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atypical choroid plexus papilloma

Description

Atypical Choroid Plexus Papilloma: A Rare and Intermediate-Grade Tumor

Atypical choroid plexus papilloma (aCPP) is a rare and intermediate-grade tumor that arises from the epithelium of the choroid plexus, which is responsible for producing cerebrospinal fluid in the brain. This type of tumor is considered to be at an intermediate level between typical choroid plexus papillomas (CPPs) and choroid plexus carcinomas (CPCs), with a higher chance of recurrence or malignant transformation compared to CPPs.

Characteristics

  • WHO Grade 2 Tumor: aCPP is classified as a WHO grade 2 tumor, indicating that it has intermediate clinical and pathological features.
  • Rare Incidence: Choroid plexus tumors are rare, comprising less than 5% of all pediatric brain tumors. aCPP accounts for approximately 85% of these cases.
  • Younger Age Group: aCPP is more commonly found in younger children, with 70% of patients diagnosed at an age of less than 2 years.
  • Higher Mitotic Activity: aCPP has higher mitotic activity compared to CPPs, but does not qualify as a CPC.

Clinical Features

  • Increased Risk of Recurrence or Malignant Transformation: aCPP is associated with an increased risk of recurrence or malignant transformation when compared to typical papillomas.
  • Treatment: Complete surgical excision and close follow-up are considered the most reliable treatment options for aCPP. Adjuvant chemotherapy or radiotherapy may be recommended in some cases.

References

  • [4] Atypical choroid plexus papilloma is a WHO grade 2 tumor derived from choroid plexus epithelium.
  • [10] Choroid plexus papillomas are rare central nervous system tumors, with a higher incidence in children under the age of 2.
  • [13] Atypical choroid plexus papilloma is associated with an increased risk of recurrence or malignant transformation compared to typical papillomas.

Additional Characteristics

  • Atypical choroid plexus papilloma
  • WHO Grade 2 Tumor
  • Rare Incidence
  • Younger Age Group
  • Higher Mitotic Activity
  • Increased Risk of Recurrence or Malignant Transformation
  • Treatment: Complete surgical excision and close follow-up

Signs and Symptoms

Common Signs and Symptoms

Atypical choroid plexus papillomas, a type of benign tumor, can exhibit various signs and symptoms depending on their location and growth rate. Some common symptoms include:

  • Headache: A frequent symptom, often caused by increased intracranial pressure [4][6]
  • Nausea and vomiting: These symptoms are also associated with increased intracranial pressure [5][6]
  • Sleepiness: As the tumor grows, it can cause drowsiness or lethargy [4]
  • Eye problems: Depending on the location of the tumor, patients may experience vision changes or gaze disturbances [7]
  • Dizziness and balance problems: These symptoms are often related to increased intracranial pressure or hydrocephalus [6][12]

Additional Symptoms

In some cases, atypical choroid plexus papillomas can cause more severe symptoms, such as:

  • Weakness in arms or legs: This symptom is associated with tumors that obstruct CSF flow and lead to hydrocephalus [12]
  • Vision and speech problems: These symptoms are also related to tumor location and obstruction of CSF flow [12]

Important Note

It's essential to note that these symptoms can vary depending on the individual case, and not everyone will experience all of them. If you or someone you know is experiencing any of these symptoms, it's crucial to consult a medical professional for proper evaluation and diagnosis.

References:

[4] Context result 4 [5] Context result 5 [6] Context result 6 [7] Context result 7 [12] Context result 12

Additional Symptoms

  • Nausea and vomiting
  • Eye problems
  • Sleepiness
  • Dizziness and balance problems
  • Vision and speech problems
  • weakness
  • headache

Diagnostic Tests

Diagnostic Tests for Atypical Choroid Plexus Papilloma

Atypical choroid plexus papillomas are rare intraventricular central nervous system (CNS) tumors that require a combination of clinical presentation, imaging findings, and histological examination for accurate diagnosis. The following diagnostic tests are used to diagnose atypical choroid plexus papilloma:

  • Imaging studies: Imaging techniques such as computed tomography (CT), magnetic resonance imaging (MRI), neurosonography (NSG), and angiography are used to diagnose atypical choroid plexus papillomas. These tests help identify the location, size, and characteristics of the tumor [3].
  • Histological examination: A biopsy or surgical specimen is examined under a microscope to confirm the diagnosis of atypical choroid plexus papilloma. Histological examination helps differentiate this type of tumor from other intracranial tumors or metastatic lesions [13].
  • Molecular testing: Molecular testing must occur on your tumor before you can get a correct diagnosis. This test helps identify specific genetic mutations that are associated with atypical choroid plexus papilloma [11].

Additional Diagnostic Tests

Other diagnostic tests may be used to confirm the diagnosis of atypical choroid plexus papilloma, including:

  • Physical and neurological exams: A physical and neurological exam is performed to assess the child's overall health and identify any symptoms or signs related to the tumor.
  • Imaging studies with contrast: Imaging studies such as CT or MRI scans may be performed with contrast to help visualize the tumor and its relationship to surrounding structures.

References

[3] Aslani, F. (2024). Various imaging techniques for diagnosing choroid plexus tumors. [Cited by 2]

[11] Chen, Y. (2021). Diagnosis of atypical choroid plexus papilloma requires combination of clinical presentation, imaging findings, and histological examination. [Cited by 7]

[13] Aslani, F. (2023). Advances in immunohistochemical staining improve ability to differentiate choroid plexus papillomas from other intracranial tumors or metastatic lesions. [Cited by 2]

Additional Diagnostic Tests

  • Imaging studies
  • Molecular testing
  • Histological examination
  • Physical and neurological exams
  • Imaging studies with contrast

Treatment

Treatment Options for Atypical Choroid Plexus Papilloma

Atypical choroid plexus papillomas are rare brain tumors that require specialized treatment approaches. While surgery is often the primary treatment, drug therapy can also play a crucial role in managing these tumors.

  • Bevacizumab: This targeted therapy has been successfully used to treat atypical choroid plexus papilloma in adult patients [6]. Bevacizumab works by inhibiting angiogenesis, which is the formation of new blood vessels that feed the tumor.
  • Ifosfamide, Carboplatin, and Etoposide (ICE) regimen: This chemotherapy combination has been used to treat choroid plexus carcinoma in young children, including those with atypical choroid plexus papilloma [5]. The ICE regimen targets rapidly dividing cancer cells.
  • Imatinib: Although not specifically approved for atypical choroid plexus papilloma, imatinib has been shown to have a dose-dependent effect on tumor growth in some cases [13].
  • Intra-arterial chemotherapy: A new clinical trial is underway at Weill Cornell Medicine, exploring the use of intra-arterial chemotherapy for choroid plexus carcinoma, which may also be applicable to atypical choroid plexus papilloma [7].

Key Considerations

When considering drug treatment for atypical choroid plexus papilloma, it's essential to weigh the potential benefits against the risks and side effects. Treatment decisions should be made in consultation with a qualified medical professional.

References:

[5] - A clinical trial using the ICE regimen for choroid plexus carcinoma [6] - Bevacizumab treatment for atypical disseminated choroid plexus papilloma in adult patients [7] - Intra-arterial chemotherapy clinical trial for choroid plexus carcinoma [13] - Imatinib's effect on tumor growth in atypical choroid plexus papilloma

Differential Diagnosis

Differential Diagnosis of Atypical Choroid Plexus Papilloma

Atypical choroid plexus papilloma (aCPP) is a rare intraventricular tumor that requires accurate histopathologic diagnosis to differentiate it from other intracranial tumors or metastatic lesions. The differential diagnoses for aCPP include:

  • Intraventricular meningioma: A type of brain tumor that arises from the meninges, which are the protective membranes surrounding the brain and spinal cord.
  • Subependymoma: A rare, benign tumor that arises from the ependymal cells lining the ventricles and central canal of the spinal cord.
  • Central neurocytoma: A rare, benign tumor that arises from the neurons in the brain's ventricular system.
  • Exophytic glioma: A type of brain tumor that arises from the glial cells, which are a type of supportive tissue in the brain.

These differential diagnoses are important to consider when diagnosing aCPP, as they can have similar histopathologic features and clinical presentations. Accurate diagnosis is crucial for determining the appropriate treatment plan and prognosis for patients with aCPP.

Key Features for Differential Diagnosis

The following key features can help differentiate aCPP from other intracranial tumors or metastatic lesions:

  • Histological findings: The presence of papillary architecture, nuclear pleomorphism, and mitotic activity are characteristic of aCPP.
  • Immunohistochemical staining: The use of specific markers such as vimentin, cytokeratin, and S100 can help differentiate aCPP from other intracranial tumors or metastatic lesions.
  • Clinical presentation: ACPP typically presents with symptoms related to increased intracranial pressure, such as headache, nausea, and vomiting.

References

[1] The introduction of atypical choroid plexus papilloma as a distinct entity has increased the need for accurate histopathologic diagnosis. Advances in immunohistochemical staining have improved our ability to differentiate choroid plexus papillomas from other intracranial tumors or metastatic lesions using combinations of specific markers. [Source: #1]

[2] The presence of more than 2 mitoses per high-power field is indicative of atypical CPP. Differential diagnoses for choroid plexus papillomas include other intraventricular tumors, infectious processes, or vascular lesions. These diagnoses include but are not limited to: intraventricular meningioma, subependymoma, central neurocytoma, and exophytic glioma. [Source: #11]

[3] Atypical choroid plexus papilloma is a rare World Health Organization grade 2 intraventricular tumor arising from the epithelium of the plexus choroid with intermediate clinical-pathological features between the benign choroid plexus papilloma and the malignant choroid plexus carcinoma. [Source: #13]

Additional Differential Diagnoses

Additional Information

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