Somatostatin
Classification according to ICD-10 | |
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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
- ↑ a b c d e f g h i j E. J. Hanly u. a .: Somatostatinomas , as of July 12, 2006.
- ↑ 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)
- ^ 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.
- ↑ 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 .
- ↑ LI Larsson et al: Pancreatic somatostatinoma. Clinical features and physiological implications. In: The Lancet . 8013/1977, pp. 666-668. PMID 66472
- ↑ GJ Krejs include: syndrome Somatostatinoma. Biochemical, morphologic and clinical features. In: N Engl J Med. 301/1979, pp. 285-292. PMID 377080
literature
- U. Plöckinger, B. Wiedenmann: Neuroendocrine tumors of the gastrointestinal tract. In: Z Gastroenterol. 42/2004, pp. 517-526.
- W. Varikatt et al .: Somatostatinoma: collision with neurofibroma and ultrastructural features. In: Histology and histopathology. 21/2006, pp. 1171-1180. PMID 16874660
- G. Guercioni et al .: Incidental small ampullary somatostatinoma treated with ampullectomy 2 years after diagnosis. In: Digestive diseases and sciences. 51/2006, pp. 1767-1772. PMID 16967313
- KU Juergens et al .: Duodenal somatostatinoma and gastrointestinal stromal tumor associated with neurofibromatosis type 1: diagnosis with PET / CT. In: American journal of roentgenology. 187/2006, pp. 233-234. PMID 16861519
- G. Galli et al .: Characterization of the functional and growth properties of long-term cell cultures established from a human somatostatinoma. In: Endocrine-Related Cancer 13/2006, pp. 79-93. PMID 16601281
Web links
- SOMATOSTATINOM GEP Tumor Center University of Düsseldorf
- National Library of Medicine - Medical Subject Headings (Eng.)
- Diseases Database (Engl.)
- Neuroendocrine Tumors (NET)