Differences in clinical characteristics, treatment, and outcomes of sporadic and MEN-1-related insulinomas
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.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2023 |
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| Erschienen: |
2023 |
| Enthalten in: |
Zur Gesamtaufnahme - year:2023 |
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| Enthalten in: |
Endokrynologia Polska - (2023) vom: 11. Juli |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Opalińska, Marta [VerfasserIn] |
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| Links: |
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| Themen: |
Hypoglycaemia |
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| Anmerkungen: |
Date Revised 11.07.2023 published: Print-Electronic Citation Status Publisher |
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| doi: |
10.5603/EP.a2023.0049 |
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| Förderinstitution / Projekttitel: |
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| 520 | |a 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 | ||
| 520 | |a 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 | ||
| 520 | |a 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 | ||
| 520 | |a 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 | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a MEN-1 | |
| 650 | 4 | |a PanNET | |
| 650 | 4 | |a hypoglycaemia | |
| 650 | 4 | |a insulinoma | |
| 650 | 4 | |a pancreatic tumours | |
| 700 | 1 | |a Gilis-Januszewska, Aleksandra |e verfasserin |4 aut | |
| 700 | 1 | |a Morawiec-Sławek, Karolina |e verfasserin |4 aut | |
| 700 | 1 | |a Kurzyńska, Anna |e verfasserin |4 aut | |
| 700 | 1 | |a Sowa-Staszczak, Anna |e verfasserin |4 aut | |
| 700 | 1 | |a Bogusławska, Anna |e verfasserin |4 aut | |
| 700 | 1 | |a Rzepka, Ewelina |e verfasserin |4 aut | |
| 700 | 1 | |a Hubalewska-Dydejczyk, Alicja |e verfasserin |4 aut | |
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