Neuroendocrine Tumors: Examinations, Diagnosis, and Therapies

What are neuroendocrine tumors? The news of rapper Fedez’s surgery due to a neuroendocrine tumor has spread across the web, raising public awareness. At Nefrocenter, it is possible to perform all diagnostic tests for the prevention of this pathology.

Neuroendocrine tumors fall into a category of rare diseases that affect the neuroendocrine system. They are defined as slow-growing in most cases and share some common characteristics that allow for similar methods of diagnosis and therapy. They can affect, in most cases, the pancreatic islets, but there are also cases localized in the gastro-enteric-pancreatic tract with faster growth. The latter can originate in all districts and therefore require different therapies and treatments compared to other forms.

Functioning and Non-Functioning Neuroendocrine Tumors

Neuroendocrine tumors that originate from endocrine cells present in the pancreas can be divided into “functioning” and “non-functioning“. Those that produce significant amounts of hormonal substances causing various symptoms are defined as “functioning”. “Non-functioning” ones are characterized by the lack of substance production. There are four main “functioning” tumors.

Insulinoma is characterized by a large production of insulin, a hormone that regulates blood glucose levels; symptoms, related to a lowering of glucose values, may include dizziness, sweating, feeling faint, and tachycardia.

VIPoma causes excessive production of VIP, which is the vasoactive intestinal peptide that regulates the production and absorption of water by the intestine; the characteristic symptom is severe diarrhea.

Glucagonoma is a rarer form, characterized by the production of glucagon, which causes skin rashes and diabetes

Gastrinoma causes production of gastrin, resulting in increased stomach acid production; the main symptoms are diarrhea and ulcers

Somatostatinoma, another rare form of tumor that causes diabetes, gallstones, and difficulty in digesting fatty foods.

Symptoms of Endocrine Tumors

The symptomatology of neuroendocrine tumors is related to the type of hormone produced. However, there are various symptoms associated with these types of pathologies: Cholelithiasis, Diabetes, Diarrhea, Hypoglycemia, Duodenal ulcers. Other symptoms depend on the effects of the tumor mass itself, particularly in cases of “non-functioning” pathologies: Weight loss, Pain, Jaundice, Vomiting.

The Diagnosis

In most cases, neuroendocrine tumors are discovered almost by chance, during examinations for other reasons. In other circumstances, they are detected due to the presence of symptoms, for example through blood tests. These tests can reveal abnormal values of the hormones previously mentioned or the presence of other substances produced by the tumor itself, such as chromogranin A.

Instrumental Examinations

Various instrumental examinations are useful to accurately identify the tumor, its location, and its size. Genetic analysis to verify the presence of Multiple Endocrine Neoplasia type 1 syndrome (MEN 1). Biopsy of the tumor tissue. Endoscopic ultrasound of the stomach and duodenum, using an ultrasound probe that allows accurate visualization of the pancreas, with the possibility of also taking small samples of the tumor through the fine needle aspiration technique. Magnetic resonance imaging (MRI) of the abdomen. Scintigraphy receptor that uses somatostatin receptors to identify neuroendocrine tumors

Computed Tomography (CT) triphasic of the abdomen, which allows verification of the presence of the tumor in the liver or lymph nodes. The technique consists of the passage of the contrast medium through the liver in three phases.

Positron emission tomography with CT fusion (PET-CT), probably the most effective examination for a correct diagnosis and re-evaluation of the tumor after oncological therapy. There are three types of this examination.

PET-CT with Dopamine, which can identify the tumor through its peculiar metabolism. PET-CT with FDG, which uses a radiopharmaceutical that concentrates in neoplastic lesions characterized by a high sugar metabolism; it serves to classify the aggressiveness of tumors. PET-CT with Gallium-DOTA-peptide is characterized by the use of a radiopharmaceutical that binds to somatostatin receptors. These often abound on the surface of these particular neoplasms

Therapies for Neuroendocrine Tumors

The treatment of neuroendocrine tumors may include a combination of different “disciplines”. Biotherapies, used in cases where surgical removal of the tumor is impossible. They consist of monthly injections of a synthetic hormone corresponding to somatostatin in patients.

Chemotherapy, used in cases where the tumor grows despite hormone therapy or in cases of rapidly growing neoplasms.

Surgery, the most effective therapy because it allows for the removal of the tumor. Surgical interventions for this type of tumor, also performed laparoscopically, are less destructive compared to those for other carcinomas

Radiometabolic and radioreceptor therapy is substantially linked to the massive presence of receptors for somatostatin on the surface of neuroendocrine tumors. It therefore involves the use of a drug corresponding to somatostatin, characterized by a radioactive portion.

This drug, injected intravenously, is able to identify the tumor thanks to the receptors, thus allowing the radioactive component to act directly on the tumor cells

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