Central neurocytoma
![Axial T1 fat saturation pre-contrast showed intraventricular tumour mass with (subacute haemorrhage) high signal intensity in](/sites/default/files/styles/figure_image_teaser_large/public/figure_image/2024-05//18602_1_1.png?itok=tQJmMD0O)
![Axial T1 fat saturation post-contrast showed mild heterogeneous enhancement with (subacute haemorrhage) high signal intensity](/sites/default/files/styles/figure_image_teaser_large/public/figure_image/2024-05//18602_1_2.png?itok=OZgLjXXO)
![Axial T2 weighted image showed intraventricular tumour mass containing central high signal intensity with a blood product in](/sites/default/files/styles/figure_image_teaser_large/public/figure_image/2024-05//18602_1_3.png?itok=HLAcPFI-)
![Axial T T2 FFE weighted image showed intraventricular tumour mass containing blood product signal intensity.](/sites/default/files/styles/figure_image_teaser_large/public/figure_image/2024-05//18602_1_4.png?itok=qt2BX5M5)
Neuroradiology
Case TypeClinical Case
Authors
Ratna Sutanto 1,2, Rusli Muljadi 1,2, Koesbandono 1,2, Jessica Christanti 2,3
Patient31 years, female
A 31-year-old female with vertigo underwent a brain magnetic resonance imaging (MRI) scan.
Brain MRI with contrast showed intraventricular tumour mass mainly in the midline and body of the right lateral ventricle containing subacute haemorrhage, and mild heterogeneous enhancement in post-contrast, accompanied by a dilatation of the right lateral ventricle (Figures 1a, 1b, 1c and 1d). The apparent diffusion coefficient (ADC) / diffusion-weighted image (DWI) (Figures 2a and 2b) showed no fluid restriction area of ischaemic lesions, and axial FLAIR (Figure 2c) showed no deep white matter ischaemic foci.
After admission for craniotomy and biopsy, then brain computed tomography (CT) without contrast (Figures 3a, 3b and 3c) was done and showed heterogeneous intraventricular lesions, mainly in the body of the right lateral ventricle with dilatation and intraventricular haemorrhage. Right parietal VP shunt with a tip at the third ventricle. Pneumocephalic inside the anterior horn and inferior horn of the right lateral ventricle. Post-operative results cause intraventricular haemorrhage and pneumocephalus.
Histopathology reveals central neurocytoma (CNS WHO grade 2).
Central neurocytoma (CN) is a rare tumour that often occurs in young adults and is often found in the lateral ventricles [1]. The cellular and molecular origin of central neurocytoma is not fully known. CN is likely to originate from neural stem cells in the subventricular zone [2].
CN has various signs and symptoms. Most of the signs and symptoms experienced by CN patients are headaches, found in about 58.8% of cases, followed by paresis, which is as much as 35.3%. The heaviest symptoms of CN patients are seizures and paralysis. On average, the age that often shows symptoms is under 30 years old. Therefore, if there are similar signs and symptoms of intracranial tumours, it is essential to perform diagnostic imaging to find the cause of the tumour so that it can be treated as early as possible [3].
Diagnosis of CN is multimodal and multidisciplinary, from clinical imaging to histopathological aspects. Radiological examinations used to diagnose CN are MRI and CT scans. These examinations can help visualise the tumour's location, size, and characteristics. CN often occurs in the lateral ventricle close to the interventricular foramen. We found subacute intratumoural haemorrhage in our case. It is scarce for CN presentation with tumoural haemorrhaging, and very few cases have been documented. Histopathological examination is the definitive examination of CN. A biopsy or surgical resection is performed to remove the tumour tissue and analysed by a pathologist. Histopathological characteristics of CN include uniform round cells with small nuclei, perivascular pseudorosettes, and immunohistochemical markers such as synaptophysin and NeuN [1].
Differential diagnoses of central neurocytoma, such as ependymoma subependymoma, must be considered, as these other tumours have similar clinical and radiological characteristics. Ependymoma can also arise in the lateral ventricles, with radiological features similar to CN. However, histopathologically, ependymoma and CN have different histological characteristics and immunohistochemical profiles from CN. Subependymoma is a tumour that originates from the subependymal cells lining the ventricles. Subependymoma can also arise in the lateral ventricle and has radiological features similar to CN's. Histopathologically, subependymoma and CN have different histological characteristics and immunohistochemical profiles from CN [1].
The first-line treatment of CN is surgical resection, which removes as many tumours as possible. Therapeutic options after resection, such as radiotherapy, are still controversial. The decision to perform post-operative radiotherapy is tailored to the tumour progression of the patient, especially in patients with multiple recurrences and residual disease [4]. The complication of CN therapy is hydrocephalus, so to avoid this complication, ventriculoperitoneal shunts (VP shunts) are used as a treatment option if dehydration therapy is unsuccessful. The survival rate of patients with CN is approximately 100% [4].
[1] Alsadiq MN, Al Sadah ZM, Butt S, Aldahmen AA (2022) Central Neurocytoma with Hemorrhagic Presentation Case Report and Review of the Literature. Case Rep Surg 2022:9731987. doi: 10.1155/2022/9731987. (PMID: 35310930)
[2] Kim H, Lee K, Shim YM, Kim EE, Kim SK, Phi JH, Park CK, Choi SH, Park SH (2023) Epigenetic Alteration of H3K27me3 as a Possible Oncogenic Mechanism of Central Neurocytoma. Lab Invest 103(8):100159. doi: 10.1016/j.labinv.2023.100159. (PMID: 37088465)
[3] Tish S, Habboub G, Jones J, Ostrom QT, Kruchko C, Barnholtz-Sloan JS, Recinos PF, Kshettry VR (2019) The epidemiology of central and extraventricular neurocytoma in the United States between 2006 and 2014. J Neurooncol 143(1):123-7. doi: 10.1007/s11060-019-03144-9. (PMID: 30859483)
[4] Xie Q, Xie B, Ou L, Wang M, Tang Z, He Y, Yang X, Hong J, Lyu Z, Wei R (2022) Clinical Outcomes and Prognostic Analysis of 101 Patients of Central Neurocytoma: A 10-Year Treatment Experience at a Single Institution. Front Oncol 12:881460. doi: 10.3389/fonc.2022.881460. (PMID: 35692765)
URL: | https://www.eurorad.org/case/18570 |
DOI: | 10.35100/eurorad/case.18570 |
ISSN: | 1563-4086 |
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.