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Table of Contents    
LETTER TO EDITOR
Year : 2014  |  Volume : 62  |  Issue : 4  |  Page : 438-439

Primary cerebellopontine angle choroid plexus papilloma in a child: A rare occurrence


1 Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi, India
2 Department of Neuropathology, All India Institute of Medical Sciences, New Delhi, India

Date of Web Publication19-Sep-2014

Correspondence Address:
Rajinder Kumar
Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi
India
Rajinder Kumar
Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.141226

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How to cite this article:
Prasad G L, Kumar R, Suri V, Prasad G L, Kumar R, Suri V. Primary cerebellopontine angle choroid plexus papilloma in a child: A rare occurrence. Neurol India 2014;62:438-9

How to cite this URL:
Prasad G L, Kumar R, Suri V, Prasad G L, Kumar R, Suri V. Primary cerebellopontine angle choroid plexus papilloma in a child: A rare occurrence. Neurol India [serial online] 2014 [cited 2019 Aug 23];62:438-9. Available from: http://www.neurologyindia.com/text.asp?2014/62/4/438/141226


Sir,

Choroid plexus papillomas (CPP) are one of the commonest childhood intraventricular tumors. [1] Primary CPP of the cerebellopontine (CP) angle is very rare. [2],[3],[4] We report a young child with primary CPP in the CP angle.

An 8-year-old boy presented with 5-months duration of diplopia, facial asymmetry, gait imbalance, left-sided hearing loss along with features of raised intracranial pressure. Neurological examination revealed a visual acuity of 6/12 bilaterally with left-sided abducent nerve paresis, grade 3 lower motor facial palsy, sensorineural hearing loss, and cerebellar signs. Magnetic resonance imaging (MRI) revealed an extra-axial 4.2 Χ 3.8 Χ 3 cm sized left CP angle lesion isointense and hyperintense on T1 and T2-weighted sequences, respectively, homogenous enhancement with compression of brainstem and moderate ventriculomegaly. Non-contrast computed tomography (NCCT) head revealed no evidence of calcification, hemorrhage, or adjacent bony changes [Figure 1]a-c. Patient underwent retrosigmoid approach and gross total removal of the tumor. Intra-operatively, the tumor was extra-axial, reddish grey, highly vascular with finger-like projections appearing to arising from the choroid plexus of foramen of Lushka. The tumor was, however, adherent to the adjacent cranial nerves. Complete total excision was achieved. Follow-up MRI showed no residual lesion [Figure 1]d. Histology showed features of papillary tumor with smooth-surfaced papillae lined by single layer of uniform columnar cells having round to oval monomorphic nuclei without mitotic activity or necrosis. Immunohistochemical staining for pancytokeratin, S-100 protein and synaptophysin was positive, while epithelial membrane antigen (EMA) staining was negative. These findings were suggestive of choroid plexus papilloma [Figure 2].
Figure 1: Axial MR images showing the lesion to be isointense on T1 WI; (a), heterogeneously hyperintense on T2WI; (b) and showing intense homogeneous enhancement; (c); post-op MRI with contrast 3 months after surgery showing no residual lesion (d)

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Figure 2: H and E photomicrographs at low (×100) magnifi cation (Fig 2) showing a papillary tumor with smooth-surfaced papillae lined by single layer of uniform columnar cells having round to oval monomorphic nuclei with no mitotic activity or necrosis suggestive of choroid plexus papillom

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CPP are located in the CP angle in approximately 9% of cases and is extremely rare in children with only three cases reported. [2],[3],[4] Bochdalek's basket, the extension of fourth ventricular choroid plexus through the foramen of Lushka into the CP angle has been postulated to be the site of origin. They may also arise primarily in the CP angle from embryonic remnant of choroid plexus anlage. [1] Other explanations include direct extension from a primary intraventricular papilloma and seeding along CSF pathways. MRI shows a well-demarcated extra-axial mass lesion possessing frond-like projections and showing intense enhancement with no parenchymal invasion. These lesions are hypo- to isointense on T1 and iso- to hyperintense on T2-weighted sequences. Hydrocephalus is either because of over production of cerebrospinal fluid (CSF), fourth ventricular obstruction or spontaneous micro-hemorrhages with secondary thickening of basal arachnoid and ependymitis. [1] Role of angiography has waned with the advent of MRI but has been suggested that enlargement of AICA and its branches in a case of a CP angle tumor is pathognomonic of CPP. Grossly, they are capsulated, soft, reddish-pink, friable tumors with cauliflower-like appearance. They resemble normal choroid plexus, although with minimal/no atypia. Immunohistochemically, they show strong positivity for cytokeratin and CK7. Vimentin, S-100 protein and synaptophysin are also strongly expressed in all cases. [1]

These tumors are benign and carry an excellent prognosis. Surgical resection is the treatment of choice. [1] A standard retrosigmoid approach would suffice in most cases. In the CP angle region, these papillomas are often adhered to the brainstem or the lower cranial nerves. In cases in which these characteristics preclude complete removal, safe surgical resection should be the goal. Pre-op embolization might be an option to secure the main feeder. [5] Adjuvant treatment is not of much benefit; however, there are anecdotal reports of CPP responding to these modalities. [6]

To conclude, primary choroid plexus papillomas of the cerebellopontine angle are extremely rare tumors. They should be always considered as remote differentials in tumors of the CP angle, especially in children.

 
  References Top

1.Jaiswal S, Vij M, Mehrotra A, Kumar B, Nair A, Jaiswal AK, et al. Choroid plexus tumors: A clinico-pathological and neuro-radiological study of 23 cases. Asian J Neurosurg 2013;8:29-35.  Back to cited text no. 1
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2.Hammock MK, Milhorat TH, Breckbill BL. Primary choroid plexus papilloma of the cerebellopontine angle presenting as brainstem tumour in a child. Childs Brain 1976;2:132-42.  Back to cited text no. 2
    
3.Piguet V, de Tribolet N. Choroid plexus papilloma of the cerebellopontine angle presenting as subarachnoid hemorrhage: Case report. Neurosurgery 1984;15:114-6.  Back to cited text no. 3
    
4.Spallone A, Pastore FS, Hagi Mao M. Choroid plexus papillomas of the cerebellopontine angle in a child. Ital J Neurol Sci 1986;7:613-6.  Back to cited text no. 4
    
5.Do HM, Marx WF, Khanam H, Jensen ME. Choroid plexus papilloma of the third ventricle: Angiography, preoperative embolization, and histology. Neuroradiology 2001;43:503-6.  Back to cited text no. 5
    
6.Kim IY, Niranjan A, Kondziolka D, Flickinger JC, Lunsford LD. Gamma knife radiosurgery for treatment resistant choroid plexus papillomas. J Neurooncol 2008;90:105-10.  Back to cited text no. 6
    


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