Pseudomyxoma peritonei in a pediatric patient: A case report and literature review

Rev Assoc Med Bras (1992). 2018 Feb;64(2):195-199. doi: 10.1590/1806-9282.64.02.195.

Abstract

Introduction: Pseudomyxoma peritonei (PMP) is a rare clinical condition, with an incidence of 1-2 cases per million, characterized by the dissemination of mucinous implants on the peritoneal surface and progressive gelatinous ascites. Although it usually presents an indolent behavior, its non-specific clinical presentation contributes to many cases remaining undiagnosed until a laparotomy is performed. With late diagnosis, performed after a long period of clinical deterioration and disease progression, it is common to find complications such as the formation of intestinal fistulas and obstruction.

Method: Review of the medical record and search for references in the Medline, Lilacs, SciELO and MD Consult databases.

Results: There are rare case reports found in the literature demonstrating atypical PMP presentations. Our report is that of a 17-year-old adolescent with a sporadic tumor diagnosed in a primary site in the transverse colon, contrary to data commonly found in the literature that mention a more frequent occurrence in women in the fifth decade of life and with a primary site in the ovary and appendix. The development of mucinous adenocarcinoma is rare in the pediatric population, and topography in the transverse colon and non-familial sporadic pattern are unusual.

Conclusion: The case reported not only raises awareness about the atypical presentations of the disease, but also emphasizes the use of imaging examinations for diagnosis, which has an important impact on prognosis and survival if performed timely.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adenocarcinoma, Mucinous / pathology
  • Adolescent
  • Colon, Transverse / pathology
  • Fatal Outcome
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Peritoneal Neoplasms / diagnostic imaging*
  • Peritoneal Neoplasms / pathology
  • Peritoneal Neoplasms / therapy
  • Pseudomyxoma Peritonei / diagnostic imaging*
  • Pseudomyxoma Peritonei / pathology
  • Pseudomyxoma Peritonei / therapy
  • Tomography