Immediate and long-term outcomes of surgical treatment in patients with retroperitoneal and abdominal desmoid fibromatosis: A retrospective study

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Background. The main method of treatment of desmoid fibromatosis is surgical, especially in patients with symptomatic disease or in cases of progression during the Look and Stay period. Due to the rarity of the disease, different localization, unpredictability of the clinical course, the lack of generally accepted clear criteria for choosing a treatment method and/or a sequence of treatment methods, the determination of prognostic criteria for the course of the disease is of great scientific and practical interest.

Aim. To study the immediate and long-term outcomes of surgical treatment in patients with retroperitoneal and abdominal desmoid fibromatosis.

Materials and methods. The study analyzed the data of 121 patients with histologically verified retroperitoneal and abdominal desmoid fibromatosis who underwent surgical treatment at the Blokhin National Medical Research Center of Oncology from 1999 to 2022.

Results. In 89% of cases, desmoid tumors are resectable; however, resections of adjacent organs are often required to remove the tumor mass completely. The frequency of combined interventions in the abdominal and retroperitoneal groups was 7.0 and 60.4%, respectively. Tumor cells along the edge of the incision are identified in 15.8% of patients, including 10% of patients with macroscopically detectable residual tumors. Surgical treatment of patients with desmoid tumors is associated with an acceptable complication rate and provides high rates of overall and relapse-free survival. Risk factors for disease-free survival of operated patients are retroperitoneal localization, multicentric tumor growth, and R2 category.

Conclusion. The treatment of patients with retroperitoneal and abdominal desmoid tumors should be carried out in specialized clinics with sufficient experience in performing surgical interventions, including combined ones. The treatment approach in patients with desmoid tumors should be selected by a multidisciplinary team based on personalized oncological and functional prognoses in accordance with the prognostic risk groups.

作者简介

Kirill Turupaev

Blokhin National Medical Research Center of Oncology; Russian University of Medicine

编辑信件的主要联系方式.
Email: Kir-turupaev@yandex.ru
ORCID iD: 0000-0001-8887-5108

oncologist, Graduate Student

俄罗斯联邦, Moscow; Moscow

Marina Budurova

Russian University of Medicine; Hertsen Moscow Oncology Research Institute – branch of the National Medical Research Radiological Centre

Email: Kir-turupaev@yandex.ru
ORCID iD: 0000-0003-1391-0516

D. Sci. (Med.)

俄罗斯联邦, Moscow; Moscow

Maxim Nikulin

Blokhin National Medical Research Center of Oncology; Russian University of Medicine

Email: Kir-turupaev@yandex.ru
ORCID iD: 0000-0002-9608-4696
SPIN 代码: 9455-5566

Cand. Sci. (Med.)

俄罗斯联邦, Moscow; Moscow

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2. Fig. 1. Three-year survival rate depending on the localization of desmoid tumor: a – Kaplan-Meier OS curve depending on the desmoid tumor site; b – Kaplan-Meier RFS curve depending on the desmoid tumor site.

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3. Fig. 2. Recurrence-free survival rate depending on tumor location, type and growth, macroscopically positive resection margin: a – Kaplan-Meier RFS curve depending on the desmoid tumor site; b – Kaplan-Meier RFS curve depending on the type of desmoid tumor growth; c – Kaplan-Meier RFS curve depending on the nature of desmoid tumor growth; d – Kaplan-Meier RFS curve depending on the surgical margin status.

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