Cerebellar liponeurocytomas were recognized in the 2000 WHO 3rd edition of CNS tumors as a distinct grade I pathological entity, a tumor with a more favorable prognosis than medulloblastoma. But reports of long-term recurrences and some possible aggressive behavior led to an upgrade on the latest WHO 4th edition of CNS tumors. The case of a 64-year-old female patient is reported in this paper. More than 30 cases of this lately recognized pathological entity have been reported to date. The diagnostic, radiological, and pathological features associated with this tumor are discussed through a literature review. 1. Introduction The cerebellar liponeurocytoma was first included in the 2000 World Health Organisation’s (WHO) 3rd edition of Central Nervous System (CNS) tumors’ classification as a separate grade I entity. Thanks to a better recognition and longer follow-up, evidences of a possible tumor recurrence were growing. Rates of recurrences were estimated to 60% during follow-up, with some cases presenting even an aggressive behavior [1–3]. This way, the cerebellar liponeurocytoma was upgraded II on the 2007 WHO’s 4th edition of CNS tumors’ classification. Through this case report we reviewed the literature for a better understanding of this pathology and the controversy around, with a listing of published cases of cerebellar liponeurocytoma. 2. A Case Report A 64-year-old woman, with a 10-year history of Parkinson’s disease, was admitted to our department for a 7-month clinical course of progressive worsening, right-sided weakness, and signs of increased intracranial pressure. On examination, her consciousness was clear, and, beside the Parkinson’s related tremor, she had a mild right hemiparesia (3/5) with a right Babinski sign and hyperreflexia on the right side. Ocular investigations showed bilateral papilledema and horizontal nystagmus. MRI scan showed a 60 × 40 × 42?mm heterogeneous, enhancing lesion of the right cerebellum extending to the CPA (cerebellopontine angle), causing mass effect and mild hydrocephalous. On T1-weighted images (Figure 1(a)) the mass was iso-intense to brain tissue, with sparse, hyperintense areas that turned hypointense on FAT-SAT sequences (Figure 1(b), arrow). Figure 1: MRI of the brain in sagittal T1 (a), axial fat sat sequence (b), and axial T1 with gadolinium, respectively (c). Note the fat component (arrows in (a) and (c)) that turns hypointense after fat exclusion (arrow in (b)). Irregular enhancement was noted after gadolinium administration (Figure 1(c)). There was no associated edema. The patient underwent
References
[1]
T. R. Jackson, W. F. Regine, D. Wilson, and D. G. Davis, “Cerebellar liponeurocytoma: case report and review of the literature,” Journal of Neurosurgery, vol. 95, no. 4, pp. 700–703, 2001.
[2]
M. D. Jenkinson, J. J. D. Bosma, D. Du Plessis et al., “Cerebellar liponeurocytoma with an unusually aggressive clinical course: case report,” Neurosurgery, vol. 53, no. 6, pp. 1425–1428, 2003.
[3]
G. Pasquale, B. A. Maria, P. Vania, P. Gastone, and D. L. Nicola, “Cerebellar liponeurocytoma: an updated follow-up of a case presenting histopathological and clinically aggressive features,” Neurology India, vol. 57, no. 2, pp. 194–196, 2009.
[4]
D. J. Brat, J. E. Parisi, B. K. Kleinschmidt-DeMasters et al., “Surgical neuropathology update: a review of changes introduced by the WHO classification of tumours of the central nervous system, 4th edition,” Archives of Pathology and Laboratory Medicine, vol. 132, no. 6, pp. 993–1007, 2008.
[5]
P. Kleihues, L. Chimelli, F. Giangaspero, and H. Ohgaki, “Cerebellar liponeurocytoma,” in WHO Classification of Tumours of the Central Nervous System, P. Kleihues and W. K. Cavenee, Eds., pp. 110–112, IARC Press, Lyon, France, 2007.
[6]
F. Giangaspero, G. Cenacchi, F. Roncaroli et al., “Medullocytoma (lipidized medulloblastoma): a cerebellar neoplasm of adults with favorable prognosis,” American Journal of Surgical Pathology, vol. 20, no. 6, pp. 656–664, 1996.
[7]
J. T. Bechtel, J. M. Patton, and Y. Takei, “Mixed mesenchymal and neuroectodermal tumor of the cerebellum,” Acta Neuropathologica, vol. 41, no. 3, pp. 261–263, 1978.
[8]
D. W. Ellison, S. C. Zygmunt, and R. O. Weller, “Neurocytoma/lipoma (neurolipocytoma) of the cerebellum,” Neuropathology and Applied Neurobiology, vol. 19, no. 1, pp. 95–98, 1993.
[9]
D. G. Davis, D. Wilson, M. Schmitz, and W. R. Markesbery, “Lipidized medulloblastoma in adults,” Human Pathology, vol. 24, no. 9, pp. 990–995, 1993.
[10]
C. H. Alleyne Jr., S. Hunter, J. J. Oison, and D. L. Barrow, “Lipomatous glioneurocytoma of the posterior fossa with divergent differentiation: case report,” Neurosurgery, vol. 42, no. 3, pp. 639–643, 1998.
[11]
K.-M. Fung, W. Fang, R. E. Norton, N. Tortes, A. Chu, and L. A. Langford, “Cerebellar central liponeurocytoma,” Ultrastructural Pathology, vol. 27, no. 2, pp. 109–114, 2003.
[12]
P.-W. Hsu, T.-C. Hsieh, C.-N. Chang et al., “Fourth ventricle central neurocytoma: case report,” Neurosurgery, vol. 50, no. 6, pp. 1365–1367, 2002.
[13]
B. K. Owler, J. M. Makeham, M. Shingde, and M. Besser, “Cerebellar liponeurocytoma,” Journal of Clinical Neuroscience, vol. 12, no. 3, pp. 326–329, 2005.
[14]
S. Chakraborti, A. Mahadevan, A. Govindan et al., “Supratentorial and cerebellar liponeurocytomas: report of four cases with review of literature,” Journal of Neuro-Oncology, vol. 103, no. 1, pp. 121–127, 2011.
[15]
M. T. Giordana, P. Schiffer, A. Boghi, P. Buoncristiani, and F. Benech, “Medulloblastoma with lipidized cells versus lipomatous medulloblastoma,” Clinical Neuropathology, vol. 19, no. 6, pp. 273–277, 2000.
[16]
M. C. Sharma, M. Agarwal, A. Suri, S. Gaikwad, P. Mukhopadhyay, and C. Sarkar, “Lipomedulloblastoma in a child: a controversial entity,” Human Pathology, vol. 33, no. 5, pp. 564–569, 2002.
[17]
F. Soylemezoglu, D. Soffer, B. Onol, K. Schwechheimer, and P. Kleihues, “Lipomatous medulloblastoma in adults: a distinct clinicopathological entity,” American Journal of Surgical Pathology, vol. 20, no. 4, pp. 416–418, 1996.
[18]
H. Alkadhi, M. Keller, S. Brandner, Y. Yonekawa, and S. S. Kollias, “Neuroimaging of cerebellar liponeurocytoma,” Journal of Neurosurgery, vol. 95, no. 2, pp. 324–331, 2001.
[19]
E. Anghileri, M. Eoli, R. Paterra et al., “FABP4 is a candidate marker of cerebellar liponeurocytomas,” Journal of Neuro-Oncology, vol. 108, pp. 513–519, 2012.
[20]
F. Limaiem, S. Bellil, I. Chelly et al., “Recurrent cerebellar liponeurocytoma with supratentorial extension,” Canadian Journal of Neurological Sciences, vol. 36, no. 5, pp. 662–665, 2009.
[21]
R. Kachhara, R. N. Bhattacharya, S. Nair, and V. V. Radhakrishnan, “Liponeurocytoma of the cerebellum—a case report,” Neurology India, vol. 51, no. 2, pp. 274–276, 2003.
[22]
J. T. Guan, Y. Q. Geng, Y. Cheng, Y. L. Guo, and R. L. Wu, “Magnetic resonance imaging of cerebellar liponeurocytoma. A case report and review of the literature,” Journal of Neuroradiology, vol. 25, no. 3, pp. 331–336, 2012.
[23]
F. V. Aker, S. ?zkara, P. Eren, ?. Peker, S. Arma?an, and T. Hakan, “Cerebellar liponeurocytoma/lipidized medulloblastoma. Case report and review of the literature,” Journal of Neuro-Oncology, vol. 71, no. 1, pp. 53–59, 2005.
[24]
S. Hostmann, A. Perry, G. Reifenberger et al., “Genetic and expression profiles of cerebellar liponeurocytomas,” Brain Pathology, vol. 14, no. 3, pp. 281–289, 2004.
[25]
T. Nishimoto and B. Kaya, “Cerebellar liponeurocytoma,” Archives of Pathology & Laboratory Medicine, vol. 136, pp. 965–969, 2012.
[26]
G. L. Taddei, A. M. Buccoliero, A. Caldarella et al., “Cerebellar liponeurocytoma: Immunohistochemical and ultrastructural study of a case,” Ultrastructural Pathology, vol. 25, no. 1, pp. 59–63, 2001.
[27]
F. Cacciola, R. Conti, G. L. Taddei, A. M. Buccoliero, and N. Di Lorenzo, “Cerebellar liponeurocytoma: case report with considerations on prognosis and management,” Acta Neurochirurgica, vol. 144, no. 8, pp. 829–833, 2002.
[28]
A. M. Buccoliero, A. Caldarella, S. Bacci et al., “Cerebellar liponeurocytoma: morphological, immunohistochemical, and ultrastructural study of a relapsed case,” Neuropathology, vol. 25, no. 1, pp. 77–83, 2005.
[29]
S. B. Chung, Y. L. Suh, and J. I. Lee, “Cerebellar liponeurocytoma with an unusually aggressive histopathology: case report and review of the literature,” Journal of Korean Neurosurgical Society, vol. 52, no. 3, pp. 250–253, 2012.
[30]
L. Chimelli, M. D. Hahn, and H. Budka, “Lipomatous differentiation in a medulloblastoma,” Acta Neuropathologica, vol. 81, no. 4, pp. 471–473, 1991.
[31]
A. Orlandi, B. Marino, M. Brunori, R. Greco, and L. G. Spagnoli, “Lipomatous medulloblastoma,” Clinical Neuropathology, vol. 16, no. 4, pp. 175–179, 1997.
[32]
N. Montagna, D. Moreira, L. C. Vaz, and M. Reis, “Cerebellar liponeurocytoma: a newly recognized clinico-pathological entity,” Arquivos de Neuro-Psiquiatria, vol. 60, no. 3, pp. 725–729, 2002.
[33]
A. Akhaddar, I. Zrara, M. Gazzaz, B. El Moustarchid, S. Benomar, and M. Boucetta, “Cerebellar liponeurocytoma (lipomatous medulloblastoma),” Journal of Neuroradiology, vol. 30, no. 2, pp. 121–126, 2003.
[34]
S. Elshihabi, M. Husain, and M. Linskey, “Lipomatous medulloblastoma: a rare adult tumor variant with a uniquely favorable prognosis,” Surgical Neurology, vol. 60, no. 6, pp. 566–570, 2003.
[35]
M. Amina, B. Saadia, N. Kais et al., “Cerebellar liponeurocytoma: a case report and review of the literarute,” Pathologica, vol. 95, no. 6, pp. 456–459, 2003.
[36]
C. A. Valéry, L. J. Sakka, and J. Poirier, “Problematic differential diagnosis between cerebellar liponeurocytoma and anaplastic oligodendroglioma,” British Journal of Neurosurgery, vol. 18, no. 3, pp. 300–303, 2004.
[37]
H. M. Bazarbacha, S. Nagi, W. Zouauoi, L. Belghith, R. Sebai, and S. Touibi, “Cerebellar liponeurocytoma. Case report,” Tunisie Medicale, vol. 83, no. 2, pp. 120–122, 2005.
[38]
M. Tatke and A. K. Singh, “Cerebellar liponeurocytoma—a case report,” Indian Journal of Pathology and Microbiology, vol. 48, no. 1, pp. 29–31, 2005.
[39]
N. Patel, A. Fallah, J. Provias, and N. K. Jha, “Cerebellar liponeurocytoma,” Canadian Journal of Surgery, vol. 52, no. 4, pp. E117–E119, 2009.
[40]
C. E. Chatillon, M. C. Guiot, D. Roberge, and R. Leblanc, “Cerebellar liponeurocytoma with high proliferation index: treatment options,” Canadian Journal of Neurological Sciences, vol. 36, no. 5, pp. 658–661, 2009.
[41]
N. Beizig, S. Ziadi, M. Ladib, and M. Mokni, “Cerebellar liponeurocytoma: case report,” Neurochirurgie, vol. 59, pp. 39–42, 2013.
[42]
S. Dey, M. K. Chaudhury, S. K. Basu et al., “Cerebellar liponeurocytoma,” Archives of Iranian Medicine, vol. 16, no. 3, pp. 199–200, 2013.