Sourrouille Isabelle, Vilcot Laurence, Honoré Charles, Coppola Sara, Terrier Philippe, le Cesne Axel, Le Péchoux Cécile, Bonvalot Sylvie
1 Department of Surgical Oncology, Institut Gustave Roussy, Villejuif, France 2 Department of Radiology, Institut Gustave Roussy, Villejuif, France 3 Department of Pathology, Institut Gustave Roussy, Villejuif, France 4 Department of Medical Oncology, Institut Gustave Roussy, Villejuif, France 5 Department of Radiotherapy, Institut Gustave Roussy, Villejuif, France.
Dis Colon Rectum. 2015 Mar;58(3):304-13. doi: 10.1097/DCR.0000000000000272.
Perineal soft tissue tumors are rare, so that little is known about their management and the outcome of treatment.
The aim of this study is to describe the presentation, management, and outcome of the surgical treatment of soft tissue tumors and to provide a final decision algorithm.
This is a retrospective study.
The study was conducted in a single tertiary care hospital with a dedicated unit on sarcoma.
Fifty-one consecutive patients from 1998 to 2013 were included.
The primary outcomes measured are patient demographics, treatment decisions, and outcome of surgical treatment.
Forty-nine patients presented with a primary soft tissue tumor, and 2 underwent simple excisions for isolated metastases. The median tumor size was 75 mm (50-110). Symptoms were nonspecific, and MRI had insufficient specificity for malignancy so that a preoperative biopsy was systematically performed according to European Society for Medical Oncology and National Comprehensive Cancer Network soft tissue tumor guidelines. Six benign soft tissue tumors (3 lipomas, 3 leiomyomas), 16 intermediate soft tissue tumors (12 aggressive angiomyxoma, 4 desmoid tumors), and 27 sarcomas were identified. Treatments and surgery were tailored from the beginning according to histology. All but 1 benign soft tissue tumor were treated by 'shelling out.' Aggressive angiomyxoma were treated with en bloc resection sparing uninvolved organs. Nonsurgical treatments were our first choice for desmoid tumors. Wide en bloc surgery was planned for all sarcomas (n = 27) after the induction treatment for 16 patients (chemotherapy, n = 12; radiotherapy, n = 4). In the sarcoma group, the 5-year estimated metastasis-free, local recurrence-free, and overall survival rates were 68.1% (95% CI, 50.7-91.5), 84.7% (95% CI, 66.7-100), and 85.7% (95% CI, 71.8-100). In the benign and intermediate tumor groups, there were no deaths, local recurrences, or progression.
This study was limited by the small number of patients, given the rarity of this disease in the perineum.
We provide useful indications for the best strategy necessary to treat these rare tumors located in a complex site.
会阴部软组织肿瘤较为罕见，因此对其治疗及治疗结果了解甚少。
本研究旨在描述软组织肿瘤手术治疗的表现、治疗方法及结果，并提供最终决策算法。
这是一项回顾性研究。
该研究在一家设有肉瘤专科病房的三级护理医院进行。
纳入了1998年至2013年连续的51例患者。
所测量的主要结果为患者人口统计学资料、治疗决策及手术治疗结果。
49例患者表现为原发性软组织肿瘤，2例因孤立性转移瘤接受了单纯切除术。肿瘤中位大小为75mm（50 - 110）。症状不具特异性，MRI对恶性肿瘤的特异性不足，因此根据欧洲医学肿瘤学会和美国国立综合癌症网络软组织肿瘤指南系统地进行了术前活检。共识别出6例良性软组织肿瘤（3例脂肪瘤、3例平滑肌瘤）、16例中间型软组织肿瘤（12例侵袭性血管黏液瘤、4例硬纤维瘤）和27例肉瘤。治疗及手术从一开始就根据组织学进行了调整。除1例良性软组织肿瘤外，所有肿瘤均采用“剜除术”治疗。侵袭性血管黏液瘤采用整块切除，保留未受累器官。非手术治疗是硬纤维瘤的首选治疗方法。16例患者（化疗12例，放疗4例）在诱导治疗后，计划对所有肉瘤（n = 27）进行广泛整块手术。在肉瘤组中，5年无转移、无局部复发及总生存率估计分别为68.1%（95%CI，50.7 - 91.5）、84.7%（95%CI，66.7 - 100）和85.7%（95%CI，71.8 - 100）。在良性和中间型肿瘤组中，无死亡、局部复发或病情进展。
鉴于会阴部这种疾病罕见，本研究受患者数量少的限制。
我们为治疗这些位于复杂部位的罕见肿瘤所需的最佳策略提供了有用的指导。
