While this tumor is still being studied, a few reports have discussed its common symptoms, the best treatment approaches, and survival rate. This guide comprises everything you need to know about Neuroendocrine tumors in the liver.
What Is A Neuroendocrine Tumor In The Liver?
In general, neuroendocrine tumors are uncommon and rare tumors originating in specialized cells in the neuroendocrine system. These cells are distributed throughout your body and link to the endocrine system, which is responsible for managing your nervous system and hormones.
Since neuroendocrine tumors are rare, diagnosing this condition and determining the right treatment is extremely challenging. Now, regarding metastatic spread to the liver, there are surely differences if we compare it with gastrointestinal and pancreatic adenocarcinomas. Also, neuroendocrine liver metastases are typically multiple and occur in different sizes. Furthermore, these tumors mostly affect both your liver lobes, i.e., left, right, quadrate, and caudate. However, the case of miliary seeding throughout the liver is extremely uncommon and seen only occasionally.
The primary cause of liver metastases is a common neuroendocrine tumor called carcinoid. This is especially the case when the tumor is of midgut origin. Neuroendocrine tumor (NET) comes in several types as well as subtypes. So, it’s common to mistake their systems with some less severe conditions. Early diagnosis and treatment are key to surviving this cancer for prolonged years.
How Common Is A Neuroendocrine Tumor In The Liver, And Who Can Develop It?
Primary hepatic neuroendocrine tumors (PHNETs) or NET in the liver are an extremely uncommon subtype of neuroendocrine tumors (NETs). This condition usually arises from cells of your neuroendocrine system. Because of the rarity of this condition, there isn’t sufficient data to back up how common this tumor is in the liver. However, we have some evidence regarding who can develop NET in the liver.
Typically, any gender or age group can develop neuroendocrine tumors, including children. However, NETs are extremely uncommon in children, teenagers, and young adults. And even if they occur, there are rare chances they will develop in the liver. The average age of getting diagnosed with neuroendocrine tumors in the liver ranges between 50 and 60. Furthermore, females are more prone to develop this condition compared to males.
While there isn’t enough data regarding how NETs in the liver affect the population, we have some general data regarding the condition as a whole. According to reports, NTS affects about five in 100,000 individuals. Also, around 12,000 individuals are diagnosed with this condition annually in the US. Furthermore, a vast population of 125,000 people lives in the US with these tumors.
What Are The Symptoms Of Neuroendocrine Tumors In the Liver?
As per the original sources from the National Library of Medicine, around one-half of neuroendocrine tumor cases are classified as non-functioning. This means the patients don’t show any hormone-related symptoms. Because they are described as non-functioning tumors, neuroendocrine tumors in the liver cause symptoms similar to exocrine pancreatic neoplasm, i.e., vomiting, nausea, back pain, and/or abdominal pain.
How Are Neuroendocrine Tumors In The Liver Diagnosed?
Now, as mentioned, NETs, in general, are uncommon neoplasms. And, since neuroendocrine tumors in the liver are even rarer, it becomes challenging and complex to diagnose this condition effectively.
Neuroendocrine tumors are known to have slow growth, so you may only realize you have this condition after several years have passed. Also, as providing a proper diagnosis is hard for this condition, it can be several years before you develop the symptoms of neuroendocrine tumors in the liver. While it’s challenging to find the right diagnosis for NETs in the liver, we have some recommended diagnostic methods that may help spot your tumor development in the early stage.
There are multiple tests your healthcare provider may use to diagnose a neuroendocrine tumor. While there are many, not all may be required for everyone. Also, before choosing the most suitable diagnostic test for you, your healthcare provider may consider the following factors:
- Your NET type (in this case, in the liver)
- The current signs and symptoms
- Your age and overall health
- The results from the earlier tests
There are several cases where NETs are spotted when individuals undergo an x-ray or some other medical procedure unrelated to the tumor. If your healthcare provider suspects these tumors, they can perform a thorough physical examination and ask for your complete family and medical history. Other than that, they may use various tests to diagnose a NET, including:
Biopsy
In a biopsy, the healthcare provider will remove a small amount of tissue from a certain part of your body to examine it under a microscope. This is a crucial part of making a diagnosis of NET. After they have your tissue, a pathologist will analyze it under a microscope. However, if your doctor suspects a pheochromocytoma, they may not recommend a biopsy as a diagnostic test unless it’s absolutely necessary. This is because a biopsy of these types of tumors can be fatal.
Endoscopy
In endoscopy, the healthcare provider looks inside your body. This is one of the best diagnostic tests for confirming certain types of NETs. It may even help spot rare conditions such as neuroendocrine tumors in the liver. Your medical practitioner will use an endoscope, a flexible, thin, and lighted tube for this. Also, endoscopy has different types, such as upper endoscopy and colonoscopy. Upper endoscopy helps you examine the stomach and esophagus. Conversely, a colonoscopy helps you examine the colon and rectum.
Your doctor may use a sedative drug on you before performing an endoscopy and inserting a tube into your body. This way, you’ll stay calm, relaxed, and sleepy during the diagnosis. If your doctor finds any abnormality, they can recommend a biopsy.
X-ray
In an x-ray, your doctor will scan your body’s inside using a small amount of radiation. This helps them get a clear picture of your inside structure that may help locate a NET. However, sometimes a tumor may not appear on an x-ray, so your healthcare provider can recommend other scans to diagnose your condition. This can be due to the size of the tumor and its location.
Blood And Urine Tests
Your doctor may ask for your urine and blood sample to determine the abnormal levels of hormones, as well as other substances. They can look for certain proteins or substances that are produced by neuroendocrine tumors in the liver.
CT Or CAT Scan
The machine will take pictures of your body’s inside through X-rays from various angles in a CT scan. After that, the computer combines these images into a detailed, 3D picture showing any tumors or abnormalities the patient may have. Also, doctors use a CT scan to measure the tumor’s size. Furthermore, a CT scan can detect if the tumor has spread to your liver. So, for patients with neuroendocrine tumors in the liver, a CT scan is a great way to diagnose your condition at the early stages.
Biomarker Testing Of The Tumor
Sometimes your healthcare provider may suggest running laboratory tests on your tumor. This test helps identify specific proteins, genes, and other factors that are unique to your tumor. Doctors usually call this test molecular testing of the tumor. Sometimes, these test results can help determine the right treatment for your condition.
MRI
In MRI, the machine will use magnetic fields instead of X-rays to give detailed pictures of your body. Your doctor can use Magnetic resonance imaging (MRI) to measure the size of your tumor.
Ultrasound
In ultrasound, the machine uses sound waves to image your internal organs. Tumors are said to generate unique echoes of waves compared to normal tissue. Therefore, when these waves bounce back to your computer, they create images through which your doctor can learn about the position of the mass inside your body. Ultrasounds are also different and can vary based on the body part being examined. A biopsy can sometimes accompany this procedure.
What Are The Best Neuroendocrine Tumors In Liver Treatments?
Before concluding which treatments will be best if you have NETs in the liver, visiting a cancer care center is suggested. Here, a team of doctors works together in order to create a patient’s treatment plan. As a neuroendocrine tumor is extremely rare, you’ll need a multidisciplinary team’s help to develop the best treatment plan combining various treatments.
A multidisciplinary team comprises multiple healthcare professionals, such as oncology nurses, physician assistants, social workers, nurse practitioners, counselors, pharmacists, dietitians, and others.
At University Cancer Centers, we help our patients by creating the best treatment plans for them. Our recommendations and treatment options depend on various factors, including:
- The origin site of the NET
- Whether the tumor is functional or nonfunctional (NET in the liver is nonfunctional)
- How fast the cells are diving, meaning the tumor grade and degree of differentiation
- Where the tumor is located and its stage
- Tumor growth pace
- Potential side effects
- Patient’s general health and preferences
As mentioned, there are multiple treatment options for a neuroendocrine tumor in the liver, including:
- Surgery
- Targeted therapy
- Somatostatin analogs
- Immunotherapy
- Radiation therapy
- Chemotherapy
- PRRT
- Liver-directed therapy
Of all these treatments, surgical resection of neuroendocrine tumors in the liver is the most effective treatment in providing the best opportunity for long-term survival. However, depending on your condition, your doctor may not recommend surgery. So, let’s learn about each of these neuroendocrine tumor treatments individually to determine your best option.
Surgery
In surgery, the doctor removes your tumor and a few surrounding healthy tissues while operating. Normally, a surgical oncologist performs this operation; its standard approach is removing the entire tumor. In most cases, localized NETs are completely treated through surgery. Furthermore, during the operation, the surgeon may even remove some surrounding tissue, known as a margin. This is to ensure there are no traces of cancer in your body.
However, if complete tumor removal isn’t possible, your healthcare provider may recommend debulking surgery. In this surgery, your doctor will try to remove the maximum amount of tumor from the affected area. This way, you can enjoy some relief from the symptoms of the tumor, but it will not completely cure your tumor.
If your tumor cannot be removed through surgery, that condition is known as an inoperable tumor. However, your doctor will recommend other treatment options in such cases.
Targeted Therapy
In target therapy, the doctor tries to target the tumor’s specific proteins, genes, or the environment of the tissue that is responsible for the survival and growth of the tumor. This treatment primarily focuses on stopping the growth and spread of NET cells and helps limit damage to your healthy cells. However, you should know not every tumor has the same targets. Hence, your doctor may run tests to identify the proteins, genes, and other tumor factors to find the most effective treatment for you.
Somatostatin Analogs
Everyone has a hormone called somatostatin responsible for controlling the release of multiple hormones, like glucagon and insulin. Your healthcare provider may recommend somatostatin analogs, which work the same as the somatostatin hormone. These drugs help control symptoms caused by the substances released by neuroendocrine tumors. Also, these drugs may help slow the tumor growth, but they can’t shrink it.
Immunotherapy
In immunotherapy, the doctor helps boost your immune system, which is your natural defense for fighting allergens and viruses. They work on enhancing the immune system’s ability to fight off cancer cells. Intron A is an immunotherapy type that has shown some results in treating NETs. Also, interferon can help shrink tumors. Well, this type of tumor treatment isn’t that popular now since FDA-approved treatment came into existence.
Radiation Therapy
In radiation therapy, doctors use high-energy X-rays or other particles to eliminate tumor cells. This type of therapy is delivered to patients in specific numbers over a time period. A radiation oncologist is usually responsible for delivering the therapy so no complications arise.
In the case of neuroendocrine tumors in the liver, external-beam radiation therapy is recommended. This involves giving radiation from a machine outside your body.
Chemotherapy
In chemotherapy, your doctor will give you certain drugs that will help eliminate tumor cells from your body. These drugs help prevent the growth and division of these cells. Like radiation therapy, chemotherapy is also delivered to patients in specific cycles over a time period. You can either get one drug or a combination of multiple drugs at the same time.
Your healthcare provider will recommend this therapy if NETs in the liver metastases. Or if the tumor is causing you severe symptoms and targeted or hormonal therapies aren’t working.
Chemotherapy can also be given to a person with a combination of other treatments. Usually, a person can experience fatigue, vomiting, nausea, loss of appetite, diarrhea, and hair loss after this therapy. The side effects may vary depending on the dose amount. Also, not all chemotherapies can lead to hair loss. You may not experience any of these side effects after the treatment gets over.
PRRT
PRRT, or peptide receptor radionuclide therapy, typically uses a radioactive drug that binds to your cell’s somatostatin receptor on certain tumors. Once it binds itself to the receptor, it enters your cell, which leads to radiation damaging the tumor cells.
A few common side effects of PRRT include high enzyme levels in particular organs, low levels of white blood cells, vomiting, fatigue, and nausea. However, there is also a risk of developing blood cancer after the treatment. So, doctor advisory is a must before proceeding with this treatment.
Liver-Directed Therapy
Finally, if you have developed a neuroendocrine tumor in your liver, your doctor may recommend either RFA or hepatic artery embolization. Professional interventional radiologists usually perform such types of procedures. Also, if you get either of these treatments, you may need to stay in the hospital that day.
Common side effects of these procedures include fever, pain around the liver, and higher enzyme levels in the liver.
Radiofrequency Ablation (RFA)
This procedure eliminates the tumor using an electric current to heat it. Your healthcare provider will recommend it for small liver metastasis. It doesn’t show great effects on larger tumors.
Hepatic Artery Embolization
The procedure helps block the blood supply to the tumor by restricting the blood vessels leading to your tumor. It is known as bland embolization when performed by itself. However, if used with other therapies like chemotherapy, it is referred to as chemoembolization. On the other hand, if your healthcare provider combines it with radiation therapy, this procedure will be known as radioembolization.
You must take your time to learn about all the possible treatments for a neuroendocrine tumor in the liver. Don’t hesitate to consult your doctor if you have any doubts or questions regarding the abovementioned treatments. Also, you can talk to your healthcare provider about the goals of each treatment and what you may experience after receiving these treatments.
Since NETs in the liver are rare, determining the right treatment options can be challenging. However, always believe your healthcare provider, as they will suggest the best treatment for your cancer.
What Is The Survival Rate Of Neuroendocrine Tumors In The Liver?
The symptoms of NETs in the liver are usually not that serious at the start. Due to this, this tumor often goes undetected for years before you finally get diagnosed with it. You may confuse it with another disease, spreading the tumor even further.
The National Library Of Medicine discusses this tumor’s surgical management and survival rate. According to the reports, the 5-year survival rate of neuroendocrine tumors in the liver after the curative resection was 75%. On the other hand, the 5-years survival rate of the patients with unresected liver tumors came to about 40%.
There were other studies in which the 5-year survival rate after receiving curative liver resection was 60%. Meanwhile, patients who underwent palliative curative had a 40% survival rate.
While we don’t have sufficient data to develop conclusive treatment methods, the most successful is resection, which has the highest 5-year survival rate. This is especially true for patients with neuroendocrine liver metastases.
Outlook And Living With NETs
People generally confuse NETs in the liver with other less severe conditions. Your outcome and prognosis depend on numerous factors, such as your tumor location, its types, and how much it has spread. If we take an estimate of all NETs types of cases, about 77% of patients survive the 5-year plan after diagnosis.
Receiving the right treatment at the right time can significantly help you have a prolonged life. However, this isn’t the end of your journey since NETs are known for their slow growth. Hence, your doctor will want to track your health status for upcoming years.
Living with NETs alone can be challenging; however, you don’t have to be alone in this cancer-fighting journey. You may experience symptoms like fatigue, tiredness, and diarrhea after treatment. So, you must prioritize rest. Please consult your doctor about joining support groups; they can help you find people who understand what you are going through.
Follow a healthy diet, and talk with your doctor if you have difficulty eating the food because of your symptoms. They can recommend foods that you can tolerate easily. Also, don’t suppress your emotions and hesitate to seek emotional support if you’re experiencing emotional difficulty. Try managing the amount of alcohol you consume, as they have the potential to trigger some NET side effects. Your healthcare provider can help you with that.
Furthermore, you can book an appointment with University Cancer Centers if you have any concerns regarding your condition. We have the right staff to help you with all your cancer-related concerns. Our team can even guide you on the best treatment options and where to receive the best treatment for your condition. Get the right help today!