tumorsMalignant tumoursMalignant tumours2224-50572587-6813Rosoncoweb10.18027/2224-5057-2024-14-3s1-4-13tumors-1341Research ArticleСтатьиКонсенсус по лечению больных псевдомиксомой брюшиныhttps://orcid.org/0000-0001-7457-3889КалининА. Е.

Алексей Евгеньевич Калинин 

115478 Москва, Каширское шоссе, 24

main2001@inbox.ruhttps://orcid.org/0000-0002-6151-5660ГущинВ.

21202 Балтимор

https://orcid.org/0000-0003-0077-9555ДоманскийА. А.

197758 Санкт-Петербург, Ленинградская улица, 68

https://orcid.org/0000-0001-7753-3081КузнецоваО. А.

115478 Москва, Каширское шоссе, 24

https://orcid.org/0000-0002-0884-561XШаленковВ. А.

115478 Москва, Каширское шоссе, 24

https://orcid.org/0000-0002-6040-407XАбудеевС. А.

115478 Москва, Каширское шоссе, 24

https://orcid.org/0000-0003-3852-3969КозловН. А.

115478 Москва, Каширское шоссе, 24

https://orcid.org/0009-0005-9043-4199КолышкинВ. А.

660133 Красноярск, 1-я Смоленская ул., 16

https://orcid.org/0000-0002-9827-5584ПриваловА. В.

454087 Челябинск, улица Блюхера, 42

https://orcid.org/0000-0002-3919-9067РыбаковЕ. Г.

123423 Москва, ул. Саляма Адиля, 2

https://orcid.org/0000-0001-8893-1894АбгарянМ. Г.

115478 Москва, Каширское шоссе, 24

https://orcid.org/0000-0001-9780-7916СушковО. И.

123423 Москва, ул. Саляма Адиля, 2

ФГБУ «Национальный медицинский исследовательский центр онкологии им. Н. Н. Блохина» Минздрава РоссииRussian FederationИнститут по лечению рака при Медицинском центре MercyUnited StatesФГБУ «Национальный медицинский исследовательский центр онкологии им. Н. Н. Петрова» Минздрава РоссииRussian FederationКГБУЗ «Красноярский краевой онкологический диспансер им. А. И. Крыжановского»Russian FederationГАУЗ «Челябинский областной клинический центр онкологии и ядерной медицины»Russian FederationФГБУ «Национальный медицинский исследовательский центр колопроктологии имени А. Н. Рыжих» Минздрава РоссииRussian Federation202418112024143s1413Copyright © Калинин А.Е., Гущин В., Доманский А.А., Кузнецова О.А., Шаленков В.А., Абудеев С.А., Козлов Н.А., Колышкин В.А., Привалов А.В., Рыбаков Е.Г., Абгарян М.Г., Сушков О.И., 20242024Калинин А.Е., Гущин В., Доманский А.А., Кузнецова О.А., Шаленков В.А., Абудеев С.А., Козлов Н.А., Колышкин В.А., Привалов А.В., Рыбаков Е.Г., Абгарян М.Г., Сушков О.И.Калинин А.Е., Гущин В., Доманский А.А., Кузнецова О.А., Шаленков В.А., Абудеев С.А., Козлов Н.А., Колышкин В.А., Привалов А.В., Рыбаков Е.Г., Абгарян М.Г., Сушков О.И.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.malignanttumors.org/jour/article/view/1341Введение

Введение: Лечение больных псевдомиксомой брюшины — одна из сложных и нерешенных проблем в онкологии. В отечественной медицинской литературе тема псевдомиксомы брюшины представлена единичными разрозненными публикациями. В основных зарубежных национальных клинических руководствах данная тема также не освещена. Основными документами, определяющими подходы к лечению псевдомиксомы брюшины, являются национальные консенсусы. Настоящий Консенсус является первой попыткой предложить стандартизованные подходы к проблеме псевдомиксомы брюшины в России.

Методы

Методы: Настоящий Консенсус принят на основании анализа литературных данных, анализа известных зарубежных консенсусов, а также в результате серии обсуждений с участием специалистов, имеющих значительный личный опыт в диагностике и лечении псевдомиксомы брюшины.

Результаты

Результаты: Итоговый документ Консенсуса представлен в формате расширенных клинических рекомендаций, освещающих все аспекты данной патологии — эпидемиологию, патогенез, классификацию, клиническую картину, диагностику, и возможные варианты лечения, наблюдение после лечения и организационные вопросы.

Заключение

Заключение: Настоящий Консенсус создан с целью предложить практикующим врачам оптимальную стратегию лечения псевдомиксомы брюшины, а также информировать врачей (как онкологов, так и общего профиля) об особенностях данной редкой патологии.

псевдомиксома брюшинымуцинозная опухоль аппендиксациторедукциягипертермическая внутрибрюшинная химиотерапияHIPECReferencesSugarbaker P.H. Are there curative options to peritoneal carcinomatosis?. Ann Surg 2005;242(5):748–751. https://doi.org/10.1097/00000658-200511000-00023Sugarbaker P.H. Are there curative options to peritoneal carcinomatosis?. Ann Surg 2005;242(5):748–751. https://doi.org/10.1097/00000658-200511000-00023Smeenk R.M., van Velthuysen M.L., Verwaal V.J., Zoetmulder F.A. Appendiceal neoplasms and pseudomyxoma peritonei: a population based study. Eur J Surg Oncol. 2008;34(2):196–201. https://doi.org/10.1016/j.ejso.2007.04.002Smeenk R.M., van Velthuysen M.L., Verwaal V.J., Zoetmulder F.A. Appendiceal neoplasms and pseudomyxoma peritonei: a population based study. Eur J Surg Oncol. 2008;34(2):196–201. https://doi.org/10.1016/j.ejso.2007.04.002Sugarbaker P.H. New standard of care for appendiceal epithelial neoplasms and pseudomyxoma peritonei syndrome? Lancet Oncol 2006;7(1):69–76. https://doi.org/10.1016/S1470-2045(05)70539-8Sugarbaker P.H. New standard of care for appendiceal epithelial neoplasms and pseudomyxoma peritonei syndrome? Lancet Oncol 2006;7(1):69–76. https://doi.org/10.1016/S1470-2045(05)70539-8Циторедуктивная хирургия и периоперационная химиотерапия для лечения опухолей с перитонеальной диссеминаций. Руководство и видеоатлас. Второе издание. Под редакцией Пол Шугабейкер, главный редактор русскоязычного издания Вадим Гущин 2019.Cytoreductive surgery and perioperative chemotherapy for the treatment of tumors with peritoneal dissemination. Manual and video atlas. Second edition. Edited by Paul Sugarbaker, editor-in-chief of the Russian-language edition Vadimshchin 2019Govaerts K., Lurvink R.J., De Hingh IHJ.T., et al. Appendiceal tumours and pseudomyxoma peritonei: Literature review with PSOGI/EURACAN clinical practice guidelines for diagnosis and treatment. Eur J Surg Oncol 2021;47(1):11–35. https://doi.org/10.1016/j.ejso.2020.02.012Govaerts K., Lurvink R.J., De Hingh IHJ.T., et al. Appendiceal tumours and pseudomyxoma peritonei: Literature review with PSOGI/EURACAN clinical practice guidelines for diagnosis and treatment. Eur J Surg Oncol 2021;47(1):11–35. https://doi.org/10.1016/j.ejso.2020.02.012Chicago Consensus Working Group. The Chicago Consensus on Peritoneal Surface Malignancies: Management of Colorectal Metastases. Ann Surg Oncol 2020;27(6):1761–1767. https://doi.org/10.1245/s10434-020-08315-xChicago Consensus Working Group. The Chicago Consensus on Peritoneal Surface Malignancies: Management of Colorectal Metastases. Ann Surg Oncol 2020;27(6):1761–1767. https://doi.org/10.1245/s10434-020-08315-xWHO Classification of Tumours Editorial Board. Digestive system tumours (5th edition). – IARC; Lyon, 2019:35–155.WHO Classification of Tumours Editorial Board. Digestive system tumours (5th edition). – IARC; Lyon, 2019:35–155.Carr N.J., Cecil T.D., Mohamed F., et al. A Consensus for Classification and Pathologic Reporting of Pseudomyxoma Peritonei and Associated Appendiceal Neoplasia: The Results of the Peritoneal Surface Oncology Group International (PSOGI) Modified Delphi Process. Am J Surg Pathol 2016;40(1):14–26.9. https://doi.org/10.1097/PAS.0000000000000535Carr N.J., Cecil T.D., Mohamed F., et al. A Consensus for Classification and Pathologic Reporting of Pseudomyxoma Peritonei and Associated Appendiceal Neoplasia: The Results of the Peritoneal Surface Oncology Group International (PSOGI) Modified Delphi Process. Am J Surg Pathol 2016;40(1):14–26.9. https://doi.org/10.1097/PAS.0000000000000535AJCC Staging https://www.facs.org/quality-programs/cancer-programs/american-joint-committee-on-cancer/ajcc-staging-online/AJCC Staging https://www.facs.org/quality-programs/cancer-programs/american-joint-committee-on-cancer/ajcc-staging-online/Umetsu S.E., Kakar S. Staging of appendiceal mucinous neoplasms: challenges and recent updates. Hum Pathol 2023;132:65–76. https://doi.org/10.1016/j.humpath.2022.07.004Umetsu S.E., Kakar S. Staging of appendiceal mucinous neoplasms: challenges and recent updates. Hum Pathol 2023;132:65–76. https://doi.org/10.1016/j.humpath.2022.07.004National Comprehensive Cancer Network v.5.2024 https://www.nccn.org/guidelines/guidelines-detail?category = 1&id = 1428National Comprehensive Cancer Network v.5.2024 https://www.nccn.org/guidelines/guidelines-detail?category = 1&id = 1428Carmignani C.P., Hampton R., Sugarbaker C.E., et al. Utility of CEA and CA 19–9 tumor markers in diagnosis and prognostic assessment of mucinous epithelial cancers of the appendix. J Surg Oncol 2004;87(4):162–166. https://doi.org/10.1002/jso.20107Carmignani C.P., Hampton R., Sugarbaker C.E., et al. Utility of CEA and CA 19–9 tumor markers in diagnosis and prognostic assessment of mucinous epithelial cancers of the appendix. J Surg Oncol 2004;87(4):162–166. https://doi.org/10.1002/jso.20107Munoz-Zuluaga C.A., King M.C., Diaz-Sarmiento V.S., et al. Defining “Complete Cytoreduction» After Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy (CRS/HIPEC) for the Histopathologic Spectrum of Appendiceal Carcinomatosis. Ann Surg Oncol 2020 Dec;27(13):5026–5036. doi: 10.1245/s10434-020-08844-5, PMID: 32705513.Munoz-Zuluaga C.A., King M.C., Diaz-Sarmiento V.S., et al. Defining “Complete Cytoreduction» After Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy (CRS/HIPEC) for the Histopathologic Spectrum of Appendiceal Carcinomatosis. Ann Surg Oncol 2020 Dec;27(13):5026–5036. doi: 10.1245/s10434-020-08844-5, PMID: 32705513.Chua TC, Moran BJ, Sugarbaker PH, et al. Early- and long-term outcome data of patients with pseudomyxoma peritonei from appendiceal origin treated by a strategy of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. J Clin Oncol. 2012;30(20):2449–2456. https://doi.org/10.1200/JCO.2011.39.7166Chua TC, Moran BJ, Sugarbaker PH, et al. Early- and long-term outcome data of patients with pseudomyxoma peritonei from appendiceal origin treated by a strategy of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. J Clin Oncol. 2012;30(20):2449–2456. https://doi.org/10.1200/JCO.2011.39.7166Nikiforchin A., Sardi A., King MC., et al. Safety of Nephrectomy Performed During CRS/HIPEC: A Propensity Score-Matched Study. Ann Surg Oncol 2023;30(4):2520–2528. https://doi.org/10.1245/s10434-022-12862-wNikiforchin A., Sardi A., King MC., et al. Safety of Nephrectomy Performed During CRS/HIPEC: A Propensity Score-Matched Study. Ann Surg Oncol 2023;30(4):2520–2528. https://doi.org/10.1245/s10434-022-12862-wGlehen O., Mohamed F., Sugarbaker P.H. Incomplete cytoreduction in 174 patients with peritoneal carcinomatosis from appendiceal malignancy. Ann Surg 2004 Aug;240(2):278–85. https://doi.org/10.1097/01.sla.0000133183.15705.71Glehen O., Mohamed F., Sugarbaker P.H. Incomplete cytoreduction in 174 patients with peritoneal carcinomatosis from appendiceal malignancy. Ann Surg 2004 Aug;240(2):278–85. https://doi.org/10.1097/01.sla.0000133183.15705.71Baratti D., Kusamura S., Nonaka D., et al. Pseudomyxoma peritonei: biological features are the dominant prognostic determinants after complete cytoreduction and hyperthermic intraperitoneal chemotherapy. Ann Surg. 2009;249(2):243–249. https://doi.org/10.1097/SLA.0b013e31818eec64Baratti D., Kusamura S., Nonaka D., et al. Pseudomyxoma peritonei: biological features are the dominant prognostic determinants after complete cytoreduction and hyperthermic intraperitoneal chemotherapy. Ann Surg. 2009;249(2):243–249. https://doi.org/10.1097/SLA.0b013e31818eec64Somashekhar S.P., Rohit K.C., Ramya Y., et al. Bowel Anastomosis After or Before HIPEC: A Comparative Study in Patients Undergoing CRS + HIPEC for Peritoneal Surface Malignancy. Ann Surg Oncol 2022 Jan;29(1):214–223. https://doi.org/10.1245/s10434-021-10661-3Somashekhar S.P., Rohit K.C., Ramya Y., et al. Bowel Anastomosis After or Before HIPEC: A Comparative Study in Patients Undergoing CRS + HIPEC for Peritoneal Surface Malignancy. Ann Surg Oncol 2022 Jan;29(1):214–223. https://doi.org/10.1245/s10434-021-10661-3Hübner M., Kusamura S., Villeneuve L., et al. Guidelines for Perioperative Care in Cytoreductive Surgery (CRS) with or without hyperthermic IntraPEritoneal chemotherapy (HIPEC): Enhanced recovery after surgery (ERAS®) Society Recommendations - Part I: Preoperative and intraoperative management. Eur J Surg Oncol 2020;46(12):2292–2310. https://doi.org/10.1016/j.ejso.2020.07.041Hübner M., Kusamura S., Villeneuve L., et al. Guidelines for Perioperative Care in Cytoreductive Surgery (CRS) with or without hyperthermic IntraPEritoneal chemotherapy (HIPEC): Enhanced recovery after surgery (ERAS®) Society Recommendations - Part I: Preoperative and intraoperative management. Eur J Surg Oncol 2020;46(12):2292–2310. https://doi.org/10.1016/j.ejso.2020.07.041Hübner M., Kusamura S., Villeneuve L., et al. Guidelines for Perioperative Care in Cytoreductive Surgery (CRS) with or without hyperthermic IntraPEritoneal chemotherapy (HIPEC): Enhanced recovery after surgery (ERAS®) Society Recommendations - Part II: Preoperative and intraoperative management. Eur J Surg Oncol 2020;46(12):2311–2323. https://doi.org/10.1016/j.ejso.2020.08.006Hübner M., Kusamura S., Villeneuve L., et al. Guidelines for Perioperative Care in Cytoreductive Surgery (CRS) with or without hyperthermic IntraPEritoneal chemotherapy (HIPEC): Enhanced recovery after surgery (ERAS®) Society Recommendations - Part II: Preoperative and intraoperative management. Eur J Surg Oncol 2020;46(12):2311–2323. https://doi.org/10.1016/j.ejso.2020.08.006Solanki S.L., Mukherjee S., Agarwal V., et al. Society of Onco-Anaesthesia and Perioperative Care consensus guidelines for perioperative management of patients for cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS-HIPEC). Indian J Anaesth 2019;63(12):972–987. https://doi.org/10.4103/ija.IJA_765_19Solanki S.L., Mukherjee S., Agarwal V., et al. Society of Onco-Anaesthesia and Perioperative Care consensus guidelines for perioperative management of patients for cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS-HIPEC). Indian J Anaesth 2019;63(12):972–987. https://doi.org/10.4103/ija.IJA_765_19Lopez-Ramirez F., Gushchin V., Sittig M., et al. Iterative Cytoreduction and Hyperthermic Intraperitoneal Chemotherapy for Recurrent Mucinous Adenocarcinoma of the Appendix. Ann Surg Oncol 2022;29(6):3390–3401. https://doi.org/10.1245/s10434-021-11233-1Lopez-Ramirez F., Gushchin V., Sittig M., et al. Iterative Cytoreduction and Hyperthermic Intraperitoneal Chemotherapy for Recurrent Mucinous Adenocarcinoma of the Appendix. Ann Surg Oncol 2022;29(6):3390–3401. https://doi.org/10.1245/s10434-021-11233-1Bhutiani N., Grotz T.E., Concors S.J., et al. Repeat Cytoreduction and Hyperthermic Intraperitoneal Chemotherapy for Recurrent Mucinous Appendiceal Adenocarcinoma: A Viable Treatment Strategy with Demonstrable Benefit. Ann Surg Oncol 2024;31(1):614–621. https://doi.org/1010.1245/s10434-023-14422-2Bhutiani N., Grotz T.E., Concors S.J., et al. Repeat Cytoreduction and Hyperthermic Intraperitoneal Chemotherapy for Recurrent Mucinous Appendiceal Adenocarcinoma: A Viable Treatment Strategy with Demonstrable Benefit. Ann Surg Oncol 2024;31(1):614–621. https://doi.org/1010.1245/s10434-023-14422-2Blackham A.U., Swett K., Eng C., et al. Perioperative systemic chemotherapy for appendiceal mucinous carcinoma peritonei treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. J Surg Oncol 2014;109(7):740–745. https://doi.org/10.1002/jso.23547Blackham A.U., Swett K., Eng C., et al. Perioperative systemic chemotherapy for appendiceal mucinous carcinoma peritonei treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. J Surg Oncol 2014;109(7):740–745. https://doi.org/10.1002/jso.23547Asare E.A., Compton C.C., Hanna N.N., et al. The impact of stage, grade, and mucinous histology on the efficacy of systemic chemotherapy in adenocarcinomas of the appendix: Analysis of the National Cancer Data Base. Cancer 2016;122(2):213–221. https://doi.org/10.1002/cncr.29744Asare E.A., Compton C.C., Hanna N.N., et al. The impact of stage, grade, and mucinous histology on the efficacy of systemic chemotherapy in adenocarcinomas of the appendix: Analysis of the National Cancer Data Base. Cancer 2016;122(2):213–221. https://doi.org/10.1002/cncr.29744Milovanov V., Sardi A., Ledakis P., et al. Systemic chemotherapy (SC) before cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS/HIPEC) in patients with peritoneal mucinous carcinomatosis of appendiceal origin (PMCA). Eur J Surg Oncol 2015;41(5):707–712. https://doi.org/10.1016/j.ejso.2015.01.005Milovanov V., Sardi A., Ledakis P., et al. Systemic chemotherapy (SC) before cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS/HIPEC) in patients with peritoneal mucinous carcinomatosis of appendiceal origin (PMCA). Eur J Surg Oncol 2015;41(5):707–712. https://doi.org/10.1016/j.ejso.2015.01.005Pietrantonio F., Maggi C., Fanetti G., et al. FOLFOX-4 chemotherapy for patients with unresectable or relapsed peritoneal pseudomyxoma. Oncologist 2014;19(8):845–850. https://doi.org/10.1634/theoncologist.2014-0106Pietrantonio F., Maggi C., Fanetti G., et al. FOLFOX-4 chemotherapy for patients with unresectable or relapsed peritoneal pseudomyxoma. Oncologist 2014;19(8):845–850. https://doi.org/10.1634/theoncologist.2014-0106Baron E., Sardi A., King M.C., et al. Adjuvant chemotherapy for high-grade appendiceal cancer after cytoreductive surgery with hyperthermic intraperitoneal chemotherapy. Eur J Surg Oncol 2023;49(1):179–187. https://doi.org/10.1016/j.ejso.2022.08.022Baron E., Sardi A., King M.C., et al. Adjuvant chemotherapy for high-grade appendiceal cancer after cytoreductive surgery with hyperthermic intraperitoneal chemotherapy. Eur J Surg Oncol 2023;49(1):179–187. https://doi.org/10.1016/j.ejso.2022.08.022Tejani M.A., ter Veer A., Milne D., et al. Systemic therapy for advanced appendiceal adenocarcinoma: an analysis from the NCCN Oncology Outcomes Database for colorectal cancer. J Natl Compr Canc Netw 2014;12(8):1123–1130. https://doi.org/10.6004/jnccn.2014.0109Tejani M.A., ter Veer A., Milne D., et al. Systemic therapy for advanced appendiceal adenocarcinoma: an analysis from the NCCN Oncology Outcomes Database for colorectal cancer. J Natl Compr Canc Netw 2014;12(8):1123–1130. https://doi.org/10.6004/jnccn.2014.0109Baron E., King M.C., Gushchin V., et al. ASO Author Reflections: CRS/HIPEC Cannot be Done-Now what? Systemic Chemotherapy in Mucinous Appendiceal Cancer. Ann Surg Oncol 2022;29(11):6590–6591. https://doi.org/10.1245/s10434-022-11925-2Baron E., King M.C., Gushchin V., et al. ASO Author Reflections: CRS/HIPEC Cannot be Done-Now what? Systemic Chemotherapy in Mucinous Appendiceal Cancer. Ann Surg Oncol 2022;29(11):6590–6591. https://doi.org/10.1245/s10434-022-11925-2Logan-Collins J.M., Lowy A.M., Robinson-Smith T.M., et al. VEGF expression predicts survival in patients with peritoneal surface metastases from mucinous adenocarcinoma of the appendix and colon. Ann Surg Oncol 2008;15(3):738–744. https://doi.org/10.1245/s10434-007-9699-7Logan-Collins J.M., Lowy A.M., Robinson-Smith T.M., et al. VEGF expression predicts survival in patients with peritoneal surface metastases from mucinous adenocarcinoma of the appendix and colon. Ann Surg Oncol 2008;15(3):738–744. https://doi.org/10.1245/s10434-007-9699-7Choe J.H., Overman M.J., Fournier K.F., et al. Improved Survival with Anti-VEGF Therapy in the Treatment of Unresectable Appendiceal Epithelial Neoplasms. Ann Surg Oncol 2015;22(8):2578–2584. https://doi.org/10.1245/s10434-014-4335-9Choe J.H., Overman M.J., Fournier K.F., et al. Improved Survival with Anti-VEGF Therapy in the Treatment of Unresectable Appendiceal Epithelial Neoplasms. Ann Surg Oncol 2015;22(8):2578–2584. https://doi.org/10.1245/s10434-014-4335-9Franke A.J., Iqbal A., Starr J., et al. Management of malignant bowel obstruction associated with gi cancers. J Oncol Pract 2017;13(7):426–434. https://doi.org/10.1200/jop.2017.022210.Franke A.J., Iqbal A., Starr J., et al. Management of malignant bowel obstruction associated with gi cancers. J Oncol Pract 2017;13(7):426–434. https://doi.org/10.1200/jop.2017.022210.

The authors declare that there are no conflicts of interest present.