Рецидивирующий десмоидный забрюшинный фиброматоз: клиническое наблюдение

Будурова М.Д., Файнштейн И.А., Турупаев К.А. и др. Рецидивирующий десмоидный забрюшинный фиброматоз: клиническое наблюдение. Современная Онкология. 2020; 22 (4): 125–129. DOI: 10.26442/18151434.2020.4.200424

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Budurova M.D., Fainshtein I.A., Turupaev K.A. et al. Recurrent desmoid-type retroperitoneal fibromatosis: clinical observation. Journal of Modern Oncology. 2020; 22 (4): 125–129. DOI: 10.26442/18151434.2020.4.200424

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Аннотация

В настоящей статье описано редкое клиническое наблюдение пациента с рецидивирующим десмоидным фиброматозом, у которого достигнута длительная стабилизация после хирургического лечения. У больного 24 лет в 2013 г. выявлена забрюшинная опухоль размером 8,8×5,6×13 см, инфильтрирующая левую поясничную мышцу, левую почку, левую общую и левую наружную подвздошные артерии, нисходящую ободочную и сигмовидные кишки. Пациент оперирован в ФГБУ «НМИЦ онкологии им. Н.Н. Блохина» в объеме удаления опухоли, резекции левой общей подвздошной артерии с протезированием протезом GORЕ-TEX, левосторонней гемиколэктомии, левосторонней нефрэктомии. Через 9 мес после операции выявлен 1-й рецидив опухоли. В связи последующим ростом опухолевых очагов, локализовавшихся по ходу левой наружной подвздошной артерии и в паховом канале, выполнена повторная операция. В дальнейшем у пациента наблюдался 2-й рецидив, по поводу которого также предпринято хирургическое вмешательство. Через 7 мес после перенесенного хирургического лечения зафиксирован 3-й рецидив опухоли. На мультидисциплинарном обсуждении, учитывая отсутствие жалоб и небольшие размеры рецидивной опухоли, а также отсутствие риска развития угрожающих жизни осложнений, решено ограничиться наблюдением. Больному проводится ежегодное обследование с частотой один раз в полгода – данных по поводу роста рецидивной опухоли не отмечено. На сегодняшний день больной жив, жалоб не предъявляет, трудоспособен. Наше клиническое наблюдение демонстрирует, что активная хирургическая тактика при рецидиве забрюшинного фиброматоза не всегда приводит к достижению длительного времени без прогрессирования и некоторым пациентам может быть рекомендована наблюдательная тактика Look and stay.

Ключевые слова: десмоидный фиброматоз, десмоидная опухоль, рецидивирующая десмоидная опухоль, забрюшинный фиброматоз, брыжеечный фиброматоз.

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This article deals with the rare clinical observation of the patient with recurrent desmoid-type fibromatosis, who have achieved long-term stability after surgical treatment. A 24-year-old patient was diagnosed with retroperitoneal tumor which size was 8.8×5.6×13 cm in 2013, infiltrating the left psoas muscle, left kidney, left common and left external iliac arteries, descending colon and sigmoid colon. The patient underwent surgery in the volume of the tumor removal, resection of the left common iliac artery and prosthetics using GORE-TEX prosthesis, left hemicolectomy, left nephrectomy at Blokhin National Medical Research Center of Oncology. The first recurrence of the tumor was detected nine months after the surgery. Due to the subsequent growth of tumor mass, located along the left external iliac artery and in the inguinal canal, the repeated surgery was performed. Then the patient had a second relapse, and underwent surgery again. The third recurrence was detected seven months after the last surgery. During the multidisciplinary discussion, according to the absence of complaints and the small size of the recurrent tumor, as well as the absence of the risk of life-threatening complications, it was decided to stop on the observation. The patient was examined once every six months – there were no data concerning recurrent tumor growth. Today, the patient is alive, does not have any complaints and is able-bodied. Our clinical observation demonstrates that active surgical tactics in case of the retroperitoneal fibromatosis recurrence not always can lead to long-term progression-free survival time and several patients can stay under the observation, using Look and Stay tactic.

Key words: desmoid-type fibromatosis, desmoid tumor, recurrent desmoid tumor, retroperitoneal fibromatosis, mesenteric fibromatosis.

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1. Novikova O.V. Polovye gomony v etiologii, patogeneze i lechenii desmoidnykh fibrom'. Dis. … d-ra med. nauk. Moscow, 2008; p. 197–9 (in Russian).
2. Khomiakov V.M., Cheremisov V.V., Chaika A.V. et al. Opyt khirurgicheskogo lecheniia desmoidnykh fibrom abdominal'noi i intraabdominal'noi lokalizatsii. Khirurgiia. Zhurn. im. N.I. Pirogova. 2014; 11: 17–25 (in Russian).
3. Huang K, Fu H, Shi YQ et al. Prognostic factors for extra-abdominal and abdominal wall desmoids: a 20-year experience at a single institution. J Surg Oncol 2009; 100: 563–9. http://www.ncbi.nlm.nih.gov/pubmed/19722232
4. Kaprin A.D., Riabov A.B., Cheremisov V.V. et al. Desmoidnaia fibroma zabriushinnogo prostranstva s vovlecheniem nizhnei poloi veny i mochetochnika. Onkologiia. Zhurn. im. P.A. Gertsena. 2016; 3: 65–70 (in Russian).
5. Kostrygin A.K., Riabov A.B., Khomiakov V.M. et al. Rezul'taty khirurgicheskogo lecheniia patsientov s desmoidnymi fibromami abdominal'noi lokalizatsii. Onkologiia. Zhurn. im. P.A. Gertsena. 2018; 7 (2): 4–15 (in Russian).
6. Escobar C, Munker R, Thomas JO et al. Update on desmoid tumors. Ann Oncol 2012; 23 (3): 562–9. DOI: 10.1093/annonc/mdr386
7. Gronchi A, Jones RL. Treatment of Desmoid Tumors in 2019. JAMA Oncol 2019; 5 (4): 567–8. DOI: 10.1001/jamaoncol.2018.6449
8. Reitamo JJ, Scheinin TM, Häyry P. The desmoid syndrome. New aspects in the cause, pathogenesis and treatment of the desmoid tumor. Am J Surg 1986; 151 (2): 230–7.
9. Couto Netto SD, Teixeira F Jr, Menegozzo CAM et al. Abdominal wall reconstruction after desmoid type fibromatosis radical resection: Case series from a single institution and review of the literature. Int J Surg Case Rep 2017; 33: 167–72. DOI: 10.1016/j.ijscr.2017.02.050
10. Bright-Thomas RM, Agrawal A, Hargest R. Preclinical studies of gene transfer for the treatment of desmoid disease in familial adenomatous polyposis. Br J Surg 2002; 89: 1563–9.
11. Abate M, Pigazzi A. Mesenteric fibromatosis in a patient with a history of neuroblastoma: a case report. J Surg Case Rep 2018; 2018 (9): rjy209. DOI: 10.1093/jscr/rjy209
12. Fritz A, Percy C, Jack A et al. International classification of diseases for oncology (ICD-O), 3rd ed. WHO, Geneva. 2000.
13. Bolotina L.V., Novikova O.V., Prokof'eva E.A. Rol' khimioterapii v lechenii agressivnogo fibromatoza. Onkologiia. Zhurn. im. P.A. Gertsena. 2012; 3: 72–6 (in Russian).
14. Karakousis CP, Mayordomo J, Zografos GC. Desmoid tu-mors of the trunk and extremity.Cancer 1993; 72: 1637–41.
15. Nath MC, Torbenson MS, Erickson LA. Desmoid Tumor. Mayo Clin Proc 2018; 93 (4): 549–50. DOI: 10.1016/j.mayocp.2018.02.009
16. Caglar K, Ozyönüm H, Akalin M, Balci S. Effective treatment of multifocal aggressive fibromatosis with low-dose chemotherapy. Turk J Pediatr 2006; 48 (4): 365–8.
17. Libertini M, Mitra I, van der Graaf WTA et al. Aggressive fibromatosis response to tamoxifen: lack of correlation between MRI and symptomatic response. Clin Sarcoma Res 2018; 8: 13. DOI: 10.1186/s13569-018-0100-3
18. Mukherjee A, Malcolm A, de la Hunt M, Neal DE. Pelvic fibromatosis (desmoid)--treatment with steroids and tamoxifen. Br J Urol 1995; 75 (4): 559–60. DOI: 10.1111/j.1464-410x.1995.tb07289.x
19. Colombo C, Urbini M, Astolfi A et al. Novel intra-genic large deletions of CTNNB1 gene identified in WT desmoid-type fibromatosis. Genes Chromosomes Cancer 2018; 57 (10): 495–503. DOI: 10.1002/gcc.22644
20. Seinen JM, Niebling MG, Bastiaannet E et al. Four different treatment strategies in aggressive fibromatosis: a systematic review. Clin Transl Radiat Oncol 2018; 12: 1–7.
21. Plukker JT, van Oort I, Vermey A. Aggressive fibromatosis (non-familial desmoid tumour): therapeutic problems and the role of adjuvant radiotherapy. Br J Surg 1995; 82: 510–4.
22. Dalén BPM, Geijer M, Kvist H et al. Gunterberg, Clinical and imaging observations of desmoid tumors left without treatment, Acta Orthop 2006; 77: 932–7. DOI: 10.1080/17453670610013259
23. Barbier O, Anract P, Pluot E et al. Tomeno – Primary or recurring extra-abdominal desmoid fibromatosis: assessment of treatment by observation only. Orthop Traumatol Surg Res 2010; 96: 884–9. DOI: 10.1016/j.otsr.2010.07.007
24. Colombo C, Miceli R, Le Péchoux C et al. Bonvalot, Sporadic extra abdominal wall desmoid-type fibromatosis: surgical resection can be safely limited to a minority of patients. Eur J Cancer Oxf Engl 2015 186–92, DOI: 10.1016/j.ejca.2014.11.019
25. Salas S, Dufresne A, Bui B et al. Prognostic factors influencing progression-free survival determined from a series of sporadic desmoid tumors: a wait-and-see policy according to tumor presentation. J Clin Oncol 2011; 29: 3553–8. http://www.ncbi.nlm.nih.gov/pubmed/21844500
26. Fiore M, Rimareix F, Mariani L et al. Desmoid-type fibromatosis: a front-line conservative approach to sel ect patients for surgical treatment. Ann Surg Oncol 2009; 16: 2587–93. http://www.ncbi.nlm.nih.gov/pubmed/19568815
27. Rodriguez‐Bigas MA, Mahoney MC, Karakousis CP, Petrelli NJ. Desmoid tumors in patients with familial adenomatous polyposis. Cancer 1994; 74: 1270–4.
28. Pikaar A, Nortier JW, Griffioen G, Vasen HF. Desmoid tumors in patients with familial adenomatous polyposis. Ned Tijdschr Geneeskd 2002; 20 (146): 1355–9.
29. Torres JC, Xin C. An unusual finding in a desmoid-type fibromatosis of the pancreas: a case report and review of the literature. J Med Case Rep 2018; 12 (1): 123. DOI: 10.1186/s13256-018-1635-x
30. Melis M, Zager JS, Sondak VK. Multimodality management of desmoid tumors: how important is a negative surgical margin? J Surg Oncol 2008; 98: 594–602. http://www.ncbi.nlm.nih.gov/pubmed/19072851
31. Peng PD, Hyder O, Mavros MN et al. Management and recurrence patterns of desmoids tumors: a multi-institutional analysis of 211 patients. Ann Surg Oncol 2012; 19: 4036–42. http://www.ncbi.nlm.nih.gov/pubmed/22972507
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Авторы

М.Д. Будурова*1, И.А. Файнштейн1, К.А. Турупаев2, А.Е. Калинин1, Н.А. Козлов1

1 ФГБУ «Национальный медицинский исследовательский центр онкологии им. Н.Н. Блохина» Минздрава России, Москва, Россия;
2 ФГБОУ ВО «Московский государственный медико-стоматологический университет им. А.И. Евдокимова» Минздрава России, Москва, Россия
*m.budurova@rambler.ru

________________________________________________

Marina D. Budurova*1, Igor A. Fainshtein1, Kirill A. Turupaev2, Aleksei E. Kalinin1, Nikolai A. Kozlov1

1 Blokhin National Medical Research Center of Oncology, Moscow, Russia;
2 Yevdokimov Moscow State University of Medicine and Dentistry, Moscow, Russia
*m.budurova@rambler.ru

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