8156/3 Somatostatin cell tumor, malignant



Definitions

Pancreas
ICD-O-3 topography code: C25
ICD10: C25
           

A somatostatinoma is a functionally active and usually malignant endocrine tumour with evidence of D-cell differentiation and clinical symptoms reflecting the diverse pathophysiologic effects of chronic inappropriate secretion of somatostatin (hypersomatostatinemia; somatostatinoma syndrome)1
 
Ronald A. DeLellis, Ricardo V. Lloyd, Philipp U. Heitz, Charis Eng
World Health Organization Classification of Tumours. Pathology and Genetics of Tumors of Endocrine Organs
IARC Press: Lyon 2004



. Somatostatinomas account for between 1 to 2% of endocrine tumours of the gastroenteropancreaticohepatic (GEPH) axis and generally arise in adults.
Some somatostatinomas are associated with NF1, MEN1 and Von Hippel-Lindau syndromes; the etiology of their sporadic counterparts is unclear.
Because somatostatinomas are generally large by the time they are detected, nearly two-thirds have already metastasized to the regional lymph nodes or the liver
2
Click to access Pubmed
Axelrod L, Bush MA, Hirsch HJ, Loo SW (1981)
Malignant somatostatinoma: clinical features and metabolic studies.
J Clin Endocrinol Metab 52: 886-96



3
Click to access Pubmed
Shirouzu K, Miyamoto Y, Shiramizu T, Morimatsu M (1985)
Somatostatinoma of the pancreas.
Acta Pathol Jpn 35: 1285-92



.





Region of the ampulla of Vater
ICD-O-3 topography code: C24.1
ICD10: C24.1
           

A somatostatinoma is a functionally active and usually malignant endocrine tumour with evidence of D-cell differentiation and clinical symptoms reflecting the diverse pathophysiologic effects of chronic inappropriate secretion of somatostatin (hypersomatostatinemia; somatostatinoma syndrome)
4
 
Ronald A. DeLellis, Ricardo V. Lloyd, Philipp U. Heitz, Charis Eng
World Health Organization Classification of Tumours. Pathology and Genetics of Tumors of Endocrine Organs
IARC Press: Lyon 2004



.

Somatostatinomas account for between 1 to 2% of endocrine tumours of the gastroenteropancreaticohepatic (GEPH) axis and generally arise in adults. While some somatostatinomas are associated with neurofibromatosis type 1 (NF1) OMIM , multiple endocrine neoplasia (MEN1) OMIM and Von Hippel-Lindau syndromes OMIM , the etiology of their sporadic counterparts is unclear.





Small intestine
ICD-O-3 topography code: C17
ICD10: C17
           

A somatostatinoma is a functionally active and usually malignant endocrine tumour with evidence of D-cell differentiation and clinical symptoms reflecting the diverse pathophysiologic effects of chronic inappropriate secretion of somatostatin (hypersomatostatinemia; somatostatinoma syndrome)
5
 
Ronald A. DeLellis, Ricardo V. Lloyd, Philipp U. Heitz, Charis Eng
World Health Organization Classification of Tumours. Pathology and Genetics of Tumors of Endocrine Organs
IARC Press: Lyon 2004



. Somatostatinomas account for between 1 to 2% of endocrine tumours of the gastroenteropancreaticohepatic (GEPH) axis and generally arise in adults. Some somatostatinomas are associated with NF1, MEN1 and Von Hippel-Lindau syndromes; the etiology of their sporadic counterparts is unclear. Because somatostatinomas are generally large by the time they are detected, nearly two-thirds have already metastasized to the regional lymph nodes or the liver 6
Click to access Pubmed
Axelrod L, Bush MA, Hirsch HJ, Loo SW (1981)
Malignant somatostatinoma: clinical features and metabolic studies.
J Clin Endocrinol Metab 52: 886-96



7
Click to access Pubmed
Shirouzu K, Miyamoto Y, Shiramizu T, Morimatsu M (1985)
Somatostatinoma of the pancreas.
Acta Pathol Jpn 35: 1285-92



.