Authors : Mohamed Arsath Shamsudeen; Chandrasekaran Selvaraju; Muhammad Azri Bin Mohamadu Thahir; Intan Nor Elmyra Binti Nor Azliman; Nurul Najihah Binti Johari; Syarifah Nur Fatnin Binti Syed Amran

Volume/Issue : Volume 10 - 2025, Issue 6 - June

Google Scholar : https://tinyurl.com/yv7c9h82

DOI : https://doi.org/10.38124/ijisrt/25jun1825

Note : A published paper may take 4-5 working days from the publication date to appear in PlumX Metrics, Semantic Scholar, and ResearchGate.

Note : Google Scholar may take 30 to 40 days to display the article.

Abstract : Appendicular neoplasms, although rare, represent a diverse group of tumours arising from the appendix, including adenocarcinomas, carcinoid tumours, and mucinous neoplasms. This review aims to synthesize current knowledge on the epidemiology, diagnostic approaches, and treatment options for appendicular neoplasms. We conducted a comprehensive literature search across multiple databases, identifying key studies that elucidate the incidence, clinical presentation, and histopathological characteristics of these tumours. The epidemiological data highlight a slight male predominance and variability in incidence across different populations. Clinical symptoms often mimic acute appendicitis, leading to potential diagnostic challenges. Advanced imaging techniques, including ultrasound and computed tomography, play a critical role in diagnosis, often supplemented by histological analysis. Treatment typically involves surgical resection, with chemotherapy considered for advanced or high-grade tumours. Prognosis varies significantly depending on tumour type and stage at diagnosis, underscoring the need for early detection and tailored management strategies. This review identifies critical gaps in the literature and suggests directions for future research to enhance understanding and treatment of appendicular neoplasms.

Keywords : Epidemiology, Adenocarcinomas, Carcinoid Tumours, Mucinous Neoplasms, Clinical Presentations.

References :

1. Turaga KK, Pappas SG, Gamblin T. Importance of histologic subtype in the staging of appendiceal tumors. Ann Surg Oncol. 2012;19:1379-1385.
2. Leonards LM, Pahwa A, Patel MK, Petersen J, Nguyen MJ, Jude CM. Neoplasms of the appendix: pictorial review with clinical and pathologic correlation. Radiographics. 2017 Jul;37(4):1059-83.
3. Connor SJ, Hanna GB, Frizelle FA. Appendiceal tumors: retrospective clinicopathologic analysis of appendiceal tumors from 7,970 appendectomies. Dis Colon Rectum. 1998;41(1):75–80.
4. Turaga KK, Pappas SG, Gamblin T. Importance of histologic subtype in the staging of appendiceal tumors. Ann Surg Oncol. 2012;19(5):1379–85.
5. Deshmukh S, Verde F, Johnson PT, Fishman EK, Macura KJ. Anatomical variants and pathologies of the vermix. Emerg Radiol. 2014;21(5):543–52.
6. Hoehn, R. S., Rieser, C. J., Choudry, M. H., Melnitchouk, N., Hechtman, J., & Bahary, N. (2021). Current management of appendiceal neoplasms. American Society of Clinical Oncology Educational Book, 41, 118-132.
7. Stopenski SJ, Grigorian A, Carmichael J, Mills S, Brady M, Dolich M, et al. Risk factors for Appendiceal Cancer after Appendectomy. Am Surg. 2021;87(6):994–8. 10.1177/0003134820960077.
8.  Brunner M, Lapins P, Langheinrich M, Baecker J, Krautz C, Kersting S, et al. Risk factors for appendiceal neoplasm and malignancy among patients with acute appendicitis. Int J Colorectal Dis. 2020;35:157–13. 10.1007/s00384-019-03453-5.
9. Loftus TJ, Raymond SL, Sarosi GA, Croft CA, Smith RS, Efron PA, et al. Predicting appendiceal tumors among patients with appendicitis. J Trauma Acute Care Surg. 2017;82(4):771–5. 10.1097/TA.0000000000001378.
10. Connor SJ, Hanna GB, Frizelle FA. Appendiceal tumors: retrospective clinicopathologic analysis of appendiceal tumors from 7,970 appendectomies. Dis Colon Rectum. 1998;41:75-80.
11. Brassil M, Lee R, O’Neill D, Woods G, Moloney BM, Dunne R, et al. Appendiceal tumours – a correlation of CT features and histopathological diagnosis. J Med Imaging Radiat Oncol. 2022;66(1):92–101. 10.1111/1754-9485.13329
12. Kwag KS, Kim HJ, Jang SK, Yeon JW, Paik S, Lean BG, et al. Sonographic findings of malignant appendix tumors in seven cases. J Med Ultrasound. 2018;26(1):52–5. 10.4103/JMU.JMU_16_17.
13. Tang LH, Shia J, Soslow RA, Dhall D, Wong WD, O’Reilly E, et al. Pathologic classification and clinical behavior of the Spectrum of Goblet Cell Carcinoid tumors of the appendix. Am J Surg Pathol. 2008;32(10):1429–43. 10.1097/PAS.0b013e31817f1816
14. Tan, Sanda A., and Luca Stocchi. "Appendiceal neoplasms." The ASCRS Textbook of Colon and Rectal Surgery (2022): 577-586.
15. Loftus, T.J.; Raymond, S.L.; Sarosi, G.A.J.; Croft, C.A.; Smith, R.S.; Efron, P.A.; Moore, F.A.; Brakenridge, S.C.M.; Mohr, A.M.; Jordan, J.R. Predicting appendiceal tumors among patients with appendicitis. J. Trauma Acute Care Surg. 2017, 82, 771–775.
16. Tajima, T.; Tajiri, T.; Mukai, M.; Sugiyama, T.; Hasegawa, S.; Yamamoto, S.; Sadahiro, S.; Shimada, H.; Makuuchi, H. Single-center analysis of appendiceal neoplasms. Oncol. Lett. 2018, 15, 6393–6399.
17. Govaerts, K.; Lurvink, R.; De Hingh, I.; Van der Speeten, K.; Villeneuve, L.; Kusamura, S.; Kepenekian, V.; Deraco, M.; Glehen, O.; Moran, B.; et al. Appendiceal tumours and pseudomyxoma peritonei: Literature review with PSOGI/EURACAN clinical practice guidelines for diagnosis and treatment. Eur. J. Surg. Oncol. 2021, 47, 11–35.
18.  Hatch, Q.M.; Gilbert, E.W. Appendiceal Neoplasms. Clin. Colon Rectal. Surg. 2018, 31, 278–287.
19.  Bastiaenen, V.P.; Allema, W.M.; Klaver, C.E.; van Dieren, S.; Koens, L.; Tanis, P.J.; Bemelman, W.A. Routine histopathologic examination of the appendix after appendectomy for presumed appendicitis: Is it really necessary? A systematic review and meta-analysis. Surgery 2020, 168, 305–312.
20. Lohsiriwat, V.; Vongjirad, A.; Lohsiriwat, D. Value of Routine Histopathologic Examination of Three Common Surgical Specimens: Appendix, Gallbladder, and Hemorrhoid. World J. Surg. 2009, 33, 2189–2193.  
21. Marmor, S.; Portschy, P.R.; Tuttle, T.M.; Virnig, B.A. The Rise in Appendiceal Cancer Incidence: 2000–2009. J. Gastrointest. Surg. 2015, 19, 743–750.
22. Todd, R.D.; Sarosi, G.A.; Nwariaku, F.; Anthony, T. Incidence and predictors of appendiceal tumors in elderly males presenting with signs and symptoms of acute appendicitis. Am. J. Surg. 2004, 188, 500–504.
23.  Naar, L.; Kim, P.; Byerly, S.; Vasileiou, G.; Zhang, H.; Yeh, D.D.; Kaafarani, H.M.; Alouidor, R.; Hing, K.K.; Sharp, V.; et al. Increased risk of malignancy for patients older than 40 years with appendicitis and an appendix wider than 10 mm on computed tomography scan: A post hoc analysis of an EAST multicenter study. Surgery 2020, 168, 701–706.
24. Orchard, P.; Preece, R.; Thomas, M.G.; Dixon, S.W.; Wong, N.A.C.S.; Chambers, A.C.; Messenger, D.E. Demographic trends in the incidence of malignant appendiceal tumours in England between 1995 and 2016: Population-based analysis. BJS Open 2022, 6, zrac103.  
25. . Nagtegaal, I.D.; Odze, R.D.; Klimstra, D.; Paradis, V.; Rugge, M.; Schirmacher, P.; Washington, K.M.; Carneiro, F.; Cree, I.A.; The WHO Classification of Tumours Editorial Board. The 2019 WHO classification of tumours of the digestive system. Histopathology 2020, 76, 182–188.
26.  Matsui, S.; Murata, K.M.; Fukunaga, Y.M.; Takeda, T.; Fujii, M.; Yamaguchi, T.M.; Kagawa, Y.M.; Mizushima, T.M.; Ohno, Y.; Yao, T.M.; et al. Analysis of Clinicopathological Characteristics of Appendiceal Tumors in Japan: A Multicenter Collaborative Retrospective Clinical Study—A Japanese Nationwide Survey. Dis. Colon Rectum 2020, 63, 1403–1410.
27.  Bolmers, M.D.M.; de Jonge, J.; van Rossem, C.C.; van Geloven, A.A.W.; Bemelman, W.A.; van Acker, G.J.; Akkermans, B.; Akkersdijk, G.J.; Algie, G.D.; Allema, J.H.; et al. Appendicular neoplasms and consequences in patients undergoing surgery for suspected acute appendicitis. Int. J. Color. Dis. 2020, 35, 2065–2071.
28. Wang, Y.; Geng, Y.; Hu, W. Survival and prognostic factors for postoperative primary appendiceal cancer: A retrospective cohort study based on the Surveillance, Epidemiology, and End Results database. J. Gastrointest. Oncol. 2022, 13, 1433–14.
29.  Johansson, J.; Andersson, R.E.; Landerholm, K.; Redéen, S. Incidence of Appendiceal Malignancies in Sweden Between 1970 and 2012. Mol. Med. 2020, 44, 4086–4092.
30.  Tan HL, Tan GH, Teo M. Two rare cases of appendiceal collision tumours involving an appendiceal mucinous neoplasm and carcinoid. BMJ Case Rep. (2016) 2016: bcr2015213938
31. Singh NG, Mannan AA, Kahvic M, Nur AM. Mixed adenocarcinoma-carcinoid (collision tumor) of the appendix. Med Princ Pract. (2011) 20(4):384–6
32. Villa M, Sforza D, Siragusa L, Guida AM, Ciancio Manuelli M, Pirozzi BM, et al. A low-grade appendiceal mucinous neoplasia and neuroendocrine appendiceal collision tumor: a case report and review of the literature. Am J Case Rep. (2021) 22:e927876.
33. Dellaportas D, Vlahos N, Polymeneas G, Gkiokas G, Dastamani C, Carvounis E, et al. Collision tumor of the appendix: mucinous cystadenoma and carcinoid. A Case Report. Chirurgia (Bucur). (2014) 109(6):843–5.25560511
34. Sholi AN, Gray KD, Pomp A. Management and outcome of an appendiceal collision tumour composed of neuroendocrine and mucinous neoplasms. BMJ Case Rep. (2019) 12(7):e229414.
35. Baena-del-Valle J, Palau-Lázaro M, Mejía-Arango M, Otero J, Londoño-Schimmer E, Cortes N, et al. Well differentiated neuroendocrine tumor of the appendix and low-grade appendiceal mucinous neoplasm presenting as a collision tumor. Rev Esp Enferm Dig. (2015) 107(6):396–8.26031879
36. Sugarbaker PH, Ben-Yaacov A, Hazzan D, Nissan A. Synchronous primary neuroendocrine and mucinous epithelial tumors present in the same appendix. Case report of 2 patients. Int J Surg Case Rep. (2020) 67:76–9.
37. Rossi G, Bertolini F, Sartori G, Bigiani N, Cavazza A, Foroni M, et al. Primary mixed adenocarcinoma and small cell carcinoma of the appendix: a clinicopathologic, immunohistochemical, and molecular study of a hitherto unreported tumor. Am J Surg Pathol. (2004) 28(9):1233–9.
38. Ekinci N, Gün E, Avcı A, Er A. Coexistence of low-grade mucinous neoplasm and carcinoid (collision tumor) within multiple appendiceal diverticula: a case report. Turk J Surg. (2018) 31:1–3.
39. Cafaro MA, Yaryura Montero JG, Bianco A, Petersen ML, Cárdenas Villa RD, Cardozo Dutra B, et al. Tumor de colisión apendicular: neoplasia epitelial mucinosa y tumor neuroendocrino [appendiceal collision tumor: mucinous epithelial neoplasm and neuroendocrine tumor]. Rev Fac Cien Med Univ Nac Cordoba. (2020) 77(2):113–6.
40. Viel G, Ciarleglio FA, Frisini M, Marcucci S, Valcanover S, Bragantini E, Barbareschi M, Mereu L, Tateo S, Merola E, Armelao F. Appendiceal collision tumors: case reports, management and literature review. Frontiers in Surgery. 2023 Jun 7;10:1184322.