Preview

Malignant tumours

Advanced search

Агрессивный фиброматоз

https://doi.org/10.18027/2224-5057-2024-14-3s1-35-41

Abstract

Агрессивный фиброматоз (АФ) или десмоидные опухоли (ДО) — это локально агрессивные и инвазивные опухоли мягких тканей мезенхимального происхождения, которые сложно лечить из‑за их вариабельных клинических проявлений, непредсказуемого клинического течения и отсутствия в настоящее время одобренных методов лечения. Это группа ЗНО редко встречается в клинической практике онколога, в связи с чем диагностика и опции лечения данной патологии существенно ограничены. На сегодняшний день, нет стандартного подхода к ведению больных с АФ, поэтому в зависимости от локализации опухоли, ее симптомов и риска развития осложнений и прогрессирования подбирается один из оптимальных методов лечения: хирургическое вмешательство или другие локальные методы, системная терапия или динамическое наблюдение. Лекарственная терапия активно развивается на протяжении последних десятилетий: от химиотерапии до внедрения тирозинкиназных ингибиторов (ТКИ) и одобрения новых молекул. В настоящее время сорафениб и пазопаниб являются одними из наиболее используемых ТКИ. Кроме того, новые ингибиторы гамма‑секретазы (нирогацестат и AL102) обещают быть эффективными средствами для лечения АФ с управляемым профилем безопасности.

About the Authors

Л. Владимирова
ФГБУ «НМИЦ онкологии» Минздрава России
Russian Federation


А. Алькина
ФГБУ «НМИЦ онкологии» Минздрава России
Russian Federation


References

1. Napolitano A., Mazzocca A., Spalato Ceruso M., et al. Recent advances in desmoid tumor therapy. Cancers (Basel) 2020;12(8):2135. https://doi.org/10.3390/cancers12082135

2. Husson O., Younger E., Dunlop A., et al. Desmoid fibromatosis through the patients' eyes: time to change the focus and organisation of care? Support Care Cancer 2019;27(3):965-980. https://doi.org/10.1007/s00520-018-4386-8

3. Bektas M., Bell T., Khan S., et al. Desmoid tumors: a comprehensive review. Adv Ther 2023;40(9):3697-3722. https://doi.org/10.1007/s12325-023-02592-0

4. Bonvalot S., Desai A., Coppola S., et al. The treatment of desmoid tumors: a stepwise clinical approach. Ann Oncol 2012;23:x158-x166. https://doi.org/10.1093/annonc/mds298

5. Constantinidou A., Scurr M., Judson I., Litchman C. Clinical presentation of desmoid tumors. In: Litchman C, editor. Desmoid tumors. Dordrecht, the Netherlands: Springer 2012.p.5-16.

6. Kasper B., Strobel P., Hohenberger P. Desmoid tumors: clinical features and treatment options for advanced disease. Oncologist 2011;16(5):682-693. https://doi.org/10.1634/theoncologist.2010-0281

7. Bertario L., Russo A., Sala P., et al. Multiple approach to the exploration of genotype-phenotype correlations in familial adenomatous polyposis. J Clin Oncol 2003;21(9):1698-1707. https://doi.org/10.1200/JCO.2003.09.118

8. Fiore M., Rimareix F., Mariani L., et al. Desmoid-type fibromatosis: a front-line conservative approach to select patients for surgical treatment. Ann Surg Oncol 2009;16(9):2587-2593. https://doi.org/10.1245/s10434-009-0586-2

9. Riedel R.F., Agulnik M. Evolving strategies for management of desmoid tumor. Cancer 2022;128(16):3027-3040. https://doi.org/10.1002/cncr.34332

10. Hughes D., Kummar S., Lazar A. New, tolerable Y-secretase inhibitor takes desmoid down a notch. Clin Cancer Res 2015;21(1):7-9. https://doi.org/10.1158/1078-0432.CCR-14-1660

11. Zhang Z., Shi J., Yang T., et al. Management of aggressive fibromatosis. Oncol Lett 2021;21(1):43. https://doi.org/10.3892/ol.2020.12304

12. Zhan T., Rindtorff N., Boutros M. Wnt signaling in cancer. Oncogene 2017;36(11):1461-73. https://doi.org/10.1038/onc.2016.304

13. Timbergen M. JM., Smits R., Grunhagen D.J., et al. Activated signaling pathways and targeted therapies in desmoid-type fibromatosis: a literature review. Front Oncol 2019;9:397. https://doi.org/10.3389/fonc.2019.00397

14. Lazar A. J, Tuvin D., Hajibashi S., et al. Specific mutations in the beta-catenin gene (CTNNB1) correlate with local recurrence in sporadic desmoid tumors. Am J Pathol 2008;173(5):1518-1527. https://doi.org/10.2353/ajpath.2008.080475

15. Colombo C., Miceli R., Lazar A.J., et al. CTNNB1 45F mutation is a molecular prognosticator of increased postoperative primary desmoid tumor recurrence: An independent, multicenter validation study. Cancer 2013;119(20):3696-3702. https://doi.org/10.1002/cncr.28271

16. Mullen J.T., DeLaney T.F., Rosenberg A.E., et al. в-Catenin mutation status and outcomes in sporadic desmoid tumors. Oncologist 2013;18(9):1043-1049. https://doi.org/10.1634/theoncologist.2012-0449

17. Referenced with permission from the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for Soft Tissue Sarcoma V. 1.2023. National Comprehensive Cancer Network, Inc. 2023. All rights reserved. Accessed March 20, 2023. To view the most recent and complete version of the guideline, go online to NCCN.org.

18. Gounder M. The Desmoid Tumor Research Foundation. Diagnosis and treatment. 2021. https://dtrf.org/diagno-sis-and-treatment/. Accessed 13 December 2021.

19. Kasper B., Baumgarten C., Garcia J., et al. An update on the management of sporadic desmoid-type fibromatosis: a European Consensus Initiative between Sarcoma Patients EuroNet (SPAEN) and European Organization for Research and Treatment of Cancer (EORTC)/Soft Tissue and Bone Sarcoma Group (STBSG). Ann Oncol 2017;28(10):2399-2408. https://doi.org/10.1093/annonc/mdx323

20. Penel N., Chibon F., Salas S. Adult desmoid tumors: biology, management and ongoing trials. Curr Opin Oncol 2017;29(4):268-74. https://doi.org/10.1097/CCO.0000000000000374

21. Garcia-Ortega D.Y., Martin-Tellez K.S., Cuellar-Hubbe M., et al. Desmoid-type fibromatosis. Cancers (Basel) 2020;12(7):1851. https://doi.org/10.3390/cancers12071851

22. Penel N., Coindre J.M., Bonvalot S., et al. Management of desmoid tumours: a nationwide survey of labelled reference centre networks in France. Eur J Cancer 2016;58:90-6. https://doi.org/10.1016/j.ejca.2016.02.008

23. Bonvalot S., Eldweny H., Haddad V., et al. Extra-abdominal primary fibromatosis: Aggressive management could be avoided in a subgroup of patients. Eur J Surg Oncol 2008;34(4):462-468. https://doi.org/10.1016/j.ejso.2007.06.006

24. Ma D., Li S., Fu R., et al. Long-term outcomes of 47 patients with aggressive fibromatosis of the chest treated with surgery. Eur J Surg Oncol 2016;42(11):1693-1698. https://doi.org/10.1016/j.ejso.2016.06.396

25. Seinen J.M., Niebling M.G., Bastiaannet E., et al. Four different treatment strategies in aggressive fibromatosis: a systematic review. Clin Transl Radiat Oncol 2018;12:1-7. https://doi.org/10.1016/j.ctro.2018.03.001

26. Yang T., Liu H., Liao Z., et al. Postoperative adjuvant radiotherapy can delay the recurrence of desmoid tumors after R0 resection in certain subgroups. Front Surg 2021;8:697793. https://doi.org/10.3389/fsurg.2021.697793

27. Van Broekhoven D.L., Deroose J.P., Bonvalot S., et al. Isolated limb perfusion using tumour necrosis factor a and melphalan in patients with advanced aggressive fibromatosis. Br J Surg 2014;101(13):1674-1680. https://doi.org/10.1002/bjs.9659

28. Kurtz J.-E., Buy X., Deschamps F., et al. CRYODESMO-O1: a prospective, open phase II study of cryoablation in desmoid tumour patients progressing after medical treatment. Eur J Cancer 2021;143:78-87. https://doi.org/10.1016/j.ejca.2020.10.035

29. Skapek S.X., Ferguson W.S., Granowetter L., et al. Vinblastine and methotrexate for desmoid fibromatosis in children: results of a pediatric oncology group phase II trial. J Clin Oncol 2007;25(5):501-506. https://doi.org/10.1200/JCO.2006.08.2966

30. Palassini E., Frezza A., Marianai L., et al. Long-term efficacy of methotrexate plus vinblastine/vinorelbine in a large series of patients affected by desmoid-type fibromatosis. Cancer J 2017;23(2):86-91. https://doi.org/10.1097/PPO.0000000000000254

31. Mir O., Rahal C., Rimareix F., et al. Efficacy of oral vinorelbine in advanced/progressive desmoid tumors: an updated retrospective study of 50 patients. Presented at: Annual Meeting of the American Society of Clinical Oncology; June 3-7, 2016. Abstract 11050

32. de Camargo V.P., Keohan M.L., D'Adamo D.R., et al. Clinical outcomes of systemic therapy for patients with deep fibromatosis (desmoid tumor). Cancer 2010;116(9):2258-2265. https://doi.org/10.1002/cncr.25089

33. Constantinidou A., Jones R.L., Scurr M., et al. Pegylated liposomal doxorubicin, an effective, well-tolerated treatment for refractory aggressive fibromatosis. Eur J Cancer 2009;45(17):2930-2934. https://doi.org/10.1016/j.ejca.2009.08.016

34. Constantinidou A., Jones R.L., Scurr M., et al. Advanced aggressive fibromatosis: effective palliation with chemotherapy. Acta Oncol 2011;50(3):455-461. https://doi.org/10.3109/0284186X.2010.509105

35. Pang A., Gouveia Macedo D.V., Carbini M., et al. Pegylated liposomal doxorubicin (PLD) as an active treatment option for desmoid tumors (DT) patients. Presented at: Annual Meeting of the American Society of Clinical Oncology; June 3-7, 2016. Abstract 11032

36. Fiore M., Colombo C., Radaelli S., et al. Hormonal manipulation with toremifene in sporadic desmoid-type fibromatosis. Eur J Cancer 2015;51(18):2800-2807. https://doi.org/10.1016/j.ejca.2015.08.026

37. Quast D.R., Schneider R., Burdzik E., et al. Long-term outcome of sporadic and FAP-associated desmoid tumors treated with high-dose selective estrogen receptor modulators and sulindac: a single-center long-term observational study in 134 patients. Fam Cancer 2016;15(1):31—40. https://doi.org/10.1007/s10689-015-9830-z

38. Mitra I., Szucs Z., Libertini M., et al. Aggressive fibromatosis response to tamoxifen: MRI features with symptomatic correlation—the Royal Marsden experience. In CTOS Annual Meeting 2016, Lisbon. Abstract 2549467

39. Mir O., Rahal C., Rimareix F., et al. Efficacy of oral vinorelbine in advanced/progressive desmoid tumours: an updated retrospective study in 50 patients. J Clin Oncol 2016;34. Abstract 11050

40. Garbay D., Le Cesne A., Penel N., et al. Chemotherapy in patients with desmoid tumors: a study from the French Sarcoma Group (FSG). Ann Oncol 2012;23(1):182-186. https://doi.org/10.1093/annonc/mdr051

41. Kasper B., Raut C.P., Gronchi A. Desmoid tumors: to treat or not to treat, that is the question. Cancer 2020;126(24):5213-5221. https://doi.org/10.1002/cncr.33233

42. Chugh R., Wathen J.K., Patel S.R., et al. Efficacy of imatinib in aggressive fibromatosis: Results of a phase II multicenter Sarcoma Alliance for Research through Collaboration (SARC) trial. Clin Cancer Res 2010;16(19):4884-4891. https://doi.org/10.1158/1078-0432.CCR-10-1177

43. Heinrich M.C., McArthur G.A., Demetri G.D., et al. Clinical and molecular studies of the effect of imatinib on advanced aggressive fibromatosis (desmoid tumor). J Clin Oncol 2006;24(7):1195-203. https://doi.org/10.1200/JCO.2005.04.0717

44. Gounder M.M., Mahoney M.R., Van Tine B.A., et al. Sorafenib for advanced and refractory desmoid tumors, N Engl J Med 2018;379(25):2417-2428. https://doi.org/10.1056/NEJMoa1805052

45. Toulmonde M., Pulido M., Ray-Coquard I., et al. Pazopanib or methotrexate-vinblastine combination chemotherapy in adult patients with progressive desmoid tumours (DESMOPAZ): a non-comparative, randomised, open-label, multicentre, phase 2 study. Lancet Oncol 2019;20(9):1263-1272. https://doi.org/10.1016/S1470-2045(19)30276-1

46. Kummar S., O'Sullivan Coyne G., Do K.T., et al. Clinical activity of the Y-Secretase inhibitor PF-03084014 in adults with desmoid tumors (aggressive fibromatosis). J Clin Oncol 2017;35(14):1561-1569. https://doi.org/10.1200/JCO.2016.71.1994

47. Shang H., Braggio D., Lee Y.J., et al. Targeting the Notch pathway: a potential therapeutic approach for desmoid tu-mors. Cancer 2015;121(22):4088-4096. https://doi.org/10.1002/cncr.29564

48. Purow B. Notch inhibition as a promising new approach to cancer therapy. Adv Exp Med Biol 2012;727:305-319. https://doi.org/10.1007/978-1-4614-0899-4_23

49. Kasper B., Ratan R., Alcindor T., et al. DeFi: a phase III, randomized controlled trial of nirogacestat versus placebo for progressing desmoid tumors (DT). Presented at: Annual Congress for the European Society for Medical Oncology; September 9-13, 2022. Abstract LBA2

50. Gounder M., Ratan R., Alcindor T., et al. Nirogacestat, a gamma-secretase inhibitor for desmoid tumors. N Engl J Med 2023;388(10):898-912. https://doi.org/10.1056/NEJMoa2210140

51. Gounder M.M., Jones R.L., Chugh R., et al. Initial results of phase II/III trial of AL102 for treatment of desmoid tumors (DT). Presented at: Annual Congress for the European Society for Medical Oncology; September 9-13, 2022. Abstract 1488M0.


Review

For citations:


 ,   . Malignant tumours. 2024;14(3s1):35-41. (In Russ.) https://doi.org/10.18027/2224-5057-2024-14-3s1-35-41

Views: 437


Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 License.


ISSN 2224-5057 (Print)
ISSN 2587-6813 (Online)