Clin Colon Rectal Surg 2015; 28(04): 247-255
DOI: 10.1055/s-0035-1564433
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Management of Appendix Cancer

Kaitlyn J. Kelly
1   Department of Surgery, University of California San Diego, San Diego, California
› Institutsangaben
Weitere Informationen

Publikationsverlauf

Publikationsdatum:
22. November 2015 (online)

Abstract

Primary cancers of the appendix are rare and are frequently diagnosed after surgery for appendicitis, presumed ovarian primary malignancy, or other indications. Primary appendix cancers are histologically diverse, and classification of these tumors has historically been confusing because of the nonstandardized nomenclature that is used. This review aimed to describe the epidemiology, presentation, workup, staging, and management of primary appendix cancers using current, recommended nomenclature. For this purpose, tumors were broadly classified as colonic-type or mucinous adenocarcinoma, goblet cell adenocarcinoma, or neuroendocrine carcinoma. Signet ring cell carcinoma was not regarded as an individual entity. The presence of signet ring cells is a histologic feature that may or may not be present in colonic-type or mucinous adenocarcinoma. The management of primary appendix cancer is complex and is dependent on the histologic subtype and extent of disease. Randomized, prospective trials do not exist for these rare tumors and management is largely guided by retrospective data expert consensus guidelines, which are summarized here.

 
  • References

  • 1 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
  • 2 Nitecki SS, Wolff BG, Schlinkert R, Sarr MG. The natural history of surgically treated primary adenocarcinoma of the appendix. Ann Surg 1994; 219 (1) 51-57
  • 3 Alakus H, Babicky ML, Ghosh P , et al. Genome-wide mutational landscape of mucinous carcinomatosis peritonei of appendiceal origin. Genome Med 2014; 6 (5) 43
  • 4 Benedix F, Reimer A, Gastinger I, Mroczkowski P, Lippert H, Kube R ; Study Group Colon/Rectum Carcinoma Primary Tumor. Primary appendiceal carcinoma—epidemiology, surgery and survival: results of a German multi-center study. Eur J Surg Oncol 2010; 36 (8) 763-771
  • 5 Davison JM, Choudry HA, Pingpank JF , et al. Clinicopathologic and molecular analysis of disseminated appendiceal mucinous neoplasms: identification of factors predicting survival and proposed criteria for a three-tiered assessment of tumor grade. Mod Pathol 2014; 27 (11) 1521-1539
  • 6 Jacquet P, Sugarbaker PH. Clinical research methodologies in diagnosis and staging of patients with peritoneal carcinomatosis. Cancer Treat Res 1996; 82: 359-374
  • 7 Low RN, Barone RM. Combined diffusion-weighted and gadolinium-enhanced MRI can accurately predict the peritoneal cancer index preoperatively in patients being considered for cytoreductive surgical procedures. Ann Surg Oncol 2012; 19 (5) 1394-1401
  • 8 Turaga K, Levine E, Barone R , et al. Consensus guidelines from The American Society of Peritoneal Surface Malignancies on standardizing the delivery of hyperthermic intraperitoneal chemotherapy (HIPEC) in colorectal cancer patients in the United States. Ann Surg Oncol 2014; 21 (5) 1501-1505
  • 9 Kelly KJ, Nash GM. Peritoneal debulking/intraperitoneal chemotherapy-non-sarcoma. J Surg Oncol 2014; 109 (1) 14-22
  • 10 McConnell YJ, Mack LA, Gui X , et al. Cytoreductive surgery with hyperthermic intraperitoneal chemotherapy: an emerging treatment option for advanced goblet cell tumors of the appendix. Ann Surg Oncol 2014; 21 (6) 1975-1982
  • 11 Baratti D, Kusamura S, Nonaka D , et al. Pseudomyxoma peritonei: clinical pathological and biological prognostic factors in patients treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC). Ann Surg Oncol 2008; 15 (2) 526-534
  • 12 Chua TC, Moran BJ, Sugarbaker PH , et al. Early- and long-term outcome data of patients with pseudomyxoma peritonei from appendiceal origin treated by a strategy of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. J Clin Oncol 2012; 30 (20) 2449-2456
  • 13 Chua TC, Yan TD, Smigielski ME , et al. Long-term survival in patients with pseudomyxoma peritonei treated with cytoreductive surgery and perioperative intraperitoneal chemotherapy: 10 years of experience from a single institution. Ann Surg Oncol 2009; 16 (7) 1903-1911
  • 14 El Halabi H, Gushchin V, Francis J , et al. The role of cytoreductive surgery and heated intraperitoneal chemotherapy (CRS/HIPEC) in patients with high-grade appendiceal carcinoma and extensive peritoneal carcinomatosis. Ann Surg Oncol 2012; 19 (1) 110-114
  • 15 Blackham AU, Swett K, Eng C , et al. Perioperative systemic chemotherapy for appendiceal mucinous carcinoma peritonei treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. J Surg Oncol 2014; 109 (7) 740-745
  • 16 Turaga KK, Pappas SG, Gamblin T. Importance of histologic subtype in the staging of appendiceal tumors. Ann Surg Oncol 2012; 19 (5) 1379-1385
  • 17 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
  • 18 van Eeden S, Offerhaus GJ, Hart AA , et al. Goblet cell carcinoid of the appendix: a specific type of carcinoma. Histopathology 2007; 51 (6) 763-773
  • 19 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 (2) 135-156
  • 20 Jiang Y, Long H, Li T, Wang W, Liu H, Zhang X. Schistosomiasis may contribute to goblet cell carcinoid of the appendix. J Parasitol 2012; 98 (3) 565-568
  • 21 Jiang Y, Long H, Wang W, Liu H, Tang Y, Zhang X. Clinicopathological features and immunoexpression profiles of goblet cell carcinoid and typical carcinoid of the appendix. Pathol Oncol Res 2011; 17 (1) 127-132
  • 22 Yao JC, Hassan M, Phan A , et al. One hundred years after “carcinoid”: epidemiology of and prognostic factors for neuroendocrine tumors in 35,825 cases in the United States. J Clin Oncol 2008; 26 (18) 3063-3072
  • 23 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 (6) 753-766
  • 24 Murray SE, Lloyd RV, Sippel RS, Chen H, Oltmann SC. Postoperative surveillance of small appendiceal carcinoid tumors. Am J Surg 2014; 207 (3) 342-345 , discussion 345
  • 25 Fornaro R, Frascio M, Sticchi C , et al. Appendectomy or right hemicolectomy in the treatment of appendiceal carcinoid tumors?. Tumori 2007; 93 (6) 587-590
  • 26 Moertel CG, Weiland LH, Nagorney DM, Dockerty MB. Carcinoid tumor of the appendix: treatment and prognosis. N Engl J Med 1987; 317 (27) 1699-1701
  • 27 Arnold R, Rinke A, Klose KJ , et al. Octreotide versus octreotide plus interferon-alpha in endocrine gastroenteropancreatic tumors: a randomized trial. Clin Gastroenterol Hepatol 2005; 3 (8) 761-771