The main form of treatment of a chordoma of the mobile spine is total en bloc spondylectomy (TES), but the clinical results are not satisfactory. Stand-alone carbon ion radiotherapy (CIRT) for bone and soft-tissue sarcomas has recently been reported to have a high rate of local control with a low rate of local recurrence. . We report two patients who underwent TES after CIRT for treating a chordoma in the lumbar spine with good medium-term outcomes. At operation, there remained histological evidence of viable tumour cells in both cases. After the combination use of TES following CIRT, neither patient showed signs of recurrence at the follow-up examination. These two cases suggest that CIRT should be combined with total spondylectomy in the treatment of chordoma of the mobile spine. Cite this article: Bone Joint J 2013;95-B:1392–5

A chordoma which occurs as a primary tumour outside the axial skeleton is known as an extra-axial chordoma, parachordoma or chordoma periphericum. It is extremely rare and therefore survival, recurrence and the rates of metastasis are not known. Whilst few recurrences have been described, the extra-axial chordoma has the potential for late recurrence at up to 12 years. Metastases are even less frequent. We report the case of a 56-year-old woman who developed an extra-axial chordoma of the right thoracic wall in close relationship with the tenth rib. The tumour was completely removed and the prognosis is excellent

Chordoma of the cervical spine is a rare but life-threatening disease with a relentless tendency towards local recurrence. Wide en bloc resection is recommended, but it is frequently not feasible in the cervical spine. Radiation therapy including high-energy particle therapy is commonly used as adjuvant therapy. The goal of this study was to examine treatment and outcome of patients with chordoma of the cervical spine. Patients affected by cervical spine chordoma who underwent surgery at the Rizzoli Institute and University Hospital of Modena, between 2007 and 2021 were identified. The clinical, pathologic, and radiographic data were reviewed in all cases. Patient outcomes including local recurrence and disease-specific survival (DSS) were analyzed using chi-square test and Kaplan-Meier survival analysis. Characteristics of the 29 patients (10 females; 19 males) included: median age at surgery 52.0 years (IQR 35.5 - 62.5 years), 10 (35%) involved upper cervical spine, 16 (55%) with tumors in the mid cervical spine, and 4 in the lower cervical spine (10%). Median tumor volume was 16 cm. 3. (IQR 8.7 - 20.8). Thirteen patients (45%) were previously treated surgically while 9 patients (31%) had previous radiation therapy. All patients underwent surgery: en bloc resection was passible in 4 patients (14 %), seventeen patients (59%) were treated with gross total resection while 8 patients (27%) underwent subtotal resection. Tumor volume was associated with a significantly higher risk of intraoperative complications (p < 0.01). Nineteen patients (65%) received adjuvant high-energy particle therapy. The median follow-up was 26 months (IQR 11 - 44). Twelve patients (41%) had local recurrence of disease. Patients treated with adjuvant high-energy particle therapy had a significant higher local control than patients who received photons or no adjuvant treatment (p = 0.01). Recurrence was the only factor significantly associated with worse DSS (p = 0.03 – OR 1.7), being the survival of the group of patients with recurrent disease 58.3% while the survival of the group of patients with no recurrent disease was 100%. Post-operative high-energy particle therapy improved local control in patients with cervical chordoma after surgical resection. Increased tumor volume was associated with increased risk of intraoperative complications. Recurrence of the disease was the only factor significantly associated with disease mortality

We have treated 175 patients with a chordoma over a ten-year period. Only two had a family history of the condition and we describe these in this paper. In one patient the tumour was at the craniocervical junction and in the other the lesion affected the sacrum. We have undertaken a literature review of familial chordoma and have identified chromosomal abnormalities associated with the condition

Purpose: Dedifferentiated chordomas are a rare and aggressive variant of chordoma. They usually occur in recurrences or following radiotherapy. We describe the rare occurrence of three cases arising de novo within conventional chordoma. Methods and Results: 29 cases of histopathologically verified chordoma were identified from our unit database. From these, we identified three cases of primary dedifferentiated chordoma. The study group included three men, with an average age of 58.3 years (range 57–61). Presenting features were comparable and included lower back pain and rectal discomfort, with a mass palpable per rectum. A needle biopsy was undertaken in one patient, which demonstrated evidence of a pleomorphic spindle cell tumour without evidence of chordoma. Surgical management involved subtotal sacrectomy, with complete excision achieved in two cases. Histopathological examination confirmed dedifferentiated chordoma with varying amounts of sarcomatous elements in each case. Post-operative radiotherapy was administered in two patients and chemotherapy in one patient. Two patients died at 7 and 10 months following presentation with pulmonary metastases. The third patient remains well and alive at two years follow-up. This patient had a complete excision with post-operative radiotherapy, however histopathological examination revealed only a small focus of the dedifferentiated component. Conclusions: Dedifferentiated chordoma is a fatal tumour, with metastases and death the likely outcome. Most cases in the literature and the two cases reported in this series, uphold this ominous prognosis. However this may not always hold true. Small areas of dedifferentiation within the chordoma tumour may carry a more favourable prognosis

Dedifferentiated chordoma is a rare and aggressive variant of the conventional tumour in which an area undergoes transformation to a high-grade lesion, typically fibrous histiocytoma, fibrosarcoma, and rarely, osteosarcoma or rhabdomyosarcoma. The dedifferentiated component dictates overall survival, with smaller areas of dedifferentiation carrying a more favourable prognosis. Although it is more commonly diagnosed in recurrences and following radiotherapy, there have been a few reports of spontaneous development. We describe four such cases, which were diagnosed de novo following primary excision, and discuss the associated clinical and radiological features

Chordoma is the second most common primary malignant tumor of the spine. These tumors rarely metastasize but are considered malignant and, when present in younger individuals, can be aggressive. In the setting of unresectable primary, recurrent, or metastatic tumors the current armamentarium of adjuvant therapy for this condition is very limited. Recent research, however, has identified potential targets for immunotherapy, including the tumor associate antigens High Molecular Weight Melanoma Associated Antigen (HMW-MAA) and B7H3. The goal of this investigation was to correlate expression of B7H3 and HMW-MAA in chordoma tumors with disease severity and clinical outcome. Tissue MicroArrays (TMA) were constructed using an automated arrayer to include 70 conventional chordoma tumors obtained from archives at our institution. Triplicate cores (0.6 mm in diameter) from each sample were created and two sets of cores were created for each chordoma specimen. One triplicate sample was incubated in a closed humid chamber with a pool of HMW-MAA-specific mAb, while the other was incubated with mAb specific for B7H3. Samples were washed in PBS and incubated with a secondary antibody for one hour. Staining was evaluated independently by two researchers and scored using validated systems. A retrospective chart review was performed for each chordoma specimen to determine demographic data, disease course, disease status at final follow-up and mortality. Clinical outcomes were then correlated to the expression of HMW-MAA and B7H3 within the chordoma lesions. Kaplan-Meier curves and Cox proportional hazard regression analysis were utilized to facilitate comparisons. Chordoma tumors from 70 patients were included in this study. Average age at the time of presentation was 57.4 years (31–88 years). Average follow-up was 5.5 years (3.6 months-21 years). Forty-three patients developed recurrences and 10 had metastatic disease. Twenty-three patients (33%) had died of disease at the time of final follow-up. Ninety-seven percent of chordoma tumors stained positive for B7H3 while 44% stained positive for HMW-MAA. No correlation could be drawn between clinical course, recurrence rate, or mortality and tumor expression of B7H3 and HMW-MAA. Kaplan-Meier analysis did demonstrate a shorter survival time for patients whose tumors stained positive for HMW-MAA compared to those whose tumors were negative for the antigen. The goal of this investigation was to correlate expression of B7H3 and HMW-MAA in chordoma tumorswith disease severity and clinical outcome. Results indicate that expression of HMW-MAA may be predictive of more aggressive disease and shorter survival. HMW-MAA and especially B7H3, in light of its near universal expression in the chordoma tumors studied here, may serve as potential targets for adjuvant immunotherapy

To determine whether sacral chordoma is monoclonal or polyclonal in origin, a new assay to study the polymorphic human androgen receptor locus (HUMARA) was applied. The ratio of maternal inactive X-chromosone to the paternal inactive X (Lyon hypothesis) was determined via a methylation-specific polymerase chain reaction (PCR) technique. Seven of seven informative samples showed a polyclonal proliferation pattern. This study suggests that chordomas are more comparable to mesenchymal neoplasms than to monoclonal hematopoeitic neoplasms. The purpose of this study was to determine whether sacral chordoma is monoclonal or polyclonal in origin via a new molecular genetic assay of the x-chromosome. A polyclonal proliferation pattern was identified in all informative samples studied. Characterization of the genetic tumorigenesis of this unpredictable neoplasm may lend insight into its biological behavior and offer novel therapeutic intervention. Utilizing a new assay to study the polymorphic human androgen receptor locus (HUMARA), the ratio of maternal inactive X-chromosome to the paternal inactive X (Lyon hypothesis) is determined via a methylation-specific polymerase chain reaction (PCR) technique to detect X-chromosome polymorphisms. Eight female chordoma patients had their DNA harvested and their x-chromosome inactivation pattern and polymorphisms determined and compared to control. A polyclonal proliferation pattern was identified in seven of seven informative samples. The eighth sample showed a single x chromosome allele in normal and tumor tissue and was thus viewed as uninformative

Purpose: To report the genetic correlation of familial chordoma, a rare tumour of embryonic notochordal remnant. Method: We present two patients with a family history of chordoma. Both patients had surgery at our unit, one for a clival and one for a sacral tumour. These two cases comprise 1.14% (2 out of total 175 chordoma cases) of our unit’s surgical experience with chordoma (79 cases involving craniocervical junction, 4 cases involving thoracolumbar spine, 92 cases involving sacral region) over the period of 15 years (1990– 2005). Patient1 had clival chordoma and Patient2 had sacral chordoma. Both the patients had excision of the tumour followed by postoperative radiotherapy and annual follow up. There was no recurrence eight years later in Patient 1 and Patient 2 died three years after the surgery. Results: Patient 1 had ten other family members affected by chordoma (mostly clival) and Patient 2 had two other family members affected by clival chordoma. Genetic analysis for the Patient 1 and of her relatives (National Cancer Institute, Bethesda) showed that there was loss of heterozygosity on chromosome 7q 33. None of the affected members of the Patient 2 were alive to do the genetic study. A literature search on genetic studies was performed using the key term as familial chordoma and following studies have been found-. Kelly et al- the study had 10 affected members and showed linkage to chromosome 7q 33. Miozszo et al- the study had 3 affected family members and showed tumour suppressor locus on chromosome 1p36. Stepanek et al –the study had affected 4 members in a family and showed probable autosomal dominant inheritance. Conclusion : Familial chordoma is a very rare tumour. Further genetic studies will hopefully reveal valuable insight into the pathogenesis and possible therapeutic measures of this tumour

Aims: Chordoma is an uncommon malignant tumour with developing the remnants of notochord and usually manifesting itself in patients in their forties and fifties, aggressive local tumour in some cases resulting metastatic progression and might its histological picture show malignancy in long-lived patients. The objective are the presentation of the surgical technique of wide resection in the sacrococcygeal region, discuss the technical aspects of body wall reconstruction and presentation the long-term results of the surgical treatment of sacrococcygeal chordoma in our department. Methods: Although we have treated 48 patients surgically between 1992 and 2005, this lecture includes 37 patients with at least 2 years follow-up. We applied wide resection in all cases and if it was necessary, reconstruction of the body wall was performed by insertingmetal wire frame and artificial fascia layer. Results: We performed 48 wide resections, in 28 cases combined with reconstruction. Most of the patients have had local pain and 26 patients have had preoperative neurological symptoms. It was attained significant local pain relief in 90 percent of the patients and in 77 percent of the patients improvement of the neurological symptoms. Due to radiologically proved local recurrence of the tumour in twelve cases we had to perform second, and in two cases third surgery. Conclusion: The wide resection of sacrococcygeal chordoma following with reconstruction of the body wall is a well designed and safe surgical procedure which results long survival in combining in certain cases with radiotherapy

Background. Resection of sacral chordoma remains challenging because complex anatomy and important nerves in the sacrum make it difficult to achieve wide surgical margins. Computer-assisted navigation has shown promise in aiding in optimal preoperative planning and in providing accurate and precise tumour resection during surgery. Purpose. To evaluate the benefit of using computer-assisted navigation in precise resection of sacral chordoma. Methods. From 2007 to 2012, we performed sacral chordoma resections with computer-assisted navigation in 19 consecutive patients, of which 15 were primary and 4 were recurrent. There were 11 male and 8 female patients with a mean age of 53.5 years (range, 36–81 years). Eighteen lesions had their upper extent above S3 and the remaining one was below S3. Reconstructed three-dimensional images were used to plan the bone resection before operation. Five patients were treated with CT-based navigation system. 14 cases got ISO-C scanned during operation and CT and MR images were fused using the navigation software. Results. The mean intra-operative blood loss was 2821 mL and the mean operating time was 300 minutes. The mean deviation of registration during operation was 1.5 mm. Wide margins and marginal margins proved by specimen evaluation were achieved in 3 patients and 14 patients, respectively. Two patients received extensive curettage followed by post-operative radiation. With mean 25.1 (range, 7–60) months of follow-up, the overall local recurrence rate was 10.5% (2/19). No recurrence was observed in 15 primary patients treated with wide or marginal margins. A second local recurrence occurred in 2 out of 4 recurrent patients. One was treated with extensive curettage and the other with marginal margin resection. Conclusion. Computer-assisted navigation allows precise execution of intended tumour resection and therefore may improve the local control of sacral chordoma. Comparative clinical studies with long-term follow-up are necessary to confirm this benefit

It is generally accepted that there is a high rate of local recurrence following surgical excision of chordoma of the sacrum, even if the margins of excision appear clear. There is uncertainty as to whether the addition of postoperative radiotherapy may decrease the risk of local recurrence, particularly if there are close or involved margins. We aimed to determine the effect of conventional radiotherapy, in the post-operative setting, on the effect of local recurrence, metastases and patient survival in a multi-centre study. Methods. 57 patients were identified from the combined databases of the RNOH and ROH, who underwent surgical excision of a primary sacral chordoma and who had a minimum of three years follow-up. Results. There were 17 women and 40 men, with a median age of 64 (25-81 range). Median tumour length was 10cm (2-20 range). 22 of the 57 patients died. Survival was 60% at 5 years and 45% at ten years. 28 of the 57 patients developed local recurrence (49%) and margins of excision did not affect the rates of local recurrence. Adjuvant radiotherapy was used in 9 patients, to with intra-lesional resections, five marginal and two wide. Without radiotherapy the local recurrence rate was 45% at five years. With radiotherapy, the local recurrence rate was 62.5% at five years. There were no statistically significant differences between the two. Conclusions. Local recurrence rates for chordoma are high. The biology of the tumour and its extent may well be predictive of local recurrence rather than just margin status per se. Radiotherapy, in the small numbers of patients in this study, seemed not to have an effect in preventing local recurrence. The role of high dose conventional radiotherapy may make surgery for these tumours obsolete

Purpose of the study: Chordoma is a malignant neoplasm believed to arise from notochord remnants. It accounts for approximately 3 to 4 p. 100 of primary bone tumors and is localized along the axial skeleton, 50 p. 100 being sacrococcygeal. Clinical, radiographical and histological findings have been well established since the first description by Ribbert in 1894. Sacral chordomas are however difficult to manage and remain a challenge for surgeons and radiotherapists alike. The purpose of this study was to evaluate the long-term results of surgical treatment and patterns of failure in patients treated for chordoma of the sacrum in our department. Materials and methods: This retrospective study included 11 cases of sacral chordomas treated from 1973 to 1998. Patient age ranged from 36 to 77 years (mean 59 years). Six patients were female and five male. The initial treatment was surgery in all cases including intralesional removal in two cases, marginal resection in seven and complete en bloc resection in two. Results: Median follow-up was 6 years (1 month to 14 years). Tumoral recurrences were observed in nine cases 5 months to 8 years after treatment. In two cases, recurrence was observed 8 years after radical sacrectomy. Treatment of recurrences was partial surgical removal with radiotherapy (40 to 70 Grays). Three patients developed metastases in lungs, liver and bone, respectively. Seven patients died, two from metastatic disease. The 5-year overall survival was 64 p. 100 but only 18 p. 100 of the patients survived 10 years. Average disease-free survival was 18 p. 100 at 5 years and 0 p. 100 at 10 years. Discussion: Chordoma is a slow-growing tumor allowing survival for several years despite recurrent disease. However, only 10 to 20 p. 100 of the patients survive free of disease at 5 years. Recurrences are frequent (45 to 80 p. 100) and often multiple. Chordoma inevitably recurs and eventually leads to death after intralesional removal or marginal resection. Radical surgery should be attempted whenever technically feasible. When performed early, particularly for smaller lesions, it offers the best chance for cure. However, tumoral recurrence can occur postoperatively despite a macroscopically complete resection. Because radiation therapy seems to be more successful in controlling microscopic disease, it should be considered as a pre- or postoperative adjuvant to a macroscopically complete resection

Purpose. We present results of high intensity focussed ultrasound (HIFU) therapy on 2 patients with recurrent sacrococcygeal chordoma with the aim to promote recruitment of patients into a larger clinical trial. Method. Extracorporeal high intensity focussed ultrasound, outcome measure: radiological evidence of involution, clinical status at follow up. Sacrococygeal chordomas are rare, histologically benign tumours that tend to grow inexorably causing pain, and loss of function in the lower limbs and genitor-urinary symptoms. Results after surgical resection with conventional radiotherapy have been generally disappointing. Carbon ion therapy has been reported to be more successful, but this is not available in the UK. Both patients presented some years after surgical resection with recurrent sacral chordoma, causing intractable pain, and declining mobility. The patients underwent several (4 and 2) sessions of HIFU, using a protocol adapted for renal cell carcinoma and without any adverse events. Follow up MRI 2 weeks and 3 months post procedure showed reduction in tumour enhancement in targeted areas. This correlated with a patient-reported improvement in pain control and improving lower limb function sustained during a 3 month period. We propose the establishment of a clinical trial examining the utility of HIFU on recurrent sacral/coccygeal chordoma in adults after surgical resection. The outcomes measured will be pain scores, functional status using validated scoring scales to allow systematic comparisons with other treatments, and survival. We aim to recruit at least 14 patients initially. Side-effects and complications of the treatment will be recorded. Treatment and follow up of patients will take place in the Churchill Hospital, Oxford, UK

Introduction and Aims: Sacrococcygeal chordoma presents a difficult diagnostic and therapeutic problem with a high incidence of local recurrence. The report aims to define the importance of adequate surgical treatment on outcome and survival. Method: Fifty-eight patients underwent surgical treatment for sacrococcygeal chordoma between 1979 and 2001. The series included 19 women and 39 men with an average age of 56.2 (range 13–76) years at diagnosis. Depending on the level and extent of the lesion, a posterior approach was performed in 25 patients, and a combined antero-posterior approach in 33 patients. A wide surgical margin was achieved in 22 patients, 14 marginal and 22 intralesional. Results: At average follow-up of 92.2 (range 18–276) months. Thirty-three patients were alive with no evidence of disease. Twenty-one patients had local recurrence. Recurrence-free survival at five years was 67% and at 10 years 57%. The overall survival was 74%, 51%, 42% at five years, 10 years, and 15 years, respectively. All patients with wide margins survived (100%), which was significantly different from patients who had either marginal or intralesional excision (p=0.0001). The type of surgical approach (p=0.138) does not influence the likelihood to obtain a good margin, although patients with wide margins were also more likely to have small tumors. A wide margin was achieved in 48% using a combined antero-posterior approach, whereas this was the case only in 29% using a posterior approach. Tumor volume univariately assessed, however, does not seem to compromise the possibility of obtaining a wide margin (p=0.21). Multivariate analysis identified – in contrast to tumor volume (p=0.13) – margin (p=0.0001) and age (p=0.04) as predictors of survival. Whereas survival is independent of age in the case with wide margins, patients with marginal or intralesional margins and an age above 60 years have a better survival (43% at 10 years) than patients younger than 60 years with those parameters (20% at 10 years; p=0.0776). Conclusion: A wide margin is the most important predictor of survival in patients with sacrococcygeal chordoma. Tumor volume per se has no negative impact on survival as long as a wide margin is obtained. Therefore, for large tumors and tumors above S3 we prefer combined antero-posterior approach. If a wide margin cannot be obtained, then young patients have a worse prognosis than older patients

Sacrococcygeal chordoma is a slow growing, malignant tumour with a clinical poor outcome due to a high local recurrence (LR) rate. Several studies emphasize that margin-free tumour resection is the most important predictor of survival and LR in patients with sacrococcygeal chordoma. However, a high recurrence rate still remains. The purpose of this report is to define the role of postoperative radiotherapy (RT). 15 patients (7 females and 8 males) underwent surgical treatment for sacrococcygeal chordoma between 1981 and 2003. The mean age at surgery was 54 (range 31–70) years. The mean follow up was 8.5 (range 4 – 20) years. Most patients suffered from local swelling and pain; only one patient had a mild urinary continence being the only pre- and postoperative neurological deficit. Mean time of preoperative complaints was 4.5 (range 0.8 – 8) years. In 9 patients an en bloc resection was performed, in 6 patients a subtotal resection was achieved. Most patients with a subtotal resection received RT (5/6 patients) following surgery, patients with en bloc resection only received RT (> 50Gray) after LR (6/9 patients). After en bloc resection (no initial RT) all patients had local recurrence of the tumour with a mean time to recurrence of 3 (range 0.8 – 13) yrs. Only two patients in the group with subtotal resection had LR after 11 yrs. Six of 9 patients with LR after en bloc surgery received RT after recurrence and had a survival of at least 9 (range 5 – 20) years. There were no major complications. The time to recurrence was significantly longer in the group that received immediate RT after surgery, even after resection with irradical margins. There was no difference in survival between both surgical groups. Our results suggest that postoperative RT is more important in the prevention of local recurrence than margin-free tumour resection. This supports the strategy to add radiotherapy as a standard adjuvant therapy to tumour resection in patients with sacrococcygeal chordoma

Aims. The aim of the study was to compare measures of the quality of life (QOL) after resection of a chordoma of the mobile spine with the national averages in the United States and to assess which factors influenced the QOL, symptoms of anxiety and depression, and coping with pain post-operatively in these patients. Patients and Methods. A total of 48 consecutive patients who underwent resection of a primary or recurrent chordoma of the mobile spine between 2000 and 2015 were included. A total of 34 patients completed a survey at least 12 months post-operatively. The primary outcome was the EuroQol-5 Dimensions (EQ-5D-3L) questionnaire. Secondary outcomes were the Patient-Reported Outcome Measurement Information System (PROMIS) anxiety, depression and pain interference questionnaires. Data which were recorded included the indication for surgery, the region of the tumour, the number of levels resected, the status of the surgical margins, re-operations, complications, neurological deficit, length of stay in hospital and rate of re-admission. Results. The median EQ-5D-3L score was 0.71 (interquartile range (IQR) 0.44 to 0.79) which is worse than the national average in the United States of 0.85 (p < 0.001). Anxiety (median: 55 (IQR 49 to 61), p = 0.031) and pain (median: 61 (IQR 56 to 68), p < 0.001) were also worse than the national average in the United States (50), while depression was not (median: 52 (IQR 38 to 57), p = 0.513). Patients who underwent a primary resection had better QOL and less anxiety, depression and pain compared with those who underwent resection for recurrent or residual disease. The one- and five-year probabilities were 0.96 and 0.74 for survival, 0.07 and 0.25 for tumour recurrence, and 0.02 and 0.16 for developing distant metastasis. A total of 25 local complications occurred in 20 patients (42%), and there were 50 systemic and other complications in 25 patients (52%) within 90 days. Conclusion. These patient reported outcomes and oncological and surgical outcomes can be used when counselling patients and to aid decision-making when planning surgery. Cite this article: Bone Joint J 2017;99-B:979–86

The purpose of this study was to evaluate the long-term outcome of patients with a sacral chordoma and the surgical management of locally recurrent disease. Between October 1990 and August 2013 we operated on 54 consecutive patients with a sacral chordoma. There were 34 men and 20 women with a mean age of 60 years (25 to 86). The mean maximum diameter of the tumour was 9.3 cm (3 to 20). The mean follow-up was 7.8 years (2 months to 23.4 years). The disease-specific survival was 82% at five years, 57% at ten years and 45% at 15 years. The local recurrence-free survival was 49% at five years, 37% at ten years and 20% at 15 years. Local recurrence occurred in 30 patients (56%) at a mean of 3.8 years (3 months to 13 years) post-operatively. Survival after the treatment of recurrence was 89% at two years, 56% at five years and 19% at ten years. Of nine patients who had complete resection of a recurrence, one died after 72 months and eight remain disease-free. Incomplete resection of recurrent disease resulted in a survival of 54% at two years and 36% at five years. For 12 patients with a local recurrence who were treated palliatively, survival was 81% at two years and 31% at five years. A wide margin of resection gave the best chance of long-term survival and complete resection of local recurrence the best chance of control of disease. Cite this article: Bone Joint J 2015;97-B:711–15

Chordoma is low grade, locally aggressive and mainly in the sacrococcygeal region. Treatment is a combination of surgery and radiotherapy. We reviewed, to determine our outcome and functional deficits, the cases treated over 15 years by the senior author. Out of 26 chordoma’s referred 14 were in the sacrococcygeal region. We reviewed them retrospectively looking at presentation, diagnosis, surgical approach, neurological result, complications and survival. The mean age was 55 years (range 26–80 years), 9 males and 5 females. 13 were primary and 1 was recurrent. Patients reported 18 months of symptoms prior to diagnosis. The tumour sites were S1-5, S2-2, S3-2, S4-3, S5-1 and coccyx −1. Surgery was performed via an anterior/posterior-combined approach in 10, a posterior approach in 2, anterior in 1 and posterior/perineal combined in 1. Complete excision was possible in 11 cases. Surgical resection with radiotherapy was used for inadequate surgical margins, in 3 cases. Neurologically we found that we needed an intact unilateral S3 nerve root for continence in our series. All patients had minor wound complications, 2 wounds required further surgical intervention, and there were 2 cardiac arrhythmias, 1 pulmonary embolus. There were 5 recurrences, 3 were local and 2 metastatic. The survival data for 5 and 7 years is 88% and 71% respectively. Our disease free survival at 5 and 7-years was 44% and 57% respectively. We achieved an excellent 5 and 7-year survival in our series. The results following complete excision were best, but those treated with adjuvant radiotherapy also responded well. We saw that a solitary S3 nerve root is needed for continence. Treating by a combined anterior/ posterior approach suggests improved survival

Aim: To review treatment outcomes in patients with sacral chordoma treated at our centre over the past 20 years. Methods: Retrospective review of prospectively kept data. Previously treated patients were excluded. The surgical objective was to obtain clear margins. If sacrifice of S2,3,4 was necessary, this was usually combined with colostomy. Results: 30 patients were treated (20 males, 10 females), median age 63.5 (28 to 94). Median duration of symptoms before presentation was 79 weeks (3–260), mean tumour size 11 cm. Most had neurological symptoms. Eight tumours involved the S2 roots, 1 the entire sacrum. Treatment was palliative in 7 patients, resection in 23. Operation time averaged 4.5 hours (1.5 to 8). Margins were wide in 7, marginal in 12, and intralesional in 4 patients. There was a high rate of postoperative complications, mostly wound problems (61% of patients). In 1 case this resulted in septicaemia and post-operative death. Average operative blood loss was 1600ml (0–3500). 65% of patients were incontinent of urine and/or faeces. Local recurrence (LR) occurred in 52% of operated patients at a median of 32 months (4–134). Incidence of LR was 60% after intralesional, 57% after marginal and 25% after wide surgery (p=0.49). LR was treated with re-excision, radiofrequency ablation, radio- and occasionally chemotherapy. Overall survival (Kaplan-Meier) of all patients was 57% at 5, and 40% at 10 years. Of operated patients this was 67% and 47%. There was a trend for better survival after wide resection margin. Metastatic disease only occurred in 3 patients. Conclusion: Chordoma of the sacrum is frequently diagnosed late. Resection is associated with a high complication rate. Local recurrence is the most common cause of death. Early referral to a specialist centre is recommended to optimize treatment. The role of adjuvant therapy remains unclear