Bone Tumour Registry Research Articles

Twentieth-century survival from osteosarcoma in childhood

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.

Book Review: Pediatric Orthopedics in Practice, by Fritz Hefti, M.D. and co-authors Reinald Breuner, M.D., Carol Hasler, M.D. and Gernot Jundt, M.D.

This is a comprehensive textbook on pediatric orthopedics written by Professor Fritz Hefti and his colleagues from Basel University Children’s Hospital, Switzerland. The textbook is arranged into sections, the first of which is broken down into parts representing regional segments of the body, such as the spine, pelvis, knee, foot and ankle, and upper extremities. The latter section covers systemic conditions, such as trauma, infections, tumors, hereditary diseases and neuro-orthopedics. It is beautifully organized with general sections defining a brief history of orthopedics and a great deal of data on normal measurements and pertinent examination of the child. It is extremely well illustrated and has numerous cartoons drawn by Dr. Franz Freuler (very similar to those of Mercer Rang), as well as the schematic drawing by Dr. Fritz Hefti. Also, throughout each section, there are interspersed aphorisms by famous people that link each section and add to your enjoyment when reading this text. Because of the large number of cartoons, drawings and radiographs, it is an easy to follow text and most enjoyable reading. In the section about hips, the authors discuss the examination of the hip and radiographs. Then they discuss the various disorders of the hip with a thorough management of each, ranging from Perthes, slipped epiphysis, hip dysplasia, toxic synovitis, to femoral anteversion, etc. All of this information and this type of organization would be most useful for pediatricians, medical students and orthopedic residents on the pediatric orthopedic service. There is much information in each section elucidating the diagnostic tests and treatment options for each disorder. In each of these regional areas, a differential diagnosis table is also presented. Dr. Hefti and his colleagues are international experts on the hip, spine and tumors and this is quite apparent in the discussion of these areas in their text. The references are very up to date and, yet, not overwhelming as they cover each of the topics. Tumors are considered regionally, such as the distal femur (under knee), but also considered in more detail in the systemic chapters. Thus, there is some overlap between these areas and perhaps some repetition. The Basel Bone Tumor registry may be biased toward more serious tumors, as the most common tumors of the distal femur were osteochondromas 37%, osteosarcomas 20% and non-ossifying fibromas only 15%. Likely, at our center, non-ossifying fibroma and osteochondromas would be far more common than osteosarcoma. Overall, this is a wonderful textbook that should be a part of pediatric orthopedic training programs for orthopedic residents and medical students. It is an excellent introduction to our specialty and most enjoyable to peruse. The author's keen insight into clinical care of the pediatric orthopedic patient and warm sense of humor makes this a wonderful text. Pediatric orthopedics is indeed fun!

Outcome of 17 pigmented villonodular synovitis (PVNS) of the Knee at 6 years mean follow-up

Between January 1950 and December 2000, 16 patients were identified from Scottish Bone Tumour Registry with 17 histologically proven pigmented villonodular synovitis (PVNS) of the knee. The mean follow-up was 6 years (range, 1–14 years). A knee swelling of chronic duration with dull ache was the common presenting symptom. The mean duration of symptoms prior to presentation was 25 months (1–108 months), however it was much less (mean, 7 months) in four patients with a history of trauma. The mean age was 33 years (range, 16–58 years) with a slight male predominance. The lesion was predominantly anterior in nine patients, posterior in four, anterolateral in two, and medial and lateral in one each. Three patients (four knees) had localised disease and 13 diffuse. Anteroposterior and lateral radiographs of the knee revealed normal findings in 11 patients, features of gonarthrosis in four and a large suprapatellar loose body in one patient (both knees). Open (incisional-eight, excisional-eight) biopsy was carried out in all and all were histologically confirmed as PVNS. Removal of a localised synovial mass or loose body with surrounding partial synovectomy (four) was carried out for the localised variety, whilst open partial (three) or total (radical) synovectomy (10) was performed in all cases of diffuse PVNS. Three of seventeen knees had a recurrence, noted at 4, 6 and 8 years postoperatively (0% — localised, 23% — diffuse variety). A total (radical) synovectomy should be considered in diffuse PVNS in order to obtain optimal outcome.

Metastatic bone disease: A 36‐Year single centre trend‐analysis of patients admitted to a tertiary orthopaedic surgical department

The treatment and outcome of primary malignant bone tumours has changed with the advances in diagnostic and treatment modalities. A trend-analysis on a large cohort of patients with metastatic bone disease was performed. A retrospective chart review of all cases with metastatic bone disease admitted to a single tertiary orthopaedic referral centre, registered with the Vienna Bone and Soft Tissue Tumour Registry between 1968 and 2003 was conducted. For trend-analysis of frequency, survival, primary site, treatment methods, and others, the 36-year study duration was divided into four periods. The study identified 601 females and 580 males (mean: 60 years) with metastatic bone disease. The most common metastases were secondary to breast cancer (n = 275; 23%) and renal cell carcinoma (n = 242; 21%) and the majority were located in the femur (n = 332; 28%) and spine (n = 348; 29%). Overall, the proportion of patients who underwent surgery decreased. At follow-up, 887 (75%) patients were verified to have died of their disease. Over the 36-year period, the frequency of bone metastases has increased at our centre. Although survival increased over time, the difference was not significant; this was most likely attributable to the seriousness of cases referred to our tertiary care centre.

Pigmented Villonodular Synovitis of the Foot and Ankle: Forty Years of Experience from the Scottish Bone Tumor Registry

Fourteen cases of pigmented villonodular synovitis (PVNS) of the foot and ankle accrued from the Scottish Bone Tumor Registry are presented with an average follow-up of 4.6 years. This study analyzed the clinical, radiological, and histopathological features and investigated their clinical behavior and the factors influencing recurrence. The mean age of the patients was 26.4 years (range, 8-52 years). There were 8 women and 6 men. The mean delay in presentation was 10.3 months. The anatomical sites were phalanges (n = 2), tarso-metatarsal area (n = 3), and hindfoot (n = 9) (6 extraarticular soft tissue swellings around the ankle, 2 ankle, 1 subtalar joint). Eight (57.1%) cases presented with a painless lump, 5 (35.7%) patients had painful masses, and 1 case had a lump associated with toe deformity. Peri-articular tissue invasion and cortical infiltration were found in one third on plain films. Magnetic resonance imaging findings were suggestive of synovial sarcoma in 2 cases because of extensive low-signal soft tissue hypertrophy and bone erosion. Excision of the lump was performed in 4 cases with a complete recovery. Phalangeal lesions were treated with toe amputation through the metatarsophalangeal joint, and no cases had recurrence. There were 2 recurrences affecting the ankle and the subtalar joint. There was a 14.3% recurrence rate, while complete recovery was achieved in 85.7% cases (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.

Chondromyxoid fibroma of the sternum

Bjork-Shiley valve in the mitral position. Ann Thorac Surg. 1981;32: 58-62. 3. Trites PN, Kiser JC, Johnson C, Tycast FJ, Gobel FL. Occlusion of Medtronic Hall mitral valve prosthesis by ruptured papillary muscle and chordae tendineae. J Thorac Cardiovasc Surg. 1984;88:301-2. 4. Mok CK, Cheung DL, Chiu CS, Aung-Khin M. An unusual lethal complication of preservation of chordae tendineae in mitral valve replacement. J Thorac Cardiovasc Surg. 1988;95:534-6. 5. Lemke P, Roth M, Kraus B, Hohe S, Klovekorn WP, Bauer EP. Ruptured papillary muscle after mitral valve replacement with preservation of chordae tendineae. Ann Thorac Surg. 2001;72:1384-6.

Primary bone tumours of the spine: a 42-year survey from the Leeds Regional Bone Tumour Registry.

We conducted a review of the Leeds Regional Bone Tumour Registry for primary bone tumours of the spine since establishment in 1958 until year 2000. To analyse the incidence of primary tumours of the spine and to record the site of occurrence, sex distribution, survival and pathology of these tumours. Primary tumours of the spine are particularly rare, accounting for between 4 and 13% of published series of primary bone tumours. The Leeds Bone Tumour Registry was reviewed and a total of 2,750 cases of bone tumours and tumour-like cases were analysed. Consultants in orthopaedic surgery, neurosurgery, oncology and pathology in North and West Yorkshire and Humberside contribute to the Registry. Primary bone tumours of the osseous spine constitute only 126 of the 2,750 cases (4.6%). Chordoma was the most frequent tumour in the cervical and sacral regions, while the most common diagnosis overall was multiple myeloma and plasmacytoma. Osteosarcoma ranked third. The mean age of presentation was 42 years and pain was the most common presenting symptom, occurring in 95% of malignant and 76% of benign tumours. Neurological involvement occurred in 52% of malignant tumours and usually meant a poor prognosis. The establishment of Bone Tumour Registries is the only way that sufficient data on large numbers of these rare tumours can be accumulated to provide a valuable and otherwise unavailable source of information for research, education and clinical follow-up.

Paget Sarcoma of the Spine: Scottish Bone Tumor Registry Experience

Retrospective case study of 13 cases of Paget sarcoma of the spine accrued from a prospectively collected Tumor Registry database. To analyze the clinical, radiologic, and histologic features of Paget sarcoma of the spine and to determine the factors influencing the prognosis. Paget disease of bone is a common disorder with the spine being involved in over 50% of patients. However, sarcomatous degeneration in the vertebral column is an extremely rare complication. There is very little in the literature with regard to clinical presentation and prognosis of patients with Paget sarcoma affecting the vertebral column. Between January 1944 and December 2003, 89 patients were registered with a diagnosis of Paget sarcoma in the Scottish Bone Tumor Registry. Thirteen patients with Paget sarcoma of the spine were analyzed with regard to their clinical, radiologic, and histopathologic features along with the prognostic predictors. The mean age was 66.9 years (range: 56-79 years). There were 10 males and three females. There were seven cases involving the sacral spine (63.6%), three cases involving lumbar vertebrae, two affecting the dorsal spine, and one with diffuse dorsolumbar involvement (D11-L3). The mode of presentation was progressively increasing low back pain (in all 13), unilateral sciatica (six; left-sided, five; right-sided, one), bilateral sciatica (two), lower limb weakness (eight), and autonomic dysfunction (four). Ten of 13 cases (76.9%) were osteosarcoma. The rest were chondrosarcoma (n = 1), fibrosarcoma (n = 1), and malignant fibrous histiocytoma (n = 1). Decompression laminectomy was performed in three patients with progressive neurologic deficit. Eight patients had received radiotherapy. The mean survival was 4.22 months. This series confirmed that Paget sarcoma of the spine has a very poor prognosis. We found a constellation of symptomatology in patients with sarcomatous Paget spine resulting from radiculomedullary compression, primarily lumbosacral involvement and predominantly osteosarcomatous histology. There was no significant difference observed on the overall prognosis of the patients with Paget sarcoma of the spine in the last 6 decades.

Chondromyxoid fibroma of the foot and ankle: 40 years’ Scottish bone tumour registry experience

Ten cases of histologically proven chondromyxoid fibroma (CMF) of the foot and ankle with a mean follow-up of 6.1 years were retrospectively reviewed using the Scottish Bone Tumour Registry. The patients' mean age was 19 years; there were six males and four females. The anatomical locations were five phalangeal, three metatarsal, one tarsal affecting body of os calcis and one distal tibial. The median delay in presentation was 4.5 months. The modes of presentation were pain only (n=4), painful lump (n=4) and painless lump (n=2). The typical radiological finding was an expansile, lobulated, cystic lesion. Cortical erosion was documented in 80% patients. In four cases, curettage alone was carried out, while five patients underwent curettage along with autogenous bone grafting. One patient with distal phalangeal CMF had a primary toe amputation. Two patients had recurrences 9 and 16 months after their initial curettage. Both of them were males with proximal phalangeal CMF, associated with cortical erosion. Foot and phalangeal CMF initially treated with curettage only should be closely followed up, as we observed a 20% recurrence rate within a 2-year period. Cases featuring cortical erosion require thorough curettage and may require autogenous bone grafting to prevent fracture.

Giant cell tumour around the foot and ankle

Giant cell tumour (GCT) has been known to behave unpredictably when located in the foot and ankle bones. Involvement of foot and ankle bones is rare and comprises of less than 4% of all the GCTs. Out of 175 cases of GCT documented in the Scottish Bone Tumour Registry, 8 cases arose from foot and ankle. The mean age was 28.8 years (13–47). These included six males and two females. Pain and swelling was the common mode of presentation. All the cases had minimum follow-up of 36 months (36–180) and had definitive treatment within the first 3 months of initial presentation. The Campannacci radiographic staging system was used in grading the tumours. Seven of eight cases had intralesional procedure, while the remaining one had primary below knee amputation. One case had local recurrence within 2 years of the initial treatment requiring further surgery. Five out of seven cases with curettage involved the distal tibia or fibula. We had one recurrence of the three cases involving the tarsal bones, which reflects the difficulty in achieving thorough curettage in small bone tumours. Thorough curettage and bone grafting with adjuvant therapy has a low recurrence rate and should be considered when possible particularly in the long bones. Use of bone cement may be a better option in small bone involvement in view of difficulty in achieving thorough curettage. Regular follow-up helps detect early recurrence.

Local recurrence in primary osteosarcoma of the femur.

Abstract Cases of primary osteosarcoma of the lower femur treated by through-femur amputation and reported to the Bristol Bone Tumour Registry have been reviewed from the viewpoint of local recurrence. There were 2 recurrences within the stump and 4 cases with metastases in the ilio-inguinal region. It is argued that these figures do not justify the routine use of hip disarticulation with its necessarily cumbersome prosthesis in the primary treatment of these tumours.

Patterns of care and survival for patients aged under 40 years with bone sarcoma in Britain, 1980–1994

The purpose of the study was to calculate population-based survival rates for osteosarcoma (OS) and Ewing's sarcoma (ES) in Great Britain during 1980–1994, determine proportions of patients treated at specialist centres or entered in national and international clinical trials, and investigate effects of these factors on survival. Data on a population-based series of 1349 patients with OS and 849 with ES were compiled from regional and national cancer registries, UK Children's Cancer Study Group, regional bone tumour registries and clinical trials. Follow-up was through population registers. Survival was analysed by actuarial analysis with log-rank tests and by Cox's proportional hazards analysis. Five-year survival rates during 1980–1984, 1985–1989 and 1990–1994 were 42% (95% CI: 37, 46), 54% (95% CI: 50, 59) and 53% (95% CI: 48, 57), respectively, for OS and 31% (95% CI: 26, 37), 46% (95% CI: 40, 51) and 51% (95% CI: 45, 57) for ES. Proportions of patients treated at a supraregional bone tumour centre or a paediatric oncology centre in the three quinquennia were 36, 56 and 67% for OS and 41, 60 and 69% for ES. In 1983–1992, 48% of OS patients were entered in a national trial; for ES, 27% were entered in 1980–1986 and 54% in 1987–1994. Survival was similar for trial and nontrial patients with OS. For ES, trial patients had consistently higher 5-year survival than nontrial patients: 1980–1986, 42 vs 30%; 1987–1992, 59 vs 42%; 1993–1994, 54 vs 43%. During 1985–1994, patients with OS or ES whose main treatment centre was a nonteaching hospital had lower survival rates. In multivariate analyses of patients diagnosed during 1985–1994 that also included age, sex, primary site, surgical treatment centre, the results relating to main treatment centre for both OS and ES retained significance but the survival advantage of trial entry for ES became nonsignificant. For both OS and ES diagnosed since 1985, patients whose main treatment centre was a nonspecialist hospital had a lower survival rate.

Sarcomatous change in the Pagetoid tibiae

We reviewed 13 cases of tibial Paget's sarcoma constituting 14% of all registered Paget's sarcoma cases of the Scottish Bone Tumour Registry between January 1947 and June 2004. Eleven patients were male, and in ten patients the tumour involved the upper half of the tibia. In all cases, the main presenting feature was progressively worsening pain followed by a mass in six, a pathological fracture in five and tibial bowing in four. In nine patients, a lytic lesion was seen radiologically. Histologically, there were three osteosarcomas and ten malignant fibrous histiocytomas. Limb ablation was carried out in 11 patients, of whom nine had trans-femoral amputation. Six patients received adjuvant radiotherapy and/or chemotherapy. Post-operative complications included stump revision in two cases, non-union of a pathological fracture of the tibial tuberosity and a stress fracture. The median survival was 17 months.

Primary malignant bone tumours in Western Australia, 1972-1996

To study the incidence of primary malignant bone tumours in Western Australia (WA) from 1972 to 1996 using the database of the WA Bone Tumour Registry (BTR) and to compare these rates with those in the United States of America (USA). In addition, to undertake a linkage study between the BTR database and that of the WA Cancer Registry (WACR) for the years 1980-1996 to determine if the BTR records could be regarded as population-based and valid for determining incidence rates in WA. For each year of the review, standardised incidence rates were calculated for osteosarcoma, chondrosarcoma, Ewing's sarcoma and for all bone sarcomas combined. For comparison, incidence rates were calculated using data obtained from the Surveillance, Epidemiology and End Results (SEER) Program Registry in the USA. Excluding myeloma, there were 263 cases of primary malignant bone tumours. Osteosarcoma (94 cases), chondrosarcoma (64 cases) and Ewing's sarcoma (49 cases), were the three most common and represented 78.7% of all primary bone sarcomas. Age-sex-standardised incidence rates for osteosarcoma in WA were 28% lower than in the USA. For chondrosarcoma and Ewing's sarcoma, no significant difference was found between rates in WA and the USA. The BTR provides a valuable resource for the study of primary bone tumours. This review has established reliable incidence rates for individual types of bone sarcoma. In both geographical sites there is a slight upward trend in the incidence of primary bone sarcoma.

Scapulo-humeral Paget's sarcoma: Scottish Bone Tumour Registry experience

This study aimed to analyse the clinical, radiological and histological features of 16 scapulo-humeral Paget's sarcoma cases accrued from Scottish Bone Tumour Registry between January 1950 and December 2000. The mean age was 61.5 (range, 44-77) years with 12 men and 4 women. There were three scapular cases and 13 humeral (1 whole length, 5 upper humeral, 5 lower humeral and 2 mid-humeral). These patients presented with progressively increasing pain in the shoulder, arm or elbow (n = 5), a painful mass associated with a pathological fracture (n = 4), a pathological fracture with progressively worsening pain (n = 3), a painful mass (n = 3) and a painless mass associated with wrist drop (n = 1). Histology showed predominantly osteosarcoma (n = 12), followed by pleomorphic sarcoma (n = 2), malignant fibrous histiocytoma (n = 1) and fibrosarcoma (n = 1). Overall, the median survival period was 4.5 months. In summary, Paget's sarcoma of the scapulo-humeral area presents with progressively worsening pain, a painful mass with or without a pathological fracture, predominantly lytic in humeral and purely sclerotic in scapular lesions, osteosarcoma-dominant histology, primarily treated with an early limb ablative surgery and associated with a poor 1-year survival (12.5%) and 5-year survival (6.2%) rate.

Prognostic factors associated with local recurrence, metastases, and tumor-related death in patients with synovial sarcoma.

Prognostic factors associated with local recurrence, metastases, and tumor-related death in synovial sarcoma were studied in 51 patients in the Scottish Bone Tumor Registry from 1955 to 1999. In a multivariate analysis, the presence of poorly differentiated (PD) areas was the strongest prognostic factor associated with local recurrence (Hazard ratio [HR] = 11.3, 95% CI 2.3, 122.5, p = 0.033), metastases (HR = 16.9, 95% CI 2.3,122.5, p = 0.005), and tumor-related death (HR = 6.9, 95% CI 1.1,41.8, p = 0.036). Other significant independent risk factors included bone invasion (HR = 16.6, 95% CI 1.1, 252.5, p = 0.043) and necrosis (HR = 5.1, 95% CI 1.4, 18.99, p = 0.016) for metastases and bone invasion (HR = 17.6, 95% CI 1.2, 253.2, p = 0.035) for tumor-related death. Increasing percentages of PD areas and necrosis were associated with increasing hazard ratios for metastases and death. In the univariate analysis, PD areas, tumor size, and a mitotic count over 10/10 high-power fields were significantly associated with recurrence, whereas necrosis, vascular invasion, and age more than 25 years were additional risk factors for metastases and death. Local recurrence was significantly associated with increased risks for metastases (OR = 6.8, 95% CI 1.6, 28.7, p = 0.006), and death (all cases). Histologic features such as PD areas, necrosis, vascular invasion, and bone invasion should be considered when deciding about adjuvant therapy.

Chondrosarcoma of the bones of the feet

Twelve chondrosarcomas of the bones of the feet from 11 patients in the Scottish Bone Tumor Registry were reviewed. One patient with diaphyseal aclasis (osteochondromatosis) developed 2 chondrosarcomas. The mean age of patients was 52.3 years (range, 17 to 83 years). Men were predominantly affected. Four tumors affected the tarsal bones; the rest involved the short tubular bones. The usual clinical presentation was a painful, progressively enlarging swelling. Radiologically, most showed some bone expansion, cortical destruction with indistinct margins, and soft-tissue extension. Histologically, the majority were middle-grade tumors. Treatment included curettage or local excision for 4 tumors and amputation or ray resection for 8 tumors. Follow-up varied from 6 months to 18 years (average, 5.8 years). Local recurrence after surgery was seen in 3 patients. All 3 died because of metastases to the lungs or brain.

Tumors of the footbones- a report from the Hamburg Bone Tumor Registry

Aim of the present study was a systematic examination of the tumors of the foot registered in the Hamburg Bone Tumor Registry regarding age, sex, location, diagnosis and morphological characteristics. The registry files and histological specimens of 367 tumors and tumor-like lesions of the foot were reviewed. Males were more frequently affected (ratio 4 : 3). Some 20.4 % of all mass forming tumors were malignant. Cartilage tumors (146 cases), cystic (132 cases) and osteoblastic lesions (33 cases) were most common. Several tumors showed a strong predilection for certain parts of the foot. In the calcaneus a solitary bone cyst was the most common lesion. Lesions in the talus and metatarsals with a similar morphology mostly represented intraosseous ganglion cysts. Other tumors in the calcaneus were mainly cartilaginous. Besides chondroblastoma, chondrosarcoma was regularly diagnosed. Chondroma was extremely rare at this site. Contrary to that chondroma was regularly found at the metatarsal and phalangeal bones, it was 4 times more common than chondrosarcoma. An even distribution showed aneurysmal bone cysts and osteoidosteoma. Very rare lesions were fibrous dysplasia, eosinophilic granuloma and intraosseous lipoma. The knowledge of the distribution of tumors in the foot is important for an exact differential diagnosis and thereby for biopsy planning, diagnosis and therapy.

Osteosarcoma in Ga-Rankuwa Hospital: a 10 year experience in an African population.

This is a clinicopathological correlation of cases of osteosarcoma diagnosed and treated at Ga-Rankuwa Teaching Hospital. A retrospective study. Department of Orthopaedics and Anatomical Pathology at Ga-Rankuwa/MEDUNSA Academic complex, a tertiary referral centre. 66 patients with a diagnosis of osteosarcoma. Records of the bone tumour registry were reviewed and cases of osteosarcoma were identified. Slides were retrieved and the diagnoses confirmed in all the cases. Information pertaining to clinical presentation and patients' survival was obtained from the clinical records. The patients' ages ranged from nine years to 75 years with a mean of 19.7 years (median 17 years). Most cases occurred around the knee and the male to female ratio was 2.7:1. The five year survival rate was 7.5% and only 25.8% survived for one year. Our patients present with advanced disease leading to poor survival.

Ewing′s Sarcoma of the Upper Extremity: Presenting Symptoms, Diagnostic Delay and Outcome

Purpose:To look at the presenting features, Enneking stage, size of primary tumour, method of treatment and patient and doctor delays in upper extremity Ewing's sarcoma to observe the effects on local recurrence, metastasis and survival. Patients and methods:Nineteen patients with upper extremity Ewing's sarcoma were identified using the Scottish Bone Tumour Registry over the past 40 years. Results:With increasing tumour Enneking stage at presentation there was a significantly higher mortality (P=0.02). Patients with a higher Enneking stage also had an increased trend towards local recurrence (P=0.08). Stage did not 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. There was a trend towards a higher Enneking Stage in patients presenting with a longer duration of symptoms (P=0.1). 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 stage IIA, 56% at 5 and 10 years for stage IIB and 0% at 5 years for stage III. Discussion:This study re-emphasises the potential importance of a diagnostic delay on outcome. Longer symptom duration is associated with a higher Enneking stage at presentation. In turn a higher presenting stage results in a higher mortality. Pain and swelling are prominent clinical findings at first presentation in upper extremity Ewing's.