Preoperative screening of patients included standard X rays, CT and bone technetium scan in all cases and MRI in 15 cases. Diagnosis was made by open biopsy except for 4 cases of chondrosarcoma for these preoperative screening was sufficient (and diagnosis confirmed by postoperative histological examination). Following limb salvage using reconstruction of pelvis was performed with methyl metacrylate without prosthesis Titanium screws were inserted in remaining bone before moulding of acrylic cement (2 to 3 packs of antibiotic loaded cement).
Desmoid tumour is an histological benign tumour. Nevertheless, peri-scapular relapses can decrease the function and intra thoracic progression threaten life. To prevent these complications, damaging treatment (radiotherapy, amputation) are sometimes proposed. To precise the optimal indications of treatment, we reviewed our cases.
Treatment was adapted to each patient, in function of age, history of illness, and risks of spontaneous evolution. En bloc extratumoral resection was performed each time, when it didn’t expose to heavy functional risk (8). The other patients were treated by contaminated resection, but never invaliding. 4 patients received pre or/and post-operative chemotherapy. 1 received Interferon alpha, and 7 tamoxifen.
Congenital fibrosarcoma (CFS) is a rare tumor most often affecting extremities of babies. Considering age, surgery of primary is preferred. Nevertheless amputation rate remains high. Preoperative chemotherapy (CT) role must be emphasised. We present 3 cases receiving preoperative CT. Patients and methods in 1985, we treated a 3 months old girl for CFS of the thigh. To avoid amputation, preoperative CT (3 Ifosfamide- Vincristine- Actinomycine D) was performed leading to complete radiological and histological response. She benefited of conservative surgery She is in first complete remission 23 years later. In September 1999, a 3 ½ y old boy with recurrent l buttock CFS operated elsewhere twice (6 months old, 2 years old), received preoperative chemotherapy with good clinical and radiological response. “En-bloc” extra tumoral resection was performed. Histology showed viable tumoral cells. We completed treatment by chemotherapy. In 01/ 2003 bilateral pulmonary metastases occurred leading to surgery and chemotherapy. In 09/ 2003 a new local recurrence appeared treated by surgery and postoperative chemotherapy. From this time, he received Alpha interferon. He is in complete remission for 6 years. In 12/2005, a 14 y old girl, with local recurrence of CFS, treated elsewhere at the age of 5 months by partial surgery and chemotherapy (remained in remission for 13 years)was admitted. Since this time, she recurred locally despite resections and multiple lines of chemotherapy, but without metastasising. She was amputated in 2008.
The stiffness of the shoulder can result of many illness. Nevertheless, we observed a severe stiffness of the scapulo thoracic space only in fibromatosis. To precise the real diagnostic value of this symptom, we examined patients with different diseases of shoulder (tumoral and non tumoral). The passive mobility of the shoulder of 11 patients with peri-scapular fibromatosis was compared to the mobility of those in 50 patients with non tumoral diseases of shoulder (arthritis and rotator cuff pathology), 50 peri-scapular soft tissues tumours, and 100 patients with primitive or secondary malignancies of humerus or scapula.
After treatment of fibromatosis, 9/11 patients are in complete remission and the mobility of their scapulo thoracic space restored. 2 patients are in stable disease and one suffers of a residual stiffness of the scapulothoracic space. We conclude that the frozen scapulo thoracic is a specific symptom of peri scapulo thoracic fibromatosis. The restoration of the mobility of the scapulo thoracic after cure of the desmoid tumour confirms its specific role and represents a good marker of the tumoral evolution.
The purpose of this study was to investigate the importance of the timing of surgery for disease-free survival (DFS). The increasing efficacy of neo-adjuvant chemotherapy in Ewing’s sarcoma modifies the prognostic factors. In a recent monocentric study the classical prognostic value of size and location of the primary disappeared ( Seventy-five patients with an average age of 19 years (range 4 to 40) years with Ewing’s sarcoma of bone fulfilled the inclusion criteria for this study: localised tumour at first screening (CT of lungs + bone scan) and location of the tumour in resectional bones (limb, scapula, innominate, rib, maxilla, skull). Metastatic patients and vertebral locations were excluded. The patients received multi-drug chemotherapy and were treated by surgery and radiotherapy in cases of bad responders and/or marginal surgery. The histologic response was evaluated according to Picci’s criteria ( After a mean follow-up of 54 months, 41 patients were in first complete remission. Patients operated before the tenth week had a higher chance (68%) of first complete remission than patients operated later (DFS: 43%). The difference is significant (p<
0.03). Further analysis shows that the difference is due to late local control, which causes a dismal prognosis for bad responders. Local treatment must be performed early, especially when histologic response is incomplete or uncertain. Preoperative chemotherapy that is too long increases the risk of metastases in bad responders. These factors must be taken into account when analysing multicentre protocols.
Nowadays 80% of patients with bone sarcomas can benefit from limb salvage. Their disease-free life expectancy is not jeopardised by conservative surgery as long as safe margins are obtained. For this reason, the oncological result relies on the accuracy of pre-operative and per-operative surgical measurements. Pre-operative evaluation of tumours is now quite accurate with digital margins (computed tomography, MNR, digital angiography). However, surgeons are still using centimeters or conventional radiographs with their own technical limitations for per-operative evaluation. A more accurate technique is needed. The system is composed of three components: 1) a color, graphic computer workstation with software to calculate and present the location of the surgical instrument on a three-dimensional, reconstructed bone image, 2) a complete set of hand-held instruments containing infrared emitters, 3) an infrared receiver linked to the work station. This measuring system enables determination of the position and incidence of a surgical instrument in real time during surgery, with an accuracy of less than one mm. The system requires four steps: 1) recording data with C.T., N.M.R. or angiography, 2) creating a three-dimensional image displayed on the computer screen for preoperative simulation of a virtual operation, 3) recording the very important anatomical points of the patient and optimal incidences of the surgical instruments, 4) preoperative location of surgical instruments and control of their location on bone. This system is very useful for resection of bone tumours when the conventional location is uncertain (innonimate bone, rib), when very sharp accuracy is needed to preserve the growth plate of the distal femur in young children, and to avoid medullary damage in a spinal tumour. The frameless stereotactic device is also very accurate in the reconstructive phase of limb salvage. After an internal hemipelvectomy, the device permits localisation of the acetabular prosthesis in the precise location before resection. In our practice, the accuracy of the video guiding system is always within two mm as compared to conventional measurements usually between one or two cm for long bones and three to five cm for innominate bone. The use of a video guidance system is very beneficial for limb salvage surgery for pelvic bone tumours.
A “hands-on” composite gives a similar functional result as a custom-made prosthesis and has a much better function than alternative techniques. Less expensive and more flexible than custom-made prostheses, it can be used even when no part of the iliac wing remains. The use of cement permits the adjunction of antibiotics needed for these complicated cases. After peri-acetabular resection for bone sarcoma, a reconstructive procedure is necessary to stabilize the hip, avoid limb discrepancy, and permit full weight bearing. This procedure needs to be easy to perform because resection of the area is time and blood consuming. This leads to the use of a “hands-on” composite prosthesis. Our reconstructive procedure uses a titanium cup with a long screw that is fixed in the remaining bone (sacrum or spine). When the cup is firmly fixed to the bone, the gap between the cup and bone is filled with cement loaded with antibiotics, and the polyethylene component is cemented on the innominate prosthesis. The femoral component of a usual hip total prosthesis is then implanted. Since 1990 we have used this reconstructive procedure in 50 patients, 27 with bone sarcomas involving the acetabulum (11 chondrosarcomas, 9 Ewing’s sarcomas and 7 other sarcomas) and 23 for metastatic disease. Thirty of these patients were already metastatic when operated. The average duration of the reconstructive procedure was 45 minutes. Walking started from the fourth to tenth day after operation, but full weight bearing was usually authorised after six weeks. Postoperative complications were frequent. Seven deep infections occurred, four required ablation of the prosthesis, and one would benefit from a saddle prosthesis. 33% of the patients had postoperative dislocation of the hip prosthesis and 13 patients had to be reoperated. Only two loosenings have been observed – one after deep infection and one after local recurrence in the sacral bone. Oncologic results: With a mean follow-up of five years, 28 patients died of disease and one from an unrelated disease. Four others with disease are still living. Seven local recurrences were observed (four in chondrosarcomas with a contaminated resection). The difficulty in obtaining wide margins explains the high rate of local recurrence (14 %). For patients with localised disease, the five-year overall survival rate is 75% and the five-year disease-free survival rate is 60%. According to the Society for Musculoskeletal Oncology criteria, orthopaedic results were excellent in 7 patients, good in 30, fair in 6, and bad in 6. The mean functional score of 46 patients who still have their prostheses is 83% with usually no pain, excellent acceptance, length discrepancy of less than 1 cm, average flexion of 100 degrees, and unlimited walking without support. We conclude that the rapidity and flexibility of this procedure are the positive aspects of this reconstructive technique. However, perfect positioning of the prosthesis remains difficult in a very large peri-acetabular resection. A computed guide is of great help to specify safe margins and prosthesis positioning. Longer follow-up is needed to ensure that the rate of late loosening will not be too high.
Nowadays 80% of patients with bone sarcomas can benefit from limb salvage. Their disease-free life expectancy is not jeopardised by conservative surgery as long as safe margins are obtained. For this reason, the oncological result relies on the accuracy of pre-operative and per-operative surgical measurements. Pre-operative evaluation of tumours is now quite accurate with digital margins (computed tomography, MNR, digital angiography). However, surgeons are still using centimeters or conventional radiographs with their own technical limitations for per-operative evaluation. A more accurate technique is needed. The system is composed of three components: 1) a color, graphic computer workstation with software to calculate and present the location of the surgical instrument on a three-dimensional, reconstructed bone image, 2) a complete set of hand-held instruments containing infrared emitters, 3) an infrared receiver linked to the work station. This measuring system enables determination of the position and incidence of a surgical instrument in real time during surgery, with an accuracy of less than one mm. The system requires four steps: 1) recording data with C.T., N.M.R. or angiography, 2) creating a three-dimensional image displayed on the computer screen for preoperative simulation of a virtual operation, 3) recording the very important anatomical points of the patient and optimal incidences of the surgical instruments, 4) preoperative location of surgical instruments and control of their location on bone. This system is very useful for resection of bone tumours when the conventional location is uncertain (innonimate bone, rib), when very sharp accuracy is needed to preserve the growth plate of the distal femur in young children, and to avoid medullary damage in a spinal tumour. The frameless stereotactic device is also very accurate in the reconstructive phase of limb salvage. After an internal hemipelvectomy, the device permits localisation of the acetabular prosthesis in the precise location before resection. In our practice, the accuracy of the video guiding system is always within two mm as compared to conventional measurements usually between one or two cm for long bones and three to five cm for innominate bone. The use of a video guidance system is very beneficial for limb salvage surgery for pelvic bone tumours.
The purpose of this study was to investigate the importance of the timing of surgery for disease-free survival (DFS). The increasing efficacy of neo-adjuvant chemotherapy in Ewing’s sarcoma modifies the prognostic factors. In a recent monocentric study the classical prognostic value of size and location of the primary disappeared ( Seventy-five patients with an average age of 19 years (range 4 to 40) years with Ewing’s sarcoma of bone fulfilled the inclusion criteria for this study: localised tumour at first screening (CT of lungs + bone scan) and location of the tumour in resectional bones (limb, scapula, innominate, rib, maxilla, skull). Metastatic patients and vertebral locations were excluded. The patients received multi-drug chemotherapy and were treated by surgery and radiotherapy in cases of bad responders and/or marginal surgery. The histologic response was evaluated according to Picci’s criteria ( After a mean follow-up of 54 months, 41 patients were in first complete remission. Patients operated before the tenth week had a higher chance (68%) of first complete remission than patients operated later (DFS: 43%). The difference is significant (p<
0.03). Further analysis shows that the difference is due to late local control, which causes a dismal prognosis for bad responders. Local treatment must be performed early, especially when histologic response is incomplete or uncertain. Preoperative chemotherapy that is too long increases the risk of metastases in bad responders. These factors must be taken into account when analysing multicentre protocols.
A “hands-on” composite gives a similar functional result as a custom-made prosthesis and has a much better function than alternative techniques. Less expensive and more flexible than custom-made prostheses, it can be used even when no part of the iliac wing remains. The use of cement permits the adjunction of antibiotics needed for these complicated cases. After periacetabular resection for bone sarcoma, a reconstructive procedure is necessary to stabilize the hip, avoid limb discrepancy, and permit full weight bearing. This procedure needs to be easy to perform because resection of the area is time and blood consuming. This leads to the use of a “hands-on” composite prosthesis. Our reconstructive procedure uses a titanium cup with a long screw that is fixed in the remaining bone (sacrum or spine). When the cup is firmly fixed to the bone, the gap between the cup and bone is filled with cement loaded with antibiotics, and the polyethylene component is cemented on the innominate prosthesis. The femoral component of a usual hip total prosthesis is then implanted. Since 1990 we have used this reconstructive procedure in 50 patients, 27 with bone sarcomas involving the acetabulum (11 chondrosarcomas, 9 Ewing’s sarcomas and 7 other sarcomas) and 23 for metastatic disease. Thirty of these patients were already metastatic when operated. The average duration of the reconstructive procedure was 45 minutes. Walking started from the fourth to tenth day after operation, but full weight bearing was usually authorised after six weeks. Postoperative complications were frequent. Seven deep infections occurred, four required ablation of the prosthesis, and one would benefit from a saddle prosthesis. 33% of the patients had postoperative dislocation of the hip prosthesis and 13 patients had to be reoperated. Only two loosenings have been observed – one after deep infection and one after local recurrence in the sacral bone. Oncologic results: With a mean follow-up of five years, 28 patients died of disease and one from an unrelated disease. Four others with disease are still living. Seven local recurrences were observed (four in chondrosarcomas with a contaminated resection). The difficulty in obtaining wide margins explains the high rate of local recurrence (14 %). For patients with localised disease, the five-year overall survival rate is 75% and the five-year disease-free survival rate is 60%. According to the Society for Musculoskeletal Oncology criteria, orthopaedic results were excellent in 7 patients, good in 30, fair in 6, and bad in 6. The mean functional score of 46 patients who still have their prostheses is 83% with usually no pain, excellent acceptance, length discrepancy of less than 1 cm, average flexion of 100 degrees, and unlimited walking without support. We conclude that the rapidity and flexibility of this procedure are the positive aspects of this reconstructive technique. However, perfect positioning of the prosthesis remains difficult in a very large periacetabular resection. A computed guide is of great help to specify safe margins and prosthesis positioning. Longer follow-up is needed to ensure that the rate of late loosening will not be too high.