Neuroendocrine tumors are rare and almost always “silent”: only in two cases out of 10 do they give specific symptoms. Massimo Falconi, from the San Raffaele Pancreas Center in Milan, explains the symptoms and how they can be treated
They are rare and almost always “silent”, because only in two cases out of 10 do they give specific symptoms. In Italy, approximately 3,000 new cases of these neoplasms are estimated every 12 months, classified as rare because they affect less than six people for every 100,000 inhabitants.
I neuroendocrine tumors (also known as NETs, from the English Neuro-Endocrine Tumors) – one of which, to the pancreas, hit Fedez, as he himself told on Instagram – are a group of very different neoplasms, some aggressive, others “indolent” (the medical term used to describe slowly evolving diseases).
What are neuroendocrine tumors?
“They originate from the neuroendocrine system, made up of cells that have the typical characteristics of both endocrine cells, which produce hormones, and nerve cells – he explains Massimo Falconi, director of the Pancreas Center of the IRCCS San Raffaele Hospital in Milan, where the rapper was operated, reference center of the European network for the diagnosis and treatment of NETs -. Neuroendocrine cells are present throughout the body and perform specific functions in different organs, such as regulating the flow of air in the lungs or the rate of transit of food in the gastrointestinal tract or the release of digestive juices in the intestine. Therefore NETs can also affect very different organs: in addition to the pancreas, intestines, lungs, thyroid, thymus or adrenal glands. In 60 percent of cases, they develop in the gastro-entero-pancreatic tract, where the neuroendocrine cellular component is most widespread, affecting the entire tract from the esophagus to the rectum, including the pancreas. The second most frequent site is represented by the bronchopulmonary tract (25 percent of cases) “.
They are dangerous?
«It depends on the type of NET in question – replies Falconi, who is also president of Itanet (Italian Association for Neuroendocrine Tumors) -. Based on the appearance of the tumor cells under the microscope, neuroendocrine tumors can be divided into “well differentiated”, which grow rather slowly and are less aggressive than other neoplasms (but they are still malignant and can metastasize even after many years from the discovery of primary tumor) and “poorly differentiated”, which develop faster and are more likely to metastasize ».
What are the symptoms?
«It is very difficult to give a single answer for the dozens of different subtypes of neuroendocrine tumors – says the expert -. They often give vague signals common to many other diseases, or they can even go silent for years. Functioning NETs are characterized by excessive secretion of hormones or other active substances (eg insulin, in the case of insulinomas, with patients presenting with hypoglycemic crises and fainting especially when fasting). There are also NETs that produce gastrin with relapsing ulcers. Of the syndromes, carcinoid syndrome is the most frequent and accounts for more than 40 percent of all syndromes of the functioning forms. It is associated with excessive secretion of serotonin by cancer cells, with diarrhea, flushing of the face and neck, bronchospasm and heart failure. Additional symptoms may be hyperhidrosis, weight loss and the appearance of skin lesions similar to those associated with pellagra. About 80 per cent of cases, however, is made up of non-functioning or non-secreting forms, which therefore do not produce substances or hormones capable of causing specific symptoms ».
What is the prognosis? Can it be healed?
«Also in this case everything depends on the type of tumor present in the single patient – replies Falconi -. And then from the stage of the malignancy to the time of diagnosis: whether it is in the early or advanced stage. Unfortunately, the unclear or absent symptoms mean that 60% of patients with neuroendocrine tumors discover the disease late, only when the tumor mass reaches significant size or compromises the functionality of specific organs.
Non-functioning NETs are often identified at random in the course of investigations conducted for other causes and belatedly. But 5-year survival in our country is high, more than 60%. Also because, in recent years, the weapons available have made it possible to make significant steps forward ».
How are they treated?
“We are facing very different pathologies, which require a personalized approach and integrated management by various specialists – underlines the San Raffale expert, certified since 2019 as a center of excellence by the European Neuroendocrine Tumor Society -. If the pathology is localized, surgery can lead to healing in high percentages of patients. Metastatic disease can benefit from surgery only in rare cases. In this phase, drug therapies come into play. From chemotherapy, which is however only effective in some forms, to somatostatin analogues, to “target” drugs, to locoregional strategies such as hepatic embolization or thermal ablation. Radioreceptor therapy has also recently been approved in Italy, which, through the administration of a radiopharmaceutical, is capable of delivering destructive energy specifically targeted on tumor cells “.
Due to the rarity and complexity of NETs, an alliance between the various competent professionals is needed
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«It is necessary to concentrate resources and knowledge in reference centers and share the experiences acquired as much as possible – adds Falconi -. One of Itanet’s objectives is to indicate both clinicians and patients a clear and defined path of care, promoting the dissemination of new knowledge. Furthermore, national and international research projects must be promoted, to identify increasingly evidence-based therapies ».
March 24, 2022 (change March 25, 2022 | 17:01)
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