PMID- 30795813
OWN - NLM
STAT- MEDLINE
DCOM- 20190419
LR  - 20200225
IS  - 1750-1172 (Electronic)
IS  - 1750-1172 (Linking)
VI  - 14
IP  - 1
DP  - 2019 Feb 22
TI  - Intertumor heterogeneity in 60 pancreatic neuroendocrine tumors associated with 
      multiple endocrine neoplasia type 1.
PG  - 54
LID - 10.1186/s13023-019-1034-4 [doi]
LID - 54
AB  - BACKGROUND: Patients with multiple endocrine neoplasia type 1 (MEN-1) develop 
      multiple pancreatic neuroendocrine neoplasias (PNENs). Size at diagnosis and 
      growth during follow-up are crucial parameters. According to the WHO 2017, 
      grading is another important parameter. The impact of grading compared to size 
      (WHO 2000) on the clinical course needs to be evaluated. METHODS: Sixty PNENs of 
      six patients with MEN-1 were retrospectively evaluated. RESULTS: Fifty-one tumors 
      with a diameter of < 20 mm were graded as G1. Two of 9 tumors with diameters of 
      >/=20 mm were graded as G2. Tumor size of >/=20 mm correlated significantly with 
      higher proliferation (p = 0.000617). Lymph node metastases were documented in two 
      patients with a total of 19 tumors. In one patient, all 13 tumors (diameter: 0.4 
      to 100 mm) were classified as G1. However, metastases were documented in 9/29 
      lymph nodes. In the other patient, 5 tumors (3.5 to 20 mm) were classified as G1. 
      The sixth tumor (30 mm) was classified as G2 (Ki-67: 8%). Metastases were 
      revealed in 2/20 lymph nodes. CONCLUSIONS: Tumor size of >/=20 mm seems to 
      correlate with more aggressive MEN-1 related pancreatic disease, regardless of 
      individual proliferation. Tumors >/=20 mm and tumors graded as G2 should be treated 
      surgically regardless of their size.
FAU - Selberherr, Andreas
AU  - Selberherr A
AUID- ORCID: 0000-0002-6545-7023
AD  - Section "Endocrine Surgery", Division of General Surgery, Department of Surgery, 
      Medical University, Wahringer Gurtel 18-20, A-1090, Vienna, Austria. 
      andreas.selberherr@meduniwien.ac.at.
FAU - Koperek, Oskar
AU  - Koperek O
AD  - Department of Pathology, Medical University, Wahringer Gurtel 18-20, A-1090, 
      Vienna, Austria.
FAU - Riss, Philipp
AU  - Riss P
AD  - Section "Endocrine Surgery", Division of General Surgery, Department of Surgery, 
      Medical University, Wahringer Gurtel 18-20, A-1090, Vienna, Austria.
FAU - Scheuba, Christian
AU  - Scheuba C
AD  - Section "Endocrine Surgery", Division of General Surgery, Department of Surgery, 
      Medical University, Wahringer Gurtel 18-20, A-1090, Vienna, Austria.
FAU - Niederle, Martin B
AU  - Niederle MB
AD  - Department of Anesthesiology, Medical University, Wahringer Gurtel 18-20, A-1090, 
      Vienna, Austria.
FAU - Kaderli, Reto
AU  - Kaderli R
AD  - Section "Endocrine Surgery", Division of General Surgery, Department of Surgery, 
      Medical University, Wahringer Gurtel 18-20, A-1090, Vienna, Austria.
FAU - Perren, Aurel
AU  - Perren A
AD  - Institute of Pathology, University of Bern, Murtenstrasse 31, CH-3012, Bern, 
      Switzerland.
FAU - Niederle, Bruno
AU  - Niederle B
AD  - Section "Endocrine Surgery", Division of General Surgery, Department of Surgery, 
      Medical University, Wahringer Gurtel 18-20, A-1090, Vienna, Austria.
LA  - eng
PT  - Journal Article
DEP - 20190222
PL  - England
TA  - Orphanet J Rare Dis
JT  - Orphanet journal of rare diseases
JID - 101266602
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Aged
MH  - Aged, 80 and over
MH  - Female
MH  - Humans
MH  - Immunohistochemistry
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/metabolism/*pathology
MH  - Neuroendocrine Tumors/metabolism/*pathology
MH  - Pancreatic Neoplasms/metabolism/*pathology
MH  - Retrospective Studies
MH  - Young Adult
PMC - PMC6387504
OTO - NOTNLM
OT  - Intertumor heterogeneity
OT  - MEN-1
OT  - Multiple endocrine neoplasia
OT  - NET
OT  - Pancreatic neuroendocrine tumors
COIS- ETHICS APPROVAL AND CONSENT TO PARTICIPATE: All procedures performed in this 
      study involving human material were in accordance with the ethical standards of 
      the institutional research committee and with the 1964 Helsinki declaration and 
      its later amendments or comparable ethical standards. Informed consent was 
      obtained from all individual participants included in the study. CONSENT FOR 
      PUBLICATION: Consent for publication was obtained from all participants. 
      COMPETING INTERESTS: The authors declare that they have no competing interests. 
      PUBLISHER'S NOTE: Springer Nature remains neutral with regard to jurisdictional 
      claims in published maps and institutional affiliations.
EDAT- 2019/02/24 06:00
MHDA- 2019/04/20 06:00
PMCR- 2019/02/22
CRDT- 2019/02/24 06:00
PHST- 2018/08/17 00:00 [received]
PHST- 2019/02/17 00:00 [accepted]
PHST- 2019/02/24 06:00 [entrez]
PHST- 2019/02/24 06:00 [pubmed]
PHST- 2019/04/20 06:00 [medline]
PHST- 2019/02/22 00:00 [pmc-release]
AID - 10.1186/s13023-019-1034-4 [pii]
AID - 1034 [pii]
AID - 10.1186/s13023-019-1034-4 [doi]
PST - epublish
SO  - Orphanet J Rare Dis. 2019 Feb 22;14(1):54. doi: 10.1186/s13023-019-1034-4.