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Pediatric plexiform fibromyxoma: A PRISMA-compliant systematic literature review

Mitsuharu Fukazawa1, Hiroshi Koga1, Shoji Hiroshige2

  • 1Department of Pediatrics.

Medicine
|January 18, 2019
PubMed

Insights

Plexiform fibromyxoma (PF) is a rare gastric tumor. Pediatric PF cases show larger tumor size, but both pediatric and adult PFs share similar pathological and clinical behaviors.

Area of Science:

  • Gastroenterology
  • Pathology
  • Pediatric Oncology

Background:

  • Plexiform fibromyxoma (PF) is a rare gastric mesenchymal tumor, with limited reported cases.
  • Gastrointestinal stromal tumors exhibit distinct characteristics in adult versus pediatric patients.

Purpose of the Study:

  • To systematically review and compare the pathological and clinical features of pediatric plexiform fibromyxoma (PF) against adult PF.

Main Methods:

  • A comprehensive literature search of MEDLINE and EMBASE databases (1947-2018) was performed.
  • Data extraction from 41 English articles included immunohistochemistry, demographics, clinical presentation, tumor characteristics, and outcomes.
  • Two authors independently reviewed and extracted data for 80 confirmed PF patients.

Main Results:

  • Eighty plexiform fibromyxoma (PF) cases were analyzed (70 adult, 10 pediatric).
  • Tumors were predominantly located in the gastric antrum (78%) and frequently presented with ulceration (65%).
  • Pediatric PF cases exhibited significantly larger median tumor size (5.3 cm) compared to adult cases (4.0 cm).

Conclusions:

  • Plexiform fibromyxoma (PF) demonstrates similar pathological and benign clinical behavior across age groups.
  • Increased tumor growth is the primary distinguishing feature observed in pediatric PF.
  • PF is considered a single disease entity irrespective of patient age at onset.
Abstract

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