Choroid plexus tumor (medicine)

Choroid plexus tumor (medicine)

Summarize

 

       The choroid plexus is a plexiform plexus of tissue in the ventricles of the brain consisting of the soft meninges and the recurrently branching blood vessels on them together with the ventricular epithelium.

 

       It is the main structure for the production of cerebrospinal fluid and consists of a large number of capillaries as well as choroidal epithelial cells separated from the ventricles of the brain, through which the plasma filters to form the cerebrospinal fluid, and on the other hand, the components of the cerebrospinal fluid enter and exit through the choroid plexus.

 

       Choroid plexus tumors usually occur in the ventricles of the brain. The cerebrospinal fluid produced by the choroid plexus tissue surrounds the brain and spinal cord, so as choroid plexus tumors grow, they block the flow of cerebrospinal fluid, which, after failing to move in and out of equilibrium, collects inside the skull and can increase intracranial pressure that can, over time, lead to a larger head.

 

       Choroid plexus tumors can be classified as benign or malignant. Most choroid plexus tumors are benign choroid plexus papillomas and atypical choroid plexus papillomas. These tumors grow slowly and rarely spread to other parts of the brain or spinal cord. Choroid plexus carcinomas, which are malignant tumors, grow rapidly and can spread through the cerebrospinal fluid and metastasize to other normal brain and spinal cord tissues.

 

Epidemiological

 

       Overall, choroid plexus tumors are very rare. The annual incidence of choroid plexus tumors is 0.3 cases per million. Although these tumors may appear in all age groups, their peak incidence is during adolescence.

 

       In adults, choroid plexus tumors account for less than 1% of primary intracranial tumors. In children, choroid plexus tumors, on the other hand, account for 3% of childhood brain tumors. They are most common in infants, and choroid plexus tumors account for 10 to 20% of all brain tumors that may occur in children from birth to one year of age. Of these, choroid plexus papillomas account for about 80% of all choroid plexus tumors, while choroid plexus carcinomas account for only 10-20% of choroid plexus tumors. Choroid plexus tumors occur in equal proportions in men and women.

 

Etiolog & Risk factors

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Classification & Stage

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Clinical Department

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Clinical manifestations

 

       Clinical manifestations of choroid plexus tumors, which are associated with increased intracranial pressure from the inability of cerebrospinal fluid to flow, include:

 

● Headaches: especially when waking up in the morning

● Irritability: for infants and toddlers who are unable to recount their discomfort, manifested as irritability

● Head enlargement: most common in infants

● Hydrocephalus: normal flow of cerebrospinal fluid is blocked, intracranial pressure rises, and the head becomes enlarged

● Nausea and vomiting: symptoms of nausea and vomiting tend to be worse in the morning and improve over the following day

● listless

● Impaired eating or walking

● Enlarged fontanel (xìn): most often seen in infants

● sideways glance

 

Examination & Diagnosis

 

       The first step in treating choroid plexus tumors in children is an accurate and complete diagnosis. The predominant diagnostic method for choroid plexus tumors is imaging combined with histopathology, including:

 

● Physical examination and complete disease history interrogation;

● Neurologic function tests, including reflexes, muscle strength, eye and mouth movements, coordination, and state of consciousness;

● Magnetic Resonance Imaging (MRI);

● A small sample of cerebrospinal fluid is removed by lumbar puncture for biopsy, a step that determines whether any tumor cells have spread.

 

       In addition to the above points, the doctor may suggest other diagnostic tests depending on the individual circumstances of the child.

 

Clinical Management

 

       The appropriate treatment is determined primarily by the child's age, medical history, the type, location and size of the tumor, and the extent of the disease.

 

       Choroid plexus tumor treatment may include the following:

 

 1. Neurosurgery

 

       For choroid plexus papillomas, the chances of successful resection are high, with 85% to 100% of the tumor likely to be removed and no further treatment required. For choroid plexus cancer, it is important to remove as much of the tumor as possible, but further treatment is needed.

 

2. Chemotherapy

 

       Chemotherapy drugs are medications that interfere with the ability of cancer cells to grow or reproduce and can be used before surgery to shrink tumors or after surgery to kill remaining cancer cells. Different chemotherapy drugs work in different ways and usually have a systemic effect.

 

       The child may receive oral chemotherapy; or intramuscular injections, such as injecting chemotherapy drugs into the muscle or under the skin, or intravenous injections, in which chemotherapy drugs are injected directly into the bloodstream, or intrathecal injections, in which chemotherapy drugs are instantly injected directly into the cerebrospinal fluid. Moreover, patients using combination therapies are treated with more than one chemotherapy drug at a time.

 

3. Radiotherapy

 

       This means that radiation (such as X-rays) is used to kill or shrink tumors, pinpointing and killing cancer cells that remain after a child's surgery. Radiotherapy is important to control the local growth of the tumor.

 

      However, due to the potential side effects of radiotherapy, such as effects on learning ability and hormone function, it is best avoided if the child is young (especially under 3 years of age).

 

Prognosis

  Patients with choroid plexus papillomas have excellent surgical outcomes. Patients with choroid plexus cancer have a fair prognosis and usually require aggressive treatment.

 

       The 5-year survival rate for children with choroid plexus papillomas can be 80-100% after complete removal of the tumor, compared to 70% for children with partial removal of the tumor. When surgery for choroid plexus cancer is combined with other treatments, the survival rate is about 60 to 65%.

 

       For children with progressive or recurrent choroidal plexus tumors, there are a variety of standard or experimental treatments available. For recurrent tumors, a second surgery followed by chemotherapy or radiation may be necessary.

 

 

Follow-up & Review

 

            Children who have completed treatment for choroid plexus tumors should be followed annually to check for recurrence or late onset of treatment side effects. Routine follow-up may include physical examination, laboratory biochemical markers and imaging tests.

 

     

Routine

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Cutting-edge Therapeutic & Clinical Research

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References

        1. St. Jude Children's Research Hospital. https://www.stjude.org/disease/choroid-plexus-tumor.html

        2. Boston Children's Cancer and Blood Disorders Center. http://www.danafarberbostonchildrens.org/conditions/brain-tumor/choroid-plexus-tumor.aspx

          3. Pathology of Choroid Plexus Neoplasms https://emedicine.medscape.com/article/1744050-overview

 

Audit specialists

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