====== Choroid plexus papilloma ======

Choroid [[plexus papilloma]]s (CPP) are benign brain tumors.

These highly vascular tumors retain the physiological function of choroid plexus and thus lead to overproduction of cerebrospinal fluid (CSF), besides obstructing the CSF pathway. 

====Epidemiology====
The overall incidence is less than 1% of all intracranial tumors. 

They are predominantly located in the [[lateral ventricle]]s in children, and in the [[fourth ventricle]] in adults.

They occur in the lateral ventricle in most cases, but have also been described in the third or fourth ventricles
((Severino M, Schwartz ES, Thurnher MM, Rydland J, Nikas I, Rossi A. Congenital 
tumors of the central nervous system. Neuroradiology. 2010 Jun;52(6):531-48. doi:
10.1007/s00234-010-0699-0. Epub 2010 Apr 29. PubMed PMID: 20428859.
))

Most of these tumors occur in the [[lateral ventricle]]s in neonates. 

[[Third ventricle]] location is uncommon, limited to a few case reports. 


Tumors of the choroid plexus have been described previously in the fetus
((Romano F, Bratta FG, Caruso G, Naro ED, Serio R, Resta M, Loizzi P. Prenatal
diagnosis of choroid plexus papillomas of the lateral ventricle. A report of two 
cases. Prenat Diagn. 1996 Jun;16(6):567-71. PubMed PMID: 8809901.
))
((Barber MA, Eguiluz I, Plasencia W, Medina M, Valle L. Intracranial fetal
hemorrhage due to choroid plexus papilloma. Int J Gynaecol Obstet. 2009
May;105(2):172-3. doi: 10.1016/j.ijgo.2008.11.041. Epub 2009 Feb 5. PubMed PMID: 
19200541.
))
((Adra AM, Mejides AA, Salman FA, Landy HJ, Helfgott AW. Prenatal sonographic
diagnosis of a third ventricle choroid plexus papilloma. Prenat Diagn. 1994
Sep;14(9):865-7. PubMed PMID: 7845895.
))
((Cohen ZR, Achiron R, Feldman Z. Prenatal sonographic diagnosis of lateral
ventricle choroid plexus papilloma in an in vitro fertilization-induced
pregnancy. Pediatr Neurosurg. 2002 Nov;37(5):267-70. PubMed PMID: 12411720.
)).

In two cases of choroid plexus papilloma reported at 21 weeks of pregnancy, the diagnosis was suspected because of ventriculomegaly. Both pregnancies were terminated and pathological examination showed bilateral papillomas of the choroid plexus
((Romano F, Bratta FG, Caruso G, Naro ED, Serio R, Resta M, Loizzi P. Prenatal
diagnosis of choroid plexus papillomas of the lateral ventricle. A report of two 
cases. Prenat Diagn. 1996 Jun;16(6):567-71. PubMed PMID: 8809901.
)).
===== Classification =====

They are histologically classified as plexus papilloma, atypical plexus papilloma, and plexus carcinoma.

----

[[Atypical choroid plexus papilloma]].

[[Choroid plexus papilloma in the posterior fossa]].

[[Extraventricular choroid plexus papilloma]].

[[Fetal choroid plexus tumor]]


===== Etiology =====
They can correspond to two different etiologies: [[papilloma]], which is a benign tumor, and [[carcinoma]] of the [[choroid plexus]]. 


===== Diagnosis =====

[[Choroid plexus papilloma diagnosis]].


===== Differential diagnosis =====

[[Choroid plexus papilloma differential diagnosis]]


===== Treatment =====

[[Choroid plexus papilloma treatment]].


====Outcome====
Total removal is possible for 96% of papillomas and for 61% of carcinomas. Surgical procedures are associated with significant operative morbidity and mortality due to uncontrolled bleeding. The prognosis is widely variable between the two forms, with a survival rate at 5 years of approximately 100% for papillomas and 40% for carcinomas
((Pencalet P, Sainte-Rose C, Lellouch-Tubiana A, Kalifa C, Brunelle F, Sgouros
S, Meyer P, Cinalli G, Zerah M, Pierre-Kahn A, Renier D. Papillomas and
carcinomas of the choroid plexus in children. J Neurosurg. 1998 Mar;88(3):521-8. 
PubMed PMID: 9488307.
)).

===== Case reports =====
A 43-year-old female with a [[hemifacial spasm]] of typical characteristics 6 months after onset. Upon [[clinical examination]], a severe contraction of the orbicularis oculi, orbicularis oris, and superficial muscles of the neck displaying 50 crisis per hour was revealed. Brain magnetic resonance imaging showed the absence of the facial nerve vascular loop in the cisternal portion, with evidence of an intraventricular tumor in relation to the medial portion of the fourth ventricle at the facial colliculus level, indicating a secondary origin of hemifacial spasm. Preoperative electromyography demonstrated irritative electric activity in the muscular branches of the facial nerve. A [[telovelar approach]] was performed to the fourth ventricle with intraoperative electrophysiology monitoring, with immediate resolution of the irritative activity after complete tumor resection. The result of the histopathologic study was a [[choroid plexus papilloma]].

[[Fourth ventricle tumor]]s with extrinsic compression of the facial colliculus represent <0.6% of the causes of hemifacial spasm. Its relationship with choroid plexuses papilloma is being described as the first case reported in the literature. Clinical correlation, imaging, and intraoperative findings in conjunction with intraoperative electrophysiology recordings allow predicting the resolution of symptoms after resecting the lesion
((Navarro-Olvera JL, Covaleda-Rodriguez JC, Diaz-Martinez JA, Aguado-Carrillo G,
Carrillo-Ruiz JD, Velasco-Campos F. Hemifacial Spasm Associated with Compression 
of the Facial Colliculus by a Choroid Plexus Papilloma of the Fourth Ventricle.
Stereotact Funct Neurosurg. 2020 Apr 21:1-5. doi: 10.1159/000507060. [Epub ahead 
of print] PubMed PMID: 32316018.
)).