Purpose of study: To test the hypothesis that previous hip involvement leads to earlier presentation and a better outcome for the contra-lateral hip in bilateral Legg-Calve-Perthes’ disease (LCPD).

Method: Case notes and radiographs of 250 patients with LCPD treated by a single surgeon between 1984 and 2003 were retrospectively reviewed. Thirty three patients (4 girls: 29 boys) with a minimum 1 year follow-up were identified with bilateral involvement from a prospectively collected database. Patients were grouped according to age at presentation (Group A-< 6 years; Group B- 6–8 years; Group C-> 8 years). All radiographs were reviewed and consensus was obtained on the presenting Waldenstrom stages. The severity of disease was rated by Catterall and lateral pillar classifications. The outcome was determined by the Stulberg classification. The right hip was the first affected in 25 of the 33 hips.

Results: These are summarized below.

Conclusions: The present report, with 33 patients, is the second largest series of patients with bilateral LCPD to our knowledge. The second hip involvement was milder than the first, but the improvement in outcome was statistically insignificant.

Introduction: Pigmented villonodular synovitis is an uncommon, benign, proliferative, neoplastic process of the synovial membrane presumed to be of histiocytic origin and is likely to cause diagnostic dilemma. We present 4 cases with varied presentations in the form of increasing groin pain, inguinal mass, co-existing osteoarthritis which were subsequently confirmed to have PVNS.

Methods and results: Clinical records and imaging modalities of 4 patients with histologically confirmed Pigmented villonodular synovitis of the hip, accrued from Scottish Bone Tumour Registry between 1969 and 2000 were reviewed.

Discussion: PVNS of the hip is an important differential diagnosis when osteoarthritis is associated with atypical clinical picture or lytic lesions. Although it remains confined to the joint, soft tissue masses extending beyond the capsule in to retroperitoneum or anterior and posterior aspects of hip have been reported as shown here. Radiographs in early stages are normal or include a concentric joint space narrowing. MR is an important non-invasive modality for surgical planning and to define the size and extent of the lesion, recurrence, delineating between synovial proliferations and periarticular or intra-abdominal organs. The role of arthroscopy, both diagnostic and therapeutic, is rapidly emerging, although, it has its own limitations. A carefully performed total excision often prevents recurrence as can be seen in this series.

Objective: To analyse long term outcome of pedicled patellar implantation in treating the defect of the knee joint after the excision of giant cell tumor of distal femur.

Methods: The geometry of patella has lead to its use for condylar reconstruction following resection for giant cell tumour around knee. 15 such patients were treated at our institute and followed up for a mean period of 7.3 years. Average age at presentation was 14.8 years. All patients had plain x-rays, angiography as needed, CT scan and MRI to check integrity of articular cartilage and ACL. Size of lesion was measured on CT. Predominant lateral condyle involvement was found in 6 and medial in 9 patients. The patella was dissociated with a slip of quadriceps attached to its proximal superior-medial pole and rotated to place it horizontally in the zone of resection. Firm osteosynthesis to intact condyle and autogenous bone grafting was carried out.

Results: Grafts consolidated at a mean of 10.8 months. All joints were fairly stable and 70% had movement of 90 degrees without pain. 11 patients had excellent outcome with ability to carry on occupations involving manual labour. There were reoperations for 2 deep infections, 1 recurrence leading to arthrodesis, 1 supracondylar fracture, 2 arthrolysis and 1 valgus osteotomy. 4 patients had arthritic changes and extensor leg at 6 years follow up. No AVN changes or extensor mechanism problems occurred. Using chi square test for log rank analysis significant relationship was found between size of lesion and range of motion (P=0.03) as well as articular cartilage grading and development of arthrosis. The rate of healing was faster than other series reporting free patella grafting.

Conclusion: Patellar implantation is a suitable way to repair the defect of the knee with better immediate functional results subsequently delaying eventual prosthesis surgery.

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.

Limb salvage surgery includes all of the surgical procedures designed to accomplish removal of a malignant tumour and reconstruction of the limb in order to achieve an acceptable oncological, functional, and cosmetic result. The aim of this study was to evaluate the functional outcome following endoprosthetic replcament for primary upper & lower extremity musculoskeletal neolplasms.

Between 1983 and 2004, we found 68 patients from the Scottish Tumor Register having had an endoprosthetic reconstruction for upper and lower-extremity malignant musculoskeletal tumours, of which 32 were alive for performing functional assessment (lower extremity-26, upper extremity-6). The clinical, radiological and oncological outcomes were evaluated. The functional outcome was measured by the Musculoskeletal Tumor Society and Toronto Extremity Salvage Score.

The average follow-up was 59 months (range, 1 to 21 years). There were 19 female and 13 male patients. These were anatomically distributed as around the knee (n=18 cases), hip (n=8) and shoulder (n=4). The most common diagnosis was chondrosarcoma (n=10) and osteosarcoma (n=11). Most of the patients were completely satisfied with their condition, with a decreased walking distance as the only notable restriction. There was no correlation between the functional outcome and the type or site of resection. Complications occurred in ten cases, including two cases of aseptic loosening and one case each of recurrent instability, sciatic nerve palsy and femoral nerve palsy. The median functional score using the Musculoskeletal Tumour Society system was 56% and Toronto Extremity Salvage Score was 72%.

Limb salvage for malignant musculoskeletal tumours continues to pose therapeutic and oncological challenges with considerable functional issues, but the good function and local tumour control in most patients justifies its continued use.

Primary lymphomas of bone are uncommon malignancies with involvement of spine, long bones, pelvis, ribs and skull in decreasing order of frequency. We describe an unusual case who presented with recurrent knee effusions turned out to be a high grade B-cell non-Hodgkin lymphoma.

A 34-year-old man is presented with painful recurrent knee effusions without any systemic aberration. Multiple aspirations were carried out to help knee pain and swelling with immediate response, but had negative cytology. Hematological and biochemical parameters were normal. Plain radiographic examination of the knee failed to show any bony abnormalities. An MRI scan of the knee showed extensive marrow oedema of the distal femur with breach in the anterior cortex. Subsequently, open biopsy of the lesion was carried out which confirmed the diagnosis of a high grade B-cell Non-Hodgkin lymphoma. Staging CT-scan of the chest, abdomen and pelvis failed to show any other lesions elsewhere. He was subsequently treated with the help of multi-drug chemotherapy and radiotherapy. At 7 months post treatment, the patient had no relapse and a repeat MRI at 7 months showed reduction in the size of lesion.

This case illustrates the limitations of plain radiograph and the usefulness of MRI in the diagnosis of bony lymphoma. The diagnostic dilemmas while investigating recurrent knee effusions due to primary skeletal Non Hodgkin lymphoma should be resolved by timely MRI scan and histological diagnosis.

Background: Extraarticular distal radius fracture is the second most common osteoporotic fracture seen in the elderly patients.

Purpose: To establish relationship between radiological parameters and final functional outcome in conservatively treated displaced extraarticular distal radial fractures in elderly patients.

Methods: Twenty-two wrists with displaced extraar-ticular distal radial fractures in twenty sedentary, low demand elderly patients treated with manipulation under anaesthesia and plaster application between May 1999 and June 2000. The case notes and radiographs of these patients were assessed retrospectively and subjective outcome was evaluated with validated DASH Questionnaire at 3 years post- reduction. Overall satisfaction, ability to return to the previous level of activity and concern over wrist appearance was further analyzed. Only those patients with more than 5 mm of shortening and more than 15 degrees of dorsal angulation at initial radiographs were included. Results were analysed using Pearson Correlation Sig.(2 –Tailed) formula.

Results: There were 16 female and 4 male patients with a mean age of 71 years. The mean follow-up was 3 years. DASH score of less than 25 was seen in 14, between 25-50 in 3 and between 50-75 in 3 patients with mean of 21.426 and standard deviation of 22.353. Despite residual deformity in some patients, there was high degree of patient satisfaction consistent with low level of DASH score found in 71% patients. It was also noticed that Males in the study group were younger and have lower dash scores. Patients with more than 5 mm of shortening and more than 15 degrees of dorsal angu-lation at initial radiographs showed no adverse correlation with subjective outcome. Statistical analysis of the results confirmed that higher the age lower the DASH score there by better functional result.

Conclusion: It was noted that the higher the age the lower the dash score. Radiological picture and functional outcome are found to be two independent variables in the elderly subgroup of patients with displaced extrar-ticular distal radius fractures. Nonoperative treatment yields satisfactory results with high patient satisfaction rate and is advocated in elderly patients.

Does the type of implant have any correlation with critical fusion time of hallux metatarsophalangeal joint? There are few cadaveric biomechanical studies published in the literature assessing the strength and rigidity of different fixation methods. Although it is still unclear whether the amount of metal affects the fusion rate, the aim of this study was to assess whether using a supplementary dorsal ¼ tubular plate in addition to a compression screw gives any added rigidity to the fusion area leading to an earlier fusion.

A retrospective analysis was conducted on the first metatarsophalangeal joint fusion in 26 consecutive patients (34 feet) between April 1998 to February 2002 comparing using single screw versus a screw supplemented with a dorsal ¼ tubular plate. There were 18 females and 8 males with a mean age of 51.5 years and a mean follow-up of 2.9 years. The final fusion was assessed clinically and radiologically by trans-articular trabeculation.

There was a fusion rate of 97%. All patients except one had solid fusion. One case had non-union. Four cases had superficial wound infection, which settled down with appropriate antibiotic therapy. Paraesthesia over the dorso-medial aspect of the big toe in three patients and transfer metatarsalgia in two patients were documented.

The type of implant did not show any direct correlation with the complication rate. There is no evidence to suggest in this study relating the amount of implant to final outcome. Therefore the choice of implant fixation can be at the discretion of the operating surgeon.

Necrotising fasciitis is a rapidly spreading, life-threatening condition. We report a case of necrotising fasciitis in a 71-year-old woman after a primary total hip replacement which became deeply infected as a result.

There are few reports describing dislocation of the metacarpophalangeal joint of the thumb in children. This study describes the clinical features and outcome of 37 such dislocations and correlates the radiological pattern with the type of dislocation.

The mean age at injury was 7.3 years (3 to 13). A total of 33 children underwent closed reduction (11 under general anaesthesia). Four needed open reduction in two of which there was soft-tissue interposition. All cases obtained a good result. There was no infection, recurrent dislocation or significant stiffness.

So-called ‘simple complete’ dislocations that present with the classic radiological finding of the joint at 90° dorsal angulation may be ‘complex complete’ injuries and require open reduction.

Introduction: The thumb metacarpophalangeal (MCP) joint dislocations in children are relatively uncommon and scarcely described in the English literature. The aim of this study was to report the clinical course and outcome of traumatic dislocations of the thumb metacarpophalangeal joints in children.

Materials and methods: We retrospectively reviewed a cohort of 37 traumatic dislocations of the thumb metacarpophalangeal joints in 37 children between 1990 and 2005. All patients were treated by five orthopaedic surgeons at a tertiary referral children’s hospital. The outcome measures included patient demographics, method of reduction and short-term outcome. The mean follow-up was 6 weeks.

Results: The mean age at injury was 7.3 years. These occurred predominantly in boys (78.3%) and were dorsal dislocations in 97.2%. Thirty-three presented acutely on the day of injury, while 3 within 1–2 weeks. Four patients needed open reduction with or without temporary stabilisation. Thirty-three had a closed reduction (under general anaesthesia-12, under ring block-5, under sedation-9 and without anaesthesia-7). All patients undergoing closed or open reduction under anaesthesia had 1–4 unsuccessful relocation attempts. Two of four open reductions revealed soft tissue interposition of volar plate and flexor pollicis longus. Post-reduction, the thumb was immobilised in a thumb spica or plaster for 2 to 3 weeks period. All gained good result. There were no infections, recurrent dislocation or gross stiffness.

Conclusion: Thumb metacarpophalangeal (MCP) joint dislocations in children are mostly dorsal and managed non-operatively in majority with satisfactory outcome. Irreducible dislocations may need open reduction due to volar plate and flexor pollicis longus tendon interposition.

Introduction: There is paucity of literature describing complex lateral condylar mass (LCM) fractures of the elbow in children, which we define as a LCM fracture occurring concurrently with another fracture or dislocation in the same elbow. The aim of this study was to evaluate the management, outcome and complication rate of 26 complex LCM fractures and to analyse difference in the outcome between the isolated and complex LCM fractures.

Materials and methods: Between 1990 and 2005, we identified 26 complex LCM fractures in the departmental database (1% of 2502 elbow/humeral injuries). Information was collected from theatre-charts, casenotes and radiographs. The mean follow-up was 5.9 months (range, 6 weeks to 4 years).

Results: These were complex because of their association with elbow dislocation (n=12; mean age 8.2 years), olecranon fracture (n=8; mean age 4.1 years) and medial condylar fracture (n=6; mean age 8 years). Nine were treated conservatively. The remaining 17 were fixed with K-wires (9), a screw (7) or both (1). A concomitant elbow dislocation was managed by closed reduction followed by open K-wiring or screw fixation of the LCM fracture. An associated olecranon fracture was treated non-operatively for minimally displaced fractures, although one needed internal fixation. All displaced T-condylar fractures required open reduction and internal fixation. There were no complications of non-union, mal-union, avascular necrosis, cubitus valgus or tardy ulnar palsy. Healing and return of normal function occurred in all, although six patients had minor loss of extension.

Conclusion: We found no obvious difference in the outcome between the isolated displaced LCM fractures described in the literature and our complex LCM group. However the importance of careful assessment of the preoperative radiographs and testing of elbow stability by examination under anaesthesia is stressed.

Introduction: There remains little evidence to discern whether K-wires or screws have different outcomes in the management of lateral condylar mass (LCM) fractures in children. We studied 77 displaced (Jacob types II and III) fractures of the lateral humeral condyle in 77 children in order to infer the relative benefit of one strategy over another.

Materials and methods: Between 1995 and 2005, we identified 77 LCM fractures in the departmental database. Information was collected from theatre-charts, casenotes and radiographs. We analysed demographic data, fracture features, treatment modalities, complications, and clinical and radiographic results. We excluded all complex LCM associated with elbow dislocations, olecranon fractures and bi-condylar fractures. The mean follow-up was 5.3 months (range, 6 weeks to 3 years).

Results: We reviewed the results of screw osteosynthesis (n=44) versus K-wire (n=33) at an average age of 5.3 years (range, 8 months to 10.9 years). There were 49 boys and 28 girls. The average interval between the injury and the operation was 1.6 days. The mean duration of implant removal was 3.6 weeks (for K-wires, removed without anaesthesia) and 20.7 weeks (for screws, removed under general anaesthesia). There was no non-union in this series. None of the patient needed a revision of osteosynthesis. Superficial wound infection (all K-wires) was found in three patients, which was completely settled with antibiotic therapy. One patient had cubitus valgus deformity (screw), which required a corrective osteotomy. Loss of range of motion of 10–50° was found in 6 cases (3 in each group).

Conclusions: Based on our observations, we believe that K-wire fixation had comparatively similar outcome to screw fixation, although, this necessitates a second procedure for removal of screw.

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.

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.

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.