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The Journal of Bone & Joint Surgery British Volume
Vol. 90-B, Issue 5 | Pages 652 - 656
1 May 2008
Hanna SA Tirabosco R Amin A Pollock RC Skinner JA Cannon SR Saifuddin A Briggs TWR

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


Orthopaedic Proceedings
Vol. 94-B, Issue SUPP_XXX | Pages 21 - 21
1 Jul 2012
Bhumbra R Hanna S Jeys L Briggs T Grimer R
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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


The Bone & Joint Journal
Vol. 99-B, Issue 7 | Pages 979 - 986
1 Jul 2017
Schwab JH Janssen SJ Paulino Pereira NR Chen YLE Wain JC DeLaney TF Hornicek FJ

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 Bone & Joint Journal
Vol. 97-B, Issue 5 | Pages 711 - 715
1 May 2015
Xie C Whalley N Adasonla K Grimer R Jeys L

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


Aims

This study aimed to analyze the accuracy and errors associated with 3D-printed, patient-specific resection guides (3DP-PSRGs) used for bone tumour resection.

Methods

We retrospectively reviewed 29 bone tumour resections that used 3DP-PSRGs based on 3D CT and 3D MRI. We evaluated the resection amount errors and resection margin errors relative to the preoperative plans. Guide-fitting errors and guide distortion were evaluated intraoperatively and one month postoperatively, respectively. We categorized each of these error types into three grades (grade 1, < 1 mm; grade 2, 1 to 3 mm; and grade 3, > 3 mm) to evaluate the overall accuracy.


The Bone & Joint Journal
Vol. 105-B, Issue 5 | Pages 551 - 558
1 May 2023
Wang H Ji T Qu H Yan T Li D Yang R Tang X Guo W

Aims

The aim of this study was to determine the rate of indocyanine green (ICG) staining of bone and soft-tissue tumours, as well as the stability and accuracy of ICG fluorescence imaging in detecting tumour residuals during surgery for bone and soft-tissue tumours.

Methods

ICG fluorescence imaging was performed during surgery in 34 patients with bone and soft-tissue tumours. ICG was administered intravenously at a dose of 2 mg/kg over a period of 60 minutes on the day prior to surgery. The tumour stain rate and signal-to-background ratio of each tumour were post hoc analyzed. After tumour resection, the tumour bed was scanned to locate sites with fluorescence residuals, which were subsequently inspected and biopsied.


Bone & Joint Research
Vol. 11, Issue 5 | Pages 278 - 291
12 May 2022
Hu X Fujiwara T Houdek MT Chen L Huang W Sun Z Sun Y Yan W

Aims

Socioeconomic and racial disparities have been recognized as impacting the care of patients with cancer, however there are a lack of data examining the impact of these disparities on patients with bone sarcoma. The purpose of this study was to examine socioeconomic and racial disparities that impact the oncological outcomes of patients with bone sarcoma.

Methods

We reviewed 4,739 patients diagnosed with primary bone sarcomas from the Surveillance, Epidemiology and End Results (SEER) registry between 2007 and 2015. We examined the impact of race and insurance status associated with the presence of metastatic disease at diagnosis, treatment outcome, and overall survival (OS).


The Bone & Joint Journal
Vol. 103-B, Issue 8 | Pages 1414 - 1420
1 Aug 2021
Wellings EP Houdek MT Owen AR Bakri K Yaszemski MJ Sim FH Moran SL Rose PS

Aims

Orthopaedic and reconstructive surgeons are faced with large defects after the resection of malignant tumours of the sacrum. Spinopelvic reconstruction is advocated for resections above the level of the S1 neural foramina or involving the sacroiliac joint. Fixation may be augmented with either free vascularized fibular flaps (FVFs) or allograft fibular struts (AFSs) in a cathedral style. However, there are no studies comparing these reconstructive techniques.

Methods

We reviewed 44 patients (23 female, 21 male) with a mean age of 40 years (SD 17), who underwent en bloc sacrectomy for a malignant tumour of the sacrum with a reconstruction using a total (n = 20), subtotal (n = 2), or hemicathedral (n = 25) technique. The reconstructions were supplemented with a FVF in 25 patients (57%) and an AFS in 19 patients (43%). The mean length of the strut graft was 13 cm (SD 4). The mean follow-up was seven years (SD 5).


The Bone & Joint Journal
Vol. 101-B, Issue 4 | Pages 484 - 490
1 Apr 2019
Nandra R Matharu G Stevenson J Parry M Grimer R Jeys L

Aims

The aim of this study was to investigate the local recurrence rate at an extended follow-up in patients following navigated resection of primary pelvic and sacral tumours.

Patients and Methods

This prospective cohort study comprised 23 consecutive patients (nine female, 14 male) who underwent resection of a primary pelvic or sacral tumour, using computer navigation, between 2010 and 2012. The mean age of the patients at the time of presentation was 51 years (10 to 77). The rates of local recurrence and mortality were calculated using the Kaplan–Meier method.


The Bone & Joint Journal
Vol. 101-B, Issue 7 | Pages 880 - 888
1 Jul 2019
Wei R Guo W Yang R Tang X Yang Y Ji T Liang H

Aims

The aim of this study was to describe the use of 3D-printed sacral endoprostheses to reconstruct the pelvic ring and re-establish spinopelvic stability after total en bloc sacrectomy (TES) and to review its outcome.

Patients and Methods

We retrospectively reviewed 32 patients who underwent TES in our hospital between January 2015 and December 2017. We divided the patients into three groups on the basis of the method of reconstruction: an endoprosthesis group (n = 10); a combined reconstruction group (n = 14), who underwent non-endoprosthetic combined reconstruction, including anterior spinal column fixation; and a spinopelvic fixation (SPF) group (n = 8), who underwent only SPF. Spinopelvic stability, implant survival (IS), intraoperative haemorrhage rate, and perioperative complication rate in the endoprosthesis group were documented and compared with those of other two groups.


The Bone & Joint Journal
Vol. 95-B, Issue 10 | Pages 1417 - 1424
1 Oct 2013
Jeys L Matharu GS Nandra RS Grimer RJ

We hypothesised that the use of computer navigation-assisted surgery for pelvic and sacral tumours would reduce the risk of an intralesional margin. We reviewed 31 patients (18 men and 13 women) with a mean age of 52.9 years (13.5 to 77.2) in whom computer navigation-assisted surgery had been carried out for a bone tumour of the pelvis or sacrum. There were 23 primary malignant bone tumours, four metastatic tumours and four locally advanced primary tumours of the rectum. The registration error when using computer navigation was <  1 mm in each case. There were no complications related to the navigation, which allowed the preservation of sacral nerve roots (n = 13), resection of otherwise inoperable disease (n = 4) and the avoidance of hindquarter amputation (n = 3). The intralesional resection rate for primary tumours of the pelvis and sacrum was 8.7% (n = 2): clear bone resection margins were achieved in all cases. At a mean follow-up of 13.1 months (3 to 34) three patients (13%) had developed a local recurrence. The mean time alive from diagnosis was 16.8 months (4 to 48).

Computer navigation-assisted surgery is safe and has reduced our intralesional resection rate for primary tumours of the pelvis and sacrum. We recommend this technique as being worthy of further consideration for this group of patients.

Cite this article: Bone Joint J 2013;95-B:1417–24.


The Bone & Joint Journal
Vol. 100-B, Issue 6 | Pages 798 - 805
1 Jun 2018
Zhang Y Guo W Tang X Yang R Ji T Yang Y Wang Y Wei R

Aims

The sacrum is frequently invaded by a pelvic tumour. The aim of this study was to review our experience of treating this group of patients and to identify the feasibility of a new surgical classification in the management of these tumours.

Patients and Methods

We reviewed 141 patients who, between 2005 and 2014, had undergone surgical excision of a pelvic tumour with invasion of the sacrum.

In a new classification, pelvisacral (Ps) I, II, and III resections refer to a sagittal osteotomy through the ipsilateral wing of the sacrum, through the sacral midline, or lateral to the contralateral sacral foramina, respectively. A Ps a resection describes a pelvic osteotomy through the ilium and a Ps b resection describes a concurrent resection of the acetabulum with osteotomies performed through the pubis and ischium or the pubic symphysis. Within each type, surgical approaches were standardized to guide resection of the tumour.


The Bone & Joint Journal
Vol. 100-B, Issue 5 | Pages 652 - 661
1 May 2018
Lawrenz JM Styron JF Parry M Grimer RJ Mesko NW

Aims

The primary aim of this study was to determine the effect of the duration of symptoms (DOS) prior to diagnosis on the overall survival in patients with a primary bone sarcoma.

Patients and Methods

In a retrospective analysis of a sarcoma database at a single institution between 1990 and 2014, we identified 1446 patients with non-metastatic and 346 with metastatic bone sarcoma. Low-grade types of tumour were excluded. Our data included the demographics of the patients, the characteristics of the tumour, and the survival outcome of patients. Cox proportional hazards analysis and Kaplan–Meier survival analysis were performed, and the survivorship of the non-metastatic and metastatic cohorts were compared.


The Bone & Joint Journal
Vol. 95-B, Issue 5 | Pages 683 - 688
1 May 2013
Chen Y Tai BC Nayak D Kumar N Chua KH Lim JW Goy RWL Wong HK

There is currently no consensus about the mean volume of blood lost during spinal tumour surgery and surgery for metastatic spinal disease. We conducted a systematic review of papers published in the English language between 31 January 1992 and 31 January 2012. Only papers that clearly presented blood loss data in spinal surgery for metastatic disease were included. The random effects model was used to obtain the pooled estimate of mean blood loss.

We selected 18 papers, including six case series, ten retrospective reviews and two prospective studies. Altogether, there were 760 patients who had undergone spinal tumour surgery and surgery for metastatic spinal disease. The pooled estimate of peri-operative blood loss was 2180 ml (95% confidence interval 1805 to 2554) with catastrophic blood loss as high as 5000 ml, which is rare. Aside from two studies that reported large amounts of mean blood loss (> 5500 ml), the resulting funnel plot suggested an absence of publication bias. This was confirmed by Egger’s test, which did not show any small-study effects (p = 0.119). However, there was strong evidence of heterogeneity between studies (I2 = 90%; p < 0.001).

Spinal surgery for metastatic disease is associated with significant blood loss and the possibility of catastrophic blood loss. There is a need to establish standardised methods of calculating and reporting this blood loss. Analysis should include assessment by area of the spine, primary pathology and nature of surgery so that the amount of blood loss can be predicted. Consideration should be given to autotransfusion in these patients.

Cite this article: Bone Joint J 2013;95-B:683–8.


The Journal of Bone & Joint Surgery British Volume
Vol. 89-B, Issue 7 | Pages 943 - 947
1 Jul 2007
Wong KC Kumta SM Chiu KH Antonio GE Unwin P Leung KS

The use of a navigation system in musculoskeletal tumour surgery enables the integration of pre-operative CT and MRI images to generate a precise three-dimensional anatomical model of the site and the extent of the tumour.

We carried out six consecutive resections of musculoskeletal tumour in five patients using an existing commercial computer navigation system. There were three women and two men with a mean age of 41 years (24 to 47). Reconstruction was performed using a tumour prosthesis in three lesions and a vascularised fibular graft in one. No reconstruction was needed in two cases. The mean follow-up was 6.9 months (3.5 to 10). The mean duration of surgery was 28 minutes (13 to 50). Examination of the resected specimens showed clear margins in all the tumour lesions and a resection that was exactly as planned.


The Journal of Bone & Joint Surgery British Volume
Vol. 93-B, Issue 7 | Pages 980 - 983
1 Jul 2011
Malhas AM Grimer RJ Abudu A Carter SR Tillman RM Jeys L

We investigated the eventual diagnosis in patients referred to a tertiary centre with a possible diagnosis of a primary bone malignancy.

We reviewed our database from between 1986 and 2010, during which time 5922 patients referred with a suspicious bone lesion had a confirmed diagnosis. This included bone sarcoma in 2205 patients (37%), benign bone tumour in 1309 (22%), orthopaedic conditions in 992 (17%), metastatic disease in 533 (9%), infection in 289 (5%) and haematological disease in 303 (5%). There was a similar frequency of all diagnoses at different ages except for metastatic disease. Only 0.6% of patients (17 of 2913) under the age of 35 years had metastatic disease compared with 17.1% (516 of 3009) of those over 35 years (p < 0.0001). Of the 17 patients under 35 years with metastatic disease, only four presented with an isolated lesion, had no past history of cancer and were systematically well.

Patients under the age of 35 years should have suitable focal imaging (plain radiography, CT or MRI) and simple systemic studies (blood tests and chest radiography). Reduction of the time to biopsy can be achieved by avoiding an unnecessary investigation for a primary tumour to rule out metastatic disease.