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

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. 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

Sacro-coccygeal chordomas pose a difficult diagnostic and therapeutic problem due to late presentation, large size, soft-tisue extension, difficulties in obtaining adequate resection margins, higher local recurrence rate and uncertain effectiveness of adjuvant treatment. We present a series of 21 patients of sacral chordomas obtained from Scottish Bone Tumour Registry to analyse predictors of local control and survival. The clinical and morphologic features, type of treatment and follow-up of 21 consecutive patients with sacral chordoma were retrospectively reviewed and analysed. The data were obtained from Scottish Bone Tumour Registry. The average age at time of the biopsy was 59 years (range, 12 to 82 years): twelve patients were male and nine were female. Pain was the presenting symptom in all patients. Two had intralesional (both recurred), 9 marginal (4 recurred) and 3 wide resections (1 recurred). Fifteen of the twenty-one patients were treated with adjuvant radiation therapy. In seven patients, the chordoma was inoperable and all but one were treated with adjuvant radiotherapy. Local recurrence and metastases occurred in 7 (50%) and 5 (23.8%) patients. The 5-year and 10-year survival were 38% and 14.2%, respectively. Excision of the lesion combined with adjuvant radiation therapy provided satisfactory results. Local recurrence presents a major problem in the management of sacral chordomas (50%). Intralesional resection should be avoided as it is associated with 100% local recurrence in our series

Chordomas are slowly growing, locally aggressive primary malignant neoplasms derived from primitive notochordal cells. They tend to occur at the extreme of ages and at the two ends of the spinal column. The purpose of this study was to evaluate the presentation, morphology and behaviour of 20 non-clival, non-sacral chordoma cases. Details of 20 cases of histologically confirmed Chordomas of the mobile spine (Cervical-7, Thoracic-7, Lumbar-6) between 1967 and 2006, were extracted from the Scottish Bone Tumour Registry. The casenotes and radiographs were retrospectively reviewed. Non-sacral chordomas comprised 48.7% (20/41 cases) of total chordomas registered. There was a slight feminine predominance (11/20 cases). Mean age was 58.1 years (range, 12 to 82 years). Progressively worsening back pain was the first symptom in all the patients. Seven had intra-lesional (five recurred), six marginal (1 recurred) and 2 wide resections (none recurred). Eleven of the twenty-one patients were treated with adjuvant radiation therapy. In five patients, the chordoma was inoperable and all but one were treated with radiotherapy. There were 2 recurrences in the group of patients treated without adjuvant radiotherapy. Six patients (40%) developed local recurrences and 3 patients (15%) developed metastases. There were 4 survivors including one with local recurrence. Sixteen patients died including 3 with metastases, 7 with persistent primary disease, 1 with local recurrence and 5 with no evidence of disease or died of unrelated causes. Median survival was 19 months (mean-30.2 months), with 5- and 10-year survival rates 10% and 0.0% respectively. We found that non-sacral/non-clival chordomas had a much poorer prognosis than published in the literature. Local recurrences occurred in 40%. Intra-lesional resection should be avoided as it is associated with 71.4% local recurrence in our series

Introduction. Treatment of spinal metastatic disease has evolved with the advent of advanced interventional, surgical and radiation techniques. Spinal Oligometastatic disease is a low volume disease state where en bloc resection of the tumour, based on oncological principles, can achieve maximum local control (MLC). Hybrid therapy incorporating Separation surgery (>2mm clearance of the thecal sac) and Stereotactic Ablative Radiotherapy (SABR) offer an alternative approach to achieving MLC. Hybrid therapy is also a viable option in patients eligible for SBRT who have failed conventional radiation therapy. En-bloc surgery may be a suitable option for those patients who are ineligible for or have failed SBRT. A multidisciplinary approach is particularly important in the decision-making process for these patients. Metal free instrumentation is aiding the optimization of these surgeries. The authors present a supra-regional centre's experience in managing spinal oligometastases. Methods. Retrospective review of oligometastatic spinal disease at a supra-regional centre between 2017 and 2021. Demographics, operative course, complications and Instrument type are examined. Results. Demographics: 24 patients with mean age 53.8y (range 12–77), 44% (40y–59y), 40% (60y–69y); 51% Male. Histology: Breast, Renal and Sarcoma accounted for 16.7% each; Thyroid, Prostate and Chordoma accounted for 8.3% each. Primary disease 7%, Synchronous 15%, Metachronous 78%. Instrumentation: Carbon-fibre (85%), TiAl (11%), Non-Instrumented (3%). Separation Surgery (70%), En-bloc resection/Tomita surgery (30%); SABR/Proton Beam Planned: 70%. Average length of hospital stays 19.1 days; twenty patients required intensive care admission for an average 2.7 days. 30 Day Mortality 8.3% (n=2: COVID-19 during admission and ventriculitis post discharge), 1y Mortality – 16.7%, 3y Mortality – 25%; Synchronous Mortality 75% (n=3) at 3 years. 30 Day infection rate 3%; 1y infection rate 7%. 1 Non-instrumented case developed proximal junctional failure post proton beam therapy and required a vascularised fibular strut graft. 2-year Revision for Local Recurrence 5% (Revision at 23 months). Conclusion. There are very few case series of oligometastatic spinal disease due to the relatively new concept of adjuvant SABR and its limited availability. Solid tumours pre-dominated the histology in our series with metachronous disease being the most commonly operated disease state. 92% of cases were eligible for SABR. The majority (85%) of cases were performed with Carbon-fibre instrumentation and has been shown to be safe with no mechanical failures in this series. Infection rates are in keeping with patients requiring radical radiotherapy with 3% early and 7% late. 30-day mortality was 8.3%, 1y=16.7% and 25% at last follow up. Mortality, as expected, is highest within the synchronous disease group and should be operated on sparingly. With the current management strategy, there was no local recurrence at 1 year and excellent local recurrence rate at 2 years (5%). Although radical en bloc surgery carries significant morbidity, it should be considered in selective cases to achieve MLC. All Oligometastatic cases deserve extra consideration and specialist MDT as not all are suitable for SABR. Multimodal Hybrid therapy, incorporating less invasive surgical techniques and SABR, represents a paradigm shift in achieving MLC in oligometastatic spinal disease

Aims

The aim of this study was to investigate the incidence and characteristics of instrumentation failure (IF) after total en bloc spondylectomy (TES), and to analyze risk factors for IF.

Statement of purpose. We review the current state of development of proton therapy and the implications for beam therapy in the management of primary bone tumours. Introduction. The principle of radiotherapy is to deliver a high dose, accurately, to the tumour. Conventional photon and proton therapy irradiates adjacent tissue significantly. This is reduced with intensity modulated proton therapy (IMPT). This has been demonstrably effective in treating tumours refractory to chemotherapy and conventional radiotherapy such as chrondrosarcomas and chordomas. Case Report. We present a patient with an isolated chondrosarcoma involving the anterior and posterior element of the L3 vertebral body with a significant soft tissue component displacing the IVC. The patient underwent a 2 stage en-bloc excision of the tumour: Stabilization was achieved by posterior L2-4 instrumented fusion with PEEK rods, an anterior PEEK cage and bone graft. Post-operatively the patient underwent 12 weeks of bed rest followed by rehabilitation. Due to pedicle involvement (giving a high risk of contamination) and the narrow clear margin found on histology the patient has been accepted for post-operative IMPT. Discussion. The high risk of contamination and the narrow margins presented an ideal case for post-operative IMPT. However, conventionally the stabilization would have been metallic, distorting the treatment mapping and the delivery of the IMPT, reducing any possible benefit. Thus, we used PEEK as it is proven to maintain its properties when subjected to a wide range of conditions while also being tolerant of, and not interfering with, most forms of radiation including proton therapy, maximising the chance of a positive outcome. One concern is that PEEK is less biomechanically stable then metal, hence the prolonged period of bed rest. Conclusion. This report discusses the current evidence for proton therapy while describing a successful technique for stabilization to facilitate delivery of proton treatment post-operatively

Statement of purpose. We review the current state of development of proton therapy and the implications for beam therapy in the management of primary bone tumours. Introduction. The principle of radiotherapy is to deliver a high dose, accurately, to the tumour. Conventional photon and proton therapy irradiates adjacent tissue significantly. This is reduced with intensity modulation proton therapy (IMPT). This has been demonstrably effective in treating tumours refractory to chemotherapy and conventional radiotherapy such as chrondrosarcomas and chordomas. Case Report. We present a patient with an isolated chondrosarcoma involving the anterior and posterior element of the L3 vertebral body with a significant soft tissue component displacing the IVC. The patient underwent a 2 stage en-bloc excision of the tumour: Stabilisation was achieved by posterior L2-4 instrumented fusion with Peek rods and screws, an anterior Peek cage and bone graft. Post-operatively the patient underwent 12 weeks of bed rest followed by rehabilitation. Due to pedicle involvement (giving a high risk of contamination) and the narrow clear margin found on histology the patient has been accepted for post-operative IMPT. Discussion. The high risk of contamination and the narrow margins presented an ideal case for post-operative IMPT. However, conventionally the stabilisation would have been metallic, distorting the treatment mapping and the delivery of the IMPT, reducing any possible benefit. Thus, we used Peek as it is proven to maintain its properties when subjected to a wide range of conditions while also being tolerant of, and not interfering with, most forms of radiation including proton therapy, maximising the chance of a positive outcome. One concern with Peek is that it is less biomechanically stable then metal, hence the prolonged period of bed rest. Conclusion. This report discusses the current evidence for proton therapy while describing a successful technique for stabilisation to facilitate delivery of proton treatment post-operatively. Ethics approval. None Audit/service standard in trust Ethics committee COREC number:. Interest Statement. None Local grant/National grant Commercial/industry support

Aims

The aim of this study was to explore the prognostic factors for postoperative neurological recovery and survival in patients with complete paralysis due to neoplastic epidural spinal cord compression.

Total en bloc spondylectomy (TES) is the total resection of a vertebra containing a tumour. Many authors have investigated patient-reported outcomes after routine spinal surgery and surgery for tumours in general. However, this is the first report of patient-reported outcomes, including health-related quality of life (HRQoL) and satisfaction, after en bloc vertebral resection for a spinal tumour.

Of the 54 patients who underwent TES for a primary tumour between 1993 and 2010, 19 died and four were lost to follow-up. In January 2012, a questionnaire was sent to the 31 surviving patients. This included the short form-36 to assess HRQoL and questions about the current condition of their disease, activities of daily living (ADL) and surgery. The response rate was high at 83.9% (26/31 patients). We found that most patients were satisfied and maintained good performance of their ADLs.

The mental health status and social roles of the HRQoL scores were nearly equivalent to those of healthy individuals, regardless of the time since surgery. There was significant impairment of physical health in the early post-operative years, but this usually returned to normal approximately three years after surgery.

Cite this article: Bone Joint J 2014;96-B:1693–8.

Mesenchymal stem-cell based therapies have been proposed as novel treatments for intervertebral disc degeneration, a prevalent and disabling condition associated with back pain. The development of these treatment strategies, however, has been hindered by the incomplete understanding of the human nucleus pulposus phenotype and by an inaccurate interpretation and translation of animal to human research. This review summarises recent work characterising the nucleus pulposus phenotype in different animal models and in humans and integrates their findings with the anatomical and physiological differences between these species. Understanding this phenotype is paramount to guarantee that implanted cells restore the native functions of the intervertebral disc.

Cite this article: Bone Joint Res 2013;2:169–78.