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孤立性纤维性肿瘤(SFT)的临床特征与预后:单中心经验

Clinical Characteristics and Outcomes for Solitary Fibrous Tumor (SFT): A Single Center Experience.

作者信息

DeVito Nicholas, Henderson Evita, Han Gang, Reed Damon, Bui Marilyn M, Lavey Robert, Robinson Lary, Zager Jonathan S, Gonzalez Ricardo J, Sondak Vernon K, Letson G Douglas, Conley Anthony

机构信息

Sarcoma Department, H. Lee Moffitt Cancer Center and Research Institute, Tampa, Florida, United States of America.

Sarcoma Department, H. Lee Moffitt Cancer Center and Research Institute, Tampa, Florida, United States of America; Anatomic Pathology Department, H. Lee Moffitt Cancer Center and Research Institute, Tampa, Florida, United States of America.

出版信息

PLoS One. 2015 Oct 15;10(10):e0140362. doi: 10.1371/journal.pone.0140362. eCollection 2015.

Abstract

Solitary fibrous tumor (SFT) is a mesenchymal neoplasm of fibrous origin. The 2013 WHO classification of soft tissue tumors defines malignant forms as hypercellular, mitotically active (>4 mitosis/10 high-power fields), with cytological atypia, tumor necrosis, and/or infiltrative margins. With an IRB-approved protocol, we investigated patient records and clinicopathologic data from our Sarcoma Database to describe the clinical characteristics of both benign and malignant SFT. All pathology specimens were reviewed by two pathologists. Descriptive statistics and univariate/multivariate survival analysis were performed. Patient records and Social Security Death Index were used to evaluate vital status. Of 82 patients, 47 (57%) were women and 73 (89%) were Caucasian. Median age was 62 years (range, 20 to 89). Thirty-two (39%) patients succumbed to the disease. Primary tumor site was lung/pleura in 28 (34%), abdomen/pelvis in 23 (28%), extremity in 13 (16%), and head/neck in 9 (11%) patients. Pathology was described as benign in 42 (51%) and malignant in 40 (49%) patients. Compared to benign SFT, malignant histology is associated with larger tumor size, higher mitotic counts, metastatic disease at diagnosis, and greater use of chemotherapy and radiation therapy. Gender, age, and tumor site were not significantly different between benign and malignant subtypes. By univariate analysis, only benign vs. malignant variant and complete resection positively impacted overall survival (P = 0.02 and P<0.0001, respectively). In the multivariable analysis of overall survival, receiving chemotherapy or not receiving surgery were two variables significantly associated with higher failure rate in overall survival: patients with chemotherapy vs. no chemotherapy (P = 0.003, HR = 4.55, with 95% CI: 1.68-12.34) and patients without surgery vs. with surgery (P = 0.005, HR = 25.49, with 95% CI: 2.62-247.57). Clear survival differences exist between benign and malignant SFT. While surgery appears to be the best treatment option for benign and malignant SFT, better systemic therapies are needed to improve outcomes of patients with metastatic, malignant SFT.

摘要

孤立性纤维瘤(SFT)是一种起源于纤维组织的间叶性肿瘤。2013年世界卫生组织软组织肿瘤分类将恶性形式定义为细胞丰富、有丝分裂活跃(>4个有丝分裂/10个高倍视野),伴有细胞学异型性肿瘤坏死和/或浸润性边缘。通过一项经机构审查委员会批准的方案,我们调查了肉瘤数据库中的患者记录和临床病理数据,以描述良性和恶性SFT的临床特征。所有病理标本均由两位病理学家进行复查。进行了描述性统计和单因素/多因素生存分析。使用患者记录和社会保障死亡指数评估生存状态。82例患者中,47例(57%)为女性,73例(89%)为白种人。中位年龄为62岁(范围20至89岁)。32例(39%)患者死于该病。原发肿瘤部位为肺/胸膜28例(34%),腹部/骨盆23例(28%),四肢13例(16%),头颈部9例(11%)。病理检查42例(51%)为良性,40例(49%)为恶性。与良性SFT相比,恶性组织学与更大的肿瘤大小、更高的有丝分裂计数、诊断时的转移性疾病以及更多地使用化疗和放疗相关。良性和恶性亚型之间的性别、年龄和肿瘤部位无显著差异。单因素分析显示,只有良性与恶性变体及完整切除对总生存有积极影响(分别为P=0.02和P<0.0001)。在总生存的多因素分析中,接受化疗与否及是否接受手术是与总生存失败率显著相关的两个变量:接受化疗与未接受化疗的患者(P=0.003,HR=4.55,95%CI:1.68-12.34),未接受手术与接受手术的患者(P=0.005,HR=25.49,95%CI:2.62-247.57)。良性和恶性SFT之间存在明显的生存差异。虽然手术似乎是良性和恶性SFT的最佳治疗选择,但需要更好的全身治疗来改善转移性恶性SFT患者的预后。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7aa9/4607370/354db81f3a6f/pone.0140362.g001.jpg

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