Neuroblastoma, also known as neuroblastoma, is a cancer that occurs in the nerve branches. Because the nerve terminals are distributed to many parts of the body, this tumor can appear in many parts of the body, about 65% originate in the abdomen, 15% to 20% in the chest, and the remaining 15% in different areas such as the neck and pelvis. Neuroblastoma is a highly malignant tumor, often multiple, and can involve surrounding tissues at an early age.
Neuroblastoma occurs most often in infants and children and is the fourth most common childhood cancer, but remains a rare disease overall. Approximately 75% of patients develop the disease before the age of five and 50% before the age of two, and some patients have a familial predisposition.
The tumor can grow fast or slow, and can reach a large size with an unsmooth surface. It can metastasize through blood and lymph in early stages, and the metastases are widespread, among which skeletal metastases, especially cranial orbital metastases, and blood metastases mostly to the liver.
Neuroblastoma in the abdominal cavity usually originates from the adrenal medulla or the posterior sympathetic ganglion of the abdominal wall and presents as a non-painful mass felt in the lower back or abdomen, with calcified spots within the tumor visible on abdominal x-ray and a deviation of the kidney to the anterior and inferior sides.
If the tumor grows from the sympathetic ganglion next to the spine, it can be seen that the ipsilateral ureter will be pushed and deviated to the side, and sometimes hydronephrosis will occur due to the compression of the tumor. If the tumor grows from the ganglion next to the spine, it will lead to compression of the spine and cause neurological symptoms, such as paralysis of the lower limbs and weakness of the extremities. If it is a tumor in the posterior mediastinal cavity, it will lead to difficulty in breathing or pneumonia due to obstruction of breathing. If the tumor appears in the sympathetic ganglion of the neck, it will cause symptoms such as constricted pupils, drooping eyelids, and inversion of the eyeballs.
Some patients also develop metastatic symptoms first, such as metastatic subcutaneous nodules, hepatomegaly due to liver metastases, painful or lumpy bone metastases, and ocular metastases causing ptosis and petechiae near the upper and lower eyelids. More than half of the children will have bone marrow invasion, resulting in weight loss, anemia, enlarged lymph glands, fever, and restlessness.
Staging of the disease Cancer staging is very important to diagnose and treat the disease. Neuroblastoma can be classified as
Stage I: The tumor is confined to the original site of origin, without lymph node metastasis, and can be completely removed.
Stage II: The tumor has extended beyond the organ or tissue of origin, but has not crossed the midline of the body, and the local lymph nodes on the same side may have been invaded.
Stage III: The tumor has crossed the midline of the body and the local lymph nodes on both sides may have been invaded.
Stage IV: Metastasis to bone marrow, bone, liver, soft tissue, brain, skin, lung, distant lymph, etc.
Stage IV-S: The child has a small primary tumor within six months but has one or more metastases that are limited to the liver, skin, and bone marrow, but no skeletal invasion is found on a full body X-ray.
What tests do patients need to have?
1. 24-hour urine is collected and tested for catecholamine compounds in the urine. Approximately 80-95% of patients with neuroblastoma have high levels of catecholamines in their urine, and epinephrine, norepinephrine, and their metabolites, 3 methoxy 4 hydroxy bitter amygdalin, are all elevated in the urine. The measurement of urinary catecholamine metabolites can not only help diagnosis, but also help to judge the treatment effect and whether recurrence.
Blood tests: The tumor itself and the treatment may cause changes in blood cells. Regular blood tests should be performed during the treatment period to observe the changes in the types of white blood cells, as well as the number of white blood cells, red blood cells and platelets.
Bone marrow examination: To determine if bone marrow metastases are present, 70% of patients may have bone marrow metastases, so all children suspected of having neuroblastoma should have a bone marrow aspiration and biopsy before surgery.
4.Nuclear bone scan: It can help to detect bone metastasis, and osteolytic changes can be seen in X-ray bone film to help confirm neuroblastoma.
5.Computer scan: Computer scan is very sensitive to detect and show primary and recurrent tumors.
6.Ultrasound: It can help to identify the primary organ of the tumor.
7.Venous urography: If the tumor is in the abdomen, this test can help determine the tumor size, location and blood flow supply status.
There are three common treatments: surgery, radiation and chemotherapy. The specific treatment depends on the age of the child, the location and stage of the tumor, and only surgery is needed for low-risk patients. Stage I patients have a 95% survival rate after surgery, and intermediate risk patients have an 80-90% survival rate after chemotherapy. Children who have metastasized or spread are often treated with a combination of surgery, chemotherapy, radiation therapy, and bone marrow transplantation.
Surgical treatment must be preceded by correction of anemia and metabolic disorders, and blood pressure control if hypertension is a complication.
If the tumor is only limited to the adrenal lesion and has not yet invaded the surrounding tissues, only the tumor can be removed and the kidney can be preserved. If complete resection of the tumor is not possible, as much of the tumor as possible should be removed within a safe range and lymph node biopsies should be taken for staging. If the tumor cannot be removed but only biopsy can be performed, the tumor boundary can be marked with silver clips, and then surgical exploration can be performed after 2-3 months of radiotherapy and chemotherapy.
2. Radiation therapy can shrink the tumor to the extent that it can be treated by surgery. Because neuroblastoma often spreads to many parts of the body or involves important organs, tumors of important organs should be treated with radiation first and then surgically removed after they shrink. Radiation therapy can also be used for bone pain caused by spinal cord compression and metastasis. However, young children under one year old as well as stage I and II patients are not helped by radiation therapy.
Chemotherapy with chemotherapy cannot improve the survival rate of patients with stage I and II disease, but for stage III lesions, treatment with cyclophosphamide, vincristine and azulfiram can prevent recurrence.
4. When metastatic lesions are found to be metastatic by X-ray examination, the diagnosis should be confirmed by lymph node biopsy and bone marrow aspiration, and then postponed for surgical exploration after radiotherapy and chemotherapy.
Children with stage IV-S generally do not need treatment or rarely treated, most of them are less than one year old and have a high rate of self-elimination. Surgical exploration is performed only when the primary tumor is clinically estimated to be surgically resectable. Radiotherapy and chemotherapy are available if observation reveals progressive lesions, and hepatomegaly responds well to radiotherapy.
The prognosis of neuroblastoma is determined by the age and stage of the patient. The younger the age of onset, the better the prognosis. For stage I and II lesions, the two-year survival rate is about 80% if the tumor can be completely removed, and about 65-70% for stage IV-S lesions.