Central neurocytoma (CNC) is an extremely rare, ordinarily benign intraventricular brain tumour that typically forms from the neuronal cells of the septum pellucidum.[1] The majority of central neurocytomas grow inwards into the ventricular system forming interventricular neurocytomas. This leads to two primary symptoms of CNCs, blurred vision and increased intracranial pressure. Treatment for a central neurocytoma typically involves surgical removal, with an approximate 1 in 5 chance of recurrence.[2] Central neurocytomas are classified as a grade II tumor under the World Health Organization's classification of tumors of the nervous system.[3]
Central neurocytoma
Axial T1-weighted gadolinium-enhanced MRI image showing an enhancing mass with cystic changes consistent with central neurocytoma in the right lateral ventricle.
There is a wide range of symptoms that patients show. Symptoms can lie dormant, but come about due to Obstructive hydrocephalus. These symptoms include:[4]
On the macroscopic scale, CNC tumors are grayish in color, resembling the gray matter that comes with areas of hemorrhage. The tumors are soft, ovid, lobulated to nodular masses that are generally well circumscribed. When dissecting the tumor, scientists experienced some grittiness, which they attribute to the presence of calcification.[citation needed]
Tumor samples stained using H&E stain and examined under the microscope revealed that CNC is a well-differentiated tumor with benign histological features. The tumor is composed of “uniform, small-to-medium-sized cells with rounded nuclei, finely stippled chromatin and inconspicuous nucleoli, along with scant cytoplasm.” CNC are characterized by perivascular pseudorosettes, circular/flower-like arrangements of cells with a small blood vessel at the centre, and polygonal small cells with a clear perinuclear halo, sometimes called the ‘fried egg’ appearance, and is clear or slightly eosinophilic. The main histomorphologic differential diagnosis is oligodendroglioma. While the tumor cells are dense in some areas, areas with anuclear, less dense tumor parts were dispersed throughout. The anuclear areas may have a fine fibrillary matrix, like that of neuropil regions. Long, thin-walled, capillary-sized vessels represent the vascularity of CNC. These vessels are arranged in a linear branching pattern, with an endocrine appearance. Thin-walled dilated vascular channels, as well as foci of calcification, were readily identified in many cases.[4]
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Diagnosisedit
This section is empty. You can help by adding to it. (December 2021)
Treatmentedit
The mainstay of treatment is surgical excision.[7] Two adjuvant therapeutic strategies are Stereotactic surgery (SRS) and fractionated convention radiotherapy (FCRT). Both are highly effective means of treatment.[8]
Surgeryedit
Surgical excision of the central neurocytoma is the primary consensus among practicing physicians. The surgeons perform a craniotomy to remove the tumor. The ability to remove the tumor and to what extent it is removed is dependent upon the location of the tumor and surgeon experience and preference. The extent of the disease plays a large part in determining how effective the surgery will be. The main goal of a complete surgical resection, of the tumor, can also be hindered by the adherence of the tumor to adjoining structures or hemorrhages.[5] If there is a recurrence of the central neurocytoma, surgery is again the most notable treatment.
Radiotherapyedit
There is not much evidence supporting the claim that radiotherapy is a beneficial and effective means of treatment. Typically, radiotherapy is used postoperatively in respect to whether or not a partial or complete excision of the tumor has been accomplished.[9] The histopathological features of CNC, neuronal differentiation, low mitotic activity, absence of vascular endothelial proliferation, and tumor necrosis, suggest that the tumor may be resistant to ionizing radiation. However, when radiotherapy is used, whole brain or involved-field treatment is given. This method utilizes a standard fractionation schedule and a total tumor dose of 50-55 Gy.[5] Gamma knife surgery is a form of radiotherapy, more specifically radiosurgery that uses beams of gamma rays to deliver a certain dosage of radiation to the tumor. Gamma knife surgery is incredibly effective at treating neurocytoma and maintaining tumor control after the procedure when a complete excision has been performed. Some studies have found that the success rate of tumor control is around 90% after the first five years and 80% after the first ten years.[2][10] Gamma knife surgery is the most recorded form of radiotherapy performed to treat remnants of the CNC tumor after surgery.[10]
Chemotherapyedit
Chemotherapy is typically limited to patients with recurrent central neurocytoma. The course of chemotherapy used for CNC is one of two platinum-based regimes. The two regimes are:
Carboplatin + VP-16 + ifosfamide
cisplatin + VP-16 + cyclophosphamide
Because chemotherapy is used in rare cases there is still information to be gathered as to the efficacy of chemotherapy to treat benign CNC. Therefore, recommendations must be viewed as limited and preliminary.[5]
Outcome and recurrenceedit
The majority of patients can be expected to be cured of their disease and become long-term survivors of central neurocytoma. As with any other type of tumor, there is a chance for recurrence. The chance of recurrence is approximately 20%.[2] Some factors that predict tumor recurrence and death due to progressive states of disease are high proliferative indices, early disease recurrence, and disseminated disease with or without the spread of disease through the cerebral spinal fluid.[5] Long-term follow up examinations are essential for the evaluation of the outcomes that each treatment brings about. It is also essential to identify possible recurrence of CN. It is recommended that a cranial MRI is performed between every 6–12 months.[2]
Historyedit
It was first described in 1982 by Hassoun.[11] Central neurocytomas are rare brain tumors that are located most of the times in the lateral ventricles near the Monro foramina. They were first discovered by Hassoun and co-workers in 1982, and were classified as grade II tumors.[12] In 1985, Wilson had also described a rare case of "differentiated neuroblastoma" in the lateral ventricle that resembles oligodendroglioma on light microscopy. However, the name central neurocytoma was given by Hassoun.[13]
Primary neuronal tumors occurring in the ventricular system were considered to be rare. Most cases described were of non-neuronal origin such as oligodendroglioma, ependymoma, meningioma, choroid plexus papilloma and giant cell. Neurocytomas were probably historically misdiagnosed as intraventricular oligondedronglioma or clear cell ependymoma prior to this. With its non-aggressive behavior the tumor has often been called "benign central neurocytoma". It is believed to occur in young adults from the neuronal cells of the septum pullicidum and the subependymal cells of the lateral ventricles. Most of the initial incidents reported in the lateral ventricle were benign. However, as more information was gathered the name benign central neurocytoma was started to be seen as a double misnomer because these tumors are not always benign nor centrally located. Many recent studies suggest that their location, biological potential and clinical behavior are observed be more variable than previously thought. Recent studies indicate their uncommon location, aggressive biological behavior and frequent recurrences following after surgical resection have generated significant interest in various treatment modalities and also in their terminology, lineage potential and molecular regulation.[13]
Epidemiologyedit
CNC represent 0.1-0.5% of primary brain tumours.[14][8] There is a genetic component to the formation of these tumors, causing a larger proportion of tumors to form in people of Asian descent than of other ethnic groups.[15] Central neurocytomas predominantly form in young adults, most commonly during the second or third decade of life.[16] There is no evidence that the sex of a person is a determinant in the frequency of central neurocytomas.[15]
^Kerkeni, A.; Ben Lakhdher, Z.; Rkhami, M.; Sebai, R.; Belguith, L.; Khaldi, M.; Ben Hamouda, M. (Oct 2010). "[Central neurocytoma: Study of 32 cases and review of the literature]". Neurochirurgie. 56 (5): 408–14. doi:10.1016/j.neuchi.2010.07.001. PMID 20692674.
^ abcdKim JW, Kim DG, Chung HT, Choi SH, Han JH, Park CK, Kim CY, Paek SH, Jung HW (December 2013). "Radiosurgery for central neurocytoma: long-term outcome and failure pattern". J. Neurooncol. 115 (3): 505–11. doi:10.1007/s11060-013-1253-9. PMID 24065568. S2CID 21557148.
^Louis David N.; Ohgaki Hiroko; Wiestler Otmar D.; Cavenee Webster K.; Burger Peter C.; Jouvet Anne; Scheithauer Bernd W.; Kleihues Paul (2007). "The 2007 WHO Classification of Tumours of the Central Nervous System". Acta Neuropathol. 114 (2): 97–109. doi:10.1007/s00401-007-0243-4. PMC1929165. PMID 17618441.
^ abLi Y, Ye XF, Qian G, Yin Y, Pan QG (2012). "Pathologic features and clinical outcome of central neurocytoma: analysis of 15 cases". Chin J Cancer Res. 24 (4): 284–290. doi:10.1007/s11670-012-0265-x. PMC3551323. PMID 23358787.
^Ouma, JR (2004). "Psychotic manifestations in brain tumour patients: 2 case reports from South Africa". Afr Health Sci. 4 (3): 190–194. PMC2688330. PMID 15687074.
^ abKarlsson Bengt; Guo Wan-Yuo; Kejia Teo; Dinesh Nivedh; Pan David Hung-Chi; Jokura Hidefumi; Kawagishi Jun; van Eck Albertus; Horstmann Gerhard A.; Yeo Tseng Tsai; Yamamoto Masaaki (2012). "Gamma Knife surgery for central neurocytomas". J Neurosurg. 117 (Suppl): 96–101. doi:10.3171/2012.6.GKS12214. PMID 23205795.
^Hassoun, J.; Gambarelli, D.; Grisoli, F.; Pellet, W.; Salamon, G.; Pellissier, JF.; Toga, M. (1982). "Central neurocytoma. An electron-microscopic study of two cases". Acta Neuropathol. 56 (2): 151–6. doi:10.1007/bf00690587. PMID 7064664. S2CID 6524741.
^Qian H.; Lin S.; Zhang M.; Cao Y. (2012). "Surgical management of intraventricular central neurocytoma: 92 cases". Acta Neurochirurgica. 154 (11): 1951–60. doi:10.1007/s00701-012-1446-6. PMID 22941394. S2CID 11054237.
^ abChoudhari K. A.; Kaliaperumal C.; Jain A.; Sarkar C.; Soo M.; Rades D.; Singh J. (2009). "Central neurocytoma: A multi-disciplinary review". British Journal of Neurosurgery. 23 (6): 585–595. doi:10.3109/02688690903254350. PMID 19922271. S2CID 1245015.
^Chuang, MT.; Lin, WC.; Tsai, HY.; Liu, GC.; Hu, SW.; Chiang, IC. (2005). "3-T proton magnetic resonance spectroscopy of central neurocytoma: 3 case reports and review of the literature". J Comput Assist Tomogr. 29 (5): 683–8. doi:10.1097/01.rct.0000171240.95430.29. PMID 16163043.
^Hassoun J, Soylemezoglu F, Gambarelli D, Figarella-Branger D, von Ammon K, Kleihues P (1993). "Central neurocytoma: a synopsis of clinical and histological features". Brain Pathology. 3 (3): 297–306. doi:10.1111/j.1750-3639.1993.tb00756.x. PMID 8293189. S2CID 19604295.
External linksedit
Wikimedia Commons has media related to Central neurocytoma.
October 26, 2023
central, neurocytoma, extremely, rare, ordinarily, benign, intraventricular, brain, tumour, that, typically, forms, from, neuronal, cells, septum, pellucidum, majority, central, neurocytomas, grow, inwards, into, ventricular, system, forming, interventricular,. Central neurocytoma CNC is an extremely rare ordinarily benign intraventricular brain tumour that typically forms from the neuronal cells of the septum pellucidum 1 The majority of central neurocytomas grow inwards into the ventricular system forming interventricular neurocytomas This leads to two primary symptoms of CNCs blurred vision and increased intracranial pressure Treatment for a central neurocytoma typically involves surgical removal with an approximate 1 in 5 chance of recurrence 2 Central neurocytomas are classified as a grade II tumor under the World Health Organization s classification of tumors of the nervous system 3 Central neurocytomaAxial T1 weighted gadolinium enhanced MRI image showing an enhancing mass with cystic changes consistent with central neurocytoma in the right lateral ventricle SpecialtyOncology neurosurgery Contents 1 Signs and symptoms 2 Pathology 3 Diagnosis 4 Treatment 4 1 Surgery 4 2 Radiotherapy 4 3 Chemotherapy 4 4 Outcome and recurrence 5 History 6 Epidemiology 7 See also 8 References 9 External linksSigns and symptoms editThere is a wide range of symptoms that patients show Symptoms can lie dormant but come about due to Obstructive hydrocephalus These symptoms include 4 Intracranial pressure Headache Papilledema Vomiting Light headedness Impaired mental activity Gait instabilityIn rare and extreme cases more severe symptoms can be observed Memory disturbance Dementia Hemiparesis 5 Seizures Hemorrhage Psychosis 6 Pathology edit nbsp Micrograph of a central neurocytoma H amp E stain On the macroscopic scale CNC tumors are grayish in color resembling the gray matter that comes with areas of hemorrhage The tumors are soft ovid lobulated to nodular masses that are generally well circumscribed When dissecting the tumor scientists experienced some grittiness which they attribute to the presence of calcification citation needed Tumor samples stained using H amp E stain and examined under the microscope revealed that CNC is a well differentiated tumor with benign histological features The tumor is composed of uniform small to medium sized cells with rounded nuclei finely stippled chromatin and inconspicuous nucleoli along with scant cytoplasm CNC are characterized by perivascular pseudorosettes circular flower like arrangements of cells with a small blood vessel at the centre and polygonal small cells with a clear perinuclear halo sometimes called the fried egg appearance and is clear or slightly eosinophilic The main histomorphologic differential diagnosis is oligodendroglioma While the tumor cells are dense in some areas areas with anuclear less dense tumor parts were dispersed throughout The anuclear areas may have a fine fibrillary matrix like that of neuropil regions Long thin walled capillary sized vessels represent the vascularity of CNC These vessels are arranged in a linear branching pattern with an endocrine appearance Thin walled dilated vascular channels as well as foci of calcification were readily identified in many cases 4 nbsp Very high mag nbsp High mag nbsp Very high mag Diagnosis editThis section is empty You can help by adding to it December 2021 Treatment editThe mainstay of treatment is surgical excision 7 Two adjuvant therapeutic strategies are Stereotactic surgery SRS and fractionated convention radiotherapy FCRT Both are highly effective means of treatment 8 Surgery edit Surgical excision of the central neurocytoma is the primary consensus among practicing physicians The surgeons perform a craniotomy to remove the tumor The ability to remove the tumor and to what extent it is removed is dependent upon the location of the tumor and surgeon experience and preference The extent of the disease plays a large part in determining how effective the surgery will be The main goal of a complete surgical resection of the tumor can also be hindered by the adherence of the tumor to adjoining structures or hemorrhages 5 If there is a recurrence of the central neurocytoma surgery is again the most notable treatment Radiotherapy edit There is not much evidence supporting the claim that radiotherapy is a beneficial and effective means of treatment Typically radiotherapy is used postoperatively in respect to whether or not a partial or complete excision of the tumor has been accomplished 9 The histopathological features of CNC neuronal differentiation low mitotic activity absence of vascular endothelial proliferation and tumor necrosis suggest that the tumor may be resistant to ionizing radiation However when radiotherapy is used whole brain or involved field treatment is given This method utilizes a standard fractionation schedule and a total tumor dose of 50 55 Gy 5 Gamma knife surgery is a form of radiotherapy more specifically radiosurgery that uses beams of gamma rays to deliver a certain dosage of radiation to the tumor Gamma knife surgery is incredibly effective at treating neurocytoma and maintaining tumor control after the procedure when a complete excision has been performed Some studies have found that the success rate of tumor control is around 90 after the first five years and 80 after the first ten years 2 10 Gamma knife surgery is the most recorded form of radiotherapy performed to treat remnants of the CNC tumor after surgery 10 Chemotherapy edit Chemotherapy is typically limited to patients with recurrent central neurocytoma The course of chemotherapy used for CNC is one of two platinum based regimes The two regimes are Carboplatin VP 16 ifosfamide cisplatin VP 16 cyclophosphamideBecause chemotherapy is used in rare cases there is still information to be gathered as to the efficacy of chemotherapy to treat benign CNC Therefore recommendations must be viewed as limited and preliminary 5 Outcome and recurrence edit The majority of patients can be expected to be cured of their disease and become long term survivors of central neurocytoma As with any other type of tumor there is a chance for recurrence The chance of recurrence is approximately 20 2 Some factors that predict tumor recurrence and death due to progressive states of disease are high proliferative indices early disease recurrence and disseminated disease with or without the spread of disease through the cerebral spinal fluid 5 Long term follow up examinations are essential for the evaluation of the outcomes that each treatment brings about It is also essential to identify possible recurrence of CN It is recommended that a cranial MRI is performed between every 6 12 months 2 History editIt was first described in 1982 by Hassoun 11 Central neurocytomas are rare brain tumors that are located most of the times in the lateral ventricles near the Monro foramina They were first discovered by Hassoun and co workers in 1982 and were classified as grade II tumors 12 In 1985 Wilson had also described a rare case of differentiated neuroblastoma in the lateral ventricle that resembles oligodendroglioma on light microscopy However the name central neurocytoma was given by Hassoun 13 Primary neuronal tumors occurring in the ventricular system were considered to be rare Most cases described were of non neuronal origin such as oligodendroglioma ependymoma meningioma choroid plexus papilloma and giant cell Neurocytomas were probably historically misdiagnosed as intraventricular oligondedronglioma or clear cell ependymoma prior to this With its non aggressive behavior the tumor has often been called benign central neurocytoma It is believed to occur in young adults from the neuronal cells of the septum pullicidum and the subependymal cells of the lateral ventricles Most of the initial incidents reported in the lateral ventricle were benign However as more information was gathered the name benign central neurocytoma was started to be seen as a double misnomer because these tumors are not always benign nor centrally located Many recent studies suggest that their location biological potential and clinical behavior are observed be more variable than previously thought Recent studies indicate their uncommon location aggressive biological behavior and frequent recurrences following after surgical resection have generated significant interest in various treatment modalities and also in their terminology lineage potential and molecular regulation 13 Epidemiology editCNC represent 0 1 0 5 of primary brain tumours 14 8 There is a genetic component to the formation of these tumors causing a larger proportion of tumors to form in people of Asian descent than of other ethnic groups 15 Central neurocytomas predominantly form in young adults most commonly during the second or third decade of life 16 There is no evidence that the sex of a person is a determinant in the frequency of central neurocytomas 15 See also editNeurocytomaReferences edit Kerkeni A Ben Lakhdher Z Rkhami M Sebai R Belguith L Khaldi M Ben Hamouda M Oct 2010 Central neurocytoma Study of 32 cases and review of the literature Neurochirurgie 56 5 408 14 doi 10 1016 j neuchi 2010 07 001 PMID 20692674 a b c d Kim JW Kim DG Chung HT Choi SH Han JH Park CK Kim CY Paek SH Jung HW December 2013 Radiosurgery for central neurocytoma long term outcome and failure pattern J Neurooncol 115 3 505 11 doi 10 1007 s11060 013 1253 9 PMID 24065568 S2CID 21557148 Louis David N Ohgaki Hiroko Wiestler Otmar D Cavenee Webster K Burger Peter C Jouvet Anne Scheithauer Bernd W Kleihues Paul 2007 The 2007 WHO Classification of Tumours of the Central Nervous System Acta Neuropathol 114 2 97 109 doi 10 1007 s00401 007 0243 4 PMC 1929165 PMID 17618441 a b Li Y Ye XF Qian G Yin Y Pan QG 2012 Pathologic features and clinical outcome of central neurocytoma analysis of 15 cases Chin J Cancer Res 24 4 284 290 doi 10 1007 s11670 012 0265 x PMC 3551323 PMID 23358787 a b c d e Chamberlain Marc C Treatment of Central Neurocytoma USC Norris Cancer Center Seattle Cancer Care Alliance lt https www seattlecca org client Chamberlain Treatment 20of 20Central 20Neurocytomas pdf Archived 2014 03 24 at the Wayback Machine gt Feb 20 2014 Ouma JR 2004 Psychotic manifestations in brain tumour patients 2 case reports from South Africa Afr Health Sci 4 3 190 194 PMC 2688330 PMID 15687074 Schmidt MH Gottfried ON von Koch CS Chang SM McDermott MW Feb 2004 Central neurocytoma a review J Neurooncol 66 3 377 84 doi 10 1023 b neon 0000014541 87329 3b PMID 15015671 S2CID 8320493 a b Garcia RM Ivan ME Oh T Barani I Parsa AT 2014 Intraventricular neurocytomas A systematic review of stereotactic radiosurgery and fractionated conventional radiotherapy for residual or recurrent tumors Clinical Neurology and Neurosurgery 117 55 64 doi 10 1016 j clineuro 2013 11 028 PMID 24438806 S2CID 23101170 Chen H Zhou R Liu J Tang J June 2012 Central neurocytoma J Clin Neurosci 19 6 849 53 doi 10 1016 j jocn 2011 06 038 PMID 22537657 S2CID 12995192 a b Karlsson Bengt Guo Wan Yuo Kejia Teo Dinesh Nivedh Pan David Hung Chi Jokura Hidefumi Kawagishi Jun van Eck Albertus Horstmann Gerhard A Yeo Tseng Tsai Yamamoto Masaaki 2012 Gamma Knife surgery for central neurocytomas J Neurosurg 117 Suppl 96 101 doi 10 3171 2012 6 GKS12214 PMID 23205795 Hassoun J Gambarelli D Grisoli F Pellet W Salamon G Pellissier JF Toga M 1982 Central neurocytoma An electron microscopic study of two cases Acta Neuropathol 56 2 151 6 doi 10 1007 bf00690587 PMID 7064664 S2CID 6524741 Qian H Lin S Zhang M Cao Y 2012 Surgical management of intraventricular central neurocytoma 92 cases Acta Neurochirurgica 154 11 1951 60 doi 10 1007 s00701 012 1446 6 PMID 22941394 S2CID 11054237 a b Choudhari K A Kaliaperumal C Jain A Sarkar C Soo M Rades D Singh J 2009 Central neurocytoma A multi disciplinary review British Journal of Neurosurgery 23 6 585 595 doi 10 3109 02688690903254350 PMID 19922271 S2CID 1245015 Chuang MT Lin WC Tsai HY Liu GC Hu SW Chiang IC 2005 3 T proton magnetic resonance spectroscopy of central neurocytoma 3 case reports and review of the literature J Comput Assist Tomogr 29 5 683 8 doi 10 1097 01 rct 0000171240 95430 29 PMID 16163043 a b Sharma Mehar Chand Deb Prabal Sharma Suash Sarkar Chitra August 2006 Neurocytoma a comprehensive review Neurosurgical Review 29 4 270 285 doi 10 1007 s10143 006 0030 z PMID 16941163 S2CID 7854296 Hassoun J Soylemezoglu F Gambarelli D Figarella Branger D von Ammon K Kleihues P 1993 Central neurocytoma a synopsis of clinical and histological features Brain Pathology 3 3 297 306 doi 10 1111 j 1750 3639 1993 tb00756 x PMID 8293189 S2CID 19604295 External links edit nbsp Wikimedia Commons has media related to Central neurocytoma Retrieved from https en wikipedia org w index php title Central neurocytoma amp oldid 1146512507, wikipedia, wiki, book, books, library,
