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Peer-reviewedCC BY-NCAnnals of coloproctology

Clinicopathologic characteristics and molecular landscape for pseudomyxoma peritonei of appendiceal origin.

  1. 1Graduate School of Medical Science and Engineering, Korea Advanced Institute of Science and Technology (KAIST), Daejeon, Korea
  2. 2Department of Pathology, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Korea
  3. 3Division of Colon and Rectal Surgery, Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea

Abstract

Pseudomyxoma peritonei (PMP) of appendiceal origin presents a unique challenge in oncology owing to its rarity, varied progression, and complex molecular characteristics. The current standard treatment using cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy has shown some success in extending survival rates; however, it is associated with significant physical burdens and high recurrence rates. Advances in understanding the molecular and genetic features, particularly mutations in genes such as KRAS, GNAS, and BRAF, have led to a promising shift toward more targeted and less invasive treatment options, potentially moving beyond the limitations of current therapies. Additionally, novel approaches targeting the tumor microenvironment in PMP have gained traction, given the role of factors such as mucin accumulation, hypoxia, and inflammatory cytokines in sustaining tumor persistence and resistance. Emerging personalized therapies and immunotherapies, particularly targeted treatments and peptide vaccine development for GNAS-mutated cancers, offer the potential to enhance treatment specificity and effectiveness. The challenges of multiomics approaches to capture the complex genomic and epigenomic landscape of PMP highlight the need for individualized optimal treatment in patients with PMP.

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BibTeX
@article{yoon2026clinicopathologic,
  title = {{Clinicopathologic characteristics and molecular landscape for pseudomyxoma peritonei of appendiceal origin.}},
  author = {Taehan Yoon and Su-Jin Shin and Ji Min Lee and Eun Jung Park},
  journal = {Annals of coloproctology},
  year = {2026},
  month = oct,
  doi = {10.3393/ac.2026.00031.0004},
  url = {https://europepmc.org/article/MED/42958777},
  note = {CC BY-NC},
}
RIS
TY  - JOUR
TI  - Clinicopathologic characteristics and molecular landscape for pseudomyxoma peritonei of appendiceal origin.
AU  - Taehan Yoon
AU  - Su-Jin Shin
AU  - Ji Min Lee
AU  - Eun Jung Park
PY  - 2026
DA  - 2026/10/15
JO  - Annals of coloproctology
DO  - 10.3393/ac.2026.00031.0004
UR  - https://europepmc.org/article/MED/42958777
AB  - Pseudomyxoma peritonei (PMP) of appendiceal origin presents a unique challenge in oncology owing to its rarity, varied progression, and complex molecular characteristics. The current standard treatment using cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy has shown some success in extending survival rates; however, it is associated with significant physical burdens and high recurrence rates. Advances in understanding the molecular and genetic features, particularly mutations in genes such as KRAS, GNAS, and BRAF, have led to a promising shift toward more targeted and less invasive treatment options, potentially moving beyond the limitations of current therapies. Additionally, novel approaches targeting the tumor microenvironment in PMP have gained traction, given the role of factors such as mucin accumulation, hypoxia, and inflammatory cytokines in sustaining tumor persistence and resistance. Emerging personalized therapies and immunotherapies, particularly targeted treatments and peptide vaccine development for GNAS-mutated cancers, offer the potential to enhance treatment specificity and effectiveness. The challenges of multiomics approaches to capture the complex genomic and epigenomic landscape of PMP highlight the need for individualized optimal treatment in patients with PMP.
SN  - 2287-9714
SN  - 2287-9722
ER  -