christiansen H, Merkenschlager A, Hirsch FW, Kiess W, Muller 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. https://doi.org/10.1007/s11060-015-1735-z
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. Case report. J Neurosurg 103(2 Suppl):184–190. https://doi.org/10.3171/ped.2005.103.2.0184
Demir HA, Varan A, Akyuz C, Soylemezoglu F, Cila A, Buyukpamukcu M (2011) Spinal low-grade neoplasm with leptomeningeal dissemination mimicking tuberculous meningitis in a child. Child’s Nervous System: ChNS: Official J Int Soc Pediatr Neurosurg 27(1):187–192. https://doi.org/10.1007/s00381-010-1218-7
Kessler BA, Bookhout C, Jaikumar S, Hipps J, Lee YZ. Disseminated oligodendroglial-like leptomeningeal tumor with anaplastic progression and presumed extraneural disease: case report. Clin Imaging 2014
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
The authors declare that there is no conflict of interest.
Rights and permissions
About this article
Cite this article
Aguilera, D., Castellino, R.C., Janss, A. et al. Clinical responses of patients with diffuse leptomeningeal glioneuronal tumors to chemotherapy. Childs Nerv Syst 34, 329–334 (2018). https://doi.org/10.1007/s00381-017-3584-x
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00381-017-3584-x