Choroid Plexus Papilloma

1. Choroid Plexus Papilloma
Ade Wijaya, MD – February 2018

2. Outline:
 Introduction
 Choroid plexus
tumors
 Predilection
 Clinical
presentation
 Complication
 Neuroradiology
 Pathology
 Differential
Diagnosis
 Gene expression
 Management
 Prognosis
 Summary

3. Introduction
 Intraventricular papillary neoplasms
derived from choroid plexus epithelium
 0.4-0.6% of all brain tumors
 Choroid plexus papillomas (CPP)
outnumber choroid plexus carcinomas
(CPC) by a ratio of at least 5:1
 CPP are confined to lateral (50%),
third (5%), and fourth (40%) ventricles
with two or three ventricles involved in
5% of cases.
Aguzzi A, Brandner S, Paulus W. Choroid plexus tumours. World Health Organization Classification of Tumours. In: Kleihues P, Cavenee WK, editors.
Pathology and Genetics: Tumours of the Nervous System. Lyon: IARC Press; 2000. pp. 84–6.

4. Choroid plexus tumors
 Papilloma (WHO gr. 1)
 Atypical papilloma – intermediate
(WHO gr. 2)
 Carcinoma (WHO gr. 3)
McGirr SJ, Ebersold MJ, Scheithauer BW, Quast LM, Shaw EG. Choroid plexus papillomas: Long-term follow-up results in a surgically treated series. J
Neurosurg. 1988;69:843–9.

5. Predilection
 Most common: 4th ventricle (in adults)
(benign)
 Lateral ventricle (in children)
(anaplastic)
Harsh GR, Wilson CB. Neurological Surgery. 3rd ed. Philadelphia: WB Saunders Co; 1990. Neuroepithelial tumors of the adult brain; pp. 3103–5.

6. Clinical Presentation
Lateral Ventricle 4th Ventricle
 Convulsion
 Mental changes
 Papiledema (loss of
vision)
 Hemiparesis
 Headache
 Ataxia
 Nystagmus
 Cerebellar signs
 Dizziness
 Loss of vision
 Vomiting
 Diplopia
Gradin WC, Taylon C, Fruin AH. Choroid plexus papilloma of the third ventricle: Case report and review of the literature. Neurosurgery. 1983;12:217–20.
Bohm E, Strang R. Choroid plexus papillomas. J Neurosurg. 1961;18:493–500.

7. Complication
 Gradual deterioration
 Spontaneous hemorrhage
 Dissemination of tumor fragments
 Hydrocephalus
Harsh GR, Wilson CB. Neurological Surgery. 3rd ed. Philadelphia: WB Saunders Co; 1990. Neuroepithelial tumors of the adult brain; pp. 3103–5.

8. Neuroradiology
Brain CT Brain MRI
 Hypodense,
 Lobulated intraventricular
mass,
 May have finely speckled
calcification
 Enhances brightly after
contrast
 T1: Hypo-iso intense
 T2: Hyperintense

9. Pathology
 Gross pathology: soft, pink, globular
masses with irregular projections and
are highly vascular and friable
 Histopathology: Papillary fronds lined
by bland columnar epithelium
 Carcinoma: mitotic activity, nuclear
pleomorphism, and necrosis
Aguzzi A, Brandner S, Paulus W. Choroid plexus tumours. World Health Organization Classification of Tumours. In: Kleihues P, Cavenee WK, editors.
Pathology and Genetics: Tumours of the Nervous System. Lyon: IARC Press; 2000. pp. 84–6.

10. Differential Diagnosis
 Bilateral villous hypertrophy, which
show dense and homogenously
enhanced lesion and by their tendency
to extend that leads to hydrocephalus,
which usually resolves after complete
resection
Tacconi L, Delfini R, Cantore G. Choroid plexus papillomas: Consideration of a surgical series of 33 cases. Acta Neurochir (Wien) 1996;138:802–10.
Erman T, Göçer AI, Erdoğan S, Tuna M, Ildan F, Zorludemir S. Choroid plexus papilloma of bilateral lateral ventricle. Acta Neurochir (Wien)
2003;145:139–43.

11. Gene expression
 Expression of cytokeratin, vimentin,
transthyretin and S-100 are well
documented in CPP.
 Epithelial Membrane Antigen (EMA)
and Glial Fibrillary Acidic Protein
(GFAP) more frequent in CPC
 S100  4th ventricle tumor
 GFAP & TTR  Lateral ventricle
tumor and older (> 20 y.o) patients
 p53 expression  cancer genesis
Paulus W, Jänisch W. Clinicopathologic correlations in epithelial choroid plexus neoplasms: A study of 52 cases. Acta Neuropathol. 1990;80:635–41.
Jay V, Ho M, Chan F, Malkin D. P53 expression in choroid plexus neoplasms: An immunohistochemical study. Arch Pathol Lab Med. 1996;120:1061–5.

12. Management & Prognostic
 Surgery
 Favorable outcome
 Reccurents are occasionally seen
 Rare cases of metastases to
suprasellar and even craniospinal
seeding have been reported
McEvoy AW, Galloway M, Revesz T, Kitchen ND. Metastatic choroid plexus papilloma: A case report. J Neurooncol. 2002;56:241–6.

13. Prognostic Factors
 Tumor size,
 Preoperative symptoms,
 The difficult surgical intervention
related to its anatomical location
McEvoy AW, Galloway M, Revesz T, Kitchen ND. Metastatic choroid plexus papilloma: A case report. J Neurooncol. 2002;56:241–6.

14. Summary
 Rare, benign neoplasm of choroid
plexus
 Most common: lateral and 3th
ventricles
 Neuroimaging: Brain CT/MRI
 Histopathology: Papillary fronds lined
by bland columnar epithelium
 Treatment: surgery
 Favorable prognosis

15. THANK YOU