somatostatinoma

Introduction

Introduction to growth hormone releasing hormone tumor Somatostatinoma is a tumor derived from islet D cells. It is called growth, because it releases a large amount of somatostatin (SS), which causes fat sputum, diabetes, hypoacidity and cholelithiasis. Inhibinoma syndrome, a polypeptide consisting of 14 amino acids, was first isolated from the rat hypothalamus in 1968 and was found to inhibit the release of growth hormone, named growth hormone. Somatotropin Release-Inhibiting Factor (SRIF). It has now been found that somatostatin is present in the hypothalamus, pancreatic D cells, stomach, duodenum and small intestine, and can inhibit the release of various peptides widely. Therefore, the hormone not only inhibits endocrine and exocrine, but also inhibits intestinal peristalsis and gallbladder contraction, so it is also called inhibitory hormone. In 1977, Ganda and Larsson first independently described somatostatin tumors in their own reports. In 1979, Krejs comprehensively described the clinical features of this disease. Somatostatin tumor is one of the rarest functional endocrine tumors. So far, the foreign data is not 50 cases. It has not been reported in the literature in China, and the age of onset of this disease is relatively large. 26 to 84 years old, with an average of 51 years old, male and female patients are almost equal. basic knowledge Sickness ratio: 0.0001% Susceptible people: no special people Mode of infection: non-infectious Complications: Diabetes Cholelithiasis

Cause

Growth hormone releasing hormone tumor etiology

Somatostatin is one of the rarest functional endocrine tumors, and its cause is still under investigation.

1. Endocrine system: Somatostatin can significantly inhibit the release of endocrine hormones in the gastrointestinal system, especially the pancreas. These hormones include: insulin, glucagon, gastrin, motilin, secretin, cholecystokinin , pancreatic polypeptide and vasoactive intestinal peptide. Whether it is normal tissue or tumor tissue, somatostatin can inhibit the release of the above-mentioned peptide hormones through specific or receptor action.

2, the central nervous system: inhibition of the pituitary release hormones, such as growth hormone, thyrotropin, adrenocorticotropic hormone and prolactin.

3, gastrointestinal tract: somatostatin can directly inhibit gastric acid secretion, gastric emptying, duodenal movement, biliary and gallbladder movement, pancreatic exocrine function and absorption of glucose, amino acids and triglycerides. Somatostatin has a paracrine effect on the gastrointestinal tract, and negatively regulates the digestion and metabolism processes that are finely regulated by peptides.

Prevention

Growth hormone releasing hormone tumor prevention

There are no special preventive measures, mainly to early diagnosis and early treatment, pay attention to the health diet.

Complication

Growth hormone releasing hormone tumor complications Complications, diabetes, cholelithiasis

Diabetes, cholelithiasis, abdominal pain, hypoacidity and other symptoms.

Symptom

Growth Hormone Repressor Tumor Symptoms Common Symptoms Diabetes Abdominal Pain Indigestion Fatty Diarrhea Diarrhea

Due to the complex diversity of the clinical manifestations of the disease, the diagnosis is very difficult, especially difficult to achieve early diagnosis, if the patient has diabetes, cholelithiasis, dysentery and triad manifestations, as well as indigestion, hypoacidity, weight loss Symptoms such as abdominal pain or abdominal mass, should be thought of the possibility of suffering from somatostatin, combined with laboratory tests, gastrointestinal barium meal, duodenal angiography, B-ultrasound, CT, MRI, selective abdominal cavity A localization examination such as arteriography is used to determine the location of the tumor.

Clinical manifestation

Diabetes

2. Too little stomach acid

3. Cholelithiasis

4. Abdominal pain

5. Diarrhea

In short, the clinical manifestations of somatostatin are very complex, showing diversity changes, and these symptoms are common in many other diseases. Some people have diabetes, cholelithiasis and steatorrhea as somatostatin. The "triple syndrome".

Examine

Growth hormone releasing hormone tumor examination

Laboratory examination

(1) Analysis of gastric juice: too little gastric acid or even stomach acid.

(2) Increased blood glucose, or decreased glucose tolerance test.

(3) Determination of basal plasma somatostatin: For the diagnosis of this disease, any patient suspected of having somatostatin tumors should be tested for plasma somatostatin levels, normal human growth in the early morning fasting state Inhibin levels <100pg/ml, patients with somatostatin tumors ranged from 0.16 to 107 ng/ml, with an average of 15.5 ng/ml, but a small number of patients may also have false negative results.

(4) Stimulation test: For patients who are clinically suspected of having somatostatin tumors and whose plasma somatostatin levels are not elevated, the diagnosis can be further confirmed by the challenge test.

1 Toluene sulfonylbutyrate (D860) challenge test: After intravenous injection of toluene sulfonylbutyrate, the presence of tumors can increase the level of plasma somatostatin by stimulating the release of somatostatin, while those without tumors do not. Raise.

2 Calcium-Pentagastrine Test: In patients with this disease, plasma somatostatin levels can be increased by 2 times 3 minutes after intravenous calcium (calcium gluconate) and pentagastrin gastrin. After a minute, it gradually returned to normal; regardless of pancreatic or pancreatic somatostatin patients, with liver metastasis, plasma somatostatin levels were also significantly increased, this test can not make plasma somatostatin concentration in normal or pancreatic adenocarcinoma patients Increase.

2. Positioning diagnosis

(1) Gastrointestinal barium meal or duodenal hypotension examination: for the duodenal descending or pancreatic head tumor, the filling defect is visible, the duodenal ring becomes larger, the impression is changed, but the pancreas Body, tail tumor does not help.

(2) B-ultrasound, CT or MRI examination: Because the tumor is usually large, the primary tumor of the pancreas and metastatic tumor of the liver can often be found, and the diagnosis rate is high.

(3) Selective celiac angiography: can show multiple blood supply tumors of the pancreas and liver metastases. The diagnosis of this disease is similar to that of B-ultrasound, CT and MRI. The diagnosis rate is more than 85%, but these tests are only Can determine the presence of a tumor, but can not make a qualitative diagnosis.

Diagnosis

Diagnosis and identification of growth hormone releasing hormone tumor

diagnosis

Diagnosis can be based on medical history, clinical symptoms, and laboratory tests.

Differential diagnosis

1. Diabetes.

2. Cholelithiasis.

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