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,     

38例腹膜后脂肪肉瘤的临床分析

目的 分析腹膜后脂肪肉瘤的治疗结果。方法 1958～1998年我院收治38例腹膜后脂肪肉瘤。21例为首程治疗,17例局部复发后再程治疗。21例初治患者均接受手术治疗,手术完整切除率为86％。3例接受术后放射治疗。17例外院术后复发再程治疗患者有13例在我院接受手术,4例接受单纯放疗。再程手术完整切除率为54％,1例接受术后放放射治疗。放疗剂量DT30Gy～70Gy,中位剂量50Gy。结果 全组患者5年总生存率为50％,5年远转率为7.3％,5年局部复发率为65％,初程治疗和再程治疗组的5年总生存率为53％和48％,无显著性差异(P=0.293,5年局部复发率为59％和74％,无显著差别(P=0.615)。全组手术完整切除组5年生存率为58％,部分切除或未切除者5年生存率为27％,有显著性差异(P=0.006)。34例有肿瘤大小记录的患者,其中4例肿瘤<10cm,均存活;22例肿瘤10～30cm,有13例死亡;8例肿瘤≥30cm,有6例死亡。结论 腹膜后脂肪肉瘤的治疗以手术为主,手术完整切除可显著提高生存率。经积极治疗,复发患者仍能取得很好的效果。     

原发性腹膜后软组织肉瘤20例临床分析

原发性腹膜后软组织肉瘤是一类临床上比较罕见的局部侵袭性肿瘤。本文对１９７６～１９９３年收治２０例病人作回顾性分析：首次诊断符合率仅３０％，结合影像学检查，诊断符合率达８５％，Ｂ超和ＣＴ在诊断中价值极大。手术是治疗本病的主要手段。手术完整切除肿瘤占４５％，部分切除占２０％，活检占３５％，指出提高本病治愈率关键是完全切除肿瘤，对切除后复发者，强调再次、多次手术，以提高生存率。     

多见于青壮年的软组织肉瘤

软组织肉瘤是一类起源于间叶组织、周围及植物神经组织和一些不明来源的高异质性恶性肿瘤。据欧美及国内的统计数据表明,软组织肉瘤每年发病率约为2～3/10万,占成人实体恶性肿瘤的比例不足1%,却占儿童约10%;患者多见于青壮年,发病的高峰年龄在20～50岁之间,男女比例为3:2;肿瘤可发生于躯体的任何部位,其中约50%发生于四肢(下肢占多数),躯干和腹膜后可占40%,头颈部可占约10%。软组织肉瘤中最常见的类型依次为:恶性纤维组织细胞瘤、脂肪肉瘤、平滑肌肉瘤、纤维肉瘤、横纹肌肉瘤、滑膜肉瘤及间质瘤等,良恶状况与其组织来源密切相关。比如隆突性皮肤纤维肉瘤、纤维肉瘤、脂肪样脂肪肉瘤等属低度恶性;表浅的恶性纤维组织细胞瘤、粘液脂肪肉瘤、平滑肌肉瘤等属中度恶性;滑膜肉瘤、深部的恶性纤维组织细胞瘤、腺泡状及胚胎性横纹肌肉瘤、血管肉瘤等属高度恶性。     

腹膜后脂肪肉瘤的治疗

脂肪肉瘤是四肢及腹膜后最常见的软组织肉瘤,约占成人腹膜后软组织肉瘤的40%[1].腹膜后脂肪肉瘤(retroperitoneal liposarcomas,RPLS)发病年龄多在在40~60岁之间,男女发病率大致相等.     

大腿软组织肉瘤术后淋巴液漏4例临床分析

自20世纪70和80年代，Enneking提出肉瘤切除的手术原则及外科分期系统以后，软组织肉瘤单独扩大广泛切除术取得了与截肢相似的效果，局部复发率为5％～10％。然而有关于软组织肉瘤广泛切除术后并发淋巴液漏的报道国内外较少，我科自1996年8月～2005年2月共收治大腿软组织肉瘤22例，其中术后并发淋巴液漏4例，均经保守治疗治愈，现报道如下。[第一段]     

原发性256例软组织肉瘤手术治疗的临床分析

目的：探讨软组织肉瘤的诊断、手术治疗方法及其疗效。方法：对256例原发性软组织肉瘤患者的治疗情况进行回顾性分析。行局部广泛切除及根治切除术,对侵犯骨组织的软组织肉瘤则按微波原位灭活保肢手术处理。切除肿瘤后,行血管修复重建、带血管蒂游离皮瓣转移、局部皮瓣转移、肌腱移位、肌皮瓣移位。结果：发病年龄为20-70岁,占62.11%（159/256）,常见的病理类型为滑膜肉瘤、恶性纤维组织细胞瘤、脂肪肉瘤、横纹肌肉瘤、纤维肉瘤,占65.23%（167/256）。Kaplan-Meier法计算5年生存率为63.28%。结论：诊断采用CT、MRI为主的影像学,治疗以手术为主,辅助放疗和化疗。     

原发性256例软组织肉瘤手术治疗的临床分析

目的:探讨软组织肉瘤的诊断、手术治疗方法及其疗效。方法:对256例原发性软组织肉瘤患者的治疗情况进行回顾性分析。行局部广泛切除及根治切除术,对侵犯骨组织的软组织肉瘤则按微波原位灭活保肢手术处理。切除肿瘤后,行血管修复重建、带血管蒂游离皮瓣转移、局部皮瓣转移、肌腱移位、肌皮瓣移位。结果:发病年龄为20-70岁,占62.11%(159/256),常见的病理类型为滑膜肉瘤、恶性纤维组织细胞瘤、脂肪肉瘤、横纹肌肉瘤、纤维肉瘤,占65.23%(167/256)。Kaplan-Meier法计算5年生存率为63.28%。结论:诊断采用CT、MRI为主的影像学,治疗以手术为主,辅助放疗和化疗。     

腹腔肉瘤的治疗进展

腹腔肉瘤系临床较难治疗的一类肿瘤，由于累及部位不同，与其它癌肿处理有所区别。现将此类肿瘤近期治疗现状予以介绍。1腹膜后软组织肉瘤腹膜后肿瘤约80％为恶性，而软组织肉瘤占其中40％－50％。目前腹膜后肉瘤的治疗仍存在许多难点，由于肿瘤体积较大，常累及邻近器官，外科手术如何确定切缘，是否行联合脏器切除，如何控制术中大出血，术后防止复发等均系临床考虑的问题。1．l手术切口腹膜后肉瘤的手术切口应以充分暴露肿瘤，便于解剖、易于止血及减少肿瘤细胞扩散和避免创面污染为原则。应认识到切口选择是决定肿瘤是否能彻底切除的保…     

软组织肉瘤动脉灌注新辅助化疗后的保肢治疗

目的探讨术前动脉内灌注化疗对软组织肉瘤的疗效及在保肢治疗中的意义。方法28例肢体软组织肉瘤,其中恶性纤维组织细胞瘤18例,滑膜肉瘤3例,脂肪肉瘤6例,原始神经外胚层肿瘤（PNET）1例。术前均给予选择性肿瘤供血动脉灌注化疗2-3周期,化疗药物为阿霉素／顺铂和异环磷酰胺。化疗后均行保肢手术治疗。结果28例术前化疗后均有疼痛缓解、皮温降低、肿胀减轻、瘤体不同程度缩小、边缘变清楚及关节活动度增加。化疗后均行肿瘤广泛切除术,术中获得良好的外科切除界限。随访6-46个月,平均25个月。2例死亡,1例局部复发行截肢术。术后12个月后采用MSTS93评分系统评分为24-29分,平均为27分。28例3年生存率为92.9％,初次保肢率100％,最终保肢率89.3％。结论肢体软组织肉瘤术前动脉灌注新辅助化疗是一种有效的保肢治疗方法。     

前列腺癌重碳离子放疗计划与调强放疗计划的比较

目的比较前列腺癌重碳离子放疗（C-ion RT）与调强放疗（IMRT）在剂量学方面的差异。方法随机选取5例前列腺癌患者,分别设计4野共面的C-ion RT计划和7野共面的IMRT计划。剂量均采用百分剂量,95％的等剂量面必须包括100％的计划靶体积（PTV）。比较靶区剂量分布的适形度指数（CI）和异质性指数（IC）,根据剂量体积直方图（DVH）,比较相同剂量水平下C-ion RT计划与IMRT计划中周围器官及非靶区正常组织的照射体积。结果在C—-ion RT计划中,CI50%、CI94%、IC分别为3．36、1．20和0．03,与IMRT计划比较差异有统计学意义（P均〈0．01）,靶区剂量分布的CI和IC均优于IMRT计划。除了95％的剂量水平外,在10％、30％、50％、70％和90％剂量水平,采用C—ion RT均可明显减少直肠的受照射体积（P均〈0．05）,同时完全保护直肠的后壁;在任何剂量水平,C—ion RT可明显减少膀胱和非靶区正常组织的受照射体积（P均〈0．05）;在10％、20％、30％和40％剂量水平,C—ion RT可明显减少双侧股骨头的受照射体积（P均〈0．05）。结论在前列腺癌的放射治疗中,与IMRT计划相比,C—ion RT计划在剂量学方面有明显优势,C—ion RT的这些优势将能够进一步提高前列腺癌的局部控制率,减少放疗引起的并发症。     

Mesenchymal stem cells are resistant to carbon ion radiotherapy

Mesenchymal stem cells (MSCs) participate in regeneration of tissues damaged by ionizing radiation. However, radiation can damage MSCs themselves.Here we show that cellular morphology, adhesion and migration abilities were not measurably altered by photon or carbon ion irradiation. The potential for differentiation was unaffected by either form of radiation, and established MSC surface markers were found to be stably expressed irrespective of radiation treatment. MSCs were able to efficiently repair DNA double strand breaks induced by both high-dose photon and carbon ion radiation. We have shown for the first time that MSCs are relatively resistant to therapeutic carbon ion radiotherapy. Additionally, this form of radiation did not markedly alter the defining stem cell properties or the expression of established surface markers in MSCs.     

重离子(12C6+)束治疗肿瘤初步临床报告

目的探讨碳离子（^12C^6＋）辐射对恶性肿瘤的治疗作用，初步确定治疗安全剂量，观察其急性副反应及近期疗效。方法12例表浅肿瘤患者（受试者）分为2个组，A组5．10～8．79GyE／次，1次／d，连续9d总剂量50．94～70．41GyE；B组5．04～8．58GyE／次，1次／d，连续7d总剂量35．88～60．06GyE。采用RTOG急性放射损伤分级标准判断急性损伤并通过局部肿物缩小程度评价近期疗效。结果治疗结束随访2个月后，A、B组受试者皮肤反应发生率分别为2／4、3／8，两组无明显血液毒性和严重皮肤反应。A、B组局部肿物缩小程度≥50％分别占3／4、5／8。结论 ^12C^6＋束治疗表浅肿瘤显示出良好的安全性和近期疗效。     

Impact of carbon ion radiotherapy for unresectable osteosarcoma of the trunk

BACKGROUND:

The authors summarized the outcomes of patients with unresectable osteosarcoma of the trunk who received carbon ion radiotherapy (CIRT).

METHODS:

The authors performed a retrospective analysis of 78 patients who had medically inoperable osteosarcoma of the trunk and received treatment with CIRT between 1996 and 2009. Tumor sites included the pelvis in 61 patients, the spine and paraspinal region in 15 patients, and other sites in 2 patients. The median applied CIRT dose was 70.4 Gray equivalent (GyE) in a total of 16 fixed fractions over 4 weeks.

RESULTS:

The minimum duration of follow‐up for survivors was 14 months. Forty‐eight patients remained alive. The 5‐year overall survival rate was 33%, and the local control rate was 62%. Thirty‐eight patients who had a clinical target volume <500 cm³ had a 5‐year overall survival rate of 46% and a 5‐year local control rate of 88%. Except for 3 patients who experienced severe skin/soft tissue complications requiring skin grafts, no other severe toxicities were observed. Of 9 patients who were continuously disease free for >5 years, 8 were able to walk with or without the help of a cane, and 6 were free from pain killers.

CONCLUSIONS:

CIRT appeared to be a safe and effective modality for the management of unresectable osteosarcoma of the trunk, providing good local control and offering a survival advantage and good long‐term functional results without unacceptable morbidity. Cancer 2012. © 2012 American Cancer Society.     

Carbon ion radiotherapy eradicates medulloblastomas with chromothripsis in an orthotopic Li-Fraumeni patient-derived mouse model

BackgroundMedulloblastomas with chromothripsis developing in children with Li-Fraumeni Syndrome (germline TP53 mutations) are highly aggressive brain tumors with dismal prognosis. Conventional photon radiotherapy and DNA-damaging chemotherapy are not successful for these patients and raise the risk of secondary malignancies. We hypothesized that the pronounced homologous recombination deficiency in these tumors might offer vulnerabilities that can be therapeutically utilized in combination with high linear energy transfer carbon ion radiotherapy.MethodsWe tested high-precision particle therapy with carbon ions and protons as well as topotecan with or without PARP inhibitor in orthotopic primary and matched relapsed patient-derived xenograft models. Tumor and normal tissue underwent longitudinal morphological MRI, cellular (markers of neurogenesis and DNA damage-repair), and molecular characterization (whole-genome sequencing).ResultsIn the primary medulloblastoma model, carbon ions led to complete response in 79% of animals irrespective of PARP inhibitor within a follow-up period of 300 days postirradiation, as detected by MRI and histology. No sign of neurologic symptoms, impairment of neurogenesis or in-field carcinogenesis was detected in repair-deficient host mice. PARP inhibitors further enhanced the effect of proton irradiation. In the postradiotherapy relapsed tumor model, median survival was significantly increased after carbon ions (96 days) versus control (43 days, P < .0001). No major change in the clonal composition was detected in the relapsed model.ConclusionThe high efficacy and favorable toxicity profile of carbon ions warrants further investigation in primary medulloblastomas with chromothripsis. Postradiotherapy relapsed medulloblastomas exhibit relative resistance compared to treatment-naïve tumors, calling for exploration of multimodal strategies.     

成人胶质瘤质子与重离子治疗现状及进展

脑胶质瘤是最常见的原发脑恶性肿瘤,标准治疗是手术联合术后放疗,但疗效并不理想。质子和碳离子治疗是目前最先进的放疗技术,在多种肿瘤中显示出优于光子放疗疗效和生存质量。然而,质子与碳离子治疗应用于胶质瘤的作用尚未明确,本文将就质子与碳离子治疗成人胶质瘤的基础研究和临床结果进行详细阐述。     

Single fraction carbon ion radiotherapy for colorectal cancer liver metastasis: A dose escalation study

Prognosis is usually grim for those with liver metastasis from colorectal cancer (CRC) who cannot receive resection. Radiation therapy can be an option for those unsuitable for resection, with carbon ion radiotherapy (CIRT) being more effective and less toxic than X‐ray due to its physio‐biological characteristics. The objective of this study is to identify the optimal dose of single fraction CIRT for colorectal cancer liver metastasis. Thirty‐one patients with liver metastasis from CRC were enrolled in the present study. Twenty‐nine patients received a single‐fraction CIRT, escalating the dose from 36 Gy (RBE) in 5% to 10% increments until unacceptable incidence of dose‐limiting toxicity was observed. Dose‐limiting toxicity was defined as grade ≥3 acute toxicity attributed to radiotherapy. The prescribed doses were as follows: 36 Gy (RBE) (3 cases), 40 Gy (2 cases), 44 Gy (4 cases), 46 Gy (6 cases), 48 Gy (3 cases), 53 Gy (8 cases) and 58 Gy (3 cases). Dose‐limiting toxicity was not observed, but late grade 3 liver toxicity due to biliary obstruction was observed in 2 patients at 53 Gy (RBE). Both cases had lesions close to the hepatic portal region, and, therefore, the dose was escalated to 58 Gy (RBE), limited to peripheral lesions. The 3‐year actuarial overall survival rate of all 29 patients was 78%, and the median survival time was 65 months. Local control improved significantly at ≥53 Gy (RBE), with a 3‐year actuarial local control rate of 82%, compared to 28% in lower doses. Treatment for CRC liver metastasis with single‐fraction CIRT appeared to be safe up to 58 Gy (RBE) as long as the central hepatic portal region was avoided.