Tenosynovial giant cell tumour (TGCT) is a rare benign tumour of the musculoskeletal system. Surgical management is fraught with challenges due to high recurrence rates. The aim of this study was to describe surgical treatment and evaluate surgical outcomes of TGCT at an Australian tertiary referral centre for musculoskeletal tumours and to identify factors affecting recurrence rates. A prospective database of all patients with TGCT surgically managed by two orthopaedic oncology surgeons was reviewed. All cases irrespective of previous treatment were included and patients without follow-up were excluded. Pertinent tumour characteristics and surgical outcomes were collected for analysis.Aims
Methods
Low-grade central osteosarcoma (LGCOS), a rare type of osteosarcoma, often has misleading radiological and pathological features that overlap with those of other bone tumours, thereby complicating diagnosis and treatment. We aimed to analyze the clinical, radiological, and pathological features of patients with LGCOS, with a focus on diagnosis, treatment, and outcomes. We retrospectively analyzed the medical records of 49 patients with LGCOS (Broder’s grade 1 to 2) treated between January 1985 and December 2017 in a single institute. We examined the presence of malignant features on imaging (periosteal reaction, cortical destruction, soft-tissue invasion), the diagnostic accuracy of biopsy, surgical treatment, and oncological outcome.Aims
Methods
Dislocation of the hip remains a major complication after periacetabular tumour resection and endoprosthetic reconstruction. The position of the acetabular component is an important modifiable factor for surgeons in determining the risk of postoperative dislocation. We investigated the significance of horizontal, vertical, and sagittal displacement of the hip centre of rotation (COR) on postoperative dislocation using a CT-based 3D model, as well as other potential risk factors for dislocation. A total of 122 patients who underwent reconstruction following resection of periacetabular tumour between January 2011 and January 2020 were studied. The risk factors for dislocation were investigated with univariate and multivariate logistic regression analysis on patient-specific, resection-specific, and reconstruction-specific variables.Aims
Methods
We report the results of the treatment of nine children with an aneurysmal bone cyst of the distal fibula (seven cysts were juxtaphyseal, and two metaphyseal). The mean age of the children was 10 years and 3 months (7 years and 4 months to 12 years and 9 months). All had open physes. All cysts were active and in seven cases substituted and expanded the entire width of the bone (type-2 lesions). The mean longitudinal extension was 5.7 cm (3 to 10). The presenting symptoms were pain,
Tenosynovial giant cell tumour (TGCT) is one of the most common soft-tissue tumours of the foot and ankle and can behave in a locally aggressive manner. Tumour control can be difficult, despite the various methods of treatment available. Since treatment guidelines are lacking, the aim of this study was to review the multidisciplinary management by presenting the largest series of TGCT of the foot and ankle to date from two specialized sarcoma centres. The Oxford Tumour Registry and the Leiden University Medical Centre Sarcoma Registry were retrospectively reviewed for patients with histologically proven foot and ankle TGCT diagnosed between January 2002 and August 2019.Aims
Methods
Aims Although chondrosarcomas (CSs) display true malignant features, including local recurrence (LR) and metastases, their behaviour in the hands and feet is thought to differ from that in other parts of the axial and appendicular skeleton by having a lower metastatic potential. The purpose of this study was to investigate the disease-specific and surgical factors that affect the local and systemic prognosis of CS of the hands and feet. A multicentre retrospective study was carried out at two tertiary sarcoma centres. A database search identified all patients with a CS treated between January 1995 and January 2018. There were 810 CSs of which 76 (9.4%) were located in the fingers, toes, metacarpals, and metatarsal bones. The median age of the study population was 55 years (36 to 68) with a median follow-up of 52 months (22 to 87) months. Overall, 70% of the tumours were in the hand (n = 54) and 30% in the foot (n = 22). Predictors for LR were margin (p = 0.011), anatomical location (p = 0.017), and method of surgical management (p = 0.003). Anatomical location (p = 0.026), histological grade between 1 and 3 (p = 0.004) or 2 and 3 (p = 0.016), and surgical management (p = 0.001) were significant factors for LR-free survival. Disease-specific survival was affected by histological grade (p < 0.001), but not by LR (p = 0.397).Methods
Results
Aneurysmal bone cysts (ABCs) are locally aggressive lesions typically found in the long bones of children and adolescents. A variety of management strategies have been reported to be effective in the treatment of these lesions. The purpose of this review was to assess the effectiveness of current strategies for the management of primary ABCs of the long bones. A systematic review of the published literature was performed to identify all articles relating to the management of primary ABCs. Studies required a minimum 12-month follow-up and case series reporting on under ten participants were not included.Aims
Methods
Local recurrence remains a challenging and common problem following curettage and joint-sparing surgery for giant cell tumour of bone (GCTB). We previously reported a 15% local recurrence rate at a median follow-up of 30 months in 20 patients with high-risk GCTB treated with neoadjuvant Denosumab. The aim of this study was to determine if this initial favourable outcome following the use of Denosumab was maintained with longer follow-up. Patients with GCTB of the limb considered high-risk for unsuccessful joint salvage, due to minimal periarticular and subchondral bone, large soft tissue mass, or pathological fracture, were treated with Denosumab followed by extended intralesional curettage with the goal of preserving the joint surface. Patients were followed for local recurrence, metastasis, and secondary sarcoma.Aims
Methods
Our aim was to investigate the outcome of excision of osteochondromas. Between 1994 and 1998, 92 symptomatic osteochondromas in 86 patients were excised. There were 40 women and 46 men with a mean age of 20 years (3 to 62). Of these, 56 had a solitary osteochondroma and 30 had multiple hereditary tumours. The presenting symptoms were pain (79.1%),
A mucoid pseudocyst of a peripheral nerve is a rare and benign tumour of controversial origin. We have reviewed ten patients with a mean follow-up of 3.2 years. The tumour affected the common peroneal nerve in eight and the ulnar nerve in two. The mean time between the onset of symptoms and diagnosis was 7.4 months (1.2 months to 2 years). On examination, there was pain in eight patients and
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
Giant-cell tumour of the tendon sheath (GCT-TS) is a benign solitary tumour which usually arises in the limbs. It occurs most often in the hand where local recurrence after excision has been reported in up to 45% of cases. It is less common in the foot where the biological behaviour and risk of local recurrence have not been defined. We have studied 17 cases of GCT-TS of the foot and ankle in which treatment was by excision. Fifteen presented as a solitary, painless, slow-growing soft-tissue
The aim of this study was to evaluate health-related quality of life (HRQoL) and joint function in tenosynovial giant cell tumour (TGCT) patients before and after surgical treatment. This prospective cohort study run in two Dutch referral centres assessed patient-reported outcome measures (PROMs; 36-Item Short-Form Health Survey (SF-36), visual analogue scale (VAS) for pain, and Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC)) in 359 consecutive patients with localized- and diffuse-type TGCT of large joints. Patients with recurrent disease (n = 121) and a wait-and-see policy (n = 32) were excluded. Collected data were analyzed at specified time intervals preoperatively (baseline) and/or postoperatively up to five years.Aims
Patients and Methods
The aim of this study was to report the outcomes of patients who underwent definitive surgery for secondary chondrosarcomas arising from osteochondromas. A total of 51 patients with secondary chondrosarcomas occurring from osteochondromas were reviewed. Median age was 36 years (interquartile range (IQR) 15 to 82). Median follow-up was 6.9 years (IQR 2.8 to 10.6). The pelvis was the most commonly affected site (59%). Histological grades were grade I in 35 (69%), grade II in 13 (25%), and grade III in three patients (6%).Aims
Patients and Methods
The aim of this study was to identify factors that determine outcomes of treatment for patients with chondroblastic osteosarcomas (COS) of the limbs and pelvis. The authors carried out a retrospective review of prospectively collected data from 256 patients diagnosed between 1979 and 2015. Of the 256 patients diagnosed with COS of the pelvis and the limbs, 147 patients (57%) were male and 109 patients (43%) were female. The mean age at presentation was 20 years (0 to 90).Aims
Patients and Methods
The aims of this study were to evaluate the efficacy of preoperative denosumab in achieving prospectively decided intention of therapy in operable giant cell tumour of bone (GCTB) patients, and to document local recurrence-free survival (LRFS). A total of 44 patients received preoperative denosumab: 22 to facilitate curettage, 16 to facilitate resection, and six with intent of converting resection to curettage. There were 26 male and 18 female patients. The mean age was 27 years (13 to 47).Aims
Patients and Methods
Intra-articular 90Yttrium (90Y) is an adjunct
to surgical treatment by synovectomy for patients with diffuse-type tenosynovial
giant-cell tumour (dtTGCT) of the knee, with variable success rates.
Clinical information is, however, sparse and its value remains unclear.
We investigated the long-term outcome of patients who underwent synovectomy
with and without adjuvant treatment with 90Yttrium. All patients with dtTGCT of the knee who underwent synovectomy
between 1991 and 2014 were included in the study. Group A patients
underwent synovectomy and an intra-articular injection of 90Yttrium
between six and eight weeks after surgery. Group B patients underwent
surgery alone.Aims
Patients and Methods
The aim of this study was to identify any progression between
benign osteofibrous dysplasia (OFD), OFD-like adamantinoma and malignant
adamantinoma, and to investigate the rates of local recurrence,
metastases and survival, in order to develop treatment algorithms
for each. A single institution retrospective review of all patients presenting
with OFD, OFD-like adamantinoma and adamantinoma between 1973 and
2012 was undertaken. Complete data were available for 73 patients
(42 with OFD; ten with an OFD-like adamantinoma and 21 with an adamantinoma).
The mean follow-up was 10.3 years (3 to 25) for OFD, 9.2 years (3.0
to 26.3) for OFD-like and 11.6 years (0.25 to 33) for adamantinoma.Aims
Patients and Methods
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 Cite this article:
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. 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.Aims
Patients and Methods
Free vascularised fibular grafting has been used for the treatment
of large bony defects for more than 40 years. However, there is
little information about the risk factors for failure and whether
newer locking techniques of fixation improve the rates of union.
The purpose of this study was to compare the rates of union of free
fibular grafts fixed with locking and traditional techniques, and
to quantify the risk factors for nonunion and failure. A retrospective review involved 134 consecutive procedures over
a period of 20 years. Of these, 25 were excluded leaving 109 patients
in the study. There were 66 men and 43 women, with a mean age of
33 years (5 to 78). Most (62) were performed for oncological indications,
and the most common site (52) was the lower limb. Rate of union
was estimated using the Kaplan-Meier method and risk factors for
nonunion were assessed using Cox regression. All patients were followed
up for at least one year.Aims
Patients and Methods
The pre-operative differentiation between enchondroma,
low-grade chondrosarcoma and high-grade chondrosarcoma remains a
diagnostic challenge. We reviewed the accuracy and safety of the
radiological grading of cartilaginous tumours through the assessment
of, first, pre-operative radiological and post-operative histological agreement,
and second the rate of recurrence in lesions confirmed as high-grade
on histology. We performed a retrospective review of major long
bone cartilaginous tumours managed by curettage as low grade between
2001 and 2012. A total of 53 patients with a mean age of 47.6 years
(8 to 71) were included. There were 23 men and 30 women. The tumours
involved the femur (n = 20), humerus (n = 18), tibia (n = 9), fibula
(n = 3), radius (n = 2) and ulna (n = 1). Pre-operative diagnoses
resulted from multidisciplinary consensus following radiological
review alone for 35 tumours, or with the addition of pre-operative
image guided needle biopsy for 18. The histologically confirmed diagnosis
was enchondroma for two (3.7%), low-grade chondrosarcoma for 49
(92.6%) and high-grade chondrosarcoma for two (3.7%). Three patients
with a low-grade tumour developed a local recurrence at a mean of 15
months (12 to 17) post-operatively. A single high-grade recurrence
(grade II) was treated with tibial diaphyseal replacement. The overall
recurrence rate was 7.5% at a mean follow-up of 4.7 years (1.2 to
12.3). Cartilaginous tumours identified as low-grade on pre-operative
imaging with or without additional image-guided needle biopsy can
safely be managed as low-grade without pre-operative histological
diagnosis. A few tumours may demonstrate high-grade features histologically,
but the rates of recurrence are not affected. Cite this article:
We investigated whether the presence of a pathological
fracture increased the risk of local recurrence in patients with
a giant cell tumour (GCT) of bone. We also assessed if curettage
is still an appropriate form of treatment in the presence of a pathological
fracture. We conducted a comprehensive review and meta-analysis
of papers which reported outcomes in patients with a GCT with and
without a pathological fracture at presentation. We computed the
odds ratio (OR) of local recurrence in those with and without a
pathological fracture. We selected 19 eligible papers for final analysis. This included
3215 patients, of whom 580 (18.0%) had a pathological fracture.
The pooled OR for local recurrence between patients with and without
a pathological fracture was 1.05 (95% confidence interval (CI) 0.66
to 1.67, p = 0.854). Amongst the subgroup of patients who were treated with
curettage, the pooled OR for local recurrence was 1.23 (95% CI 0.75
to 2.01, p = 0.417). A There is no difference in local recurrence rates between patients
who have a GCT of bone with and without a pathological fracture
at the time of presentation. The presence of a pathological fracture
should not preclude the decision to perform curettage as carefully
selected patients who undergo curettage can have similar outcomes
in terms of local recurrence to those without such a fracture. Cite this article:
We retrospectively reviewed 30 patients with
a diffuse-type giant-cell tumour (Dt-GCT) (previously known as pigmented
villonodular synovitis) around the knee in order to assess the influence
of the type of surgery on the functional outcome and quality of
life (QOL). Between 1980 and 2001, 15 of these tumours had been
treated primarily at our tertiary referral centre and 15 had been
referred from elsewhere with recurrent lesions. The mean follow-up was 64 months (24 to 393). Functional outcome
and QOL were assessed with range of movement and the Knee injury
and Osteoarthritis Outcome Score (KOOS), the Musculoskeletal Tumour
Society (MSTS) score, the Toronto Extremity Salvage Score (TESS)
and the SF-36 questionnaire. There was recurrence in four of 14
patients treated initially by open synovectomy. Local control was
achieved after a second operation in 13 of 14 (93%). Recurrence
occurred in 15 of 16 patients treated initially by arthroscopic
synovectomy. These patients underwent a mean of 1.8 arthroscopies
(one to eight) before open synovectomy. This achieved local control
in 8 of 15 (53%) after the first synovectomy and in 12 of 15 (80%)
after two. The functional outcome and QOL of patients who had undergone
primary arthroscopic synovectomy and its attendant subsequent surgical
procedures were compared with those who had had a primary open synovectomy
using the following measures: range of movement (114º Those who had undergone open synovectomy needed fewer subsequent
operations. Most patients who had been referred with a recurrence
had undergone an initial arthroscopic synovectomy followed by multiple
further synovectomies. At the final follow-up of eight years (2
to 32), these patients had impaired function and QOL compared with
those who had undergone open synovectomy initially. We conclude that the natural history of Dt-GCT in patients who
are treated by arthroscopic synovectomy has an unfavourable outcome,
and that primary open synovectomy should be undertaken to prevent
recurrence or residual disease. Cite this article:
Clinical studies of patients with bone sarcomas have been challenged
by insufficient numbers at individual centres to draw valid conclusions.
Our objective was to assess the feasibility of conducting a definitive
multi-centre randomised controlled trial (RCT) to determine whether
a five-day regimen of post-operative antibiotics, in comparison
to a
24-hour regimen, decreases surgical site infections in patients
undergoing endoprosthetic reconstruction for lower extremity primary
bone tumours. We performed a pilot international multi-centre RCT. We used
central randomisation to conceal treatment allocation and sham antibiotics
to blind participants, surgeons, and data collectors. We determined
feasibility by measuring patient enrolment, completeness of follow-up,
and protocol deviations for the antibiotic regimens. Objective
Methods
Giant cell tumours (GCT) of the synovium and
tendon sheath can be classified into two forms: localised (giant
cell tumour of the tendon sheath, or nodular tenosynovitis) and
diffuse (diffuse-type giant cell tumour or pigmented villonodular
synovitis). The former principally affects the small joints. It
presents as a solitary slow-growing tumour with a characteristic
appearance on MRI and is treated by surgical excision. There is
a significant risk of multiple recurrences with aggressive diffuse
disease. A multidisciplinary approach with dedicated MRI, histological assessment
and planned surgery with either adjuvant radiotherapy or systemic
targeted therapy is required to improve outcomes in recurrent and
refractory diffuse-type GCT. Although arthroscopic synovectomy through several portals has
been advocated as an alternative to arthrotomy, there is a significant
risk of inadequate excision and recurrence, particularly in the
posterior compartment of the knee. For local disease partial arthroscopic
synovectomy may be sufficient, at the risk of recurrence. For both
local and diffuse intra-articular disease open surgery is advised
for recurrent disease. Marginal excision with focal disease will
suffice, not dissimilar to the treatment of GCT of tendon sheath.
For recurrent and extra-articular soft-tissue disease adjuvant therapy,
including intra-articular radioactive colloid or moderate-dose external
beam radiotherapy, should be considered.
We describe 22 cases of bizarre parosteal osteochondromatous proliferation, or Nora’s lesion. These are surface-based osteocartilaginous lesions typically affecting the hands and feet. All patients were identified from the records of a regional bone tumour unit and were treated between 1985 and 2009. Nine lesions involved the metacarpals, seven the metatarsals, one originated from a sesamoid bone of the foot and five from long bones (radius, ulna, tibia, and femur in two). The mean age of the patients was 31.8 years (6 to 66), with 14 men and eight women. Diagnosis was based on the radiological and histological features. The initial surgical treatment was excision in 21 cases and amputation of a toe in one. The mean follow-up was for 32 months (12 to 162). Recurrence occurred in six patients (27.3%), with a mean time to recurrence of 49 months (10 to 120). Two of the eight patients with complete resection margins developed a recurrence (25.0%), compared with four of 14 with a marginal or incomplete resection (28.6%). Given the potential surgical morbidity inherent in resection, our data suggest that there may be a role for a relatively tissue-conserving approach to the excision of these lesions.
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.
Pathological fractures in children can occur
as a result of a variety of conditions, ranging from metabolic diseases and
infection to tumours. Fractures through benign and malignant bone
tumours should be recognised and managed appropriately by the treating
orthopaedic surgeon. The most common benign bone tumours that cause pathological
fractures in children are unicameral bone cysts, aneurysmal bone
cysts, non-ossifying fibromas and fibrous dysplasia. Although pathological
fractures through a primary bone malignancy are rare, these should
be recognised quickly in order to achieve better outcomes. A thorough
history, physical examination and review of plain radiographs are
crucial to determine the cause and guide treatment. In most benign
cases the fracture will heal and the lesion can be addressed at
the time of the fracture, or after the fracture is healed. A step-wise
and multidisciplinary approach is necessary in caring for paediatric
patients with malignancies. Pathological fractures do not have to
be treated by amputation; these fractures can heal and limb salvage
can be performed when indicated.
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.
We have analysed the pattern of symptoms in patients presenting with synovial sarcoma to identify factors which led to long delays in diagnosis. In 35 children, the early symptoms and the results of clinical and radiological investigation were reviewed, along with the presumed diagnoses. The duration of symptoms was separated into patient delay and doctor delay. Only half of the patients had one or more of the four clinical findings suggestive of sarcoma according to the guidance of the National Institute for Clinical Excellence at the onset of symptoms. Of the 33 children for whom data were available, 16 (48.5%) presented with a painless mass and in ten (30.3%) no mass was identified. Seven (21.2%) had an unexplained joint contracture. Many had been extensively investigated unsuccessfully. The mean duration of symptoms was 98 weeks (2 to 364), the mean patient delay was 43 weeks (0 to 156) and the mean doctor delay was 50 weeks (0 to 362). The mean number of doctors seen before referral was three (1 to 6) and for 15 patients the diagnosis was obtained after unplanned excision. Tumours around the knee and elbow were associated with a longer duration of symptoms and longer doctor delay compared with those at other sites. Delays did not improve significantly over the period of our study of 21 years, and we were unable to show that delay in diagnosis led to a worse prognosis. Our findings highlight the variety of symptoms associated with synovial sarcoma and encourage greater awareness of this tumour as a potential diagnosis in childhood.
Schwannomas are the most common tumours of the sheath of peripheral nerves. The clinical diagnosis is usually straightforward, but may be delayed for many years in a schwannoma of the posterior tibial nerve. The symptoms are often attributed to entrapment neuropathy or to lumbosacral radiculopathy. We describe 25 patients with a schwannoma of the posterior tibial nerve. Only three were diagnosed within a year of presentation. The mean time to diagnosis was 86.5 months with a median of 48 months (2 to 360). All the patients complained of pain, which was felt specifically in the sole of the foot in 18. A Tinel sign was detected in all 25 patients. MRI confirmed the diagnosis in all the cases in which it had been undertaken. Surgical resection of the lesion abolished the neuropathic pain. In patients with a long history of neuropathic pain in the lower limb in whom lumbar and pelvic lesions have been excluded, a benign tumour of the sheath of a peripheral nerve may explain the symptoms. Surgical resection of the tumour is safe and effective.
Bone loss secondary to primary or metastatic lesions of the proximal humerus remains a challenging surgical problem. Options include preservation of the joint with stabilisation using internal fixation or resection of the tumour with prosthetic replacement. Resection of the proximal humerus often includes the greater tuberosity and adjacent diaphysis, which may result in poor function secondary to loss of the rotator cuff and/or deltoid function. Preservation of the joint with internal fixation may reduce the time in hospital and peri-operative morbidity compared with joint replacement, and result in a better functional outcome. We included 32 patients with pathological fractures of the proximal humerus in this study. Functional and radiological assessments were performed. At a mean follow-up of 17.6 months (8 to 61) there was no radiological evidence of failure of fixation. The mean revised musculoskeletal Tumour Society functional score was 94.6% (86% to 99%). There was recurrent tumour requiring further surgery in four patients (12.5%). Of the 22 patients who were employed prior to presentation all returned to work without restrictions. The use of a locking plate combined with augmentation with cement extends the indications for salvage of the proximal humerus with good function in patients with pathological and impending pathological fractures.
In our database of 7935 patients referred for investigation of a soft-tissue mass, only 100 were found to have a soft-tissue metastasis (1.3%). Our aim was to define the clinical features of such patients and to identify the site of their primary tumour. The most common presentation was a painful lump, deep to the fascia, ranging between 2 cm and 35 cm (mean 8.3 cm) with 78% of the lumps located deep to the fascia. The mean age of the patients at presentation was 64 years (22 to 84) and there were almost equal numbers of men and women. Of 53 patients with a history of malignancy, 52 had metastases from the same primary (lung in 12, melanoma in ten, kidney in nine, gastrointestinal track in four, breast in five, bladder in four, and others in eight). The other 47 had no history of malignancy and the metastasis was the first presentation. The primary sites in these cases were the lung in 19, gastro-intestinal track in four, kidney in two, melanoma in nine, other in three, and unknown (despite investigations) in ten. There was no correlation between the site of the metastases and the primary tumour. Of the 7935 patients, 516 had a history of malignancy. Of these, only 10% had a soft-tissue metastasis, 29% had a benign diagnosis, 55% a soft-tissue sarcoma and 6% another malignancy. Patients with soft-tissue metastases have similar clinical features to those with soft-tissue sarcomas and should be considered for assessment at appropriate diagnostic centres for patients with suspicious soft-tissue lumps.
The purpose of this study was to assess the outcome
of 15 patients (mean age 13.6 years (7 to 25)) with a primary sarcoma
of the tibial diaphysis who had undergone excision of the affected
segment that was then irradiated (90 Gy) and reimplanted with an
ipsilateral vascularised fibular graft within it. The mean follow-up was 57 months (22 to 99). The mean time to
full weight-bearing was 23 weeks (9 to 57) and to complete radiological
union 42.1 weeks (33 to 55). Of the 15 patients, seven required
a further operation, four to obtain skin cover. The mean Musculoskeletal
Society Tumor Society functional score at final follow-up was 27
out of 30 once union was complete. The functional results were comparable
with those of allograft reconstruction and had a similar rate of
complication. We believe this to be a satisfactory method of biological reconstruction
of the tibial diaphysis in selected patients.
Between 1986 and 2002, 42 patients with synchronous multifocal osteosarcoma were treated with two different protocols of neoadjuvant chemotherapy. When feasible, the primary and secondary tumours were excised as a combined procedure. After initial chemotherapy 26 patients were excluded from simultaneous excision of all their secondary bone lesions as their disease was too advanced. In 12 patients only isolated excision of the primary lesion was possible. For 16 patients simultaneous operations were conducted to excise the primary and secondary lesions. This involved two supplementary sites in 15 patients and four additional sites in one patient. Of these, 15 attained remission but 12 relapsed and died (11 within two years). Three patients remained disease-free at five, six and 17 years. The histological response to pre-operative chemotherapy of the primary and secondary lesions was concordant in 13 of the 16 patients who underwent simultaneous operations at more than one site. The prognosis for synchronous multifocal osteosarcoma remains poor despite combined chemotherapy and surgery. The homogeneous histological responses in a large proportion of the primary and secondary lesions implies that synchronous multifocal osteosarcoma tumours are not multicentric in origin, but probably represent bone-to-bone metastases from a single tumour.
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.
We have reviewed five adult patients treated with endoprosthetic reconstruction of the proximal radius following resection of non-traumatic lesions. The patients had a mean age of 33.4 years (20 to 60) at the time of surgery and the mean follow-up was 7.6 years (0.8 to 16). Following surgery, all elbows were clinically stable and there was 100% survivorship of the prosthesis. Evaluation of function was assessed clinically and by the Mayo Elbow Performance Score, achieving a mean of 86% (70 to 100). Results at medium-term follow-up are encouraging with regards to elbow stability, implant survivorship and functional outcome.
We evaluated the long-term outcome of patients with an osteosarcoma who had undergone prior manipulative therapy, a popular treatment in Asia, and investigated its effects on several prognostic factors. Of the 134 patients in this study, 70 (52%) patients had manipulative therapy and 64 (48%) did not. The age, location, and size of tumour were not significantly different between the groups. The five-year overall survival rate was 58% and 92% in the groups with and without manipulative therapy (p = 0.004). Both the primary and overall rates of lung metastasis were significantly higher in the manipulative group (primary: 32% This form of therapy may serve as a mechanism to accelerate the spread of tumour cells, and therefore must be avoided in order to improve the outcome for patients with an osteosarcoma.
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.
Deep-seated lipomas are often atypical histologically and are considered by some to have a high risk of recurrence after excision. We reviewed 215 deep-seated lipomas of the extremities and trunk wall with reference to histology, cytogenetics, clinical features and local recurrence. We classified tumours with atypical features and/or ring chromosomes as atypical lipomas. These were more common in men, larger than ordinary lipomas and more often located in the upper leg. The annual incidence was estimated as ten per million inhabitants and the ratio of atypical to ordinary lipomas was 1:3. In total, six tumours (3%), recurred locally after a median of eight years (1 to 16); of these, four were classified as atypical. The low recurrence rate of deep-seated lipomas of the extremity or trunk wall, irrespective of histological subtype, implies that if surgery is indicated, the tumour may be shelled out, that atypical lipomas in these locations do not deserve the designation well-differentiated liposarcoma, and that routine review after surgery is not required.
We have investigated the oncological outcome of 63 patients with soft-tissue sarcomas of the hand managed at three major centres in the United Kingdom. There were 44 males and 19 females with a mean age of 45 years (11 to 92). The three most common diagnoses were synovial sarcoma, clear cell sarcoma and epithelioid sarcoma. Local excision was carried out in 45 patients (71%) and amputation in 18 (29%). All those treated by amputation had a wide margin of excision but this was only achieved in 58% of those treated by local excision. The risk of local recurrence was 6% in those treated by amputation compared with 42% for those who underwent attempted limb salvage. An inadequate margin of excision resulted in a 12 times greater risk of local recurrence when compared with those in whom a wide margin of excision had been achieved. We were unable to demonstrate any role for radiotherapy in decreasing the risk of local recurrence when there was an inadequate margin of excision. Patients with an inadequate margin of excision had a much higher risk of both local recurrence and metastasis than those with wide margins. The overall survival rate at five years was 87% and was related to the grade and size of the tumour and to the surgical margin. We have shown that a clear margin of excision is essential to achieve local control of a soft-tissue sarcoma in the hand and that failure to achieve this results in a high risk of both local recurrence and metastastic disease.
Between 1996 and 2003, 16 patients (nine female, seven male) were treated for a primary bone sarcoma of the femur by wide local excision of the tumour, extracorporeal irradiation and re-implantation. An additional vascularised fibular graft was used in 13 patients (81%). All patients were free from disease when reviewed at a minimum of two years postoperatively (mean 49.7 months (24 to 96). There were no cases of infection. Primary union was achieved after a median of nine months (interquartile range 7 to 11). Five host-donor junctions (16%) united only after a second procedure. Primary union recurred faster at metaphyseal junctions (94% (15) at a median of 7.5 months (interquartile range 4 to 12)) than at diaphyseal junctions (75% (12) at a median of 11.1 months (interquartile range 5 to 18)). Post-operatively, the median Musculoskeletal Tumour Society score was 85% (interquartile range 75 to 96) and the median Toronto Extremity Salvage score 94% (interquartile range 82 to 99). The Mankin score gave a good or excellent result in 14 patients (88%). The range of movement of the knee was significantly worse when the extracorporeally irradiated autografts were fixed by plates rather than by nails (p = 0.035). A total of 16 (62%) of the junctions of the vascularised fibular grafts underwent hypertrophy, indicating union and loading. Extracorporeal irradiation autografting with supplementary vascularised fibular grafting is a promising biological alternative for intercalary reconstruction after wide resection of malignant bone tumours of the femur.
We retrospectively studied local recurrence of giant cell tumour in long bones following treatment with curettage and cementing in 137 patients. The median follow-up time was 60 months (3 to 166). A total of 19 patients (14%) had at least one local recurrence, the first was diagnosed at a median of 17 months (3 to 29) after treatment of the primary tumour. There were 13 patients with a total of 15 local recurrences who were successfully treated by further curettage and cementing. Two patients with a second local recurrence were consequently treated twice. At the last follow-up, at a median of 53 months (3 to 128) after the most recent operation, all patients were free from disease and had good function. We concluded that local recurrence of giant cell tumour after curettage and cementing in long bones can generally be successfully treated with further curettage and cementing, with only a minor risk of increased morbidity. This suggests that more extensive surgery for the primary tumour in an attempt to obtain wide margins is not the method of choice, since it leaves the patient with higher morbidity with no significant gain with respect to cure of the disease.