Choroid plexus carcinoma

Choroid plexus carcinoma
Synonyms
Pronounce
Field	oncology
Symptoms
Complications
Onset
Duration
Types
Causes
Risks
Diagnosis
Differential diagnosis
Prevention
Treatment
Medication
Prognosis
Frequency
Deaths

A choroid plexus carcinoma (WHO grade III) is a type of choroid plexus tumor <ref name="pmid18684041">,

 Choroid plexus carcinoma, 
 Arch. Pathol. Lab. Med., 
 
 Vol. 132(Issue: 8),
 pp. 1350–4,
 DOI: [1350:CPC2.0.CO;2 10.1043/1543-2165(2008)132[1350:CPC]2.0.CO;2],
 PMID: 18684041,</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>, 
 Choroid plexus carcinoma, 
 Radiopaedia, 
 
 
 
 
 
 
 
 Full text,</ref> The disease creates lesions in the brain and increases cerebrospinal fluid volume, resulting in hydrocephalus.<ref name="AdunkaBuchman2010">, 
  
 Otology, Neurotology, and Lateral Skull Base Surgery: An Illustrated Handbook, 
  
 Thieme, 
  
  
  
 ISBN 9783131450210, 
 DOI: 10.1055/b-0034-83637,</ref>

Signs and symptoms

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 tumors. A mutation in the tumor suppressor gene TP53 is usually characterized in this disease.<ref>,

 DNA Methylation, Stem Cells and Cancer,</ref>

Pathophysiology

Main article: 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/>

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>,

 Choroid plexus carcinomas in children: MRI features and patient outcomes., 
 Neuroradiology, 
 
 Vol. 46(Issue: 9),
 pp. 770–80,
 DOI: 10.1007/s00234-004-1238-7,
 PMID: 15309348,</ref>

Treatment

Treatment of choroid plexus carcinoma depends on the location and severity of the tumor. Possible interventions include inserting shunts, 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>,

 Choroid plexus tumor epidemiology and outcomes: implications for surgical and radiotherapeutic management., 
 Journal of Neuro-Oncology, 
 
 Vol. 121(Issue: 1),
 pp. 151–7,
 DOI: 10.1007/s11060-014-1616-x,
 PMID: 25270349,</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 of about 0.3 per 1 million cases.<ref name= Menon>,

 Choroid plexus tumors: an institutional series of 25 patients., 
 Neurology India, 
 
 Vol. 58(Issue: 3),
 pp. 429–35,
 DOI: 10.4103/0028-3886.66455,
 PMID: 20644273,</ref>

It is seen mainly in children under the age of 5,<ref name="pmid10940770">,

 Management of choroid plexus tumours in children: 20 years experience at a single neurosurgical centre, 
 Pediatr Neurosurg, 
 
 Vol. 32(Issue: 4),
 pp. 192–9,
 DOI: 10.1159/000028933,
 PMID: 10940770,
 
 
 Full text,</ref> representing 5% of all pediatric tumors and 20% of tumors in children less than 1 year old.<ref name= Rickert>, 
 Tumors of the choroid plexus., 
 Microscopy Research and Technique, 
 
 Vol. 52(Issue: 1),
 pp. 104–11,
 DOI: <104::AID-JEMT12>3.0.CO;2-3 10.1002/1097-0029(20010101)52:1<104::AID-JEMT12>3.0.CO;2-3,
 PMID: 11135453,</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>,

 The 2007 WHO Classification of Tumours of the Central Nervous System, 
 Acta Neuropathologica, 
 
 Vol. 114(Issue: 2),
 pp. 97–109,
 DOI: 10.1007/s00401-007-0243-4,
 PMID: 17618441,
 PMC: 1929165,</ref><ref>, 
 Choroid plexus tumors in pediatric patients., 
 British Journal of Neurosurgery, 
 
 Vol. 26(Issue: 1),
 pp. 32–7,
 DOI: 10.3109/02688697.2011.601820,
 PMID: 21970783,</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>, 
 Choroid plexus carcinomas in childhood: clinical features and prognostic factors., 
 Neurosurgery, 
 
 Vol. 42(Issue: 3),
 pp. 470–5,
 DOI: 10.1097/00006123-199803000-00006,
 PMID: 9526979,</ref>

See also

• Choroid plexus
• Brain tumor
• Cancer of the brain

External links

   This article is a medical stub. You can help WikiMD by expanding it!