Somatostatin

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Classification according to ICD-10
C25.4 Malignant neoplasm: endocrine glandular portion of the pancreas
ICD-10 online (WHO version 2019)

A somatostatin is a malignant tumor in the endocrine pancreas or duodenum that produces the hormone somatostatin . The somatostatinoma belongs to the pancreatic tumors with an endocrine structure (islet cell tumors), which in turn are classified as gastro-entero-pancreatic neuroendocrine tumors .

description

As a functionally active tumor, a somatostatinoma releases the hormone somatostatin in an uncontrolled manner. This release causes an inhibition of the production of other endogenous hormones such as insulin , glucagon , growth hormone , gastrin , secretin , cholecystokinin (CCK) and vasoactive intestinal peptide (VIP). 95% of the affected patients therefore suffer from diabetes mellitus . Diabetes can range from a mild form of glucose intolerance to severe ketoacidosis .

The somatostatinoma affects the pancreas and the duodenum about equally often. In most cases, metastases have already formed when the tumor is first diagnosed .

Prevalence and incidence

A somatostatin is a very rare cancer. The prevalence is well below 1: 100,000. In the US , the incidence is 1:40 million.

In 93% of the cases described, the somatostatinomas occurred sporadically as single events. In 7% of cases there is an association with multiple endocrine neoplasia type 1 (MEN 1).

Men and women are equally affected. The disease usually occurs in the fourth to sixth decade of life.

diagnosis

Patients with a somatostatinoma have significantly increased somatostatin levels in their blood. The normal value in healthy people is below 100  pg / ml, while it is in the range above 1 ng / ml in patients with a somatostatinoma  . Since the disease is extremely rare, the corresponding evidence can only be provided in a few clinics. With imaging techniques such as computed tomography (CT), magnetic resonance imaging (MRI) and endoscopic ultrasonography (ultrasound examination) a Somatostatinoma can be easily diagnosed. Somatostatin receptor scintigraphy has proven particularly useful for localizing metastases .

therapy

Surgical removal of the tumor is the method of choice and offers the chance of complete cure. To chemotherapeutic agents have 5-FU and streptozocin proven. In addition, there is usually adjuvant diabetes therapy.

forecast

The prognosis for a somatostatinoma is relatively favorable. The five-year survival rate for patients with metastases is 30 to 60%. In patients without metastases, it is almost 100%.

Initial description

In 1977 Ganda and Larsson independently described the first two cases of a somatostatinoma. Ganda's patient was 46 years old and had had diabetes mellitus for eight years . After the tumor was surgically removed, her blood sugar level (euglycaemia) returned to normal.

Two years later, Krejs described the somatostatinoma comprehensively in morphology and biochemistry.

Individual evidence

  1. a b c d e f g h i j E. J. Hanly u. a .: Somatostatinomas , as of July 12, 2006.
  2. O. Schillaci et al: Somatostatin receptor scintigraphy of malignant somatostatinoma with indium-111-pentetreotide. In: J Nucl Med. 38/1997, pp. 886-887. (medscape.com)
  3. ^ F. Gibril et al .: Somatostatin receptor scintigraphy: its sensitivity compared with that of other imaging methods in detecting primary and metastatic gastrinomas. A prospective study. In: Ann Intern Med 125/1996, pp. 26-34.
  4. OP Ganda et al. a: "Somatostatinoma": a somatostatin-containing tumor of the endocrine pancreas. In: N Engl J Med . 296/1977, pp. 963-967. PMID 321960 .
  5. LI Larsson et al: Pancreatic somatostatinoma. Clinical features and physiological implications. In: The Lancet . 8013/1977, pp. 666-668. PMID 66472
  6. GJ Krejs include: syndrome Somatostatinoma. Biochemical, morphologic and clinical features. In: N Engl J Med. 301/1979, pp. 285-292. PMID 377080

literature

Web links