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Appendiceal carcinoids are rare overall but represent the most common tumor of the appendix. The appendix is also one of the most common (but not the most common) locations for gastrointestinal carcinoid tumors.
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Appendiceal carcinoids can present as the obstructive cause of acute appendicitis or less commonly a mucocele. Often they are incidental findings 3.
Appendiceal carcinoids are neuroendocrine tumors that classically arise at the appendiceal tip from subepithelial neuroendocrine cells 4.
Two types are described 4:
- classic type
- Goblet cell carcinoid
- considered a distinct tumor, separate from classic neuroendocrine tumor, with its own evolving classification based on degree of differentiation 5
- small (usually <1 cm), round masses or diffuse appendiceal thickening
- most commonly at the appendiceal tip (75%) 1,2
Treatment and prognosis
Appendiceal carcinoids have a more benign course than other gastrointestinal carcinoids, rarely metastasizing, with a 5-year survival rate of >90% 2,3.
Metastases, when they do occur are to regional nodes, and rarely the liver 6,7.
- other causes of mucocele of the appendix
- other appendiceal neoplasms
- mucinous cystadenocarcinoma: most common malignant neoplasm of the appendix
- colonic adenocarcinoma
- 1. Brant WE, Helms C. Fundamentals of Diagnostic Radiology. Lippincott Williams & Wilkins. ISBN:1608319121. Read it at Google Books - Find it at Amazon
- 2. Levy AD, Sobin LH. From the archives of the AFIP: Gastrointestinal carcinoids: imaging features with clinicopathologic comparison. Radiographics. 2007;27 (1): 237-57. Radiographics (full text) - doi:10.1148/rg.271065169 - Pubmed citation
- 3. Pickhardt PJ, Levy AD, Rohrmann CA et-al. Primary neoplasms of the appendix: radiologic spectrum of disease with pathologic correlation. Radiographics. 2003;23 (3): 645-62. Radiographics (full text) - doi:10.1148/rg.233025134 - Pubmed citation
- 4. Ganeshan D, Bhosale P, Yang T et-al. Imaging features of carcinoid tumors of the gastrointestinal tract. AJR Am J Roentgenol. 2013;201 (4): 773-86. doi:10.2214/AJR.12.9758 - Pubmed citation
- 5. Roy P, Chetty R. Goblet cell carcinoid tumors of the appendix: An overview. (2010) World journal of gastrointestinal oncology. 2 (6): 251-8. doi:10.4251/wjgo.v2.i6.251 - Pubmed
- 6. Elkbuli A, Sanchez C, McKenney M, Boneva D. Incidental neuro-endocrine tumor of the appendix: Case report and literature review. (2019) Annals of medicine and surgery (2012). 43: 44-47. doi:10.1016/j.amsu.2019.05.015 - Pubmed
- 7. Leonards LM, Pahwa A, Patel MK, Petersen J, Nguyen MJ, Jude CM. Neoplasms of the Appendix: Pictorial Review with Clinical and Pathologic Correlation. (2017) Radiographics : a review publication of the Radiological Society of North America, Inc. 37 (4): 1059-1083. doi:10.1148/rg.2017160150 - Pubmed