Background <p>Appendiceal tumors comprise a heterogeneous group of tumors that may be localized or disseminate throughout the peritoneum. Limited high quality clinical data exist and many practices have been extrapolated from colorectal cancer without validation in appendiceal cohorts. There are many controversies regarding the treatment&#xa0;of appendiceal tumors, and practices vary widely between centers and care settings. A national consensus update of best management practices for appendiceal malignancies was performed to better standardize care.</p> Methods <p>The 2018 Chicago Consensus guideline was updated through a modified Delphi consensus, performed over two rounds using nationally circulated surveys. Supporting evidence was evaluated using rapid systematic reviews. Key systemic therapy concepts were summarized by content experts.</p> Results <p>Most supporting literature consists of observational studies, but high-quality studies increasingly&#xa0;are becoming available to drive management. Two consensus-based pathways were generated for localized appendiceal tumors, one for epithelial mucinous neoplasms and another for appendiceal adenocarcinoma. Of 138 participants responding in the first round, 133 (96%) engaged in the second round. Greater than 90% consensus was achieved for all pathway blocks. Key points include minimizing intervention invasiveness where permitted by pathologic classification and margin status, and determining which margin and pathologic findings are indications for consideration of cytoreduction with or without intraperitoneal chemotherapy. Surveillance and systemic therapy recommendations are also presented.</p> Conclusion <p>With growing but still primarily observational evidence currently dictating care, these consensus recommendations provide expert guidance in the treatment of appendiceal tumors without peritoneal involvement.</p>

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Consensus Guideline for the Management of Patients with Appendiceal Tumors, Part 1: Appendiceal Tumors Without Peritoneal Involvement

  • 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,
  • James Cusack,
  • Joshua H. Winer,
  • Craig G. Gunderson,
  • Joseph Misdraji,
  • Rupen Shah,
  • Deepa R. Magge,
  • Ian Solsky,
  • Cathy Eng,
  • Oliver S. Eng,
  • Ardaman Shergill,
  • John Paul Shen,
  • Michael B. Foote,
  • 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 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 Schultz,
  • Laura Lambert,
  • Lauren Raymond,
  • Lauren Schleimer,
  • Lawrence Blaszkowsky,
  • Leanne Brown,
  • Leonard Saltz,
  • Leopoldo Fernandez,
  • Lindy Davis,
  • Lloyd Mack,
  • M. Talha 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,
  • Ryan Merkow,
  • Sabha Ganai,
  • Sam George Pappas,
  • Samantha Ruff,
  • Sameer Patel,
  • Samer Naffouje,
  • Samuel Butensky,
  • Sandy Tun,
  • Sanjay Bagaria,
  • 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 Vudatha,
  • Wenyi Luo,
  • Wilbur B. Bowne,
  • William Morano,
  • Xavier Keutgen,
  • Xiang Gao,
  • Yan Li

摘要

Background

Appendiceal tumors comprise a heterogeneous group of tumors that may be localized or disseminate throughout the peritoneum. Limited high quality clinical data exist and many practices have been extrapolated from colorectal cancer without validation in appendiceal cohorts. There are many controversies regarding the treatment of appendiceal tumors, and practices vary widely between centers and care settings. A national consensus update of best management practices for appendiceal malignancies was performed to better standardize care.

Methods

The 2018 Chicago Consensus guideline was updated through a modified Delphi consensus, performed over two rounds using nationally circulated surveys. Supporting evidence was evaluated using rapid systematic reviews. Key systemic therapy concepts were summarized by content experts.

Results

Most supporting literature consists of observational studies, but high-quality studies increasingly are becoming available to drive management. Two consensus-based pathways were generated for localized appendiceal tumors, one for epithelial mucinous neoplasms and another for appendiceal adenocarcinoma. Of 138 participants responding in the first round, 133 (96%) engaged in the second round. Greater than 90% consensus was achieved for all pathway blocks. Key points include minimizing intervention invasiveness where permitted by pathologic classification and margin status, and determining which margin and pathologic findings are indications for consideration of cytoreduction with or without intraperitoneal chemotherapy. Surveillance and systemic therapy recommendations are also presented.

Conclusion

With growing but still primarily observational evidence currently dictating care, these consensus recommendations provide expert guidance in the treatment of appendiceal tumors without peritoneal involvement.