Synovial sarcoma of the extremities and trunk: a long-lasting disease.

Synovial sarcoma (SS) of an extremity or trunk is relatively rare and is approached by limb sparing surgery (LSS), radiation therapy (RT) and chemotherapy. We conducted a retrospective analysis of the clinical and histopathological data of 73 patients with proven SS. At a median follow-up time of 6 years, local recurrence was seen in 17.8 and systemic recurrence 35.6% of patients (local-only, 6.8; systemic-only, 24.6; combined, 11%). The 10-year local recurrence-free survival (LRFS), systemic recurrence-free survival (SRFS) and overall survival (OS) rates were 78, 68 and 61%, respectively. LRFS was significantly better in patients treated with isolated limb perfusion (ILP); SRFS was influenced by the delay until diagnosis. The practical aspects of our observations are the need for long-term follow-up in order to diagnose recurrences, the fact that not all local or distant recurrences are necessarily associated with a shortening of OS time and the important role of induction ILP with TNF in cases of extremity SS.

Liposarcoma in adult limbs treated by limb-sparing surgery and adjuvant radiotherapy.

Between December 1995 and March 2003, 38 adult patients with intermediate or high-grade liposarcoma in a limb were treated by limb-sparing surgery and post-operative radiotherapy. The ten-year local recurrence-free survival was 83%, the ten-year metastasis-free survival 61%, the ten-year disease-free survival 51% and the ten-year overall survival 67%. Analysis of failure and success showed no association with the age of the patients, gender, the location of the primary tumour, the type of liposarcoma and the quality of resection. Our results indicate that liposarcoma may recur even ten years after the end of definitive therapy and may spread to unexpected sites as for soft-tissue sarcoma.

The radiological evaluation of the hip joint after prosthetic arthroplasty of the proximal femur in patients with a tumour using a bipolar femoral head.

Excision of the proximal femur for tumour with prosthetic reconstruction using a bipolar femoral head places a considerable load on the unreplaced acetabulum. We retrospectively reviewed the changes which occur around the affected hip joint by evaluating the post-operative radiographs of 65 consecutive patients who underwent proximal prosthetic arthroplasty of the femur, and in whom an acetabular component had not been used. There were 37 men and 28 women with a mean age of 57.3 years (17 to 93). Radiological assessment included the extent of degenerative change in the acetabulum, heterotopic ossification, and protrusio acetabuli. The mean follow-up was 9.1 years (2 to 11.8). Degenerative changes in the acetabulum were seen in three patients (4.6%), Brooker grade 1 or 2 heterotopic ossification in 17 (26%) and protrusion of the prosthetic head in nine (13.8%). A total of eight patients (12.3%) needed a revision. Five were revised to the same type of prosthesis and three (4.6%) were converted to a total hip arthroplasty. We conclude that radiological evidence of degenerative change, heterotopic ossification and protrusion occur in a few patients who undergo prosthetic arthroplasty of the proximal femur for tumour. The limited extent of these changes and the lack of associated symptoms do not justify the routine arthroplasty of the acetabulum in these patients.

ErbB-4 expression in limb soft-tissue sarcoma: correlation with the results of neoadjuvant chemotherapy.

ErbB-4 is a recently described growth factor receptor. Relatively little is known about its expression in human tumours. In this study, we assessed the possible role of erbB-4 as a tissue marker for soft-tissue sarcomas (STS) and its correlation with the response to chemotherapy. The histological specimen of 29 patients with STS of a limb who had received preoperative doxorubicin (ADR)-based chemotherapy were studied for the degree of necrosis and the expression of erbB-4 (by an avidin-biotin-peroxidase technique). ErbB-4 expression in the preoperative tissue samples was compared with the expression in the postchemotherapy resected tumour. The true objective response rate to preoperative chemotherapy was 34%. Wide resection of the tumour was done in 12 patients, marginal in 14, amputation in 2 and no surgery in 1. The tumour necrosis was above 90% in 9 patients, 60-90% in 12, and less than 60% in 7 patients. An increase in erbB-4 expression was more common in cases with no response to chemotherapy, while no change or a decrease in erbB-4 was more common in responsive tumours (P=0.004). No correlation could be found between the degree of necrosis or the chemotherapeutic regimen and the change in expression of erbB-4. The median disease-free survival (DFS) was longer for patients with a decrease or no change in expression of erbB-4 than for patients with increased expression. It is believed that postchemotherapy new expression or no downregulation of the erbB-4 molecule represents tumour aggressiveness and increased capability of growth and spread.

Amputation of the lower limb as palliative treatment for debilitating musculoskeletal cancer.

Limb sparing surgery has replaced amputation surgery for treating sarcomas of the lower limb in most cases. Wide resection followed by postoperative radiation therapy can achieve acceptable local control and survival rates in patients with bone and soft-tissue sarcomas of the lower limb. Recurrent or persistent disease constitutes a major oncological problem. Local symptoms such as agonizing pain, fractures, tumor fungation, inability to walk and inability to maintain daily activities, further impair the patient's quality of life. In this clinical set-up palliative amputation of the limb should be considered. Fourteen patients with soft-tissue or bone sarcomas underwent palliative major amputation. The procedures included: hemipelvectomy, hip disarticulation, knee disarticulation, above or below-knee amputation. Local control of the disease and pain, and improvement of the performance status were observed in 13 evaluable patients. The mobility was restored in 13/14 patients. The median survival following the procedure was 9 months. There was only one case of immediate post-operative death. Severe phantom pain was not reported by any of the patients. Quality of life was reported to be improved by two-thirds of the patients. We found palliative major amputation surgery worth-performing in low-performance status cancer patients with locally advanced disease of the lower limb.

Internal hemipelvectomy for bone sarcomas in children and young adults: surgical considerations.

AIMS: Pelvic bone sarcomas in children and young adults are rare, and associated with a poor prognosis and a high rate of local recurrence. Primary goals of treatment include prevention of local recurrence and distant metastases. A secondary goal is maintenance of quality of life by avoiding major amputative surgery. This is why internal hemipelvectomy (a limb-sparing surgery) is advocated whenever possible. The focus of our presentation is surgical issues in the context of resection and reconstruction of the pelvis in the first two decades of life. MATERIALS AND METHODS: Between January 1988 and June 1998, 27 patients were treated and operated on (follow-up time 1.5-12 years). There were 17 males and 10 females. Their age ranged between 2 and 22 years. There were 24 patients with Ewing's sarcoma (ES) and three with other bone sarcomas. In 19 patients the tumour involved the entire or part of the iliac bone (in some cases with extension to the sacrum). In five patients the tumour involved the pubis and/or ischium. In three patients the tumour involved the sacrum with some extension to the posterior iliac bone. All patients received neoadjuvant and adjuvant chemotherapy and radiotherapy with different protocols (related to the origin of referral). RESULTS: Twenty-seven patients underwent internal hemipelvectomy. According to Enneking's classification there were: type I-10; type II-one; type III-six; type IV-five (including one localized sacrectomy); type I+IV-five patients. In 15 patients some kind of reconstruction was needed and in 12 no reconstruction was done. Four wound infections occurred that were managed successfully by surgical debridement, antibiotics and local wound care. In one case removal of the 'implant' was needed. No primary or secondary amputations were performed in the series. The rate of local recurrence was 22%. Functional status at the last follow-up visit or before death, according to the AMSTS functional rating system: excellent-six; good-17; fair-three and poor-one. All patients except the one poor result maintained their walking ability during the follow-up time. CONCLUSIONS: Internal hemipelvectomy is achievable in most cases and justified for better quality of life in children, adolescents and young adults with sarcomas. Further efforts are needed to improve the reconstructive options in the pelvis.

Isolated limb perfusion with tumour necrosis factor-alpha and melphalan for unresectable bone sarcomas of the lower extremity.

AIMS: Isolated limb perfusion (ILP) with recombinant tumour necrosis factor-alpha (rTNF-alpha) and melphalan has recently been reported to induce major tumour responses and permit limb salvage in over 80% of patients with unresectable soft-tissue sarcomas of the extremities. We investigated whether TNF-based ILP could allow limb-sparing surgery in patients with primary, recurrent or metastatic bone sarcoma to the lower extremity who met the criteria for an amputation and had failed or refused chemotherapy. METHODS: From August 1992 to December 1997, we employed ILP with rTNF-alpha and melphalan in 13 patients with unresectable bone sarcoma of the lower extremity, all of whom were candidates for amputation. The aim was to reduce tumour size and allow the performance of a limb-sparing surgery (LSS). RESULTS: Following ILP, none of the patients had severe local toxicity and only one patient experienced significant systemic side-effects. LSS was subsequently performed in nine of the 13 patients. LSS was feasible in an additional three patients but was not performed because of the emergence of diffused metastatic disease. CONCLUSIONS: ILP with rTNF-alpha and melphalan can allow limb salvage in patients wih locally advanced bone sarcomas who had failed standard treatment options. Its potential role in the treatment of unresectable bone sarcomas of the extremities merits further evaluation.

Extraspinal bone and soft-tissue tumors as a cause of sciatica. Clinical diagnosis and recommendations: analysis of 32 cases.

STUDY DESIGN: Between 1982 and 1997, the authors treated 32 patients with sciatica who subsequently were found to have a tumor along the extraspinal course of the sciatic nerve. SUMMARY OF BACKGROUND DATA: Extraspinal compression of the sciatic nerve by a tumor is a rare cause of sciatica. Signs and symptoms overlap those of the more common causes of sciatica (i.e., herniated disc and spinal stenosis). OBJECTIVE: To characterize the unique clinical presentation of these patients and to formulate guidelines that may lead to early diagnosis. METHODS: All pertinent clinical data and studies were reviewed retrospectively, and standard demographic data were collected for analysis. RESULTS: These patients typically sought treatment for an insidious onset of sciatic pain that was constant, progressive, and unresponsive to change in position or bed rest. The mean time to final diagnosis was 11.9 months (median, 6 months). Seventeen patients were able to locate their pain to a specific point along the extraspinal course of the sciatic pain, and a mass was noted in 13 patients. Eighteen of these tumors were in the pelvis, 10 in the thigh, and 4 in the popliteal fossa and calf. CONCLUSIONS: A high index of clinical suspicion is the key to early diagnosis of bone or soft-tissue tumors as a cause of sciatica; special attention should be given to pain pattern, physical examination of the entire course of the sciatic nerve, and selection of proper imaging studies. Routine anteroposterior plain radiography of the pelvis as part of the initial imaging screening process is recommended.

Closed argon-based cryoablation of bone tumours.

We report our experience with a new technique for cryosurgical ablation of bone tumours which allows accurate determination of the temperature and freezing time within a cavity of any geometrical shape. Between 1997 and 2000, 58 patients diagnosed with 13 malignant and 45 aggressive benign bone tumours underwent argon-based cryoablation. This technique includes removal of the tumour by curettage and filling the cavity with a gel medium into which metal probes are inserted. Argon gas is delivered through the metal probes and both time and temperature are computer-controlled. After formal reconstruction, all patients were followed for more than two years. None had skin necrosis, infection, neurapraxia or thromboembolic complication. Fractures occurred in two patients (3.4%) and the tumour recurred in two patients (3.4%).

The use of three-dimensional computed tomography in evaluating snapping scapula syndrome.

This article evaluates imaging of the scapula and scapulothoracic joint in patients with snapping scapula syndrome. Between 1990 and 1996, a total of 20 patients (10 men and 10 women) with snapping scapula syndrome were evaluated. Diagnosis was based on patient complaints and physical examination findings. There were 26 affected scapulae (6 patients had bilateral presentation). Imaging of the scapula included plain radiography, computed tomography (CT), and 3-dimensional computed tomography (3-D CT) reconstruction. Plain radiography revealed bony incongruity between the anterior aspect of the scapula and the chest wall in 7 scapulae, CT revealed such incongruity in 19 scapulae, and 3-D CT revealed incongruity in all 26 scapulae. Treatment was conservative, consisting of nonsteroidal anti-inflammatory drugs, a physiotherapy program, and subscapular injection of a local anesthetic and steroids. In 5 patients who responded poorly to conservative treatment, the region responsible for the snapping was resected. Pain relief and resolution of the snapping were complete following surgery in 4 patients, while pain and crepitation persisted in the fifth. Three-dimensional CT is recommended as the main imaging modality in the evaluation of any patient with snapping scapula syndrome who is a candidate for surgical intervention.

Is it important to maintain high-dose intensity chemotherapy in the treatment of adults with osteosarcoma?

Neoadjuvant chemotherapy for osteosarcoma is the standard of care, but there is still confusion regarding the best chemotherapy regimen and the optimal intensity. This retrospective study intends to evaluate whether there is a clear correlation between the chemotherapy dose intensity (DI) and the percentage of tumor necrosis, the risk of tumor recurrence after surgery and patient survival. The medical records of all adult patients with localized osteosarcoma that received treatment between the years of 1998 and 2009 at the Tel Aviv Sourasky Medical Center were analyzed. We used multiple logistic/linear regression models to test the effect of the neoadjuvant chemotherapy relative DI (RDI) on histological response, recurrence and time to recurrence. A Cox regression analysis was conducted for the effects of neoadjuvant chemotherapy RDI, histological response, tumor location, gender and age on patient survival. Thirty medical records were analyzed. Survival, histological response, recurrence and time to recurrence were not affected by the chemotherapy RDI. The 5-year overall survival of the patient's population was found to be 63% with a median survival of 9.4 years. Patients with a good histological response had a longer survival than those with a bad response (mean survival times 11.0 vs. 6.6 years, log-rank test, P = 0.046). High DI is not a prognostic factor in osteosarcoma and maintaining it should not be a prime priority. Histological response is a prognostic but possibly not a reliable predictive factor, and further research is needed in order to find other reliable factors.

The role of the fibula head flap for joint reconstruction after osteoarticular resections.

INTRODUCTION AND AIM: Endoprosthetic reconstruction is considered the mainstay of limb salvage in periarticular bone tumours. However, this procedure has limited durability especially when performed in young patients. The free fibula head flap including the proximal articular surface represents one option for hemiarthroplasty reconstruction. The aim of this study was to investigate the role of the fibula head flap for joint reconstruction after osteoarticular resections. PATIENTS AND METHODS: All patients who underwent hemiarthroplasty procedures between 2000 and 2006 using the free fibula head flap were included in the study. Functional assessments were performed using the American Musculoskeletal Tumor Society (AMTS) classification. RESULTS: There were five males and two females (mean age: 22.6 ± 15.9 years). Five patients underwent reconstruction following resection of malignant bone tumours and two for chronic osteomyelitis of the distal humerus. In three patients, the fibula was used for distal radius and wrist joint reconstruction, and the remaining four patients for reconstruction of the distal humerus and elbow joint. A vascularised growth plate transfer based on the lateral geniculate vessels was performed in two patients. Atechnetium-bone scan confirmed viability of all flaps 10 days after surgery, and radiographic bony union was confirmed on average 5 months following surgery. There were no complications with the recipient or donor site after a median follow-up of 71 months (range: 12 months to 10 years). All patients achieved reasonable return of function and were able to perform all activities of daily living. CONCLUSIONS: We demonstrate that the hemiarthroplasty procedure using the free fibula flap with its proximal head is a safe procedure with good functional results. Performing autologous arthroplasty using a free fibula head flap may be a promising alternative to an endoprosthesis or alloplastic reconstruction with a low risk of complications and morbidity.

Hypofractionated adjuvant radiation therapy of soft-tissue sarcoma achieves excellent results in elderly patients.

OBJECTIVE: Adjuvant radiation therapy (RT) is an essential part of combined limb-sparing treatment of soft-tissue sarcoma (STS). Elderly or medically unfit patients often have difficulty in completing 6-7 weeks of standard fractionated daily treatment. Our aim was to evaluate the efficacy of a hypofractionated adjuvant approach with RT for STS in elderly and debilitated patients. METHODS: 21 elderly patients were treated with a short course of adjuvant RT (39-48 Gy, 3 Gy per fraction) for STS. The medical records of the patients were retrospectively reviewed for local or distant recurrence and side effects of RT. RESULTS: At a mean 26 months of follow-up, three local recurrences (14%) were detected. Eight patients (38%) had lung metastases during the observed period. Three of them died from metastatic disease. The hypofractionated radiation was well tolerated with minimum long-term side effects. CONCLUSION: Hypofractionated adjuvant radiation appears to be an effective treatment in terms of local control in elderly and debilitated patients. ADVANCES IN KNOWLEDGE: The results of this study might provide an alternative to commonly used standard fractionation of radiotherapy in sarcoma patients.

Fifteen years of bone tumor cryosurgery: a single-center experience of 440 procedures and long-term follow-up.

BACKGROUND: This summary of a single center's extensive cumulative experience in bone tumor cryosurgery assesses the long-term outcome of bone conservation surgery in which adjuvant cryosurgery plays a pivotal role. MATERIALS AND METHODS: We performed 440 cryosurgical procedures between January 1988 and December 2002. Two-thirds of the series comprised a variety of primary benign-aggressive and low-grade malignant lesions, and one-third were primary high-grade and metastatic bone tumors. The anatomical locations included almost every bone of the skeleton. Two methods of bone cryosurgery were used: Marcove's "open" direct-pour system using liquid nitrogen (1988-1997) and Meller's "closed" argon-based system (1998 to the present). RESULTS: The study cohort consisted of 214 males and 191 females (age range 5-82 years). The median follow-up was 7 years (range 3-18). The overall local recurrence rate was 8%: fractures=1%, infections=2% and skin burns=1.3%. There were three cases of transient nerve palsies in areas other than the sacrum, and four cases of late osteoarthritis of an adjacent joint. The functional outcome for the 372 patients with no evidence of disease was almost 100% "good" and "excellent" (American Musculo-skeletal Tumor Society System). Only two patients needed secondary amputations. CONCLUSIONS: Bone cryosurgery is a safe and effective limb-, joint- and even epiphysis-sparing surgical technique in suitable types of bone tumors, temporarily or permanently obviating the need for resection surgery.

Reconstruction of hip stability after proximal and total femur resections.

Dislocation is the most common complication after proximal and total femur endoprosthetic reconstruction. The current study describes a surgical technique of acetabular preservation and reconstruction of the joint capsule and abductor mechanism that recreates joint stability and avoids dislocation. Between 1980 and 1996, 57 patients underwent proximal or total femur resection with endoprosthetic reconstruction. Forty-six patients had primary sarcoma of bone, nine had other bone tumors, and two had metabolic bone disease. The acetabulum was spared and not resurfaced in all patients. Bipolar hemiarthroplasty was performed in 49 patients, and fixed unipolar hemiarthroplasty was performed in eight. Soft tissue reconstruction included Dacron tape capsulorrhaphy over the prosthetic neck, reattachment of the abductor mechanism to the prosthesis, and extracortical bone fixation. The average followup period was 6.5 years (range, 2-18.2 years). Dislocation occurred in only one (1.7%) patient, and aseptic prosthetic loosening occurred in three (5.3%) patients. Four patients with primary bone sarcoma had local recurrence, of whom one required amputation of the limb. The limb salvage rate was 98%. Eighty-one percent of the patients had a good to excellent functional outcome. Acetabular preservation, capsulorrhaphy, and reconstruction of the abductor mechanism recreate hip stability and avoid dislocation after proximal and total femur endoprosthetic reconstruction.