Choroid plexus papilloma

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Template:Choroid plexus papilloma Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]

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

Overview

Choroid plexus papilloma was first described by Guerard, in 1832.Choroid plexus papilloma may be classified into two groups: typical and atypical.

Historical Perspective

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

Classification

Choroid plexus papilloma may be classified into two groups:

Choroid plexus papilloma

Typical

Atypical

WHO grade I

WHO grade II

Pathophysiology

• 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.
• It may be a true congenital intracranial neoplasm.

Choroid plexus papilloma may be associated with:

• Aicardi syndrome
• Von Hippel-Lindau disease
• Li-Fraumeni syndrome
• On gross pathology, choroid plexus papilloma is characterized by a soft, solid, pink to red, capsulated, vascular, and friable cauliflower-like mass.
• 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:

• Adults: 70% occur in the fourth ventricle
• Children: most often occur in the lateral ventricles, with a predilection for the trigone

• Third ventricular, cerebellopontine angle, parenchymal and even pineal region tumors have also been described.

Gallery

• 

  _{Gross pathological specimen of choroid plexus papilloma.}

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.
• 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

• 

  _{Shown above is a micrograph of choroid plexus papilloma (H&E stain).}

Causes

• There are no established causes for choroid plexus papilloma.

Differentiating Choroid Plexus Papilloma from other Diseases

Choroid plexus papilloma may be differentiated from:

• Choroid plexus carcinoma
• Choroid plexus metastasis

Among children, choroid plexus papilloma must be differentiated from:

• Medulloblastoma
• Atypical teratoid rhabdoid tumor (AT/RT)
• Papillary anaplastic ependymoma
• Pineocytoma
• Pineoblastoma

Among adults, choroid plexus papilloma must be differentiated from:

• Intraventricular meningioma
• Subependymoma
• Central neurocytoma

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.
• They are far more common than the more aggressive choroid plexus carcinoma (WHO grade III), which they outnumber 5:1.

Age

• Choroid plexus papilloma is a rare disease that tends to affect children and adults.
• 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.

Risk Factors

• Common risk factors in the development of [disease name] are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].

Natural History, Complications and Prognosis

• The majority of patients with [disease name] remain asymptomatic for [duration/years].
• Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
• If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation ].
• Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
• Prognosis is generally [excellent/good/poor], and the [1/5/10year mortality/survival rate] of patients with [disease name] is approximately [#%].

Diagnosis

Diagnostic Criteria

• The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met:

• [criterion 1]
• [criterion 2]
• [criterion 3]
• [criterion 4]

Symptoms

• [Disease name] is usually asymptomatic.
• Symptoms of [disease name] may include the following:

• [symptom 1]
• [symptom 2]
• [symptom 3]
• [symptom 4]
• [symptom 5]
• [symptom 6]

Physical Examination

• Patients with [disease name] usually appear [general appearance].
• Physical examination may be remarkable for:

• [finding 1]
• [finding 2]
• [finding 3]
• [finding 4]
• [finding 5]
• [finding 6]

Laboratory Findings

• There are no specific laboratory findings associated with [disease name].
• A [positive/negative] [test name] is diagnostic of [disease name].
• An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
• Other laboratory findings consistent with the diagnosis of [disease name] include immunohistochemistry, [abnormal test 2], and [abnormal test 3].

Immunohistochemistry

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

Imaging Findings

• There are no [imaging study] findings associated with [disease name].
• [Imaging study 1] is the imaging modality of choice for [disease ame].
• On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
• [Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].

Other Diagnostic Studies

• [Disease name] may also be diagnosed using [diagnostic study name].
• Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].

Treatment

Medical Therapy

• There is no treatment for [disease name]; the mainstay of therapy is supportive care.
• The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
• [Medical therapy 1] acts by [mechanism of action1].
• Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

Surgery

• Surgery is the mainstay of therapy for [disease name].
• [Surgical procedure] in conjunction with [chemotherapy/radiation] is he most common approach to the treatment of [disease name].
• [Surgical procedure] can only be performed for patients with [disease tage] [disease name].

Prevention

• There are no primary preventive measures available for [disease name].
• Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].
• Once diagnosed and successfully treated, patients with [disease name] are followedup every [duration]. Followup testing includes [test 1], [test 2], and [test 3].

References

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