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 »  Introduction
 »  Case report
 »  Discussion
 »  References

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Year : 1999  |  Volume : 47  |  Issue : 1  |  Page : 71-3

Choroid plexus papilloma of cerebellopontine angle with extension to foramen magnum.


Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Science, Lucknow, Uttar Pradesh, India.

Correspondence Address:
Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Science, Lucknow, Uttar Pradesh, India.

  »  Abstract

A case of choroid plexus papilloma resembling meningioma of cerebellopontine (CP) angle with its extension to foramen magnum is presented. Occurrence of this tumour in CP angle is very rare. Its extension towards foramen magnum is further rare. It was a real diagnostic enigma preoperatively as the tumour was resembling meningioma upto some extent on radiological study. Retromastoid craniectomy with microsurgical excision of tumour and its extension was achieved in toto. Tumour was attached to few rootlets of lower cranial nerves which were preserved. Attachment of the tumour with lower cranial nerves again caused diagnostic confusion with neurofibroma intraoperatively.

How to cite this article:
Kumar R, Jain V K, Krisnani N. Choroid plexus papilloma of cerebellopontine angle with extension to foramen magnum. Neurol India 1999;47:71


How to cite this URL:
Kumar R, Jain V K, Krisnani N. Choroid plexus papilloma of cerebellopontine angle with extension to foramen magnum. Neurol India [serial online] 1999 [cited 2021 Jul 25];47:71. Available from: https://www.neurologyindia.com/text.asp?1999/47/1/71/1654




   »   Introduction Top


Choroid plexus papillomas are benign slow growing tumours of neuroectodermal origin. Their incidence among all intracranial neoplasms at all ages varies between 0.4 to 1%.[1],[2] The incidence amongst the children is, however, 1.5% to 4% with peak in the first two years of life.[2],[3] Lateral ventricle in children and fourth ventricle in adults is the most common site of this tumour.[2],[4],[5] The origin of choroid plexus papilloma in third ventricle and cerebellopontine angle is extremely rare.[6] Generally these tumours manifest with featrues of raised intracranial pressure due to concomitent hydrocephalus. By virtue of their benign nature and intraventricular localisation these are amenable to total excision. A case of choroid plexus papilloma arising in the cerebellopontine angle, extending down upto foramen magnum and its attachment to lower cranial nerves is described for its rarity.


   »   Case report Top


A 40 years male presented with one year history of mild to moderate holocranial headache which increased in frequency and intensity during the last 4 months of his illness. Headache was later associated with vomiting. He developed unsteady gait, swaying to right side, occasional diplopia on left lateral gaze and hoarseness of voice. On examination there was bilateral papilloedema, bilateral 6th nerve paresis, horizontal gaze nystagmus and right cerebellar signs. Laryngitis and a vocal cord nodule was found on throat examination. Routine investigations were normal. Contrast CT head showed hyperdense enhancing rounded large lesion in right cerebellopontine angle. Fourth ventricle was compressed and shifted to left. Hydrocephalus was present. Tumour was resembling meningioma. On MRI, the mass was seen to be extending upto foramen magnum. It measured 5.5 x 5.0 x 4.0 cm, and was hypointense on TI and (heterogenously) hyperintense on T2 weighted images with cystic component encircling the tumour inferiorly. Right CP angle cistern was dilated and brain stem was compressed by the tumour. Fourth ventricle was also compressed and shifted towards the left side [Figure 1][Figure 2]. Right 7th and 8th nerve complex could be made out separately from mass. Audiometry revealed bilateral mild sensorineural deafness.

Brain stem evoked potential showed prolonged interpeak latencies between wave 1 to 3 and 3 to 5. The tumour was approached by right retromastoid craniectomy with provisional diagnosis of CP angle meningioma. On exploration there was soft to firm partially suckable vascular tumour with well defined capsule, good arachnoid plane and arachnoid cap. It was attached with the rootlets of lower cranial nerves. Total microsurgical excision of tumour was achieved with preservation of 7th, 8th and lower cranial nerve. Histology revealed multiple arborizing papillae having central fibrovascular core, lined by cubo columnar cells [Figure 3]Papillae were oedematous at places. A histological diagnosis of choroid plexus papilloma was made.Hoarseness increased transiently post operatively but improved on conservative management. Patient developed meningitis 20 days after the surgery which was managed following readmission.


   »   Discussion Top


Lateral ventricle is the most frequent site of choroid plexus papilloma in children,[1],[2],[7] while in adults it is the fourth ventricle.[4],[5] In 57.1% children the tumour has been reported to be localized in lateral ventricle while in 66.6% of adults it is infratentorially situated.[1] Localization in third ventricle and CP angle is quite rare.[1],[6],[7],[8] Choroid plexus papilloma can arise directly from choroid plexus of foramen of Luschka, by a direct extension from fourth ventricle or through CSF dissemination. Rovit et al reported 234 cases of choroid plexus papillomas of which 17 were found in CP angle.[9] In the present case, tumour was situated in right CP angle and extended upto foramen magnum with significant brain stem compression. Fourth ventricle was also compressed and shifted towards left side.

Most patients with choroid plexus papilloma develop hydrocephalus because of either over production of CSF by tumour or by obstruction of CSF pathway, especially when the tumour is located in third or fourth ventricle.[1],[2],[5] Spontaneous microhaemorrhages with secondary thickening of basal arachnoid and ependymitis is another accepted mechanism to explain bilateral ventricular dilatation despite a unilateral lesion or persistence of hydrocephalus once the tumour has been completely removed.[1],[2] Hydrocephalus, in the present case, was secondary to compression of fourth ventricle and brain stem. There was neither clinical nor pathological evidence of microhaemorrhages. In infratentorial location, symptoms and signs of cerebellar or brain stem dysfunction can be present, the symptomatology may differ in adults and children. Tacconi et al found headache to be most common symptom in both paediatric and adults age groups with papilloedema being the main clinical sign in adults (63.1%) and unsteady gait in children (71.4%).[1] The present case presented with features of raised intracranial pressure (i.e. headache, vomiting and papilloedema) and brain stem dysfunction, however, there was no involvement of seventh and eight cranial nerves either clinically or on surgical exploration despite the location of tumour in CP angle.

CT scan reveals choroid plexus papilloma to be a well defined lobulated lesion with irregular borders, typical appearence of cauliflower and hypo or iso dense lesion with marked enhancement on contrast. Calcification may be present in 4 to 10% cases. MRI reveals a hypointense lesion on Tl weighted image and iso to hypo intense lesion on T2 weighted images. There is marked enhancement on contrast.[2],[5]

It is important to evaluate preoperatively the position, size and vascularity of the tumour by angiogram and degree of hydrocephalus (Tacconi et al 1996). On microsurgical excision the tumour was found attached with rootlets of lower cranial nerves which caused the diagnostic confusion with neurofibroma intraoperatively. Microscopically these are soft, grey, pink vascular tumours with rough and irregular borders. From histology point of view they resemble the normal choroid plexus i.e. fibrovascular stroma covered by unique layer of well differentiated cuboidal cells with round and basal nucleus.[10]

The morbidity and mortality of surgical treatment of choroid plexus papilloma has drastically decreased in the last 10-15 years to 8.6% and 4.3% respectively. This is due to improvement of microsurgical and anaesthetic techniques as well as better neuroimaging.[1] Prognosis in choroid plexus papilloma is very good.

 

  »   References Top

1.Tacconi L, Delfini R, and Cantore G : Choroid plexus papillomas, consideration of surgical series of 33 cases. Acta Neurochir 1996; 138 : 802-810.   Back to cited text no. 1    
2.Tanaka Y, Sekido K. Oltsubo Y : Choroid plexus papillomas of third ventricle in infant, Childs Nerv Syst 1995; 664-666.   Back to cited text no. 2    
3.Donald M Ho, Tai Tong Wong and Hing Chang Lin : Choroid plexus tumours in childhood. Nervous System 1991; 7 : 437-441.   Back to cited text no. 3    
4.Bohn E, Strang R : Choroid plexus papilloma. J Neurosurg 1961; 18 : 493-500.   Back to cited text no. 4    
5.Coates TL, Hinshaw DB, Peckman N, : Paediatric choroid plexus neoplasm MR. CT and pathological correlation. Radiology 1989; 173 : 81-88.   Back to cited text no. 5    
6.Harsh GR, Wilson CB : Neuroepithelial tumors of adult brain, In Neurological surgery, Julian R. Yoman, (Eds.) Third edition WB Sounders Company, Montreal, vol 5 1990; 3103-3105.   Back to cited text no. 6    
7.Fortuna A, Celli P, Ferrante L et al : A review of papillomas of the third ventricle : one case report. J Neurosurg Sci 1979; 23 : 61-76.   Back to cited text no. 7    
8.Piquet V, De Tribolet N : Choroid plexus papilloma of the cerebellopontine angle presenting as a subarachnoid haemorrhage : case report. Neurosurgery 1984; 15 : 114-116.   Back to cited text no. 8    
9.Rovit RL, Schechter MM, and Chodroft P : Choroid plexus papilloma, observation on radiographic diagnosis, AJR 1970; 110 : 608-617.   Back to cited text no. 9    
10.Coffin CM, Wick MR, Braun JT et al : Choroid plexus neoplasms : clinicopathological and immuno-histo-chemical studies. Am J Surg Pathol 1986; 10 : 394-404.   Back to cited text no. 10    

 

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