| caption = Photomicrograph of hematoxylin-eosin stained section of a choroid plexus carcinoma (grade III WHO) at 400x magnification.
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A '''choroid plexus carcinoma''' ([[WHO Grades of CNS Tumors|WHO grade III]]) is a type of [[choroid plexus tumor]] <ref name="pmid18684041">{{cite journal| vauthors=Gopal P, Parker JR, Debski R, Parker JC |title=Choroid plexus carcinoma |journal=Arch. Pathol. Lab. Med. |volume=132 |issue=8 |pages=1350–4 |date=August 2008 |pmid=18684041 |doi=10.1043/1543-2165(2008)132[1350:CPC]2.0.CO;2|doi-broken-date=2020-01-22 }}</ref> that affects the [[choroid plexus]] of the brain. It is considered the worst of the three grades of chord plexus tumors, having a much poorer prognosis than [[choroid atypical plexus papilloma]] (WHO grade II) and [[choroid plexus papilloma]] (WHO grade I).<ref>{{cite journal | vauthors=Stanislavsky, A| title=Choroid plexus carcinoma |journal=Radiopaedia |url=https://radiopaedia.org/articles/choroid-plexus-carcinoma}}</ref> The disease creates lesions in the brain and increases [[cerebrospinal fluid]] volume, resulting in [[hydrocephalus]].<ref name="AdunkaBuchman2010">{{cite book|author1=Oliver Adunka|author2=Craig Buchman|title=Otology, Neurotology, and Lateral Skull Base Surgery: An Illustrated Handbook|date=11 October 2010|publisher=Thieme|doi=10.1055/b-0034-83637|chapter=Management of Middle Ear Trauma|isbn=9783131450210}}</ref>
The symptoms of choroid plexus carcinoma are similar to those of other brain tumors. They include:<ref name="pmid10940770"/>
*Persistent or new onset [[headaches]]
*[[Macrocephaly]] or bulging [[fontanels]] in infants.
*Loss of appetite (refusal to take food in infants)
* [[Papilledema]]
*[[Nausea]] and [[emesis]]
*[[Ataxia]]
*[[Strabismus]]
*Developmental delays
*[[Altered mental status]]
==Cause==
The cause of choroid plexus carcinomas are relatively unknown, although hereditary factors are suspected. The sometimes occur in conjunction with other hereditary cancers, including [[Li–Fraumeni syndrome]] and [[malignant rhabdoid tumour|malignant rhabdoid tumors]]. A mutation in the tumor suppressor gene TP53 is usually characterized in this disease.<ref>{{cite book| vauthors= Dricua A, et al. | title=DNA Methylation, Stem Cells and Cancer |date=Dec 1999}}</ref>
==Pathophysiology==
'''Choroid Plexus Carcinoma''' is a rare, malignant tumor that arises from the [[choroid plexus]] in the [[ventricular system]] of the [[brain]]. It is classified as a [[central nervous system]] tumor and is most commonly found in children, although it can occur in adults.
{{Main|Carcinogenesis}}
Choroid plexus carcinomas typically occur in the [[lateral ventricles]] in children and in the [[fourth ventricle]] of adults. The [[third ventricle]] is the least common ventricle effected. This is unlike most other pediatric and adult tumors, as the locations of the tumors are typically reversed. These tumors are usually found in the [[infratentorial]] region in children and in the [[supratentorial]] space in adults.<ref name= Rickert/> Choroid plexus carcinomas can induce hydrocephalus through a variety of mechanisms, including blockage of normal cerebrospinal fluid (CSF) flow, the tumor overproducing CSF, spontaneous hemorrhage, and expansion of the ventricles.<ref name= Menon/>
=== Anatomy and Function of the Choroid Plexus ===
The tumors most frequently spread through the CSF. As a result, metastases frequently occur along the [[central nervous system]], particularly in the [[leptomeninges]]. In rare cases, metastases have been reported to spread to the [[abdomen]] and extra-cranial sites.<ref name=Meyers>{{cite journal| vauthors=Meyers SP, et al. |title=Choroid plexus carcinomas in children: MRI features and patient outcomes. | journal=Neuroradiology |volume=46 |issue=9 |pages=770–80 |date=Sep 2004 |pmid=15309348 |doi=10.1007/s00234-004-1238-7}}</ref>
The [[choroid plexus]] is a network of cells that produces [[cerebrospinal fluid]] (CSF) in the [[ventricles]] of the brain. It plays a crucial role in maintaining the [[homeostasis]] of the [[central nervous system]] by regulating the composition of the CSF, which cushions the brain and spinal cord, removes waste, and provides nutrients.
==Diagnosis==
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==Treatment==
=== Pathophysiology ===
Treatment of choroid plexus carcinoma depends on the location and severity of the tumor. Possible interventions include inserting [[Shunt (medical)|shunt]]s, surgical resection, radiotherapy, and chemotherapy. Inserting a shunt could help to drain the CSF and relieve pressure on the brain. The best outcomes occur when total resection of the tumor is combined with adjuvant [[chemotherapy]] and [[radiotherapy]].<ref>{{cite journal| vauthors= Cannon DM, et al. |title=Choroid plexus tumor epidemiology and outcomes: implications for surgical and radiotherapeutic management. |journal=Journal of Neuro-Oncology |volume=121 |issue=1 |pages=151–7 |date=Jan 2015 |pmid=25270349 |doi=10.1007/s11060-014-1616-x}}</ref> In the event of subtotal resection or widespread leptomeningeal disease, craniospinal irradiation is often used.<ref name=Meyers/>
==Incidence==
Choroid plexus tumors have an annual [[Incidence (epidemiology)|incidence]] of about 0.3 per 1 million cases.<ref name= Menon>{{cite journal| vauthors= Menon G, Nair SN, Baldawa SS, Rao RB | title=Choroid plexus tumors: an institutional series of 25 patients. |journal=Neurology India| volume=58 | issue=3 | date=May–June 2010| pmid=20644273 | doi=10.4103/0028-3886.66455 | pages=429–35}}</ref>
It is seen mainly in children under the age of 5,<ref name="pmid10940770">{{cite journal |vauthors=McEvoy AW, Harding BN, Phipps KP |title=Management of choroid plexus tumours in children: 20 years experience at a single neurosurgical centre |journal=Pediatr Neurosurg |volume=32 |issue=4 |pages=192–9 |date=April 2000 |pmid=10940770 |doi= 10.1159/000028933|url=http://content.karger.com/produktedb/produkte.asp?typ=fulltext&file=pne32192|display-authors=etal}}</ref> representing 5% of all pediatric tumors and 20% of tumors in children less than 1 year old.<ref name= Rickert>{{cite journal| vauthors= Rickert CH, Paulus W | title=Tumors of the choroid plexus. |journal=Microscopy Research and Technique| volume=52 | issue=1 | date=Jan 2001| pmid=11135453 | doi=10.1002/1097-0029(20010101)52:1<104::AID-JEMT12>3.0.CO;2-3 | pages=104–11}}</ref> There has been no link between sex and occurrence.<ref name= Louis/>
Although choroid plexus carcinomas are significantly more aggressive and have half the survival rate as choroid plexus papillomas, they are outnumbered in incidence by 5:1 in all age groups.<ref name= Louis>{{cite journal| vauthors= Louis D, et al.| title=The 2007 WHO Classification of Tumours of the Central Nervous System | journal=Acta Neuropathologica| volume= 114 | issue= 2| date= Aug 2007| pmc=1929165| pmid=17618441| doi=10.1007/s00401-007-0243-4| pages=97–109}}</ref><ref>{{cite journal | vauthors= Ogiwara H, et al. |title= Choroid plexus tumors in pediatric patients.| journal=British Journal of Neurosurgery | volume=26 | issue=1 | pages=32–7 |date=Feb 2012 | pmid=21970783 | doi=10.3109/02688697.2011.601820}}</ref> Clinical studies have shown that patients who receive a total resection of a tumor have an 86% survival rate, while patients who only receive a partial resection have a 26% 5-year survival rate. Many incomplete resections result in recurrence within 2 years of primary surgery.<ref>{{cite journal |vauthors= Berger C, et al. |title=Choroid plexus carcinomas in childhood: clinical features and prognostic factors. |journal=Neurosurgery |volume=42 |issue=3 |pages=470–5 |date=Mar 1998 |pmid=9526979 |doi=10.1097/00006123-199803000-00006}}</ref>
Choroid Plexus Carcinoma is characterized by the uncontrolled proliferation of epithelial cells of the choroid plexus. This leads to the formation of a mass that can obstruct the flow of CSF, causing [[hydrocephalus]] and increased [[intracranial pressure]]. The tumor is highly aggressive and can metastasize to other parts of the central nervous system.
==See also==
=== Clinical Presentation ===
*[[Choroid plexus]]
*[[Brain tumor]]
*[[Cancer of the brain]]
==References==
Patients with Choroid Plexus Carcinoma may present with symptoms of increased intracranial pressure, such as [[headache]], [[nausea]], [[vomiting]], and [[papilledema]]. In infants, signs may include [[macrocephaly]] and [[irritability]]. Neurological deficits may occur depending on the location and size of the tumor.
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{{Central nervous system tumors}}
[[Category:Brain tumor]]
=== Diagnosis ===
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Diagnosis of Choroid Plexus Carcinoma typically involves [[neuroimaging]] techniques such as [[MRI]] and [[CT scan]] to visualize the tumor. A definitive diagnosis is made through [[histopathological]] examination of a biopsy sample, which reveals atypical epithelial cells with high mitotic activity.
=== Treatment ===
The primary treatment for Choroid Plexus Carcinoma is surgical resection. Complete removal of the tumor is the goal, but it may be challenging due to the tumor's location and potential involvement with critical brain structures. [[Adjuvant therapy]] such as [[radiation therapy]] and [[chemotherapy]] may be employed to manage residual disease and reduce the risk of recurrence.
=== Prognosis ===
The prognosis for patients with Choroid Plexus Carcinoma varies depending on factors such as the extent of surgical resection, age at diagnosis, and response to adjuvant therapy. Overall, the prognosis is poor due to the aggressive nature of the tumor and its tendency to recur.
Choroid Plexus Carcinoma is a rare, malignant tumor that arises from the choroid plexus in the ventricular system of the brain. It is classified as a central nervous system tumor and is most commonly found in children, although it can occur in adults.
Anatomy and Function of the Choroid Plexus
The choroid plexus is a network of cells that produces cerebrospinal fluid (CSF) in the ventricles of the brain. It plays a crucial role in maintaining the homeostasis of the central nervous system by regulating the composition of the CSF, which cushions the brain and spinal cord, removes waste, and provides nutrients.
Pathophysiology
Choroid Plexus Carcinoma is characterized by the uncontrolled proliferation of epithelial cells of the choroid plexus. This leads to the formation of a mass that can obstruct the flow of CSF, causing hydrocephalus and increased intracranial pressure. The tumor is highly aggressive and can metastasize to other parts of the central nervous system.
Clinical Presentation
Patients with Choroid Plexus Carcinoma may present with symptoms of increased intracranial pressure, such as headache, nausea, vomiting, and papilledema. In infants, signs may include macrocephaly and irritability. Neurological deficits may occur depending on the location and size of the tumor.
Diagnosis
Diagnosis of Choroid Plexus Carcinoma typically involves neuroimaging techniques such as MRI and CT scan to visualize the tumor. A definitive diagnosis is made through histopathological examination of a biopsy sample, which reveals atypical epithelial cells with high mitotic activity.
Treatment
The primary treatment for Choroid Plexus Carcinoma is surgical resection. Complete removal of the tumor is the goal, but it may be challenging due to the tumor's location and potential involvement with critical brain structures. Adjuvant therapy such as radiation therapy and chemotherapy may be employed to manage residual disease and reduce the risk of recurrence.
Prognosis
The prognosis for patients with Choroid Plexus Carcinoma varies depending on factors such as the extent of surgical resection, age at diagnosis, and response to adjuvant therapy. Overall, the prognosis is poor due to the aggressive nature of the tumor and its tendency to recur.
