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Choroid Plexus Papilloma
Prem Sheth*, Rahul Verma**, Ryan Dias***
 
Choroid plexus papillomas are one of the rare intracranial tumours, benign in nature and seen more in childhood than in adult life. We present below one such rare case.
 
Introduction
Choroid plexus papillomas are one of the rare intracranial tumours. They are benign in nature. They are seen more in childhood than in adult life. They originate from the tufts of villi within the ventricular system which normally produce cerebrospinal fluid. They have a varied mode of presentation. Radioimagining is a very good and accurate tool to their diagnosis. Their management requires the use of medical therapies in addition to the preferred surgical removal of the tumour. Certain cases are known to have residual complications with poor neuropsychological outcome.
 
Case Report

A 3 year old child with no prior illness in the past sustained a fall (trivial in nature) following which she had vomiting, was admitted for head injury in a hospital. CT scan was done, it revealed - a choroid plexus papilloma in the body and trigone of the left lateral ventricle and a uncompensated communicating hydrocephalus.

Three days later the patient was admitted in hospital for the same. The child had complaints of headache and the fundus examination revealed gross papilloedema. An MRI done revealed a well defined lesion within the posterior left lateral ventricle suggestive of a choroid plexus papilloma with associated periventricular ooze.

Surgery was done for the removal of the tumour. “Left parieto occipital craniotomy with complete excision of the left trigonal papilloma (endoscopically assisted) with the insertion of an omaya reservoir”. Postoperatively the child was doing well with no complaints of headache or irritability, the skin flap was flush with the surrounding skin. The CT scan showed complete resection of the lesion. The patient was discharged on phenytoin, acetazolamide and sodamint.

Few days following the discharge the child had complaints of vomiting, excessive crying and swelling of the skin flap. CT scan done revealed hydrocephalus with periventricular ooze and haemorrhage in the ventricle. The child was readmitted because of increasing complaints and aspiration from both the swollen skin flap and the omaya reservoir was done. Patient improved and was discharged. Thereafter weekly aspirations were done till the haemorrhage in the ventricle decreased. Patient was admitted again 3 weeks later and in view of increasing hydrocephalus a ventriculo peritoneal shunt inserted. Thereafter patient was well.

 

Fig. 1a

Fig. 1b
 

Fig. 2

Fig. 1c
Figs. 1a,b,c : Pre operative MRI shows a well defined intensely enhancing cauliflower like lesion within the posterior left lateral ventricle suggestive of choroid plexus papilloma. There is associated hydrocephalus with periventricular ooze.
1. Sagittal view, 2. Coronal view, 3. Axial view
Fig. 2 : Post operative CT scan - choroid plexus papilloma excised, some amount of ventricular distortion with moderate hydrocephalus, decrease in the periventricular ooze with some amount of iv blood noted, intraventricular shunt noted.
 
Discussion

Choroid plexus tumours account for 0.4 to 1% of all intracranial tumours. 70% of these present before the age of 2 years. They may present in the form of hydrocephalus and raised intracranial pressure or seizures or sub arachnoid haemorrhage or focal neurological deficits. Hydrocephalus, the more commoner presentation is due to increased production of cerebrospinal fluid or from blockage of the normal drainage pathways.

Some theories state that they are congenital in origin while others state that they are related to infections with simian vacuolating virus (sv40). In children they normally present in the lateral ventricle whereas in adults they are normally infratentorial. A male to female ratio of 2.8:1 has been noted in studies. Most are histologically benign (papilloma) whereas a malignant form (carcinoma) can also exist.

Histologically a choroid plexus papilloma is a neuroepithelial lined papillary projection of the ventricular ependyma, it has a core of fibrovascular tissue lined with low cuboidal neuroepithelium.

The management of such papilloma is surgical removal of the tumour in toto. Analysis and studies have revealed the benefit of gross total resection. Just shunting without resection is not good enough. Surgery normally has good results but at times multiple surgeries may be needed for complete excision. Total removal of the tumour may not cure the hydrocephalus which may be due to high CSF protein, haemorrhage from tumour or surgery and ependidymitis. Nowadays in addition to surgery use of medical therapy (with drugs like acetazolamide) in combination is found to have good results. Complications which may be neurological or psychological are known to influence the outcome.

 
References
1. Cheryl Palmer, et al. Update on cpp. Emedicine 2003.
2. Buxton N, Punt J. Choroids plexus papilloma producing symptoms. Paed Neurosurgery 1997; 27 (2) : 108-11.
3. Fitzpatrick LK, Aronson LJ, Cohen KJ. Is there requirement of adjunct therapy for CPP that is completely resected? J Neuroncol 2002; 57 (2).
4. Huang H, Reis R, Yonekawa Y. Identification of human brain tumours of DNA sequences specific for sv40. Brain Pathol 1999; 9 (1) : 33-34.


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