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Pancreatic carcinoids (serotonin-producing pancreatic neuroendocrine neoplasms): Report of 5 cases and review of the literature.

Abstract Pancreatic neuroendocrine neoplasms (pNENs) are relatively rare tumors representing 1% to 2% of all pancreatic neoplasms. These tumors can secrete a variety of biologically active substances giving rise to distinct clinical symptoms or can be clinically nonfunctioning. Apart from insulinomas and gastrinomas, which constitute the majority of functioning pNENs, some tumors may secrete serotonin presenting with the features of the carcinoid syndrome. These so-called pancreatic carcinoids are considered relatively rare tumors and are associated with increased urinary levels of 5-hydroxyindoleacetic acid (5-HIAA). It has recently been suggested that the prevalence of such tumors might be underestimated.
PMID
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Authors

Mayor MeshTerms
Keywords
Journal Title medicine
Publication Year Start




PMID- 28422824
OWN - NLM
STAT- In-Process
DA  - 20170419
LR  - 20170419
IS  - 1536-5964 (Electronic)
IS  - 0025-7974 (Linking)
VI  - 96
IP  - 16
DP  - 2017 Apr
TI  - Pancreatic carcinoids (serotonin-producing pancreatic neuroendocrine neoplasms): 
      Report of 5 cases and review of the literature.
PG  - e6201
LID - 10.1097/MD.0000000000006201 [doi]
AB  - INTRODUCTION: Pancreatic neuroendocrine neoplasms (pNENs) are relatively rare
      tumors representing 1% to 2% of all pancreatic neoplasms. These tumors can
      secrete a variety of biologically active substances giving rise to distinct
      clinical symptoms or can be clinically nonfunctioning. Apart from insulinomas and
      gastrinomas, which constitute the majority of functioning pNENs, some tumors may 
      secrete serotonin presenting with the features of the carcinoid syndrome. These
      so-called pancreatic carcinoids are considered relatively rare tumors and are
      associated with increased urinary levels of 5-hydroxyindoleacetic acid (5-HIAA). 
      It has recently been suggested that the prevalence of such tumors might be
      underestimated. CASES: We present a series of 5 patients from our database of 138
      pNENs (5/138, 3.62%), harboring serotonin-producing pNENs and describe their
      distinctive clinical, biochemical, histopathological features, and response to
      treatment along with a review of the relevant available literature. CONCLUSION:
      Such tumors are considered rare, although this may be an underestimate as
      systematic screening for the presence of serotonin in tissue or elevated urinary 
      5-HIAA levels in patients with apparently nonfunctioning pNENs is not currently
      recommended. In order to reach such a consensus, data from large prospective
      studies are needed in order to evaluate the impact of this type of tumors in
      survival and clinical outcome, since some studies have suggested a worse
      prognosis.
FAU - Tsoukalas, Nikolaos
AU  - Tsoukalas N
AD  - aDepartment of Medical Oncology, "401" General Military Hospital bEndocrine Unit,
      Department of Pathophysiology, National University of Athens cPathology
      Department, Evangelismos Hospital, Athens, Greece.
FAU - Chatzellis, Eleftherios
AU  - Chatzellis E
FAU - Rontogianni, Dimitra
AU  - Rontogianni D
FAU - Alexandraki, Krystallenia I
AU  - Alexandraki KI
FAU - Boutzios, Georgios
AU  - Boutzios G
FAU - Angelousi, Anna
AU  - Angelousi A
FAU - Kaltsas, Gregory
AU  - Kaltsas G
LA  - eng
PT  - Journal Article
PL  - United States
TA  - Medicine (Baltimore)
JT  - Medicine
JID - 2985248R
EDAT- 2017/04/20 06:00
MHDA- 2017/04/20 06:00
CRDT- 2017/04/20 06:00
AID - 10.1097/MD.0000000000006201 [doi]
AID - 00005792-201704210-00005 [pii]
PST - ppublish
SO  - Medicine (Baltimore). 2017 Apr;96(16):e6201. doi: 10.1097/MD.0000000000006201.

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