Metastatic osteosarcoma is seen in 10-20% of patients at initial presentation with the lung the most common site of metastasis. Historically, prognosis has been poor. We studied trends in survival in our small developed nation and aimed to identify correlations between the survival rate and three factors: newer chemotherapy, advances in radiological imaging and a more aggressive approach adopted by cardiothoracic surgeons for lung metastases.

Our national bone tumour registry was used to identify patients at the age of 18 or under, who presented with metastatic disease at initial diagnosis between 1933 and 2006. There were 30 patients identified. Kaplan-Meier analysis was used to determine survival rates and univariate analysis was performed using the Cox regression proportional hazards model.

Median survival has improved over the last 50 years; highlighted by the ‘Kotz’ eras demonstrating incremental improvement with more effective chemotherapy agents (p=0.004), and a current 5-year survival of 16%. Aggressive primary and metastatic surgery also show improving trends in survival. Three patients have survived beyond five years. The introduction of computerised tomography scanning has led to an increase in the prevalence of metastases at initial diagnosis.

Metastatic osteosarcoma remains with a very poor prognostic factor, however, aggressive management has been shown to prolong survival.

Introduction

Spinal osteosarcomas are quite rare and the optimal treatment strategy is unknown. We report a series of 9 cases of osteosarcoma of the spine treated with intralesional resection and adjuvant combination therapy in order to evaluate their clinico-pathological correlation, recurrence rate and survival.

Metastatic osteosarcoma is seen in 10-20% of patients at initial presentation with the lung the most common site of metastasis. Historically, prognosis has been poor. We studied trends in survival in our small developed nation and aimed to identify correlations between the survival rate and three factors: newer chemotherapy, advances in radiological imaging and a more aggressive approach adopted by cardiothoracic surgeons for lung metastases.

Our national bone tumour registry was used to identify patients at the age of 18 or under, who presented with metastatic disease at initial diagnosis between 1933 and 2006. There were 30 patients identified. Kaplan-Meier analysis was used to determine survival rates and univariate analysis was performed using the Cox regression proportional hazards model.

Median survival has improved over the last 50 years; highlighted by the ‘Kotz’ eras demonstrating incremental improvement with more effective chemotherapy agents (p=0.004), and a current 5-year survival of 16%. Aggressive primary and metastatic surgery also show improving trends in survival. Three patients have survived beyond 5 years. The introduction of computerised tomography scanning has led to an increase in the prevalence of metastases at initial diagnosis.

Metastatic osteosarcoma remains with a very poor prognostic factor, however, aggressive management has been shown to prolong survival.

Introduction

Aneurysmal bone cysts are uncommon benign lesions affecting the spinal column. They mostly occur in the lumbar spine and have a propensity to affect adjacent vertebrae. We describe 14 aneurysmal bone cysts affecting the spinal column from the Scottish Bone Tumour Registry with regard to assess the incidence, demography, biological behaviour and recurrence rate.

Chordomas are slowly growing, locally aggressive primary malignant neoplasms derived from primitive notochordal cells. They tend to occur at the extreme of ages and at the two ends of the spinal column. The purpose of this study was to evaluate the presentation, morphology and behaviour of 20 non-clival, non-sacral chordoma cases.

Details of 20 cases of histologically confirmed Chordomas of the mobile spine (Cervical-7, Thoracic-7, Lumbar-6) between 1967 and 2006, were extracted from the Scottish Bone Tumour Registry. The casenotes and radiographs were retrospectively reviewed.

Non-sacral chordomas comprised 48.7% (20/41 cases) of total chordomas registered. There was a slight feminine predominance (11/20 cases). Mean age was 58.1 years (range, 12 to 82 years). Progressively worsening back pain was the first symptom in all the patients. Seven had intra-lesional (five recurred), six marginal (1 recurred) and 2 wide resections (none recurred). Eleven of the twenty-one patients were treated with adjuvant radiation therapy. In five patients, the chordoma was inoperable and all but one were treated with radiotherapy. There were 2 recurrences in the group of patients treated without adjuvant radiotherapy.

Six patients (40%) developed local recurrences and 3 patients (15%) developed metastases. There were 4 survivors including one with local recurrence. Sixteen patients died including 3 with metastases, 7 with persistent primary disease, 1 with local recurrence and 5 with no evidence of disease or died of unrelated causes. Median survival was 19 months (mean-30.2 months), with 5- and 10-year survival rates 10% and 0.0% respectively.

We found that non-sacral/non-clival chordomas had a much poorer prognosis than published in the literature. Local recurrences occurred in 40%. Intra-lesional resection should be avoided as it is associated with 71.4% local recurrence in our series.

Chondrosarcomas are uncommon primary malignant cartilaginous tumours, even less common in spine. Surgical excision is the only mode of successful treatment as these tumours are resistant to conventional chemotherapy and radiation therapy. We share our experience of 22 cases of chondrosarcomas of the spine with special reference to their recurrence and survival.

We identified 20 conventional and 2 dedifferentiated chondrosarcomas from the Scottish Bone Tumour Registry database between 1964 and 2009. Radiology and histopathology were documented. The mean follow-up was 5.2 years.

There were 14 men and 8 women with a mean age of 50.1 years. There were 7 under the age of 40 years (31.8%). The majority of lesions occurred in the thoracic spine (16), followed by sacrum (3), lumbar (2) and cervical spine (1). The overall local recurrence rate was 45.4% (10/22 cases-once in 5, twice in 2 and thrice in 3 patients).

Four patients presented with pulmonary metastases leading to death. The estimated overall 5- and 10-year survival rates were 31.8% and 18.1% respectively. We found that 1/3^rd of chondrosarcomas of the spine occured below 40 years of age and 3/4^th in the thoracic spine. Every other case was associated with local recurrence with a 32% 5-year and 18% 10-year survival rates.

Sacro-coccygeal chordomas pose a difficult diagnostic and therapeutic problem due to late presentation, large size, soft-tisue extension, difficulties in obtaining adequate resection margins, higher local recurrence rate and uncertain effectiveness of adjuvant treatment. We present a series of 21 patients of sacral chordomas obtained from Scottish Bone Tumour Registry to analyse predictors of local control and survival.

The clinical and morphologic features, type of treatment and follow-up of 21 consecutive patients with sacral chordoma were retrospectively reviewed and analysed. The data were obtained from Scottish Bone Tumour Registry.

The average age at time of the biopsy was 59 years (range, 12 to 82 years): twelve patients were male and nine were female. Pain was the presenting symptom in all patients. Two had intralesional (both recurred), 9 marginal (4 recurred) and 3 wide resections (1 recurred). Fifteen of the twenty-one patients were treated with adjuvant radiation therapy. In seven patients, the chordoma was inoperable and all but one were treated with adjuvant radiotherapy. Local recurrence and metastases occurred in 7 (50%) and 5 (23.8%) patients. The 5-year and 10-year survival were 38% and 14.2%, respectively.

Excision of the lesion combined with adjuvant radiation therapy provided satisfactory results. Local recurrence presents a major problem in the management of sacral chordomas (50%). Intralesional resection should be avoided as it is associated with 100% local recurrence in our series.

Introduction: Giant cell tumours are locally highly aggressive and extremely unpredictable bone tumours. Treatment of spinal GCTs remains controversial. We report our experience of 11 Giant cell tumours of the spine identified from the Scottish Bone Tumour Registry.

Materials and Methods: Details of 11 cases of histologically confirmed Giant cell tumours of the spine (9 benign and 2 malignant) between 1960 and 2004, were extracted from the Scottish Bone Tumour Registry. The casenotes and radiographs were retrospectively reviewed.

Results: There was a slight feminine predominance of 7 cases. Mean age was 34 years (range, 16 to 61 years). The sacrum (5) was most common location, followed by lumbar (3), thoracic (2) and rarely in cervical (1). Operative intervention was carried out in 5 (curettage-1; excision-5). Three also received supplemented bone grafts. Radiotherapy (including some of the operative cases) was administered in 9 patients. There were 5 recurrences (45.4%). There were 7 survivors 2 of whom still had evidence of persistent primary disease. Two died with unrelated illnesses and two from local recurrences.

Conclusions: Axial GCTs behave aggressively with a high recurrence rate (45%). Radiotherapy is useful in the management of GCTs of the spine and conservative surgery with local radiotherapy is a viable treatment option in some selected patients.

Introduction: A retrospective analysis was performed to determine the oncological outcome of patients with Ewing’s sarcoma of the spine treated with combined chemotherapy and radiotherapy for definitive local control.

Materials and Methods: Fifteen patients were identified from the Scottish Bone Tumour Registry with a histologically confirmed Ewing’s sarcoma affecting the axial skeleton. All case notes and imaging were retrospectively reviewed.

Results: Primary vertebral Ewing’s sarcoma accounted for 8.3% of all malignant spinal lesions in our registry. The mean age was 17.8 years (between 4 and 39 years). There was a male predilection with 9 male and 6 female patients. Site was evenly distributed between cervical (4), dorsal (5) and lumbosacral (6) regions. Progressively worsening back pain was the first symptom in all the patients. Satisfactory imaging studies were available in all with plain radiographs (15), bone scan (11), CT-scan (12) and MR Scan (9) patients.

Biopsy was performed in 11 patients and surgical treatment was carried out in 3 patients including curettage (2) and excision with bone grafting (1). All patients were treated with adjuvant radiotherapy while 87% also received adjuvant chemotherapy. Seven patients were alive with no evidence of disease at a mean 6 year follow-up. Six patients died of metastatic disease, one due to local recurrence and one with persistent primary disease. The mean follow-up time was 65 months (median 28 months; ranging from 12 to 218 months).

Conclusions: Primary vertebral Ewing’s sarcoma comprised 8.3% of our National Registry’s primary malignant spinal lesions. Progressive vertebral pain in the late second decade and male gender should raise the suspicion of Ewing’s sarcoma. Ewing’s sarcoma of the spine treated with combined chemotherapy and radiotherapy for definitive local control achieved a 45% five year survival.

Introduction: Benign bone-forming tumours are common in children and adolescents. Careful radiographical and histological study is necessary to distinguish slow growing from more aggressive bone forming tumours. We reviewed 25 cases of primary benign bone forming tumours of the spine to investigate whether there were any obvious differences in their biological behaviour in adults compared to children.

Materials and Methods: Twenty five cases of primary benign bone forming tumours of the spine were identified from the Scottish Bone Tumour Registry: this data is collected prospectively. A retrospective review of this data was performed. There were 9 osteoid osteomas, 15 osteoblastomas and 1 aggressive osteoblastoma. These cases were divided into group A (children) and group B (adults).

Results: There were 16 patients in group A (6-osteoid osteoma, 9-osteoblastoma, 1-aggressive osteoblastoma), 10 boys and 6 girls. The mean age was 12.1 years (range, 6–16 years). There were 2 cervical, 4 thoracic, 8 lumbar and 2 sacral tumours. There were 9 patients in Group B (3-osteoid osteoma, 6-osteoblastoma), 7 boys and 2 girls. The mean age was 26.6 years (range, 18–53 years). There were 1 cervical, 6 thoracic, 2 lumbar and none sacral tumours.

Twenty two tumours were excised and 3 had curettage performed (1 child and 2 adults). There were 2 recurrences (one osteoid osteoma, one osteoblastoma), one from the excision group and one who had curettage, both in adults. These were successfully treated with re-excision. Mean follow-up was 8 years and all were alive at the time of final follow-up.

Conclusions: Benign bone forming tumours of the spine are extremely uncommon. In children they occur more commonly in lumbar spine, while thoracic involvement predominates in adult patients. Good outcomes are obtained with surgical treatment. Recurrence occurred only in the adult group: both of these patients had successful outcomes following further treatment.

Introduction: Benign bone-forming tumours are common in children and adolescents. Careful radiographical and histological study is necessary to distinguish slow growing from more aggressive bone forming tumours. We reviewed 25 cases of primary benign bone forming tumours of the spine to investigate whether there were any obvious differences in the biological behaviour of such tumours in adults compared to children.

Materials and Methods: Twenty five cases of primary benign bone forming tumours of the spine were identified from the Scottish Bone Tumour Registry: this data is collected prospectively. A retrospective review of this data was performed. There were 9 osteoid osteomas,15 osteoblastomas and 1 aggressive osteoblastoma. These cases were divided into group A (children) and group B (adults).

Results: There were 16 patients in group A (6-osteoid osteoma, 9-osteoblastoma, 1-aggressive osteoblastoma), 10 boys and 6 girls. The mean age was 12.1 years (range, 6–16 years). There were 2 cervical, 4 thoracic, 8 lumbar and 2 sacral tumours. There were 9 patients in Group B (3-osteoid osteoma, 6-osteoblastoma), 7 boys and 2 girls. The mean age was 26.6 years (range, 18–53 years). There were 1 cervical, 6 thoracic, 2 lumbar and none sacral tumours.

Twenty two tumours were excised and 3 had curettage performed (1 child and 2 adults). There were 2 recurrences (one osteoid osteoma, one osteoblastoma), one from the excision group and one who had curettage, both in adults. These were successfully treated with re-excision. Mean follow-up was 8 years and all were alive at the time of final follow-up.

Conclusions: Benign bone forming tumours of the spine are extremely uncommon. In children they occur more commonly in lumbar spine, while thoracic involvement predominates in adult patients. Good outcomes are obtained with surgical treatment. Recurrence occurred only in the adult group: both of these patients had successful outcomes following further treatment.

Introduction: Only 4–13 % of all spine tumours are primary bone tumours. We report on 180 cases of primary malignant bone tumours of the spine from the Scottish Bone Tumour Registry. The aim of the study was to analyse the incidence, demography, pathology and survival patterns of primary malignant bone tumours of the vertebral column.

Materials and Methods: All of the data in the Scottish Bone Tumour Registry is compiled prospectively. This report is based on a retrospective review of all the data from 180 cases of primary malignant bone tumours (excluding metastatic lesions).

Results: Of 4,301 registry cases 4.1% were primary malignant lesions (n=180). Seventy two percent of all spinal tumours were primary malignant neoplasms. There were 22 cervical, 72 thoracic, 45 lumbar and 41 sacrococcygeal lesions. There was a male preponderance (103 males & 77 females). The mean age at presentation was 54 years (range, 4–86 years). The top two ranked tumours were myeloma (42) and chordoma (41). Ewing’ sarcoma (15), Leukaemik-Lymphomatous lesions (13), conventional osteosarcoma (10) and Paget’s sarcoma (9) followed thereafter.

The predominant presenting symptom was pain. Pathological fracture occurred in 7 patients. The operative treatment consisted of curettage (21), excision (51) and resection (9) with supplemented bone grafting (13). Adjuvant chemo (=61) and radiotherapy (=131) was also used in selective cases. Thirty patients were alive with no evidence of disease at a mean 5 year follow-up. Six were alive with persistent primary disease and/or local recurrence and/or metastases at the time of review. Eighty four patients died with persistent primary disease, 30 patients died of metastatic disease, 9 due to local recurrence and 17 of unrelated causes.

Conclusions: Only 4.1% of the musculoskeletal tumours were spinal: 40% involved the thoracic spine. Mean age at presentation was 54 years. Myeloma, chordoma, chondrosarcoma and Ewing’ sarcoma were the most common pathologies. Myeloma predominated: osteosarcoma was much less common in our series compared to previous reports. Early diagnoses resulted into improved outcome.

Intraosseous schwannoma is a rare benign neoplasm, which most commonly arises in the head and neck region particularly the mandible, due to the long intraosseous path of sensory nerves in the mandible. We present a 27-year-old lady with an unusual presentation of an intraosseous schwannoma of the first metatarsal. There is only one report published previously of an intraossous schwannoma of the lesser metatarsal bone of the foot.

A 27-year-old woman presented with painful left forefoot following a trip while walking. Plain radiographs demonstrated a pathological fracture through a lytic lesion of the first metatarsal of the left foot. MRI scan using axial T1-weighted spin echo and axial and sagittal T2-weighted gradient echo showed an amorphous mass occupying the medulla of the bone but with a breach of the plantar aspect of cortex with apparent localised destruction. Ultrasound-guided biopsy was performed. Haematoxylin and Eosin stained specimen sections showed a proliferation of spindle cells of alternating hypercellularity and hypocellularity. This case was managed by curettage and grafting with autograft and synthetic bone substitute. At two-year follow-up, the radiographs showed complete graft incorporation and a healed cyst. The patient was clinically asymptomatic with return of full functions. There were no clinico-radiological findings to suggest any recurrence.

Due to rarity and non-specific clinico-radiological features, this case illustrates the necessity of a multi-disciplinary approach with an accurate histological diagnosis in combination with radiological and clinical appearances.

Between 1948 and 2004, we report 34 patients with Ewing’s sarcoma of pelvis accrued from Scottish Bone Tumour Registry, aiming to identify the prognostic factors and the influence of various treatment modalities on outcome. There were 19 male and 15 female patients at a mean age of 19 years (range, 3 to 48 years). The Pain was main presenting symptom in 30, swelling in 12 and restriction of hip movements in 11 patients. The commonest anatomical site was ilium. Local control was achieved by surgery, radiotherapy (n=25), chemotherapy (n=23) or a combination. The survival correlated significantly with chemotherapy protocols in favour of the group that received ifosamide (p< 0.01). Metastases at presentation was the most important factor determining survival (P< 0.01). Among the patients who presented without metastases (n=25), there was no statistically significant difference in survival based on the anatomical location of the tumour, age or sex. The mean time to lung metastases from the date of presentation was 13 months, while bone metastases presented at an average of 20 months. None of the patients with the metastasis or local recurrence survived. There were 5 local and 17 systemic (metastatic) relapses. The mean duration of survival was 13 months.

With advances in imaging, aggressive chemotherapy, surgery and conformal radiotherapy which can deliver high dose of radiation with precision, it is possible to achieve a cure rate of more that 50% in non-metastatic pelvic Ewing’s sarcoma. The results of this study favour a middle-path regime combining all treatment modalities.

Purpose: The purpose of this study was to determine factors predictive of tumour recurrence, or refracture, following curettage as treatment for pathological fracture of the proximal humerus through a benign bone lesion.

Methods: From a cohort of patients held on a national database the factors predictive of recurrence following surgical curettage in patients with pathological fractures through benign bone tumours of the proximal humerus were examined. Thirty nine cases were identified. The diagnosis was simple bone cyst in 27 patients (69.2%), aneurysmal bone cyst in 4 patients (10.3%), (en)chondroma in 4 patients (10.3%), giant cell tumour in 2 patients (5.1%), benign chondroblastoma in 1 patient (2.6%) and fibroma in 1 patient (2.6%). The mean age was 16.5 years and 70% were male.

Results: Most of the patients presented with a history of trauma (77%). Five patients were excluded as their fractures were not treated with surgical curettage. Twenty two patients (65%) had recurrence of the lesion or re-fracture following curettage. None of the patients in whom the fracture occurred after skeletal maturity had a recurrence. Obliteration of the lesion occurred more frequently in those with greatest initial fracture displacement on pre-operative radiographs and in those with impacted fractures. The average time to union and obliteration of the lesion was 4 months (range 1 to 13 months).

Conclusions: Factors predictive of recurrence following curettage were age under 21 years, undisplaced fractures and fractures without impaction on initial radiographs. Patients with these features should be followed up until obliteration of the lesion or skeletal maturity.

Introduction: Frozen shoulder is a general term denoting all causes of motion loss in the shoulder. As the syndrome is very common, many patients do not undergo detailed imaging studies before treatment.

Objectives: A series of 15 patients with primary neoplasms of the shoulder girdle mimicking frozen shoulder syndrome is presented.

Results: There were 6 male and 9 female patients. The common presentation was pain and stiffness of the shoulder joint. Mean age at diagnosis was 46.63Yrs (range 23 “ 71 Yrs). 73% were less than 50 Yrs of age. Only 2 gave history of trauma. Most received local steroids and physiotherapy before diagnosis. There were 10(66.6%) proximal humeral lesions and 5(33.3%) scapular lesions. Humeral lesions included chondrosarcoma (2), Ewing’s (2), lymphoma (2), chondroma (2) and osteoblastoma (1). Scapular lesions included chondrosarcoma (3), lymphoma (1) and fibromatosis (1). Scapular tumors involved older individuals. The mean delay in diagnosis after onset of symptoms was 15.8 mts (range 2 weeks- 48 months). All patients had X rays and CT / MRI. Treatment included a combination of surgery, chemotherapy and radiation. 3 patients with humeral lesions died at a mean of 20.6 mts, and 3 patients with scapular lesions died at a mean of 4.3 mts after diagnosis. The common cause of death was pulmonary metastasis.

Discussion: Many so called frozen shoulders are joints inhibited by pain rather than by true contracture. The commonest lesion to mimic a frozen shoulder is a slow growing low/middle grade chondrosarcoma. Young patients presenting with persistent pain or night pain must be examined for this possibility. Consideration should be given for further investigation before instituting treatment. Delay in diagnosis adversely affects survival. Surgeons are reminded that although rare, a tumor should be suspected when clinical presentations are unusual.

Purpose: To evaluate the clinico-pathological features and outcome of osteosarcoma in patients over the age of 40 in Scotland.

Methods: A retrospective review was performed using data collected by the Scottish Bone Tumour Registry on patients diagnosed with osteosarcoma over the age of 40 between 1960 and 2004. Information about tumour location, age of diagnosis, gender, lung metastasis, and survival was analysed. Histological slides were reviewed again and the diagnosis of osteosarcoma confirmed. The overall survival was calculated using Kaplan-Meier survival curves.

Results: 145 patients were identified. 78 patients had malignant change in pre-existing Paget’s disease. 60 patients had osteosarcoma and 18 malignant fibrous histiocytoma. Average age of diagnosis of Paget’s osteosarcoma was 67.8 years, male to female ratio of 2:1 and 27% of cases were within the pelvis. Median survival was 6 months. 30% had lung metastasis at presentation.

54 patients had conventional osteosarcoma. Average age of diagnosis of 58.8 years, male to female ratio of 3:2 and 37% were femoral. Median survival was 11 months.

13 patients had radiation-induced osteosarcoma. Average age of diagnosis of 67.2, male to female ratio of 1:6 and 5 out of the 13 had pelvic osteosarcoma. Median survival was 8 months.

Conclusion: We present the clinico-pathological features and outcome of osteosarcoma in patients over the age of 40 in Scotland between 1960 and 2004. Pelvic disease and metastasis at presentation were prevalent in patients with Paget’s and radiation-induced osteosarcoma reflecting their poor outcome.

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.

This study aimed to analyse clinical, radiological and histological features of 29 cases of pathological fractures of the long bones either presented as or occurred during the course of sarcomatous degeneration in Paget’s disease of bone utilising Scottish Bone Tumour Registry between 1950 to 2000. The mean age was 67.2 (range, 53–81) years with 16 males and 13 females. The commonest bone affected was the femur (n=13) followed by humeral (n=8), tibial (n=5), ilium (n=2) and os calcis (n=1) fracture. The mean duration of symptoms prior to presentation was two months. Fourteen patients presented with pain associated with a pathological fracture. One patient presented with painless lump and eleven with painful swelling followed by a pathological fracture. In the remaining three, the patients reported with a delayed or non-union of the pathological fracture. Nine patients presented acutely as pathological fracture being their main presenting symptom. The typical radiological appearance was a lytic (n=22). Histology showed predominantly osteosarcoma (n=17) followed by malignant fibrous histiocytoma (n=7) and pleomorphic sarcoma (n=5). Amputation was carried out in 16, internal fixation in 5 and replacement arthroplasty in two (1-cemented bipolar and 1-Charnley’s total hip arthroplasty). In addition, adjuvant radiotherapy in 13, adjuvant chemotherapy in 3 and combined radiotherapy-chemotherapy in 2 patients was instituted. The mean survival was 19.3 months (humeral=4.6, pelvifemoral=30, tibiocalcaneal=23.3 months). In conclusion, the majority of the pathological fractures in Paget’s sarcoma behave differently from their counterparts (non-Pagetic pathological fractures) with regard to their limb ablation treatment rather than stabilisation operation. The actual occurrence of a pathological fracture did not show to significantly affect the overall survivorship.

Between 1944 to 2003, eighty nine cases were registered with a diagnosis of Paget’s sarcoma in the Scottish Bone and Soft Tissue Tumour Registry. We found thirteen cases of sarcomatous degeneration of the spine (0.26% of the total bone tumour registry case) which were analysed in this study elaborating clinical, radiological and histopathological features. The mean age was 66.9 years (range 56 to 79 years). There were ten males and three females. There were seven cases involving sacral spine (63.6%), three cases involving lumbar vertebrae and two affecting dorsal spine. One case had diffuse dorso-lumbar involvement from D11 to L3 vertebrae. The mode of presentation was increasing low back pain (in all 13), unilateral sciatica (6, left sided-5, right sided-1), bilateral sciatica (2), lower limb weakness (8) and autonomic dysfunction (4, presented as chronic cauda equina syndrome). The majority of the cases (69.23%) were osteosarcomas. Out of these osteosarcomas, two showed giant cell rich matrix and one revealed predominant telengiectatic areas. Rest of the histological types was shared by chondrosarcoma, fibrosarcoma and malignant fibrous histiocytoma. Decompression laminectomy was performed in three cases. Eight patients had received radiotherapy. The mean survival was 3.93 months (range, 1 week to 7 months), nearly half to the whole Scottish Paget’s sarcoma series with a mean survival of 7.5 months. We found a constellation of symptomatology due to radiculo-medullary compression with a fatal evolution, predominantly lumbosacral involvement, predominantly osteosarcomatous histopathology with a poorest prognosis of all Paget’s sarcoma. Although, decompression laminectomy and adjuvant radiotherapy provided reasonable pain relief and palliation; however, there was no significant influence on the overall prognosis of the patients with Paget’s sarcoma of spine in the last six decades.

Purpose: The aim of the study was to document the trends in survival from childhood osteosarcoma in Scotland using clinical data held in the Scottish Bone Tumor Registry from 1933 onwards.

Methods and Results: From 217 osteosarcoma patients identified in Scotland aged 18 and under with case notes in the BTR, 184 with non-metastatic appendicular disease were included in the analysis. Kaplan Meier curves were constructed and log rank statistics calculated for univariate analysis. Multivariate analysis was performed using the Cox regression proportional hazards model.

Epidemiological figures reflect those of other studies. The male: female ratio was 1.4: 1, most common age at diagnosis was 16 – 18 and the most common site of tumor was the distal femur, 71% of tumors occurred at the knee. 5 year and median survival were 30% and 26 months for the entire period. 5 year survival was found to have improved from 21% between 1933–1959 to 62% in 1990 – 1999. On univariate analysis the most significant factor influencing outcome was use of chemotherapy in treatment (p< 0.00005). On multivariate analysis, date of diagnosis had most influence on the hazard ratio, the greatest difference being found between diagnoses pre and post – 1980. Site of tumor was also found to be a significant factor (p=0.044). The survival from Osteosarcoma in Scotland in recent years was found to be no worse than the rest of the UK as had previously been suggested.

Conclusion: Survival from childhood osteosarcoma in Scotland has improved significantly from the 1930s to the present day. This is largely due to the introduction of effective chemotherapy protocols into the treatment regimen. These improvements reflect those seen in other countries over the same period.

Ten cases of histologically proven chondromyxoid fibroma (CMF) of the Foot and Ankle with a mean follow-up of 6.1 years were reviewed. The mean age was 19 years (12.8 for skeletally immature and 25.2 for remaining skeletally mature patients). There were 6 males and 4 females. There were 5 phalangeal (3-proximal, 1-middle and 1-terminal phalanx), 3 metatarsal, 1 tarsal affecting body of os calcis and one case of distal tibial involvement. The mean delay in presentation was 16.7 months. The common modes of presentation were pain only (n=4), painful lump (n=4) and painless lump (n=2). Typical x-ray finding was an expansile, lobulated, cystic metaphyeal lesion. Cortical erosion was documented in 50% patients. Histology confirmed by several pathologists due to difficulty in diagnosis. There were two atypical CMF. In four cases, curettage alone was carried out, while 5 underwent curettage along with autogenous bone grafting. One case of distal phalangeal CMF had primary toe amputation. Postoperatively, calcaneal CMF had infection (treated with wound debridement and re-curettage). Two cases observed recurrences after 9 and 16 months from their initial operation. Both of them were male, had preliminary proximal phalangeal CMF, showed to have cortical erosion and were treated with curettage only. Re-curettage was done for one, while toe amputation through the metatarsal neck was performed for another recurrence. Foot phalangeal CMF which were initially treated with curettage only should be kept under close follow-up as we observed 20% recurrence rate within two year period. Cases with cortical erosion should be supplemented with autogenous bone grafting.

A 68 year old lady presented with one year history of painful right heel. She noticed slowly growing swelling resulting in broadening of the heel and difficulty in putting on her shoe-wear for a period of two months. On physical examination, there was a diffuse swelling over the posterior aspect of the right heel. The mass was firm to hard, mildly tender on palpation, fixed to bone and deep-seated. There was no erythema with increased warmth over the area of the mass. Plain x-ray of the affected heel revealed a pathological avulsion fracture of the posterosuperior corner of the calcaneus with typical Pagetoid features in the form of diffuse cortical thickening, coarsened trabeculae, loss of corticotrabecular differentiation and poorly demarcated osteolytic destruction. The lateral radiograph of the left heel showed normal radiological appearance of the os calcis. Chest x-ray was normal. The histological examination showed malignant fibrous histiocytoma characterised by the abundance of pleomorphic cells, spindle cells, lipid containing macrophages and touton type giant cells. The microvascular invasion and amount of mitosis declared the tumour as a high grade anaplastic, pleomorphic tumour. A below knee amputation was done approximately 3 weeks from the date of biopsy and after full staging. The patient died after surviving for a period of 6 years and 8 months from the date of biopsy. It is important to recognise the fact that an avulsion fracture can be pathological in nature and secondary to Paget’s sarcoma in patients with Paget’s disease. This case highlights that a high index of suspicion should be observed in patients presenting with a chronic heel pain who are shown to have pre-existing Paget’s disease on radiological grounds and who may have an avulsion fracture or other localising signs of underlying sarcomatous degeneration.

We describe a 65 year old gentleman presented with a three month history of painful lump in the right forearm which was subsequently confirmed to be osteosarcomatous degeneration in the Paget’s disease of the radius.

A 65 year old gentleman presented with a three month history of painful lump in the right forearm with overlying redness thought by the patient to be the result of recent sun exposure. On physical examination, there was an 8 x 10 cm sized mass over the radial aspect of the right forearm. The mass was soft to firm, mildly tender to palpation, fixed to bone and deep-seated. There was erythema with increased warmth over the area of the mass. There was significant restriction of forearm supination and pronation. Neurovascular examination was normal. Chest x-ray was normal. Plain x-ray of the affected forearm revealed a mixed pattern (both sclerotic and lytic appearance) affecting the entire diaphyseal portion of the radius with soft tissue extension in the mid-shaft. There were alterations in the trabecular pattern of the bone, thickened cortices, encroachment of the medullary space and an increase in the size and deformity of the entire length of the radius with cortical destruction sparing the ulna. Bone scan showed multiple hot foci with an increase uptake in the sacrum, left hemipelvis, L3 vertebra and intensely increased uptake over the right radius. On the 10th day of presentation, an open incisional biopsy was done. Microscopy showed a high grade sarcomatous tumour with areas of osteoid formation. There was evidence of vascular invasion. The presence of preexisting Paget’s disease of bone was confirmed in the specimen. An above elbow amputation was done 4 weeks from the date of biopsy and after full staging. The patient died 16 months with multiple metastases from the date of diagnosis.

Any patient with Paget’s disease who develops new pain in a previously pain-free area of Paget’s disease, or worsening of pain, or a painful progressive mass or a lytic area in sclerotic Paget’s bone should be evaluated for possible sarcomatous degeneration.

Fourteen cases of pigmented villonodular synovitis (PVNS) of the foot and ankle (between January 1957 and December 1999) accrued from Scottish bone tumor registry are presented with an average follow-up of 4.6 years aimed to analyse the clinical, radiological and histopathological features in order to investigate the clinical behaviour of PVNS in the foot and ankle, and to determine the factors influencing recurrence. The mean age was 26.4 years (range, 8 to 52 years). There were eight females and six males. The mean delay in presentation was 10.3 months. The anatomical sites were foot phalanges (n=2), tarso-metatarsal area (n=3) and hindfoot (n=9). Hindfoot cases comprised of 6 extra-articular soft tissue swelling around the ankle, two affecting the ankle joint and one involving the subtalar joint. There were eight (57.1%) cases presented with painless lump, five (35.7%) patients with painful lumps and one case with a lump associated with toe deformity. The clinical suspicion were ganglion, gout, soft tissue swelling (? tumour) and exostosis. Peri-articular tissue invasion and cortical infiltration was found in one third on plain films. CT scan showed multiple lytic lesions and MRI scan findings were consistent with extensive low signal soft tissue hypertrophy and bone erosion, two of which were suspected with synovial sarcoma. Excision of the lump was done in 4 cases with a complete recovery. Foot phalangeal PVNS were treated with toe amputation through metatarsophalangeal joint and no cases had recurrence of the lesion. There were two recurrences affecting the ankle and the subtalar joint. Recurrent ankle PVNS was treated with re-exploration, open synovectomy, curettage of talar cyst and autogenous bone grafting. The second recurrent case involving subtalar joint was treated with re-excision and curettage. Both recurred cases were primarily treated with intralesional excision for their diffuse variety. There were no recurrences in the nodular variety. Complete recovery was achieved in 85.7% case (12/14). A high index of suspicion for PVNS should be observed for cases presenting with a painless or painful mass in the foot and ankle region. Complete recovery can be achieved in the majority by complete excision. Toe amputation may be considered for foot phalangeal PVNS.

Between 1944 to 2003, ninety cases of Paget’s sarcoma from the Scottish Bone and Soft Tissue Tumour Registry were reviewed. The mean age of patients was 72.3 years (range, 30 to 85 years). There were 59 males and 30 females. The most frequent sites were the femur (26), pelvis (19), humerus (13), tibia (11), and thoraco-lumbosacral spine (9). Biopsy was done in 69 cases. In the remainder 20 cases, the histological diagnosis was confirmed either from examining amputated limb or at autopsy. The most common type was osteosarcoma. Local excision was performed in seven cases. Resection in two cases and prosthetic replacement in two cases was carried out. Twenty nine patients underwent amputation surgery. Chemotherapy was administered to 15 patients (including 2 preop chemotherapy). Fifty one patients received radiotherapy (preoperatively in eight patients). All the patients died within one year from the date of biopsy with an average survival time of 7.5 months. Patients with Paget’s sarcoma tend to have a very poor prognosis inspite of improvements in therapy strategies including surgery, radiation therapy, and chemotherapy. We emphasise the need for more research by a combined oncosurgical, oncological, radiological and histological approach in the management of Paget sarcoma to improve the prognosis.

Between 1944 to 2003, eighty nine cases of Paget’s sarcoma from the Scottish Bone and Soft Tissue Tumour Registry were reviewed. The mean age of patients was 72.3 years (range, 30 to 85 years). There were 59 males and 30 females. The most frequent sites were the femur (26), pelvis (19), humerus (13), tibia (11), and thoracolumbosacral spine (9). Biopsy was done in 69 cases. In the remainder 20 cases, the histological diagnosis was confirmed either from examining amputated limb or at autopsy. The most common type was osteosarcoma. Local excision was performed in seven cases. Resection in two cases and prosthetic replacement in two cases was carried out. Twenty nine patients underwent amputation surgery. Chemotherapy was administered to 15 patients (including 2 preop chemotherapy). Fifty one patients received radiotherapy (preoperatively in eight patients). All the patients died within one year from the date of biopsy with an average survival time of 7.5 months. Patients with Paget’s sarcoma tend to have a very poor prognosis inspite of improvements in therapy strategies including surgery, radiation therapy, and chemotherapy. We emphasise the need for more research by a combined oncosurgical, oncological, radiological and histological approach in the management of Paget sarcoma to improve the prognosis.

Introduction: Swellings of the medial end of the clavicle in children and adolescents are common. The vast majority represent either osteomyelitis, or a benign conditions such as eosinophilic granuloma. Their radiographic and early clinical appearance, however, are difficult often to discriminate from Ewing’s sarcoma. Unrewarding biopsies are often performed.

Aims: To identify incidences of malignant neoplasm at the medial end of clavicle in patients aged 18 and under from the Scottish Bone Tumour Registry (SBTR), and by questionnaire from European tumour databases.

Methods: The SBTR was searched for childhood malignant neoplasms as above. Questionnaires were sent to 171 European regional and national tumour databases. Information requested included details of patients with malignant neoplasms as above, longevity of database operation and population served. An estimate of European child population rates was made based on a conservative (North Italian) calculation of children aged under 18 representing 14.4% of the population (comparison our region 21.9%). Confidence intervals were calculated using Wilson method.

Results: The SBTR identified 7 children with neoplasms of the clavicle out of a total register of 4009 cases. Only 1 was malignant and this at the lateral end.

Responses were gained from 56 European databases. National databases were less able to identify this detailed anatomical site than regional ones. 30 databases from 12 countries responded with complete data representing 1.619 billion total population person-years. Four malignant tumours at the medial end of clavicle were identified from a total of 233.2 million children-years at risk.

Discussion: By extrapolation, in our region (population 779,000) a malignant childhood medial clavicle tumours is estimated to occur once every 342 years (95%CI 91 years – ∞). It is recommended that biopsy of such childhood swellings should be restricted to patients who present with features atypical of infection or benign lesions.

Aim: The aim of this study was to look at the presenting features, histological grade, size of primary tumour, method of treatment and patient and doctor delays in upper extremity Ewing sarcoma to observe the effects on local recurrence, metastasis and survival.

Methods: 19 patients with upper extremity Ewing sarcoma were identified using the Scottish Bone Tumour Registry which carries clinical, pathological and radiological data on the majority of bone tumours diagnosed in Scotland over the past 50 years.

Results: With increasing tumour Enneking grade at presentation there was a significantly higher mortality (X2=8.0606, p=0.0178). Patients with a higher grade also had an increased trend towards local recurrence (X2=5.1154, p=0.0775). Grade did not seem to influence the occurrence of metastasis. Patients with larger tumours tended to have a higher mortality (50% vs 27% dead at 5 years). All patients presented clinically with pain and all but two complained of some sort of swelling. It was found that there was a trend towards a higher grade in patients presenting with a longer duration of symptoms (X2=4.6269,p=0.0989). No difference in survival was noted between patients undergoing surgery and chemotherapy and patients undergoing radiotherapy and chemotherapy. Disease-free survival was 100% at both 5 and 10 years for Enneking Grade IIA, 56% at 5 and 10 years for Grade IIB and 0% at 5 years for Grade III.

Conclusions: This study re-emphasises the importance of a delay in diagnosis on outcome. Longer symptom duration results in a higher histological grade at presentation. In turn a higher presenting grade is associated with a higher mortality. In agreement with other studies a larger primary tumour correlates with a poorer outcome. Outcomes in terms of survival are comparable for groups treated with adjuvant radiotherapy or surgery.

Introduction: The aims of the study were to analyse the presenting features of chondroasarcoma for prognostic significance, to observe the effects of grade and surgical management on local recurrence, metastasis and survival: and to assess the significance of delays to consultation and treatment.

Methods: From Scottish Bone Tumour Registry Records, 24 proximal humeral chondrosarcomas were treated between 1937 and 2002 in several hospitals in Scotland. Clinical records were available for all patients. Only 4 patients were eventually lost to follow-up. Age at presentation ranged for 16 to 79 (median 56) and male to female ration was 2:1. Patients were followed up for a range of 5 months to 24 years (median 6.5 years). Tumour histology was systematically reviewed: 5 (22%) were grade 1, 12 (52%) were grade 2, and 6 (26%) were grade 3. Dedifferentiated tumours were considered as grade 3.

Results: Pain was almost invariable (23/24), but its severity increased with grade. In the absence of pathological fracture (fracture in 4 Patients), swelling was significantly associated with increasing grade (X2+8.56, p+0.0139), as was symptom progression (X2=7.52, p=0.0232). Delay in diagnosis was calculated separately as ‘patient delay’ (range 0–69 months) and ‘doctor delay’ (range 0–132 months). No improvement in diagnostic delay was noted in this time period (1937–2002)

All cases were biopsied. 37% of these were excisional biopsies, 29% were incisional biopsies and 17% were needle biopsies. 21 patients (88%) received definitive surgery. Of these 5 had forequarter amputations (24%), 11 cases were excised marginally (52%), and 5 cases curetted (24%). Surgical choice was highly dependent on grade (X2=4.9256, p=0.005). In all cases the intent was curative.

2 patients had metastasis disease at diagnosis, and 5 developed metastases after definitive surgery. 4 patients had local recurrence (all had undergone wide local excision). All patients with grade 1 tumours remained disease free. Cumulative survival at 5 years was 57% and at 10 years 42%. Patient age did not affect survival. 5 year survival in grade 1, 2 and 3 tumours was 100%, 83% and 20% respectively. 4 of 5 patients undergoing amputation developed metastases and survival was significantly worse in the amputation group. Local recurrence in the wide local excision group did not diminish prospects for survival.

Discussion: Swelling predicts aggressive disease; as found in studies in childhood sarcomas. Progressive symptoms and serve pain are additional features indicative of high histological grade. In contract to improvements noted in several other studies, patient delay is highly variable and has not been demonstrably reduced over time.

Curettage was chosen for most grade 1 and some grade 2 tumours. Although maintenance of function is far better, our study provides no evidence that curettage results in increased local recurrence rates. Indeed, local recurrence in the wide local excision group did not depress survival figures. Because of early death in the amputation group, we would recommend avoidance of amputation in favour of wide local excision in almost all cases if possible. Age alone should not be a factor in determining surgical treatment.

The Government has set a target of two weeks for referral of suspicious tumours to specialist centres, but what symptoms should raise suspicion of chondrosarcoma and what factors affect survival and local recurrence? A retrospective study of 320 cases of chondrosarcoma from the Scottish Bone Tumour Registry was performed. Presenting symptoms and were related to tumour grade and duration of disease free status and survival. Pain, swelling and loss of function were the most common presenting features. Rapid progression of pain was significantly associated with high-grade tumours. Longer duration of symptoms from onset to presentation was associated with low-grade tumours. High-grade tumour and metastasis at diagnosis were associated with poor prognosis. Thus, patients with a longer history of symptoms actually appear to have longer disease free survival after presentation. Expert opinion should be sought for patients presenting with pain, swelling and loss of function. Practitioners should make prompt referral of patients presenting with pain that is rapidly increasing in severity.

Solitary benign peripheral-nerve tumours are rare and may be difficult to diagnose correctly. Surgical excision may increase the patient's symptoms and may not be necessary. We have reviewed the presentation, clinical findings and histology of 104 solitary tumours presenting at one centre between 1959 and 1990. Male patients predominated for both schwannoma and neurofibroma. There was considerable but variable delay before presentation; 94% of patients complained primarily of a mass and less than half had pain or paraesthesia. The correct diagnosis had been made in only a few cases before operation, and the incidence of neurological symptoms doubled after exploration. We emphasise the need for vigilance, accurate preoperative diagnosis, and careful surgery.

We reviewed the records and radiographs of seven children who presented with knee pain, local tenderness over the medial femoral condyle, and radiological irregularity of the distal medial metaphysis of the femur suggestive of malignancy. In the five patients who had biopsies, histological changes were consistent with musculotendinous avulsion, and the dissection of ten cadavers confirmed the site to be the insertion of part of the adductor magnus. The recognition of this lesion and knowledge of its benign nature may avoid unnecessary anxiety and needless biopsy.

We studied the pedigrees of 17 index patients with osteosarcoma, recording malignant disease and cause of death for first- and second-degree relatives. There were seven cancers and five cancer deaths per 2151.5 person-years in first-degree relatives of osteosarcoma patients under the age of 50 years, a significantly greater incidence than in an age- and sex-matched population group (p < 0.001). This excess of malignancy was largely due to two families which fulfilled the criteria for the Li-Fraumeni cancer family syndrome. Both of these families were shown to have the genetic alterations in the p53 gene which have been implicated in this syndrome. Our study suggests that orthopaedic surgeons seeing new cases of osteosarcoma should arrange screening for familial malignancy.

We report a case of malignant fibrous histiocytoma of the hip which occurred 30 years after the insertion of a single chrome-cobalt alloy screw for a slipped femoral epiphysis. The possible aetiological association between malignant tumours and metallic implants is discussed.