Download PDF
Clin Colon Rectal Surg 2018; 31(05): 278-287
DOI: 10.1055/s-0038-1642051
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Appendiceal Neoplasms

Quinton M. Hatch
1   Division of Gastrointestinal and General Surgery, Department of Surgery, Oregon Health & Science University, Portland, Oregon
,
Erin W. Gilbert
1   Division of Gastrointestinal and General Surgery, Department of Surgery, Oregon Health & Science University, Portland, Oregon
› Author Affiliations
Further Information

Publication History

Publication Date:
04 September 2018 (online)

    Permissions and Reprints

Abstract

Appendiceal neoplasms are identified in 0.9 to 1.4% of appendiceal specimens, and the incidence is increasing. It has long been professed that neuroendocrine tumors (formerly carcinoids) are the most common neoplastic process of the appendix; recent data, however, has suggested a shift in epidemiology. Our intent is to distill the complex into an algorithm, and, in doing so, enable the surgeon to seamlessly maneuver through operative decisions, treatment strategies, and patient counseling. The algorithm for evaluation and treatment is complex, often starts from the nonspecific presenting complaint of appendicitis, and relies heavily on often subtle histopathologic differences.

Keywords

appendiceal neoplasms - gastrointestinal neuroendocrine tumors - appendiceal neuroendocrine tumors - pseudomyxoma peritonei - adenoneuroendocrine carcinoma
 

  • References

  • 1 Deaver JB. Appendicitis, 3rd ed. Philadelphia: P Blakiston's Son & Co.; 1905
    Google Scholar
  • 2 Williams GR. Presidential address: a history of appendicitis. With anecdotes illustrating its importance. Ann Surg 1983; 197 (05) 495-506
    CrossrefPubMedGoogle Scholar
  • 3 Cope Z. A History of the Acute Abdomen. London: Oxford University Press; 1965
    Google Scholar
  • 4 Major RH. Classic Descriptions of Disease, 3rd ed. Springfield: Charles C Thomas; 1945
    Google Scholar
  • 5 Shepherd JA. Acute appendicitis: a historical survey. Lancet 1954; 267 (6833): 299-302
    PubMedGoogle Scholar
  • 6 Fitz RH. Perforating inflammation of the appendix; with special reference to its early diagnosis and treatment. Am J Med Sci 1886; 92: 321-346
    PubMedGoogle Scholar
  • 7 McBurney C. Experience with early operative interference in cases of disease of the vermiform appendix. NY Med J 1889; 50: 676-684
    PubMedGoogle Scholar
  • 8 Elting AW. IX. Primary carcinoma of the vermiform appendix, with a report of three cases. Ann Surg 1903; 37 (04) 549-574
    PubMedGoogle Scholar
  • 9 Barrett ML, Hines AL, Andrews RM. Trends in rates of perforated appendix, 2001–2010: statistical brief #159. 2006. Available at: http:// www.ncbi.nlm.nih.gov/pubmed/24199256 . Accessed January 22, 2016
    PubMedGoogle Scholar
  • 10 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) 3307-3312
    CrossrefPubMedGoogle Scholar
  • 11 Spanos CP, Kaiser AM. Appendiceal neoplasms. In: Steele SR, Hull TL, Read TE, Saclarides TJ, Senagore AJ, Whitlow CB. , eds. The ASCRS Textbook of Colon and Rectal Surgery, 3rd ed. New York, NY: Springer; 2016: 617-629
    Google Scholar
  • 12 Marmor S, Portschy PR, Tuttle TM, Virnig BA. The rise in appendiceal cancer incidence: 2000-2009. J Gastrointest Surg 2015; 19 (04) 743-750
    CrossrefPubMedGoogle Scholar
  • 13 Whitfield CG, Amin SN, Garner JP. Surgical management of primary appendiceal malignancy. Colorectal Dis 2012; 14 (12) 1507-1511
    CrossrefPubMedGoogle Scholar
  • 14 Overman MJ, Fournier K, Hu CY. , et al. Improving the AJCC/TNM staging for adenocarcinomas of the appendix: the prognostic impact of histological grade. Ann Surg 2013; 257 (06) 1072-1078
    CrossrefPubMedGoogle Scholar
  • 15 McGory ML, Maggard MA, Kang H, O'Connell JB, Ko CY. Malignancies of the appendix: beyond case series reports. Dis Colon Rectum 2005; 48 (12) 2264-2271
    CrossrefPubMedGoogle Scholar
  • 16 Maggard MA, O'Connell JB, Ko CY. Updated population-based review of carcinoid tumors. Ann Surg 2004; 240 (01) 117-122
    CrossrefPubMedGoogle Scholar
  • 17 Shaib W, Krishna K, Kim S. , et al. Appendiceal neuroendocrine, goblet and signet-ring cell tumors: a spectrum of diseases with different patterns of presentation and outcome. Cancer Res Treat 2016; 48 (02) 596-604
    CrossrefPubMedGoogle Scholar
  • 18 Alexandraki KI, Kaltsas GA, Grozinsky-Glasberg S, Chatzellis E, Grossman AB. Appendiceal neuroendocrine neoplasms: diagnosis and management. Endocr Relat Cancer 2016; 23 (01) R27-R41
    CrossrefPubMedGoogle Scholar
  • 19 Yilmaz M, Akbulut S, Kutluturk K. , et al. Unusual histopathological findings in appendectomy specimens from patients with suspected acute appendicitis. World J Gastroenterol 2013; 19 (25) 4015-4022
    CrossrefPubMedGoogle Scholar
  • 20 Carr NJ, Sobin LH. Adenocarcinoma of the appendix. In: Bosman FT, Carneiro F, Hruban RH, Theise ND. , eds. WHO Classification of Tumors of the Digestive System (IARC WHO Classification of Tumours), 4th ed. Lyon: World Health Organization; 2010: 122-125
    Google Scholar
  • 21 Misdraji J. Tumors of the appendix. In: Shepherd NA, Warren BF, Williams GT, Greenson JK, Lauwers GY, Novelli MR. , eds. Morson's and Dawson's Gastrointestinal Pathology, 5th ed. London: Wiley; 2013: 490-498
    Google Scholar
  • 22 Ruoff C, Hanna L, Zhi W, Shahzad G, Gotlieb V, Saif MW. Cancers of the appendix: review of the literatures. ISRN Oncol 2011; 2011: 728579
    PubMedGoogle Scholar
  • 23 Sugarbaker PH. The natural history, gross pathology, and histopathology of appendiceal epithelial neoplasms. Eur J Surg Oncol 2006; 32 (06) 644-647
    CrossrefPubMedGoogle Scholar
  • 24 Sugarbaker PH. Epithelial appendiceal neoplasms. Cancer J 2009; 15 (03) 225-235
    CrossrefPubMedGoogle Scholar
  • 25 Weaver CH. Mucocele of appendix with pseudomucinous degeneration. Am J Surg 1937; 36: 347-351
    PubMedGoogle Scholar
  • 26 Werth R. Pseudomyxoma peritonei. Arch Gynecol 1884; 24: 100-118
    PubMedGoogle Scholar
  • 27 Ronnett BM, Zahn CM, Kurman RJ, Kass ME, Sugarbaker PH, Shmookler BM. Disseminated peritoneal adenomucinosis and peritoneal mucinous carcinomatosis. A clinicopathologic analysis of 109 cases with emphasis on distinguishing pathologic features, site of origin, prognosis, and relationship to “pseudomyxoma peritonei”. Am J Surg Pathol 1995; 19 (12) 1390-1408
    CrossrefPubMedGoogle Scholar
  • 28 Compton C, Fenoglio-Preiser CM, Pettigrew N, Fielding LP. American Joint Committee on Cancer Prognostic Factors Consensus Conference: Colorectal Working Group. Cancer 2000; 88 (07) 1739-1757
    CrossrefPubMedGoogle Scholar
  • 29 Pai RK, Longacre TA. Appendiceal mucinous tumors and pseudomyxoma peritonei: histologic features, diagnostic problems, and proposed classification. Adv Anat Pathol 2005; 12 (06) 291-311
    CrossrefPubMedGoogle Scholar
  • 30 O'Connell JT, Tomlinson JS, Roberts AA, McGonigle KF, Barsky SH. Pseudomyxoma peritonei is a disease of MUC2-expressing goblet cells. Am J Pathol 2002; 161 (02) 551-564
    CrossrefPubMedGoogle Scholar
  • 31 Jackson SL, Fleming RA, Loggie BW, Geisinger KR. Gelatinous ascites: a cytohistologic study of pseudomyxoma peritonei in 67 patients. Mod Pathol 2001; 14 (07) 664-671
    PubMedGoogle Scholar
  • 32 Lundqvist M, Wilander E. Subepithelial neuroendocrine cells and carcinoid tumours of the human small intestine and appendix. A comparative immunohistochemical study with regard to serotonin, neuron-specific enolase and S-100 protein reactivity. J Pathol 1986; 148 (02) 141-147
    CrossrefPubMedGoogle Scholar
  • 33 Shaw PA. The topographical and age distributions of neuroendocrine cells in the normal human appendix. J Pathol 1991; 164 (03) 235-239
    CrossrefPubMedGoogle Scholar
  • 34 Komminoth P, Arnold R, Capella C. , et al. Neuroendocrine neoplasms of the appendix. In: Bosman FT, Carneiro F, Hruban RH, Theise ND. , eds. WHO Classification of Tumors of the Digestive System, 4th ed. Lyon: World Health Organization; 2010
    Google Scholar
  • 35 Modlin IM, Kidd M, Latich I. , et al. Genetic differentiation of appendiceal tumor malignancy: a guide for the perplexed. Ann Surg 2006; 244 (01) 52-60
    CrossrefPubMedGoogle Scholar
  • 36 Pape UF, Perren A, Niederle B. , et al; Barcelona Consensus Conference participants. ENETS Consensus Guidelines for the management of patients with neuroendocrine neoplasms from the jejuno-ileum and the appendix including goblet cell carcinomas. Neuroendocrinology 2012; 95 (02) 135-156
    CrossrefPubMedGoogle Scholar
  • 37 Stewart RJ, Mirakhur M. Primary malignant lymphoma of the appendix. Ulster Med J 1986; 55 (02) 187-189
    PubMedGoogle Scholar
  • 38 Roy P, Chetty R. Goblet cell carcinoid tumors of the appendix: an overview. World J Gastrointest Oncol 2010; 2 (06) 251-258
    PubMedGoogle Scholar
  • 39 Pham TH, Wolff B, Abraham SC, Drelichman E. Surgical and chemotherapy treatment outcomes of goblet cell carcinoid: a tertiary cancer center experience. Ann Surg Oncol 2006; 13 (03) 370-376
    CrossrefPubMedGoogle Scholar
  • 40 Tang LH, Shia J, Soslow RA. , 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) 1429-1443
    CrossrefPubMedGoogle Scholar
  • 41 Reid MD, Basturk O, Shaib WL. , et al. Adenocarcinoma ex-goblet cell carcinoid (appendiceal-type crypt cell adenocarcinoma) is a morphologically distinct entity with highly aggressive behavior and frequent association with peritoneal/intra-abdominal dissemination: an analysis of 77 cases. Mod Pathol 2016; 29 (10) 1243-1253
    PubMedGoogle Scholar
  • 42 Trivedi AN, Levine EA, Mishra G. Adenocarcinoma of the appendix is rarely detected by colonoscopy. J Gastrointest Surg 2009; 13 (04) 668-675
    CrossrefPubMedGoogle Scholar
  • 43 Connor SJ, Hanna GB, Frizelle FA. Appendiceal tumors: retrospective clinicopathologic analysis of appendiceal tumors from 7,970 appendectomies. Dis Colon Rectum 1998; 41 (01) 75-80
    CrossrefPubMedGoogle Scholar
  • 44 Madwed D, Mindelzun R, Jeffrey Jr RB. Mucocele of the appendix: imaging findings. AJR Am J Roentgenol 1992; 159 (01) 69-72
    CrossrefPubMedGoogle Scholar
  • 45 Puvaneswary M, Proietto A. Mucocele of the appendix with magnetic resonance imaging findings. Australas Radiol 2006; 50 (01) 71-74
    CrossrefPubMedGoogle Scholar
  • 46 Mullen JT, Savarese DM. Carcinoid tumors of the appendix: a population-based study. J Surg Oncol 2011; 104 (01) 41-44
    CrossrefPubMedGoogle Scholar
  • 47 Kusamura S, Moran BJ, Sugarbaker PH. , et al; Peritoneal Surface Oncology Group International (PSOGI). Multicentre study of the learning curve and surgical performance of cytoreductive surgery with intraperitoneal chemotherapy for pseudomyxoma peritonei. Br J Surg 2014; 101 (13) 1758-1765
    CrossrefPubMedGoogle Scholar
  • 48 Esquivel J, Chua TC, Stojadinovic A. , et al. Accuracy and clinical relevance of computed tomography scan interpretation of peritoneal cancer index in colorectal cancer peritoneal carcinomatosis: a multi-institutional study. J Surg Oncol 2010; 102 (06) 565-570
    CrossrefPubMedGoogle Scholar
  • 49 Franko J, Shi Q, Goldman CD. , et al. Treatment of colorectal peritoneal carcinomatosis with systemic chemotherapy: a pooled analysis of north central cancer treatment group phase III trials N9741 and N9841. J Clin Oncol 2012; 30 (03) 263-267
    CrossrefPubMedGoogle Scholar
  • 50 Turaga KK, Pappas SG, Gamblin T. Importance of histologic subtype in the staging of appendiceal tumors. Ann Surg Oncol 2012; 19 (05) 1379-1385
    CrossrefPubMedGoogle Scholar
  • 51 Edge SB, Byrd DR, Compton CC. , et al. AJCC (American Joint Committee on Cancer) Cancer Staging Manual, 7th ed. New York: Springer; 2010: 133
    Google Scholar
  • 52 Sugarbaker PH. New standard of care for appendiceal epithelial neoplasms and pseudomyxoma peritonei syndrome?. Lancet Oncol 2006; 7 (01) 69-76
    CrossrefPubMedGoogle Scholar
  • 53 González-Moreno S, Sugarbaker PH. Right hemicolectomy does not confer a survival advantage in patients with mucinous carcinoma of the appendix and peritoneal seeding. Br J Surg 2004; 91 (03) 304-311
    CrossrefPubMedGoogle Scholar
  • 54 Verwaal VJ, van Ruth S, de Bree E. , et al. Randomized trial of cytoreduction and hyperthermic intraperitoneal chemotherapy versus systemic chemotherapy and palliative surgery in patients with peritoneal carcinomatosis of colorectal cancer. J Clin Oncol 2003; 21 (20) 3737-3743
    CrossrefPubMedGoogle Scholar
  • 55 Stinner B, Kisker O, Zielke A, Rothmund M. Surgical management for carcinoid tumors of small bowel, appendix, colon, and rectum. World J Surg 1996; 20 (02) 183-188
    CrossrefPubMedGoogle Scholar
  • 56 Anthony T, Simmang C, Hyman N. , et al; Standards Practice Task Force, The American Society of Colon and Rectal Surgeons. Practice parameters for the surveillance and follow-up of patients with colon and rectal cancer. Dis Colon Rectum 2004; 47 (06) 807-817
    CrossrefPubMedGoogle Scholar
  • 57 Lieberman DA, Rex DK, Winawer SJ, Giardiello FM, Johnson DA, Levin TR. Guidelines for colonoscopy surveillance after screening and polypectomy: a consensus update by the US Multi-Society Task Force on Colorectal Cancer. Gastroenterology 2012; 143 (03) 844-857
    CrossrefPubMedGoogle Scholar
  • 58 Boudreaux JP, Klimstra DS, Hassan MM. , et al; North American Neuroendocrine Tumor Society (NANETS). The NANETS consensus guideline for the diagnosis and management of neuroendocrine tumors: well-differentiated neuroendocrine tumors of the jejunum, ileum, appendix, and cecum. Pancreas 2010; 39 (06) 753-766
    CrossrefPubMedGoogle Scholar