Lymph node metastases are more frequent in paediatric appendiceal NET ≥1.5 cm but without impact on outcome - Data from the German MET studies
© 2024 Published by Elsevier Ltd..
BACKGROUND: Paediatric appendiceal neuroendocrine tumours (appNET) are very rare tumours, mostly detected incidentally by histopathological evaluation after appendectomy. Treatment recommendations are based on adult data considering high-risk NET as defined by European Neuroendocrine Tumour Society (ENETS) guidelines for completion right-sided hemicolectomy (RHC). Recent data suggest that less aggressive therapy may be justified.
PROCEDURE: Analysis of children and adolescents with appNET prospectively registered with the German Malignant Endocrine Tumour (MET) studies between 1997 and 2022.
RESULTS: By December 2022, 662 patients (64.7% females, 35.3% male) had been reported. Median age was 13.3 years [4.5-17.9], median duration of follow-up 2.2 years [0-10.9]. No distant metastases were reported. Tumour size was <1 cm in 63.5%, 1-2 cm in 33.2%, and >2 cm in 3.2% of patients. WHO grade 1 and 2 tumours were diagnosed in 76.9% and 23.1% of patients, respectively. Lymphovascular invasion and lymph node metastases were associated with tumour size ≥1.5 cm. 27.0% of patients presented with high-risk NET according to ENETS criteria. Of those, only 55.9% underwent secondary oncological right hemicolectomy. Neither distant metastases, nor recurrences or disease-related deaths occurred in patients with appendectomy only as well as in patients with completion RHC. Overall and event-free survival were both 100%.
CONCLUSIONS: Internationally harmonized consensus recommendations on treatment of children and adolescents with appendiceal NET are urgently needed to avoid completion RHC in high-risk patients.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2024 |
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| Erschienen: |
2024 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:50 |
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| Enthalten in: |
European journal of surgical oncology : the journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology - 50(2024), 4 vom: 29. Apr., Seite 108051 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Kuhlen, Michaela [VerfasserIn] |
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| Links: |
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Appendix |
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| Anmerkungen: |
Date Completed 01.04.2024 Date Revised 01.04.2024 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.1016/j.ejso.2024.108051 |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM369205804 |
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| 245 | 1 | 0 | |a Lymph node metastases are more frequent in paediatric appendiceal NET ≥1.5 cm but without impact on outcome - Data from the German MET studies |
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| 500 | |a Citation Status MEDLINE | ||
| 520 | |a © 2024 Published by Elsevier Ltd. | ||
| 520 | |a BACKGROUND: Paediatric appendiceal neuroendocrine tumours (appNET) are very rare tumours, mostly detected incidentally by histopathological evaluation after appendectomy. Treatment recommendations are based on adult data considering high-risk NET as defined by European Neuroendocrine Tumour Society (ENETS) guidelines for completion right-sided hemicolectomy (RHC). Recent data suggest that less aggressive therapy may be justified | ||
| 520 | |a PROCEDURE: Analysis of children and adolescents with appNET prospectively registered with the German Malignant Endocrine Tumour (MET) studies between 1997 and 2022 | ||
| 520 | |a RESULTS: By December 2022, 662 patients (64.7% females, 35.3% male) had been reported. Median age was 13.3 years [4.5-17.9], median duration of follow-up 2.2 years [0-10.9]. No distant metastases were reported. Tumour size was <1 cm in 63.5%, 1-2 cm in 33.2%, and >2 cm in 3.2% of patients. WHO grade 1 and 2 tumours were diagnosed in 76.9% and 23.1% of patients, respectively. Lymphovascular invasion and lymph node metastases were associated with tumour size ≥1.5 cm. 27.0% of patients presented with high-risk NET according to ENETS criteria. Of those, only 55.9% underwent secondary oncological right hemicolectomy. Neither distant metastases, nor recurrences or disease-related deaths occurred in patients with appendectomy only as well as in patients with completion RHC. Overall and event-free survival were both 100% | ||
| 520 | |a CONCLUSIONS: Internationally harmonized consensus recommendations on treatment of children and adolescents with appendiceal NET are urgently needed to avoid completion RHC in high-risk patients | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Appendix | |
| 650 | 4 | |a Children and adolescents | |
| 650 | 4 | |a Hemicolectomy | |
| 650 | 4 | |a Neuroendocrine tumour | |
| 700 | 1 | |a Kunstreich, Marina |e verfasserin |4 aut | |
| 700 | 1 | |a Pape, Ulrich-Frank |e verfasserin |4 aut | |
| 700 | 1 | |a Seitz, Guido |e verfasserin |4 aut | |
| 700 | 1 | |a Lessel, Lienhard |e verfasserin |4 aut | |
| 700 | 1 | |a Vokuhl, Christian |e verfasserin |4 aut | |
| 700 | 1 | |a Frühwald, Michael C |e verfasserin |4 aut | |
| 700 | 1 | |a Vorwerk, Peter |e verfasserin |4 aut | |
| 700 | 1 | |a Redlich, Antje |e verfasserin |4 aut | |
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