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Somatostatin tumor (somatostatinoma) is one of the neuroendocrine neoplasms. Somatostatin tumor is quite a serious disease, as it turns out that at the time of diagnosis, most patients already have metastatic cancer. What are the causes and symptoms of this cancer? How is somatostatinoma treated?

Somatostatin tumor( somatostatinoma ) can lead to a number of different ailments, e.g. diabetes, diarrhea or gallstone disease - responsible for their the occurrence of excessive amounts of somatostatin and the effect of this substance on the action of other gastrointestinal hormones. Within the pancreas, various types of cells secreting different hormones are secreted. For example, the product of the so-called Alpha cells are glucagon, while beta cells produce arguably the most famous hormone of the pancreas, insulin. Another type of hormonally active cell found in the pancreas are delta cells that secrete somatostatin. Various types of neuroendocrine neoplasms can develop from these cells. When a tumor is made by delta cells, it is called a somatostatin tumor (also known as a somatostatinoma). Most of these lesions are malignant, but there are also benign forms of somatostatin tumors. A typical location for these pathological masses is in the pancreas, but they can also be found in other parts of the gastrointestinal tract. This tumor occurs in patients of both sexes with a similar frequency, the disease usually appears in patients between 40 and 60 years of age. Somatostatinoma is a rare disease, according to American statistics, 1 in 40 million people are diagnosed with the disease each year.

Somatostatin tumor: causes

The factors leading to the excessive proliferation of delta cells and the occurrence of somatostatin tumors have not been known so far. However, there are hypotheses as to the role of genetic disorders in the pathogenesis of somatostatinoma. They stem from the fact that this type of cancer is more common in people with certain genetic syndromes, such as type I neurofibromatosis or multiple endocrine neoplasia syndrome type I (MEN 1).

Somatostatin tumor: symptoms

Patients' symptoms of a somatostatin tumorare mainly related to the action of somatostatin on the human body. It is a hormone that inhibits the secretion of other substances from the gastrointestinal tract, such as insulin, gastrin or glucagon. When contracting somatostatinoma, patients may experience:

  • Insulin-related diabetes
  • gallbladder stones
  • diarrhea
  • reduced or even complete inhibition of hydrochloric acid secretion (i.e. conditions referred to as hypochlorhydria and achlorhydria, respectively)
  • changes in the nature of the stools (there may be so-called fatty stools)

Other ailments related to a somatostatin tumor can be quite uncharacteristic and may be related to the location or mass of the neoplastic lesion. These symptoms can be:

  • weight loss
  • anemia
  • gastrointestinal bleeding
  • stomach pains
  • gastrointestinal obstruction
  • jaundice

Somatostatin tumor: diagnosis

The diagnosis of a somatostatin tumor may not be easy - due to the exceptional rarity of this disease, it is rarely considered as a factor causing the symptoms described above in patients. When carrying out various diagnostic tests in a patient, however, it is possible - even unexpectedly - to visualize pathological tissue masses. They may be noticeable both during endoscopic examinations (e.g. during endoscopic ultrasonography), but also in computed tomography of the abdominal cavity or in the imaging of the same area of ​​the body by magnetic resonance imaging. - scintigraphy. Before its performance, patients are given a specific marker - octreotide - which, by binding to the somatostatin receptors of the tumor, enables the imaging of the neoplasm. This examination allows not only to visualize the tumor itself, but also to determine the presence of its possible metastases.

Somatostatin tumor: treatment

In the treatment of somatostatinoma, surgical procedures are primarily used - they give the best chances of curing the patient. Different types of procedures are performed in patients, the choice of the method depends both on the mass of the tumor and its location. Only the neoplastic lesion can be removed, as well as resection of the pathological formation together with the head of the pancreas or even with the entire organ. Sometimes a so-called Whipple's operation, during which the head of the pancreas and part of the stomach are resectedin some patients (due to the location of the somatostatin tumor or some other he alth burden), surgery may not be possible. In such situations, other methods of treating somatostatinoma may be used, such as, for example, chemotherapy (consisting in administering drugs such as streptozocin or fluorouracil to patients). In symptomatic treatment (i.e. relieving patients' ailments), they are used, in turn, e.g. anti-diabetic drugs (both oral agents and insulin) as well as pancreatic enzyme preparations.

Somatostatin tumor: prognosis

Even 70-80 percent patients with somatostatin tumors, at the time of diagnosis, are already metastatic neoplastic, the most common location is the liver. In such a situation, after surgical treatment, 5-year survival is recorded in up to 60% of patients. sick. In turn, the prognosis of those who did not develop somatostatinoma metastases is much better - after undergoing surgery, 5-year survival is observed in up to 100% of patients. sick.

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Category:

Oncology