Somatostatin-producing neuroendocrine tumors of the duodenum and pancreas: incidence, types, biological behavior, association with inherited syndromes, and functional activity.
Somatostatin-producing neuroendocrine tumors (SOM-NETs) of the duodenum and pancreas appear to be heterogeneous. To determine their clinicopathological profiles, respective data were analyzed on a series of 82 duodenal and 541 pancreatic NETs. In addition, the clinical records of 821 patients with duodenal or pancreatic NETs were reviewed for evidence of a somatostatinoma syndrome. Predominant or exclusive expression of somatostatin was found in 21 (26%) duodenal and 21 (4%) pancreatic NETs. They were classified as sporadic (n=31) or neurofibromatosis type 1 (NF1)-associated duodenal NETs (n=3), gangliocytic paragangliomas (GCPGs; n=6), or poorly differentiated neuroendocrine carcinomas (pdNECs; n=2). In addition, five duodenal and four pancreatic SOM-NETs were found in five patients with multiple endocrine neoplasia type 1 (MEN1). Metastases occurred in 13 (43%) patients with sporadic or NF1-associated SOM-NETs, but in none of the duodenal or pancreatic MEN1-associated SOM-NETs or GCPGs. Sporadic advanced (stage IV) SOM-NETs were more commonly detected in the pancreas than in the duodenum. None of the patients (including the 821 patients for whom only the clinical records were reviewed) fulfilled the criteria of a somatostatinoma syndrome. Our data show that somatostatin expression is not only seen in sporadic NETs but may also occur in GCPGs, pdNECs, and hereditary NETs. Surgical treatment is effective in most duodenal and many pancreatic SOM-NETs. MEN1-associated SOM-NETs and GCPGs follow a benign course, while somatostatin-producing pdNECs are aggressive neoplasms. The occurrence of the so-called somatostatinoma syndrome appears to be extremely uncommon.
Garbrecht
Nele
N
Department of Pathology, University of Kiel, Michaelisstr 11, Kiel, Germany. ngarbrecht@path.uni-kiel.de
Anlauf
Martin
M
Schmitt
Anja
A
Henopp
Tobias
T
Sipos
Bence
B
Raffel
Andreas
A
Eisenberger
Claus F
CF
Knoefel
Wolfram T
WT
Pavel
Marianne
M
Fottner
Christian
C
Musholt
Thomas J
TJ
Rinke
Anja
A
Arnold
Rudolf
R
Berndt
Uta
U
Plöckinger
Ursula
U
Wiedenmann
Bertram
B
Moch
Holger
H
Heitz
Philipp U
PU
Komminoth
Paul
P
Perren
Aurel
A
Klöppel
Günter
G
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D004379:Duodenal Neoplasms / Q000378:metabolism / Q000473:pathology*; D005260:Female; D005500:Follow-Up Studies; D020022:Genetic Predisposition to Disease; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D018761:Multiple Endocrine Neoplasia Type 1 / Q000378:metabolism / Q000473:pathology; D018358:Neuroendocrine Tumors / Q000235:genetics / Q000378:metabolism* / Q000473:pathology*; D010190:Pancreatic Neoplasms / Q000378:metabolism / Q000473:pathology*; D010235:Paraganglioma / Q000378:metabolism / Q000473:pathology; D011379:Prognosis; D013004:Somatostatin / Q000378:metabolism*; D013005:Somatostatinoma / Q000378:metabolism / Q000473:pathology; D013577:Syndrome
D013004:Somatostatin