附睾脂肪肉瘤腹膜后及腹腔内转移1例报告并文献复习

目的:报告1例罕见的附睾脂肪肉瘤伴腹膜后及腹腔内多次转移病例,提高对本病的诊治水平。方法:回顾分析1例附睾脂肪肉瘤伴腹膜后及腹腔内多次转移患者的临床资料,结合国内外相关文献,探讨附睾脂肪肉瘤的临床表现、诊断及治疗。结果:经腹股沟切口行根治性高位睾丸切除术完整切除肿瘤,10年后出现腹膜后复发转移,肿块上至肾脏上极,下达盆腔,大小15.6 cm×9.4 cm×25.5 cm,包裹左肾及输尿管上段,行左肾及输尿管全长、腹膜后肿瘤切除手术,4年后再次出现腹膜后、腹腔内多发复发转移灶,再次行手术切除,随访1年,死于肺功能衰竭。结论:附睾脂肪肉瘤极为罕见,仔细的临床体检和影像学检查可协助诊断,但最终需病理确诊,应早期行根治性手术切除。放化疗对附睾脂肪肉瘤的作用存在争议。本病有较高的复发率,长期的密切随访是必要的。     

原发性腹膜后脂肪肉瘤的诊断治疗

目的探讨腹膜后脂肪肉瘤的诊疗方法。方法回顾性分析近10年来我院收治的15例腹膜后脂肪肉瘤的诊疗效果。结果 15例患者共手术治疗28次,首次手术15例,7例复发;再次手术14例次.首次手术肿瘤完整切除率93.3%(14/15),较再次手术肿瘤完整切除率76.9%(10/13)高;首次手术联合脏器切除率26.7%(4/15)较再次手术38.5%(5/13)低。再次术后并发症较首次手术并发症高,术中出血较首次手术多。术后复发时间1.5～75个月,平均26.5个月。术后复发的间期随复发次数的增加而缩短,手术切除越彻底,术后复发的间期越长。肿瘤完整切除患者预后良好,全组手术完整切除组5年生存率为45.5%,术后并发症2例,1例术后并发肠瘘,1例术后腹腔淋巴瘘,均痊愈出院。首次术后病理3例黏液型、8例分化良好型、3例去分化型、1例多形性;再次术后病理4例次黏液型、5例次分化良好型、2例次去分化型、1例次多形性和1例次圆形细胞型,其中4例再次手术后病理类型与首次手术后不同。结论腹膜后脂肪肉瘤常以腹部包块、腹胀为表现,CT和MRI等影像学为主要检查方法,病理类型多样,放化疗效果不明确,手术治疗为主,复发时可反复多次手术,预后与病理类型相关。     

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。     

以结肠巨大息肉为临床表现的脂肪肉瘤一例

1 临床资料 患者男,46岁.因腹膜后脂肪肉瘤复发、大便带血1个月于2008年8月5日收治人院.2006年该患者因左上腹包块行腹膜后包块切除+腹腔内包块切除术,术后病理检查为腹膜后黏液型和硬化性脂肪肉瘤;腹腔内肉瘤,伴平滑肌细胞分化.术后3个月行CT检查提示脂肪肉瘤复发,患者拒绝治疗.患者定期复查发现包块逐渐增大,近1个月来出现大便带鲜血,为求进一步诊断和治疗入住我科.体格检查:腹部未触及包块,直肠指诊无异常发现.钡剂灌肠检查:结肠近脾区管壁僵硬,腔内多发不规则团块状充盈缺损,表面黏膜破坏,管腔明显狭窄.     

以结肠巨大息肉为临床表现的脂肪肉瘤一例

1 临床资料 患者男,46岁.因腹膜后脂肪肉瘤复发、大便带血1个月于2008年8月5日收治人院.2006年该患者因左上腹包块行腹膜后包块切除+腹腔内包块切除术,术后病理检查为腹膜后黏液型和硬化性脂肪肉瘤;腹腔内肉瘤,伴平滑肌细胞分化.术后3个月行CT检查提示脂肪肉瘤复发,患者拒绝治疗.患者定期复查发现包块逐渐增大,近1个月来出现大便带鲜血,为求进一步诊断和治疗入住我科.体格检查:腹部未触及包块,直肠指诊无异常发现.钡剂灌肠检查:结肠近脾区管壁僵硬,腔内多发不规则团块状充盈缺损,表面黏膜破坏,管腔明显狭窄.     

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目的 探讨原发性腹膜后及肠系膜脂肪肉瘤的外科治疗方法。方法 回顾性分析1993～2001年收治的18例原发性腹膜后及肠系膜脂肪肉瘤病人经手术治疗27例次的临床资料。结果 脂肪肉瘤占同期经手术探查的原发性腹膜后及肠系膜实体瘤的31．6％(18／57)；临床表现主要为进行性增大的腹部包块与消化道症状：病理类型以高分化型多见(13／18)；首诊后16例获得临床根治性切除，6例复发性脂肪肉瘤病人，共接受15次手术；全组合并单侧肾切除6例，部分结肠切除4例，部分空肠切除和胃大部切除各1例；肠系膜脂肪肉瘤手术多采用完整切除术(8／10)。结论 手术切除是目前唯一有效的治疗方法，必要时可行联合脏器切除术，但不应强求根治；为改善生存质量、延长生存期，对多发肿瘤不能完整切除者应积极实施姑息性减瘤术，多次复发者有条件可多次手术；采用完整切除术、尽量避免肠切除治疗对多发性及复发性肠系膜脂肪肉瘤具有重要临床价值。     

肠系膜巨大脂肪瘤合并肠系膜血管压迫1例报告

本文报道2016年6月手术切除肠系膜巨大脂肪瘤1例,39岁男性,体检发现腹腔内占位,肿瘤几乎占满腹腔。入院后完善检查,考虑腹腔内巨大脂肪瘤可能性大,不排除高分化脂肪肉瘤。术中见肿瘤来源于肠系膜,包膜完整,瘤体几乎占满整个腹腔,术中将肿瘤完整切除,术后恢复良好,随访3个月无复发。肠系膜脂肪瘤是一种十分罕见的实体肿瘤,通过该病例报道使外科医生加深对这种"沉默的肿瘤"的认识。     

腹膜后脂肪肉瘤诊断及治疗现状与展望

腹膜后脂肪肉瘤是腹膜后软组织肉瘤中最常见的病理分型,早期无典型的临床表现。腹膜后腔潜在空间巨大,肿瘤生长至非常巨大时才能触及肿块或产生压迫症状,多数病人肿瘤较大时才被发现,对邻近组织器官易产生压迫或推移作用。本病的临床表现不明显,无典型症状及体征,诊断主要依靠影像学检查及病理证实。此病放化疗无明显效果,手术切除仍是此疾病最有效的治疗手段,手术切除后多为原位复发,很少远处转移。由于本病恶性程度较低,多数病人可长期生存,早期完整手术切除可显著提高该病的无瘤生存时间及总生存时间。     

原发性腹膜后肉瘤的外科治疗68例临床报告

目的探讨原发性腹膜后肉瘤的诊断与治疗。方法对2000年1月至2010年1月经手术治疗的68例原发性腹膜后肉瘤的临床资料进行回顾性分析。结果原发性腹膜后肉瘤病理类型多样,本组68例中恶性纤维组织瘤9例,脂肪肉瘤33例,平滑肌肉瘤9例,未分化肉瘤7例,滑膜肉瘤4例,恶性外周神经鞘瘤6例。其中术后局部复发43例,5年存活率广泛切除为29%,局部切除的为43%。结论原发性腹膜后肉瘤的临床表现多为无症状性包块,组织学类型复杂,以脂肪肉瘤多见,预后与手术的彻底性和病理类型相关。     

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。     

Management of high-grade retroperitoneal liposarcomas: personal experience

Sarcomas are uncommon malignant tumors arising from mesenchymal tissue, accounting for approximately 10% of soft-tissue sarcomas and less than 1% of all malignant neoplasms. The most frequent subtypes of retroperitoneal sarcomas are liposarcoma. Total surgical extirpation appears the only chance for patient cure since medical therapies have shown little efficacy. We have analyzed our series of 32 retroperitoneal liposarcoma that undergone surgical resection and with at least 5 years of follow-up. We have included 21 patients who underwent a complete resection (R0) and 6 patients who underwent an incomplete resection (R1). However, the operation's goal was a complete tumor resection with tumor-free resection margins. The 5-year survival was 85.7% when we performed a R0 resection, while it was 33.3% when we performed a R1 resection with a significant statistical difference (p = 0.02). Surgery is the gold standard for treatment of liposarcoma. Despite advances in diagnostic modalities, surgical techniques and the adoption of more aggressive procedure, such disease still has a propensity for local recurrence, even after an apparent complete resection. Therefore, locoregional recurrence remains the main cause of death in patients with a retroperitoneal liposarcoma. Moreover when the tumor recurs locally, the treatment of choice is to remove the recurrence. Our experience shows that a complete resection of primary lesions and further resection of recurrences early detected with CT examination could improve the overall survival of patients with retroperitoneal liposarcoma. Moreover in our series, the patients who undergo R1 resection had a worse prognosis.     

63例原发性腹膜后肿瘤的诊断和外科治疗

目的 总结原发性腹膜后肿瘤诊断和外科治疗经验.方法 回顾性分析1990年1月至2007年3月63例经手术治疗且病理证实的原发性腹膜后肿瘤的临床表现、手术治疗、病理类型和随访结果.结果 63例中良性25例,恶性38例.主要临床表现为腹部包块,CT对良恶性判断的约登指数为85%,良性肿瘤完整切除率为88%,恶性肿瘤为68%,联合脏器切除占肿瘤完整切除总数的40%.肿瘤切除程度和病理类型与术后复发密切相关.良、恶性肿瘤完整切除的5年生存率分别为83.6%和27.3%.恶性肿瘤完整切除的患者平均随访36个月(5～168个月),53%局部复发,平均复发时间25个月(3～108个月),恶性肿瘤局部复发再手术完整切除率62.5%.全组有1例术后第1天腹腔内出血死亡.结论 应当重视临床表现争取早期诊断;影像学检查是判断手术范围的重要依据;肿瘤完整切除,必要时联合脏器切除是治疗本病的最佳手段;术后规律复查有助于及早发现复发肿瘤并争取再手术治疗.     

Retroperitoneal liposarcoma extending into the thigh

The authors describe a case of a giant retroperitoneal liposarcoma that extended into the thigh along the course of the iliopsoas muscle. The tumor was successfully excised en bloc through an abdominal and a proximal thigh incision. Histopathologic examination revealed a well-differentiated, lipoma-like liposarcoma with clear surgical margins. The patient remains well without any evidence of tumor recurrence 18 months after surgery.     

Histologic subtype and margin of resection predict pattern of recurrence and survival for retroperitoneal liposarcoma

OBJECTIVE: The aim of this study was to determine the pattern of recurrence and prognostic significance of histologic subtype in a large series of patients with primary retroperitoneal liposarcoma. SUMMARY BACKGROUND DATA: Classification of liposarcoma into subtypes, based on morphologic features and cytogenetic aberrations, is now widely accepted. Previous studies have shown that high histologic grade and incomplete gross resection are the most important prognostic factors for survival in patients with retroperitoneal sarcoma and suggest that patients with liposarcoma have a 3-fold higher risk of local recurrence compared with other histologies. METHODS: A prospective database was used to identify 177 patients with primary retroperitoneal liposarcoma treated between July 1982 and June 2002. Histology at primary presentation was reviewed by a sarcoma pathologist and subtyped into 4 distinct groups according to strict criteria. The influence of clinicopathological factors on local recurrence, distant recurrence, and disease-specific survival was analyzed. RESULTS: Of 177 patients with primary retroperitoneal liposarcoma operated on for curative intent, 99 (56%) presented with well-differentiated, 65 (37%) with dedifferentiated, 9 (5%) with myxoid, and 4 (2%) with round cell morphology. The tumor burden was determined by the sum of the maximum tumor diameters. The median tumor burden was 26 cm (5-139). Median follow-up time for 92 (52%) surviving patients was 37 (mean, 0.5-192) months. Multivariate analysis showed that dedifferentiated liposarcoma subtype was associated with a 6-fold increased risk of death compared with well-differentiated histology (P < 0.0001). In addition to histologic subtype, incomplete resection (P < 0.0001), contiguous organ resection (excluding nephrectomy; P = 0.05), and age (P = 0.03) were important independent prognostic factors for survival in retroperitoneal liposarcoma. Retroperitoneal dedifferentiated liposarcoma was associated with an 83% local recurrence rate and 30% distant recurrence rate at 3 years. CONCLUSIONS: The histologic subtype and margin of resection are prognostic for survival in primary retroperitoneal liposarcoma. Dedifferentiated histologic subtype and the need for contiguous organ resection (excluding nephrectomy) was associated with an increase risk of local and distant recurrence. Nephrectomy may be needed to achieve complete resection, but has no measurable influence on disease specific survival.     

A case of a large retroperitoneal liposarcoma presenting as an incarcerated inguinal hernia

We report a rare case of retroperitoneal liposarcoma developing within an incarcerated inguinal hernia. A 53-year-old man presented to our hospital with left inguinal mass. Preoperative computed tomography revealed an intraabdominal huge mass, and a clinical diagnosis of liposarcoma. Physical findings except for the inguinal mass and laboratory examination, including tumor markers (CEA, CA19-9), were within normal range. Intraoperatively, the mass was incarcerated in the inguinal canal and involved the left testis. We performed a radical tumor resection, including a left orchiectomy. The resected specimen measured 45 × 30 cm and weighed 7,510 g. Histopathologically, the tumor was diagnosed as a well-differentiated liposarcoma, and originated from retroperitoneum. We did not undergo adjuvant therapy in consideration of histopathological subtype and curable surgical treatment. The patient is well and shows no evidence of recurrence four months after the operation.     

原发性腹膜后脂肪肉瘤复发和预后的影响因素

目的探讨原发性腹膜后脂肪肉瘤复发和预后的影响因素。方法回顾性分析2011年6月至2020年1月郑州大学第一附属医院接受手术治疗的89例原发性腹膜后脂肪肉瘤患者的临床资料,男42例,女47例。中位年龄53(26~78)岁。我院首次手术治疗65例,外院术后复发再次手术治疗24例。初诊临床表现:体检发现腹膜后肿物41例,腹胀12例,腹痛10例,发热11例,恶心、呕吐、纳差8例,尿频、排尿困难6例,双下肢水肿1例。术前CT检查示肿瘤主体位于腹膜后肾区58例,位于腹膜后间隙、盆腔腹膜外间隙31例;肿瘤单发55例,多发34例。肿瘤长径中位值20(3~52)cm。首诊65例中通过术前影像学检查考虑为原发性腹膜后脂肪肉瘤47例(72.3%)。89例手术中,腔镜手术78例,其中腹腔镜手术21例,后腹腔镜手术38例,达芬奇机器人辅助腹腔镜手术19例;开放手术11例。87例行根治性切除术,2例行姑息性切除术;42例术中行邻近脏器切除术。对患者复发及生存情况进行随访。结果本组89例手术均顺利完成。术中出血量中位值200(10~2000)ml。病理类型为高分化型23例,去分化型40例,黏液样/圆形细胞型20例,多形性型5例,混合型1例。组织学分级低级别42例,高级别47例。术后中位随访时间28(3~108)个月。患者5年无复发生存率、无病生存率和总生存率分别为16.7%、16.1%和52.6%。57例局部复发,1例肺转移,1例肝转移,中位无病生存时间24个月;42例死亡,中位生存时间64个月。单因素分析结果显示,术中出血量(P<0.01)、肿瘤是否多发(P<0.01)、病理类型(P<0.01)、组织学分级(P<0.01)是影响无病生存时间的相关因素;术中出血量(P<0.01)、肿瘤是否多发(P<0.05)、病理类型(P<0.05)、是否复发(P<0.01)是影响总生存时间的相关因素。性别、年龄、肿瘤大小、肿瘤部位、是否初次手术、是否根治性切除、是否联合邻近脏器切除对患者的预后生存无影响(P>0.05)。Cox回归模型多因素分析结果显示,术中出血量(RR=2.360,95%CI 1.313~4.241,P=0.004)、肿瘤是否多发(RR=1.899,95%CI 1.068~3.375,P=0.029)、病理类型(RR=4.976,95%CI 1.622~15.264,P=0.005)是影响无病生存时间的独立因素;肿瘤是否复发是影响患者总生存时间的独立因素(RR=31.495,95%CI 1.062~933.684,P=0.046)。结论腹膜后脂肪肉瘤临床少见,复发率高。手术出血量、肿瘤是否多发、病理类型是影响患者无病生存时间的独立因素,肿瘤是否复发是影响患者总生存时间的独立因素。     

Retroperitoneal Liposarcoma With Leiomyosarcomatous Differentiation

We herein describe a 60-year-old Japanese man with a giant retroperitoneal liposarcoma undergoing leiomyosarcomatous differentiation. He was admitted to our hospital because of a 5-month history of dysphagia and abdominal distention. Abdominal computed tomography showed a giant tumor that occupied the entire retroperitoneal space. The majority of the mass was lipomatous and low density; both a heterogenous and solid mass were also present. A giant retroperitoneal liposarcoma was diagnosed, and tumor resection was performed. At surgery, the tumor was mostly isolated from the retroperitoneum and other organs. Histopathologically, the tumor comprised well-differentiated and dedifferentiated liposarcoma with heterologous differentiation of the leiomyosarcomatous components, which is a rare phenomenon in liposarcoma. The patient was alive 3 years after the first treatment, although he has had 3 local recurrences (approximately one recurrence yearly) and has been treated by repeated resection and radiotherapy.Key words: Retroperitoneum, Liposarcoma, Leiomyosarcomatous differentiationDedifferentiated liposarcoma (DL) is one of the most frequent sarcomas of the retroperitoneum. It is defined by the association of an atypical lipomatous tumor, namely, areas of well-differentiated liposarcoma (WDL), with a dedifferentiated component. WDL is composed of mature adipocytes and atypical stromal cells with an enlarged, hyperchromatic nucleus. Usually, the dedifferentiated part of the liposarcoma is composed of either a spindle/pleomorphic high-grade sarcoma or a mixoid/spindle cell low-grade sarcoma. The WDL component may be easily overlooked, and DL may thus be mistaken for another high-grade sarcoma. It has been reported in one study that approximately 5% of the dedifferentiated component showed heterologous differentiation, such as leiomyosarcoma, rhabdomyosarcoma, osteosarcoma, and angiosarcoma.¹ A less common phenomenon is the occurrence of WDL with leiomyosarcomatous (LMS) differentiation.² Limited to the retroperitoneum, only 8 cases of liposarcoma with LMS components have been reported.^3–7 We herein report a case of retroperitoneal liposarcoma comprising WDL and DL, with LMS components, treated by surgical resection.     

Giant recurrent retroperitoneal liposarcoma initially presenting as inguinal hernia: Review of literature

INTRODUCTIONLiposarcomas comprise around 15% of soft tissue tumors. These tumors of mesodermal origin arise as single tumors, present one histologic type and diverse locations (including the retroperitoneum). Diagnosis of liposarcomas of retroperitoneum is difficult because of this unspecific presentation and in 50–100% of the cases there is recurrence from residual tissue.PRESENTATION OF CASEAn 86 year old male patient was admitted in 1996 due to a right and voluminous inguinal hernia. During the herniaplasty, a right paratesticular tumor was isolated and removed. The histologic exam revealed a well-differentiated liposarcoma. A CT scan was performed and a large abdominal mass was detected. The patient underwent a laparotomy and an incomplete resection of the tumor was achieved. After the surgery the patient remained asymptomatic during a long period. Nine years later, the patient underwent another laparotomy with partial removal of the giant recurrent retroperitoneal liposarcoma.CONCLUSIONThe purpose of this publication is to report the recurrence of giant retroperitoneal liposarcoma, which is an unusual presentation in surgery today. Furthermore, we would like to emphasize the long-term survival of this patient despite partial resection and the possibility of performing a re-resection in this type of cases.     

Locoregional Disease Patterns in Well-Differentiated and Dedifferentiated Retroperitoneal Liposarcoma: Implications for the Extent of Resection?

Background

Well-differentiated (WD)/dedifferentiated (DD) liposarcoma is the most common soft tissue sarcoma of the retroperitoneum. The frequency of distant metastasis is low and the major burden of disease is locoregional. We sought to define the patterns of locoregional disease to help guide surgical decision making.

Methods

Data were collected from 247 patients with de novo or recurrent tumors treated at our institution from 1993 to early 2012. The number and location of tumors at both initial presentation and subsequent locoregional recurrence were determined by combined analysis of operative dictations and radiologic imaging.

Results

Thirty-four percent of patients had multifocal locoregional disease (two or more tumors) at initial presentation to our institution, including 9 % who had tumors at synchronous remote retroperitoneal sites. The impact of multifocal disease on overall survival was dependent on histologic subtype (WD vs. DD) and disease presentation (de novo vs. recurrence) at the time of resection. Among patients with initial unifocal disease, 57 % progressed to multifocal locoregional disease with subsequent recurrence, including 11 % with new tumors outside of the original resection field. No clinicopathologic or treatment-related variable, including the type or extent of resection, was predictive of either multifocal or ‘outside field’ progression.

Conclusions

Multifocal disease is common in patients with WD/DD retroperitoneal liposarcoma, and tumors can also develop at remote, locoregional sites. Surgical resection remains the primary method of locoregional control in this disease; however, the aggressiveness of resection should be individualized, with consideration of both tumor and patient-related factors.