“The death of Steve Jobs, the father of Apple, has brought a rare disease called pancreatic neuroendocrine tumor into the public eye. Many media on the Internet blamed Steve Jobs’ death on pancreatic cancer, which misled many people. In fact, Jobs suffered from pancreatic neuroendocrine tumor, not the so-called pancreatic cancer. The presence of neuroendocrine tumors is not high, but the incidence of the disease has been on a significant rise in recent years, with a five-fold increase in incidence in 30 years. It is reported that the incidence of this disease is second only to colon and rectal cancer in western countries, and we must also pay attention to it. Below we will take you to understand pancreatic neuroendocrine tumors.
What is neuroendocrine tumor
Neuroendocrine tumors originate from neuroendocrine cells, and these cells can secrete neurohormones and regulate the functions of other organs. Since neuroendocrine cells are distributed all over the body, the disease can also occur in all parts of the body, but most commonly in the digestive system such as the stomach, intestines and pancreas. Neuroendocrine tumors are both benign tumors that exhibit slow growth and tumors that exhibit low malignancy, as well as tumors that exhibit high metastasis and high malignancy, and it is a collective term for a large group of tumors. According to the differentiation status, they can be divided into well-differentiated neuroendocrine tumors, poorly differentiated neuroendocrine carcinomas, and mixed neuroendocrine-non-neuroendocrine tumors. It is important to note that neuroendocrine tumors are not cancer and can also develop metastasis.
Neuroendocrine cellsNeuroendocrine cells
Difference between pancreatic neuroendocrine tumors and pancreatic cancer
Neuroendocrine tumors of the pancreas and pancreatic cancer are easily confused, but the two are very different in many aspects such as pathogenesis, biological characteristics and prognosis. These tumors are more malignant, insensitive to radiotherapy and chemotherapy, have poor prognosis and are prone to recurrence. Another type of tumor originates from the neuroendocrine cells of the pancreas and is called pancreatic neuroendocrine tumor, which is a relatively less malignant tumor with a better prognosis and longer survival time for patients. Jobs had liver metastases at the time of diagnosis. In the case of pancreatic cancer with liver metastases, the survival period is only 8 months, while Jobs’ survival period is up to 8 years.
Etiology of pancreatic neuroendocrine tumors
The etiology is not yet clear and may be related to genetic mutations. Certain genetic syndromes increase the probability of disease, including
Multiple endocrine adenomatosis type I
VHL syndrome
Neurofibromatosis type I
tuberous sclerosis
Clinical manifestations of pancreatic neuroendocrine tumors
Functional tumors secrete a large amount of hormones and produce corresponding symptoms; non-functional tumors do not or cannot produce sufficient amount of hormones to cause obvious symptoms, so they are difficult to detect in early stages and are often diagnosed only after they have spread.
Depending on the type of hormone, functional pancreatic neuroendocrine tumors include
Insulinomas: large amounts of insulin are secreted, which can lead to intractable hypoglycemia; these are most common.
Gastrinoma: the second most common disease, with symptoms often manifesting as gastric ulcers, epigastric pain, and diarrhea.
glucagonomas: diverse clinical manifestations, including diabetes mellitus, wandering erythema, and cachexia
vasoactive intestinal peptide tumors: relatively rare, with characteristic clinical manifestations of watery diarrhea, hypokalemia, dehydration, and gastric acid deficiency
growth-inhibiting hormone tumors: may present with diabetes mellitus, gallstones, and diarrhea
adrenocorticotropinoma: symptoms are Cushing’s syndrome, mainly manifested by acne, full-moon face, secondary diabetes, centripetal obesity, hypertension, and osteoporosis
Screening of pancreatic neuroendocrine tumors
Diagnosis is often made through the patient’s clinical symptoms, blood tests for tumor markers (chromogranin A), CT, MRI, endoscopic ultrasound and other imaging means combined with pathological examination. Functional neuroendocrine tumors often show obvious symptoms and therefore are not difficult to diagnose, while non-functional neuroendocrine tumors have no typical clinical symptoms and are more difficult to diagnose.
Many people find a lump on the pancreas during physical examination and are terrified that they are suffering from pancreatic cancer, but in fact they are not. In addition to pancreatic cancer, there are also pancreatic cancer neuroendocrine tumors on the pancreas, which may also be benign tumors. The exact type of tumor needs a puncture biopsy or surgical removal for pathological examination to be clear.
If you are unfortunately suffering from pancreatic neuroendocrine tumor, you should keep an upward attitude and actively cooperate with your doctor for treatment. Pancreatic neuroendocrine tumors are far less scary than pancreatic cancer. The survival rate of patients with radical surgery is >50% in 20 years, and patients can even live with tumors for a long time. The disease is not scary, please believe in medicine.
