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。     

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.     

腹膜后脂肪肉瘤29例的诊断和治疗

探讨原发性及复发性腹膜后脂肪肉瘤（PRLS）的临床病理特征、诊疗原则及预后情况。回顾性分析2015年1月—2021年12月新疆医科大学第一附属医院消化血管外科中心收治的29例腹膜后肿瘤患者的临床资料。其中,侵犯周围器官15例（51.7%）,联合器官切除16例(55.2%)。中位手术时间235 min;中位出血量300 m L。术后病理检查结果证实脂肪肉瘤诊断,术后共12例患者出现并发症：肠梗阻4例、感染性休克1例、胸腹盆腔积液6例与低钾血症1例。以上患者经积极治疗后均痊愈出院。原发性腹膜后脂肪肉瘤前期诊断困难,切除难度大、术后易复发。肿瘤切除范围、肿瘤分化程度是腹膜后脂肪肉瘤术后复发的重要影响因素。     

腹膜后脂肪肉瘤的生物学特性及其诊断和治疗

目的 探讨腹膜后脂肪肉瘤生物学行为及诊断与治疗的有关问题。方法 回顾性分析1970—2005年中国医科大学附属第一医院收治的32例（共接受手术43例次）原发及复发腹膜后脂肪肉瘤病人的临床和病理资料。结果 该病主要临床表现是腹胀、腹部包块进行性增大。复发病例较原发病例生物学行为差，肿瘤侵袭力强，组织学亚型恶性程度高，手术完全切除率低。多次复发者复发间期逐渐缩短。难治性大出血是术后主要且致命的并发症。结论 腹膜后脂肪肉瘤术后易复发，很少转移，手术切除是最有效的治疗手段。完全切除者复发间期长，控制术中出血是减少术后死亡的关键。放、化疗对腹膜后脂肪肉瘤的作用有限。     

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。     

原发性腹膜后肉瘤的外科治疗

目的　探讨原发性腹膜后肉瘤的外科治疗方法。方法　回顾性分析 1992年 1月～ 2 0 0 0年 12月收治的 63例原发性腹膜后肉瘤 (脂肪肉瘤和平滑肌肉瘤 )的治疗效果、预后因素等临床资料。结果　肿瘤全切除率为 88.2 % ,其中联合脏器切除为 2 1.6% ,无围手术期死亡。脂肪肉瘤的术后平均生存时间 3 6.5个月 ,平滑肌肉瘤为 2 7.6个月。高分化肿瘤术后平均生存时间 3 7.4个月 ,中低分化肿瘤仅 18.1个月 (P <0 .0 1)。 3 2例作再次手术 ,其中肿瘤完全切除率为 62 .5 % (2 0 /3 2 ) ,术后平均生存时间为 19.8个月 ,其中 2 8.1%术后病理类型发生变化。结论　手术切除整个肿瘤及侵及的周围脏器是治疗的最佳手段 ,对局部复发的肿瘤争取再次手术 ,肿瘤的分化程度及能否完整切除肿瘤是影响预后的主要因素。     

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.     

Multicentric liposarcoma: Report of two cases

We present herein two unusual cases of multicentric liposarcoma which highlight the problems associated with the management of this disease entity. When the surgeon is confronted with multicentric liposarcomas, it is necessary to define whether they are secondary tumors or independently arising multicentric liposarcomas, since the latter situation merits a more aggressive approach. Guidelines for this differentiation are suggested in the discussion following the case reports. Surgery, which is often multiple, remains the mainstay of treatment, although adjuvant chemotherapy and radiotherapy are also usually required.     

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。     

Giant retroperitoneal liposarcoma

Liposarcomas are one of the most common soft tissue sarcomas of adulthood. Liposarcomas are remarkable because of their frequently large size. We report a case with an extremely large dedifferentiated liposarcoma that weighed 18 kg. Although it was capsulated and could be excised en bloc, the patient suffered a relapse 9 months later and died 1 year after the operation.     

Retroperitoneal and scrotal giant liposarcoma: Report of a case

(Received for publication on Aug. 5, 1996; accepted on Mar. 4, 1997)     

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.     

Pulmonary pleomorphic liposarcoma

Pulmonary liposarcoma is extremely rare with 6 cases previously reported in the English literature. The following is a report of a 36-year-old man with pulmonary liposarcoma. The patient had chest pain and a mass shadow in the right lower lung field was revealed on a chest X-ray film. A computed tomography and magnetic resonance imaging showed a heterogeneous tumor, compressing the right lower lobe of the lung. Because of the fatty tissue component in the tumor on the chest computed tomography, and the appearance as an extrapulmonary mass, preoperative diagnosis was posterior mediastinal liposarcoma. But thoracotomy revealed that the tumor originated from the lung. Right lower lobectomy and lymph node dissection was carried out with no additional therapy. The pathological diagnosis was pleomorphic liposarcoma originated from the lung. Two months after the operation, the patient died of respiratory failure due to local recurrence.     

Multicentric liposarcoma

Abstract A 72-year-old man complaining of upper abdominal discomfort was diagnosed as having retroperitoneal liposarcoma by means of diagnostic imaging. He then underwent an operation. One mass existed on the curvatura ventriculi major, extending to the hilum splenicum and pressing back the pancreal head and body. There was another mass to the left of the first, situated on the ventral side of the left kidney. Also, another mass was intramurally found adjacent to the curvatura ventriculi major. Histologically, the mass on the curvatura ventriculi major ranged from the peritoneal cavity to the retroperitoneum Its intraperitoneal portion was classified as a differentiated lipoma-like type and the retroperitoneal mass was of mucous type. The mass on the left kidney was of a differentiated fibrosing type. The intramural mass in the gastric curvature was found to be a differentiated lipoma-like type. The patient has been under observation for 12 months and has shown no recurrence.     

Clinical features of multiply recurrent retroperitoneal liposarcoma: A single-center experience

BackgroundTo elucidate the clinicopathological features of multiply recurrent retroperitoneal liposarcoma referred to a tertiary center.MethodsWe retrospectively analyzed the clinical data of 40 patients with pathologically proven primary retroperitoneal liposarcoma treated between January 2015 and June 2019.ResultsThe initial pathology was well-differentiated liposarcoma (WDLS) in 23 patients, dedifferentiated liposarcoma (DDLS) in 15 patients, and myxoid liposarcoma in two patients. Before and after referral to our hospital, the patients underwent 142 surgeries (median number of surgeries: 3) for initial and recurrent tumors. Of these, 35 (87.5%) patients underwent surgeries for recurrent tumors. In 11 (47.8%) of the 23 patients with initial WDLS, pathological progression (PP) to DDLS was observed in recurrent tumors at the median interval of 7.8 years. In eight patients (72.7%), the PP occurred at the first recurrence. In contrast, all 15 patients with DDLS recurred as DDLS. Distant metastases developed in eight patients (34.8%) with WDLS and five patients (29.4%) with DDLS/myxoid subtype. All eight WDLS patients who developed metastasis were alive with disease at the median interval of 3.6 years, whereas four of the five patients with primary diagnosis of DDLS/myxoid subtype died at the median interval of 5.6 months from the development of metastasis. The 5-year overall survival of the patients with initial WDLS and those with DDLS/myxoid subtype were 100% and 67.4% (p = 0.0006), respectively.ConclusionsThe prognosis of the initial-WDLS patients was favorable despite multiple recurrences. In WDLS patients, if distant metastases develop, it is possible to remain alive with disease for years.     

原发性腹膜后肿瘤的外科治疗(附85例报告)

目的探讨和总结原发性腹膜后肿瘤的手术治疗经验。方法回顾性分析1993年1月至2005年9月经手术治疗的85例原发性腹膜后肿瘤,分析总结其病理学类型、影像学检查、外科手术治疗等临床资料。结果良性肿瘤33例,恶性肿瘤51例,交界性肿瘤1例,均经手术治疗且病理证实。良性肿瘤完整切除31例(93.94%);恶性肿瘤完整切除39例(76.47%);交界性肿瘤行完整切除。手术中联合脏器切除共20例,其中良性2例,恶性18例。良性肿瘤术后复发再手术4例,均完整切除;恶性肿瘤术后复发再手术19例,完整切除14例,部分切除3例,探查活检2例。结论影像学检查对术前诊断和手术切除范围有重要意义。手术完整切除肿瘤是治疗的关键因素。再次手术仍是治疗复发性腹膜后肿瘤的有效手段。