PMID- 37431872
OWN - NLM
STAT- Publisher
LR  - 20230711
IS  - 2299-8306 (Electronic)
IS  - 0423-104X (Linking)
DP  - 2023 Jul 11
TI  - Differences in clinical characteristics, treatment, and outcomes of sporadic and 
      MEN-1-related insulinomas.
LID - 10.5603/EP.a2023.0049 [doi]
AB  - INTRODUCTION: Although in most cases insulinomas are small, benign, sporadic 
      tumours, they can also be associated with hereditary syndromes, most commonly 
      multiple endocrine neoplasia type 1 (MEN-1). Such a diagnosis significantly 
      affects patient management. The objective was to elucidate the clinical 
      differences between sporadic and MEN-1-linked insulinoma. MATERIAL AND METHODS: 
      Comparison of clinical and histopathological characteristics, types of surgery, 
      and outcomes of patients with sporadic and MEN-1-related insulinoma diagnosed 
      between 2015 and 2022. RESULTS: There were 17 cases of insulinomas that underwent 
      MEN-1 genetic testing (10 women and 7 men). In 7 cases, the mutation in the menin 
      gene was confirmed. The median age at the time of diagnosis of sporadic 
      insulinoma related to MEN-1 was 69 years (range 29-87) and 31.5 years (16-47), 
      respectively. Primary hyperparathyroidism (PHP) was found in 6 of 7 patients with 
      MEN-1-related insulinoma, while in none of the patients without MEN-1 mutations. 
      Multifocal pancreatic NETs were found in 3 patients with MEN-1 syndrome, while in 
      all sporadic cases there was a single pancreatic tumour. Two patients with 
      insulinoma related to MEN-1 had a positive familial history of MEN-1-related 
      diseases, while none with sporadic form. Dissemination at diagnosis was found in 
      4 cases, including 3 patients with insulinoma related to MEN-1-related 
      insulinoma. Patients with sporadic and MEN-1-related insulinoma did not differ in 
      tumour size, Ki-67 proliferation index, and outcome. CONCLUSIONS: Of all the 
      features evaluated, only the multifocal nature of pancreatic neuroendocrine 
      tumour (PanNET) lesions and a positive family history differentiated between 
      patients with sporadic and MEN-1-related insulinomas. An age of insulinoma 
      diagnosis of less than 30 years may be a strong indicator of an increased risk of 
      MEN-1 syndrome.
FAU - Opalinska, Marta
AU  - Opalinska M
AUID- ORCID: 0000-0002-5919-9291
AD  - Chair and Department of Endocrinology, Jagiellonian University Medical College, 
      Krakow, Poland.
FAU - Gilis-Januszewska, Aleksandra
AU  - Gilis-Januszewska A
AD  - Chair and Department of Endocrinology, Jagiellonian University Medical College, 
      Krakow, Poland. myjanusz@cyf-kr.edu.pl.
FAU - Morawiec-Slawek, Karolina
AU  - Morawiec-Slawek K
AD  - Chair and Department of Endocrinology, Jagiellonian University Medical College, 
      Krakow, Poland.
FAU - Kurzynska, Anna
AU  - Kurzynska A
AD  - Chair and Department of Endocrinology, Jagiellonian University Medical College, 
      Krakow, Poland.
FAU - Sowa-Staszczak, Anna
AU  - Sowa-Staszczak A
AD  - Chair and Department of Endocrinology, Jagiellonian University Medical College, 
      Krakow, Poland.
FAU - Boguslawska, Anna
AU  - Boguslawska A
AD  - Chair and Department of Endocrinology, Jagiellonian University Medical College, 
      Krakow, Poland.
FAU - Rzepka, Ewelina
AU  - Rzepka E
AD  - Chair and Department of Endocrinology, Jagiellonian University Medical College, 
      Krakow, Poland.
FAU - Hubalewska-Dydejczyk, Alicja
AU  - Hubalewska-Dydejczyk A
AD  - Chair and Department of Endocrinology, Jagiellonian University Medical College, 
      Krakow, Poland.
LA  - eng
PT  - Journal Article
DEP - 20230711
PL  - Poland
TA  - Endokrynol Pol
JT  - Endokrynologia Polska
JID - 0370674
SB  - IM
OTO - NOTNLM
OT  - MEN-1
OT  - PanNET
OT  - hypoglycaemia
OT  - insulinoma
OT  - pancreatic tumours
EDAT- 2023/07/11 13:10
MHDA- 2023/07/11 13:10
CRDT- 2023/07/11 07:42
PHST- 2023/04/04 00:00 [received]
PHST- 2023/05/07 00:00 [accepted]
PHST- 2023/07/11 13:10 [medline]
PHST- 2023/07/11 13:10 [pubmed]
PHST- 2023/07/11 07:42 [entrez]
AID - VM/OJS/J/95007 [pii]
AID - 10.5603/EP.a2023.0049 [doi]
PST - aheadofprint
SO  - Endokrynol Pol. 2023 Jul 11. doi: 10.5603/EP.a2023.0049.