We performed a systematic literature review to define features of patients, treatment, and biological behaviour of multicentric giant cell tumour (GCT) of bone. The search terms used in combination were “multicentric”, “giant cell tumour”, and “bone”. Exclusion criteria were: reports lacking data, with only an abstract; papers not reporting data on multicentric GCT; and papers on multicentric GCT associated with other diseases. Additionally, we report three patients treated under our care.Aims
Methods
The aim of this study was to report the patterns of symptoms and insufficiency fractures in patients with tumour-induced osteomalacia (TIO) to allow the early diagnosis of this rare condition. The study included 33 patients with TIO who were treated between January 2000 and June 2022. The causative tumour was detected in all patients. We investigated the symptoms and evaluated the radiological patterns of insufficiency fractures of the rib, spine, and limbs.Aims
Methods
Internal hemipelvectomy without reconstruction of the pelvis is a viable treatment for pelvic sarcoma; however, the time it takes to return to excellent function is quite variable. Some patients require greater time and rehabilitation than others. To determine if psoas muscle recovery is associated with changes in ambulatory function, we retrospectively evaluated psoas muscle size and limb-length discrepancy (LLD) before and after treatment and their correlation with objective functional outcomes. T1-weighted MR images were evaluated at three intervals for 12 pelvic sarcoma patients following interval hemipelvectomy without reconstruction. Correlations between the measured changes and improvements in Timed Up and Go test (TUG) and gait speed outcomes were assessed both independently and using a stepwise multivariate regression model.Aims
Methods
The aim of this study was to investigate the feasibility of application of a 3D-printed megaprosthesis with hemiarthroplasty design for defects of the distal humerus or proximal ulna following tumour resection. From June 2018 to January 2020, 13 patients with aggressive or malignant tumours involving the distal humerus (n = 8) or proximal ulna (n = 5) were treated by en bloc resection and reconstruction with a 3D-printed megaprosthesis with hemiarthroplasty, designed in our centre. In this paper, we summarize the baseline and operative data, oncological outcome, complication profiles, and functional status of these patients.Aims
Methods
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. 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).Aims
Methods
There is an increased risk of dislocation of the hip after the resection of a periacetabular tumour and endoprosthetic reconstruction of the defect in the hemipelvis. The aim of this study was to determine the rate and timing of dislocation and to identify its risk factors. To determine the dislocation rate, we conducted a retrospective single-institution study of 441 patients with a periacetabular tumour who had undergone a standard modular hemipelvic endoprosthetic reconstruction between 2003 and 2019. After excluding ineligible patients, 420 patients were enrolled. Patient-specific, resection-specific, and reconstruction-specific variables were studied using univariate and multivariate analyses.Aims
Methods
To investigate the benefits of denosumab in combination with nerve-sparing surgery for treatment of sacral giant cell tumours (GCTs). This is a retrospective cohort study of patients with GCT who presented between January 2011 and July 2017. Intralesional curettage was performed and patients treated from 2015 to 2017 also received denosumab therapy. The patients were divided into three groups: Cohort 1: control group (n = 36); cohort 2: adjuvant denosumab group (n = 9); and cohort 3: neo- and adjuvant-denosumab group (n = 17).Aims
Methods
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. 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.Aims
Patients and Methods
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. 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.Aims
Patients and Methods
Resection of the proximal humerus for the primary malignant bone
tumour sometimes requires We reviewed 45 patients who had undergone resection of a primary
malignant tumour of the proximal humerus. There were 29 in the deltoid
sparing group and 16 in the deltoid resecting group. Imaging studies
were reviewed to assess tumour extension and soft-tissue involvement.
The presence of a fat rim separating the tumour from the deltoid
on MRI was particularly noted. The cumulative probability of local
recurrence was calculated in a competing risk scenario.Aims
Patients and Methods
We report a prospective cohort study of the midterm results of
surgical dislocation of the hip (according to Ganz) to perform resection
of osteochondromas involving the femoral neck in patients with multiple
hereditary exostoses (MHE). Hip range of movement (ROM) was assessed pre- and post-operatively.
Patients’ judgment of post-operative reduction of pain, symptoms,
the Rand 36-item Health Survey (RAND-36) and complications were
analysed. Aims
Methods
Avascular necrosis (AVN) is a serious complication
of high-dose chemotherapy for haematological malignancy in childhood.
In order to describe its incidence and main risk factors and to
evaluate the current treatment options, we reviewed 105 children
with a mean age of 8.25 years (1 to 17.8) who had acute lymphoblastic
or acute myeloid leukaemia, or a non-Hodgkin’s lymphoma. Overall,
eight children (7.6%) developed AVN after a mean of 16.8 months (8
to 49). There were four boys and four girls with a mean age of 14.4
years (9.8 to 16.8) and a total of 18 involved sites, 12 of which
were in the femoral head. All these children were aged >
nine years
(p <
0.001). All had received steroid treatment with a mean cumulative
dose of prednisone of 5967 mg (4425 to 9599) compared with a mean
of 3943 mg (0 to 18 585) for patients without AVN (p = 0.005). No
difference existed between genders and no thrombophilic disorders
were identified. Their initial treatment included 11 core decompressions
and two bipolar hip replacements. Later, two salvage osteotomies
were done and three patients (four hips) eventually needed a total
joint replacement. We conclude that AVN mostly affects the weight-bearing
epiphyses. Its risk increases with age and higher steroid doses.
These high-risk patients may benefit from early screening for AVN. Cite this article:
Guidelines for the management of patients with metastatic bone
disease (MBD) have been available to the orthopaedic community for
more than a decade, with little improvement in service provision
to this increasingly large patient group. Improvements in adjuvant
and neo-adjuvant treatments have increased both the number and overall
survival of patients living with MBD. As a consequence the incidence
of complications of MBD presenting to surgeons has increased and
is set to increase further. The British Orthopaedic Oncology Society
(BOOS) are to publish more revised detailed guidelines on what represents
‘best practice’ in managing patients with MBD. This article is designed
to coincide with and publicise new BOOS guidelines and once again
champion the cause of patients with MBD. A series of short cases highlight common errors frequently being
made in managing patients with MBD despite the availability of guidelines.Objectives
Methods
Elastofibroma dorsi is an uncommon, benign, slow-growing soft-tissue tumour of uncertain aetiology. It classically presents as an ill-defined mass at the inferior pole of the scapula with symptoms which include swelling, discomfort, snapping, stiffness and occasionally pain. We report the symptoms, function and outcome after treatment of 21 elastofibromas in 15 patients. All were diagnosed by MRI and early in the series four also underwent CT-guided biopsy to confirm the diagnosis. In all, 18 tumours were excised and three were observed. After excision, the mean visual analogue score for pain decreased from 4.6 (0 to 10) pre-operatively to 2.4 (0 to 8) post-operatively (p = 0.04). The mean shoulder function, at a mean follow-up of 4.2 years (3 months to 16 years), was 78.1% (30 to 100) using the Stanmore percentage of normal shoulder assessment scoring system. The mean range of forward flexion improved from 135° (70° to 180°) to 166° (100° to 180°) after excision (p = 0.005). In four patients a post-operative haematoma formed; one required evacuation. Three patients developed a post-operative seroma requiring needle aspiration and one developed a superficial infection which was treated with antibiotics. Our findings support previous reports suggesting that a pre-operative tissue diagnosis is not necessary in most cases since the lesion can be confidently diagnosed by MRI, when interpreted in the light of appropriate clinical findings. Surgical excision in symptomatic patients, is helpful. It has been suggested that elastofibroma is caused by a local tissue reaction and is not a true neoplastic process. A strong association has been noted between elastofibroma and repetitive use of the shoulder, which is supported by our findings.
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 current indications for functional restoration
of extension of the knee following quadriceps resection or loss require
reappraisal. The contribution of pedicled and free functional muscle
transfer is likely to be over-emphasised in many studies, with good
functional outcomes predominantly reported only in the context of
cases with residual quadriceps function. In cases with total quadriceps
resection or loss, all forms of reconstruction perform poorly. Furthermore,
in smaller resections with loss of two or fewer components of the
quadriceps, minimal impairment of function occurs in the absence
of functional reconstruction, suggesting that functional restoration
may not be warranted. Thus there is a paradox in the current approach
to quadriceps reconstruction, in that small resections are likely
to be over-treated and large resections remain under-treated. This review suggests a shift is required in the approach and
rationale for reconstructing functional extension of the knee after
quadriceps resection or loss. A classification based on current
evidence is suggested that emphasises more clearly the indications
and rationale for functional transfers.
Endoprosthetic reconstruction following resection of 31 tumours of the proximal femur in 30 patients was performed using a Wagner SL femoral revision stem. The mean follow-up was 25.6 months (0.6 to 130.0). Of the 28 patients with a metastasis, 27 died within a mean follow-up period of 18.1 months (0.6 to 56.3) after the operation, and the remaining patient was excluded from the study 44.4 months post-operatively when the stem was removed. The two patients with primary bone tumours were still alive at the latest follow-up of 81.0 and 130.0 months, respectively. One stem only was removed for suspected low-grade infection 44.4 months post-operatively. The worst-case survival rate with removal of the stem for any cause and/or loss to follow-up was 80.0% (95% confidence interval 44.9 to 100) at 130.0 months. The mean Karnofsky index increased from 44.2% (20% to 70%) pre-operatively to 59.7% (0% to 100%) post-operatively, and the mean Merle d’Aubigné score improved from 4.5 (0 to 15) to 12.0 (0 to 18). The mean post-operative Musculoskeletal Tumour Society score was 62.4% (3.3% to 100%). The Wagner SL femoral revision stem offers an alternative to special tumour prostheses for the treatment of primary and secondary tumours of the proximal femur. The mid-term results are very promising, but long-term experience is necessary.
The humerus is a common site for skeletal metastases in the adult. Surgical stabilisation of such lesions is often necessary to relieve pain and restore function. These procedures are essentially palliative and should therefore provide effective relief from pain for the remainder of the patient’s life without the need for further surgical intervention. We report a retrospective analysis of 35 patients (37 nails) with symptomatic metastases in the shaft of the humerus which were treated by locked, antegrade nailing. There were 27 true fractures (73.0%) and ten painful deposits (27.0%). Relief from pain was excellent in four (11.4%), good in 29 (82.9%) and fair in two (5.7%) on discharge. Function was improved in all but one patient. One case of palsy of the radial nerve was noted. The mean postoperative survival was 7.1 months (0.2 to 45.5) which emphasises the poor prognosis in this group of patients. There were no failures of fixation and no case in which further surgery was required. Antegrade intramedullary nailing is an effective means of stabilising the humerus for the palliative treatment of metastases. It relieves pain and restores function to the upper limb with low attendant morbidity.
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
A retrospective analysis was performed of eight patients with an open triradiate cartilage, who underwent resection for osteosarcoma and reconstruction of the proximal femur with a hemiarthroplasty, in order to identify changes of acetabular development. An analysis of the centre-edge angle, teardrop-to-medial prosthesis distance, superior joint space, teardrop-to-superior prosthesis distance, degree of lateral translation, and arthritic changes, was performed on serial radiographs. The median age at the time of the initial surgery was 11 years (5 to 14). All patients developed progressive superior and lateral migration of the prosthetic femoral head. Following hemiarthroplasty in the immature acetabulum, the normal deepening and enlargement of the acetabulum is arrested. The degree of superior and lateral migration of the prosthetic head depends on the age at diagnosis and the length of follow-up.
