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。     

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：     

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     

腹膜后黏液样脂肪肉瘤1例及文献复习

<正>腹膜后脂肪肉瘤(retroperitoneal liposarcoma,RPLS)是罕见的间叶细胞来源的恶性肿瘤,发病率占全部恶性肿瘤的1%以下,但在腹膜后软组织肉瘤中则是最常见的类型。因其症状隐匿,发现时多数肿瘤体积巨大且与邻近脏器关系复杂,手术难度大,彻底切除较困难,往往需要联合脏器切除,术后复发率高,化疗效果不佳,因此是临床亟待解决的难点。现就本院收治的1例腹膜后黏液样脂肪肉瘤,     

腹膜后脂肪肉瘤患者1例

1病例资料 患者,女,46岁,因查体发现腹部肿物10 d入院.患者10 d前于当地医院查体时彩超检查发现左侧腹部一肿物,无疼痛,左侧腹部轻微腹胀,排便后腹胀无缓解,大便规律,约1次/d,为成形软便,偶尔大便较稀不成形,无便血、黑便,无发热,无恶心、呕吐,无腹痛,无肛门停止排气、排便.入院时查体:一般状况良好,双侧下肢无水肿,腹部查体:左下腹部可触及一包块,大小约9 cm×10 cm,类圆形,质硬,活动度差,锁骨上及腹股沟淋巴结均未触及肿大.腹部彩超示:左下腹腹腔内探及一实性略强回声包块,大小约11.1 cm×6.6 cm ×9.6 cm,边界清,形态规则,内部回声不均质,内可见少许血流信号显示.腹部CT示:左下腹部可见一软组织密度肿块,其内密度不均匀,部分CT值约-15 HU,边界区清楚;增强扫描肿块呈不均匀强化.入院诊断:腹膜后肿物,脂肪肉瘤?卵巢畸胎瘤?.     

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.     

腹膜后巨大脂肪肉瘤1例并文献复习

目的 探讨腹膜后脂肪肉瘤的诊断和治疗,提高少见病例的诊疗水平。方法 回顾江汉大学附属医院收治的1例腹膜后巨大脂肪肉瘤患者的临床资料,结合文献讨论腹膜后脂肪肉瘤的临床特点以及诊断、治疗方法。结果 患者经影像学诊断后行左侧腹膜后肿瘤切除+左肾部分切除术,术后病理提示腹膜后脂肪肉瘤,手术顺利,术后随访20个月,未见肿瘤复发及转移。结论 腹膜后脂肪肉瘤临床少见,无特异性临床表现,CT有助于该病的诊断,手术是首选的治疗方法。     

Challenging surgical treatment of giant retroperitoneal liposarcoma: A case report

Liposarcoma is a rare malignant tumor type and surgical resection is the gold standard treatment. The present study reported on the case of a 51-year-old woman who presented with a mass in the left upper abdomen. Computed tomography revealed a 32-cm giant retroperitoneal liposarcoma. Complete tumor resection was performed without the removal of other organs. Postoperative pathological examination indicated retroperitoneal well-differentiated liposarcoma and immunohistochemistry revealed S-100(−), MDM2(+), vimentin(+), CDK4(+), p16(+) and STAT6(+) results. The patient recovered well after the surgery. Complete tumor resection during the first surgery is key to cure liposarcoma. The present case report will be helpful for clinical oncologists to fully understand giant retroperitoneal liposarcoma and treat it accordingly.     

Diagnostic accuracy of biopsy after neoadjuvant treatment for well-differentiated and dedifferentiated retroperitoneal liposarcoma

PurposeAccurate histopathological grading of percutaneous biopsies is essential to guide adequate management of patients with suspected retroperitoneal liposarcoma. In this regard, however, limited reliability has been described. Therefore, we conducted a retrospective study to assess the diagnostic accuracy in retroperitoneal soft tissue sarcomas and simultaneously investigate its impact on patients' survival.Materials and methodsReports of an interdisciplinary sarcoma tumor board between 2012 and 2022 were systematically screened for patients with well-differentiated (WDLPS) and dedifferentiated retroperitoneal liposarcoma (DDLPS). Histopathological grading on pre-operative biopsy was correlated with corresponding postoperative histology. Additionally, patients' survival outcomes were examined. All analyses were performed in two subgroups: patients with primary surgery and patients with neoadjuvant treatment.ResultsA total of 82 patients met our inclusion criteria. Diagnostic accuracy of patients who underwent upfront resection (n = 32) was significantly inferior to patients with neoadjuvant treatment (n = 50) (66% versus 97% for WDLPS, p < 0.001; 59% versus 97% for DDLPS, p < 0.001). For patients with primary surgery, histopathological grading on biopsy and surgery was concordant in only 47% of cases. Sensitivity for detecting WDLPS was higher than for DDLPS (70% versus 41%). Higher histopathological grading in surgical specimens correlated with worse survival outcomes (p = 0.01).ConclusionHistopathological grading of RPS may no longer be reliable after neoadjuvant treatment. The true accuracy of the percutaneous biopsy may need to be studied in patients who do not receive neoadjuvant treatment. Future biopsy strategies should aim to improve identification of DDLPS to inform patient management.     

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.     

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.     

Radiosensitivity translates into excellent local control in extremity myxoid liposarcoma

BACKGROUND:

Myxoid liposarcoma has been reported to be more radiosensitive compared with other soft tissue sarcomas (STS). The authors report the results of multidisciplinary treatment of extremity myxoid liposarcoma compared with a contemporary cohort of other STS subtypes with an emphasis on the role of radiotherapy (RT) in improving local control.

METHODS:

Between 1989 and 2004, 691 patients were identified from a prospective STS database who underwent combined management for localized extremity STS and were followed for a minimum of 12 months or until death. All patients underwent surgery together with pre or postoperative RT, depending on their presenting characteristics and resection margins. Demographics and outcomes were compared between patients with myxoid liposarcoma and other STS subtypes (other‐STS).

RESULTS:

Of 691 patients, 88 patients had myxoid liposarcoma and 603 had other STS subtypes (other‐STS). Median age was 48 and 60 years for the myxoid liposarcoma and other‐STS groups, respectively. Median follow‐up was 86 and 61 months, respectively. For myxoid liposarcoma and other‐STS groups, preoperative RT was used in 57% versus 61% of patients and postoperative RT in 43% versus 39%, respectively. The 5‐year local recurrence‐free survival was 97.7% for patients with myxoid liposarcoma compared with 89.6% for patients with other‐STS tumors (P = .008). High‐grade tumors were present in 7% and 59% of myxoid liposarcoma and other‐STS patients, respectively (P = .0003). Two myxoid liposarcoma patients with local recurrence had positive resection margins, whereas only 33% of patients in the other‐STS group who developed a local recurrence had positive resection margins. No patients with myxoid liposarcoma required amputation as primary management, whereas 8 (1.3%) required amputation as primary management in the other‐STS group. Systemic disease control was superior in myxoid liposarcoma compared with other‐STS patients, with 5‐year overall and metastasis‐free survival rates of 93.9% versus 76.4% (P = .0008) and 89.1% versus 66.0% (P = .0001) respectively. Of 12 myxoid liposarcoma patients with distant metastases, 7 appeared in nonpulmonary sites. In comparison, 205 (34%) patients with other‐STS tumors developed systemic disease but 78% had pulmonary metastases.

CONCLUSIONS:

Multidisciplinary management of extremity STS achieves high rates of local control. Myxoid liposarcoma is associated with higher rates of local control compared with other STS subtypes, after combined surgery and radiation, suggesting a particular radiosensitivity that can be exploited to improve oncologic outcome in appropriate cases. Cancer 2009. © 2009 American Cancer Society.     

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.     

后腹腔镜治疗肾上腺较大肿瘤的经验分析

目的探讨后腹腔镜肾上腺肿瘤切除术治疗肾上腺较大肿瘤的有效性及安全性。方法回顾性选取2010年3月至2020年2月间大连市中心医院收治的1 000例肾上腺肿瘤患者,按肿瘤直径分为肾上腺较小肿瘤(直径<6cm) 665例和肾上腺较大肿瘤(直径≥6cm) 335例,术前均完善肾上腺增强CT(其中95例因过敏史行平扫CT)确定肿瘤最大直径,均采用后腹腔镜手术,评价临床价值。结果 995例患者成功完成后腹腔镜下肾上腺肿瘤切除术,5例患者中转开放手术。较小肿瘤组患者手术时间15～110min,平均40.5min;较大肿瘤组30～220min,平均78.5min。较小肿瘤组术中出血量10～220ml,平均35.5ml;较大肿瘤组50～1 200ml,平均205.5ml。较小肿瘤组术中及术后输血5例,较大肿瘤组15例。较小肿瘤组中转开放2例,较大肿瘤组3例。以上两组比较,差异均有统计学意义(P <0.05)。较小肿瘤组患者术后拔除引流管时间1～3天,平均1.55天;较大肿瘤组1～4天,平均1.59天。较小肿瘤组住院时间4～11天,平均5.1天;较大肿瘤组4～14天,平均5.25天。两组比较...