Aims

Current literature suggests that survival outcomes and local recurrence rates of primary soft-tissue sarcoma diagnosed in the very elderly age range, (over 90 years), are comparable with those in patients diagnosed under the age of 75 years. Our aim is to quantify these outcomes with a view to rationalizing management and follow-up for very elderly patients.

Aims

The aims of the study were to analyze differences in surgical and oncological outcomes, as well as quality of life (QoL) and function in patients with ankle sarcomas undergoing three forms of surgical treatment, minor or major limb salvage surgery (LSS), or amputation.

Aims

The aim of this study was to report the results of three forms of reconstruction for patients with a ditsl tibial bone tumour: an intercalary resection and reconstruction, an osteoarticular reconstruction, and arthrodesis of the ankle.

Aims. We retrospectively report our experience of managing 30 patients with a primary malignant tumour of the distal tibia; 25 were treated by limb salvage surgery and five by amputation. We compared the clinical outcomes of following the use of different methods of reconstruction. Patients and Methods. There were 19 male and 11 female patients. The mean age of the patients was 19 years (6 to 59) and the mean follow-up was 5.1 years (1.25 to 12.58). Massive allograft was used in 11 patients, and autograft was used in 14 patients. The time to union, the survival time of the reconstruction, complication rate, and functional outcomes following the different surgical techniques were compared. The overall patient survival was also recorded. Results. Out of 14 patients treated with an autograft, 12 (86%) achieved union at both the proximal and distal junctions. The time to union at both junctions of the autograft was significantly shorter than in those treated with an allograft (11.1 vs 17.2 months, p = 0.02; 9.5 vs 16.2 months, p = 0.04). The complication rate of allograft reconstruction was 55%. The five patients treated with an amputation did not have a complication. Out of the 25 patients who were treated with limb salvage, three (12%) developed local recurrence and underwent amputation. The mean functional Musculoskeletal Tumor Society (MSTS) score after autograft reconstruction was higher than after allograft reconstruction (81% vs 67%; p = 0.06), and similar to that after amputation (81% vs 82%; p = 0.82). The two- and five-year overall rates of survival were 83% and 70%, respectively. Conclusions. This consecutive case series supports the safety of limb salvage and the effectiveness of biological reconstruction after the resection of a primary tumour of the distal tibia. Autograft might be a preferable option. In some circumstances, below-knee amputation remains a valid option

Patients who have limb amputation for musculoskeletal tumours are a rare group of cancer survivors. This was a prospective cross-sectional survey of patients from five specialist centres for sarcoma surgery in England. Physical function, pain and quality of life (QOL) outcomes were collected after lower extremity amputation for bone or soft-tissue tumours to evaluate the survivorship experience and inform service provision.

Of 250 patients, 105 (42%) responded between September 2012 and June 2013. From these, completed questionnaires were received from 100 patients with a mean age of 53.6 years (19 to 91). In total 60 (62%) were male and 37 (38%) were female (three not specified). The diagnosis was primary bone sarcoma in 63 and soft-tissue tumour in 37. A total of 20 tumours were located in the hip or pelvis, 31 above the knee, 32 between the knee and ankle and 17 in the ankle or foot. In total 22 had hemipelvectomy, nine hip disarticulation, 35 transfemoral amputation, one knee disarticulation, 30 transtibial amputation, two toe amputations and one rotationplasty. The Toronto Extremity Salvage Score (TESS) differed by amputation level, with poorer scores at higher levels (p < 0.001). Many reported significant pain. In addition, TESS was negatively associated with increasing age, and pain interference scores. QOL for Cancer Survivors was significantly correlated with TESS (p < 0.001). This relationship appeared driven by pain interference scores.

This unprecedented national survey confirms amputation level is linked to physical function, but not QOL or pain measures. Pain and physical function significantly impact on QOL. These results are helpful in managing the expectations of patients about treatment and addressing their complex needs.

Cite this article: Bone Joint J 2015;97-B:1284–90.

In this case study, we describe the clinical presentation and treatment of 36 patients with periosteal chondrosarcoma collected over a 59-year period by the archive of the Netherlands Committee on Bone Tumours. The demographics, clinical presentation, radiological features, treatment and follow-up are presented with the size, location, the histological grading of the tumour and the survival.

We found a slight predominance of men (61%), and a predilection for the distal femur (33%) and proximal humerus (33%). The metaphysis was the most common site (47%) and the most common presentation was with pain (44%). Half the tumours were classified histologically as grade 1. Pulmonary metastases were reported in one patient after an intra-lesional resection. A second patient died from local recurrence and possible pulmonary and skin metastases after an incomplete resection.

It is clearly important to make the diagnosis appropriately because an incomplete resection may result in local recurrence and metastatic spread. Staging for metastatic disease is recommended in grade II or III lesions.

These patients should be managed with a contrast-enhanced MRI of the tumour and histological confirmation by biopsy, followed by en-bloc excision.

Cite this article: Bone Joint J 2014;96-B:823–8.

Bone sarcomas are rare cancers and orthopaedic surgeons come across them infrequently, sometimes unexpectedly during surgical procedures. We investigated the outcomes of patients who underwent a surgical procedure where sarcomas were found unexpectedly and were subsequently referred to our unit for treatment. We identified 95 patients (44 intra-lesional excisions, 35 fracture fixations, 16 joint replacements) with mean age of 48 years (11 to 83); 60% were males (n = 57). Local recurrence arose in 40% who underwent limb salvage surgery versus 12% who had an amputation. Despite achieving local control, overall survival was worse for patients treated with amputation rather than limb salvage (54% vs 75% five-year survival). Factors that negatively influenced survival were invasive primary surgery (fracture fixation, joint replacement), a delay of greater than two months until referral to our oncology service, and high-grade tumours. Survival in these circumstances depends mostly on factors that are determined prior to definitive treatment by a tertiary orthopaedic oncology unit. Limb salvage in this group of patients is associated with a higher rate of inadequate marginal surgery and, consequently, higher local recurrence rates than amputation, but should still be attempted whenever possible, as local control is not the primary determinant of survival.

Cite this article: Bone Joint J 2014;96-B:665–72.

There are eight reported cases in the literature of osteosarcomas secreting β-hCG. Our primary aim was to investigate the rate of β-hCG expression in osteosarcoma and attempt to understand the characteristics of osteosarcomas that secrete β-hCG. We reviewed 37 histopathology slides (14 biopsies and 23 surgical specimens) from 32 patients with osteosarcoma. The slides were retrospectively stained for β-hCG expression. Patient and tumour characteristics, including age, gender, tumour location, subtype, proportion of necrosis, presence of metastases and recurrence were recorded. A total of five of the 32 tumours were found to be positive for β-hCG expression (one strongly and four weakly). This incidence of this expression was found in tumours with poor histological response to neoadjuvant chemotherapy.

The use of β-hCG expression as a diagnostic, prognostic or follow-up marker is questionable and needs further investigation with a larger sample size.

The best method of reconstruction after resection of malignant tumours of the tibial diaphysis is unknown. In the absence of any long-term studies analysing the results of intercalary endoprosthetic replacement, we present a retrospective review of 18 patients who underwent limb salvage using a tibial diaphyseal endoprosthetic replacement following excision of a malignant bone tumour. There were ten men and eight women with a mean age of 42.5 years (16 to 76). Mean follow-up was 58.5 months (20 to 141) for all patients and 69.3 months (20 to 141) for the 12 patients still alive. Cumulative patient survival was 59% (95% confidence interval (CI) 32 to 84) at five years. Implant survival was 63% (95% CI 35 to 90) at ten years. Four patients required revision to a proximal tibial replacement at a mean follow-up of 29 months (10 to 54). Complications included metastases in five patients, aseptic loosening in four, peri-prosthetic fracture in two, infection in one and local recurrence in one. The mean Musculoskeletal Tumor Society score and the mean Toronto Extremity Salvage Score were 23 (17 to 28) and 74% (53 to 91), respectively.

Although rates of complication and revision were high, custom-made tibial diaphyseal replacement following resection of malignant bone tumours enables early return to function and provides an attractive alternative to other surgical options, without apparent compromise of patient survival.

We evaluated the oncological and functional outcome of 27 patients who had limb salvage for a soft-tissue sarcoma of the foot or ankle between 1992 and 2007, with a mean follow-up of 7.5 years (1.05 to 16.2). There were 12 men and 15 women, with a mean age at presentation of 47 years (12 to 84). Referrals came from other hospitals for 16 patients who had previous biopsy or unplanned excision, and 11 presented de novo. There were 18 tumours located in the foot and nine around the ankle. Synovial sarcoma was the most frequent histological diagnosis. Excision was performed in all cases, with 16 patients requiring plastic surgical reconstruction with 13 free and three local flaps. Adjuvant treatment was undertaken in 20 patients, 18 with radiotherapy and two by chemotherapy. Limb salvage was successful in 26 of the 27 patients. There have been two local recurrences and two mesenchymal metastases. Four patients have died of their sarcoma and two of other causes.

Function was evaluated with the Toronto Extremity Salvage Score and a mean overall score of 89.40 (52.1 to 100) was obtained. A questionnaire revealed that all surviving patients are able to wear normal shoes and none require a walking aid.

Limb salvage can achieve good oncological and functional results with additional treatment.

Endoprosthetic replacement of the distal tibia and ankle joint for a primary bone tumour is a rarely attempted and technically challenging procedure. We report the outcome of six patients treated between 1981 and 2007. There were four males and two females, with a mean age of 43.5 years (15 to 75), and a mean follow-up of 9.6 years (1 to 27). No patient developed a local recurrence or metastasis. Two of the six went on to have a below-knee amputation for persistent infection after a mean 16 months (1 to 31). The four patients who retained their endoprosthesis had a mean musculoskeletal tumour society score of 70% and a mean Toronto extremity salvage score of 71%. All were pain free and able to perform most activities of daily living in comfort. A custom-made endoprosthetic replacement of the distal tibia and ankle joint is a viable treatment option for carefully selected patients with a primary bone tumour. Patients should, however, be informed of the risk of infection and the potential need for amputation if this cannot be controlled

This paper describes the preliminary results of a proximal tibial endoprosthesis which spares the knee joint and enables retention of the natural articulation by replacing part of the tibial metaphysis and diaphysis. In eight patients who had a primary malignant bone tumour of the proximal tibia, the distal stem, which had a hydroxyapatite-coated collar to improve fixation, was cemented into the medullary canal. The proximal end had hydroxyapatite-coated extracortical plates which were secured to the remaining proximal tibial metaphysis using cortical screws. The mean age of the patients at operation was 28.9 years (8 to 43) and the mean follow-up was for 35 months (4 to 48). The mean Musculoskeletal Tumour Society score was 79% (57% to 90%), the mean Oxford Knee score was 40 points of 48 (36 to 46) and the mean knee flexion was 112° (100° to 120°). In one patient, revision to a below-knee amputation through the prosthesis was required because of recurrence of the tumour. Another patient sustained a periprosthetic fracture which healed with a painful malunion. This was revised to a further endoprosthesis which replaced the knee. In the remaining six patients the prosthesis allowed preservation of the knee joint with good function and no early evidence of loosening. Further follow-up is required to assess the longevity of these prostheses

We evaluated the results of fibular centralisation as a stand alone technique to reconstruct defects that occurred after resection of tumours involving the tibial diaphysis and distal metaphysis. Between January 2003 and December 2006, 15 patients underwent excision of tumours of the tibial diaphysis or distal metaphysis and reconstruction by fibular centralisation. Their mean age was 17 years (7 to 40). Two patients were excluded; one died from the complications of chemotherapy and a second needed a below-knee amputation for a recurrent giant-cell tumour. A total of 13 patients were reviewed after a mean follow-up of 29 months (16 to 48). Only 16 of 26 host graft junctions united primarily. Ten junctions in ten patients needed one or more further procedure before union was achieved. At final follow-up 12 of the 13 patients had fully united grafts; 11 walked without aids. The mean time to union at the junctions that united was 12 months (3 to 36). The mean Musculoskeletal Tumor Society Score was 24.7 (16 to 30). Fibular centralisation is a durable reconstruction for defects of the tibial diaphysis and distal metaphysis with an acceptable functional outcome. Stable osteosynthesis is the key to successful union. Additional bone grafting is recommended for patients who need postoperative radiotherapy

We evaluated the long-term functional outcome in 118 patients treated for osteosarcoma or Ewing’s sarcoma in the extremities a minimum of five years after treatment. We also examined if impaired function influenced their quality of life and ability to work.

The function was evaluated according to the Musculoskeletal Tumor Society (MSTS) score and the Toronto Extremity Salvage Score (TESS). Quality of life was assessed by using the Short Form-36 (SF-36).

The mean age at follow-up was 31 years (15 to 57) and the mean follow-up was for 13 years (6 to 22). A total of 67 patients (57%) initially had limb-sparing surgery, but four had a secondary amputation. The median MSTS score was 70% (17% to 100%) and the median TESS was 89% (43% to 100%). The amputees had a significantly lower MSTS score than those with limb-sparing surgery (p < 0.001), but there was no difference for the TESS. Tumour localisation above knee level resulted in significantly lower MSTS scores and TESS (p = 0.003 and p = 0.02, respectively).

There were no significant differences in quality of life between amputees and those with limb-sparing surgery except in physical functioning. Of the patients 11% (13) did not work or study. In multivariate analysis, amputation, tumour location above the knee and having muscular pain were associated with low physical function.

We conclude that most of the bone tumour survivors managed well after adjustment to their physical limitations. A total of 105 are able to work and have an overall good quality of life.

Four patients who developed malignant synovial tumours are described; one with chondromatosis developed a synovial chondrosarcoma and three with pigmented villonodular synovitis developed malignant change. The relevant literature is discussed.

We report the results of limb salvage for non-metastatic osteosarcoma of the distal tibia using resection arthrodesis, autogenous fibular graft and fixation by an Ilizarov external fixator.

In six patients with primary osteosarcoma of the distal tibia who refused amputation, treatment with wide en bloc resection and tibiotalar arthrodesis was undertaken. The defect was reconstructed using non-vascularised free autogenous fibular strut graft in three patients and a vascularised pedicular fibular graft in three, all supplemented with iliac cancellous graft at the graft-host junction. An Ilizarov external fixator was used for stabilisation of the reconstruction.

In five patients sound fusion occurred at a mean of 13.2 months (8 to 20) with no evidence of local recurrence or deep infection at final follow-up. The mean post-operative functional score was 70% (63% to 73%) according to the Musculoskeletal Tumour Society scoring system. All five patients showed graft hypertrophy.

Union of the graft was faster in cases reconstructed by vascularised fibular grafts. One patient who had a poor response to pre-operative chemotherapy developed local tumour recurrence at one year post-operatively and required subsequent amputation.

We undertook this retrospective study to determine the rate of recurrence and functional outcome after intralesional curettage for chondroblastoma of bone. The factors associated with aggressive behaviour of the tumour were also analysed. We reviewed 53 patients with histologically-proven chondroblastoma who were treated by intralesional curettage in our unit between 1974 and 2000. They were followed up for at least two years to a maximum of 27 years. Seven (13.2%) had a histologically-proven local recurrence. Three underwent a second intralesional curettage and had no further recurrence. Two had endoprosthetic replacement of the proximal humerus and two underwent below-knee amputation after aggressive local recurrence. One patient had the rare malignant metastatic chondroblastoma and eventually died. The mean Musculoskeletal Tumour Society functional score of the survivors was 94.2%. We conclude that meticulous intralesional curettage alone can achieve low rates of local recurrence and excellent long-term function