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Leiomyosarcoma of bone is just one of the variants of spindle cell sarcoma of bone characterised by the expression of desmin and other markers indicating a significant element of smooth muscle in the tumour, without osteoid production we have investigated the management and outcome of this rare type of primary malignant bone tumour. Method. Retrospective review of data stored on a prospective database. Results. In a database of 3364 patients with primary malignant bone sarcomas, 31 patients were identified with a primary leiomyosarcoma of bone. There were 12 males and 19 females with a mean age of 46 and tumour size of 8 cm. The most common site was the distal femur followed by the proximal tibia. Treatment was with chemotherapy and surgical resection. Seven of the patients had metastases at diagnosis. Surgery was carried out in 28 patients, 8 having amputation and 20 limb salvage. Three patients developed local recurrence, but half developed metastases. All patient disease-specific survival was 57% at five years and 44% at 10 yrs but for those without metastases was 82% and 60%, respectively. The only prognostic factors were metastases at diagnosis. Conclusion. Leiomyosarcoma of bone is a very rare primary malignant bone tumour affecting a predominantly older population. Despite the high incidence of metastases, survival is better than for other bone sarcomas for those without metastases at diagnosis.

Low-grade central osteosarcoma (LGCO) is a rare variant of osteosarcoma which is difficult to diagnose. If not treated appropriately, the tumour can recur with higher-grade disease. We reviewed our experience of this condition to try and identify factors that could improve both diagnosis and outcome. 18 patients out of 1540 osteosarcoma cases (over 25 years) had LGCO (1.2%). Only 11 patients (61%) were direct primary referrals. Almost 40% (7 of 18) cases were referred after treatment elsewhere when the diagnosis had not been made initially and all presented with local recurrence. Of the 11 who presented primarily, the first biopsy was diagnostic in only 6 (55%) cases. Of the remaining cases, up to three separate biopsies were required before a definitive diagnosis was made. Overall survivorship at 5 years was 90%. 17 patients were treated with limb salvage procedures, and one patient had an amputation. The diagnosis of LGCO remains challenging due to the relatively nonspecific radiological and histological findings. Since treatment of LGCO is so different to a benign lesion, accurate diagnosis is essential. Any difficult or nondiagnostic biopsies of solitary bone lesions should be referred to specialist tumour units for a second opinion.

Background. This study aimed to investigate prognostic factors for patients with myxoid/round-cell liposarcoma (MRCLS), in particular the significance of the round cell component, and to identify metastatic patterns as well as possibly suggest a suitable strategy for followup. Methods. Clinical, morphologic, and follow-up data from 160 patients with MRCLS was reviewed and statistically analysed. Results. Of 130 tumours with the round cell component evaluated, 61 had no round cell component, 27 had <5% round cell component, and 42 had >5%. All patients underwent surgical excision, 15 requiring amputation. 107 patients received adjuvant radiotherapy. Local recurrence occurred in 19 patients (12%), predominantly in patients with marginal or intralesional margins and a round cell component. Overall disease specific survival was 75% at 5 years and 56% at 10 years and was related to the proportion of round cell component. Of 52 patients who developed metastases, 38 (73%) had purely extrapulmonary metastases. We could not identify any factors predicting the site of metastases developing. Conclusions. The occurrence of any round cell component is the most important adverse prognostic factor for patients with MRCLS; patients with >5% round cell component are at higher risk of local recurrence, metastasis and tumour-related death and should be considered for adjuvant radiotherapy and possibly chemotherapy. The best method of monitoring extrapulmonary metastases remains to be established.

Introduction. The ''two-week wait" was established as a potential means of diagnosing malignant tumours earlier. This paper investigated whether these clinics are leading to earlier diagnosis of malignant soft-tissue lumps. Method. We identified all referrals to our centre from a database over a 4-year period. Results. 2225 patients were referred to our unit for investigation of a soft-tissue mass. 576 (26%) were referred under the ''two-week wait" criteria. 153 (27%) of which were found to have a malignant or borderline malignant diagnosis. 1649 patients were referred nonurgently. 855 (52%) of which were diagnosed with a malignant or borderline lesion. The average size at diagnosis was 9.4 cm with no difference in size between the different referral routes. Conclusion. There is little evidence that the two-week wait clinic is leading to earlier diagnosis of soft-tissue sarcomas with the majority still being referred nonurgently.

Between 1996 and 2006 a total of 278 patients with soft tissue Leiomyosarcoma were treated at our centre. We identified 16 patients (5.8%) where the tumour directly arose from the blood vessels. These tumours were studied to determine their prognosis and behaviour. All tumors were in the lower limbs: 11 from the femoral vein, 3 popliteal vein, and 2 from the posterior tibial vein. Mean tumour size was 10.4 cm (3 to 33). Histological grade was high in all patients. Surgical treatment was amputation in one, excision with or without vascular reconstruction in 12 followed by radiotherapy, and 3 patients had no surgery because of advanced disease at diagnosis. Seven out of the 16 patients (44%) had metastasis at diagnosis, and five patients without metastasis at diagnosis rapidly developed metastases at a median time of 5 months from diagnosis (2-30 months). The overall survival of the patients at 5 years was 25% which was considerably worse than those with nonvascular leiomyosarcoma. We conclude that patients with leiomyosarcoma of vascular origin have a very high risk of metastases and poor prognosis when treated in the conventional way.

We have retrospectively analysed the experience of a musculoskeletal oncological unit in the management of adult head and neck soft tissue sarcomas from 1990 to 2005. Thirty-six patients were seen, of whom 24 were treated at this unit, the remainder only receiving advice. The median age of the patients was 46 years. Most of the sarcomas were deep and of high or intermediate grade with a median size of 5.5 cm. Eleven different histological subtypes were identified. Wide excision was possible only in 21% of the cases. 42% of the patients developed local recurrence and 42% developed metastatic disease usually in the lungs. Overall survival was 49% at 5 years. Tumour size was the most important prognostic factor. Adult head and neck soft tissue sarcomas have a high mortality rate with a high risk of local recurrence and metastatic disease. The rarity of the disease would suggest that centralisation of care could lead to increased expertise and better outcomes.

Elastofibroma dorsi is an uncommon benign soft tissue pseudotumour usually located at the lower pole of the scapula, deep to serratus anterior, and often attached to the periosteum of the ribs, presenting with long history of swelling and occasionally pain and discomfort. This lesion is usually seen in patients over the age of 50 years and is not uncommonly mistaken as a malignant tumour because of its size and location deep to the periscapular muscles. Review of the orthopaedic oncology database of 17 500 patients revealed that there were 15 patients with elastofibroma dorsi. There were 12 males and 3 females, mean age at diagnosis of 68.4 years range 51-79 years. The diagnosis was confirmed by MRI in 3 patients, excision biopsy in 3 patients, trucut biopsy in 8 patients and open biopsy in 1 patient. Eight patients had excision of the lesion which was symptomatic. There have been no recurrences. We highlight the clinical and radiological presentation of elastofibroma dorsi to increase awareness of its existence and management.

Purpose: Periosteal osteosarcomas are rare cartilage-rich bone tumours characterized by a juxtacortical eccentric position and are normally regarded oncologically as of intermediate to high grade.Their low incidence is mirrored by a small number of reported cases in the world literature. While there is general agreement that wide surgical excision is required, there is a paucity of evidence regarding adjuvant therapy. Previous reports have not indicated any consistent approach to this to allow appraisal., Patients and Methods: We report 17 cases treated at our centre over 16 years. Our policy was to use chemotherapy when the tumour showed any features of high grade., Results: To date, no deaths have resulted from recurrence or metastasis of the tumour although there have been two deaths from other causes., Discussion: Comparison of survival with existing studies is made to draw conclusions regarding future treatment of this condition in terms of surgical and adjuvant approaches.

Purpose Limb salvage surgery of soft tissue sarcomas is associated with both a risk of local recurrence and wound complications. Although the lower limb appears to be at greater risk of wound-related morbidity, few studies separate anatomical compartments. We believe that the adductor compartment of the thigh has a particularly high rate of complications and so performed a retrospective analysis of all soft tissue sarcomas arising in this region undergoing limb salvage.Patients Patients with intermediate and high grade adductor compartment tumours were identified from our database and the case notes were reviewed for patient, tumour, surgical and wound variables, identifying those with wound complications both before and after discharge.Results Of 49 patients who underwent limb salvage surgery, 22 (42.9%) developed complications. Twelve patients (24.5%) required further surgery prior to wound healing and 10 patients had delays in post-operative radiotherapy. There were significant differences in the rates of preceding surgery, open biopsy performed at other centres and previous radiotherapy to this region between the complicated and uncomplicated groups.Discussion The management of these difficult tumours carries a high rate of wound complications and requires careful planning prior to tissue biopsy. Open biopsies should be performed by the tumour surgeon to allow easy inclusion of this site in the definitive procedure. In previously irradiated or operated limbs, alternative strategies for wound management may need to be considered.

Method. We performed a two-stage vascularized ipsilateral fibular graft transfer for segmental tibial defect following excision of malignant bone tumours.Results. We report 10 patients who had this procedure with an average follow-up of 116 months.The graft was transposed medialy on its vascular pedicle by two-stage surgery. Full weight bearing was achieved in six patients at 8-43 months postoperatively, but every patient had a significant complication.Discussion. The use of this method in isolation is not recommended for reconstruction of the tibia following tumour excision.

Purpose. Transmission of malignant tumour cells to a bone graft donor site is a rare complication of bone grafting.We report a case of seeding of malignant fibrous histiocytoma from the femur to a pelvic bone graft donor site.Discussion. We review the literature, discuss the possible mechanism of tumour transfer and offer advice aimed at avoiding this complication.

Purpose. Clear cell chondrosarcoma is a rare variant of chondrosarcoma. Six cases are herein reported.Subjects. We have treated six patients with clear cell chondrosarcoma in the past 28 years, representing 1.6% of all chondrosarcomas seen in this time period.Results and Discussion. Half the patients had been initially underdiagnosed and inappropriately treated.Conclusions. Our results and our review of the literature highlight the fact that inadequate initial treatment leads to a high rate of both local recurrence and metastasis whilst wide initial excision is usually curative.

Unlabelled: Purpose Paget' s osteosarcoma has a fearful reputation with a quoted survival of at best 5% at 5 years.We therefore reviewed our experience of 26 patients treated over the last 25 years using modern staging and limb salvage techniques to see if there had been any improvement in survival., Subjects: We identified 26 patients on the Royal Orthopaedic Hospital Oncological database with a diagnosis of sarcoma secondary to Paget's disease., Results: The survival rate was 53% at 1 year, 25% at 2 years and no patient survived for 5 years.The median survival was 21 months for those treated with curative intent and 7 months for those treated palliatively. Four of the five patients treated with limb-sparing surgery developed local recurrence between 5 and 12 months, the fifth died at 14 months.There was no difference in survival between amputation and limb salvage., Discussion: The development of sarcomatous change in Paget's disease is well recognised. It represents an important segment of primary bone tumours in patients over 40 years of age.The prognosis is appalling. Indeed only 15 of 368 cases (4%) from a number of historical series have survived more than 5 years. Our results are similarly disappointing with no survivors at 5 years despite modern methods of management of bone tumours.While there is no difference in local recurrence rates or survival between limb reconstruction and limb ablation the poor prognosis for both means that neither can be recommended at present. Sarcomatous change in Pagetoid bone should therefore be regarded as a different disease to primary osteosarcoma. It remains an incurable disease with a poor prognosis.

Purpose. The purpose of this study was to analyse the height at diagnosis and growth in 72 skeletally immature children who had been treated for osteosarcoma in the area of the knee.Subjects. Of the patients, the average age at diagnosis was 10 years in girls and 12 years in boys. All children received neo-adjuvant chemotherapy, and had limb salvage by endoprosthetic replacement.Results and conclusion. The results of this study indicate that there is no evidence that children with osteosarcoma are taller at diagnosis than their normal counterparts. However, there was a marked retardation in growth in the year following the administration of cytotoxic chemotherapy. There were 19 children who reached skeletal maturity. The final height in these children was not significantly different from the normal population.

Patient. We describe a case of chondroblastoma of the os calcis which metastasized to the tibia, soft tissues and lung. A complete response of the lung lesions was noted with chemotherapy.Discussion. Review of the published literature shows that metastatic chondroblastoma only arises following local recurrence of the tumour.

Purpose. The aim of this paper was to assess the growth in the unaffected lower limb of children who had received chemotherapy for a malignant primary bone tumour around the knee.Subjects/methods. Following diagnosis, all children (45, of which 32 were boys and 13 were girls) were staged. If limb-salvage surgery was thought appropriate, measured radiographs of both legs was performed, the bone age was estimated and the expected growth in the femur and tibia was calculated according to Tupman. These procedures were repeated at follow-up and the data plotted. Regression and correlation coefficients were also calculated.Results. The observed regression line in boys was almost identical to Tupman's curve. However, the observed growth in girls was larger than the expected growth.Discussion. It is recommended that the regression lines presented here are used in the calculation of the expected growth in the lower limb of children who have received chemotherapy for a malignant primary bone tumour, especially in girls.