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

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Pelvic Ewing's sarcoma: a review from Scottish Bone Tumour Registry.

PURPOSE: To review various treatment modalities on outcome of patients with pelvic Ewing's sarcoma. METHODS: 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. RESULTS: 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%. CONCLUSION: 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.

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.

Paget sarcoma of the spine: Scottish Bone Tumor Registry experience.

STUDY DESIGN: Retrospective case study of 13 cases of Paget sarcoma of the spine accrued from a prospectively collected Tumor Registry database. OBJECTIVES: To analyze the clinical, radiologic, and histologic features of Paget sarcoma of the spine and to determine the factors influencing the prognosis. SUMMARY OF BACKGROUND DATA: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

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.

Disease incidence and results of extremity lesion treatment: mersey region soft tissue sarcomas (1975-1985).

Purpose. The incidence and treatment results of extremity soft tissue sarcoma (STS) in the Mersey Region, in the absence of a Multi-Disciplinary Unit, for the period 1975-1985, have been analysed.Subjects and methods. Data from cases presenting with STS within the Mersey region, from 1 January 1975 until 31 December 1985, were reviewed. Only patients with sarcoma of head and neck, thoracic wall, abdominal wall, retroperitoneum, limb girdle or extremity were included. Extremity lesions were staged according to the MTS system. Pathological data also were assigned a grade according to tumour differentiation, mitosis count and tumour necrosis. Data from patients with a minimum follow-up of 5 years were collated, and patterns of treatment failure were investigated. Finally, time to first occurrence was analysed.Results and Discussion. The incidence of STS in this study was identical to that reported by the US Department of Health in 1976. Five year survival rate for Stage I tumours was only 51.7% which compares very unfavourably with contemporary series from Multi-Disciplinary Units. Five year survival rate following wide local excision +/- adjuvant therapy is 52.4%, while that following amputation +/- adjuvant therapy is 45.5%. While not attaining the results reported by other centres, limb-sparing surgery does not appear to appreciably prejudice long-term survival.Conclusions. STS are rare in the UK, leading to poor classification and suboptimal treatment of lesions. It is important to establish multidisciplinary teams of surgeons, radiologists, radiotherapists and oncologists to plan and organise multimodality therapy for STS.

Diffuse intramuscular lipomatosis of a lower limb.

Patient. A 40-year-old man presented with a swelling of the left thigh which had been increasing in size over 10 months. Surgery confirmed a diagnosis of lipoma. After 6 months, another swelling appeared, this time in the left calf. Ultrasound-guided biopsies revealed that the tissue showed appearances consistent with intramuscular lipoma. No further surgery was performed and the man is to be reviewed regularly, with possible debulking if necessary.Discussion. This case presents an atypical case of lipomatosis. Magnetic resonance imaging is useful for assessing the extent of the lesion.

Bizarre parosteal osteochondromatous proliferation with cortical invasion.

A 15-year-old male presented with an 18-month history of increasing swelling on the radial aspect of his left forearm. Radiographs and MR images showed a partly calcified mass on the lateral border of the radius with erosion of the underlying cortex. Following a CT-guided needle biopsy, the mass was excised by segmental resection of the radial shaft with replacement by a vascularised fibular graft. Histological examination of the resected specimen showed many features typical of a bizarre parosteal osteochondromatous proliferation (BPOP) but with destruction of the underlying cortical bone by cellular spindle cell tissue. Although there were no cytological signs of malignancy, we believe that this unusual disease should be regarded as a particularly aggressive form of BPOP and treated by wide excision. Clinical follow-up of our patient at 2 years shows no sign of local recurrence or metastasis.