Neurosurgery notes/Tumours/Choroid plexus tumours

Choroid plexus tumours

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Tumours
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Choroid plexus papilloma
Atypical choroid plexus papilloma
Choroid plexus carcinoma

General

  • Have marked epithelial characteristics, are separated from the category of Gliomas, Glioneuronal Tumours, and Neuronal Tumours.
  • Rare ( < 1% of intracranial neoplasms)
  • 85% occur at age 10 years or less; often present at birth
    • 2-4% of those that occur in children aged < 15 years
    • 10- 20% of those occurring in the first year of life.

Location

  • Most commonly
    • Lateral ventricle in children
    • Fourth ventricle in adults.

Clinical features

  • HCP
    • Due to
      • Increased CSF production
      • Obstruction

Radiology

  • Avidly enhancing tumours
  • Lobulated margins
  • Flow voids
  • Frequently reach a large size → can breach through the ependyma into the surrounding parenchyma.
Carcinoma
Papilloma
MRS
Higher choline and lactate peak
Higher myoinositol

Management CCLG

  • Surgery
    • Gross total resection
      • The best management regardless the WHO grade of the tumour
      • Choroid plexus tumours are very vascular and there is a considerable risk of bleeding during surgery, which may be catastrophic
        • Preoperative embolization should be considered as it can reduce intraoperative bleeding which can be catastrophic.
    • Neoadjuvant Chemo for Choroid plexus carcinoma
      • Neoadjuvant chemotherapy is reported to facilitate second look surgery by reducing tumour vascularity and blood loss (Lafay-Cousin et al. 2010; Schneider et al. 2015).
        • The possibility of surgical resection should be reconsidered after every 2 cycles of chemotherapy.
  • Chemotherapy
    • Adjuvant chemotherapy in Choroid plexus carcinoma has been shown to confer a survival advantage with the most effective agents being etoposide, carboplatin and cyclophosphamide
    • As many patients with CPTs are very young, chemotherapy strategies have been used to delay or avoid radiation.
  • Postoperative radiotherapy is used for CPCs but the single most important prognostic factor is the presence or not of residual tumour.

Prognosis

  • The location, size, and enhancement characteristics of the choroid plexus tumours can occasionally make difficult to differentiate from meningiomas in this area. CSF seeding is rare for the CPP I, II, but individual cases have been reported. CSF seeding is frequent for CPCs. Whole spine MRI before any definite management decision is therefore mandatory.
  • The 5-year survival outcomes in 2018
    • CPP: (n=85) 5y OS 100%, 5y EFS 95%
    • APP: (n=89) 5y OS 97%, 5y EFS 79%
    • CPC: (n=77) 5y OS 59%, 5y EFS 32%

Differential for all choroid plexus neoplasm

  • Meningioma
  • Metastases
  • Ependymoma
  • Medulloblastoma
  • Choroid plexus papilloma
  • Haemangioma (sturge-weber)
    • Localization: lateral ventricles
    • Marked enhancement
    • Calcification
    • Association with sturge-weber syndrome
      • Choroid plexus hemangiomas ipsilateral to the leptomeningeal vascular malformation may be present
  • Lymphoma
  • Choroid plexus carcinoma
Tumor Type
Location
CT characteristics
Ependymoma
4th ventricle > supratentorial when
supratentorial more commonly extraventricular
- Isodense to hypodense
- Occasionally hyperdense (maybe higher grade)
- Heterogeneous
Subependymoma
4th ventricle > lateral ventricles (Commonly in frontal horn/body)
- Hypodense to isodense
- Well-defined, lobulated
- Heterogeneous
Choroid Plexus Papilloma
- Lateral ventricles (more common in children)
- 4th ventricle (more common in adults)
- Isodense to hyperdense
- No brain invasion
- May engulf glomus
Choroid Plexus Carcinoma
- Same as CPP
- May more commonly involve/invade 3ʳᵈ ventricle than CPP
More heterogeneous with necrosis and brain invasion