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Clinical course of the untreated calcified big cerebellar liponeurocytoma

Clinical course of the untreated calcified big cerebellar liponeurocytoma Cerebellar liponeurocytoma (CLN) is a very rare tumor of central nervous system, classified as glioneuronal tumor by the lat- est classification of World Health Organization (WHO) in 2016. There are limited data in the literature about biologic behav- ior, growth pattern and radiological features of this tumor. In this case report, we operated a big calcified CLN patient who had denied any surgical procedures suggested by an another institute and had been followed for 4 years. INTRODUCTION CASE REPORT Cerebellar liponeurocytomas (CLN) are very rare neoplasms A 55-year-old woman presented with headache, dizziness and that have been included in the 2016 World Health Organization gait imbalance for 2 months. Patient’s history revealed a poster- (WHO) classification of grade II tumors of the central nervous ior fossa tumor that had been diagnosed 4 years ago prior to system under the heading of glioneuronal tumors [1]. Anatomically, admission to our clinic. At that time, contrast enhanced of mag- these tumors commonly occur in cerebellar hemisphere followed netic resonance imaging (MRI)showed the ill defined lesion, by the vermis. CLN is a slow-growing tumor with a relatively 5.5×3.5×4 cm in size without perilesional edema (Fig. 1A,B). benign clinical prognosis and an indolent course. There is really a During this 4 years period, her follow-up had been performed dearth of data regarding annual growth pattern, biological behavior with serial MRI (Fig. 2A and B) and CT assessments (Fig. 3). Until and precise radiological features of this tumor [2–5]. In the litera- 2 months ago, she had only intermittent headache. On neuro- ture, the reported ill-defined radiological features of these logical examination, she had cerebellar signs including ataxia tumors consist of mild edema or lack of any edematous pat- and also bilateral papilledema through ocular investigations. CT tern including fatty content. CT images were not reported to assessment showed punctate calcified lesion demonstrated with be a definitive diagnostic tool for all the previous presented undifferentiated margins of the lesion from the cerebellar paren- cases. To the best of our knowledge, there is an absence of chyma. The last MRI findings showed heterogeneous ill defined clinical course of an untreated CLN case with a long follow-up lesion without perilesional edema in vermis and extending to period in the English literature. We presented an operated the adjoining cerebellar hemisphere at the supracerebellar infra- unusual CLN case who had been followed with big calcified tentorial space with a dimension of 6.7×4×4cm size causing com- posterior fossa tumor without surgery for 4 years. pression of the fourth ventricle and marked hydrocephalous. The Received: June 22, 2018. Accepted: November 15, 2018 Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2018. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com 1 Downloaded from https://academic.oup.com/jscr/article-abstract/2018/11/rjy316/5197536 by Ed 'DeepDyve' Gillespie user on 16 October 2019 2 V. Hamzaoglu et al. Figure 1: (A) Post-contrast coronal T1 weighted image 4 years ago. Heterogeneous contrast enhancement of the lesion, (B) Axial-T2 weighted image showing slightly hyperintense signal of the big tumor compared with the cortex. Note that the absence of perilesional edema 4 years ago. Figure 2: Preoperative MRI (A) Axial T1-weighted MRI showing hypointense lesion located in the cerebellar vermis. Note the hyperintense area correspond- ing to the fat component. (B) The lesion is hyperintense and well circumscribed patient underwent surgery through a midline supracerebellar on sagittal T2-weighted MRI. Note the supratentorial hydrocephalus secondary to compression of the fourth ventricule. infratentorial approach in the prone position. It was a soft whitish intra-axial tumor, moderately vascular with a good plane between the vermis and cerebellum. A complete resection was achieved. the 2016 World Health Organization (WHO) classification [1, 2]. The cerebrospinal fluidshunt wasnot decidedtobeplaced(Fig. 4). Based on the current literature, precise radiologic features and Pathological diagnosis was reported as CLN. Immunohistochemical biological behaviors of these tumors are still unclear. study, synaptophysin and neuron-specific enolase (NSE) immu- Radiological characteristics of CLN cannot be differentiated nopositivity were found (Fig. 5). In the mean time, glial fibrillary from adult medulloblastomas and ependymomas preopera- acidic protein (GFAP) was immunonegative and Ki-67 index was tively. There were restricted number of cases about the CT and 2%. After operation, she had uneventful postoperative period MRI features of these tumors in the literature [2, 6]. CT shows and was discharged on seventh day. hypo-iso-or hyperdense ill-defined or well-defined variable contrast enhanced lesions with focal areas of marked hypoden- DISCUSSION sity due to fatty tissue [6]. Our case might be suggested as CLN is a rare central nervous system tumor mostly affecting unique for the CT appearance that had included multiple intra- adult population. It was defined as a neuronal-glial tumor in tumoral calcifications without fat density appearance which Downloaded from https://academic.oup.com/jscr/article-abstract/2018/11/rjy316/5197536 by Ed 'DeepDyve' Gillespie user on 16 October 2019 Calcified cerebellar liponeurocytoma 3 Figure 5: Tumor composed of uniform population of small neurocystic cells arranged in sheets and lobules and with regular round nuclei, clear cytoplasm. The histological hallmark of this entity is the focal accumulation of lipid-laden cells that resemble adipocytes (arrow). Figure 3: Computed tomography image showing calcifications in the lesion. The margins of the lesion cannot be differentiated from cerebellar parenchyma. present case, slow tumor progression rate was related to low Ki-67 index which was found as 2% after total tumor resection. To the best of our knowledge, this is the first nonoperated big CLN patient in the literature who survived for a long time with a very slow tumor progression. CONCLUSIONS In summary, the present study reported nonoperated, longlast- ing big CLN with a very slow-growing pattern (1.2×0.5 cm in 4 years) which was undergone total resection. Preclinical radio- logical diagnosis is difficult, as our case, which showed multiple calcifications and mild fat component. In CLN, calcification fea- ture should be taken into consideration for the radiological evaluation in CT. Even in untreated big sized CLNs, low prolifer- ation index (Ki-67) appears to be the main factor for benign bio- logical behavior and slow-growing pattern of these tumors. FUNDING This publication was prepared without any external sources of Figure 4: The postoperative sagittal contrast-enhanced CT scan showing that funding. the tumor had been totally excised. CONFLICT OF INTEREST STATEMENT made this tumor more challenging in differantiation from the We declare that there is no potential or actual conflicts of inter- ependymomas. To the best of our knowledge, there were no est and disclosure of support related our manuscript. reported calcified CLN on CT images in the English literature. Big sized tumors without peritumoral edema and low attenuat- REFERENCES ing fatty tissue might be predictable for CLN through the MRI findings for posterior fossa tumors as our case. CLN may recur 1. Louis DN, Perry A, Reifenberger G, Deimling A, Figarella- with a mean interval of 8.5 years [7]. The related literature Branger D, Cavenee WK, et al. The 2016 World Health shows 5-year survival rate postoperatively as 48%, the recur- Organization. Classification of tumors of the central ner- rence up to 40% [8]. To date, most published cases proliferation vous system: a summary. Acta Neuropathol 2016;131:803–20. index as measured by KI-67/MIB-1 was usually low (<5%). The 2. Alkadhi H, Keller M, Brandner S, Yonekawa Y, Kollias SS. tumor with high rates of Ki-67 was associated with high rates Neuroimaging of cerebellar liponeurocytoma. Case report. of recurrences and more agressive biological behavior [9, 10]. In J Neurosurg 2001;95:324–31. our case, despite the big size of the lesion in the initial MRI 3. Bechtel JT, Patton JM, Takei Y. Mixed mesenchymal and (5.5×3.5×4 cm), the growth pattern of the tumor was quite slow. neuroectodermal tumor of the cerebellum. Acta Neuropath However, radiological enlargement had been measured only (Berl.) 1978;41:261–3. 1.2 cm on sagittal plane and 0,5 mm in antero-posterior direc- 4. Owler B, Makeham J, Shingde M, Besser M. Cerebellar lipo- tion (6.7×4×4 cm) during preoperative 4 years period. In the neurocytoma. J Cli Neurosci 2005;12:326–9. Downloaded from https://academic.oup.com/jscr/article-abstract/2018/11/rjy316/5197536 by Ed 'DeepDyve' Gillespie user on 16 October 2019 4 V. Hamzaoglu et al. 5. Kakkar A, Sable M, Suri V, Sarkar C, Garg A, Satyarthee GD, 8. Kleihues P, Chimelli L, Giangaspero F, Ohgaki H. WHO et al. Cerebellar liponeurocytoma, an unusual tumor of the Classification of Tumours of the Central Nervous System. 4th ed. central nervous system ultrastructural examination. Lyon: IARC, 2007;110–2. Ultrastruct Pathol 2015;39:419–23. 9. Oudrhiri MY, Raouzi N, El Kacemi I, El Fatemi N, Gana R, 6. Satyarthee GD, Mahapatra AK. Liponeurocytoma of Maaqili MR, et al. Understanding cerebellar liponeurocyto- Cerebellum: rare entity, case based study. Rom Neurosurg mas: case report and literature review. Case Rep Neurol Med 2015;29:183–7. 2014;2014:1–6. 7. Limaiem F, Bellil S, Chelly I, Mekni A, Jemel H, Haouet S, 10. Qu LM, Yu JL, Ma HX, Wang YP. Cerebellar liponeurocytoma et al. Recurrent cerebellar liponeurocytoma with supraten- in the posterior fossa: a case report and review of the litera- torial extension. Can J Neurol Sci 2009;36:662–5. ture. Int J Clin Exp Pathol 2016;9:8724–9. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Journal of Surgical Case Reports Oxford University Press

Clinical course of the untreated calcified big cerebellar liponeurocytoma

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Oxford University Press
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Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2018.
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2042-8812
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10.1093/jscr/rjy316
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Abstract

Cerebellar liponeurocytoma (CLN) is a very rare tumor of central nervous system, classified as glioneuronal tumor by the lat- est classification of World Health Organization (WHO) in 2016. There are limited data in the literature about biologic behav- ior, growth pattern and radiological features of this tumor. In this case report, we operated a big calcified CLN patient who had denied any surgical procedures suggested by an another institute and had been followed for 4 years. INTRODUCTION CASE REPORT Cerebellar liponeurocytomas (CLN) are very rare neoplasms A 55-year-old woman presented with headache, dizziness and that have been included in the 2016 World Health Organization gait imbalance for 2 months. Patient’s history revealed a poster- (WHO) classification of grade II tumors of the central nervous ior fossa tumor that had been diagnosed 4 years ago prior to system under the heading of glioneuronal tumors [1]. Anatomically, admission to our clinic. At that time, contrast enhanced of mag- these tumors commonly occur in cerebellar hemisphere followed netic resonance imaging (MRI)showed the ill defined lesion, by the vermis. CLN is a slow-growing tumor with a relatively 5.5×3.5×4 cm in size without perilesional edema (Fig. 1A,B). benign clinical prognosis and an indolent course. There is really a During this 4 years period, her follow-up had been performed dearth of data regarding annual growth pattern, biological behavior with serial MRI (Fig. 2A and B) and CT assessments (Fig. 3). Until and precise radiological features of this tumor [2–5]. In the litera- 2 months ago, she had only intermittent headache. On neuro- ture, the reported ill-defined radiological features of these logical examination, she had cerebellar signs including ataxia tumors consist of mild edema or lack of any edematous pat- and also bilateral papilledema through ocular investigations. CT tern including fatty content. CT images were not reported to assessment showed punctate calcified lesion demonstrated with be a definitive diagnostic tool for all the previous presented undifferentiated margins of the lesion from the cerebellar paren- cases. To the best of our knowledge, there is an absence of chyma. The last MRI findings showed heterogeneous ill defined clinical course of an untreated CLN case with a long follow-up lesion without perilesional edema in vermis and extending to period in the English literature. We presented an operated the adjoining cerebellar hemisphere at the supracerebellar infra- unusual CLN case who had been followed with big calcified tentorial space with a dimension of 6.7×4×4cm size causing com- posterior fossa tumor without surgery for 4 years. pression of the fourth ventricle and marked hydrocephalous. The Received: June 22, 2018. Accepted: November 15, 2018 Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2018. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com 1 Downloaded from https://academic.oup.com/jscr/article-abstract/2018/11/rjy316/5197536 by Ed 'DeepDyve' Gillespie user on 16 October 2019 2 V. Hamzaoglu et al. Figure 1: (A) Post-contrast coronal T1 weighted image 4 years ago. Heterogeneous contrast enhancement of the lesion, (B) Axial-T2 weighted image showing slightly hyperintense signal of the big tumor compared with the cortex. Note that the absence of perilesional edema 4 years ago. Figure 2: Preoperative MRI (A) Axial T1-weighted MRI showing hypointense lesion located in the cerebellar vermis. Note the hyperintense area correspond- ing to the fat component. (B) The lesion is hyperintense and well circumscribed patient underwent surgery through a midline supracerebellar on sagittal T2-weighted MRI. Note the supratentorial hydrocephalus secondary to compression of the fourth ventricule. infratentorial approach in the prone position. It was a soft whitish intra-axial tumor, moderately vascular with a good plane between the vermis and cerebellum. A complete resection was achieved. the 2016 World Health Organization (WHO) classification [1, 2]. The cerebrospinal fluidshunt wasnot decidedtobeplaced(Fig. 4). Based on the current literature, precise radiologic features and Pathological diagnosis was reported as CLN. Immunohistochemical biological behaviors of these tumors are still unclear. study, synaptophysin and neuron-specific enolase (NSE) immu- Radiological characteristics of CLN cannot be differentiated nopositivity were found (Fig. 5). In the mean time, glial fibrillary from adult medulloblastomas and ependymomas preopera- acidic protein (GFAP) was immunonegative and Ki-67 index was tively. There were restricted number of cases about the CT and 2%. After operation, she had uneventful postoperative period MRI features of these tumors in the literature [2, 6]. CT shows and was discharged on seventh day. hypo-iso-or hyperdense ill-defined or well-defined variable contrast enhanced lesions with focal areas of marked hypoden- DISCUSSION sity due to fatty tissue [6]. Our case might be suggested as CLN is a rare central nervous system tumor mostly affecting unique for the CT appearance that had included multiple intra- adult population. It was defined as a neuronal-glial tumor in tumoral calcifications without fat density appearance which Downloaded from https://academic.oup.com/jscr/article-abstract/2018/11/rjy316/5197536 by Ed 'DeepDyve' Gillespie user on 16 October 2019 Calcified cerebellar liponeurocytoma 3 Figure 5: Tumor composed of uniform population of small neurocystic cells arranged in sheets and lobules and with regular round nuclei, clear cytoplasm. The histological hallmark of this entity is the focal accumulation of lipid-laden cells that resemble adipocytes (arrow). Figure 3: Computed tomography image showing calcifications in the lesion. The margins of the lesion cannot be differentiated from cerebellar parenchyma. present case, slow tumor progression rate was related to low Ki-67 index which was found as 2% after total tumor resection. To the best of our knowledge, this is the first nonoperated big CLN patient in the literature who survived for a long time with a very slow tumor progression. CONCLUSIONS In summary, the present study reported nonoperated, longlast- ing big CLN with a very slow-growing pattern (1.2×0.5 cm in 4 years) which was undergone total resection. Preclinical radio- logical diagnosis is difficult, as our case, which showed multiple calcifications and mild fat component. In CLN, calcification fea- ture should be taken into consideration for the radiological evaluation in CT. Even in untreated big sized CLNs, low prolifer- ation index (Ki-67) appears to be the main factor for benign bio- logical behavior and slow-growing pattern of these tumors. FUNDING This publication was prepared without any external sources of Figure 4: The postoperative sagittal contrast-enhanced CT scan showing that funding. the tumor had been totally excised. CONFLICT OF INTEREST STATEMENT made this tumor more challenging in differantiation from the We declare that there is no potential or actual conflicts of inter- ependymomas. To the best of our knowledge, there were no est and disclosure of support related our manuscript. reported calcified CLN on CT images in the English literature. Big sized tumors without peritumoral edema and low attenuat- REFERENCES ing fatty tissue might be predictable for CLN through the MRI findings for posterior fossa tumors as our case. CLN may recur 1. Louis DN, Perry A, Reifenberger G, Deimling A, Figarella- with a mean interval of 8.5 years [7]. The related literature Branger D, Cavenee WK, et al. The 2016 World Health shows 5-year survival rate postoperatively as 48%, the recur- Organization. Classification of tumors of the central ner- rence up to 40% [8]. To date, most published cases proliferation vous system: a summary. Acta Neuropathol 2016;131:803–20. index as measured by KI-67/MIB-1 was usually low (<5%). The 2. Alkadhi H, Keller M, Brandner S, Yonekawa Y, Kollias SS. tumor with high rates of Ki-67 was associated with high rates Neuroimaging of cerebellar liponeurocytoma. Case report. of recurrences and more agressive biological behavior [9, 10]. In J Neurosurg 2001;95:324–31. our case, despite the big size of the lesion in the initial MRI 3. Bechtel JT, Patton JM, Takei Y. Mixed mesenchymal and (5.5×3.5×4 cm), the growth pattern of the tumor was quite slow. neuroectodermal tumor of the cerebellum. Acta Neuropath However, radiological enlargement had been measured only (Berl.) 1978;41:261–3. 1.2 cm on sagittal plane and 0,5 mm in antero-posterior direc- 4. Owler B, Makeham J, Shingde M, Besser M. Cerebellar lipo- tion (6.7×4×4 cm) during preoperative 4 years period. In the neurocytoma. J Cli Neurosci 2005;12:326–9. Downloaded from https://academic.oup.com/jscr/article-abstract/2018/11/rjy316/5197536 by Ed 'DeepDyve' Gillespie user on 16 October 2019 4 V. Hamzaoglu et al. 5. Kakkar A, Sable M, Suri V, Sarkar C, Garg A, Satyarthee GD, 8. Kleihues P, Chimelli L, Giangaspero F, Ohgaki H. WHO et al. Cerebellar liponeurocytoma, an unusual tumor of the Classification of Tumours of the Central Nervous System. 4th ed. central nervous system ultrastructural examination. Lyon: IARC, 2007;110–2. Ultrastruct Pathol 2015;39:419–23. 9. Oudrhiri MY, Raouzi N, El Kacemi I, El Fatemi N, Gana R, 6. Satyarthee GD, Mahapatra AK. Liponeurocytoma of Maaqili MR, et al. Understanding cerebellar liponeurocyto- Cerebellum: rare entity, case based study. Rom Neurosurg mas: case report and literature review. Case Rep Neurol Med 2015;29:183–7. 2014;2014:1–6. 7. Limaiem F, Bellil S, Chelly I, Mekni A, Jemel H, Haouet S, 10. Qu LM, Yu JL, Ma HX, Wang YP. Cerebellar liponeurocytoma et al. Recurrent cerebellar liponeurocytoma with supraten- in the posterior fossa: a case report and review of the litera- torial extension. Can J Neurol Sci 2009;36:662–5. ture. Int J Clin Exp Pathol 2016;9:8724–9.

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Journal of Surgical Case Reports – Oxford University Press

Published: Nov 1, 2018

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