Not only Irrfan Khan and Steve Jobs fought neuroendocrine cancer I am fighting it too
Neuroendocrine tumors (NET) are tumors arising from neuroendocrine cells in the body and are found in the gastrointestinal tract, and less commonly pancreas, lungs,thymus and thyroid C-cells. There has been a recent increase in the prevalence of the NETs, which is attributed to better diagnostic procedures. NETs were thought to be slow growing benign tumors in the past but almost all NETs are now considered to have malignant potential.
These tumors cause systemic symptoms by releasing vasoactive substances to the bloodstream and are often associated with psychiatric symptoms like depression,anxiety or psychosis. Treating such individuals would require multidisciplinary team approach due to the complexity of the illness. The purpose of this article is to review the various aspects of this illness and challenges of treating the associated psychiatric symptoms.
Neuroendocrine tumors (NETs) form in cells that interact with the nervous system or in glands that produce hormones. These cells, called neuroendocrine cells, can be found throughout the body, but NETs are most often found in the abdomen, especially in the gastrointestinal tract. These tumors may also be found in the lungs, pancreas and adrenal glands. Merkel cell cancer, a type of skin cancer, also is considered a neuroendocrine cancer.
KEY POINTS
The plan for cancer treatment depends on where the NET is found in the pancreas and whether it has spread.
There are three ways that cancer spreads in the body.
Cancer may spread from where it began to other parts of the body.
Treatment of pancreatic NETs is based on the following:
Whether the cancer is found in one place in the pancreas.
Whether the cancer is found in several places in the pancreas.
Whether the cancer has spread to lymph nodes near the pancreas or to other parts of the body such as the liver, lung, peritoneum, or bone.
Cancer may spread from where it began to other parts of the body.
When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood.
Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body.
Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.
The metastatic tumor is the same type of tumor as the primary tumor. For example, if a pancreatic neuroendocrine tumor spreads to the liver, the tumor cells in the liver are actually neuroendocrine tumor cells. The disease is a metastatic pancreatic neuroendocrine tumor, not liver cancer.
Many cancer deaths are caused when cancer moves from the original tumor and spreads to other tissues and organs. This is called metastatic cancer. This animation shows how cancer cells travel from the place in the body where they first formed to other parts of the body.
Symptoms of neuroendocrine carcinoma can include:
Hyperglycemia (too much sugar in the blood)
Hypoglycemia (too little sugar in the blood)
Diarrhea
Persistent pain in a specific area
Loss of appetite/weight loss
Persistent cough or hoarseness
Thickening or lump in any part of the body
Changes in bowel or bladder habits
Unexplained weight gain or loss
Jaundice (yellowing of the skin)
Unusual bleeding or discharge
Persistent fever or night sweats
Headache
Anxiety
Gastric ulcer disease
In metastatic carcinoid tumor,the prevalence of depression is about 50% and anxiety about 35% .
Presence of psychotic symptoms with carcinoid tumors was reported in two studies . Two article reviews reported presence of cognitive symptoms in cases of carcinoid tumor .
The psychiatric symptoms may be due to multiple causes including the release of biogenic amines. Prevalence of psychiatric symptoms in cancer is high in general but studies have reported that symptoms of anxiety and depression are relatively higher in carcinoid tumors.
Serotonin: Serotonin is one of the biogenic amines secreted by the carcinoid tumors that play an important role in producing the symptoms of carcinoid crisis .
• About 95% of body’s serotonin is found in the enterochromaffin cells in the GI tract and the remaining 5%
in the platelet and the brain. The platelets do not produce serotonin, they uptake the serotonin produced by the enterochromaffin cells. In the brain, serotonin is secreted by the serotonergic neurons.
• Serotonin is produced from its precursor tryptophan which is also a precursor of Niacin. In carcinoid syndrome,
excessive production of serotonin causes depletion of tryptophan and decreased production of niacin as a result.
This leads to niacin deficiency symptoms, namely pellagra,
which can also contribute to the psychiatric symptoms.
• In the brain, serotonin production is dependent on the availability of tryptophan. As only L- tryptophan or free tryptophan [unbound to albumin] can enter the brain by crossing the blood brain barrier; it affects serotonin biosynthesis by the neurons. L- tryptophan level is also reduced by substances like cortisol, the level of which is high in cases of depression, which can further reduce brain serotonin production.
• Half-life of serotonin is about 5 days in the platelet, same as the life of platelet, whereas in the brain it is only few minutes and is quickly deactivated by the monoamine oxidase enzymes.
• Peripheral effects of serotonin are local vasoconstriction where it is released and also vasodilatation and increased capillary permeability; constriction of veins and induction of venous thrombosis and promotion of platelet aggregation.
Serotonin has a positive chronotropic effect on the heart through 5HT4 receptors and can cause cardiac rhythm disorders.
• Excessive serotonin gives rise to debilitating diarrhea affecting the quality of life of patients with carcinoid syndrome.
• Prolonged exposure to high level of serotonin can cause fibrosis of heart valves, more commonly on the right side,
causing valvular heart disease. Excessive serotonin is also linked to fibrosis of uterus, skin (scleroderma),
pulmonary and retroperitoneal fibrosis in the long run,causing multiple complications.
Low levels of serotonin are often associated with many behavioral and emotional disorders. Studies have shown that low levels of serotonin can lead to depression, anxiety, suicidal behavior, and obsessive-compulsive disorder. If you are experiencing any of these thoughts or feelings, consult a health care professional immediately. The sooner treatment starts, the faster you’ll see improvements.
Psychological aspect
• Supportive psychotherapy, instillation of hope and providing support.
• Teaching coping skills and grounding techniques using mindfulness-based training.
• Getting connected with social worker and therapist.
Social and community aspect • Discharge planning meeting involving multidisciplinary team.
• Assignment of outreach worker to continue with the supportive work.
• Outpatient mental health follow up appointments.
• Pain clinic referral.
• Continuation with follow up with cancer clinic, cardiologist and other specialists follow ups.
• Follow up with her family doctor, Social worker and dietician.
Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.
Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
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