Aims. The aim of this study was to report the long-term prognosis of patients with multiple Langerhans cell histiocytosis (LCH) involving the spine, and to analyze the risk factors for progression-free survival (PFS). Methods. We included 28 patients with multiple LCH involving the spine treated between January 2009 and August 2021. Kaplan-Meier methods were applied to estimate overall survival (OS) and PFS. Univariate Cox regression analysis was used to identify variables associated with PFS. Results. Patients with multiple LCH involving the spine accounted for 15.4% (28/182 cases) of all cases of spinal LCH: their lesions primarily involved the thoracic and lumbar spines. The most common symptom was pain, followed by neurological dysfunction. All patients presented with osteolytic bone destruction, and 23 cases were accompanied by a paravertebral soft-tissue mass. The incidence of vertebra plana was low, whereas the oversleeve-like sign was a more common finding. The alkaline phosphatase was significantly higher in patients with single-system multifocal bone LCH than in patients with multisystem LCH. At final follow-up, one patient had been lost to follow-up, two patients had died, three patients had local recurrence, six patients had distant involvement, and 17 patients were alive with disease. The median PFS and OS were 50.5 months (interquartile range (IQR) 23.5 to 63.1) and 60.5 months (IQR 38.0 to 73.3), respectively. Stage (hazard ratio (HR) 4.324; p < 0.001) and chemotherapy (HR 0.203; p < 0.001) were prognostic factors for PFS. Conclusion. Pain is primarily due to segmental instability of the spine from its destruction by LCH. Chemotherapy can significantly improve PFS, and radiotherapy has achieved good results in local control. The LCH lesions in some patients will continue to progress. It may initially appear as an isolated or single-system LCH, but will gradually involve multiple sites or systems. Therefore, long-term follow-up and timely intervention are important for patients with spinal LCH. Cite this article: Bone Joint J 2023;105-B(6):679–687

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

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

Aims. Patients with differentiated thyroid carcinomas (DTCs) have a favourable long-term survival. Spinal metastases (SMs) cause a decline in performance status (PS), directly affecting mortality and indirectly preventing the use of systemic therapies. Metastasectomy is indicated, if feasible, as it yields the best local tumour control. Our study aimed to examine the long-term clinical outcomes of metastasectomy for SMs of thyroid carcinomas. Methods. We collected data on 22 patients with DTC (16 follicular and six papillary carcinomas) and one patient with medullary carcinoma who underwent complete surgical resection of SMs at our institution between July 1992 and July 2017, with a minimum postoperative follow-up of five years. The cancer-specific survival (CSS) from the first spinal metastasectomy to death or the last follow-up was determined using Kaplan-Meier analysis. Potential factors associated with survival were evaluated using the log-rank test. We analyzed the clinical parameters and outcome data, including pre- and postoperative disability (Eastern Cooperative Oncology Group PS 3), lung and non-spinal bone metastases, and history of radioiodine and kinase inhibitor therapies. Results. Lung and other bone metastases at the time of surgery were observed in ten and eight patients, respectively. Three patients experienced local tumour recurrences at the operated site. The five- and ten-year CSS rates in the 22 patients with DTC were 77% and 52%, respectively. Pre- and postoperative disability and operative site tumour recurrence were identified as risk factors for short postoperative survival. Conclusion. Metastasectomy for resectable SM from DTC yielded favourable results and has the potential to improve survival. Cite this article: Bone Joint J 2023;105-B(5):575–582

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

Chondrosarcomas are uncommon primary malignant cartilaginous tumours, even less common in spine. Surgical excision is the only mode of successful treatment as these tumours are resistant to conventional chemotherapy and radiation therapy. We share our experience of 22 cases of chondrosarcomas of the spine with special reference to their recurrence and survival. We identified 20 conventional and 2 dedifferentiated chondrosarcomas from the Scottish Bone Tumour Registry database between 1964 and 2009. Radiology and histopathology were documented. The mean follow-up was 5.2 years. There were 14 men and 8 women with a mean age of 50.1 years. There were 7 under the age of 40 years (31.8%). The majority of lesions occurred in the thoracic spine (16), followed by sacrum (3), lumbar (2) and cervical spine (1). The overall local recurrence rate was 45.4% (10/22 cases-once in 5, twice in 2 and thrice in 3 patients). Four patients presented with pulmonary metastases leading to death. The estimated overall 5- and 10-year survival rates were 31.8% and 18.1% respectively. We found that 1/3. rd. of chondrosarcomas of the spine occured below 40 years of age and 3/4. th. in the thoracic spine. Every other case was associated with local recurrence with a 32% 5-year and 18% 10-year survival rates

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

Introduction. Spinal osteosarcomas are quite rare and the optimal treatment strategy is unknown. We report a series of 9 cases of osteosarcoma of the spine treated with intralesional resection and adjuvant combination therapy in order to evaluate their clinico-pathological correlation, recurrence rate and survival. Materials and Methods. Between 1980 and 2009, nine histologically confirmed cases of primary conventional osteogenic sarcoma of the spine were identified from Scottish Bone Tumour Registry. This prospectively collected registry database was retrospectively reviewed. Results. There were 9 cases with male predominance (77.7%) at a median age of 39 years (range, 16 – 73 years) and were anatomically distributed as 2-cervical, 6-thoracic, 1-lumbar and 1-sacral. Local and distant staging and histological grading was carried out in all. Seven patients underwent intralesional (including open biopsies) and 2 marginal resections. Seven patients received adjuvant chemotherapy and 8 adjuvant radiotherapy. The overall local recurrence rate was 66.6%. All but one patient died of the disease with a median length of survival of 30 months. The 2-year and 5-year survival rates were 66.6% and 22.2% respectively. Two patients who survived more than 5 years were male patients below the age of 40 years involving thoracic spine (one had a marginal resection without receiving any adjuvant therapy subsequently had a recurrence; other one underwent intralesional resection with adjuvant chemo- and radiotherapy, died with no evidence of disease). Conclusions. Spinal osteosarcoma in Scottish patients showed a higher median age, thoracic spine and male gender predilection with overall poor prognosis. Local recurrence did not affect the survival adversely, however patients older than 40 years and metastases at presentation showed poor survival compared to age under 40 and non-metastatic presentations

The skeleton is the most common site to be affected by metastatic cancer. The place of surgical treatment and of different techniques of reconstruction has not been clearly defined. We have studied the rate of survival of 94 patients and the results of the surgical treatment of 91 metastases of the limbs and pelvis, and 18 of the spine. Variables included the different primary tumours, the metastatic load at the time of operation, the surgical margin, and the different techniques of reconstruction. The survival rate was 0.54 at one year and 0.27 at three years. Absence of visceral metastases and of a pathological fracture, a time interval of more than three years between the diagnosis of cancer and that of the first skeletal metastasis, thyroid carcinoma, prostate carcinoma, renal-cell carcinoma, breast cancer, and plasmacytoma were positive variables with regard to survival. The metastatic load of the skeleton and the surgical margin were not of significant influence. In tumours of the limbs and pelvis, the local failure rate was 0% after biological reconstruction (10), 3.6% after cemented or uncemented osteosynthesis (28) and 1.8% after prosthetic replacement (53). The local failure rate after stabilisation of the spine (18) was 16.6%. There was local recurrence in seven patients (6.4%), and in four of these the primary tumour was a renal-cell carcinoma. The local recurrence rate was 0% after extralesional (24) and 8.2% after intralesional resection (85). Improvements in the oncological management of patients with primary and metastatic disease have resulted in an increased survival rate. In order to avoid additional surgery, it is essential to consider the expected time of survival of the reconstruction and, in bony metastases with a potentially poor response to radiotherapy, the surgical margin

Patients with solitary spinal metastases from Renal Cell Carcinoma (RCC) have better prognosis and survival rates compared to other spinal metastatic disease. Adjuvant therapy has been proven ineffective. Selected patients can be treated with Total En bloc Spondylectomy (TES) for solitary intra-osseous metastasis in the thoracolumbar spine secondary to renal cell carcinoma. Five patients with solitary vertebral metastasis secondary to RCC underwent TES for radical resection of the spinal pathology after pre-operative embolisation. The procedure involves en bloc laminectomy and corpectomy with posterior instrumented fusion and anterior instrumentation with cage reconstruction following the spondylectomy. All patients were fully staged pre-operatively and assessed according to the Tokuhashi scoring system. Recurrence of spinal metastasis and radiological failure of reconstruction. All patients demonstrated full neurological recovery and reported significant pain relief. One patient died at 11 months post-op due to a recurrence of the primary. The other four are well at 24, 45, 52 and 66 months post-op without evidence of recurrence in the spine. There were no major surgical complications. Careful patient selection is required to justify this procedure. The indication is limited to solitary intra-osseous lesions where complete resection of the tumour is possible. The main advantage of this treatment is that it affords significant pain relief and restores spinal stability whilst minimizing local recurrence

Objective. To evaluate functional and oncological outcomes following sacral resection. Methods. Retrospective review of 97 sacral tumours referred to spinal or oncology units between 2004 and 2009. Results. 61 males, 37 females (average age of 47 (range 3 – 82). Average duration of symptoms 13 months. 17 metastatic disease, excluded from further discussion. Of the remainder 36/81(44%) underwent surgery – 21 excision, 9 excision and instrumented stabilisation, and 6 curettage. 13(16%) patients were inoperable - 8 advanced disease, 3 unable to establish local control, 2 recurrence. Colostomy was performed in 11/21(52%) patients who underwent excision. Deep wound infections in 6/21(29%). No difference in infection rates between definitive surgery with or without colostomy – 3/11(27%) vs. 3/10(30%). In the instrumented group, no colostomies were performed due to concerns about deep infection and none resulted (0/9). Radiological failure of stabilisation was noted in 7/9(78%). However, functionally, 3/9(33%) were mobilising independently, 3/9(33%) crutches, 2/9(22%) able to transfer and 1/9(11%) undocumented. Mean follow-up 25 months (range 0-70). Local recurrence in 9/36(25%) of operated patients. Metastasis occurred in 4/36(11%) and mortality 8/36(22%) although follow-up period was noted to be short. Conclusions. Results are comparable with current literature. Mechanical stabilisation for extensive sacral lesions is challenging. Despite radiological failure in 7/9 instrumented stabilisations, patients were relatively asymptomatic and only 1/9 required revision stabilisation surgery. By design none had colostomies and there were no deep infections

Objective. To evaluate functional and oncological outcomes following resection of sacral tumours and discuss the strategies for instrumentation. Introduction. Primary malignant tumours of the sacrum are rare, arising from bony or neural elements, or bone marrow in haematological malignancies. Management of such lesions is dictated by anatomy and the behaviour of tumours. Three key issues which arise are the adequacy of tumour resection, mechanical stabilisation and the need for colostomy. Stabilisation is often extensive and can be challenging. Methods. A retrospective review of the surgical management of primary malignant sacral tumours from 2004 - 2009. Results. The study included 46 patients (34 males, 12 females) with an average age of 49 (range 7 – 82). Median duration of symptoms before presentation was 26 months. 25 patients (54%) underwent surgical resection. 8 underwent instrumented stabilisations with fibula strut graft vs. 17 uninstrumented. Mechanical failure of stabilisation was noted in 75% over the follow up period but only one required revision surgery. Colostomy was performed in 10 patients (40%). Mean follow post-operatively was 19.0 months. Wound healing problems were present in 5/25 (20%). There was no difference in infection rates between definitive surgery with and without colostomy. There was one peri-operative death. Local recurrence occurred in 12%(3/25) of operated patients although follow-up period was noted to be short. Conclusions. Mechanical stabilisation for extensive lesions in the sacrum are particularly challenging in tumour surgery. Despite radiological failure in 7/8 instrumented stabilisations, patients were relatively asymptomatic and only 1/8 required revision stabilisation surgery. Ethics approval:. None: Audit. Interest Statement: None

To evaluate functional and oncological outcomes following resection of primary malignant bone tumours. Primary malignant tumours of the sacrum are rare, arising from bony or neural elements, or bone marrow in haematological malignancies. Management of these lesions is dictated by anatomical considerations and the behaviour of tumours. The three key issues which arise are the adequacy of tumour resection, mechanical stabilisation and the need for colostomy. A retrospective review of the surgical management of primary malignant sacral tumours from 2004 - 2009. The study included 46 patients (34 males, 12 females) with an average age of 49 (range 7 – 82). Median duration of symptoms before presentation was 26 months. 10 patients had inoperable tumours at presentation. 6 patients had chemotherapy. 2 patients opted for palliative radiotherapy. 1 patient was unfit for surgery. 25 patients (54%) underwent surgical resection. 8 underwent instrumented stabilisations with fibula strut graft vs. 17 uninstrumented. Colostomy was performed in 10 patients (40%). Mean follow post-operatively was 19.0 months. Wound healing problems were present in 5/25 (20%). There was no difference in infection rates between definitive surgery with and without colostomy. Mechanical failure of stabilisation was noted in 75%. There was one peri-operative death. Local recurrence occurred in 12%(3/25) of operated patients although follow-up period was noted to be short. Mechanical stabilisation for extensive lesions in the sacrum are particularly challenging in tumour surgery. Despite radiological failure in 7/8 instrumented stabilisations, patients were relatively asymptomatic and only 1/8 required revision stabilisation surgery. Ethics approval: None: Audit Interest Statement: None

Aims

To evaluate the perioperative complications associated with total en bloc spondylectomy (TES) in patients with spinal tumours, based on the extent and level of tumour resection.

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.