Choroid plexus carcinoma

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Choroid plexus carcinoma
Photomicrograph of hematoxylin-eosin stained section of a choroid plexus carcinoma (grade III WHO) at 400x magnification
SpecialtyNeuro-oncology

A choroid plexus carcinoma (

WHO grade III) is a type of choroid plexus tumor[1] 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).[2] The disease creates lesions in the brain and increases cerebrospinal fluid volume, resulting in hydrocephalus.[3]

Signs and symptoms

The symptoms of choroid plexus carcinoma are similar to those of other brain tumors. They include:[4]

  • Persistent or new onset
    headaches
  • fontanels
    in infants.
  • Loss of appetite (refusal to take food in infants)
  • Papilledema
  • emesis
  • Ataxia
  • Strabismus
  • Developmental delays
  • Altered mental status

Cause

The cause of choroid plexus carcinomas are relatively unknown, although hereditary factors are suspected. They 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.[5]

Pathophysiology

Main article: Carcinogenesis

Choroid plexus carcinomas typically occur in the

supratentorial space in adults.[6] 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.[7]

The tumors most frequently spread through the CSF. As a result, metastases frequently occur along the

leptomeninges. In rare cases, metastases have been reported to spread to the abdomen and extra-cranial sites.[8]

Diagnosis

Treatment

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

radiotherapy.[9] In the event of subtotal resection or widespread leptomeningeal disease, craniospinal irradiation is often used.[8]

Incidence

Choroid plexus tumors have an annual incidence of about 0.3 per 1 million cases.[7] It is seen mainly in children under the age of 5,[4] representing 5% of all pediatric tumors and 20% of tumors in children less than 1 year old.[6] There has been no link between sex and occurrence.[10]

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.[10][11] 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.[12]

See also

References

  1. PMID 18684041
    .
  2. ^ Stanislavsky, A. "Choroid plexus carcinoma". Radiopaedia.
  3. ISBN 9783131450210
    .
  4. ^
    S2CID 20244382
    .
  5. ^ Dricua A, et al. (Dec 1999). DNA Methylation, Stem Cells and Cancer.
  6. ^
    PMID 11135453
    .
  7. ^
    PMID 20644273
    .
  8. ^
    S2CID 22235664
    .
  9. S2CID 11127634
    .
  10. ^
    PMID 17618441
    .
  11. S2CID 12096860
    .
  12. PMID 9526979
    .

External links
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