The neoplastic appendix: a practical approach

      Abstract

      Neoplastic processes in the appendix are histologically similar to their colonic counterparts, and recognition of these neoplasms is relatively straightforward. However, reporting and tumour staging of the neoplastic appendix can be complex, given numerous proposed classification systems, the uncertainty surrounding extra-appendiceal spread, and the frequently low-grade histology. Prognostication and clinical decisions regarding treatment rely heavily upon the completeness of the pathology report. This review discusses the clinicopathologic features of the epithelial neoplasms of the appendix, including colonic-type (non-mucinous) adenomas and adenocarcinomas, mucinous neoplasms, classical carcinoid tumours (well-differentiated neuroendocrine tumours), and goblet cell carcinoids with a focus on prognosis, terminology and pertinent points to include in the pathology report.

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      References

        • Carr N.J.
        • Sobin L.H.
        Epithelial noncarcinoid tumors and tumor-like lesions of the appendix.
        Cancer. 1995; 76: 2383-2384
        • Carr N.J.
        • Emory T.S.
        • Sobin L.H.
        Epithelial neoplasms of the appendix and colorectum: an analysis of cell proliferation, apoptosis, and expression of p53, CD44, bcl-2.
        Arch Pathol Lab Med. 2002; 126: 837-841
      1. Edge S. Byrd D. Compton C. AJCC Cancer Staging Manual. Springer, New York2010
      2. Sobin L. Gospodarowicz M. Wittekind C. International Union against Cancer TNM Classification of Malignant Tumours. Wiley-Blackwell, Lyon2009
      3. Bosman F. Carneiro F. Hruban R. WHO Classification of Tumours of the Digestive System. IARC, Lyon2010
        • Bradley R.F.
        • Stewart J.H.T.
        • Russell G.B.
        • et al.
        Pseudomyxoma peritonei of appendiceal origin: a clinicopathologic analysis of 101 patients uniformly treated at a single institution, with literature review.
        Am J Surg Pathol. 2006; 30: 551-559
        • Gibbs N.M.
        Mucinous cystadenoma and cystadenocarcinoma of the vermiform appendix with particular reference to mucocele and pseudomyxoma peritonei.
        J Clin Pathol. 1973; 26: 413-421
        • Higa E.
        • Rosai J.
        • Pizzimbono C.A.
        • et al.
        Mucosal hyperplasia, mucinous cystadenoma, and mucinous cystadenocarcinoma of the appendix. A re-evaluation of appendiceal “mucocele”.
        Cancer. 1973; 32: 1525-1541
        • Melcher D.H.
        • Rayan A.S.
        Columnar-cell (non-carcinoid) tumours of the appendix.
        Br J Surg. 1968; 55: 693-696
        • Misdraji J.
        • Yantiss R.K.
        • Graeme-Cook F.M.
        • et al.
        Appendiceal mucinous neoplasms: a clinicopathologic analysis of 107 cases.
        Am J Surg Pathol. 2003; 27: 1089-1103
        • Pai R.K.
        • Longacre T.A.
        Appendiceal mucinous tumors and pseudomyxoma peritonei: histologic features, diagnostic problems, and proposed classification.
        Adv Anat Pathol. 2005; 12: 291-311
        • Smeenk R.M.
        • van Velthuysen M.L.
        • Verwaal V.J.
        • et al.
        Appendiceal neoplasms and pseudomyxoma peritonei: a population based study.
        Eur J Surg Oncol. 2008; 34: 196-201
        • Yantiss R.K.
        • Shia J.
        • Klimstra D.S.
        • et al.
        Prognostic significance of localized extra-appendiceal mucin deposition in appendiceal mucinous neoplasms.
        Am J Surg Pathol. 2009; 33: 248-255
        • Ronnett B.M.
        • Zahn C.M.
        • Kurman R.J.
        • et al.
        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: 1390-1408
        • Pai R.K.
        • Beck A.H.
        • Norton J.A.
        • et al.
        Appendiceal mucinous neoplasms: clinicopathologic study of 116 cases with analysis of factors predicting recurrence.
        Am J Surg Pathol. 2009; 33: 1425-1439
        • Gonzalez-Moreno S.
        • Sugarbaker P.H.
        Right hemicolectomy does not confer a survival advantage in patients with mucinous carcinoma of the appendix and peritoneal seeding.
        Br J Surg. 2004; 91: 304-311
        • Sugarbaker P.H.
        Cytoreductive surgery and peri-operative intraperitoneal chemotherapy as a curative approach to pseudomyxoma peritonei syndrome.
        Eur J Surg Oncol. 2001; 27: 239-243
        • Modlin I.M.
        • Lye K.D.
        • Kidd M.
        A 5-decade analysis of 13,715 carcinoid tumors.
        Cancer. 2003; 97: 934-959
        • Modlin I.M.
        • Sandor A.
        An analysis of 8305 cases of carcinoid tumors.
        Cancer. 1997; 79: 813-829
        • Plockinger U.
        • Couvelard A.
        • Falconi M.
        • et al.
        Consensus guidelines for the management of patients with digestive neuroendocrine tumours: well-differentiated tumour/carcinoma of the appendix and goblet cell carcinoma.
        Neuroendocrinology. 2008; 87: 20-30
        • Hemminki K.
        • Li X.
        Incidence trends and risk factors of carcinoid tumors: a nationwide epidemiologic study from Sweden.
        Cancer. 2001; 92: 2204-2210
        • Godwin 2nd, J.D.
        Carcinoid tumors. An analysis of 2,837 cases.
        Cancer. 1975; 36: 560-569
        • Caplin M.E.
        • Buscombe J.R.
        • Hilson A.J.
        • et al.
        Carcinoid tumour.
        Lancet. 1998; 352: 799-805
        • Kulke M.H.
        • Mayer R.J.
        Carcinoid tumors.
        N Engl J Med. 1999; 340: 858-868
        • Maggard M.A.
        • O’Connell J.B.
        • Ko C.Y.
        Updated population-based review of carcinoid tumors.
        Ann Surg. 2004; 240: 117-122
        • O’Donnell M.E.
        • Carson J.
        • Garstin W.I.
        Surgical treatment of malignant carcinoid tumours of the appendix.
        Int J Clin Pract. 2007; 61: 431-437
        • Stinner B.
        • Rothmund M.
        Neuroendocrine tumours (carcinoids) of the appendix.
        Best Pract Res Clin Gastroenterol. 2005; 19: 729-738
        • Deans G.T.
        • Spence R.A.
        Neoplastic lesions of the appendix.
        Br J Surg. 1995; 82: 299-306
        • Gustafsson B.I.
        • Siddique L.
        • Chan A.
        • et al.
        Uncommon cancers of the small intestine, appendix and colon: an analysis of SEER 1973–2004, and current diagnosis and therapy.
        Int J Oncol. 2008; 33: 1121-1131
        • Burke A.P.
        • Sobin L.H.
        • Federspiel B.H.
        • et al.
        Goblet cell carcinoids and related tumors of the vermiform appendix.
        Am J Clin Pathol. 1990; 94: 27-35
        • Tang L.H.
        • Shia J.
        • Soslow R.A.
        • et al.
        Pathologic classification and clinical behavior of the spectrum of goblet cell carcinoid tumors of the appendix.
        Am J Surg Pathol. 2008; 32: 1429-1443