What Is the Main Idea?

Neuroblastoma is a type of solid tumor that is rare in adults. As a result, the disease course of neuroblastoma in adults is not well studied and there is no guideline-recommended chemotherapy strategy specifically for adults. In the open-access article “Adrenal Neuroblastoma Producing Catecholamines Diagnosed in Adults: Case Report”, published in the journal Case Reports in Oncology, the authors describe the case of a 24-year-old female patient and discuss considerations regarding the care of adults with neuroblastoma.

What Else Can You Learn?

In this blog post, neuroblastoma is discussed, along with issues relating to the treatment of adult patients compared with children and common complications that may develop. Embryogenesis and the role of the sympathetic nervous system are also addressed.

What Does the Case Report Describe?

In this case report, the case of a 24-year-old female patient with neuroblastoma is described. Although classification of her tumor according to an international staging system suggested that it was unlikely to be an aggressive tumor, it recurred 4 months after surgery and she needed further drug treatment.

What Is Neuroblastoma?

Neuroblastoma is a type of solid tumor, which means that it forms an abnormal mass (lump) of tissue that doesn’t usually contain any liquid areas. In childhood, neuroblastoma is the most common type of solid tumor that develops outside of the cranium (the bones that surround the brain) and the third most common childhood cancer worldwide. Neuroblastomas can develop at any location in the sympathetic nervous system but most commonly in the adrenal medulla, the inner regions of the adrenal glands, which are located on top of the kidneys.

What Is the Sympathetic Nervous System?

The sympathetic nervous system is the part of the autonomic nervous system (which controls things that you do without thinking about them, so it can be thought of as the “automatic” nervous system). The sympathetic nervous system controls rapid, involuntary responses of the body to dangerous or stressful situations, or when you are physically active. These include increasing your heart rate, improving oxygen delivery to your lungs, activating energy stores in your liver so that you can use energy quickly, and slowing down your digestion so that energy being used to digest food can go to other areas of the body that need it. Most of the signals that the sympathetic nervous system sends start in the spinal cord (a long, tube-like band of tissue that runs through the center of your spine, connecting your brain to your lower back) and are relayed all over your body. To communicate, the sympathetic nervous system uses chemicals called neurotransmitters. One family of neurotransmitters is the catecholamines, which include dopamine and epinephrine (adrenaline). Catecholamines are made by the brain and adrenal glands. Once they have been used, they are removed from your body via the urine.

How Does Neuroblastoma Develop?

When a human egg is fertilized, an embryo starts to develop through a series of processes that are together called “embryogenesis”. These processes include cell division and growth, and different groups of cells begin to develop that have specific roles in the body (this is called differentiation). In vertebrates, a group of cells called neural crest cells temporarily exists that go on to give rise to cells with very different roles, including nerve cells, melanin(pigment)-producing cells, and smooth muscle. It is thought that neuroblastoma can develop if neural crest cells start to gain mutations and changes during embryogenesis that disrupt their differentiation, although it’s not yet clear how.

Can Neuroblastoma Occur in Adults?

Neuroblastoma is considered by many to occur almost exclusively in children, with more than 90% of patients diagnosed before 10 years of age. However, although neuroblastoma is rare in adolescents and even rarer in adults, cases do occur, and while the clinical course of childhood neuroblastoma tends to be benign (mild), with some patients aged less than 1 year experiencing remission, there is evidence that the course of the disease in adults is more severe. Unfortunately, the fact that adult neuroblastoma is so rare means that it is not well understood.

What Are Specific Considerations for the Treatment of Adults with Neuroblastoma?

Children diagnosed with neuroblastoma are usually treated with intense polychemotherapy (chemotherapy involving several different drugs), which has been reported to be poorly tolerated by adult patients. However, there is currently no specific chemotherapy regimen for adult patients, so polychemotherapy is used with adjustments made on an individual-patient basis, depending on the needs of the patient and their ability to tolerate the drugs. As well as the treatment that adult patients receive, a key consideration is the level of catecholamines in the patient’s urine.

Why Are Catecholamine Levels Important?

As mentioned earlier, catecholamines are neurotransmitters that are used by the sympathetic nervous system and are involved in stress responses. It has been reported that 85–90% of patients with neuroblastoma have increased levels of catecholamines. As well as potentially indicating the presence of a rare tumor in the adrenal glands, high catecholamine levels can cause high blood pressure. As a result, patients with neuroblastoma can be at increased risk of stroke, kidney failure, and cardiovascular complications in the future, like arterial stiffness and thickening of the ventricles in the heart. Care needs to be taken so that high catecholamine levels and any complications are detected and followed up effectively.

What’s the Take-Home Message?

Although neuroblastoma is adults is rare, cases do occur. It is particularly important that the treatment of adults with neuroblastoma is tailored to the individual and any cardiovascular complications are detected and followed up properly. If a person has a high catecholamine level in their urine, the cause should be investigated because it may indicate the presence of a rare adrenal tumor.

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