Skip to main content
SpringerLink
Log in

Appendiceal Tumors

  • Chapter
  • First Online:
Oncologic Surgical Emergencies

Part of the book series: Hot Topics in Acute Care Surgery and Trauma ((HTACST))

  • 223 Accesses

Abstract

Appendiceal adenocarcinoma was first described by Berger in 1882 [1], whereas a tumor arising from neuroendocrine cells [neuroendocrine tumors (NETs)] was reported by Masson in 1928 [2]. Primary appendiceal tumors (ATs) are rare entities, occurring in <2% of all appendectomies, with an age-adjusted incidence of 0.12 cases per million people per year, even if in large databases the incidence may reach 0.97 per year [3, 4]. Thanks to the current detection methods, including both imaging and colonoscopy, there was an increased incidence and decreased age at diagnosis of AT [5].

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 129.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Hardcover Book
USD 169.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Berger A. Ein Fall von Krebs des Wurmfortsatzes. Ber Klin Wochenschr. 1882;19:610.

    Google Scholar 

  2. Masson P. Carcinoids (Argentaffin-cell tumors) and nerve hyperplasia of the appendicular mucosa. Am J Pathol. 1928;4(3):181–212.

    CAS  PubMed  PubMed Central  Google Scholar 

  3. Hanna M, Hwang G, Moghadamyeghaneh Z, et al. Incidental appendiceal cancer at appendectomy: an analysis of incidence, trends and risk factors. Dis Colon Rectum. 2015;58:E339.

    Google Scholar 

  4. Connor SJ, Hanna GB, Frizelle FA. Appendiceal tumors: retrospective clinicopathologic analysis of appendiceal tumors from 7970 appendectomies. Dis Colon Rectum. 1998;41(1):75–80. https://doi.org/10.1007/BF02236899.

    Article  CAS  PubMed  Google Scholar 

  5. Turaga KK, Pappas SG, Gamblin T. Importance of histologic subtype in the staging of appendiceal tumors. Ann Surg Oncol. 2012;19(5):1379–85. https://doi.org/10.1245/s10434-012-2238-1.

    Article  PubMed  Google Scholar 

  6. Marmor S, Portschy PR, Tuttle TM, Virnig BA. The rise in appendiceal cancer incidence: 2000–2009. J Gastrointest Surg. 2015;19(4):743–50. https://doi.org/10.1007/s11605-014-2726-7.

    Article  PubMed  Google Scholar 

  7. O'Donnell ME, Badger SA, Beattie GC, Carson J, Garstin WI. Malignant neoplasms of the appendix. Int J Color Dis. 2007;22(10):1239–48. https://doi.org/10.1007/s00384-007-0304-0.

    Article  Google Scholar 

  8. Stinner B, Rothmund M. Neuroendocrine tumors (carcinoids) of the appendix. Best Pract Res Clin Gastroenterol. 2005;19:729–38.

    Article  CAS  PubMed  Google Scholar 

  9. Smeenk RM, van Velthuysen ML, Verwaal VJ, Zoetmulder FA. Appendiceal neoplasms and pseudomyxoma peritonei: a population based study. Eur J Surg Oncol. 2008;34(2):196–201. https://doi.org/10.1016/j.ejso.2007.04.002.

    Article  CAS  PubMed  Google Scholar 

  10. Cerame MA. A 25-year review of adenocarcinoma of the appendix. A frequently perforating carcinoma. Dis Colon Rectum. 1988;31(2):145–50. https://doi.org/10.1007/BF02562650.

    Article  CAS  PubMed  Google Scholar 

  11. Sandor A, Modlin IM. A retrospective analysis of 1570 appendiceal carcinoids. Am J Gastroenterol. 1998;93(3):422–8. https://doi.org/10.1111/j.1572-0241.1998.00422.x.

    Article  CAS  PubMed  Google Scholar 

  12. Cortina R, McCormick J, Kolm P, Perry RR. Management and prognosis of adenocarcinoma of the appendix. Dis Colon Rectum. 1995;38(8):848–52. https://doi.org/10.1007/BF02049842.

    Article  CAS  PubMed  Google Scholar 

  13. Murphy EM, Farquharson SM, Moran BJ. Management of an unexpected appendiceal neoplasm. Br J Surg. 2006;93(7):783–92. https://doi.org/10.1002/bjs.5385.

    Article  CAS  PubMed  Google Scholar 

  14. Smith JW, Kemeny N, Caldwell C, Banner P, Sigurdson E, Huvos A, Pseudomyxoma peritonei of appendiceal origin. The Memorial Sloan-Kettering Cancer Center experience. Cancer. 1992;70(2):396–401.

    Article  CAS  PubMed  Google Scholar 

  15. Didolkar MS, Fanous N. Adenocarcinoma of the appendix: a clinicopathologic study. Dis Colon Rectum. 1977;20(2):130–4. https://doi.org/10.1007/BF02587328.

    Article  CAS  PubMed  Google Scholar 

  16. Ussia A, Vaccari S, Gallo G, Grossi U, Ussia R, Sartarelli L, Minghetti M, Lauro A, Barbieri P, Di Saverio S, Cervellera M, Tonini V. Laparoscopic appendectomy as an index procedure for surgical trainees: clinical outcomes and learning curve. Updates Surg. 2021;73(1):187–95. https://doi.org/10.1007/s13304-020-00950-z.

    Article  PubMed  Google Scholar 

  17. Guaitoli E, Gallo G, Cardone E, Conti L, Famularo S, Formisano G, Galli F, Giuliani G, Martino A, Pasculli A, Patini R, Soriero D, Pappalardo V, Casoni Pattacini G, Sparavigna M, Meniconi R, Mazzari A, Barra F, Orsenigo E, Pertile D. Consensus Statement of the Italian Polispecialistic Society of Young Surgeons (SPIGC): diagnosis and treatment of acute appendicitis. J Investig Surg. 2021;34(10):1089–103. https://doi.org/10.1080/08941939.2020.1740360.

    Article  Google Scholar 

  18. Di Saverio S, Podda M, De Simone B, Ceresoli M, Augustin G, Gori A, Boermeester M, Sartelli M, Coccolini F, Tarasconi A, De' Angelis N, Weber DG, Tolonen M, Birindelli A, Biffl W, Moore EE, Kelly M, Soreide K, Kashuk J, Ten Broek R, Gomes CA, Sugrue M, Davies RJ, Damaskos D, Leppäniemi A, Kirkpatrick A, Peitzman AB, Fraga GP, Maier RV, Coimbra R, Chiarugi M, Sganga G, Pisanu A, De' Angelis GL, Tan E, Van Goor H, Pata F, Di Carlo I, Chiara O, Litvin A, Campanile FC, Sakakushev B, Tomadze G, Demetrashvili Z, Latifi R, Abu-Zidan F, Romeo O, Segovia-Lohse H, Baiocchi G, Costa D, Rizoli S, Balogh ZJ, Bendinelli C, Scalea T, Ivatury R, Velmahos G, Andersson R, Kluger Y, Ansaloni L, Catena F. Diagnosis and treatment of acute appendicitis: 2020 update of the WSES Jerusalem guidelines. World J Emerg Surg. 2020;15(1):27. https://doi.org/10.1186/s13017-020-00306-3.

    Article  PubMed  PubMed Central  Google Scholar 

  19. O’Donnell ME, Carson J, Garstin WI. Surgical treatment of malignant carcinoid tumors of the appendix. Int J Clin Pract. 2007;61:431–7.

    Article  PubMed  Google Scholar 

  20. Stark C, Jousi M, Enholm B. Preoperative assessment and treatment of appendiceal mucocele complicated by acute torsion: a case report. BMC Res Notes. 2014;7:1. https://doi.org/10.1186/1756-0500-7-1.

    Article  PubMed  PubMed Central  Google Scholar 

  21. Moertel CG, Weiland LH, Nagorney DM, Dockerty MB. Carcinoid tumor of the appendix: treatment and prognosis. N Engl J Med. 1987;317(27):1699–701. https://doi.org/10.1056/NEJM198712313172704.

    Article  CAS  PubMed  Google Scholar 

  22. Feldman JM, Jones RS. Carcinoid syndrome from gastrointestinal carcinoids without liver metastasis. Ann Surg. 1982;196(1):33–7. https://doi.org/10.1097/00000658-198207000-00008.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  23. Baskin LS, Stoller ML. Unusual appendiceal pathology presenting as urologic disease. Urology. 1991;38(5):432–6. https://doi.org/10.1016/0090-4295(91)80232-v.

    Article  CAS  PubMed  Google Scholar 

  24. Young RH, Gilks CB, Scully RE. Mucinous tumors of the appendix associated with mucinous tumors of the ovary and pseudomyxoma peritonei. A clinicopathological analysis of 22 cases supporting an origin in the appendix. Am J Surg Pathol. 1991;15(5):415–29. https://doi.org/10.1097/00000478-199105000-00001.

    Article  CAS  PubMed  Google Scholar 

  25. Gamble HA 2nd. Adenocarcinoma of the appendix: an unusual case and review. Dis Colon Rectum. 1976;19(7):621–5. https://doi.org/10.1007/BF02590980.

    Article  PubMed  Google Scholar 

  26. Van de Moortele M, De Hertogh G, Sagaert X, Van Cutsem E. Appendiceal cancer: a review of the literature. Acta Gastroenterol Belg. 2020;83(3):441–8.

    PubMed  Google Scholar 

  27. Glasgow SC, Gaertner W, Stewart D, Davids J, Alavi K, Paquette IM, Steele SR, Feingold DL. The American Society of Colon and Rectal Surgeons, clinical practice guidelines for the management of appendiceal neoplasms. Dis Colon Rectum. 2019;62(12):1425–38. https://doi.org/10.1097/DCR.0000000000001530.

    Article  PubMed  Google Scholar 

  28. Nagtegaal ID, Odze RD, Klimstra D, Paradis V, Rugge M, Schirmacher P, Washington KM, Carneiro F, Cree IA, WHO Classification of Tumors Editorial Board. The 2019 WHO classification of tumors of the digestive system. Histopathology. 2020;76(2):182–8. https://doi.org/10.1111/his.13975.

    Article  PubMed  Google Scholar 

  29. 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;37(4):1059–83. https://doi.org/10.1148/rg.2017160150.

    Article  PubMed  Google Scholar 

  30. Amin MB, Greene FL, Edge SB, Compton CC, Gershenwald JE, Brookland RK, Meyer L, Gress DM, Byrd DR, Winchester DP. The eighth edition AJCC cancer staging manual: continuing to build a bridge from a population-based to a more “personalized” approach to cancer staging. CA Cancer J Clin. 2017;67(2):93–9. https://doi.org/10.3322/caac.21388.

    Article  PubMed  Google Scholar 

  31. Carr NJ, Cecil TD, Mohamed F, Sobin LH, Sugarbaker PH, González-Moreno S, Taflampas P, Chapman S, Moran BJ. Peritoneal surface oncology group international. A consensus for classification and pathologic reporting of pseudomyxoma peritonei and associated appendiceal neoplasia: the results of the peritoneal surface oncology group international (PSOGI) modified Delphi process. Am J Surg Pathol. 2016;40(1):14–26. https://doi.org/10.1097/PAS.0000000000000535.

    Article  PubMed  Google Scholar 

  32. https://documents.cap.org/protocols/cp-appendix-net-17protocol-4001.pdf.

  33. Pape UF, Niederle B, Costa F, Gross D, Kelestimur F, Kianmanesh R, Knigge U, Öberg K, Pavel M, Perren A, Toumpanakis C, O'Connor J, Krenning E, Reed N, O'Toole D, Vienna Consensus Conference Participants. ENETS consensus guidelines for neuroendocrine neoplasms of the appendix (excluding goblet cell carcinomas). Neuroendocrinology. 2016;103(2):144–52. https://doi.org/10.1159/000443165.

    Article  CAS  PubMed  Google Scholar 

  34. Deshmukh S, Verde F, Johnson PT, Fishman EK, Macura KJ. Anatomical variants and pathologies of the vermix. Emerg Radiol. 2014;21(5):543–52. https://doi.org/10.1007/s10140-014-1206-4.

    Article  PubMed  PubMed Central  Google Scholar 

  35. Pickhardt PJ, Levy AD, Rohrmann CA Jr, Kende AI. Primary neoplasms of the appendix manifesting as acute appendicitis: CT findings with pathologic comparison. Radiology. 2002;224(3):775–81. https://doi.org/10.1148/radiol.2243011545.

    Article  PubMed  Google Scholar 

  36. Pickhardt PJ, Levy AD, Rohrmann CA Jr, Kende AI. Primary neoplasms of the appendix: radiologic spectrum of disease with pathologic correlation. Radiographics. 2003;23(3):645–62. https://doi.org/10.1148/rg.233025134.

    Article  PubMed  Google Scholar 

  37. Low RN, Barone RM, Gurney JM, Muller WD. Mucinous appendiceal neoplasms: preoperative MR staging and classification compared with surgical and histopathologic findings. AJR Am J Roentgenol. 2008;190(3):656–65. https://doi.org/10.2214/AJR.07.2018.

    Article  PubMed  Google Scholar 

  38. Misdraji J. Mucinous epithelial neoplasms of the appendix and pseudomyxoma peritonei. Mod Pathol. 2015;28(Suppl 1):S67–79. https://doi.org/10.1038/modpathol.2014.129.

    Article  PubMed  Google Scholar 

  39. Misdraji J, Young RH. Primary epithelial neoplasms and other epithelial lesions of the appendix (excluding carcinoid tumors). Semin Diagn Pathol. 2004;21(2):120–33. https://doi.org/10.1053/j.semdp.2004.11.005.

    Article  PubMed  Google Scholar 

  40. Low RN, Barone RM, Lucero J. Comparison of MRI and CT for predicting the peritoneal cancer index (PCI) preoperatively in patients being considered for cytoreductive surgical procedures. Ann Surg Oncol. 2015;22(5):1708–15. https://doi.org/10.1245/s10434-014-4041-7.

    Article  PubMed  Google Scholar 

  41. Tchana-Sato V, Detry O, Polus M, Thiry A, Detroz B, Maweja S, Hamoir E, Defechereux T, Coimbra C, De Roover A, Meurisse M, Honoré P. Carcinoid tumor of the appendix: a consecutive series from 1237 appendectomies. World J Gastroenterol. 2006;12(41):6699–701. https://doi.org/10.3748/wjg.v12.i41.6699.

    Article  PubMed  PubMed Central  Google Scholar 

  42. Bayhan Z, Yildiz YA, Akdeniz Y, Gonullu E, Altintoprak F, Mantoglu B, Capoglu R, Kahyaoglu AZ. Appendix neuroendocrine tumor: retrospective analysis of 4026 appendectomy patients in a single center. Emerg Med Int. 2020;2020:4030527. https://doi.org/10.1155/2020/4030527.

    Article  PubMed  PubMed Central  Google Scholar 

  43. Squires MH 3rd, Volkan Adsay N, Schuster DM, Russell MC, Cardona K, Delman KA, Winer JH, Altinel D, Sarmiento JM, El-Rayes B, Hawk N, Staley CA 3rd, Maithel SK, Kooby DA. Octreoscan versus FDG-PET for neuroendocrine tumor staging: a biological approach. Ann Surg Oncol. 2015;22(7):2295–301. https://doi.org/10.1245/s10434-015-4471-x.

    Article  PubMed  Google Scholar 

  44. van Eeden S, Offerhaus GJ, Hart AA, Boerrigter L, Nederlof PM, Porter E, van Velthuysen ML. Goblet cell carcinoid of the appendix: a specific type of carcinoma. Histopathology. 2007;51(6):763–73. https://doi.org/10.1111/j.1365-2559.2007.02883.x.

    Article  PubMed  Google Scholar 

  45. Lee KS, Tang LH, Shia J, Paty PB, Weiser MR, Guillem JG, Temple LK, Nash GM, Reidy D, Saltz L, Gollub MJ. Goblet cell carcinoid neoplasm of the appendix: clinical and CT features. Eur J Radiol. 2013;82(1):85–9. https://doi.org/10.1016/j.ejrad.2012.05.038.

    Article  CAS  PubMed  Google Scholar 

  46. Guo J, Wu G, Chen X, Li X. Primary appendiceal lymphoma presenting as suspected perforated acute appendicitis: clinical, sonography and CT findings with pathologic correlation. Int J Clin Exp Pathol. 2014;7(10):7068–71.

    PubMed  PubMed Central  Google Scholar 

  47. Pickhardt PJ, Levy AD, Rohrmann CA Jr, Abbondanzo SL, Kende AI. Non-Hodgkin’s lymphoma of the appendix: clinical and CT findings with pathologic correlation. AJR Am J Roentgenol. 2002;178(5):1123–7. https://doi.org/10.2214/ajr.178.5.1781123.

    Article  PubMed  Google Scholar 

  48. McCusker ME, Coté TR, Clegg LX, Sobin LH. Primary malignant neoplasms of the appendix: a population-based study from the surveillance, epidemiology and end-results program, 1973–1998. Cancer. 2002;94(12):3307–12. https://doi.org/10.1002/cncr.10589.

    Article  PubMed  Google Scholar 

  49. https://www.nccn.org/patients/guidelines/content/PDF/colon-patient.pdf.

  50. Bucher P, Gervaz P, Ris F, Oulhaci W, Egger JF, Morel P. Surgical treatment of appendiceal adenocarcinoid (goblet cell carcinoid). World J Surg. 2005;29(11):1436–9. https://doi.org/10.1007/s00268-005-7958-y.

    Article  PubMed  Google Scholar 

  51. Varisco B, McAlvin B, Dias J, Franga D. Adenocarcinoid of the appendix: is right hemicolectomy necessary? A meta-analysis of retrospective chart reviews. Am Surg. 2004;70(7):593–9.

    Article  PubMed  Google Scholar 

  52. Shaib W, Krishna K, Kim S, Goodman M, Rock J, Chen Z, Brutcher E, Staley CI, Maithel SK, Abdel-Missih S, El-Rayes BF, Bekaii-Saab T. Appendiceal neuroendocrine, goblet and signet-ring cell tumors: a spectrum of diseases with different patterns of presentation and outcome. Cancer Res Treat. 2016;48(2):596–604.

    Article  PubMed  Google Scholar 

  53. Gilmore G, Jensen K, Saligram S, Sachdev TP, Arekapudi SR. Goblet cell carcinoid of the appendix—diagnostic challenges and treatment updates: a case report and review of the literature. J Med Case Rep. 2018;12(1):275. https://doi.org/10.1186/s13256-018-1789-6.

    Article  PubMed  PubMed Central  Google Scholar 

  54. Shapiro R, Eldar S, Sadot E, Papa MZ, Zippel DB. Appendiceal carcinoid at a large tertiary center: pathologic findings and long-term follow-up evaluation. Am J Surg. 2011;201(6):805–8. https://doi.org/10.1016/j.amjsurg.2010.04.016.

    Article  PubMed  Google Scholar 

  55. Boxberger N, Redlich A, Böger C, Leuschner I, von Schweinitz D, Dralle H, Vorwerk P. Neuroendocrine tumors of the appendix in children and adolescents. Pediatr Blood Cancer. 2013;60(1):65–70. https://doi.org/10.1002/pbc.24267.

    Article  PubMed  Google Scholar 

  56. Murray SE, Lloyd RV, Sippel RS, Chen H, Oltmann SC. Postoperative surveillance of small appendiceal carcinoid tumors. Am J Surg. 2014;207(3):342–5. https://doi.org/10.1016/j.amjsurg.2013.08.038.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Unit of General Surgery and Surgical Oncology, Department of Medicine, Surgery and Neurosciences, University of Siena, Siena, Italy

    Gaetano Gallo & Franco Roviello

  2. Department of General Surgery, University Hospital Sant’Andrea, La Sapienza University of Rome, Rome, Italy

    Marta Goglia

  3. Department of Emergency and Organ Transplantation, University “Aldo Moro” of Bari, Bari, Italy

    Arcangelo Picciariello

  4. Department of Digestive and Emergency Surgery, Hospital Saint Mary of Terni, Nutrition Science Degree, University of Perugia, Terni, Italy

    Maria Chiara Ranucci

  5. University of Catania, Catania, Italy

    Elena Lamirata

  6. Department of Medicine, Universidad el Bosque, Bogota, Colombia

    Maria Paula Forero Rios

  7. Department of General Surgery, ASUR Marche, AV5, Hospital of San Benedetto del Tronto, San Benedetto del Tronto, Italy

    Salomone Di Saverio

Authors
  1. Gaetano Gallo
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Marta Goglia
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Arcangelo Picciariello
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Maria Chiara Ranucci
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Elena Lamirata
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Maria Paula Forero Rios
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Franco Roviello
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Salomone Di Saverio
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Salomone Di Saverio .

Editor information

Editors and Affiliations

  1. Emergency Surgery Dept., Parma University Hospital, Parma, Italy

    Antonio Tarasconi

  2. Oncology Dept., Parma University Hospital, Parma, Italy

    Simona Bui

  3. Digestive Surgery Dept, Ctr. Hosp. Univ. Grenoble Alpes, La Tronche, France

    Mircea Chirica

  4. Hepato-gastroenterology and Digestive oncology Dept., Grenoble-Alpes University Hospital, Grenoble, France

    Gaël Roth

  5. Department of Surgery, University of California, Irvine, Orange, CA, USA

    Jeffry Nahmias

Ethics declarations

This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

Conflicts of Interest

The authors declare no conflict of interest.

Informed Consent

Not required.

Rights and permissions

Reprints and permissions

Copyright information

© 2023 The Author(s), under exclusive license to Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Gallo, G. et al. (2023). Appendiceal Tumors. In: Tarasconi, A., Bui, S., Chirica, M., Roth, G., Nahmias, J. (eds) Oncologic Surgical Emergencies. Hot Topics in Acute Care Surgery and Trauma. Springer, Cham. https://doi.org/10.1007/978-3-031-36860-8_9

Download citation

  • DOI: https://doi.org/10.1007/978-3-031-36860-8_9

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-031-36859-2

  • Online ISBN: 978-3-031-36860-8

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation