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childhood choroid plexus cancer

Description

Childhood Choroid Plexus Cancer: A Rare but Aggressive Brain Tumor

Choroid plexus cancer, also known as choroid plexus carcinoma (CPC), is a rare and aggressive form of brain cancer that primarily affects children. It accounts for only 3% of brain tumors in children, with the majority occurring in infants and children under the age of 5 [1].

Characteristics and Symptoms

Choroid plexus carcinoma grows faster than its benign counterpart, choroid plexus papilloma, and is more likely to spread through the cerebrospinal fluid (CSF) to other tissues. This can lead to a buildup of CSF in the brain, causing increased pressure on the brain and potentially resulting in hydrocephalus [7].

Treatment Options

The primary treatment for choroid plexus carcinoma includes surgery to remove the tumor, followed by chemotherapy and radiation therapy to shrink any remaining tumor cells or prevent recurrence [3]. The goal of treatment is to remove as much of the tumor as possible while minimizing damage to surrounding brain tissue.

Prognosis and Survival Rates

Unfortunately, the prognosis for children diagnosed with choroid plexus carcinoma is poor, especially in cases where the tumor has acquired many mutations. Children under the age of 2 are more likely to be affected, and those with a TP53 mutation have a particularly poor survival prognosis [10].

Rare Genetic Associations

Choroid plexus carcinoma has been associated with rare genetic conditions, such as Li-Fraumeni syndrome, which increases the risk of developing this aggressive brain cancer [8].

In summary, childhood choroid plexus cancer is a rare but aggressive form of brain cancer that primarily affects infants and young children. Early diagnosis and treatment are crucial to improving survival rates and minimizing long-term damage.

References: [1] Context result 1 [3] Context result 3 [7] Context result 7 [8] Context result 8 [10] Context result 10

Additional Characteristics

  • Choroid plexus carcinoma
  • rare and aggressive form of brain cancer
  • primarily affects children
  • accounts for only 3% of brain tumors in children
  • grows faster than choroid plexus papilloma
  • more likely to spread through the cerebrospinal fluid (CSF) to other tissues
  • can lead to a buildup of CSF in the brain, causing increased pressure on the brain and potentially resulting in hydrocephalus
  • primary treatment includes surgery, chemotherapy, and radiation therapy
  • goal of treatment is to remove as much of the tumor as possible while minimizing damage to surrounding brain tissue
  • prognosis for children diagnosed with choroid plexus carcinoma is poor, especially in cases where the tumor has acquired many mutations
  • children under the age of 2 are more likely to be affected, and those with a TP53 mutation have a particularly poor survival prognosis
  • associated with rare genetic conditions, such as Li-Fraumeni syndrome

Signs and Symptoms

Symptoms of Childhood Choroid Plexus Tumor

Choroid plexus tumors in children can cause a range of symptoms due to increased pressure in the brain. The most common signs and symptoms include:

  • Headache: This is often the first symptom, particularly in the mornings [9].
  • Pressure headaches: These can be severe and may be accompanied by nausea or vomiting.
  • Weakness or loss of sensation in the arms and/or legs: As the tumor grows, it can put pressure on nearby nerves, leading to weakness or numbness in the arms and/or legs.
  • Speech, vision, or swallowing difficulties: In some cases, the tumor may affect areas of the brain that control speech, vision, or swallowing, leading to difficulties with these functions.

It's essential for parents or caregivers to be aware of these symptoms and seek medical attention if they suspect a child has a choroid plexus tumor. Early diagnosis and treatment can significantly improve outcomes for children with this condition.

References:

  • [9] The first symptoms of a choroid plexus carcinoma may, therefore, be pressure headaches, particularly in the mornings.
  • [10] Symptoms of Childhood Choroid Plexus Tumor.

Additional Symptoms

  • Pressure headaches
  • or legs
  • Speech, vision, or swallowing difficulties
  • headache

Diagnostic Tests

Diagnostic Tests for Childhood Choroid Plexus Cancer

Childhood choroid plexus cancer, a rare and aggressive brain tumor, requires accurate diagnosis to determine the best course of treatment. The following diagnostic tests are commonly used to diagnose this condition:

  • Neurological Exam: A thorough neurological exam is performed to assess the child's vision, hearing, balance, coordination, and reflexes. This can help identify any potential issues related to the tumor's location in the brain.
  • Brain Imaging: Advanced imaging techniques such as Magnetic Resonance Imaging (MRI) and Computerized Tomography (CT) scans are used to visualize the tumor and its surrounding tissues. These tests provide valuable information about the tumor's size, location, and potential impact on nearby structures.
  • Biopsy: A biopsy is performed to obtain a tissue sample from the tumor. This sample is then examined by a pathologist under a microscope to confirm the diagnosis of choroid plexus cancer.

Additional Diagnostic Tests

Other diagnostic tests may be ordered depending on the child's specific condition and medical history. These can include:

  • Molecular Testing: To determine the genetic makeup of the tumor, molecular testing is performed to identify any potential mutations or abnormalities.
  • Imaging Techniques: Additional imaging techniques such as Nuclear Medicine Scans (NSG) may be used to further evaluate the tumor's extent and potential impact on surrounding tissues.

Early Detection and Accurate Diagnosis

Prompt and accurate diagnosis are crucial for effective treatment of childhood choroid plexus cancer. A multidisciplinary team of healthcare professionals, including neurosurgeons, oncologists, and radiologists, work together to provide comprehensive care and develop a personalized treatment plan for each child.

References:

  • [1] (Context 3) - The Childhood Brain Tumor Center at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center is a world-renowned destination for children with malignant and non-malignant brain and spinal cord tumors.
  • [2] (Context 4) - Doctors test for choroid plexus tumors in several ways, including a biopsy to diagnose CPT.
  • [3] (Context 6) - Physical and neurological examination; Brain imaging including magnetic resonance imaging (MRI) scan of the child's brain.
  • [4] (Context 11) - Tests and procedures used to diagnose choroid plexus carcinoma include: Neurological exam, MRI or CT scans.

Treatment

Treatment Options for Childhood Choroid Plexus Cancer

Childhood choroid plexus cancer, a rare and aggressive brain tumor, requires prompt and effective treatment to improve survival rates. While surgery is often the primary treatment approach, drug therapy plays a crucial role in managing this condition.

Chemotherapy

Chemotherapy is a common treatment option for childhood choroid plexus cancer. The goal of chemotherapy is to kill cancer cells that may have spread beyond the original tumor site. According to search results [2], treatments include surgery to remove the tumor and other treatments like chemotherapy and radiation to shrink any remaining bits of tumor or prevent it from coming back.

Targeted Therapy

Targeted therapy, a type of drug treatment, has shown promise in treating childhood choroid plexus cancer. This approach involves using medications that specifically target cancer cells, reducing harm to healthy cells. The ifosfamide, carboplatin, and etoposide (ICE) regimen [9] has been successfully used for choroid plexus carcinoma in young children.

Clinical Trials

Clinical trials are ongoing to explore new treatment options for childhood choroid plexus cancer. These trials involve testing innovative therapies like intra-arterial chemotherapy [11], which delivers medication directly to the tumor site, reducing side effects. Additionally, researchers are investigating the use of vorinostat and temozolomide in treating relapsed or refractory primary brain tumors [13].

Treatment Approaches

The healthcare team will suggest treatments based on your child's needs and work with you to develop a treatment plan [7]. Newly diagnosed choroid plexus tumors may be treated with surgery, radiation therapy, or chemotherapy. Recurrent choroid plexus tumors may be treated with surgery, followed by radiation therapy, chemotherapy, or both.

References

  • Search result 2: Choroid plexus carcinoma is a rare, aggressive brain cancer that most often affects infants and children.
  • Search result 9: The ifosfamide, carboplatin, and etoposide (ICE) regimen has been successfully used for choroid plexus carcinoma in young children.
  • Search result 11: Intra-arterial chemotherapy for newly diagnosed, residual, or recurrent atypical choroid plexus papilloma (ACPP) and choroid plexus carcinoma (CPC).
  • Search result 13: Vorinostat and temozolomide in treating young patients with relapsed or refractory primary brain tumors or spinal cord tumors.

Recommended Medications

  • Chemotherapy
  • Targeted Therapy
  • Intra-arterial chemotherapy
  • Ifosfamide, carboplatin, and etoposide (ICE) regimen
  • Vorinostat and temozolomide

💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.

Differential Diagnosis

Differential Diagnoses for Childhood Choroid Plexus Cancer

Childhood choroid plexus cancer, also known as choroid plexus carcinoma (CPC), is a rare and aggressive brain cancer that primarily affects infants and children. When diagnosing CPC, it's essential to consider other possible conditions that may exhibit similar symptoms or characteristics.

Possible Differential Diagnoses:

  • Ependymoma: A type of brain tumor that arises from the ependymal cells lining the ventricles and central canal of the spinal cord.
  • Intraventricular Meningioma: A rare, non-cancerous tumor that grows in the fluid-filled spaces within the brain.
  • Subependymoma: A slow-growing, benign tumor that develops near the ependymal cells.
  • Central Neurocytoma: A rare, benign tumor that occurs in the central part of the brain.
  • Exophytic Glioma: A type of brain tumor that grows outward from the surface of the brain.

Other Considerations:

  • Choroid Plexus Papilloma: A non-cancerous tumor that arises from the choroid plexus, which can be a precursor to CPC.
  • Papillary Ependymoma: A rare type of ependymal tumor that may exhibit similar characteristics to CPC.

Key Factors for Differential Diagnosis:

  • Age: Most CPC cases occur in children under 5 years old.
  • Imaging Patterns: While imaging patterns are not always diagnostic, they can help narrow the differential diagnosis.
  • Histological Analysis: A thorough examination of tumor tissue is crucial for accurate diagnosis and differentiation from other CNS tumors.

References:

  • [3] Lack of necrosis and cerebral parenchymal invasion may favor choroid plexus papilloma over CPC.
  • [4] The differential diagnosis of a choroid plexus tumor includes several conditions, including ependymoma, astrocytoma, germinoma, meningioma, xanthogranuloma, and others.
  • [9] Ependymoma is one of the possible differential diagnoses for CPC, along with intraventricular meningioma, subependymoma, central neurocytoma, and exophytic glioma.

Note: The above information is based on the provided context and may not be an exhaustive list of all possible differential diagnoses.

Additional Differential Diagnoses

Additional Information

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It is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified healthcare provider with questions about your medical condition.