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Neuroblastoma Cancer - Stages and Types of Neuroblastoma Cancer

Home> Neuroblastoma> Overview and Types

Neuroblastoma, a nerve cancer, primarily affects young children. The tumor originates from the immature nerve cells. It is one of the most common extracranial solid tumors seen in pediatric patients. Oncologists should determine the type of neuroblastoma for treatment optimization and prognosis prediction.

What is Neuroblastoma Cancer?

Neuroblastoma cancer occurs in immature nerve cells that are commonly found in a fetus or embryo. These immature nerve cells are known as neuroblasts and are involved in nervous system development. Neuroblastoma cancer occurs most often in young children and infants (≤5 years of age). This cancer most frequently affects the adrenal glands (glands above the kidneys). The other sites for neuroblastoma development include the abdomen, neck, chest, and spinal cord. The symptoms range from a benign, palpable mass to serious complications due to the spread of the tumor.

How Common is Neuroblastoma?

Neuroblastoma cancer constitutes about 8 - 12% of all childhood cancer cases. The rate of neuroblastoma incidence is about 11 to 13 per million in children <15 years of age. The incidence rate varies according to age. It is 1 per million in children between 10 and 14 years of age and 65 per million in children below the age of 1 year.

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What are the Different Types of Neuroblastoma Cancer?

Some of the common types of neuroblastoma cancer are:

Localized Neuroblastoma

Localized neuroblastoma refers to the type of neuroblastoma that is confined to the origin site and has not spread to other body parts. Localized neuroblastoma is further divided into various risk groups based on several factors, such as tumor location, potential to spread, and age of the patient. These risk groups include low-risk, intermediate-risk, and high-risk. The low-risk neuroblastoma has a small tumor, and the prognosis is favorable, while tumors of intermediate risk have a high risk of recurrence. High-risk neuroblastoma is the most aggressive and has the highest potential to metastasize. Localized neuroblastoma may be completely resectable or unresectable.

Regional Neuroblastoma

Regional neuroblastoma is the stage of neuroblastoma in which the tumor has spread to the nearby lymph nodes and/or surrounding tissues or organs. Still, it has not spread to distant organs. Regional neuroblastoma is generally categorized as stage 3 of the disease. The treatment generally involves a combination of surgery, radiation therapy, and chemotherapy. The customized treatment plan depends upon the tumor's location, the age and overall health of the child, and the characteristics of the tumor.

Metastatic Neuroblastoma

Metastatic neuroblastoma is the neuroblastoma stage characterized by the spread of the tumor to various parts of the body, including distant lymph nodes, skin, bones, bone marrow, and liver. Patients with metastatic neuroblastoma require a very aggressive treatment approach and usually have a relatively unfavorable prognosis.

Histopathological Classification

Histopathological classification for neuroblastoma is important to determine the disease's characteristics and optimize treatment choices. The oncologists histopathologically classify neuroblastoma through several methods. The two most common methods include the Shimada system and the International Neuroblastoma Pathology Classification (INPC). The classification under the Shimada system includes differentiated neuroblastoma and undifferentiated or poorly differentiated neuroblastoma. The INPC provides a more detailed classification and has three major categories. These are neuroblastoma (Schwannian stroma-poor), ganglioneuroblastoma (intermixed), and ganglioneuroma (Schwannian stroma-rich).

Segmental Chromosome Aberration

Neuroblastoma is also classified based on the genetic characteristics of tumor cells. The genetic characteristics also affect the prognosis of the disease. Segmental chromosomal aberrations, such as chromosomal alterations or deletions, also categorize neuroblastoma into various groups. Two of the most clinically relevant chromosomal aberrations include the loss of chromosome 1p and the gain of chromosome 17q. Both are associated with high-risk, aggressive neuroblastoma with a poor prognosis.

What are the Stages of Neuroblastoma?

Following are the different neuroblastoma stages:

Neuroblastoma stage 1

At neuroblastoma cancer stage 1, the tumor is localized and is not spread to other body parts or surrounding lymph nodes. The cancer is completely removed through surgery.

Neuroblastoma stage 2A

At neuroblastoma stage 2A, the neuroblastoma cells are confined to the area of origin and have not invaded the lymph nodes or distant organs. Though surgery removes all the visible tumors, they are not completely resectable.

Neuroblastoma stage 2B

At this stage, the tumors are not completely removed by the surgery. Although the tumor has not spread to distant organs, it has spread to the surrounding lymph nodes on the same side.

Neuroblastoma stage 3

At stage 3 neuroblastoma, the cancer is not completely resectable and has spread to the lymph nodes on the other side of the body. However, cancer does not spread to distant lymph nodes or distant organs.

Neuroblastoma stage 4

At this stage, the tumor spreads to the lymph nodes and organs, including the liver, bone, bone marrow, and skin. However, the patient does not meet the criteria for stage 4s.

Neuroblastoma stage 4s

There are several criteria for neuroblastoma cancer type 4s. These are the age of the child <1 year, cancer is on the single side, may affect the lymph nodes of the same side, may spread to the skin, liver, or bone marrow, has not affected >10% of bone marrow cells, and is not shown on the MIBG scan of the bone marrow.

Recurrent

In recurrent neuroblastoma cancer, the cancer recurs after it has been completely treated. The cancer may recur at the same or different sites.



Conclusion

Neuroblastoma is a cancer that originates from immature nerve cells. It is one of the most common childhood cancers and occurs often in children less than five years old. There are several methods to determine types of neuroblastoma cancer. One classification is based on the spread of the disease. These include localized, regional, and metastatic neuroblastomas. The other classification is based on histopathological characteristics and sequential chromosome aberrations. The stages of neuroblastoma range from stage 1 to stage 4.

Frequently Asked Questions

What part of the body does neuroblastoma affect?

The most common site for neuroblastoma is the adrenal glands. The other sites for neuroblastoma development include the neck, chest, spine, and some areas of the abdomen.

Some types of neuroblastoma cancer are relatively more aggressive than others. This includes neuroblastoma tumor cells with specific chromosomal aberrations like loss of chromosome 1p and gain of chromosome 17q.

The rate of growth of neuroblastoma depends on the type of neuroblastoma. Some forms of neuroblastoma have spontaneous regression and require no treatment. The other forms of neuroblastoma are divided based on the speed of progression. These include high-risk, intermediate-risk, and low-risk.

Although stage 4 neuroblastoma has a poor prognosis, the mortality rate depends upon several factors, such as the age of the patients, overall health, and response to treatment. It has been estimated that almost 50% of the patients with stage 4 neuroblastoma may survive.

The survival rate of patients with neuroblastoma depends upon various factors, such as the stage of the disease, the characteristics of the tumor, the overall health, the age of the patient, and the response to treatment. The estimated 5-year survival rate of patients with intermediate-risk neuroblastoma is about 90 to 95%.