Unusual site of pseudomyxoma peritonei recurrence after cytoreductive surgery and hyperthermic intraperitoneal chemotherapy: a case report of intraluminal disease manifestation in the small bowel

Paulien Rauwerdink, Lodewijk A.A. Brosens, Karin K. van Diepen, Okan N. Ghedri, Onno Kranenburg, Djamila Boerma, Arjen J. Witkamp, Helma M.U. van Grevenstein*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

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Abstract

Background: Pseudomyxoma peritonei (PMP) is an uncommon clinical condition characterized by the presence of mucinous ascites, mainly induced by perforated appendiceal mucinous neoplasms (AMN). The peritoneal surface of the small bowel is usually spared from disease manifestation due to peristaltic movements. Mucinous tumours can disseminate as PMP on the entire peritoneum, but are rarely intraluminal. For the first time in literature, we report a case of intraluminal PMP involving the ileum. Case presentation: A 75-year-old male was treated for perforated AMN and disseminated PMP with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. During follow-up, the patient developed intraperitoneal recurrence together with intraluminal depositions in the ileum, both disease manifestations with identical KRAS and SMAD4 mutations. Hereafter, the patient was treated with palliative care. Conclusion: This case illustrates the variation in the biological and clinical behaviour of this rare disease. Clinicians should be aware of unusual tumour distribution patterns of PMP, including the presence of mucinous tumour within the small bowel.

Original languageEnglish
Article number147
Pages (from-to)1-7
JournalWorld Journal of Surgical Oncology
Volume20
Issue number1
DOIs
Publication statusPublished - 10 May 2022

Keywords

  • CRS-HIPEC
  • High-grade appendiceal mucinous neoplasm
  • Pseudomyxoma peritonei
  • Recurrence
  • SMAD4
  • Small bowel
  • Peritoneal Neoplasms/pathology
  • Pseudomyxoma Peritonei/pathology
  • Humans
  • Male
  • Hyperthermia, Induced/adverse effects
  • Appendiceal Neoplasms/pathology
  • Hyperthermic Intraperitoneal Chemotherapy
  • Cytoreduction Surgical Procedures/adverse effects
  • Aged
  • Retrospective Studies

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