Rare Childhood Tumour

Essential Information

Summarize

1. Childhood rare cancer refers to a cancer that is extremely rare in children.

Cancer in childhood and adolescence is generally rare. Since 1975, although the number of new cases of childhood cancer has risen slowly, it is reassuring that the number of deaths from childhood cancer has more than halved.

Rare cancers in childhood are even rarer, with most children's hospitals seeing only a few types within a few years. Due to their rarity, there is very little information available on which treatment is most effective. Treatment for children with rare cancers often relies on the experiences of other patients. Sometimes, doctors can only refer to the diagnostic, treatment, and follow-up reports of one or a small group of patients who have undergone the same type of treatment to gain insights.

(This section covers many different types of cancer. We will discuss them by body part.)

2. Detection methods for the detection, diagnosis and staging of rare cancers in childhood.

The choice of detection method for identifying, diagnosing, and staging cancer depends on the type of cancer. After a cancer diagnosis, it is crucial to check if the cancer cells have spread to other parts of the body. This process is known as cancer staging. The information from these staging tests helps doctors determine the stage of cancer development, which is essential for developing the best treatment plan.

The following tests and procedures can be used to detect, diagnose and stage cancer:

A. Physical examination and medical history: A physical examination is a general check of the body's health, including signs of disease such as lumps or anything that seems abnormal. Medical history is a record of a patient's health habits, past illnesses and treatments.

B. Blood chemistry tests: a method of detecting the amount of certain substances released into the blood by organs and tissues in the body. Certain substances may be too high or too low, which may be a sign of disease.

C. X-rays: A beam of energy that penetrates the body and forms an image on film.

d. CT scan (also known as CAT scan): This is a diagnostic procedure that uses X-rays to capture detailed images of the body's interior from multiple angles. The images are produced by a computer connected to an X-ray machine. This process is also known as computed tomography (CT) or computerized axial tomography (CAT).

e. Positron Emission Tomography (PET scan): This is a method used to detect malignant tumor cells in the body. Before the scan, healthcare providers will inject a small amount of radioactive glucose into the patient's vein. During the scan, the PET scanner rotates around the patient's body to capture images, observing how the glucose is absorbed by the body. Malignant tumor cells appear brighter on the image because they are more active and absorb more glucose than normal cells.

f. Magnetic Resonance Imaging (MRI, magnetic resonance imaging): A type of examination that uses a magnetic field and radio waves to take a series of detailed images of the body's internal regions. The images are created by a computer. This process is also known as Nuclear Magnetic Resonance Imaging (NMRI, nuclear magnetic resonance imaging).

G. Ultrasound examination: a type of examination that produces echoes and images of tissues or organs in the body through high-energy sound waves. The image of the tissue formed by the echo is called an acoustic image. The photo can be printed for later viewing.

h. Endoscopy: A method for detecting abnormal organs and tissues within the body. An endoscope is a thin, flexible tube equipped with a light and a lens for observation, which can be inserted through a skin incision or an opening in the body (such as the mouth or rectum). It can also be used to collect tissue or lymph node samples, which can then be examined under a microscope for signs of disease.

I. Bone scan: This is a test to detect the presence of rapidly dividing cells (such as cancer cells) in the bones. Before the scan, health care providers inject a small amount of radioactive material into the patient's veins. The radioactive material accumulates in the bones affected by cancer and is then detected by a scanner.

J. Biopsy (biopsy for short): This is a procedure in which cells or tissue from the patient are removed so that a pathologist can look under a microscope for signs of cancer.

There are many different types of biopsies, and the most common types include:

Fine needle aspiration (FNA) biopsy: Use a fine needle to obtain tissue or fluid.

Core biopsy: tissue is taken with a wide needle.

Incisional biopsy: A portion of the mass or tissue that appears abnormal is removed.

Biopsy: Remove an entire mass or area that appears abnormal.

3. There are three ways in which cancer can spread in the body.

Most malignant tumors are invasive and can spread throughout the body in various ways. Malignant tumors spread through three main pathways: tissue, lymphatic system, and blood. Tissue spread involves cancer spreading from its origin to nearby areas. Lymphatic system spread occurs when cancer enters the lymphatic system and spreads to other parts of the body through the lymphatic vessels. Blood spread happens when cancer enters the bloodstream and spreads to other parts of the body through the blood vessels

4. Cancer may spread from the primary site to other parts of the body.

When the primary tumor spreads to another part of the body, it is referred to as metastasis (metastatic tumor). A metastatic tumor is a cancer of the same type as the original tumor. For example, if renal cell carcinoma spreads to the bones, the cancer cells in the bones are actually cancerous renal cells, making this condition metastatic renal cell carcinoma, not bone cancer. In many cancers, when cancer moves from the original tumor and spreads to other tissues and organs, it can lead to the patient's death.

Epidemiological

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Etiology & Risk Factors

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Classification & Staging

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Clinical Manifestations

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Clinical Department

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Examination & Diagnosis

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Clinical Management

1. There are different types of treatment for children with rare cancers.

There are various treatment options available for children with cancer. Some treatments are standard therapies currently in use, while others are still in the testing phase of clinical trials. Therapeutic clinical trials aim to improve current treatments or gather information on new therapies for cancer patients. If a new therapy proves more effective than the standard treatment in clinical trials, it may become the new standard.

Because childhood cancers are extremely rare, participation in clinical trials should be considered. Some clinical trials are only open to children who have not yet started treatment.

2. Treatment plans for children with rare cancers should be developed by a healthcare team of cancer specialists.

The treatment of cancer patients should be supervised by pediatric oncologists, specialists in the medical care of children with cancer and in certain medical fields. Pediatric oncologists work with other pediatric health care providers. The team may include the following specialists:

pediatrician

A pediatric surgeon

Pediatric hematologist

neurosurgeon

Neurologist

neuropathologist

neuroradiologist

Radiologist, oncology

Pediatric nursing specialist

Rehabilitation specialist

endocrinologist

social work

psychologist

3. Seven existing standard treatments:

3.1 Surgical operations

There are three purposes of surgery: to find out if cancer is present, to remove it from the body, and to repair parts of the body. Palliative surgery is designed to relieve symptoms caused by cancer.

After a surgeon has removed all the tumors found during surgery, some patients may also need to receive chemotherapy or radiation therapy after surgery to kill any remaining cancer cells in the body. The treatment used after surgery to reduce the risk of cancer recurrence is called adjuvant therapy.

3.2 Radiotherapy

Radiation therapy (RT) is a cancer treatment that uses high-energy rays (X-rays or other types of radiation) to kill cancer cells or prevent them from growing. There are several different types of radiation therapy:

External beam radiation therapy: Radiation is delivered to the cancer site using an external radiation machine.

Proton beam radiation therapy (PBRT) is a high-energy form of external radiation therapy. Radiation therapy aims at a stream of protons (tiny, invisible, positively charged particles) on cancer cells and then kills them. This type of treatment causes less damage to nearby healthy tissue.

Intracorporeal radiotherapy: Radioactive material is injected directly into the body, or sealed in a needle, particle (seed), wire or catheter and placed directly into or near the cancer lesion for treatment.

Radioembolization is a form of in vivo radiotherapy used to treat neuroendocrine tumors of the liver. In this procedure, healthcare professionals attach a small amount of radioactive material to tiny beads, which are then injected into the patient's hepatic artery via a catheter. The beads mix with the material that blocks the artery, cutting off the blood flow to the tumor. As a result, most of the radiation is trapped near the tumor, allowing it to kill cancer cells at a close range.

Radioactive iodine (131I-MIBG) therapy is a form of in vivo radiation therapy used to treat pheochromocytoma and paraganglioma. Radioactive iodine is injected into the bloodstream through a vein, where it accumulates in certain types of tumor cells and is used to kill them with the radiation emitted.

The type of radiation therapy a patient should use depends on the type of cancer being treated.

3.3 Chemotherapy

Chemotherapy, or simply 'chemotherapy,' is a cancer treatment that uses drugs to inhibit the growth of cancer cells (either by killing them or preventing their division). When chemotherapy drugs are administered orally or through injection into the veins or muscles, they enter the bloodstream and affect cancer cells throughout the body (known as systemic chemotherapy). When drugs are directly injected into the cerebrospinal fluid, body cavities (such as the abdominal cavity), or specific organs, they primarily affect the cancer cells in those areas. Combination chemotherapy involves using more than one anticancer drug simultaneously. The choice of chemotherapy regimen depends on the type and stage of the cancer being treated.

3.4. Hormone therapy

Hormone therapy is a cancer treatment that works by either removing hormones or blocking their effects to inhibit the growth of cancer cells. Hormones are produced by glands in the body and circulate in the bloodstream. Some hormones can promote the growth of certain cancers. If tests reveal that cancer cells have receptors for these hormones, treatments such as medication, surgery, or radiation therapy may be used to reduce hormone production or block their function. In the treatment of thymoma or thymic cancer, corticosteroid hormone therapy is often used.

Growth hormone inhibitors, such as octreotide or lanreotide, are used to treat neuroendocrine tumors that have spread or cannot be surgically removed. When administered subcutaneously or intramuscularly, these drugs prevent the tumor from producing excessive hormones. Sometimes, these drugs are attached with a small amount of radioactive material, allowing them to kill cancer cells through radiation, a process known as peptide receptor radioisotope therapy.

3.5 Immunotherapy

Immunotherapy is a treatment that uses the patient's immune system to fight cancer. Immunotherapy drugs, either synthesized in the body or made in the laboratory, enhance, control, or restore the body's natural defenses against cancer. This type of cancer treatment is also known as biological therapy.

Interferon: Interferon slows tumor growth by interfering with the division of cancer cells. It is used to treat nasopharyngeal carcinoma and papilloma.

Epstein-Barr virus (EBV)-specific cytotoxic T lymphocytes are a type of white blood cell (T lymphocyte) in the patient's body. After being modified by EB virus in the lab and reinfused into the patient, these cells can stimulate the immune system to fight cancer. Research is ongoing on using EB virus-specific cytotoxic T lymphocytes to treat nasopharyngeal carcinoma.

Vaccine therapy: Vaccine therapy uses certain substances (cancer cell proteins) to stimulate the immune system and destroy tumors. Vaccine therapy can be used to treat papillomas.

Immunotherapy with immune checkpoint inhibitors: Certain types of immune cells, such as T cells and some cancer cells, have proteins on their surfaces that restrict the immune response. These proteins are known as checkpoint proteins. When cancer cells have a high level of these checkpoint proteins, they are less likely to be targeted and killed by T cells. Immune checkpoint inhibitors can block these proteins, thereby enhancing the ability of T cells to attack and kill cancer cells. CTLA-4 is an example of an immune checkpoint inhibitor.

CTLA-4 is a protein on the surface of T cells that helps suppress the body's immune response. When CTLA-4 binds to a protein called B7 on cancer cells, it prevents T cells from attacking these cells. However, when a CTLA-4 inhibitor binds to CTLA-4, it reactivates T cells, enabling them to attack cancer cells. Ipilimumab, a CTLA-4 inhibitor, has not been tested in children, but it may be considered for high-risk melanoma patients who have had their tumors completely removed through surgery.

3.6. Observational waiting

Observational waiting involves closely monitoring a patient's condition without giving any treatment until signs or symptoms of the disease appear or change. It can be used when the tumor is growing slowly or is likely to disappear without treatment.

3.7. Targeted therapy

Targeted therapy is a treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Several targeted therapies are used to treat rare childhood cancers:

Kinase inhibitors: These targeted therapy drugs block the signals essential for tumor growth. Vandetanib and Cabozantinib are used to treat medullary thyroid cancer. Sunitinib is used to treat pheochromocytoma, paraganglioma, neuroendocrine tumors, and thymic cancer. Crizotinib is used to treat tracheobronchial tumors. Everolimus is used to treat tumors of the heart, neuroendocrine system, and pancreatic cells.

Monoclonal Antibodies: This type of targeted therapy uses antibodies produced in the laboratory from a single type of immune system cell. These antibodies can target substances on cancer cells or normal substances that may aid in their growth, attaching to these targets to kill cancer cells, inhibit their growth, or prevent their spread. Monoclonal antibodies are administered via intravenous infusion. They can be used alone or as carriers to deliver drugs, toxins, or radioactive materials directly into cancer cells. Bevacizumab (Bevacizumab) is a monoclonal antibody used to treat papillomatosis.

Histone methyltransferase inhibitors: This type of targeted therapy can slow the growth and division of cancer cells. A study is currently underway to treat recurrent chordoma with Tazametostat.

Targeted treatments for rare childhood cancers are being studied.

4. Novel therapies in clinical trials.

Use Cabozantinib-S-malic acid for the treatment of young patients with recurrent, refractory or newly diagnosed sarcoma, Wilms tumor, or other rare tumors:

Status: Recruiting

Age: 2 to 30 years old

Gender: Not limited

Location: 123 different locations

Web site: https://clinicaltrials.gov/ct2/show/NCT02867592

Molecular oriented treatment of childhood cancer:

Status: In progress (recruitment stopped)

Age: 13 months to 21 years old

Gender: Not limited

Venue: 5 different venues

Web site: https://clinicaltrials.gov/ct2/show/NCT02162732

Targeted radiotherapy for pulmonary metastases of Ewing's sarcoma, rhabdomyosarcoma or nephroblastoma:

Status: Recruiting

Age: 21 and under

Gender: Not limited

Venue: Three different venues

Web site: https://clinicaltrials.gov/ct2/show/NCT02581384

Research on young patients with renal tumors

Status: Recruiting

Age: 29 and under

Gender: Not limited

Location: 194 different locations

Web site: https://clinicaltrials.gov/ct2/show/NCT00898365

5. Gene therapy

Gene therapy is a treatment that inserts foreign genetic material (DNA or RNA) into human cells to prevent and fight disease. Gene therapy for papilloma is under study.

The treatment of rare childhood cancers can cause side effects

Late effects are side effects that begin after cancer treatment and last for months or years. Late effects of cancer treatment may include:

physical problem 。

Changes in mood, feelings, thinking, learning or memory.

Secondary tumors (new cancers).

Some late effects may be treated or controlled, so it is important to talk to your child's doctor about the possible late effects of cancer and cancer treatment ——. (For more information, see the PDQ summary of late effects of Childhood Cancer Treatment: https://www.cancer.gov/types/childhood-cancers/late-effects-pdq).

Prognosis

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Follow-up & Review

Follow-up tests may be required.

Patients may need to repeat some of the tests used to diagnose cancer or stage cancer. Some of these tests are repeated to see how well the treatment is working. The results of these tests determine whether the treatment should continue, be changed, or stopped.

Some tests need to be done periodically after treatment. The results of these tests can show whether the child's condition has changed or if the cancer has returned. Some sources call these tests follow-up tests.

Daily Care

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Cutting-edge therapeutic and clinical Trials

Patients may want to consider participating in a clinical trial.

For some patients, participating in a clinical trial may be the best treatment option. Clinical trials are part of cancer research and are conducted to find out whether new cancer treatments are safe and effective or better than standard care.

Many of today's standard cancer treatments are based on early clinical trials. Patients who participate in clinical trials can receive standard care or be the first to receive a new treatment.

Patients who participate in clinical trials can help improve the way cancer is treated in the future. Even if clinical trials do not lead to effective new therapies, they often answer important questions in research and help advance research.

Patients can enroll in clinical trials before, during or after cancer treatment.

Some clinical trials only accept patients who have not yet started treatment, some look at patients who have not improved after cancer treatment, and others aim to test new ways to prevent cancer recurrence or reduce the side effects of cancer treatment.