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Background <p>Appendiceal tumors comprise a heterogeneous group of tumors that frequently disseminate to the peritoneum. Management of appendiceal tumors is lacking high quality data given their rarity and heterogeneity. In general, appendiceal tumor treatment is extrapolated in part from colorectal cancer or pooled studies, without definitive evidence of disease-specific benefit. Many practices are controversial and vary widely between institutions. A national consensus update of best management practices for appendiceal malignancies was performed to better standardize care. Herein the authors present recommendations for the&#xa0;management of appendiceal tumors with peritoneal involvement.</p> Methods <p>As previously described, modified Delphi consensus was performed to update the previous 2018 Chicago consensus guideline. Recommendations were supported by&#xa0;using rapid systematic reviews of key issues in surgical and systemic therapy. Key pathology concepts and recommendations were synthesized in collaboration with content experts.</p> Results <p>A consensus-based pathway was generated for any type of non-neuroendocrine appendiceal tumor with peritoneal involvement. The first round of Delphi consensus included 138 participants, of whom 133 (96%) participated in the second round, and greater&#xa0;than 90% consensus&#xa0;was achieved for all pathway blocks. Key items include recommending evaluation for cytoreduction to most patients with low-grade peritoneal disease who are surgical candidates, and to many patients with high-grade disease, as well as timing of systemic chemotherapy and surveillance protocols. Common pitfalls in pathologic classification and their clinical implications are also presented.</p> Conclusion <p>These consensus recommendations provide guidance regarding the management of appendiceal tumors with peritoneal involvement, including a review of current evidence in the&#xa0;management of recurrent and unresectable disease.</p>

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Consensus Guideline for the Management of Patients with Appendiceal Tumors, Part 2: Appendiceal Tumors with Peritoneal Involvement

  • 期刊论文

  • 发表日期：

    2025年6月25日

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  • Elizabeth L. Godfrey,
  • Forest Mahoney,
  • Varun V. Bansal,
  • David G. Su,
  • David N. Hanna,
  • Felipe Lopez-Ramirez,
  • Ekaterina Baron,
  • Kiran K. Turaga,
  • Al B. Benson III,
  • Namrata Setia,
  • Joshua H. Winer,
  • Craig G. Gunderson,
  • Rupen Shah,
  • Deepa R. Magge,
  • Ian Solsky,
  • Cathy Eng,
  • Oliver S. Eng,
  • Ardaman Shergill,
  • John Paul Shen,
  • Joseph Misdraji,
  • Michael B. Foote,
  • Wenyi Luo,
  • Ahmed Hamed,
  • Adam Khader,
  • Alan Venook,
  • Alda Tam,
  • Alex Fisher,
  • Alex Kim,
  • Alexander Loftus,
  • Alexander Thomas,
  • Alexandra Gangi,
  • Alicia Greene,
  • Alissa Greenbaum,
  • Amanda Arrington,
  • Amer K. Abu Alfa,
  • Ana Gleisner,
  • Anai Kothari,
  • Anand Govindarajan,
  • Andreana N. Holowatyj,
  • Andrei Nikiforchin,
  • Andres Abreu,
  • Andrew Blakely,
  • Andrew Łowy,
  • Andrew M. Bellizzi,
  • Ankit Dhiman,
  • Anthony Villano,
  • Antoine Bouchard-Fortier,
  • Anwaar Saeed,
  • Armando Sardi,
  • Baylee Bakkila,
  • Beatrice Sun,
  • Benjamin Powers,
  • Beth Helmink,
  • Biren Reddy,
  • Blase Polite,
  • Bradley White,
  • Brian Sparkman,
  • Byrne Lee,
  • Carla Harmath,
  • Carlos Chan,
  • Carrie Luu,
  • Catalina Mosquera,
  • Charles J. Schneider,
  • Charles Staley,
  • Christopher Lieu,
  • Chukwuemeka Ihemelandu,
  • Colette Brottman,
  • Colette Pameijer,
  • Colin Court,
  • Conor O’Neill,
  • Dan Blazer,
  • Daniel Abbott,
  • Daniel Labow,
  • Daniel Milgrom,
  • Daniel Tuvin,
  • Darryl Outlaw,
  • Darryl Schuitevoerder,
  • David Bartlett,
  • David Ilson,
  • Devon Freudenberger,
  • Deyali Chatterjee,
  • Divya Sood,
  • Edward Levine,
  • Elizabeth Olecki,
  • Emily Papai,
  • Emmanuel Gabriel,
  • Eric Pletcher,
  • Erin P. Ward,
  • Fabian Johnston,
  • Fadi Dahdaleh,
  • Farin Amersi,
  • Filip Bednar,
  • Francisco Izquierdo,
  • Frederick A. Godley,
  • Garrett Nash,
  • Gary N. Mann,
  • George Salti,
  • Georgios Georgakis,
  • Georgios Karagkounis,
  • Gilbert Murimwa,
  • Giorgos Karakousis,
  • Haejin In,
  • Hallbera Gudmundsdottir,
  • Hanlin L. Wang,
  • Hanna Sanoff,
  • Harveshp Mogal,
  • Hatem Halabi,
  • Hunter Witmer,
  • Ioannis Konstantinidis,
  • Ira Leeds,
  • J. Randolph Hecht,
  • Jackson Baril,
  • Jacqueline Birkness-Gartman,
  • Jacquelyne J. Gaddy,
  • James C. Cusack Jr,
  • James W Fleshman,
  • Jane Wang,
  • Jason Foster,
  • Jason L. Hornick,
  • Javid Sadjadi,
  • Jeff Sussman,
  • Jeffrey M. Sutton,
  • Jennifer Keller,
  • Jeremy Sharib,
  • Jesus Esquivel,
  • Jig Deneve,
  • Joanna Buchheit,
  • Joel Baumgartner,
  • John Hart,
  • John Hays,
  • John Miura,
  • Jonathan B. Greer,
  • Jordan Cloyd,
  • Jorge L. Gomez,
  • Joseph Kim,
  • Jula Veerapong,
  • Jun Gong,
  • Kamran Idrees,
  • Katrina S. Pedersen,
  • Kay Washington,
  • Kevin Sullivan,
  • Kimberly Kopecky,
  • Konstantinos Chouliaras,
  • Kostas Votanopoulos,
  • Kristina A. Matkowskyj,
  • Kurt S. Schultz,
  • Laura Lambert,
  • Lauren Raymond,
  • Lauren Schleimer,
  • Lawrence Blaszkowsky,
  • Leanne M. Brown,
  • Leonard Saltz,
  • Leopoldo Fernandez,
  • Lindsay Alpert,
  • Lindy Davis,
  • Lloyd Mack,
  • MTalha Waheed,
  • Madeline B. Torres,
  • Maheswari Senthil,
  • Manju George,
  • Marcello Deraco,
  • Marcovalerio Melis,
  • Maria Russell,
  • Marie Robert,
  • Mary Garland-Kledzik,
  • Mason Vierra,
  • Masoumeh Ghayouri,
  • Mazin Al-Kasspooles,
  • Mecker Moller,
  • Mehraneh D. Jafari,
  • Melanie Ongchin,
  • Michael Drage,
  • Michael D. Kluger,
  • Michael Wach,
  • Michael White,
  • Mikaël Soucisse,
  • Mio Kitano,
  • Mohamed Adam,
  • Mohammad Haroon Asif Choudry,
  • Mohammed Suraju,
  • Mojgan Hosseini,
  • Monica Malec,
  • Mustafa Raoof,
  • Nabil Wasif,
  • Nadège Fackche,
  • Nader Hanna,
  • Namrata Vijayvergia,
  • Nancy Baxter,
  • Neal Bhutiani,
  • Nelya Melnitchouk,
  • Niraj J. Gusani,
  • Olivier Glehen,
  • Pathik Aravind,
  • Patricio Polanco,
  • Patrick Wagner,
  • Paul Helft,
  • Paul Sugarbaker,
  • Pompiliu Piso,
  • Prakash Pandalai,
  • Rachel Kim,
  • Rachyl Shanker,
  • Raed Al-Rajabi,
  • Raghav Sundar,
  • Rajiv Agarwal,
  • Randall Zuckerman,
  • Ravi Chokshi,
  • Rebecca Wiatrek,
  • Reetesh Pai,
  • Ricardo Bello,
  • Richard Alexander,
  • Richard Berri,
  • Richard Hoehn,
  • Rifat Mannan,
  • Riom Kwakman,
  • Rita Akumuo,
  • Robert Sticca,
  • Raul Gonzalez,
  • Ryan Merkow,
  • Sabha Ganai,
  • Sam George Pappas,
  • Samantha Ruff,
  • Sameer Patel,
  • Samer Naffouje,
  • Samuel D. Butensky,
  • Sandy Tun,
  • Sanjay Bagaria,
  • Sanjay Kakar,
  • Sara Daniel,
  • Sarah Wilkins,
  • Scott Sherman,
  • Sean Dineen,
  • Sepideh Gholami,
  • Seth Concors,
  • Shannon Altpeter,
  • Shannon Radomski,
  • Sherif Abdel Misih,
  • Stacey Cohen,
  • Stacey Stein,
  • Stephanie Greco,
  • Steven Ahrendt,
  • Steven Libutti,
  • Susan Campbell,
  • T. Clark Gamblin,
  • Taylor Jaraczewski,
  • Thinzar Lwin,
  • Thorvardur Halfdanarson,
  • Trang Nguyen,
  • Travis Grotz,
  • Ugwuji Maduekwe,
  • Umut Sarpel,
  • Uqba Khan,
  • Vadim Gushchin,
  • Van Morris,
  • Vignesh Vudhata,
  • Wenyi Luo,
  • Wilbur B. Bowne,
  • William Morano,
  • Xavier Keutgen,
  • Xiang Gao,
  • Yan Li,
  • Kiran Turaga

摘要

Background

Appendiceal tumors comprise a heterogeneous group of tumors that frequently disseminate to the peritoneum. Management of appendiceal tumors is lacking high quality data given their rarity and heterogeneity. In general, appendiceal tumor treatment is extrapolated in part from colorectal cancer or pooled studies, without definitive evidence of disease-specific benefit. Many practices are controversial and vary widely between institutions. A national consensus update of best management practices for appendiceal malignancies was performed to better standardize care. Herein the authors present recommendations for the management of appendiceal tumors with peritoneal involvement.

Methods

As previously described, modified Delphi consensus was performed to update the previous 2018 Chicago consensus guideline. Recommendations were supported by using rapid systematic reviews of key issues in surgical and systemic therapy. Key pathology concepts and recommendations were synthesized in collaboration with content experts.

Results

A consensus-based pathway was generated for any type of non-neuroendocrine appendiceal tumor with peritoneal involvement. The first round of Delphi consensus included 138 participants, of whom 133 (96%) participated in the second round, and greater than 90% consensus was achieved for all pathway blocks. Key items include recommending evaluation for cytoreduction to most patients with low-grade peritoneal disease who are surgical candidates, and to many patients with high-grade disease, as well as timing of systemic chemotherapy and surveillance protocols. Common pitfalls in pathologic classification and their clinical implications are also presented.

Conclusion

These consensus recommendations provide guidance regarding the management of appendiceal tumors with peritoneal involvement, including a review of current evidence in the management of recurrent and unresectable disease.