TY - JOUR

T1 - Somatostatin-Immunoreactive Pancreaticoduodenal Neuroendocrine Neoplasms

T2 - Twenty-three cases evaluated according to the WHO 2010 Classification

AU - Engelund Luna, Iben

AU - Monrad, Nina

AU - Binderup, Tina

AU - Boisen Thoegersen, Christina

AU - Hilsted, Linda

AU - Jensen, Claus

AU - Federspiel, Birgitte

AU - Knigge, Ulrich

N1 - © 2015 S. Karger AG, Basel.

PY - 2016

Y1 - 2016

N2 - OBJECTIVE: Neuroendocrine neoplasms in the pancreas and duodenum with predominant or exclusive immunoreactivity for somatostatin (p-dSOMs) are rare, and knowledge on tumour biology, treatment, survival and prognostic factors is limited. This study aimes to describe clinical, pathological, and biochemical features as well as treatment and prognosis.DESIGN: Twenty-three patients with p-dSOM (9 duodenal, 12 pancreatic, 2 unknown primary tumour) were identified from our prospective neuroendocrine tumour (NET) database, and data according to the study aims were recorded.RESULTS: Of the 9 patients with duodenal SOM the m/f ratio was 4/5. All males and one female had NF-1. Seven patients had stage 1A-B and 2 had stage 2B disease. The Ki-67 index was 1-5% (median 2%). Plasma somatostatin was elevated in patients with 2B disease. Of the 14 patients with pancreatic SOM or unknown primary tumour the m/f ratio was 2/12. One male had MEN-1. Five had stage 1A-2B and nine had stage 4. The Ki-67 index was 1-40% (median 7%). Plasma somatostatin was elevated in seven patients. Patients reported symptoms related to the somatostatinoma syndrome, but none fulfilled the criteria for a full syndrome. Primary tumour in the pancreas, metastatic disease at diagnosis and higher tumour grade were all associated with a significantly poorer survival.CONCLUSION: None of the patients with p-dSOM presented with the full somatostatinoma syndrome. Prognostic factors are localisation of the primary tumour, dissemination and tumour grade. A Ki-67 of 5% may discriminate the course of disease.

AB - OBJECTIVE: Neuroendocrine neoplasms in the pancreas and duodenum with predominant or exclusive immunoreactivity for somatostatin (p-dSOMs) are rare, and knowledge on tumour biology, treatment, survival and prognostic factors is limited. This study aimes to describe clinical, pathological, and biochemical features as well as treatment and prognosis.DESIGN: Twenty-three patients with p-dSOM (9 duodenal, 12 pancreatic, 2 unknown primary tumour) were identified from our prospective neuroendocrine tumour (NET) database, and data according to the study aims were recorded.RESULTS: Of the 9 patients with duodenal SOM the m/f ratio was 4/5. All males and one female had NF-1. Seven patients had stage 1A-B and 2 had stage 2B disease. The Ki-67 index was 1-5% (median 2%). Plasma somatostatin was elevated in patients with 2B disease. Of the 14 patients with pancreatic SOM or unknown primary tumour the m/f ratio was 2/12. One male had MEN-1. Five had stage 1A-2B and nine had stage 4. The Ki-67 index was 1-40% (median 7%). Plasma somatostatin was elevated in seven patients. Patients reported symptoms related to the somatostatinoma syndrome, but none fulfilled the criteria for a full syndrome. Primary tumour in the pancreas, metastatic disease at diagnosis and higher tumour grade were all associated with a significantly poorer survival.CONCLUSION: None of the patients with p-dSOM presented with the full somatostatinoma syndrome. Prognostic factors are localisation of the primary tumour, dissemination and tumour grade. A Ki-67 of 5% may discriminate the course of disease.

U2 - 10.1159/000441605

DO - 10.1159/000441605

M3 - Journal article

C2 - 26505735

VL - 103

SP - 567

EP - 577

JO - Neuroendocrinology

JF - Neuroendocrinology

SN - 0028-3835

IS - 5

M1 - 000441605

ER -