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Abstract
Primary central nervous system neuroblastomas and ganglioneuroblastomas (CNS-NB and CNS-GNB) are rare and poorly studied malignant tumors in adult patients. As histological verification of the diagnosis is difficult, there is currently a shortage of extensive epidemiological data. Moreover, protocols for adjuvant therapy are largely empirical, resulting in considerable variability of clinical outcome.
The article discusses the problems of diagnosis and treatment of CNS-NB and CNS-GNB in adult patients based on the analysis of the medical literature and our own clinical observations. It describes the epidemiological, clinical, morphological, and molecular genetic characteristics, as well as current approaches to adjuvant therapy of CNS-NB and CNS-GNB in patients over 18 years old. To prepare the review, a literature search was conducted using the Scopus, Web of Science, Medline, PubMed, and RSCI databases. We used 36 sources, 44.5 % of which were published in the last 5 years.
References
Louis DN, Perry A, Wesseling P, et al. The 2021 WHO classification of tumors of the central nervous system: a summary. Neuro Oncol. 2021;23(8):1231–51. doi:10.1093/neuonc/noab106.
Horten BC, Rubinstein LJ. Primary cerebral neuroblastoma. A clinicopathological study of 35 cases. Brain. 1976;99(4):73556. doi:10.1093/brain/99.4.735.
Bennett JP Jr, Rubinstein LJ. The biological behavior of primary cerebral neuroblastoma: a reappraisal of the clinical course in a series of 70 cases. Ann Neurol. 1984;16(1):217. doi:10.1002/ana.410160106.
Louis DN, Perry A, Reifenberger G, et al. The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary. Acta Neuropathol. 2016;131(6):80320. doi:10.1007/s00401-016-1545-1.
Louis DN, Wesseling P, Aldape K, et al. cIMPACT-NOW update 6: new entity and diagnostic principle recommendations of the cIMPACT-Utrecht meeting on future CNS tumor classification and grading. Brain Pathol. 2020;30(4):844856. doi:10.1111/bpa.12832.
National Comprehensive Cancer Network. Central Nervous System Cancers (Version 1.2022) [Internet]. Available online: https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1425.
Ostrom QT, Patil N, Cioffi G, et al. CBTRUS statistical report: Primary brain and other central nervous system tumors diagnosed in the United States in 2013-2017. Neuro Oncol. 2020;22(12 Suppl 1):iv1iv96. doi:10.1093/neuonc/noaa200.
Lu X, Zhang X, Deng X, et al. Incidence, treatment, and survival in primary central nervous system neuroblastoma. World Neurosurg. 2020;140:e61–72. doi:10.1016/j.wneu.2020.04.145.
Louis DN, Ohgaki H, Wiestler OD, et al. The 2007 WHO classification of tumours of the central nervous system. Acta Neuropathol. 2007;114(2):97109. doi:10.1007/s00401-007-0243-4.
Schwalbe EC, Hayden JT, Rogers HA, et al. Histologically defined central nervous system primitive neuro-ectodermal tumours (CNS-PNETs) display heterogeneous DNA methylation profiles and show relationships to other paediatric brain tumour types. Acta Neuropathol. 2013;126(6):9436. doi:10.1007/s00401-013-1206-6.
Łastowska M, Trubicka J, Sobocińska A, et al. Molecular identification of CNS NB-FOXR2, CNS EFT-CIC, CNS HGNET-MN1 and CNS HGNET-BCOR pediatric brain tumors using tumor-specific signature genes. Acta Neuropathol Commun. 2020;8(1):105. doi:10.1186/s40478-020-00984-9.
Sturm D, Orr BA, Toprak UH, et al. New Brain Tumor Entities Emerge from Molecular Classification of CNS-PNETs. Cell. 2016;164(5):10601072. doi:10.1016/j.cell.2016.01.015.
Blessing MM, Alexandrescu S. Embryonal Tumors of the Central Nervous System: An Update. Surg Pathol Clin. 2020;13(2):235247. doi:10.1016/j.path.2020.01.003.
Davis PC, Wichman RD, Takei Y, et al. Primary cerebral neuroblastoma: CT and MR findings in 12 cases. AJR Am J Roentgenol. 1990;154(4):8316. doi:10.2214/ajr.154.4.2107684.
Akın M, Ergen SA, Oz B, et al. Ventricular ganglioneuroblastoma in an adult and successful treatment with radiotherapy. Balkan Med J. 2014;31(2):1736. doi:10.5152/balkanmedj.2014.13155.
Black-Tiong SP, Sandler SJ, Otto S, et al. Adult supratentorial primitive neuroectodermal tumour presenting as intracranial haemorrhage: Case report. J Clin Neurosci. 2017;37:4142. doi:10.1016/j.jocn.2016.10.043.
Nishihara H, Ozaki Y, Ito T, et al. A case of cerebral ganglioneuronal tumor in the parietal lobe of an adult. Brain Tumor Pathol. 2008;25(1):459. doi:10.1007/s10014-008-0229-5.
Schipper MH, van Duinen SG, Taphoorn MJ, et al. Cerebral ganglioneuroblastoma of adult onset: Two patients and a review of the literature. Clin Neurol Neurosurg. 2012;114(6):529–34. doi:10.1016/j.clineuro.2012.03.015.
Мацко М.В., Мацко Д.Е., Имянитов Е.Н. и др. Эмбриональные опухоли центральной нервной системы у взрослых. Три наблюдения из практики. Обзор литературы. Сибирский онкологический журнал. 2021;20(1):105114. doi:10.21294/1814-4861-2021-20-1-105-114 [Matsko MV, Matsko DE, Imyanitov EN, et al. Embryonal tumors of the central nervous system in adults: a report of three cases. Review of the literature. Siberian Journal of Oncology. 2021;20(1):105–14. doi:10.21294/1814-4861-2021-20-1-105-114 (In Russ.)].
Ren AJ, Ning HY, Lin E. Serial diffusion-weighted and conventional mr imaging in primary cerebral neuroblastoma treated with radiotherapy and chemotherapy. A case report and literature review. Neuroradiol J. 2014;27(4):41721. doi:10.15274/NRJ-2014-10059.
Holsten T, Lubieniecki F, Spohn M, et al. Detailed Clinical and Histopathological Description of 8 Cases of Molecularly Defined CNS Neuroblastomas. J Neuropathol Exp Neurol. 2021;80(1):5259. doi:10.1093/jnen/nlaa128.
Gessi M, Setty P, Bisceglia M, et al. Supratentorial primitive neuroectodermal tumors of the central nervous system in adults: molecular and histopathologic analysis of 12 cases. Am J Surg Pathol. 2011;35(4):57382. doi:10.1097/PAS.0b013e31820f1ce0.
Korshunov A, Okonechnikov K, Schmitt-Hoffner F, et al. Molecular analysis of pediatric CNS-PNET revealed nosologic heterogeneity and potent diagnostic markers for CNS neuroblastoma with FOXR2-activation. Acta Neuropathol Commun. 2021;9(1):20. doi:10.1186/s40478-021-01118-5.
Furuta T, Moritsubo M, Muta H, et al. Central nervous system neuroblastic tumor with FOXR2 activation presenting both neuronal and glial differentiation: a case report. Brain Tumor Pathol. 2020;37(3):100104. doi:10.1007/s10014-020-00370-2.
Nielsen LAG, Bangsø JA, Lindahl KH, et al. Evaluation of the proliferation marker Ki-67 in gliomas: Interobserver variability and digital quantification. Diagn Pathol. 2018;13(1):38. doi:10.1186/s13000-018-0711-2.
Poh B, Koso H, Momota H, et al. Foxr2 promotes formation of CNS-embryonal tumors in a Trp53-deficient background. Neuro Oncol. 2019;21(8):993–1004. doi:10.1093/neuonc/noz067.
Hwang EI, Kool M, Burger PC, et al. Extensive molecular and clinical heterogeneity in patients with histologically diagnosed cns-pnet treated as a single entity: A report from the children's oncology group randomized ACNS0332 trial. J Clin Oncol. 2018;36(34):JCO2017764720. doi:10.1200/JCO.2017.76.4720.
Weller M, van den Bent M, Preusser M, et al. EANO guidelines on the diagnosis and treatment of diffuse gliomas of adulthood. Nat Rev Clin Oncol. 2021;18(3):170186. doi:10.1038/s41571-020-00447-z.
Korshunov A, Capper D, Reuss D, et al. Histologically distinct neuroepithelial tumors with histone 3 G34 mutation are molecularly similar and comprise a single nosologic entity. Acta Neuropathol. 2016;131(1):13746. doi:10.1007/s00401-015-1493-1.
Wang L, Liang B, Li YI, et al. What is the advance of extent of resection in glioblastoma surgical treatment-a systematic review. Chin Neurosurg J. 2019;5:2. doi:10.1186/s41016-018-0150-7.
De Braganca KC, Packer RJ. Treatment options for medulloblastoma and CNS primitive neuroectodermal tumor (PNET). Curr Treat Options Neurol. 2013;15(5):593606. doi:10.1007/s11940-013-0255-4.
Yao PS, Chen GR, Shang-Guan HC, et al. Adult hippocampal ganglioneuroblastoma: Case report and literature review. Medicine (Baltimore). 2017;96(51):e8894. doi:10.1097/MD.0000000000008894.
Nakazato Y, Hosaka N. A 32-year-old man with left temporal lobe tumor. Neuropathology. 2004;24(3):2612. doi:10.1111/j.1440-1789.2004.00556.x.
Sabatino G, Lauriola L, Sioletic S, et al. Occipital ganglio-neuroblastoma in an adult. Acta Neurochir (Wien). 2009;151(5):4956. doi:10.1007/s00701-009-0227-3.
Hosaka T, Nukui H, Koizumi H et al. Ganglioneuroblastoma of the anterior skull base with a long-term follow-up. Case Report. Neurol Med Chir (Tokyo). 1982;22(9):757–62. doi:10.2176/nmc.22.757.
Tanaka M, Shibui S, Nomura K et al. Pineal ganglioneuroblastoma in an adult. J Neurooncol. 1999;44:169–73. doi:10.1023/a:1006308205805.
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