质子重离子放射治疗在腹膜后肉瘤治疗中的价值

<正>软组织肉瘤主要是指一大类源于间叶组织的肿瘤,发病约占全身恶性肿瘤的1%。手术是腹膜后肉瘤（retroperitoneal sarcomas, RPS）的主要治疗方法,但因解剖的复杂性和多样性,即使达到肿瘤完整切除,45%～86%的病人仍会出现局部复发。放射治疗（放疗）在RPS中的作用目前尚未明确,缺乏高级别循证医学证据的支持。     

重视腹腔及腹膜后肉瘤的诊治

腹腔及腹膜后肉瘤是临床上最难处理的肿瘤之一,正如法国肉瘤专家Bonvalot在2011年雅典国际会议上强调,肉瘤外科医师是最伟大的,因为需涉及多脏器、高复杂性的肿瘤,所以要求肉瘤外科医师具有全面的手术技巧及胆识。目前,腹腔肉瘤进展最为显著的是胃肠道间质瘤(GIST),涉及分     

腹膜后肉瘤的外科处理及影响预后的因素

软组织肉瘤有 1 0 %～ 1 5%原发于腹膜后。尽管辅助治疗对肢体肉瘤有一定疗效 ,但迄今化学治疗对腹膜后肉瘤无效 ,而放射线因对胃肠道和神经的损伤限制了足够放射剂量 ,故放射治疗的效果亦较差 ,因此积极的外科手术对腹膜后肉瘤显得尤为重要 ,只有完全切除肉眼可见的肿瘤组织才能为患者长期存活提供机会。但要达到完全切除肿瘤而又不切除部分周围组织或器官则很困难 ,甚至不可能。本文就此探讨腹膜后软组织肉瘤的外科处理和影响生存的主要预后因素。一、处理方法1 .手术治疗手术切除是治疗腹膜后软组织肉瘤的主要手段。手术治疗前需判断肿瘤…     

腹膜后肿瘤外科发展的历史、现状与前景展望

<正>随着近年来腹膜后肿瘤（retroperitoneal tumor,RPT）发病率和死亡率的持续攀升,越来越多外科医师通过技术的改进、理念的更迭,多学科合作等方法来持续改善病人预后,并不断成立了专门的RPT亚专科、学会以及团体,使得RPT外科在近20年来的发展速度远超过去百年。然而,RPT仍属罕见病,病理类型多达百余种,80%以上为恶性的腹膜后肉瘤（retroperitoneal sarcoma,RPS）亚群。     

腹膜后脂肪肉瘤的治疗进展

脂肪肉瘤是一种少见但复发率较高的恶性肿瘤,最常见于四肢,腹膜后间隙次之,约占成人腹膜后软组织肉瘤的40%[1].腹膜后脂肪肉瘤(retroperitoneal liposarcomas,RPLS)男女发生比例大致相等,发病年龄跨度较大,但多在40～60岁之间.由于腹膜后间隙较大,位置隐蔽,多数患者早期缺乏特异性症状和体征,就诊时肿瘤往往长得很大,给手术带来很大的困难.诊断主要依靠体格检查和影像学检查,穿刺活检并未为临床所推崇,因其可增加肿瘤沿针道种植、播散的风险[2].手术完整切除仍是RPLS的标准治疗方式.对于未能完整切除及高复发风险者,可选择结合放化疗的综合治疗,但其效果目前仍存在争议.     

三维可视化重建辅助下诊治腹膜后巨大脂肪肉瘤1例报告

正腹膜后肿瘤由于位置深在,通常起病隐匿,发现时瘤体多已巨大,往往造成周围器官的受累~([1])。腹膜后脂肪肉瘤是最常见的腹膜后肿瘤,约占原发性腹膜后软组织肉瘤的45%~([2])。近年来三维可视化重建技术已广泛应用于肝脏外科等领域,腹膜后肿瘤由于其与周围组织密度差不显著以及该技术自身局限性等原因应用较少。哈尔滨医科大学附属第二医院近期在该技术辅助下成功诊治了1例腹膜后脂肪肉瘤病人。现报告如下。     

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目的 对原发性腹膜后平滑肌肉瘤的外科治疗进行总结。方法 １９７０￣１９９７年收治原发性腹膜后平滑肌肉瘤３３例,就其临床表现、辅助检查和外科治疗进行总结分析。结果 临床表现多为腹痛及腹部包块,辅助检查以Ｂ超及ＣＴ诊断率最高。肿瘤完全切除率为４２．４％,肿瘤完全切除组２年内复发９例,７例为局部复发,再手术８例,３例完全切除肿瘤。结论 影像学检查尤其是Ｂ超及ＣＴ是诊断本病的主要手段,手术完全切除肿瘤是治     

化疗在晚期腹膜后肉瘤治疗中的临床应用

腹膜后软组织肉瘤(retroperitoneal soft tissuesarcoma,RPS)是腹膜后恶性肿瘤的主要类型,女性略少于男性,40～70岁的病人多见,病理类型以脂肪     

腹腔及腹膜后软组织肿瘤的外科治疗:共识与争议

<正>腹膜后软组织肉瘤(retroperitoneal sarcoma,RPS)占所有软组织肉瘤的10%~15%,在美国其年发病率约为0.27/10万~([1-2]),目前无确切的关于中国患者的流行病学数据。迄今为止(至2016年1月),未发现该疾病的发病特征在不同的地区、人种及性别间存在显著的差异。从空间概念上,RPS系指原发于腹膜后间隙、不包括原发于腹膜后实质脏器如     

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外科手术是治疗原发性腹膜后肿瘤的重要手段 ,但切除后的复发率较高 ,达 4 9%～ 88% [1] 。多数原发性腹膜后肿瘤恶性程度较低 ,对于原发性腹膜后肿瘤术后复发者仍应积极争取再手术治疗。1　原发性腹膜后肿瘤复发的原因腹膜后肿瘤大多恶性程度较低 ,多数为局部复发或种植转移 ,较少远处转移。复发的病例中以脂肪肉瘤为多见 ,国内田氏[2 ] 报告 34例中 ,脂肪肉瘤占 16例。蔡氏[3 ] 报告33例复发的肉瘤中脂肪肉瘤也为 16例 ,其次为平滑肌肉瘤、恶性纤维组织肉瘤、神经纤维肉瘤、恶性间皮瘤等。原发性腹膜后肿瘤术后复发的原因主要有 :(1)首次…     

Retroperitoneal tumors: Review of diagnosis and management

Retroperitoneal tumors are extremely rare tumors occurring in the retroperitoneum. Retroperitoneal tumors are divided into benign tumors and malignant tumors, including retroperitoneal sarcoma. Approximately 70–80% of primary retroperitoneal soft-tissue tumors are malignant; however, these only account for 0.1–0.2% of all malignancies. Retroperitoneal sarcoma is an orphan malignant disease with a low incidence. The information on benign retroperitoneal tumors is limited. The American Joint Committee on Cancer/TNM classification updated to the 8th edition in 2017. In 2010, three new drugs for soft tissue sarcoma were approved based on the results of phase III trials, but the histological subtypes of the patients enrolled in the trials of each drug differed. Recently, in addition to surgery for retroperitoneal sarcoma, the effectiveness of perioperative radiation therapy has become interesting. For malignant retroperitoneal tumors and retroperitoneal sarcoma, survival improvement and locoregional recurrence prevention can be undertaken by carrying out surgery to secure negative margins with wide and combined resection of some adjacent organs, and cooperation with a trained medical team comprising of radiologists, pathologists and medical oncologists in centralized hospitals. Some clinical trials aimed at further improving treatment results by adding preoperative chemotherapy and radiation therapy based on histological confirmation using a correct needle biopsy are in progress. In recent years, molecular profiling has been used to select eligible patients for chemotherapy. In the future, precision medicine with next-generation sequencing technology will be expected among the diverse and potential future treatments for retroperitoneal sarcoma. In this review, we summarized the current state of retroperitoneal tumors and retroperitoneal sarcoma.     

Retroperitoneal sarcoma: Time for a change in attiude?

Background : Retroperitoneal sarcoma (RPS) is considered a disease with poor prognosis partly because of the difficulty with diagnosis at an early stage. This review assesses the current best practice principles for RPS and finds evidence suggesting a better outlook for appropriately managed cases. Recommendations are made for improving diagnostic certainty before laparotomy and inappropriate transperitoneal biopsy occur. Methods : A critical review of the English language literature was conducted using MEDLINE software and searching the terms ‘retroperitoneal sarcoma’ alone or in combination with ‘prognosis’, ‘surgery’ and ‘adjuvant therapy’. Conclusions : Retroperitoneal sarcoma is a rare disease but when appropriately managed the disease‐free survival can be improved and may even approach that of extremity soft tissue sarcoma. One of the greatest barriers to improving outcome is the misinterpretation of clinical signs and an over‐reliance on ultrasound diagnosis in pelvic presentations, or misinterpretation of clinical signs and/or computer tomography (CT) scans in abdominal masses. Physicians referring patients with a retroperitoneal mass should consider more frequently the less common differential diagnoses of an abdominopelvic mass including retroperitoneal sarcoma. This is especially true in circumstances where there is a circumscribed, predominantly solid tumour, with clinical or radiological signs of vascular or rectal displacement, ureteric obstruction and/or classic renal rotational displacement. The more frequent use of CT scans with intravenous and oral contrast with referral prior to inappropriate transperitoneal biopsy is recommended. In atypical cases where preoperative biopsy is necessary, extraperitoneal routes are preferable. Complete en bloc surgical excision at the first laparotomy is the treatment of choice in RPS. Macroscopic clearance may necessitate resection of adjacent viscera, neurovascular structures or abdominopelvic walls but, if achieved, may lead to long‐term survival depending on individual tumour biology.     

Retroperitoneal Sarcoma: Is It Time to Change the Surgical Policy?

Background  

Complete tumor resection is the mainstay of treatment for retroperitoneal sarcoma (RPS), but the size and quality of surgical margins for radical resection in RPS are unknown. They are believed to be pushing tumors, but recently, aggressive surgical policies leading to multivisceral resection have seemed to suggest better local control compared with simple tumor resection. We analyzed a single-institution series of RPS to provide information useful to surgical decision-making.     

Giant Retroperitoneal Liposarcoma- Renal Salvage by Autotransplantation

Liposarcomas are the most common retroperitoneal soft tissue tumors. We here in report a case of giant retroperitoneal sarcoma which weighed 24 kgs. Renal autotransplant of right kidney was done for organ preservation. The patient has done well after a follow up of 63 months. Complete surgical resection with organ preservation is the goal of treatment in patients with retroperitoneal liposarcomas.     

Combined modality treatment of extremity soft tissue sarcomas

Background: Substantial progress has been made in the multimodality treatment of soft tissue sarcoma over the past decade. This review summarizes current state-of-the-art management for patients with extremity soft tissue sarcoma. Methods: Literature-based review of combined modality treatment of extremity sarcoma. Results: The revised staging system of the International Union Against Cancer/American Joint Committee on Cancer (UICC/AJCC) for this disease is described and a practical approach to diagnosis, evaluation, management, and follow-up of patients is presented. Brief summaries of the roles of surgical therapy, pre- and postoperative radiotherapy, pre- and postoperative chemotherapy, and concurrent chemoradiation in the management of extremity soft tissue sarcoma are provided, with summaries of the available data from randomized prospective trials. The data referenced and discussed herein provide the basis for stage-specific treatment recommendations for the management of patients with localized soft tissue sarcoma of the extremities. Conclusions: Soft tissue sarcoma remains a challenging disease to treat. The majority of patients require an integrated combined modality approach. Future research should be directed at developing more effective systemic therapies and minimizing the toxicities of local therapies.     

Initial results of a trial of preoperative external-beam radiation therapy and postoperative brachytherapy for retroperitoneal sarcoma

Background Surgical resection alone does not cure the majority of patients with retroperitoneal sarcoma (RPS). We evaluated the effects of preoperative external-beam radiotherapy (XRT) and postoperative brachytherapy (BT) combined with complete surgical resection. Methods Fifty-five patients with primary or locally recurrent RPS judged to be resectable were entered onto a trial of combined therapy and observed prospectively. Forty-six patients underwent complete gross resection with curative intent. Of these, 41 patients completed preoperative XRT and 23 patients received BT. Outcome measures were treatment toxicity, overall survival, and disease-free survival (DFS). Results Preoperative XRT was very well tolerated and was associated with Radiation Therapy Oncology Group acute toxicity scores of ≤2 in all patients. Acute postoperative and BT-related toxicity resulted in modified RTOG scores of ≥3 in 39.1% (18 of 46) of patients. Late toxicity was associated with death in 4.3% (2 of 46) and with life-threatening illness in 2.2% (1 of 46) of patients, all of whom had been treated with BT to the upper abdomen. The 2-year overall survival and DFS for resected RPS were 88% and 80%, respectively. Significantly better 2-year DFS was achieved in patients with primary RPS and in those with low-grade tumors (93% and 95%, respectively). Conclusions The initial results of combined therapy are promising. Although preoperative XRT was very well tolerated, BT to the upper abdomen was associated with substantial toxicity. Our current protocol includes selective application of BT to the lower abdomen only. Presented at the 54th Annual Cancer Symposium of the Society of Surgical Oncology, Washington, DC, March 15–18, 2001.     

Effect of resection and outcome in patients with retroperitoneal sarcoma

BACKGROUND: A consecutive series of 47 patients with retroperitoneal sarcoma (RPS) were resected and prospectively followed. METHOD: Between July 1994 and March 2005, 47 patients (24 men, 23 women; mean age, 56 years; range, 17-82 years) were evaluated. RESULTS: A total of 23 patients had primary RPS and 24 patients had recurrent RPS. A total of 30 out of 47 patients (64%) underwent removal of contiguous intra-abdominal organs. The peroperative mortality was nil and significant preoperative complications occurred in eight cases only (17%). High tumour grade and incomplete resection were significant variables for a worse survival in all 47 patients, both in the univariate and multivariate analyses (P = 0.008 and P = 0.016, respectively). Among 28 radically resected patients, only histological grade affected overall survival (90% 5-year survival for low-grade tumour vs 26% 5-year survival for high-grade tumour; P = 0.006) with a similar effect noted for disease-free survival. CONCLUSIONS: Histological grade was the only factor that affected overall and disease-free survival for RPS tumours. An aggressive surgical approach in both primary and recurrent RPS is associated with long-term survival.     

Malignant mesenchymoma of the pleura

Malignant mesenchymomas are rare soft tissue tumors of mesenchymal origin. We report a case of pleural localization with liposarcomatous, leiomyosarcomatous and osteocartilaginous elements. The treatment associated surgical resection, chemotherapy and radiotherapy. Sixteen months after the diagnosis a metastatic retroperitoneal sarcoma with osteosarcomatous differentiation appeared without any sign of thoracic recurrence. A surgical resection was performed, but a rapid retroperitoneal recurrence led to death.     

The importance of the margin of resection and external radiation in non-lipomatous retroperitoneal sarcoma

BackgroundPrior studies evaluating the impact of adjuvant or neoadjuvant radiation on clinical outcomes of patients with non-lipomatous retroperitoneal sarcoma have been underpowered.MethodsWe queried the National Cancer Database to identify patients undergoing surgical resection of retroperitoneal sarcoma with non-lipomatous histology from 2004 to 2016. Multivariable logistic regression and Cox proportional hazards modelling with patients stratified by tumor size were used to identify factors associated with overall survival.Results3,394 patients met inclusion criteria. 592 had small (<5 cm), 1,186 had intermediate (5–10 cm), and 1,616 had large (>10 cm) tumors. Use of either neoadjuvant or adjuvant radiotherapy was associated with improved survival for patients with intermediate (neoadjuvant HR 0.67, CI [0.46, 0.98]; adjuvant HR 0.61, CI [0.50, 0.76]) and large (neoadjuvant HR 0.50, CI [0.37, 0.68]; adjuvant HR 0.56, CI [0.47, 0.69]) tumors, while adjuvant radiation therapy was associated with a survival benefit for small-sized tumors (HR 0.67, CI [0.46, 0.99]).ConclusionsRadiation therapy is associated with an overall survival benefit in patients presenting undergoing resection of non-lipomatous retroperitoneal sarcoma.     

The importance of the margin of resection and radiotherapy in retroperitoneal liposarcoma

BackgroundPrior studies evaluating the impact of adjuvant or neoadjuvant radiotherapy on clinical outcomes in retroperitoneal liposarcoma have been underpowered.MethodsWe queried the National Cancer Database for patients undergoing resection of retroperitoneal liposarcoma from 2004 to 2016. Cox proportional hazards modeling stratified by tumor size was used to identify factors associated with overall survival.Results4018 patients met inclusion criteria. 251 had small (<5 cm), 574 intermediate (5–10 cm), and 3193 large (>10 cm) tumors. Positive surgical margins were correlated with risk of death across all tumor size categories (<5 cm HR 2.33, CI [1.20, 4.55]; 5–10 cm HR 1.49, CI [1.03, 2.14]; >10 cm HR 1.30, CI [1.12, 1.51]). Adjuvant radiotherapy was associated with improved survival for patients with large tumors only (HR 0.75, CI [0.64, 0.89]).ConclusionsIn retroperitoneal liposarcoma, adjuvant radiation is associated with improved survival only for patients with tumors larger than 10 cm. Radiation should be used sparingly in patients with smaller tumors.SummaryThe use of radiotherapy in the management of retroperitoneal sarcoma remains controversial. We isolated retroperitoneal liposarcomas only and identified a survival benefit from radiotherapy treatment only in tumors larger than 10 cm and only in the adjuvant setting.