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.

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.

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