114例腹膜后脂肪肉瘤临床病理特征及预后因素分析

目的:探讨腹膜后脂肪肉瘤(retroperitoneal liposarcoma)患者的临床病理学特征与预后的关系,寻找影响预后的独立临床病理指标。方法:收集本院2008年1月至2018年1月行手术治疗的腹膜后脂肪肉瘤病理标本,研究组织病理学特征包括肿瘤大小、组织学类型、淋巴结转移、脉管瘤栓、神经侵犯等,免疫组织化学特征包括Ki-67、P53蛋白表达,分子病理特征包括MDM2基因扩增;分析临床治疗及随访信息,采用Kaplan-Meier法计算患者生存率;以生存预后模型进行单因素和多因素分析,分析影响预后的独立临床病理学因素。结果:共纳入114例腹膜后脂肪肉瘤,男59例(51.8%),女55例(48.2%),中位年龄52(22～80)岁。高分化脂肪肉瘤48例(42.1%),去分化脂肪肉瘤66例(57.9%);淋巴结有转移者2例(1.4%);有脉管瘤栓者3例(2.6%);Ki-67阳性指数<60%者88例(77.2%),≥60%者26例(22.8%);P53<70%者85例(74.6%),≥70%者29例(25.4%),荧光原位杂交发现MDM2基因扩增。单因素分析发现影响患者生...     

巨大腹膜后脂肪肉瘤1例

<正>患者女性,53岁,因发现腹部肿物3年,腹围快速增大。2019年7月收治于河北医科大学第四医院。查体:身高165 cm,体质量86 kg,腹部弥漫性高度膨隆(图1A),自肋缘下至耻骨联合上扪及巨大包块,上下径69 cm,前后径45 cm,左右径30 cm,腹围134 cm,立位可见Ⅲ度子宫脱垂(包括部分膀胱壁)。肿瘤巨大几乎占据整个腹腔,肿瘤分叶状,部分包膜光     

腹膜后脂肪肉瘤的诊断与治疗策略的研究进展

腹膜后脂肪肉瘤是一种罕见的后腹膜间隙内的间质来源肿瘤,典型恶性肿瘤的表现并且通常预后不良。由于该肿瘤较罕见并且解剖位置隐蔽,因此诊断比较困难,在治疗方法上也存在诸多挑战。对肿瘤进行彻底切除后其复发率依然较高,因此需要长时间不定期随访。同时,除外科手术切除外,还有放疗、化疗、免疫治疗以及综合治疗等方式。     

原发性腹膜后脂肪肉瘤20例报告

自１９６５年５月至１９９１年７月共收治原发性腹膜后脂肪肉瘤２０例，均经手术及病理证实。２０例中完全切除者１５例，部分切除者４例，活检１例。肿瘤切除合并肾，肠襻等邻近器官切除者达１２例。全组的５年，１０年生存率分别是５７．１％和２０．０％，完全切除组的５年１０年生存率分别是６３．６％和２８．６％。２０例病人共进行了４８次切除手术。首次手术死亡２例。着重讨论了本病的诊断、治疗和突出的临床特点。     

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。      

腹膜后脂肪肉瘤1例报告

脂肪肉瘤在软组织肉瘤中层第二位，约占14～15%，男女发病率相似，好发年龄40-60岁，以分化不良型脂肪肉瘤多见，幼年及青年以分化较好型脂肪内瘤多见，我院曾收一例腹膜后脂肪肉瘤，现报道如下：     

原发性腹膜后软组织肉瘤预后因素的COX模型分析

自１９６４年４月至１９９２年４月，我院共收治原发性腹膜后软组织肉瘤４８例，总的２．５，１０年生存率为５７．８％，２２．４％和１０．７％，本文运用ＣＯＸ模型，对可能影响本病预后的有关因素，分别作单因素及多因素分析，分析结果表明肿瘤有无全切除，是影响本病预后的最重要因素，因此，我们主在明确本病诊断后，尽可能完全切除肿瘤，对术后复发者，也要争取再次，多次手术切除，术后酌情辅加放疗。     

38例手术治疗腹膜后脂肪肉瘤病例分析

目的：通过分析38例手术治疗的腹膜后脂肪肉瘤病例,探讨治疗复发性脂肪肉瘤的方法。方法：分析1987年6 月至2010年6 月辽宁省肿瘤医院共38例有完整随访资料的原发性脂肪肉瘤病历资料。结果：行2 次以上手术23例（60.5%）,3 次以上手术11例（28.9%）,联合脏器切除14例（36.8%）,5 年生存率47.4% ,10年生存率28.9% 。结论：腹膜后脂肪肉瘤有术后易于复发的特点,对于复发病例争取再次手术是延长患者生存期的重要办法,扩大切除（包括联合脏器切除）是降低复发率的重要手段。     

Clinicopathological characteristics and experience in the treatment of giant retroperitoneal liposarcoma: A case report and review of the literature

Retroperitoneal liposarcoma (RPLS) is a rare tumor, especailly those over 20 kg that are called “giant liposarcoma," whose characteristics and treatments remain relatively unknown. Herein, we report a giant RPLS measuring 65 × 45 × 30 cm in diameter and 31 kg in weight, which we successfully performed complete excision through interdisciplinary cooperation. The patient had an uneventful postoperative course and was discharged without complications. Afterwards he underwent radiotherapy and had no evidence of tumor recurrence or symptoms of metastasis at 3-month CT scan and 8-month follow-up. We also first review the 13 cases reported in literature published in PubMed regarding giant RPLS. Giant RPLS commonly occurs in adults aged 40–60 y and presents atypical clinical manifestations. CT scan is the most useful examination and preoperative biopsy is controversial. Complete surgical resection still remains the principal treatment. Giant RPLS can also be removed, even reach to R0 excision, by a multidisciplinary team in a specialized center after meticulous planning even though its gigantic tumor size. Local radiotherapy following surgery may improve the rate of recurrence. Besides, closely follow-up and routine examinations are required.     

Cardiac metastasis of liposarcoma

The authors describe a retroperitoneal liposarcoma with secondary involvement of the left ventricle. Therapy has been disappointing, and is guided by the nature of the primary tumor, previous therapy, extent of metastatic spread, and feasibility of cardiac resection. In selected patients whose primary tumor is well-controlled and progressing slowly, with no evidence of widespread disease, resection of the cardiac metastases can be performed when technically feasible.     

Metastatic patterns of extremity myxoid liposarcoma and their outcome

BACKGROUND AND OBJECTIVES: Extremity myxoid liposarcomas have a unique extrapulmonary metastatic potential. We studied the metastatic pattern of extremity liposarcomas to determine what types of posttreatment imaging may be of value in the follow-up these patients. METHODS: Twenty-two patients from a total of 128 patients with primary extremity liposarcoma were treated at a tertiary care institution for subsequent metastases from January 1981 to January 2000. Median follow-up was 45 months (range: 6-270 months). Data on these patients was prospectively collected and then retrospectively analyzed for effect of metastatic pattern and treatment on outcome. RESULTS: Of these 22 patients, extrapulmonary metastases developed in 10, combined pulmonary and extrapulmonary metastases developed in 6, and isolated pulmonary metastases developed in 6. Of the 16 patients with extrapulmonary metastases, 13 were of the myxoid subtype. Of the 49 patients with extremity myxoid liposarcomas, metastases developed in 14 (29%). The most common sites of metastases among these 14 patients include: the retroperitoneum, 10 patients (71)%; intra-abdominal extra-hepatic, 7 patients (50%); spinal/paraspinal, 6 patients (43%). Only 3 of the patients are alive and disease free and all 3 of these patients are from the subgroup of 10 patients with only extra-pulmonary metastases (2 intra-abdominal and 1 retroperitoneal). CONCLUSIONS: Extremity myxoid liposarcomas have an unusually high predilection for extra-pulmonary metastases, frequently without any pulmonary metastases. After treatment of the primary tumor, these patients should be followed with periodic chest X-ray and abdominal/pelvic computed tomography (CT) scans. Any back or neurologic complaints should prompt additional imaging of the appropriate spinal area. Consideration should be given to surgical and adjuvant treatment of metastatic disease when appropriate.     

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.     

123例子宫内膜癌的预后相关因素分析

目的:探讨子宫内膜癌的预后相关因素。方法:回顾性分析1996年1月至2006年12月汕头大学医学院附属肿瘤医院收治的123例子宫内膜癌患者的临床资料,并随访患者生存情况,进行生存及预后分析。结果:123例子宫内膜癌患者总体5年生存率为90.2%。单因素分析显示:病理组织分级、病理类型、深肌层浸润、腹水细胞学阳性、附件转移、淋巴结转移、手术-病理分期及术后放化疗等因素与预后相关(P<0.05)。多因素分析显示:手术-病理分期、深肌层浸润以及是否行腹膜后淋巴结切除术与预后相关(P<0.05)。结论:子宫内膜癌总体生存率高,手术-病理分期、肌层受累程度以及是否行腹膜后淋巴结切除术是影响子宫内膜癌预后的独立因素。     

肢体脂肪肉瘤的预后相关因素及放化疗疗效分析

目的：统计分析肢体脂肪肉瘤的临床预后相关因素;回顾性分析辅助放疗、化疗对脂肪肉瘤的治疗效果。方法：随访2007年7 月至2012年12月在北京大学肿瘤医院接受综合治疗的肢体脂肪肉瘤患者,统计分析预后与年龄、部位、入院状态等临床因素的关系。分析辅助放疗和化疗对总生存时间（overallsurvival,OS）和无病生存时间（diseasefreesurvival,DFS）的影响。结果：共82例患者接受手术为主的综合治疗,失访9 例,73例患者获得随访,随访时间24～88个月,中位随访时间47个月。总生存率为83.6%（61/ 73）,无病生存率为68.5%（50/ 73）。 Cox 多因素回归分析提示部位、病理分级和入院状态是影响DFS 的独立相关因素;年龄和病理分级是影响OS的独立相关因素。Kaplan-Meier 生存分析显示,放疗能够显著改善G 2、G 3 级脂肪肉瘤的DFS（59.1个月vs . 28.4 个月,P < 0.01）,并且能改善OS（70.8 个月vs . 55.1 个月,P < 0.05）。 结论：脂肪肉瘤的预后与患者年龄、部位及病理分级相关。辅助放疗可改善G 2、G 3 级肢体脂肪肉瘤的生存与预后,但化疗在脂肪肉瘤治疗中的作用尚不明确。     

The prognostic impact of dedifferentiation in retroperitoneal liposarcoma: a series of surgically treated patients at a single institution

BACKGROUND.

A series of patients with well differentiated (WD)/dedifferentiated (DD) retroperitoneal liposarcoma (RLS) was studied to evaluate the prognostic value of the presence, extension, and grade of the DD component.

METHODS.

Among 148 patients with RLS who underwent surgery over 20 years, the authors retrieved data on patients who had localized WD/DD RLS. For the current analysis, patients were included only if they had primary disease or a first recurrence at their initial presentation. The DD component, when present, was graded according to National Federation of Centers in the Fight Against Cancer (FNCLCC) criteria, and the extension of the DD component was described as a percentage. Univariate and multivariate analyses were carried out for local recurrence‐free survival (LRFS), event‐free survival (EFS), and disease‐specific survival (DSS).

RESULTS.

Of 93 patients who were identified, 36 patients (39%) had WD RLS, and 57 patients (61%) had DD RLS. The median follow‐up was 71 months (range, 28‐132 months). Seven patients (7.5%) developed distant metastases, including 5 patients who had DD RLS. The 5‐year DSS rate was 42% in patients with DD RLS and 71.6% in patients with WD RLS (P = .018). The corresponding rates for LRFS were 22% and 43.3%, respectively (P = .007). The presence of the DD component and its FNCLCC grade were independent prognostic factors for DSS and LRFS. The administration of radiation therapy was associated independently with better LRFS.

CONCLUSIONS.

Patients with high‐grade DD RLS had a worse prognosis in terms of both DSS and LRFS. The extension of the DD component and its mitotic index were relevant for EFS. The results indicated that radiation therapy may improve LRFS. These data may help stratify the risk of recurrence for patients with RLS. Clinical studies on new multimodality approaches are warranted. Cancer 2008. © 2008 American Cancer Society.     

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.     

Myxoid/round cell and pleomorphic liposarcomas: prognostic factors and survival in a series of patients treated at a single institution

BACKGROUND: The objective of this study was to investigate prognostic factors and clinical outcome of myxoid/round cell and pleomorphic liposarcoma. METHODS: Three hundred twenty-nine patients with localized myxoid/round cell or pleomorphic liposarcoma who underwent surgery at the Istituto Nazionale per lo Studio e la Cura dei Tumori (Milan, Italy) over 25 years were reviewed. The rates of local recurrence, distant metastases, and survival were studied. RESULTS: Two hundred fourteen patients presented with primary disease, and 115 patients had locally recurrent tumors. The disease-specific survival rate was 75% at 10 years, and the local recurrence and distant metastases incidence were 25% and 15%, respectively. Presentation with recurrent disease, tumor size (>10 cm), tumor grade (French Federation of Cancer Centers grade II or III vs grade I), and positive surgical margins were independent predictors of death. Tumor site and radiation therapy also played a role, mostly related to their effect on local outcome. Pathologic grade and histologic subtype influenced distant metastases. Extrapulmonary metastases were associated with poorer postmetastatic disease-specific survival. CONCLUSIONS: Myxoid/round cell liposarcomas shared similar prognostic factors with other soft tissue sarcomas and had a relatively good clinical outcome. The presence of >5% of round cell component singled out a group of patients at greater risk of metastases and death but with a broad spectrum of disease aggressiveness. Extrapulmonary metastases were a peculiar pattern of myxoid/round cell liposarcoma that require special consideration for treatment and prognosis.