颅内67例脊索瘤临床治疗的回顾性分析

目的 探讨发生于颅内脊索瘤的临床表现、影像学特点、组织学类型、手术方法、辅助治疗及远期预后.方法 回顾性总结分析67例颅内脊索瘤患者的临床资料,并介绍2例斜坡复发脊索瘤.结果 发生于颅底鞍斜区的脊索瘤临床表现复杂,主要表现为颅内站位效应及肿瘤对周围神经损害,首发症状以头痛、视力下降、耳鸣、耳聋及后组颅神经受损症状为主,病理分型:普通型即经典型脊索瘤56例,软骨型脊索瘤8例,去分化型3例.影像学表现67例患者均行CT及MRI检者表现为欠规则肿块影,以膨胀性生长为主,颅底多有骨质破坏肿块与周围软组织边界清楚,2、5年复发率为37.3％、67.1％.结论 手术治疗为主要方法,但术后复发率高,根据病变位置、涉及范围选择不同手术人路,必要时联合入路可增加全切几率,尽可能全切除肿瘤对预后至关重要,术后放疗可延缓肿瘤复发时间、减少复发率.     

骶骨脊索瘤病理分型与预后

目的 探讨骶骨脊索瘤临床病理分型与预后的关系。方法,分析24例患者的临床表现、病理分型,手术治疗及随访结果。结果 软骨瘤9例,平均生存5．5年,典型脊索瘤15例,平均生存3．5年。3例手术完全切除,随访2、2．5、4年末复发;不完全切除21例,2年复发13例。结论 软骨型脊索瘤比典型脊索瘤预后佳。包括骶骨的肿瘤完全切除术预后良好,但常难以实现。     

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。     

乳腺癌保乳治疗十年随访

目的研究乳腺癌保乳治疗的远期疗效及规范化治疗。方法对1990年3月至2010年3月行保乳治疗的93例乳腺癌进行回顾性分析。结果经过125个月的中位随访期,局部复发率3．2％（3／93）,总生存率89．2％（83／93）,美容效果满意率90．3％（84／93）。5年生存率92．3％,元瘤生存率为89．2％;10年生存率89．3％,无瘤生存率为85．7％。在预后相关指标单因素分析中,未能发现年龄、肿瘤大小、临床分期、腋淋巴结转移等因素对预后产生影响。结论早期乳腺癌行保乳治疗,原发肿瘤较大甚至临床分期较晚的乳腺癌经新辅助化疗缩小病灶后行保乳治疗,局部复发率较低,生存率较高,并且明显改善了患者的生活质量。     

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.     

224例无淋巴结转移的进展期胃癌预后因素分析

目的探讨无淋巴结转移的进展期胃癌预后危险因素。方法回顾性分析福建省立医院1998—2008年10年间行D2胃癌根治术的236例淋巴结清扫数目在12枚以上、无淋巴结转移（N0期）的进展期胃癌患者的临床病理资料。结果本组患者中224例（94．9％）接受了随访,其5年总体生存率和5年无瘤生存率分别为75．2％和66．4％。T2期和T3期患者的复发率分别为5．8％（8／138）和14．0％（12／86）：5年总体生存率分别为82．5％和59．0％;5年无瘤生存率分别为70．4％和52．2％：差异均有统计学意义（均P〈0．05）。单因素预后分析显示,浸润深度、Lauren分型及淋巴结清扫数目与本组胃癌患者预后有关（P〈0．05）。多因素预后分析证实,浸润深度是本组患者的独立预后因素（P〈0．05）。结论T2N0期胃癌患者的预后明显优于T3N0期患者;对N0期胃癌患者决定是否行辅助治疗及制定随访策略时应考虑浸润深度这一因素。     

直肠癌尖淋巴结转移的危险因素分析

为探讨直肠癌尖淋巴结（APN）转移与其预后及与转移淋巴结总数的关系,回顾性研究510例行直肠癌切除术病理诊断淋巴结阳性患者的临床资料,并对APN与患者预后和N分期之间的关系进行单因素及多因素分析。全组患者术后平均随访47个月,术后5年局部复发率18．3％,无瘤生存率55．5％,总生存率58．8％。     

综合治疗侵犯海绵窦包绕颈内动脉的斜坡脊索瘤1例

脊索瘤是罕见的骨恶性肿瘤,起源于胚胎残留的脊索组织,过去认为脊索瘤相对于颅底更多见于骶骨,然而,现有证据表明,其在颅底( 32％)、脊柱(32.8％)和骶骨(29.2％)的分布几乎是均等的[1].虽然脊索瘤为低度恶性肿瘤,但易局部侵犯且预后不良.对于发生于斜坡的脊索瘤,目前公认的治疗原则是最大限度地安全地切除肿瘤,术中...     

胸腺瘤患者预后因素分析

目的 探讨影响胸腺瘤患者术后远期生存率的相关因素。方法 回顾性分析我科1973—2000年间手术治疗的69例胸腺瘤，应用Kaplan—Meier法和Cox比例风险模型对可能影响胸腺瘤术后远期生存率的因素进行单因素和多因素分析。结果 全组患者5年、10年、15年生存率分别为83．3％、67．4％、41．9％。单因素分析显示年龄、Masaoka分期、WHO组织学分类、肿瘤切除范围、Rosai／Levine分类对胸腺瘤患者术后长期生存率有显著影响(P＜0．01)，但经多因素分析表明仅Masaoka分期(P＜0．01)、肿瘤切除范围(P＜0．05)、年龄(P＜0．05)是独立的预后因素。结论 对胸腺瘤应积极进行手术治疗，即使姑息性切除亦有助于提高远期生存。     

二期翻修治疗人工全髋关节置换术后感染的中期疗效评估

目的对全髋关节置换术后感染行二期翻修术治疗的患者进行中期的随访,并进行疗效评估。方法自2004年1月～2007年12月,本组采用二期翻修术治疗人工髋关节术后感染22例（22髋）。一期手术彻底清创,取出感染假体,置入抗生素骨水泥间隔器;二期手术植入骨水泥型或非骨水泥型假体。以感染复发率、疼痛评分（VAS评分）、髋关节功能评分（Harris评分）评估术后疗效。结果平均随访周期为（6．6±1．8）年（5～8年）,22例患者均未发现感染复发、假体松动和脱位等。治疗前VAS评分平均为7．2分（5—9．1分）,一期手术后VAS评分平均为3．8分（1．2～5．3分）,最后一次随访时VAS评分平均为1．2分（0—3．2分）。治疗前Harris评分平均为36．4分（20～49分）,一期手术后Harris评分平均为56．3分（40～66分）,最后随访时Harris评分平均为84．2分（72—93分）。结论使用抗生素骨水泥间隔器二期翻修术中期随访控制感染效果显著,为全髋置换术后感染提供了有效治疗途径。     

Skull base chordomas: efficacy of surgery followed by carbon ion radiotherapy

Summary Background  Skull base chordomas are challenging to treat because of their invasive nature, critical location, and aggressive recurrence. We report the effectiveness of combined radical skull base surgery with carbon ion radiotherapy for treating skull base chordomas. Methods  Between November 1996 and August 2007, 32 patients (12 males and 20 females; mean age at initial presentation 41.4 years, range, 10–75 years) with skull base chordomas underwent 59 operations. Sub-total resection (resection of >90% of the pre-operative tumour volume) was achieved in 24 out of 59 (40.7%) operations. After surgical excision, 9/32 (28.1%) patients underwent adjuvant carbon ion radiotherapy. The patients were followed up for a mean period of 36.3 months (range, 3–93 months) from the initial presentation. Findings  Fifty percent of the patients had to be re-treated due to tumour regrowth during the follow-up period. Thus far, 3 patients have died from the neoplasms. The overall 7 year survival rate of 9 patients who underwent carbon ion radiotherapy was 85.7%. The rate was higher compared to that of others (76.4%). The 3 year recurrence free survival rates of carbon ion therapy treated group was 70.0%, being higher than that of the other groups treated with radiotherapy or untreated (57.1%, and 7.1% respectively). Log-rank analysis showed a significant difference in the recurrence free survival rates between the group treated with adjunctive carbon ion radiotherapy and the untreated group (P = 0.001146). Conclusions  Surgical removal of the tumour around the brainstem and the optic nerve combined with post-operative carbon ion radiotherapy will improve the survival rate and quality of life of patients with complicated skull base chordomas.     

Gamma Knife surgery for skull base chordomas and chondrosarcomas

OBJECT: The purpose of this study was to evaluate radiosurgical outcomes in skull base chordomas and chondrosarcomas, and to determine which tumors are appropriate for stereotactic radiosurgery as adjuvant therapy following maximum tumor resection. METHODS: Thirty-seven patients (48 lesions) were treated using Gamma Knife surgery (GKS); 27 had chordomas, seven had chondrosarcomas, and three had radiologically diagnosed chordomas. The mean tumor volume was 20 ml, and the mean maximum and marginal doses were 28 and 14 Gy, respectively. The mean follow-up period was 97 months from diagnosis and 59 months from GKS. RESULTS: The actuarial 5- and 10-year survival rates after GKS were 80 and 53%, respectively. The actuarial 5- and 10-year local tumor control (LTC) rates after single or multiple GKS sessions were 76 and 67%, respectively. All patients with low-grade chondrosarcomas achieved good LTC. A tumor volume of less than 20 ml significantly affected the high rate of LTC (p = 0.0182). No patient had adverse radiation effects, other than one in whom facial numbness worsened despite successful tumor control. CONCLUSIONS: As an adjuvant treatment after resection, GKS is a reasonable option for selected patients harboring skull base chordomas or chondrosarcomas with a residual tumor volume of less than 20 ml. Dose planning with a generous treatment volume to avoid marginal treatment failure should be made at a marginal dose of at least 15 Gy to achieve long-term tumor control.     

Prognostic factors for long-term outcome of patients with surgical resection of skull base chordomas—106 cases review in one institution

Skull base chordoma are still challenging. Between May 1993 and June 2005, 106 consecutive patients with skull base chordoma underwent surgical removal at Skull Base Division of Neurosurgery, Beijing Tiantan Hospital, China. Retrospective analysis included medical charts and images. The age of the patients ranged from 7 to 65 years old, with an average age of 35.6 years. Sixty patients were male; the other 46 were female (1.3:1). Follow up data were available in 79 cases ranging from 10 to 158 months (average 63.9 months) after operation. The prognostic factors for recurrence and survival were analyzed with Kaplan‐Meier, Cox regression and t‐test. Overall, 1, 3, 5 and 10 years survival rates were 87.2%, 79.4%, 67.6%and 59.5% respectively. One, 3, 5 and 10 year recurrent rates were 19.1%, 34.7%, 52.9% and 88.3%, respectively. The long term outcome of the skull base chordomas is poor. The previous radiotherapy or surgery, dedifferentiated pathology, and less tumor resection are risk factors for longterm survival and recurrence (p < 0.05). Although there is no statistic significant role of tumor adherent to vital structure for outcome (p = 0.051), it can not exclude its importance for favorable outcome. Gender, age, tumor size and staging are not independent risk factors for outcome. Surgical technique leading to radical tumor resection with less morbidity is advocatory and beneficial for patients with skull base chordoma with long term outcome, if the tumor could be exposed and resected completely, the recurrence rate was very low for most benign chordomas.     

Proton Therapy for Skull Base Chordomas: An Outcome Study from the University of Florida Proton Therapy Institute

Objectives Skull base chordoma is a rare, locally aggressive tumor located adjacent to critical structures. Gross total resection is difficult to achieve, and proton therapy has the conformal advantage of delivering a high postoperative dose to the tumor bed. We present our experience using proton therapy to treat 33 patients with skull base chordomas. Design Retrospective outcomes study. Setting University of Florida Proton Therapy Institute; 2007 to 2011. Participants A total of 33 patients with skull base chordomas received postoperative three-dimensional conformal proton therapy. The patients were 79% male and 6% diabetic; 27% had received a gross total resection. Main Outcome Measures The gross tumor/tumor bed received a dose between 77.4 CGE and 79.4 CGE. Local control and overall survival were tracked, and radiation toxicity was assessed using a modified Radiation Therapy Oncology Group/European Organization for Research and Treatment of Cancer Late Radiation Morbidity Scoring Scheme. Results Median follow-up for all patients was 21 months. Local control and overall survival rates at 2 years were 86% and 92%, respectively. Grade 2 toxicity was observed in 18% of our cohort in the form of unilateral hearing loss partially corrected with a hearing aid. No grade 2 or higher optic or brainstem toxicities were observed. Conclusions Proton therapy is an effective treatment modality for skull base chordomas.     

A multidisciplinary team approach to skull base chordomas.

OBJECT: A multidisciplinary team devised a protocol for long-term care of patients with skull base chordomas. In this study they describe their approach. METHODS: Forty-two patients presented between 1986 and 1998 and were treated by maximum surgical cytoreduction and photon radiation therapy. Tumor volume-doubling time determined on the basis of magnetic resonance imaging, immunostaining, and cell proliferation (Ki67 labeling index [LI]) studies indicated growth rates of individual chordomas. The best outlook was associated with the greatest extent of tumor removal achieved during the first operation. There were no deaths associated with patients who underwent first-time surgery, but there was a 7.1% mortality rate associated with those who underwent subsequent operations. Cerebrospinal fluid leaks, additional cranial nerve palsies, and pharyngeal wound problems were the most difficult management problems encountered after second and subsequent surgeries. The time interval between operations was usually between 2 years and 3 years after the first surgery; very few patients required a second surgery, with a quiescent period in excess of 5 years. Life-table 5- and 10-year survival rates were 77% and 69%, respectively. CONCLUSIONS: The authors believe that this series of skull base chordomas provides new insights into the management of these lesions, particularly with regard to techniques that increase survival times and studies that aid in formulating prognoses.     

A multidisciplinary team approach to skull base chondrosarcomas.

OBJECT: The authors review their experience with treating skull base chondrosarcomas, which are much rarer than skull base chordomas and differ from them in prognosis and treatment. METHODS: Seventeen patients (12 male and five female patients) with histologically verified chondrosarcomas were followed up prospectively over a 12-year period. The mean age at presentation was 35.9 years. Most patients presented with cranial nerve palsies. Seven had undergone surgery prior to referral to the authors' unit. All underwent maximum surgical cytoreduction by the most direct surgical approach; only the two patients harboring the mesenchymal variant underwent radiotherapy. CONCLUSIONS: One patient died of a pulmonary embolus; the patients harboring mesenchymal chondrosarcomas died at 20 and 36 months, respectively, after treatment. Of the remaining patients, 93% were alive 5 years postsurgery and had a projected 10-year survival rate of 84% (mean survival time 9.3 years). These data emphasize the very slow progression of this tumor compared with skull base chordoma.     

Endoscopic endonasal approach in the management of skull base chordomas—clinical experience on a large series,technique, outcome,and pitfalls

Skull base chordomas represent very interesting neoplasms, due to their rarity, biological behavior, and resistance to treatment. Their management is very challenging. Recently, the use of a natural corridor, through the nose and the sphenoid sinus, improved morbidity and mortality allowing also for excellent removal rates. Prospective analysis of 54 patients harboring a skull base chordoma that were managed by extended endonasal endoscopic approach (EEA). Among the 54 patients treated (during a 72 months period), 21 were women and 33 men, undergoing 58 procedures. Twenty-two cases (40 %) were recurrent and 32 (60 %) newly diagnosed chordomas. Among the 32 newly diagnosed chordomas, a gross total resection was achieved in 28 cases (88 %), a near total (>95 % of tumor) in 2 cases (6 %), a partial (>50 % of tumor) in 2 cases (6 %). Among the 22 recurrent chordomas, resection was complete in 7 cases (30 %), near total in 7 (30 %), and partial in 8 (40 %). The global gross total resection rate was 65 % (35/54 cases). Four patients (11 %) recurred and 4 (11 %) progressed within a mean follow-up of 34 months (range 12–84 months). Four patients (11 %) were re-operated; one patient (1.8 %) died due to disease progression, one patient (1.8 %) died 2 weeks after surgery due to a massive bleeding from an ICA pseudo aneurysm. CSF leakage occurred in four patients (8 %), and meningitis in eight cases (14 %). No new permanent neurological deficit occurred. The EEA management of skull base chordomas requires a long and gradual learning curve that once acquired offers the possibility of either similar or better resection rates as compared to traditional approaches while morbidity is improved.     

Les chordomes

Purposes

To review in the literature, all the epidemiological, clinical, radiological, histological and therapeutic data regarding chordomas as well as various notochordal entities: ecchordosis physaliphora, intradural and intraparenchymatous chordomas, benign notochordal cell tumors, parachordomas and extra-axial chordomas. To identify different types of chordomas, including familial forms, associations with tuberous sclerosis, Ollier's disease and Maffucci's syndrome, forms with metastasis and seeding. To assess the recent data regarding molecular biology and progress in targeted therapy. To compare the different types of radiotherapy, especially protontherapy and their therapeutic effects. To review the largest series of chordomas in their different localizations (skull base, sacrum and mobile spine) from the literature.

Materials

The series of 136 chordomas treated and followed up over 20 years (1972–2012) in the department of neurosurgery at Lariboisière hospital is reviewed. It includes: 58 chordomas of the skull base, 47 of the craniocervical junction, 23 of the cervical spine and 8 from the lombosacral region. Similarly, 31 chordomas in children (less than 18 years of age), observed in the departments of neurosurgery of les Enfants-Malades and Lariboisière hospitals, are presented. They were observed between 1976 and 2010 and were located intracranially (n = 22 including 13 with cervical extension), 4 at the craniocervical junction level and 5 in the cervical spine.

Methods

In the entire Lariboisière series and in the different groups of localization, different parameters were analyzed: the delay of diagnosis, of follow-up, of occurrence of metastasis, recurrence and death, the number of primary patients and patients referred to us after progression or recurrence and the number of deaths, recurrences and metastases. The influence of the quality of resection (total, subtotal and partial) on the prognosis is also presented. Kaplan-Meier actuarial curves of overall survival and disease free survival were performed in the entire series, including the different groups of localization based on the following 4 parameters: age, primary and secondary patients, quality of resection and protontherapy. In the pediatric series, a similar analysis was carried-out but was limited by the small number of patients in the subgroups.

Results

In the Lariboisière series, the mean delay of diagnosis is 10 months and the mean follow-up is 80 months in each group. The delay before recurrence, metastasis and death is always better for the skull base chordomas and worse for those of the craniocervical junction, which have similar results to those of the cervical spine. Similar figures were observed as regards the number of deaths, metastases and recurrences. Quality of resection is the major factor of prognosis with 20.5 % of deaths and 28 % of recurrences after total resection as compared to 52.5 % and 47.5 % after subtotal resection. This is still more obvious in the group of skull base chordomas. Adding protontherapy to a total resection can still improve the results but there is no change after subtotal resection. The actuarial curve of overall survival shows a clear cut in the slope with some chordomas having a fast evolution towards recurrence and death in less than 4 years and others having a long survival of sometimes more than 20 years. Also, age has no influence on the prognosis. In primary patients, disease free survival is better than in secondary patients but not in overall survival. Protontherapy only improves the overall survival in the entire series and in the skull base group. Total resection improves both the overall and disease free survival in each group. Finally, the adjunct of protontherapy after total resection is clearly demonstrated. In the pediatric series, the median follow-up is 5.7 years. Overall survival and disease free survival are respectively 63 % and 54.3 %. Factors of prognosis are the histological type (atypical forms), localization (worse for the cervical spine and better for the clivus) and again it will depend on the quality of resection.

Conclusions

Many different pathologies derived from the notochord can be observed: some are remnants, some may be precursors of chordomas and some have similar features but are probably not genuine chordomas. To-day, immuno-histological studies should permit to differentiate them from real chordomas. Improving knowledge of molecular biology raises hopes for complementary treatments but to date the quality of surgical resection is still the main factor of prognosis. Complementary protontherapy seems useful, especially in skull base chordomas, which have better overall results than those of the craniocervical junction and of the cervical spine. However, we are still lacking an intrinsic marker of evolution to differentiate the slow growing chordomas with an indolent evolution from aggressive types leading rapidly to recurrence and death on which more aggressive treatments should be applied.     

Radiosurgery for chordomas and chondrosarcomas of the skull base

OBJECT: Chordomas and chondrosarcomas of the skull base are aggressive and locally destructive tumors with a high tendency for local progression despite treatment. The authors evaluated the effect of stereotactic radiosurgery (SRS) on local tumor control and survival. METHODS: Twenty-eight patients with histologically confirmed chordomas (18) or chondrosarcomas (10) underwent Gamma Knife SRS either as primary or adjuvant treatment. Their ages ranged from 17 to 72 years (median 44 years). The most common presenting symptom was diplopia (26 patients, 93%). In two patients, SRS was the sole treatment. Twenty-six patients underwent between one and five additional surgical procedures. Two underwent an initial transsphenoidal biopsy. The average tumor volume was 9.8 cm3. The median dose to the tumor margin was 16 Gy. RESULTS: No patient was lost to follow-up. Transient symptomatic adverse radiation effects developed in only one patient. The actuarial local tumor control for chondrosarcomas at 5 years was 80 +/- 10.1%. For chordomas both the actuarial tumor control and survival was 62.9 +/- 10.4%. CONCLUSIONS: Stereotactic radiosurgery is an important option for skull base chordomas and chondrosarcomas either as primary or adjunctive treatment. Multimodal management appears crucial to improve tumor control in most patients.     

Proton radiation therapy for chordomas and chondrosarcomas of the skull base.

OBJECT: Local tumor control, patient survival, and treatment failure outcomes were analyzed to assess treatment efficacy in 58 patients in whom fractionated proton radiation therapy (RT) was administered for skull base chordomas and chondrosarcomas. METHODS: Between March 1992 and January 1998, a total of 58 patients who could be evaluated were treated for skull base tumors, 33 for chordoma and 25 for chondrosarcoma. Following various surgical procedures, residual tumor was detected in 91% of patients; 59% demonstrated brainstem involvement. Target dosages ranged from 64.8 and 79.2 (mean 70.7) Co Gy equivalent. The range of follow up was 7 to 75 months (mean 33 months). In 10 patients (17%) the treatment failed locally, resulting in local control rates of 92% (23 of 25 patients) for chondrosarcomas and 76% (25 of 33 patients) for chordomas. Tumor volume and brainstem involvement influenced control rates. All tumors with volumes of 25 ml or less remained locally controlled, compared with 56% of tumors larger than 25 ml (p = 0.02); 94% of patients without brainstem involvement did not experience recurrence; in patients with brainstem involvement (and dose reduction because of brainstem tolerance constraints) the authors achieved a tumor control rate of 53% (p = 0.04). Three patients died of their disease, and one died of intercurrent disease. Actuarial 5-year survival rates were 100% for patients with chondrosarcoma and 79% for patients with chordoma. Grade 3 and 4 late toxicities were observed in four patients (7%) and were symptomatic in three (5%). CONCLUSIONS: High-dose proton RT offers excellent chances of lasting tumor control and survival, with acceptable risks. In this series all small- and medium-sized tumors with no demonstrable brainstem involvement have been controlled; all such patients are alive. Surgical debulking enhanced delivery of full tumoricidal doses, but even patients with large tumors and disease abutting crucial normal structures benefited.