原发性腹膜后脂肪肉瘤的诊断及治疗

原发性腹膜后脂肪肉瘤(primary retroperitoneal liposarcoma，PRPLS)临床上少见，我院1983年1月至2003年12月共收治经手术和病理证实的PRPLS患者13例，现报告如下。     

原发性腹膜后脂肪肉瘤16例临床分析

原发性腹膜后脂肪肉瘤在临床上虽不常见，但也不是很少见，因其发病部位深在，难以早期发现，即使诊断明确后行手术切除，也经常复发。我们回顾分析了我院1994—2003年收治的16例原发性腹膜后脂肪肉瘤病例，结合近年来的相关文献，对该病例的临床特点及治疗作初步的探讨。     

腹膜后巨大脂肪肉瘤一例

腹膜后脂肪肉瘤是一种少见的恶性肿瘤，早期不易发现，手术彻底切除较困难，术后易复发。现将我院收治的1例腹膜后巨大脂肪肉瘤报道如下。[第一段]     

原发性腹膜后脂肪肉瘤23例报道

目的总结原发性腹膜后脂肪肉瘤(primary retroperitoneal liposarcoma,PRL)的临床病理特征、诊断及治疗方法。方法回顾性分析经手术和活检证实的23例PRL患者的临床病理资料。结果首发症状及体征表现为腹部肿块(91.3%,21/23),腹胀(56.5%,13/23)及腹痛(30.4%,7/23)。B超及CT的定位诊断准确率分别为66.7%(12/18)和85.7%(12/14)。首次手术肿瘤完整切除16例(69.6%),其中8例联合脏器切除;部分切除3例(13.0%);仅行活检4例(17.4%)。PRL肿瘤完整切除术后复发率为75.0%(12/16),该12例中再次手术8例(66.7%)可完全切除肿瘤。结论CT是诊断PRL的重要手段,优于B超;手术以完整切除肿瘤为主,对侵犯脏器者采用累及脏器一并切除;术后复发者可再次手术。     

原发性腹膜后脂肪肉瘤的外科治疗

目的探讨原发性腹膜后脂肪肉瘤（PRPI_S）的外科治疗方法。方法回顾性分析1996年1月至2008年3月经手术治疗17例PRPLS患者的治疗效果。结果17例患者共进行手术30例次,其中13例次为首次手术,17例次为再次手术。肿瘤完整切除24例次（80．0％）,其中联合脏器切除12例次（40．0％）,肿瘤部分切除5例次（16．7％）,探查活检1例次（3．3％）。结论原发性腹膜后脂肪肉瘤无论是首发还是复发,手术切除都是标准的治疗手段,晚期肿瘤姑息切除亦能减轻邻近压迫症状,改善患者生存质量,延长生存时间。     

腹膜后脂肪肉瘤15例分析

作者报告10年中收治的腹膜后脂肪肉瘤15例.术前根据病人年龄较大、病史较短、腹部可触及能轻微左右移动的巨大平软或中等深在肿块等特点,若病人全身情况较好,再结合消化道钡餐、B超、CT等检查,可初步诊断、定位和了解与周围组织关系.治疗主张尽可能整块或大部切除,对复发者争取再次、多次手术切除,如有困难术中不要勉强切除.15例中整块或大部切除11例.15例中已有5例死亡,多为不能切除者,大部切除的病例目前已有3例分别作了4次、3次、2次手术,存活时间最长已10年,最短2年,整块全切除复发率低,疗效较好.     

原发性腹膜后肉瘤预后研究进展

原发性腹膜后肉瘤在临床上较为少见,然而病理类型繁多。手术切除是其主要的治疗方法,但术后的高复发率是此类疾病治疗的一大困扰。此文分析总结了相关文献,结果显示腹膜后肉瘤病人的预后主要与病理类型、肿瘤级别、手术范围及肿瘤数目等有关。     

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,PRPLS）的临床病理特点、诊治及预后.方法 对华中科技大学同济医学院附属协和医院普通外科初诊的43例PRPLS患者临床病理资料进行回顾性分析.结果 43例PRPLS患者中男性32例,女性11例,年龄22~75岁,中位年龄49岁.肿瘤平均大小（17.6＆#177;10.8） cm.行CT检查38例,定性准确率为81.3%;行MRI检查8例,定性准确率为80.0%.43例患者均接受手术,其中首次手术肿瘤完整切除者40例,联合切除率为32.5%,中位随访39个月,首次手术完整切除且未复发的患者3、5年生存率分别为85.0%、80.0%,术后复发患者3、5年生存率为72.2%、61.1%.结论 PRPLS早期诊断较为困难,CT和MRI是PRPLS诊断和术后复查的重要手段.PRPLS首次手术应争取完整切除肿瘤,对大多数复发病例仍应以积极手术治疗为主,完整切除仍可获得较好的预后.     

腹膜后巨大脂肪肉瘤1例

1病历简介病人女性,68岁。于2008-09-18来我院就诊,以腹腔肿物收入院。入院前3个月出现腹胀,无腹痛,无呼吸困难,无恶心呕吐,排黄色稀便,入院前1个月前行CT检查发现腹膜后巨大占位病变,入院查体:T37.0℃,P82次/分,     

原发性腹膜后脂肪肉瘤的发病机制及综合治疗

目的了解原发性腹膜后脂肪肉瘤(PRLPS)的发病机制及综合治疗的研究进展,以期为临床医生诊疗提供依据。方法复习近年来关于PRLPS的病理分型、发病机制以及包括手术、放疗、化疗及分子靶向治疗在内的综合治疗研究进展的相关文献并加以综述。结果 PRLPS病理分型分为高分化、去分化、黏液样或圆细胞型、多形型及混合型5种。分子学发病机制主要是基因表达的变化,如MDM2与相关基因的协同作用、c-myc基因表达异常、Prune-nm23-H1机制、miRNA异常表达及FUS-CHOP融合基因的异常蛋白质产物调节肿瘤的生长。PRLRS的治疗包括肿瘤根治切除术、扩大切除术、姑息性切除术,辅之以放化疗以及分子靶向治疗。结论 PRLPS是一种复发率极高、较罕见的恶性肿瘤,但早期诊疗比较困难。随着对PRLPS分子机制的进一步研究,其治疗方式已转变为以手术切除治疗为主、辅以放化疗及分子靶向治疗的综合治疗方式。     

Surgical management of primary and recurrent retroperitoneal liposarcoma

BACKGROUND: Surgery plays a dominant role in the initial and subsequent treatment of retroperitoneal liposarcoma (RPLS). This study was a review of outcomes of patients treated at the Royal Marsden Hospital. METHODS: Records of all patients who had surgery for RPLS since 1990 were reviewed, with particular attention to local recurrence and disease-specific survival. Patients with primary RPLS and those with recurrent RPLS, who had palliative surgery after a variable number of operations performed elsewhere, were considered separately. RESULTS: Seventy-two patients had surgery for primary RPLS, over half of whom underwent resection of a contiguous organ to achieve clearance. Follow-up of at least 12 months was available for 58 patients. Thirty-four patients had no evidence of recurrence after median follow-up of 26 (range 12-151) months. Low-grade tumour and macroscopic clearance of tumour were significantly associated with a reduced risk of local recurrence and improved survival. Forty-seven patients had palliative surgery for recurrent RPLS. Median survival from time of last operation to death was 27 (range 0-79) months. Follow-up was to a median of 68 (range 14-261) months. CONCLUSION: Patients with low-grade RPLS that has been completely resected at the initial operation have the most favourable prognosis. Palliative resection is worthwhile to treat troublesome symptoms of recurrence.     

原发性腹膜后脂肪肉瘤术后复发的多因素分析

目的探讨原发性腹膜后脂肪肉瘤术后复发的影响因素以减少复发。方法回顾分析10年手术治疗31例原发性腹膜后脂肪肉瘤患者的临床资料,通过随访了解术后5年复发情况,以与术后复发有关的因素为变量组单因素及多因素非条件性回归分析。结果 5年复发率为48.39%（15/31）,单因素分析及多因素分析均显示手术方式、组织学亚型、肿瘤体积和是否外侵对原发性腹膜后脂肪肉瘤术后复发均有影响（P〈0.05）。结论手术方式、组织学亚型、是否外侵和肿瘤体积与原发性腹膜后脂肪肉瘤患者的术后复发相关。     

Giant retroperitoneal liposarcoma

Liposarcoma is a malignant tumor that has an embryologic origin from mesodermal tissue depending on fatty tissue. Although liposarcoma is only 0.1% of all human neoplasms, it is the most common histology subtype of retroperitoneal soft tissue sarcomas. This tumor grows slowly. Diffuse abdominal pain is its most frequent symptom and abdominal mass is the most common sign. Aggressive surgical treatment is basic to get a complete resection and a local disease control. This objective is difficult because of the large tumor size it gets in the retroperitoneal location and the multiorgan involvement that require the resection of a high percentage of contiguous organs. We report a case of a giant retroperitoneal liposarcoma presenting like continuous left hemiabdominal pain because of the visceral compression. The right kidney was involved and suffering from renal vessel enlargement without renal function. There is a high probability of microscopic residual disease and a good follow-up of the patients is necessary as well as an adjuvant radiation therapy in some cases.     

Calcified retroperitoneal liposarcoma

Case report of a retroperitoneal liposarcoma pleomorfico with calcification in diagnosed in our hospital. We comment on its clinical characteristics, diagnostic methods and treatment. We likewise comment the possible differential diagnoses.     

Giant retroperitoneal liposarcoma

Liposarcoma is a malignancy of fat cells and is the most frequent soft tissue sarcoma localized in the retroperitoneum. It can reach substantial proportions. It is a slow-growing tumor, and the most frequent symptom is nonspecific abdominal pain and diffuse abdominal enlargement. Treatment is radical surgery and complete resection is essential for local control of the disease. We present a case of giant right retroperitoneal liposarcoma, which was well-encapsulated and could be completely excised. The patient is currently in follow up and at 2 years is disease-free.