Aims. Treatment of high-grade limb bone sarcoma that invades a joint requires en bloc extra-articular excision. MRI can demonstrate joint invasion but is frequently inconclusive, and its predictive value is unknown. We evaluated the diagnostic accuracy of direct and indirect radiological signs of intra-articular tumour extension and the performance characteristics of MRI findings of intra-articular tumour extension. Methods. We performed a retrospective case-control study of patients who underwent extra-articular excision for sarcoma of the knee, hip, or
We retrospectively reviewed the outcomes of 33
consecutive patients who had undergone an extra-articular, total or
partial scapulectomy for a malignant tumour of the
Aims. The scapula is a rare site for a primary bone tumour. Only a small number of series have studied patient outcomes after treatment. Previous studies have shown a high rate of recurrence, with functional outcomes determined by the preservation of the glenohumeral joint and deltoid. The purpose of the current study was to report the outcome of patients who had undergone tumour resection that included the scapula. Methods. We reviewed 61 patients (37 male, 24 female; mean age 42 years (SD 19)) who had undergone resection of the scapula. The most common resection was type 2 (n = 34) according to the Tikhoff-Linberg classification, or type S1A (n = 35) on the Enneking classification. Results. The ten-year disease-specific survival was 76%. High tumour grade (hazard ratio (HR) 4.27; p = 0.016) and a total resection of the scapula (HR 3.84; p = 0.015) were associated with worse survival. The ten-year metastasis-free and local recurrence-free survivals were 82% and 86%, respectively. Total scapular resection (HR 6.29; p = 0.004) was associated with metastatic disease and positive margins were associated with local recurrence (HR 12.86; p = 0.001). At final follow-up, the mean
Aims. Limb salvage in bone tumour patients replaces the bone with massive segmental prostheses where achieving bone integration at the
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
Endoprosthetic reconstruction following distal femur tumour resection has been widely advocated. In this paper, we present the design of an uncemented endoprosthesis system featuring a short, curved stem, with the goal of enhancing long-term survivorship and functional outcomes. This study involved patients who underwent implantation of an uncemented distal femoral endoprosthesis with a short and curved stem between 2014 and 2019. Functional outcomes were assessed using the 1993 version of the Musculoskeletal Tumour Society (MSTS-93) score. Additionally, we quantified five types of complications and assessed osseointegration radiologically. The survivorship of the endoprosthesis was evaluated according to two endpoints. A total of 134 patients with a median age of 26 years (IQR 16 to 41) were included in our study. The median follow-up time was 61 months (IQR 56 to 76), and the median functional MSTS-93 was 83% (IQR 73 to 91) postoperatively.Aims
Methods
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
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
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
Patients with soft-tissue sarcoma (STS) who undergo unplanned excision (UE) are reported to have worse outcomes than those who undergo planned excision (PE). However, others have reported that patients who undergo UE may have similar or improved outcomes. These discrepancies are likely to be due to differences in characteristics between the two groups of patients. The aim of the study is to compare patients who underwent UE and PE using propensity score matching, by analyzing data from the Japanese Bone and Soft Tissue Tumor (BSTT) registry. Data from 2006 to 2016 was obtained from the BSTT registry. Only patients with STS of the limb were included in the study. Patients with distant metastasis at the initial presentation and patients with dermatofibrosarcoma protuberans and well-differentiated liposarcoma were excluded from the study.Aims
Methods
We retrospectively studied 14 patients with proximal and diaphyseal tumours and disappearing bone (Gorham’s) disease of the humerus treated with wide resection and reconstruction using an allograft-resurfacing composite (ARC). There were ten women and four men, with a mean age of 35 years (8 to 69). At a mean follow-up of 25 months (10 to 89), two patients had a fracture of the allograft. In one of these it was revised with a similar ARC and in the other with an intercalary prosthesis. A further patient had an infection and a fracture of the allograft that was revised with a megaprosthesis. In all patients with an ARC, healing of the ARC-host bone interface was observed. One patient had failure of the locking mechanism of the total elbow replacement. The mean post-operative Musculoskeletal Tumor Society score for the upper extremity was 77% (46.7% to 86.7%), which represents good and excellent results; one patient had a poor result (46.7%). In the short term ARC effectively relieves pain and restores
The purpose of this study was to report the long-term results of extendable endoprostheses of the humerus in children after the resection of a bone sarcoma. A total of 35 consecutive patients treated with extendable endoprosthetic replacement of the humerus in children were included. There were 17 boys and 18 girls in the series with a median age at the time of initial surgery of nine years (interquartile range (IQR) 7 to 11).Aims
Methods
We evaluated the construct validity of the Musculoskeletal Tumour Society rating scale (Enneking score) as a functional measure for patients with sarcoma involving the upper limb. We compared the Enneking score by examining the correlation between two patient-derived outcome measures, the Disability of the Arm,
The aim of this study was to report the results of custom-made endoprostheses with extracortical plates plus or minus a short, intramedullary stem aimed at preserving the physis after resection of bone sarcomas in children. Between 2007 and 2017, 18 children aged less than 16 years old who underwent resection of bone sarcomas, leaving ≤ 5 cm of bone from the physis, and reconstruction with a custom-made endoprosthesis were reviewed. Median follow-up was 67 months (interquartile range 45 to 91). The tumours were located in the femur in 11 patients, proximal humerus in six, and proximal tibia in one.Aims
Patients and Methods
The primary aim of this study was to determine the morbidity
of a tibial strut autograft and characterize the rate of bony union
following its use. We retrospectively assessed a series of 104 patients from a single
centre who were treated with a tibial strut autograft of > 5 cm
in length. A total of 30 had a segmental reconstruction with continuity
of bone, 27 had a segmental reconstruction without continuity of
bone, 29 had an arthrodesis and 18 had a nonunion. Donor-site morbidity
was defined as any event that required a modification of the postoperative
management. Union was assessed clinically and radiologically at
a median of 36 months (IQR, 14 to 74).Aims
Patients and Methods
The aim of this study was to assess the current trends in the estimation of survival and the preferred forms of treatment of pathological fractures among national and international general and oncological orthopaedic surgeons, and to explore whether improvements in the management of these patients could be identified in this way. All members of the Dutch Orthopaedic Society (DOS) and the European Musculoskeletal Oncology Society (EMSOS) were invited to complete a web-based questionnaire containing 12 cases.Aims
Materials and Methods
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
A prospective single-cohort study was designed to include 20 patients with enchondromas but was stopped because of poor early results. Four patients with an enchondroma, three in the proximal humerus and one in the distal femur, were treated by curettage and filling of the defect with Norian SRS cement. Clinical and radiological follow-up including CT and MRI was carried out for 18 months. All three patients with lesions in the proximal humerus had severe pain and limited movement of the
We studied 100 patients who had undergone endoprosthetic replacement of the proximal humerus between 1976 and 1998. The outcome was assessed with regard to the survivorship of the patients, the salvaged limbs and the prostheses. Function was determined in the 47 surviving patients, of whom 30 were assessed using the Musculo-Skeletal Tumour Society (MSTS) rating scale and 38 completed the Toronto Extremity Salvage Score (TESS) questionnaire. The median age of the patients was 34 years (10 to 80). The mean follow-up period for surviving patients was nine years (2 to 20). The mean MSTS score at follow-up was 79% and the mean TESS score was 72%. The length of bone which was resected influenced the functional outcome. Abduction of the
The aims of this study were to analyse the long-term outcome
of vascularised fibular graft (VFG) reconstruction after tumour
resection and to evaluate the usefulness of the method. We retrospectively reviewed 49 patients who had undergone resection
of a sarcoma and reconstruction using a VFG between 1988 and 2015.
Their mean follow-up was 98 months (5 to 317). Reconstruction was
with an osteochondral graft (n = 13), intercalary graft (n = 12),
inlay graft (n = 4), or resection arthrodesis (n = 20). We analysed
the oncological and functional outcome, and the rate of bony union
and complications.Aims
Patients and Methods
Massive endoprostheses rely on extra-cortical bone bridging (ECBB)
to enhance fixation. The aim of this study was to investigate the
role of selective laser sintered (SLS) porous collars in augmenting
the osseointegration of these prostheses. The two novel designs of porous SLS collars, one with small pores
(Ø700 μm, SP) and one with large pores (Ø1500 μm, LP), were compared
in an ovine tibial diaphyseal model. Osseointegration of these collars
was compared with that of a clinically used solid, grooved design
(G). At six months post-operatively, the ovine tibias were retrieved and
underwent radiological and histological analysis.Aims
Materials and Methods
We present a retrospective review of patients treated with extracorporeally
irradiated allografts for primary and secondary bone tumours with
the mid- and long-term survivorship and the functional and radiographic
outcomes. A total of 113 of 116 (97.4%) patients who were treated with
extracorporeally irradiated allografts between 1996 and 2014 were
followed up. Forms of treatment included reconstructions, prostheses
and composite reconstructions, both with and without vascularised
grafts. Survivorship was determined by the Kaplan-Meier method.
Clinical outcomes were assessed using the Musculoskeletal Tumor
Society (MSTS) scoring system, the Toronto Extremity Salvage Score
(TESS) and Quality of Life-C30 (QLQ-30) measures. Radiographic outcomes
were assessed using the International Society of Limb Salvage (ISOLS)
radiographic scoring system.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
To assess complications and failure mechanisms of osteoarticular
allograft reconstructions for primary bone tumours. We retrospectively evaluated 38 patients (28 men, 74%) who were
treated at our institution with osteoarticular allograft reconstruction
between 1989 and 2010. Median age was 19 years (interquartile range
14 to 32). Median follow-up was 19.5 years (95% confidence interval
(CI) 13.0 to 26.1) when 26 patients (68%) were alive. In addition, we
systematically searched the literature for clinical studies on osteoarticular
allografts, finding 31 studies suitable for analysis. Results of
papers that reported on one site exclusively were pooled for comparison.Aims
Patients and Methods
We investigated the functional outcome in patients
who underwent reverse shoulder replacement (RSR) after removal of
a tumour of the proximal humerus. A total of 16 patients (ten women
and six men) underwent this procedure between 1998 and 2011 in our
hospital. Five patients died and one was lost to follow-up. Ten
patients were available for review at a mean follow-up of 46 months
(12 to 136). Eight patients had a primary and two patients a secondary
bone tumour. At final follow up the mean range of active movement was: abduction
78° (30° to 150°); flexion 98° (45° to 180°); external rotation
32° (10° to 60°); internal rotation 51° (10° to 80°). The mean Musculoskeletal
Tumor Society score was 77% (60% to 90%) and the mean Toronto Extremity
Salvage Score was 70% (30% to 91%). Two patients had a superficial
infection and one had a deep infection and underwent a two-stage
revision procedure. In two patients there was loosening of the RSR;
one dislocated twice. All patients had some degree of atrophy or
pseudo-atrophy of the deltoid muscle. Use of a RSR in patients with a tumour of the proximal humerus
gives acceptable results. Cite this article:
The purpose of this study was to develop a prognostic model for
predicting survival of patients undergoing surgery owing to metastatic
bone disease (MBD) in the appendicular skeleton. We included a historical cohort of 130 consecutive patients (mean
age 64 years, 30 to 85; 76 females/54 males) who underwent joint
arthroplasty surgery (140 procedures) owing to MBD in the appendicular
skeleton during the period between January 2003 and December 2008.
Primary cancer, pre-operative haemoglobin, fracture Aims
Methods
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:
In patients with a tumour affecting the distal
ulna it is difficult to preserve the function of the wrist following extensive
local resection. We report the outcome of 12 patients (nine female,
three male) who underwent excision of the distal ulna without local
soft-tissue reconstruction. In six patients, an aggressive benign
tumour was present and six had a malignant tumour. At a mean follow-up
of 64 months (15 to 132) the mean Musculoskeletal Tumour score was
64% (40% to 93%) and the mean DASH score was 35 (10 to 80). The
radiological appearances were satisfactory in most patients. Local
recurrence occurred in one patient with benign disease and two with
malignant disease. The functional outcome was thus satisfactory
at a mean follow-up in excess of five years, with a relatively low
rate of complications. The authors conclude that complex reconstructive
soft-tissue procedures may not be needed in these patients. Cite this article:
The aim of this study was to determine whether
the high-sensitivity modified Glasgow prognostic score (Hs-mGPS) could
predict the disease-specific survival and oncological outcome in
adult patients with non-metastatic soft-tissue sarcoma before treatment.
A total of 139 patients treated between 2001 and 2012 were retrospectively reviewed.
The Hs-mGPS varied between 0 and 2. Patients with a score of 2 had
a poorer disease-specific survival than patients with a score of
0 (p <
0.001). The estimated five-year rate of disease-specific
survival for those with a score of 2 was 0%, compared with 85.4%
(95% CI 77.3 to 93.5) for those with a score of 0. Those with a
score of 2 also had a poorer disease-specific survival than those
with a score of 1 (75.3%, 95% CI 55.8 to 94.8; p <
0.001). Patients
with a score of 2 also had a poorer event-free rate than those with
a score of 0 (p <
0.001). Those with a score of 2 also had a
poorer event-free survival than did those with a score of 1 (p =
0.03). A multivariate analysis showed that the Hs-mGPS remained
an independent predictor of survival and recurrence. The Hs-mGPS
could be a useful prognostic marker in patients with a soft-tissue
sarcoma. Cite this article:
Rarely, the extent of a malignant bone tumour
may necessitate resection of the complete humerus to achieve adequate
oncological clearance. We present our experience with reconstruction
in such cases using a total humeral endoprosthesis (THER) in 20
patients (12 male and eight female) with a mean age of 22 years
(6 to 59). We assessed the complications, the oncological and functional
outcomes and implant survival. Surgery was performed between June
2001 and October 2009. The diagnosis included osteosarcoma in nine,
Ewing’s sarcoma in eight and chondrosarcoma in three. One patient
was lost to follow-up. The mean follow-up was 41 months (10 to 120)
for all patients and 56 months (25 to 120) in survivors. There were
five local recurrences (26.3%) and 11 patients were alive at time
of last follow-up, with overall survival for all patients being
52% (95% confidence interval (CI) 23.8 to 74) at five years. The
mean Musculoskeletal Tumor Society score for the survivors was 22
(73%; 16 to 23). The implant survival was 95% (95% CI 69.5 to 99.3)
at five years. The use of a THER in the treatment of malignant tumours of bone
is oncologically safe; it gives consistent and predictable results
with low rates of complication.
Between 1997 and 2007, 68 consecutive patients underwent replacement of the proximal humerus for tumour using a fixed-fulcrum massive endoprosthesis. Their mean age was 46 years (7 to 87). Ten patients were lost to follow-up and 16 patients died. The 42 surviving patients were assessed using the Musculoskeletal Tumor Society (MSTS) Score and the Toronto Extremity Salvage Score (TESS) at a mean follow-up of five years and 11 months (one year to ten years and nine months). The mean MSTS score was 72.3% (53.3% to 100%) and the mean TESS was 77.2% (58.6% to 100%). Four of 42 patients received a new constrained humeral liner to reduce the risk of dislocation. This subgroup had a mean MSTS score of 77.7% and a mean TESS of 80.0%. The dislocation rate for the original prosthesis was 25.9; none of the patients with the new liner had a dislocation at a mean of 14.5 months (12 to 18). Endoprosthetic replacement for tumours of the proximal humerus using this prosthesis is a reliable operation yielding good results without the documented problems of unconstrained prostheses. The performance of this prosthesis is expected to improve further with a new constrained humeral liner, which reduces the risk of dislocation.
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:
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.
Pathological fractures of the humerus are associated with pain, morbidity, loss of function and a diminished quality of life. We report our experience of stabilising these fractures using polymethylmethacrylate and non-locking plates. We undertook a retrospective review over 20 years of patients treated at a tertiary musculoskeletal oncology centre. Those who had undergone surgery for an impending or completed pathological humeral fracture with a diagnosis of metastatic disease or myeloma were identified from our database. There were 63 patients (43 men, 20 women) in the series with a mean age of 63 years (39 to 87). All had undergone intralesional curettage of the tumour followed by fixation with intramedullary polymethylmethacrylate and plating. Complications occurred in 14 patients (22.2%) and seven (11.1%) required re-operation. At the latest follow-up, 47 patients (74.6%) were deceased and 16 (25.4%) were living with a mean follow-up of 75 months (1 to 184). A total of 54 (86%) patients had no or mild pain and 50 (80%) required no or minimal assistance with activities of daily living. Of the 16 living patients none had pain and all could perform activities of daily living without assistance. Intralesional resection of the tumour, filling of the cavity with cement, and plate stabilisation of the pathological fracture gives immediate rigidity and allows an early return of function without the need for bony union. The patient’s local disease burden is reduced, which may alleviate tumour-related pain and slow the progression of the disease. The cemented-plate technique provides a reliable option for the treatment of pathological fractures of the humerus.
Between June 2005 and March 2008, 14 patients with a Campanacci grade-3 giant-cell tumour of the distal radius were treated by en bloc resection and reconstruction by ulnar translocation with arthrodesis of the wrist. The mean length of radius resected was 7.9 cm (5.5 to 15). All the patients were followed to bony union and 12 were available at a mean follow-up of 26 months (10 to 49). The mean time to union was four months (3 to 7) at the ulnocarpal junction and five months (3 to 8) at the ulnoradial junction. All except one patient had an excellent range of pronation and supination. The remaining patient developed a radio-ulnar synostosis. The mean Musculoskeletal Tumor Society score was 26 (87%, range 20 to 28). Three patients had a soft-tissue recurrence, but with no bony involvement. They underwent a further excision and are currently well and free from disease. Ulnar translocation provides a local vascularised bone graft to reconstruct the defect left after excision of the distal radius for giant cell tumour. It avoids the need for a microvascular procedure while retaining rotation of the forearm and good function of the hand.
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.
In skeletally immature patients, resection of
bone tumours and reconstruction of the lower limb often results
in leg-length discrepancy. The Stanmore non-invasive extendible
endoprosthesis, which uses electromagnetic induction, allows post-operative
lengthening without anaesthesia. Between 2002 and 2009, 55 children
with a mean age of 11.4 years (5 to 16) underwent reconstruction
with this prosthesis; ten patients (18.2%) died of disseminated
disease and one child underwent amputation due to infection. We
reviewed 44 patients after a mean follow-up of 41.2 months (22 to
104). The mean Musculoskeletal Tumor Society score was 24.7 (8 to
30) and the Toronto Extremity Salvage score was 92.3% (55.2% to
99.0%). There was no local recurrence of tumour. Complications developed
in 16 patients (29.1%) and ten (18.2%) underwent revision. The mean length gained per patient was 38.6 mm (3.5 to 161.5),
requiring a mean of 11.3 extensions (1 to 40), and ten component
exchanges were performed in nine patients (16.4%) after attaining
the maximum lengthening capacity of the implant. There were 11 patients
(20%) who were skeletally mature at follow-up, ten of whom had equal
leg lengths and nine had a full range of movement of the hip and
knee. This is the largest reported series using non-invasive extendible
endoprostheses after excision of primary bone tumours in skeletally
immature patients. The technique produces a good functional outcome,
with prevention of limb-length discrepancy at skeletal maturity.
We treated 50 patients with bony malignancy by The mean survivor follow-up was 38 months (12 to 92) when 42 patients were alive and without disease. There were four recurrences. The functional results were good according to the Mankin score (17 excellent, 13 good, nine fair, three failures), the Musculoskeletal Tumour Society score (mean 77) and the Toronto Extremity Salvage score (mean 81). There was solid union, but bone resorption was seen in some cases. The dose of radiation was lethal to all cells and produced a dead autograft of perfect fit. Extracorporeal irradiation is a useful technique for limb salvage when there is reasonable residual bone stock. It allows effective re-attachment of tendons and produces a lasting biological reconstruction. There should be no risk of local recurrence or of radiotherapy-induced malignancy in the replanted bone.
The use of allografts for the treatment of bone tumours in children is limited by nonunion and the difficulty of finding a suitable graft. Furthermore, appositional growth can’t be expected of an allograft. We used an overlapping allograft in 11 children, with a mean age of ten years (4 to 15), with a mean follow-up of 24.1 months (20 to 33). There were five intercalary and six intra-articular resections, and the tumours were in the femur in six cases and the humerus in five. Rates of union, times to union, remodelling patterns and allograft-associated complications were evaluated. No allograft was removed due to a complication. Of the 16 junctional sites, 15 (94%) showed union at a mean of 3.1 months (2 to 5). Remodelling between host and allograft was seen at 14 junctions at a mean of five months (4 to 7). The mean Musculoskeletal Tumor Society score was 26.5 of 30 (88.3%). One case of nonunion and another with screw protrusion required re-operation. Overlapping allografts have the potential to shorten time to union, decrease rates of nonunion and have positive appositional growth effect.
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.
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.
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 have reviewed the data from our regional Bone Tumour Registry on patients with osteosarcoma diagnosed between 1933 and 2004 in order to investigate the relationship between survival and changes in treatment. There were 184 patients with non-metastatic appendicular osteosarcoma diagnosed at the age of 18 or under. Survival was calculated using Kaplan-Meier curves, and multivariate analysis was performed using the Cox regression proportional hazards model. The five-year survival improved from 21% between 1933 and 1959, to 62% between 1990 and 1999. During this time, a multi-disciplinary organisation was gradually developed to manage treatment. The most significant variable affecting outcome was the date of diagnosis, with trends in improved survival mirroring the introduction of increasingly effective chemotherapy. Our experience suggests that the guidelines of the National Institute for Clinical Excellence on the minimum throughput of centres for treatment should be enforced flexibly in those that can demonstrate that their historical and contemporary results are comparable to those published nationally and internationally.
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.
Between December 1995 and March 2003, 38 adult patients with intermediate or high-grade liposarcoma in a limb were treated by limb-sparing surgery and post-operative radiotherapy. The ten-year local recurrence-free survival was 83%, the ten-year metastasis-free survival 61%, the ten-year disease-free survival 51% and the ten-year overall survival 67%. Analysis of failure and success showed no association with the age of the patients, gender, the location of the primary tumour, the type of liposarcoma and the quality of resection. Our results indicate that liposarcoma may recur even ten years after the end of definitive therapy and may spread to unexpected sites as for soft-tissue sarcoma.
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.