Choroid plexus carcinoma is a rare form of tumor which is preponderantly noted in kid . It is an intraventricular , key flighty system ( CNS ) tumour that is derived from the choroid rete epithelium . Choroid plexus carcinoma is characterise by papillary and intraventricular growth .
As per the WHO ( World Health Organization ) , choroid rete neoplasm are classified under 3 main category as follows :
Choroid plexus carcinoma is the most severe flesh of malignance among the 3 grades of choroid coat plexus tumour and thus it is important to differentiate the 3 grades of malignancy from each other for providing appropriate treatment .
Symptoms of Choroid Plexus Carcinoma
symptom of Choroid plexus carcinoma may admit :
Other signs and symptom of Choroid plexus carcinoma include :
Choroid plexus carcinoma generally affects nipper and it presents with symptoms of cerebrospinal fluid obstruction . Among the pediatric universe , the most common site of involvement is the sidelong ventricle follow by the quaternary ventricle . When this condition affects the adult universe , the pattern is reversed with the most common site of involvement being the fourth heart ventricle stick to by the 2d ventricle . It has been noted that the third ventricle is the least vulgar web site of involvement irrespective of the patient ’s historic period . In rare cases , primary spare ventricular papilloma may come about at the cerebellopontine angle . In some cases , either both the sidelong ventricles gets involved or multiple ventricle get involve .
Epidemiology of Choroid Plexus Carcinoma
Choroid plexus carcinoma is more coarse among nestling than in adult . The incidence pace of this experimental condition in all age is about 0.3 case per million . The peak incidence is within mid teenaged years . It account for less than 1 % of primary intracranial neoplasm and upto 5 % of paediatricbrain tumorsand about 20 % of those in children aged below 1 year and new . It may also have fetal and inborn relation .
The incidence proportion between choroid plexus villoma and choroid plexus carcinomas is about 5:1 . About 90 % of all choroid rete neoplasm in child are choroid rete papilloma , 70 % of which occur in children below 2 year . This condition does not have significant sexuality taste .
Prognosis of Choroid Plexus Carcinoma
The forecast for choroid plexus carcinoma is not very good as the tumour rise very rapidly and the 5 year natural selection rate is only 40 % . Among the total number of tumour , two - third of them metastasise through cerebrospinal fluid , and some malignant cells might be present in the cerebrospinal fluid even before resection is done which increases the risk of spreading of cancer . Systemicmetastasisis observe in patients who survived longer . accomplished resection of the neoplasm is the only objective which increases the chance of survival and the overall prognosis also improves . However , the prognosis is life - sinister if accomplished resection is not done .
Causes of Choroid Plexus Carcinoma
Most cases of choroid plexus carcinoma are sporadic in nature . It has been seen that patrimonial divisor play a vital use in exploitation of choroid rete villoma and carcinoma . Choroid rete papilloma is usually seen in association with Aicardi syndrome , Down syndrome , von Hippel Lindau diseaseandneurofibromatosistype 2 . Choroid plexus carcinoma can arise is affiliation with familial cancer sensitivity syndrome including Li - Fraumeni and rhabdoid sensitivity syndrome with familial mutation of TP53 and hSNF5 / INI1 / SMARCB1 , respectively .
Diagnosis of Choroid Plexus Carcinoma
diagnosing of choroid plexus carcinoma may affect the following procedures :
Treatment of Choroid Plexus Carcinoma
intervention sensory system are design to promote physical and psychological well - being and may differ based on the watch over component :
The most commonly used treatment modalities are :
Other fashion of discourse may demand radiation therapy , acupuncture , massages , dietary changes etc .
Choroid plexus carcinoma is a fast growing malignant condition and is very severe in nature . The prognosis is not very bright as 5 twelvemonth selection rate is only 40 % along with gamey risk of systemic metastasis in somebody who live longer . The only way to improve the prospect is by completely resecting the neoplasm . It is a potentially lifespan - threatening shape and hence , one must not delay in essay aesculapian attending for shaver in case any of the symptom are follow .
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