99 patients with new diagnosed soft tissue sarcomas involving the pelvic region were studied to determine the outcome and prognostic factors for survival and local recurrence. The mean age at diagnosis was 57 years. There were 55 males and 44 females. The mean tumor size was 12cm. The tumor was deep in 79 patients and superficial in 20. Surgical treatment was excision in 93 patients and hindquarter amputation in 6 patients. Histological grade was low grade in 23 and high grade in 75 patients. 7% of the patients had metastases at presentation. The 5 year overall survival was 57% and local recurrence occurred in 22% of the patients. The risk of inadequate surgical margins in patients with tumors within the pelvic brim was 50% compared to 18% for those with tumors located outside the pelvic brim. The significant predictors of local recurrence were inadequate margins and location of the tumor within the pelvic brim. Tumor size, grade and depth did not influence development of local recurrence. Significant predictors of survival included metastases at presentation, tumor grade and depth. The cumulative 5 year survival for patients with deep high grade tumors, deep low grade tumors, superficial high grade and superficial low grade tumors were 45%, 74%, 63% and 100% respectively (p=0.01). The 5-year overall survival was 66% in those patients without local recurrence compared to 37% in those who develop local recurrence (p=0.005). Multivariate analysis revealed that development of local recurrence was the most important determinant of overall and metastases free survival. We conclude that patients with pelvic soft tissue sarcoma who develop local recurrence have an extremely poor prognosis. Patients with high grade and inadequate surgical margins represent a particular group with very high risk of metastases and death even with radiotherapy and perhaps should be considered for other adjuvant treatment.
130 consecutive patients with metastastic tumours of the extremity bones treated with resection with or without major endoprosthetic reconstruction were studied retrospectively to determine the indication for surgery, complications, clinical outcome and oncological results of treatment. The mean age at diagnosis was 61 (22 – 84). The tumours originated from a variety of organs. Lower extremity was involved in 104 and upper extremity in 26. Metastatic disease was solitary in 55 patients and multiple in 75 at the time of surgery. The median follow-up possible from the time of operation to review was 48 months (0-103). The indication for surgery was radical treatment of solitary metastases with curative intent in 33, pathological fracture in 46, impending fracture in 27, failure of prior fixation devices in 17, painful swelling or extremity in 37. Surgical treatment included excision of expendable bones without reconstruction in 20 patients and resection with endoprosthetic reconstruction in 110 patients. 7 patients received adjuvant chemotherapy and the majority received adjuvant radiotherapy. At the time of review, 58 patients had died at a mean time of 23 months (0–90) from surgery (53 from progressive metastatic disease and 5 from other causes). 72 were alive at mean follow-up of 22 months (1–103) from surgery. 36 patients (28%) were alive at 2 years post-surgery and 8 (6%) at 5 years. One patient died intra-operatively. Post-operative complications occurred in 32 patients (25%). 18 patients required further surgical procedures for dislocation, infection haematoma, stiff joint, plastic surgical procedures. All the patients had control of pain and 90% achieved desired mobility. There was no difference in the survival of patients who presented with solitary and multiple metastases, renal and non-renal metastases, and upper or lower limb metastases.
The unusual phenomenon of histological grade change in locally recurrent soft tissue sarcomas is examined by retrospective review of a large sarcoma database. Increased histological grade was found to occur in 20% of recurrent tumours. Several possible factors predisposing to grade change were examined, and only the histologic diagnosis of myxoid malignant fibrous histiocytoma was found to be significant. Despite increased histologic grade, these tumours do not appear to have a worse prognosis in terms of developing systemic disease. Soft tissue sarcomas (STS) have a reported local recurrence rate of between five and thirty percent. Recurrent tumours are often similar histologically to the initial tumour, however they are occasionally of higher histological grade than the original lesion. Factors that predispose to this change in grade are not known. We sought to identify the frequency at which locally recurrent STS demonstrate a change in histological grade, and to investigate the possible factors leading to this change. We also investigate whether a change in grade is associated with a poorer prognosis. We identified one hundred and seventy-three patients who developed locally recurrent STS, one hundred and twenty-four of which met inclusion criteria and who will form the basis of this study. Ninety-two patients (74%) had no change in histological grade, twenty-four (19%) demonstrated an increase in histological grade and eight (7%) a decreased histological grade. Univariate analysis of time to local recurrence, histological diagnosis and use of radiotherapy and chemotherapy did not reveal significant differences between the groups who did and did not undergo change in grade. When the diagnosis of myxoid MFH was looked at separately, there was a higher proportion in the group that developed increased histological grade. Development of a change in grade was not associated with a poorer survival rate. Increase in histological grade occurs in approximately 20% of locally recurrent STS, but this phenomenon is not associated with a poorer prognosis than if the grade remains the same. A histological diagnosis of myxoid MFH predicts for an increase in histological grade.
Non-union of long bone fractures can be a challenging problem. There are several methods of treatment and they depend upon various patient factors, biology of non-union, and presence of infection. When faced with failure of treatment with biological reconstructive procedures patients have little choice. At our institute we have treated 10 such patients with radical excision and reconstruction using tumour endoprostheses as a last attempt to save the limb. Median age of the patients was 71 years (25–85). 2 patients were male and 8 were female. Median follow-up was 49 months (8–229). 5 had infected non-union. Resection and massive endoprosthetic reconstruction involved the distal femur in 4 patients, proximal femur 3, distal humerus 2 and total Humerus in 1 patient. Time from diagnosis of non-union to treatment was 0 to 96 months (median 11 months) and patients had had 0 to 6 (median 3) previous operations 5 infected non-unions were operated as 2 stage procedures and received long term antibiotics. 4 out of 5 infected non-unions were salvaged. There were 5 complications, namely periprosthetic fracture, infection, a dislocated shoulder, radial nerve palsy, suture of bosing. All the patients achieved immediate mobility and stability. Extendible prosthesis allowed partial correction of limb shortening.
Pathological fracture occurs in 5–10% of all primary malignant bone tumours. It is thought that they unfavourably influence survival, because the fracture haema-toma may contaminate adjacent tissues. Management is often more aggressive and one is less inclined to consider limb saving surgery. Aim of this study was to determine whether the presence of pathological fracture had an effect on rate of limb salvage surgery, role of adjuvant treatment and survival. A retrospective study was done on all patients with a pathological fracture through localised Ewing’s sarcoma, treated between 1979 and 2001. Of 289 patients with localised Ewing’s sarcoma, 27 had a pathological fracture. Eighteen presented with fracture, in 9 fracture occurred after biopsy. All were treated with chemotherapy according to protocol. Two fractures were already treated by osteosynthesis elsewhere, the rest healed with conservative treatment. After chemotherapy, 20 patients were treated surgically: 19 with limb saving surgery, 1 with amputation. Apart from chemotherapy, treatment was surgery alone in 15, surgery and radiotherapy in 5, and radiotherapy alone in 7 patients. Indications for radiotherapy were close margins, poor chemotherapy response, or pelvic tumours. Surgical margins were wide in 16 patients, marginal in 2, and intralesional in 1 patient. Local recurrence occurred in 2 patients, primarily treated with chemotherapy and radiotherapy alone. Five year survival was 60%, metastasis free survival 59%, both comparable with rates reported in literature.
The median survival for patients without metastases at the time of LR was 3 years with a 31% survival at 10 years. The most important prognostic factor in this group was grade with low grade tumours having a much better outlook (70% survival at 10 years) than intermediate or high grade tumours (24% at 10 years). Complete control of the first local recurrence could not be shown to be a prognostic factor.
Metachronous multifocal osteosarcoma (MFOS) is a rare form of osteosarcoma manifested by one or more new tumors developing after the initial treatment of primary osteosarcoma. We present a 61 year old Asian male who was referred with 10 week history of pain and discomfort around his left shoulder and low back pain. In 1968, he received radiotherapy followed by disarticulation of left hip for an osteosarcoma of lower end of left femur complicated with pathological fracture. He did not show any clinical features suggestive of recurrence of disease since then until he presented again in February 2005. CT scan of left scapula has revealed extensive osteosarcoma of lower part of scapula, involving subscapularis and infraspinatus muscles. An MRI scan of whole spine has shown evidence of multiple lesions in sacrum, ilium and acetabulum. A core biopsy from scapula has confirmed the histological diagnosis as high grade osteoblastic osteosarcoma. Slides from 1968 were reviewed in the context of recent recurrence and were consistent with features of intermediate grade fibroblastic osteosarcoma. He is currently undergoing chemotherapy prior to planning definitive treatment as there is evidence in the literature that MFOS is a potentially curable condition.
The accuracy was 96.9%. CT overestimated metastases in 4%.
Limb preserving surgery following segmental resection of the distal end of the radius and its articular surface presents a major challenge. We have studied 11 consecutive patients with aggressive tumours located in the distal radius that required segmental resection of the distal radius and its articular surface to evaluate the clinical and functional outcome of reconstruction of such defects. The mean age at the time of diagnosis was 33 years (7–60). Follow up ranged from 12 to 306 months (median 56). Histological diagnosis was osteosarcoma in 4 patients, chondrosarcoma in 2, giant cell tumour in 5 and meta-static carcinoma in 1 patient. Four patients received chemotherapy. The length of excised bone ranged from 6 to 14cm. Reconstruction was performed with non-vascularised proximal fibula strut graft in 6 patients, ulna transposition in 3 and custom made endoprosthesis in 2 patients. The wrist joint was arthrodesed in 5 patients. At the time of review 2 patients had died of disease, one was alive with disease and 8 were alive and free of disease. Non-union of the graft occurred in one patient, reflex sympathetic dystrophy in 2 and prosthetic dislocation in one. One patient had local recurrence. Four patients required further surgery including one patient who needed an amputation for severe reflex sympathetic dystrophy, one graft revision for non-union, one secondary wrist arthrodesis and one closed reduction of dislocated endoprosthesis. Patients without arthrodesis often had clinical and radiological signs of wrist instability. The majority of the patients achieved satisfactory function with little or no discomfort and ability to perform activities of daily living. We conclude that limb salvage surgery is worthwhile in patients with resectable tumours of the distal radius.
Myxoid liposarcoma (MLS) is an unusual type of soft tissue sarcoma as it tends to metastasize frequently to sites other than the lungs. This study was aimed to investigate the natural history of patients with MLS to try and identify prognostic factors which could help predict outcome and aid earlier detection of metastases. Data was prospectively collected from patient notes and analysed retrospectively. Prognostic factors and metastatic pattern were examined using Kaplan-Meier curves. There were 124 patients with MLS, aged between 28 and 93, the median size of the tumours was 12cm and the most common site was the thigh. Following treatment with excision and radiotherapy the 5yr survival was 65%. Survival was related to younger age (p=0.010) and proximal site (p=0.003) and was also related to the % round cell component of the tumour but was not related to either size or depth of the tumour. Site and margins of excision were significant prognostic factors for local recurrence of disease. 32% of patients developed metastases, of whom 18 cases (46.2%) developed pulmonary metastases and 21 (53.8%) developed extra pulmonary metastases. The sites of these varied hugely and was not significantly related to the site or size of the primary tumour. There was no difference in time to develop metastases or in overall survival between the two groups. Median survival following metastases was 24 months. Although MLS has an unusual pattern of metastases the site of metastases does not predict a better or worse outcome. Intensive follow up for extraskeletal metastases is probably not justified until they become symptomatic.
30 patients were studied with a mean follow-up of 32 months. The mean age at diagnosis was 34yrs (range 19–79). The male to female ratio was 1:1. The most common location was the ilium (19 patients), with the pubis and ischium accounting for a third of patients. Only 1 patient had an acetabular osteochondroma. Median duration of symptoms prior to referral was 6 months (1–79). Pain without a lump was the main presenting symptom (16 patients), followed by lump with pain (6), and lump alone (6). Two patients presented with obstructive labour requiring emergency procedures. The lesions were solitary in 24 and associated with hereditary multiple exostosis (HME) in 6 patients. 1 patient had a radiation induced lesion. The lesions showed increased uptake on bone scans and the cartilage cap was less than 10mm in all but 2 patients. Treatment was surgical excision in 21 patients and observation with serial radiographs in 9. Histological examination confirmed osteochondroma in all patients, however 1 patient with HME had areas of Grade I malignancy. Significant surgical complications occurred in 1 patient who developed pulmonary embolism.
Large benign lytic lesions of the proximal femur present a significant risk of pathological fractures. We report our experience of treating 9 consecutive patients with such defects treated with curettage and fibula strut grafting without supplementary osteosynthesis to evaluate the outcome of this type of reconstruction.. The mean age at the time of diagnosis was 13 years (8–21). Follow up ranged from 2 to 215 months (median 15). Histological diagnosis was fibrous dysplasia in 10 patients and unicameral cyst in 2. All the patients were at risk of pathological fracture. None of the patients developed pathological fracture after surgery and the lesions consolidated fully within one year. Local recurrence occurred in one patient (8%). Minor donor site complications occurred four patients. All the patients were able to fully weight usually within 3 months of surgery. At the time of review all but one patient were completely asymptomatic and fully weight bearing. The only symptomatic patient was the patient with local recurrence which has recently been treated. We conclude that fibula strut graft is a good method of reconstruction of cystic defects in the proximal femut. It prevents pathological fracture, allows mechanical reinforcement of the lesion and delivers biological tissue allowing early consolidation of the defect.
We reviewed our experience with diaphyseal endoprostheses to determine the survival of this type of reconstruction and factors affecting that survival.
The weights of evidence (WE) are logs of the likelihood ratios and can be added and a probability then calculated. e.g. a 36 yr old with a 10cm, deep, painless lump that is increasing in size scores −0.39 + 0.4 + 0.4 – 0.11 + 0.58 = 0.88. This equates to a risk of the lump being malignant of 70%.
The histological findings from the heads of femur or bone biopsy taken from 90 patients with suspected pathological fractures of the femoral neck were studied to determine the rates of significant abnormal pathological findings.The mean age at the time of fracture was 80.41 years (44–99). 29 patients were males and 71 females. The patients were divided into four groups. Group I: 34 patients with fracture without history of fall or trauma. Group II: 21 patients with suspicious radiology of pelvis. Group III: 27 patients with past history of malignancy without known bone metastases. Group IV: 8 patients with past history of malignancy and known bone metastases None of the patients in groups I and II had significant abnormalities other than osteoporosis. 4 patients (15%) in group III had metastases and 6 patients (75%) in group IV had metastases on histological examination. We conclude that the absence of history of fall or trauma or subtle radiographic findings in patients with fracture of the neck of the femur is usually not associated with sinister pathology and the cause of fracture in these patients is often osteoporosis. Patients with previous history of malignancy without known bone metastases have a 15% risk of finding of metastatic disease even in the absence of radiological abnormalities. Patients with fractured neck of femur with past history of malignancy and who are known to have bone metastases must be considered as having pathological fractures through metastatic disease until unless proven otherwise
The generally unfavourable prognosis and perceived risks have led surgeons to palliate, stabilise in situ or amputate for distal femoral metastases despite recognised morbidity and life style restrictions. We conclude that DF EPR should be considered as a limb salvage option in patients with distal femoral mets.