骶骨脊索瘤的手术治疗结果评价

例出现局部复发(42.5%).28例第2～3次手术的患者中,再次复发者18例(64.3%).51例患者共进行79次手术,25例次(31.6%)术后出现伤口并发症.11例次(13.9%)患者术后出现脑脊液漏.结论 骶骨脊索瘤为低度恶性局部侵袭性强的肿瘤,广泛切除是治疗骶骨脊索瘤的关键.S3以下的肿瘤可行广泛切除,对于累及S3以上的肿瘤为最大限度的保留神经功能,采用切刮术加高压冲洗可以有效地降低局部复发率.     

Role of intraoperative radiotherapy in the treatment of sacral chordoma

Background Context

Sacral chordoma is a rare entity with high local recurrence rates when complete resection is not achieved. To date, there are no series available in literature combining surgery and intraoperative radiotherapy (IORT).

Review of current treatment of sacral chordoma

Chordoma is a relatively rare, locally aggressive tumor which is known to arise from embryonic remnants of the notochord and to occur exclusively along the spinal axis, with a predilection for the sacrum. Although chordoma typically presents as a single lesion, a few cases of metastasis have been reported and the prognosis of such patients may be poor. Chordomas are slowly growing tumors with insidious onset of symptoms, making early diagnosis difficult. Recent improvements in imaging have provided valuable information for early diagnosis. The optimal treatment for sacral chordoma is en bloc sacral resection with wide surgical margins. Improvement in surgical techniques has widened the opportunities to provide effective treatment. However, the effects of adjuvant treatment options are still both unclear and controversial. Substantial progress has been made in the study of molecular‐targeted therapy. The authors review the current surgical and adjuvant treatment modalities, including molecular‐targeted therapy, available for management of sacral chordoma.     

骶骨脊索瘤的临床特点及预后因素分析

目的探讨骶骨脊索瘤的临床特点和影响手术预后的相关因素,从而判定影响骶骨脊索瘤的局部复发和总体生存的因素。方法回顾性分析1991年1月~2012年12月在本院行手术治疗的骶骨脊索瘤患者资料95例。应用单因素生存分析和多因素Cox比例风险模型判定独立危险因素。生存率通过Kaplan-Meier曲线计算,通过对数秩检验判定统计学差异。选择P≤0.1的影响因素进入多因素Cox比例风险模型,判定独立危险因素。结果本组病例平均随访29个月,其中33例患者术后局部复发,局部复发率为34.7%(33/95);20例患者在本研究随访终止前死亡,总体生存率78.9%(75/95),术后生存时间(78.8±4.7)个月。在各临床因素中,术前Frankel分级、Enneking分期及手术方式与局部复发密切相关;年龄、性别、术前Frankel分级、术前选择性肿瘤动脉栓塞术与患者死亡密切相关。结论外科手术可提高骶骨脊索瘤患者的生存质量,延长患者生存期。30~70岁的男性患者总体生存率较高,术前较好的Frankel分级、EnnekingⅠ期、全脊椎en-bloc切除术可明显降低骶骨脊索瘤复发率;Frankel分级可作为术后总体生存率的独立预后因素。     

Hand metastasis from a sacral chordoma

Chordomas are rare, low grade, malignant tumours derived from the ectopic remnants of the notochord that line the axial skeleton. They are characterised by their slow growth, long disease course and propensity for local relapse. Furthermore, up to 40% of non-cranial chordomas metastasise. We describe the first reported case of a hand metastasis arising from a conventional sacral chordoma after carbon ion radiotherapy. The common occurrence of distant metastasis with chordomas makes it important to perform a systemic examination, in part because their resection might improve patient prognosis.     

骶骨脊索瘤的临床特点及综合治疗

目的介绍骶骨脊索瘤的临床特点并对其综合治疗的疗效和相关问题进行分析。方法对1999年12月~2006年12月收治的行以手术切除为主的综合治疗的40例骶骨脊索瘤病例进行回顾性分析。结果本组病例无术中死亡,术后肿瘤局部复发13例,复发率为32.5%,其中3例复发2次;发生肺部转移2例,转移发生率为5%。术后5年内共5例死亡,患者经过综合治疗后的术后5年生存率达87.5%。结论以手术切除为主、辅以术中化疗、术后放疗的综合治疗方法,对降低骶骨脊索瘤的局部复发率、延长患者生存期、提高患者生活质量有重要作用,是治疗骶骨脊索瘤的有效方法。     

En bloc resection of a C4 chordoma: surgical technique

The prognosis of aggressive benign and low-grade malignant tumors in the spine as in the limbs, seems to be mostly related to the feasibility of en bloc resection, while in the treatment of high-grade malignant tumors the protocols of treatment include the combination of chemotherapy, radiation and surgery. Indications, criteria of feasibility and surgical technique are extensively reported for the thoracic and lumbar spine. In the cervical spine few cases are reported of resection, due not only to anatomical constraint, but also to the rarity of finding a tumor accomplishing the criteria of feasibility. A case of double-approach vertebrectomy finalized to remove en bloc the body of C4 for a stage IA chordoma is reported. The first stage was posterior, aiming to remove the posterior healthy elements by piecemeal technique. The anterior approach consisted of contemporary right and left prevascular presternocleidomastoid approaches The specimen was submitted for the histological study of the margins, which resulted tumor-free. This technical note is finalized to confirm that en bloc resection of the vertebral body through total vertebrectomy is feasible in the midcervical spine by double approaches, provided the tumor involves only layers B and C, maximum extension sectors 5–8.     

Endoscopic transsphenoidal resection of a large chordoma in the posterior fossa

Summary Encouraged by an experience with endoscopic transsphenoidal pituitary surgery, an endoscopic transsphenoidal technique was applied in a patient with a large chordoma in the posterior fossa. The patient was a 40-year-old man with a two-year history of progressive ataxia, a memory disorder and emotional instability. A magnetic resonance (MR) scan of the brain revealed a midline posterior fossa mass measuring 4 cm in diameter located between the clivus and the brainstem. The basilar artery and its bifurcation were encased by the tumor and the brainstem was also distorted by the tumor. Obstructive hydrocephalus was treated previously with a ventriculoperitoneal shunt and fractionated external beam radiation treatment was given without histological diagnosis at another hospital. Subtotal resection of the tumor was achieved utilizing an endoscopic transsphenoidal technique through the patient's nostril. The portion of the tumor located behind the basilar artery was not resected in order to protect the brainstem perforating arteries. The patient showed dramatic improvement of his symptoms postoperatively. Residual tumor located behind the basilar artery was treated by stereotactic gamma-knife surgery. This is the first reported case of a large posterior fossa chordoma being treated by an endoscopic transsphenoidal technique.     

Wide resection of sacral chordoma via a posterior approach

Purpose  

The study was carried out to report the results of wide resection in sacral chordoma using a posterior approach and gauze packing technique.     

Routine shave margins are not necessary in early stage breast cancer treated with Breast Conserving Surgery

Introduction

Breast Conserving Surgery (BCS) is considered standard of care for women with early stage breast cancer. Between 20 and 50% of women treated with BCS will require re-operation for positive or close margins and it has been suggested that routine cavity shave margins may reduce the frequency of positive margins.

胸腰椎脊索瘤的临床特点和治疗分析

目的:探讨胸、腰椎脊索瘤的临床特点和治疗方法。方法:对7例胸、腰椎脊索瘤患者的临床资料进行总结,根据WBB分期,实施椎体或全脊椎切除,其中囊内切除3例、扩大切除4例。结果:术后5例患者症状完全消失。1例T7、T8脊索瘤患者出现切口下缘顽固性疼痛,封闭后疼痛减轻。1例L1~L3脊索瘤全瘫患者,双下肢肌力恢复至2级。病理检查镜下均可见“印章样细胞环”和“合胞体”。术后平均随访5.2年,局部复发1例,无远处转移。结论:胸腰椎脊索瘤相对少见,临床表现不典型,容易误诊。“印章样细胞环”和“合胞体”是胸、腰椎脊索瘤的典型病理表现。全脊椎切除肿瘤能降低局部复发率。     

脊索瘤的发病特点及治疗进展

<正>脊索瘤是一种相对罕见的间叶源性恶性肿瘤,发生率为0.08/10万^[1-2],占原发恶性脊柱肿瘤的20%^[3]。脊索瘤好发于中老年男性,主要位于融合节段脊柱,如颅底斜坡、骶尾骨^[2]。虽然脊索瘤生长相对缓慢,但其具有较强的局部侵袭性,常导致患区局部疼痛。同时,由于脊索瘤常毗邻脑干、脊髓等重要神经组织,其生长常压迫周围神经组织,导致肢体感觉运动障碍、瘫痪等并发症,严重影响患者生活质量,甚至威胁患者生命^[4]。目前,     

Safety of MRI at 1.5Tesla in patients with implanted sacral nerve neurostimulator

OBJECTIVES: Sacral neuromodulation has become an established method to treat voiding dysfunction. Currently the use of implanted sacral nerve stimulators is becoming more popular worldwide. Magnetic resonance imaging (MRI) is an important diagnostic tool for many medical and neurological disorders. Many radiology centers do not perform MRI examinations on patients with implanted sacral nerve stimulator. The basis for this policy is that potential hazards such as motion, dislocation or torquing of the implanted pulse generator (IPG), heating of the leads, and damage to the IPG may occur, resulting in painful stimulation. In contrast, many studies conducted on MRI at 1.5Tesla in patients with implantable devices have found the examination to be safe if the area to be imaged is out of the isocenter of the MRI scanner and other precautions are taken. METHODS: Eight MRI examinations at 1.5Tesla were conducted in areas outside the pelvis on six patients with implanted sacral nerve stimulator (InterStim neurostimulator; Medtronic, Inc, Minneapolis, MN, USA). Implanted pulse generators were examined before and after MRI procedures. All patients had their parameters recorded; then the IPGs were put to "nominal" status. Patients were monitored continuously during and after the procedure. After the MRI session, the site of the implanted device was examined and changes were reported. Devices were then re-programmed to their previous setup with the use of a programmer (model 7432; Medtronic, Inc). Voiding diaries were collected after MRI procedures and compared with previous records. RESULTS AND CONCLUSION: During the MRI session, no patient showed symptoms that required stopping the examination. There was no change in perception of the stimulation after re-programming of the implanted sacral nerve stimulator, according to patients' feedback. Devices were functioning properly, and no change in bladder functions was reported after MRI examinations. Finally, we hope that presenting these cases will encourage performance of more comprehensive studies on implanted sacral nerve stimulators on a larger patient population in the near future.     

显微手术切除颅底沟通性脊索瘤

目的 探讨不同颅面入路治疗颅底沟通性脊索瘤的入路选择和显微手术方法.方法 手术治疗并经病理证实的颅底沟通性脊索瘤22例.采用颅面入路或联合入路显微手术切除,包括扩大的前颅底入路8例,改良Weber-Ferguson入路8例,下颌入路6例.其中部分向颅内生长明显的沟通性肿瘤,联合经颅手术,包括翼点入路3例,颞颧入路1例,枕下乙状窦后入路2例.对患者预后进行长期随访.结果 肿瘤全切除10例,次全切除7例,大部切除4例,部分切除1例.肿瘤全切除或次全切除占77.3%.术后无死亡和严重神经功能障碍患者.术后脑脊液漏1例,伤口感染1例,动眼神经不全麻痹2例,展神经麻痹1例.本组中20例进行了随访,随访1～10年,平均3.4年.恢复正常工作或部分工作12例,不能工作但能生活自理5例,生活不能自理1例,死于肿瘤复发2例.结论 颅底沟通性脊索瘤侵犯范围广泛,应争取手术尽可能全切.根据肿瘤的不同部位和生长方向,选择不同入路或联合入路.显微外科手术可提高切除率、降低并发症,同时应注意硬脑膜的修补和颅底的重建.     

Close surgical margins after radical prostatectomy mimic biochemical recurrence rates of positive margins

IntroductionThe significance of a “close” but negative surgical margin after radical prostatectomy (RP) is controversial. We evaluated the effect of a close surgical margin (CSM) on biochemical recurrence (BCR) compared to a negative margin after RP.Materials and methodsPathologic records of men who underwent RP from 2005-2011 were retrospectively reviewed. Margin status was classified as “positive” (PSM), “negative” (NSM), or “close” (<1 mm from margin). BCR was defined as 2 consecutive postoperative prostate specific antigen measurements >0.2 ng/ml. Probability of BCR was estimated using the Kaplan-Meier method and stratified by margin status. Univariable and multivariable Cox proportional hazards models were used to determine whether close margin status was associated with an increased rate of BCR.ResultsA total of 609 consecutive patients underwent RP (93% robotic) and had complete pathologic data. A total of 126 (20.7%) had PSM, 453 (74.4%) had NSM, and 30 (4.9%) had CSM (mean<0.44 mm). The 3-year BCR-free survival for patients with CSM was similar to those with PSM (70.4% vs. 74.5%, log rank P = 0.66) and significantly worse than those with NSM (90%, log rank P<0.001). On multivariable regression, positive margin status (HR = 3.26, P<0.001) was significantly associated with a higher risk of BCR, along with close margins (HR = 2.7, P = 0.04).ConclusionsBCR for patients with CSM at RP is tantamount to PSM patients. CSM <1 mm should be explicitly noted on pathology reports. Patients with this finding should be followed up closely and offered adjuvant therapy.     

颈椎脊索瘤术后5次迟发性出血1例报告

椎动脉出血（VAH）是颈椎手术中或术后出现的一类极为罕见的并发症,大出血可导致中枢神经系统永久性损伤,甚至导致患者死亡,其发生率为0.20%～1.96%^[1-5]。有研究^[3-5]表明,VAH多发生于颈椎退行性疾病的前路手术显露及后路手术椎弓根置钉等过程。目前VAH的文献报道较少,尚无确切的治疗方式。本院收治1例颈椎脊索瘤术后单侧椎动脉反复出血5次的病例,通过总结其诊疗经过探讨VAH的原因及诊治措施,现报告如下。     

Positive surgical margins during robotic radical prostatectomy: a contemporary analysis of risk factors

OBJECTIVE

To determine the risk factors (clinical, pathological and technical) for positive surgical margins (PSMs) after robotically assisted radical prostatectomy (RARP), as a PSM is associated with an increased risk of biochemical recurrence and often responsible for significant patient anxiety.

PATIENTS AND METHODS

Between November 2003 and March 2007, 216 consecutive patients had an RARP by one fellowship‐trained urological oncologist. The surgical pathological specimens were fixed and processed using standard techniques, and assessed by a pathologist at the same institution. A PSM was defined as the presence of cancer adjacent to the inked margin. The clinical charts were reviewed retrospectively under an approved institutional review board protocol. Univariable and multivariable methods, including logistic regression models, were used to analyse the clinical, pathological and technical risk factors for PSM.

RESULTS

The overall prevalence of PSM was 14.8% (32/216), and 5.4% (8/149) for pT2 cancers. The only preoperative factor that was associated with a greater risk of a PSM was the serum prostate‐specific antigen (PSA) level (P = 0.012) and PSA density (P = 0.005). Age, clinical stage and clinical Gleason grade were not predictors of a PSM. The overall and pT2 PSM rate remained constant throughout the series of 216 patients (P = 0.371), indicating that the initial experience for RARP was not associated with a significantly greater risk of a PSM. However, there was a small independent ‘learning curve’ effect, with a lower rate of PSM associated with each increment of 25 patients (odds ratio 0.8, 95% confidence interval 0.6–1.0), supported by the significantly decreasing trend in PSM for pT3 cancers over time (P = 0.031) Although there was no significant increase over time in PSM with the use of an endostapler to control the dorsal venous complex (DVC), there was a significant learning effect, with a decrease in the PSM rate specifically in pT3 cancers using the suture technique (P = 0.005). A nerve‐sparing procedure increased the risk of PSM in multivariable analysis (P = 0.03). As expected, pathological stage and pathological Gleason grade were the strongest predictors of PSM (P < 0.001).

CONCLUSION

The most important risk factors for a PSM after RARP are the preoperative PSA level, PSA density, pathological stage and Gleason grade. PSM rates for a surgeon in their initial experience can be comparable to that of a surgeon experienced in RARP. Using a stapling device to control the DVC does not appear to increase the risk of a PSM, although nerve‐sparing increases the rates of PSM in extraprostatic prostate cancer.     

脊索瘤与脆性组氨酸三联体基因的相关性研究

目的 探讨脆性组氨酸三联体（FHIT）基因改变与脊索瘤发生的关系。方法 应用逆转录-聚合酶链反应（RT—PCR）和免疫组织化学（SP）方法对18例脊索瘤和瘤旁正常组织FHIT基因RNA转录本和蛋白表达进行检测。结果 RT-PCR检测可见11例（61．1％）存在FHIT基因RNA转录本缺失．1例还同时含有正常和异常转录本。对其中2例进行测序，证实1例转录本缺失全部E5～E7外显子及部分E8外显子和部分E10外显子等2个片段；另1例转录本缺失全部E5～E9外显子及绝大部分E10外显子。脊索瘤中FHIT蛋白表达阳性百分比为55．6％（10／18）．瘤周正常组织为100．0％（18／18），差异有统计学意义（P〈0．05），FHIT蛋白在脊索瘤中表达减低。结论 FHIT基因表达缺失是脊索瘤发生的重要事件之一，可能与脊索瘤的发生机制有关。