腹膜后血管平滑肌肉瘤1例

患者男，73a。于2001年3月5日开始发热，体温37．5℃左右，轻微咳嗽，无咳痰、咳血、呼吸困难、胸痛、心慌等症状。按上呼吸道感染处理，疗效不佳。1mo后，仍间断发热，出现肺部湿罗音。胸片提示：慢性支气管炎。于2001年4月26日以慢性支气管炎、肺部感染入住老年病科。血常规检查正常，B超检查提示：脾脏肿大，其余未见异常。     

腹膜后肿瘤切除联合重要血管重建12例

背景与目的:腹膜后肿瘤的治疗,手术切除仍是惟一可能根治的有效方法.但临床确诊时,多数肿瘤发现较晚,常累及腹腔内重要血管,即属手术相对禁忌症.本研究旨在探讨累及腹腔内重要血管的腹膜后肿瘤切除的处理方法,以提高切除率及生存率.方法:回顾性分析了2003年1月-2007年6月我院外科手术治疗的12例腹膜后肿瘤累及腹腔内重要血管的患者,经手术切除并行人工血管重建术.结果:成功完成了12例腹膜后肿瘤及受累血管的完整切除和相应的重要血管重建术,术后无1例围手术期死亡.结论:累及腹腔内重要血管的腹膜后肿瘤不是根治性切除的手术禁忌症,联合重要血管切除并行血管重建手术是安全的,并可明显提高切除率,降低复发率,延长患者存活时间.     

腹膜后巨大血管肉瘤一例报告

腹膜后巨大血管肉瘤一例报告青海省人民医院（西宁市８１００００）李豫闽刘丽萍１病例报告患者，女性，２５岁。因发现腹部肿块１０个月，于１９９６年５月入院。患者１０个月前无明显诱因自感左大腿腹股沟部及左腰部酸痛不适，下腹部有一肿物进行性生长。入院时体检：一...     

复发性腹膜后软组织肉瘤的疗效分析

我们分析了局部复发性腹膜后软组织肉瘤３０例的外科治疗效果。行肿瘤完全切除者１８例（６０％），其中联合脏器切除６例，占３３％（６／１８）。全组总３年和５年生存率为３９％和１４％。完全切除者３年和５年生存率为４９％和２４％，而部分切除及探查活检者（１２例）为２５％和０％，两者比较差异显著（Ｐ＜０．０５）。结果表明完全切除复发肿瘤仍可获得长期疗效。并就肿瘤复发前无瘤间隙期、肿瘤大小、病理类型等因素对疗效的影响及其临床意义进行了探讨。     

原发性腹膜后副神经节瘤14例

回顾性分析14例原发性腹膜后副神经节瘤患者的临床表现、辅助检查、病理特征及外科治疗结果。结果示14例患者均以腹部包块就诊，所有病例均行手术完整切除。病理诊断良性者为11例，恶性3例。随访17例0．4～10年，2例死亡。该病对化疗、放疗均不敏感．外科手术是惟一确切疗效的治疗方法。术前应肠道：住备充分、备血，术中防止大出血、控制高血压，防止心跳骤停。     

原发性腹膜后肿瘤303例分析

原发性腹膜后肿瘤３０３例，有病理诊断２８８例，其中良性肿瘤９１例，占３１．６％；恶性肿瘤１９７例，占６８．４％。本病解剖部位特殊，发病隐蔽，症状无特异性，临床诊断困难，故强调影象学检查，尤其是Ｂ超和ＣＴ的诊断。不能切除的腹膜后恶性肿瘤预后极差，作者主张放宽剖腹探查指征，常规作术中冰冻切片检查。若是恶性肿瘤，则应尽可能完全或大部分切除，必要时可行受累脏器的联合切除术。肿瘤无法切除者，术后可用放疗或化疗，待肿瘤缩小，再争取手术切除。术后复发者，要争取再次、多次手术切除。若为良性肿瘤，则不应施行危及患者生命的冒险手术，不要勉强行肿瘤完全切除。     

腹膜后肿瘤手术治疗探讨

对２６例腹腊后肿瘤的治疗结果表明，腹膜后肿瘤所在产以及选择合适的手术径路，是肿瘤能否彻底切除的前提。对不能一期切除的较大恶性肿瘤，采取肿瘤的包膜内切除术或减量手术，可取得良好的临床效果。术后病人应缩短复查时间，争取复发时早期手术，并切除这受累器官。     

原发性腹膜后肿瘤36例

 目的 总结原发性腹膜后肿瘤的诊治经验。方法 回顾1996年12月至2006年12月经术后病理证实的36例原发性腹膜后肿瘤的临床特点、影像学检查及手术情况。结果 辅助检查超声诊断率90 %，CT诊断率95 %，MRI诊断率100 %。其中恶性肿瘤26例，完全切除率50 %，合并脏器切除率31 %；良性肿瘤10例，完全切除率80 %，合并脏器切除率10 %。术后复发6例，再手术5例，均完全切除，1例拒绝再手术，1年内死亡。结论 超声和CT检查是诊断该病的主要手段，手术完全切除是治疗的关键。对复发肿瘤应争取再次手术，达到延长生存的目的。     

腹膜后肿瘤32例诊治分析

由于腹膜后肿瘤发生部位的缘故，使完全切除肿瘤有一定难度。作者分析了32例腹膜后肿瘤的诊治情况，认为充分的术前检查，良好的术前准备及仔细的术中分离操作，是提高手术成功率的关键。     

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.

EJSO educational Special issue from the TARPSWG - Standard medical treatment and new options in retroperitoneal sarcoma

Retroperitoneal soft tissue sarcomas mainly consist histologically of liposarcomas and leiomyosarcomas. For the liposarcoma subgroup, the local relapse rate seems to determine patients' overall prognosis. In contrast, leiomyosarcoma patients are challenged by the development of metastatic disease; therefore, effective systemic therapies are the cornerstone to improve patients’ outcome. No doubt, the limited number of active regimens currently available makes the treatment of patients with locally advanced and/or metastatic disease challenging and results in the overall poor prognosis of this population. In this European Journal of Surgical Oncology Educational Special Issue from the Transatlantic Australasian RetroPeritoneal Sarcoma Working Group (TARPSWG), we aim to summarize state-of-the-art systemic treatments for patients with retroperitoneal sarcomas with a focus on the locally advanced and metastatic disease setting including conventional standard chemotherapies as well as new innovative treatment approaches in order to identify current unmet medical needs guiding the sarcoma community to initiate appropriate translational research projects and design innovative clinical trials.     

Treatment for local control of retroperitoneal and pelvis sarcomas: A review of the literature

Retroperitoneal and pelvis sarcomas are uncommon tumors for which complete surgical resection is the mainstay of treatment. However, achieving complete gross resection with microscopically negative margins is challenging, and local recurrence rates can be high. Patients often succumb to uncontrolled local disease. Radiation therapy offers a potential means for sterilizing microscopic residual disease, although its use continues to be controversial. Chemotherapy alone or in combination with radiation continues to be investigated as an adjunct to surgery, along with immunotherapy and targeted therapies. In this review, we discuss the current management of retroperitoneal and pelvis sarcomas, focusing on studies of surgery and radiation therapy to maximize local control.     

The impact of postoperative radiological surveillance intensity on disease free and overall survival from primary retroperitoneal,abdominal and pelvic soft-tissue sarcoma

AimThis observational study aimed to evaluate the impact of intensity of radiological surveillance on survival following resection of retroperitoneal sarcoma.MethodRetrospective cohort study of patients undergoing primary resection of soft tissue sarcoma arising in the retroperitoneum, abdomen or pelvis at a single, high-volume sarcoma centre. Intensity of follow-up regimes up to 5 postoperative years were categorized as ‘European Society for Medical Oncology (ESMO) compliant’ (intense), or ‘non-ESMO compliant’ (less-intense). The primary outcome measure was overall survival (OS). The secondary outcome measures were disease-free survival (DFS) and reoperation rate. Analyses were stratified by high (grade 2 or 3) or low (grade 1) tumour grade.ResultsOf 168 patients, 67.1% had high-grade and 32.9% had low-grade disease. Overall, 40.0% of patients had ESMO-compliant radiological follow-up (high-grade:25.7%, low-grade:66.7%). 41.7% of patients died and 48.2% suffered local or distant recurrence by cessation of follow up. Upon univariable analysis for high-grade tumours, ESMO compliance reduced DFS (p = 0.066) but had no impact on OS. There was no significant difference in the reoperation rate in patients with ESMO-compliant and non-compliant follow-up (p = 0.097). In low-grade tumours, ESMO compliance significantly reduced DFS (p < 0.001), but without effecting OS. In risk-adjusted models for high-grade tumours, ESMO compliant follow-up was associated with reduced OS (HR:3.47, 1.40–8.61, p = 0.007) and no difference in DFS. In low-grade tumours, there was no association between overall ESMO compliance and OS or DFS.ConclusionThis study did not find a benefit for high-intensity radiological surveillance and overall survival in patients undergoing primary resection for high or low-grade retroperitoneal sarcoma.     

New developments in the pathology and molecular biology of retroperitoneal sarcomas

Retroperitoneal sarcomas (RPS) refer to a heterogeneous group of malignancies of mesenchymal origin developing from retroperitoneal tissues and vessels. The most frequent RPS are well differentiated/dedifferentiated liposarcomas and leiomyosarcomas, but other rare histological subtypes can be observed. Over the last decade, significant advances have been made in the pathological and molecular characterization of sarcomas. These advances have led to major changes in their diagnostic management as well as in the development of new therapeutic strategies based on tumor biology and microenvironment. This review describes the current knowledge and recent findings in the pathology and molecular biology of the most frequent RPS subtypes.     

Major vascular reconstruction in colorectal adenocarcinoma and retroperitoneal sarcoma: A retrospective study of safety and margins in a tertiary referral centre

BackgroundThe role of en bloc vascular resection and reconstruction (EVRR) is controversial in colorectal adenocarcinoma (CRC), but well-established in retroperitoneal sarcoma (RPS). Sparse data exists regarding these complex procedures.MethodsPatients undergoing curative intent EVRR for advanced CRC and RPS between 2014 and 2021 at a tertiary centre were included. Morbidity, margins, recurrence, and survival were evaluated.Results24 patients underwent EVRR with 48 reconstructions (11 CRC and 13 RPS). For CRC, 100% of patients underwent Iliac system reconstructions. For RPS, inferior vena cava reconstructions were the most common (69.2%). There were 2 arterial and 1 venous graft thromboses. Primary graft patency was 89.4% arterial and 93.1% venous, while secondary patency was 100% arterial and 96.5% venous at last follow up. 1 venous and 1 arterial graft required reoperation for bleeding. There were no compromised limbs. Major complications occurred in 6 patients (25.0%) with no observed difference between CRC and RPS (OR 0.43 95%CI[0.60,3.19], P = 0.41). R1 margins occurred 1 CRC (90.9%) and 3 RPS (76.9%), with no R2 resections. All vascular resection margins were clear. There were 6 CRC (50%) and 4 RPS (33.3%) recurrences. Median recurrence time was 20.9 months for CRC and ‘not yet reached’ for RPS. Median follow-up was 19.4 months for CRC and 21.4 months for RPS.ConclusionEVRR for locally advanced CRC or RPS is safe and achieves favorable R0 resection rates. CRC patients with major vascular invasion can still be considered for curative intent surgery. Larger cohorts with longer follow up are needed to assess oncologic outcomes.     

食管平滑肌肉瘤的外科治疗(附11例临床分析)

目的　探讨食管平滑肌肉瘤的临床生物学特征、诊断方法、手术方式及其预后。方法　总结分析11 例食管平滑肌肉瘤的诊断及手术治疗经验。结果　本组11 例手术切除率为100 % ,5 年生存率为54-5 % (6/11) 。诊断主要依靠食管造影、拉网及纤维食管镜(FOE) 。临床分为息肉型和侵袭型,本组息肉型4 例,侵袭型7 例,5 年生存率分别为3 例/4 例和3 例/7 例。远期肿瘤死于局部复发2 例,血行转移2 例。结论　食管平滑肌肉瘤的预后优于食管癌,息肉型预后优于浸润型。远期肿瘤死因为局部复发及血行转移。本病手术切除率高,宜采取手术治疗。     

开腹与后腹腔镜术式治疗肾上腺良性肿瘤的比较

目的 探讨开腹与后腹腔镜术式对肾上腺良性肿瘤患者围手术期临床指标、ICU转送率及并发症的影响。方法 选取120例肾上腺良性肿瘤患者为研究对象,以随机数字表法分为对照组和观察组,每组各60例;其中对照组患者采用开腹术式治疗,观察组患者则采用后腹腔镜术式治疗;比较两组患者的手术时间、术中失血量、肛门排气时间、术后引流量、术后拔管时间、住院时间、ICU转送率及并发症发生率。结果 观察组患者手术时间、肛门排气时间、术后拔管时间及住院时间均显著短于对照组（P＜0.05）;观察组患者术中失血量和术后引流量均显著少于对照组（P＜0.05）;两组患者ICU转送率比较差异无统计学意义（P＞0.05）;同时观察组患者术中和术后并发症发生率均显著低于对照组（P＜0.05）。结论 后腹腔镜术式治疗肾上腺良性肿瘤可有效减轻医源性损伤程度,促进术后早期康复,且有助于避免术中及术后出现并发症,其临床应用价值优于开腹术式。     

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。     

Vascular reconstruction after retroperitoneal and lower extremity sarcoma resection

Purpose

Soft tissue sarcomas (STS) of the retroperitoneum and the lower limb with invasion of major blood vessels are very rare malignancies. This study analyses the outcome of patients with vascular replacement during resection of STS of the retroperitoneum and the lower extremity with either arterial or concomitant arterial and venous infiltration.