Scottish Bone Tumour Registry Research Articles

Radiofrequency ablation of osteoid osteoma: outcomes from the West of Scotland

Osteoid osteoma is a classically described benign bone tumour. Traditionally, the surgical treatment of choice was excision, but this can have significant morbidity. Percutaneous radiofrequency ablation (RFA) has grown in popularity as an alternative treatment. This study reports the outcomes using this technique in our regional bone tumour unit. Between May 2003 and October 2007, 14 patients underwent CT-guided RFA. All patients were subsequently offered follow-up in the out-patient clinic. Outcomes were taken from the Scottish Bone Tumour Registry database. Eleven patients (78.6%) had complete resolution of symptoms after one RFA. Three (21.4%) cases were unsuccessful but one of these was due to technical failure. All three of the above patients had complete relief of symptoms after one further RFA. One (7.1%) patient initially had complete relief of symptoms, but suffered recurrence after 9 months. This patient also had a second curative treatment. Percutaneous RFA for osteoid osteoma is an attractive treatment due to its efficacy and low morbidity. Our results showed a primary success rate of 78.6%, a secondary success rate of 100% (after one additional procedure). Our results add to the growing literature supporting RFA as the preferred treatment for osteoid osteoma.

Primary Osseous Tumors of the Hindfoot: Why the Delay in Diagnosis and Should We Be Concerned?

Bony tumors of the foot account for approximately 3% of all osseous tumors. Diagnosis is frequently delayed as a result of lack of clinician familiarity and as a result of their rarity. The reasons for the delays, however, are unclear. We therefore determined (1) how hindfoot tumors present and the specific reasons for delay in diagnosis; (2) whether the spectrum of disease varies between the talus and calcaneus; and (3) how these patients were treated. We retrospectively reviewed the medical notes and imaging for all patients with 34 calcaneal and 23 talar tumors recorded in the Scottish Bone Tumour Registry. Demographics, presentation, investigation, histology, management, recurrence, and mortality were recorded. Hindfoot tumors present with pain and often swelling around the heel (calcaneus) or ankle (talus), most often misdiagnosed as soft tissue injury. Calcaneal lesions were more likely to be malignant than talar lesions: 13 of 34 versus three of 23. Clinicians should be aware that hindfoot tumors can be initially misdiagnosed as soft tissue injuries and suspicion of a tumor should be raised in the absence of trauma or persistent symptoms. Lesions affecting the calcaneus are more likely to be malignant. Early diagnosis and adjuvant therapy are important. Level IV, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.

Aneurysmal Bone Cysts of the Vertebrae

To review records of 14 patients with aneurysmal bone cysts (ABCs) of the spine. Using the Scottish Bone Tumour Registry for the period of October 1952 to November 2005, records of 9 females and 5 males aged 8 to 63 (mean, 25.3) years who had ABCs of the spine and were followed up for a mean of 7.1 years were reviewed. The most commonly involved site was the lumbar vertebrae (n=6), followed by the thoracic (n=4), cervical (n=3), and sacral (n=1) vertebrae. The mean duration of symptoms at presentation was 8.8 (range, 0.3-24) months. The symptoms included gradually increasing pain in the back (n=14), a palpable spinal mass (n=4), spinal deformity (n=2), and neurological deficits (n=5). All the patients underwent surgery: intra-lesional excision (curettage) without bone grafting (n=3), excision (n=7, 2 of whom had adjuvant radiotherapy), and open excisional biopsy (n=4, 2 of whom had iliac crest bone grafting). One patient with a cervical ABC underwent preoperative angiographic embolisation. Another patient with a sacral ABC underwent percutaneous sclerotherapy. Two patients had recurrence. One had recurrence within 4 months and underwent adjuvant radiotherapy; another had recurrence 16.8 years later and underwent repeat curettage. No major complications were encountered. Most ABCs of the spine occurred in young females. Intra-lesional excision was an effective treatment.

Primary bone tumours of the talus: The Scottish Bone Tumour Registry experience

Primary bone tumours of the talus are rare and the existing literature is limited. The aim of this study was to investigate the epidemiology of primary bone tumours affecting this uncommon site and suggest a management protocol for these tumours. We retrospectively reviewed the Scottish Bone Tumour Register from January 1954 to May 2010 and included all primary bone tumours of the talus. We identified only twenty three bone tumours over fifty six years highlighting the rarity of these tumours. There were twenty benign and three malignant tumours with a mean age of twenty eight years. A delay in presentation was common with a mean time from onset of symptoms to diagnosis of ten months. Tumour types identified were consistent with previous literature. We identified cases of desmoplastic fibroma and intraosseous lipodystrophy described for the first time. We suggest an investigatory and treatment protocol for patients with a suspected primary bone tumour of the talus.

Bizarre Parosteal Osteochondromatous Proliferation: A Locally Aggressive Benign Tumor

Bizarre parosteal osteochondromatous proliferation (BPOP) is a benign lesion of bone, and numerous questions remain unresolved regarding its etiology, diagnosis, and treatment. We present the Scottish Bone Tumour Registry experience of this rare lesion. We performed a retrospective analysis of the Scottish Bone Tumour Registry records. Histologic specimens were reexamined by a musculoskeletal pathologist. Radiographs were reevaluated by a musculoskeletal radiologist. From 1983 to 2009, 13 cases (13 patients; six male, seven female) were identified. Their ages ranged from 13 to 65 years. All patients presented with localized swelling. Pain was present in five. Antecedent trauma was present in two. Nine lesions affected the hand, three the foot, and one the tibial tuberosity. Twelve lesions were excised and one was curetted. There were seven recurrences of which six were excised. One lesion recurred a second time and was excised. There were no metastases. Radiographs showed densely mineralized lesions contiguous with an uninvolved cortex. Cortical breakthrough was present in one case and scalloping in another. Histologic analysis characteristically showed hypercellular cartilage with pleomorphism and calcification/ossification without atypia, bone undergoing maturation, and a spindle cell stroma. BPOP is a rare benign lesion that probably is neoplastic, with no gender predilection, and affecting patients over a wide age range. Previously trauma was considered an etiologic factor, but this no longer seems to be the case. The rate of recurrence was 50%, which may indicate a more extensive resection is required for this locally aggressive lesion. No metastases were reported. BPOP should not be mistaken for, or treated as, a malignant tumor. Level IV, retrospective case series. See Guidelines for Authors for a complete description of levels of evidence.

Pelvic Ewing's Sarcoma: A Review from Scottish Bone Tumour Registry

To review various treatment modalities on outcome of patients with pelvic Ewing's sarcoma. Between January 1948 and December 2004, 18 male and 15 female patients aged 3 to 48 (mean, 20) years with pelvic Ewing's sarcoma were retrospectively reviewed from the Scottish Bone Tumour Registry. The commonest site involved was the ilium. The main presenting symptom was pain, followed by swelling and restricted hip movements. Treatments included surgery, radiotherapy, chemotherapy, or any of them in combination. Patients who had no metastasis at presentation (p<0.01) and underwent chemotherapy with ifosfamide (p<0.01) had significantly longer survival. Age (p=0.09), gender (p=0.95), delay in presentation (p=0.31), tumour site (p=0.9), surgery (p=0.73), and radiotherapy (p=0.23) were not predictive of survival in the univariate analysis. The 5-year event-free survival rate in patients with no metastasis at presentation was 35%. Intense multi-agent neo-adjuvant chemotherapy including ifosfamide followed by wide excision and postoperative radiation are recommended. Local therapy should not take precedence over or interfere with systemic chemotherapy.

Survival trends in osteosarcoma of humerus

Management of osteosarcoma has evolved during the last 50 years. A review of 53 cases of osteosarcoma affecting the humerus included in the Scottish Bone Tumour registry from 1950 to 2000 is presented. Survival trends and the various factors influencing survival during this period are analysed. Patients were chronologically divided into two cohorts, one presenting before 1975 (18 patients) and the other presenting after 1975 (35 patients). The second half of the study period (1975 onwards) was characterized by availability of new treatment modalities like limb salvage and chemotherapy. A distinct improvement in survival was noted during the second half of the study (35% 5 years survival post 1975 vs. 18% pre 1975, P = 0.007). Survival and metastasis appeared to depend on multiple factors. Chemotherapy in isolation did not significantly affect survival. Limb salvage surgery did not have an adverse effect on survival rates. This improvement in survival could be due to a change in the natural history of the disease along with advances in diagnostics and therapeutics.

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.

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.

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.

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.

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.

Multifocal synchronous osteosarcoma: The Scottish bone tumour registry experience

We report 3 cases of multifocal synchronous osteosarcoma constituting 1% of osteosarcoma cases in the Scottish Bone Tumour Registry over 30 years. All were children and had a median age of 10 years. They all presented with multiple bony lesions in the absence of demonstrable pulmonary metastases. Treatment was with palliative radiotherapy in two and combination chemotherapy and radiotherapy in the most recent case. Survival was poor in all cases, with a median of 7 months. A proposal is made for genetic studies in future cases to help elucidate the origin of these tumours. The multicentric and metastatic hypotheses are discussed.

The Scottish Bone-Tumour Registry

The Scottish Bone-Tumour Registry