Management of Appendix Neuroendocrine Neoplasms: Insights on the Current Guidelines
Abstract
:1. Introduction
2. Epidemiology
3. Classification of Appendiceal Neuroendocrine Neoplasms
4. Clinical Presentation
5. Diagnostic Assessment
6. Circulating Biomarkers
7. Staging and Imaging
8. Management
8.1. Treatment of Localized Disease
8.2. Treatment of Metastatic Disease
8.3. Surgery
8.4. Liver-Directed Therapy
8.5. Somatostatin Analog Therapy
8.6. Radiolabeled Somatostatin Analog Therapy
8.7. Targeted Therapies (mTORi and Anti-Angiogenesis)
8.8. Cytotoxic Chemotherapy
9. Conclusions
Author Contributions
Funding
Conflicts of Interest
Abbreviations
Abbreviation | Description |
NETs | neuroendocrine tumors |
NECs | neuroendocrine carcinomas |
ANENs | appendiceal neuroendocrine neoplasms |
ANETs | Appendiceal neuroendocrine tumors |
ANECs | appendiceal neuroendocrine carcinoma |
MiNENs | mixed neuroendocrine-non-neuroendocrine neoplasms |
pNETs | pancreatic neuroendocrine tumors |
GEP-NENs | gastroenteropancreatic neuroendocrine neoplasms |
G1 | low grade (Grade 1) |
G2 | intermediate grade (Grade 2) |
G3 | high grade (Grade 3) |
WHO | World Health Organization |
MI | mitotic index |
CgA | Chromogranin A |
5-HIAA | 5-hydroxyindoleacetic acid |
CT | computed tomography |
MRI | magnetic resonance imaging |
PET | positron emission tomography |
SPECT | single-photon emission computed tomography |
SSTR-PET | somatostatin receptor PET |
NANETS | North American Neuroendocrine Tumor Society |
ENETS | European Neuroendocrine Tumor Society |
NCI | national cancer institute |
RFA | radiofrequency ablation |
RHC | Right hemicolectomy |
SSAs | somatostatin analogues |
SSTR | somatostatin receptor |
LAR | long-acting release |
TTP | time to progression |
PFS | progression-free survival |
PRRT | peptide receptor radionuclide therapy |
HR | hazard ratio |
MDS | myelodysplastic syndrome |
VEGF | vascular endothelial growth factor |
PDGFRs | platelet-derived growth factor receptors |
FGFRs | Fibroblast growth factor receptors |
TKIs | tyrosine kinase inhibitors |
CAPTEM | capecitabine and temozolomide |
ORR | objective response rate |
IP | Irinotecan cisplatin doublet |
EP | platinum etoposide |
CI | confidence interval |
RR | relative risk |
OS | overall survival |
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Stage | TNM | 5-Year OS % | 10-Year OS % |
---|---|---|---|
I | T1N0M0 | 100 | 100 |
II | T1N1M0 T2N0M0 | 100 | 100 |
III | T2N1M0 T3, any N, M0 | 78 | 63 |
IV | Any T, Any N, M1 | 32 | 17 |
Guidelines | Lesion ≤ 1 cm | Lesion 1–2 cm | Lesion ≥ 2 cm |
---|---|---|---|
NCCN | Appendectomy | Appendectomy | RHC |
NANETs | Appendectomy | Appendectomy Discuss RHC if tumor at base with HRF | RHC |
ENETS | Appendectomy Discuss RHC if tumor at base with HRF or R1 | Appendectomy Discuss RHC if tumor at tip or middle with HRF Consider RHC if tumor at base with HRF or R1 | RHC |
UK NETs | Appendectomy | Appendectomy Discuss RHC if tumor at base with HRF | RHC |
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Mohamed, A.; Wu, S.; Hamid, M.; Mahipal, A.; Cjakrabarti, S.; Bajor, D.; Selfridge, J.E.; Asa, S.L. Management of Appendix Neuroendocrine Neoplasms: Insights on the Current Guidelines. Cancers 2023, 15, 295. https://doi.org/10.3390/cancers15010295
Mohamed A, Wu S, Hamid M, Mahipal A, Cjakrabarti S, Bajor D, Selfridge JE, Asa SL. Management of Appendix Neuroendocrine Neoplasms: Insights on the Current Guidelines. Cancers. 2023; 15(1):295. https://doi.org/10.3390/cancers15010295
Chicago/Turabian StyleMohamed, Amr, Sulin Wu, Mohamed Hamid, Amit Mahipal, Sakti Cjakrabarti, David Bajor, J. Eva Selfridge, and Sylvia L. Asa. 2023. "Management of Appendix Neuroendocrine Neoplasms: Insights on the Current Guidelines" Cancers 15, no. 1: 295. https://doi.org/10.3390/cancers15010295