腹膜后脂肪肉瘤的多学科治疗现状

腹膜后脂肪肉瘤是腹膜后间隙的恶性间叶源性肿瘤,其发病率较低,病因和发病机制尚未明确,影像学和病理学表现多样,恶性程度高低不等。目前腹膜后脂肪肉瘤主要依赖手术切除,但复发率高,复发速度快,严重影响预后。近年来放疗、化疗、靶向治疗等手段开始应用,免疫治疗等新理念也逐渐兴起,形成了以手术为主体、多学科治疗相结合的综合治疗体系,现对腹膜后脂肪肉瘤的多学科治疗现状予以综述。      

Recent progress in the management of retroperitoneal sarcoma

Retroperitoneal sarcomas (RPS) are rare tumours that typically present late and carry a poor prognosis even following grossly complete resection. In an attempt to improve the outlook for patients with RPS, sarcoma specialists have employed various adjuvant therapies, including extermal beam radiation, intraoperative radiation, brachyradiation and systemic chemotherapy. This article reviews the presentation and prognosis of RPS, and focuses on the results of new treatment strategies compared with conventional management.A Medline search of the English literature was performed to identify all retrospective and prospective reports relating to the management of adult RPS published since 1980. Series that did not analyse RPS separately from other intra-abdominal or extra-abdominal sarcomas or other malignancies were excluded, and information on investigation, presentation, prognostic factors, treatment and outcome was extracted from the remaining reports. Survival and local control data were collected from reports that contained at least 30 cases of RPS (n = 31).While surgical resection remains the cornerstone of treatment for RPS, the majority of patients will relapse and die from sarcoma within 5 years of resection. Adjuvant radiation may improve these results, but further trials are required to definitively demonstrate its benefit. Possible reasons for the failure of conventional treatment are discussed, and alternative strategies designed to overcome these obstacles are presented.     

A review of retroperitoneal liposarcoma genomics

Retroperitoneal liposarcomas are rare tumours that carry a poorer prognosis than their extremity counterparts. Within their subtypes – well differentiated (WDL), dedifferentiated (DDL), myxoid (MLS) and pleomorphic (PLS) - they exhibit a diverse genomic landscape. With recent advances in next generation sequencing, the number of studies exploring this have greatly increased. The recent literature has deepened our understanding of the hallmark MDM2/CDK4 amplification in WDL/DDL and addressed concerns about toxicity and resistance when targeting this. The FUS-DDIT3 fusion gene remains the primary focus of interest in MLS with additional potential targets described. Whole genome sequencing has driven identification of novel genes and pathways implicated in WDL/DDL outside of the classic 12q13-15 amplicon. Due to their rarity; anatomical location and histologic subtype are infrequently mentioned when reporting the results of these studies. Reports can include non-adipogenic or extremity tumours, making it difficult to draw specific retroperitoneal conclusions. This narrative review aims to provide a summary of retroperitoneal liposarcoma genomics and the implications for therapeutic targeting.     

38例腹膜后脂肪肉瘤的临床分析

目的 分析腹膜后脂肪肉瘤的治疗结果。方法 1958～1998年我院收治38例腹膜后脂肪肉瘤。21例为首程治疗,17例局部复发后再程治疗。21例初治患者均接受手术治疗,手术完整切除率为86％。3例接受术后放射治疗。17例外院术后复发再程治疗患者有13例在我院接受手术,4例接受单纯放疗。再程手术完整切除率为54％,1例接受术后放放射治疗。放疗剂量DT30Gy～70Gy,中位剂量50Gy。结果 全组患者5年总生存率为50％,5年远转率为7.3％,5年局部复发率为65％,初程治疗和再程治疗组的5年总生存率为53％和48％,无显著性差异(P=0.293,5年局部复发率为59％和74％,无显著差别(P=0.615)。全组手术完整切除组5年生存率为58％,部分切除或未切除者5年生存率为27％,有显著性差异(P=0.006)。34例有肿瘤大小记录的患者,其中4例肿瘤<10cm,均存活;22例肿瘤10～30cm,有13例死亡;8例肿瘤≥30cm,有6例死亡。结论 腹膜后脂肪肉瘤的治疗以手术为主,手术完整切除可显著提高生存率。经积极治疗,复发患者仍能取得很好的效果。     

腹膜后去分化脂肪肉瘤临床病理分析

目的 探讨腹膜后去分化脂肪肉瘤(dedifferentiated liposarcoma,DDL)的临床病理学特点.方法 回顾性分析16例腹膜后去分化脂肪肉瘤的临床表现、影像学资料、光学显微镜形态和免疫学表型,并对患者进行随访.结果 16例中为复发再次手术者10例.所有患者肿瘤均发生于腹膜后.CT能够清楚地显示肿瘤的生长范围及对周围脏器的推挤、浸润,影像学具有一定的提示作用.镜下观察显示去分化脂肪肉瘤含有分化性成分和去分化成分,去分化成分可表现为纤维肉瘤样、恶性纤维组织细胞瘤样、恶性外周神经鞘瘤样、平滑肌肉瘤样、横纹肌肉瘤样、血管肉瘤样、血管外皮瘤样等.肿瘤可以以一种成分为主,也可以几种成分同时存在,免疫表型表达各异.16例患者均通过手术治疗,其中9例为单纯肿物切除,7例同时切除了肿物及周围粘连的脏器.随访至2012年1月(随访3-24月)均无复发及转移.结论 去分化脂肪肉瘤中的去分化成分表现各异,低级别和高级别区域可在同一肿瘤内并存.对于复发病例仍然应该争取再次手术切除,以缓解症状,延长生存期.     

Pancreaticoduodenectomy in the surgical management of primary retroperitoneal sarcoma

Background

In retroperitoneal sarcoma (RPS), the optimal extent of resection must balance adequate disease control with potential for morbidity. We sought to study the frequency and outcomes after a Whipple procedure or pancreaticoduodenectomy (PD) in patients undergoing resection for primary RPS.

Optimal management of primary retroperitoneal sarcoma: an update

Soft tissue sarcomas are a group of heterogeneous neoplasms with more than 50 histological subtypes exhibiting major differences in terms of pathogenesis, genetic alterations and clinical behavior. Sarcomas represent approximately 1% of malignancies with retroperitoneal sarcomas representing 10–15% of all soft tissue sarcomas. Surgery is currently the only modality which offers the chance of cure. Surgery for retroperitoneal sarcomas presents specific challenges due their location in a complex space surrounded by vital structures and visceral organs often prohibiting resection with wide margins. Furthermore, even after complete resection local recurrence is common and the leading cause of death. In this article the authors describe the initial investigations, prognostic factors and optimal surgical management. The evidence and current research as regards the role of multimodality treatment is reviewed and discussed.     

Rare synchronous primary large B-cell gastric lymphoma and huge retroperitoneal liposarcoma with inguinal hernia in chronic hepatitis B patient

Multiple primary neoplasms with synchronous or metachronous presentation are rare, although the incidence has recently increased because of several factors. We present the case of a 53-year-old patient with chronic hepatitis B who presented with abdominal mass, mild abdominal pain, and inguinal hernia. Computed tomography imaging demonstrated diffuse thickening of the gastric antral wall, together with a huge heterogeneous abdominal mass with predominant fat attenuation with septa that showed mild enhancement on contrast-enhanced scans. Distal gastrectomy and wide resection of the retroperitoneal mass was performed. Pathology exam led to a diagnosis of diffuse large B-cell gastric lymphoma with retroperitoneal liposarcoma. This is a rare case of a primary gastric lymphoma with another primary (sarcomatous) malignancy occurring synchronously in same patient.     

Follow-up of patients with retroperitoneal sarcoma

Retroperitoneal sarcomas (RPS) are rare malignancies that are potentially curable by complete surgical resection. A regular surveillance program is normally commenced following surgery due to the risk of local recurrence (LR), especially in low-intermediate grade disease, and distant metastases (DM), especially in high-grade RPS. Consensus guidelines usually advocate for more frequent imaging during the first 2–3 years and less intensive imaging over a prolonged period thereafter, reflecting the incidence pattern of LR and DM. Definitive evidence for the most effective imaging schedule has never been provided, and retrospective studies have not shown an association between follow-up intensity and survival. Improvement in the prediction of recurrence patterns has been sustained by prognostic dynamic nomograms, which are now capable of forecasting disease behaviour in each patient according to specific features. Incorporation of such tools in clinical practice may help to stratify patients and tailor ongoing surveillance to the risk of recurrence. This may help to relieve patients’ anxiety while awaiting results of surveillance investigations, and also reduce the economic and environmental burden of repeated imaging. A randomized controlled study (SARveillance Trial) is proposed to shed light on this controversial topic, allowing clinicians to harmonize the follow-up protocol of patients undergoing surgery for RPS.     

The management of retroperitoneal soft tissue sarcoma: a single institution experience with a review of the literature.

AIM: Ten percent of soft tissue sarcomas (STS) arise in the retroperitoneal tissues. The prognosis for patients with retroperitoneal sarcoma is poor with a 5-year survival rate between 12% and 70%. Stage at presentation, high histological grade, unresectable primary tumour and incomplete resection are associated with a less favourable outcome. METHODS: Complete follow-up data were available on 22 patients who underwent surgery for retroperitoneal STS in our institution between 1990 and 2000. Patient, tumour and treatment variables were analysed including use of adjuvant therapy and survival status. RESULTS: Eighteen patients underwent surgery for primary disease, four patients were treated for recurrent disease or metastases. Ten patients presented with pain, seven with an abdominal mass, other presentation included weight loss and haematuria. Thirteen patients presented with tumours larger than 10 cm. The tumours were seven liposarcomas, six leiomyosarcomas, three malignant fibrous histiocytomas, two rhabdomyosarcomas, two malignant schwannomas and two undifferentiated sarcomas. Six primary tumours were completely excised, five patients received radiotherapy and five received chemotherapy. Local recurrence rate was 45% and recurrence-free interval for 10 patients with recurrence was 11 months. Five patients received radiotherapy and five received chemotherapy. The median survival for patients with primary tumours was 36 months, and 5-year survival was 44%. Adjuvant therapy was not associated with higher survival rates. CONCLUSION: This study re-emphasizes the poor outcome of patients with retroperitoneal STS. Adjuvant radiotherapy and chemotherapy do not appear to be any proven benefit and the single most important prognostic factor is aggressive successful en bloc resection of the primary tumour. Our resection rate and 5-year survival rates are comparable with previous reported UK series although lower than large reports from North American centres. This might partly be explained by difficulty in data collection in a retrospective analysis, but may reflect inadequate subspecialization in UK centres. Copyright Harcourt Publishers Limited.     

原发性腹膜后脂肪肉瘤的诊断与治疗

目的 探讨原发性腹膜后脂肪肉瘤(primary retroperitoneal liposarcoma,PRPL)诊断与手术治疗情况,分析其影响复发的因素.方法 回顾性分析1995～2010年中山大学附属第一医院收治的23例原发性腹膜后脂肪肉瘤患者的临床病理资料,分析其术后复发情况.结果 PRPL临床表现主要为腹部包块进行性增大、腹胀.所有患者均接受剖腹探查手术治疗.23例患者共进行手术5l例次,首次手术肿瘤肉眼完整切除19例(82.6%),部分切除2例(8.7%),2例仅行术中单纯活检(8.7%),联合脏器切除14例.术后中位复发时间为22.1个月,术后2年复发率达61.9%.患者术后复发与肿瘤大小、联合脏器切除以及手术切缘阳性有关.多次复发者复发间期逐渐缩短.结论 PRPLS首次手术应争取完整切除肿瘤,复发常见,定期复查是诊断术后复发的重要手段,对于复发的病例仍应尽早手术治疗.     

Weekly paclitaxel therapy is curative for patients with retroperitoneal liposarcoma

In March 2004, we resected a giant retroperitoneal liposarcoma and the transverse colon, spleen and left kidney in a 58-year-old woman. In July, recurrence was detected in the right pelvis and left upper abdomen; therefore, we resected the tumor. In September 2004, computed tomography (CT) revealed multiple recurrences in the right lower abdomen, left upper abdomen, front of the left lobe of the liver, and at the back of the stomach. In October 2004, we started mesna, doxorubicin, ifosfamide, and dacarbazine therapy (MAID); however, after 1 course, the disease progressed, and the patient developed edema in the bilateral legs due to inferior vena cava (IVC) compression. In November 2004, we started weekly paclitaxel therapy (100 mg/m(2), once a week for 3 weeks followed by 1 drug-free week). CT revealed no change as a result of chemotherapy; however, IVC compression had improved, and leg edema had decreased. In August 2005, chemotherapy was stopped; therefore,the patient's condition worsened. She died in September 2005. We performed weekly paclitaxel therapy for the patient with recurrent liposarcoma. This improved her symptoms and quality of life (QOL). Therefore,we consider weekly paclitaxel therapy to be effective for liposarcoma treatment.     

腹膜后脂肪肉瘤CT诊断价值的研究

24例经手术确诊且CT检查资料完整的病例中,男10例,女14例。发病年龄45～78岁,平均年龄56岁,中位年龄58.5岁。其中发生于肾周围囊者13例,肾周围囊外11例。单发者22例,多发2例。肿瘤直径>10cm者23例,<10cm者1例。瘤体内含有脂肪组织密度者20例,其中10例呈均匀脂肪密度改变,9例可见脂肪组织与条片状或块状肌肉样组织混合存在,1例以肌肉样改变为主。肿瘤侵犯周围组织器官9例。回顾性分析结果显示,含有脂肪组织密度的巨大腹膜后肿块是诊断本病的可靠征象,CT扫描能准确地判断腹膜后肿瘤的大小、范围、术后复发及周围组织受侵程度等。     

腹膜后脂肪肉瘤的影像学和病理学分析

目的探讨不同组织学亚型腹膜后脂肪肉瘤的影像学和病理组织学特征。方法回顾性分析21例腹膜后原发性脂肪肉瘤的CT和MRI表现,按不同病理组织学亚型进行对照观察。结果21例腹膜后原发性脂肪肉瘤中,高分化11例,黏液性4例,圆形细胞性3例,多形性2例,去分化脂肪肉瘤1例。分化型脂肪肉瘤主要由脂肪瘤样和硬化性成分组成,脂肪瘤样成分的CT密度、MRI信号与脂肪成分相似,而硬化性成分与肌肉的密度和信号相似。黏液性脂肪肉瘤CT平扫密度和MRI信号同水相似,CT增强扫描表现为网状延迟强化。圆形细胞性和多形性脂肪肉瘤表现为无明显脂肪、但有灶性坏死的软组织肿块。去分化脂肪肉瘤表现为分化良好的脂肪瘤样成分与明显强化的软组织肿块成分,二者分界清楚。结论不同病理组织学亚型的脂肪肉瘤,其CT和MRI表现有所不同,这取决于肿瘤所含的主要组织学成分。了解肿瘤不同的组织学亚型与影像学表现之问的关系,有助于腹膜后脂肪肉瘤的准确诊断。     

The management of clear cell sarcoma.

Clear cell sarcoma is a rare soft tissue tumour, constituting approximately 1% of all soft tissue sarcomas. Prognosis is reported to be poor due to the great propensity to metastasise regionally and distantly. In this paper, we report the surgical experience of two university hospitals. Both disease-free and overall survival after resection of clear cell sarcoma in this limited study were excellent, which may be explained by relatively small tumour size in seven out of eight patients and adjuvant radiation treatment. The current treatment for clear cell sarcoma is wide local tumour excision, with adjuvant radiation therapy for resection margins of less than 1 mm.     

Diagnosis and treatment of primary and recurrent retroperitoneal liposarcoma

We retrospectively reviewed 16 patients in whom primary retroperitoneal liposarcoma was diagnosed and treated. These patients were classified according to the first definitive surgical procedure employed: complete excision with or without radiotherapy; partial excision followed by radiotherapy; only biopsy of tumor followed by radiotherapy. Distribution of tumors according to histologic type was: well differentiated myxoid type, 10; poorly differentiated myxoid type, 4; and mixed type, 2. The main factor in determining resectability was extent and fixation of tumor to vital structures. Recurrent retroperitoneal disease developed in four patients all of whom underwent secondary intervention. We concluded from our experience that complete surgical excision is the most effective primary therapeutic approach for patients with primary and recurrent retroperitoneal liposarcoma.     

Long-term prognosis of primary retroperitoneal soft tissue sarcoma.

AIMS: To evaluate the result of treatment and long-term outcome of a population-based cohort of patients with retroperitoneal soft tissue sarcoma (RSTS). METHODS: Between 1 January 1989 and 1 January 1994, 143 patients diagnosed as having primary RSTS were selected from a national pathology database (PALGA) in the Netherlands. In this population-based group of patients, the result of surgery, overall survival (OS) and disease-free survival (DFS) were analysed as well as factors affecting OS and DFS. Median follow-up was 10.2 years. RESULTS: Operative treatment resulted in a complete tumour resection in 55% of the patients (n=78), low- and intermediate-grade tumours were more often completely resected than high-grade tumours (P=0.016). Five- and 10-year cumulative OS was 39% and 21%, respectively, while DFS was 22% and 17%, respectively. In a multivariate analysis low malignancy grade (P=0.017) and a complete tumour resection (P<0.001) were associated with better OS. CONCLUSIONS: Complete tumour resection and low malignancy grade were independent favourable prognosticators. However, these factors were related too, since surgical success was influenced by malignancy grade.