Chemotherapy
Despite the success of surgery and chemotherapy in the early treatment of neuroblastoma, the long-term survival of children with advanced diseases is poor. A number of meta-analyses have addressed this issue and have shown that more aggressive treatment is necessary for patients with neuroblastoma with metastases. In this study, the Children’s Cancer Study Group (CCSG) conducted a randomized phase III trial comparing standard myeloablative chemotherapy, fractionated TBI, and intensive consolidation chemotherapy in patients with high-risk neuroblastoma.
The results showed that the combination of myeloablation and autologous hematopoietic cell rescue (AHR) significantly improved overall survival (OS). Specifically, the 5-year OS was around 40% in the randomized trial.
High-risk neuroblastoma requires extensive neoadjuvant chemotherapy. In this treatment, children are given chemotherapy before surgery. They may also receive radiation therapy to shrink the tumor before surgery. Some of the common chemotherapy agents used in the treatment of neuroblastoma are carboplatin, cyclophosphamide, and doxorubicin. In addition to these common therapies, children may also receive immunotherapy, such as CAR T-cell. These are experimental treatments that involve triggering the body’s immune system to attack cancer cells. They are currently used in conjunction with bone marrow transplantation.
A patient may also receive radiation therapy to kill cancer cells, which may be used before surgery or after stem cell transplantation. It is generally believed that the prognosis for radiation therapy is better than for chemotherapy alone. The risk of complications is low, but a child can experience nausea, vomiting, and diarrhea. Other complications include infection, fistula, and bleeding.
In addition to surgery, chemotherapy, and radiation, patients with neuroblastoma may also receive immunotherapy, such as CAR-T-cell. CAR-T-cell is a newer type of immunotherapy that uses cells from the immune system of the child. These cells will attack the cancer cells in the body. However, this is still a new technology, so the results are not yet conclusive.
Patients with neuroblastoma can be classified into three groups based on the stage of their disease. These groups include high-risk, intermediate-risk, and low-risk. The treatment plan for each group will vary depending on the original risk group, the child’s age, and the location of the tumor. A child with neuroblastoma should be referred to a pediatric hematologist/oncologist for the entire course of the disease.
High-risk neuroblastoma can be treated using neoadjuvant chemotherapy, myeloablative mega therapy, autologous bone marrow transplantation, and radiation therapy. In the Children’s Cancer Study Group trial, patients were randomized to receive either myeloablation or intensive consolidation chemotherapy at week 8. Patients in the high-risk group underwent neurosurgical debulking. The high-risk group was also given 13-cis-retinoic acid.
These agents are taken in two or three doses over a fortnight. This treatment may be added to high-dose chemotherapy or may be given after surgery. The high-dose therapy is usually inpatient.
High-risk neuroblastoma patients are also treated with immunotherapy. In addition to immunotherapy, children may be given iodine 131-MIBG therapy. The radioactive iodine 131-MIBG kills the tumor cells. This therapy is also administered on an outpatient basis. Patients with low-risk neuroblastoma can be managed with low-intensity neoadjuvant chemotherapy. However, the chemotherapy used will vary based on the patient’s age and risk group.
Surgery
Surgical treatment of neuroblastoma aims to remove cancer and reduce the risk of recurrence. It is considered ideal to remove the entire tumor, if possible. However, this can be difficult, and some children may not require surgery. Biological therapies may also be used. Neuroblastomas are tumors that form from nerve cells. They are usually found near the adrenal glands. They may cause lumps under the skin, and changes in bowel function. In addition, they may cause pain. They may be treated by surgery, chemotherapy, radiation, or biological therapies.
For most stages of neuroblastoma, surgery is a standard treatment. However, the extent of surgery and the complications associated with it are often debated. It is also difficult to determine if surgery has an effect on the survival of a child with neuroblastoma. The goal of surgery is to remove the tumor and to investigate whether the tumor has spread to other parts of the body.
Anomalies can be identified by using imaging technologies. In addition, tumor markers can be tested prior to surgery. Several prospective trials are now assessing the effectiveness of surgery on recurrence and survival.
Surgery for neuroblastoma may be performed by a multidisciplinary team. This may include oncologists, surgeons, oncology nurses, social workers, and psychologists. In addition, chemotherapy may be given prior to surgery. In addition, some children will receive immunotherapy. This is a type of cancer vaccine that targets two proteins in neuroblastoma cells. These antibodies may be given by IV or in an outpatient setting.
Neuroblastoma is a common pediatric cancer, accounting for 6% of all childhood cancers. It accounts for 12-15% of all cancer-related deaths in children. Surgery for neuroblastoma aims to remove all cancer and to ensure that the child is near-normal in size and growth. It is also important to follow up on the child’s condition to ensure that cancer does not recur. Anomalies can be identified by a panel of tumor markers, which are obtained through cross-sectional imaging.
Surgery for neuroblastoma is usually performed using a multidisciplinary team. This may include an oncologist, an oncology nurse, a surgeon, a child life therapist, a psychologist, and a rehabilitation specialist. In addition, chemotherapy may be given prior to, during, or after surgery. Chemotherapy may be used to shrink the tumor and may also be given to treat cancer that remains.
In addition, the International Neuroblastoma Surgical Report Form (INSRF) was developed as a standard for reporting surgery for neuroblastoma. It is an initiative of the International Pediatric Oncological Cooperative Group (COG), the German cooperative group (SIOP), and the German cooperative (GPOH). It aims to provide a common reporting format for the surgical treatment of neuroblastoma. It will hopefully influence clinical practice. The new report format includes a total of 111 patients who were treated from January 1, 2015, to April 1, 2021.
Neuroblastoma surgery was performed by pediatric surgeons at five academic centers. The average operative time was 85 minutes. The operative time was longer in patients with CIN. The CDC system was used to grade complications. However, none of the patients died during surgery.
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