原发性骶骨脊索瘤的手术治疗

目的 介绍原发性骶骨脊索瘤手术治疗的初步经验。方法 采用前后路联合入路切除或刮除术治疗原发性骶骨脊索瘤8例，局部病灶清除减压治疗复发性骶骨脊索瘤1例。结果 7例术后2.5～4年获得随访，均无瘤正常生存，1例失访。1例死亡。结论 早期前后路联合入路手术是治疗骶骨脊索瘤的较好方法。对于脊索瘤术后复发或转移，施行姑息性病灶清除减压术，有利于减轻患者痛苦。     

骶骨脊索瘤手术切除及并发症的防治

目的 探讨骶骨脊索瘤的诊治及并发症的预防。方法 本组12例骶骨脊索瘤患者，术中5例行腹主动脉暂时套扎，7例行球囊导管腹主动脉血流阻断后，再将骶骨肿瘤切除。初次手术患者11例，第二次手术患者1例。结果 12例患者中，2例发生术后切口部分感染、坏死；1例出现局部巨大血肿；2例患者术后肿瘤复发。随访3个月至7年，平均3.2年。术中无一例死亡。结论 骶骨脊索瘤的手术切除成功的关键在于有效地控制术中失血。球囊导管腹主动脉阻断术阻断血流安全、可靠，能明显减少术中出血。术前准备工作在保证手术的安全方面具有同样的重要性。     

明胶海绵靶血管栓塞后手术治疗原发性骶骨肿瘤

目的探讨明胶海绵靶血管栓塞后手术治疗骶骨脊索瘤与骶骨巨细胞瘤的临床疗效。方法收集1994年1月至2008年1月靶血管栓塞后手术治疗61例原发性骶骨肿瘤患者的临床资料,对资料完整的35例骶骨脊索瘤与11例骨巨细胞瘤进行回顾性研究。所有患者术前均用明胶海绵进行靶血管栓塞,栓塞后平均1．5d内后方入路手术切除肿瘤。记录术中出血量与输血量,并观察大小便功能和肿瘤复发情况。结果所有患者瘤体均顺利切除,术中出血平均1230mL（200～5100mL）,平均输血量1000mL（0～4200mL）。平均随访53．6个月（8～156个月）,脊索瘤患者复发11例（31．4％）,其中死亡5例（14．3％）,骨巨细胞瘤复发1例（9．1％）。保留双侧S1-3神经根或保留双侧S1-2神经根及一侧S3神经根者术后大小便功能基本正常,余均有不同程度的大小便功能障碍。结论明胶海绵靶血管栓塞后手术治疗原发性骶骨肿瘤,可有效减少术中出血,增加肿瘤切除率和彻底切除的可能性;对肿瘤较大的患者后方入路仍能较好完成手术,疗效满意。     

骶骨外脊柱少见部位脊索瘤的临床病理特征分析

目的 探讨发生于脊柱少见部位的脊索瘤的临床特点及诊断和治疗方法。方法 收集2001年12月至2013年10月发生于颈、胸、腰椎的6例脊索瘤患者的临床资料,包括临床表现、影像学和病理学特点以及接受外科治疗的情况。结果 6例脊索瘤的发生部位：腰椎3例,颈椎2例,胸椎1例。6例均接受手术治疗,术后随访时间平均38.7个月（12~45个月）。术后复发2例,均为囊内切除边界。1例全身多发转移,术后5个月死亡。病理组织学检查均表现为脊索瘤经典型形态：肿瘤细胞片状分布或呈条索状和散在分布于丰富的黏液性间质中,肿瘤细胞主要由含空泡的液滴样细胞和嗜酸性星形细胞构成。结论 颈、胸、腰椎脊索瘤罕见,临床症状多不典型,容易漏诊或误诊,需结合影像学和病理学表现诊断,囊内切除后复发率高。     

靶血管栓塞后手术治疗复发性骶骨脊索瘤的疗效分析

目的探讨靶血管栓塞后手术治疗复发性骶骨脊索瘤的临床疗效。方法对1993～2008年间36例骶骨脊索瘤患者进行回顾性分析,将27例初发患者列为初发组,外院初次手术后复发来我院9例列为复发组。所有患者均采用靶血管栓塞后行后路手术。统计学分析两组病例术中出血量、输血量、术后引流量、伤口并发症、术后二便功能和复发情况。结果两组患者平均出血量、平均输血量、术后引流量差异无统计学意义,P〉0．05。平均随访68个月,复发组术后大小便功能障碍较初发组多（P〈0．05）。初发组复发10例,复发组再次复发7例,差异有统计学意义（P〈0．05）。结论选择性靶血管栓塞可有效地减少术中出血,对于复发性骶骨脊索瘤仍是安全有效的方法。复发性骶骨脊索瘤术后大小便功能恢复较初发患者差,并且较初发患者更易复发。     

21例骶尾部脊索瘤临床分析

目的：探讨骶尾部脊索瘤的临床特点及治疗方法。方法：回顾性分析天津医科大学附属肿瘤医院1989年1月至2004年12月收治的21例骶尾部脊索瘤患者的临床资料，11例接受单纯手术治疗，10例接受手术＋术后放疗，放射治疗剂量为40～50Gy。结果：9例患者死亡，6例死于局部复发，1例死于双肺转移，2例死于非肿瘤相关疾病。全组1、3、5、10年生存率分别为100％、95％、85％、41％。单纯手术组的5年生存率为81％，手术＋术后放疗组的5年生存率为89％，二者无显著性差异（P=0．256〉0．05）。局部复发15例，单纯手术组9例，局部复发率为81．8％，手术＋放疗组6例，局部复发率为60％，二者无显著性差异（x^2=1．222，P=0．269〉0．05）。单纯手术组局部复发时间为术后2个月-38个月，中位复发时间22个月；手术＋放疗组发生复发时间为术后23个月～个72月，中位复发时间48个月。结论：脊索瘤极易复发，患者就诊状态及治疗手段可能为影响复发的因素，手术切除为其主要治疗手段，术后辅以放疗未能明显提高生存率及减少局部复发，但推迟了局部复发时间。     

超选择肿瘤动脉栓塞联合单纯后路手术切除治疗骶骨脊索瘤的疗效观察

目的:探讨肿瘤动脉栓塞后手术切除治疗原发性骶骨脊索瘤的疗效。方法:回顾性分析我院2012年01月至2018年01月收治的30例原发性骶骨脊索瘤患者。其中19例采用DSA引导下超选择肿瘤动脉栓塞联合骶后方入路一期完全切除骶骨脊索瘤(A组),11例未行肿瘤动脉栓塞直接采用单纯后路一期手术切除(B组)。对两组患者的手术时间、术中出血量、术后引流量、术后引流管拔除时间、并发症发生率及术后肿瘤复发率进行比较分析。结果:所有患者均顺利完成手术,无术中直肠损伤、失血性休克或死亡,A组手术时间155～248 min(202.42±29.75)min,术中出血量685～1 885 mL(1 349.84±282.84)mL,术后引流量150～300 mL(227.26±35.82)mL,术后拔管时间7～9 d(7.42±0.61)d; B组手术时间220～300 min (271.45±21.78)min,术中出血量890～2 400 mL(1 846.45±396.38)mL,术后引流量220～330 mL(284.09±38.26)mL,术后拔管时间8～10 d(9.45±0.69)d。A组手术时间...     

计算机导航辅助骶骨脊索瘤的精确切除的手术效果和安全性研究

目的 骶骨脊索瘤发病率低,手术切除复杂,获得安全的外科边界困难,术后复发风险较高.本研究的目的 是评价计算机导航辅助骶骨脊索瘤切除的手术效果和安全性.方法 分析2009年至2016年,在我院接受计算机导航辅助切除的骶骨脊索瘤病例资料,共纳入32例,包括27例原发肿瘤和5例复发肿瘤.肿瘤的最高水平有29例位于S3水平以上...     

骶骨脊索瘤的外科治疗

骶骨脊索瘤的外科治疗郝云东副主任医师揭玉蓉，董秀英，韦孝明青岛市人民医院（２６６００１）１９８０年－１９９４年我院共收治原发性骶骨肿瘤１３例，其中脊索瘤７例，现报告如下：临床资料：本组原发骶骨肿瘤１３例，其中脊索瘤７例，（均经病理证实），男４例，女３...     

骶骨脊索瘤外科治疗长期随访

目的 回顾骶骨脊索瘤外科治疗后长期随访的结果,分析评估影响外科治疗效果的临床因素.方法 1978年10月至2000年10月,我院共收治68例骶骨脊索瘤,男性60例,女性8例,年龄25～74岁,中位年龄55.5岁,部位为S1～5 7例,S2～5 15例,S3～5 33例,S4～5 5例,其他8例.外科手术共104例次,首次在我院外科治疗的原发病例48例,肿瘤外科切除边界为广泛切除4例（8.3%）;边缘切除21例（43.7%）;囊内切除23例（48.0%）;复发病例20例.结果 随访1-365个月,平均81.84个月.存活53例（77.9%）;死亡15例（22.1%）,其中,围手术期内死亡7例,占死亡总数的46.7%.总体5年生存率87.3%,10年生存率73.3%,中位生存时间（月）282.0±88.7.其中囊内切除者、边缘切除者和广泛切除者5年生存率,三者比较统计学无明显差异（P=0.18）.手术囊内切除者复发34例（81.0%）,边缘切除者复发8例（36.4%）,统计学差异明显（P=0.000）;总体1年无复发生存率77.4%;3年33.2%,5年24.3%.我院首次手术与非我院首次手术患者的无复发生存率比较,统计学差异明显（log-rank P=0.000）.边缘切除与囊内切除患者的无复发生存率比较,统计学差异明显（log-rank P=0.000）.Cox回归分析显示是否我院首次手术及不同手术边界均为预测局部复发的独立因素.结论 骶骨脊索瘤外科切除,局部复发率高,生存期较长,外科切除边界是影响局部复发的重要因素,首次手术对于预后有重要影响.     

Chordoma and chondrosarcoma: similar, but quite different, skull base tumors

BACKGROUND: Chordoma and chondrosarcoma of the skull base are frequently amalgamated because of similar anatomic location, clinical presentation, and radiologic findings. The chondroid chordoma variant has been reported to carry a better prognosis. The objective of the current study was to investigate the distinctions between these 3 entities. METHODS: The data concerning 109 patients with chordoma, chondroid chordoma, and chondrosarcoma who were treated by a single surgeon with maximum surgical resection and frequently by adjunct proton beam radiotherapy between 1990 and 2006 were analyzed retrospectively. Pathologic distinction was established by cytokeratin and epithelial membrane antigen staining. Clinical, radiologic, pathologic, and cytogenetic studies were analyzed in relation to disease recurrence and death. RESULTS: The average follow-up was 48+/-37.5 months (range, 1-191 months). There were no reliable distinguishing clinical or radiologic features noted between the groups. Chondrosarcoma patients had a significantly better outcome compared with chordoma patients with regard to survival and recurrence-free survival (P=.028 and P<.001, respectively), whereas patients with chondroid chordoma had a poor outcome similar to chordoma patients with regard to survival and recurrence-free survival (P=.337 and P=.906, respectively). CONCLUSIONS: Chordoma and chondrosarcoma differ with regard to their origin and histology, and differ markedly with regard to outcome. Chondroid chordomas behave in a manner that is clinically similar to chordomas, with the same prognosis. Both chordoma types demonstrate an aggressive clinical course and poor outcome after disease recurrence. The optimal treatment for all groups of patients involves radical surgical resection followed by high-dose radiotherapy in patients with chordomas. Radiotherapy may not be necessary in patients with low-grade chondrosarcoma.     

Ectopic Recurrence of Skull Base Chordoma after Proton Therapy

Simple SummaryChordoma are very rare tumors of the spine and skull base. Due to close proximity of crucial organs, like the brain stem, complete removal can often not be achieved, and tumor tissue, either macroscopic or microscopic, remains in situ. Local recurrence up to 88% occurs in 10 years. Ectopic recurrence as an early sign of treatment failure is considered rare. We retrospectively reviewed five patients with ectopic recurrence as a first sign of treatment failure after treatment with surgery and proton therapy, and studied the applied treatment strategies and imaging follow-up. We found 18 ectopic recurrences in these five patients, of which 17 (94%) could be related to prior surgical tracts. Our theory is that these relapses occur due to microscopic tumor spill during surgery. These cells did not receive a therapeutic radiation dose. Advances in surgical possibilities and adjusted radiotherapy target volumes might improve local control and survival.AbstractBackground: Chordoma are rare tumors of the axial skeleton. The treatment gold standard is surgery, followed by particle radiotherapy. Total resection is usually not achievable in skull base chordoma (SBC) and high recurrence rates are reported. Ectopic recurrence as a first sign of treatment failure is considered rare. Favorable sites of these ectopic recurrences remain unknown. Methods: Five out of 16 SBC patients treated with proton therapy and surgical resection developed ectopic recurrence as a first sign of treatment failure were critically analyzed regarding prior surgery, radiotherapy, and recurrences at follow-up imaging. Results: Eighteen recurrences were defined in five patients. A total of 31 surgeries were performed for primary tumors and recurrences. Seventeen out of eighteen (94%) ectopic recurrences could be related to prior surgical tracts, outside the therapeutic radiation dose. Follow-up imaging showed that tumor recurrence was difficult to distinguish from radiation necrosis and anatomical changes due to surgery. Conclusions: In our cohort, we found uncommon ectopic recurrences in the surgical tract. Our theory is that these recurrences are due to microscopic tumor spill during surgery. These cells did not receive a therapeutic radiation dose. Advances in surgical possibilities and adjusted radiotherapy target volumes might improve local control and survival.     

Chordoma: 6 Years’ experience at a tertiary centre

Nine patients with a histologically proven diagnosis of chordoma seen at the Department of Radiation Oncology, Maulana Azad Medical College and Lok Nayak Hospital between January 1999 and December 2004 were retrospectively reviewed with respect to age, sex, presentation, location of tumour, treatment, response, recurrence, metastasis and follow up. Chordoma constituted 0.07% of total cancer cases registered over 6 years. Out of nine patients, eight were males and one was female with median age at time of diagnosis 52 years (range 34–68 years). All had sacrococcygeal lesions except one who had a spheno‐occipital lesion. Seven patients had undergone either subtotal or gross total resection whereas only biopsy had been carried out in two of them. All patients received radiation therapy, seven in a postoperative setting and two for palliation. Follow‐up period ranged from 2 to 50 months. Four patients died – the first after fourth fraction of radiation, second after 10 days of treatment, third of progressive lesion in sphenoidal region despite resection and radiation and fourth of local recurrence in the sacrococcyx. One patient developed distant metastases in the lungs and subcutaneous tissue over the scalp along with local recurrence; he is still alive. Two patients are locally free of disease whereas the other two were lost to follow up. The present analysis was undertaken to review our institutional experience with an aim to provide a practical approach to these tumours. In this report, these cases are discussed and the published works have been reviewed for the optimal management of patients with chordoma.     

咽喉部软骨样脊索瘤(附2例报告)

目的:探讨咽喉部软骨样脊索瘤的特点、诊断、治疗以及预后。方法:分析两例咽喉部软骨样脊索瘤患者的临床资料并进行文献回顾。结果:两例分别随访8月和1年均无复发。1例侵及环状软骨,行气管切开术。结论:咽喉部软骨样脊索瘤早期症状不典型,容易误诊和漏诊。彻底切除肿瘤是治疗关键。     

小肝癌切缘复发预防及临床处理方法的探讨

背景与目的随着医疗诊断技术的提高,越来越多的小肝癌被发现,目前治疗小肝癌首选的方法仍是手术切除,但其术后复发率高达60%以上。本研究中我们对临床小肝癌进行回顾对照分析,拟探讨降低小肝癌手术切缘复发率的方法。方法回顾性分析广东省开平市中心医院和中山大学肿瘤医院在1991年1月～2003年5月间收治的283例小肝癌患者的临床病理资料。由于85%以上的肝癌患者合并不同程度肝硬化和肝储备功能不良,故以非规则性肝切除术为主。其中作肿瘤剔出术加残端切缘注射无水乙醇或无水乙醇明胶海绵创面填塞术140例(研究组);143例仅作普通常规处理(对照组)。比较两组患者的年龄、性别、肿瘤部位、肿瘤分期、Child-Pugh分期无统计学意义。结果研究组和对照组切缘1年复发率分别为21.4%和4.4%,有显著性差异(P<0.05);肝内转移/再发率分别为26.1%和24.5%,无统计学意义(P>0.05)。研究组和对照组5年总生存率分别为57.3%、52.8%(P=0.48),5年无瘤生存率分别为35.2%、36.9%(P=0.51)。两组患者术后一周内体温均在39℃以下;均无明显白细胞升高、胆漏及出血出现。结论不规则肝切除术后残端用无水乙醇明胶海绵填塞,或在残端处注射无水乙醇,对减少术后残端局部复发有一定效果,但对术后转移复发再发无作用,此法简单经济实用,便于推广应用,副作用少。     

骶尾部脊索瘤11例的临床特征与治疗

目的：探讨骶尾部脊索瘤的临床特征与治疗方案。方法：分析１１例脊索瘤的临床表现、Ｘ线与病理组织学资料,总结诊治要点。结果：术后全组五年生存率为４５．５％,根治切除组为５５．６％。结论：手术切除加术后放疗为最佳治疗方法。     

腹部恶性纤维组织细胞瘤47例临床和预后分析

目的探讨腹部恶性纤维组织细胞瘤(MHF)的临床病理特点、治疗策略及预后相关因素。方法回顾性分析1970年1月至2000年1月间天津医科大学附属肿瘤医院47例腹部恶性纤维组织细胞瘤患者手术切除治疗情况。所有患者均经手术病理证实,病变单一。结果术后随访中共有32例患者出现复发、转移,术后单纯局部复发13例(29.5%),出现肺及胸膜转移7例(15.9%),肝转移4例(9.0%),骨转移6例(13.6%),肾脏转移2例(4.5%),复发 转移5例(11.4%)。术后1,3,5年无瘤生存率分别为65.9%、40.9%和29.5%,累计生存率分别为70.5%、52.3%和31.8%,中位生存期为18个月。影响预后的主要因素为肿瘤发生的部位、治疗方式、病理类型、肿瘤的局部复发等。以手术为主的综合治疗较单纯手术及放化疗预后好,术后辅以放疗可降低局部复发率,尤其对那些切端阳性患者。结论腹部MHF侵袭性强,复发率高,病理分级差,以手术切除为主辅以放疗有助于提高患者的远期生存率。     

心脏黏液瘤的外科治疗

目的 探讨心脏黏液瘤的诊断和治疗经验。方法 回顾性分析自1995年10月～2001年10月收治的83例心脏黏液瘤病例(其中左心房黏液瘤57例,右心房黏液瘤19例,左心室黏液瘤2例,右心室黏液瘤5例)。术前经彩色超声心动图确诊,均在体外循环下行黏液瘤摘除术,同期行二尖瓣成形术8例,二尖瓣机械瓣置换术3例,三尖瓣成形术62例,三尖瓣机械瓣置换术1例,房间隔缺损修补术6例,隔膜型主动脉瓣下狭窄解除术1例。结果 无围术期及手术死亡,随访72例,时间1个月到6年,心功能(NYHA分级):Ⅰ级51例,Ⅱ级11例,Ⅲ级4例,1例发生脑梗死死亡,1例死于车祸,1例死因不明,3例复发再次手术,复发率4.2％。结论 心脏黏液瘤一经确诊应尽快手术,手术效果满意,复发率低,彩色超声心动图对诊断及术后随访有重要作用,应注意术后随访。     

Surgical treatment of dermatofibrosarcoma protuberans. A retrospective study of 20 cases with review of literature

The clinicopathological features and results of surgical treatment of 20 patients with dermatofibrosarcoma protuberans (DFSP) were reviewed. All patients have been observed until the present time or death. The primary treatment usually consisted of marginal, wide or radical excision. Re-excision because of recurrence was performed in six of the 20 patients who were treated primarily only by marginal excision. Following re-excision two patients have remained disease-free until the present, the other four of these six patients had a second recurrence. Three of these four patients with a second relapse were successfully treated by excision. Although six patients out of 20 had 13 recurrences at the site of primary therapy, no patient developed lymph node or distant metastases. To date, none of the remaining 14 patients has had recurrence, all having been treated by excision of from 1 to 5 cm, the mean follow-up time was 8.75 years. Based on our data, the high recurrence rate after marginal surgical treatment implies an initial radical resection.