Choroid plexus papilloma

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]; Ammu Susheela, M.D. [3]

Synonyms and Keywords: Choroid plexus papillomas; Papilloma of choroid plexus; Papilloma of the choroid plexus; CPP

Overview

Choroid plexus papilloma is a a rare, slow-growing, histologically benign intracranial tumor that is commonly located in the ventricular system of the choroid plexus. It may obstruct the cerebrospinal fluid flow, causing elevated intracranial pressure. Choroid plexus papilloma is neuroectodermal in origin and similar in structure to a normal choroid plexus. They may be created by epithelial cells of the choroid plexus.[1] Choroid plexus papilloma may be associated with Aicardi syndrome, Von Hippel-Lindau disease, and Li-Fraumeni syndrome.[2][3] On gross pathology, choroid plexus papilloma is characterized by a soft, solid, pink to red, capsulated, vascular, and friable cauliflower-like mass.[2] On microscopic histopathological analysis, choroid plexus papilloma is characterized by papillary structures with a delicate fibrovascular core lined by columnar or cuboidal epithelial cells with vesicular nuclei. Its appearance is very similar to normal choroid plexus.[2] Choroid plexus papilloma is demonstrated by positivity to tumor marker such as cytokeratin (CK7).[2] Choroid plexus papilloma may be differentiated from choroid plexus carcinoma, choroid plexus metastasis, medulloblastoma, and intraventricular meningioma.[4] Choroid plexus papilloma accounts for approximately 1% of all brain tumors, 2-6% of all pediatric brain tumors, and 0.5% of the adult brain tumors.[5] Choroid plexus papilloma is a rare disease that tends to affect children and adults.[5] Males are more commonly affected with choroid plexus papilloma than females. The male to female ratio is approximately 2.8 to 1.[6] If left untreated, patients with choroid plexus papilloma may progress to develop seizures, hydrocephalus, focal neurological deficits, cranioneuropathies, psychosis, and coma.[7][8] Common complications of choroid plexus papilloma include CSF seeding, obstructive hydrocephalus, blindness, psychosis, and coma. The prognosis is excellent, and the 5-year survival rate after complete excision of choroid plexus papilloma is approximately 100%.[8][9] Symptoms of choroid plexus papilloma include headache, vomiting, seizures, and vision loss.[10][7] Common physical examination findings of choroid plexus papilloma include bulging fontanelle, enlarged head size, papilledema, altered mental status, and cranioneuropathies (6th and 7th nerve palsy).[10][7][8] Head CT scan and brain MRI are helpful in the diagnosis of choroid plexus papilloma. On CT scan, choroid plexus papilloma is characterized by iso- to hyperdense well-defined lobulated masses with intense homogenous enhancement and associated hydrocephalus. If there is markedly heterogeneous contrast enhancement, a choroid plexus carcinoma should be suspected.[11][7] On MRI, choroid plexus papilloma is characterized by a well demarcated, extra-axial mass that is iso- to hypointense on T1-weighted imaging and iso- to hyperintense on T2-weighted imaging. On contrast administration, there is generally marked homogenous enhancement.[11][7] The predominant therapy for choroid plexus papilloma is surgical resection. Radiotherapy is indicated for the inoperable residual tumor or resistant/aggressive subtype, whereas chemotherapy is reserved for the recurrent tumors.[7][12]

Historical Perspective

Choroid plexus papilloma was first described by Guerard, in 1832.[7]

Classification

Choroid plexus papilloma may be classified into two groups:[13]

 
 
 
 
 
 
 
 
 
 
 
 
Choroid plexus papilloma
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Typical
 
 
 
 
 
 
 
 
 
Atypical
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
WHO grade I
 
 
 
 
 
 
 
 
 
WHO grade II
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 

Pathophysiology

  • On gross pathology, choroid plexus papilloma is characterized by a soft, solid, pink to red, capsulated, vascular, and friable cauliflower-like mass.[2]
  • Unlike most other brain tumors which are more common in the posterior fossa in children and supratentorial compartment in adults, the relationship is reversed for choroid plexus papillomas:
  • Third ventricular, cerebellopontine angle, parenchymal and even pineal region tumors have also been described.

Gallery

Microscopic Pathology

  • On microscopic histopathological analysis, choroid plexus papilloma is characterized by papillary structures with a delicate fibrovascular core lined by columnar or cuboidal epithelial cells with vesicular nuclei. Its appearance is very similar to normal choroid plexus.[2]
  • If two or more mitoses are present per 10 high power field (HPF), then the tumor is designated an atypical choroid plexus papilloma and is classified as a WHO grade II tumor.

Gallery

Causes

  • There are no established causes for choroid plexus papilloma.[8]

Differentiating Choroid Plexus Papilloma from other Diseases

Choroid plexus papilloma may be differentiated from:[4][7]

Among children, choroid plexus papilloma must be differentiated from:[4]

Among adults, choroid plexus papilloma must be differentiated from:[4]

Epidemiology and Demographics

Prevalence

  • Choroid plexus papilloma accounts for approximately 1% of all brain tumors, 2-6% of all pediatric brain tumors, and 0.5% of the adult brain tumors.[5]
  • They are far more common than the more aggressive choroid plexus carcinoma (WHO grade III), which they outnumber 5:1.[14]

Age

  • Choroid plexus papilloma is a rare disease that tends to affect children and adults.[5]
  • Approximately 85% of all choroid plexus papilloma occurs in children under the age of 5 years.

Gender

  • Males are more commonly affected with choroid plexus papilloma than females. The male to female ratio is approximately 2.8 to 1.[6]

Risk Factors

  • There are no established risk factors for choroid plexus papilloma.

Screening

  • There is insufficient evidence to recommend routine screening for choroid plexus papilloma.[15]

Natural History, Complications and Prognosis

Diagnosis

Symptoms

Physical Examination

Common physical examination findings of choroid plexus papilloma include:[10][7][8]

HEENT

Neurological

Laboratory Findings

  • There are no specific laboratory findings associated with choroid plexus papilloma.
  • Other laboratory findings consistent with the diagnosis of choroid plexus papilloma is immunohistochemistry.

Immunohistochemistry

  • Choroid plexus papilloma is demonstrated by positivity to tumor marker such as cytokeratin (CK7).[2]
  • Some reactivity for GFAP and epithelial membrane antigen may be present.[7]

Imaging Findings

CT

  • Head CT scan is helpful in the diagnosis of choroid plexus papilloma.
  • On CT scan, choroid plexus papilloma is characterized by:[11][7]
  • Well-defined lobulated masses
  • Iso- or somewhat hyperdense compared to the adjacent brain
  • Associated hydrocephalus
  • Fine, speckled calcification (25%)
  • Microhemorrhage
  • Intense homogenous enhancement, with an irregular frond-like pattern
  • If there is markedly heterogeneous contrast enhancement, a choroid plexus carcinoma should be suspected

Gallery

MRI

  • Brain MRI is helpful in the diagnosis of choroid plexus papilloma.
  • On MRI, choroid plexus papilloma is characterized by:[11]
MRI component Findings

T1

  • Iso- to hypointense compared to adjacent brain

T2

  • Iso- to hyperintense
  • Small flow-voids may be observed within the tumor

T1 with contrast

  • Marked homogenous enhancement

Gallery

Other Imaging Findings

Other Diagnostic Studies

  • Other diagnostic studies for choroid plexus papilloma include angiography, which demonstrate intense vascular blush owing to the vascularity of the tumor. Enlarged choroidal arteries may be observed feeding the tumor, with shunting.[11]

Treatment

Medical Therapy

The treatment options for choroid plexus papilloma include:[7]

 
 
 
 
 
 
 
 
 
 
 
 
Treatment of choroid plexus papilloma
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Surgery
(mainstay)
 
Radiotherapy
 
Radiosurgery
 
Chemotherapy
 
 
 
 
 
 
 
 
 
 
 
 
 

Radiotherapy

  • If complete resection of choroid plexus papilloma is done, then no adjuvant radiotherapy is necessary.
  • Indications for radiotherapy include:[7]
  • Inoperable residual tumor
  • Resistant / aggressive tumor

Chemotherapy

  • Adjuvant chemotherapy is not required if choroid plexus papilloma is completely resected.[7]
  • Combination chemotherapy is used in the case of recurrent tumors.

Surgery

  • Surgery is the mainstay of treatment for choroid plexus papilloma.[7]
  • Complete resection of the tumor must be attempted in all cases.
  • Owing to the highly vascular nature of the tumor, care should be taken prior to and during surgery to prevent any life-threatening bleeding.
  • Techniques used to minimize this complication of surgery include:[7]
  • Preoperative embolization of small caliber feeding vessels
  • Intraoperative usage of tranexamic acid
  • Hydrocephalus may be managed either by the placement of an external ventricular drain (EVD), VP shunt, or endoscopic third ventriculostomy (ETV).
  • The approach to the surgery depends on the size, location, and extent of the tumor.
    • Tumor is in the posterior third ventricle: infratentorial supracerebellar, transcallosal, occipital transtentorial, or transcortical-transforaminal approaches may be used for resection.
    • Tumor is in the fourth ventricle: midline suboccipital approach is preferred.
    • Tumor is in the cerebellopontine angle: midline cerebellomedullary fissure or the conventional retrosigmoid approach may be utilized.

Radiosurgery

  • If complete resection of choroid plexus papilloma is done, then no adjuvant radiosurgery is necessary.
  • Indications for radiotherapy include:[7]
  • Inoperable residual tumor
  • Resistant / aggressive tumor

Prevention

  • There are no primary or secondary preventive measures available for choroid plexus papilloma.

References

  1. 1.0 1.1 Choroid plexus papilloma. Wikipedia 2016. https://en.wikipedia.org/wiki/Choroid_plexus_papilloma. Accessed on January 13, 2016
  2. 2.0 2.1 2.2 2.3 2.4 2.5 2.6 2.7 2.8 Clinical presentation of choroid plexus papilloma. Dr Tim Luijkx and Dr Paresh K Desai et al. Radiopaedia 2016. http://radiopaedia.org/articles/choroid-plexus-papilloma-1. Accessed on January 13, 2016
  3. 3.0 3.1 3.2 Gozali AE, Britt B, Shane L, Gonzalez I, Gilles F, McComb JG; et al. (2012). "Choroid plexus tumors; management, outcome, and association with the Li-Fraumeni syndrome: the Children's Hospital Los Angeles (CHLA) experience, 1991-2010.". Pediatr Blood Cancer. 58 (6): 905–9. PMID 21990040. doi:10.1002/pbc.23349. 
  4. 4.0 4.1 4.2 4.3 Differential diagnosis of choroid plexus. Dr Tim Luijkx and Dr Paresh K Desai et al. Radiopaedia 2016. http://radiopaedia.org/articles/choroid-plexus-papilloma-1. Accessed on January 13, 2016
  5. 5.0 5.1 5.2 5.3 Epidemiology of choroid plexus papilloma. Dr Tim Luijkx and Dr Paresh K Desai et al. Radiopaedia 2016. http://radiopaedia.org/articles/choroid-plexus-papilloma-1. Accessed on January 13, 2016
  6. 6.0 6.1 Epidemiology of choroid plexus papilloma. DR.SAGAR JUNG RANA 2016. http://drsagarjungrana.blogspot.com/2012/11/choroid-plexus-papilloma.html. Accessed on January 14, 2016
  7. 7.00 7.01 7.02 7.03 7.04 7.05 7.06 7.07 7.08 7.09 7.10 7.11 7.12 7.13 7.14 7.15 7.16 7.17 7.18 Prasad GL, Mahapatra AK (2015). "Case series of choroid plexus papilloma in children at uncommon locations and review of the literature.". Surg Neurol Int. 6: 151. PMC 4596056Freely accessible. PMID 26500797. doi:10.4103/2152-7806.166167. 
  8. 8.0 8.1 8.2 8.3 8.4 8.5 8.6 Koeller, Kelly K.; Sandberg, Glenn D. (2002). "From the Archives of the AFIP". RadioGraphics. 22 (6): 1473–1505. ISSN 0271-5333. doi:10.1148/rg.226025118. 
  9. 9.0 9.1 Pencalet P, Sainte-Rose C, Lellouch-Tubiana A, Kalifa C, Brunelle F, Sgouros S; et al. (1998). "Papillomas and carcinomas of the choroid plexus in children.". J Neurosurg. 88 (3): 521–8. PMID 9488307. doi:10.3171/jns.1998.88.3.0521. 
  10. 10.0 10.1 10.2 10.3 Signs and symptoms of choroid plexus papilloma. Wikipedia 2016. https://en.wikipedia.org/wiki/Choroid_plexus_papilloma. Accessed on January 13, 2016
  11. 11.0 11.1 11.2 11.3 11.4 11.5 Radiographic features of choroid plexus papilloma. Dr Tim Luijkx and Dr Paresh K Desai et al. Radiopaedia 2016. http://radiopaedia.org/articles/choroid-plexus-papilloma-1. Accessed on January 13, 2016
  12. Treatment and prognosis of choroid plexus papilloma. Dr Tim Luijkx and Dr Paresh K Desai et al. Radiopaedia 2016. http://radiopaedia.org/articles/choroid-plexus-papilloma-1. Accessed on January 14, 2016
  13. Choroid plexus papillomas. Dr Tim Luijkx and Dr Paresh K Desai et al. Radiopaedia 2016. http://radiopaedia.org/articles/choroid-plexus-papilloma-1. Accessed on January 13, 2016
  14. Choroid plexus papilloma. Dr Tim Luijkx and Dr Paresh K Desai et al. Radiopaedia 2016. http://radiopaedia.org/articles/choroid-plexus-papilloma-1. Accessed on January 13, 2016
  15. Early detection, diagnosis, and staging of brain tumors. American cancer society. http://www.cancer.org/cancer/braincnstumorsinadults/detailedguide/brain-and-spinal-cord-tumors-in-adults-detection
  16. Image courtesy of Dr. Michael Sargent. Radiopaedia (original file here). Creative Commons BY-SA-NC
  17. Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC
  18. Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC

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