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CASE REPORT Table of Contents   
Year : 2009  |  Volume : 52  |  Issue : 1  |  Page : 108-109
Central neurocytoma in the vermis of the cerebellum


1 Department of Pathology, Gandhi Medical College, Bhopal - 462 001, India
2 Department of Surgery, Gandhi Medical College, Bhopal - 462 001, India

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   Abstract 

In neuropathology, anatomic landmarks and topographic relationships bear particularly on issues of differential diagnosis. A case of central neurocytoma of the vermis of the cerebellum is being reported in this article. A 45-year-old male with features of hydrocephalous and a posterior fossa space-occupying lesion was diagnosed as having central neurocytoma upon a microscopy of Hematoxylene and Eosin stained sections. The diagnosis was confirmed by a positive immunohistochemical reaction for Neuron Specific Enolase (NSE) and Synaptophysin. This case report is being made to emphasize that a high index of suspicion is required even for reporting neuropathology where the site does not match the conventional location of the lesion seen and also to emphasize the importance of immunohistochemistry in proving such unusually located lesions

Keywords: Cerebellum, central neurocytoma, perinuclear clearing

How to cite this article:
Kapoor N, Gandhi A, Chaurasia A K. Central neurocytoma in the vermis of the cerebellum. Indian J Pathol Microbiol 2009;52:108-9

How to cite this URL:
Kapoor N, Gandhi A, Chaurasia A K. Central neurocytoma in the vermis of the cerebellum. Indian J Pathol Microbiol [serial online] 2009 [cited 2019 Dec 11];52:108-9. Available from: http://www.ijpmonline.org/text.asp?2009/52/1/108/44989



   Introduction Top


The cerebellum of the brain is located infratentorially in the posterior fossa of the cranium.[1] The central part of the cerebellum is called the vermis, which connects the left and right side of the cerebellum. It forms the roof of the fourth ventricle. [2] The hemispheric cerebellum, vermian and fourth ventricular masses can usually be differentially diagnosed as pilocytic astrocytoma (in the 5-15 year age group), hemangioblastoma (in the 35-45 year age group), posterior fossa cyst, ependymoma and choroid plexus papilloma. [3] We are reporting a case of central neurocytoma of the vermis of the cerebellum. Central neurocytoma is in itself a rare and distinctive tumor described by Hassoun, et al. in 1982. [4] This tumor comprises of less then 1% of all brain tumors [5] and is usually found in the lateral or third ventricle. [6]


   Clinical Summery Top


A 45-year-old male presented to neurosurgery out patient department with a complaint of fever and sporadic vomiting over the past 2 months. He also had a persistent headache for past 8 days. He was diagnosed as having hydrocephalus. Routine hematological and biochemical investigations were performed, as well as a magnetic resonance imaging (MRI). Hematological and biochemical investigations were found to be within normal limits. The MRI findings suggested a space-occupying lesion in the posterior fossa. The patient was operated upon and a space-occupying lesion was identified in the vermis of the cerebellum measuring approximately 2x0.5 cm. It was surgically excised completely. The material was sent for a histopathology examination. The patient was discharged on postoperative recovery and was doing well on the first follow-up.

Histopathological features

The tissue was received and fixed in 10% buffered formalin. It was routinely processed and Hematoxylene and Eosin (H and E) stained sections were prepared for microscopy. Under light microscope, the sections revealed sheets of monomorphic cells embedded in a delicate fibrillary matrix. The nuclear contours were surrounded by a perinuclear clearing. A branching pattern of thin walled capillaries and a collection of micro calcifications were seen [Figure 1]. A diagnosis of central neurocytoma was made, which was confirmed immunohistochemically by a positive reaction to neuron specific enolase (NSE) and synaptophysin.


   Discussion Top


Although central neurocytoma is a relatively new tumor entity, it is important to explore all the locations of occurrence of these tumors for proper management. This is because these tumors have a relatively favorable prognosis and their current treatment of choice is complete surgical removal without adjuvant chemotherapy or radiotherapy. [7] Since the establishment of the designation of central neurocytoma, some reports of this tumor have been published [7],[8],[9] but the preferred locations of the tumor in all of the reports have been the third or lateral ventricle of the brain. In an Indian study of twenty cases it was found that 50% of the tumors were in the lateral ventricle. [7] It is hypothesized that the usual location of central neurocytoma may be anywhere within the ventricular confines, possibly because the tumor derives from remnants of the subependymal matrix that retains prenatal proliferative capacity.[10] This may be correct because embryologically the cerebellum develops from the dorsolateral part of the alar lamina of the metencephalon. The developing cerebellum can be divided into an intra ventricular part and an extra ventricular part. During the later stage of embryonic development, the extra ventricular part becomes much larger then the intraventricular part [2] and thus subependymomal remnants sometimes being retained in cerebellum is a possibility.

If we take a correlative look at the above anatomical facts and at the predicted pathogenesis of the central neurocytoma, there would appear to be a strong likelihood of quite a few previously diagnosed cerebellar tumors in the pre immunohistochemistry era of being central neurocytoma. Such lesions may have been misdiagnosed or confused with other common cerebellar lesions under the light microscope with only H and E staining. Especially confusing would be the cerebellar abscess, which shows mononuclear cells, as well as calcification and medulloblastoma, which commonly presents with hydrocephalous and is usually found in the midline of the cerebellum in the younger age group. These entities require a different management protocol then central neurocytoma. So a misdiagnosed lesion would mean misguided management protocol and would result in undue morbidity and mortality to the patient.

 
   References Top

1.Voogd J. Nervous system. In: William PL, editor. Gray's anatomy. 38th ed. New York: Churchill Livingstone; 1995. p. 1028-9.  Back to cited text no. 1    
2.Singh I, Pal GP. Human embryology. 8th ed. India: MacMillan; 2007.  Back to cited text no. 2    
3.Available from: http://rad.usuhs.mil/index.html. cited 2008 May 9   Back to cited text no. 3    
4.Hassoun J, Gambarelli D, Grisoli F, Pellet W, Salamon G, Pellisier JF, et al . Central neurocytoma. An electron microscopic study of two cases. Acta Neuropathol 1982;56:151-6.  Back to cited text no. 4    
5.Rajesh LS, Jain D, Radotra B, Banerjee AK, Khosla VK, Vasishta RK. Central neurocytoma: A clinico-pathological study of eight cases. Indian J Pathol Microbiol 2006;49:543-5.  Back to cited text no. 5  [PUBMED]  
6.Townsend JJ, Seaman JP. Central neurocytoma: A rare benign intraventricular tumour. Acta Neuropathol 1986;71:167-70.  Back to cited text no. 6  [PUBMED]  
7.Sharma MC, Sarkar C, Karak AK, Gaikwad S, Mahapatra AK, Mehta VS. Intraventricular neurocytoma: A clinicopathological study of 20 cases with review of literature. J Clin Neurosci 1999;6:319-23.   Back to cited text no. 7  [PUBMED]  [FULLTEXT]
8.Schid SF, Scheithauer BW, Haddock MG, Schiff D, Burger PC, Wong WW, et al . Central neurocytoma. Cancer 1997;79:790-5.  Back to cited text no. 8    
9.Figarella Branger D, Pellisier JF, Daumas-Duport C, Delisle MB, Pasquier B, Parent M, et al . Central neurocytoma: Critical evaluation of a small cell neuronal tumor. Am J Surg Pathol 1992;16:97-109.  Back to cited text no. 9    
10.Rosenblum MK, Bilbao JM, Ang LC. Neuromuscular System. In: Rosai J, editor. Surgical pathology. 9th ed. Missouri: Mosby; 2004. p. 2545.  Back to cited text no. 10    

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Correspondence Address:
Neelkamal Kapoor
E-6/14 Arera Colony, Bhopal 462 016
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.44989

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    Figures

  [Figure 1]

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    Abstract
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