CLINICAL AND EPIDEMIOLOGIC EVALUATION OF DESMOID TUMORS IN A BRAZILIAN SARCOMA REFERENCE CENTER.

IF 0.5 4区医学 Q4 ORTHOPEDICS Acta Ortopedica Brasileira Pub Date : 2024-06-24 eCollection Date: 2024-01-01 DOI:10.1590/1413-785220243202e274225

Cassia da Silva, Fábio Fernando Eloi Pinto, Ademar Lopes, Suely Akiko Nakagawa, Marcelo Porfirio Sunagua Aruquipa, Samuel Aguiar, Celso Abdon Lopes de Mello

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Abstract

Introduction: Desmoid Tumors (DT) are rare neoplasms with higher incidence in younger women.

Methods: Retrospective, single-center analysis of patients with DT. Variables were age, sex, biopsy, treatment and recurrence. The disease-free survival (DFS) was calculated with the Kaplan-Meier method.

Results: 242 patients were evaluated, mean age was 34 years, 70.7% women, 44.4% originated in the trunk/abdomen and 54.5% had size > 5cm. Surgery was performed in 70.2%, 31% with negative margin and only 57% with previous biopsy. Recurrence rate was 38% and 1,2,5-year DFS was 75.3%, 64.2%, 57.8%, respectively. Size (p = 0.018) and tumor location in the dorsum (p = 0.001), extremities (p = 0.003) and pelvis (p = 0.003) were related to higher relapse rate.

Conclusion: our data reinforces the need to gather data from real world practice and the importance of awareness of DT and medical education about DT behavior and best approach due to the high rates of surgery and elevated number of patients treated without biopsy. Level of Evidence III; Retrospective Comparative Study.

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巴西肉瘤参考中心对脱模瘤的临床和流行病学评估。

导言蝶形细胞瘤（DT）是一种罕见肿瘤，在年轻女性中发病率较高：方法：对DT患者进行回顾性单中心分析。变量包括年龄、性别、活检、治疗和复发。结果：242名患者接受了评估，平均年龄34岁，70.7%为女性，44.4%起源于躯干/腹部，54.5%大小大于5厘米。70.2%的患者接受了手术治疗，31%的患者边缘阴性，只有57%的患者曾接受过活检。复发率为 38%，1、2、5 年的 DFS 分别为 75.3%、64.2% 和 57.8%。肿瘤大小（p = 0.018）、肿瘤位置在背侧（p = 0.001）、四肢（p = 0.003）和盆腔（p = 0.003）与较高的复发率有关。结论：我们的数据加强了从真实世界的实践中收集数据的必要性，以及对 DT 的认识和有关 DT 行为和最佳方法的医学教育的重要性，因为手术率高，未经活检治疗的患者数量增加。证据等级 III；回顾性比较研究。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Acta Ortopedica Brasileira Orthopedics-

CiteScore

0.90

自引率

14.30%

发文量

审稿时长

25 weeks

期刊介绍： A Revista Acta Ortopédica Brasileira, órgão oficial do Departamento de Ortopedia e Traumatologia da Faculdade de Medicina da Universidade de São Paulo (DOT/FMUSP), é publicada bimestralmente em seis edições ao ano (jan/fev, mar/abr, maio/jun, jul/ago, set/out e nov/dez) com versão em inglês disponível nos principais indexadores nacionais e internacionais e instituições de ensino do Brasil. Sendo hoje reconhecidamente uma importante contribuição para os especialistas da área com sua seriedade e árduo trabalho para as indexações já conquistadas.