Mucinous Appendiceal Neoplasms: Incidence, Diagnosis and Surgical Treatment

Cir Esp. Jun-Jul 2017;95(6):321-327. doi: 10.1016/j.ciresp.2017.05.008. Epub 2017 Jun 24.
[Article in English, Spanish]

Abstract

Introduction: Mucinous tumors of the appendix are a rare pathology, with a prevalence below 0.5%. Clinical presentation usually occurs during the sixth decade of life, and mucinous tumors can clinically mimic acute appendicitis. The aim of this study is to describe the clinical and demographic variables, therapeutic procedure and diagnosis of these tumors. We analyze the association between mucinous tumors and pseudomyxoma peritonei (PP), as well as the association with colorectal and ovarian tumors.

Methods: A retrospective study was performed including patients who underwent an appendectomy between December 2003 and December 2014.

Results: Seventy-two mucinous tumors of the appendix were identified among 7.717 patients reviewed, resulting in a prevalence of 0.9%. Mean age at presentation was 64 years, 62% patients were female and 38% males. An incidental diagnosis was made in 43% of patients. Mucinous tumors of low malignant potential were significantly related to the presence of pseudomyxoma peritonei, identified in 16 (22%) of the cases. We also observed an increased risk of ovarian mucinous tumors in patients with a diagnosis of appendiceal mucinous neoplasm. In our sample, 22 (30.5%) patients showed a synchronous or metachronous colorectal cancer.

Conclusions: Appendiceal mucinous tumors are frequently an incidental finding. The diagnosis of mucinous tumors of low malignant potential is a factor associated with the development of pseudomyxoma peritonei. Histologic tumor grade and the presence of peritoneal dissemination will determine surgical treatment that can vary, from appendectomy to cytoreductive surgery.

Keywords: Appendiceal neoplasm; Mucinous neoplasm; Mucinous tumor; Neoplasia mucinosa maligna; Pseudomixoma peritoneal; Pseudoymoxa peritonei; Tumor de apéndice; Tumor mucinoso.

MeSH terms

  • Adenocarcinoma, Mucinous* / diagnosis
  • Adenocarcinoma, Mucinous* / epidemiology
  • Adenocarcinoma, Mucinous* / surgery
  • Adult
  • Aged
  • Appendectomy
  • Appendiceal Neoplasms* / diagnosis
  • Appendiceal Neoplasms* / epidemiology
  • Appendiceal Neoplasms* / surgery
  • Female
  • Humans
  • Incidence
  • Male
  • Middle Aged
  • Retrospective Studies