We describe a case of oncogenic osteomalacia in an adult male who presented with low back pain and bilateral hip pain. Extensive investigations had failed to find a cause. A plain pelvic radiograph showed Looser’s zones in both femoral necks. MRI confirmed the presence of insufficiency fractures bilaterally in the femoral head and neck. Biochemical investigations confirmed osteomalacia which was unresponsive to treatment with vitamin D and calcium. A persistently low serum phosphate level suggested a diagnosis of hypophosphataemic osteomalacia. The level of fibroblast growth factor-23 was highly raised, indicating the cause as oncogenic osteomalacia. This was confirmed on positron-emission tomography, MRI and excision of a benign fibrous histiocytoma following a rapid recovery. The diagnosis of oncogenic osteomalacia may be delayed due to the non-specific presenting symptoms. Subchondral insufficiency fractures of the femoral head may be missed unless specifically looked for.
Disarticulation of the hip in patients with high-grade tumours in the upper thigh results in significant morbidity. In patients with no disease of the proximal soft tissue a femoral stump may be preserved, leaving a fulcrum for movement and weight-bearing. We reviewed nine patients in whom the oncological decision would normally be to disarticulate, but who were treated by implantation of an endoprosthesis in order to create a functioning femoral stump. The surgery was undertaken for chondrosarcoma in four patients, pleomorphic sarcoma in three, osteosarcoma in one and fibrous dysplasia in one. At follow-up at a mean of 80 months (34 to 132), seven patients were alive and free from disease, one had died from lung metastases and another from a myocardial infarction. The mean functional outcome assessment was 50 (musculoskeletal tumor society), 50 and 60 (physical and mental Short-form 36 scores). Implantation of an endoprosthesis into the stump in carefully selected patients allows fitting of an above-knee prosthesis and improves wellbeing and the functional outcome.
Limb salvage involving wide resection and reconstruction is now well established for managing musculoskeletal sarcomas. However, involvement of major nerves and vessels with a large volume of muscle and skin may result in a useless limb, contributing to depression and a low quality of life. We have been studying alternative treatments for musculoskeletal sarcoma since 1990, and have recently established a regime using photodynamic surgery with cells labelled with acridine orange, photodynamic therapy with cells treated similarly and radiodynamic treatment using the effect of X-rays on such cells. These techniques have been used after marginal or intralesional resection of tumours since 1999 and have enabled maintenance of excellent limb function in patients with sarcomas.
Primary giant-cell tumour of soft tissue arising in the ligamentum teres has not been previously described. We report a case of such a tumour in a 46-year-old woman. The lesion was only detected at the time of hip arthroscopy despite pre-operative MRI being performed. It was successfully excised arthroscopically with resolution of the symptoms.
This study reports the experience of one treatment centre with routine surveillance MRI following excision of musculoskeletal sarcoma. The case notes, MRI and histology reports for 57 patients were reviewed. The primary outcome was local tumour recurrence detected on either surveillance MRI in asymptomatic patients, or interval MRI in patients with clinical concern. A total of 47 patients had a diagnosis of soft-tissue sarcoma and ten of a primary bone tumour. A total of 13 patients (22%) had local recurrence. Nine were identified on a surveillance scan, and four by interval scans. The cost of surveillance is estimated to be £4414 per recurrence detected if low-grade tumours with clear resection margins are excluded. Surveillance scanning has a role in the early detection of local recurrence of bone and soft-tissue sarcoma.
We evaluated the oncological and functional outcome of 27 patients who had limb salvage for a soft-tissue sarcoma of the foot or ankle between 1992 and 2007, with a mean follow-up of 7.5 years (1.05 to 16.2). There were 12 men and 15 women, with a mean age at presentation of 47 years (12 to 84). Referrals came from other hospitals for 16 patients who had previous biopsy or unplanned excision, and 11 presented de novo. There were 18 tumours located in the foot and nine around the ankle. Synovial sarcoma was the most frequent histological diagnosis. Excision was performed in all cases, with 16 patients requiring plastic surgical reconstruction with 13 free and three local flaps. Adjuvant treatment was undertaken in 20 patients, 18 with radiotherapy and two by chemotherapy. Limb salvage was successful in 26 of the 27 patients. There have been two local recurrences and two mesenchymal metastases. Four patients have died of their sarcoma and two of other causes. Function was evaluated with the Toronto Extremity Salvage Score and a mean overall score of 89.40 (52.1 to 100) was obtained. A questionnaire revealed that all surviving patients are able to wear normal shoes and none require a walking aid. Limb salvage can achieve good oncological and functional results with additional treatment.
As metal-on-metal arthroplasty becomes more widespread, concerns are being raised about the potential dangers of metal particulate debris. We present the case of a benign psoas mass secondary to the presence of such particles. The mass was excised and the hip resurfacing subsequently revised to a total hip replacement.
Dupuytren’s disease of the hand has only been rarely reported in children and is rarer still in infants. We report a case in a six-month-old infant who required surgery when aged ten months. Histology confirmed the diagnosis of Dupuytren’s disease.
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.
In developing countries locally-made low-cost prostheses are mainly used in limb-salvage surgery to alleviate the economic burden. We retrospectively collected data on 104 patients treated by limb-salvage surgery between July 1997 and July 2005. We used a locally-designed and fabricated stainless-steel endoprosthesis in each case. Oncological and functional outcomes were evaluated at a mean follow-up of 47 months (12 to 118). A total of 73 patients (70.2%) were free from disease, nine (8.7%) were alive with disease, 19 (18.2%) had died from their disease and three (2.9%) from unrelated causes. According to the Musculoskeletal Tumor Society scoring system, the mean functional score was 76.3% (SD 17.8). The five-year survival for the implant was 70.5%. There were nine cases (8.7%) of infection, seven early and two late, seven (6.7%) of breakage of the prosthesis, three (2.9%) of aseptic loosening and two (1.9%) of failure of the polyethylene bushing. Multivariate analysis showed that a proximal tibial prosthesis and a resection length of 14 cm or more were significant negative prognostic factors. Our survival rates and Musculoskeletal Tumor Society functional scores are similar to those reported in the literature. Although longer follow-up is needed to confirm our results, we believe that a low-cost custom-made endoprosthesis is a cost-effective and reliable reconstructive option for limb salvage in developing countries.
Despite local treatment with systemic chemotherapy in Ewing’s sarcoma family tumours (ESFT), patients with detectable metastases at presentation have a markedly worse prognosis than those with apparently localised disease. We investigated the clinical, pathological and laboratory differences in 888 patients with ESFT, 702 with localised disease and 186 with overt metastases at presentation, seen at our institution between 1983 and 2006. Multivariate analyses showed that location in the pelvis, a high level of serum lactic dehydrogenase, the presence of fever and a short interval between the onset of symptoms and diagnosis were indicative of metastatic disease. The rate of overt metastases at presentation was 10% without these four risk factors, 22.7% with one, 31.4% with two, and 50% for those with three or four factors. We concluded that in ESFT the site, the serum level of lactic dehydrogenase, fever, and the interval between the onset of symptoms and diagnosis are indicators of tumours having a particularly aggressive metastatic behaviour.
Vertebral haemangiomas are usually asymptomatic and discovered fortuitously during imaging. A small proportion may develop variable degrees of pain and neurological deficit. We prospectively studied six patients who underwent eight surgical procedures on 11 vertebral bodies. There were 11 balloon kyphoplasties, six lumbar and five thoracic. The mean follow-up was 22.3 months (12 to 36). The indications for operation were pain in four patients, severe back pain with Frankel grade C paraplegia from cord compression caused by soft-tissue extension from a thoracic vertebral haemangioma in one patient, and acute bleeding causing Frankel grade B paraplegia from an asymptomatic vascular haemangioma in one patient. In four patients the exhibited aggressive vascular features, and two showed lipomatous, non-aggressive, characteristics. One patient who underwent a unilateral balloon kyphoplasty developed a recurrence of symptoms from the non-treated side of the vertebral body which was managed by a further similar procedure. Balloon kyphoplasty was carried out successfully and safely in all patients; four became asymptomatic and two showed considerable improvement. Neurological recovery occurred in all cases but bleeding was greater than normal. To avoid recurrence, complete obliteration of the lesion with bone cement is indicated. For acute bleeding balloon kyphoplasty should be combined with emergency decompressive laminectomy. For intraspinal extension with serious neurological deficit, a combination of balloon kyphoplasty with intralesional alcohol injection is effective.
We describe a method of reconstruction using tumour-bearing autograft treated by liquid nitrogen in 28 patients. The operative technique consisted of The limb function was rated as excellent in 20 patients (71.4%), good in three (10.7%), fair in three (10.7%), and poor in two (7.1%). At the final follow-up six patients had died at a mean of 19.8 months after the operation, while 21 remained free from disease with a mean follow-up of 28.1 months (10 to 54). One patient is alive with disease. Bony union was seen at a mean of 6.7 months after the operation in 26 patients. Complications were encountered in seven patients, including three deep infections, two fractures, and two local recurrences. All were managed successfully. Our results suggest that this is a simple and effective method of biological reconstruction.
We reviewed 20 patients who had undergone a Coonrad-Morrey total elbow arthroplasty after resection of a primary or metastatic tumour from the elbow or distal humerus between 1980 and 2002. Eighteen patients underwent reconstruction for palliative treatment with restoration of function after intralesional surgery and two after excision of a primary bone tumour. The mean follow-up was 30 months (1 to 192). Five patients (25%) were alive at the final follow-up; 14 (70%) had died of their disease and one of unrelated causes. Local control was achieved in 15 patients (75%). The mean Mayo Elbow Performance Score improved from 22 (5 to 45) to 75 points (55 to 95). Four reconstructions (20%) failed and required revision. Seven patients (35%) had early complications, the most frequent being nerve injury (25%). There were no infections or wound complications although 18 patients (90%) had radiotherapy, chemotherapy or both. The Coonrad-Morrey total elbow arthroplasty provides good relief from pain and a good functional outcome after resection of tumours of the elbow. The rates of complications involving local recurrence of tumour (25%) and nerve injury (25%) are of concern.
