Somatostatinoma

Somatostatinoma
a) Large subepithelial gastric mass b) duodenal invasion with bleeding (endoscopy)
SpecialtyOncology

Somatostatinomas are a tumor of the delta cells of the endocrine pancreas that produces somatostatin. Increased levels of somatostatin inhibit pancreatic hormones and gastrointestinal hormones. Thus, somatostatinomas are associated with mild diabetes mellitus (due to inhibition of insulin release), steatorrhoea and gallstones (due to inhibition of cholecystokinin release), and achlorhydria (due to inhibition of gastrin release). Somatostatinomas are commonly found in the head of pancreas. Only ten percent of somatostatinomas are functional tumours [9], and 60-70% of tumours are malignant. Nearly two-thirds of patients with malignant somatostatinomas will present with metastatic disease.

Signs and symptoms

The clinical presentation in individuals (particularly with duodenal tumors) is:[1]

  • Gastrointestinal bleeding
  • Jaundice
  • Abdominal pain

Pathophysiology

Main article: Somatostatin

In a normal subject, actions of somatostatin include:

  • Lowers the rate of gastric emptying, and reduces smooth muscle contractions and blood flow within the intestine
  • Suppresses the release of pancreatic hormones
  • Suppresses the exocrine secretory action of pancreas.

This explains how abnormally elevated somatostatin can cause diabetes mellitus, by inhibiting insulin secretion, steatorrhoea by inhibiting cholecystokinin and secretin, gall stones by inhibiting cholecystokinin which normally induce gallbladder myocytes to contract, and hypochlorhydria caused by inhibiting gastrin, which normally stimulate acid secretion.

Somatostatinomas are associated with calcium deposits called psammoma bodies.[4]

Diagnosis

Fasting plasma somatostatin greater than 30 pg/mL.

SRS (Somatostatin Receptor Scintigraphy) – It is a radio-nucleotide scan by giving Octreotide tagged with Indium111 isotope, which shows an increase in uptake by the tumour cells.[5]

Treatment

Treatment is by chemotherapy with streptozocin, dacarbazine, doxorubicin or by 'watchful waiting' and surgical debulking via Whipple procedure and other resections of the gastrointestinal organs affected.[6]

References

  1. ↑ de Herder, Wouter W.; Zandee, Wouter T.; Hofland, Johannes (2000). "Somatostatinoma". Endotext. MDText.com, Inc. Archived from the original on 29 September 2021. Retrieved 22 December 2021.
  2. ↑ "Insulin". The Lecturio Medical Concept Library. Archived from the original on 1 October 2021. Retrieved 1 October 2021.
  3. ↑ Voet D, Voet JG (2011). Biochemistry (4th ed.). New York: Wiley.
  4. ↑ Johannessen JV, Sobrinho-Simões M (September 1980). "The origin and significance of thyroid psammoma bodies". Lab. Invest. 43 (3): 287–96. PMID 7401638.
  5. ↑ de Herder, Wouter W.; Zandee, Wouter T.; Hofland, Johannes (2000). Feingold, KR; Anawalt, B; Boyce, A (eds.). Somatostatinoma. South Dartmouth (MA): Endotext. Archived from the original on 2021-09-29. Retrieved 2021-10-18.
  6. ↑ Ellison TA, Edil BH (2012). "The current management of pancreatic neuroendocrine tumors". Adv Surg. 46 (46): 283–296. doi:10.1016/j.yasu.2012.04.002. PMID 22873046.
  • 9. Soga J, Yakuwa Y. Somatostatinoma/inhibitory syndrome: a statistical evaluation of 173 reported cases as compared to other pancreatic endocrinomas. J Exp Clin Cancer Res, 1999; 18:13–22. [PMID: 10374671]
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