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   Abstract
   Introduction
   Case Report
   Discussion
   Conclusion
   References
   Article Figures

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CASE REPORT
Year : 2009  |  Volume : 57  |  Issue : 4  |  Page : 486-488

Choroid plexus papilloma presenting as a non-contrast-enhancing fourth ventricular mass in a child


1 Departments of Neurological Sciences, Christian Medical College, Vellore, India
2 Department of Pathology, Christian Medical College, Vellore, India

Date of Acceptance26-Apr-2009
Date of Web Publication10-Sep-2009

Correspondence Address:
Vedantam Rajshekhar
Department of Neurological Sciences, Christian Medical College, Vellore - 632 004
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.55601

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 ╗ Abstract 

Choroid plexus papilloma (CPP) is a rare benign tumor of the central nervous system with a propensity for location within the lateral ventricle in children. We report a case of a 14-year-old girl who presented with transient facial paresis and ataxia. Her imaging showed a non-enhancing intra fourth ventricular mass, the histology of which was reported as CPP. The atypical clinical and radiological features in this case are discussed. Choroid plexus papillomas should be considered in the differential diagnosis of non-enhancing fourth ventricular masses.


Keywords: Choroid plexus papilloma, computed tomography, fourth ventricle, magnetic resonance imaging


How to cite this article:
Pratheesh R, Moorthy RK, Singh R, Rajshekhar V. Choroid plexus papilloma presenting as a non-contrast-enhancing fourth ventricular mass in a child. Neurol India 2009;57:486-8

How to cite this URL:
Pratheesh R, Moorthy RK, Singh R, Rajshekhar V. Choroid plexus papilloma presenting as a non-contrast-enhancing fourth ventricular mass in a child. Neurol India [serial online] 2009 [cited 2021 Jun 20];57:486-8. Available from: https://www.neurologyindia.com/text.asp?2009/57/4/486/55601



 ╗ Introduction Top


Choroid plexus papillomas (CPP) are benign intraventricular tumors derived from the choroid plexus epithelium. These brilliantly contrast-enhancing tumors occur more commonly in children and in this age group the location is predominantly in the lateral ventricle. [1] This report highlights the unusual clinical and radiological findings in a patient who was diagnosed to have a CPP.


 ╗ Case Report Top


A 14-year-old girl presented three months after an episode of transient left-sided facial paresis and imbalance while walking that had lasted for ten days. She was asymptomatic at the time of admission. She did not have any neurological deficits. Fundus examination was normal. Computed tomography (CT) showed a non-contrast-enhancing hypodense intra fourth ventricular mass. There were no calcifications [Figure 1]. Magnetic resonance imaging (MRI) of the brain showed a hypointense fourth ventricular mass on short TR sequences that did not show enhancement after administration of gadolinium [Figure 2]. There was no hydrocephalus. She underwent radical excision of the mass and she made an uneventful recovery. During surgery the mass was found to have a small attachment to the floor of the fourth ventricle. The histopathology showed a tumor composed of papillary structures lined by cuboidal epithelium with regular, rounded centrally placed nuclei with no evidence of mitosis, necrosis or anaplasia. This was reported as CPP [Figure 3]. Contrast CT did not show any residual lesion at 16 months follow-up and she was asymptomatic [Figure 4].


 ╗ Discussion Top


Clinical features of choroid plexus papillomas

Choroid plexus papillomas account for 0.4-0.6% of all brain tumors and in children they account for 2-4% of brain tumors. [1] The reported annual incidence is 0.3 per 1,000,000 population. [2] The most common site of origin in the pediatric age group is within the atria of the lateral ventricles, whereas fourth ventricular tumors are more common in adults. [1] The commonest presentation is with raised intracranial pressure as a consequence of block of cerebrospinal fluid (CSF) pathways and/or overproduction of CSF. However, these tumors can have varied presentations including cerebellar dysfunction and cranial nerve involvement like hearing loss if it extends into the subarachnoid space. [3] Our patient had a transient left facial paresis that improved without any medical or surgical intervention, and she was asymptomatic at presentation to us.

Radiological features

On CT imaging, CPPs have been reported to be iso- to hyperdense and intensely contrast-enhancing with cystic areas and spotty calcifications. [4],[5] The MRI characteristics of these tumors have been described in literature as iso to hypointense in short TR sequences, variably hyperintense in long TR sequences and commonly associated with flow voids. [4],[5],[6],[7] The most important characteristic is the intense contrast enhancement which is homogeneous in most cases. Inhomogeneous enhancement has also been reported in a few cases.[6] A literature search revealed only one case of non-enhancing CPP, reported by Vasquez et al.[7] Imaging in our patient showed a hypodense fourth ventricular lesion with no areas of calcification and no enhancement after contrast administration.

Pathology

Macroscopically, these tumors are well-circumscribed cauliflower-like masses and may be adherent to the ventricular wall. At surgery, these tumors usually are free from the floor of the fourth ventricle except in rare instances. [8] There have been reports of CSF seeding in these benign tumors. [8] In our patient, the tumor was found to be adherent to the floor of the fourth ventricle and this was another atypical feature. Gross total excision is the treatment of choice and the reported five-year survival is 100%. [9] The presence of mitoses, less than 50% of the tumor cells heavily positive for S100, absence of TTR(transthyretin)-positive cells, brain invasion by cell nests, absence of marked stromal edema, and presence of necrotic areas have shown to correlate with a poor prognosis. [10]


 ╗ Conclusion Top


Choroid plexus papilloma is a benign tumor seen in children and has a good prognosis. Although known to brilliantly enhance with contrast, a non-enhancing variant is present and should be included in the differential diagnoses for non-enhancing fourth ventricular masses in children.

 
 ╗ References Top

1.Aguzzi A, Brandner S, Paulus W. Choroid plexus tumours. In: Kleihues P, Cavenee WK, editors. Pathology and genetics of tumours of the nervous system. Lyon, France: IARC; 2000. p. 84-6.  Back to cited text no. 1    
2.Jänisch W, Staneczek W. Primary tumors of the choroid plexus. Frequency, localization and age. Zentralbl Allg Pathol 1989;135:235-40.  Back to cited text no. 2    
3.van Swieten JC, Thomeer RT, Vielvoye GJ, Bots GT. Choroid plexus papilloma in the posterior fossa. Surg Neurol 1987;28:129-34.  Back to cited text no. 3    
4.Coates TL, Hinshaw DB Jr, Peckman N, Thompson JR, Hasso AN, Holshouser BA, et al. Pediatric choroid plexus neoplasms: MR, CT, and pathologic correlation. Radiology 1989;173:81-8.  Back to cited text no. 4    
5.Okuyama T, Sohma T, Tsuchita H, Kitami K, Kohama I, Saito K. Magnetic resonance imaging characteristics of choroid plexus papilloma in the fourth ventricle. Neurol Med Chir (Tokyo) 1995;35:442-4.  Back to cited text no. 5    
6.Talacchi A, De Micheli E, Lombardi C, Turrazi S, Bricolo A. Choroid plexus papillomas of the cerebellopontine angle: A twelve patient series. Surg Neurol 1999;51:621-9.  Back to cited text no. 6    
7.Vazquez E, Ball WS Jr, Prenger EC, Castellote A, Crone KR. Magnetic resonance imaging of fourth ventricular choroid plexus neoplasms in childhood. A report of two cases. Pediatr Neurosurg 1991-1992;17:48-52.  Back to cited text no. 7    
8.Jinhu Y, Jianping D, Jun M, Hui S, Yepeng F. Metastasis of a histologically benign choroid plexus papilloma: Case report and review of the literature. J Neurooncol 2007;83:47-52.  Back to cited text no. 8    
9.Pencalet P, Sainte-Rose C, Lellouch-Tubiana A, Kalifa C, Brunelle F, Sgouros S, et al. Papillomas and carcinomas of the choroid plexus in children. J Neurosurg 1998;88:521-8.  Back to cited text no. 9    
10.Paulus W, Jänisch W. Clinicopathologic correlations in epithelial choroid plexus neoplasms: A study of 52 cases. Acta Neuropathol 1990; 80:635-41.  Back to cited text no. 10    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

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