Identifying rare tumors is a major issue as it might be associated with delay in the diagnosis or unsuitable treatments. Brain tumors and neuroendocrine tumors are some of the examples of rare tumors. People hardly have any knowledge regarding these tumors and their associated causes or symptoms. An understanding regarding these topics can be really helpful in early diagnosis and treatment.
Neuroendocrine tumor is a rare heterogeneous tumor type, comprising ~2% of all malignancies. It is heterogeneous types of tumor in which a patient requires multidisciplinary care along with an oncologist, radiologist, pathologist, surgeon, endocrinologist, gastroenterologist and pulmonologist.
Definitely you might have many questions regarding neuroendocrine tumors, like what it is, how it affects our body and how it can be diagnosed or treated. Let's spare some minutes and explore this tumor in little detail:
- What is the neuroendocrine system?
- What is a neuroendocrine tumor?
- How are neuroendocrine tumors classified?
- Signs and symptoms of neuroendocrine tumors
- What are the causes of neuroendocrine tumors
- How are neuroendocrine tumors diagnosed?
- Oncological management of neuroendocrine tumors
- Healthy lifestyle to prevent cancer
What is the neuroendocrine system?
The Neuroendocrine system is a complex system of hormones released by endocrine glands to the other parts of the body. The two systems, viz. Nervous systems and endocrine systems communicate with each other to maintain the homeostasis of the body. The stimuli from the nerves may influence the release of many hormones and vice versa.
Hypothalamus releases many neuroendocrine cells, the cells which have both endocrine and neuronal characteristics. Contrast to other “traditional”neurons, these cells release neurotransmitters straight into a blood system. Many neurotransmitters release from these cells also referred to as neurohormones due to their endocrine feature. These cells start growing abnormally, that state considered to be neuroendocrine tumors.
What is a neuroendocrine tumor?
Neuroendocrine tumors (NETs) are the cancerous growth of neuroendocrine cells. These are mostly developed in the GI (gastrointestinal) tract, lungs and pancreas. Other types of NETs are prostate, skin, thymus and breast.
How are neuroendocrine tumors classified?
Now the next question is how these tumors are classified. As per their clinicopathologic features, these tumors can be classified according to grade, stage and differentiation.
- Grades: According to World Health Organisation grading system, neuroendocrine tumors can be classified as:
- Grade 1(Low) – These tumor cells divide at a lower pace, thereby growing slowly.
- Grade 2(Intermediate) – These tumor cells divide at intermediate rate.
- Grade 3(High) – On the hand, cells of this grade divide fast and grow speedily.
- Stage: It represents the extent of the tumour spread in the body. Neuroendocrine tumors can be classified in
- Early stage
- Advanced stage (metastatic)
- Differentiation: This presents how similarly the neoplastic cells resemble their nonneoplastic parts.
- Grade 1 and 2 neuroendocrine cells are well-differentiated and
- Grade 3 are poorly differentiated tumor cells.
Signs and symptoms of neuroendocrine tumors
The signs and symptoms of neuroendocrine tumors depend upon the place of the tumor. For instance, a neuroendocrine tumor in the GI tract shows symptoms like stomach pain, diarrhea and constipation. Tumor in the lungs can lead to persistent cough, wheezing, shortness of breath or chest pain.
Other than these, there are functional tumors (tumors that release abnormal levels of hormones directly into the bloodstream) can lead to flushing, low blood sugar, wheezing, diarrhea, fluctuating blood pressure, cramps or certain heart problems.
What are the causes of neuroendocrine tumors?
The exact cause behind neuroendocrine tumors is not clear. However, there are certain risk factors, which can lead to such tumors. These are -
- Multiple endocrine neoplasia type 1 (MEN1) – This condition is associated with the tumors of glands that produce hormones, like pituitary glands, parathyroid gland and islet cells of the pancreas.
- Multiple endocrine neoplasias type 2 (MEN2) - This is a rare condition, which is associated with the medullary thyroid cancer, pheochromocytoma and parathyroid tumors.
- Neurofibromatosis type 1 (NF1) – This condition is known to cause abnormal skin color, tumors of skin and nerves.
- Von Hippel-Lindau syndrome (VHL) – This problem is associated with tumors developing in the multiple organs. Other than this, some medical problems like reduced acid production in the stomach, chronic atrophic gastritis, pernicious anemia, zollinger-ellison syndrome, extreme alcohol drinking, cigarette smoking, diabetes or chronic pancreatitis might also lead to this condition.
How are neuroendocrine tumors diagnosed?
Diagnosis of neuroendocrine tumors needs a multidisciplinary approach. It involves pathological testing, diagnostic imaging, syndrome and non-syndrome specific testing.
Oncological management of neuroendocrine tumors
The treatment options to manage neuroendocrine tumors depends upon many factors like site, grade, differentiation, stage, symptoms, age of patient or associated comorbidities. This problem can be treated with surgery along with proper adjuvant therapy.
Many medications like somatostatin analogues (lanreotide or octreotide) aid in managing the condition. These analogues restrict the production of excessive hormonal growth and lower the severity of symptoms. Some medications can also help to shrink the size of the tumor.
Healthy lifestyle to prevent cancer
Healthy lifestyle and proper nutrition in any way is essential to keep the body fit and fine. It is observed that lower physical activity, accumulation of visceral fat and unhealthy diet patterns are the key factors behind many common cancers. These factors also play a crucial role in the management of patients of neuroendocrine tumors.
A diet full of vegetables, fruits, low meat, high legumes are really beneficial. Avoid having spicy, fatty food or foods containing high levels of amine like, alcohol, fermented drinks, aged cheese, yeast, vinegar and soybean products. Also follow all the restrictions and advice of your physician, if you are undergoing neuroendocrine treatment.
There are some drugs which can adversely interact with others medications, like, sunitinib and everolimus can react with eating things like garlic, ginseng, grapefruit, resveratrol, soy, camomile, green tea extract and cranberry), which might lead to toxicity of the medicine.
Awareness regarding neuroendocrine tumors can lower the delay and physicians will be able to provide a multidisciplinary approach. Follow healthy lifestyle and do go for regular checkup to prevent yourself from such rare hard-to-diagnose cancers.
Along with this, avoiding having tobacco, limiting alcohol consumption, stopping cigarette smoking and being physically active significantly helps in improving quality of life and regaining the lost immunity and health.