Facial flushing and frequent diarrhea? Beware of neuroendocrine tumors

  Continued frequent facial flushing, frequent diarrhea and abdominal pain, recurrent hypoglycemia and even asthma …… These seemingly unrelated symptoms may in fact point to the same disease: it has taken away the admiration of millions of “fruit fans” Apple’s founder Steve Jobs, and is also destroying It has taken away Apple’s founder Steve Jobs, who was admired by many “fruit fans”, and is also destroying patients of both sexes, from teenagers to the elderly. Although it occurs in the gastrointestinal and pancreatic system, it has a strange name “neuroendocrine tumor”. Recently, the first Southern Regional Pancreatic Neuroendocrine Tumor Expert Seminar (hereinafter referred to as “Southern Meeting”) was held at the First Hospital of Sun Yat-sen University, and the Southern Neuroendocrine Tumor Diagnosis and Treatment Center was inaugurated at the same time. The reporter learned that, as one of the largest neuroendocrine tumor treatment centers in China, Zhongshan First Hospital receives a variety of patients from different sources every year. There are patients who go to the endocrinology department for treatment of symptoms such as recurrent hypoglycemia, and those who go to the gastroenterology department for treatment of recurrent skin flushing and diarrhea. Experts pointed out that the symptoms of this disease are complex and changeable, so the treatment cannot rely on the specialist alone, but should take the road of “multidisciplinary treatment”.  ”Neuroendocrine tumors are a group of tumors with high heterogeneity, not a single tumor as we usually know, but a general term for a large group of tumors.” Professor Chen F. Lake, vice president of the First Hospital of Sun Yat-sen University and chief expert of gastroenterology, pointed out that neuroendocrine tumors can occur in many organs and tissues throughout the body, with gastroenteropancreatic neuroendocrine tumors being the most common, accounting for about two-thirds of all such tumors.  When it comes to the clinical manifestations of this disease, “complex and diverse” becomes the most apt description. Neuroendocrine tumors can be divided into non-hormone-secreting non-functional neuroendocrine tumors and hormone-secreting functional neuroendocrine tumors, and their clinical manifestations can be described as “thousand and one”. Take functional neuroendocrine tumors as an example, patients may have different symptoms depending on the hormones secreted by the tumors. For example, insulinoma may secrete excessive insulin, causing patients to have recurrent hypoglycemia; gastrinoma may secrete gastrin, causing excessive secretion of gastric acid and peptic ulcers. Carcinoid tumors may secrete vasoactive substance 5-hydroxytryptamine, causing recurrent skin flushing on the head, face and trunk, abdominal pain and diarrhea, and even asthma. There are also a large proportion of neuroendocrine tumors that have no clinical symptoms at all and are only discovered by chance during CT, ultrasound and gastroscopy.  Slow growth rate is not equal to benign tumor. Many diseases have population preferences, for example, some diseases favor men, some prefer women, some are more prevalent in the elderly, and some are attracted to teenage boys. However, all these rules fail to apply to neuroendocrine tumors.  ”There is no gender difference in the onset of neuroendocrine tumors, and there is a wide range of patient ages, from elderly people in their seventies to teenagers.” Dr. Chen Jie, deputy director of the Department of Gastroenterology at Zhongshan First Hospital, pointed out that about 10 percent of all neuroendocrine tumor patients have a genetic background and carry defective genes that cause the disease, while the vast majority of patients have nothing to do with genetics.  Patients often think that slow-growing tumors are not malignant tumors. “Whether a neuroendocrine tumor is malignant and has a good or bad prognosis has nothing to do with how fast the tumor grows or whether it secretes hormones.” Chen Jie said that neuroendocrine tumors that do not grow fast can also metastasize everywhere. An important index to evaluate the malignancy of such tumors is Ki-67 index, which reflects the speed of tumor growth and proliferation. Based on the range of this index, gastroenteropancreatic neuroendocrine tumors are classified into three grades. The vast majority of grade 1 and 2 neuroendocrine tumors have a survival period of more than five years, while the average survival time for patients with grade 3 neuroendocrine cancer is only ten months.  ”Among all neuroendocrine tumors, the most famous one is pancreatic neuroendocrine tumor. The disease was made famous by the death of Steve Jobs, the founder of Apple Inc. and is commonly known as “Steve Jobs Cancer”. Due to the same location of growth and malignant tumor, pancreatic neuroendocrine tumor is often mistaken as “life-threatening demon” pancreatic cancer, but in fact they are two completely different tumors originating from different cells.  Pancreatic neuroendocrine tumors originate from the neuroendocrine cells of the pancreas and have specific histological neuroendocrine markers, and some of them have the function of secreting related hormones. In general, pancreatic neuroendocrine tumors are much better treated than pancreatic cancer. However, once it develops distant metastases, the median survival of patients is only about three years. Steve Jobs passed away after an eight-year journey against tumor. Chen Jie emphasized that early and accurate diagnosis and timely and appropriate treatment are very important for patients with neuroendocrine tumors. There are various treatment methods for neuroendocrine tumors. Patients with no metastasis and only local metastasis in early stage can be completely eradicated through surgery and achieve clinical cure.  Chen F. Lake pointed out that for advanced neuroendocrine tumors that cannot be radically resected by surgery, there are also options such as radiointerventional therapy, nuclear therapy, chemotherapy, biological therapy and molecular targeted therapy to improve the condition and prolong the survival time of patients.  High rate of misdiagnosis and underdiagnosis Low rate of early diagnosis “The three most common problems of neuroendocrine tumor patients are under-treatment, delayed treatment and over-treatment.” Chen F. Lake pointed out that because of the great differences in the site of occurrence, clinical manifestations, pathological features, malignancy and treatment of neuroendocrine tumors, the clinical understanding of neuroendocrine tumors is still inadequate, so misdiagnosis and underdiagnosis are easy to occur. Take abdominal pain and diarrhea as an example, which happen to be symptoms common to many GI diseases, and thus are very easy to be misdiagnosed as general GI diseases. Due to the low rate of early diagnosis, more than half of neuroendocrine tumors are already in the middle to late stages when diagnosed.  ”We receive patients from a wide variety of sources each year. There are those who go to endocrinology for symptoms such as recurrent hypoglycemia, and those who go to gastroenterology for skin flushing and recurrent diarrhea.” Chen Jie pointed out that, in fact, each department alone cannot fix gastroenteropancreatic neuroendocrine tumors. As it involves multiple tissues and organs, there are significant individual differences in clinical manifestations, and its diagnosis and treatment need to be based on individual patient’s situation and adopt multidisciplinary treatment (MDT) mode to reduce misdiagnosis and mismanagement of such diseases.  For patients, the first “South Meeting” delivered good news, as this “South Meeting” facilitated the exchange of experience among doctors from several hospitals in South China and Southwest China, which helped strengthen multidisciplinary and multi-hospital collaboration and promote clinical understanding and standardized treatment of neuroendocrine tumors. It will help strengthen the multidisciplinary and multi-hospital collaboration and promote the clinical understanding and standardized treatment of neuroendocrine tumors.  ”For neuroendocrine tumors, which are rare in clinical practice, the establishment of patient-centered multidisciplinary centers in large medical institutions is a better treatment model at present.” Chen Fhu pointed out that, taking the hospital as an example, the multidisciplinary diagnosis and treatment center for neuroendocrine tumors collects experts from specialized outpatient clinics, diagnostic imaging, gastrointestinal surgery, gastroenterology, hepatobiliary and pancreatic surgery, endocrinology, pathology, tumor intervention, nuclear medicine and other related departments, so that patients can enjoy one-stop standardized and high-quality medical services and thus obtain good diagnosis and treatment results.  Chen F. Lake reminded that the following symptoms may be the signal of neuroendocrine tumor and should be beware of: 1. Skin flushing: sudden or persistent skin flushing on head, face and trunk, which may be triggered by alcohol, strenuous activities, mental stress or eating chocolate or banana; 2. It is easy to occur after exercise, and some patients will have blurred vision and abnormal mental performance.  4. Gastroduodenal ulcers with recurrent attacks and poor results of conventional treatment, patients are mostly accompanied by diarrhea.