Skip to main content

Advertisement

Log in

Malignant neoplasms of the appendix

  • Original Article
  • Published:
International Journal of Colorectal Disease Aims and scope Submit manuscript

Abstract

Background

Appendiceal neoplasms, first described in 1882, are still rare, with pre-operative diagnosis invariably difficult. We present our 10-year experience of these lesions with a review of current epidemiology, pathology and treatment modalities.

Materials and methods

A retrospective histopathological review of all appendicectomy specimens was completed between April 1994 and December 2003 to identify patients diagnosed with malignant neoplasms. Patient demographics, operative details, histopathology and clinical outcomes were obtained from case notes. A literature search of the PubMed database was then performed using the medical search headings; appendix, tumour, neoplasm and malignancy.

Results

Twenty-two patients (eight men) were identified during the study period, with no age difference between gender (mean age in women 58, range 14–83 vs mean age in men 55, range 16–78). Eleven patients were found to have carcinoid-type tumours, eight patients with adenocarcinomas and three patients with lymphomas. Other appendiceal pathologies were identified after appendicectomies, hemicolectomy and oophorectomy. Mean follow-up was 41 months (range 1–125 months). Fourteen patients were alive at the end of follow-up. Patients with classical carcinoid tumours (CCT) had better outcomes than patients with the goblet cell carcinoid, adenocarcinoma and lymphoma.

Conclusions

From our own experience and a subsequent review of the literature, we recommend right hemicolectomy as the treatment of choice for all malignant appendiceal neoplasms, except for small CCT less than 2 cm in diameter at the tip of the appendix, with a low proliferative index, without angiolymphatic or mesoappendiceal extension. Further adjuvant therapy should be considered after oncological assessment.

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

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5

Similar content being viewed by others

References

  1. Adiss DG, Shaffer N, Fowler BS, Tauxe RV (1990) The epidemiology of appendicitis and appendectomy in the United States. Am J Epidemiol 132:910–925

    Google Scholar 

  2. Berger A (1882) Ein Fall von Krebs des Wurmfortsatzes. Ber Klin Wochenschr 19:610

    Google Scholar 

  3. Collins DC (1963) 71,000 human appendix specimens: a final report summarizing forty years’ study. Am J Proctol 14:365–381

    Google Scholar 

  4. Connor SJ, Hanna GB, Frizelle FA (1998) Appendiceal tumors: retrospective clinicopathologic analysis of appendiceal tumors from 7,970 appendectomies. Dis Colon Rectum 41:75–80

    Article  PubMed  CAS  Google Scholar 

  5. O’Donnell ME, Carson J, Garstin WIH (2007) Surgical treatment of malignant carcinoid tumours of the appendix. Int J Clin Pract 61(3):431–437

    Article  PubMed  CAS  Google Scholar 

  6. McGory ML, Maggard MA, Kang H, O’Connell JB, Ko CY (2005) Malignancies of the appendix: beyond case series reports. Dis Colon Rectum 48:2264–2271

    Article  PubMed  Google Scholar 

  7. Butler JA, Houshair A, Lin F, Wilson SE (1994) Goblet cell carcinoid of the appendix. Am J Surg 168:685–687

    Article  PubMed  CAS  Google Scholar 

  8. Cortina R, McCormick J, Kolm P, Perry RR (1995) Management and prognosis of adenocarcinoma of the appendix. Dis Colon Rectum 38:848–852

    Article  PubMed  CAS  Google Scholar 

  9. Deans GT, Spence RAJ (1995) Neoplastic lesions of the appendix. Br J Surg 82:229–306

    Article  Google Scholar 

  10. Nitecki SS, Wolff BG, Schlinkert R, Sarr MG (1994) The natural history of surgically treated primary adenocarcinoma of the appendix. Ann Surg 219:51–57

    Article  PubMed  CAS  Google Scholar 

  11. Lyss AP (1988) Appendiceal malignancies. Semin Oncol 15:129–137

    PubMed  CAS  Google Scholar 

  12. Sandor A, Modulin IM (1998) A retrospective analysis of 1570 appendiceal carcinoids. Am J Gastroenterol 93:422–428

    Article  PubMed  CAS  Google Scholar 

  13. Rutledge RH, Alexander JW (1992) Primary appendiceal malignancies. Surgery 111:244–250

    PubMed  CAS  Google Scholar 

  14. Hananel N, Powsner E, Wolloch Y (1998) Adenocarcinoma of the appendix: an unusual case. Eur J Surg 164:859–862

    Article  PubMed  CAS  Google Scholar 

  15. Arnold R, McCallion K, McGailie C (2006) Goblet cell carcinoids of the appendix. Ulster Med J 75: 40–45

    PubMed  CAS  Google Scholar 

  16. McCusker ME, Cote TR, Clegg LX, Sobin LH (2002) Primary malignant neoplasms of the appendix: a population-based study from the surveillance, epidemiology and end-results program, 1973–1998. Cancer 94:3307–3312

    Article  PubMed  Google Scholar 

  17. Hemminki K, Li X (2001) Incidence trends and risk factors of carcinoid tumors: a nationwide epidemiological study from Sweden. Cancer 92:2204–2210

    Article  PubMed  CAS  Google Scholar 

  18. Godwin JD II (1975) Carcinoid tumors. An analysis of 2837 cases. Cancer 36:560–569

    Article  PubMed  Google Scholar 

  19. Anderson JR, Wilson BG (1985) Carcinoid tumours of the appendix. Br J Surg 72:545–546

    Article  PubMed  CAS  Google Scholar 

  20. Moertel CL, Weiland LH, Telander RL (1990) Carcinoid tumor of the appendix in the first two decades of life. J Pediatr Surg 25:1073–1075

    Article  PubMed  CAS  Google Scholar 

  21. Modlin IM, Lye KD, Kidd M (2003) A 5-decade analysis of 13 715 carcinoid tumours. Cancer 97:934–959

    Article  PubMed  Google Scholar 

  22. Roggo A, Wood WC, Ottinger LW (1993) Carcinoid tumours of the appendix. Ann Surg 217:385–390

    Article  PubMed  CAS  Google Scholar 

  23. Hermans JJ, Hermans AL, Risseeuw GA, Verhaar JC, Meradji M (1993) Appendicitis caused by carcinoid tumour. Radiology 188:71–72

    PubMed  CAS  Google Scholar 

  24. Nwiloh JO, Pillarisetty S, Moscovic EA, Freeman HP (1990) Carcinoid tumours. J Surg Oncol 45(4):261–264

    Article  PubMed  CAS  Google Scholar 

  25. Markgraf WH, Dunn TM (1964) Appendiceal carcinoid with carcinoid syndrome. Am J Surg 107:730–732

    Article  PubMed  CAS  Google Scholar 

  26. Johnston WH, Waisman J (1971) Carcinoid tumour of the vermiform appendix with Cushing’s syndrome. Cancer 27:681–686

    Article  PubMed  CAS  Google Scholar 

  27. Kema IP, de Vries EG, Schellings AM, Postmus PE, Muskiet FA (1992) Improved diagnosis of carcinoid tumours by measurement of platelet serotonin. Clin Chem 38:534–540

    PubMed  CAS  Google Scholar 

  28. King CM, Reznek RH, Bomanji J, Ur E, Britton KE, Grossman AB, Besser GM (1993) Imaging neuroendocrine tumours with radiolabelled somatostatin analogues and X-ray computed tomography: a comparative study. Clin Radiol 48:386–391

    Article  PubMed  CAS  Google Scholar 

  29. Stivanello M, Berruti A, Torta M, Termine A, Tampellini M, Gorzegno G, Angeli A, Dogliotti L (2001) Circulating chromogranin A in the assessment of patients with neuroendocrine tumours. A single institutional experience. Ann Oncol 12 (Suppl 2):S73–S77

    Article  PubMed  Google Scholar 

  30. Janson ET, Holmberg L, Stridsberg M, Eriksson B, Theodorsson E, Wilander E, Oberg K (1997) Carcinoid tumors: analysis of prognostic factors and survival in 301 patients from a tertiary referral centre. Ann Oncol 8:685–690

    Article  PubMed  CAS  Google Scholar 

  31. Williams ED, Sandler M (1963) The classification of carcinoid tumours. Lancet 1:238–239

    Article  PubMed  CAS  Google Scholar 

  32. Soga J, Tazawa K (1971) Pathologic analysis of carcinoids. Histologic re-evaluation of 62 cases. Cancer 28:990–998

    Article  PubMed  CAS  Google Scholar 

  33. Capella C, Heitz PU, Hofler H, Solcia E, Kloppel G (1995) Revised classification of neuroendocrine tumours of the lung, pancreas and gut. Virchows Arch 425:547–560

    Article  PubMed  CAS  Google Scholar 

  34. Van Eeden S, Quaedvlieg PF, Taal BG, Offerhaus GJ, Lamers CB, Van Velthuysen ML (2002) Classification of low-grade neuroendocrine tumours of the midgut and unknown origin. Hum Pathol 33:1126–1132

    Article  PubMed  Google Scholar 

  35. Syracuse DC, Perzin KH, Price JB, Wiedel PD, Mesa-Tejada R (1979) Carcinoid tumours of the appendix. Mesoappendiceal extension and nodal metastases. Ann Surg 190:58–63

    Article  PubMed  CAS  Google Scholar 

  36. Moertel CG, Weiland LH, Nagorney DM, Dockerty MB (1987) Carcinoid tumour of the appendix: treatment and prognosis. N Engl J Med 317:1699–1701

    Article  PubMed  CAS  Google Scholar 

  37. Goede AC, Caplin ME, Winslet MC (2003) Carcinoid tumour of the appendix. Br J Surg 90:1317–1322

    Article  PubMed  CAS  Google Scholar 

  38. Jonsson T, Johannsson JH, Hallgrimsson JG (1989) Carcinoid tumours of the appendix in children younger than 16 years. A retrospective clinical and pathologic study. Acta Chir Scand 155:113–116

    PubMed  CAS  Google Scholar 

  39. Dhillon AP, Williams RA, Rode J (1992) Age, site and distribution of subepithelial neurosecretory cells in the appendix. Pathology 24:56–59

    PubMed  CAS  Google Scholar 

  40. Ridolfi R, Amaducci L, Derni S, Fabbri L, Innocenti MP, Vignuteilli P (1991) Chemotherapy with 5-fluoracil and streptozotocin in carcinoid tumours of gastrointestinal origin: experiences with 13 patients. J Chemother 3:328–331

    PubMed  CAS  Google Scholar 

  41. Arnold R, Neuhaus C, Benning R, Schwerk WB, Trautmann ME, Joseph K, Bruns C (1993) Somatostatin analog Sandostatin and inhibition of tumor growth in patients with metastatic endocrine gastroenteropancreatic tumors. World J Surg 72:511–519

    Article  Google Scholar 

  42. Buchanan KD (1993) Effects of Sandostatin on neuroendocrine tumours of the gastrointestinal system. Recent Results Cancer Res 129:45–55

    PubMed  CAS  Google Scholar 

  43. Debas HT, Gittes G (1993) Somatostatin analogue therapy in functioning neuroendocrine gut tumors. Digestion 54(Suppl 1):68–71

    Article  PubMed  Google Scholar 

  44. Saltz L, Trochanowski B, Buckley M, Heffernan B, Niedzwiecki D, Tao Y, Kelsen D (1993) Octreotide as an antineoplastic agent in the treatment of functional and nonfunctional neuroendocrine tumors. Cancer 72:244–248

    Article  PubMed  CAS  Google Scholar 

  45. Janson ET, Oberg K (1993) Long-term management of the carcinoid syndrome. Treatment with octreotide alone and in combination with α-interferon. Acta Oncol 72:244–248

    Google Scholar 

  46. Moertel CG, Johnson CM, McKusick MA, Martin JK Jr, Nagorney DM, Kvols LK, Rubin J, Kunselman S (1994) The management of patients with advanced carcinoid tumors and islet cell carcinomas. Ann Intern Med 120:302–309

    PubMed  CAS  Google Scholar 

  47. Gagne F, Fortin P, Dufour V, Delage C (1969) Tumeurs de l’appendice associant des carretteres histologiques de cardinoide et d’adenocarcinome. Ann Anat Pathol 14:393–406

    CAS  Google Scholar 

  48. Berardi RS, Lee SS, Chen HP (1988) Goblet cell carcinoids of the appendix. Surg Gynecol Obstet 167:81–86

    PubMed  CAS  Google Scholar 

  49. Anderson NH, Somerville JE, Johnston CF, Hayes DM, Buchanan KD, Sloan JM (1991) Appendiceal goblet cell carcinoids: a clinicopathological and immunohistochemical study. Histopathology 18:61–65

    Article  PubMed  CAS  Google Scholar 

  50. Kanthan R, Saxena A, Kanthan SC (2001) Goblet cell carcinoids of the appendix: immunophenotype and ultrastructural study. Arch Pathol Lab Med 125:386–390

    PubMed  CAS  Google Scholar 

  51. Edmonds P, Merino MJ, LiVolsi VA, Duray PH (1984) Adenocarcinoid (mucinous carcinoid) of the appendix. Gastroenterology 86:302–309

    PubMed  CAS  Google Scholar 

  52. Ramnani DM, Wistuba II, Behrens C, Gazdar AF, Sobin LH, Albores-Saavedra J (1999) K-ras and p53 mutations in the pathogenesis of classical and goblet cell carcinoids of the appendix. Cancer 86:14–21

    Article  PubMed  CAS  Google Scholar 

  53. Chen KTK (1990) Appendiceal adenocarcinoid with ovarian metastases. Gynecol Oncol 38:268–286

    Article  Google Scholar 

  54. Warkel RL, Cooper PH, Helwig EB (1978) Adenocarcinoid, a mucin-producing tumour of the appendix. Cancer 42:2781–2793

    Article  PubMed  CAS  Google Scholar 

  55. Klein E, Rosen M (1996) Bilateral Krukenberg tumours due to appendiceal mucinous carcinoid. Int J Gynecol Pathol 15:85–88

    Article  PubMed  CAS  Google Scholar 

  56. Collins DC (1955) A study of 50,000 specimens of the human vermiform appendix. Surg Gynecol Obstet 101:437–445

    PubMed  CAS  Google Scholar 

  57. Gilhome RW, Johnston DH, Clark J, Kyld J (1984) Primary adenocarcinoma of the vermiform appendix: report of a series of ten cases and review of the literature. Br J Surg 71:553–555

    Article  PubMed  CAS  Google Scholar 

  58. Burgess P, Done HJ (1989) Adenocarcinoma of the appendix. J R Soc Med 82:28–29

    PubMed  CAS  Google Scholar 

  59. Nielsen GP, Isaksson HJ, Finnbogason H, Gunnlaugsson GH (1991) Adenocarcinoma of the vermiform appendix. A population study. APMIS 99:653–656

    Article  PubMed  CAS  Google Scholar 

  60. Uihlein A, McDonald JR (1943) Primary carcinoma of the appendix resembling carcinoma of the colon. Surg Gynecol Obstet 76:711–714

    Google Scholar 

  61. Steinberg M, Cohn I (1967) Primary adenocarcinoma of the appendix. Surgery 61:644–660

    PubMed  CAS  Google Scholar 

  62. Wilson R (1962) Primary carcinoma of the appendix. Am J Surg 104:238–249

    Article  PubMed  CAS  Google Scholar 

  63. Gamble HA (1976) Adenocarcinoma of the appendix: an unusual case and review. Dis Colon Rectum 19:621–625

    Article  PubMed  Google Scholar 

  64. Hopkins BG, Tullis RH, Kristensen KAB (1973) Primary adenocarcinoma of the vermiform appendix: report of seven cases and review of the literature. Dis Colon Rectum 16:140–144

    Article  PubMed  CAS  Google Scholar 

  65. Higa E, Rosai J, Pizzimbono CA, Wise L (1973) Mucosal hyperplasia, mucinous cystadenoma and mucinous cystadenocarcinoma of the appendix. Cancer 32:1525–1541

    Article  PubMed  CAS  Google Scholar 

  66. McGregor JK, McGregor DD (1960) Adenocarcinoma of the appendix. Surgery 48:925–935

    Google Scholar 

  67. Sugarbaker PH, Kern K, Lack E (1987) Malignant pseudomyxoma peritonei of colonic origin. Dis Colon Rectum 30:772–779

    Article  PubMed  CAS  Google Scholar 

  68. Wolff M, Ahmed N (1976) Epithelial neoplasms of the vermiform appendix (exclusion of carcinoid). Cancer 37:2493–2510

    Article  PubMed  CAS  Google Scholar 

  69. Niceberg DM, Feldman S, Mandleberg A (1956) Adenocarcinoma of the vermiform appendix. A case presentation and review of the literature. Surgery 40:560–570

    PubMed  Google Scholar 

  70. Hata K, Tanaka N, Nomura Y, Wada I, Nagawa H (2002) Early appendiceal adenocarcinoma. A review of the literature with special reference to optimal surgical procedures. J Gastroenterol 37:210–214

    Article  PubMed  Google Scholar 

  71. Andersson A, Bergdahl L, Boquist L (1976) Primary carcinoma of the appendix. Ann Surg 183:53–57

    Article  PubMed  CAS  Google Scholar 

  72. Sieracki JC, Tesluk H (1956) Primary adenocarcinoma of the vermiform appendix. Cancer 9:997–1011

    Article  PubMed  CAS  Google Scholar 

  73. Sugarbaker PH (1991) Cytoreductive surgery and intraperitoneal chemotherapy with peritoneal spread of cytoadenocarcinoma. Eur J Surg Suppl 561:75–82

    PubMed  Google Scholar 

  74. Lewin KJ, Ranchod M, Dorfman RF (1978) Lymphomas of the gastrointestinal tract. Cancer 42:693–707

    Article  PubMed  CAS  Google Scholar 

  75. Contreary K, Nance FC, Becker VF (1980) Primary lymphoma of the gastrointestinal tract. Ann Surg 191:593–598

    Article  PubMed  CAS  Google Scholar 

  76. Carpender BW (1991) Lymphoma of the appendix. Gastrointest Radiol 16:256–258

    Article  Google Scholar 

  77. D’Amore F, Brincker H, Grønbæk K, Thorling K, Pedersen M, Jensen MK (1994) Non-Hodgkin’s lymphoma of the gastrointestinal tract: a population-based analysis of incidence, geographic distribution, clinicopathologic presentation features, and prognosis. Danish Lymphoma Study Group. J Clin Oncol 12:1673–1684

    PubMed  CAS  Google Scholar 

  78. Pascuale MD, Shabahang M, Bitterman P, Lack EE, Evans SRT (1994) Primary lymphoma of the appendix. Case report and review of the literature. Surg Oncol 3:243–248

    Article  Google Scholar 

  79. Kanavaros P (1998) Primary gastric peripheral T-cell malignant lymphoma with helper/inducer phenotype. First case report with a complete histological ultrastructural and immunohistochemical study. Cancer 61:1602–1610

    Article  Google Scholar 

  80. Moulbayed P (1987) T-cell lymphoma of the stomach: morphological and immunological studies characterizing two cases of T-cell lymphoma. Virchows Arch 411:523–529

    Article  Google Scholar 

  81. Kitamura Y, Ohta T, Terada T (2000) Primary T-cell non-Hodgkin’s malignant lymphoma of the appendix. Pathol Int 50:313–317

    Article  PubMed  CAS  Google Scholar 

  82. Mori M, Kusunoki T, Kikunoki M, Motoori T, Sugimachi K (1985) Primary malignant lymphoma of the appendix. Jpn J Surg 15:230–233

    Article  PubMed  CAS  Google Scholar 

  83. Sin IC, Ling ET, Prentice RSA (1980) Burkitt’s lymphoma of the appendix: report of two cases. Human Pathol 11:465–470

    Article  CAS  Google Scholar 

  84. Stewart RJ, Mirakhur M (1986) Primary malignant lymphoma of the appendix. Ulster Med J 55:187–189

    PubMed  CAS  Google Scholar 

  85. Freeman C, Berg JW, Cutler SJ (1972) Occurrence and prognosis of extranodal lymphomas. Cancer 29:252–260

    Article  PubMed  CAS  Google Scholar 

  86. Dawson IMP, Cornes JS, Morson BC (1961) Primary malignant lymphoid tumours of the intestinal tract. Report of 37 cases with a study of factors influencing prognosis. Br J Surg 49:80–89

    Article  PubMed  CAS  Google Scholar 

  87. Uncu H, Erdem E, Tüzüner A (1998) Primary malignant lymphoma of the appendix: case report and review of the literature. Acta Chir Hung 37(1–2):11–16

    PubMed  CAS  Google Scholar 

  88. Jones PA (1979) Leiomyosarcoma of the appendix: report of two cases. Dis Colon Rectum 22:175–178

    Article  PubMed  CAS  Google Scholar 

  89. Michalany J, Galindo W (1973) Classification of neuromas of the appendix. Beitr Pathol 150:213–228

    PubMed  CAS  Google Scholar 

  90. Zarabi M, LaBach JP (1982) Ganglioneuroma causing acute appendicitis. Hum Pathol 13:1143–1146

    PubMed  CAS  Google Scholar 

  91. Deziel DJ, Saclarides TJ, Marshall JS, Yaremko LM (1991) Appendiceal Kaposi’s sarcoma: a cause of right lower quadrant pain in the acquired immunodeficiency syndrome. Am J Gastroenterol 86:901–903

    PubMed  CAS  Google Scholar 

  92. Zebrowska G, Walsh NM (1991) Human immunodeficiency virus related Karposi’s sarcoma of the appendix and acute appendicitis. Report of a case and review of the literature. Arch Pathol Lab Med 115:1157–1160

    PubMed  CAS  Google Scholar 

  93. Johnston J, Helwig EB (1981) Granular cell tumours of the gastrointestinal tract and perianal region: a study of 74 cases. Dig Dis Sci 26:807–816

    Article  PubMed  CAS  Google Scholar 

  94. Bernhardt H, Young JM (1965) Mucocele and pseudomyxoma peritonei of appendiceal origin: clinicopathologic aspects. Am J Surg 109:235

    Article  PubMed  CAS  Google Scholar 

  95. Burney RE, Koss N, Goldberg IS (1974) Acute appendicitis secondary to metastatic carcinoma of the breast. Arch Surg 108:872

    PubMed  CAS  Google Scholar 

  96. Maddox PR (1990) Acute appendicitis secondary to metastatic carcinoma of the breast. Br J Clin Pract 44:376–378

    PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Mark E. O’Donnell.

Rights and permissions

Reprints and permissions

About this article

Cite this article

O’Donnell, M.E., Badger, S.A., Beattie, G.C. et al. Malignant neoplasms of the appendix. Int J Colorectal Dis 22, 1239–1248 (2007). https://doi.org/10.1007/s00384-007-0304-0

Download citation

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00384-007-0304-0

Keywords

Navigation