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The INDEPSO-ISPSM Consensus on Peritoneal Malignancies-Mucinous Appendiceal Neoplasms and Pseudomyxoma Peritonei
Vivekanand Sharma1, Somashekhar Sp2, Rohit Kumar2
1Dept of Colorectal Surgery, East Suffolk and North Essex NHS Foundation, Ipswich, UK.
Abstract:
Mucinous appendiceal neoplasm (MANs) and pseudomyxoma peritonei (PMP) are very rare conditions with distinct therapeutic challenges. This consensus exercise was carried out to address clinically pertinent issues not addressed in existing international guidelines on the subject and reinforce some important recommendations from these guidelines. The modified Delphi method was employed with two rounds of voting. There were 42 questions on seven key issues (treatment approach and pre-operative work up; management of incidental MANs; management of high-volume PMP, the role of hyperthermic intraperitoneal chemotherapy (HIPEC); management of PMP with extraperitoneal disease; the role of systemic chemotherapy; follow-up after cytoreductive surgery (CRS), management of recurrence). A consensus was achieved if anyone option received > 70 votes (strong consensus > 90%). In round 1, 45/45 (100%) panelists voted; round 2, 39/45 (86.6%). A consensus was achieved on 36/42 (88.0%) questions (strong consensus-16.6%). In post-appendicectomy scenarios, CRS-HIPEC was recommended for patients with perforated (pT4) high grade appendiceal mucinous neoplasms (HAMN) with involved margins, pT4 poorly differentiated tumours, pT4 signet ring cell tumours in the absence of peritoneal disease and in all patients with overt peritoneal disease. No consensus was reached on post-appendicectomy management of pT3 HAMNs and intraoperatively detected MANs with peritoneal spread. HIPEC in addition to CRS was recommended for PMP. Some debatable interventions were recommended in selected patients like a total gastrectomy with a total colectomy, fertility-sparing surgery, two-stage CRS, and CRS in the presence of extraperitoneal disease. The panelists agreed with international recommendations on the management of established peritoneal disease but was more stringent in recommending prophylactic CRS and HIPEC in the post-appendicectomy scenarios.
Supplementary Information:
The online version contains supplementary material available at 10.1007/s13193-025-02265-0.
Insights
This consensus exercise provides recommendations for managing rare mucinous appendiceal neoplasms (MANs) and pseudomyxoma peritonei (PMP). Cytoreductive surgery with hyperthermic intraperitoneal chemotherapy (CRS-HIPEC) is recommended for specific advanced MANs and PMP cases.
Area of Science:
- Gastroenterology
- Surgical Oncology
- Oncology
Background:
- Mucinous appendiceal neoplasms (MANs) and pseudomyxoma peritonei (PMP) present unique and rare therapeutic challenges.
- Existing international guidelines may not fully address all clinical scenarios for these conditions.
Purpose of the Study:
- To establish expert consensus on clinically pertinent issues in MANs and PMP management.
- To reinforce and update recommendations from existing international guidelines.
Main Methods:
- A modified Delphi method involving two rounds of voting by 45 international panelists.
- 42 questions covering seven key issues in MANs and PMP management were addressed.
- Consensus was defined as >70% agreement, with strong consensus >90%.
Main Results:
- Consensus was achieved on 36 out of 42 questions (88%).
- CRS-HIPEC was recommended for perforated (pT4) high-grade MANs, pT4 poorly differentiated/signet ring cell tumors without peritoneal disease, and all overt peritoneal disease.
- HIPEC in addition to CRS was recommended for PMP; consensus was not reached for pT3 HAMNs or intraoperatively detected MANs with peritoneal spread.
Conclusions:
- Expert consensus supports CRS-HIPEC for specific advanced MANs and PMP, aligning with but also refining international guidelines.
- The consensus provides guidance on debatable interventions like fertility-sparing surgery and CRS for extraperitoneal disease.
- A more stringent approach to prophylactic CRS and HIPEC in post-appendicectomy scenarios was recommended.
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